Treatment of extrahepatic portal hypertension following a whipple procedure with a Rex shunt: report of a case.

PubMed

Reichman, Trevor W; Anthony, Tiffany; Testa, Giuliano

2011-02-01

The Rex shunt is a mesenteric vein to left portal vein decompressive shunt used for the treatment of portal vein thrombosis and portal hypertension. Its use has been reported primarily in the pediatric population where portal vein thrombosis occurs with some frequency. The shunt is thought to represent a more physiologic shunt, since it restores hepatopedal blood flow through the liver. This report describes the use of this shunt in an adult who had frequent gastrointestinal bleeding secondary to extrahepatic portal vein thrombosis, which occurred as a complication after a pancreaticoduodenectomy.

Treatment for hepatitis C virus-induced portal hypertension in leukemic children.

PubMed

El-Ashry, Rasha; Malek, Hala Abdel; Ghayaty, Essam A Desoky; El-Gendy, Ahmed A; Darwish, Ahmad; Al-Tonbary, Youssef

2013-01-01

Children with acute leukemia are at high risk of hepatitis C infection, either by immunosuppression secondary to chemotherapy or by multiple transfusions of blood products during the course of the disease. Hepatitis C virus (HCV) infection constitutes a major problem during management of acute leukemia due to resultant portal hypertension or bleeding esophageal varices. Chronic HCV infection is a major cause of liver cirrhosis and hepatocellular carcinoma in leukemic survivors. The effect of amlodipine treatment on children with acute lymphoblastic leukemia (ALL) having portal hypertension secondary to HCV infection during maintenance chemotherapy has been studied. Sixty male children (mean age 11.83 ± 1.1 years) with ALL in remission and have HCV infection were included. Diagnosis of HCV infection was confirmed by real-time PCR. Thirty patients received 5 mg amlodipine orally per day for 4 weeks and compared to another 30 patients received placebo therapy and 30 age- and sex-matched children as a control group. Amlodipine significantly reduced the elevated portal blood pressure to normal level in doses which did not interfere with mechanism of action of chemotherapy (p ≤ 0.001). Treatment with amlodipine can be used to control portal hypertension in leukemic children having HCV-induced portal hypertension. HCV in leukemics could be virtually eliminated by proper testing of the blood transfusion pool.

[Idiopathic portal hypertension].

PubMed

Orozco, H; Takahashi, T; García-Tsao, G; Mercado, M A; Diliz, H; Hernández-Ortiz, J

1991-01-01

Patients with portal hypertension without a demonstrable cause have been reported in the literature under several different terms, such as tropical splenomegaly, phlebosclerosis, obliterative portal venopathy of the liver, hepatoportal sclerosis, noncirrhotic portal fibrosis and idiopathic portal hypertension (IPH). Such patients have been described worldwide, with a greater frequency in India and Japan. The etiology of IPH is still unknown, although some of the theories that have been proposed are: exposure to toxic substances or drugs, relationship with the hepatitis-B virus, immunologic abnormalities, systemic or intra-abdominal infections and clotting abnormalities. The main histopathologic findings are periportal fibrosis, obliteration of small portal veins and sclerosis of the interhepatic portal system. Although these abnormalities could be secondary to portal hypertension, it has been proposed that the vascular changes are the primary event that leads to portal hypertension. The site of increased resistance in IPH is found at the presinusoidal level with some component at the sinusoidal and postsinusoidal level. The main symptoms and signs in IPH are upper gastrointestinal tract bleeding secondary to esophago-gastric varices, symptoms related to anemia, and splenomegaly. The long-term prognosis for patients with IPH is better than for cirrhotic patients, with a 77% survival at ten years. Variceal bleeding is the main cause of death, and some treatment to prevent bleeding or its recurrence is warranted. Although no comparative trial has been performed in IPH patients, the surgical management could be the first choice for elective treatment in these patient without liver failure, because of the high re-bleeding rates with chronic sclerotherapy. Pharmacologic management could be considered for prophylactic treatment of these patients.

Clinical value of liver and spleen shear wave velocity in predicting the prognosis of patients with portal hypertension

PubMed Central

Zhang, Yan; Mao, Da-Feng; Zhang, Mei-Wu; Fan, Xiao-Xiang

2017-01-01

AIM To explore the relationship of liver and spleen shear wave velocity in patients with liver cirrhosis combined with portal hypertension, and assess the value of liver and spleen shear wave velocity in predicting the prognosis of patients with portal hypertension. METHODS All 67 patients with liver cirrhosis diagnosed as portal hypertension by hepatic venous pressure gradient in our hospital from June 2014 to December 2014 were enrolled into this study. The baseline information of these patients was recorded. Furthermore, 67 patients were followed-up at 20 mo after treatment, and liver and spleen shear wave velocity were measured by acoustic radiation force impulse at the 1st week, 3rd month and 9th month after treatment. Patients with favorable prognosis were assigned into the favorable prognosis group, while patients with unfavorable prognosis were assigned into the unfavorable prognosis group. The variation and difference in liver and spleen shear wave velocity in these two groups were analyzed by repeated measurement analysis of variance. Meanwhile, in order to evaluate the effect of liver and spleen shear wave velocity on the prognosis of patients with portal hypertension, Cox’s proportional hazard regression model analysis was applied. The ability of those factors in predicting the prognosis of patients with portal hypertension was calculated through receiver operating characteristic (ROC) curves. RESULTS The liver and spleen shear wave velocity in the favorable prognosis group revealed a clear decline, while those in the unfavorable prognosis group revealed an increasing tendency at different time points. Furthermore, liver and spleen shear wave velocity was higher in the unfavorable prognosis group, compared with the favorable prognosis group; the differences were statistically significant (P < 0.05). The prognosis of patients with portal hypertension was significantly affected by spleen hardness at the 3rd month after treatment [relative risk (RR) = 3.481]. At the 9th month after treatment, the prognosis was affected by liver hardness (RR = 5.241) and spleen hardness (RR = 7.829). The differences between these two groups were statistically significant (P < 0.05). The ROC analysis revealed that the area under the curve (AUC) of spleen hardness at the 3rd month after treatment was 0.644, while the AUCs of liver and spleen hardness at the 9th month were 0.579 and 0.776, respectively. These might predict the prognosis of patients with portal hypertension. CONCLUSION Spleen hardness at the 3rd month and liver and spleen shear wave velocity at the 9th month may be used to assess the prognosis of patients with portal hypertension. This is hoped to be used as an indicator of predicting the prognosis of patients with portal hypertension. PMID:29259380

Clinical value of liver and spleen shear wave velocity in predicting the prognosis of patients with portal hypertension.

PubMed

Zhang, Yan; Mao, Da-Feng; Zhang, Mei-Wu; Fan, Xiao-Xiang

2017-12-07

To explore the relationship of liver and spleen shear wave velocity in patients with liver cirrhosis combined with portal hypertension, and assess the value of liver and spleen shear wave velocity in predicting the prognosis of patients with portal hypertension. All 67 patients with liver cirrhosis diagnosed as portal hypertension by hepatic venous pressure gradient in our hospital from June 2014 to December 2014 were enrolled into this study. The baseline information of these patients was recorded. Furthermore, 67 patients were followed-up at 20 mo after treatment, and liver and spleen shear wave velocity were measured by acoustic radiation force impulse at the 1 st week, 3 rd month and 9 th month after treatment. Patients with favorable prognosis were assigned into the favorable prognosis group, while patients with unfavorable prognosis were assigned into the unfavorable prognosis group. The variation and difference in liver and spleen shear wave velocity in these two groups were analyzed by repeated measurement analysis of variance. Meanwhile, in order to evaluate the effect of liver and spleen shear wave velocity on the prognosis of patients with portal hypertension, Cox's proportional hazard regression model analysis was applied. The ability of those factors in predicting the prognosis of patients with portal hypertension was calculated through receiver operating characteristic (ROC) curves. The liver and spleen shear wave velocity in the favorable prognosis group revealed a clear decline, while those in the unfavorable prognosis group revealed an increasing tendency at different time points. Furthermore, liver and spleen shear wave velocity was higher in the unfavorable prognosis group, compared with the favorable prognosis group; the differences were statistically significant ( P < 0.05). The prognosis of patients with portal hypertension was significantly affected by spleen hardness at the 3 rd month after treatment [relative risk (RR) = 3.481]. At the 9 th month after treatment, the prognosis was affected by liver hardness (RR = 5.241) and spleen hardness (RR = 7.829). The differences between these two groups were statistically significant ( P < 0.05). The ROC analysis revealed that the area under the curve (AUC) of spleen hardness at the 3 rd month after treatment was 0.644, while the AUCs of liver and spleen hardness at the 9 th month were 0.579 and 0.776, respectively. These might predict the prognosis of patients with portal hypertension. Spleen hardness at the 3 rd month and liver and spleen shear wave velocity at the 9 th month may be used to assess the prognosis of patients with portal hypertension. This is hoped to be used as an indicator of predicting the prognosis of patients with portal hypertension.

Effect of verapamil on nitric oxide synthase in a portal vein-ligated rat model: Role of prostaglandin

PubMed Central

Lay, Chii-Shyan; May, CMY; Lee, Fa-Yauh; Tsai, Yang-Te; Lee, Shou-Dong; Chien, Shu; Sinchon, Shlomoh

2006-01-01

AIM: To investigate the effects of verapamil on nitric oxide (NO) synthesis in a portal vein-ligated rat model. METHODS: Systemic and splanchnic hemodynamics were measured by radiolabeled microspheres in portal hypertensive rats after acute administration of verapamil (2 mg/kg) on chronic treatment with Nw–nitro-L-arginine (NNA)(80 mg/kg) and/or indomethacin (2 mg/kg) . RESULTS: Verapamil (2 mg/kg) caused a marked fall in both arterial pressure and cardiac output accompanied by an insignificant change in the portal pressure and no change in portal venous inflow. This result suggested that verapamil did not cause a reduction in portal vascular resistance of portal hypertensive rats, which was similar between Nw- nitro–L-arginine-treated and indomethacin-treated groups. CONCLUSION: In portal hypertensive rats pretreated with NNA and/or indomethacin, acute verapamil administration can not reduce the portal pressure, suggesting that NO and prostaglandin play an important role in the pathogenesis of splanchnic arterial vasodilation in portal hypertension. PMID:16688824

Impact of hepatitis C oral therapy in portal hypertension.

PubMed

Libânio, Diogo; Marinho, Rui Tato

2017-07-14

Chronic hepatitis C is a leading cause of morbidity and mortality, mainly related to fibrosis/cirrhosis and portal hypertension. Direct antiviral agents are highly effective and safe and can now cure > 90% of the patients. Sustained viral response (SVR) after interferon-based regimens has been associated with improvement in liver function, fibrosis and portal hypertension in a significant proportion of patients, although a point of no return seems to exist from which viral elimination is no longer capable of preventing portal hypertension progression and liver decompensation. Indeed, although SVR is associated with improvement of hepatic venous pressure gradients and therefore a decreased risk of de novo esophageal varices, several studies show that viral clearance does not eliminate the risk of variceal progression, liver decompensation and death in patients with pre-established portal hypertension. Although evidence about the effects of direct antiviral agents (DAAs) on clinically significant outcomes is still scarce and with short follow-up, DAAs can decrease the burden of the disease if patients are timely treated before significant fibrosis and portal hypertension develops. Studies with longer follow-up are waited to establish the real magnitude of hepatitis C treatment on portal hypertension. Future studies should also focus on predictors of portal hypertension resolution since it can influence management and avoid unnecessary monitoring.

[Evaluation and treatment of portal hypertension].

PubMed

Brůha, Radan; Petrtýl, Jaromír

Liver cirrhosis is a serious disease shortening the life expectancy. Unavoidable consequence of cirrhosis is portal hypertension, which usually limits the prognosis by its complications. Portal hypertension is a prognostic factor for cirrhosis decompensation, variceal bleeding and even the mortality in cirrhotic patients. In the evaluation of portal hypertension hepatic venous pressure gradient (HVPG) measurement is used.Measurement of HVPG is used in clinical praxis in these situations: diagnosis of portal hypertension, evaluation of prognosis of patients with cirrhosis, monitoring the treatment efficacy in the prevention of variceal bleeding, management of acute variceal bleeding. Decrease of HVPG below 12 mmHg or at least for more than 20% of initial value in the treatment by beta-blockers is associated with the lower risk of bleeding from varices or other complications. HVPG above 20 mm Hg is associated with the high risk of early rebleeding from varices and can discriminate those patients profiting from early TIPS.HVPG measurement is an invasive, but simple, reproducible and safe catheterization technique with minimal complication rate. The most frequent complication could be incorrect assessment of obtained values. HVPG measurement should be a routine technique in centers specialized to liver diseases.

Gastrointestinal Bleeding in Cirrhotic Patients with Portal Hypertension

PubMed Central

Biecker, Erwin

2013-01-01

Gastrointestinal bleeding related to portal hypertension is a serious complication in patients with liver cirrhosis. Most patients bleed from esophageal or gastric varices, but bleeding from ectopic varices or portal hypertensive gastropathy is also possible. The management of acute bleeding has changed over the last years. Patients are managed with a combination of endoscopic and pharmacologic treatment. The endoscopic treatment of choice for esophageal variceal bleeding is variceal band ligation. Bleeding from gastric varices is treated by injection with cyanoacrylate. Treatment with vasoactive drugs as well as antibiotic treatment is started before or at the time point of endoscopy. The first-line treatment for primary prophylaxis of esophageal variceal bleeding is nonselective beta blockers. Pharmacologic therapy is recommended for most patients; band ligation is an alternative in patients with contraindications for or intolerability of beta blockers. Treatment options for secondary prophylaxis include variceal band ligation, beta blockers, a combination of nitrates and beta blockers, and combination of band ligation and pharmacologic treatment. A clear superiority of one treatment over the other has not been shown. Bleeding from portal hypertensive gastropathy or ectopic varices is less common. Treatment options include beta blocker therapy, injection therapy, and interventional radiology. PMID:27335828

[Late complications of liver cirrhosis - management of gastrointestinal bleeding in the presence of portal hypertension].

PubMed

Hejda, Václav

Cirrhosis is the end stage of progressive development of different liver diseases and is associated with significant morbidity and mortality rates. Cirrhosis is associated with a number of potential complications, in particular with development of portal hypertension. Portal hypertension with the production of ascites, hepatic and gastric varices bleeding in the upper part of the gastrointestinal tract, presents the breakpoint in the natural course of cirrhosis, and it is associated with a considerably worse prognosis of patients, with a dramatically increased risk of mortality. A major progress was reached during the past 10-20 years in diagnosing liver cirrhosis (including non-invasive methods), in primary prevention of the initial episode of upper gastrointestinal bleeding and in the therapy of acute bleeding due to modern pharmacotherapy, with regard to expanding possibilities of therapeutic endoscopy and relatively new options for management of acute bleeding (esophageal stents, TIPS and suchlike). However acute upper gastrointestinal bleeding associated with portal hypertension still presents a considerable risk of premature death (15-20 %). Early diagnosing and causal treatment of numerous liver diseases may lead to slowing or regression of fibrosis and cirrhosis and possibly even of the degree of portal hypertension and thereby also the risk of bleeding.Key words: cirrhosis - esophageal varices - treatment of bleeding - portal hypertension.

Recurrent recessive mutation in deoxyguanosine kinase causes idiopathic noncirrhotic portal hypertension.

PubMed

Vilarinho, Sílvia; Sari, Sinan; Yilmaz, Güldal; Stiegler, Amy L; Boggon, Titus J; Jain, Dhanpat; Akyol, Gulen; Dalgic, Buket; Günel, Murat; Lifton, Richard P

2016-06-01

Despite advances in the diagnosis and management of idiopathic noncirrhotic portal hypertension, its pathogenesis remains elusive. Insight may be gained from study of early-onset familial idiopathic noncirrhotic portal hypertension, in which Mendelian mutations may account for disease. We performed exome sequencing of eight subjects from six kindreds with onset of portal hypertension of indeterminate etiology during infancy or childhood. Three subjects from two consanguineous families shared the identical rare homozygous p.N46S mutation in DGUOK, a deoxyguanosine kinase required for mitochondrial DNA replication; haplotype sharing demonstrated that the mutation in the two families was inherited from a remote common ancestor. All three affected subjects had stable portal hypertension with noncirrhotic liver disease for 6-16 years of follow-up. This mutation impairs adenosine triphosphate binding and reduces catalytic activity. Loss-of-function mutations in DGUOK have previously been implicated in cirrhosis and liver failure but not in isolated portal hypertension. Interestingly, treatment of patients with human immunodeficiency viral infection with the nucleoside analogue didanosine is known to cause portal hypertension in a subset of patients and lowers deoxyguanosine kinase levels in vitro; the current findings implicate these effects on deoxyguanosine kinase in the causal mechanism. Our findings provide new insight into the mechanisms mediating inherited and acquired noncirrhotic portal hypertension, expand the phenotypic spectrum of DGUOK deficiency, and provide a new genetic test for a specific cause of idiopathic noncirrhotic portal hypertension. (Hepatology 2016;63:1977-1986). © 2016 by the American Association for the Study of Liver Diseases.

Contemporary concepts of the medical therapy of portal hypertension under liver cirrhosis

PubMed Central

Garbuzenko, Dmitry Victorovich

2015-01-01

Severe complications of liver cirrhosis are mostly related to portal hypertension. At the base of the pathogenesis of portal hypertension is the increase in hepatic vascular resistance to portal blood flow with subsequent development of hyperdynamic circulation, which, despite of the formation of collateral circulation, promotes progression of portal hypertension. An important role in its pathogenesis is played by the rearrangement of vascular bed and angiogenesis. As a result, strategic directions of the therapy of portal hypertension under liver cirrhosis include selectively decreasing hepatic vascular resistance with preserving or increasing portal blood flow, and correcting hyperdynamic circulation and pathological angiogenesis, while striving to reduce the hepatic venous pressure gradient to less than 12 mmHg or 20% of the baseline. Over the last years, substantial progress in understanding the pathophysiological mechanisms of hemodynamic disorders under liver cirrhosis has resulted in the development of new drugs for their correction. Although the majority of them have so far been investigated only in animal experiments, as well as at the molecular and cellular level, it might be expected that the introduction of the new methods in clinical practice will increase the efficacy of the conservative approach to the prophylaxis and treatment of portal hypertension complications. The purpose of the review is to describe the known methods of portal hypertension pharmacotherapy and discuss the drugs that may affect the basic pathogenetic mechanisms of its development. PMID:26034348

Contemporary concepts of the medical therapy of portal hypertension under liver cirrhosis.

PubMed

Garbuzenko, Dmitry Victorovich

2015-05-28

Severe complications of liver cirrhosis are mostly related to portal hypertension. At the base of the pathogenesis of portal hypertension is the increase in hepatic vascular resistance to portal blood flow with subsequent development of hyperdynamic circulation, which, despite of the formation of collateral circulation, promotes progression of portal hypertension. An important role in its pathogenesis is played by the rearrangement of vascular bed and angiogenesis. As a result, strategic directions of the therapy of portal hypertension under liver cirrhosis include selectively decreasing hepatic vascular resistance with preserving or increasing portal blood flow, and correcting hyperdynamic circulation and pathological angiogenesis, while striving to reduce the hepatic venous pressure gradient to less than 12 mmHg or 20% of the baseline. Over the last years, substantial progress in understanding the pathophysiological mechanisms of hemodynamic disorders under liver cirrhosis has resulted in the development of new drugs for their correction. Although the majority of them have so far been investigated only in animal experiments, as well as at the molecular and cellular level, it might be expected that the introduction of the new methods in clinical practice will increase the efficacy of the conservative approach to the prophylaxis and treatment of portal hypertension complications. The purpose of the review is to describe the known methods of portal hypertension pharmacotherapy and discuss the drugs that may affect the basic pathogenetic mechanisms of its development.

Combined administration of propranolol + AG490 offers better effects on portal hypertensive rats with cirrhosis.

PubMed

Wang, Dong; Wang, Qin; Yin, Jikai; Dong, Rui; Wang, Qing; Du, Xilin; Lu, Jianguo

2016-05-01

AG490, the specific inhibitor of JAK2/STAT3 signaling, has been shown to decrease portal pressure, splanchnic hyperdynamic circulation and liver fibrosis in cirrhotic rats. Nonselective betablockers such as propranolol are the only drugs recommended in the treatment of portal hypertension. The aim of this study was to explore the combinative effect of treatment with propranolol and AG490 on portal hypertension. Rats induced by common bile duct ligation were treated with vehicle, AG490, propranolol, or AG490 + propranolol for 2 weeks. Hemodynamics parameters were assessed. Expressions of phospho-STAT3 protein and its down-regulated cytokines in splanchnic organs were detected by ELISA or western blot. Lipopolysaccharide binding protein (LBP) and IL-6 were assessed by ELISA or western blot. Characterization of liver and mesentery was performed by histological analyses. Highly expressed phospho-STAT3 protein in cirrhotic rats could successfully be inhibited by AG490 or AG490 + propranolol treatments but not by propranolol alone. Both AG490 and propranolol significantly reduced portal pressure and hyperdynamic splanchnic circulation, and combination of AG490 and propranolol achieved an additive effect than with either drug alone. AG490, alone or in combination with propranolol, inhibited liver fibrosis, splenomegaly and splanchnic angiogenesis. Increased markers of bacterial translocation (LBP and IL6) were greatly reduced by propranolol but not by AG490. The combination of propranolol and AG490 caused a greater improvement of portal hypertension and might therefore offer a potentially promising therapy in the portal hypertension treatment. © 2015 Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.

Elastography methods for the non-invasive assessment of portal hypertension.

PubMed

Roccarina, Davide; Rosselli, Matteo; Genesca, Joan; Tsochatzis, Emmanuel A

2018-02-01

The gold standard to assess the presence and severity of portal hypertension remains the hepatic vein pressure gradient, however the recent development of non-invasive assessment using elastography techniques offers valuable alternatives. In this review, we discuss the diagnostic accuracy and utility of such techniques in patients with portal hypertension due to cirrhosis. Areas covered: A literature search focused on liver and spleen stiffness measurement with different elastographic techniques for the assessment of the presence and severity of portal hypertension and oesophageal varices in people with chronic liver disease. The combination of elastography with parameters such as platelet count and spleen size is also discussed. Expert commentary: Non-invasive assessment of liver fibrosis and portal hypertension is a validated tool for the diagnosis and follow-up of patients. Baveno VI recommended the combination of transient elastography and platelet count for ruling out varices needing treatment in patients with compensated advanced chronic liver disease. Assessment of aetiology specific cut-offs for ruling in and ruling out clinically significant portal hypertension is an unmet clinical need. The incorporation of spleen stiffness measurements in non-invasive algorithms using validated software and improved measuring scales might enhance the non-invasive diagnosis of portal hypertension in the next 5 years.

[Treatment of non-cirrhotic, non-tumoural portal vein thrombosis].

PubMed

Llop, Elba; Seijo, Susana

2016-01-01

Thrombosis of the splenoportal axis not associated with liver cirrhosis or neoplasms is a rare disease whose prevalence ranges from 0.7 to 3.7 per 100,000 inhabitants. However, this entity is the second most common cause of portal hypertension. Prothrombotic factors are present as an underlying cause in up to 70% of patients and local factors in 10-50%. The coexistence of several etiological factors is frequent. Clinical presentation may be acute or chronic (portal cavernomatosis). The acute phase can present as abdominal pain, nausea, vomiting, fever, rectorrhagia, intestinal congestion, and ischemia. In this phase, early initiation of anticoagulation is essential to achieve portal vein recanalization and thus improve patient prognosis. In the chronic phase, symptoms are due to portal hypertension syndrome. In this phase, the aim of treatment is to treat or prevent the complications of portal hypertension. Anticoagulation is reserved to patients with a proven underlying thrombophilic factor. Copyright © 2016 Elsevier España, S.L.U. y AEEH y AEG. All rights reserved.

Variant meso-Rex bypass with transposition of abdominal autogenous vein for the management of idiopathic extrahepatic portal vein obstruction: a retrospective observational study.

PubMed

Ha, Tae-Yong; Kim, Kyung-Mo; Ko, Gi-Young; Oh, Seak Hee; Kwon, Tae-Won; Cho, Yong-Pil; Lee, Sung-Gyu

2015-10-17

The aim of this study was to evaluate whether variant meso-Rex bypass with transposition of abdominal autogenous vein can be used as an alternative treatment modality for selected patients with symptomatic extrahepatic portal vein obstruction. This was a retrospective review of six consecutive patients who received this alternative procedure for the treatment of symptomatic portal hypertension secondary to idiopathic extrahepatic portal vein obstruction. Their clinical characteristics, operative procedures and outcomes were analyzed retrospectively. The procedure was attempted in six patients, and all had a patent shunt established by intraoperative portography at the end of the procedure; the coronary vein was used in four patients and the inferior mesenteric vein was used in two. During the median period of 23.5 months (range 10-30 months), follow-up was uneventful except one patient; reduced portal hypertension and no new episodes of gastrointestinal bleeding were observed in all patients, with the exception of one patient with shunt stenosis and recurrent varix bleeding who had to undergo endovascular treatment to restore portal vein blood flow. Technical and clinical success was achieved in all patients. This procedure could be used safely and effectively to treat selected patients with portal hypertension secondary to extrahepatic portal vein obstruction.

Managing portal hypertension in patients with liver cirrhosis

PubMed Central

Sauerbruch, Tilman; Schierwagen, Robert; Trebicka, Jonel

2018-01-01

Portal hypertension is one cause and a part of a dynamic process triggered by chronic liver disease, mostly induced by alcohol or incorrect nutrition and less often by viral infections and autoimmune or genetic disease. Adequate staging - continuously modified by current knowledge - should guide the prevention and treatment of portal hypertension with defined endpoints. The main goals are interruption of etiology and prevention of complications followed, if necessary, by treatment of these. For the past few decades, shunts, mostly as intrahepatic stent bypass between portal and hepatic vein branches, have played an important role in the prevention of recurrent bleeding and ascites formation, although their impact on survival remains ambiguous. Systemic drugs, such as non-selective beta-blockers, statins, or antibiotics, reduce portal hypertension by decreasing intrahepatic resistance or portal tributary blood flow or by blunting inflammatory stimuli inside and outside the liver. Here, the interactions among the gut, liver, and brain are increasingly examined for new therapeutic options. There is no general panacea. The interruption of initiating factors is key. If not possible or if not possible in a timely manner, combined approaches should receive more attention before considering liver transplantation. PMID:29780579

The somatostatin analogue octreotide inhibits angiogenesis in the earliest, but not in advanced, stages of portal hypertension in rats.

PubMed

Mejias, Marc; Garcia-Pras, Ester; Tiani, Carolina; Bosch, Jaime; Fernandez, Mercedes

2008-01-01

Angiogenesis is an important determinant of the pathophysiology of portal hypertension contributing to the formation of portosystemic collateral vessels and the hyperdynamic splanchnic circulation associated to this syndrome. Somatostatin and its analogues, like octreotide, have been shown to be powerful inhibitors of experimental angiogenesis. To determine whether octreotide has angioinhibitory effects in portal hypertensive rats. Partial portal vein-ligated (PPVL) rats were treated with octreotide or vehicle during 4 or 7 days. Splanchnic neovascularization and VEGF expression were determined by histological analysis and western blotting. Expression of the somatostatin receptor subtype 2 (SSTR2), which mediates the anti-angiogenic effects of octreotide, was also analyzed. Formation of portosystemic collaterals (radioactive microspheres) and hemodynamic parameters were also measured. Octreotide treatment during 4 days markedly and significantly decreased splanchnic neovascularization, VEGF expression by 63% and portal pressure by 15%, whereas portosystemic collateralization and splanchnic blood flow were not modified. After 1 week of octreotide injection, portal pressure was reduced by 20%, but inhibition of angiogenesis escaped from octreotide therapy, a phenomenon that could be related to the finding that expression of SSTR2 receptor decreased progressively (up to 78% reduction) during the evolution of portal hypertension. This study provides the first experimental evidence showing that octreotide may be an effective anti-angiogenic therapy early after induction of portal hypertension, but not in advanced stages most likely due to SSTR2 down-regulation during the progression of portal hypertension in rats. These findings shed light on new mechanisms of action of octreotide in portal hypertension.

The portal hypertension syndrome: etiology, classification, relevance, and animal models.

PubMed

Bosch, Jaime; Iwakiri, Yasuko

2018-02-01

Portal hypertension is a key complication of portal hypertension, which is responsible for the development of varices, ascites, bleeding, and hepatic encephalopathy, which, in turn, cause a high mortality and requirement for liver transplantation. This review deals with the present day state-of-the-art preventative treatments of portal hypertension in cirrhosis according to disease stage. Two main disease stages are considered, compensated and decompensated cirrhosis, the first having good prognosis and being mostly asymptomatic, and the second being heralded by the appearance of bleeding or non-bleeding complications of portal hypertension. The aim of treatment in compensated cirrhosis is preventing clinical decompensation, the more frequent event being ascites, followed by variceal bleeding and hepatic encephalopathy. Complications are mainly driven by an increase of hepatic vein pressure gradient (HVPG) to values ≥10 mmHg (defining the presence of Clinically Significant Portal Hypertension, CSPH). Before CSPH, the treatment is limited to etiologic treatment of cirrhosis and healthy life style (abstain from alcohol, avoid/correct obesity…). When CSPH is present, association of a non-selective beta-blocker (NSBB), including carvedilol should be considered. NSBBs are mandatory if moderate/large varices are present. Patients should also enter a screening program for hepatocellular carcinoma. In decompensated patients, the goal is to prevent further bleeding if the only manifestation of decompensation was a bleeding episode, but to prevent liver transplantation and death in the common scenario where patients have manifested first non-bleeding complications. Treatment is based on the same principles (healthy life style..) associated with administration of NSBBs in combination if possible with endoscopic band ligation if there has been variceal bleeding, and complemented with simvastatin administration (20-40 mg per day in Child-Pugh A/B, 10-20 mg in Child C). Recurrence shall be treated with TIPS. TIPS might be indicated earlier in patients with: 1) Difficult/refractory ascites, who are not the best candidates for NSBBs, 2) patients having bleed under NSBBs or showing no HVPG response (decrease in HVPG of at least 20% of baseline or to values equal or below 12 mmHg). Decompensated patients shall all be considered as potential candidates for liver transplantation. Treatment of portal hypertension has markedly improved in recent years. The present day therapy is based on accurate risk stratification according to disease stage.

Endovascular interventions for traumatic portal venous hemorrhage complicated by portal hypertension

PubMed Central

Sundarakumar, Dinesh Kumar; Smith, Crysela Mirta; Lopera, Jorge Enrique; Kogut, Matthew; Suri, Rajeev

2013-01-01

Life-threatening hemorrhage rarely occurs from the portal vein following blunt hepatic trauma. Traditionally, severe portal bleeding in this setting has been controlled by surgical techniques such as packing, ligation, and venorrhaphy. The presence of portal hypertension could potentially increase the amount of hemorrhage in the setting of blunt portal vein trauma making it more difficult to control. This case series describes the use of indirect carbon dioxide portography to identify portal hemorrhage. Furthermore, these cases illustrate attempted endovascular treatment utilizing a transjugular intrahepatic portosystemic shunt in one scenario and transmesocaval shunt coiling of a jejunal varix in the other. PMID:24179633

Beneficial long term effect of a phosphodiesterase-5-inhibitor in cirrhotic portal hypertension: A case report with 8 years follow-up.

PubMed

Deibert, Peter; Lazaro, Adhara; Stankovic, Zoran; Schaffner, Denise; Rössle, Martin; Kreisel, Wolfgang

2018-01-21

Non-selective beta-blockers are the mainstay of medical therapy for portal hypertension in liver cirrhosis. Inhibitors of phosphodiesterase-5 (PDE-5-inhibitors) reduce portal pressure in the acute setting by > 10% which may suggest a long-term beneficial effect. Currently, there is no available data on long-term treatment of portal hypertension with PDE-5-inhibitors. This case of a patient with liver cirrhosis secondary to autoimmune liver disease with episodes of bleeding from esophageal varices is the first documented case in which a treatment with a PDE-5-inhibitor for eight years was monitored. In the acute setting, the PDE-5-inhibitor Vardenafil lowered portal pressure by 13%. The portal blood flow increased by 28% based on Doppler sonography and by 16% using MRI technique. As maintenance medication the PDE-5-inhibitor Tadalafil was used for eight consecutive years with comparable effects on portal pressure and portal blood flow. There were no recurrence of bleeding and no formation of new varices. Influencing the NO-pathway by the use of PDE-5 inhibitors may have long-term beneficial effects in compensated cirrhosis.

Pregnancy with Portal Hypertension

PubMed Central

Aggarwal, Neelam; Negi, Neha; Aggarwal, Aakash; Bodh, Vijay; Dhiman, Radha K.

2014-01-01

Even though pregnancy is rare with cirrhosis and advanced liver disease, but it may co-exist in the setting of non-cirrhotic portal hypertension as liver function is preserved but whenever encountered together is a complex clinical dilemma. Pregnancy in a patient with portal hypertension presents a special challenge to the obstetrician as so-called physiological hemodynamic changes associated with pregnancy, needed for meeting demands of the growing fetus, worsen the portal hypertension thereby putting mother at risk of potentially life-threatening complications like variceal hemorrhage. Risks of variceal bleed and hepatic decompensation increase many fold during pregnancy. Optimal management revolves round managing the portal hypertension and its complications. Thus management of such cases requires multi-speciality approach involving obstetricians experienced in dealing with high risk cases, hepatologists, anesthetists and neonatologists. With advancement in medical field, pregnancy is not contra-indicated in these women, as was previously believed. This article focuses on the different aspects of pregnancy with portal hypertension with special emphasis on specific cause wise treatment options to decrease the variceal bleed and hepatic decompensation. Based on extensive review of literature, management from pre-conceptional period to postpartum is outlined in order to have optimal maternal and perinatal outcomes. PMID:25755552

Nodular regenerative hyperplasia: Evolving concepts on underdiagnosed cause of portal hypertension

PubMed Central

Hartleb, Marek; Gutkowski, Krzysztof; Milkiewicz, Piotr

2011-01-01

Nodular regenerative hyperplasia (NRH) is a rare liver condition characterized by a widespread benign transformation of the hepatic parenchyma into small regenerative nodules. NRH may lead to the development of non-cirrhotic portal hypertension. There are no published systematic population studies on NRH and our current knowledge is limited to case reports and case series. NRH may develop via autoimmune, hematological, infectious, neoplastic, or drug-related causes. The disease is usually asymptomatic, slowly or non-progressive unless complications of portal hypertension develop. Accurate diagnosis is made by histopathology, which demonstrates diffuse micronodular transformation without fibrous septa. Lack of perinuclear collagen tissue distinguishes NRH from typical regenerative nodules in the cirrhotic liver. While the initial treatment is to address the underlying disease, ultimately the therapy is directed to the management of portal hypertension. The prognosis of NRH depends on both the severity of the underlying illness and the prevention of secondary complications of portal hypertension. In this review we detail the epidemiology, pathogenesis, diagnosis, management, and prognosis of NRH. PMID:21472097

Interventional Radiologic Treatment for Idiopathic Portal Hypertension

DOE Office of Scientific and Technical Information (OSTI.GOV)

Hirota, Shozo; Ichikawa, Satoshi; Matsumoto, Shinichi

1999-07-15

Purpose: To evaluate the usefulness of interventional radiological treatment for idiopathic portal hypertension. Methods: Between 1995 and 1998, we performed an interventional radiological treatment in five patients with idiopathic portal hypertension, four of whom had refused surgery and one of whom had undergone surgery. Three patients with gastroesophageal varices (GEV) were treated by partial splenic embolization (PSE), one patient with esophageal varices (EV) and massive ascites by transjugular intrahepatic portosytemic shunt (TIPS) and PSE, and one patient with GEV by percutaneous transhepatic obliteration (PTO). Midterm results were analyzed in terms of the effect on esophageal and/or gastric varices. Results: Inmore » one woman with severe GEV who underwent three sessions of PSE, there was endoscopic confirmation that the GEV had disappeared. In one man his EV shrunk markedly after two sessions of PSE. In two patients slight reduction of the EV was obtained with one application of PSE combined with endoscopic variceal ligation therapy. PTO for GV in one patient resulted in good control of the varices. All patients have survived for 16-42 months since the first interventional treatment, and varices are well controlled. Conclusion: Interventional radiological treatment is effective for patients with idiopathic portal hypertension, whether or not they have undergone surgery.« less

Hydrodynamics Analysis and CFD Simulation of Portal Venous System by TIPS and LS.

PubMed

Wang, Meng; Zhou, Hongyu; Huang, Yaozhen; Gong, Piyun; Peng, Bing; Zhou, Shichun

2015-06-01

In cirrhotic patients, portal hypertension is often associated with a hyperdynamic changes. Transjugular Intrahepatic Portosystemic Shunt (TIPS) and Laparoscopic splenectomy are both treatments for liver cirrhosis due to portal hypertension. While, the two different interventions have different effects on hemodynamics after operation and the possibilities of triggering PVT are different. How hemodynamics of portal vein system evolving with two different operations remain unknown. Based on ultrasound and established numerical methods, CFD technique is applied to analyze hemodynamic changes after TIPS and Laparoscopic splenectomy. In this paper, we applied two 3-D flow models to the hemodynamic analysis for two patients who received a TIPS and a laparoscopic splenectomy, both therapies for treating portal hypertension induced diseases. The current computer simulations give a quantitative analysis of the interplay between hemodynamics and TIPS or splenectomy. In conclusion, the presented computational model can be used for the theoretical analysis of TIPS and laparoscopic splenectomy, clinical decisions could be made based on the simulation results with personal properly treatment.

[Selective portal-systemic shunts for bleeding portal hypertension].

PubMed

Orozco, H; Mercado, M A; Takahashi, T; García-Tsao, G; Guevara, L; Hernandez-Ortiz, J; Tielve, M

1990-07-01

At the beginning of the seventies, we began to perform regularly selective shunts for the treatment of portal hypertension. In a 15 year period, 177 patients (155 with liver cirrhosis) were operated with three kinds of selective shunts: 128 with a Warren shunt, 29 with an end to end renosplenic shunt and 20 with a splenocaval shunt. 167 cases were operated in an elective fashion. The 15 years global operative mortality, was 14.4%. Operative mortality of the Child A patients, was 11.6%. Survival for the Child A group was 74.6% at 1 year, 68.2% at 5 years and 64.6% at 15 years. Incapacitating encephalopathy was observed in 6.9%, rebleeding 6.2% and shunt thrombosis in 6.2%. Portal vein alterations in the postoperative period were observed: in 13.3% a reduction in diameter ocurred and in 20.5%, thrombosis was recorded. It is concluded that when feasible, the selective shunts are the treatment of choice for portal hypertension in those patients with good liver function.

Review: pharmacotherapeutic agents in the treatment of portal hypertension.

PubMed

Lebrec, D

1997-02-01

Certain vasoactive substances reduce portal pressure in patients or animals with portal hypertension by either inducing splanchnic vasoconstriction or reducing hepatic vascular resistance. Studies have shown that propranolol or nadolol significantly reduce the risk of a first episode of gastrointestinal (GI) bleeding and increase the survival rate in patients with cirrhosis and oesophageal varices. Isosorbide-5-mononitrate is also effective in the prevention of bleeding. The combination of beta-blockers and nitrates may be more effective than one drug alone. These results show that beta-adrenoceptor antagonists must be used to prevent the first episode of GI bleeding. Beta-blocker administration also significantly reduces the risk of recurrent GI bleeding and increases the survival rate in patients with cirrhosis. Studies have shown that propranolol is as effective as endoscopic sclerotherapy. The combination of a beta-blocker with endoscopic sclerotherapy may be more effective than pharmacological or endoscopic treatment alone for the prevention of rebleeding. Finally, new experimental and clinical studies are needed to improve the pharmacological treatment of portal hypertension.

Mesocaval Shunt Creation for Jejunal Variceal Bleeding with Chronic Portal Vein Thrombosis.

PubMed

Yoon, Ja Kyung; Kim, Man Deuk; Lee, Do Yun; Han, Seok Joo

2018-01-01

The creation of transjugular intrahepatic portosystemic shunt (TIPS) is a widely performed technique to relieve portal hypertension, and to manage recurrent variceal bleeding and refractory ascites in patients where medical and/or endoscopic treatments have failed. However, portosystemic shunt creation can be challenging in the presence of chronic portal vein occlusion. In this case report, we describe a minimally invasive endovascular mesocaval shunt creation with transsplenic approach for the management of recurrent variceal bleeding in a portal hypertension patient with intra- and extrahepatic portal vein occlusion. © Copyright: Yonsei University College of Medicine 2018.

Evolution in the understanding of the pathophysiological basis of portal hypertension: How changes in paradigm are leading to successful new treatments

PubMed Central

Bosch, Jaume; Groszmann, Roberto J.; Shah, Vijay H.

2015-01-01

Summary Among the common complication of cirrhosis portal hypertension witnessed a major improvement of prognosis during the past decades. Principally due to the introduction of rational treatments based on new pathophysiological paradigms (concepts of thought) developed in the 1980s. The best example being the use of non-selective beta-blockers and of vasopressin analogs, somatostatin, and its analogs. Further refinement in the knowledge of the molecular mechanisms involved in the regulation of both the splanchnic and hepatic circulation has led to the emergence of new treatments, which are based on evidence that show not only structural but also vasoactive components increase the hepatic vascular resistance, as well as of angiogenesis. This knowledge and future improvements will most likely result in more effective treatment of portal hypertension and effective prevention of its complications in early stages. PMID:25920081

Portal Hypertension Over the Last 25 Years: Where Did It Go?

PubMed

Rosemurgy, Alexander; Raitano, Olivia; Srikumar, Thejal; Sawangkum, Peeraya; Luberice, Kenneth; Ryan, Carrie; Ross, Sharona

2016-06-01

Portal hypertension has seemingly vanished from surgery; this study was undertaken to determine where it has gone. Data from the Agency for Health Care Administration for 33,166,201 hospital inpatients in Florida for the periods 1988 to 1992, 1998 to 2002, and 2008 to 2012 were analyzed. Admissions with a diagnosis of portal hypertension dramatically increased: 5,473 patients from 1988 to 1992, 7,366 patients from 1998 to 2002, and 36,554 patients from 2008 to 2012. Endoscopic treatment of esophageal varices also dramatically increased. The number of decompressive shunts placed nominally increased, but application of endoscopic therapy increased significantly faster than the application of decompressive shunts (p < 0.0001). The percentage of patients who underwent shunting dramatically and significantly decreased (p < 0.0001), and surgeons undertook proportionally fewer shunts (42% in 1992 to 4% in 2012; p < 0.0001). For patients with a diagnosis of portal hypertension, in-hospital mortality progressively decreased, from 9% in 1988 to 1992 to 3% in 2008 to 2012 (p < 0.0001). In the state of Florida, over 25 years, there has been a 7-fold increase in the number of patients admitted with a diagnosis of portal hypertension, with a 65% reduction of in-hospital mortality. Application of endoscopic treatment of varices has increased dramatically. Decompressive shunts are applied to an ever-decreasing percentage of patients, and when applied, are now routinely undertaken by nonsurgeons. Therefore, portal hypertension has disappeared from the purview of surgery and has migrated toward the world of medical and endoscopic therapy, probably never to return. Copyright © 2016. Published by Elsevier Inc.

Celecoxib and octreotide synergistically ameliorate portal hypertension via inhibition of angiogenesis in cirrhotic rats.

PubMed

Gao, Jin-Hang; Wen, Shi-Lei; Feng, Shi; Yang, Wen-Juan; Lu, Yao-Yao; Tong, Huan; Liu, Rui; Tang, Shi-Hang; Huang, Zhi-Yin; Tang, Ying-Mei; Yang, Jin-Hui; Xie, Hui-Qi; Tang, Cheng-Wei

2016-10-01

Abnormal angiogenesis is critical for portal hypertension in cirrhosis. Except for etiological treatment, no efficient medication or regime has been explored to treat the early stage of cirrhosis when angiogenesis is initiated or overwhelming. In this study, we explored an anti-angiogenesis effort through non-cytotoxic drugs octreotide and celecoxib to treat early stage of cirrhotic portal hypertension in an animal model. Peritoneal injection of thioacetamide (TAA) was employed to induce liver cirrhosis in rats. A combination treatment of celecoxib and octreotide was found to relieve liver fibrosis, portal venous pressure, micro-hepatic arterioportal fistulas, intrahepatic and splanchnic angiogenesis. Celecoxib and octreotide exerted their anti-angiogenesis effect via an axis of cyclooxygenase-2/prostaglandin E2/EP-2/somatostatin receptor-2, which consequently down-regulated phosphorylation of extracellular signal-regulated kinase (p-ERK)-hypoxia-inducible factor-1α (HIF-1α)-vascular endothelial growth factor (VEGF) integrated signaling pathways. In conclusions, combination of celecoxib and octreotide synergistically ameliorated liver fibrosis and portal hypertension of the cirrhotic rats induced by TAA via the inhibition of intrahepatic and extrahepatic angiogenesis. The potential mechanisms behind the regimen may due to the inactivation of p-ERK-HIF-1α-VEGF signaling pathway.

Combined transjugular intrahepatic portosystemic shunt and other interventions for hepatocellular carcinoma with portal hypertension.

PubMed

Qiu, Bin; Zhao, Meng-Fei; Yue, Zhen-Dong; Zhao, Hong-Wei; Wang, Lei; Fan, Zhen-Hua; He, Fu-Liang; Dai, Shan; Yao, Jian-Nan; Liu, Fu-Quan

2015-11-21

To evaluate combination transjugular intrahepatic portosystemic shunt (TIPS) and other interventions for hepatocellular carcinoma (HCC) and portal hypertension. Two hundred and sixty-one patients with HCC and portal hypertension underwent TIPS combined with other interventional treatments (transarterial chemoembolization/transarterial embolization, radiofrequency ablation, hepatic arterio-portal fistulas embolization, and splenic artery embolization) from January 1997 to January 2010 at Beijing Shijitan Hospital. Two hundred and nine patients (121 male and 88 female, aged 25-69 years, mean 48.3 ± 12.5 years) with complete clinical data were recruited. We evaluated the safety of the procedure (procedure-related death and serious complications), change of portal vein pressure before and after TIPS, symptom relief [e.g., ascites, hydrothorax, esophageal gastric-fundus variceal bleeding (EGVB)], cumulative rates of survival, and distributary channel restenosis. The characteristics of the patients surviving ≥ 5 and < 5 years were also analyzed. The portosystemic pressure was decreased from 29.0 ± 4.1 mmHg before TIPS to 18.1 ± 2.9 mmHg after TIPS (t = 69.32, P < 0.05). Portosystemic pressure was decreased and portal hypertension symptoms were ameliorated. During the 5 year follow-up, the total recurrence rate of resistant ascites or hydrothorax was 7.2% (15/209); 36.8% (77/209) for EGVB; and 39.2% (82/209) for hepatic encephalopathy. The cumulative rates of distributary channel restenosis at 1, 2, 3, 4, and 5 years were 17.2% (36/209), 29.7% (62/209), 36.8% (77/209), 45.5% (95/209) and 58.4% (122/209), respectively. No procedure-related deaths and serious complications (e.g., abdominal bleeding, hepatic failure, and distant metastasis) occurred. Moreover, Child-Pugh score, portal vein tumor thrombosis, lesion diameter, hepatic arterio-portal fistulas, HCC diagnosed before or after TIPS, stent type, hepatic encephalopathy, and type of other interventional treatments were related to 5 year survival after comparing patient characteristics. TIPS combined with other interventional treatments seems to be safe and efficacious in patients with HCC and portal hypertension.

Novel treatment options for portal hypertension

PubMed Central

Laleman, Wim

2017-01-01

Abstract Portal hypertension is most frequently associated with cirrhosis and is a major driver for associated complications, such as variceal bleeding, ascites or hepatic encephalopathy. As such, clinically significant portal hypertension forms the prelude to decompensation and impacts significantly on the prognosis of patients with liver cirrhosis. At present, non-selective β-blockers, vasopressin analogues and somatostatin analogues are the mainstay of treatment but these strategies are far from satisfactory and only target splanchnic hyperemia. In contrast, safe and reliable strategies to reduce the increased intrahepatic resistance in cirrhotic patients still represent a pending issue. In recent years, several preclinical and clinical trials have focused on this latter component and other therapeutic avenues. In this review, we highlight novel data in this context and address potentially interesting therapeutic options for the future. PMID:28533907

Therapeutic effect of captopril, pentoxifylline, and cordyceps sinensis in pre-hepatic portal hypertensive rats.

PubMed

Ahmed, Ahmed F; El-Maraghy, Nabila N; Abdel Ghaney, Rasha H; Elshazly, Shimaa M

2012-01-01

Portal hypertension is an important and potentially fatal complication of liver disease whereby cellular and fibrotic alterations manifest to increase portal venous pressure. The aim of this study is to investigate the effect of captopril, pentoxifylline (PTX), and cordyceps sinensis in pre-hepatic portal hypertensive rats. Wistar male rats were divided at random into 3 main groups: the first group: control rats. The second group: sham-operated rats and the third group: prehepatic portal hypertensive rats (PHPHT) induced by regulated pre-hepatic portal vein ligation. After 14 days, Group 3 was subdivided into 5 subgroups. Subgroup (1): portal vein-ligated (PVL) was killed at once; Subgroup (2): received distilled water for 30 days (untreated PVL group); subgroups 3-5 were treated with captopril (60 mg/kg, orally); PTX (100 mg/kg, orally); and C. sinensis (200 mg/kg, orally), respectively, as a single daily dose for 30 days. Portal pressure, nitric oxide (NO), antioxidant enzymes, Liver enzymes, and creatinine levels were measured to evaluate the status of the liver state. Portal vein ligation produced significant increments in liver enzymes, NO, creatinine and portal pressure concomitant with significant decrements in glutathione content and superoxide dismutase activity. Treatment with captopril, PTX, and C. sinensis resulted in a significant reduction in liver enzymes, NO, creatinine and portal pressure and observable increase in antioxidant enzymes. captopril, PTX, and C. sinensis have promising effect in controlling PHPHT and reducing hyperdynamic circulatory state through reduction of portal pressure and NO level.

[Hepatopulmonary syndrome and portopulmonary hypertension].

PubMed

Marcu, Cristina; Schiffer, Eduardo; Aubert, John-David; Vionnet, Julien; Yerly, Patrick; Deltenre, Pierre; Marot, Astrid

2017-08-30

Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are two frequent pulmonary complications of liver disease. Portal hypertension is a key element in the pathogenesis of both disorders, which are however distinct in terms of pathogenesis, diagnosis and treatment. HPS corresponds to an abnormal arterial oxygenation in relation with the development of intrapulmonary vascular dilatations. POPH is a pulmonary arterial hypertension in the setting of portal hypertension and elevated pulmonary vascular resistance. As both diseases are associated with an increased risk of morbidity and mortality, it is important to screen and evaluate the severity of these two disorders particularly in liver transplant candidates.

Three months of simvastatin therapy vs. placebo for severe portal hypertension in cirrhosis: A randomized controlled trial.

PubMed

Pollo-Flores, Priscila; Soldan, Mônica; Santos, Ubiratan Cassano; Kunz, Danielle Gobbi; Mattos, Denise Espindola; da Silva, Alexandre Cerqueira; Marchiori, Roberta Cabral; Rezende, Guilherme Ferreira da Motta

2015-11-01

Pleiotropic effects of statins decrease intrahepatic resistance and portal hypertension. We evaluated the effects of simvastatin on hepatic venous pressure gradient (HVPG) and azygos vein blood flow in cirrhotic patients. A 3-month prospective, randomized, triple-blind trial with simvastatin (40 mg/day) vs. placebo was conducted in patients with cirrhotic portal hypertension. HVPG and azygos blood flow, measured by colour Doppler endoscopic ultrasound, were assessed before and after treatment. The primary endpoint was a decrease in the HVPG of at least 20% from baseline or to ≤12 mmHg after the treatment. 34 patients were prospectively enrolled, and 24 completed the protocol. In the simvastatin group 6/11 patients (55%) presented a clinically relevant decrease in the HVPG; no decrease was observed in the placebo group (p=0.036). Patients with medium/large oesophageal varices and previous variceal bleeding had a higher response rate to simvastatin. HVPG and azygos blood flow values were not correlated. No significant adverse events occurred. Simvastatin lowers portal pressure and may even improve liver function. The haemodynamic effect appears to be more evident in patients with severe portal hypertension. Copyright © 2015 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

Mouse and Rat Models of Induction of Hepatic Fibrosis and Assessment of Portal Hypertension.

PubMed

Klein, Sabine; Schierwagen, Robert; Uschner, Frank Erhard; Trebicka, Jonel

2017-01-01

Portal hypertension either develops due to progressive liver fibrosis or is the consequence of vascular liver diseases such as portal vein thrombosis or non-cirrhotic portal hypertension. This chapter focuses on different rodent models of liver fibrosis with portal hypertension and also in few non-cirrhotic portal hypertension models. Importantly, after the development of portal hypertension, the proper assessment of drug effects in the portal and systemic circulation should be discussed. The last part of the chapter is dedicated in these techniques to assess the in vivo hemodynamics and the ex vivo techniques of the isolated liver perfusion and vascular contractility.

Therapeutic Effect of Captopril, Pentoxifylline, and Cordyceps Sinensis in Pre-Hepatic Portal Hypertensive Rats

PubMed Central

Ahmed, Ahmed F.; El-Maraghy, Nabila N.; Ghaney, Rasha H. Abdel; Elshazly, Shimaa M.

2012-01-01

Background/Aim: Portal hypertension is an important and potentially fatal complication of liver disease whereby cellular and fibrotic alterations manifest to increase portal venous pressure. The aim of this study is to investigate the effect of captopril, pentoxifylline (PTX), and cordyceps sinensis in pre-hepatic portal hypertensive rats. Settings and Design: Wister male rats were divided at random into 3 main groups: the first group: control rats. The second group: sham-operated rats and the third group: prehepatic portal hypertensive rats (PHPHT) induced by regulated pre-hepatic portal vein ligation. After 14 days, Group 3 was subdivided into 5 subgroups. Subgroup (1): portal vein-ligated (PVL) was killed at once; Subgroup (2): received distilled water for 30 days (untreated PVL group); subgroups 3-5 were treated with captopril (60 mg/kg, orally); PTX (100 mg/kg, orally); and C. sinensis (200 mg/kg, orally), respectively, as a single daily dose for 30 days. Patients and Methods: Portal pressure, nitric oxide (NO), antioxidant enzymes, Liver enzymes, and creatinine levels were measured to evaluate the status of the liver state. Results: Portal vein ligation produced significant increments in liver enzymes, NO, creatinine and portal pressure concomitant with significant decrements in glutathione content and superoxide dismutase activity. Treatment with captopril, PTX, and C. sinensis resulted in a significant reduction in liver enzymes, NO, creatinine and portal pressure and observable increase in antioxidant enzymes. Conclusions: captopril, PTX, and C. sinensis have promising effect in controlling PHPHT and reducing hyperdynamic circulatory state through reduction of portal pressure and NO level. PMID:22626797

 Schistosomal portal hypertension: Randomized trial comparing endoscopic therapy alone or preceded by esophagogastric devascularization and splenectomy.

PubMed

Costa Lacet, Celina Maria; Neto, João Batista; Ribeiro, Laercio Tenório; Oliveira, Francisco Silva; Wyszomirska, Rozangela Fernandes; Strauss, Edna

2016-01-01

 Background. Upper gastrointestinal bleeding is a major cause of morbidity and mortality in patients with portal hypertension secondary to schistosomiasis mansoni. To evaluate the efficacy of combined surgery and sclerotherapy versus endoscopic treatment alone in the prophylaxis of esophageal variceal rebleeding due to portal hypertension in schistosomiasis. During a two-years period consecutive patients with schistosomiasis and a recent bleeding history were evaluated for prospective randomization. Absolute exclusion criteria were alcoholism or other liver diseases, whereas platelet count < 50,000/mm3, INR > 1.5 or presence of gastric varices were relative exclusion criteria. By random allocation 25 (group A) have received endoscopic sclerotherapy for esophageal varices alone and 22 (group B) combined treatment: esophagogastric devascularization with splenectomy followed by sclerotherapy. Interim analysis at 24 months has shown significant statistical differences between the groups and the randomization was halted. Mean age was 38.9 ± 15.4 years and 58.46% were male. Mean follow-up was 38.6 ± 20.1 months. Endoscopic comparison of the size of esophageal varices before and after treatment did not show significant differences among the two groups. Treatment efficacy was assessed by the rate of recurrent esophageal variceal bleeding, that was more common in group A- 9/25 patients (36.0%) vs. 2/22 (9.0%) in group B (p = 0.029). Other complications were odynophagia, dysphagia and esophageal ulcer in group A and ascites and portal vein thrombosis in the surgical group. In portal hypertension due to schistosomiasis, combined surgical and endoscopic treatment was more effective for the prevention of recurrent esophageal variceal bleeding.

Combination therapy using PSE and TIO ameliorates hepatic encephalopathy due to intrahepatic portosystemic venous shunt in idiopathic portal hypertension

PubMed Central

Kojima, Seiichiro; Ito, Hiroyuki; Takashimizu, Shinji; Ichikawa, Hitoshi; Matsumoto, Tomohiro; Hasebe, Terumitsu

2016-01-01

A 64-year-old woman treated for anemia and ascites exhibited hepatic encephalopathy. Abdominal ultrasonography and computed tomography (CT) showed communication between the portal vein and the middle hepatic vein, indicating an intrahepatic portosystemic venous shunt (PSS). Since hepatic encephalopathy of the patient was resistant to medical treatment, interventional radiology was performed for the treatment of shunt obliteration. Hepatic venography showed anastomosis between the hepatic vein branches, supporting the diagnosis of idiopathic portal hypertension (IPH). To minimize the increase in portal vein pressure after shunt obliteration, partial splenic artery embolization (PSE) was first performed to reduce portal vein blood flow. Transileocolic venous obliteration (TIO) was then performed, and intrahepatic PSS was successfully obliterated using coils with n-butyl-2-cyanoacrylate (NBCA). In the present case, hepatic encephalopathy due to intrahepatic PSS in the patient with IPH was successfully treated by combination therapy using PSE and TIO. PMID:27651930

Modification of splenic stiffness on acoustic radiation force impulse parallels the variation of portal pressure induced by transjugular intrahepatic portosystemic shunt.

PubMed

De Santis, Adriano; Nardelli, Silvia; Bassanelli, Chiara; Lupo, Marinella; Iegri, Claudia; Di Ciesco, Carmela Anna; Forlino, Mariana; Farcomeni, Alessio; Riggio, Oliviero

2018-03-01

Spleen and liver stiffness (LS) measured by acoustic radiation force impulse (ARFI) imaging has been shown to be useful in identifying patients with portal hypertension. The study aims to establish if the modification of portal pressure induced by a transjugular intrahepatic portosystemic shunt (TIPS) parallels the modification of spleen or LS measured by ARFI in order to understand if ARFI may be used to monitor the modification of portal pressure in patients with cirrhosis. Thirty-eight patients with severe portal hypertension underwent LS and spleen stiffness (SS) before TIPS and 1 week after TIPS. Portal atrial gradient (PAG) was measured before and after the shunt opening. Portal atrial gradient decreased significantly from 19.5 to 6 mmHg (P < 0.001). SS decreased significantly after TIPS (pre-TIPS 3.7 m/s vs post-TIPS 3. 1 m/s; P < 0.001), and LS was also significantly modified by TIPS (pre-TIPS 2.8 m/s vs post-TIPS 2.4 m/s; P = 0.003). PAG and SS values measured before and after TIPS were significantly correlated (r = 0.56; P < 0.001); on the other hand, PAG and LS were not (r = 0.19; P = 0.27). Two patients developed a persistent hepatic encephalopathy refractory to medical treatment and were submitted to the reduction of the stent diameter. The modification of SS was parallel to the modification of PAG. Spleen stiffness is superior to LS in detecting the modification of portal pressure induced by TIPS. This makes SS a potential non-invasive method to monitor the modification of portal hypertension. Further investigations are needed to establish applicability and clinical utility of this promising tool in the treatment of portal hypertension. © 2017 Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.

Idiopathic Non-Cirrhotic Intrahepatic Portal Hypertension (NCIPH)—Newer Insights into Pathogenesis and Emerging Newer Treatment Options

PubMed Central

Goel, Ashish; Elias, Joshua E.; Eapen, Chundamannil E.; Ramakrishna, Banumathi; Elias, Elwyn

2014-01-01

Chronic microangiopathy of portal venules results in idiopathic non-cirrhotic intrahepatic portal hypertension (NCIPH). Recent data suggest a role for vasoactive factors of portal venous origin in the pathogenesis of this ‘pure’ vasculopathy of the liver. Enteropathies (often silent), are an important ‘driver’ of this disease. NCIPH is under-recognized and often mis-labeled as cryptogenic cirrhosis. Liver biopsy is needed to prove the diagnosis of NCIPH. In these patients, with advancing disease and increased porto-systemic shunting, the portal venous vasoactive factors bypass the liver filter and contribute to the development of pulmonary vascular endothelial disorders—porto-pulmonary hypertension and hepato-pulmonary syndrome as well as mesangiocapillary glomerulonephritis. Prognosis in NCIPH patients is determined by presence, recognition and management of associated disorders. With better understanding of the pathogenesis of NCIPH, newer treatment options are being explored. Imbalance in ADAMTS 13 (a disintegrin and metalloprotease with thrombospondin type 1 motif, member 13): vWF (von-Willebrand factor) ratio is documented in NCIPH patients and may have a pathogenic role. Therapeutic interventions to correct this imbalance may prove to be important in the management of NCIPH. PMID:25755567

Effect of transjugular intrahepatic portosystemic shunt on pulmonary gas exchange in patients with portal hypertension and hepatopulmonary syndrome

PubMed Central

Martínez-Pallí, Graciela; Drake, Britt B; García-Pagán, Joan-Carles; Barberà, Joan-Albert; Arguedas, Miguel R; Rodriguez-Roisin, Robert; Bosch, Jaume; Fallon, Michael B

2005-01-01

AIM: To assess the impact of transjugular intrahepatic portosystemic shunt (TIPS) on pulmonary gas exchange and to evaluate the use of TIPS for the treatment of hepatopulmonary syndrome ( HPS ). METHODS: Seven patients, three of them with advanced HPS, in whom detailed pulmonary function tests were performed before and after TIPS placement at the University of Alabama Hospital and at the Hospital Clinic, Barcelona, were considered. RESULTS: TIPS patency was confirmed by hemodynamic evaluation. No changes in arterial blood gases were observed in the overall subset of patients. Transient arterial oxygenation improvement was observed in only one HPS patient, early after TIPS, but this was not sustained 4 mo later. CONCLUSION: TIPS neither improved nor worsened pulmonary gas exchange in patients with portal hypertension. This data does not support the use of TIPS as a specific treatment for HPS. However, it does reinforce the view that TIPS can be safely performed for the treatment of other complications of portal hypertension in patients with HPS. PMID:16425397

Portal hypertension and gastrointestinal bleeding: Diagnosis, prevention and management

PubMed Central

Biecker, Erwin

2013-01-01

Bleeding from esophageal varices is a life threatening complication of portal hypertension. Primary prevention of bleeding in patients at risk for a first bleeding episode is therefore a major goal. Medical prophylaxis consists of non-selective beta-blockers like propranolol or carvedilol. Variceal endoscopic band ligation is equally effective but procedure related morbidity is a drawback of the method. Therapy of acute bleeding is based on three strategies: vasopressor drugs like terlipressin, antibiotics and endoscopic therapy. In refractory bleeding, self-expandable stents offer an option for bridging to definite treatments like transjugular intrahepatic portosystemic shunt (TIPS). Treatment of bleeding from gastric varices depends on vasopressor drugs and on injection of varices with cyanoacrylate. Strategies for primary or secondary prevention are based on non-selective beta-blockers but data from large clinical trials is lacking. Therapy of refractory bleeding relies on shunt-procedures like TIPS. Bleeding from ectopic varices, portal hypertensive gastropathy and gastric antral vascular ectasia-syndrome is less common. Possible medical and endoscopic treatment options are discussed. PMID:23964137

Prevalence and Indicators of Portal Hypertension in Patients with Nonalcoholic Fatty Liver Disease

PubMed Central

Mendes, Flavia D.; Suzuki, Ayako; Sanderson, Schuyler O.; Lindor, Keith D.; Angulo, Paul

2012-01-01

Background & Aims Little is known about the prevalence and severity of portal hypertension in patients with non-alcoholic fatty liver disease (NAFLD). We investigated the prevalence and non-invasive predictors of portal hypertension in patients with NAFLD. Methods Signs of portal hypertension, including esophageal varices, splenomegaly, portosystemic encephalopathy, and ascites where investigated in 354 patients with NAFLD. Results One-hundred patients had portal hypertension at the time of NAFLD diagnosis (28.2%), 88 of these with septal fibrosis or cirrhosis (88%). Fibrosis stage correlated with presence (r=0.41, P<.0001) and number of findings (r=0.48, P=.006) of portal hypertension. Of the 204 patients with no or mild fibrosis (stages 0–2), 12 had portal hypertension (6%); they had a significantly higher grade of steatosis, based on biopsy analysis, compared to the 192 patients without portal hypertension (94%). Thrombocytopenia, hyperbilirubinemia, cirrhosis, and obesity were independently associated with portal hypertension. Esophageal varices were found in 57 of the 128 patients undergoing endoscopic screening (44.5%) and independently associated with thrombocytopenia, type 2 diabetes, and splenomegaly. Conclusions Signs of portal hypertension are present in 25% of patients at the time of diagnosis of NAFLD; most had advanced fibrosis or cirrhosis. Portal hypertension can occur in a small proportion of patients with mild or no fibrosis and is associated with the extent of steatosis. Features of advanced liver disease and insulin resistance might identify patients with NAFLD and portal hypertension, and those expected to derive the most benefit from endoscopic screening for esophageal varices. PMID:22610002

A novel canine model of portal vein stenosis plus thioacetamide administration-induced cirrhotic portal hypertension with hypersplenism.

PubMed

Lin, Dexin; Wu, Xianbin; Ji, Xiaoke; Zhang, Qiyu; Lin, YuanWei; Chen, WeiJian; Jin, Wangxun; Deng, Liming; Chen, Yunzhi; Chen, Bicheng; Li, Jianmin

2012-01-01

Current large animal models that could closely resemble the typical features of cirrhotic portal hypertension in human have not been well established. Thus, we aimed to develop and describe a reliable and reproducible canine cirrhosis model of portal hypertension. A total of 30 mongrel dogs were randomly divided into four groups: 1 (control; n = 5), 2 (portal vein stenosis [PVS]; n = 5], 3 (thioacetamide [TAA]; n = 5), and 4 (PVS plus TAA; n = 15). After 4-months modeling period, liver and spleen CT perfusion, abdominal CT scans, portal hemodynamics, gastroscopy, hepatic function, blood routine, the bone marrow, liver, and spleen histology were studied. The animals in group 2 (PVS) developed extrahepatic portosystemic collateral circulation, particularly esophageal varices, without hepatic cirrhosis and portal hypertension. Animals from group 3 (TAA) presented mild cirrhosis and portal hypertension without significant symptoms of esophageal varices and hypersplenism. In contrast, animals from group 4 (PVS + TAA) showed well-developed micronodular and macronodular cirrhosis, associated with significant portal hypertension and hypersplenism. The combination of PVS and TAA represents a novel, reliable, and reproducible canine cirrhosis model of portal hypertension, which is associated with the typical characteristics of portal hypertension, including hypersplenism.

[Portal hypertension. Evidence-based guide].

PubMed

Mercado, Miguel Angel; Orozco Zepeda, Héctor; Plata-Muñoz, Juan José

2004-01-01

Treatment of portal hypertension has evolved widely during the last decades. Advances in physiopathology have allowed better application of therapeutic options and also have permitted to know the natural history of varices and variceal bleeding, predicting which patients have a higher risk of bleeding. It also permits probability of designing patient treatment. According to liver function and subadjacent liver disease, it is possible to offer different alternatives within the three possible scenarios (primary prophylaxis, acute bleeding episode, and secondary prophylaxis). For primary prophylaxis, pharmacotherapy offers the best choice. Endoscopic banding is also growing in these scenarios and probably will be accepted in the near future. For the acute bleeding episode, endoscopic therapy (sclerosis and/or bands) and/or pharmacologic therapy (octreotide, terlipresin) represent best choice, considering TIPS as a rescue option. Surgery is not used routinely in this scenario in most centers. For secondary prophylaxis, pharmaco- and endoscopic therapy are first-line treatments, while TIPS and surgery as second-line treatments. TIPS is mainly used in patients on a waiting list for liver transplantation. Surgery offers good results for low-risk patients, with good liver function and with portal blood-flow preserving procedures (selective shunts, extensive devascularizations). Liver transplantation is recommended for patients with poor liver function because together with portal hypertension, it treats subadjacent liver disease.

Liver surgery in cirrhosis and portal hypertension.

PubMed

Hackl, Christina; Schlitt, Hans J; Renner, Philipp; Lang, Sven A

2016-03-07

The prevalence of hepatic cirrhosis in Europe and the United States, currently 250 patients per 100000 inhabitants, is steadily increasing. Thus, we observe a significant increase in patients with cirrhosis and portal hypertension needing liver resections for primary or metastatic lesions. However, extended liver resections in patients with underlying hepatic cirrhosis and portal hypertension still represent a medical challenge in regard to perioperative morbidity, surgical management and postoperative outcome. The Barcelona Clinic Liver Cancer classification recommends to restrict curative liver resections for hepatocellular carcinoma in cirrhotic patients to early tumor stages in patients with Child A cirrhosis not showing portal hypertension. However, during the last two decades, relevant improvements in preoperative diagnostic, perioperative hepatologic and intensive care management as well as in surgical techniques during hepatic resections have rendered even extended liver resections in higher-degree cirrhotic patients with portal hypertension possible. However, there are few standard indications for hepatic resections in cirrhotic patients and risk stratifications have to be performed in an interdisciplinary setting for each individual patient. We here review the indications, the preoperative risk-stratifications, the morbidity and the mortality of extended resections for primary and metastatic lesions in cirrhotic livers. Furthermore, we provide a review of literature on perioperative management in cirrhotic patients needing extrahepatic abdominal surgery and an overview of surgical options in the treatment of hepatic cirrhosis.

Variceal bleeding and portal hypertension: new lights on old horizon.

PubMed

Bhasin, D K; Siyad, I

2004-02-01

New clinical, endoscopic, and imaging modalities for diagnosing varices and predicting bleeding are being investigated. Transnasal endoscopy and ultrathin battery-powered esophagoscopes are being used to improve patient comfort and compliance. Patients who respond to portal pressure-reducing drugs not only have a reduced risk of bleeding, but also a reduced risk of developing other complications, with improved survival. Nitrates have been shown to have no definite role in primary prophylaxis against variceal bleeding. The hemodynamic response to treatment has an independent prognostic value for the risk of variceal bleeding. Newer drugs have been investigated for reducing the hepatic venous pressure gradient, but with little success. Survival after bleeding has increased due to improved patient care and technological advances. Combined radiographic and endoscopic management of gastric varices is evolving and appears to be promising. Nonvariceal bleeding from portal hypertensive gastropathy is increasingly being recognized as a potential cause of bleeding in patients with portal hypertension, and pharmacotherapy with octreotide appears to be promising for the treatment of this condition. Variceal band ligation in children has been found to be as safe and effective as in adults.

Portal hypertension: a review of portosystemic collateral pathways and endovascular interventions.

PubMed

Pillai, A K; Andring, B; Patel, A; Trimmer, C; Kalva, S P

2015-10-01

The portal vein is formed at the confluence of the splenic and superior mesenteric vein behind the head of the pancreas. Normal blood pressure within the portal system varies between 5 and 10 mmHg. Portal hypertension is defined when the gradient between the portal and systemic venous blood pressure exceeds 5 mmHg. The most common cause of portal hypertension is cirrhosis. In cirrhosis, portal hypertension develops due to extensive fibrosis within the liver parenchyma causing increased vascular resistance. In addition, the inability of the liver to metabolise certain vasodilators leads to hyperdynamic splanchnic circulation resulting in increased portal blood flow. Decompression of the portal pressure is achieved by formation of portosystemic collaterals. In this review, we will discuss the pathophysiology, anatomy, and imaging findings of spontaneous portosystemic collaterals and clinical manifestations of portal hypertension with emphasis on the role of interventional radiology in the management of complications related to portal hypertension. Copyright © 2015 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.

5 CFR 842.707 - Partial deferred payment of the lump-sum credit if annuity commences after January 3, 1988, and...

Code of Federal Regulations, 2013 CFR

2013-01-01

... hypertension with hypertensive encephalopathy. (K) Cardiac aneurysm not amenable to surgical treatment. (L) Agranulocytosis. (M) Severe hepatic failure. (N) Severe hypoxic brain damage. (O) Severe portal hypertension with...

5 CFR 842.707 - Partial deferred payment of the lump-sum credit if annuity commences after January 3, 1988, and...

Code of Federal Regulations, 2014 CFR

2014-01-01

... hypertension with hypertensive encephalopathy. (K) Cardiac aneurysm not amenable to surgical treatment. (L) Agranulocytosis. (M) Severe hepatic failure. (N) Severe hypoxic brain damage. (O) Severe portal hypertension with...

5 CFR 842.707 - Partial deferred payment of the lump-sum credit if annuity commences after January 3, 1988, and...

Code of Federal Regulations, 2012 CFR

2012-01-01

... hypertension with hypertensive encephalopathy. (K) Cardiac aneurysm not amenable to surgical treatment. (L) Agranulocytosis. (M) Severe hepatic failure. (N) Severe hypoxic brain damage. (O) Severe portal hypertension with...

5 CFR 842.707 - Partial deferred payment of the lump-sum credit if annuity commences after January 3, 1988, and...

Code of Federal Regulations, 2010 CFR

2010-01-01

... hypertension with hypertensive encephalopathy. (K) Cardiac aneurysm not amenable to surgical treatment. (L) Agranulocytosis. (M) Severe hepatic failure. (N) Severe hypoxic brain damage. (O) Severe portal hypertension with...

5 CFR 842.707 - Partial deferred payment of the lump-sum credit if annuity commences after January 3, 1988, and...

Code of Federal Regulations, 2011 CFR

2011-01-01

... hypertension with hypertensive encephalopathy. (K) Cardiac aneurysm not amenable to surgical treatment. (L) Agranulocytosis. (M) Severe hepatic failure. (N) Severe hypoxic brain damage. (O) Severe portal hypertension with...

One step minilaparotomy-assisted transmesenteric portal vein recanalization combined with transjugular intrahepatic portosystemic shunt placement: A novel surgical proposal in pediatrics

PubMed Central

Pelizzo, Gloria; Quaretti, Pietro; Moramarco, Lorenzo Paolo; Corti, Riccardo; Maestri, Marcello; Iacob, Giulio; Calcaterra, Valeria

2017-01-01

Transjugular intrahepatic portosystemic shunt (TIPS) placement is a standard procedure for the treatment of portal hypertension complications. When this conventional approach is not feasible, alternative procedures for systemic diversion of portal blood have been proposed. A one-step interventional approach, combining minilaparotomy-assisted transmesenteric (MAT) antegrade portal recanalization and TIPS, is described in an adolescent with recurrent esophageal varice bleeding and portal cavernoma (PC). A 16-year-old girl was admitted to our Unit because of repeated bleeding episodes over a short period of time due to esophageal varices in the context of a PC. A portal vein recanalization through an ileocolic vein isolation with the MAT approach followed by TIPS during the same session was performed. In the case of failed portal recanalization, this approach, would also be useful for varice endovascular embolization. Postoperative recovery was uneventful. Treatment consisting of propanolol, enoxaparin and a proton pump inhibitor was prescribed after the procedure. One month post-op, contrast enhanced computed tomography confirmed the patency of the portal and intrahepatic stent grafts. No residual peritoneal fluid was detected nor opacification of the large varices. Endoscopy showed good improvement of the varices. Doppler ultrasound confirmed the accelerated flow in the portal stent and hepatopetal flow inside the intrahepatic portal branches. Three months post-op, TIPS maintained its hourglass shape despite a slight expansion. Portal hypertension and life threatening conditions related to PC would benefit from one-step portal recanalization. MAT-TIPS is feasible and safe for the treatment of PC even in children. This minimally invasive procedure avoids or delays surgical treatment or re-transplantation when necessary in pediatric patients. PMID:28487619

Therapeutic approaches for portal biliopathy: A systematic review

PubMed Central

Franceschet, Irene; Zanetto, Alberto; Ferrarese, Alberto; Burra, Patrizia; Senzolo, Marco

2016-01-01

Portal biliopathy (PB) is defined as the presence of biliary abnormalities in patients with non-cirrhotic/non-neoplastic extrahepatic portal vein obstruction (EHPVO) and portal cavernoma (PC). The pathogenesis of PB is due to ab extrinseco compression of bile ducts by PC and/or to ischemic damage secondary to an altered biliary vascularization in EHPVO and PC. Although asymptomatic biliary abnormalities can be frequently seen by magnetic resonance cholangiopancreatography in patients with PC (77%-100%), only a part of these (5%-38%) are symptomatic. Clinical presentation includes jaundice, cholangitis, cholecystitis, abdominal pain, and cholelithiasis. In this subset of patients is required a specific treatment. Different therapeutic approaches aimed to diminish portal hypertension and treat biliary strictures are available. In order to decompress PC, surgical porto-systemic shunt or transjugular intrahepatic porto-systemic shunt can be performed, and treatment on the biliary stenosis includes endoscopic (Endoscopic retrograde cholangiopancreatography with endoscopic sphincterotomy, balloon dilation, stone extraction, stent placement) and surgical (bilioenteric anastomosis, cholecystectomy) approaches. Definitive treatment of PB often requires multiple and combined interventions both on vascular and biliary system. Liver transplantation can be considered in patients with secondary biliary cirrhosis, recurrent cholangitis or unsuccessful control of portal hypertension. PMID:28018098

Inflammation: a way to understanding the evolution of portal hypertension

PubMed Central

Aller, María-Angeles; Arias, Jorge-Luis; Cruz, Arturo; Arias, Jaime

2007-01-01

Background Portal hypertension is a clinical syndrome that manifests as ascites, portosystemic encephalopathy and variceal hemorrhage, and these alterations often lead to death. Hypothesis Splanchnic and/or systemic responses to portal hypertension could have pathophysiological mechanisms similar to those involved in the post-traumatic inflammatory response. The splanchnic and systemic impairments produced throughout the evolution of experimental prehepatic portal hypertension could be considered to have an inflammatory origin. In portal vein ligated rats, portal hypertensive enteropathy, hepatic steatosis and portal hypertensive encephalopathy show phenotypes during their development that can be considered inflammatory, such as: ischemia-reperfusion (vasodilatory response), infiltration by inflammatory cells (mast cells) and bacteria (intestinal translocation of endotoxins and bacteria) and lastly, angiogenesis. Similar inflammatory phenotypes, worsened by chronic liver disease (with anti-oxidant and anti-enzymatic ability reduction) characterize the evolution of portal hypertension and its complications (hepatorenal syndrome, ascites and esophageal variceal hemorrhage) in humans. Conclusion Low-grade inflammation, related to prehepatic portal hypertension, switches to high-grade inflammation with the development of severe and life-threatening complications when associated with chronic liver disease. PMID:17999758

Glutamine prevents gastric oxidative stress in an animal model of portal hypertension gastropathy.

PubMed

Marques, Camila; Mauriz, José L; Simonetto, Douglas; Marroni, Claudio A; Tuñon, María J; González-Gallego, Javier; Marrón, Norma P

2011-01-01

Portal hypertension (PHI) is a clinical syndrome characterized by increases of the blood flow and/or of the vascular resistance in the portal system. A direct consequence of PHI can appearance different lesions on the gastric mucosa and submucosa, cumulatively termed portal hypertensive gastropathy (PHG). To investigate the effects of glutamine on oxidative stress in an experimental model of PHG induced by partial portal vein ligation (PPVL). Portal pressure, transaminase and alkaline phosphatase activity were quantified. Gastric tissue damage was assessed by histological analysis. Oxidative stress was measured by quantification of cytosolic concentration of thiobarbituric acid reactive substances (TBARS), hydroperoxide-initiated chemiluminescence (QL), and nitric oxide (NO) production. Moreover, activities of the antioxidant enzymes superoxide dismutase (SOD), glutathione peroxidase (GPx), and catalase (CAT) were analyzed. Transaminase and alkaline phosphatase activities were not significantly modified by PPVL, indicating absence of liver injury. Histological analysis of gastric sections showed a lost of normal architecture, with edema and vasodilatation. TBARS, QL, and NO production were significantly increased in PPVL animals. A reduction of SOD activity was found. Glutamine administration markedly alleviated histological abnormalities and oxidative stress, normalized SOD activity, and blocked NO overproduction. Our results confirm that the use of molecules with antioxidant capacity can provide protection of the gastric tissue in portal hypertension. Glutamine treatment can be useful to reduce the oxidative damage induced by PHI on gastric tissue.

[Surgery in portal hypertension. Which patient and which operation?].

PubMed

Mercado, M A; Takahashi, T; Rojas, G; Prado, E; Hernández, J; Tielve, M; Orozco, H

1993-01-01

A prospective trial of a cohort of patients (N = 94) with portal hypertension and history of bleeding was selected for surgery based on strict clinical and laboratory criteria. All of them were treated with portal blood flow preserving procedures. The following selection criteria were used: good cardiopulmonary function without pulmonary hypertension and good liver function (Child-Pugh A). All patients were operated in an elective fashion and the operations performed were: selective shunts (N = 38) (distal splenorenal and splenocaval), low diameter mesocaval shunts (N = 13) and the esophagogastric devascularization with esophageal transection (Sugiura-Futagawa) (N = 43). Patients were selected for each operation according to their anatomical conditions. Sixty-one of the patients were cirrhotics. Operative mortality was 8% and rebleeding was observed in 5% of the cases. Postoperative encephalopathy was seen in seven patients (three selective shunts, two low diameter mesocaval shunts and two devascularizations). In 13 of 62 patients postoperatively evaluated by means of angiography, portal vein thrombosis was shown (seven selective shunts, two low diameter shunts and four devascularizations). Twenty-two patients with preoperative portal vein thrombosis (and treated with a Sugiura-Futagawa operation) were excluded from postoperative angiographic evaluation. Survival (Kaplan-Meier) was 85% at 60 months. Portal blood flow preserving procedures are the treatment of choice for patients with hemorrhagic portal hypertension and good liver function. The kind of operation is selected according to the individual anatomical status of the patient.

Obliterative portal venopathy without portal hypertension: an underestimated condition.

PubMed

Guido, Maria; Sarcognato, Samantha; Sonzogni, Aurelio; Lucà, Maria Grazia; Senzolo, Marco; Fagiuoli, Stefano; Ferrarese, Alberto; Pizzi, Marco; Giacomelli, Luciano; Colloredo, Guido

2016-03-01

Obliterative portal venopathy without portal hypertension has been described by a single study in a limited number of patients, thus very little is known about this clinical condition. This study aimed to investigate the prevalence of obliterative portal venopathy and its clinical-pathological correlations in patients with cryptogenic chronic liver test abnormalities without clinical signs of portal hypertension. We analysed 482 liver biopsies from adults with non-cirrhotic cryptogenic chronic liver disorders and without any clinical signs of portal hypertension, consecutively enrolled in a 5-year period. Twenty cases of idiopathic non-cirrhotic portal hypertension diagnosed in the same period, were included for comparison. Histological findings were matched with clinical and laboratory features. Obliterative portal venopathy was identified in 94 (19.5%) of 482 subjects and in all 20 cases of idiopathic non-cirrhotic portal hypertension: both groups shared the entire spectrum of histological changes described in the latter condition. The prevalence of incomplete fibrous septa and nodular regenerative hyperplasia was higher in the biopsies of idiopathic non-cirrhotic portal hypertension (P = 0.006 and P = 0.002), a possible hint of a more advanced stage of the disease. The two groups also shared several clinical laboratory features, including a similar liver function test profile, concomitant prothrombotic conditions and extrahepatic autoimmune disorders. Obliterative portal venopathy occurs in a substantial proportion of patients with unexplained chronic abnormal liver function tests without portal hypertension. The clinical-pathological profile of these subjects suggests that they may be in an early (non-symptomatic) stage of idiopathic non-cirrhotic portal hypertension. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Management of small hepatocellular carcinoma in cirrhosis: Focus on portal hypertension

PubMed Central

Hernandez-Gea, Virginia; Turon, Fanny; Berzigotti, Annalisa; Villanueva, Augusto

2013-01-01

The incidence of hepatocellular carcinoma (HCC) is rising worldwide being currently the fifth most common cancer and third cause of cancer-related mortality. Early detection of HCC through surveillance programs have enabled the identification of small nodules with higher frequency, and nowadays account for 10%-15% of patients diagnosed in the West and almost 30% in Japan. Patients with small HCC can be candidates for potential curative treatments: liver transplantation, surgical resection and percutaneous ablation, depending on the presence of portal hypertension and co-morbidities. This review will analyze recent advancements in the clinical management of these individuals, focusing on issues related to the role of portal hypertension, the debate between resection and ablative therapies and the future impact of molecular technologies. PMID:23482437

Management of Portal Hypertension After Liver Transplantation.

PubMed

Korda, D; Deák, P Á; Kiss, G; Gerlei, Z; Kóbori, L; Görög, D; Fehérvári, I; Piros, L; Máthé, Z; Doros, A

2017-09-01

Post-transplantation portal hypertension has severe complications, such as esophageal varix bleeding, therapy refractory ascites, extreme splenomegaly, and graft dysfunction. The aim of our study was to analyze the effectiveness of the therapeutic strategies and how to visualize the procedure. A retrospective study involving liver transplantation patients from the Semmelweis University Department of Transplantation and Surgery was performed between 2005 and 2015. The prevalence, etiology, and leading complications of the condition were determined. The applied interventions' effects on the patients' ascites volume, splenic volume, and the occurrence of variceal bleeding were determined. Mean portal blood flow velocity and congestion index values were calculated using Doppler ultrasonography. The prevalence of post-transplantation portal hypertension requiring intervention was 2.8%. The most common etiology of the disease was portal anastomotic stenosis. The most common complications were esophageal varix bleeding and therapy refractory ascites. The patients' ascites volume decreased significantly (2923.3 ± 1893.2 mL vs. 423.3 ± 634.3 mL; P < .05), their splenic volume decreased markedly. After the interventions, only one case of recurrent variceal bleeding was reported. The calculated Doppler parameters were altered in the opposite direction in cases of pre-hepatic versus intra- or post-hepatic portal hypertension. After the interventions, these parameters shifted towards the physiologic ranges. The interventions performed in our clinic were effective in most cases. The patients' ascites volume, splenic volume, and the prevalence of variceal bleeding decreased after the treatment. Doppler ultrasonography has proved to be a valuable imaging modality in the diagnosis and the follow-up of post-transplantation portal hypertension. Copyright © 2017 Elsevier Inc. All rights reserved.

Treatment of hepatocellular carcinoma with portal vein tumor thrombus: advances and challenges.

PubMed

Jiang, Jin-Fang; Lao, Yong-Cong; Yuan, Bao-Hong; Yin, Jun; Liu, Xin; Chen, Long; Zhong, Jian-Hong

2017-05-16

Portal vein tumor thrombus is a frequent, challenging complication in hepatocellular carcinoma. Hepatocellular carcinoma patients with portal vein tumor thrombus may show worse liver function, less treatment tolerance and worse prognosis than patients without portal vein tumor thrombus, and they may be at higher risk of comorbidity related to portal hypertension. Western and some Asian guidelines stratify hepatocellular carcinoma with portal vein tumor thrombus together with metastatic hepatocellular carcinoma and therefore recommend only palliative treatment with sorafenib or other systemic agents. In recent years, more treatment options have become available for hepatocellular carcinoma patients with portal vein tumor thrombus, and an evidence-based approach to optimizing disease management and treatment has become more widespread. Nevertheless, consensus policies for managing hepatocellular carcinoma with portal vein tumor thrombus have not been established. This comprehensive literature review, drawing primarily on studies published after 2010, examines currently available management options for patients with hepatocellular carcinoma and portal vein tumor thrombus.

Pathology of idiopathic non-cirrhotic portal hypertension.

PubMed

Guido, Maria; Sarcognato, Samantha; Sacchi, Diana; Colloredo, Guido

2018-04-12

Idiopathic non-cirrhotic portal hypertension is an under-recognized vascular liver disease of unknown etiology, characterized by clinical signs of portal hypertension in the absence of cirrhosis. By definition, any disorder known to cause portal hypertension in the absence of cirrhosis and any cause of chronic liver disease must be excluded to make a diagnosis of idiopathic non-cirrhotic portal hypertension. However, the diagnosis is often difficult because the disease resembles cirrhosis and there is no gold standard test. Liver biopsy is an essential tool: it is able to exclude cirrhosis and other causes of portal hypertension and it allows the identification of the characteristic lesions. Nonetheless, the histological diagnosis of idiopathic non-cirrhotic portal hypertension is not always straightforward, in particular by needle biopsy samples, because there is no pathognomonic lesion, but rather a variety of vascular changes which are unevenly distributed, very subtle, and not all necessarily identified in a single specimen. Pathologists should be able to recognize several patterns of injury, involving portal/periportal areas as well as parenchymal structures.The histological features of idiopathic non-cirrhotic portal hypertension are described in this review, focusing on their interpretation in needle biopsy specimens.

The FXR agonist PX20606 ameliorates portal hypertension by targeting vascular remodelling and sinusoidal dysfunction.

PubMed

Schwabl, Philipp; Hambruch, Eva; Seeland, Berit A; Hayden, Hubert; Wagner, Michael; Garnys, Lukas; Strobel, Bastian; Schubert, Tim-Lukas; Riedl, Florian; Mitteregger, Dieter; Burnet, Michael; Starlinger, Patrick; Oberhuber, Georg; Deuschle, Ulrich; Rohr-Udilova, Nataliya; Podesser, Bruno K; Peck-Radosavljevic, Markus; Reiberger, Thomas; Kremoser, Claus; Trauner, Michael

2017-04-01

Steroidal farnesoid X receptor (FXR) agonists demonstrated potent anti-fibrotic activities and lowered portal hypertension in experimental models. The impact of the novel non-steroidal and selective FXR agonist PX20606 on portal hypertension and fibrosis was explored in this study. In experimental models of non-cirrhotic (partial portal vein ligation, PPVL, 7days) and cirrhotic (carbon tetrachloride, CCl 4 , 14weeks) portal hypertension, PX20606 (PX,10mg/kg) or the steroidal FXR agonist obeticholic acid (OCA,10mg/kg) were gavaged. We then measured portal pressure, intrahepatic vascular resistance, liver fibrosis and bacterial translocation. PX decreased portal pressure in non-cirrhotic PPVL (12.6±1.7 vs. 10.4±1.1mmHg; p=0.020) and cirrhotic CCl 4 (15.2±0.5 vs. 11.8±0.4mmHg; p=0.001) rats. In PPVL animals, we observed less bacterial translocation (-36%; p=0.041), a decrease in lipopolysaccharide binding protein (-30%; p=0.024) and splanchnic tumour necrosis factor α levels (-39%; p=0.044) after PX treatment. In CCl 4 rats, PX decreased fibrotic Sirius Red area (-43%; p=0.005), hepatic hydroxyproline (-66%; p<0.001), and expression of profibrogenic proteins (Col1a1, α smooth muscle actin, transforming growth factor β). CCl 4 -PX rats had significantly lower transaminase levels and reduced hepatic macrophage infiltration. Moreover, PX induced sinusoidal vasodilation (upregulation of cystathionase, dimethylaminohydrolase (DDAH)1, endothelial nitric oxide synthase (eNOS), GTP-cyclohydrolase1) and reduced intrahepatic vasoconstriction (downregulation of endothelin-1, p-Moesin). In cirrhosis, PX improved endothelial dysfunction (decreased von-Willebrand factor) and normalized overexpression of vascular endothelial growth factor, platelet-derived growth factor and angiopoietins. While short-term 3-day PX treatment reduced portal pressure (-14%; p=0.041) by restoring endothelial function, 14week PX therapy additionally inhibited sinusoidal remodelling and decreased portal pressure to a greater extent (-22%; p=0.001). In human liver sinusoidal endothelial cells, PX increased eNOS and DDAH expression. The non-steroidal FXR agonist PX20606 ameliorates portal hypertension by reducing liver fibrosis, vascular remodelling and sinusoidal dysfunction. The novel drug PX20606 activates the bile acid receptor FXR and shows beneficial effects in experimental liver cirrhosis: In the liver, it reduces scarring and inflammation, and also widens blood vessels. Thus, PX20606 leads to an improved blood flow through the liver and decreases hypertension of the portal vein. Additionally, PX20606 improves the altered intestinal barrier and decreases bacterial migration from the gut. Copyright © 2016 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.

Liver surgery in cirrhosis and portal hypertension

PubMed Central

Hackl, Christina; Schlitt, Hans J; Renner, Philipp; Lang, Sven A

2016-01-01

The prevalence of hepatic cirrhosis in Europe and the United States, currently 250 patients per 100000 inhabitants, is steadily increasing. Thus, we observe a significant increase in patients with cirrhosis and portal hypertension needing liver resections for primary or metastatic lesions. However, extended liver resections in patients with underlying hepatic cirrhosis and portal hypertension still represent a medical challenge in regard to perioperative morbidity, surgical management and postoperative outcome. The Barcelona Clinic Liver Cancer classification recommends to restrict curative liver resections for hepatocellular carcinoma in cirrhotic patients to early tumor stages in patients with Child A cirrhosis not showing portal hypertension. However, during the last two decades, relevant improvements in preoperative diagnostic, perioperative hepatologic and intensive care management as well as in surgical techniques during hepatic resections have rendered even extended liver resections in higher-degree cirrhotic patients with portal hypertension possible. However, there are few standard indications for hepatic resections in cirrhotic patients and risk stratifications have to be performed in an interdisciplinary setting for each individual patient. We here review the indications, the preoperative risk-stratifications, the morbidity and the mortality of extended resections for primary and metastatic lesions in cirrhotic livers. Furthermore, we provide a review of literature on perioperative management in cirrhotic patients needing extrahepatic abdominal surgery and an overview of surgical options in the treatment of hepatic cirrhosis. PMID:26973411

Endoscopic treatments for portal hypertension.

PubMed

Lo, Gin-Ho

2018-02-01

Acute esophageal variceal hemorrhage is a dreaded complication of portal hypertension. Its management has evolved rapidly in recent years. Endoscopic therapy is often employed to arrest bleeding varices as well as to prevent early rebleeding. The combination of vasoconstrictor and endoscopic therapy is superior to vasoconstrictor or endoscopic therapy alone for control of acute esophageal variceal hemorrhage. After control of acute variceal bleeding, combination of banding ligation and beta-blockers is generally recommended to prevent variceal rebleeding. To prevent the catastrophic event of acute variceal bleeding, endoscopic banding ligation is an important tool in the prophylaxis of first bleeding. Endoscopic obturation with cyanoacrylate is usually utilized to arrest acute gastric variceal hemorrhage as well as to prevent rebleeding. It can be concluded that endoscopic therapies play a pivotal role in management of portal hypertensive bleeding.

Hand-assisted laparoscopic Hassab's procedure for esophagogastric varices with portal hypertension.

PubMed

Kobayashi, Takashi; Miura, Kohei; Ishikawa, Hirosuke; Soma, Daiki; Zhang, Zhengkun; Ando, Takuya; Yuza, Kizuki; Hirose, Yuki; Katada, Tomohiro; Takizawa, Kazuyasu; Nagahashi, Masayuki; Sakata, Jun; Kameyama, Hitoshi; Wakai, Toshifumi

2017-10-23

Laparoscopic surgery for patients with portal hypertension is considered to be contraindicated because of the high risk of massive intraoperative hemorrhaging. However, recent reports have shown hand-assisted laparoscopic surgery for devascularization and splenectomy to be a safe and effective method of treating esophagogastric varices with portal hypertension. The aim of this study is to evaluate the efficacy of hand-assisted laparoscopic devascularization and splenectomy (HALS Hassab's procedure) for the treatment of esophagogastric varices with portal hypertension. From 2009 to 2016, seven patients with esophagogastric varices with portal hypertension were treated with hand-assisted laparoscopic devascularization and splenectomy in our institute. Four men and three women with a median age of 61 years (range 35-71) were enrolled in this series. We retrospectively reviewed the medical records for the perioperative variables, postoperative mortality and morbidity, and postoperative outcomes of esophagogastric varices. The median operative time was 455 (range 310-671) min. The median intraoperative blood loss was 695 (range 15-2395) ml. The median weight of removed spleen was 507 (range 242-1835) g. The conversion rate to open surgery was 0%. The median postoperative hospital stay was 21 (range 13-81) days. During a median 21 (range 3-43) months of follow-up, the mortality rate was 0%. Four postoperative complications (massive ascites, enteritis, intra-abdominal abscess, and intestinal ulcer) were observed in two patients. Those complications were treated successfully without re-operation. Esophagogastric varices in all patients disappeared or improved. Bleeding from esophagogastric varices was not observed during the follow-up period. Although our data are preliminary, hand-assisted laparoscopic devascularization and splenectomy proved an effective procedure for treating esophagogastric varices in patients with portal hypertension.

[Gastric vascular lesions in cirrhosis: gastropathy and antral vascular ectasia].

PubMed

Casas, Meritxell; Calvet, Xavier; Vergara, Mercedes; Bella, Maria Rosa; Junquera, Félix; Martinez-Bauer, Eva; Campo, Rafael

2015-02-01

Portal hypertensive gastropathy (GHP) is a complication of portal hypertension usually associated with liver cirrhosis. The pathogenesis is unclear but the presence of portal hypertension is an essential factor for its development. GHP may be asymptomatic or present as gastrointestinal bleeding or iron deficiency anemia. Endoscopic lesions vary from a mosaic pattern to diffuse red spots; the most common location is the fundus. Treatment is indicated when there is acute or chronic bleeding, as secondary prophylaxis. There is insufficient evidence to recommend primary prophylaxis in patients who have never bled. Drugs that decrease portal pressure, such as non-cardioselective beta-blockers, and/or endoscopic ablative treatments, such as argon-beam coagulation, may be used. The role of transarterial intrahepatic portosystemic shunt) or bypass surgery has been insufficiently analyzed. Antral vascular ectasia (EVA) is a rare entity in liver cirrhosis, whose pathophysiology is still unknown. Clinical presentation is similar to that of GHP and endoscopy usually shows red spots in the antrum. Biopsy is often required to differentiate EVA from GHP. There is no effective medical therapy, so endoscopic ablative therapy and, in severe cases, antrectomy are recommended. Copyright © 2014 Elsevier España, S.L.U. and AEEH y AEG. All rights reserved.

Nitro-oxidative stress, VEGF and MMP-9 in patients with cirrhotic and non-cirrhotic portal hypertension.

PubMed

Muti, Leon Adrian; Pârvu, Alina Elena; Crăciun, Alexandra M; Miron, Nicolae; Acalovschi, Monica

2015-01-01

Nitro-oxidative stress may have pathophysiological consequences. The study aimed to assess the nitro-oxidative stress, the vascular growth factor, and metalloproteinase-9 levels in patients with noncirrohic and cirrhotic portal hypertension. Patients with noncirrhotic portal hypertension (n=50) and cirrhotic portal hypertension (n=50) from the 3rd Medical Clinic in Cluj-Napoca Romania were prospectively enrolled between October 2004 and October 2006. A control group of healthy volunteers (n=50) was also evaluated. Nitro-oxidative stress was assessed by measuring serum concentration of nitrites and nitrate, 3-nitrotyrosine, total oxidative status, total antioxidant reactivity, and oxidative stress index. Serum vascular growth factor and matrix metalloproteinase-9 were also determined. Serum nitrites and nitrate levels significantly increased in both noncirrhotic (p<0.001) and cirrhotic portal hypertension (p=0.057). 3-nitrotyrosine also increased in noncirrhotic (p=0.001) and cirrhotic portal hypertension patients (p=0.014). Total oxidative status showed a significant increase in noncirrhotic (p<0.001) and in cirrhotic portal hypertension (p<0.001), but total antioxidant reactivity did not change significantly. The oxidative stress index increased in both noncirrhotic (p <0.001) and cirrhotic portal hypertension (p<0.001), as well as the serum vascular growth factor (p=0.005 and p=0.01, respectively). In NCPHT patients serum MMP-9 was significantly lower than in the healthy controls (p=0.03) and CPHT patients (p=0.05). In patients with noncirrhotic and cirrhotic portal hypertension a significant systemic nitro-oxidative stress was found, correlated with an increase of VEGF. MMP-9 decreased in noncirrhotic portal hypertension.

Pulmonary hypertension in patients with hepatic cirrhosis and portal hypertension. An echographic study.

PubMed

Gurghean, Adriana V; Tudor, Ioana A

2017-01-01

The aim of the study is to determine the frequency of pulmonary hypertension in patients with hepatic cirrhosis and portal hypertension, to determine the possibility of an accurate ultrasound diagnosis of the characteristics of this complication. 347 patients with liver cirrhosis consecutively hospitalized at Coltea Clinical Hospital were screened. 61 were excluded because of other possible causes of portal or pulmonary hypertension. All patients were investigated clinically and by abdominal and cardiac ultrasonography. Of the remaining 286 patients, 116 had portal hypertension, 27 of them (23%) having pulmonary hypertension. In this group we found a higher cardiac index and right atrial volume, higher pressures in the right atrium, suggesting a hyperdynamic state. Porto-pulmonary hypertension was found in only one patient. Echocardiography permits characterization of patients with cirrhosis and portal hypertension.

Apelin signaling modulates splanchnic angiogenesis and portosystemic collateral vessel formation in rats with portal hypertension.

PubMed

Tiani, Carolina; Garcia-Pras, Ester; Mejias, Marc; de Gottardi, Andrea; Berzigotti, Annalisa; Bosch, Jaime; Fernandez, Mercedes

2009-02-01

Angiogenesis is a pathological hallmark of portal hypertension. Although VEGF is considered to be the most important proangiogenic factor in neoangiogenesis, this process requires the coordinated action of a variety of factors. Identification of novel molecules involved in angiogenesis is highly relevant, since they may represent potential new targets to suppress pathological neovascularization in angiogenesis-related diseases like portal hypertension. The apelin/APJ signaling pathway plays a crucial role in angiogenesis. Therefore, we determined whether the apelin system modulates angiogenesis-driven processes in portal hypertension. Partial portal vein-ligated rats were treated with the APJ antagonist F13A for seven days. Splanchnic neovascularization and expression of angiogenesis mediators (Western blotting) was determined. Portosystemic collateral formation (microspheres), and hemodynamic parameters (flowmetry) were also assessed. Apelin and its receptor APJ were overexpressed in the splanchnic vasculature of portal hypertensive rats. F13A effectively decreased, by 52%, splanchnic neovascularization and expression of proangiogenic factors VEGF, PDGF and angiopoietin-2 in portal hypertensive rats. F13A also reduced, by 35%, the formation of portosystemic collateral vessels. This study provides the first experimental evidence showing that the apelin/APJ system contributes to portosystemic collateralization and splanchnic neovascularization in portal hypertensive rats, presenting a potential novel therapeutic target for portal hypertension.

Relevance of plasma malondialdehyde level and severity of portal hypertension in cirrhotic patients.

PubMed

Wang, Sheng-Lan; Zhu, Xin-Yan; Zhang, Dong-Wei; Zhang, Zhao-Jie; Gao, Heng-Jun; Yang, Chang-Qing

2015-01-01

Portal hypertension is one of the death reasons for the liver cirrhosis patients. The oxidative stress is related to the occurrence and development of portal hypertension in cirrhosis. Malondialdehyde (MDA), one of the lipid peroxides, increases substantially in cirrhotic patients. To evaluate the relevance between the MDA level and portal hypertension in cirrhotic patients. 60 liver cirrhotic patients and 30 healthy controls were enrolled. The plasma MDA level and general blood tests including ALT, AST, ALB, total bilirubin, and platelet were measured. All people enrolled accepted endoscopic examination and B-Ultrasound check to evaluate the severity of portal hypertension. The MDA plasma level of cirrhotic patients was significantly higher than the controls (P<0.001) and increased significantly accompanied by the severity of liver fibrosis and portal hypertension (P<0.01). Further, the plasma MDA level of cirrhotic patients was significantly correlated with Child-Pugh classification of cirrhosis (r=0.820, P<0.001), the degree of esophageal varices (r=0.857, P<0.001) and the width of portal vein (r=0.652, P<0.001). The ROC curve analyses showed that the plasma MDA level is a strong predictor of liver cirrhosis and portal hypertension. Plasma MDA level may correlate with the severity of portal hypertension in cirrhotic patients.

Rational classification of portal vein thrombosis and its clinical significance.

PubMed

Ma, Jingqin; Yan, Zhiping; Luo, Jianjun; Liu, Qingxin; Wang, Jianhua; Qiu, Shijing

2014-01-01

Portal vein thrombosis (PVT) is commonly classified into acute (symptom duration <60 days and absence of portal carvernoma and portal hypertension) and chronic types. However, the rationality of this classification has received little attention. In this study, 60 patients (40 men and 20 women) with PVT were examined using contrast-enhanced computed tomography (CT). The percentage of vein occlusion, including portal vein (PV) and superior mesenteric vein (SMV), was measured on CT image. Of 60 patients, 17 (28.3%) met the criterion of acute PVT. Symptoms occurred more frequently in patients with superior mesenteric vein thrombosis (SMVT) compared to those without SMVT (p<0.001). However, there was no significant difference in PV occlusion between patients with and without symptoms. The frequency of cavernous transformation was significantly higher in patients with complete PVT than those with partial PVT (p<0.001). Complications of portal hypertension were significantly associated with cirrhosis (p<0.001) rather than with the severity of PVT and presence of cavernoma. These results suggest that the severity of PVT is only associated with the formation of portal cavernoma but unrelated to the onset of symptoms and the development of portal hypertension. We classified PVT into complete and partial types, and each was subclassified into with and without portal cavernoma. In conclusion, neither symptom duration nor cavernous transformation can clearly distinguish between acute and chronic PVT. The new classification system can determine the pathological alterations of PVT, patency of portal vein and outcome of treatment in a longitudinal study.

Rational Classification of Portal Vein Thrombosis and Its Clinical Significance

PubMed Central

Ma, Jingqin; Yan, Zhiping; Luo, Jianjun; Liu, Qingxin; Wang, Jianhua; Qiu, Shijing

2014-01-01

Portal vein thrombosis (PVT) is commonly classified into acute (symptom duration <60 days and absence of portal carvernoma and portal hypertension) and chronic types. However, the rationality of this classification has received little attention. In this study, 60 patients (40 men and 20 women) with PVT were examined using contrast-enhanced computed tomography (CT). The percentage of vein occlusion, including portal vein (PV) and superior mesenteric vein (SMV), was measured on CT image. Of 60 patients, 17 (28.3%) met the criterion of acute PVT. Symptoms occurred more frequently in patients with superior mesenteric vein thrombosis (SMVT) compared to those without SMVT (p<0.001). However, there was no significant difference in PV occlusion between patients with and without symptoms. The frequency of cavernous transformation was significantly higher in patients with complete PVT than those with partial PVT (p<0.001). Complications of portal hypertension were significantly associated with cirrhosis (p<0.001) rather than with the severity of PVT and presence of cavernoma. These results suggest that the severity of PVT is only associated with the formation of portal cavernoma but unrelated to the onset of symptoms and the development of portal hypertension. We classified PVT into complete and partial types, and each was subclassified into with and without portal cavernoma. In conclusion, neither symptom duration nor cavernous transformation can clearly distinguish between acute and chronic PVT. The new classification system can determine the pathological alterations of PVT, patency of portal vein and outcome of treatment in a longitudinal study. PMID:25393320

Biology of portal hypertension.

PubMed

McConnell, Matthew; Iwakiri, Yasuko

2018-02-01

Portal hypertension develops as a result of increased intrahepatic vascular resistance often caused by chronic liver disease that leads to structural distortion by fibrosis, microvascular thrombosis, dysfunction of liver sinusoidal endothelial cells (LSECs), and hepatic stellate cell (HSC) activation. While the basic mechanisms of LSEC and HSC dysregulation have been extensively studied, the role of microvascular thrombosis and platelet function in the pathogenesis of portal hypertension remains to be clearly characterized. As a secondary event, portal hypertension results in splanchnic and systemic arterial vasodilation, leading to the development of a hyperdynamic circulatory syndrome and subsequently to clinically devastating complications including gastroesophageal varices and variceal hemorrhage, hepatic encephalopathy from the formation of portosystemic shunts, ascites, and renal failure due to the hepatorenal syndrome. This review article discusses: (1) mechanisms of sinusoidal portal hypertension, focusing on HSC and LSEC biology, pathological angiogenesis, and the role of microvascular thrombosis and platelets, (2) the mesenteric vasculature in portal hypertension, and (3) future directions for vascular biology research in portal hypertension.

Correlation Between Severity Of Portal Hypertensive Gastropathy And Size Of Oesophageal Varices In Cirrhotic Hepatitis-C Patients.

PubMed

Saleem, Khurram; Baig, Faisal Amin; Nida, Mahwish; Javed, Munaza

2018-01-01

Portal hypertension can lead to oesophageal varices (EV) and portal hypertensive gastropathy (PHG). The aim of this study is to determine the relationship between severity of Portal hypertensive gastropathy and size of oesophageal varices. One hundred and ninety-five patients of hepatitis C positive chronic liver disease having oesophageal varices were assessed for severity of portal hypertensive gastropathy. Mild Portal Hypertensive Gastropathy was observed in 16 (8.2 %), moderate in 54 (27.7 %) and severe in 120 (61.6 %) patients. Grade 1 Oesophageal Varices were present in 79 (40.5%) patients, grade 2 in 44 (21.9%) patients, grade 3 in 62 (31.8%) and grade 4 in 10 (5.2%) patients. No significant correlation was observed between grades of gastropathy and size of varices. The frequency of portal hypertensive gastropathy was 97.5% in Hepatitis C positive cirrhotic patients having oesophageal varices. Severity of gastropathy is not related to the grade or size of oesophageal varices.

Very Early Presentation of Extrahepatic Portal Vein Obstruction Causing Portal Hypertension in an Infant: Uncertainties in the Management and Therapeutic Limitations

PubMed Central

Khodayar-Pardo, Parisá; Peña Aldea, Andrés; Ramírez González, Ana; Meseguer Carrascosa, Adela; Calabuig Bayo, Cristina

2016-01-01

Extrahepatic portal vein obstruction, although rare in children, is a significant cause of portal hypertension (PHT) leading to life-threatening gastrointestinal bleeding in the pediatric age group. PHT may also lead to other complications such as hyperesplenism, cholangyopathy, ascites, and even hepatopulmonary syndrome and portopulmonary hypertension that may require organ transplantation. Herein we report the case of an asymptomatic 11-month-old infant wherein a hepatomegaly and cavernous transformation of the portal vein was detected by liver ultrasound. Neither signs of thrombosis in arteriovenous system, nor affectation of biliary tract were identified in the magnetic resonance imaging study. A significant enlargement of the caudate lobe of the liver was reported. No risk factors were detected. The differential diagnosis performed was extensive. Inherited thrombophilia and storage disorders were especially considered. Liver biopsy was normal. Upper gastrointestinal esophagogastroduodenoscopy detected two small varicose cords on the distal third of the esophagus. Finding a cavernous transformation of the portal vein with evidence of collateral circulation in such an early age is a challenging condition for professionals, since PHT may lead to severe complications during childhood and can compromise growth and development. Evidence-based guidelines for the management of PHT in adults have been published. However, follow-up and treatment of pediatric patients have not yet been standardized. Moreover, management of PHT in infants faces particular difficulties such as technical restrictions that could hinder their treatment. PMID:27504083

Giant Intrahepatic Portal Vein Aneurysm: Leave it or Treat it?

PubMed

Shrivastava, Amit; Rampal, Jagdeesh S; Nageshwar Reddy, D

2017-03-01

Portal vein aneurysm (PVA) is a rare vascular dilatation of the portal vein. It is a rare vascular anomaly representing less than 3% of all visceral aneurysms and is not well understood. Usually, PVA are incidental findings, are asymptomatic, and clinical symptoms are proportionally related to size. Patients present with nonspecific epigastric pain or gastrointestinal bleeding with underlying portal hypertension. PVA may be associated with various complications such as biliary tract compression, portal vein thrombosis/rupture, duodenal compression, gastrointestinal bleeding, and inferior vena cava obstruction. Differential diagnoses of portal vein aneurysms are solid, cystic, and hypervascular abdominal masses, and it is important that the radiologists be aware of their multi-modality appearance; hence, the aim of this article was to provide an overview of the available literature to better simplify various aspects of this rare entity and diagnostic appearance on different modality with available treatment options. In our case, a 55-year-old male patient came to the gastroenterology OPD for further management of pancreatitis with portal hypertension and biliary obstruction with plastic stents in CBD and PD for the same. In this article, we have reported a case of largest intrahepatic portal vein aneurysm and its management by endovascular technique. As per our knowledge, this is the largest intrahepatic portal vein aneurysm and first case where the endovascular technique was used for the treatment of the same.

Murine study of portal hypertension associated endothelin-1 hypo-response.

PubMed

Theodorakis, Nicholas; Maluccio, Mary; Skill, Nicholas

2015-04-28

To investigate endothelin-1 hypo-responsive associated with portal hypertension in order to improve patient treatment outcomes. Wild type, eNOS(-/-) and iNOS(-/-) mice received partial portal vein ligation surgery to induce portal hypertension or sham surgery. Development of portal hypertension was determined by measuring the splenic pulp pressure, abdominal aortic flow and portal systemic shunting. To measure splenic pulp pressure, a microtip pressure transducer was inserted into the spleen pulp. Abdominal aortic flow was measured by placing an ultrasonic Doppler flow probe around the abdominal aorta between the diaphragm and celiac artery. Portal systemic shunting was calculated by injection of fluorescent microspheres in to the splenic vein and determining the percentage accumulation of spheres in liver and pulmonary beds. Endothelin-1 hypo-response was evaluated by measuring the change in abdominal aortic flow in response to endothelin-1 intravenous administration. In addition, thoracic aorta endothelin-1 contraction was measured in 5 mm isolated thoracic aorta rings ex-vivo using an ADI small vessel myograph. In wild type and iNOS(-/-) mice splenic pulp pressure increased from 7.5 ± 1.1 mmHg and 7.2 ± 1 mmHg to 25.4 ± 3.1 mmHg and 22 ± 4 mmHg respectively. In eNOS(-/-) mice splenic pulp pressure was increased after 1 d (P = NS), after which it decreased and by 7 d was not significantly elevated when compared to 7 d sham operated controls (6.9 ± 0.6 mmHg and 7.3 ± 0.8 mmHg respectively, P = 0.3). Abdominal aortic flow was increased by 80% and 73% in 7 d portal vein ligated wild type and iNOS when compared to shams, whereas there was no significant difference in 7 d portal vein ligated eNOS(-/-) mice when compared to shams. Endothelin-1 induced a rapid reduction in abdominal aortic blood flow in wild type, eNOS(-/-) and iNOS(-/-) sham mice (50% ± 8%, 73% ± 9% and 47% ± 9% respectively). Following portal vein ligation endothelin-1 reduction in blood flow was significantly diminished in each mouse group. Abdominal aortic flow was reduced by 19% ± 9%, 32% ± 10% and 9% ± 9% in wild type, eNOS(-/-) and iNOS(-/-) mice respectively. Aberrant endothelin-1 response in murine portal hypertension is NOS isoform independent. Moreover, portal hypertension in the portal vein ligation model is independent of ET-1 function.

Infection as a Trigger for Portal Hypertension.

PubMed

Steib, Christian J; Schewe, Julia; Gerbes, Alexander L

2015-01-01

Microbial infections are a relevant problem for patients with liver cirrhosis. Different types of bacteria are responsible for different kinds of infections: Escherichia coli and Klebsiella pneumoniae are frequently observed in spontaneous bacterial peritonitis or urinary tract infections, and Streptococcus pneumoniae and Mycoplasma pneumoniae in pulmonary infections. Mortality is up to 4-fold higher in infected patients with liver cirrhosis than in patients without infections. Key Messages: Infections in patients with liver cirrhosis are due to three major reasons: bacterial translocation, immune deficiency and an increased incidence of systemic infections. Nonparenchymal liver cells like Kupffer cells, sinusoidal endothelial cells and hepatic stellate cells are the first liver cells to come into contact with microbial products when systemic infection or bacterial translocation occurs. Kupffer cell (KC) activation by Toll-like receptor (TLR) agonists and endothelial sinusoidal dysfunction have been shown to be important mechanisms increasing portal pressure following intraperitoneal lipopolysaccharide pretreatment in cirrhotic rat livers. Reduced intrahepatic vasodilation and increased intrahepatic vasoconstriction are the relevant pathophysiological pathways. Thromboxane A2 and leukotriene (LT) C4/D4 have been identified as important vasoconstrictors. Accordingly, treatment with montelukast to inhibit the cysteinyl-LT1 receptor reduced portal pressure in cirrhotic rat livers. Clinical studies have demonstrated that activation of KCs, estimated by the amount of soluble CD163 in the blood, correlates with the risk for variceal bleeding. Additionally, intestinal decontamination with rifaximin in patients with alcohol-associated liver cirrhosis reduced the portal pressure and the risk for variceal bleeding. TLR activation of nonparenchymal liver cells by pathogens results in portal hypertension. This might explain the pathophysiologic correlation between microbial infections and portal hypertension in patients with liver cirrhosis. These findings are the basis for both better risk stratifying and new treatment options, such as specific inhibition of TLR for patients with liver cirrhosis and portal hypertension. © 2015 S. Karger AG, Basel.

Diagnostic accuracy of point shear wave elastography in the detection of portal hypertension in pediatric patients.

PubMed

Burak Özkan, M; Bilgici, M C; Eren, E; Caltepe, G

2018-03-01

The purpose of this study was to determine the usefulness of point shear wave elastography (p-SWE) of the liver and spleen for the detection of portal hypertension in pediatric patients. The study consisted of 38 healthy children and 56 pediatric patients with biopsy-proven liver disease who underwent splenic and liver p-SWE. The diagnostic performance of p-SWE in detecting clinically significant portal hypertension was assessed using receiver operating characteristic (ROC) curves. Reliable measurements of splenic and liver stiffness with p-SWE were obtained in 76/94 (81%) and 80/94 patients (85%), respectively. The splenic stiffness was highest in the portal hypertension group (P<0.01). At ROC curve analysis, the area under the curve in the detection of portal hypertension was lower for splenic p-SWE than for liver p-SWE (0.906 vs. 0.746; P=0.0239). The cut-off value of splenic p-SWE for portal hypertension was 3.14m/s, with a specificity of 98.59% and a sensitivity of 68.18%. The cut-off value of liver p-SWE for portal hypertension was 2.09m/s, with a specificity of 80.28% and a sensitivity of 77.27%. In pediatric patients, p-SWE is a reliable method for detecting portal hypertension. However, splenic p-SWE is less accurate than liver p-SWE for the diagnosis of portal hypertension. Copyright © 2017 Editions françaises de radiologie. Published by Elsevier Masson SAS. All rights reserved.

Rapamycin Attenuates Splenomegaly in both Intrahepatic and Prehepatic Portal Hypertensive Rats by Blocking mTOR Signaling Pathway

PubMed Central

Wang, Huakai; Li, Yongjian; Shi, Minmin; Li, Hongwei; Yan, Jiqi

2016-01-01

Background Spleen enlargement is often detected in patients with liver cirrhosis, but the precise pathogenetic mechanisms behind the phenomenon have not been clearly elucidated. We investigated the pathogenetic mechanisms of splenomegaly in both portal hypertensive patients and rats, and tried to identify the possible therapy for this disease. Methods Spleen samples were collected from portal hypertensive patients after splenectomy. Rat models of portal hypertension were induced by common bile duct ligation and partial portal vein ligation. Spleen samples from patients and rats were used to study the characteristics of splenomegaly by histological, immunohistochemical, and western blot analyses. Rapamycin or vehicle was administered to rats to determine the contribution of mTOR signaling pathway in the development of splenomegaly. Results We found that not only spleen congestion, but also increasing angiogenesis, fibrogenesis, inflammation and proliferation of splenic lymphoid tissue contributed to the development of splenomegaly in portal hypertensive patients and rats. Intriguingly, splenomegaly developed time-dependently in portal hypertensive rat that accompanied with progressive activation of mTOR signaling pathway. mTOR blockade by rapamycin profoundly ameliorated splenomegaly by limiting lymphocytes proliferation, angiogenesis, fibrogenesis and inflammation as well as decreasing portal pressure. Conclusions This study provides compelling evidence indicating that mTOR signaling activation pathway plays a key role in the pathogenesis of splenomegaly in both portal hypertensive patients and rats. Therapeutic intervention targeting mTOR could be a promising strategy for patients with portal hypertension and splenomegaly. PMID:26734934

Rapamycin Attenuates Splenomegaly in both Intrahepatic and Prehepatic Portal Hypertensive Rats by Blocking mTOR Signaling Pathway.

PubMed

Chen, Yunyang; Wang, Weijie; Wang, Huakai; Li, Yongjian; Shi, Minmin; Li, Hongwei; Yan, Jiqi

2016-01-01

Spleen enlargement is often detected in patients with liver cirrhosis, but the precise pathogenetic mechanisms behind the phenomenon have not been clearly elucidated. We investigated the pathogenetic mechanisms of splenomegaly in both portal hypertensive patients and rats, and tried to identify the possible therapy for this disease. Spleen samples were collected from portal hypertensive patients after splenectomy. Rat models of portal hypertension were induced by common bile duct ligation and partial portal vein ligation. Spleen samples from patients and rats were used to study the characteristics of splenomegaly by histological, immunohistochemical, and western blot analyses. Rapamycin or vehicle was administered to rats to determine the contribution of mTOR signaling pathway in the development of splenomegaly. We found that not only spleen congestion, but also increasing angiogenesis, fibrogenesis, inflammation and proliferation of splenic lymphoid tissue contributed to the development of splenomegaly in portal hypertensive patients and rats. Intriguingly, splenomegaly developed time-dependently in portal hypertensive rat that accompanied with progressive activation of mTOR signaling pathway. mTOR blockade by rapamycin profoundly ameliorated splenomegaly by limiting lymphocytes proliferation, angiogenesis, fibrogenesis and inflammation as well as decreasing portal pressure. This study provides compelling evidence indicating that mTOR signaling activation pathway plays a key role in the pathogenesis of splenomegaly in both portal hypertensive patients and rats. Therapeutic intervention targeting mTOR could be a promising strategy for patients with portal hypertension and splenomegaly.

Venous outflow obstruction and portopulmonary hypertension after orthotopic liver transplantation

PubMed Central

Aguirre-Avalos, Guadalupe; Covarrubias-Velasco, Marco Antonio; Rojas-Sánchez, Antonio Gerardo

2013-01-01

Patient: Female, 54 Final Diagnosis: Suprahepatic inferior vena cava anastomosis stricture Symptoms: Ascites • fatigue • lower limb edema • hepatomegaly Medication: — Clinical Procedure: — Specialty: Transplantology • Critical Care Medicine Objective: Unusual clinical course Background: Suprahepatic inferior vena cava anastomosis stricture is an unusual vascular complication after orthotopic liver transplantation with the “piggyback” technique. Clinical manifestations are dependent upon the severity of the stenosis. Portopulmonary hypertension after orthotopic liver transplantation is a complication that carries high mortality due to cardiopulmonary dysfunction. The pathogenesis of pulmonary vascular disorders after orthotopic liver transplantation remains uncertain. Case Report: We report a case of acute right heart pressure overload after surgical correction of the suprahepatic inferior vena cava anastomotic stricture in a 54-year-old woman who had preexisting pulmonary arterial hypertension associated with portal hypertension after orthotopic liver transplantation. Twenty months posttransplantation, she developed fatigue and progressive ascites. On admission, the patient had hepatomegaly, ascites, and lower limb edema. Symptoms in the patient developed gradually over time. Conclusions: Recurrent portal hypertension by vascular complications is a cause of pulmonary arterial hypertension after orthotopic liver transplantation. Clinical manifestations of suprahepatic inferior vena cava anastomotic stenosis are dependent upon their severity. Sildenafil is an effective drug for treatment of pulmonary arterial hyper-tension after portal hypertension by vascular complications. PMID:24046802

Splanchnic-aortic inflammatory axis in experimental portal hypertension

PubMed Central

Aller, Maria-Angeles; de las Heras, Natalia; Nava, Maria-Paz; Regadera, Javier; Arias, Jaime; Lahera, Vicente

2013-01-01

Splanchnic and systemic low-grade inflammation has been proposed to be a consequence of long-term prehepatic portal hypertension. This experimental model causes minimal alternations in the liver, thus making a more selective study possible for the pathological changes characteristic of prehepatic portal hypertension. Low-grade splanchnic inflammation after long-term triple partial portal vein ligation could be associated with liver steatosis and portal hypertensive intestinal vasculopathy. In fact, we have previously shown that prehepatic portal hypertension in the rat induces liver steatosis and changes in lipid and carbohydrate metabolism similar to those produced in chronic inflammatory conditions described in metabolic syndrome in humans. Dysbiosis and bacterial translocation in this experimental model suggest the existence of a portal hypertensive intestinal microbiome implicated in both the splanchnic and systemic alterations related to prehepatic portal hypertension. Among the systemic impairments, aortopathy characterized by oxidative stress, increased levels of proinflammatory cytokines and profibrogenic mediators stand out. In this experimental model of long-term triple portal vein ligated-rats, the abdominal aortic proinflammatory response could be attributed to oxidative stress. Thus, the increased aortic reduced-nicotinamide-adenine dinucleotide phosphate [NAD(P)H] oxidase activity could be associated with reactive oxygen species production and promote aortic inflammation. Also, oxidative stress mediated by NAD(P)H oxidase has been associated with risk factors for inflammation and atherosclerosis. The splanchnic and systemic pathology that is produced in the long term after triple partial portal vein ligation in the rat reinforces the validity of this experimental model to study the chronic low-grade inflammatory response induced by prehepatic portal hypertension. PMID:24307792

Contrast-enhanced sonography for quantitative assessment of portal hypertension in patients with liver cirrhosis.

PubMed

Qu, En-Ze; Zhang, Ying-Cai; Li, Zhi-Yan; Liu, Yang; Wang, Jin-Rui

2014-11-01

The clinical utility of contrast-enhanced sonography in portal hypertension remains unclear. We explored the feasibility of using contrast-enhanced sonography for noninvasive assessment of portal venous pressure. Twenty healthy individuals (control group; 9 men; mean age, 46.4 years) and 18 patients with portal hypertension (15 men; mean age, 46.2 years) were enrolled in this study. The portal hypertension group included patients who underwent splenectomy and pericardial blood vessel disarticulation at our hospital from October 2010 to March 2011. One week before surgery, patients with portal hypertension underwent preoperative liver contrast-enhanced sonography. Two-dimensional, Doppler, and contrast-enhanced sonographic parameters were compared between the groups. Portal venous pressure was measured intraoperatively by portal vein puncture in the portal hypertension group, and its relationship with the other parameters was analyzed. The 2-dimensional, Doppler, and contrast-enhanced sonographic parameters differed between the groups (P < .01). Portal venous pressure was inversely correlated with the area under the portal vein/hepatic artery time-intensity curve ratio (Qp/Qa), portal vein/hepatic artery strength ratio (Ip/Ia), and portal vein/hepatic artery wash-in perfusion slope ratio (βp/βa), with correlation coefficients of -0.701, -0.625, and -0.494, respectively. Measurement of the liver contrast-enhanced sonographic parameters Qp/Qa, Ip/Ia, and βp/βa could be used as a new quantitative method for noninvasively assessing portal venous pressure. © 2014 by the American Institute of Ultrasound in Medicine.

[Surgical treatment of hemorrhage of esophageal varices secondary to thrombosis of the portal vein].

PubMed

Orozco-Zepeda, H; Takahashi, T; Angel Mercado, M; García-Tsao, G; Hernández-Ortiz, J

1990-01-01

The Sugiura Procedure (SP) was performed in 27 patients with hemorrhagic portal hypertension secondary to extrahepatic portal vein thrombosis without associated liver disease (EPVT). There were fourteen females and 13 males. Mean age was 28 +/- 14 years. The causes of EPVT were: protein C deficiency-2 cases, antithrombin III deficiency-1 case, omphalitis history-2 cases, pancreatitis history-1 case and idiopathic-21 cases. The SP was completed with two surgical stages in 14 patients and with one operation in nine. There was one operative death. One patient developed mild postoperative encephalopathy, and two patients re-bled at long-term. Actuarial survival was 82% at five and ten years. It is concluded that the SP is a good alternative for the management of hemorrhagic portal hypertension secondary to EPVT.

Intrahepatic upregulation of MRTF-A signaling contributes to increased hepatic vascular resistance in cirrhotic rats with portal hypertension.

PubMed

Zheng, Lei; Qin, Jun; Sun, Longci; Gui, Liang; Zhang, Chihao; Huang, Yijun; Deng, Wensheng; Huang, An; Sun, Dong; Luo, Meng

2017-06-01

Portal hypertension in cirrhosis is mediated, in part, by increased intrahepatic resistance, reflecting massive structural changes associated with fibrosis and intrahepatic vasoconstriction. Activation of the Rho/MRTF/SRF signaling pathway is essential for the cellular regulatory network of fibrogenesis. The aim of this study was to investigate MRTF-A-mediated regulation of intrahepatic fibrogenesis in cirrhotic rats. Portal hypertension was induced in rats via an injection of CCl 4 oil. Hemodynamic measurements were obtained using a polyethylene PE-50 catheter and pressure transducers. Expression of hepatic fibrogenesis was measured using histological staining. Expression of protein was measured using western blotting. Upregulation of MRTF-A protein expression in the livers of rats with CCl 4 -induced cirrhosis was relevant to intrahepatic resistance and hepatic fibrogenesis in portal hypertensive rats with increased modeling time. Inhibition of MRTF-A by CCG-1423 decelerated hepatic fibrosis, decreased intrahepatic resistance and portal pressure, and alleviated portal hypertension. Increased intrahepatic resistance in rats with CCl 4 -induced portal hypertension is associated with an upregulation of MRTF-A signaling. Inhibition of this pathway in the liver can decrease hepatic fibrosis and intrahepatic resistance, as well as reduce portal pressure in cirrhotic rats with CCl 4 -induced portal hypertension. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Endothelial dysfunction in the regulation of portal hypertension

PubMed Central

Iwakiri, Yasuko

2013-01-01

Portal hypertension is caused by an increased intrahepatic resistance, a major consequence of cirrhosis. Endothelial dysfunction in liver sinusoidal endothelial cells (LSECs) decreases the production of vasodilators, such as nitric oxide (NO) and favors vasoconstriction. This contributes to an increased vascular resistance in the intrahepatic/sinusoidal microcirculation. Portal hypertension, once developed, causes endothelial cell (EC) dysfunction in the extrahepatic, i.e. splanchnic and systemic, circulation. Unlike LSEC dysfunction, EC dysfunction in the splanchnic and systemic circulation overproduces vasodilator molecules, leading to arterial vasodilatation. In addition, portal hypertension leads to the formation of portosystemic collateral vessels. Both arterial vasodilatation and portosystemic collateral vessel formation exacerbate portal hypertension by increasing the blood flow through the portal vein. Pathologic consequences, such as esophageal varices and ascites, result. While the sequence of pathological vascular events in cirrhosis and portal hypertension have been elucidated, the underlying cellular and molecular mechanisms causing EC dysfunctions are not yet fully understood. This review article summarizes the current cellular and molecular studies on EC dysfunctions found during the development of cirrhosis and portal hypertension with a focus on intra- and extrahepatic circulation. The article ends by discussing future directions of study for EC dysfunctions. PMID:21745318

Systems Level Analysis and Identification of Pathways and Networks Associated with Liver Fibrosis

DTIC Science & Technology

2014-11-07

portal hypertension , and liver failure [4]. Liver fibrosis typically starts with apoptosis or necrosis of hepatocytes, which causes reactive oxygen...Arroyo V, et al. (2007) Antiangiogenic treatment with sunitinib ameliorates inflammatory infiltrate, fibrosis, and portal pressure in cirrhotic rats

Idiopathic Noncirrhotic Portal Hypertension: An Appraisal

PubMed Central

Lee, Hwajeong; Rehman, Aseeb Ur; Fiel, M. Isabel

2016-01-01

Idiopathic noncirrhotic portal hypertension is a poorly defined clinical condition of unknown etiology. Patients present with signs and symptoms of portal hypertension without evidence of cirrhosis. The disease course appears to be indolent and benign with an overall better outcome than cirrhosis, as long as the complications of portal hypertension are properly managed. This condition has been recognized in different parts of the world in diverse ethnic groups with variable risk factors, resulting in numerous terminologies and lack of standardized diagnostic criteria. Therefore, although the diagnosis of idiopathic noncirrhotic portal hypertension requires clinical exclusion of other conditions that can cause portal hypertension and histopathologic confirmation, this entity is under-recognized clinically as well as pathologically. Recent studies have demonstrated that variable histopathologic entities with different terms likely represent a histologic spectrum of a single entity of which obliterative portal venopathy might be an underlying pathogenesis. This perception calls for standardization of the nomenclature and formulation of widely accepted diagnostic criteria, which will facilitate easier recognition of this disorder and will highlight awareness of this entity. PMID:26563701

Single-Session Percutaneous Endovascular Mesocaval Shunt Creation and Balloon-Occluded Retrograde Transvenous Obliteration for the Treatment of Gastric Varices.

PubMed

Srinivasa, Ravi Nara; Majdalany, Bill S; Chick, Jeffrey Forris Beecham; Meadows, J Matthew; Fenlon, Jordan Bruce; Brewerton, Charles; Saad, Wael E

2018-01-01

In the setting of portal hypertension, the body responds by creating portosystemic venous shunts, which may lead to the development of varices. Endoscopic treatment of these varices is often warranted to prevent catastrophic bleeding. During the course of variceal treatment, 1 or more portosystemic shunts may be sacrificed, which may acutely exacerbate portal hypertension and reduce systemic venous return. This report describes percutaneous creation of a mesocaval shunt and balloon-occluded retrograde transvenous obliteration (BRTO) in a patient with cavernous transformation of the portal vein. The patient had previously undergone an unsuccessful attempt at transjugular intrahepatic portosystemic shunt (TIPS) creation with postoperative bleeding requiring splenectomy. As TIPS was not feasible, creation of a percutaneous mesocaval shunt provided an alternate pathway for portosystemic decompression, facilitating safe treatment of gastric varices with BRTO via a gastrorenal shunt. These procedures were performed simultaneously to reduce the risk of variceal bleeding from acute changes in portal venous pressures and redirect blood flow through the shunt to maintain patency. This is the first reported case of combined mesocaval shunt placement and BRTO in a single session. Copyright © 2017 Elsevier Inc. All rights reserved.

[Predictive value of ultrasonography in portal hypertension].

PubMed

Moreno, E; Torres, P; Trejo, C; Barra Ostoni, V; Ortega, C; Römer, H

1991-01-01

Portal hypertension is a common pathology in childhood and one of its most common causes is cavernomatosis of the portal vein. This obstruction causes hemodynamic changes which lead to splenomegaly and collateral circulation. Esophageal varices are one of the most important sequelae, which endanger the patient's life because of a bleeding tendency. Ecosonography helps to detect the thickening of the lesser omentum vis a vis the aortic diameter, caused by the collateral circulation. We studied 15 children presenting with portal hypertension resulting from portal vein cavernomatosis; we performed an upper GI endoscopy and abdominal ecosonography. The endoscopy revealed grade II esophageal varices in 20% of cases, the remaining 80% had grade III and grade IV. Ecosonography revealed an increased lesser omentum/aorta ratio in children with portal hypertension, compared to controls (p < 0.001). Our results suggest that the lesser omentum/aorta ratio has diagnostic value in pediatric portal hypertension.

Assessment of contrast-enhanced ultrasonography of the hepatic vein for detection of hemodynamic changes associated with experimentally induced portal hypertension in dogs.

PubMed

Morishita, Keitaro; Hiramoto, Akira; Michishita, Asuka; Takagi, Satoshi; Hoshino, Yuki; Itami, Takaharu; Lim, Sue Yee; Osuga, Tatsuyuki; Nakamura, Sayuri; Ochiai, Kenji; Nakamura, Kensuke; Ohta, Hiroshi; Yamasaki, Masahiro; Takiguchi, Mitsuyoshi

2017-04-01

OBJECTIVE To assess the use of contrast-enhanced ultrasonography (CEUS) of the hepatic vein for the detection of hemodynamic changes associated with experimentally induced portal hypertension in dogs. ANIMALS 6 healthy Beagles. PROCEDURES A prospective study was conducted. A catheter was surgically placed in the portal vein of each dog. Hypertension was induced by intraportal injection of microspheres (10 to 15 mg/kg) at 5-day intervals via the catheter. Microsphere injections were continued until multiple acquired portosystemic shunts were created. Portal vein pressure (PVP) was measured through the catheter. Contrast-enhanced ultrasonography was performed before and after establishment of hypertension. Time-intensity curves were generated from the region of interest in the hepatic vein. Perfusion variables measured for statistical analysis were hepatic vein arrival time, time to peak, time to peak phase (TTPP), and washout ratio. The correlation between CEUS variables and PVP was assessed by use of simple regression analysis. RESULTS Time to peak and TTPP were significantly less after induction of portal hypertension. Simple regression analysis revealed a significant negative correlation between TTPP and PVP. CONCLUSIONS AND CLINICAL RELEVANCE CEUS was useful for detecting hemodynamic changes associated with experimentally induced portal hypertension in dogs, which was characterized by a rapid increase in the intensity of the hepatic vein. Furthermore, TTPP, a time-dependent variable, provided useful complementary information for predicting portal hypertension. IMPACT FOR HUMAN MEDICINE Because the method described here induced presinusoidal portal hypertension, these results can be applied to idiopathic portal hypertension in humans.

Primary Biliary Cirrhosis

MedlinePlus

... other complications. Increased pressure in the portal vein (portal hypertension). Blood from your intestine, spleen and pancreas enters ... specific complications: Increased pressure in the portal vein (portal hypertension). Your doctor is likely to screen and monitor ...

Two surgical procedures for esophagogastric variceal bleeding in patients with portal hypertension

PubMed Central

Yang, Lin; Yuan, Li-Juan; Dong, Rui; Yin, Ji-Kai; Wang, Qing; Li, Tao; Li, Jiang-Bin; Du, Xi-Lin; Lu, Jian-Guo

2013-01-01

AIM: To determine the clinical value of a splenorenal shunt plus pericardial devascularization (PCVD) in portal hypertension (PHT) patients with variceal bleeding. METHODS: From January 2008 to November 2012, 290 patients with cirrhotic portal hypertension were treated surgically in our department for the prevention of gastroesophageal variceal bleeding: 207 patients received a routine PCVD procedure (PCVD group), and 83 patients received a PCVD plus a splenorenal shunt procedure (combined group). Changes in hemodynamic parameters, rebleeding, encephalopathy, portal vein thrombosis, and mortality were analyzed. RESULTS: The free portal pressure decreased to 21.43 ± 4.35 mmHg in the combined group compared with 24.61 ± 5.42 mmHg in the PCVD group (P < 0.05). The changes in hemodynamic parameters were more significant in the combined group (P < 0.05). The long-term rebleeding rate was 7.22% in the combined group, which was lower than that in the PCVD group (14.93%), (P < 0.05). CONCLUSION: Devascularization plus splenorenal shunt is an effective and safe strategy to control esophagogastric variceal bleeding in PHT. It should be recommended as a first-line treatment for preventing bleeding in PHT patients when surgical interventions are considered. PMID:24409071

Coil-Assisted Retrograde Transvenous Obliteration (CARTO) for the Treatment of Portal Hypertensive Variceal Bleeding: Preliminary Results

PubMed Central

Lee, Edward W; Saab, Sammy; Gomes, Antoinette S; Busuttil, Ronald; McWilliams, Justin; Durazo, Francisco; Han, Steven-Huy; Goldstein, Leonard; Tafti, Bashir A; Moriarty, John; Loh, Christopher T; Kee, Stephen T

2014-01-01

OBJECTIVES: To describe the technical feasibility, safety, and clinical outcomes of coil-assisted retrograde transvenous obliteration (CARTO) in treating portal hypertensive non-esophageal variceal hemorrhage. METHODS: From October 2012 to December 2013, 20 patients who received CARTO for the treatment of portal hypertensive non-esophageal variceal bleeding were retrospectively evaluated. All 20 patients had at least 6-month follow-up. All patients had detachable coils placed to occlude the efferent shunt and retrograde gelfoam embolization to achieve complete thrombosis/obliteration of varices. Technical success, clinical success, rebleeding, and complications were evaluated at follow-up. RESULTS: A 100% technical success rate (defined as achieving complete occlusion of efferent shunt with complete thrombosis/obliteration of bleeding varices and/or stopping variceal bleeding) was demonstrated in all 20 patients. Clinical success rate (defined as no variceal rebleeding) was 100%. Follow-up computed tomography after CARTO demonstrated decrease in size with complete thrombosis and disappearance of the varices in all 20 patients. Thirteen out of the 20 had endoscopic confirmation of resolution of varices. Minor post-CARTO complications, including worsening of esophageal varices (not bleeding) and worsening of ascites/hydrothorax, were noted in 5 patients (25%). One patient passed away at 24 days after the CARTO due to systemic and portal venous thrombosis and multi-organ failure. Otherwise, no major complication was noted. No variceal rebleeding was noted in all 20 patients during mean follow-up of 384±154 days. CONCLUSIONS: CARTO appears to be a technically feasible and safe alternative to traditional balloon-occluded retrograde transvenous obliteration or transjugular intrahepatic portosystemic shunt, with excellent clinical outcomes in treating portal hypertensive non-esophageal variceal bleeding. PMID:25273155

Targeting the transsulfuration-H2S pathway by FXR and GPBAR1 ligands in the treatment of portal hypertension.

PubMed

Fiorucci, Stefano; Distrutti, Eleonora

2016-09-01

Cirrhosis is a end-stage disease of the liver in which fibrogenesis, angiogenesis and distortion of intrahepatic microcirculation lead to increased intrahepatic resistance to portal blood flow, a condition known as portal hypertension. Portal hypertension is maintained by a variety of molecular mechanisms including sinusoidal endothelial cells (LSECs) hyporeactivity, activation of hepatic stellate cells (HSCs), reduction in hepatic endothelial nitric oxide synthase (eNOS) activity along with increased eNOS-derived NO generation in the splanchnic and systemic circulations. A reduction of the expression/function of the two major hydrogen sulfide (H2S)-producing enzymes, cystathionine γ-lyase (CSE) and cystathionine β-synthase (CBS), has also been demonstrated. A deficit in the transsulfuration pathway leading to the accumulation of homocysteine might contribute to defective generation of H2S and endothelial hyporeactivity. Bile acids are ligands for nuclear receptors, such as farnesoid X receptor (FXR), and G-protein-coupled receptors (GPCRs), such as the G-protein bile acid receptor 1 (GPBAR1). FXR and GPBAR1 ligands regulate the expression/activity of CSE by both genomic and non-genomic effects and have been proved effective in protecting against endothelial dysfunction observed in rodent models of cirrhosis. GPBAR1, a receptor for secondary bile acids, is selectively expressed by LSECs and its activation increases the expression of CSE and attenuates the production of endotelin-1, a potent vasoconstrictor agent. In vivo GPBAR1 ligand attenuates the imbalance between vasodilatory and vaso-constricting agents, making GPBAR1 a promising target in the treatment of portal hypertension. Copyright © 2016 Elsevier Ltd. All rights reserved.

The effect of partial portal decompression on portal blood flow and effective hepatic blood flow in man: a prospective study.

PubMed

Rosemurgy, A S; McAllister, E W; Godellas, C V; Goode, S E; Albrink, M H; Fabri, P J

1995-12-01

With the advent of transjugular intrahepatic porta-systemic stent shunt and the wider application of the surgically placed small diameter prosthetic H-graft portacaval shunt (HGPCS), partial portal decompression in the treatment of portal hypertension has received increased attention. The clinical results supporting the use of partial portal decompression are its low incidence of variceal rehemorrhage due to decreased portal pressures and its low rate of hepatic failure, possibly due to maintenance of blood flow to the liver. Surprisingly, nothing is known about changes in portal hemodynamics and effective hepatic blood flow following partial portal decompression. To prospectively evaluate changes in portal hemodynamics and effective hepatic blood flow brought about by partial portal decompression, the following were determined in seven patients undergoing HGPCS: intraoperative pre- and postshunt portal vein pressures and portal vein-inferior vena cava pressure gradients, intraoperative pre- and postshunt portal vein flow, and pre- and postoperative effective hepatic blood flow. With HGPCS, portal vein pressures and portal vein-inferior vena cava pressure gradients decreased significantly, although portal pressures remained above normal. In contrast to the significant decreases in portal pressures, portal vein blood flow and effective hepatic blood flow do not decrease significantly. Changes in portal vein pressures and portal vein-inferior vena cava pressure gradients are great when compared to changes in portal vein flow and effective hepatic blood flow. Reduction of portal hypertension with concomitant maintenance of hepatic blood flow may explain why hepatic dysfunction is avoided following partial portal decompression.

Unexpected disappearance of portal cavernoma on long-term anticoagulation.

PubMed

Silva-Junior, Gilberto; Turon, Fanny; Hernandez-Gea, Virginia; Darnell, Anna; García-Criado, Ángeles; García-Pagán, Juan Carlos

2014-08-01

Idiopathic non-cirrhotic portal hypertension is a rare disease of unknown etiology. Patients with idiopathic non-cirrhotic portal hypertension have an increased risk of developing portal vein thrombosis and this is especially prevalent when HIV is also present. We describe a unique case of a patient with idiopathic non-cirrhotic portal hypertension associated to HIV, who developed acute portal vein thrombosis that despite anticoagulation transformed in portal cavernoma and disappeared completely after five years of follow-up on continuous anticoagulation. Copyright © 2014 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.

Outcomes of pregnancies complicated by liver cirrhosis, portal hypertension, or esophageal varices.

PubMed

Puljic, Anela; Salati, Jennifer; Doss, Amy; Caughey, Aaron B

2016-01-01

To evaluate pregnancy outcomes in women with liver cirrhosis, portal hypertension, or esophageal varices. We analyzed a retrospective cohort of 2,284,218 pregnancies in 2005-2009 recorded in the California Birth Registry database. Utilizing ICD-9 codes we analyzed the following outcomes for liver cirrhosis, portal hypertension, or esophageal varices in pregnancy: preeclampsia (PET), preterm delivery (PTD; <37 weeks), cesarean section, low birth weight (LBW; <2500 g), small for gestational age (SGA; <10th percentile), neonatal death (NND), and postpartum hemorrhage (PPH). Cirrhosis in pregnancy conferred an increased risk of PET, PTD, CS in multiparous women, LBW, and NND. Portal hypertension in pregnancy was associated with PTD, LBW, NND, and PPH. Non-bleeding esophageal varices in pregnancy were not associated with the outcomes assessed in a statistically significant manner. One case of bleeding esophageal varices was observed, resulting in PTD with a LBW infant. There were three cases of concomitant portal hypertension or concomitant esophageal varices with cirrhosis in pregnancy. Pregnancy in women with concomitant liver cirrhosis, portal hypertension, or esophageal varices can be successful. However, pregnancy outcomes are worse and may warrant closer antenatal monitoring and patient counseling. Cirrhosis in pregnancy with concomitant portal hypertension or esophageal varices is rare.

Idiopathic noncirrhotic portal hypertension: current perspectives

PubMed Central

Riggio, Oliviero; Gioia, Stefania; Pentassuglio, Ilaria; Nicoletti, Valeria; Valente, Michele; d’Amati, Giulia

2016-01-01

The term idiopathic noncirrhotic portal hypertension (INCPH) has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals), splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis. Physicians should learn to look for this condition in a number of clinical settings, including cryptogenic cirrhosis, a disease known to be associated with INCPH, drug administration, and even chronic alterations in liver function tests. Once INCPH is clinically suspected, liver histology becomes mandatory for the correct diagnosis. However, pathologists should be familiar with the histological features of INCPH, especially in cases in which histology is not only requested to exclude liver cirrhosis. PMID:27555800

Idiopathic noncirrhotic portal hypertension: current perspectives.

PubMed

Riggio, Oliviero; Gioia, Stefania; Pentassuglio, Ilaria; Nicoletti, Valeria; Valente, Michele; d'Amati, Giulia

2016-01-01

The term idiopathic noncirrhotic portal hypertension (INCPH) has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals), splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis. Physicians should learn to look for this condition in a number of clinical settings, including cryptogenic cirrhosis, a disease known to be associated with INCPH, drug administration, and even chronic alterations in liver function tests. Once INCPH is clinically suspected, liver histology becomes mandatory for the correct diagnosis. However, pathologists should be familiar with the histological features of INCPH, especially in cases in which histology is not only requested to exclude liver cirrhosis.

Congenital abnormalities associated with extrahepatic portal hypertension.

PubMed Central

Odièvre, M; Pigé, G; Alagille, D

1977-01-01

Congenital abnormalities were present in 12 out of 30 (40%) children with extrahepatic portal hypertension of unknown cause, but in only 2 out of 17 (12%) children with extnahepatic portal hypertension secondary to umbilical vein catheterization or omphalitis. The most frequent abnormalities in this series and in published reports were atrial septal defect, malformation of the biliary tract, and anomalous inferior vena cava. These findings are consistent with the view that some cases with extrahepatic portal hypertension are congenital in origin. PMID:869567

Liver stiffness measurement, better than APRI, Fibroindex, Fib-4, and NBI gastroscopy, predicts portal hypertension in patients with cirrhosis.

PubMed

Zhang, Wei; Wang, Liqiong; Wang, Lei; Li, Gang; Huang, Aoshuang; Yin, Ping; Yang, Zhenhua; Ling, Changquan; Wang, Lingtai

2015-03-01

Liver stiffness measurement (LSM) is frequently used as non-invasive alternative for liver fibrosis including cirrhosis, which can lead to portal hypertension. This study was conducted to evaluate the predictive value of LSM in cirrhosis-induced portal hypertension patients. Between July 2011 and December 2013, 153 participants were enrolled into a single-center, prospective, cross-sectional study. Each subject received both gastroscopy and LSM. Baseline biochemical, APRI, Fibroindex, and Fib-4 were also performed. LSM of cirrhosis patients with portal hypertension was significantly higher compared to those without portal hypertension (P < 0.05). A LSM ≥ 13.6 kPa had a sensitivity of 83.87 % and a specificity of 72.53 % with an accuracy of 77.1 for the prediction of portal hypertension, which are higher than those of APRI, Fib-4, and Fibroscan separately. A combination of Fibroscan combined with Fib-4 achieved a maximum AUC of 0.833 and accuracy of 77.8. Discriminant and internal validation analysis showed that Fibroscan plus APRI obtained a lower false negative rate compared to Fibroscan plus Fib-4 and Fibroscan plus Fibroindex (9.68, 17.74, and 11.29 %, respectively). A good relationship was found between LSM and NBI mean optical density both by linear and polynomial correlation analysis (r = 0.5533 and r = 0.7349, both P < 0.001). There was a trend toward a better performance of LSM for assessing portal hypertension compared with NBI gastroscopy mean optical density (P = 0.028 and P = 0.05, respectively). Better than APRI, Fibroindex, Fib-4, and NBI gastroscopy, LSM can predict portal hypertension in cirrhosis patients. A LSM of 13.6 kPa can be considered to be the predictive value for portal hypertension.

[Postoperative complications and survival analysis of 1 118 cases of open splenectomy and azygoportal disconnection in the treatment of portal hypertension].

PubMed

Qi, R Z; Zhao, X; Wang, S Z; Zhang, K; Chang, Z Y; Hu, X L; Wu, M L; Zhang, P R; Yu, L X; Xiao, C H; Shi, X J; Li, Z W

2018-06-01

Objective: To analyze the recent postoperative and long-term postoperative complications of open-splenectomy and disconnection in patients with portal hypertension. Methods: There were 1 118 cases with portal hypertension who underwent open splenectomy and azygoportal disconnection from April 2010 to September 2015 at Department of Surgery, People's Liberation Army 302 Hospital. Retrospective case investigation and telephone follow-up were conducted in October 2016. All patients had history of upper gastrointestinal bleeding before operation. Short-term complications after surgery were recorded including secondary laparotomy of postoperative abdominal hemostasis, severe infection, intake disorders, liver insufficiency, postoperative portal vein thrombosis and perioperative mortality. Long-term data including postoperative upper gastrointestinal rebleeding, postoperative survival rate and incidence of postoperative malignancy were recorded, too. GraphPad Prism 5 software for data survival analysis and charting. Results: Postoperative short-term complications in 1 118 patients included secondary laparotomy of postoperative abdominal hemostasis(1.8%, 21/1 118), severe infection(2.9%, 32/1 118), intake disorders(1.0%, 11/1 118), liver dysfunction (1.6%, 18/1 118), postoperative portal vein thrombosis(47.1%, 526/1 118)and perioperative mortality(0.5%, 5/1 118). After phone call following-up, 942 patients' long-term data were completed including 1, 3, 5 years postoperative upper gastrointestinal rebleeding rate(4.4%, 12.1%, 17.2%), 1, 3, 5-year postoperative survival rate(97.0%, 93.5%, 90.3%); the incidence of postoperative malignant tumors in 1, 3 and 5 years were 1.7%, 4.4% and 6.2%. Conclusions: Reasonable choosing of surgical indications and timing, proper performing the surgery process, effective conducting perioperative management of portal hypertension are directly related to the patient's short-term prognosis after portal hypertension. Surgical intervention can reduce the rates of patients with upper gastrointestinal rebleeding, improve survival, and do not increase the incidence of malignant tumors.

Effects of raloxifene on portal hypertension and hepatic encephalopathy in cirrhotic rats.

PubMed

Chang, Ching-Chih; Lee, Wen-Shin; Chuang, Chiao-Lin; Hsin, I-Fang; Hsu, Shao-Jung; Chang, Ting; Huang, Hui-Chun; Lee, Fa-Yauh; Lee, Shou-Dong

2017-05-05

Raloxifene, a selective estrogen receptor modulator, has been used extensively for osteoporosis. In addition to the effect of osteoporosis treatment, emerging evidences show that raloxifene affects the vascular function in different tissues. Cirrhosis is characterized with portal hypertension and complicated with hepatic encephalopathy. Portal hypertension affects portal-systemic shunt which leads to hepatic encephalopathy that the vascular modulation might influence severity of hepatic encephalopathy. Herein, we evaluated the impact of raloxifene on bile duct ligation (BDL)-induced cirrhotic rats. The female Sprague-Dawley rats received BDL plus ovariectomy or sham-operation. Four weeks later, rats were divided into 2 subgroups respectively to receive of raloxifene (10mg/kg/day) or saline (vehicle) for 14 days. On the 43th day, motor activities and hemodynamic parameters were measured. Hepatic and vascular mRNA and protein expressions were determined. The histopathological change of liver was examined. We found that the liver biochemistry, ammonia level and motor activity were similar between cirrhotic rats with or without raloxifene administration. The hemodynamic parameters were not significantly different except that raloxifene reduced portal venous inflow. Raloxifene exacerbated hepatic fibrosis and up-regulated hepatic endothelin-1 and cyclooxygenase 2 protein expressions. In addition, raloxifene modulated the mRNA expressions of endothelial nitric oxide synthase, cyclooxygenase and endothelin-1 in the superior mesenteric artery and collateral vessel. In conclusion, raloxifene aggravates hepatic fibrosis and decreases portal venous inflow in cirrhotic rats without adversely affecting portal hypertension and hepatic encephalopathy. The modulation of hepatic and vascular endothelin-1, endothelial nitric oxide synthase and cyclooxygenase expressions may play a role in the mechanism. Copyright © 2017 Elsevier B.V. All rights reserved.

Gastric Varices: An Overview for the Gastroenterology Nurse.

PubMed

Dale, Cheryl

2016-01-01

Gastric varices can occur in as many as one-third of patients with portal hypertension. Within the nursing literature, however, articles focus on the management of esophageal varices and portal hypertensive gastrointestinal bleeding with few publications about management of gastric varices. Given the advancement in therapies, it is prudent for gastroenterology nurses to have an understanding of its management and treatment options. This article reviews the pathophysiology, classification, and management of patients with gastric varices and outlines the importance of the nurse's role in the education and ongoing care for this patient group.

Imaging diagnosis of portal hypertension.

PubMed

Conangla-Planes, M; Serres, X; Persiva, O; Augustín, S

2018-02-19

Portal hypertension is a clinical entity defined by a hydrostatic pressure greater than 5mm Hg in the portal territory, being clinically significant when it is greater than or equal to 10mm Hg. Starting from this threshold, complications can develop, such as the bleeding of esophageal varices, the appearance of ascites, or hepatic encephalopathy. Imaging techniques play an important role as a noninvasive method for determining whether portal hypertension is present. This article analyzes various imaging findings that can suggest the presence of portal hypertension and can help to define its etiology, severity, and possible complications. Copyright © 2018. Publicado por Elsevier España, S.L.U.

[Recurrent variceal bleeding in a patient with portal and splenic vein thrombosis secondary to complex thrombophilia].

PubMed

Ławniczak, Małgorzata; Raszeja-Wyszomirska, Joanna; Marlicz, Wojciech; Białek, Andrzej; Wiechowska-Kozłowska, Anna; Lubikowski, Jerzy; Wójcicki, Maciej; Starzyńska, Teresa

2008-08-01

Thrombophilia in adults is one of main causes of portal vein thrombosis. Esophageal and gastric varices, ascites and hypersplenism are well known complications of portal hypertension. There are controversial issues on the management, especially anticoagulant therapy and surgical treatment of these patients. We present a 42-years old woman with a history of three acute coronary episodes suffering from recurrent variceal bleeding due to portal and splenic vein thrombosis in the course of myeloproliferative disorder and protein C deficiency. It was 10 months delay of diagnosis. She was successfully treated with medical and surgical treatment (esophageal stapler transection, cardial devascularization, and splenectomy). In the paper we discuss complexity of diagnosis and surgical treatment.

Establishment of a reversible model of prehepatic portal hypertension in rats

PubMed Central

Zhao, Xin; Dou, Jian; Gao, Qing-Jun

2016-01-01

The aim of the present study was to improve upon the traditional model of pre-hepatic portal hypertension in rats, and simulate the anhepatic phase of orthotopic liver transplantation without veno-venous bypass. A reversible model of portal hypertension was induced by portal vein ligation, with a label ring ligated along the portal vein. A total of 135 male Wistar rats were divided into three groups: i) Normal control (NC) group; ii) portal hypertensive control (PHTC) group; and iii) reperfusion (R) group. In the R group, rats with portal hypertension underwent simultaneous clamping of the portal triad and retrohepatic vena cava for 1 h, followed by removal of the clamps to enable blood reperfusion. Portal venography and portal vein pressure were recorded during the surgery. Arterial oxygen pressure (PaO2), and alanine aminotransferase (ALT), aspartate aminotransferase (AST) and total bilirubin (TBil) levels were determined, and pathological changes of the liver were investigated by immunohistochemical staining. The results demonstrated that, 3 weeks after portal vein ligation, the vein area and the free portal pressures in the PHTC group were significantly increased compared with those in the NC group. The serum ALT and AST levels in the R group at different time points were significantly elevated compared with those in the PHTC group, and reached their maximal levels at 24 h after reperfusion. Furthermore, the PaO2 at 24 h after reperfusion was significantly decreased. In conclusion, the reversible model of pre-hepatic portal hypertension in rats was successfully established using the introduction of a label ring. This model may be useful for basic research focusing on the anhepatic phase of orthotopic liver transplantation without veno-venous bypass. PMID:27446299

Establishment of a reversible model of prehepatic portal hypertension in rats.

PubMed

Zhao, Xin; Dou, Jian; Gao, Qing-Jun

2016-08-01

The aim of the present study was to improve upon the traditional model of pre-hepatic portal hypertension in rats, and simulate the anhepatic phase of orthotopic liver transplantation without veno-venous bypass. A reversible model of portal hypertension was induced by portal vein ligation, with a label ring ligated along the portal vein. A total of 135 male Wistar rats were divided into three groups: i) Normal control (NC) group; ii) portal hypertensive control (PHTC) group; and iii) reperfusion (R) group. In the R group, rats with portal hypertension underwent simultaneous clamping of the portal triad and retrohepatic vena cava for 1 h, followed by removal of the clamps to enable blood reperfusion. Portal venography and portal vein pressure were recorded during the surgery. Arterial oxygen pressure (PaO 2 ), and alanine aminotransferase (ALT), aspartate aminotransferase (AST) and total bilirubin (TBil) levels were determined, and pathological changes of the liver were investigated by immunohistochemical staining. The results demonstrated that, 3 weeks after portal vein ligation, the vein area and the free portal pressures in the PHTC group were significantly increased compared with those in the NC group. The serum ALT and AST levels in the R group at different time points were significantly elevated compared with those in the PHTC group, and reached their maximal levels at 24 h after reperfusion. Furthermore, the PaO 2 at 24 h after reperfusion was significantly decreased. In conclusion, the reversible model of pre-hepatic portal hypertension in rats was successfully established using the introduction of a label ring. This model may be useful for basic research focusing on the anhepatic phase of orthotopic liver transplantation without veno-venous bypass.

Intrahepatic portal hypertension secondary to metastatic carcinoma of the prostate.

PubMed

Attila, Tan; Datta, Milton W; Sudakoff, Gary; Abu-Hajir, Majed; Massey, Benson T

2007-02-01

While the liver is a common site of metastasis, tumor metastases are not a common cause of portal hypertension. We report a case of a patient with symptomatic portal hypertension due to diffuse metastatic prostate carcinoma infiltration of liver parenchyma that was not appreciated with routine imaging.

[Management of ascites due to portal hypertension].

PubMed

Godat, S; Antonino, A T; Dehlavi, M-A; Moradpour, D; Doerig, C

2012-09-05

Portal hypertension is regularly encountered by the general practitioner. It is defined by an elevation of the porto-systemic pressure gradient, with complications such as ascites, spontaneous bacterial peritonitis, hepatorenal syndrome, variceal bleeding, hypersplenism, hepatopulmonary syndrome or hepatic encephalopathy occuring when a significant elevation of this gradient is reached. Cirrhosis is the primary cause of portal hypertension in industrialized countries. Symptomatic portal hypertension carries a poor prognosis. Management should be initiated rapidly, including the identification and correction of any reversible underlying condition. Liver transplantation should be considered in advanced cases.

Portal hypertensive gastropathy: association with Child-Pugh score in liver cirrhosis

NASA Astrophysics Data System (ADS)

Sungkar, T.; Zain, L. H.; Siregar, G. A.

2018-03-01

Portal Hypertensive Gastropathy (PHG) occurs as a complication of cirrhotic or non-cirrhotic portal hypertension. The association between the severity of portal hypertensive gastropathy and the hepatic function, as assessed by the Child-Pugh score in patients with liver cirrhosis are poorly defined. We evaluated association between PHG and Child-Pugh score in patients with liver cirrhosis. Adults liver cirrhosis patients admitted at Adam Malik Hospital Medan during January 2016-December 2016, were included in this study. Endoscopic PHG grade, Child-Pugh score were assessed. A total of 49 patients were enrolled. Majority of cases of liver cirrhosis are due to chronic viral hepatitis B infections (65.3 %). Portal hypertensive gastropathy were observed in 46 cases; twenty-five patients (51%) showed severe portal hypertensive gastropathy. The overall prevalence of PHG and the proportion of patients with severe PHG differ about the Child-Pugh classification. PHG was present in 66.7 % of patients from Child-Pugh class A, 96 % of patients with class B, and 95.2 % of those from class C, and severe forms were present in 0 %, 36 %, and 76.2 %, respectively (P< 0.000). In conclusions, the present data suggest that the severity of portal hypertensive gastropathy is related to Child-Pugh score.

Effect of viral suppression on hepatic venous pressure gradient in hepatitis C with cirrhosis and portal hypertension.

PubMed

Afdhal, N; Everson, G T; Calleja, J L; McCaughan, G W; Bosch, J; Brainard, D M; McHutchison, J G; De-Oertel, S; An, D; Charlton, M; Reddy, K R; Asselah, T; Gane, E; Curry, M P; Forns, X

2017-10-01

Portal hypertension is a predictor of liver-related clinical events and mortality in patients with hepatitis C and cirrhosis. The effect of interferon-free hepatitis C treatment on portal pressure is unknown. Fifty patients with Child-Pugh-Turcotte (CPT) A and B cirrhosis and portal hypertension (hepatic venous pressure gradient [HVPG] >6 mm Hg) were randomized to receive 48 weeks of open-label sofosbuvir plus ribavirin at Day 1 or after a 24-week observation period. The primary endpoint was sustained virologic response 12 weeks after therapy (SVR12) in patients who received ≥1 dose of treatment. Secondary endpoints included changes in HVPG, laboratory parameters, and MELD and CPT scores. A subset of patients was followed 48 weeks posttreatment to determine late changes in HVPG. SVR12 occurred in 72% of patients (33/46). In the 37 patients with paired HVPG measurements at baseline and the end of treatment, mean HVPG decreased by -1.0 (SD 3.97) mm Hg. Nine patients (24%) had ≥20% decreases in HVPG during treatment. Among 39 patients with pretreatment HVPG ≥12 mm Hg, 27 (69%) achieved SVR12. Four of the 33 (12%) patients with baseline HVPG ≥12 mm Hg had HVPG <12 mm Hg at the end of treatment. Of nine patients with pretreatment HVPG ≥12 mm Hg who achieved SVR12 and completed 48 weeks of follow-up, eight (89%) had a ≥20% reduction in HVPG, and three reduced their pressure to <12 mm Hg. Patients with chronic HCV and compensated or decompensated cirrhosis who achieve SVR can have clinically meaningful reductions in HVPG at long-term follow-up. (EudraCT 2012-002457-29). © 2017 John Wiley & Sons Ltd.

Neuropeptide Y restores non-receptor-mediated vasoconstrictive action in superior mesenteric arteries in portal hypertension.

PubMed

Hartl, Johannes; Dietrich, Peter; Moleda, Lukas; Müller-Schilling, Martina; Wiest, Reiner

2015-12-01

Vascular hyporeactivity to vasoconstrictors contributes to splanchnic arterial vasodilatation and hemodynamic dysregulation in portal hypertension. Neuropeptide Y (NPY), a sympathetic cotransmitter, has been shown to improve adrenergic vascular contractility in portal hypertensive rats and markedly attenuate hyperdynamic circulation. To further characterize the NPY-effects in portal hypertension, we investigated its role for non-receptor-mediated vasoconstriction in the superior mesenteric artery (SMA) of portal vein ligated (PVL) and sham-operated rats. Ex vivo SMA perfusion of PVL and sham rats was used to analyse the effects of NPY on pressure response to non-receptor-mediated vasoconstriction. Dose-response curves to KCl (30-300 mM) were used to bypass G protein-coupled receptor mechanisms. Potential involvement of the cyclooxygenase-pathway was tested by non-selective cyclooxygenase-inhibition using indomethacin. KCl-induced vascular contractility but not vascular sensitivity was significantly attenuated in PVL rats as compared with sham rats. Administration of NPY resulted in an augmentation of KCl-evoked vascular sensitivity being not different between study groups. However, KCl-induced vascular contractility was markedly more enhanced in PVL rats, thus, vascular response was no more significantly different between PVL and sham rats after addition of NPY. Administration of indomethacin abolished the NPY-induced enhancement of vasoconstriction. Receptor-independent vascular contractility is impaired in mesenteric arteries in portal hypertension. NPY improves non-receptor mediated mesenteric vasoconstriction more effective in portal hypertension than in healthy conditions correcting splanchnic vascular hyporesponsiveness. This beneficial vasoactive action of NPY adds to its well known more pronounced effects on adrenergic vasoconstriction in portal hypertension making it a promising therapeutic agent in portal hypertension. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Role of hydrogen sulfide in portal hypertension and esophagogastric junction vascular disease

PubMed Central

Wang, Chao; Han, Juan; Xiao, Liang; Jin, Chang-E; Li, Dong-Jian; Yang, Zhen

2014-01-01

AIM: To investigate the association between endogenous hydrogen sulfide (H2S) and portal hypertension as well as its effect on vascular smooth muscle cells. METHODS: Portal hypertension patients were categorized by Child-Pugh score based on bilirubin and albumin levels, prothrombin time, ascites and hepatic encephalopathy. Plasma H2S concentrations and portal vein diameters (PVDs) were compared between portal hypertension patients and control participants, as well as between portal hypertension patients with varying degrees of severity. In addition, we established a rabbit hepatic schistosomiasis portal hypertension (SPH) model and analyzed liver morphology, fibrosis grade, plasma and liver tissue H2S concentrations, as well as cystathionine γ-lyase (CSE) activity and phosphorylated extracellular signal-regulated kinase (pERK)1/2, B cell lymphoma (Bcl)-2 and Bcl-XL expression in portal vein smooth muscle cells, in addition to their H2S-induced apoptosis rates. RESULTS: In portal hypertension patients, endogenous H2S levels were significantly lower than those in healthy controls. The more severe the disease was, the lower were the H2S plasma levels, which were inversely correlated with PVD and Child-Pugh score. Liver tissue H2S concentrations and CSE expression were significantly lower in the SPH rabbit livers compared with the control animals, starting at 3 wk, whereas pERK 1/2 expressions gradually increased 12-20 wk after SPH model establishment. In portal vein smooth muscle cells, increasing H2S levels led to increased apoptosis, while Bcl-2 and Bcl-XL expression decreased. CONCLUSION: H2S prevents vascular restructuring caused by excessive proliferation of smooth muscle cells via apoptosis induction, which helps to maintain normal vascular structures. PMID:24574782

[Caroli's disease: congenital cystic and segmentary dilatation of the intrahepatic bile ducts].

PubMed

Serra, C A; Recalde, E B; Martellotto, G I

1987-01-01

Congenital cystic segmentary dilatation of the intrahepatic biliary ducts is a rare disease. Although several cases has been described in medical literature, its occurrence such as it was originally referred is exceptional. Some cases coexist with congenital hepatic fibrosis and portal hypertension. A characteristic phenomenon is the formation of stones and canalicular infection. In this article, three cases of Caroli disease (females, 57, 53 and 58 years old) are described. Case n 1 was a complex malformation including: a) cystic and segmentary dilatations of the intrahepatic biliar ducts; b) intra and extrahepatic lithiasis and recurrent infections; c) fusion of the choledocus and gallbladder in a common sac; d) hepatic cavernosus hemangioma and a supernumerarious lobe. Case n 2 showed: a) cystic and segmentary dilatation of the intrahepatic biliar ducts; b) portal fibrosis; c) polycystic kidneys; d) portal hypertension. Case n 3 showed: a) cystic and segmentary dilatation of the intrahepatic biliar ducts; b) portal fibrosis; c) polycystic kidneys and spleen; d) portal hypertension. Prognostic of this patients is variable and depends on the recurrent infections and the coexistence of other malformations which are frequently severe. Surgery is the only treatment for the disease although there are no definite conclusions concerning the long-term results.

Percutaneous Mesocaval Shunt Creation in a Patient with Chronic Portal and Superior Mesenteric Vein Thrombosis

DOE Office of Scientific and Technical Information (OSTI.GOV)

Bercu, Zachary L., E-mail: zachary.bercu@mountsinai.org; Sheth, Sachin B., E-mail: sachinsheth@gmail.com; Noor, Amir, E-mail: amir.noor@gmail.com

The creation of a transjugular intrahepatic portosystemic shunt (TIPS) is a critical procedure for the treatment of recurrent variceal bleeding and refractory ascites in the setting of portal hypertension. Chronic portal vein thrombosis remains a relative contraindication to conventional TIPS and options are limited in this scenario. Presented is a novel technique for management of refractory ascites in a patient with hepatitis C cirrhosis and chronic portal and superior mesenteric vein thrombosis secondary to schistosomiasis and lupus anticoagulant utilizing fluoroscopically guided percutaneous mesocaval shunt creation.

Role of the renin-angiotensin system in hepatic fibrosis and portal hypertension.

PubMed

Shim, Kwang Yong; Eom, Young Woo; Kim, Moon Young; Kang, Seong Hee; Baik, Soon Koo

2018-05-01

The renin-angiotensin system (RAS) is an important regulator of cirrhosis and portal hypertension. As hepatic fibrosis progresses, levels of the RAS components angiotensin (Ang) II, Ang-(1-7), angiotensin-converting enzyme (ACE), and Ang II type 1 receptor (AT1R) are increased. The primary effector Ang II regulates vasoconstriction, sodium homoeostasis, fibrosis, cell proliferation, and inflammation in various diseases, including liver cirrhosis, through the ACE/Ang II/AT1R axis in the classical RAS. The ACE2/Ang-(1-7)/Mas receptor and ACE2/Ang-(1-9)/AT2R axes make up the alternative RAS and promote vasodilation, antigrowth, proapoptotic, and anti-inflammatory effects; thus, countering the effects of the classical RAS axis to reduce hepatic fibrogenesis and portal hypertension. Patients with portal hypertension have been treated with RAS antagonists such as ACE inhibitors, Ang receptor blockers, and aldosterone antagonists, with very promising hemodynamic results. In this review, we examine the RAS, its roles in hepatic fibrosis and portal hypertension, and current therapeutic approaches based on the use of RAS antagonists in patients with portal hypertension.

Portacaval shunting

MedlinePlus

... cannot flow through it easily. This is called portal hypertension (increased pressure and backup of the portal vein.) ... open (rupture), causing serious bleeding. Common causes of portal hypertension are: Alcohol use causing scarring of the liver ( ...

Esophageal Varices

MedlinePlus

... that carries blood to your liver. This pressure (portal hypertension) forces the blood to seek other pathways through ... the amount of pressure in the portal vein (portal hypertension). Large varices. The larger the varices, the more ...

Wall shear stress in portal vein of cirrhotic patients with portal hypertension.

PubMed

Wei, Wei; Pu, Yan-Song; Wang, Xin-Kai; Jiang, An; Zhou, Rui; Li, Yu; Zhang, Qiu-Juan; Wei, Ya-Juan; Chen, Bin; Li, Zong-Fang

2017-05-14

To investigate wall shear stress (WSS) magnitude and distribution in cirrhotic patients with portal hypertension using computational fluid dynamics. Idealized portal vein (PV) system models were reconstructed with different angles of the PV-splenic vein (SV) and superior mesenteric vein (SMV)-SV. Patient-specific models were created according to enhanced computed tomography images. WSS was simulated by using a finite-element analyzer, regarding the blood as a Newtonian fluid and the vessel as a rigid wall. Analysis was carried out to compare the WSS in the portal hypertension group with that in healthy controls. For the idealized models, WSS in the portal hypertension group (0-10 dyn/cm 2 ) was significantly lower than that in the healthy controls (10-20 dyn/cm 2 ), and low WSS area (0-1 dyn/cm 2 ) only occurred in the left wall of the PV in the portal hypertension group. Different angles of PV-SV and SMV-SV had different effects on the magnitude and distribution of WSS, and low WSS area often occurred in smaller PV-SV angle and larger SMV-SV angle. In the patient-specific models, WSS in the cirrhotic patients with portal hypertension (10.13 ± 1.34 dyn/cm 2 ) was also significantly lower than that in the healthy controls ( P < 0.05). Low WSS area often occurred in the junction area of SV and SMV into the PV, in the area of the division of PV into left and right PV, and in the outer wall of the curving SV in the control group. In the cirrhotic patients with portal hypertension, the low WSS area extended to wider levels and the magnitude of WSS reached lower levels, thereby being more prone to disturbed flow occurrence. Cirrhotic patients with portal hypertension show dramatic hemodynamic changes with lower WSS and greater potential for disturbed flow, representing a possible causative factor of PV thrombosis.

Wall shear stress in portal vein of cirrhotic patients with portal hypertension

PubMed Central

Wei, Wei; Pu, Yan-Song; Wang, Xin-Kai; Jiang, An; Zhou, Rui; Li, Yu; Zhang, Qiu-Juan; Wei, Ya-Juan; Chen, Bin; Li, Zong-Fang

2017-01-01

AIM To investigate wall shear stress (WSS) magnitude and distribution in cirrhotic patients with portal hypertension using computational fluid dynamics. METHODS Idealized portal vein (PV) system models were reconstructed with different angles of the PV-splenic vein (SV) and superior mesenteric vein (SMV)-SV. Patient-specific models were created according to enhanced computed tomography images. WSS was simulated by using a finite-element analyzer, regarding the blood as a Newtonian fluid and the vessel as a rigid wall. Analysis was carried out to compare the WSS in the portal hypertension group with that in healthy controls. RESULTS For the idealized models, WSS in the portal hypertension group (0-10 dyn/cm2) was significantly lower than that in the healthy controls (10-20 dyn/cm2), and low WSS area (0-1 dyn/cm2) only occurred in the left wall of the PV in the portal hypertension group. Different angles of PV-SV and SMV-SV had different effects on the magnitude and distribution of WSS, and low WSS area often occurred in smaller PV-SV angle and larger SMV-SV angle. In the patient-specific models, WSS in the cirrhotic patients with portal hypertension (10.13 ± 1.34 dyn/cm2) was also significantly lower than that in the healthy controls (P < 0.05). Low WSS area often occurred in the junction area of SV and SMV into the PV, in the area of the division of PV into left and right PV, and in the outer wall of the curving SV in the control group. In the cirrhotic patients with portal hypertension, the low WSS area extended to wider levels and the magnitude of WSS reached lower levels, thereby being more prone to disturbed flow occurrence. CONCLUSION Cirrhotic patients with portal hypertension show dramatic hemodynamic changes with lower WSS and greater potential for disturbed flow, representing a possible causative factor of PV thrombosis. PMID:28566887

Transjugular intrahepatic portosystemic shunt (TIPS)

MedlinePlus

... flow through it very easily. This is called portal hypertension (increased pressure and backup of the portal vein). ... open (rupture), causing serious bleeding. Common causes of portal hypertension are: Alcohol use causing scarring of the liver ( ...

Ultrasonography for Noninvasive Assessment of Portal Hypertension

PubMed Central

Maruyama, Hitoshi; Yokosuka, Osamu

2017-01-01

Portal hypertension is a major pathophysiology in patients with cirrhosis. Portal pressure is the gold standard to evaluate the severity of portal hypertension, and radiological intervention is the only procedure for pressure measurement. Ultrasound (US) is a simple and noninvasive imaging modality available worldwide. B-mode imaging allows broad applications for patients to detect and characterize chronic liver diseases and focal hepatic lesions. The Doppler technique offers real-time observation of blood flow with qualitative and quantitative assessments, and the application of microbubble-based contrast agents has improved the detectability of peripheral blood flow. In addition, elastography for the liver and spleen covers a wider field beyond the original purpose of fibrosis assessment. These developments enhance the practical use of US in the evaluation of portal hemodynamic abnormalities. This article reviews the recent progress of US in the assessment of portal hypertension. PMID:28267700

Ultrasonography for Noninvasive Assessment of Portal Hypertension.

PubMed

Maruyama, Hitoshi; Yokosuka, Osamu

2017-07-15

Portal hypertension is a major pathophysiology in patients with cirrhosis. Portal pressure is the gold standard to evaluate the severity of portal hypertension, and radiological intervention is the only procedure for pressure measurement. Ultrasound (US) is a simple and noninvasive imaging modality available worldwide. B-mode imaging allows broad applications for patients to detect and characterize chronic liver diseases and focal hepatic lesions. The Doppler technique offers real-time observation of blood flow with qualitative and quantitative assessments, and the application of microbubble-based contrast agents has improved the detectability of peripheral blood flow. In addition, elastography for the liver and spleen covers a wider field beyond the original purpose of fibrosis assessment. These developments enhance the practical use of US in the evaluation of portal hemodynamic abnormalities. This article reviews the recent progress of US in the assessment of portal hypertension.

Prevalence of histological features of idiopathic noncirrhotic portal hypertension in general population: a retrospective study of incidental liver biopsies.

PubMed

Zuo, Chunlai; Chumbalkar, Vaibhav; Ells, Peter F; Bonville, Daniel J; Lee, Hwajeong

2017-09-01

Idiopathic noncirrhotic portal hypertension (INCPH) is associated with histologic changes secondary to obliterative portal venopathy without cirrhosis. We studied the prevalence of individual histological features of INCPH in liver biopsies obtained incidentally during unrelated elective procedures and in elective liver biopsies with the diagnosis of fatty liver disease. A total of 53 incidental liver biopsies obtained intraoperatively during unrelated elective procedures and an additional 28 elective biopsies with the diagnosis of fatty liver disease without portal hypertension and cirrhosis were studied. Various histologic features of INCPH were evaluated. Shunt vessel (30%), phlebosclerosis (27%), increased number of portal vessels (19%) and incomplete septa (17%) were common in these liver biopsies after confounding factors such as co-existing fatty liver disease or fibrosis were excluded. At least one feature of INCPH was noted in 90% of the biopsies. Eight (10%) biopsies showed 5-6 features of INCPH. In total, 11 (14%) of 81 patients had risk factors associated with INCPH, including hypercoagulability, autoimmune disease, exposure to drugs, and infections. No patient had portal hypertension at the end of the follow-up. The histologic features of INCPH are seen in incidental liver biopsies and fatty liver disease without portal hypertension. Ten percent of the biopsies show 5-6 features of INCPH without portal hypertension. Interpreting histologic features in the right clinical context is important for proper patient care.

Endothelin antagonism in portal hypertensive mice: implications for endothelin receptor-specific signaling in liver disease

PubMed Central

Feng, Hong-Qiang; Weymouth, Nate D.; Rockey, Don C.

2009-01-01

Endothelin-1 (ET-1), a potent vasoactive peptide, plays an important role in the pathogenesis of liver disease and portal hypertension. Two major endothelin receptors (ET-A and ET-B) mediate biological effects, largely on the basis of their known downstream signaling pathways. We hypothesized that the different receptors are likely to mediate divergent effects in portal hypertensive mice. Liver fibrosis and cirrhosis and portal hypertension were induced in 8-wk-old male BALB/c mice by gavage with carbon tetrachloride (CCl4). Portal pressure was recorded acutely during intravenous infusion of endothelin receptor antagonists in normal or portal hypertensive mice. In vivo microscopy was used to monitor sinusoidal dynamics. Additionally, the effect of chronic exposure to endothelin antagonists was assessed in mice during induction of fibrosis and cirrhosis with CCl4 for 8 wk. Intravenous infusion of ET-A receptor antagonists into normal and cirrhotic mice reduced portal pressure whereas ET-B receptor antagonism increased portal pressure. A mixed endothelin receptor antagonist also significantly reduced portal pressure. Additionally, the ET-A receptor antagonist caused sinusoidal dilation, whereas the ET-B receptor antagonist caused sinusoidal constriction. Chronic administration of each the endothelin receptor antagonists during the induction of fibrosis and portal hypertension led to reduced fibrosis, a significant reduction in portal pressure, and altered sinusoidal dynamics relative to controls. Acute effects of endothelin receptor antagonists are likely directly on the hepatic and sinusoidal vasculature, whereas chronic endothelin receptor antagonism appears to be more complicated, likely affecting fibrogenesis and the hepatic microcirculation. The data imply a relationship between hepatic fibrogenesis or fibrosis and vasomotor responses. PMID:19299580

Life-threatening hypersplenism due to idiopathic portal hypertension in early childhood: case report and review of the literature

PubMed Central

2010-01-01

Background Idiopathic portal hypertension (IPH) is a disorder of unknown etiology and is characterized clinically by portal hypertension, splenomegaly, and hypersplenism accompanied by pancytopenia. This study evaluates the pathogenic concept of the disease by a systematic review of the literature and illustrates novel pathologic and laboratory findings. Case Presentation We report the first case of uncontrolled splenic hyperperfusion and enlargement with subsequent hypersplenism leading to life-threatening complications of IPH in infancy and emergent splenectomy. Conclusions Our results suggest that splenic NO and VCAM-1, rather than ET-1, have a significant impact on the development of IPH, even at a very early stage of disease. The success of surgical interventions targeting the splenic hyperperfusion suggests that the primary defect in the regulation of splenic blood flow seems to be crucial for the development of IPH. Thus, beside other treatment options splenectomy needs to be considered as a prime therapeutic option for IPH. PMID:20961440

Diagnosis of cirrhosis and portal hypertension: imaging, non-invasive markers of fibrosis and liver biopsy

PubMed Central

Procopet, Bogdan

2017-01-01

Abstract The concept of ‘cirrhosis’ is evolving and it is now clear that compensated and decompensated cirrhosis are completely different in terms of prognosis. Furthermore, the term ‘advanced chronic liver disease (ACLD)’ better reflects the continuum of histological changes occurring in the liver, which continue to progress even after cirrhosis has developed, and might regress after removing the etiological factor causing the liver disease. In compensated ACLD, portal hypertension marks the progression to a stage with higher risk of clinical complication and requires an appropriate evaluation and treatment. Invasive tests to diagnose cirrhosis (liver biopsy) and portal hypertension (hepatic venous pressure gradient measurement and endoscopy) remain of crucial importance in several difficult clinical scenarios, but their need can be reduced by using different non-invasive tests in standard cases. Among non-invasive tests, the accepted use, major limitations and major benefits of serum markers of fibrosis, elastography and imaging methods are summarized in the present review. PMID:28533906

Mitochondria-targeted antioxidant mitoquinone deactivates human and rat hepatic stellate cells and reduces portal hypertension in cirrhotic rats.

PubMed

Vilaseca, Marina; García-Calderó, Héctor; Lafoz, Erica; Ruart, Maria; López-Sanjurjo, Cristina Isabel; Murphy, Michael P; Deulofeu, Ramon; Bosch, Jaume; Hernández-Gea, Virginia; Gracia-Sancho, Jordi; García-Pagán, Juan Carlos

2017-07-01

In cirrhosis, activated hepatic stellate cells (HSC) play a major role in increasing intrahepatic vascular resistance and developing portal hypertension. We have shown that cirrhotic livers have increased reactive oxygen species (ROS), and that antioxidant therapy decreases portal pressure. Considering that mitochondria produce many of these ROS, our aim was to assess the effects of the oral mitochondria-targeted antioxidant mitoquinone on hepatic oxidative stress, HSC phenotype, liver fibrosis and portal hypertension. Ex vivo: Hepatic stellate cells phenotype was analysed in human precision-cut liver slices in response to mitoquinone or vehicle. In vitro: Mitochondrial oxidative stress was analysed in different cell type of livers from control and cirrhotic rats. HSC phenotype, proliferation and viability were assessed in LX2, and in primary human and rat HSC treated with mitoquinone or vehicle. In vivo: CCl 4 - and thioacetamide-cirrhotic rats were treated with mitoquinone (5 mg/kg/day) or the vehicle compound, DecylTPP, for 2 weeks, followed by measurement of oxidative stress, systemic and hepatic haemodynamic, liver fibrosis, HSC phenotype and liver inflammation. Mitoquinone deactivated human and rat HSC, decreased their proliferation but with no effects on viability. In CCl 4 -cirrhotic rats, mitoquinone decreased hepatic oxidative stress, improved HSC phenotype, reduced intrahepatic vascular resistance and diminished liver fibrosis. These effects were associated with a significant reduction in portal pressure without changes in arterial pressure. These results were further confirmed in the thioacetamide-cirrhotic model. We propose mitochondria-targeted antioxidants as a novel treatment approach against portal hypertension and cirrhosis. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Portal vein aneurysm: What to know.

PubMed

Laurenzi, Andrea; Ettorre, Giuseppe Maria; Lionetti, Raffaella; Meniconi, Roberto Luca; Colasanti, Marco; Vennarecci, Giovanni

2015-11-01

Portal vein aneurysm is an unusual vascular dilatation of the portal vein, which was first described by Barzilai and Kleckner in 1956 and since then less than 200 cases have been reported. The aim of this article is to provide an overview of the international literature to better clarify various aspects of this rare nosological entity and provide clear evidence-based summary, when available, of the clinical and surgical management. A systematic literature search of the Pubmed database was performed for all articles related to portal vein aneurysm. All articles published from 1956 to 2014 were examined for a total of 96 reports, including 190 patients. Portal vein aneurysm is defined as a portal vein diameter exceeding 1.9 cm in cirrhotic patients and 1.5 cm in normal livers. It can be congenital or acquired and portal hypertension represents the main cause of the acquired version. Surgical indication is considered in case of rupture, thrombosis or symptomatic aneurysms. Aneurysmectomy and aneurysmorrhaphy are considered in patients with normal liver, while shunt procedures or liver transplantation are the treatment of choice in case of portal hypertension. Being such a rare vascular entity its management should be reserved to high-volume tertiary hepato-biliary centres. Copyright © 2015 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

Splenic artery ligature associated with endoscopic banding for schistosomal portal hypertension.

PubMed

Colaneri, Renata Potonyacz; Coelho, Fabrício Ferreira; de Cleva, Roberto; Perini, Marcos Vinícius; Herman, Paulo

2014-11-28

To propose a less invasive surgical treatment for schistosomal portal hypertension. Ten consecutive patients with hepatosplenic schistosomiasis and portal hypertension with a history of upper gastrointestinal hemorrhage from esophageal varices rupture were evaluated in this study. Patients were subjected to a small supraumbilical laparotomy with the ligature of the splenic artery and left gastric vein. During the procedure, direct portal vein pressure before and after the ligatures was measured. Upper gastrointestinal endoscopy was performed at the 30(th) postoperative day, when esophageal varices diameter were measured and band ligature performed. During follow-up, other endoscopic procedures were performed according to endoscopy findings. There was no intra-operative mortality and all patients had confirmed histologic diagnoses of schistosomal portal hypertension. During the immediate postoperative period, two of the ten patients had complications, one characterized by a splenic infarction, and the other by an incision hematoma. Mean hospitalization time was 4.1 d (range: 2-7 d). Pre- and post-operative liver function tests did not show any significant changes. During endoscopy thirty days after surgery, a decrease in variceal diameters was observed in seven patients. During the follow-up period (57-72 mo), endoscopic therapy was performed and seven patients had their varices eradicated. Considering the late postoperative evaluation, nine patients had a decrease in variceal diameters. A mean of 3.9 endoscopic banding sessions were performed per patient. Two patients presented bleeding recurrence at the late postoperative period, which was controlled with endoscopic banding in one patient due to variceal rupture and presented as secondary to congestive gastropathy in the other patient. Both bleeding episodes were of minor degree with no hemodynamic consequences or need for blood transfusion. Ligature of the splenic artery and left gastric vein with supraumbilical laparotomy is a promising and less invasive method for treating presinusoidal schistosomiasis portal hypertension.

Extrahepatic angiogenesis hinders recovery of portal hypertension and collaterals in rats with cirrhosis resolution.

PubMed

Hsu, Shao-Jung; Tsai, Ming-Hung; Chang, Ching-Chih; Hsieh, Yu-Hsin; Huang, Hui-Chun; Lee, Fa-Yauh; Chuang, Chiao-Ling; Hou, Ming-Chih; Lee, Shou-Dong

2018-03-30

Liver cirrhosis is characterized by portal hypertension. However, the alteration of portal hypertension-related derangements during cirrhosis resolution is not well known. The present study aimed to establish animal models with cirrhosis resolution and to investigate the relevant changes during this process. Male Sprague-Dawley rats were applied. In reverse thioacetamide (rTAA) model, rats were randomly allocated into four groups with control, thioacetamide (TAA) cirrhosis and rTAA groups that discontinued TAA for 4 or 8 weeks after cirrhosis induction. In reverse bile duct ligation (rBDL) model, rats received choledochoduodenal shunt surgery upon the establishment of cirrhosis and 4, 8, or 16 weeks were allowed after the surgery. At the end, portal hypertension-related parameters were evaluated. Cirrhosis resolution was observed in rTAA groups. Portal pressure (PP) decreased after cirrhosis resolution but remained higher than control group (control, TAA, rTAA4, rTAA8 (mmHg): 5.4 ± 0.3, 12.9 ± 0.3, 8.6 ± 0.4, 7.6 ± 0.6). Further survey found the increased splanchnic blood flow did not reduce during cirrhosis resolution. The extrahepatic pathological angiogenesis was not ameliorated (% of mesenteric window area: 1.2 ± 0.3, 7.3 ± 1.1, 8.3 ± 1.0, 11.3 ± 2.7). In collateral system, the shunting degree reduced while the vessels structure remained. The vascular contractility of all systems and nitric oxide (NO) production were normalized. In rBDL series, PP decreased in rBDL16 groups but the extrahepatic angiogenesis persisted. In conclusion, cirrhosis resolution attenuates but not completely normalizes portal hypertension because of persistently high splanchnic inflow and angiogenesis. In clinical setting, vascular complications such as varices could persist after cirrhosis resolution and further investigation to define the follow-up and treatment strategies is anticipated. © 2018 The Author(s). Published by Portland Press Limited on behalf of the Biochemical Society.

Zolmitriptan: a novel portal hypotensive agent which synergizes with propranolol in lowering portal pressure.

PubMed

Reboredo, Mercedes; Chang, Haisul C Y; Barbero, Roberto; Rodríguez-Ortigosa, Carlos M; Pérez-Vizcaíno, Francisco; Morán, Asunción; García, Mónica; Banales, Jesús M; Carreño, Norberto; Alegre, Félix; Herrero, Ignacio; Quiroga, Jorge; Prieto, Jesús; Sangro, Bruno

2013-01-01

Only a limited proportion of patients needing pharmacological control of portal hypertension are hemodynamic responders to propranolol. Here we analyzed the effects of zolmitriptan on portal pressure and its potential interaction with propranolol. ZOLMITRIPTAN, PROPRANOLOL OR BOTH WERE TESTED IN TWO RAT MODELS OF PORTAL HYPERTENSION: common bile duct ligation (CBDL) and CCl4-induced cirrhosis. In these animals we measured different hemodynamic parameters including portal venous pressure, arterial renal flow, portal blood flow and cardiac output. We also studied the changes in superior mesenteric artery perfusion pressure and in arterial wall cAMP levels induced by zolmitriptan, propranolol or both. Moreover, we determined the effect of splanchnic sympathectomy on the response of PVP to zolmitriptan. In both models of portal hypertension zolmitriptan induced a dose-dependent transient descent of portal pressure accompanied by reduction of portal flow with only slight decrease in renal flow. In cirrhotic rats, splanchnic sympathectomy intensified and prolonged zolmitriptan-induced portal pressure descent. Also, propranolol caused more intense and durable portal pressure fall when combined with zolmitriptan. Mesenteric artery perfusion pressure peaked for about 1 min upon zolmitriptan administration but showed no change with propranolol. However propranolol enhanced and prolonged the elevation in mesenteric artery perfusion pressure induced by zolmitriptan. In vitro studies showed that propranolol prevented the inhibitory effects of β2-agonists on zolmitriptan-induced vasoconstriction and the combination of propranolol and zolmitriptan significantly reduced the elevation of cAMP caused by β2-agonists. Zolmitriptan reduces portal hypertension and non-selective beta-blockers can improve this effect. Combination therapy deserves consideration for patients with portal hypertension failing to respond to non-selective beta-blockers.

Efficacy of carvedilol versus propranolol versus variceal band ligation for primary prevention of variceal bleeding.

PubMed

Abd ElRahim, Ayman Yosry; Fouad, Rabab; Khairy, Marwa; Elsharkawy, Aisha; Fathalah, Waleed; Khatamish, Haytham; Khorshid, Omayma; Moussa, Mona; Seyam, Moataz

2018-01-01

Band ligation and propranolol are the current therapies for primary prevention of variceal bleeding. Carvedilol is a rising nonselective beta-blocker used for reducing portal pressure with favorable outcome. The aim of this study to assess the efficacy of carvedilol, propranolol, and band ligation for primary prevention of variceal bleeding based on the effect of each regimen on progression of Child score and portal hypertensive gastropathy after 1 year. The study included 264 cirrhotic patients with medium/large-sized varices who were candidates for primary prophylaxis of variceal bleeding. Patients were randomly divided into three groups: group I: band ligation; group II: propranolol; group III: carvedilol. Group I showed higher success rate of 75 %, followed by group III with 70.2 % and group II with 65.2 %. Risk of bleeding was comparable between the three groups, with group II carrying the highest rate of complications (34.7 %) followed by group III (14.2 %) and finally group I (5.7 %). After 1 year of follow-up, Child score did not improve in any of the studied groups, while portal hypertensive gastropathy significantly increased in group I but decreased in groups II and III. Band ligation is the best treatment option for primary prevention of variceal bleeding with minimal complications. Carvedilol is a good pharmaceutical alternative medicine to propranolol with lesser side-effects. Progress of liver disease as represented by Child score is not affected by any of the primary variceal prophylactic regimens, although medical treatment reduces portal hypertensive gastropathy. Choice of treatment depends on patient will, compliance with treatment, and endoscopist competence.

Severe bleeding from esophageal varices resistant to endoscopic treatment in a non cirrhotic patient with portal hypertension

PubMed Central

Caronna, Roberto; Bezzi, Mario; Schiratti, Monica; Cardi, Maurizio; Prezioso, Giampaolo; Benedetti, Michele; Papini, Federica; Mangioni, Simona; Martino, Gabriele; Chirletti, Piero

2008-01-01

A non cirrhotic patient with esophageal varices and portal vein thrombosis had recurrent variceal bleeding unsuccessfully controlled by endoscopy and esophageal transection. Emergency transhepatic portography confirmed the thrombosed right branch of the portal vein, while the left branch appeared angulated, shifted and stenotic. A stent was successfully implanted into the left branch and the collateral vessels along the epatoduodenal ligament disappeared. In patients with esophageal variceal hemorrhage and portal thrombosis if endoscopy fails, emergency esophageal transection or nonselective portocaval shunting are indicated. The rare patients with only partial portal thrombosis can be treated directly with stenting through an angioradiologic approach. PMID:18644135

Current status of portal vein thrombosis in Japan: Results of a questionnaire survey by the Japan Society for Portal Hypertension.

PubMed

Kojima, Seiichiro; Watanabe, Norihito; Koizumi, Jun; Kokubu, Shigehiro; Murashima, Naoya; Matsutani, Shoichi; Obara, Katsutoshi

2018-03-01

To investigate the current status of portal vein thrombosis (PVT) in Japan, the Clinical Research Committee of the Japan Society of Portal Hypertension undertook a questionnaire survey. A questionnaire survey of 539 cases of PVT over the previous 10 years was carried out at institutions affiliated with the Board of Trustees of the Japan Society of Portal Hypertension. The most frequent underlying etiology of PVT was liver cirrhosis in 75.3% of patients. Other causes included inflammatory diseases of the hepatobiliary system and the pancreas, malignant tumors, and hematologic diseases. The most frequent site was the main trunk of the portal vein (MPV) in 70.5%, and complete obstruction of the MPV was present in 11.5%. Among the medications for PVT, danaparoid was given to 45.8%, warfarin to 26.2%, heparin to 17.3%, and anti-thrombin III to 16.9%. Observation of the course was practiced in 22.4%. Factors contributing to therapeutic efficacy were implementation of various medications, thrombi localized to either the right or left portal vein only, non-complete obstruction of the MPV and Child-Pugh class A liver function. A survival analysis showed that the prognosis was favorable with PVT disappearance regardless of treatment. The questionnaire survey showed the current status of PVT in Japan. Any appropriate medication should be given to a patient with PVT when PVT is recognized. It is necessary to compile a large amount of information and reach a consensus on safe and highly effective management of PVT. © 2017 The Japan Society of Hepatology.

Portal hypertension in children: High-risk varices, primary prophylaxis and consequences of bleeding.

PubMed

Duché, Mathieu; Ducot, Béatrice; Ackermann, Oanez; Guérin, Florent; Jacquemin, Emmanuel; Bernard, Olivier

2017-02-01

Primary prophylaxis of bleeding is debated for children with portal hypertension because of the limited number of studies on its safety and efficacy, the lack of a known endoscopic pattern carrying a high-risk of bleeding for all causes, and the assumption that the mortality of a first bleed is low. We report our experience with these issues. From 1989 to 2014, we managed 1300 children with portal hypertension. Endoscopic features were recorded; high-risk varices were defined as: grade 3 esophageal varices, grade 2 varices with red wale markings, or gastric varices. Two hundred forty-six children bled spontaneously and 182 underwent primary prophylaxis. The results of primary prophylaxis were reviewed as well as bleed-free survival, overall survival and life-threatening complications of bleeding. High-risk varices were found in 96% of children who bled spontaneously and in 11% of children who did not bleed without primary prophylaxis (p<0.001), regardless of the cause of portal hypertension. Life-threatening complications of bleeding were recorded in 19% of children with cirrhosis and high-risk varices who bled spontaneously. Ten-year probabilities of bleed-free survival after primary prophylaxis in children with high-risk varices were 96% and 72% for non-cirrhotic causes and cirrhosis respectively. Ten-year probabilities of overall survival after primary prophylaxis were 100% and 93% in children with non-cirrhotic causes and cirrhosis respectively. In children with portal hypertension, bleeding is linked to the high-risk endoscopic pattern reported here. Primary prophylaxis of bleeding based on this pattern is fairly effective and safe. In children with liver disease, the risk of bleeding from varices in the esophagus is linked to their large size, the presence of congestion on their surface and their expansion into the stomach but not to the child's age nor to the cause of portal hypertension. Prevention of the first bleed in children with high-risk varices can be achieved by surgery or endoscopic treatment, and decreases mortality and morbidity. Copyright © 2016 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.

Advances in the management of childhood portal hypertension.

PubMed

McKiernan, Patrick; Abdel-Hady, Mona

2015-05-01

Portal hypertension is one of the most serious complications of childhood liver disease, and variceal bleeding is the most feared complication. Most portal hypertension results from cirrhosis but extra hepatic portal vein obstruction is the single commonest cause. Upper gastrointestinal endoscopy endoscopy remains necessary to diagnose gastro-esophageal varices. Families of children with portal hypertension should be provided with written instructions in case of gastrointestinal bleeding. Children with large varices should be considered for primary prophylaxis on a case-by-case basis. The preferred method is variceal band ligation. Children with acute bleeding should be admitted to hospital and treated with antibiotics and pharmacotherapy before urgent therapeutic endoscopy. All children who have bled should then receive secondary prophylaxis. The preferred method is variceal band ligation and as yet there is little evidence to support the use of β-blockers. Children with extrahepatic portal vein obstruction should be assessed for suitability of mesoportal bypass.

[Changes in the peritoneum of the small intestine and diaphragm in experimental portal hypertension].

PubMed

Khoroshaev, V A; Vorozheĭkin, V M; Baĭbekov, I M

1991-04-01

Diaphragm and small intestine peritoneum morphology was studied in experimental portal hypertension in rats with the help of luminescent, transmission and scanning electron microscopy techniques. Structural organizations of these peritoneum portions and performance function were different: fluid transudation realized through the small intestine peritoneum and resorption occurred via diaphragm peritoneum. Morphological signs allowed to judge about the increasing of fluid transudation in abdominal cavity and diaphragmatic resorption in early period of portal hypertension. Morphological alterations appeared in peritoneum resorption sites (pumping diaphragmatic hatchs) according to progress of portal hypertension that indicated decompensation process of peritoneal fluid absorption and led to ascites.

Future therapy of portal hypertension in liver cirrhosis – a guess

PubMed Central

Trebicka, Jonel

2014-01-01

In patients with chronic liver disease, portal hypertension is driven by progressive fibrosis and intrahepatic vasoconstriction. Interruption of the initiating and perpetuating etiology—mostly leading to necroinflammation—is possible for several underlying causes, such as autoimmune hepatitis, hepatitis B virus (HBV) infection, and most recently hepatitis C virus (HCV) infection. Thus, in the long run, lifestyle-related liver damage due to chronic alcoholism or morbid obesity will remain the main factor leading to portal hypertension. Both causes are probably more easily countered by socioeconomic measures than by individual approaches. If chronic liver injury supporting fibrogenesis and portal hypertension cannot be interrupted, a wide variety of tools are available to modulate and reduce intrahepatic resistance and therewith portal hypertension. Many of these have been evaluated in animal models. Also, some well-established drugs, which are used in humans for other indications (for example, statins), are promising if applied early and concomitantly to standard therapy. In the future, more individually tailored strategies must also be considered in line with the spectrum of portal hypertensive complications and risk factors defined by high-throughput analysis of the patient’s genome, transcriptome, metabolome, or microbiome. PMID:25374673

Arachidonic acid metabolites and endothelial dysfunction of portal hypertension.

PubMed

Sacerdoti, David; Pesce, Paola; Di Pascoli, Marco; Brocco, Silvia; Cecchetto, Lara; Bolognesi, Massimo

2015-07-01

Increased resistance to portal flow and increased portal inflow due to mesenteric vasodilatation represent the main factors causing portal hypertension in cirrhosis. Endothelial cell dysfunction, defined as an imbalance between the synthesis, release, and effect of endothelial mediators of vascular tone, inflammation, thrombosis, and angiogenesis, plays a major role in the increase of resistance in portal circulation, in the decrease in the mesenteric one, in the development of collateral circulation. Reduced response to vasodilators in liver sinusoids and increased response in the mesenteric arterioles, and, viceversa, increased response to vasoconstrictors in the portal-sinusoidal circulation and decreased response in the mesenteric arterioles are also relevant to the pathophysiology of portal hypertension. Arachidonic acid (AA) metabolites through the three pathways, cyclooxygenase (COX), lipoxygenase, and cytochrome P450 monooxygenase and epoxygenase, are involved in endothelial dysfunction of portal hypertension. Increased thromboxane-A2 production by liver sinusoidal endothelial cells (LSECs) via increased COX-1 activity/expression, increased leukotriens, increased epoxyeicosatrienoic acids (EETs) (dilators of the peripheral arterial circulation, but vasoconstrictors of the portal-sinusoidal circulation), represent a major component in the increased portal resistance, in the decreased portal response to vasodilators and in the hyper-response to vasoconstrictors. Increased prostacyclin (PGI2) via COX-1 and COX-2 overexpression, and increased EETs/heme-oxygenase-1/K channels/gap junctions (endothelial derived hyperpolarizing factor system) play a major role in mesenteric vasodilatation, hyporeactivity to vasoconstrictors, and hyper-response to vasodilators. EETs, mediators of liver regeneration after hepatectomy and of angiogenesis, may play a role in the development of regenerative nodules and collateral circulation, through stimulation of vascular endothelial growth factor (VEGF) inside the liver and in the portal circulation. Pharmacological manipulation of AA metabolites may be beneficial for cirrhotic portal hypertension. Copyright © 2015 Elsevier Inc. All rights reserved.

Portal Hypertension in Patients with Liver Cirrhosis: Diagnostic Accuracy of Spleen Stiffness.

PubMed

Takuma, Yoshitaka; Nouso, Kazuhiro; Morimoto, Youichi; Tomokuni, Junko; Sahara, Akiko; Takabatake, Hiroyuki; Matsueda, Kazuhiro; Yamamoto, Hiroshi

2016-05-01

To evaluate the accuracy of spleen stiffness (SS) and liver stiffness (LS) measured by using acoustic radiation force impulse imaging in the diagnosis of portal hypertension in patients with liver cirrhosis, with the hepatic venous pressure gradient (HVPG) as a reference standard. Institutional review board approval and informed consent were obtained for this prospective single-center study. From February 2012 to August 2013, 60 patients with liver cirrhosis (mean age, 70.8 years; age range, 34-88 years; 34 men, 26 women) with HVPG, LS, and SS measurements and gastrointestinal endoscopy and laboratory data were included if they met the following criteria: no recent episodes of gastrointestinal bleeding, no history of splenectomy, no history of partial splenic embolization, no history of β-blocker therapy, and absence of portal thrombosis. The efficacy of the parameters for the evaluation of portal hypertension was analyzed by using the Spearman rank-order correlation coefficient and receiver operating characteristic (ROC) curve analysis. The correlation coefficient between SS and HVPG (r = 0.876) was significantly better than that between LS and HVPG (r = 0.609, P < .0001). The areas under the ROC curve of SS for the identification of clinically important portal hypertension (HVPG ≥ 10 mm Hg), severe portal hypertension (HVPG ≥ 12 mm Hg), esophageal varices (EVs), and high-risk EVs were significantly higher (0.943, 0.963, 0.937, and 0.955, respectively) than those of LS, spleen diameter, platelet count, and platelet count to spleen diameter ratio (P < .05 for all). SS could be used to accurately rule out the presence of clinically important portal hypertension, severe portal hypertension, EVs, and high-risk EVs (negative likelihood ratios, 0.051, 0.056, 0.054, and 0.074, respectively). SS is reliable and has better diagnostic performance than LS for identifying portal hypertension in liver cirrhosis. (©) RSNA, 2015 Online supplemental material is available for this article.

Sustained virologic response to interferon-free therapies ameliorates HCV-induced portal hypertension.

PubMed

Mandorfer, Mattias; Kozbial, Karin; Schwabl, Philipp; Freissmuth, Clarissa; Schwarzer, Rémy; Stern, Rafael; Chromy, David; Stättermayer, Albert Friedrich; Reiberger, Thomas; Beinhardt, Sandra; Sieghart, Wolfgang; Trauner, Michael; Hofer, Harald; Ferlitsch, Arnulf; Ferenci, Peter; Peck-Radosavljevic, Markus

2016-10-01

We aimed to investigate the impact of sustained virologic response (SVR) to interferon (IFN)-free therapies on portal hypertension in patients with paired hepatic venous pressure gradient (HVPG) measurements. One hundred and four patients with portal hypertension (HVPG ⩾6mmHg) who underwent HVPG and liver stiffness measurement before IFN-free therapy (baseline [BL]) were retrospectively studied. Among 100 patients who achieved SVR, 60 patients underwent HVPG and transient elastography (TE) after antiviral therapy (follow-up [FU]). SVR to IFN-free therapies significantly decreased HVPG across all BL HVPG strata: 6-9mmHg (BL: 7.37±0.28 vs. FU: 5.11±0.38mmHg; -2.26±0.42mmHg; p<0.001), 10-15mmHg (BL: 12.2±0.4 vs. FU: 8.91±0.62mmHg; -3.29±0.59mmHg; p<0.001) and ⩾16mmHg (BL: 19.4±0.73 vs. FU: 17.1±1.21mmHg; -2.3±0.89mmHg; p=0.018). In the subgroup of patients with BL HVPG of 6-9mmHg, HVPG normalized (<6mmHg) in 63% (12/19) of patients, while no patient progressed to ⩾10mmHg. Among patients with BL HVPG ⩾10mmHg, a clinically relevant HVPG decrease ⩾10% was observed in 63% (26/41); 24% (10/41) had a FU HVPG <10mmHg. Patients with Child-Pugh stage B were less likely to have a HVPG decrease (hazard ratio [HR]: 0.103; 95% confidence interval [CI]: 0.02-0.514; p=0.006), when compared to Child-Pugh A patients. In the subgroup of patients with BL CSPH, the relative change in liver stiffness (per %; HR: 0.972; 95% CI: 0.945-0.999; p=0.044) was a predictor of a HVPG decrease ⩾10%. The area under the receiver operating characteristic curve for the diagnosis of FU CSPH by FU liver stiffness was 0.931 (95% CI: 0.865-0.997). SVR to IFN-free therapies might ameliorate portal hypertension across all BL HVPG strata. However, changes in HVPG seemed to be more heterogeneous among patients with BL HVPG of ⩾16mmHg and a HVPG decrease was less likely in patients with more advanced liver dysfunction. TE might be useful for the non-invasive evaluation of portal hypertension after SVR. We investigated the impact of curing hepatitis C using novel interferon-free treatments on portal hypertension, which drives the development of liver-related complications and mortality. Cure of hepatitis C decreased portal pressure, but a decrease was less likely among patients with more pronounced hepatic dysfunction. Transient elastography, which is commonly used for the non-invasive staging of liver disease, might identify patients without clinically significant portal hypertension after successful treatment. Copyright © 2016 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.

Liver Disease and Pulmonary Hypertension

MedlinePlus

... Liver disease can cause what is known as “portal hypertension,” meaning increased blood pressure in the veins that ... diagnosed? A specialist can diagnose POPH by identifying portal hypertension (high pressure in the veins of the liver), ...

Clinical role of non-invasive assessment of portal hypertension.

PubMed

Bolognesi, Massimo; Di Pascoli, Marco; Sacerdoti, David

2017-01-07

Measurement of portal pressure is pivotal in the evaluation of patients with liver cirrhosis. The measurement of the hepatic venous pressure gradient represents the reference method by which portal pressure is estimated. However, it is an invasive procedure that requires significant hospital resources, including experienced staff, and is associated with considerable cost. Non-invasive methods that can be reliably used to estimate the presence and the degree of portal hypertension are urgently needed in clinical practice. Biochemical and morphological parameters have been proposed for this purpose, but have shown disappointing results overall. Splanchnic Doppler ultrasonography and the analysis of microbubble contrast agent kinetics with contrast-enhanced ultrasonography have shown better accuracy for the evaluation of patients with portal hypertension. A key advancement in the non-invasive evaluation of portal hypertension has been the introduction in clinical practice of methods able to measure stiffness in the liver, as well as stiffness/congestion in the spleen. According to the data published to date, it appears to be possible to rule out clinically significant portal hypertension in patients with cirrhosis ( i.e ., hepatic venous pressure gradient ≥ 10 mmHg) with a level of clinically-acceptable accuracy by combining measurements of liver stiffness and spleen stiffness along with Doppler ultrasound evaluation. It is probable that the combination of these methods may also allow for the identification of patients with the most serious degree of portal hypertension, and ongoing research is helping to ensure progress in this field.

Clinical role of non-invasive assessment of portal hypertension

PubMed Central

Bolognesi, Massimo; Di Pascoli, Marco; Sacerdoti, David

2017-01-01

Measurement of portal pressure is pivotal in the evaluation of patients with liver cirrhosis. The measurement of the hepatic venous pressure gradient represents the reference method by which portal pressure is estimated. However, it is an invasive procedure that requires significant hospital resources, including experienced staff, and is associated with considerable cost. Non-invasive methods that can be reliably used to estimate the presence and the degree of portal hypertension are urgently needed in clinical practice. Biochemical and morphological parameters have been proposed for this purpose, but have shown disappointing results overall. Splanchnic Doppler ultrasonography and the analysis of microbubble contrast agent kinetics with contrast-enhanced ultrasonography have shown better accuracy for the evaluation of patients with portal hypertension. A key advancement in the non-invasive evaluation of portal hypertension has been the introduction in clinical practice of methods able to measure stiffness in the liver, as well as stiffness/congestion in the spleen. According to the data published to date, it appears to be possible to rule out clinically significant portal hypertension in patients with cirrhosis (i.e., hepatic venous pressure gradient ≥ 10 mmHg) with a level of clinically-acceptable accuracy by combining measurements of liver stiffness and spleen stiffness along with Doppler ultrasound evaluation. It is probable that the combination of these methods may also allow for the identification of patients with the most serious degree of portal hypertension, and ongoing research is helping to ensure progress in this field. PMID:28104976

Portal hypertensive gastropathy: A systematic review of the pathophysiology, clinical presentation, natural history and therapy.

PubMed

Gjeorgjievski, Mihajlo; Cappell, Mitchell S

2016-02-08

To describe the pathophysiology, clinical presentation, natural history, and therapy of portal hypertensive gastropathy (PHG) based on a systematic literature review. Computerized search of the literature was performed via PubMed using the following medical subject headings or keywords: "portal" and "gastropathy"; or "portal" and "hypertensive"; or "congestive" and "gastropathy"; or "congestive" and "gastroenteropathy". The following criteria were applied for study inclusion: Publication in peer-reviewed journals, and publication since 1980. Articles were independently evaluated by each author and selected for inclusion by consensus after discussion based on the following criteria: Well-designed, prospective trials; recent studies; large study populations; and study emphasis on PHG. PHG is diagnosed by characteristic endoscopic findings of small polygonal areas of variable erythema surrounded by a pale, reticular border in a mosaic pattern in the gastric fundus/body in a patient with cirrhotic or non-cirrhotic portal hypertension. Histologic findings include capillary and venule dilatation, congestion, and tortuosity, without vascular fibrin thrombi or inflammatory cells in gastric submucosa. PHG is differentiated from gastric antral vascular ectasia by a different endoscopic appearance. The etiology of PHG is inadequately understood. Portal hypertension is necessary but insufficient to develop PHG because many patients have portal hypertension without PHG. PHG increases in frequency with more severe portal hypertension, advanced liver disease, longer liver disease duration, presence of esophageal varices, and endoscopic variceal obliteration. PHG pathogenesis is related to a hyperdynamic circulation, induced by portal hypertension, characterized by increased intrahepatic resistance to flow, increased splanchnic flow, increased total gastric flow, and most likely decreased gastric mucosal flow. Gastric mucosa in PHG shows increased susceptibility to gastrotoxic chemicals and poor wound healing. Nitrous oxide, free radicals, tumor necrosis factor-alpha, and glucagon may contribute to PHG development. Acute and chronic gastrointestinal bleeding are the only clinical complications. Bleeding is typically mild-to-moderate. Endoscopic therapy is rarely useful because the bleeding is typically diffuse. Acute bleeding is primarily treated with octreotide, often with concomitant proton pump inhibitor therapy, or secondarily treated with vasopressin or terlipressin. Nonselective β-adrenergic receptor antagonists, particularly propranolol, are used to prevent bleeding after an acute episode or for chronic bleeding. Iron deficiency anemia from chronic bleeding may require iron replacement therapy. Transjugular-intrahepatic-portosystemic-shunt and liver transplantation are highly successful ultimate therapies because they reduce the underlying portal hypertension. PHG is important to recognize in patients with cirrhotic or non-cirrhotic portal hypertension because it can cause acute or chronic GI bleeding that often requires pharmacologic therapy.

Cirrhotic portal hypertension: current and future medical therapy for primary and secondary prevention of variceal bleeding.

PubMed

Laleman, W; Nevens, F

2006-08-01

Portal hypertension (PHT) is the most common complication of chronic liver disease and develops in the vast majority of patients with cirrhosis. It is characterized by an increase of the portal vein pressure, and leads to the development of gastroesophageal varices, ascites, renal dysfunction and hepatic encephalopathy. Over the years, it has become clear that a decrease in portal pressure is not only protective against the risk of variceal (re)bleeding but is also associated with a lower long-term risk of developing other complications and with an improved long-term survival. At present, non-selective b-blockers remain the medical treatment of choice for both primary and secondary prophylaxis. However, recent advances in the knowledge of the pathophysiology of cirrhotic PHT have directed future therapy towards the increased intrahepatic vascular resistance, which in part is determined by an increased hepatic vascular tone. This increased vasculogenic component provides the motivation to the use of therapies aimed at increasing intrahepatic vasorelaxing capacity on the one hand and at antagonizing excessive intrahepatic vasoconstrictor force on the other hand. This review covers current and future developments in the treatment of PHT with regard to primary and secondary prophylaxis.

The keys to successful TIPS in patients with portal vein thrombosis and cavernous transformation.

PubMed

Lombardo, S; Espejo, J J; Pérez-Montilla, M E; Zurera, L J; González-Galilea, Á

Portal vein thrombosis is a common complication in patients with cirrhosis. Anticoagulation involves a high risk of bleeding secondary to portal hypertension, so placing transjugular intrahepatic portosystemic shunts (TIPS) has become an alternative treatment for portal vein thrombosis. Three strategies for TIPS placement have been reported: 1) portal recanalization and conventional implantation of the TIPS through the jugular vein; 2) portal recanalization through percutaneous transhepatic/transsplenic) access; and (3) insertion of the TIPS between the suprahepatic vein and a periportal collateral vessel without portal recanalization. We describe different materials that can be used as fluoroscopic targets for the TIPS needle and for portal recanalization. This article aims to show the success of TIPS implantation using different combinations of the techniques listed above, which is a good treatment alternative in these patients whose clinical condition makes them difficult to manage, and to show that portal vein thrombosis/cavernous transformation should not be considered a contraindication for TIPS. Copyright © 2017 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.

Vasodilator responses to nitric oxide are enhanced in mesenteric arteries of portal hypertensive rats.

PubMed

Heinemann, A; Stauber, R E

1996-09-01

Nitric oxide (NO) is discussed as a mediator of the splanchnic hyperaemia in portal hypertension. We assessed the vasorelaxation by the NO-dependent vasodilator acetylcholine, the NO donor 3-morpholino-sydnonimine (SIN-1) and forskolin, a stimulator of the adenylate cyclase pathway in potassium-preconstricted isolated perfused mesenteric arteries of portal vein-ligated and sham-operated rats. Dilator responses to acetylcholine and SIN-1 were significantly enhanced in vessels of portal vein-ligated rats as compared to sham-operated rats, whereas no difference was found in forskolin-induced vasodilatation. This suggests enhanced reactivity of the vasculature to NO in experimental portal hypertension.

A Novel Predictor of Posttransplant Portal Hypertension in Adult-To-Adult Living Donor Liver Transplantation: Increased Estimated Spleen/Graft Volume Ratio.

PubMed

Gyoten, Kazuyuki; Mizuno, Shugo; Kato, Hiroyuki; Murata, Yasuhiro; Tanemura, Akihiro; Azumi, Yoshinori; Kuriyama, Naohisa; Kishiwada, Masashi; Usui, Masanobu; Sakurai, Hiroyuki; Isaji, Shuji

2016-10-01

In adult living donor liver transplantation (ALDLT), graft-to-recipient weight ratio of less than 0.8 is incomplete for predicting portal hypertension (>20 mm Hg) after reperfusion. We aimed to identify preoperative factors contributing to portal venous pressure (PVP) after reperfusion and to predict portal hypertension, focusing on spleen volume-to-graft volume ratio (SVGVR). In 73 recipients with ALDLT between 2002 and 2013, first we analyzed survival according to PVP of 20 mm Hg as the threshold, evaluating the efficacy of splenectomy. Second, we evaluated various preoperative factors contributing to portal hypertension after reperfusion. All of the recipients with PVP greater than 20 mm Hg (n = 19) underwent PVP modulation by splenectomy, and their overall survival was favorable compared with 54 recipients who did not need splenectomy (PVP ≤ 20 mm Hg). Graft-to-recipient weight ratio had no correlation with PVP.Multivariate analysis revealed that estimated graft and spleen volume were significant factors contributing to PVP after reperfusion (P < 0.0001 and P < 0.0001, respectively). Furthermore, estimated SVGVR showed a significant negative correlation to PVP after reperfusion (R = 0.652), and the best cutoff value for portal hypertension was 0.95. In ALDLT, preoperative assessment of SVGVR is a good predictor of portal hypertension after reperfusion can be used to indicate the need for splenectomy before reperfusion.

Dihydroartemisinin counteracts fibrotic portal hypertension via farnesoid X receptor-dependent inhibition of hepatic stellate cell contraction.

PubMed

Xu, Wenxuan; Lu, Chunfeng; Zhang, Feng; Shao, Jiangjuan; Yao, Shunyu; Zheng, Shizhong

2017-01-01

Portal hypertension is a frequent pathological symptom occurring especially in hepatic fibrosis and cirrhosis. Current paradigms indicate that inhibition of hepatic stellate cell (HSC) activation and contraction is anticipated to be an attractive therapeutic strategy, because activated HSC dominantly facilitates an increase in intrahepatic vein pressure through secreting extracellular matrix and contracting. Our previous in vitro study indicated that dihydroartemisinin (DHA) inhibited contractility of cultured HSC by activating intracellular farnesoid X receptor (FXR). However, the effect of DHA on fibrosis-related portal hypertension still requires clarification. In this study, gain- and loss-of-function models of FXR in HSC were established to investigate the mechanisms underlying DHA protection against chronic CCl 4 -caused hepatic fibrosis and portal hypertension. Immunofluorescence staining visually showed a decrease in FXR expression in CCl 4 -administrated rat HSC but an increase in that in DHA-treated rat HSC. Serum diagnostics and morphological analyses consistently indicated that DHA exhibited hepatoprotective effects on CCl 4 -induced liver injury. DHA also reduced CCl 4 -caused inflammatory mediator expression and inflammatory cell infiltration. These improvements were further enhanced by INT-747 but weakened by Z-guggulsterone. Noteworthily, DHA, analogous to INT-747, significantly lowered portal vein pressure and suppressed fibrogenesis. Experiments on mice using FXR shRNA lentivirus consolidated the results above. Mechanistically, inhibition of HSC activation and contraction was found as a cellular basis for DHA to relieve portal hypertension. These findings demonstrated that DHA attenuated portal hypertension in fibrotic rodents possibly by targeting HSC contraction via a FXR activation-dependent mechanism. FXR could be a target molecule for reducing portal hypertension during hepatic fibrosis. © 2016 Federation of European Biochemical Societies.

[Gastric ulcers in liver cirrhosis and their exposure to low-intensity infrared laser radiation].

PubMed

Vorozheĭkin, V M; Nazyrov, F G; Artykov, Sh N; Baĭbekov, I M

1992-01-01

By means of light, scanning electron microscopy and planimetry, the pathomorphology of gastric ulcers (GU) was investigated in patients who had died from liver cirrhosis (LC) and in rats with experimental portal hypertension (PH). It was noticed that GU are frequent (13.3%) in patients with LC. Experimental GU induced in rats with PH persist longer and their areas are larger than in ulcers without cirrhosis. The treatment of ulcers through abdominal wall with low-intensive infrared laser irradiation (LILR) causes significant acceleration of their epithelialization in the rat without cirrhosis. Ulcers in the animals with cirrhosis do not repair under LILR influence, but their area significantly decreases after forming collateral shunting. The congestion in the portal system is the main pathogenetic element in gastroesophageal ulcerogenesis. The laser therapy without decreasing portal hypertension is not efficient.

Twenty-five years after the first TIPS in Spain.

PubMed

Bilbao Jaureguízar, J I

2016-01-01

The incorporation, 25 years ago, of transjugular intrahepatic portosystemic shunting, better known by the acronym TIPS, represents an indisputable improvement in the treatment and management of patients with symptoms due to portal hypertension. This article discusses the origins of the technique and the technical innovations that have been progressively added through the years. The implantation of coated stents, which protect the stent from processes in the parenchymal track that can lead to stenosis, have helped ensure long-term patency, thus reducing the need for reintervention. Solid evidence from valuable publications has situated TIPS at the forefront of the treatment options in a wide variety of clinical situations associated with portal hypertension. Copyright © 2016 SERAM. Published by Elsevier España, S.L.U. All rights reserved.

Current status and future possibilities of transjugular intrahepatic portosystemic shunts in the management of portal hypertension.

PubMed

Radosevich, P M; LaBerge, J M; Gordon, R L

1994-01-01

Transjugular intrahepatic portosystemic shunt (TIPS) is an exciting new method for treating complications of cirrhosis. Technical advances have allowed TIPS to be widely applied in the treatment of variceal bleeding. This article presents and discusses the results of recent experiences in TIPS placement. TIPS can be successfully placed in almost all patients. The complication rate of the procedure is low. TIPS is an effective means of controlling variceal bleeding and is especially useful for controlling bleeding in patients awaiting liver transplantation. It may also have a role in the treatment of ascites and other conditions related to portal hypertension. The most important issue facing TIPS is the long-term patency of the shunt. Potential solutions to the problem of long-term shunt patency are discussed.

TIPS treatment in a patient with severe lower gastrointestinal bleeding with a misdiagnosis of cirrhotic portal hypertension.

PubMed

Laborda, Alicia; Guirola, José Andrés; Medrano, Joaquín; Simón, Miguel Ángel; Ioakeim, Ignatios; de-Gregorio, Miguel Ángel

2015-12-01

Abernethy malformation is a rare abnormal embryological development of splanchnic venous system characterised by the presence of a congenital extrahepatic portosystemic shunt. We present a rare case of an adult male patient that was admitted with severe lower gastrointestinal bleeding, requiring multiple blood transfusions. The patient's medical history and the laboratory tests performed led to the misdiagnosis of a congenital Abernethy malformation. We present a rare case, discussing the reasons for the misdiagnosis and we conclude that management of clinical data and imaging are highly important to discard these types of congenital malformations that can mimic a portal hypertension condition.

Case report of a modified Meso-Rex bypass as a treatment technique for late-onset portal vein cavernous transformation with portal hypertension after adult deceased-donor liver transplantation.

PubMed

Han, Dongdong; Tang, Rui; Wang, Liang; Li, Ang; Huang, Xin; Shen, Shan; Dong, Jiahong

2017-06-01

Portal vein thrombosis is a complication after liver transplantation and cavernous transformation of the portal vein (CTPV) is a result of portal vein thrombosis, with symptoms of portal hypertension revealed by an enhanced CT scan. Meso-Rex bypass is an artificial shunt connecting the left portal vein to the superior mesenteric vein and is mainly used for idiopathic cavernomas. This technique is also used for post-transplant portal vein thrombosis in pediatric patients thereby bypassing obstructed sites of the extrahepatic portal vein. Here we report about an adult patient who was treated by connecting the cystic part of the portal vein to the splenic vein instead of the superior mesenteric vein. An adult male patient with post-liver transplantation portal vein cavernous transformation suffered from hypersplenism and elevated hepatic enzymes. The last follow up revealed irregular and obvious hypersplenism, and splenomegaly had occurred, while an enhanced CT scan revealed serious esophagogastric varices and CTPV in addition to occluded right and common PV trunks. The patient was treated by connecting the cystic part of the portal vein to the splenic vein instead of the superior mesenteric vein. After the operation, a satisfactory velocity was confirmed 1 month postoperatively and the shunt still remained patent at the 6-month postoperation follow-up. A Meso-Rex bypass intervention connecting the left portal vein to the splenic vein instead of the superior mesenteric vein after liver transplantation in an adult patient with right and common portal vein occlusions has been successfully performed as an alternative approach.

Pattern of venous collateral development after splenic vein occlusion in an extended Whipple procedure : comparison with collateral vein pattern in cases of sinistral portal hypertension.

PubMed

Strasberg, Steven M; Bhalla, Sanjeev; Sanchez, Luis A; Linehan, David C

2011-11-01

The risks of developing sinistral portal hypertension as a result of occlusion of the splenic vein close to its termination during a Whipple procedure are unclear. Our purpose was to compare the pattern of venous collateral development after splenic vein ligation in an extended Whipple procedure with the pattern of collateral development in cases of sinistral portal hypertension. Five patients underwent an extended Whipple procedure in which the splenic vein was divided and not reconstructed. Six to eight months later detailed mapping of venous return from the spleen was determined by contrast-enhanced multidetector computed tomography or in one case by 3D contrast-enhanced MRI. Spleen size and length of residual patent splenic vein were also measured. The literature on sinistral portal hypertension was evaluated to ascertain whether the venous collateral pattern in cases of left-sided portal hypertension was similar to the pattern that developed when the splenic vein was ligated at its termination in the Whipple procedure. A length of splenic vein remained patent in all five patients, measuring 4.5 to 11.5 cm from the spleen. Splenomegaly did not develop. Blood returned from the spleen by multiple collaterals including collaterals in the omentum and mesocolon. These types of collaterals do not develop in sinistral portal hypertension, nor is residual patent splenic vein seen. Ligation of the splenic vein close to its termination in five patients resulted in a pattern of venous return different from patients that have developed left-sided portal hypertension.

An update on the management of Budd-Chiari syndrome: the issues of timing and choice of treatment.

PubMed

Mancuso, Andrea

2015-03-01

Because of the rarity of Budd-Chiari syndrome (BCS), the flow chart of management comes from expert opinion and is not evidence based. To report an update on the management of BCS. I performed a review on published papers on BCS in an attempt to speculate in particular on the timing and the choice of treatment. Some authors suggest that the management of BCS should follow a step-wise strategy. Anticoagulation and medical therapy should be the first-line treatment. Revascularization or transjugular intrahepatic portosystemic shunt should be performed in case of no response to medical therapy. Orthotopic liver transplant should be used as a rescue therapy. The biggest criticism of this flow chart is that it is based on the assumption that patients with BCS should receive further treatment only when hemodynamic effects on portal hypertension become clinically evident, thus paying little attention to the chronic ischemic liver damage effects on hepatic function and to the possibility of preventing liver failure by relieving impaired hepatic veins outflow. Recently, I presented a proposal of a new algorithm for the management of BCS, in which medical therapy alone is suggested only for patients without any sign of portal hypertension, irrespective of whether early interventional treatment is suggested when either any symptoms or signs of portal hypertension appear, with the aim of preventing hepatic fibrosis development, disease progression, and finally improving outcome. Given that the benefit of treatments for BCS is not under debate, guidelines for the management of BCS should be re-evaluated and updated, with particular attention to both the timing and the choice of treatment.

Histological Spectrum of Idiopathic Noncirrhotic Portal Hypertension in Liver Biopsies From Dialysis Patients.

PubMed

Lee, Hwajeong; Ainechi, Sanaz; Singh, Mandeep; Ells, Peter F; Sheehan, Christine E; Lin, Jingmei

2015-09-01

Liver biopsy is performed for various indications in dialysis patients. Being a less-common subset, the hepatic pathology in renal dialysis is not well documented. Idiopathic noncirrhotic portal hypertension (INCPH) is a clinical entity associated with unexplained portal hypertension and/or a spectrum of histopathological vascular changes in the liver. After encountering INCPH and vascular changes of INCPH in 2 renal dialysis patients, we sought to further investigate this noteworthy association. A random search for patients on hemodialysis or peritoneal dialysis with liver biopsy was performed. Hematoxylin and eosin, reticulin, trichrome, and CK7 stains were performed on formalin-fixed, paraffin-embedded tissue sections. Histopathological features were reviewed, and the results were correlated with clinical findings. In all, 13 liver biopsies were retrieved. The mean cumulative duration of dialysis was 50 months (range = 17 months to 11 years). All patients had multiple comorbidities. Indications for biopsy were a combination of abnormal liver function tests (6), portal hypertension (4), ascites (3), and possible cirrhosis (3). Two patients with portal hypertension underwent multiple liver biopsies for diagnostic purposes. All (100%) biopsies showed some histological features of INCPH, including narrowed portal venous lumen (9), increased portal vascular channels (8), shunt vessels (3), dilated sinusoids (9), regenerative nodule (5), and features of venous outflow obstruction (3). No cirrhosis was identified. Liver biopsies from patients on dialysis demonstrate histopathological vascular changes of INCPH. Some (31%) patients present with portal hypertension without cirrhosis. The histological changes may be reflective of underlying risk factors for INCPH in this group. © The Author(s) 2015.

Surgical strategy according to the anatomical types of congenital portosystemic shunts in children.

PubMed

Matsuura, Toshiharu; Takahashi, Yoshiaki; Yanagi, Yusuke; Yoshimaru, Koichiro; Yamamura, Kenichiro; Morihana, Eiji; Nagata, Hazumu; Uike, Kiyoshi; Takada, Hidetoshi; Taguchi, Tomoaki

2016-12-01

Congenital portosystemic shunts (CPSS) with intrahepatic portal vein (IHPV) hypoplasia or absence cause encephalopathy or pulmonary hypertension (PH). Acute shunt closure may result in postoperative portal hypertension. The aim of this study was to propose a surgical strategy according to the anatomical types of CPSS and IHPV. Twenty-three CPSS patients were diagnosed from1990 to 2015. All patients were evaluated by computed tomography, angiography, and PV pressure monitoring under a shunt occlusion test. CPSS were categorized into 5 types according to the anatomical shunt location. The median age at diagnosis was 34months. Three of 23 total patients, who had an extrahepatic portosystemic shunt with a hypoplastic IHPV, died before treatment initiation because of severe PH. Fourteen cases received surgical or interventional treatment at the median age of 5years. A total of 6 cases received surgical therapy, including liver transplants for 2 absent IHPV cases. The remaining 8 cases received interventional coiling. All shunt ligations were successfully accomplished in 1-stage ligation without any complications. After the treatment, the hypoplastic IHPV gradually enlarged with an efficient portal inflow. A precise pretreatment anatomical evaluation of CPSS and IHPV types is mandatory for the selection of surgical treatment. Diagnostic study - level II and treatment study - level III. Copyright © 2016 Elsevier Inc. All rights reserved.

Reliability in endoscopic diagnosis of portal hypertensive gastropathy

PubMed Central

de Macedo, George Fred Soares; Ferreira, Fabio Gonçalves; Ribeiro, Maurício Alves; Szutan, Luiz Arnaldo; Assef, Mauricio Saab; Rossini, Lucio Giovanni Battista

2013-01-01

AIM: To analyze reliability among endoscopists in diagnosing portal hypertensive gastropathy (PHG) and to determine which criteria from the most utilized classifications are the most suitable. METHODS: From January to July 2009, in an academic quaternary referral center at Santa Casa of São Paulo Endoscopy Service, Brazil, we performed this single-center prospective study. In this period, we included 100 patients, including 50 sequential patients who had portal hypertension of various etiologies; who were previously diagnosed based on clinical, laboratory and imaging exams; and who presented with esophageal varices. In addition, our study included 50 sequential patients who had dyspeptic symptoms and were referred for upper digestive endoscopy without portal hypertension. All subjects underwent upper digestive endoscopy, and the images of the exam were digitally recorded. Five endoscopists with more than 15 years of experience answered an electronic questionnaire, which included endoscopic criteria from the 3 most commonly used Portal Hypertensive Gastropathy classifications (McCormack, NIEC and Baveno) and the presence of elevated or flat antral erosive gastritis. All five endoscopists were blinded to the patients’ clinical information, and all images of varices were deliberately excluded for the analysis. RESULTS: The three most common etiologies of portal hypertension were schistosomiasis (36%), alcoholic cirrhosis (20%) and viral cirrhosis (14%). Of the 50 patients with portal hypertension, 84% were Child A, 12% were Child B, 4% were Child C, 64% exhibited previous variceal bleeding and 66% were previously endoscopic treated. The endoscopic parameters, presence or absence of mosaic-like pattern, red point lesions and cherry-red spots were associated with high inter-observer reliability and high specificity for diagnosing Portal Hypertensive Gastropathy. Sensitivity, specificity and reliability for the diagnosis of PHG (%) were as follows: mosaic-like pattern (100; 92.21; High); fine pink speckling (56; 76.62; Unsatisfactory); superficial reddening (69.57; 66.23; Unsatisfactory); red-point lesions (47.83; 90.91; High); cherry-red spots (39.13; 96.10; High); isolated red marks (43.48; 88.31; High); and confluent red marks (21.74; 100; Unsatisfactory). Antral elevated erosive gastritis exhibited high reliability and high specificity with respect to the presence of portal hypertension (92%) and the diagnosis of portal hypertensive gastropathy (88.31%). CONCLUSION: The most suitable endoscopic criteria for the diagnosis of PHG were mosaic-like pattern, red-point lesions and cherry-red spots with no subdivisions, which were associated with a high rate of inter-observer reliability. PMID:23858376

Blood in the gastric lumen increases splanchnic blood flow and portal pressure in portal-hypertensive rats.

PubMed

Chen, L; Groszmann, R J

1996-10-01

In portal-hypertensive humans, portal blood flow and pressure increase after a meal. These hemodynamic changes may increase variceal rupture risk. The aim of this study was to determine whether blood in the stomach lumen increases splanchnic flow and portal pressure (PP) in portal-hypertensive rats. superior mesenteric artery flow and PP were measured in conscious, unrestrained, fasted partial portal vein-ligated rats with chronically implanted Doppler flow probes or portal vein catheters before and after gavage with heparinized, warmed blood from donor rats, air, standard meal, or empty tube. Percentage of changes in flow and pressure from baseline were significantly greater after gavage with blood (an increase of 22.6% +/- 3.5% and an increase of 16.4% +/- 3.1%, respectively) than empty tube (an increase of 3.4% +/- 0.6% and a decrease of 5.4% +/- 3.5%, respectively) (P < 0.005). Percentage of changes in flow and pressure were slightly but insignificantly greater after gavage with air vs. empty tube (P < 0.005). In portal-hypertensive rats, blood in the stomach lumen significantly increases splanchnic blood flow and PP. Splanchnic hyperemia from absorption of blood's calories probably contributes to these hemodynamic changes. In patients with variceal hemorrhage, blood in the stomach may increase the risk of persistent variceal bleeding or rebleeding.

Laparoscopic splenectomy and azygoportal disconnection for bleeding varices with hypersplenism.

PubMed

Wang, Yue D; Ye, Huan; Ye, Zai Y; Zhu, Yang W; Xie, Zhi J; Zhu, Jin H; Liu, Jin M; Zhao, Ting

2008-02-01

Bleeding from esophageal varices is an important cause of morbidity and mortality in patients with portal hypertension. The ideal surgical procedure should control bleeding with as little impairment of liver function as possible and with low rates of encephalopathy. Recently, significant progress in laparoscopic technology has enabled laparoscopic splenectomy and devascularization of the lower esophagus and upper stomach in a less invasive way. In this paper, we present preliminary results for 25 patients in whom laparoscopic splenectomy and azygoportal disconnection were performed. Laparoscopic splenectomy and devascularization of the lower esophagus and upper stomach were performed in 25 patients with cirrhosis, bleeding portal hypertension, and secondary hypersplenism between January 2000 and October 2006. Among them, 5 patients underwent a laparoscopic modified Sugiura procedure, the lower esophagus was transected, and then reanastomosed with a circular stapler. Laparoscopic splenectomy and azygoportal disconnection were completed in all patients, except in 1 conversion, without significant morbidity. The operation time ranged from 4.0 to 5.5 hours and the blood loss was 100-400 mL. The postoperative hospital stay was 6-15 days. During a postoperative follow-up period of 3 months to 5 years in 22 patients, neither esophagus variceal bleeding nor encephalopathy has recurred. Laparoscopic splenectomy and azygoportal disconnection are feasible, effective, and safe surgical procedures, and have all the benefits of minimally invasive surgery for patients with bleeding portal hypertension and hypersplenism. Laparoscopic splenectomy and azygoportal disconnection offer a new operative method for treatment of bleeding portal hypertension with hypersplenism.

Biophysics and clinical practice for regenerative processes in cirrhosis of the liver/of liver cirrhosis assisted by Delta-S Entropy Variation Systems.

PubMed

Marineo, G; Fesce, E

2006-12-01

The therapy in question uses an innovative bioengineering device denoted as ''Delta-S DVD Entropy Variation System''. Previous research indicated regression of cirrhosis as evaluated in its morphofunctional and symptomatological aspects. The aim of the study is to confirm and extend previous experimental observations by enhancing hemodynamic evaluation techniques. In order to clarify scar regression, it was decided to include in the endpoints a quantitative evaluation of portal hypertension called HVPG, which is sensitive to the breakdown of hepatic architecture and the influence of regeneration nodules and therefore the advance of cirrhosis. The experimental design consists of a self-controlled study carried out on Child A-B cirrhosis patients with portal hypertension (hepatic venous pressure gradient, HVPG > or = 10 mmHg). Five patients were enrolled, 4 HCV positive, one with autoimmune cirrhosis, all showing extensive symptoms. At the end of the treatment all patients showed a reduction in portal hypertension (mean reduction HVPG = 40.2%, P<0.011), together with an improved ultrasound flowmeter pattern and a sharp decrease or disappearance of the symptoms. No adverse effects were reported. Efficacy on autoimmune cirrhosis was unaffected. By means of a quantitative analysis of portal hypertension and of functional aspects, this study confirms that the Delta-S DVD system can lead to the regression of the scar component of cirrhosis, promote the regeneration of functioning liver tissue with positive effects on hepatic functionality and prevent symptoms and the risk of varicose vein rupture.

A Novel Predictor of Posttransplant Portal Hypertension in Adult-To-Adult Living Donor Liver Transplantation: Increased Estimated Spleen/Graft Volume Ratio

PubMed Central

Gyoten, Kazuyuki; Mizuno, Shugo; Kato, Hiroyuki; Murata, Yasuhiro; Tanemura, Akihiro; Azumi, Yoshinori; Kuriyama, Naohisa; Kishiwada, Masashi; Usui, Masanobu; Sakurai, Hiroyuki; Isaji, Shuji

2016-01-01

Background In adult living donor liver transplantation (ALDLT), graft-to-recipient weight ratio of less than 0.8 is incomplete for predicting portal hypertension (>20 mm Hg) after reperfusion. We aimed to identify preoperative factors contributing to portal venous pressure (PVP) after reperfusion and to predict portal hypertension, focusing on spleen volume-to-graft volume ratio (SVGVR). Methods In 73 recipients with ALDLT between 2002 and 2013, first we analyzed survival according to PVP of 20 mm Hg as the threshold, evaluating the efficacy of splenectomy. Second, we evaluated various preoperative factors contributing to portal hypertension after reperfusion. Results All of the recipients with PVP greater than 20 mm Hg (n = 19) underwent PVP modulation by splenectomy, and their overall survival was favorable compared with 54 recipients who did not need splenectomy (PVP ≤ 20 mm Hg). Graft-to-recipient weight ratio had no correlation with PVP. Multivariate analysis revealed that estimated graft and spleen volume were significant factors contributing to PVP after reperfusion (P < 0.0001 and P < 0.0001, respectively). Furthermore, estimated SVGVR showed a significant negative correlation to PVP after reperfusion (R = 0.652), and the best cutoff value for portal hypertension was 0.95. Conclusions In ALDLT, preoperative assessment of SVGVR is a good predictor of portal hypertension after reperfusion can be used to indicate the need for splenectomy before reperfusion. PMID:27472097

Proinflammatory Liver and Antiinflammatory Intestinal Mediators Involved in Portal Hypertensive Rats

PubMed Central

Aller, Maria Angeles; Vara, Elena; Garcia, Cruz; Palma, Maria Dolores; Arias, Jorge L.; Nava, Maria Paz; Arias, Jaime

2005-01-01

Proinflammatory (TNF-α, IL-1β, and NO) and antiinflammatory (IL-10, CO) levels were assayed in serum, liver, and small bowel in order to verify a hypothetic inflammatory etiopathogeny of portal hypertension that could be the cause of its evolutive heterogeneity. Male Wistar rats were divided into one control group (n = 11) and one group with a triple stenosing ligation of the portal vein (n = 23) after 28 days of evolution. In one subgroup of portal hypertensive rats, portal pressure, collateral venous circulation, mesenteric vasculopathy, and liver and spleen weights were determined. In the remaining rats with portal hypertension TNF-α, IL-1β, and IL-10 were quantified in liver and ileum by enzyme-linked immunosorbent assay. NO synthase activity was studied in liver and ileum. CO and NO were measured in portal and systemic blood by spectrophotometry and Griess reaction, respectively. Portal hypertensive rats with mayor spleen weight show hepatomegaly and mayor development of collateral circulation. Ileum release of IL-10 (0.30 ± 0.12 versus 0.14 ± 0.02 pmol/mg protein; P < .01) is associated with a liver production of both proinflammatory mediators (TNF-α: 2 ± 0.21 versus 1.32 ± 0.60 pmol/mg protein; P < .05, IL-1β: 19.17 ± 2.87 versus 5.96 ± 1.84 pmol/mg protein; P = .005, and NO: 132.10 ± 34.72 versus 61.05 ± 8.30 nmol/mL; P = .005) and an antiinflammatory mediator (CO: 6.49 ± 2.99 versus 3.03 ± 1.59 pmol/mL; P = .005). In short-term prehepatic portal hypertension a gut-liver inflammatory loop, which could be fundamental in the regulation both of the portal pressure and of its complications, could be proposed. PMID:16030393

Extension of a Computer Assisted Decision Support (CADS) Study to Improve Outcomes in Patients with Type 2 DM Treated by Primary Care Providers. Addendum

DTIC Science & Technology

2015-04-01

Type 2 diabetes (7-8). Hypertension is one of the most common co-morbidities associated with DM and substantially contributes to the macrovascular...challenged when attempting to upload their glucose data. Access to the patient portal , Diabetes Mellitus Everywhere (DME) in CDMP required installation of...antihypertensive treatment on cardiovascular disease risk in older diabetic patients with isolated systolic hypertension . Systolic hypertension in

Patient Portal Use and Blood Pressure Control in Newly Diagnosed Hypertension.

PubMed

Manard, William; Scherrer, Jeffrey F; Salas, Joanne; Schneider, F David

2016-01-01

Current evidence that patient portal use improves disease management is inconclusive. Randomized controlled trials have found no benefit of Web-based patient-provider communication for blood pressure (BP) control, but patients from these studies were not selected for uncontrolled hypertension, nor did measures of portal use occur in a real-world setting, as captured in the electronic medical record. This study determined whether patient portal use by patients with treated, incident hypertension was associated with achieving BP control. Between 2008 to 2010, 1571 patients with an incident hypertension diagnosis, ages 21 to >89 years, were identified from an academic medical center primary care patient data registry. Cox proportional hazard models were computed to estimate the association between portal use and incident BP control during follow-up (2011-2015), before and after adjusting for covariates. Covariates included sociodemographics, smoking, obesity and other physical and mental health comorbidities, and volume of health care utilization. After adjusting for age, portal users were more likely than nonusers to achieve BP control (hazard ratio, 1.24; 95% confidence interval, 1.06-1.45). After adjustment for sociodemographics, portal use was no longer associated with BP control (hazard ratio, 0.98; 95% confidence interval, 0.83-1.16). Patient sociodemographic factors, including race, sex, and socioeconomic status, account for the observation that portal use leads to BP control among persons with newly diagnosed hypertension. Further research is warranted to determine whether there are benefits of portal use for other chronic conditions. © Copyright 2016 by the American Board of Family Medicine.

von Willebrand factor as a novel noninvasive predictor of portal hypertension and esophageal varices in hepatitis B patients with cirrhosis.

PubMed

Wu, Hao; Yan, Shiping; Wang, Guangchuan; Cui, Shaobo; Zhang, Chunqing; Zhu, Qiang

2015-01-01

At present, there is no perfect noninvasive method to assess portal hypertension and esophageal varices. Early predicting esophageal varices can provide evidence for managing cirrhotic patients. We aimed to further investigate von Willebrand factor (vWF) as a noninvasive predictor of portal hypertension, especially of esophageal varices. A total of 60 hepatitis B patients with cirrhosis and 45 healthy subjects were enrolled in this study. Levels of six markers were examined. All patients underwent hepatic venous pressure gradient (HVPG) and esophagogastroduodenoscopy. We evaluated the performance of six factors for diagnosis of portal hypertension and esophageal varices. The vWF levels in liver tissues were observed by immunohistochemistry. Correlations between the level of vWF in liver tissues and HVPG and between levels of vWF in tissues and plasma were examined. Cutoff values of plasma vWF (1510.5 mU/mL and 1701 mU/mL) showed high positive predictive value (PPV, 90.2% and 87.5%) in predicting clinically significant portal hypertension and severe portal hypertension. Cutoff values of vWF (1414 mU/ml and 1990 mU/mL, PPV 90.3% and 86.3%, respectively) were provided to detect the presence and degree of esophageal varices. Linear correlations were observed between levels of vWF in liver tissues and HVPG (r(2) = 0.552, p < 0.001) and between the level of vWF in liver tissues and in plasma (r(2) = 0.461, p < 0.001). The vWF is a noninvasive predictor of portal hypertension and esophageal varices in hepatitis B patients with cirrhosis. Increased levels of vWF in liver tissues may induce the elevated plasma vWF levels, but molecular mechanism is needed for further study.

Role of the transjugular intrahepatic portosystemic shunt in the management of severe complications of portal hypertension in idiopathic noncirrhotic portal hypertension.

PubMed

Bissonnette, Julien; Garcia-Pagán, Juan Carlos; Albillos, Agustín; Turon, Fanny; Ferreira, Carlos; Tellez, Luis; Nault, Jean-Charles; Carbonell, Nicolas; Cervoni, Jean-Paul; Abdel Rehim, Mohamed; Sibert, Annie; Bouchard, Louis; Perreault, Pierre; Trebicka, Jonel; Trottier-Tellier, Félix; Rautou, Pierre-Emmanuel; Valla, Dominique-Charles; Plessier, Aurélie

2016-07-01

Idiopathic noncirrhotic portal hypertension is a heterogeneous group of diseases characterized by portal hypertension in the absence of cirrhosis. The efficacy and safety of transjugular intrahepatic portosystemic shunt (TIPS) in this population are unknown. The charts of patients with idiopathic noncirrhotic portal hypertension undergoing TIPS in seven centers between 2000 and 2014 were retrospectively reviewed. Forty-one patients were included. Indications for TIPS were recurrent variceal bleeding (n = 25) and refractory ascites (n = 16). Patients were categorized according to the presence (n = 27) or absence (n = 14) of significant extrahepatic comorbidities. Associated conditions were hematologic, prothrombotic, neoplastic, immune, and exposure to toxins. During follow-up (mean 27 ± 29 months), variceal rebleeding occurred in 7/25 (28%), including three with early thrombosis of the stent. Post-TIPS overt hepatic encephalopathy was present in 14 patients (34%). Eleven patients died, five due the liver disease or complications of the procedure and six because of the associated comorbidities. The procedure was complicated by hemoperitoneum in four patients (10%), which was fatal in one case. Serum creatinine (P = 0.005), ascites as indication for TIPS (P = 0.04), and the presence of significant comorbidities (P = 0.01) at the time of the procedure were associated with death. Mortality was higher in patients with significant comorbidities and creatinine ≥100 μmol/L (P < 0.001). In patients with idiopathic noncirrhotic portal hypertension who have normal kidney function or do not have severe extrahepatic conditions, TIPS is an excellent option to treat severe complications of portal hypertension. (Hepatology 2016;64:224-231). © 2016 by the American Association for the Study of Liver Diseases.

Place de la splénectomie dans la prise en charge de l’hypertension portale non cirrhotique: à propos de 3 cas

PubMed Central

Belhamidi, Mohamed Said; Hammi, Salah Eddine; Bouzroud, Mohamed; Benmoussa, Mustapha; ali, Abdelmounaim Ait; Bounaim, Ahmed

2017-01-01

L’hypertension portale non cirrhotique est une affection décrite pour la première fois par Guido BANTI en 1898 comme une affection associant une hypertension portale avec splénomégalie et anémie sur foie sain. Le diagnostic repose sur l’échographie abdominale, la splénoportographie et la biopsie hépatique. Le but de notre travail est d’évaluer la place de la splénectomie dans l’hypertension portale non cirrhotique à travers une étude rétrospective portant sur 3 malades dont 2 femmes et un homme pris en charge dans notre formation entre Janvier 2010 et Septembre 2016. Le diagnostic de l’hypertension portale idiopathique a été basé sur les critères suivants : une hypertension portale, la présence des varices oesophagiènnes avec une splénomégalie, l’absence de cirrhose ou d’autres affections hépatiques responsables de l’hypertension portale. La splénectomie a été réalisée chez les 3 malades. L’évolution après la splénectomie était marquée par la normalisation des signes cliniques, radiologiques et biologiques de cette affection, avec absence de récidive des varices œsophagiennes. La splénectomie associée à la ligature des varices œsophagiennes pourraient être suffisantes pour traiter ce syndrome et surtout ses conséquences sans avoir recours à une dérivation spléno-rénale. PMID:29255554

Adipokines in Liver Cirrhosis.

PubMed

Buechler, Christa; Haberl, Elisabeth M; Rein-Fischboeck, Lisa; Aslanidis, Charalampos

2017-06-29

Liver fibrosis can progress to cirrhosis, which is considered a serious disease. The Child-Pugh score and the model of end-stage liver disease score have been established to assess residual liver function in patients with liver cirrhosis. The development of portal hypertension contributes to ascites, variceal bleeding and further complications in these patients. A transjugular intrahepatic portosystemic shunt (TIPS) is used to lower portal pressure, which represents a major improvement in the treatment of patients. Adipokines are proteins released from adipose tissue and modulate hepatic fibrogenesis. These proteins affect various biological processes that are involved in liver function, including angiogenesis, vasodilation, inflammation and deposition of extracellular matrix proteins. The best studied adipokines are adiponectin and leptin. Adiponectin protects against hepatic inflammation and fibrogenesis, and leptin functions as a profibrogenic factor. These and other adipokines are supposed to modulate disease severity in patients with liver cirrhosis. Consequently, circulating levels of these proteins have been analyzed to identify associations with parameters of hepatic function, portal hypertension and its associated complications in patients with liver cirrhosis. This review article briefly addresses the role of adipokines in hepatitis and liver fibrosis. Here, studies having analyzed these proteins in systemic blood in cirrhotic patients are listed to identify adipokines that are comparably changed in the different cohorts of patients with liver cirrhosis. Some studies measured these proteins in systemic, hepatic and portal vein blood or after TIPS to specify the tissues contributing to circulating levels of these proteins and the effect of portal hypertension, respectively.

Adipokines in Liver Cirrhosis

PubMed Central

Haberl, Elisabeth M.; Rein-Fischboeck, Lisa; Aslanidis, Charalampos

2017-01-01

Liver fibrosis can progress to cirrhosis, which is considered a serious disease. The Child-Pugh score and the model of end-stage liver disease score have been established to assess residual liver function in patients with liver cirrhosis. The development of portal hypertension contributes to ascites, variceal bleeding and further complications in these patients. A transjugular intrahepatic portosystemic shunt (TIPS) is used to lower portal pressure, which represents a major improvement in the treatment of patients. Adipokines are proteins released from adipose tissue and modulate hepatic fibrogenesis. These proteins affect various biological processes that are involved in liver function, including angiogenesis, vasodilation, inflammation and deposition of extracellular matrix proteins. The best studied adipokines are adiponectin and leptin. Adiponectin protects against hepatic inflammation and fibrogenesis, and leptin functions as a profibrogenic factor. These and other adipokines are supposed to modulate disease severity in patients with liver cirrhosis. Consequently, circulating levels of these proteins have been analyzed to identify associations with parameters of hepatic function, portal hypertension and its associated complications in patients with liver cirrhosis. This review article briefly addresses the role of adipokines in hepatitis and liver fibrosis. Here, studies having analyzed these proteins in systemic blood in cirrhotic patients are listed to identify adipokines that are comparably changed in the different cohorts of patients with liver cirrhosis. Some studies measured these proteins in systemic, hepatic and portal vein blood or after TIPS to specify the tissues contributing to circulating levels of these proteins and the effect of portal hypertension, respectively. PMID:28661458

Inhibition of soluble epoxide hydrolase lowers portal hypertension in cirrhotic rats by ameliorating endothelial dysfunction and liver fibrosis.

PubMed

Deng, Wensheng; Zhu, Yiming; Lin, Jiayun; Zheng, Lei; Zhang, Chihao; Luo, Meng

2017-07-01

Epoxyeicostrienoic acids (EETs) are arachidonic acid derived meditators which are catalyzed by soluble epoxide hydrolase (sEH) to less active dihydroeicostrienoics acids (DHETS). The aim of our study is to investigate the effects of sEH inhibition on hepatic and systemic hemodynamics, hepatic endothelial dysfunction, and hepatic fibrosis in CCl4 cirrhotic rats. The sEH inhibitor,trans-4-{4-[3-(4-trifluoromethoxyphenyl)-ureido]cyclohexyloxy}benzoic acid (t-TUCB) was administered to stabilize hepatic EETs by gavage at a dose of 1mg/kg/d. Our results showed that hepatic sEH expression was markedly increased in portal hypertension, and led to a lower ratio of EETs/DHETs which was effectively reversed by t-TUCB administration. t-TUCB significantly decreased portal pressure without significant changes in systemic hemodynamics, which was associated with the attenuation of intrahepatic vascular resistance (IHVR) and liver fibrosis. t-TUCB ameliorated endothelial dysfunction, increased hepatic endothelial nitric oxide synthase (eNOS) phosphorylation and nitric oxide (NO) production. In addition, t-TUCB significantly reduced alpha-Smooth Muscle Actin (α-SMA) expression and liver fibrosis, which was associated with a decrease in NF-κB signaling. Taken together, inhibition of sEH reduces portal pressure, liver fibrosis and attenuates hepatic endothelial dysfunction in cirrhotic rats. Our results indicate that sEH inhbitors may be useful in the treatment of portal hypertension in patients with cirrhosis. Copyright © 2017 Elsevier Inc. All rights reserved.

[Small-diameter portosystemic shunts: indications and limitations].

PubMed

Angel Mercado, M; Granados-García, J; Barradas, F; Chan, C; Contreras, J L; Orozco, H; Angel-Mercado, M

1998-01-01

Low diameter porto-systemic shunts for the treatment of portal hypertension bleeding have emerged as a consequence of the technical development of vascular grafts (PTFE) that allow the use of a narrow lumen. The experience with this kind of operation at the Instituto Nacional de la Nutrición Salvador Zubirán, Mexico City during a 6-year period is reported. There were twenty-seven patients with good liver function (Child-Pugh A-B) were operated or electively, average Age 47.5 years (range 17-71), twenty three patients with liver cirrhosis, one with portal fibrosis and three with idiopathic portal hypertension. Operative mortality: 4%. Rebleeding: 14%. Postoperative encephalopathy was observed in 14 of 27, three of them being grade III-IV (11%). In the remaining 11 cases, it was mild and easily controlled. Postoperative angiography showed shunt patency in 81% of the cases; in 33% of the cases, portal vein diameter reduction was shown, as well as two cases with portal vein thrombosis. In 77% of the cases, adequate postoperative quality of life was observed. Survival (Kaplan-Meier): 86% at 12 months and 56% at 60 months. These kinds of shunts are a good alternate choice for patients considered for surgery, in which other portal blood flow preserving procedures (selective shunts, devascularization with transection) are not feasible.

Severe portopulmonary hypertension in congenital hepatic fibrosis.

PubMed

Hsu, Chen-Ming; Chiu, Cheng-Tang; Lien, Jau-Min; Ng, Kwai-Fong

2003-03-01

Portopulmonary hypertension is a rare complication of portal hypertension. Although epoprostenol infusion, nitric oxide inhalation, isosorbide-5-mononitrate, nitroglycerin, and calcium channel blockers may reduce pulmonary artery pressure in patients with portopulmonary hypertension, the prognosis remains poor. We present a case of congenital hepatic fibrosis associated with pulmonary hypertension. A 42-year-old man with congenital hepatic fibrosis visited our hospital with syncope. The man had suffered from breathlessness on exertion for 2 weeks before the episode of syncope. He also had a history of portal hypertension with documented gastric cardiac varices at the age of 28 years. Despite undergoing intensive care, the patient died 1 week after admission owing to severe right-sided heart failure. Autopsy revealed dilatation of the right atrium and right ventricle grossly and plexogenic pulmonary arteriopathy microscopically. Accurate diagnosis of portopulmonary hypertension requires awareness of the disease and a high index of suspicion when examining patients with portal hypertension and dyspnea.

Gut-liver axis, cirrhosis and portal hypertension: the chicken and the egg.

PubMed

Arab, Juan P; Martin-Mateos, Rosa M; Shah, Vijay H

2018-02-01

The term gut-liver axis is used to highlight the close anatomical and functional relationship between the intestine and the liver. The intestine has a highly specialized epithelial membrane which regulates transport across the mucosa. Due to dysbiosis, impairment of the intestinal barrier and altered immunity status, bacterial products can reach the liver through the portal vein, where they are recognized by specific receptors, activate the immune system and lead to a proinflammatory response. Gut microbiota and bacterial translocation play an important role in the pathogenesis of chronic liver diseases, including alcoholic and non-alcoholic fatty liver disease, cirrhosis, and its complications, such as portal hypertension, spontaneous bacterial peritonitis and hepatic encephalopaty. The gut microbiota also plays a critical role as a modulator of bile acid metabolism which can also influence intestinal permeability and portal hypertension through the farnesoid-X receptor. On the other hand, cirrhosis and portal hypertension affect the microbiota and increase translocation, leading to a "chicken and egg" situation, where translocation increases portal pressure, and vice versa. A myriad of therapies targeting gut microbiota have been evaluated specifically in patients with chronic liver disease. Further studies targeting intestinal microbiota and its possible hemodynamic and metabolic effects are needed. This review summarizes the current knowledge about the role of gut microbiota in the pathogenesis of chronic liver diseases and portal hypertension.

Wounded Warrior Care and Reintegration Requires a Public-Private Partnership

DTIC Science & Technology

2013-06-01

hypertensive shock documentation. 8 Treatment of battle fatigue also changed in the Korean War from World War II by treating people on the front...Assistance Branch (JAL), “Integrated Disability Evaluation System Process”, April 2012, 1. http://www.hqmc.marines.mil/ Portals /135/Docs/JAL/Overview...located at http://www.woundedwarriorresourcecenter.com and established an e-Benefits portal which the service member can access after leaving the

Vascular diseases of the liver. Clinical Guidelines from the Catalan Society of Digestology and the Spanish Association for the Study of the Liver.

PubMed

Martín-Llahí, Marta; Albillos, Agustín; Bañares, Rafael; Berzigotti, Annalisa; García-Criado, M Ángeles; Genescà, Joan; Hernández-Gea, Virginia; Llop-Herrera, Elba; Masnou-Ridaura, Helena; Mateo, José; Navascués, Carmen A; Puente, Ángela; Romero-Gutiérrez, Marta; Simón-Talero, Macarena; Téllez, Luis; Turon, Fanny; Villanueva, Cándido; Zarrabeitia, Roberto; García-Pagán, Juan Carlos

2017-10-01

Despite their relatively low prevalence, vascular diseases of the liver represent a significant health problem in the field of liver disease. A common characteristic shared by many such diseases is their propensity to cause portal hypertension together with increased morbidity and mortality. These diseases are often diagnosed in young patients and their delayed diagnosis and/or inappropriate treatment can greatly reduce life expectancy. This article reviews the current body of evidence concerning Budd-Chiari syndrome, non-cirrhotic portal vein thrombosis, idiopathic portal hypertension, sinusoidal obstruction syndrome, hepatic vascular malformations in hereditary haemorrhagic telangiectasia, cirrhotic portal vein thrombosis and other rarer vascular diseases including arterioportal fistulas. It also includes a section on the diagnostic imaging of vascular diseases of the liver and their treatment from a haematological standpoint (study of thrombotic diathesis and anticoagulation therapy). All recommendations are based on published studies extracted from PubMed. The quality of evidence and strength of recommendations were rated in accordance with the GRADE system (Grading of Recommendations, Assessment Development and Evaluation). In the absence of sufficient evidence, recommendations were based on the opinion of the committee that produced the guide. Copyright © 2017 Elsevier España, S.L.U., AEEH y AEG. All rights reserved.

Glutamine synthetase activity and glutamate uptake in hippocampus and frontal cortex in portal hypertensive rats

PubMed Central

Acosta, Gabriela Beatriz; Fernández, María Alejandra; Roselló, Diego Martín; Tomaro, María Luján; Balestrasse, Karina; Lemberg, Abraham

2009-01-01

AIM: To study glutamine synthetase (GS) activity and glutamate uptake in the hippocampus and frontal cortex (FC) from rats with prehepatic portal vein hypertension. METHODS: Male Wistar rats were divided into sham-operated group and a portal hypertension (PH) group with a regulated stricture of the portal vein. Animals were sacrificed by decapitation 14 d after portal vein stricture. GS activity was determined in the hippocampus and FC. Specific uptake of radiolabeled L-glutamate was studied using synaptosome-enriched fractions that were freshly prepared from both brain areas. RESULTS: We observed that the activity of GS increased in the hippocampus of PH rats, as compared to control animals, and decreased in the FC. A significant decrease in glutamate uptake was found in both brain areas, and was more marked in the hippocampus. The decrease in glutamate uptake might have been caused by a deficient transport function, significantly and persistent increase in this excitatory neurotransmitter activity. CONCLUSION: The presence of moderate ammonia blood levels may add to the toxicity of excitotoxic glutamate in the brain, which causes alterations in brain function. Portal vein stricture that causes portal hypertension modifies the normal function in some brain regions. PMID:19533812

Rapamycin Ameliorates Inflammation and Fibrosis in the Early Phase of Cirrhotic Portal Hypertension in Rats through Inhibition of mTORC1 but Not mTORC2

PubMed Central

Wang, Weijie; Yan, Jiqi; Wang, Huakai; Shi, Minmin; Zhang, Mingjun; Yang, Weiping; Peng, Chenghong; Li, Hongwei

2014-01-01

Objective Hepatic stellate cells (HSCs) transdifferentiation and subsequent inflammation are important pathological processes involved in the formation of cirrhotic portal hypertension. This study characterizes the pathogenetic mechanisms leading to cholestatic liver fibrosis and portal hypertension, and focuses on mammalian target of rapamycin (mTOR) pathway as a potential modulator in the early phase of cirrhotic portal hypertension. Methods Early cirrhotic portal hypertension was induced by bile duct ligation (BDL) for three weeks. One week after operation, sham-operated (SHAM) and BDL rats received rapamycin (2 mg/kg/day) by intraperitoneal injection for fourteen days. Vehicle-treated SHAM and BDL rats served as controls. Fibrosis, inflammation, and portal pressure were evaluated by histology, morphometry, and hemodynamics. Expressions of pro-fibrogenic and pro-inflammatory genes in liver were measured by RT-PCR; alpha smooth muscle actin (α-SMA) and antigen Ki67 were detected by immunohistochemistry; expressions of AKT/mTOR signaling molecules, extracellular-signal-regulated kinase 1/2 (ERK1/2), p-ERK1/2, and interleukin-1 beta (IL-1β) were assessed by western blot. Results The AKT/mTOR signaling pathway was markedly activated in the early phase of cirrhotic portal hypertension induced by BDL in rats. mTOR blockade by rapamycin profoundly improved liver function by limiting inflammation, fibrosis and portal pressure. Rapamycin significantly inhibited the expressions of phosphorylated 70KD ribosomal protein S6 kinase (p-P70S6K) and phosphorylated ribosomal protein S6 (p-S6) but not p-AKT Ser473 relative to their total proteins in BDL-Ra rats. Those results suggested that mTOR Complex 1 (mTORC1) rather than mTORC2 was inhibited by rapamycin. Interestingly, we also found that the level of p-ERK1/2 to ERK1/2 was significantly increased in BDL rats, which was little affected by rapamycin. Conclusions The AKT/mTOR signaling pathway played an important role in the early phase of cirrhotic portal hypertension in rats, which could be a potential target for therapeutic intervention in the early phase of such pathophysiological progress. PMID:24404143

Rapamycin ameliorates inflammation and fibrosis in the early phase of cirrhotic portal hypertension in rats through inhibition of mTORC1 but not mTORC2.

PubMed

Wang, Weijie; Yan, Jiqi; Wang, Huakai; Shi, Minmin; Zhang, Mingjun; Yang, Weiping; Peng, Chenghong; Li, Hongwei

2014-01-01

Hepatic stellate cells (HSCs) transdifferentiation and subsequent inflammation are important pathological processes involved in the formation of cirrhotic portal hypertension. This study characterizes the pathogenetic mechanisms leading to cholestatic liver fibrosis and portal hypertension, and focuses on mammalian target of rapamycin (mTOR) pathway as a potential modulator in the early phase of cirrhotic portal hypertension. Early cirrhotic portal hypertension was induced by bile duct ligation (BDL) for three weeks. One week after operation, sham-operated (SHAM) and BDL rats received rapamycin (2 mg/kg/day) by intraperitoneal injection for fourteen days. Vehicle-treated SHAM and BDL rats served as controls. Fibrosis, inflammation, and portal pressure were evaluated by histology, morphometry, and hemodynamics. Expressions of pro-fibrogenic and pro-inflammatory genes in liver were measured by RT-PCR; alpha smooth muscle actin (α-SMA) and antigen Ki67 were detected by immunohistochemistry; expressions of AKT/mTOR signaling molecules, extracellular-signal-regulated kinase 1/2 (ERK1/2), p-ERK1/2, and interleukin-1 beta (IL-1β) were assessed by western blot. The AKT/mTOR signaling pathway was markedly activated in the early phase of cirrhotic portal hypertension induced by BDL in rats. mTOR blockade by rapamycin profoundly improved liver function by limiting inflammation, fibrosis and portal pressure. Rapamycin significantly inhibited the expressions of phosphorylated 70KD ribosomal protein S6 kinase (p-P70S6K) and phosphorylated ribosomal protein S6 (p-S6) but not p-AKT Ser473 relative to their total proteins in BDL-Ra rats. Those results suggested that mTOR Complex 1 (mTORC1) rather than mTORC2 was inhibited by rapamycin. Interestingly, we also found that the level of p-ERK1/2 to ERK1/2 was significantly increased in BDL rats, which was little affected by rapamycin. The AKT/mTOR signaling pathway played an important role in the early phase of cirrhotic portal hypertension in rats, which could be a potential target for therapeutic intervention in the early phase of such pathophysiological progress.

A successful case of deceased donor liver transplantation for a patient with intrahepatic arterioportal fistula.

PubMed

Takagi, Kosei; Yagi, Takahito; Yoshida, Ryuichi; Shinoura, Susumu; Umeda, Yuzo; Nobuoka, Daisuke; Watanabe, Nobuyuki; Kuise, Takashi; Sui, Kenta; Hirose, Akira; Tsuboi, Makiko; Ogasawara, Mitsunari; Iwasaki, Shinji; Saibara, Toshiji; Fujiwara, Toshiyoshi

2016-12-01

Intrahepatic arterioportal fistula (IAPF) is a rare cause of portal hypertension that is often difficult to treat with interventional radiology or surgery. Liver transplantation for IAPF is extremely rare. We report a case of bilateral diffuse IAPF with severe portal hypertension requiring deceased donor liver transplantation (DDLT). A 51-year-old woman with no past medical history was admitted to another hospital complaining of abdominal distension and marasmus. A computed tomography scan and digital subtraction angiography indicated a massive pleural effusion, ascites, and a very large IAPF. Several attempts of interventional embolization of the feeding artery failed to ameliorate arterioportal shunt flow. As ruptures of the esophageal varices became more frequent, hepatic encephalopathy worsened. After repeated, uncontrollable attacks of hepatic coma, the patient was referred to our facility for further treatment. Surgical approaches to IAPF other than liver transplantation were challenging because of diffuse collateralization; therefore, we placed the patient on the national waiting list for DDLT. Although her Model for End-Stage Liver Disease score was relatively low, she received a DDLT 2 months after the waiting period. The postoperative course was uneventful, and the patient was discharged 44 days after her transplant. Liver transplantation may be a valid treatment option for uncontrollable IAPF with severe portal hypertension. © 2016 The Authors Hepatology Research published by John Wiley & Sons Australia, Ltd on behalf of Japan Society of Hepatology.

[Hydatid cyst in the hepatic hilum causing a cavernous transformation in the portal vein].

PubMed

Gil-Egea, M J; Alameda, F; Girvent, M; Riera, R; Sitges-Serra, A

1998-05-01

Portal cavernomatosis consists in the substitution of the portal vein by many fine, twisting venules leading to the liver. This phenomenon is produced as a consequence of anterior thrombosis of the portal vein and is associated with chronic pancreatitis, cancer of the pancreas, intraabdominal sepsis and cholelithiasis. The symptomatology may be nul or present as obstructive jaundice or portal hypertension. Diagnosis is made by Doppler echography. The treatment is portal shunt when symptomatology is produced. In patients with cholelithiasis requiring surgery, the shunt is advised prior to biliary surgery since perioperative hemorrhage, if present, may be incoercible as in the case herein described. We present a 84-year-old woman with portal cavernomatosis the etiology of which was a hydatidic cyst located in the hepatic bifurcation and treated with mebendazol 10 years previously. This etiology has not been previously reported.

Ten-year experience of transjugular intrahepatic portosystemic shunt for noncirrhotic portal hypertension.

PubMed

Regnault, David; d'Alteroche, Louis; Nicolas, Charlotte; Dujardin, Fanny; Ayoub, Jean; Perarnau, Jean Marc

2018-05-01

Transjugular intrahepatic portosystemic shunt (TIPS) is considered to be well suited for the treatment of noncirrhotic portal hypertension (NCPHT) because of a usually severe portal hypertension (PHT) and a mild liver failure, but very less data are available. Records of patients referred for TIPS between 2004 and 2015 for NCPHT were reviewed. No patient should have clinical or biological or histological features of cirrhosis. Twenty-five patients with a wide variety of histological lesions (sinusoidal dilatations, granulomatosis, regenerative nodular hyperplasia, obliterative portal venopathy, or subnormal liver) and a wide variety of associated diseases (thrombophilia, sarcoidosis, common variable immunodeficiency, scleroderma, Castleman's disease, early primitive biliary cirrhosis, congenital liver fibrosis, chemotherapy, purinethol intake, and congenital varices) were included. Two complications occurred during the procedure: one periprosthetic hematoma and the other misposition of a covered stent. During the first month, two other patients had an early thrombosis, another had induced encephalopathy, and one died of early rebleeding. Two of these complications occurred in patients with cavernoma. With a mean follow-up of 39 months, 10 patients experienced at least one episode of spontaneous encephalopathy, with three of these patients requiring a stent reduction. Five patients had a recurrence of their initial symptoms, and one had an asymptomatic hemodynamic dysfunction. TIPS is effective in NCPHT but can be technically difficult, especially in the case of cavernoma. Good liver function does not prevent the occurrence of long-term encephalopathy.

A new rat model of portal hypertension induced by intraportal injection of microspheres

PubMed Central

Li, Xiang-Nong; Benjamin, IS; Alexander, B

1998-01-01

AIM: To produce a new rat model of portal hypertension by intraportal injection of microspheres. METHODS: Measured aliquots of single or different-sized microspheres (15, 40, 80μm) were injected into the portal vein to block intrahepatic portal radicals. The resultant changes in arterial,portal,hepatic venous and splenic pulp pressures were monitored. The liver and lungs were excised for histological examination. RESULTS: Portal venous pressure was elevated from basal value of 0.89-1.02 kPa to a steady-state of 1.98-3.19 kPa following the sequential injections of single- or different-sized microspheres, with a markedly lowered mean arterial pressure. However, a small-dose injection of 80 μm microspheres (1.8 × 105) produced a steady-state portal venous pressure of 2.53 × 0.17 kPa, and all rats showed normal arterial pressures. In addition, numerous microspheres were found in the lungs in all experimental groups. CONCLUSION: Portal hypertension can be reproduced in rats by intraportal injection of microspheres at a small dose of 80 μm (1.8 × 105). Intrahepatic portal-systemic shunts probably exist in the normal rat liver. PMID:11819236

Genetics Home Reference: progressive familial intrahepatic cholestasis

MedlinePlus

... the vein that supplies blood to the liver (portal hypertension), and an enlarged liver and spleen (hepatosplenomegaly). There ... Manual Consumer Version: Cholestasis Merck Manual Consumer Version: Portal Hypertension Orphanet: Progressive familial intrahepatic cholestasis Patient Support and ...

Management by the intensivist of gastrointestinal bleeding in adults and children

PubMed Central

2012-01-01

Intensivists are regularly confronted with the question of gastrointestinal bleeding. To date, the latest international recommendations regarding prevention and treatment for gastrointestinal bleeding lack a specific approach to the critically ill patients. We present recommendations for management by the intensivist of gastrointestinal bleeding in adults and children, developed with the GRADE system by an experts group of the French-Language Society of Intensive Care (Société de Réanimation de Langue Française (SRLF), with the participation of the French Language Group of Paediatric Intensive Care and Emergencies (GFRUP), the French Society of Emergency Medicine (SFMU), the French Society of Gastroenterology (SNFGE), and the French Society of Digestive Endoscopy (SFED). The recommendations cover five fields of application: management of gastrointestinal bleeding before endoscopic diagnosis, treatment of upper gastrointestinal bleeding unrelated to portal hypertension, treatment of upper gastrointestinal bleeding related to portal hypertension, management of presumed lower gastrointestinal bleeding, and prevention of upper gastrointestinal bleeding in intensive care. PMID:23140348

Case report and systematic literature review of a novel etiology of sinistral portal hypertension presenting with UGI bleeding: Left gastric artery pseudoaneurysm compressing the splenic vein treated by embolization of the pseudoaneurysm.

PubMed

Hakim, Seifeldin; Bortman, Jared; Orosey, Molly; Cappell, Mitchell S

2017-03-01

A novel case is reported of upper gastrointestinal (UGI) bleeding from sinistral portal hypertension, caused by a left gastric artery (LGA) pseudoaneurysm (PA) compressing the splenic vein (SV) that was successfully treated with PA embolization. A 41-year-old man with previous medical history of recurrent, alcoholic pancreatitis presented with several episodes of hematemesis and abdominal pain for 48 hours. Physical examination revealed a soft abdomen, with no abdominal bruit, no pulsatile abdominal mass, and no stigmata of chronic liver disease. The hemoglobin declined acutely from 12.3 to 9.3 g/dL. Biochemical parameters of liver function and routine coagulation profile were entirely within normal limits. Abdominal CT revealed a 5-cm-wide peripancreatic mass compressing the stomach and constricting the SV. Esophagogastroduodenoscopy showed blood oozing from portal hypertensive gastropathy, small nonbleeding gastric cardial and fundal varices, gastric compression from the extrinsic mass, and no esophageal varices. MRCP and angiography showed that the mass was vascular, arose from the LGA, compressed the mid SV without SV thrombosis, and caused sinistral portal hypertension. At angiography, the PA was angioembolized and occluded. The patient has been asymptomatic with no further bleeding and a stable hemoglobin level during 8 weeks of follow-up. Literature review of the 14 reported cases of LGA PA revealed that this report of acute UGI bleeding from sinistral portal hypertension from a LGA PA constricting the SV is novel; one previously reported patient had severe anemia without acute UGI bleeding associated with sinistral portal hypertension from a LGA PA. A patient presented with UGI bleeding from sinistral portal hypertension from a LGA PA compressing the SV that was treated by angiographic obliteration of the PA which relieved the SV compression and arrested the UGI bleeding. Primary therapy for this syndrome should be addressed to obliterate the PA and not the secondarily constricted SV.

Abnormal splenic artery diameter/hepatic artery diameter ratio in cirrhosis-induced portal hypertension

PubMed Central

Zeng, Dao-Bing; Dai, Chuan-Zhou; Lu, Shi-Chun; He, Ning; Wang, Wei; Li, Hong-Jun

2013-01-01

AIM: To determine an optimal cutoff value for abnormal splenic artery diameter/proper hepatic artery diameter (S/P) ratio in cirrhosis-induced portal hypertension. METHODS: Patients with cirrhosis and portal hypertension (n = 770) and healthy volunteers (n = 31) underwent volumetric computed tomography three-dimensional vascular reconstruction to measure the internal diameters of the splenic artery and proper hepatic artery to calculate the S/P ratio. The cutoff value for abnormal S/P ratio was determined using receiver operating characteristic curve analysis, and the prevalence of abnormal S/P ratio and associations between abnormal S/P ratio and major complications of portal hypertension were studied using logistic regression. RESULTS: The receiver operating characteristic analysis showed that the cutoff points for abnormal splenic artery internal diameter and S/P ratio were > 5.19 mm and > 1.40, respectively. The sensitivity, specificity, positive predictive value, and negative predictive value were 74.2%, 45.2%, 97.1%, and 6.6%, respectively. The prevalence of an abnormal S/P ratio in the patients with cirrhosis and portal hypertension was 83.4%. Patients with a higher S/P ratio had a lower risk of developing ascites [odds ratio (OR) = 0.708, 95%CI: 0.508-0.986, P = 0.041] and a higher risk of developing esophageal and gastric varices (OR = 1.483, 95%CI: 1.010-2.175, P = 0.044) and forming collateral circulation (OR = 1.518, 95%CI: 1.033-2.230, P = 0.034). After splenectomy, the portal venous pressure and maximum and mean portal venous flow velocities were reduced, while the flow rate and maximum and minimum flow velocities of the hepatic artery were increased (P < 0.05). CONCLUSION: The prevalence of an abnormal S/P ratio is high in patients with cirrhosis and portal hypertension, and it can be used as an important marker of splanchnic hemodynamic disturbances. PMID:23483462

Portal hypertension in patients with cirrhosis: indirect assessment of hepatic venous pressure gradient by measuring azygos flow with 2D-cine phase-contrast magnetic resonance imaging.

PubMed

Gouya, Hervé; Grabar, Sophie; Vignaux, Olivier; Saade, Anastasia; Pol, Stanislas; Legmann, Paul; Sogni, Philippe

2016-07-01

To measure azygos, portal and aortic flow by two-dimensional cine phase-contrast magnetic resonance imaging (2D-cine PC MRI), and to compare the MRI values to hepatic venous pressure gradient (HVPG) measurements, in patients with cirrhosis. Sixty-nine patients with cirrhosis were prospectively included. All patients underwent HVPG measurements, upper gastrointestinal endoscopy and 2D-cine PC MRI measurements of azygos, portal and aortic blood flow. Univariate and multivariate regression analyses were used to evaluate the correlation between the blood flow and HVPG. The performance of 2D-cine PC MRI to diagnose severe portal hypertension (HVPG ≥ 16 mmHg) was determined by receiver operating characteristic curve (ROC) analysis, and area under the curves (AUC) were compared. Azygos and aortic flow values were associated with HVPG in univariate linear regression model. Azygos flow (p < 10(-3)), aortic flow (p = 0.001), age (p = 0.001) and presence of varices (p < 10(-3)) were independently associated with HVPG. Azygos flow (AUC = 0.96 (95 % CI [0.91-1.00]) had significantly higher AUC than aortic (AUC = 0.64 (95 % CI [0.51-0.77]) or portal blood flow (AUC = 0.40 (95 % CI [0.25-0.54]). 2D-cine PC MRI is a promising technique to evaluate significant portal hypertension in patients with cirrhosis. • Noninvasive HVPG assessment can be performed with MRI azygos flow. • Azygos MRI flow is an easy-to-measure marker to detect significant portal hypertension. • MRI flow is more specific that varice grade to detect portal hypertension.

Stent Recanalization of Chronic Portal Vein Occlusion in a Child

DOE Office of Scientific and Technical Information (OSTI.GOV)

Cwikiel, Wojciech; Solvig, Jan; Schroder, Henrik

2000-07-15

An 8-year-old boy with a 21/2 year history of portal hypertension and repeated bleedings from esophageal varices, was referred for treatment. The 3.5-cm-long occlusion of the portal vein was passed and the channel created was stabilized with a balloon-expandable stent; a portosystemic stent-shunt was also created. The portosystemic shunt closed spontaneously within 1 month, while the recanalized segment of the portal vein remained open. The pressure gradient between the intrahepatic and extrahepatic portal vein branches dropped from 17 mmHg to 0 mmHg. The pressure in the portal vein dropped from 30 mmHg to 17 mmHg and the bleedings stopped. Themore » next dilation of the stent was performed 12 months later due to an increased pressure gradient; the gastroesophageal varices disappeared completely. Further dilation of the stent was planned after 2, 4, and 6 years.« less

Intraductal ultrasonographic anatomy of biliary varices in patients with portal hypertension.

PubMed

Takagi, Tadayuki; Irisawa, Atsushi; Shibukawa, Goro; Hikichi, Takuto; Obara, Katsutoshi; Ohira, Hiromasa

2015-01-01

The term, portal biliopathy, denotes various biliary abnormalities, such as stenosis and/or dilatation of the bile duct, in patients with portal hypertension. These vascular abnormalities sometimes bring on an obstructive jaundice, but they are not clear which vessels participated in obstructive jaundice. The aim of present study was clear the bile ductal changes in patients with portal hypertension in hopes of establishing a therapeutic strategy for obstructive jaundice caused by biliary varices. Three hundred and thirty-seven patients who underwent intraductal ultrasound (IDUS) during endoscopic retrograde cholangiography for biliary abnormalities were enrolled. Portal biliopathy was analyzed using IDUS. Biliary varices were identified in 11 (2.7%) patients. IDUS revealed biliary varices as multiple, hypoechoic features surrounding the bile duct wall. These varices could be categorized into one of two groups according to their location in the sectional image of bile duct: epicholedochal and paracholedochal. Epicholedochal varices were identified in all patients, but paracholedochal varices were observed only in patients with extrahepatic portal obstruction. IDUS was useful to characterize the anatomy of portal biliopathy in detail.

Endoscopic band ligation of internal haemorrhoids versus stapled haemorrhoidopexy in patients with portal hypertension.

PubMed

Zaher, Tarik; Ibrahim, Islam; Ibrahim, Amany

2011-03-01

Portal hypertension is common in Egypt as a sequela to the high prevalence of hepatitis C virus and bilharziasis. In portal hypertension internal haemorrhoids are frequently found. The aim of this work was to compare the outcome of endoscopic band ligation (EBL) of symptomatic internal haemorrhoids with that of stapled haemorrhoidopexy (SH) in Egyptian patients with portal hypertension. In this study, 26 portal hypertensive patients (with oesophageal and/or fundal varices) with a grade 2-4 internal haemorrhoids who had no coagulation disorders were randomised to treatment by EBL (13 patients) or SH (13 patients) after doing colonoscopy. Symptom scores of bleeding and prolapse were assessed before and after the intervention. Complications were recorded. Patients were followed up for 12months. Goligher's grades of internal haemorrhoids improved significantly (p=0.018) 12weeks after SH (from 2.9±0.8 to 0.4±0.5; p=0.001) and after EBL (from 2.8±0.8 to 1.1±0.8; p=0.001). Symptom (bleeding and prolapse) scores significantly improved 4weeks after both EBL (from 1.6±0.8 to 0.6±0.8; p<0.001 and from 1.6±0.9 to 0.5±0.5; p=0.002, respectively) and SH (from 1.8±0.8 to 0.2±0.4; p=0.002 and from 1.5±0.9 to 0.2±0.4; p=0.001, respectively). The differences after 4weeks between EBL and SH were not significant (p=0.168 and p=0.225). Pain requiring analgesics occurred in five patients (38.5%) after EBL, compared with six (46.2%) after SH (p=0.691). Minimal bleeding occurred in two patients (15.4%) after EBL but not with SH; urinary retention was observed in one patient after EBL compared with two after SH; and anal fissures were observed in one patient after EBL. During 1-year follow-up, increased frequency of stool occurred in one patient after EBL. Recurrence of symptoms was observed in three patients after EBL and in one after SH. For portal hypertensive patients with internal haemorrhoids and without coagulation disorders SH seems to be superior to EBL. However further studies are needed to evaluate EBL in different grades of cirrhosis. Copyright © 2011 Arab Journal of Gastroenterology. Published by Elsevier Ltd. All rights reserved.

Portal Hypertension Complications Are Frequently the First Presentation of NAFLD in Patients Undergoing Liver Transplantation Evaluation.

PubMed

Nagpal, Sajan Jiv Singh; Kabbany, Mohammad Nasser; Mohamad, Bashar; Lopez, Rocio; Zein, Nizar N; Alkhouri, Naim

2016-07-01

Nonalcoholic fatty liver disease (NAFLD) is likely to replace Hepatitis C as the leading cause of cirrhosis resulting in liver transplantation (LT) within a few years. Unfortunately, due to the lack of established guidelines for the screening of NAFLD in high-risk populations, many patients present with portal hypertension complications as their first manifestation of NAFLD require a LT evaluation. We aimed to investigate what proportion of patients who underwent LT for NAFLD-cirrhosis had knowledge of their liver disease prior to presenting with portal hypertension complications and to identify differences in clinical parameters between those with and without knowledge of preexisting NAFLD. Consecutive patients who underwent LT for NAFLD-cirrhosis at a tertiary referral center were included in the study. Demographic and clinical data at the time of the first LT evaluation visit were collected, and patient knowledge of previous NAFLD was documented. Ascites, variceal bleeding, hepatic encephalopathy, and thrombocytopenia leading to diagnosis of underlying cirrhosis were considered as the presenting symptoms of portal hypertension. A p < 0.05 was considered statistically significant. A total of 124 subjects who received LT for NAFLD-cirrhosis were included, 58 % (n = 72) were male. At the time of the first LT evaluation visit, 60 % had diabetes, the mean body mass index was 33.2 [28.6, 37.6] kg/m(2), and the mean Model for End-Stage Liver Disease (MELD) score was 14.0 [11.0, 19.0]. More importantly, 85/124 patients (68.5 %) had no knowledge of preexisting NAFLD prior to presentation with symptoms of portal hypertension. The presenting symptoms were new-onset ascites in 61 %, hepatic encephalopathy in 25 %, variceal bleeding in 18 %, thrombocytopenia in 9 %, and other in 9 % (non-exclusive). Patients with no prior knowledge of NAFLD were less likely to have a diagnosis of hypercholesterolemia (30 vs. 50 %, p = 0.035) and had a trend toward having higher MELD scores at the time of the first LT evaluation visit (15 vs. 13.5, p = 0.05) and presenting with encephalopathy (25 vs. 10 %, p = 0.06) compared to those with previous knowledge of NAFLD diagnosis. The majority of patients undergoing liver transplant evaluation for NAFLD-cirrhosis are not aware of underlying NAFLD until they present with features of portal hypertension. New guidelines should consider screening for NAFLD in certain high-risk groups as more effective treatments for NAFLD are emerging.

Portal hypertensive gastropathy: A systematic review of the pathophysiology, clinical presentation, natural history and therapy

PubMed Central

Gjeorgjievski, Mihajlo; Cappell, Mitchell S

2016-01-01

AIM: To describe the pathophysiology, clinical presentation, natural history, and therapy of portal hypertensive gastropathy (PHG) based on a systematic literature review. METHODS: Computerized search of the literature was performed via PubMed using the following medical subject headings or keywords: “portal” and “gastropathy”; or “portal” and “hypertensive”; or “congestive” and “gastropathy”; or “congestive” and “gastroenteropathy”. The following criteria were applied for study inclusion: Publication in peer-reviewed journals, and publication since 1980. Articles were independently evaluated by each author and selected for inclusion by consensus after discussion based on the following criteria: Well-designed, prospective trials; recent studies; large study populations; and study emphasis on PHG. RESULTS: PHG is diagnosed by characteristic endoscopic findings of small polygonal areas of variable erythema surrounded by a pale, reticular border in a mosaic pattern in the gastric fundus/body in a patient with cirrhotic or non-cirrhotic portal hypertension. Histologic findings include capillary and venule dilatation, congestion, and tortuosity, without vascular fibrin thrombi or inflammatory cells in gastric submucosa. PHG is differentiated from gastric antral vascular ectasia by a different endoscopic appearance. The etiology of PHG is inadequately understood. Portal hypertension is necessary but insufficient to develop PHG because many patients have portal hypertension without PHG. PHG increases in frequency with more severe portal hypertension, advanced liver disease, longer liver disease duration, presence of esophageal varices, and endoscopic variceal obliteration. PHG pathogenesis is related to a hyperdynamic circulation, induced by portal hypertension, characterized by increased intrahepatic resistance to flow, increased splanchnic flow, increased total gastric flow, and most likely decreased gastric mucosal flow. Gastric mucosa in PHG shows increased susceptibility to gastrotoxic chemicals and poor wound healing. Nitrous oxide, free radicals, tumor necrosis factor-alpha, and glucagon may contribute to PHG development. Acute and chronic gastrointestinal bleeding are the only clinical complications. Bleeding is typically mild-to-moderate. Endoscopic therapy is rarely useful because the bleeding is typically diffuse. Acute bleeding is primarily treated with octreotide, often with concomitant proton pump inhibitor therapy, or secondarily treated with vasopressin or terlipressin. Nonselective β-adrenergic receptor antagonists, particularly propranolol, are used to prevent bleeding after an acute episode or for chronic bleeding. Iron deficiency anemia from chronic bleeding may require iron replacement therapy. Transjugular-intrahepatic-portosystemic-shunt and liver transplantation are highly successful ultimate therapies because they reduce the underlying portal hypertension. CONCLUSION: PHG is important to recognize in patients with cirrhotic or non-cirrhotic portal hypertension because it can cause acute or chronic GI bleeding that often requires pharmacologic therapy. PMID:26855694

[Diagnosis and treatment of pulmonary hypertension].

PubMed

Román, J Sánchez; Hernández, F J García; Palma, M J Castillo; Medina, C Ocaña

2008-03-01

Pulmonary arterial hypertension is an idiopathic process or can be associated with another circumstances (connective tissue diseases, congenital heart disease, portal hypertension, exposure to appetite suppressants or another drugs or infectious agents such as HIV). Most patients are diagnosed as the result of an evaluation of symptoms, whereas others are diagnosed incidentally or during screening of asymptomatic populations at risk. We reviews systematic screening for the approach to diagnosing pulmonary arterial hypertension. A diagnostic algorithm can guide the evaluation but it can be modified according to specific clinical circumstances. The number of therapeutic options has increased.in the last years. We reviews the use of calcium-channel blockers, prostacyclin (and analogues), endothelin-receptor antagonists, and phosphodiesterase-5 inhibitors, and the use of combination therapy, and provides specific recommendations about the actual treatment.

Surgical implications of portal venous system malformation

PubMed Central

Marks, Charles

1974-01-01

The significance of congenital abnormalities in predisposing to portal hypertension and variceal haemorrhage needs to be remembered when these effects manifest in childhood, as portal venography will permit elucidation of the complicated congenital developmental abnormalities underlying the pathological condition and permit rational surgical amelioration. In the presence of portal hypertension the development of a collateral venous circulation may be represented by a hepatopetal or hepatofugal circulatory pattern and will closely parallel the developmental areas where portal and systemic venous circulations meet, being representative of the embryological anastomosis between the vitelloumbilical system and the posterior cardinal system of veins. ImagesFig. 3Fig. 5Fig. 6 PMID:4614690

Gender Linked Metric Analysis of Portal Vein: A Sonographic Appraisal.

PubMed

Singh, Shikha; Pankaj, Arvind Kumar; Rani, Anita; Sharma, Pradeep Kumar; Chauhan, Puja

2017-03-01

Portal hypertension is one of the most mystifying and disconcerting abdominal ailment. Ultrasonography (USG) is an effective diagnostic tool for its prompt management. Knowledge of normal calibre of portal vein in a local setting is essential as literature reports contrasting values in different regions. It helps in early diagnosis of portal hypertension even before it is clinically manifested thereby assisting clinicians and interventional radiologists in pertinent management. Study was aimed to evaluate the Portal Vein Diameter (PVD) and find its correlation with gender by using USG in North Indian population. A total of 300 healthy adults were included in the study. Portal vein diameter was measured in supine position and normal respiration by grey scale USG. The portal vein diameter was correlated with age and gender statistically using independent Student's t-test and ANOVA. Mean PVD of (9.49±1.03 mm) was observed in the present cross-sectional study. Male showed a significantly higher mean PVD (9.70±1.02 mm) as compared to females (9.10±0.94 mm). Scarcity of information concerning ultrasonographically measured standard portal vein diameter and inconstant values reported in literature necessitates the need for establishing local standard value. In the given subset of population the portal vein diameter was influenced by the gender. The information will be helpful in prompt diagnosis and management of portal hypertension.

Pulmonary hypertension and hepatic cirrhosis.

PubMed

Téllez Villajos, L; Martínez González, J; Moreira Vicente, V; Albillos Martínez, A

2015-01-01

Pulmonary hypertension is a relatively common phenomenon in patients with hepatic cirrhosis and can appear through various mechanisms. The most characteristic scenario that binds portal and pulmonary hypertension is portopulmonary syndrome. However, hyperdynamic circulation, TIPS placement and heart failure can raise the mean pulmonary artery pressure without increasing the resistances. These conditions are not candidates for treatment with pulmonary vasodilators and require a specific therapy. A correct assessment of hemodynamic, ultrasound and clinical variables enables the differential diagnosis of each situation that produces pulmonary hypertension in patients with cirrhosis. Copyright © 2015 Elsevier España, S.L.U. y Sociedad Española de Medicina Interna (SEMI). All rights reserved.

A de novo mutation in KCNN3 associated with autosomal dominant idiopathic non-cirrhotic portal hypertension.

PubMed

Koot, Bart G P; Alders, Marielle; Verheij, Joanne; Beuers, Ulrich; Cobben, Jan M

2016-04-01

Non-cirrhotic portal hypertension is characterized by histopathological abnormalities in the liver, mostly affecting small intrahepatic portal veins that cause portal hypertension in the absence of cirrhosis. It can be secondary to coagulation disorders or toxic agents. However, most cases are idiopathic non-cirrhotic portal hypertension (INCPH) and familial cases are rare. We report a family in which a father and three of his four children conceived with three different mothers are affected by INCPH. Whole exome and Sanger sequencing showed the father to have a de novo single nucleotide substitution c.1348G>C in the KCNN3 gene that was transmitted to all three of his affected offspring. The KCNN3 gene encodes small conductance calcium-activated potassium (SK) channel 3. SK channels are involved in the regulation of arterial and venous vascular tone by causing smooth muscle relaxation on activation. No data exist on the expression and function of SK channels in portal veins. The autosomal dominant inheritance in this unique pedigree and the single de novo mutation identified, strongly suggests that KCNN3 mutations have a pathogenetic role in INCPH. Copyright © 2015 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.

Management of hepatocellular carcinoma with portal vein tumor thrombosis: Review and update at 2016

PubMed Central

Chan, Stephen L; Chong, Charing C N; Chan, Anthony W H; Poon, Darren M C; Chok, Kenneth S H

2016-01-01

Portal vein tumor thrombosis (PVTT) is a common phenomenon in hepatocellular carcinoma (HCC). Compared to HCC without PVTT, HCC with PVTT is characterized by an aggressive disease course, worse hepatic function, a higher chance of complications related to portal hypertension and poorer tolerance to treatment. Conventionally, HCC with PVTT is grouped together with metastatic HCC during the planning of its management, and most patients are offered palliative treatment with sorafenib or other systemic agents. As a result, most data on the management of HCC with PVTT comes from subgroup analyses or retrospective series. In the past few years, there have been several updates on management of HCC with PVTT. First, it is evident that HCC with PVTT consists of heterogeneous subgroups with different prognoses. Different classifications have been proposed to stage the degree of portal vein invasion/thrombosis, suggesting that different treatment modalities may be individualized to patients with different risks. Second, more studies indicate that more aggressive treatment, including surgical resection or locoregional treatment, may benefit select HCC patients with PVTT. In this review, we aim to discuss the recent conceptual changes and summarize the data on the management of HCC with PVTT. PMID:27621575

Extrahepatic portal obstruction without hepatopetal pathway associated with congenital arterioportal fistula: a case report.

PubMed

Maeda, N; Horie, Y; Koda, M; Suou, T; Andachi, H; Nakamura, K; Kawasaki, H

1997-01-01

Extrahepatic portal obstruction is one of the causes of portal hypertension, in which well-developed hepatopetal pathways are commonly recognized. Herein an extremely rare case of extrahepatic portal obstruction without hepatopetal pathway, probably caused by arterioportal fistula, is reported. The patient was a normally matured 16-year-old girl admitted for further evaluation of jaundice, presenting with the clinical manifestations of the portal hypertension associated with hypersplenism and portosystemic venous shunt. Celiac angiography clearly demonstrated an intrahepatic arterial aneurysm fed by the right hepatic artery shunting to the superior mesenteric vein, and portography disclosed complete obstruction of the portal trunk with conspicuous hepatofugal pathway but no hepatopetal collateral veins. The exact mechanism of this phenomenon is not known and whether the extrahepatic portal obstruction was primary or secondary is still obscure. However, this is the first case report in the world literature describing extrahepatic portal obstruction with absence of hepatopetal pathway.

[Histology of liver lesions due to Schistosoma mekongi. About six cases with severe portal hypertension operated in Cambodia].

PubMed

Monchy, D; Dumurgier, C; Heng, T K; Hong, K; Khun, H; Hou, S V; Sok, K E; Huerre, M R

2006-12-01

Schistosomiasis mekongi was shown to be endemic, along the Mekong River, in northern Cambodia, affecting many patients with portal hypertension. Surgical procedures were proposed to some patients with digestive haemorrhage history to avoid fatal recurrence. The aim of our study was to evaluate the intensity of the liver fibrosis among these patients. During surgical treatment, liver biopsies were collected, fixed in Bouin or in formalin and processed at the Institut Pasteur of Cambodia. Sections were stained by H&E, Masson's trichrome, PAS, Ziehl-Neelsen's method and Congo Red. A total of six biopsies from patients aged 16-36 were analysed. There was complete disorganization of hepatic architecture with fibrous enlargement of portal tracts and some portal-portal bridging fibrosis, but there was no cirrhosis. In portal areas, there was blood vessel congestion and thrombosis with inflammation. Bile ducts were normal. In the parenchyma, congestion of sinusoidal capillaries was combined with focal mononuclear inflammatory infiltrate. There was no steatosis, no necrosis, no cholestasis, no iron accumulation and no amyloidosis. Numerous eggs of Schistosoma mekongi were observed in five cases, mostly in fibrous areas and more rarely in the parenchyma. Eggs were round or oval, measuring 60 x 40 microns with an acid-fast thin hyaline wall. Some eggs were surrounded by epithelioid and giant cell reaction. In conclusion, our findings illustrated a surprisingly high degree of fibrosis among young adults which contrasts with other schistosomiasis.

Prevention of Portal Hypertension: from Variceal Development to Clinical Decompensation

PubMed Central

Vorobioff, Julio D.; Groszmann, Roberto J

2015-01-01

Pharmacological treatment of portal hypertension (PH) has been exclusively devoted to gastro-esophageal varices related events at different frameworks including prophylactic, emergency or preventive therapy. The goals of treatment are to avoid the first bleeding episode, stop active bleeding and prevent bleeding recurrence, respectively. The objective of pre-primary prophylaxis (PPP) is to avoid variceal development and therefore, it necessarily deals with cirrhotic patients at earlier stages of the disease. At these earlier stages, nonselective beta blocker (NSBB) have been ineffective in preventing the development of varices and other complications of PH. Therefore, treatment should not rely on NSBB. It is possible, that at these earlier stages, etiological treatment of liver disease itself could prevent the progression of PH. This review will focus mainly on early treatment of PH, because if successful, it may translate into histological-hemodynamic improvements, avoiding not only variceal development but also other PH related complications, such as ascites and porto-systemic encephalopathy (PSE). Moreover, the advent of new therapies may allow not only the prevention of the complications of PH, but also the chance of a substantial degree of regression in the cirrhotic process with the possible prevention of hepatocellular carcinoma (HCC). PMID:24913395

[Invasion of the portal vein by a hydatid cyst. Review of the literature].

PubMed

Zubiaurre Lizarralde, Leire; Oyarzabal Pérez, Igor; Ruiz Montesinos, Inmaculada; Guisasola Gorrotxategi, Esther

2006-01-01

We have found only 3 publications in the literature that describe portal vein invasion by a hydatid cyst. This complication is very uncommon but should be kept in mind in the diagnosis of anaphylactic shock. Clinical presentation can vary from abdominal pain and fever to portal hypertension or anaphylactic reaction due to leaking of antigenic material from the cyst. Ultrasound and computed tomography scan can identify hydatid cysts and cavernomatosis, but magnetic resonance imaging shows the presence of multiple daughter vesicles replacing the lumen of the portal vein and a communication between the residual cyst and the portal vein. The treatment of choice is surgery, including removal of the cyst and local instillation of scolicide solution. In addition to surgery, administration of albendazole is recommended. Administration should begin 4 days before extirpation and should be continued for more than 4 weeks.

Transjugular Intrahepatic Portosystemic Shunt Placement During Pregnancy: A Case Series of Five Patients

DOE Office of Scientific and Technical Information (OSTI.GOV)

Ingraham, Christopher R., E-mail: cringra@uw.edu; Padia, Siddharth A., E-mail: spadia@uw.edu; Johnson, Guy E., E-mail: gej@uw.edu

Background and AimsComplications of portal hypertension, such as variceal hemorrhage and ascites, are associated with significant increases in both mortality and complications during pregnancy. Transjugular intrahepatic portosystemic shunt (TIPS) is a well-established procedure for treating portal hypertension, but the safety of TIPS during pregnancy is largely unknown. In this series, we review five patients who underwent TIPS placement while pregnant and describe their clinical outcomes.MethodsFive pregnant patients with cirrhosis and portal hypertension underwent elective TIPS for complications of portal hypertension (four for secondary prevention of variceal bleeding and one for refractory ascites). Outcomes measured were recurrent bleeding episodes or needmore » for further paracenteses during pregnancy, estimated radiation dose to the fetus and gestational age at delivery. All patients were followed after delivery to evaluate technical and clinical success of the procedure.ResultsAll five patients survived pregnancy and went on to deliver successfully. When TIPS was performed for secondary prevention of variceal bleeding (n = 4), no patients demonstrated variceal bleeding after TIPS placement. When TIPS was performed for refractory ascites (n = 1), no further paracenteses were required. All patients delivered successfully, albeit prematurely. Average radiation dose estimated to the fetus was 16.3 mGy.ConclusionsThis series suggests that TIPS can be performed in selective pregnant patients with portal hypertension, with little added risk to the mother or fetus.« less

Urotensin II modulates hepatic fibrosis and portal hemodynamic alterations in rats.

PubMed

Kemp, William; Kompa, Andrew; Phrommintikul, Arintaya; Herath, Chandana; Zhiyuan, Jia; Angus, Peter; McLean, Catriona; Roberts, Stuart; Krum, Henry

2009-10-01

The influence of circulating urotensin II (UII) on liver disease and portal hypertension is unknown. We aimed to evaluate whether UII executes a pathogenetic role in the development of hepatic fibrosis and portal hypertension. UII was administered by continuous infusion over 4 wk in 20 healthy rats divided into three treatment groups, controls (saline, n = 7), low dose (UII, 1 nmol x kg(-1) x h(-1), n = 8), and high dose (UII, 3 nmol x kg(-1) x h(-1), n = 5). Hemodynamic parameters and morphometric quantification of fibrosis were assessed, and profibrotic cytokines and fibrosis markers were assayed in hepatic tissue. UII induced a significant dose-dependent increase in portal venous pressure (5.8 +/- 0.4, 6.4 +/- 0.3, and 7.6 +/- 0.7, respectively, P = 0.03). High-dose UII infusion was associated with an increase in hepatic transcript for transforming growth factor-beta (P < 0.05) and platelet-derived growth factor-beta (P = 0.06). Liver tissue hydroxyproline was elevated in the high-dose group (P < 0.05). No systemic hemodynamic alterations were noted. We concluded that UII infusion elevates portal pressure and induces hepatic fibrosis in normal rats. This response may be mediated via induction of fibrogenic cytokines. These findings have pathophysiological implications in human liver disease where increased plasma UII levels have been observed.

Radiological score for hemorrhage in the patients with portal hypertension.

PubMed

Ge, Wei; Wang, Yi; Cao, Ya-Juan; Xie, Min; Ding, Yi-Tao; Zhang, Ming; Yu, De-Cai

2015-01-01

To analyze the risk factors from radiological indices for hemorrhage in the patients with portal hypertension and weight risk factors. We retrospectively analyzed all cases of portal hypertension with hepatitis B from June 2008 to June 2014 in Nanjing Drum Tower hospital. Patients with hepatocellular carcinoma, portal vein thrombosis, or portal hypertension with other causes, such as autoimmune hepatitis, pancreatitis, or hematological diseases were excluded. Ninety-eight patients were recruited and divided into hemorrhage and non-hemorrhage groups. There were no statistical differences in clinical indexes such as age, prothrombin time, serum albumin, serum creatinine, serum sodium, hemameba, and blood platelet count. However, the differences were statistically significant in total bilirubin, hemoglobin, and liver function with the p values of 0.023, 0.000, and 0.039 respectively. For radiological indices, hemorrhage was correlated with diameter of inferior mesenteric vein (P=0.0528), posterior gastric vein (P=0.0283), and esophageal varices scores (P=0.0221). Logistic procedure was used to construct the model with stepwise selection and finally inferior mesenteric vein, posterior gastric vein, esophageal varices, and short gastric vein were enrolled into the model. These veins were scored according to the diameters and the rates of hemorrhage were increased with the score. We then validated the model with 26 patents from July 2014 to December 2014. The AUC value was 0.8849 in ROC curves for this radiological model. A risk model was constructed including inferior mesenteric vein, esophageal varices, posterior gastric vein, and short gastric vein. This radiological scoring model may be a valuable indicator for hemorrhage of portal hypertension.

Hepatic Veins and Inferior Vena Cava Thrombosis in a Child Treated by Transjugular Intrahepatic Portosystemic Shunt

DOE Office of Scientific and Technical Information (OSTI.GOV)

Carnevale, Francisco Cesar, E-mail: fcarnevale@uol.com.br; Santos, Aline Cristine Barbosa; Tannuri, Uenis

We report the case of a 9-year-old boy with portal hypertension, due to Budd-Chiari syndrome, and retrohepatic inferior vena cava thrombosis, submitted to a transjugular intrahepatic portosystemic shunt (TIPS) by connecting the suprahepatic segment of the inferior vena cava directly to the portal vein. After 3 months, the withdrawal of anticoagulants promoted the thrombosis of the TIPS. At TIPS revision, thrombosis of the TIPS and the main portal vein and clots at the splenic and the superior mesenteric veins were found. Successful angiography treatment was performed by thrombolysis and balloon angioplasty of a severe stenosis at the distal edge ofmore » the stent.« less

Bleeding Duodenal: Varices Treatment by TIPS and Transcatheter Embolization

DOE Office of Scientific and Technical Information (OSTI.GOV)

Lopera, Jorge E.; Arthurs, Blain; Scheuerman, Christian

2008-03-15

We describe our clinical experience in 4 patients with portal hypertension who presented with bleeding mesenteric varices originating from the superior mesenteric vein with retrograde filling of collaterals draining into the inferior vena cava. The clinical presentation, imaging findings, and potential therapeutic management are discussed.

[Hepatic fibrosis and portal hypertension in chronic vitamin A poisoning].

PubMed

Verneau, A; Rosenbaum, J; Zafrani, E S; Roudot-Thoraval, F; Leclercq, M; Dhumeaux, D

1984-02-01

The authors report the case of a 36-year-old man who was hospitalized for portal hypertension. The patient had been receiving 200 millions units of vitamin A for ten years duration as treatment of psoriasis. There were no extrahepatic signs of hypervitaminosis A and the serum concentration of vitamin A was low. Microscopic examination of a liver specimen showed: a) spontaneous fluorescence due to vitamin A accumulation in sinusoidal cells; b) portal, periportal and perisinusoidal fibrosis; c) hyperplasia and hypertrophy of Ito cells. Hepatic vitamin A concentration was markedly increased. Hemodynamic study showed increased wedged hepatic venous pressure. Low serum concentration of retinol binding protein, which could be due to severe denutrition, explained the low serum vitamin A. This case report emphasizes that severe hepatic injury due to chronic hypervitaminosis A may be observed in the absence of extrahepatic signs of vitamin A intoxication and increase in serum vitamin A concentration. In such cases, histologic examination of a liver specimen and determination of hepatic vitamin A concentration are the only means of diagnosis.

Amelioration of cirrhotic portal hypertension by targeted cyclooxygenase-1 siRNA delivery to liver sinusoidal endothelium with polyethylenimine grafted hyaluronic acid.

PubMed

Lin, Liteng; Cai, Mingyue; Deng, Shaohui; Huang, Wensou; Huang, Jingjun; Huang, Xinghua; Huang, Mingsheng; Wang, Yong; Shuai, Xintao; Zhu, Kangshun

2017-10-01

Portal hypertension (PH), a leading cause of mortality in cirrhosis, lacks effective clinical therapeutic strategies. The increased thromboxane A 2 (TXA 2 ), derived primarily from the upregulation of cyclooxygenase-1 (COX-1) in cirrhotic liver sinusoidal endothelial cells (LSECs), is responsible for hepatic endothelial dysfunction and PH. Thus, blocking the COX-1 pathway in cirrhotic LSECs may benefit the treatment of PH. In this study, hyaluronate-graft-polyethylenimine (HA-PEI) was synthesized for the targeted delivery of COX-1 siRNA to LSECs. Compared to non-targeted PEI, HA-PEI mediated much more efficient siRNA delivery, which resulted in potent targeted gene silencing in LSECs. In vivo, HA-PEI notably increased the accumulation of siRNA along the sinusoidal lining of the liver, inhibited over-activation of the COX-1/TXA 2 pathway in LSECs, and successfully reduced portal pressure in cirrhotic mice. These results highlight the potential of HA-PEI complexed siRNA to serve as a LSECs-specific nanomedical system for effective gene therapy in PH. Copyright © 2017 Elsevier Inc. All rights reserved.

TIPS stent migration into the heart with 6-year follow-up.

PubMed

Fehervari, Imre; Szonyi, Laszlo; Fazakas, Janos; Gerlei, Zsuzsa; Lazar, Istvan

2011-01-01

The transjugular intrahepatic portosystemic shunt (TIPS) is widely used for the treatment of portal hypertension in adults, but no studies have defined the best approach to treat portal hypertension in pediatric patients. Pediatric use of TIPS is rare even in large centers of adult practice. The migration of stents has also been reported as a complication in adults. There is no standard way to treat this type of complication, and it is not always clear whether immediate removal or watchful waiting is safer for the patient. We report the case of an 11-year-old patient who underwent urgent TIPS implantation due to variceal bleeding, after unsuccessful sclerotherapy. During the procedure, due to the deep impact of the stent, a second, telescopic, stent was inserted. The portal pressure decreased, no further bleeding occurred, and the patient was listed for transplantation. Three weeks later a routine chest X-ray discovered the migration of the second stent into the right ventricle. No interventional radiological removal or open heart surgery was available for the transplant waiting list patient. The patient underwent uneventful combined liver-kidney transplantation. During the 6-year follow-up period the child had no signs of hemodynamic instability, and his somatic and mental development were appropriate. To our knowledge this case is the first publication on a heart-impacted TIPS stent in a child. The watchful waiting was justified by uneventful combined liver-kidney transplantation and long-term follow-up. This case also underlines the need for best practice guidelines in pediatric portal hypertension.

[To evaluate the role of OLT on splenomegaly of portal hypertension by the radiological changes of splenic morphology and collaterals].

PubMed

Liang, Ying-ying; Wang, Jin; Shan, Hong; Yan, Rong-hua; Hu, Bing; Jiang, Zai-bo; He, Bing-jun; Liu, Jing-jing; Ren, Ling-lan; Shao, Shuo

2012-11-20

To explore the effect of orthotopic liver transplantation (OLT) on portal hypertension by observing the radiological changes of splenic volume and collaterals before and after OLT. In our hospital 56 patients performing OLT due to cirrhosis, portal hypertension and splenomegaly were classified into five groups according to their following-up time: A (≤3 months), B (>3-6 months), C (>6-12 months), D (>12-24 months), and E (>24 months). Twenty health people were chose as control group (F). The splenic width, thickness, length, volume, diameter of portal and splenic vein and collaterals were measured and observed in every patient of six groups before and after OLT respectively. After OLT, the splenic volume decreased by 25.4%, 27.8%, 21.9%, 25.2%, 27.7% in five groups respectively, which was still larger than the normal group (P<0.05). Gastroesophageal varices in 31 cases (81.6%, 31/36) became normal after OLT. The opened umbilical vein disappeared and the retroperitoneal varices persisted in five cases after OLT. Splenomegaly and opened collaterals can be relieved by OLT effectively. The splenic volume didn't change obviously until it decreased by 25% in the three months after OLT. Gastroesophageal varices can be removed in most of patients after OLT. The splenomegaly could last paralled with the splenic vein and retroperitoneal varices after OLT. After OLT, correct disposal of splenic and collateral changes could improve the success rate and the long-term treatment effect of OLT.

Hepatic arterial vasodilation is independent of portal hypertension in early stages of cirrhosis.

PubMed

Moeller, Miriam; Thonig, Antje; Pohl, Sabine; Ripoll, Cristina; Zipprich, Alexander

2015-01-01

The compensatory increase in hepatic arterial flow with a decrease in portal venous flow is known as the hepatic arterial buffer response. In cirrhosis with elevated portal pressure, the vascular resistance of the hepatic artery is decreased. Whether this lower resistance of the hepatic artery is a consequence of portal hypertension or not remains unknown. The aim of the study was to investigate the hepatic arterial resistance and response to vasoconstriction in cirrhosis without portal hypertension (normal portal resistance). Cirrhosis was induced by CCl4-inhalation for 8 weeks (8W, normal portal resistance) and for 12-14 weeks (12W, elevated portal resistance). Bivascular liver perfusion was performed at 8W or 12W and dose response curves of methoxamine were obtained in the presence or absence of LNMMA (nitric oxide synthase blocker). Vascular resistances of the hepatic artery (HAR), portal vein (PVR) and sinusoids (SVR) were measured. Western Blot (WB) and Immunohistochemistry (IHC) were done to measure eNOS and HIF 1a expression. HAR in both groups of cirrhotic animals (8W and 12W) were lower compared to controls. Dose response curves to methoxamine revealed lower HAR in both cirrhotic models (8W and 12W) regardless the magnitude of portal resistance. LNMMA corrected the dose response curves in cirrhosis (8W and 12W) to control. WB and IHC show increased protein expression of eNOS and HIF1a in 8W and 12W. Hepatic arterial resistance is decreased in cirrhosis independent of portal resistance. Vasodilation of the hepatic artery in cirrhosis seems to be influenced by hypoxia rather than increase in portal resistance. Nitric oxide is the main vasodilator.

Hepatic Arterial Vasodilation Is Independent of Portal Hypertension in Early Stages of Cirrhosis

PubMed Central

Moeller, Miriam; Thonig, Antje; Pohl, Sabine; Ripoll, Cristina; Zipprich, Alexander

2015-01-01

Introduction The compensatory increase in hepatic arterial flow with a decrease in portal venous flow is known as the hepatic arterial buffer response. In cirrhosis with elevated portal pressure, the vascular resistance of the hepatic artery is decreased. Whether this lower resistance of the hepatic artery is a consequence of portal hypertension or not remains unknown. Study Aim The aim of the study was to investigate the hepatic arterial resistance and response to vasoconstriction in cirrhosis without portal hypertension (normal portal resistance). Methods Cirrhosis was induced by CCl4-inhalation for 8 weeks (8W, normal portal resistance) and for 12–14 weeks (12W, elevated portal resistance). Bivascular liver perfusion was performed at 8W or 12W and dose response curves of methoxamine were obtained in the presence or absence of LNMMA (nitric oxide synthase blocker). Vascular resistances of the hepatic artery (HAR), portal vein (PVR) and sinusoids (SVR) were measured. Western Blot (WB) and Immunohistochemistry (IHC) were done to measure eNOS and HIF 1a expression. Results HAR in both groups of cirrhotic animals (8W and 12W) were lower compared to controls. Dose response curves to methoxamine revealed lower HAR in both cirrhotic models (8W and 12W) regardless the magnitude of portal resistance. LNMMA corrected the dose response curves in cirrhosis (8W and 12W) to control. WB and IHC show increased protein expression of eNOS and HIF1a in 8W and 12W. Conclusion Hepatic arterial resistance is decreased in cirrhosis independent of portal resistance. Vasodilation of the hepatic artery in cirrhosis seems to be influenced by hypoxia rather than increase in portal resistance. Nitric oxide is the main vasodilator. PMID:25793622

The role of inducible nitric oxide synthase in vascular hyporeactivity of endotoxin-treated and portal hypertensive rats.

PubMed

Heinemann, A; Stauber, R E

1995-05-04

The involvement of the inducible nitric oxide (NO) synthase in the vascular hyporeactivity in portal vein-ligated rats was assessed in isolated perfused mesenteric arterial beds. Aminoguanidine, a selective inhibitor of the inducible NO synthase, restored the pressor responses to methoxamine in arteries of endotoxin-treated rats, but was ineffective in hyporeactive portal vein-ligated vessels. NG-Nitro-L-arginine methyl ester enhanced the responsiveness both in portal vein-ligated and sham-operated rats, without changing the difference between the two groups. These results not only indicate that the inducible NO synthase is not involved in the hyporeactivity to methoxamine in mesenteric arteries of portal hypertensive rats, but also suggest a role for factors other than NO.

Inter and intra-observer concordance for the diagnosis of portal hypertension gastropathy.

PubMed

Casas, Meritxell; Vergara, Mercedes; Brullet, Enric; Junquera, Félix; Martínez-Bauer, Eva; Miquel, Mireia; Sánchez-Delgado, Jordi; Dalmau, Blai; Campo, Rafael; Calvet, Xavier

2018-03-01

At present there is no fully accepted endoscopic classification for the assessment of the severity of portal hypertensive gastropathy (PHG). Few studies have evaluated inter and intra-observer concordance or the degree of concordance between different endoscopic classifications. To evaluate inter and intra-observer agreement for the presence of portal hypertensive gastropathy and enteropathy using different endoscopic classifications. Patients with liver cirrhosis were included into the study. Enteroscopy was performed under sedation. The location of lesions and their severity was recorded. Images were videotaped and subsequently evaluated independently by three different endoscopists, one of whom was the initial endoscopist. The agreement between observations was assessed using the kappa index. Seventy-four patients (mean age 63.2 years, 53 males and 21 females) were included. The agreement between the three endoscopists regarding the presence or absence of PHG using the Tanoue and McCormack classifications was very low (kappa scores = 0.16 and 0.27, respectively). The current classifications of portal hypertensive gastropathy have a very low degree of intra and inter-observer agreement for the diagnosis and assessment of gastropathy severity.

Noninvasive Subharmonic Pressure Estimation for Monitoring Breast Cancer Response to Neoadjuvant Therapy

DTIC Science & Technology

2013-01-01

F Forsberg. In vivo subharmonic pressure estimation of portal hypertension in canines. Proc IEEE US Symp, pp. 778-781, 2010. S. Paul, K. Sarkar...Symposium, San Diego, CA, USA. • In vivo subharmonic pressure estimation of portal hypertension in canines. November 21–23, 2010 63rd Annual Meeting...9. Less JR, et al., Interstitial hypertension in human breast and colorectal tumors. Cancer Res, 1992. 52(22): p. 6371-4. 28 10. Taghian AG, et

Novel Rat Model of Repetitive Portal Venous Embolization Mimicking Human Non-Cirrhotic Idiopathic Portal Hypertension.

PubMed

Klein, Sabine; Hinüber, Christian; Hittatiya, Kanishka; Schierwagen, Robert; Uschner, Frank Erhard; Strassburg, Christian P; Fischer, Hans-Peter; Spengler, Ulrich; Trebicka, Jonel

2016-01-01

Non-cirrhotic idiopathic portal hypertension (NCIPH) is characterized by splenomegaly, anemia and portal hypertension, while liver function is preserved. However, no animal models have been established yet. This study assessed a rat model of NCIPH and characterized the hemodynamics, and compared it to human NCIPH. Portal pressure (PP) was measured invasively and coloured microspheres were injected in the ileocecal vein in rats. This procedure was performed weekly for 3 weeks (weekly embolization). Rats without and with single embolization served as controls. After four weeks (one week after last embolization), hemodynamics were investigated, hepatic fibrosis and accumulation of myofibroblasts were analysed. General characteristics, laboratory analyses and liver histology were collected in patients with NCIPH. Weekly embolization induced a hyperdynamic circulation, with increased PP. The mesenteric flow and hepatic hydroxyproline content was significantly higher in weekly embolized compared to single embolized rats (mesenteric flow +54.1%, hydroxyproline +41.7%). Mesenteric blood flow and shunt volumes increased, whereas splanchnic vascular resistance was decreased in the weekly embolization group. Fibrotic markers αSMA and Desmin were upregulated in weekly embolized rats. This study establishes a model using repetitive embolization via portal veins, comparable with human NCIPH and may serve to test new therapies.

Arsenicosis, possibly from contaminated groundwater, associated with noncirrhotic intrahepatic portal hypertension.

PubMed

Goel, Ashish; Christudoss, Pamela; George, Renu; Ramakrishna, Banumathi; Amirtharaj, G Jayakumar; Keshava, Shyamkumar N; Ramachandran, Anup; Balasubramanian, K A; Mackie, Ian; Fleming, Jude J; Elias, Elwyn; Eapen, Chundamannil E

2016-05-01

Idiopathic noncirrhotic intrahepatic portal hypertension (NCIPH), a chronic microangiopathy of the liver caused by arsenicosis from use of contaminated groundwater, was reported from Asia. This study aimed to see, if in the twenty-first century, arsenicosis was present in NCIPH patients at our hospital and, if present, to look for groundwater contamination by arsenic in their residential locality. Twenty-seven liver biopsy proven NCIPH patients, 25 portal hypertensive controls with hepatitis B or C related cirrhosis and 25 healthy controls, matched for residential locality, were studied. Eighty-four percent to 96 % of study subjects belonged to middle or lower socioeconomic category. Arsenicosis was looked for by estimation of arsenic levels in finger/toe nails and by skin examination. Arsenic levels in nails and in ground water (in NCIPH patients with arsenicosis) was estimated by mass spectrometry. Nail arsenic levels were raised in five (10 %) portal hypertensive study subjects [two NCIPH patients (both had skin arsenicosis) and three portal hypertensive controls]. All of these five patients were residents of West Bengal or Bangladesh. Skin arsenicosis was noted in three NCIPH patients (11 %) compared to none of disease/healthy controls. Ground water from residential locality of one NCIPH patient with arsenicosis (from Bangladesh) showed extremely high level of arsenic (79.5 μg/L). Arsenicosis and microangiopathy of liver, possibly caused by environmental contamination continues in parts of Asia. Further studies are needed to understand the mechanisms of such 'poverty-linked thrombophilia'.

Antibody titers and response to vaccination against hepatitis A and B in pediatric patients with portal hypertension.

PubMed

Rosa, Mariana Nogueira de Paula; Hessel, Gabriel; Alves De Tommaso, Adriana María

2008-09-01

In Brazil, approximately 130 new cases of hepatitis A per 100,000 inhabitants occur annually and 15% of the population has been in contact with hepatitis B virus. Portal hypertension causes hypersplenism and reduces T cell production, which may lead to less effective response to hepatitis vaccination. The objective of the study was to evaluate the response to hepatitis A and B vaccination in patients with portal hypertension secondary to chronic liver disease or portal vein thrombosis. Twenty-three patients (2 to 18 years) with portal hypertension seen at the Pediatric Hepatology Service of Hospital das Clínicas, Universidade Estadual de Campinas, between 1994 and 2006 were studied. Hepatitis A and B serology was tested in all patients. Patients who had not been vaccinated before their visits received the vaccines during the study period. Patients who had been vaccinated before but had negative anti-HB antibodies received a booster dose, and their serology was repeated Blood counts were performed in each patient to assess for immunosuppression. Eighteen patients received hepatitis A vaccine and all became positive for anti-HAV antibodies. All patients had received hepatitis B vaccine and 17 (73.9%) were anti-HBs positive at the time of the study The other 6 received a booster dose and became anti-HBs positive afterward. The anti-HBs-positive and -negative patients did not differ significantly in age, leukocytes, lymphocytes, or duration between the vaccination and positive serology. In this study, hepatitis A vaccines elicited a 100% response and hepatitis B vaccine conferred protection and induced an anamnestic response in pediatric patients with portal hypertension.

Congenital portosystemic shunts: diagnosis and treatment.

PubMed

Franchi-Abella, Stéphanie; Gonzales, Emmanuel; Ackermann, Oanez; Branchereau, Sophie; Pariente, Danièle; Guérin, Florent

2018-05-05

Congenital portosystemic shunts (CPSS) are rare vascular malformations that create an abnormal connection between portal and systemic veins resulting in complete or partial diversion of the portal flow away from the liver to the systemic venous system. Different anatomic types exist and several classifications have been proposed. They can be associated with other malformations especially cardiac and heterotaxia. The main complications include hepatic encephalopathy, liver tumors, portopulmonary hypertension, and pulmonary arteriovenous shunts. Diagnosis relies on imaging, and prenatal diagnosis is possible. Spontaneous closure of the CPSS is possible in some anatomic forms during the first year of life. When the CPSS remains patent, radiologic or surgical closure of the CPSS may prevent, resolve, or stabilize complications. Interventional radiology plays a key role for both the preoperative evaluation with occlusion test to assess the exact anatomy and to measure portal pressure after occlusion of the CPSS. Endovascular closure is the first option for treatment when possible.

Hydrogen sulfide attenuates carbon tetrachloride-induced hepatotoxicity, liver cirrhosis and portal hypertension in rats.

PubMed

Tan, Gang; Pan, Shangha; Li, Jie; Dong, Xuesong; Kang, Kai; Zhao, Mingyan; Jiang, Xian; Kanwar, Jagat R; Qiao, Haiquan; Jiang, Hongchi; Sun, Xueying

2011-01-01

Hydrogen sulfide (H(2)S) displays vasodilative, anti-oxidative, anti-inflammatory and cytoprotective activities. Impaired production of H(2)S contributes to the increased intrahepatic resistance in cirrhotic livers. The study aimed to investigate the roles of H(2)S in carbon tetrachloride (CCl(4))-induced hepatotoxicity, cirrhosis and portal hypertension. Sodium hydrosulfide (NaHS), a donor of H(2)S, and DL-propargylglycine (PAG), an irreversible inhibitor of cystathionine γ-lyase (CSE), were applied to the rats to investigate the effects of H(2)S on CCl(4)-induced acute hepatotoxicity, cirrhosis and portal hypertension by measuring serum levels of H(2)S, hepatic H(2)S producing activity and CSE expression, liver function, activity of cytochrome P450 (CYP) 2E1, oxidative and inflammatory parameters, liver fibrosis and portal pressure. CCl(4) significantly reduced serum levels of H(2)S, hepatic H(2)S production and CSE expression. NaHS attenuated CCl(4)-induced acute hepatotoxicity by supplementing exogenous H(2)S, which displayed anti-oxidative activities and inhibited the CYP2E1 activity. NaHS protected liver function, attenuated liver fibrosis, inhibited inflammation, and reduced the portal pressure, evidenced by the alterations of serum alanine aminotransferase (ALT), aspartate aminotransferase (AST), hyaluronic acid (HA), albumin, tumor necrosis factor (TNF)-α, interleukin (IL)-1β, IL-6 and soluble intercellular adhesion molecule (ICAM)-1, liver histology, hepatic hydroxyproline content and α-smooth muscle actin (SMA) expression. PAG showed opposing effects to NaHS on most of the above parameters. Exogenous H(2)S attenuates CCl(4)-induced hepatotoxicity, liver cirrhosis and portal hypertension by its multiple functions including anti-oxidation, anti-inflammation, cytoprotection and anti-fibrosis, indicating that targeting H(2)S may present a promising approach, particularly for its prophylactic effects, against liver cirrhosis and portal hypertension.

Evaluation of surgical procedure selection based on intraoperative free portal pressure measurement in patients with portal hypertension.

PubMed

Sun, Yong-Wei; Chen, Wei; Luo, Meng; Hua, Rong; Liu, Wei; Huo, Yan-Miao; Wu, Zhi-Yong; Cao, Hui

2010-06-01

Various surgical procedures can be used to treat liver cirrhosis and portal hypertension. How to select the most appropriate procedure for patients with portal hypertension has become a difficult problem. This study aimed to analyze the relationship between the value of intraoperative free portal pressure (FPP) and postoperative complications, and to explore the significance of intraoperative FPP measurement with respect to surgical procedure selection. The clinical data of 187 patients with portal hypertension who received pericardial devascularization and proximal splenorenal shunt combined with devascularization (combined operation) at the Department of General Surgery in our hospital from January 2001 to September 2008 were retrospectively analyzed. Among the patients who received pericardial devascularization, those with a postoperative FPP >or=22 mmHg were included in a high-pressure group (n=68), and those with FPP <22 mmHg were in a low-pressure group (n=49). Seventy patients who received the combined operation comprised a combined group. The intraoperative FPP measurement changes at different times, and the incidence of postoperative complications in the three groups of patients were compared. The postoperative FPP value in the high-pressure group was 27.5+/-2.3 mmHg, which was significantly higher than that of the low-pressure (20.9+/-1.8 mmHg) or combined groups (21.7+/-2.5 mmHg). The rebleeding rate in the high-pressure group was significantly higher than that in the low-pressure and combined groups. The incidence rates of postoperative hepatic encephalopathy and liver failure were not statistically different among the three groups. The mortality due to rebleeding in the low-pressure and combined groups (0.84%) was significantly lower than that of the high-pressure group. The study demonstrates that FPP is a critical measurement for surgical procedure selection in patients with portal hypertension. A FPP value >or=22 mmHg after splenectomy and devascularization alone is an important indicator that an additional proximal splenorenal shunt needs to be performed.

Natural history of patients with non cirrhotic portal hypertension: Comparison with patients with compensated cirrhosis.

PubMed

Gioia, Stefania; Nardelli, Silvia; Pasquale, Chiara; Pentassuglio, Ilaria; Nicoletti, Valeria; Aprile, Francesca; Merli, Manuela; Riggio, Oliviero

2018-01-31

The knowledge of natural history of patients with portal hypertension (PH) not due to cirrhosis is less well known than that of cirrhotic patients. To describe the clinical presentation and the outcomes of 89 patients with non-cirrhotic PH (25 with non-cirrhotic portal hypertension, INCPH, and 64 with chronic portal vein thrombosis, PVT) in comparison with 77 patients with Child A cirrhosis. The patients were submitted to a standardized clinical, laboratory, ultrasonographic and endoscopic follow-up. Variceal progression, incidence of variceal bleeding, portal vein thrombosis, ascites and survival were recorded. At presentation, the prevalence of varices, variceal bleeding and ascites was similar in the 3 groups. During follow-up, the rate of progression to varices at risk of bleeding (p < 0.0001) and the incidence of first variceal bleeding (p = 0.02) were significantly higher in non-cirrhotic then in cirrhotic patients. A PVT developed in 32% of INCPH patients and in 18% of cirrhotics (p = 0.02). In the patients with non-cirrhotic PH variceal progression is more rapid and bleeding more frequent than in cirrhotics. Patients with INCPH are particularly prompt to develop PVT. This observational study suggests that the management of patients with non-cirrhotic PH should take into consideration the natural history of portal hypertension in these patients and cannot be simply derived by the observation of cirrhotic patients. Copyright © 2018 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

Partial spleen embolization reduces the risk of portal hypertension-induced upper gastrointestinal bleeding in patients not eligible for TIPS implantation.

PubMed

Buechter, Matthias; Kahraman, Alisan; Manka, Paul; Gerken, Guido; Dechêne, Alexander; Canbay, Ali; Wetter, Axel; Umutlu, Lale; Theysohn, Jens M

2017-01-01

Upper gastrointestinal bleeding (UGIB) is a severe and life-threatening complication among patients with portal hypertension (PH). Covered transjugular intrahepatic portosystemic shunt (TIPS) is the treatment of choice for patients with refractory or recurrent UGIB despite pharmacological and endoscopic therapy. In some patients, TIPS implantation is not possible due to co-morbidity or vascular disorders. Spleen embolization (SE) may be a promising alternative in this setting. We retrospectively analyzed 9 patients with PH-induced UGIB who underwent partial SE between 2012 and 2016. All patients met the following criteria: (i) upper gastrointestinal hemorrhage with primary or secondary failure of endoscopic interventions and (ii) TIPS implantation not possible. Each patient was followed for at least 6 months after embolization. Five patients (56%) suffered from cirrhotic PH, 4 patients (44%) from non-cirrhotic PH. UGIB occured in terms of refractory hemorrhage from gastric varices (3/9; 33%), hemorrhage from esophageal varices (3/9; 33%), and finally, hemorrhage from portal-hypertensive gastropathy (3/9; 33%). None of the patients treated with partial SE experienced re-bleeding episodes or required blood transfusions during a total follow-up time of 159 months, including both patients with cirrhotic- and non-cirrhotic PH. Partial SE, as a minimally invasive intervention with low procedure-associated complications, may be a valuable alternative for patients with recurrent PH-induced UGIB refractory to standard therapy.

Portosystemic shunts for extrahepatic portal hypertension in children.

PubMed

Tocornal, J; Cruz, F

1981-07-01

Twenty-three children with prehepatic portal hypertension and hemorrhage due to ruptured esophagogastric varices had portosystemic shunts. Their ages ranged from two years and seven months to 15 years. Eleven were less than eight years of age. Twenty patients had portal vein cavernomatosis and three patients had double portal veins. In 21 patients, a mesocaval type of shunt was done. A splenorenal shunt was performed in two. There was no surgical mortality. Two shunts occluded, both in rather young infants--two years and seven months and three years of age. In all the others, there was no further bleeding, and the shunts remained patent, as shown by abdominal angiograms. Neuropsychiatric disorders, probably due to hepatic encephalopathy, occurred in only one patient. On the basis of this favorable experience, we believe that an elective portosystemic shunt should, in general, be performed upon children with prehepatic portal hypertension after one major variceal hemorrhage. We favor a mesocaval type of shunt in these children because of the larger diameter of the vessels involved in the anastomosis and because it preserves the spleen, maintaining defense against subsequent infection.

Sinusoidal portal hypertension in hepatic amyloidosis.

PubMed Central

Bion, E; Brenard, R; Pariente, E A; Lebrec, D; Degott, C; Maitre, F; Benhamou, J P

1991-01-01

Hepatic venous catheterisation and transvenous liver biopsy were performed in five patients with hepatic amyloidosis. In three patients, hepatic venous pressures were normal and histological examination of the liver biopsy specimen showed discrete and sparse perisinusoidal amyloid deposits. In the other two, however, the gradient between wedged and free hepatic venous pressures was increased (12 and 16 mmHg; normal 1-4 mmHg) and amyloid deposits were abundant and diffuse in the Disse's space. This study shows that portal hypertension in patients with hepatic amyloidosis is of the sinusoidal type and is related to the reduction of vascular space of hepatic sinusoids by massive perisinusoidal amyloid deposits. Furthermore, portal hypertension is associated with a poor prognosis in patients with hepatic amyloidosis. Images Figure 1 Figure 2 PMID:1864548

Splenic artery aneurysm.

PubMed

Tcbc-Rj, Rui Antônio Ferreira; Ferreira, Myriam Christina Lopes; Ferreira, Daniel Antônio Lopes; Ferreira, André Gustavo Lopes; Ramos, Flávia Oliveira

2016-01-01

Splenic artery aneurysms - the most common visceral artery aneurysms - are found most often in multiparous women and in patients with portal hypertension. Indications for treatment of splenic artery aneurysm or pseudoaneurysm include specific symptoms, female gender and childbearing age, presence of portal hypertension, planned liver transplantation, a pseudoaneurysm of any size, and an aneurysm with a diameter of more than 2.5cm. Historically, the treatment of splenic artery aneurysm has been surgical ligation of the splenic artery, ligation of the aneurysm, or aneurysmectomy with or without splenectomy, depending on the aneurysm location. There are other percutaneous interventional techniques. The authors present a case of a splenic artery aneurysm in a 51-year-old woman, detected incidentally. RESUMO Aneurismas da artéria esplênica - os aneurismas arteriais viscerais mais comuns - são encontrados mais frequentemente em mulheres multíparas e em pacientes com hipertensão portal. As indicações para o seu tratamento incluem sintomas específicos, sexo feminino e idade fértil, presença de hipertensão portal, paciente em fila de transplante hepático, um pseudoaneurisma de qualquer tamanho, e um aneurisma com um diâmetro superior a 2,5cm. Historicamente, o tratamento do aneurisma da artéria esplênica tem sido a ligadura cirúrgica da artéria esplênica, a ligadura do aneurisma ou a aneurismectomia, com ou sem esplenectomia, dependendo do local do aneurisma. Existem outras técnicas intervencionistas percutâneas. Os autores apresentam o caso de um aneurisma de artéria esplênica em uma mulher de 51 anos de idade, diagnosticado incidentalmente.

Well-tolerated portal hypertension and favorable prognosis in adult patients with extrahepatic portal vein obstruction in Japan.

PubMed

Sekimoto, Tadashi; Maruyama, Hitoshi; Kobayashi, Kazufumi; Kiyono, Soichiro; Kondo, Takayuki; Shimada, Taro; Takahashi, Masanori; Yokosuka, Osamu

2016-05-01

To evaluate the clinical features and prognoses in adult patients with extrahepatic portal vein obstruction (EHO) from the aspect of portal hypertension during the last 20 years in Japan. There were 40 EHO patients (aged 21-77 years; mean ± standard deviation [SD], 54.6 ± 15.0). Clinical findings and prognoses were examined retrospectively during the median observation period of 71.6 months. Twenty-two patients (55%) showed positive signs of portal hypertension; 18 with esophageal varices (F0, one; F1, eight; F2, nine), two with gastric varices (F1, one; F2, one) and seven with mild ascites. Multivariate analysis showed that platelet count and spleen size were significant factors for the presence of gastroesophageal varices, with odds ratios of 0.989 (95% confidence interval [CI], 0.980-0.997; P = 0.011) for platelet count and 1.003 (95% CI, 1.001-1.005; P = 0.003) for spleen size. Ten of 20 patients with gastroesophageal varices received primary prophylaxis and only one patient (10%) showed variceal recurrence. The cumulative overall survival rate was 100% at 1 year, 94.2% at 3-7 years and 68.7% at 10 years. The cumulative survival rates did not differ between the patients with and without gastroesophageal varices, with and without ascites, and patterns of portal cavernoma at baseline. Forty-five percent of adult EHO patients in Japan were free from signs of portal hypertension, and platelet count and spleen size are predictive for identifying patients with gastroesophageal varices. EHO patients with gastroesophageal varices show favorable prognoses comparable to those without, if primary/secondary prophylaxis was performed appropriately. © 2015 The Japan Society of Hepatology.

Paradoxical arterial hypoxemia in a left-to-right shunt congenital heart disease.

PubMed

Martínez-Quintana, Efrén; Rodríguez-González, Fayna

2014-01-01

The hepatopulmonary syndrome is a rare complication of different types of chronic hepatic diseases with associated portal venous hypertension, resulting in pulmonary vascular dilatation, predominantly in the lower lung fields, and leading to ventilation-perfusion mismatch, arterial hypoxemia and a poor prognosis. We present the case of 42-year-old male patient with an anomalous drainage of the right superior pulmonary vein into the azygos vein and a portal vein cavernomatosis with associated portal venous hypertension who presented severe oxygen desaturation, during exercise, in the context of a hepatopulmonary syndrome.

Idiopathic portal hypertension and extrahepatic portal venous obstruction.

PubMed

Khanna, Rajeev; Sarin, Shiv Kumar

2018-02-01

Idiopathic portal hypertension (IPH) and extrahepatic portal venous obstruction (EHPVO) are non-cirrhotic vascular causes of portal hypertension (PHT). Variceal bleed and splenomegaly are the commonest presentations. The present review is intended to provide the existing literature on etiopathogenesis, clinical profile, diagnosis, natural history and management of IPH and EHPVO. IPH and EHPVO are both characterized by normal hepatic venous pressure gradient, moderate to massive splenomegaly with preserved liver synthetic functions. While the level of block in IPH is presinusoidal, in EHPVO it is at prehepatic level. Infections, autoimmunity, drugs, immunodeficiency and prothrombotic states are possible etiological agents in IPH. Contrastingly in EHPVO, prothrombotic disorders and local factors around the portal vein are the incriminating factors. Diagnosis is often clinical, supported by simple radiological tools. Natural history is defined by episodes of variceal bleed and symptoms related to enlarged spleen. Growth failure, portal biliopathy and minimal hepatic encephalopathy are additional concerns in EHPVO. Long-term survival is reasonably good with endoscopic surveillance; however, parenchymal extinction leading to decompensation is seen in a minority of patients in both the disorders. Surgical shunts revert the complications secondary to PHT. Meso-Rex shunt has become the standard surgery in children with EHPVO. This review gives a detailed summary of these two vascular conditions of liver-IPH and EHPVO. Further research is needed to understand the pathogenesis and natural history of these disorders.

Combined Rex-bypass shunt with pericardial devascularization alleviated prehepatic portal hypertension caused by cavernomatous transformation of portal vein.

PubMed

Wang, Ruo-Yi; Wang, Jun-Feng; Liu, Qian; Ma, Nan; Chen, Wei-Xiu; Li, Jin-Liang

2017-09-01

To evaluate the effects of combined Rex-bypass shunt and pericardial devascularization on prehepatic portal hypertension secondary to cavernomatous transformation of portal vein (CTPV). Forty-two patients aged from 3 years to 49 years (divided into 3 groups), 26 cases male and 16 female, with prehepatic vascular hepertention were treated with Rex-bypass shunt combined with pericardial devascularization. In each patient, preoperative assessment included ultrasound and computed tomographic angiography of the portal vein and blood analysis. The procedure was Rex-bypass shunt (with or without graft), and patients with moderate or severe gastroesophageal varices required additional paraesophagogastric devascularization. Splenectomy or subtotal splenectomy was performed if combined hypersplenism co-existed. All data were analyzed retrospectively. No intraoperative death occurred, blood routine analysis improved (P < 0.05), the blood flow velocity (P < 0.05) and diameter (P < 0.05) of the left portal vein (LPV) significantly increased, the esophageal and gastric varices significantly relieved in 34 patients (P < 0.05), and better effects of earlier operations were demonstrated than the delayed ones (P < 0.05). During the period of follow-up from 6 to 64 months, the overall patency rate was 85.7% and the younger the age the better of the effect. Rex-bypass shunt combined with pericardial devascularization is a safe and effective procedure for prehepatic portal hypertension caused by CTPV.

Gastroesphageal Variceal Hemorrhage Induced by Metastatic Liver Tumor of Lung Cancer

PubMed Central

Honda, Takayuki; Kobayashi, Hiroaki; Saiki, Masafumi; Sogami, Yusuke; Miyashita, Yoshihiro; Inase, Naohiko

2012-01-01

Gastroesophageal variceal hemorrhage is a lethal complication of portal hypertension. Liver cirrhosis is often the principal cause of the portal hypertensive state. Malignant tumors coexist with portal hypertension in some cases. Non-small-cell lung cancer (NSCLC) is likely to become metastatic. Liver is a frequent site of cancer metastasis, but diffuse hepatic sinusoidal metastasis is uncommon as a metastatic form of NSCLC. This report describes a patient with gastroesophageal variceal hemorrhage owing to a metastatic liver tumor of NSCLC. The patient, a male smoker with stage IV NSCLC, was free of any hepatitis viral infection and had no alcohol addiction. Liver dysfunction and liver disease had never been pointed out in his medical history. His tumor harbored an L858R epidermal growth factor receptor mutation. Gefitinib was initiated but had to be ceased because of interstitial lung disease. Sequential steroid therapy was effective and bevacizumab-containing chemotherapy was commenced. Both chemotherapy regimens produced favorable effects against the metastatic liver tumor, eliciting atrophic change regardless of the chemotherapy-free interval. One day the patient was admitted to our hospital because of black stool and hypotension. Upper gastrointestinal endoscopy revealed a beaded appearance of the gastroesophageal varix with bloody gastric contents. The portal hypertension might have been caused by changes in portal vein hemodynamics induced by the conformational changes underlying the favorable response of the liver tumor to molecular targeted chemotherapy and notable regression. PMID:23275780

Splanchnic Vein Thrombosis in the Myeloproliferative Neoplasms.

PubMed

Akpan, Imo J; Stein, Brady Lee

2018-06-01

To review the epidemiology, diagnostic challenges, pathogenesis, and treatment strategies for patients with myeloproliferative neoplasm-associated splanchnic vein thrombosis. The epidemiology of myeloproliferative neoplasm-associated splanchnic vein thrombosis (MPN-SVT) has been well characterized. While typical MPN-associated thrombosis affects older patients and involves the arterial circulation, MPN-SVT mostly impacts younger women. An association with JAK2 V617F is well-known; recent studies have demonstrated only a weak association with CALR mutations. JAK inhibition may represent a novel treatment strategy, complementing anticoagulation, and management of portal hypertension. While the epidemiology has been well characterized, more work is needed to identify novel contributors to disease pathogenesis, beyond the JAK2 V617F mutation itself, and endothelial compromise. Testing for MPN mutations in the setting of non-cirrhotic SVT is commonplace; JAK2 V617F is the most likely to be identified. Testing for CALR or MPL mutations requires clinical judgement, though not unreasonable. The mainstay of therapy is indefinite anticoagulation; the role of direct oral anticoagulants is unclear. JAK inhibition may play a role in addressing associated splenomegaly and portal hypertension.

Delayed gastric emptying of both the liquid and solid components of a meal in chronic liver disease.

PubMed

Galati, J S; Holdeman, K P; Dalrymple, G V; Harrison, K A; Quigley, E M

1994-05-01

To evaluate gastric emptying in patients with chronic liver disease and portal hypertension. We measured gastric emptying of both the liquid and solid components of a meal in 10 consecutive patients with chronic liver disease and portal hypertension, but free of ascites, and 14 age- and sex-matched healthy controls. In the patients with liver disease, relationships between emptying and liver function were examined. To measure gastric emptying, subjects consumed a test meal that consisted of scrambled eggs labeled with 99mTc-sulfur colloid and 4 oz of water labeled with 111In-diethylene triamine pentacetic acid (DTPA). Patients with liver disease and portal hypertension demonstrated delayed emptying of both the liquid (t1/2, min, mean +/- SE, patients vs. 69.4 +/- 19.4 vs. 31.4 +/- 1.8, p < 0.01) and solid (post-lag phase solid emptying: 141 +/- 32.9 vs. 69.8 +/- 4.6, p < 0.006) components of the meal. We could not identify any correlation between gastric emptying and tests of liver function. Gastric emptying is delayed in patients with liver disease and portal hypertension; this abnormal gastric motor function may contribute to the pathophysiology of foregut complaints in this patient population.

Systemic mastocytosis: A rare cause of non-cirrhotic portal hypertension.

PubMed

Martins, Cláudio; Teixeira, Cristina; Ribeiro, Suzane; Trabulo, Daniel; Cardoso, Cláudia; Mangualde, João; Freire, Ricardo; Gamito, Élia; Alves, Ana Luísa; Cremers, Isabelle; Alves, Cecília; Neves, Anabela; Oliveira, Ana Paula

2016-07-28

Mastocytosis is a clonal neoplastic disorder of the mast cells (MC) that can be limited to the skin (cutaneous mastocytosis) or involve one or more extracutaneous organs (systemic mastocytosis). The clinical manifestations of mastocytosis are heterogeneous ranging from indolent disease with a long-term survival to a highly aggressive neoplasm with survival of about 6 mo. Although liver involvement in aggressive systemic mastocytosis (ASM) is relatively common, the development of portal hypertension with or without cirrhosis is rare. We report a case of ASM without skin involvement in a 72-year-old caucasian male who presented with non-cirrhotic portal hypertension based on clinical, analytical, imagiological and endoscopic findings. Given the hematological picture, the correct diagnosis was established based on ancillary tests for MC using bone marrow aspirates and biopsy. Extensive involvement of the liver and gastrointestinal tract was histologically documented. The disease progressed rapidly and severe pancytopenia and recurrent upper gastrointestinal bleeding became the dominant problem. This case illustrates the challenge in establishing a diagnosis of ASM especially when the clinical picture is atypical and without skin involvement. Gastroenterologists should consider infiltrative disease, particularly systemic mastocytosis, as a differential diagnosis in a clinical case of portal hypertension of unknown etiology.

Living donor liver transplantation for congenital absence of the portal vein.

PubMed

Sanada, Y; Mizuta, K; Kawano, Y; Egami, S; Hayashida, M; Wakiya, T; Mori, M; Hishikawa, S; Morishima, K; Fujiwara, T; Sakuma, Y; Hyodo, M; Yasuda, Y; Kobayashi, E; Kawarasaki, H

2009-12-01

The congenital absence of the portal vein (CAPV) is a rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. Liver transplantation (OLT) may be indicated for patients with symptomatic CAPV refractory to medical treatment, especially due to hyperammonemia, portosystemic encephalopathy, hepatopulmonary syndrome, or hepatic tumors. Because portal hypertension and collateral circulation do not occur with CAPV, significant splanchnic congestion may occur when the portocaval shunt is totally clamped during portal vein (PV) reconstruction in OLT. This phenomenon results in severe bowel edema and hemodynamic instability, which negatively impact the patient's condition and postoperative recovery. We have successfully reconstructed the PV in living donor liver transplantation (LDLT) using a venous interposition graft, which was anastomosed end-to-side to the portocaval shunt by a partial side-clamp, using a patent round ligament of the liver, which was anastomosed end-to-end to the graft PV with preservation of both the portal and caval blood flows. Owing to the differences in anatomy among patients, at LDLT for CAPV liver transplant surgeons should seek to preserve both portal and caval blood flows.

Angiotensin II or epinephrine hemodynamic and metabolic responses in the liver of L-NAME induced hypertension and spontaneous hypertensive rats

PubMed Central

Kimura, Debora Conte; Nagaoka, Marcia Regina; Borges, Durval Rosa; Kouyoumdjian, Maria

2017-01-01

AIM To study hepatic vasoconstriction and glucose release induced by angiotensin (Ang)II or Epi in rats with pharmacological hypertension and spontaneously hypertensive rat (SHR). METHODS Isolated liver perfusion was performed following portal vein and vena cava cannulation; AngII or epinephrine (Epi) was injected in bolus and portal pressure monitored; glucose release was measured in perfusate aliquots. RESULTS The portal hypertensive response (PHR) and the glucose release induced by AngII of L-NAME were similar to normal rats (WIS). On the other hand, the PHR induced by Epi in L-NAME was higher whereas the glucose release was lower compared to WIS. Despite the similar glycogen content, glucose release induced by AngII was lower in SHR compared to Wistar-Kyoto rats although both PHR and glucose release induced by Epi in were similar. CONCLUSION AngII and Epi responses are altered in different ways in these hypertension models. Our results suggest that inhibition of NO production seems to be involved in the hepatic effects induced by Epi but not by AngII; the diminished glucose release induced by AngII in SHR is not related to glycogen content. PMID:28660012

Antioxidant role of heme oxygenase-1 in prehepatic portal hypertensive rats

PubMed Central

Gonzales, Soledad; Pérez, María Julia; Perazzo, Juan C; Tomaro, María Luján

2006-01-01

AIM: To study the effect of bilirubin on the oxidative liver status and the activity and expression of heme oxygenase-1 (HO-1) in rat liver injury induced by prehepatic portal hypertension. METHODS: Wistar male rats, weighing 200-250 g, were divided at random into two groups: one group with prehepatic portal hypertension (PH) induced by regulated prehepatic portal vein ligation (PPVL) and the other group corresponded to sham operated rats. Portal pressure, oxidative stress parameters, antioxidant enzymes, HO-1 activity and expression and hepatic sinusoidal vasodilatation were measured. RESULTS: In PPVL rats oxidative stress was evidenced by a marked increase in thiobarbituric acid reactive substances (TBARS) content and a decrease in reduced glutathione (GSH) levels. The activities of liver antioxidant enzymes, superoxide dismutase (SOD), catalase (CAT) and glutathione peroxidase (GSH-Px) were also diminished while activity and expression of HO-1 were enhanced. Administration of bilirubin (5 μmol/kg body weight) 24 h before the end of the experiment entirely prevented all these effects. Pretreatment with Sn-protoporphyrin IX (Sn-PPIX) (100 μg/kg body weight, i.p.), a potent inhibitor of HO, completely abolished the oxidative stress and provoked a slight decrease in liver GSH levels as well as an increase in lipid peroxidation. Besides, carbon monoxide, another heme catabolic product, induced a significant increase in sinusoidal hepatic areas in PPVL group. Pretreatment of PPVL rats with Sn-PPIX totally prevented this effect. CONCLUSION: These results suggest a beneficial role of HO-1 overexpression in prehepatic portal hypertensive rats. PMID:16830363

Functional asplenia and portal hypertension in a patient with primary splenic hemangiosarcoma

DOE Office of Scientific and Technical Information (OSTI.GOV)

Yuecel, A.E.D.; Durak, H.; Bernay, I.

1990-05-01

A 60-year-old man with primary splenic hemangiosarcoma (PSH) presented with weakness, weight loss, abdominal pain, and anemia. Physical examination revealed hepatomegaly, ascites, and firm, huge splenomegaly. Ultrasonography showed many nodular structures characterized by hypoechogenic and hyperechogenic areas. The patient also had portal hypertension, which was confirmed by physical findings and by measurement of portal vein pressure during operation. A liver-spleen scan using Tc-99m sulfur colloid and Tc-99m labeled heat denatured erythrocytes failed to demonstrate any splenic uptake, a reliable feature of functional asplenia. Although on a total body scan with Ga-67 citrate there was no splenic uptake, there was galliummore » uptake in the liver, where the presence of the metastatic lesion was histopathologically verified and confirmed by operation. There was also uptake in the middle zones of the lungs. Ga-67 citrate imaging appears to be helpful in the diagnosis of metastasis of PSH, and PSH can rarely cause portal hypertension.« less

Portal hypertension and liver lesions in chronically alcohol drinking rats prevented and reversed by stable gastric pentadecapeptide BPC 157 (PL-10, PLD-116), and propranolol, but not ranitidine.

PubMed

Prkacin, I; Separovic, J; Aralicia, G; Perovic, D; Gjurasin, M; Lovric-Bencic, M; Stancic-Rokotov, D; Staresinic, M; Anic, T; Mikus, D; Sikiric, P; Seiwerth, S; Mise, S; Rotkvic, I; Jagic, V; Rucman, R; Petek, M; Turkovic, B; Marovic, A; Sebecic, B; Boban-Blagaic, A; Kokic, N

2001-01-01

Liver lesions and portal hypertension in rats, following chronic alcohol administration, are a particular target for therapy. Portal hypertension (mm Hg) assessed directly into the portal vein, and liver lesions induced by 7.28 g/kg b.w. of alcohol given in drinking water for 3 months, were counteracted by a stable gastric pentadecapeptide BPC 157, GEPPPGKPADDAGLV, M.W. 1419, known to have a beneficial effect in a variety of models of gastrointestinal or liver lesions (10 microg or 10 ng/kg b.w. i.p. or i.g.) and propranolol (10 mg/kg b.w. i.g.), but not ranitidine (10 mg/kg b.w. i.g.) or saline (5 ml/kg b.w. i.p./i.g.; control). The medication (once daily) was throughout either the whole 3 months period (1) or the last month only (2) (last application 24 h before sacrifice). In the background of 7.28 g/kg/daily alcohol regimen similar lesions values were assessed in control rats following alcohol consumption, after 2 or 3 months of drinking. Both prophylactic and therapeutic effects were shown. After a period of 2 or 3 months, in all control saline [intragastrically (i.g.) or intraperitoneally (i.p.)] treated rats, the applied alcohol regimen consistently induced a significant rise of portal blood pressure values over values noted in healthy rats. In rats that received gastric pentadecapeptide BPC 157 or propranolol the otherwise raised portal pressure was reduced to the values noted in healthy rats. Besides, a raised surface area (microm(2)) and increased circumference (microm) of hepatocyte or hepatocyte nucleus [HE staining, measured using PC-compatible program ISSA (VAMS, Zagreb, Croatia)] and an advanced steatosis [scored (0-4), Oil Red staining] (on 100 randomly assigned hepatocytes per each liver), an increased liver weight, all together parallel a raised portal pressure in controls. Some of them were completely eliminated (not different from healthy rats, i.e. portal pressure, the circumference and area of hepatocytes, liver weight), while others were markedly attenuated (values less than in drinking controls, still higher than in healthy rats, i.e. circumference and area of hepatocytes nucleus). On the other hand, ranitidine application attenuated only steatosis development. In summary, despite continuous chronic alcohol drinking, pentadecapeptide BPC 157, and propranolol may prevent portal hypertension as well as reverse already established portal hypertension along with related liver disturbances.

[Experience in the treatment of some complications of portal hypertension in alcoholic liver cirrhosis].

PubMed

Savić, Zeljka; Vracarić, Vladimir; Hadnadjev, Ljiljana; Petrović, Zora; Damjanov, Dragomir

2011-11-01

Portal hypertension (PH) is hemodynamical abnormality associated with the most serious complications of alcoholic liver cirrhosis (ALC): ascites, varices and variceal bleeding. The aim of this study was to determine characteristics of portal hypertension, especially of upper gastrointestinal bleedings in patients with alcoholic liver cirrhosis (ALC). A total of 237 patients with ALC were observed in a 3-year period. A total of 161 patients (68%) were hospitalized because of PH elements: 86 (36.3%) had upper gastrointestinal bleeding, 75 (31.7%) were decompensated. Only 76 (32%) of the patients had icterus. General mortality was 85 (36%). According to the source of bleeding, 61 (71%) patients bled from varices, and 25 (29%) from other sources with existing varices but non-incriminated for bleeding in 16 (64%) of those patients. Active bleeding or stigmata of recent bleeding were found in 63 (73%) cases. Endoscopic treatment of variceal bleeding along with octreotide applied in 20 (32.78%) patients, just octreotide in 32 (52.46%), and octreotid plus balloon tamponade in 9 (14.75%). According to Child-Pugh classification, 25 (29%) of the bleeding patients were in class A, score 5.4; 43 (50%) in class B, score 7.8; and 18 (21%) in class C, score 10.9. Average hemoglobin level was 93 g/L, hematocrit 0.27, AST 71.52 U/L (normal to 37 U/L), ALT 37.74 U/L (normal to 40 U/L). Until this bleeding episode, 41 (47%) of the patients already bled. In the decompensated patients 3 (4%) were in Child Pugh class A, score 6; 42 (56%) in class B, score 8.3; and 30 (40%) in class C, score 10.6. Until this decompensation episode, 7 (9.3%) patients already bled. Patients with ALC need early detection of varices, primary and secondary profilaxis of variceal bleeding and adequate therapy of ascites. When bleeding occurs, patients need urgent upper endoscopy and intensive treatment.

[Results of the mesoportal bypass (Rex shunt) in the treatment of idiopathic extrahepatic portal vein obstruction in children].

PubMed

Domínguez Amillo, E; De la Torre Ramos, C; Andrés Moreno, A; Encinas Hernández, J L; Hernández Oliveros, F; López Santamaría, M

2017-01-25

Extrahepatic portal vein obstruction (EPVO) is the principal cause of portal hypertension in children. The objective of this study was to analyze the capacity of the surgical technique that creates a mesoportal shunt to treat changes caused by EPVO. Retrospective review of patients with idiopathic EPVO who underwent a mesoportal shunt and analysis of the changes in the number of leucocytes, platelets, prothrombin time and spleen size one year after the surgery. Twelve patients underwent surgery, out of which 10 had prior leukopenia, 11 thrombopenia, 9 longer prothrombin times and all had hypersplenism. One patient suffered a postoperative shunt thrombosis, was reoperated and underwent a change in the operative technique. The remaining patients (92%) have functioning shunts 4.3 ± 2.5 years after surgery, and none have suffered any episode of gastrointestinal bleeding. One year after surgery, there were significant changes in the number of platelets, prothrombin time and spleen size, with no significant changes in the number of leukocytes. However, the number of patients who went from a leukopenic to a normal state was significant, as happened with changes in prothrombin time. Mesoportal Rex shunt improves some of the disorders caused by portal hypertension in children suffering EPVO, with a high rate of surgical success. This technique should be of first choice in these patients.

Pulmonary hypertension in patients with congenital portosystemic venous shunt: a previously unrecognized association.

PubMed

Ohno, Takuro; Muneuchi, Jun; Ihara, Kenji; Yuge, Tetsuji; Kanaya, Yoshiaki; Yamaki, Shigeo; Hara, Toshiro

2008-04-01

Pulmonary arterial hypertension has been reported to be observed in association with acquired portal hypertension. However, the contribution of congenital anomalies occurring in the portal system to the development of pulmonary arterial hypertension remains to be elucidated. Nine patients with congenital portosystemic venous shunt were studied from January 1990 through September 2005. Patent ductus venosus was detected in 5 patients, including 3 patients with an absence of the portal vein. The presence of either a gastrorenal or splenorenal shunt was evident in another 4 patients. Six patients had a history of hypergalactosemia with normal enzyme activities, as seen during neonatal screening. Six (66.7%) of the 9 patients were identified to have clinically significant pulmonary arterial hypertension (mean pulmonary artery pressure: 34-79 mm Hg; pulmonary vascular resistances: 5.12-38.07 U). The median age at the onset of pulmonary arterial hypertension was 12 years and 3 months. Histologic studies of lung specimens, which were available in 4 of the 9 patients with congenital portosystemic venous shunt, showed small arterial microthrombotic lesions in 3 patients. This characteristic finding was recognized even in the congenital portosystemic venous shunt patients without PAH. This study demonstrated thromboembolic pulmonary arterial hypertension to be a crucial complication in congenital portosystemic venous shunt, and this pathologic state may be latently present in patients with pulmonary arterial hypertension of unknown etiology.

Protection of estrogen in portal hypertension gastropathy: an experimental model.

PubMed

Morgan-Martins, Maria Isabel; Jacques, Simone Iahnig; Hartmann, Renata Minuzzo; Marques, Camila Moraes; Marroni, Cláudio Augusto; Marroni, Norma Possa

2011-01-01

Portal hypertension is a complication secondary to cirrhosis that is characterized by increased blood flow and/or vascular resistance in the portal system, causing the appearance of a hyperdynamic collateral circulation. Partial portal vein ligation is an experimental model used in rats to study the pathophysiological mechanisms involved in pre-hepatic portal hypertension. Estrogen E2 is an antioxidant molecule with various physiological actions. To evaluate the antioxidant activity of endogenous estrogen in an experimental model of partial portal vein ligation by comparing intact with castrated rats. Twenty Wistar rats, weighing on average 250 g were used and divided into four groups: sham-operated (SO); intact (I) with partial portal vein ligation (I + PPVL), castrated (C) and castrated with partial ligation of the vein (C + PPVL). Day 1: castration or sham-operation; day 7, PPVL surgery; on day 15 post-PPVL, portal pressure in the mesenteric vein of rats was measured on polygraph Letica. Lipid peroxidation in the stomach was assessed using the technique of thiobarbituric acid reactive substances and activity of antioxidant enzymes superoxide dismutase, catalase and glutathione peroxidase. Statistical analysis was done with ANOVA - Student-Newman-Keuls (mean ± SE), and P<0.05 was considered as significant. Portal pressure was significantly increased in C + PPVL as compared to the other groups. There was no significant difference in the group of intact rats. TBARS showed significant damage in C and C + PPVL in relation to others. Antioxidant enzymes were significantly increased in the castrated rats with subsequent PPVL as compared to the other groups. We suggest that estrogen E2 plays a protective role in intact compared with castrated rats because it presents hydrophenolic radicals in its molecule, thus acting as an antioxidant in this experimental model.

Portal hypertension and liver cirrhosis in rats: effect of the β3-adrenoceptor agonist SR58611A

PubMed Central

Vasina, Valentina; Giannone, Ferdinando; Domenicali, Marco; Latorre, Rocco; Berzigotti, Annalisa; Caraceni, Paolo; Zoli, Marco; De Ponti, Fabrizio; Bernardi, Mauro

2012-01-01

BACKGROUND AND PURPOSE β3-Adrenoceptors participate in the regulation of vascular tone in physiological and pathological conditions. We aimed to assess the effect of pharmacological modulation of β3-adrenoceptors on portal pressure (PP) and systemic haemodynamics and their expression in the liver and mesenteric vessels of cirrhotic rats. EXPERIMENTAL APPROACH PP, central venous pressure (CVP) and systemic haemodynamics were invasively assessed in control and CCl4-treated cirrhotic rats before and during infusion of the selective β3-adrenoceptor agonist, SR58611A. Tissue samples were also collected from liver, heart, portal vein and mesenteric artery for immunohistochemistry and molecular biology analysis. The effect of SR58611A on isolated portal vein was assessed. KEY RESULTS At baseline, cirrhotic rats showed portal hypertension, reduced CVP and hyperdynamic circulation. SR58611A induced a significant, dose-dependent decrease in PP in cirrhotic rats, but not in controls. Although both groups manifested a dose-dependent reduction in mean arterial pressure, this effect was associated with decreased cardiac index (CI) and unchanged indicized peripheral vascular resistance (PVRI) in cirrhotic rats and increased CI and decreased PVRI in control animals. Pretreatment with the selective β3-adrenoceptor antagonist SR59230 prevented all SR58611A-induced changes in cirrhotic rats. SR58611A concentration-dependently relaxed portal vein in cirrhotic rats to a significantly greater extent than in healthy rats; pretreatment with SR59230A completely prevented SR58611A-induced cirrhotic portal vein relaxation. Finally, β3-adrenoceptors were identified in the liver, heart and portal vein of cirrhotic and control animals; their expression was increased in cirrhotic rats. CONCLUSIONS AND IMPLICATIONS β3-Adrenoceptors are altered in portal hypertension of experimental cirrhosis and may represent a novel therapeutic target. PMID:22708587

Altered blood-brain barrier permeability in rats with prehepatic portal hypertension turns to normal when portal pressure is lowered

PubMed Central

Eizayaga, Francisco; Scorticati, Camila; Prestifilippo, Juan P; Romay, Salvador; Fernandez, Maria A; Castro, José L; Lemberg, Abraham; Perazzo, Juan C

2006-01-01

AIM: To study the blood-brain barrier integrity in prehepatic portal hypertensive rats induced by partial portal vein ligation, at 14 and 40 d after ligation when portal pressure is spontaneously normalized. METHODS: Adult male Wistar rats were divided into four groups: Group I: Sham14d , sham operated; Group II: PH14d , portal vein stenosis; (both groups were used 14 days after surgery); Group III: Sham40d, Sham operated and Group IV: PH40d Portal vein stenosis (Groups II and IV used 40 d after surgery). Plasma ammonia, plasma and cerebrospinal fluid protein and liver enzymes concentrations were determined. Trypan and Evans blue dyes, systemically injected, were investigated in hippocampus to study blood-brain barrier integrity. Portal pressure was periodically recorded. RESULTS: Forty days after stricture, portal pressure was normalized, plasma ammonia was moderately high, and both dyes were absent in central nervous system parenchyma. All other parameters were reestablished. When portal pressure was normalized and ammonia level was lowered, but not normal, the altered integrity of blood-brain barrier becomes reestablished. CONCLUSION: The impairment of blood-brain barrier and subsequent normalization could be a mechanism involved in hepatic encephalopathy reversibility. Hemodynamic changes and ammonia could trigger blood-brain barrier alterations and its reestablishment. PMID:16552803

Establishment of a hepatic cirrhosis and portal hypertension model by hepatic arterial perfusion with 80% alcohol.

PubMed

Wang, Lei; He, Fu-Liang; Liu, Fu-Quan; Yue, Zhen-Dong; Zhao, Hong-Wei

2015-08-28

To determine the feasibility and safety of establishing a porcine hepatic cirrhosis and portal hypertension model by hepatic arterial perfusion with 80% alcohol. Twenty-one healthy Guizhou miniature pigs were randomly divided into three experimental groups and three control groups. The pigs in the three experimental groups were subjected to hepatic arterial perfusion with 7, 12 and 17 mL of 80% alcohol, respectively, while those in the three control groups underwent hepatic arterial perfusion with 7, 12 and 17 mL of saline, respectively. Hepatic arteriography and direct portal phlebography were performed on all animals before and after perfusion, and the portal venous pressure and diameter were measured before perfusion, immediately after perfusion, and at 2, 4 and 6 wk after perfusion. The following procedures were performed at different time points: routine blood sampling, blood biochemistry, blood coagulation and blood ammonia tests before surgery, and at 2, 4 and 6 wk after surgery; hepatic biopsy before surgery, within 6 h after surgery, and at 1, 2, 3, 4 and 5 wk after surgery; abdominal enhanced computed tomography examination before surgery and at 6 wk after surgery; autopsy and multi-point sampling of various liver lobes for histological examination at 6 wk after surgery. In experimental group 1, different degrees of hepatic fibrosis were observed, and one pig developed hepatic cirrhosis. In experimental group 2, there were cases of hepatic cirrhosis, different degrees of increased portal venous pressure, and intrahepatic portal venous bypass, but neither extrahepatic portal-systemic bypass circulation nor death occurred. In experimental group 3, two animals died and three animals developed hepatic cirrhosis, and different degrees of increased portal venous pressure and intrahepatic portal venous bypass were also observed, but there was no extrahepatic portal-systemic bypass circulation. It is feasible to establish an animal model of hepatic cirrhosis and portal hypertension by hepatic arterial perfusion with 80% alcohol, however, the safety of this model depends on a suitable perfusion dose.

Supersonic shear imaging for the diagnosis of liver fibrosis and portal hypertension in liver diseases: a meta-analysis.

PubMed

Deng, Han; Qi, Xingshun; Zhang, Tiansong; Qi, Xiaolong; Yoshida, Eric M; Guo, Xiaozhong

2018-01-01

The meta-analysis aimed to summarize the technical success rate of supersonic shear imaging (SSI) and to evaluate the diagnostic performance of liver and spleen stiffness measurement (LSM and SSM) with SSI for the detection of liver fibrosis, portal hypertension, and gastroesophageal varices in liver diseases. PubMed, EMBASE, and Cochrane Library databases were searched. Technical success rate of SSI was pooled. Area under curve (AUC), sensitivity, and specificity with corresponding 95% confidence interval (CI) were calculated. Included studies regarding the diagnostic performance of SSI for liver fibrosis, portal hypertension, and esophageal varices numbered 28, 4, and 4 respectively. The pooled technical success rates of LSM and SSM were 95.3% and 75.5%, respectively. The AUC, sensitivity, and specificity of LSM/SSM for different stages of liver fibrosis were 0.85-0.94, 0.7-0.89, and 0.82-0.92, respectively. The AUC, sensitivity, and specificity of LSM were 0.84 (95%CI = 0.8-0.86), 0.79 (95%CI = 0.7-0.85), and 0.82 (95%CI = 0.72-0.88) for clinically significant portal hypertension, 0.85 (95%CI = 0.82-0.88), 0.8 (95%CI = 0.68-0.88), and 0.8 (95%CI = 0.6-0.92) for any varices, and 0.86 (95%CI = 0.83-0.89), 0.86 (95%CI = 0.76-0.92), and 0.61 (95%CI = 0.35-0.83) for high-risk varices, respectively. LSM with SSI had a high diagnostic accuracy for liver fibrosis, but a moderate diagnostic accuracy for portal hypertension and esophageal varices.

Indocyanine green retention test (ICG-r15) as a noninvasive predictor of portal hypertension in patients with different severity of cirrhosis.

PubMed

Pind, Marie-Louise L; Bendtsen, Flemming; Kallemose, Thomas; Møller, Søren

2016-08-01

Portal hypertension is a severe consequence of chronic liver disease, responsible for the main clinical complications of cirrhosis. Measurement of the hepatic venous pressure gradient (HVPG) provides important clinical information, but the procedure is invasive and demands expert skills of the staff.In the present study, we aimed to investigate the relationship between the constant infusion indocyanine green (ICG) clearance, the calculated ICG retention test after 15 min (ICG-r15), and HVPG in patients with different severity of cirrhosis for validation of ICG-r15 as a noninvasive predictor of portal hypertension. A total of 325 patients were studied. During a hemodynamic investigation, the ICG clearance was determined using the constant infusion technique and ICG-r15 was calculated. Assessment of the diagnostic performance of ICG clearance and ICG-r15 as predictors of HVPG above 10 mmHg was performed by receiver operating characteristic curve analyses.The ICG clearance and ICG-r15 performed well in all three Child classes, with the most significant results among Child class A patients [area under the receiver operating characteristic (AUROC)=0.832] and less significant results in Child class B (AUROC=0.7448) and Child class C patients (AUROC=0.7392). Only six out of 102 patients in Child class C had HVPG of less than 12 mmHg. ICG-r15 can be used as an indirect assessment of significant portal hypertension in compensated cirrhotic patients. ICG-r15 may be suitable as a screening tool for the identification of patients for endoscopy and measurement of HVPG.Further validation of ICG-r15 together with other predictors of portal hypertension and its clinical use is encouraged.

Correlation of transient elastography with hepatic venous pressure gradient in patients with cirrhotic portal hypertension: A study of 326 patients from India.

PubMed

Kumar, Ashish; Khan, Noor Muhammad; Anikhindi, Shrihari Anil; Sharma, Praveen; Bansal, Naresh; Singla, Vikas; Arora, Anil

2017-01-28

To study the diagnostic accuracy of transient elastography (TE) for detecting clinically significant portal hypertension (CSPH) in Indian patients with cirrhotic portal hypertension. This retrospective study was conducted at the Institute of Liver, Gastroenterology, and Pancreatico-Biliary Sciences, Sir Ganga Ram Hospital, New Delhi, on consecutive patients with cirrhosis greater than 15 years of age who underwent hepatic venous pressure gradient (HVPG) and TE from July 2011 to May 2016. Correlation between HVPG and TE was analyzed using the Spearman's correlation test. Receiver operating characteristic (ROC) curves were prepared for determining the utility of TE in predicting various stages of portal hypertension. The best cut-off value of TE for the diagnosis of CSPH was obtained using the Youden index. The study included 326 patients [median age 52 (range 16-90) years; 81% males]. The most common etiology of cirrhosis was cryptogenic (45%) followed by alcohol (34%). The median HVPG was 16.0 (range 1.5 to 30.5) mmHg. Eighty-five percent of patients had CSPH. A significant positive correlation was noted between TE and HVPG (rho 0.361, P < 0.001). The area under ROC curve for TE in predicting CSPH was 0.740 (95%CI: 0.662-0.818) ( P < 0.01). A cut-off value of TE of 21.6 kPa best predicted CSPH with a positive predictive value (PPV) of 93%. TE has a fair positive correlation with HVPG; thus, TE can be used as a non-invasive modality to assess the degree of portal hypertension. A cut-off TE value of 21.6 kPa identifies CSPH with a PPV of 93%.

Correlation of transient elastography with hepatic venous pressure gradient in patients with cirrhotic portal hypertension: A study of 326 patients from India

PubMed Central

Kumar, Ashish; Khan, Noor Muhammad; Anikhindi, Shrihari Anil; Sharma, Praveen; Bansal, Naresh; Singla, Vikas; Arora, Anil

2017-01-01

AIM To study the diagnostic accuracy of transient elastography (TE) for detecting clinically significant portal hypertension (CSPH) in Indian patients with cirrhotic portal hypertension. METHODS This retrospective study was conducted at the Institute of Liver, Gastroenterology, and Pancreatico-Biliary Sciences, Sir Ganga Ram Hospital, New Delhi, on consecutive patients with cirrhosis greater than 15 years of age who underwent hepatic venous pressure gradient (HVPG) and TE from July 2011 to May 2016. Correlation between HVPG and TE was analyzed using the Spearman’s correlation test. Receiver operating characteristic (ROC) curves were prepared for determining the utility of TE in predicting various stages of portal hypertension. The best cut-off value of TE for the diagnosis of CSPH was obtained using the Youden index. RESULTS The study included 326 patients [median age 52 (range 16-90) years; 81% males]. The most common etiology of cirrhosis was cryptogenic (45%) followed by alcohol (34%). The median HVPG was 16.0 (range 1.5 to 30.5) mmHg. Eighty-five percent of patients had CSPH. A significant positive correlation was noted between TE and HVPG (rho 0.361, P < 0.001). The area under ROC curve for TE in predicting CSPH was 0.740 (95%CI: 0.662-0.818) (P < 0.01). A cut-off value of TE of 21.6 kPa best predicted CSPH with a positive predictive value (PPV) of 93%. CONCLUSION TE has a fair positive correlation with HVPG; thus, TE can be used as a non-invasive modality to assess the degree of portal hypertension. A cut-off TE value of 21.6 kPa identifies CSPH with a PPV of 93%. PMID:28216976

Portomesenteric Vein Thrombosis After Laparoscopic Sleeve Gastrectomy: Incidence, Analysis and Follow-Up in 1236 Consecutive Cases.

PubMed

Villagrán, Rodrigo; Smith, Gabriela; Rodriguez, Walter; Flores, Carlos; Cariaga, Mario; Araya, Sofía; Yañez, Marisol; Fuentes, Paulina; Linares, Jeannette; Zapata, Antonio

2016-11-01

Portomesenteric vein thrombosis (PMVT) is a rare but severe complication after laparoscopic bariatric surgery, with potentially serious consequences. We aimed to describe the incidence, clinical features, management, outcome, and midterm follow-up in patients with PMVT after laparoscopic sleeve gastrectomy (LSG). This retrospective and descriptive study included patients who underwent LSG between November 2009 and July 2015 and developed PMVT. The following data were analyzed: age, gender, body mass index (BMI), thrombosis risk factors, surgical technique, thromboembolic prophylaxis, primary surgery outcomes, clinical features, treatment, thrombophilia testing results, and follow-up findings, including imaging and endoscopic findings. A total of 1236 patients underwent LSG, and 5 (0.4 %) developed PMVT. The mean age was 34.4 years, and 3 patients were women. The mean BMI was 38.5 kg/m 2 . Two patients had received hormonal contraceptive treatment. Four patients had a history of smoking. All of the patients received anticoagulant treatment, and none required surgery. The mean hospitalization duration was 7.6 days. Two patients showed complete recanalization. One patient showed portal cavernomatosis on delayed images. Two patients had a positive thrombophilia test. No portal hypertension endoscopic findings were observed. PMVT is a rare complication, for which smoking was identified as a predominant risk factor. Early diagnosis and prompt anticoagulant therapy could lead to a dramatic decrease in the incidence of intestinal infarction, mortality, and extrahepatic portal hypertension in the near future. However, careful follow-up is necessary to evaluate the impact of PMVT on long-term patient outcomes.

Enhanced Y1-receptor-mediated vasoconstrictive action of neuropeptide Y (NPY) in superior mesenteric arteries in portal hypertension.

PubMed

Wiest, Reiner; Jurzik, Lars; Moleda, Lukas; Froh, Matthias; Schnabl, Bernd; von Hörsten, Stephan; Schölmerich, Juergen; Straub, Rainer H

2006-03-01

Vascular hyporeactivity to catecholamines contributes to arterial vasodilation and hemodynamic dysregulation in portal hypertension. Neuropeptide Y (NPY) is a sympathetic neurotransmitter facilitating adrenergic vasoconstriction via Y1-receptors on the vascular smooth muscle. Therefore, we investigated its role for vascular reactivity in the superior mesenteric artery (SMA) of portal vein ligated (PVL) and sham operated rats. In vitro perfused SMA vascular beds of rats were tested for the cumulative dose-response to NPY dependent on the presence and level of alpha1-adrenergic vascular tone (methoxamine MT: 0.3-10 microM). Moreover, the effect of NPY (50 nM) on vascular responsiveness to alpha1-adrenergic stimulation (MT: 0.3-300 microM) was evaluated. Y1-receptor function was tested by Y1-selective inhibition using BIBP-3226 (1 microM). NPY dose-dependently and endothelium-independently enhanced MT-pre-constriction in SMA. This potentiation was increasingly effective with increasing adrenergic pre-stimulation and being more pronounced in PVL rats as compared to sham rats at high MT concentrations. NPY enhanced vascular contractility only in PVL rats correcting the adrenergic vascular hyporeactivity. Y1-receptor inhibition completely abolished NPY-evoked vasoconstrictive effects. NPY endothelium-independently potentiates adrenergic vasoconstriction via Y1-receptors being more pronounced in portal hypertension improving mesenteric vascular contractility and thereby correcting the splanchnic vascular hyporeactivity. This makes NPY a superior vasoconstrictor counterbalancing arterial vasodilation in portal hypertension.

Percutaneous Angioplasty of Portal Vein Stenosis that Complicates Liver Transplantation: The Mid-Term Therapeutic Results

PubMed Central

Park, Kwang Bo; Do, Young Soo; Shin, Sung Wook; Cho, Sung Gi; Choo, In-Wook

2005-01-01

Objective We wanted to valuate the mid-term therapeutic results of percutaneous transhepatic balloon angioplasty for portal vein stenosis after liver transplantation. Materials and Methods From May 1996 to Feb 2005, 420 patients underwent liver transplantation. Percutaneous transhepatic angioplasty of the portal vein was attempted in six patients. The patients presented with the clinical signs and symptoms of portal venous hypertension or they were identified by surveillance doppler ultrasonography. The preangioplasty and postangioplasty pressure gradients were recorded. The therapeutic results were monitored by the follow up of the clinical symptoms, the laboratory values, CT and ultrasonography. Results The overall technical success rate was 100%. The clinical success rate was 83% (5/6). A total of eight sessions of balloon angioplasty were performed in six patients. The mean pressure gradient decreased from 14.5 mmHg to 2.8 mmHg before and after treatment, respectively. The follow up periods ranged from three months to 64 months (mean period; 32 months). Portal venous patency was maintained in all six patients until the final follow up. Combined hepatic venous stenosis was seen in one patient who was treated with stent placement. One patient showed puncture tract bleeding, and this patient was treated with coil embolization of the right portal puncture tract via the left transhepatic portal venous approach. Conclusion Percutaneous transhepatic balloon angioplasty is an effective treatment for the portal vein stenosis that occurs after liver transplantation, and our results showed good mid-term patency with using this technique. PMID:16145291

Parallel transjugular intrahepatic portosystemic shunt for controlling portal hypertension complications in cirrhotic patients.

PubMed

He, Fu-Liang; Wang, Lei; Yue, Zhen-Dong; Zhao, Hong-Wei; Liu, Fu-Quan

2014-09-07

To evaluate the feasibility of a second parallel transjugular intrahepatic portosystemic shunt (TIPS) to reduce portal venous pressure and control complications of portal hypertension. From January 2011 to December 2012, 10 cirrhotic patients were treated for complications of portal hypertension. The demographic data, operative data, postoperative recovery data, hemodynamic data, and complications were analyzed. Ten patients underwent a primary and parallel TIPS. Technical success rate was 100% with no technical complications. The mean duration of the first operation was 89.20 ± 29.46 min and the second operation was 57.0 ± 12.99 min. The mean portal system pressure decreased from 54.80 ± 4.16 mmHg to 39.0 ± 3.20 mmHg after the primary TIPS and from 44.40 ± 3.95 mmHg to 26.10 ± 4.07 mmHg after the parallel TIPS creation. The mean portosystemic pressure gradient decreased from 43.80 ± 6.18 mmHg to 31.90 ± 2.85 mmHg after the primary TIPS and from 35.60 ± 2.72 mmHg to 15.30 ± 3.27 mmHg after the parallel TIPS creation. Clinical improvement was seen in all patients after the parallel TIPS creation. One patient suffered from transient grade I hepatic encephalopathy (HE) after the primary TIPS and four patients experienced transient grade I-II after the parallel TIPS procedure. Mean hospital stay after the first and second operations were 15.0 ± 3.71 d and 16.90 ± 5.11 d (P = 0.014), respectively. After a mean 14.0 ± 3.13 mo follow-up, ascites and bleeding were well controlled and no stenosis of the stents was found. Parallel TIPS is an effective approach for controlling portal hypertension complications.

Origins of Portal Hypertension in Nonalcoholic Fatty Liver Disease.

PubMed

Baffy, Gyorgy

2018-03-01

Nonalcoholic fatty liver disease (NAFLD) advanced to cirrhosis is often complicated by clinically significant portal hypertension, which is primarily caused by increased intrahepatic vascular resistance. Liver fibrosis has been identified as a critical determinant of this process. However, there is evidence that portal venous pressure may begin to rise in the earliest stages of NAFLD when fibrosis is far less advanced or absent. The biological and clinical significance of these early changes in sinusoidal homeostasis remains unclear. Experimental and human observations indicate that sinusoidal space restriction due to hepatocellular lipid accumulation and ballooning may impair sinusoidal flow and generate shear stress, increasingly disrupting sinusoidal microcirculation. Sinusoidal endothelial cells, hepatic stellate cells, and Kupffer cells are key partners of hepatocytes affected by NAFLD in promoting endothelial dysfunction through enhanced contractility, capillarization, adhesion and entrapment of blood cells, extracellular matrix deposition, and neovascularization. These biomechanical and rheological changes are aggravated by a dysfunctional gut-liver axis and splanchnic vasoregulation, culminating in fibrosis and clinically significant portal hypertension. We may speculate that increased portal venous pressure is an essential element of the pathogenesis across the entire spectrum of NAFLD. Improved methods of noninvasive portal venous pressure monitoring will hopefully give new insights into the pathobiology of NAFLD and help efforts to identify patients at increased risk for adverse outcomes. In addition, novel drug candidates targeting reversible components of aberrant sinusoidal circulation may prevent progression in NAFLD.

Jejunal varices diagnosed by capsule endoscopy in patients with post-liver transplant portal hypertension.

PubMed

Bass, Lee M; Kim, Stanley; Superina, Riccardo; Mohammad, Saeed

2017-02-01

Portal hypertension secondary to portal vein obstruction following liver transplant occurs in 5%-10% of children. Jejunal varices are uncommon in this group. We present a case series of children with significant GI blood loss, negative upper endoscopy, and jejunal varices detected by CE. Case series of patients who had CE for chronic GI blood loss following liver transplantation. Three patients who had their initial transplants at a median age of 7 months were identified at our institution presenting at a median age of 8 years (range 7-16 years) with a median Hgb of 2.8 g/dL (range 1.8-6.8 g/dL). Upper endoscopy was negative for significant esophageal varices, gastric varices, and bleeding portal gastropathy in all three children. All three patients had significant jejunal varices noted on CE in mid-jejunum. Jejunal varices were described as large prominent bluish vessels underneath visualized mucosa, one with evidence of recent bleeding. The results led to venoplasty of the portal vein in two patients and a decompressive shunt in one patient with resolution of GI bleed and anemia. CE is useful to diagnose intestinal varices in children with portal hypertension and GI bleeding following liver transplant. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Post-traumatic arteriovenous fistula of the hepatic pedicle.

PubMed

Ibn Majdoub Hassani, K; Mohsine, R; Belkouchi, A; Bensaid, Y

2010-10-01

Hepatico-portal fistula (HPF) is a rare condition, most often of post-traumatic or iatrogenic origin and occasionally secondary to a ruptured aneurysm of the hepatic artery into the portal vein. HPF in extrahepatic locations often results in portal hypertension (PHT). While Doppler ultrasound, CT angiography, and magnetic resonance angiography are usually demonstrative, arteriography remains indispensable to clarify the exact anatomical configuration. In the treatment of these arteriovenous (AV) fistulas, open surgical approaches have increasingly given way to radiological embolization techniques, especially in intrahepatic locations, but surgery remains indicated for AV fistulas of the hepatic pedicle where maintenance of hepatic arterial flow is a priority of treatment. We report a patient who had an AV fistula of the hepatic pedicle with resultant PHT presenting 5 years after open abdominal trauma. Treatment was surgical; the immediate and long-term postoperative course was uneventful with regression of PHT. Through analysis of this case and a review of the literature, we discuss the clinical, paraclinical, therapeutic, and prognostic features of this lesion. Copyright © 2010. Published by Elsevier Masson SAS.

Portal Vein Thrombosis

PubMed Central

Chawla, Yogesh K.; Bodh, Vijay

2015-01-01

Portal vein thrombosis is an important cause of portal hypertension. PVT occurs in association with cirrhosis or as a result of malignant invasion by hepatocellular carcinoma or even in the absence of associated liver disease. With the current research into its genesis, majority now have an underlying prothrombotic state detectable. Endothelial activation and stagnant portal blood flow also contribute to formation of the thrombus. Acute non-cirrhotic PVT, chronic PVT (EHPVO), and portal vein thrombosis in cirrhosis are the three main variants of portal vein thrombosis with varying etiological factors and variability in presentation and management. Procoagulant state should be actively investigated. Anticoagulation is the mainstay of therapy for acute non-cirrhotic PVT, with supporting evidence for its use in cirrhotic population as well. Chronic PVT (EHPVO) on the other hand requires the management of portal hypertension as such and with role for anticoagulation in the setting of underlying prothrombotic state, however data is awaited in those with no underlying prothrombotic states. TIPS and liver transplant may be feasible even in the setting of PVT however proper selection of candidates and type of surgery is warranted. Thrombolysis and thrombectomy have some role. TARE is a new modality for management of HCC with portal vein invasion. PMID:25941431

Ultrasound-based elastography for the diagnosis of portal hypertension in cirrhotics

PubMed Central

Şirli, Roxana; Sporea, Ioan; Popescu, Alina; Dănilă, Mirela

2015-01-01

Progressive fibrosis is encountered in almost all chronic liver diseases. Its clinical signs are diagnostic in advanced cirrhosis, but compensated liver cirrhosis is harder to diagnose. Liver biopsy is still considered the reference method for staging the severity of fibrosis, but due to its drawbacks (inter and intra-observer variability, sampling errors, unequal distribution of fibrosis in the liver, and risk of complications and even death), non-invasive methods were developed to assess fibrosis (serologic and elastographic). Elastographic methods can be ultrasound-based or magnetic resonance imaging-based. All ultrasound-based elastographic methods are valuable for the early diagnosis of cirrhosis, especially transient elastography (TE) and acoustic radiation force impulse (ARFI) elastography, which have similar sensitivities and specificities, although ARFI has better feasibility. TE is a promising method for predicting portal hypertension in cirrhotic patients, but it cannot replace upper digestive endoscopy. The diagnostic accuracy of using ARFI in the liver to predict portal hypertension in cirrhotic patients is debatable, with controversial results in published studies. The accuracy of ARFI elastography may be significantly increased if spleen stiffness is assessed, either alone or in combination with liver stiffness and other parameters. Two-dimensional shear-wave elastography, the ElastPQ technique and strain elastography all need to be evaluated as predictors of portal hypertension. PMID:26556985

A Combined Training Program for Veterans with Amnestic Mild Cognitive Impairment

DTIC Science & Technology

2015-10-01

Acute illness or unstable chronic illness, e.g., history of severe liver disease (cirrhosis,  esophageal varices, ascites,  portal   hypertension ...unstable chronic illness, e.g., history of severe liver disease (cirrhosis,  esophageal varices, ascites,  portal   hypertension , hepatic encephalopathy...impairment such as hypertension , obesity, and type II diabetes mellitus [1; 2; 3; 4]. Hence, it may be that by improving cardiovascular health and reducing

Role of cyclooxygenase isoforms in prostacyclin biosynthesis and murine prehepatic portal hypertension

PubMed Central

Skill, N. J.; Theodorakis, N. G.; Wang, Y. N.; Wu, J. M.; Redmond, E. M.; Sitzmann, J. V.

2008-01-01

Portal hypertension (PHT) is a common complication of liver cirrhosis and significantly increases morbidity and mortality. Abrogation of PHT using NSAIDs has demonstrated that prostacyclin (PGI2), a direct downstream metabolic product of cyclooxygenase (COX) activity, is an important mediator in the development of experimental and clinical PHT. However, the role of COX isoforms in PGI2 biosynthesis and PHT is not fully understood. Prehepatic PHT was induced by portal vein ligation (PVL) in wild-type, COX-1−/−, and COX-2−/− mice treated with and without COX-2 (NS398) or COX-1 (SC560) inhibitors. Hemodynamic measurements and PGI2 biosynthesis were determined 1–7 days after PVL or sham surgery. Gene deletion or pharmacological inhibition of COX-1 or COX-2 attenuated but did not ameliorate PGI2 biosynthesis after PVL or prevent PHT. In contrast, treatment of COX-1−/− mice with NS398 or COX-2−/− mice with SC560 restricted PGI2 biosynthesis and abrogated the development of PHT following PVL. In conclusion, either COX-1 or COX-2 can mediate elevated PGI2 biosynthesis and the development of experimental prehepatic PHT. Consequently, PGI2 rather then COX-selective drugs are indicated in the treatment of PHT. Identification of additional target sites downstream of COX may benefit the >27,000 patients whom die annually from cirrhosis in the United States alone. PMID:18772366

Splenic Arterial Embolization in the Treatment of Severe Portal Hypertension Due to Pancreatic Diseases: The Primary Experience in 14 Patients

DOE Office of Scientific and Technical Information (OSTI.GOV)

Wang, Qi, E-mail: wqtjmu@gmail.com; Xiong, Bin, E-mail: herrxiong@126.com; Zheng, ChuanSheng, E-mail: hqzcsxh@sina.com

ObjectiveThis retrospective study reports our experience using splenic arterial particle embolization and coil embolization for the treatment of sinistral portal hypertension (SPH) in patients with and without gastric bleeding.MethodsFrom August 2009 to May 2012, 14 patients with SPH due to pancreatic disease were diagnosed and treated with splenic arterial embolization. Two different embolization strategies were applied; either combined distal splenic bed particle embolization and proximal splenic artery coil embolization in the same procedure for acute hemorrhage (1-step) or interval staged distal embolization and proximal embolization in the stable patient (2-step). The patients were clinically followed.ResultsIn 14 patients, splenic arterial embolizationmore » was successful. The one-step method was performed in three patients suffering from massive gastric bleeding, and the bleeding was relieved after embolization. The two-step method was used in 11 patients, who had chronic gastric variceal bleeding or gastric varices only. The gastric varices disappeared in the enhanced CT scan and the patients had no gastric bleeding during follow-up.ConclusionsSplenic arterial embolization, particularly the two-step method, proved feasible and effective for the treatment of SPH patients with gastric varices or gastric variceal bleeding.« less

[Octreotide treatment for postoperative chylous ascites in an adult].

PubMed

Senosiain Lalastra, Carla; Martínez González, Javier; Mesonero Gismero, Francisco; Moreira Vicente, Víctor

2012-10-01

Chylous ascites is infequent after abdominal surgery. We describe the case of a 43-year-old man with portal cavernomatosis who underwent surgery to insert a splenorenal shunt, which was not placed due to the absence of signs of portal hypertension. On postoperative day 20, the patient developed abdominal distension and mild dyspnea and was diagnosed with chylous ascites, which was related to the surgery. The patient was initially treated with diet and diuretics, with no clinical response, and consequently octreotide therapy was started. Four days later, the ascites was almost resolved and an ultrasound scan at 4 months showed its complete disappearance. This article demonstrates the effectiveness of octreotide in the treatment of postsurgical chylous ascites. Copyright © 2012 Elsevier España, S.L. and AEEH y AEG. All rights reserved.

Management of portal hypertension in children

PubMed Central

Gugig, Roberto; Rosenthal, Philip

2012-01-01

Portal hypertension can be caused by a wide variety of conditions. It frequently presents with bleeding from esophageal varices. The approach to acute variceal hemorrhage in children is a stepwise progression from least invasive to most invasive. Management of acute variceal bleeding is straightforward. But data on primary prophylaxis and long term management prevention of recurrent variceal bleeding in children is scarce, therefore prospective multicenter trials are needed to establish best practices. PMID:22468080

Liver disease in patients with cystic fibrosis.

PubMed

Kamal, Natasha; Surana, Pallavi; Koh, Christopher

2018-05-01

The aim of this study was to provide an overview of the current understanding of the pathophysiology, diagnosis and management of cystic fibrosis-liver disease (CFLD). CFLD has a variety of manifestations. Previously, it was thought that patients progressed from mild cholestatic disease to cirrhosis to decompensated cirrhosis with portal hypertension. Newer evidence suggests that some patients may develop cirrhosis while others develop noncirrhotic portal hypertension. Advances in our understanding of the pathophysiology of disease necessitate modifications to the current diagnostic criteria. Both fibroscan and noninvasive biomarkers can be used to identify patients with cirrhosis and portal hypertension. Ursodeoxycholic acid remains the mainstay of therapy despite a paucity of rigorous studies supporting its use. Novel therapeutic agents such as CF transmembrane conductance regulator (CFTR) modulators and potentiators are encouraging but need to be evaluated specifically in CFLD. A better understanding of the pathophysiology of disease is critical to developing more disease-specific diagnostics and therapeutics.

Complications of cirrhosis. A 50 years flashback.

PubMed

Møller, Søren; Bendtsen, Flemming

2015-06-01

In patients with cirrhosis and portal hypertension, it is largely the frequency and severity of complications relating to the diseased liver, degree of portal hypertension and hemodynamic derangement that determine the prognosis. It can be considered as a multiple organ failure that apart from the liver involves the heart, lungs, kidneys, the immune systems and other organ systems. Progressive fibrosis of the liver and subsequent metabolic impairment leads to a systemic and splanchnic arteriolar vasodilatation. With the progression of the disease development of portal hypertension leads to formation of esophageal varices and ascites. The circulation becomes hyperdynamic with cardiac, pulmonary as well as renal consequences for dysfunction and reduced survival. Infections and a changed cardiac function known as cirrhotic cardiomyopathy may be involved in further aggravation of other complications such as renal failure precipitating the hepatorenal syndrome. Patients with end-stage liver disease and related complications as for example the hepatopulmonary syndrome can only radically be treated by liver transplantation.

The Design of Clinical Trials in Portal Hypertension.

PubMed

Abraldes, Juan G; Garcia-Tsao, Guadalupe

2017-02-01

Portal hypertension (PH) is the main consequence of cirrhosis and is responsible for the majority of its complications. Gastroesophageal varices and variceal hemorrhage are direct consequences of PH; therefore, most clinical trials in PH have been directed toward treating or preventing variceal hemorrhage. However, varices and variceal hemorrhage are not isolated events; they must be considered in the context of the presence (or absence) of other complications of cirrhosis/PH. Cirrhosis progresses across different stages, each with a different prognosis and pathophysiology and hence different therapeutic targets. In this review, the authors discuss the design of proof-of-concept studies for the assessment of new drugs for the treatment of PH, that are mainly based on the drug's ability to reduce the hepatic venous pressure gradient. They further discuss the design of studies with clinical endpoints in the context of each stage of cirrhosis, specifically targets of therapy, optimal therapies in the control arm, risk stratification, and primary outcome. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Diagnosis, follow-up and treatment of cystic fibrosis-related liver disease.

PubMed

van de Peppel, Ivo P; Bertolini, Anna; Jonker, Johan W; Bodewes, Frank A J A; Verkade, Henkjan J

2017-11-01

To provide an insight and overview of the challenges in the diagnosis, follow-up and treatment of cystic fibrosis-related liver disease (CFLD). The variable pathophysiology of CFLD complicates its diagnosis and treatment. A 'gold standard' for CFLD diagnosis is lacking. Over the past years, new techniques to diagnose features of CFLD, such as transient elastography, have been investigated. Although most of these tests confirm cystic fibrosis-related liver involvement (CFLI), they are, however, not suitable to distinguish various phenotypical presentations or predict progression to clinically relevant cirrhosis or portal hypertension. A combined initiative from the European and the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition has been started, aimed to obtain consensus on CFLD criteria and definitions. Currently, only ursodeoxycholic acid is used in CFLD treatment, although it has not been convincingly demonstrated to change the natural course of the disease. Drugs that directly target cystic fibrosis transmembrane conductance regulator protein dysfunction show promising results; however, more long-term follow-up and validation studies are needed. CFLD is an umbrella term referring to a wide variety of liver manifestations with variable clinical needs and consequences. CFLD with portal hypertension is the most severe form of CFLD due to its significant implications on morbidity and mortality. The clinical relevance of other CFLI is uncertain. Consensus on CFLD definitions is essential to validate new diagnostic tools and therapeutic outcome measures.

Intrahepatic portosystemic venous shunt: diagnosis by color Doppler imaging.

PubMed

Kudo, M; Tomita, S; Tochio, H; Minowa, K; Todo, A

1993-05-01

Intrahepatic portosystemic venous shunt is a rare clinical entity; only 33 such cases have been reported. It may be congenital, or secondary to portal hypertension. Five patients with this disorder are presented, each of whom was diagnosed by color Doppler imaging, including waveform spectral analysis. One patient with clinical evidence of cirrhosis and portal hypertension had episodes of hepatic encephalopathy and elevated blood levels of ammonia. This patient had a large tubular shunt between the posterior branch of the portal vein and the inferior vena cava. Shunts of this type are considered to be collateral pathways which develop in the hepatic parenchyma as a result of portal hypertension. The other four patients had no evidence of liver disease, and all four evidenced an aneurysmal portohepatic venous shunt within the liver parenchyma. Shunts of this type are considered congenital. The diagnosis of intrahepatic portosystemic venous shunts was established by color Doppler imaging, which demonstrated a direct communication of color flow signals between the portal vein and hepatic vein, in addition to the characterization of the Doppler spectrum at each sampling point from a continuous waveform signal (portal vein) to a turbulent signal (aneurysmal cavity), and finally, to a biphasic waveform signal (hepatic vein). As demonstrated by the five patients, color Doppler imaging is useful in the diagnosis of an intrahepatic portosystemic hepatic venous shunt, and the measurement of shunt ratio may be useful in the follow-up and determining the therapeutic option.

Hepatic encephalopathy in patients with non-cirrhotic portal hypertension: Description, prevalence and risk factors.

PubMed

Nicoletti, Valeria; Gioia, Stefania; Lucatelli, Pierleone; Nardelli, Silvia; Pasquale, Chiara; Nogas Sobrinho, Stefano; Pentassuglio, Ilaria; Greco, Francesca; De Santis, Adriano; Merli, Manuela; Riggio, Oliviero

2016-09-01

Hepatic encephalopathy (HE) is a common complication of cirrhosis but it is less studied in patients with non-cirrhotic portal hypertension (NCPH). To describe the prevalence of cognitive impairment (overt and covert HE) in NCPH patients and to identify the risk factors for its development. 51 patients with NCPH, 35 with chronic portal vein thrombosis (PVT) and 16 with idiopathic non-cirrhotic portal hypertension (INCPH), were evaluated for the presence of previous or present overt HE (OHE). The psychometric hepatic encephalopathy score and the SCAN battery were used to detect the presence of covert HE (CHE). 34 compensated cirrhotic patients were used as control. In NCPH patients, abdominal scans were performed to detect the presence of shunts. None of the patients experienced OHE at evaluation while 5.7% of PVT and 12.5% of INCPH patients referred at least one documented episode of previous OHE, similarly to patients with cirrhosis (14.7%). Even if lower than in patients with cirrhosis (64.7%), a considerable proportion of patients with chronic PVT (34.3%) and INCPH (25%) had CHE (p=0.008). The presence of a large portal-systemic shunt was the only factor significantly correlated to cognitive impairment in NCPH patients. HE is a tangible complication of NCPH and is mainly related to the presence of portal-systemic shunts. Copyright © 2016 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

A traumatic hepatic artery pseudoaneurysm and arterioportal fistula, with severe diarrhea as the first symptom: A case report and review of the literature.

PubMed

Han, Ping; Yang, Lan; Huang, Xiao-Wei; Zhu, Xiu-Qin; Chen, Li; Wang, Nan; Li, Zhen; Tian, De-An; Qin, Hua

2018-02-01

Hepaticarterioportal fistula (APF) is a rare cause of portal hypertension and gastrointestinal hemorrhage, and presents as abnormal communication between the hepatic artery and portal vein. Percutaneous liver biopsy is a main iatrogenic cause of AFP. However, non-iatrogenic, abdominal, trauma-related APF is rarely reported. A 29-year-old man presenting with severe, watery diarrhea was transferred to our hospital, and his condition was suspected to be acute gastroenteritis because he ate expired food and suffered a penetrating abdominal stab wound 5 years ago. After admission, the patient suffered from hematemesis, hematochezia, ascites, anuria, and kidney failure, and he developed shock. The patient was finally diagnosed as a traumatic hepatic artery pseudoaneurysm and APF. This patient was treated with emergency transarterial embolization using coils. Since a secondary feeding vessel was exposed after the first embolization of the main feeding artery, a less-selective embolization was performed again. During the 6-month follow-up period, the patient remained asymptomatic. A penetrating abdominal stab wound is a rare cause of hepatic APFs, and occasionally leads to portal hypertension, the medical history and physical examination are the most important cornerstones of clinical diagnosis. Interventional radiology is essential for the diagnosis and treatment of an APF.

A traumatic hepatic artery pseudoaneurysm and arterioportal fistula, with severe diarrhea as the first symptom

PubMed Central

Han, Ping; Yang, Lan; Huang, Xiao-Wei; Zhu, Xiu-Qin; Chen, Li; Wang, Nan; Li, Zhen; Tian, De-An; Qin, Hua

2018-01-01

Abstract Rationale: Hepaticarterioportal fistula (APF) is a rare cause of portal hypertension and gastrointestinal hemorrhage, and presents as abnormal communication between the hepatic artery and portal vein. Percutaneous liver biopsy is a main iatrogenic cause of AFP. However, non-iatrogenic, abdominal, trauma-related APF is rarely reported. Patient concerns: A 29-year-old man presenting with severe, watery diarrhea was transferred to our hospital, and his condition was suspected to be acute gastroenteritis because he ate expired food and suffered a penetrating abdominal stab wound 5 years ago. After admission, the patient suffered from hematemesis, hematochezia, ascites, anuria, and kidney failure, and he developed shock. Diagnoses: The patient was finally diagnosed as a traumatic hepatic artery pseudoaneurysm and APF. Interventions: This patient was treated with emergency transarterial embolization using coils. Since a secondary feeding vessel was exposed after the first embolization of the main feeding artery, a less-selective embolization was performed again. Outcomes: During the 6-month follow-up period, the patient remained asymptomatic. Lessons: A penetrating abdominal stab wound is a rare cause of hepatic APFs, and occasionally leads to portal hypertension, the medical history and physical examination are the most important cornerstones of clinical diagnosis. Interventional radiology is essential for the diagnosis and treatment of an APF. PMID:29443759

Hydatid liver cyst causing portal vein thrombosis and cavernous transformation: a case report and literature review

PubMed Central

Kirmizi, Serdar; Kayaalp, Cuneyt; Yilmaz, Sezai

2016-01-01

A 33-year-old male with abdominal distention after meals was admitted to the hospital. He had a history of surgery for hydatid liver cyst. The cyst was located at the liver hilum and there were portal venous thrombosis and cavernous transformation. It had been treated with partial cystectomy, omentoplasty and albendazole. Two years later at the admission to our center, his laboratory tests were in normal ranges. Abdominal imaging methods revealed splenomegaly, portal vein thrombosis, cavernous transformation and the previously operated hydatid liver cyst. Upper gastrointestinal endoscopy demonstrated esophageal and gastric fundal varices. Due to his young age and low risk for surgery, the patient was planned for surgical treatment of both pathologies at the same time. At laparotomy, hydatid liver cyst was obliterated with omentum and there was no sign of active viable hydatid disease. A meso-caval shunt with an 8 mm in-diameter graft was created. In the postoperative period, his symptoms and endoscopic varices were regressed. There were four similar cases reported in the literature. This one was the youngest and the only one treated by a surgical shunt. Hydatid liver cysts that located around the hilum can lead to portal vein thrombosis and cavernous thrombosis. Treatment should consist of both hydatid liver cyst and portal hypertension. To the best of our knowledge, this was the first case of surgically treated portal vein thrombosis that was originated from a hydatid liver cyst. PMID:27895860

Percutaneous Microwave Ablation in the Spleen for Treatment of Hypersplenism in Cirrhosis Patients.

PubMed

Jiang, XiangWu; Gao, Fei; Ma, Yan; Feng, ShuFen; Liu, XueLian; Zhou, HongKe

2016-01-01

The aim of this study was to estimate the feasibility and therapeutic effectiveness of percutaneous microwave ablation in the treatment of hypersplenism in cirrhosis. Forty-one cirrhosis patients with hypersplenism were treated with ultrasonography-guided percutaneous microwave ablation between February 2007 and August 2011. Peripheral blood cell counts, portal vein diameter, splenic vein diameter, and blood flow of splenic vein were evaluated before and after the operation, and complications of the treatment were also investigated. All patients were followed up for 24 months. The levels of platelets and white blood cells were increased, while the splenic vein diameter narrowed gradually after the therapy and 24 months later. Moreover, patients received percutaneous microwave ablation had much lower splenic venous flow velocity. The portal vein diameter did not change significantly 6 months after the treatment, although it narrowed gradually within 3 months after the treatment. Furthermore, no complications such as uncontrollable bleeding, splenic abscess, spleen rupture, and damage in surrounding organ happened after the therapy. Graded percutaneous microwave ablation, as a minimally invasive therapy, could damage the spleen, increase the levels of platelets and white blood cells, and reduce portal hypertension effectively without serious complications. Percutaneous microwave ablation is an effective, safe, and feasible method for cirrhosis patients with hypersplenism.

Hemodynamic effects of renin-angiotensin-aldosterone inhibitor and β-blocker combination therapy vs. β-blocker monotherapy for portal hypertension in cirrhosis: A meta-analysis

PubMed Central

Wang, Jianrong; Lu, Wenxia; Li, Jingjing; Zhang, Rong; Zhou, Yuqing; Yin, Qin; Zheng, Yuanyuan; Wang, Fan; Xia, Yujing; Chen, Kan; Li, Sainan; Liu, Tong; Lu, Jie; Zhou, Yingqun; Guo, Chuan-Yong

2017-01-01

β-blockers are commonly used for the treatment of acute variceal bleeding in cirrhosis. Renin-angiotensin-aldosterone antagonists (angiotensin I-converting enzyme inhibitors, angiotensin receptor blockers and aldosterone antagonists) are potential therapies for portal hypertension. Several studies have compared the renin-angiotensin-aldosterone system (RAAS) inhibitor and β-blocker combination therapy vs. β-blocker monotherapy, with inconsistent results. The aim of the present study was to assess the efficacy of the RAAS inhibitor and β-blocker combination therapy vs. β-blocker monotherapy for hepatic vein pressure gradient (HVPG) reduction in cirrhosis. Studies were obtained using PubMed, Embase, Medline and Cochrane library databases up to July 2015, and the weighted mean difference (WMD) in HVPG reduction was used as a measure of treatment efficacy. In total, three studies (91 patients) were included. When compared to the β-blocker monotherapy, the RAAS inhibitor and β-blocker combination therapy resulted in a significant HVPG reduction [WMD 1.70; 95% confidence interval (CI): 0.52–2.88]. However, there was no significant difference in the heart rate reduction between the monotherapy and combination therapy groups (WMD −0.11; 95% CI: −3.51–3.29). In addition, no significant difference in the hemodynamic response was observed between the two groups (WMD 1.46; 95% CI: 0.93–2.30). In conclusion, the RAAS inhibitor and β-blocker combination therapy reduces portal hypertension significantly and to a greater extent than β-blocker monotherapy. Both therapies reduced the heart rate to similar levels; however, the RAAS inhibitor and β-blocker combination therapy reduced the mean arterial pressure to a greater extent. Due to the limited number of studies included, the data available do not allow a satisfactory comparison of adverse events. Moreover, further larger-scale trials are required in order to strengthen the results of the present study. PMID:28565796

[Hemodynamic study of the patient with hemorrhagic portal hypertension: importance of the left renal vein in patients with a distal splenorenal shunt (Warren)].

PubMed

Orozco, Héctor; Tielve, Manuel; Ramos, Guillermo; Mercado, Miguel Angel

2006-01-01

There is no information in the literature about surgical outcome of the distal splenorenal shunt (Warren shunt) in those patients with anomalous flow in the left renal vein to the inferior vena cava. The purpose of this manuscript was to evaluate the incidence of thrombosis in the Warren shunt in those patients with anomalous flow in the left renal vein to the inferior vena cava. We performed a prospective, descriptive and longitudinal study in those patients who performed a surgical procedure to the treatment of hemorrhagic portal hypertension in a tertiary referral center in Mexico City during a one year period (2002-2003). Before the surgical procedure an arterial and venous angiographic study was done including celiac axis, superior mesenteric artery and splenic artery. The patients were scheduled in the outpatient office the first, third, sixth month and the year after the surgical procedure. We looked in them for gastrointestinal bleeding secondary to portal hypertension. In those patients with Warren shunt an angiographic study was done during the first month after the surgical procedure. Twenty eight patients were included, 17 of them women (60.7%). Median patient age was 48 years old. In 20 patients a Warren shunt were done and in eigth patients a devascularization operation were done. The anomalous flow of the left renal vein was identified in nine patients (28.7%). In seven of them a Warren shunt were done and in two of them a devascularization operation were done. We didn't find gastrointestinal bleeding or thrombosis of the Warren shunt in any of these patients. In those cases of patients with anomalous flow in the left renal vein a Warren shunt can be performed. In this study we didn't find thrombosis of the shunt or gastrointestinal bleeding. In this way a surgical decompression of the portal system can be done preventing bleeding episodes.

Laparoscopic Splenectomy with Technical Standardization and Selection Criteria for Standard or Hand-Assisted Approach in 390 Patients with Liver Cirrhosis and Portal Hypertension.

PubMed

Kawanaka, Hirofumi; Akahoshi, Tomohiko; Kinjo, Nao; Harimoto, Norifumi; Itoh, Shinji; Tsutsumi, Norifumi; Matsumoto, Yoshihiro; Yoshizumi, Tomoharu; Shirabe, Ken; Maehara, Yoshihiko

2015-08-01

Laparoscopic splenectomy (LS) is still challenging in patients with liver cirrhosis and portal hypertension. This study was designed to establish safe and less invasive LS in patients with liver cirrhosis and portal hypertension. We analyzed 390 patients with liver cirrhosis and portal hypertension, who underwent LS between 1993 and 2013. Patients were divided into 3 time periods; early (1993 to 2004, n = 106); middle (2005 to 2008, n = 159); and late (2008 to 2013, n = 125). During the middle time period, standardized technique for LS and selection criteria for hand-assisted LS were adopted. Patients with spleen volume ≥ 1,000 mL by CT volumetry, large perisplenic collateral vessels, and/or Child-Pugh score ≥ 9, underwent hand-assisted LS. During the late time period, the selection criteria were refined and patients with spleen volume ≥ 600 mL underwent hand-assisted LS. Conversion to open splenectomy decreased (10.4% in the early time period, 1.9% in the middle time period, and 3.2% in the late time period, p = 0.004), median blood loss decreased (300g, 87g, and 98g, respectively, p < 0.001), and the success rate of pure LS tended to improve (87.2%, 89.5%, and 98.0%, respectively, p = 0.110). Mortality was 0% in each time period, Clavien-Dindo grade IIIb or more complications tended to decrease (5.7%, 2.5%, and 0.8%, respectively, p = 0.081), and technique-related complications decreased significantly (10.4%, 3.8%, and 2.4%, respectively, p = 0.014). Laparoscopic splenectomy is now a safe and less invasive approach, even in patients with liver cirrhosis and portal hypertension, because of its technical standardization with the refined selection criteria for pure or hand-assisted LS. Copyright © 2015 American College of Surgeons. Published by Elsevier Inc. All rights reserved.

Osteopontin: A non-invasive parameter of portal hypertension and prognostic marker of cirrhosis.

PubMed

Bruha, Radan; Jachymova, Marie; Petrtyl, Jaromir; Dvorak, Karel; Lenicek, Martin; Urbanek, Petr; Svestka, Tomislav; Vitek, Libor

2016-03-28

To investigate the relationship between osteopontin plasma concentrations and the severity of portal hypertension and to assess osteopontin prognostic value. A cohort of 154 patients with confirmed liver cirrhosis (112 ethylic, 108 men, age 34-72 years) were enrolled in the study. Hepatic venous pressure gradient (HVPG) measurement and laboratory and ultrasound examinations were carried out for all patients. HVPG was measured using a standard catheterization method with the balloon wedge technique. Osteopontin was measured using the enzyme-linked immunosorbent assay (ELISA) method in plasma. Patients were followed up with a specific focus on mortality. The control group consisted of 137 healthy age- and sex- matched individuals. The mean value of HVPG was 16.18 ± 5.6 mmHg. Compared to controls, the plasma levels of osteopontin in cirrhotic patients were significantly higher (P < 0.001). The plasma levels of osteopontin were positively related to HVPG (P = 0.0022, r = 0.25) and differed among the individual Child-Pugh groups of patients. The cut-off value of 80 ng/mL osteopontin distinguished patients with significant portal hypertension (HVPG above 10 mmHg) at 75% sensitivity and 63% specificity. The mean follow-up of patients was 3.7 ± 2.6 years. The probability of cumulative survival was 39% for patients with HVPG > 10 mmHg and 65% for those with HVPG ≤ 10 mmHg (P = 0.0086, odds ratio (OR), 2.92, 95% confidence interval (CI): 1.09-7.76). Osteopontin showed a similar prognostic value to HVPG. Patients with osteopontin values above 80 ng/mL had significantly lower cumulative survival compared to those with osteopontin ≤ 80 ng/mL (37% vs 56%, P = 0.00035; OR = 2.23, 95%CI: 1.06-4.68). Osteopontin is a non-invasive parameter of portal hypertension that distinguishes patients with clinically significant portal hypertension. It is a strong prognostic factor for survival.

Direct Percutaneous Embolization of Bleeding Stomal Varices

DOE Office of Scientific and Technical Information (OSTI.GOV)

Naidu, Sailen G., E-mail: naidu.sailen@mayo.ed; Castle, Erik P.; Kriegshauser, J. Scott

2010-02-15

Stomal variceal bleeding can develop in patients with underlying cirrhosis and portal hypertension. Most patients are best treated with transjugular intrahepatic portosystemic shunt (TIPS) creation because this addresses the underlying problem of portal hypertension. However, some patients are not good candidates for TIPS creation because they have end-stage liver disease or encephalopathy. We describe such a patient who presented with recurrent bleeding stomal varices, which was successfully treated with percutaneous coil embolization. The patient had bleeding-free survival for 1 month before death from unrelated causes.

[The current state of the surgery of portal hypertension].

PubMed

Mercado, M A; Orozco, H

1992-01-01

Surgery for bleeding portal hypertension has evolved widely in the last decades. The surgical procedures that preserve portal blood flow are the first operative choice for well selected patients. Operative procedures that deprive the portal blood flow to the liver, are most likely to promote deterioration of liver function in the late postoperative period. The operation most frequently performed are the selective shunts (Warren) and the thoraco abdominal devascularization (Sugiura). The best results are obtained in patients with a good liver function that are operated in an elective fashion. Non-selective shunts have a restricted indication and low diameter porto systemic shunts are still under evaluation. The combination of drug therapy and/or sclerotherapy with surgery appears to improve survival. Liver transplants are indicated for those patients with associated liver failure. For patients with good liver function, surgery is the therapy of choice.

[Treatment of liver cirrhosis - actually possibility of ambulant internist].

PubMed

Ehrmann, Jiří; Aiglová, Květa; Konečný, Michal; Procházka, Vlastimil; Vrzalová, Drahomíra

There are 40 000-60 000 patients with cirrhosis in the Czech Republic. 2 000 die of this disease yearly. This group of patients needs a complex treatment and it is mostly an internist cooperating with other specialists. The most important for an ambulant internist is to diagnose the disease as soon as possible and start with treatment of chronic liver disease that could lead to a cirrhosis. It means especially chronic viral hepatitis, alcoholic or non-alcoholic steatosis/steatohepatitis, auto-immune liver damage and metabolic disease. The next step is to diagnose the cirrhosis in time when it is in no manifest stage. The third step is to diagnose and treat the liver decompensation. It means consequences of the portal hypertension, it is ascit, esophageal or gastric varices, hepatorenal syndrome. Next there are consequences of the metabolic insufficiency, it is icterus, coagulopathy and hepatic encephalopathy. It is necessary to diagnose and cure cholestasis from the very first extrahepatic causes. For a successful treatment of the hepatocellular carcinoma originated almost exclusively in the grounds of the cirrhosis must be early diagnosed. The ambulant internist respective hepatologist must diagnose the stage of the cirrhosis and decide when a hospitalization is necessary. Also a close cooperation with other specialists is urgent if it is about a liver transplantation. The treatment of successive stages of the cirrhosis is a topic of the showed educational article. compensated/decompensated liver cirrhosis - diet/nutrition in liver cirrhosis - etiology and diagnose of liver cirrhosis - treatment of liver insufficiency/failure - treatment of portal hypertension and its complications.

Non-variceal upper gastrointestinal bleeding in cirrhotic patients in Nile Delta.

PubMed

Gabr, Mamdouh Ahmed; Tawfik, Mohamed Abd El-Raouf; El-Sawy, Abd Allah Ahmed

2016-01-01

Acute upper gastrointestinal bleeding (AUGIB) in cirrhotic patients occurs mainly from esophageal and gastric varices; however, quite a large number of cirrhotic patients bleed from other sources as well. The aim of the present work is to determine the prevalence of non-variceal UGIB as well as its different causes among the cirrhotic portal hypertensive patients in Nile Delta. Emergency upper gastrointestinal (UGI) endoscopy for AUGIB was done in 650 patients. Out of these patients, 550 (84.6%) patients who were proved to have cirrhosis were the subject of the present study. From all cirrhotic portal hypertensive patients, 415 (75.5%) bled from variceal sources (esophageal and gastric) while 135 (24.5%) of them bled from non-variceal sources. Among variceal sources of bleeding, esophageal varices were much more common than gastric varices. Peptic ulcer was the most common non-variceal source of bleeding. Non-variceal bleeding in cirrhosis was not frequent, and sources included peptic ulcer, portal hypertensive gastropathy, and erosive disease of the stomach and duodenum.

Preoperative predictors of portal vein thrombosis after splenectomy with periesophagogastric devascularization

PubMed Central

Zhang, Yu; Wen, Tian-Fu; Yan, Lu-Nan; Yang, Hong-Ji; Deng, Xiao-Fan; Li, Chuan; Wang, Chuan; Liang, Guan-Lin

2012-01-01

AIM: To evaluate the predictive value of preoperative predictors for portal vein thrombosis (PVT) after splenectomy with periesophagogastric devascularization. METHODS: In this prospective study, 69 continuous patients with portal hypertension caused by hepatitis B cirrhosis underwent splenectomy with periesophagogastric devascularization in West China Hospital of Sichuan University from January 2007 to August 2010. The portal vein flow velocity and the diameter of portal vein were measured by Doppler sonography. The hepatic congestion index and the ratio of velocity and diameter were calculated before operation. The prothrombin time (PT) and platelet (PLT) levels were measured before and after operation. The patients’ spleens were weighed postoperatively. RESULTS: The diameter of portal vein was negatively correlated with the portal vein flow velocity (P < 0.05). Thirty-three cases (47.83%) suffered from postoperative PVT. There was no statistically significant difference in the Child-Pugh score, the spleen weights, the PT, or PLT levels between patients with PVT and without PVT. Receiver operating characteristic curves showed four variables (portal vein flow velocity, the ratio of velocity and diameter, hepatic congestion index and diameter of portal vein) could be used as preoperative predictors of postoperative portal vein thrombosis. The respective values of the area under the curve were 0.865, 0.893, 0.884 and 0.742, and the respective cut-off values (24.45 cm/s, 19.4333/s, 0.1138 cm/s-1 and 13.5 mm) were of diagnostically efficient, generating sensitivity values of 87.9%, 93.9%, 87.9% and 81.8%, respectively, specificities of 75%, 77.8%, 86.1% and 63.9%, respectively. CONCLUSION: The ratio of velocity and diameter was the most accurate preoperative predictor of portal vein thrombosis after splenectomy with periesophagogastric devascularization in hepatitis B cirrhosis-related portal hypertension. PMID:22553410

Lung Transplantation in Patients with Pulmonary Hypertension

MedlinePlus

... Pulmonary Hypertension Consensus Statements Issued by the Scientific Leadership Council Download & Print PDF DISCLAIMER: This information is ... on our new PHPN/PHCR or Support Group Leadership Institute portal? Reset your password here . Login Username ...

Ammonia produces pathological changes in human hepatic stellate cells and is a target for therapy of portal hypertension.

PubMed

Jalan, Rajiv; De Chiara, Francesco; Balasubramaniyan, Vairappan; Andreola, Fausto; Khetan, Varun; Malago, Massimo; Pinzani, Massimo; Mookerjee, Rajeshwar P; Rombouts, Krista

2016-04-01

Hepatic stellate cells (HSCs) are vital to hepatocellular function and the liver response to injury. They share a phenotypic homology with astrocytes that are central in the pathogenesis of hepatic encephalopathy, a condition in which hyperammonemia plays a pathogenic role. This study tested the hypothesis that ammonia modulates human HSC activation in vitro and in vivo, and evaluated whether ammonia lowering, by using l-ornithine phenylacetate (OP), modifies HSC activation in vivo and reduces portal pressure in a bile duct ligation (BDL) model. Primary human HSCs were isolated and cultured. Proliferation (BrdU), metabolic activity (MTS), morphology (transmission electron, light and immunofluorescence microscopy), HSC activation markers, ability to contract, changes in oxidative status (ROS) and endoplasmic reticulum (ER) were evaluated to identify effects of ammonia challenge (50 μM, 100 μM, 300 μM) over 24-72 h. Changes in plasma ammonia levels, markers of HSC activation, portal pressure and hepatic eNOS activity were quantified in hyperammonemic BDL animals, and after OP treatment. Pathophysiological ammonia concentrations caused significant and reversible changes in cell proliferation, metabolic activity and activation markers of hHSC in vitro. Ammonia also induced significant alterations in cellular morphology, characterised by cytoplasmic vacuolisation, ER enlargement, ROS production, hHSC contraction and changes in pro-inflammatory gene expression together with HSC-related activation markers such as α-SMA, myosin IIa, IIb, and PDGF-Rβ. Treatment with OP significantly reduced plasma ammonia (BDL 199.1 μmol/L±43.65 vs. BDL+OP 149.27 μmol/L±51.1, p<0.05) and portal pressure (BDL 14±0.6 vs. BDL+OP 11±0.3 mmHg, p<0.01), which was associated with increased eNOS activity and abrogation of HSC activation markers. The results show for the first time that ammonia produces deleterious morphological and functional effects on HSCs in vitro. Targeting ammonia with the ammonia lowering drug OP reduces portal pressure and deactivates hHSC in vivo, highlighting the opportunity for evaluating ammonia lowering as a potential therapy in cirrhotic patients with portal hypertension. Copyright © 2015 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.

Effects of in vitro cultivated Calculus Bovis compound on pulmonary lesions in rabbits with schistosomiasis.

PubMed

Li, Tao; Yang, Zhen; Cai, Hong-Jiao; Song, Li-Wei; Lu, Ke-Yu; Zhou, Zheng; Wu, Zai-De

2010-02-14

To explore the interventional effects and mechanism of in vitro cultivated Calculus Bovis compound preparation (ICCBco) on pulmonary lesions in portal hypertensive rabbits with schistosomiasis. The experimental group included 20 portal hypertensive rabbits with schistosomiasis treated by ICCBco. The control group included 20 portal hypertensive rabbits with schistosomiasis treated by praziquantel. The morphological changes of the pulmonary tissues were observed under light and electron microscopy. The expression of fibronectin (FN) and laminin (LN) in the lung tissues was analyzed by immunohistochemistry. Under light microscope, the alveolar exudation in the lung tissue was more frequently observed in the control group, while the alveolar space was fairly dry in the lung tissue of ICCBco group. Under electron microscope, more alveolar exudation in the lung tissue, and more macrophages, alveolar angiotelectasis and the blurred three-tier structure of alveolar-capillary barrier could be seen in the control group. In ICCBco group, fibers within the alveolar interspace slightly increased in some lung regions, and the structure of type I epithelium, basement membrane and endodermis was complete, and no obvious exudation from the alveolar space, and novascular congestion could be observed. There was a positive or strong positive expression of FN and LN in the lung tissue of the control group, while there was a negative or weak positive expression of FN and LN in ICCBco group. ICCBco can effectively prevent pulmonary complications in portal hypertensive rabbits with schistosomiasis by means of improving lung microcirculation and lowering the content of extracellular matrix.

Identifying Patients for Clinical Studies from Electronic Health Records: TREC 2012 Medical Records Track at OHSU

DTIC Science & Technology

2012-11-01

causes of hypertension ") AND NOT(report_text:"pulmonary| portal hypertension " OR report_text:"secondary to hypertension ") 182 Patients with Ischemic... hypertension , and tachycardia (discharge_icd_codes_txt:293.0 OR report_text:delirium) AND (discharge_icd_codes_txt:401.* OR discharge_icd_codes_txt:405...report_text:"**AGE[in teens") 162 Patients with hypertension on anti- hypertensive medication (report_text:" hypertension " OR report_text:"high blood

Role of estrogen receptor β selective agonist in ameliorating portal hypertension in rats with CCl4-induced liver cirrhosis.

PubMed

Zhang, Cheng-Gang; Zhang, Bin; Deng, Wen-Sheng; Duan, Ming; Chen, Wei; Wu, Zhi-Yong

2016-05-14

To investigate the role of diarylpropionitrile (DPN), a selective agonist of estrogen receptor β (ERβ), in liver cirrhosis with portal hypertension (PHT) and isolated hepatic stellate cells (HSCs). Female Sprague-Dawley rats were ovariectomized (OVX), and liver cirrhosis with PHT was induced by CCl4 injection. DPN and PHTPP, the selective ERβ agonist and antagonist, were used as drug interventions. Liver fibrosis was assessed by hematoxylin and eosin (HE) and Masson's trichrome staining and by analyzing smooth muscle actin expression. Hemodynamic parameters were determined in vivo using colored microspheres technique. Protein expression and phosphorylation were determined by immunohistochemical staining and Western blot analysis. Messenger RNA levels were analyzed by quantitative real-time polymerase chain reaction (qRT-PCR). Collagen gel contraction assay was performed using gel lattices containing HSCs treated with DPN, PHTPP, or Y-27632 prior to ET-1 addition. Treatment with DPN in vivo greatly lowered portal pressure and improved hemodynamic parameters without affecting mean arterial pressure, which was associated with the attenuation of liver fibrosis and intrahepatic vascular resistance (IHVR). In CCl4-treated rat livers, DPN significantly decreased the expression of RhoA and ROCK II, and even suppressed ROCK II activity. Moreover, DPN remarkedly increased the levels of endothelial nitric oxide synthase (eNOS) and phosphorylated eNOS, and promoted the activities of protein kinase G (PKG), which is an NO effector in the liver. Furthermore, DPN reduced the contractility of activated HSCs in the 3-dimensional stress-relaxed collagen lattices, and decreased the ROCK II activity in activated HSCs. Finally, in vivo/in vitro experiments demonstrated that MLC activity was inhibited by DPN. For OVX rats with liver cirrhosis, DPN suppressed liver RhoA/ROCK signal, facilitated NO/PKG pathways, and decreased IHVR, giving rise to reduced portal pressure. Therefore, DPN represents a relevant treatment choice against PHT in cirrhotic patients, especially postmenopausal women.

Percutaneous Retrograde Sclerotherapy for Refractory Bleeding of Jejunal Varices: Direct Injection via Superficial Epigastric Vein

DOE Office of Scientific and Technical Information (OSTI.GOV)

Nakata, Manabu, E-mail: nktmnbohsu@jichi.ac.jp; Nakata, Waka, E-mail: waka-s@jichi.ac.jp; Isoda, Norio, E-mail: isodano1@jichi.ac.jp

2012-02-15

Small-bowel varices are rare and almost always occur in cases with portal hypertension. We encountered a patient with bleeding jejunal varices due to liver cirrhosis. Percutaneous retrograde sclerotherapy was performed via the superficial epigastric vein. Melena disappeared immediately after treatment. Disappearance of jejunal varices was confirmed by contrast-enhanced computed tomography. After 24 months of follow-up, no recurrent melena was observed.

Pulmonary arterial hypertension and portal hypertension in a patient with hereditary hemorrhagic telangiectasia.

PubMed

Pousada, Guillermo; Baloira, Adolfo; Valverde, Diana

2015-03-15

Pulmonary arterial hypertension (PAH) is a rare disease that could be inherited with an autosomal dominant pattern. Mutations in BMPR2 gene are described in over 70% of cases, although other genes are involved in lesser extend in PAH. Hereditary hemorrhagic telangiectasia (HHT) is another rare autosomal dominant disease. PAH is a rare complication of HHT that occurs in less than 1% of cases. Liver cirrhosis with portal hypertension is also associated with the presence of PAHs in 1-2% of cases. We present here a patient with HHT who developed PAH shortly after showing portal hypertension. Some genes (BMPR2, ACVRL1, ENG) seem to play an important role in PAH pathogenesis. We analyzed these genes, detecting mutations in BMPR2 gene (c.1021G>A (V341L), c.327G>A (p.Q109Q)), ACVRL1 (c.313+20C>A, c.1502+7A>G) and ENG (c.498G>A (Q166Q)). The patient also had 3 polymorphisms in the TRPC6 gene (c.1-361A>T, c.1-254C>G, c.1-218C>T). The study of these genes will help us to identify and track individuals susceptible for developing PAH associated with other diseases. Copyright © 2014 Elsevier España, S.L.U. All rights reserved.

Splenic artery aneurysms encountered in the ED: 10 years' experience.

PubMed

Liu, Chu-Feng; Kung, Chia-Te; Liu, Ber-Ming; Ng, Shu-Hang; Huang, Chung-Cheng; Ko, Sheung-Fat

2007-05-01

Our objective was to report 7 cases of splenic artery aneurysm (SAA) encountered in the emergency department (ED). A retrospective survey of our ED database revealed 7 cases of SAA (6 men, 1 woman; mean age, 56 years) of 651,347 ED visits over the last decade. Their clinical and imaging features, management, and outcomes were evaluated. Splenic artery aneurysm in the ED was rare (prevalence, 0.011%). Common presentations included acute abdomen (n = 5) and shock (n = 2). Five cases had liver cirrhosis and portal hypertension. Abdominal radiographs (n = 7) revealed 2 atherosclerotic patients with SAA. Abdominal computed tomography (n = 7) depicted all SAAs (size, 1.5-8 cm; mean, 3.8 cm). Four ruptured SAAs were successfully managed with coils embolization. Among them, 1 patient with ruptured mycotic SAA also received surgery, but the patient died of Klebsiella sepsis 3 months later. In the ED, ruptured SAA should be included as a rare differential consideration of acute abdomen, especially in middle-aged men with liver cirrhosis and portal hypertension. Although SAA may be an unexpected computed tomographic finding, once diagnosed, endovascular treatment is recommended.

Interferon-free regimens improve portal hypertension and histological necroinflammation in HIV/HCV patients with advanced liver disease.

PubMed

Schwabl, P; Mandorfer, M; Steiner, S; Scheiner, B; Chromy, D; Herac, M; Bucsics, T; Hayden, H; Grabmeier-Pfistershammer, K; Ferlitsch, A; Oberhuber, G; Trauner, M; Peck-Radosavljevic, M; Reiberger, T

2017-01-01

HIV/HCV co-infected patients show accelerated fibrosis progression and higher risk for complications of portal hypertension (PHT). To assess the effects of interferon-free therapy on portal pressure, liver histology and plasma biomarkers in HIV/HCV-coinfected patients with PHT. Twenty-two patients with paired hepatic venous pressure gradient (HVPG) measurements prior and after successful treatment (SVR) with interferon-free regimens were included. Liver stiffness was assessed by transient elastography and biopsies were scored according to METAVIR. Plasma biomarkers were determined by ELISA. Overall, HVPG decreased from 10.7 ± 4.1 mmHg at baseline to 7.4 ± 4.2 mmHg after HCV treatment (Δ:-3.3 ± 2.7 mmHg; p < 0.001). In patients with clinically significant PHT (HVPG≥10 mmHg, n = 11), HVPG decreased from 14.1 ± 2.9 to 10.4 ± 3.9 mmHg (Δ:-3.7 ± 3.3 mmHg; p = 0.004) and a haemodynamic response (HVPG decrease ≥10%) was observed in 73%. In 64% of patients with subclinical PHT (HVPG 6-9 mmHg, n = 11), portal pressure normalised at SVR. Mean liver stiffness decreased from 20.8 kPa to 11.5 kPa (Δ:-8.8 ± 7.4 kPa; p < 0.001). Fifty percent (7/14) of patients with cirrhosis were re-classified as METAVIR ≤F3 and all patients with decompensated cirrhosis improved their Child-Pugh stage. After successful HCV treatment, 39% still had persistent histological necroinflammatory activity (METAVIR A1), which correlated with less HVPG response and more steatosis. While most biomarkers improved with SVR, METAVIR A1 patients had significantly higher plasma levels of fibrogenic (PDGF, TGF-β) and angiogenic (VEGF, Angiopoietin1) biomarkers. Interferon-free therapy reduces PHT and halts histological necroinflammatory activity in the majority of HIV/HCV-coinfected patients after SVR, which may lead to re-compensation of liver function in cirrhosis. Biomarkers could identify patients with persisting hepatic necroinflammation. © 2016 John Wiley & Sons Ltd.

Janus-kinase-2 relates directly to portal hypertension and to complications in rodent and human cirrhosis.

PubMed

Klein, Sabine; Rick, Johanna; Lehmann, Jennifer; Schierwagen, Robert; Schierwagen, Irela Gretchen; Verbeke, Len; Hittatiya, Kanishka; Uschner, Frank Erhard; Manekeller, Steffen; Strassburg, Christian P; Wagner, Kay-Uwe; Sayeski, Peter P; Wolf, Dominik; Laleman, Wim; Sauerbruch, Tilman; Trebicka, Jonel

2017-01-01

Angiotensin II (AngII) activates via angiotensin-II-type-I receptor (AT1R) Janus-kinase-2 (JAK2)/Arhgef1 pathway and subsequently RHOA/Rho-kinase (ROCK), which induces experimental and probably human liver fibrosis. This study investigated the relationship of JAK2 to experimental and human portal hypertension. The mRNA and protein levels of JAK2/ARHGEF1 signalling components were analysed in 49 human liver samples and correlated with clinical parameters of portal hypertension in these patients. Correspondingly, liver fibrosis (bile duct ligation (BDL), carbon tetrachloride (CCl 4 )) was induced in floxed-Jak2 knock-out mice with SM22-promotor (SM22 Cre+ -Jak2 f/f ). Transcription and contraction of primary myofibroblasts from healthy and fibrotic mice and rats were analysed. In two different cirrhosis models (BDL, CCl 4 ) in rats, the acute haemodynamic effect of the JAK2 inhibitor AG490 was assessed using microsphere technique and isolated liver perfusion experiments. Hepatic transcription of JAK2/ARHGEF1 pathway components was upregulated in liver cirrhosis dependent on aetiology, severity and complications of human liver cirrhosis (Model for End-stage Liver disease (MELD) score, Child score as well as ascites, high-risk varices, spontaneous bacterial peritonitis). SM22 Cre+ - Jak2 f/f mice lacking Jak2 developed less fibrosis and lower portal pressure (PP) than SM22 Cre- -Jak2 f/f upon fibrosis induction. Myofibroblasts from SM22 Cre+ -Jak2 f/f mice expressed less collagen and profibrotic markers upon activation. AG490 relaxed activated hepatic stellate cells in vitro. In cirrhotic rats, AG490 decreased hepatic vascular resistance and consequently the PP in vivo and in situ. Hepatic JAK2/ARHGEF1/ROCK expression is associated with portal hypertension and decompensation in human cirrhosis. The deletion of Jak2 in myofibroblasts attenuated experimental fibrosis and acute inhibition of JAK2 decreased PP. Thus, JAK2 inhibitors, already in clinical use for other indications, might be a new approach to treat cirrhosis with portal hypertension. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

Applying Technology to Enhance Nursing Education in the Psychological Health and Traumatic Brain Injury Needs of Veterans and their Families

DTIC Science & Technology

2012-10-01

mhslearn.csd.disa.mil/ilearn/en/learner/mhs/ portal /civilian_login.jsp Military Health System Portal 6. http://www.dcoe.health.mil/ Defense Centers of...forget. He is in rehab for a traumatic brain injury and is seen today for evaluation of hypertension . During the intake, Andrew casually says, I don t...statement: a. Did you know that hypertension can cause damage to blood vessels that keep organs healthy? b. Are you worried about side effects? There are

Hepatic arteriovenous fistulae and portal vein hypoplasia in a Labrador retriever.

PubMed

Schaeffer, I G; Kirpensteijn, J; Wolvekamp, W T; Van den Ingh, T S; Rothuizen, J

2001-03-01

An 18-month-old male Labrador retriever was referred for investigation of chronic intermittent diarrhoea and vomiting of two months duration. A diagnosis of hepatic arteriovenous fistulae was made. These are extremely rare hepatic vascular anomalies which confer arterial pressure to the portal vein. Liver atrophy, portal vein hypoplasia, portal hypertension and multiple acquired portosystemic collateral vessels are the main complications. Surgical excision is a challenge as resection of large lesions may be associated with significant blood loss. In this dog, persistence of portal vein hypoplasia and extensive collateral pathways following surgery led to a reserved prognosis.

Vasopressin and nitroglycerin decrease portal and hepatic venous pressure and hepato-splanchnic blood flow.

PubMed

Wisén, E; Svennerholm, K; Bown, L S; Houltz, E; Rizell, M; Lundin, S; Ricksten, S-E

2018-03-26

Various methods are used to reduce venous blood pressure in the hepato-splanchnic circulation, and hence minimise blood loss during liver surgery. Previous studies show that combination of vasopressin and nitroglycerin reduces portal pressure and flow in patients with portal hypertension, and in this study we investigated this combination in patients with normal portal pressure. In all, 13 patients were studied. Measurements were made twice to confirm baseline (C1 and BL), during vasopressin infusion 4.8 U/h (V), and during vasopressin infusion combined with nitroglycerin infusion (V + N). Portal venous pressure (PVP), hepatic venous pressure (HVP), central haemodynamics and arterial and venous blood gases were obtained at each measuring point, and portal (splanchnic) and hepato-splanchnic blood flow changes were calculated. Vasopressin alone did not affect PVP, whereas HVP increased slightly. In combination with nitroglycerin, PVP decreased from 10.1 ± 1.6 to 8.9 ± 1.3 mmHg (P < 0.0001), and HVP decreased from 7.9 ± 1.9 to 6.2 ± 1.3 mmHg (P = 0.001). Vasopressin reduced portal blood flow by 47 ± 19% and hepatic venous flow by 11 ± 18%, respectively. Addition of nitroglycerin further reduced portal- and hepatic flow by 55 ± 13% and 30 ± 13%, respectively. Vasopressin alone had minor effects on central haemodynamics, whereas addition of nitroglycerin reduced cardiac index (3.2 ± 0.7 to 2.7 ± 0.5; P < 0.0001). The arterial-portal vein lactate gradient was unaffected. The combination of vasopressin and nitroglycerin decreases portal pressure and hepato-splanchnic blood flow, and could be a potential treatment to reduce bleeding in liver resection surgery. © 2018 The Acta Anaesthesiologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.

Use of portal pressure studies in the management of variceal haemorrhage.

PubMed

Addley, Jennifer; Tham, Tony Ck; Cash, William Jonathan

2012-07-16

Portal hypertension occurs as a complication of liver cirrhosis and complications such as variceal bleeding lead to significant demands on resources. Endoscopy is the gold standard method for screening cirrhotic patients however universal endoscopic screening may mean a lot of unnecessary procedures as the presence of oesophageal varices is variable hence a large time and cost burden on endoscopy units to carry out both screening and subsequent follow up of variceal bleeds. A less invasive method to identify those at high risk of bleeding would allow earlier prophylactic measures to be applied. Hepatic venous pressure gradient (HVPG) is an acceptable indirect measurement of portal hypertension and predictor of the complications of portal hypertension in adult cirrhotics. Varices develop at a HVPG of 10-12 mmHg with the appearance of other complications with HPVG > 12 mmHg. Variceal bleeding does not occur in pressures under 12 mmHg. HPVG > 20 mmHg measured early after admission is a significant prognostic indicator of failure to control bleeding varices, indeed early transjugular intrahepatic portosystemic shunt (TIPS) in such circumstances reduces mortality significantly. HVPG can be used to identify responders to medical therapy. Patients who do not achieve the suggested reduction targets in HVPG have a high risk of rebleeding despite endoscopic ligation and may not derive significant overall mortality benefit from endoscopic intervention alone, ultimately requiring TIPS or liver transplantation. Early HVPG measurements following a variceal bleed can help to identify those at risk of treatment failure who may benefit from early intervention with TIPS. Therefore, we suggest using HVPG measurement as the investigation of choice in those with confirmed cirrhosis in place of endoscopy for intitial variceal screening and, where indicated, a trial of B-blockade, either intravenously during the initial pressure study with assessment of response or oral therapy with repeat HVPG six weeks later. In those with elevated pressures, primary medical prophylaxis could be commenced with subsequent close monitoring of HVPG thus negating the need for endoscopy at this point. All patients presenting with variceal haemorrhage should undergo HVPG measurement and those with a gradient greater than 20 mmHg should be considered for early TIPS. By introducing portal pressure studies into a management algorithm for variceal bleeding, the number of endoscopies required for further intervention and follow up can be reduced leading to significant savings in terms of cost and demand on resources.

Jejunal varices after choledochojejunostomy treated with laparotomic transcatheter variceal embolization.

PubMed

Waguri, Nobuo; Azumi, Rie; Sugimura, Kazuhito; Arao, Yoshihisa; Ikarashi, Shunzo; Sakai, Norihiro; Ogawa, Masahiro; Osaki, Akihiko; Sato, Munehiro; Aiba, Tsuneo; Yoneyama, Osamu; Furukawa, Koichi; Igarashi, Kentarou

2016-01-01

Portal hypertension induces collateral shunt formation between the portal and systemic circulation, decompressing the elevated portal pressure. Ectopic varices outside of the gastroesophageal region, such as jejunal varices, are rare conditions. This report describes the successful embolization of ruptured jejunal varices resulting from an extrahepatic portal obstruction. A 62-year-old man was admitted to our hospital with recurrent massive gastrointestinal bleeding. Fourteen months earlier, he had undergone a choledochojejunostomy and pancreatic cystojejunostomy for bile duct stenosis with an enlarged pancreatic pseudocyst due to severe chronic pancreatitis. Contrast-enhanced computed tomography showed jejunal intramural dilated vessels close to the choledochojejunal anastomosis, but extravasation was not observed. Due to the lack of a rapid definitive diagnosis, the patient required massive blood transfusions. Hemorrhagic scintigraphy using 99m Tc-HSAD finally identified the site of the hemorrhage. Angiography and double-balloon endoscopy revealed the anastomotic jejunal varices to be the result of an extrahepatic portal obstruction. Laparotomic transcatheter variceal embolization with microcoils was successful in halting the refractory gastrointestinal bleeding. This surgery preserved hepatopetal portal venous flow by another route, and no complications were observed. At present, 4 years post-surgery, there has been no recurrence of gastrointestinal hemorrhage. The development of jejunal varices is often associated with postoperative adhesions. Some patients with a history of hepatico- or choledochojejunostomy may experience portal hypertension resulting from extrahepatic portal obstruction, leading to the formation of jejunal varices as hepatopetal portal collaterals. The choice of therapy in each patient should be based on the individual hemodynamics of the ectopic varices.

Spectrum of small-bowel mucosal abnormalities identified by capsule endoscopy in patients with portal hypertension of varied etiology.

PubMed

Chandrasekar, T S; Janakan, Gokul Bollu; Chandrasekar, Viveksandeep Thoguluva; Kalamegam, Raja Yogesh; Suriyanarayanan, Sathiamoorthy; Sanjeevaraya, Prasad Menta

2017-01-01

Bleeding from small intestinal ectopic varices and persistent anemia caused by portal hypertensive enteropathy (PHE) can be very challenging. Capsule endoscopy (CE) is one of the best noninvasive modalities in identifying such lesions. The aims of this study are to study the prevalence of small-bowel changes related to portal hypertension (PHT) and to correlate them with the observations related to the effects of portal hypertension in the esophagus, stomach, and colon. Thirty-two patients with various etiologies of PHT with either anemia or gastrointestinal bleed were included along with age- and sex-matched controls without PHT. All patients underwent blood tests, gastroscopy, colonoscopy, and CE. The small-bowel findings by CE were categorized as inflammatory-like and vascular lesions. The small-bowel changes were analyzed to find out any association with various demographic, clinical, and endoscopic variables. Thirty-one out of 32 patients with PHT (96.8%) had PHE identified by CE. Of them, 31 (96.8%) had inflammatory-like appearance, 11 (34.4%) had vascular lesions, and 2 (6.2%) had small-bowel varices. Inflammatory-like appearance was noted in eight (25%) and angiodysplastic lesions in two (6.2%) controls. Findings compatible with PHE were detected in 96.8% of the patients and 25% of the controls (X 2 =34.72, p=0.000).The presence of PHE was not associated with any of the above-mentioned variables. Small-bowel mucosal changes were seen in significantly higher number of patients with PHT with anemia.

Chemotherapy and Drug Targeting in the Treatment of Leishmaniasis

DTIC Science & Technology

1989-05-30

in the catabolism of adenosine to inosine, and ultimately to the final breakdown product o2 purines, uric acid . Since sinefungin is an adenosine analog...chemotherapeutic value if they inhibit nucleic acid synthesis in the pathogen but not in the host. This sometimes occurs if the host enzymes are slightly...remove these organisms from the blood and bleeding secondary to portal hypertension. Currently there are major epidemics in India and Kenya . Figure 1

5 CFR 831.2207 - Partial deferred payment of the lump-sum credit if annuity commences after January 3, 1988, and...

Code of Federal Regulations, 2014 CFR

2014-01-01

...] (H) Severe cardiomyopathy—Class IV. (I) Aplastic anemia. (J) Uncontrolled hypertension with...) Severe hepatic failure. (N) Severe Hypoxic brain damage. (O) Severe portal hypertension with esophageal...

5 CFR 831.2207 - Partial deferred payment of the lump-sum credit if annuity commences after January 3, 1988, and...

Code of Federal Regulations, 2010 CFR

2010-01-01

...] (H) Severe cardiomyopathy—Class IV. (I) Aplastic anemia. (J) Uncontrolled hypertension with...) Severe hepatic failure. (N) Severe Hypoxic brain damage. (O) Severe portal hypertension with esophageal...

5 CFR 831.2207 - Partial deferred payment of the lump-sum credit if annuity commences after January 3, 1988, and...

Code of Federal Regulations, 2011 CFR

2011-01-01

...] (H) Severe cardiomyopathy—Class IV. (I) Aplastic anemia. (J) Uncontrolled hypertension with...) Severe hepatic failure. (N) Severe Hypoxic brain damage. (O) Severe portal hypertension with esophageal...

5 CFR 831.2207 - Partial deferred payment of the lump-sum credit if annuity commences after January 3, 1988, and...

Code of Federal Regulations, 2013 CFR

2013-01-01

...] (H) Severe cardiomyopathy—Class IV. (I) Aplastic anemia. (J) Uncontrolled hypertension with...) Severe hepatic failure. (N) Severe Hypoxic brain damage. (O) Severe portal hypertension with esophageal...

5 CFR 831.2207 - Partial deferred payment of the lump-sum credit if annuity commences after January 3, 1988, and...

Code of Federal Regulations, 2012 CFR

2012-01-01

...] (H) Severe cardiomyopathy—Class IV. (I) Aplastic anemia. (J) Uncontrolled hypertension with...) Severe hepatic failure. (N) Severe Hypoxic brain damage. (O) Severe portal hypertension with esophageal...

Clinical, radiologic, and endoscopic characteristics upon diagnosis of patients with prehepatic portal hypertension at the Instituto Nacional de Pediatría from 2001 to 2011.

PubMed

Zárate Mondragón, F; Romero Trujillo, J O; Cervantes Bustamante, R; Mora Tiscareño, M A; Montijo Barrios, E; Cadena León, J F; Cázares Méndez, M; Toro Monjaraz, E M; Ramírez Mayans, J

2014-01-01

Prehepatic portal hypertension in children can be asymptomatic for many years. Once diagnosed, the therapeutic measures (pharmacologic, endoscopic, and surgical) are conditioned by the specific characteristics of each patient. In Mexico, there are no recorded data on the incidence of the disease and patient characteristics. To determine the main clinical, radiologic, and endoscopic characteristics upon diagnosis of these patients at the Instituto Nacional de Pediatría within the time frame of January 2001 and December 2011. A cross-sectional, retrolective, descriptive, and observational study was conducted in which all the medical records of the patients with portal hypertension diagnosis were reviewed. There was a greater prevalence of prehepatic etiology (32/52) (61.5%) in the portal hypertension cases reviewed. Males (62.5%) predominated and 11 of the 32 patients were under 4 years of age. The primary reason for medical consultation was upper digestive tract bleeding with anemia (71.9%) and the main pathology was cavernomatous degeneration of the portal vein (65.6%). Splenoportography was carried out on 17 of the 32 patients. A total of 65.5% of the patients received the combination therapy of propranolol and a proton pump inhibitor. Initial endoscopy revealed esophageal varices in 96.9% of the patients, 12 of whom presented with gastroesophageal varices. Congestive gastropathy was found in 75% of the patients. The varices were ligated in 8 cases, sclerotherapy for esophageal varices was carried out in 5 cases (15.6%), and sclerotherapy for gastric varices was performed in 2 patients. Seventeen patients (53.1%) underwent portosystemic diversion: 10 of the procedures employed a mesocaval shunt and 7 a splenorenal shunt. Nine patients (28.1%) underwent total splenectomy. The primary cause of the disease was cavernomatous degeneration of the portal vein; it was predominant in males and the first symptom was variceal bleeding. Copyright © 2014 Asociación Mexicana de Gastroenterología. Published by Masson Doyma México S.A. All rights reserved.

Comparison of radial 4D Flow-MRI with perivascular ultrasound to quantify blood flow in the abdomen and introduction of a porcine model of pre-hepatic portal hypertension.

PubMed

Frydrychowicz, A; Roldan-Alzate, A; Winslow, E; Consigny, D; Campo, C A; Motosugi, U; Johnson, K M; Wieben, O; Reeder, S B

2017-12-01

Objectives of this study were to compare radial time-resolved phase contrast magnetic resonance imaging (4D Flow-MRI) with perivascular ultrasound (pvUS) and to explore a porcine model of acute pre-hepatic portal hypertension (PHTN). Abdominal 4D Flow-MRI and pvUS in portal and splenic vein, hepatic and both renal arteries were performed in 13 pigs of approximately 60 kg. In six pigs, measurements were repeated after partial portal vein (PV) ligature. Inter- and intra-reader comparisons and statistical analysis including Bland-Altman (BA) comparison, paired Student's t tests and linear regression were performed. PvUS and 4D Flow-MRI measurements agreed well; flow before partial PV ligature was 322 ± 30 ml/min in pvUS and 297 ± 27 ml/min in MRI (p = 0.294), and average BA difference was 25 ml/min [-322; 372]. Inter- and intra-reader results differed very little, revealed excellent correlation (R 2  = 0.98 and 0.99, respectively) and resulted in BA differences of -5 ml/min [-161; 150] and -2 ml/min [-28; 25], respectively. After PV ligature, PV flow decreased from 356 ± 50 to 298 ± 61 ml/min (p = 0.02), and hepatic arterial flow increased from 277 ± 36 to 331 ± 65 ml/min (p = n.s.). The successful in vivo comparison of radial 4D Flow-MRI to perivascular ultrasound revealed good agreement of abdominal blood flow although with considerable spread of results. A model of pre-hepatic PHTN was successfully introduced and acute responses monitored. • Radial 4D Flow-MRI in the abdomen was successfully compared to perivascular ultrasound. • Inter- and intra-reader testing demonstrated excellent reproducibility of upper abdominal 4D Flow-MRI. • A porcine model of acute pre-hepatic portal hypertension was successfully introduced. • 4D Flow-MRI successfully monitored acute changes in a model of portal hypertension.

Transjugular portal vein recanalization with creation of intrahepatic portosystemic shunt (PVR-TIPS) in patients with chronic non-cirrhotic, non-malignant portal vein thrombosis.

PubMed

Klinger, Christoph; Riecken, Bettina; Schmidt, Arthur; De Gottardi, Andrea; Meier, Benjamin; Bosch, Jaime; Caca, Karel

2018-03-01

To determine safety and efficacy of transjugular portal vein recanalization with creation of intrahepatic portosystemic shunt (PVR-TIPS) in patients with chronic non-cirrhotic, non-malignant portal vein thrombosis (PVT). This retrospective study includes 17 consecutive patients with chronic non-cirrhotic PVT (cavernous transformation n = 15). PVR-TIPS was indicated because of variceal bleeding (n = 13), refractory ascites (n = 2), portal biliopathy with recurrent cholangitis (n = 1), or abdominal pain (n = 1). Treatment consisted of a combination of transjugular balloon angioplasty, mechanical thrombectomy, and-depending on extent of residual thrombosis-transjugular intrahepatic portosystemic shunt and additional stenting of the portal venous system. Recanalization was successful in 76.5 % of patients despite cavernous transformation in 88.2 %. Both 1- and 2-year secondary PV and TIPS patency rates were 69.5 %. Procedure-related bleeding complications occurred in 2 patients (intraperitoneal bleeding due to capsule perforation, n = 1; liver hematoma, n = 1) and resolved spontaneously. However, 1 patient died due to subsequent nosocomial pneumonia. During follow-up, 3 patients with TIPS occlusion and PVT recurrence experienced portal hypertensive complications. PVR-TIPS is safe and effective in selected patients with chronic non-cirrhotic PVT. Due to technical complexity and possible complications, it should be performed only in specialized centers with high experience in TIPS procedures. © Georg Thieme Verlag KG Stuttgart · New York.

Schistosome-induced cholangiocyte proliferation and osteopontin secretion correlate with fibrosis and portal hypertension in human and murine schistosomiasis mansoni.

PubMed

Pereira, Thiago A; Syn, Wing-Kin; Machado, Mariana V; Vidigal, Paula V; Resende, Vivian; Voieta, Izabela; Xie, Guanhua; Otoni, Alba; Souza, Márcia M; Santos, Elisângela T; Chan, Isaac S; Trindade, Guilherme V M; Choi, Steve S; Witek, Rafal P; Pereira, Fausto E; Secor, William E; Andrade, Zilton A; Lambertucci, José Roberto; Diehl, Anna Mae

2015-11-01

Schistosomiasis is a major cause of portal hypertension worldwide. It associates with portal fibrosis that develops during chronic infection. The mechanisms by which the pathogen evokes these host responses remain unclear. We evaluated the hypothesis that schistosome eggs release factors that directly stimulate liver cells to produce osteopontin (OPN), a pro-fibrogenic protein that stimulates hepatic stellate cells to become myofibroblasts. We also investigated the utility of OPN as a biomarker of fibrosis and/or severity of portal hypertension. Cultured cholangiocytes, Kupffer cells and hepatic stellate cells were treated with soluble egg antigen (SEA); OPN production was quantified by quantitative reverse transcriptase polymerase chain reaction (qRTPCR) and ELISA; cell proliferation was assessed by BrdU (5-bromo-2'-deoxyuridine). Mice were infected with Schistosoma mansoni for 6 or 16 weeks to cause early or advanced fibrosis. Liver OPN was evaluated by qRTPCR and immunohistochemistry (IHC) and correlated with liver fibrosis and serum OPN. Livers from patients with schistosomiasis mansoni (early fibrosis n=15; advanced fibrosis n=72) or healthy adults (n=22) were immunostained for OPN and fibrosis markers. Results were correlated with plasma OPN levels and splenic vein pressures. SEA-induced cholangiocyte proliferation and OPN secretion (P<0.001 compared with controls). Cholangiocytes were OPN (+) in Schistosoma-infected mice and humans. Liver and serum OPN levels correlated with fibrosis stage (mice: r=0.861; human r=0.672, P=0.0001) and myofibroblast accumulation (mice: r=0.800; human: r=0.761, P=0.0001). Numbers of OPN (+) bile ductules strongly correlated with splenic vein pressure (r=0.778; P=0.001). S. mansoni egg antigens stimulate cholangiocyte proliferation and OPN secretion. OPN levels in liver and blood correlate with fibrosis stage and portal hypertension severity. © 2015 Authors; published by Portland Press Limited.

Circulating Elastin Fragments Are Not Affected by Hepatic, Renal and Hemodynamic Changes, But Reflect Survival in Cirrhosis with TIPS.

PubMed

Nielsen, M J; Lehmann, J; Leeming, D J; Schierwagen, R; Klein, S; Jansen, C; Strassburg, C P; Bendtsen, F; Møller, S; Sauerbruch, T; Karsdal, M A; Krag, A; Trebicka, J

2015-11-01

Progressive fibrosis increases hepatic resistance and causes portal hypertension with complications. During progressive fibrosis remodeling and deposition of collagens and elastin occur. Elastin remodeling is crucially involved in fibrosis progression in animal models and human data. This study investigated the association of circulating elastin with the clinical outcome in cirrhotic patients with severe portal hypertension receiving transjugular intrahepatic porto-systemic shunt (TIPS). We analyzed portal and hepatic venous samples of 110 cirrhotic patients obtained at TIPS insertion and 2 weeks later. The circulating levels of elastin fragments (ELM) were determined using specific monoclonal ELISA. The relationship of ELM with clinical short-time follow-up and long-term outcome was investigated. Circulating levels of ELM showed a gradient across the liver before TIPS with higher levels in the hepatic vein. Interestingly, the circulating ELM levels remained unchanged after TIPS. The circulating levels of ELM in portal and hepatic veins correlated with platelet counts and inversely with serum sodium. Hepatic venous levels of ELM were higher in CHILD C compared to CHILD A and B and were associated with the presence of ascites. Patients with high levels of ELM in the hepatic veins before TIPS showed poorer survival. In multivariate analysis ELM levels in the hepatic veins and MELD were independent predictors of mortality in these patients. This study demonstrated that circulating levels of ELM are not associated with hemodynamic changes, but might reflect fibrosis remodeling and predict survival in patients with severe portal hypertension receiving TIPS independently of MELD.

Intestinal decontamination inhibits TLR4 dependent fibronectin mediated crosstalk between stellate cells and endothelial cells in liver fibrosis in mice

PubMed Central

Zhu, Qiang; Zou, Li; Jagavelu, Kumaravelu; Simonetto, Douglas A.; Huebert, Robert C.; Jiang, Zhi-Dong; DuPont, Herbert L.; Shah, Vijay H.

2012-01-01

Background/Aims Liver fibrosis is associated with angiogenesis and leads to portal hypertension. Certain antibiotics reduce complications of liver failure in humans, however, effect of antibiotics on the pathologic alterations of the disease are not fully understood. The aim of this study was to test whether the non-absorbable antibiotic rifaximin could attenuate fibrosis progression and portal hypertension in vivo, and explore potential mechanisms in vitro. Methods Effect of rifaximin on portal pressure, fibrosis, and angiogenesis was examined in wild type and toll like receptor 4 (TLR4) mutant mice after bile duct ligation (BDL). In vitro studies were carried out to evaluate the effect of the bacterial product and TLR agonist, lipopolysaccharide (LPS) on paracrine interactions between hepatic stellate cells (HSC) and liver endothelial cells (LEC) that lead to fibrosis and portal hypertension. Results Portal pressure, fibrosis, and angiogenesis were significantly lower in BDL mice receiving rifaximin compared to BDL mice receiving vehicle. Studies in TLR4 mutant mice confirmed that the effect of rifaximin was dependent on LPS/TLR4 pathway. Fibronectin (FN) was increased in BDL liver and was reduced by rifaximin administration and thus was explored further in vitro as a potential mediator of paracrine interactions of HSC and LEC. In vitro, LPS promoted FN production from HSC. Furthermore, HSC-derived FN promoted LEC migration and angiogenesis. Conclusion These studies expand our understanding of the relationship of intestinal microbiota with fibrosis development by identifying FN as a TLR4 dependent mediator of the matrix and vascular changes that characterize cirrhosis. PMID:22173161

Liver cirrhosis and portal hypertension in cystic fibrosis.

PubMed

Fustik, Stojka

2013-01-01

As the expected survival improves in individuals with the cystic fibrosis (CF), so they may be faced with a number of medical complications. The aim of this study was to analyze the prevalence of liver cirrhosis in our CF population as well as the clinical and genetic characteristics of these patients. All patients older than 2 years (n = 96) were screened for liver disease. Liver cirrhosis was defined by ultrasonographic findings of distinct heterogeneity of liver parenchyma and nodular liver surface and/or by liver biopsy findings. Enlarged spleen, distended portal vein and abnormal portal venous flow indicated portal hypertension. Clinical and genotype data were analyzed. Sixteen patients were found to have liver cirrhosis, three of them with portal hypertension. All patients had pancreatic insufficiency. Nutritional status expressed as standard deviation score (Z score) for weight, height, and body mass index was as follows: zW = -0.40 +/- 1.24, zH = -0.83 +/- 1.02, and BMI = 20.1 +/- 2.3. CF patients with liver cirrhosis generally had mild-to-moderate lung disease, with average FVC and FEV1 values of 97.1 +/- 16.5% of predicted and 87.9 +/- 23.5% of predicted, respectively. Genetic analysis showed high frequency of F508del mutation in the group with cirrhosis (90.6%). The prevalence of liver cirrhosis in our CF population older than 2 years was 16.6%. Patients with pancreatic insufficiency and severe CFTR mutations, especially F508del, were exposed to higher risk of developing liver cirrhosis. Liver cirrhosis has no significant impact on the pulmonary function and the nutritional status, until the end-stage liver disease.

Staged Transcatheter Treatment of Portal Hypoplasia and Congenital Portosystemic Shunts in Children

DOE Office of Scientific and Technical Information (OSTI.GOV)

Bruckheimer, Elchanan, E-mail: elchananb@bezeqint.net; Dagan, Tamir; Atar, Eli

2013-12-15

Purpose: Congenital portosystemic shunts (CPSS) with portal venous hypoplasia cause hyperammonemia. Acute shunt closure results in portal hypertension. A transcatheter method of staged shunt reduction to afford growth of portal vessels followed by shunt closure is reported. Methods: Pressure measurements and angiography in the CPSS or superior mesenteric artery (SMA) during temporary occlusion of the shunt were performed. If vessels were diminutive and the pressure was above 18 mmHg, a staged approach was performed, which included implantation of a tailored reducing stent to reduce shunt diameter by {approx}50 %. Recatheterization was performed approximately 3 months later. If the portal pressuremore » was below 18 mmHg and vessels had developed, the shunt was closed with a device. Results: Six patients (5 boys, 1 girl) with a median age of 3.3 (range 0.5-13) years had CPSS portal venous hypoplasia and hyperammonemia. Five patients underwent staged closure. One patient tolerated acute closure. One patient required surgical shunt banding because a reducing stent could not be positioned. At median follow-up of 3.8 (range 2.2-8.4) years, a total of 21 procedures (20 transcatheter, 1 surgical) were performed. In all patients, the shunt was closed with a significant reduction in portal pressure (27.7 {+-} 11.3 to 10.8 {+-} 1.8 mmHg; p = 0.016), significant growth of the portal vessels (0.8 {+-} 0.5 to 4.0 {+-} 2.4 mm; p = 0.037), and normalization of ammonia levels (202.1 {+-} 53.6 to 65.7 {+-} 9.6 {mu}mol/L; p = 0.002) with no complications. Conclusion: Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia.« less

Staged transcatheter treatment of portal hypoplasia and congenital portosystemic shunts in children.

PubMed

Bruckheimer, Elchanan; Dagan, Tamir; Atar, Eli; Schwartz, Michael; Kachko, Ludmila; Superina, Riccardo; Amir, Gabriel; Shapiro, Rivka; Birk, Einat

2013-12-01

Congenital portosystemic shunts (CPSS) with portal venous hypoplasia cause hyperammonemia. Acute shunt closure results in portal hypertension. A transcatheter method of staged shunt reduction to afford growth of portal vessels followed by shunt closure is reported. Pressure measurements and angiography in the CPSS or superior mesenteric artery (SMA) during temporary occlusion of the shunt were performed. If vessels were diminutive and the pressure was above 18 mmHg, a staged approach was performed, which included implantation of a tailored reducing stent to reduce shunt diameter by ~50 %. Recatheterization was performed approximately 3 months later. If the portal pressure was below 18 mmHg and vessels had developed, the shunt was closed with a device. Six patients (5 boys, 1 girl) with a median age of 3.3 (range 0.5-13) years had CPSS portal venous hypoplasia and hyperammonemia. Five patients underwent staged closure. One patient tolerated acute closure. One patient required surgical shunt banding because a reducing stent could not be positioned. At median follow-up of 3.8 (range 2.2-8.4) years, a total of 21 procedures (20 transcatheter, 1 surgical) were performed. In all patients, the shunt was closed with a significant reduction in portal pressure (27.7 ± 11.3 to 10.8 ± 1.8 mmHg; p = 0.016), significant growth of the portal vessels (0.8 ± 0.5 to 4.0 ± 2.4 mm; p = 0.037), and normalization of ammonia levels (202.1 ± 53.6 to 65.7 ± 9.6 μmol/L; p = 0.002) with no complications. Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia.

TMEM16A regulates portal vein smooth muscle cell proliferation in portal hypertension.

PubMed

Zeng, Xi; Huang, Ping; Chen, Mingkai; Liu, Shiqian; Wu, Nannan; Wang, Fang; Zhang, Jing

2018-01-01

The aim of the present study was to elucidate the effect of transmembrane protein 16A (TMEM16A) on portal vein smooth muscle cell (PVSMC) proliferation associated with portal vein remodeling in portal hypertension (PHT). Sprague-Dawley rats were subjected to bile duct ligation to establish a rat model of liver cirrhosis and PHT. Sham-operated animals served as controls. At 8 weeks after bile duct ligation, the extent of liver fibrosis and the portal vein wall thickness were assessed using hematoxylin-eosin staining. The protein expression levels of TMEM16A, extracellular signal-regulated kinase 1 and 2 (ERK1/2) and phosphorylated ERK1/2 (p-ERK1/2) in the portal vein were detected by immunohistochemistry and western blotting. In vitro , the lentivirus vectors were constructed and transfected into PVSMCs to upregulate the expression of TMEM16A. Isolated rat primary PVSMCs were treated with a small molecule inhibitor of TMEM16A, T16A-inhA01. Cell cycle was detected by flow cytometry. The activity of TMEM16A in the portal vein isolated from bile duct ligated rats was decreased, while the expression level of p-ERK1/2 was increased. However, in vitro , upregulation of TMEM16A promoted the proliferation PVSMCs, while inhibition of TMEM16A channels inhibited the proliferation of PVSMCs. The results indicated that TMEM16A contributes to PVSMCs proliferation in vitro , but in vivo , it may be a negative regulator of cell proliferation influenced by numerous factors.

Current management of long-term survivors of biliary atresia: over 40 years of experience in a single center and review of the literature.

PubMed

Sasaki, Hideyuki; Tanaka, Hiromu; Nio, Masaki

2017-12-01

Owing to several therapeutic advancements, more patients with biliary atresia now survive into adulthood while retaining their native liver. However, the optimal strategy for long-term management of such patients remains unclear. Aiming to establish the current management strategies, we reviewed previous reports of long-term outcome of BA who underwent surgery at our institution as well as the relevant literature, focusing particularly on the treatment of late complications. Approximately 30-40% of long-term survivors of biliary atresia who retain their native liver exhibit late sequelae such as cholangitis and portal hypertension. Early and appropriate intervention with Kasai portoenterostomy is essential for ensuring long-term survival with good quality of life. In our hospital, the current standard for Kasai portoenterostomy involves dissecting the fibrous remnants along the porta hepatis, just on the level of the liver capsule. Cholangitis is an important late complication in biliary atresia, and the possibility of mechanical obstruction of the biliary drainage route or deformity of the intrahepatic bile ducts with or without gallstones should be thoroughly evaluated in patients with intractable cholangitis. Regarding portal hypertension, appropriate interventions such as endoscopic variceal treatment and partial splenic embolization are considered to provide good quality of life when hepatic function is preserved. Appropriate therapeutic management is strongly recommended in selected patients with late complications.

Comparison of Technical and Clinical Outcome of Transjugular Portosystemic Shunt Placement Between a Bare Metal Stent and a PTFE-Stentgraft Device.

PubMed

Lauermann, J; Potthoff, A; Mc Cavert, M; Marquardt, S; Vaske, B; Rosenthal, H; von Hahn, T; Wacker, F; Meyer, B C; Rodt, Thomas

2016-04-01

To analyse technical and clinical success of transjugular intrahepatic portosystemic shunt (TIPS) in patients with portal hypertension and compare a stent and a stentgraft with regard to clinical and technical outcome and associated costs. 170 patients (56 ± 12 years, 32.9% females) treated with TIPS due to portal hypertension were reviewed. 80 patients received a stent (group 1) and 83 a stentgraft (group 2), and seven interventions were unsuccessful. Technical data, periprocedural imaging, follow-up ultrasound and clinical data were analysed with focus on technical success, patency, clinical outcome and group differences. Cost analysis was performed. Portal hypertension was mainly caused by ethyltoxic liver cirrhosis with ascites as dominant symptom (80%). Technical success was 93.5% with mean portosystemic gradient decrease from 16.1 ± 4.8 to 5.1 ± 2.1 mmHg. No significant differences in technical success and portosystemic gradient decrease between the groups were observed. Kaplan-Meier analysis yielded significant differences in primary patency after 14 days, 6 months and 2 years in favour of the stentgraft. Both groups showed good clinical results without significant difference in 1-year survival and hepatic encephalopathy rate. Costs to establish TIPS and to manage 2-year follow-up with constant patency and clinical success were 8876 € (group 1) and 9394 € (group 2). TIPS is a safe and effective procedure to manage portal hypertension. Stent and stentgraft enabled good technical and clinical results with a low complication rate. Primary patency rates are clearly in favour of the stentgraft, whereas the stent was more cost effective with similar clinical results in both groups.

Congenital veno-venous malformations of the liver: widely variable clinical presentations.

PubMed

Witters, Peter; Maleux, Geert; George, Christophe; Delcroix, Marion; Hoffman, Ilse; Gewillig, Marc; Verslype, Chris; Monbaliu, Diethard; Aerts, Raymond; Pirenne, Jacques; Van Steenbergen, Werner; Nevens, Frederik; Fevery, Johan; Cassiman, David

2008-08-01

Congenital portosystemic veno-venous malformations are rare abnomalities that often remain undiagnosed. Typically they are classified by their anatomical characteristics according to Morgan (extrahepatic, Abernethy malformations type Ia,b and II) and Park (intrahepatic, types 1-4). However, their clinical presentation is less dependent on the anatomical type. We reviewed the clinical characteristics of six cases drawn from our files (from 1970 to 2006). One patient, a 25-year-old male, had extrahepatic shunting whereby the liver receives only arterial blood because the portal vein (PV) connects with the inferior caval vein (ICV) (Abernethy Ib); he presented with episodes of jaundice and pruritus. Three patients had extrahepatic shunting with patent intrahepatic portal veins, but with shunting of splenomesenterial blood towards the ICV (Abernethy II); these included a 66-year-old male with hepatic encephalopathy, a 17-year-old female with (porto?-)pulmonary hypertension without portal hypertension, and a 33-year-old female with epidsodes of acute pain secondary to spontaneous bleeding within a primary liver tumor. Two patients had intrahepatic shunting; these included an 8-year-old boy who was diagnosed incidentally during work-up for abnormal liver enzymes with a communication between right PV and ICV (Park type 1), and a 59-year-old male with multiple PV-ICV-shunts in several liver segments (Park, type 4) who presented with hepatic encephalopathy. Patients often present with signs of hepatic shunting (encephalopathy, pulmonary hypertension, hepatopulmonary syndrome, and/or hypoglycemia) with relative sparing of the synthetic liver function in the absence of portal hypertension. Some shunts present with space-occupying lesions (focal nodular hyperplasia, hepatocellular carcinoma, nodular regenerative hyperplasia, etc.) or biliary atresia. Finally, some cases are detected incidentally.

Hepatic sarcoidosis in patients presenting with liver dysfunction: imaging appearance, pathological correlation and disease evolution.

PubMed

Fetzer, David T; Rees, Mitchell A; Dasyam, Anil K; Tublin, Mitchell E

2016-09-01

We hypothesize that hepatic sarcoidosis is a dynamic process that can lead to cirrhosis and portal hypertension, independent of the course of thoracic disease. Therefore, we assess the imaging appearance and progression of hepatic sarcoidosis in subjects presenting with hepatic dysfunction. An IRB-approved, HIPAA-compliant, single-institution retrospective review identified 39 subjects with sarcoidosis-related liver dysfunction. Clinical information was collected. Two abdominal radiologists analyzed baseline and follow-up imaging studies, scoring features of cirrhosis. Chest CT was also analyzed. At presentation, 23 subjects (59.0 %) exhibited >3 cirrhotic features and 15 (38.5 %) >2 findings of portal hypertension. Of subjects with available follow-up, 57.9 % (19 subjects; mean interval 4.7 years) showed worsening of >3 cirrhotic features (Pearson rho = 0.58; p = 0.009). Parenchymal nodules were uncommon (25.6 %), and most regressed. Although 87.2 % of subjects were diagnosed with thoracic sarcoidosis, there was poor correlation between severity of hepatic and chest disease (Pearson rho = 0.30; p = 0.119). A mean of 7.2 years elapsed between diagnosis of pulmonary and liver involvement. Sarcoidosis may present as liver dysfunction, cirrhosis or portal hypertension. Sarcoid-related liver disease may progress and can manifest without, alongside or significantly after a diagnosis of pulmonary disease. • Patients often present with elevated liver function tests indicating cholestasis. • Patients may present with portal hypertension, and some progress to cirrhosis. • Though biopsy can be considered for focal liver lesions, most will regress. • Extent of intra-abdominal involvement may not correlate with severity of thoracic disease. • Liver disease may manifest alongside, prior to or significantly after initial diagnosis.

Comparison of Technical and Clinical Outcome of Transjugular Portosystemic Shunt Placement Between a Bare Metal Stent and a PTFE-Stentgraft Device

DOE Office of Scientific and Technical Information (OSTI.GOV)

Lauermann, J., E-mail: jostlauermann@gmail.com; Potthoff, A.; Mc Cavert, M.

PurposeTo analyse technical and clinical success of transjugular intrahepatic portosystemic shunt (TIPS) in patients with portal hypertension and compare a stent and a stentgraft with regard to clinical and technical outcome and associated costs.Materials and Methods170 patients (56 ± 12 years, 32.9 % females) treated with TIPS due to portal hypertension were reviewed. 80 patients received a stent (group 1) and 83 a stentgraft (group 2), and seven interventions were unsuccessful. Technical data, periprocedural imaging, follow-up ultrasound and clinical data were analysed with focus on technical success, patency, clinical outcome and group differences. Cost analysis was performed.ResultsPortal hypertension was mainly caused by ethyltoxicmore » liver cirrhosis with ascites as dominant symptom (80 %). Technical success was 93.5 % with mean portosystemic gradient decrease from 16.1 ± 4.8 to 5.1 ± 2.1 mmHg. No significant differences in technical success and portosystemic gradient decrease between the groups were observed. Kaplan–Meier analysis yielded significant differences in primary patency after 14 days, 6 months and 2 years in favour of the stentgraft. Both groups showed good clinical results without significant difference in 1-year survival and hepatic encephalopathy rate. Costs to establish TIPS and to manage 2-year follow-up with constant patency and clinical success were 8876 € (group 1) and 9394 € (group 2).ConclusionTIPS is a safe and effective procedure to manage portal hypertension. Stent and stentgraft enabled good technical and clinical results with a low complication rate. Primary patency rates are clearly in favour of the stentgraft, whereas the stent was more cost effective with similar clinical results in both groups.« less

Factors affecting mortality and resource use for hospitalized patients with cirrhosis

PubMed Central

Charatcharoenwitthaya, Phunchai; Soonthornworasiri, Ngamphol; Karaketklang, Khemajira; Poovorawan, Kittiyod; Pan-ngum, Wirichada; Chotiyaputta, Watcharasak; Tanwandee, Tawesak; Phaosawasdi, Kamthorn

2017-01-01

Abstract Hospitalizations for advanced liver disease are costly and associated with significant mortality. This population-based study aimed to evaluate factors associated with in-hospital mortality and resource use for the management of hospitalized patients with cirrhosis. Mortality records and resource utilization for 52,027 patients hospitalized with cirrhosis and/or complications of portal hypertension (ascites, hepatic encephalopathy, variceal bleeding, spontaneous bacterial peritonitis, or hepatorenal syndrome) were extracted from a nationally representative sample of Thai inpatients covered by Universal Coverage Scheme during 2009 to 2013. The rate of dying in the hospital increased steadily by 12% from 9.6% in 2009 to 10.8% in 2013 (P < .001). Complications of portal hypertension were independently associated with increased in-hospital mortality except for ascites. The highest independent risk for hospital death was seen with hepatorenal syndrome (odds ratio [OR], 5.04; 95% confidence interval [CI], 4.38–5.79). Mortality rate remained high in patients with infection, particularly septicemia (OR, 4.26; 95% CI, 4.0–4.54) and pneumonia (OR, 2.44; 95% CI, 2.18–2.73). Receiving upper endoscopy (OR, 0.29; 95% CI, 0.27–0.32) and paracentesis (OR, 0.93; 95% CI, 0.87–1.00) were associated with improved patient survival. The inflation-adjusted national annual costs (P = .06) and total hospital days (P = .07) for cirrhosis showed a trend toward increasing during the 5-year period. Renal dysfunction, infection, and sequelae of portal hypertension except for ascites were independently associated with increased resource utilization. Renal dysfunction, infection, and portal hypertension-related complications are the main factors affecting in-hospital mortality and resource utilization for hospitalized patients with cirrhosis. The early intervention for modifiable factors is an important step toward improving hospital outcomes. PMID:28796076

Dose effect of patient-care team communication via secure portal messaging on glucose and blood pressure control.

PubMed

Price-Haywood, Eboni G; Luo, Qingyang; Monlezun, Dominique

2018-02-09

Organizational strategies for implementing eHealth tools influence patient and provider use of portal technology. This study examines whether the intensity of bidirectional secure portal messaging is associated with improved clinical outcomes. This is a retrospective cohort analysis of 101 019 patients with diabetes or hypertension (11 138 active portal users) who received primary care within the Ochsner Health System between 2012 and 2014. Propensity score-adjusted multivariable fixed effects regression panel analysis was used to examine associations between intensity of "medical advice" portal messaging and glucose/blood pressure control. Most portal users rarely used medical advice messaging. A higher proportion of patients who were age 50 years and older, female, white non-Hispanic, and with co-morbid diabetes and hypertension had higher frequency and intensity of medical advice messaging. Study findings revealed a dose-response effect of the intensity of messaging on glucose control, whereby, compared to nonportal users, each level of messaging among portal users was associated with greater decreases in HbA1c (β estimate [95% CI]: none -0.28 (-0.34 to -0.22); low -0.28 (-0.32 to -0.24); medium -0.41 (-0.52 to -0.31); high -0.43 (-0.60 to -0.27), all P ≤ .001). There was no observed effect on blood pressure. The digital divide exists not only between portal users and nonusers but also among portal users. Research exploring the relationship between intensity of bidirectional secure messaging and health outcomes for a broader scope of chronic conditions is needed. Future implementation research must also elucidate best practices that enhance not only the use of portals by patients and providers, but how they use portals. © The Author(s) 2018. Published by Oxford University Press on behalf of the American Medical Informatics Association. All rights reserved. For Permissions, please email: journals.permissions@oup.com

Transjugular intrahepatic portosystemic shunt creation for cirrhotic portal hypertension is well tolerated among patients with portal vein thrombosis.

PubMed

Merola, Jonathan; Fortune, Brett E; Deng, Yanhong; Ciarleglio, Maria; Amirbekian, Smbat; Chaudhary, Noami; Shanbhogue, Alampady; Ayyagari, Rajasekhara; Rodriguez-Davalos, Manuel I; Teperman, Lewis; Charles, Hearns W; Sigal, Samuel H

2018-06-01

Portal vein thrombosis (PVT) develops in cirrhotic patients because of stagnation of blood flow. Transjugular intrahepatic portosystemic shunt (TIPS) creates a low-resistance conduit that restores portal venous patency and blood flow. The effect of PVT on transplant-free survival in cirrhotic patients undergoing TIPS creation was evaluated. A multicenter, retrospective cohort study of patients who underwent TIPS creation for cirrhotic portal hypertension was carried out. A Cox model with propensity score adjustment was developed to evaluate the effect of PVT on 90-day and 3-year transplant-free survival. A subgroup analysis examining mortality of those with superior and inferior PVT was also carried out. A total of 252 consecutive TIPS creations were assessed, including 65 in patients with PVT. Survival of patients with high Model for End-stage Liver Disease scores (≥18) and PVT was not statistically different compared with patients with low Model for End-stage Liver Disease scores (<18) and no PVT at 90 days (P=0.46) and 3 years (P=0.42). Those with superior PVT had improved 90-day and 3-year survival both compared with patients with a inferior PVT and those without a PVT (P<0.01, all cases). The presence of PVT does not impair the prognosis of patients following TIPS creation, particularly in patients with superior portal occlusion.

Challenges and Management of Liver Cirrhosis: Practical Issues in the Therapy of Patients with Cirrhosis due to NAFLD and NASH.

PubMed

Traussnigg, Stefan; Kienbacher, Christian; Halilbasic, Emina; Rechling, Christian; Kazemi-Shirazi, Lili; Hofer, Harald; Munda, Petra; Trauner, Michael

2015-01-01

Nonalcoholic fatty liver disease (NAFLD) is the hepatic manifestation of the metabolic syndrome and comprises a liver disease spectrum ranging from steatosis to nonalcoholic steatohepatitis (NASH) with risk of progression to liver cirrhosis and hepatocellular carcinoma (HCC). Associated metabolic conditions and comorbidities such as obesity, diabetes and cardiovascular diseases are common and require concerted management. Adiponutrin (PNPLA3) variants may help to identify NAFLD patients at higher risk for liver disease progression towards advanced fibrosis and HCC. The therapeutic options in NAFLD/NASH include lifestyle modification, pharmacological treatment, bariatric surgery for patients with morbid obesity and treatment of complications of liver cirrhosis and HCC, including liver transplantation. Insulin sensitizers and antioxidative treatment strategies with vitamin E are among the best-established pharmacological approaches, but both drugs have long-term safety issues and there is limited evidence in cirrhotic patients. Treatment of concomitant/underlying metabolic conditions with statins or metformin may also have beneficial effects on portal hypertension, complications of liver cirrhosis and HCC prevention. The bile acid receptor FXR may be a promising novel therapeutic target for the treatment of NAFLD/NASH, fibrosis and portal hypertension, but the prognostic implications of associated changes in low- and high-density lipoprotein cholesterol require further studies. Morbidly obese NASH patients can benefit from bariatric surgery which may reduce liver fibrosis but carries a risk of decompensation in patients with advanced liver cirrhosis. When carefully selected, patients with NASH cirrhosis undergoing liver transplantation have a good outcome. This review summarizes recent progress in the management of patients with liver cirrhosis due to NASH. © 2015 S. Karger AG, Basel.

Multiple esophageal variceal ruptures with massive ascites due to myelofibrosis-induced portal hypertension

PubMed Central

Tokai, Koichi; Miyatani, Hiroyuki; Yoshida, Yukio; Yamada, Shigeki

2012-01-01

A 75-year old man had been diagnosed at 42 years of age as having polycythemia vera and had been monitored at another hospital. Progression of anemia had been recognized at about age 70, and the patient was thus referred to our center in 2008 where secondary myelofibrosis was diagnosed based on bone marrow biopsy findings. Hematemesis due to rupture of esophageal varices occurred in January and February of 2011. The bleeding was stopped by endoscopic variceal ligation. Furthermore, in March of the same year, hematemesis recurred and the patient was transported to our center. He was in irreversible hemorrhagic shock and died. The autopsy showed severe bone marrow fibrosis with mainly argyrophilic fibers, an observation consistent with myelofibrosis. The liver weighed 1856 g the spleen 1572 g, indicating marked hepatosplenomegaly. The liver and spleen both showed extramedullary hemopoiesis. Myelofibrosis is often complicated by portal hypertension and is occasionally associated with gastrointestinal hemorrhage due to esophageal varices. A patient diagnosed as having myelofibrosis needs to be screened for esophageal/gastric varices. Myelofibrosis has a poor prognosis. Therefore, it is necessary to carefully decide the therapeutic strategy in consideration of the patient’s concomitant conditions, treatment invasiveness and quality of life. PMID:22851873

Unexplained dyspnea in a patient of chronic arsenicosis: A diagnostic challenge and learning curve for physicians

PubMed Central

Sengupta, Amitabha; Maji, Arnab; Jash, Debraj; Maikap, Malay

2015-01-01

Chronic arsenic exposure causes cutaneous effects like hyperkeratosis, peripheral vascular disease, hypertension, ischemic heart disease, non-cirrhotic portal hypertension, hepatomegaly, peripheral neuropathy, respiratory involvement, bad obstetrical outcome, hematological disturbances, and diabetes mellitus. Here we present a case of a 24-year-old lady, with chronic exposure to arsenic, presenting to us with progressive dyspnea. We found pulmonary arterial hypertension (PAH) as a cause of her dyspnea. PAH can occur in arsenicosis, secondary to arsenic-induced chronic obstructive pulmonary disease (COPD), lung fibrosis, and portal hypertension, which we excluded by appropriate investigations in our case. We also excluded a familial or heritable form of PAH. Thus, with the exclusion of all these secondary causes of PAH, as well as a hereditary cause, we came to a conclusion that this PAH might be due to chronic arsenic exposure. To the best of our knowledge, no case of PAH in chronic arsenicosis has been reported to date. PMID:25814805

Diagnosis and Management of Budd Chiari Syndrome: An Update

DOE Office of Scientific and Technical Information (OSTI.GOV)

Copelan, Alexander, E-mail: alexander.copelan@beaumont.edu; Remer, Erick M., E-mail: remere1@ccf.org; Sands, Mark, E-mail: sandsm@ccf.org

Imaging plays a crucial role in the early detection and assessment of the extent of disease in Budd Chiari syndrome (BCS). Early diagnosis and intervention to mitigate hepatic congestion is vital to restoring hepatic function and alleviating portal hypertension. Interventional radiology serves a key role in the management of these patients. The interventionist should be knowledgeable of the clinical presentation as well as key imaging findings, which often dictate the approach to treatment. This article concisely reviews the etiology, pathophysiology, and clinical presentation of BCS and provides a detailed description of imaging and treatment options, particularly interventional management.

Attenuated portal hypertension in germ-free mice: Function of bacterial flora on the development of mesenteric lymphatic and blood vessels.

PubMed

Moghadamrad, Sheida; McCoy, Kathy D; Geuking, Markus B; Sägesser, Hans; Kirundi, Jorum; Macpherson, Andrew J; De Gottardi, Andrea

2015-05-01

Intestinal bacterial flora may induce splanchnic hemodynamic and histological alterations that are associated with portal hypertension (PH). We hypothesized that experimental PH would be attenuated in the complete absence of intestinal bacteria. We induced prehepatic PH by partial portal vein ligation (PPVL) in germ-free (GF) or mice colonized with altered Schaedler's flora (ASF). After 2 or 7 days, we performed hemodynamic measurements, including portal pressure (PP) and portosystemic shunts (PSS), and collected tissues for histomorphology, microbiology, and gene expression studies. Mice colonized with intestinal microbiota presented significantly higher PP levels after PPVL, compared to GF, mice. Presence of bacterial flora was also associated with significantly increased PSS and spleen weight. However, there were no hemodynamic differences between sham-operated mice in the presence or absence of intestinal flora. Bacterial translocation to the spleen was demonstrated 2 days, but not 7 days, after PPVL. Intestinal lymphatic and blood vessels were more abundant in colonized and in portal hypertensive mice, as compared to GF and sham-operated mice. Expression of the intestinal antimicrobial peptide, angiogenin-4, was suppressed in GF mice, but increased significantly after PPVL, whereas other angiogenic factors remained unchanged. Moreover, colonization of GF mice with ASF 2 days after PPVL led to a significant increase in intestinal blood vessels, compared to controls. The relative increase in PP after PPVL in ASF and specific pathogen-free mice was not significantly different. In the complete absence of gut microbial flora PP is normal, but experimental PH is significantly attenuated. Intestinal mucosal lymphatic and blood vessels induced by bacterial colonization may contribute to development of PH. © 2015 by the American Association for the Study of Liver Diseases.

Tyrosine kinase inhibition ameliorates the hyperdynamic state and decreases nitric oxide production in cirrhotic rats with portal hypertension and ascites.

PubMed Central

López-Talavera, J C; Levitzki, A; Martínez, M; Gazit, A; Esteban, R; Guardia, J

1997-01-01

Tumor necrosis factor-alpha (TNF) causes vasodilatation and a hyperdynamic state by activating nitric oxide (NO) synthesis. Tyrphostins, specific inhibitors of protein tyrosine kinase (PTK), block the signaling events induced by TNF and NO production. A hyperdynamic circulatory syndrome (HCS) is often observed in portal hypertension (PHT). TNF and NO seem to mediate these hemodynamic changes. The aim of this work was to study the effect of PTK inhibition on the systemic and portal hemodynamics, TNF and NO production, in cirrhotic rats with portal hypertension. Rats with liver cirrhosis induced by chronic inhalation of carbon tetrachloride were used. Animals were treated daily with tyrphostin AG 126 (alpha-cyano-(3-hydroxy-4-nitro) cinnamonitrile) or placebo for 5 d. Mean arterial pressure (MAP), heart rate (HR), and portal pressure (PP) were measured by indwelling catheters. Cardiac output (CI) and stroke volume (SV) were estimated by thermodilution, systemic vascular resistance (SVR) was calculated (MAP/CI), and portal systemic shunting (PSS) was quantitated using radioactive microspheres. Serum and mesenteric lymph node (MLN) TNF levels were measured using an immunoassay kit, and serum NOx was determined photometrically by its oxidation products. The AG 126-treated group showed a statistically significant increase in MAP and SVR, and decreases in CI, SV, MLN TNF, and serum NO oxidation products nitrite and nitrate (NOx) in comparison with the placebo-treated rats. No significant differences were noticed in HR, PP, PSS, or serum TNF. Significant correlations were observed between MAP and NOx, MAP and MLN TNF, PSS and NOx, and serum TNF and serum NOx. The HCS observed in PHT seems to be mediated, at least in part, by TNF and NO by the activation of PTKs and their signaling pathways. PTK activity inhibition ameliorates the hyperdynamic abnormalities that characterize animals with cirrhosis and PHT. PMID:9239414

[Mesenteric-cava shunt's results with autologous jugular vein graft in children with pre-sinusoidal portal hypertension].

PubMed

Leal, N; López Santamaría, M; Gámez, M; Murcia, J; Andolfi, G; Berrocal, T; Frauca, E; Jara, P; Tovar, J

2002-07-01

Presinusoidal portal hypertension (PPH) in children evaluates without functional hepatic damage, and with the time, trends to compensate through the creation of spontaneous portosystemic shunts. Nevertheless, some patients suffer episodes of gastrointestinal bleeding (GIB) that because of its frequency or severity, force to propose the change of surgical treatment. To evaluate the results of the mesocaval shunt (MCS) with autologous jugular vein in children with PPH. Among the 32 children with PPH treated in our Hospital in the last 7 years, 10 had episodes of GIB that forced to perform a surgical shunt. The types of shunt were distal splenorenal in 3 patients and mesocaval in 7. These 7 cases are the material of this study. The origin of the PPH was a cavernomatosis transformation of the portal vein in 6 cases and a congenital hepatic fibrosis in 1. Before the surgery the average number of episodes of GIB was 9 (range 2-15); all the patients needed transfusion of blood products and variceal sclerosis. In 2 cases a tamponade with the Sengtaken balloon was required and 5 patients were treated with somatostatin and propranolol. The Doppler ultrasounds revealed and intense hepatofugal collateral circulation in all the cases. The initial flow through the shunt was adequate in all the patients except one who required a percutaneous balloon dilatation. Only this patient has suffered an episode of GIB. The hyperesplenism signs disappeared or improved in all the seven cases and the collateral circulation was significantly reduced. The pressure in the splenic territory decreased around 50% in the 4 patients that was measured. There were no cases of encephalopasty and only one child with congenital hepatic fibrosis shows signs of mild hepatic disfunction. The medium follow up post-shunt is 32 months (range 8 m-6 years). The MCS prevents the GIB in the PPH not responsive to the conservative treatment; its effectiveness is related with an adequate permeability though the graft and at least in the cases with portal cavernomatosis (the most frequent in children) doesn't produce hepatic dysfunction. Doppler ultrasounds give a very precise information about the post-surgical situation and are an excellent method of follow up.

Portal hypertension and hypersplenism in extrahepatic portal venous obstruction: Are they related?

PubMed

Kilambi, Ragini; Singh, Anand Narayan; Madhusudhan, Kumble Seetharama; Pal, Sujoy; Saxena, Renu; Shalimar; Dash, Nihar Ranjan; Sahni, Peush

2018-06-23

Portal hypertension (PHT) due to extrahepatic portal venous obstruction (EHPVO) is common in developing countries. Hypersplenism is a near-constant feature of EHPVO, but its significance, unlike in cirrhotics, is unknown. We aimed to study the relationship between hypersplenism and the severity of PHT in patients with EHPVO. This prospective study was done at a tertiary care center from January 2014 to August 2015. All patients with EHPVO who underwent a splenectomy and a shunt or devascularization were included. Data regarding clinical profile, preoperative parameters, and intraoperative details were recorded. The correlation was studied between hypersplenism and the intraoperatively measured portal pressures and markers of PHT. Of the 40 patients studied (mean [SD] age 22.4 [8.4] years), hematological hypersplenism was present in 39 (97.5%). The mean (SD) hemoglobin, total leukocyte counts (TLC), and platelet counts were 9.9 (2.4) g/dL, 2971 (1239) cells/mm 3 , and 66,400 (32047) cells/mm 3 , respectively. The mean (SD) sonographic spleen volume (SV), splenic weight, and intraoperative portal pressure were 1084.7 (553.9) cm 3 , 1088.7 (454.7) g, and 35.6 (5.1) mmHg, respectively. The TLC and platelet counts correlated inversely with the portal pressure. Additionally, the platelet counts correlated negatively with eradicated variceal status, SV, and weight; hemoglobin with SV and weight; and TLC with SV. Multivariate analysis showed the platelet counts were an independent predictor of portal pressures and platelet counts ≤ 53,500 cells/mm 3 indicated significantly high portal pressures. The platelet counts showed a significant inverse correlation with portal pressures in patients with EHPVO and may be used as surrogate markers of PHT. A platelet count ≤ 53,500 cells/mm 3 is predictive of significantly high pressures.

Effects of asymmetric dimethylarginine on renal arteries in portal hypertension and cirrhosis

PubMed Central

Segarra, Gloria; Cortina, Belén; Mauricio, María Dolores; Novella, Susana; Lluch, Paloma; Navarrete-Navarro, Javier; Noguera, Inmaculada; Medina, Pascual

2016-01-01

AIM To evaluate the effects of asymmetric dimethylarginine (ADMA) in renal arteries from portal hypertensive and cirrhotic rats. METHODS Rat renal arteries from Sham (n = 15), pre-hepatic portal hypertension (PPVL; n = 15) and bile duct ligation and excision-induced cirrhosis (BDL; n = 15) were precontracted with norepinephrine, and additional contractions were induced with ADMA (10-6-10-3 mol/L), an endogenous inhibitor of nitric oxide (NO) synthase. Concentration-response curves to acetylcholine (1 × 10-9-3 × 10-6 mol/L) were determined in precontracted renal artery segments with norepinephrine in the absence and in the presence of ADMA. Kidneys were collected to determine the protein expression and activity of dimethylarginine dimethylaminohydrolase (DDAH), an enzyme that catabolizes ADMA. RESULTS In renal arteries precontracted with norepinephrine, ADMA caused endothelium-dependent contractions. The pD2 values to ADMA were similar in the Sham and PPVL groups (4.20 ± 0.08 and 4.11 ± 0.09, P > 0.05, respectively), but were lower than those of the BDL group (4.79 ± 0.16, P < 0.05). Acetylcholine-induced endothelium-dependent relaxation that did not differ, in terms of pD2 and maximal relaxation, among the 3 groups studied. Treatment with ADMA (3 × 10-4 mol/L) inhibited acetylcholine-induced relaxation in the 3 groups, but the inhibition was higher (P < 0.05) in the BDL group compared with that for the Sham and PPVL groups. The mRNA and protein expression of DDAH-1 were similar in kidneys from the three groups. Conversely, DDAH-2 expression was increased (P < 0.05) in PPVL and further enhanced (P < 0.05) in the BDL group. However, renal DDAH activity was significantly decreased in the BDL group. CONCLUSION Cirrhosis increased the inhibitory effect of ADMA on basal- and induced-release of NO in renal arteries, and decreased DDAH activity in the kidney. PMID:28082806

Effects of asymmetric dimethylarginine on renal arteries in portal hypertension and cirrhosis.

PubMed

Segarra, Gloria; Cortina, Belén; Mauricio, María Dolores; Novella, Susana; Lluch, Paloma; Navarrete-Navarro, Javier; Noguera, Inmaculada; Medina, Pascual

2016-12-28

To evaluate the effects of asymmetric dimethylarginine (ADMA) in renal arteries from portal hypertensive and cirrhotic rats. Rat renal arteries from Sham ( n = 15), pre-hepatic portal hypertension (PPVL; n = 15) and bile duct ligation and excision-induced cirrhosis (BDL; n = 15) were precontracted with norepinephrine, and additional contractions were induced with ADMA (10 -6 -10 -3 mol/L), an endogenous inhibitor of nitric oxide (NO) synthase. Concentration-response curves to acetylcholine (1 × 10 -9 -3 × 10 -6 mol/L) were determined in precontracted renal artery segments with norepinephrine in the absence and in the presence of ADMA. Kidneys were collected to determine the protein expression and activity of dimethylarginine dimethylaminohydrolase (DDAH), an enzyme that catabolizes ADMA. In renal arteries precontracted with norepinephrine, ADMA caused endothelium-dependent contractions. The pD 2 values to ADMA were similar in the Sham and PPVL groups (4.20 ± 0.08 and 4.11 ± 0.09, P > 0.05, respectively), but were lower than those of the BDL group (4.79 ± 0.16, P < 0.05). Acetylcholine-induced endothelium-dependent relaxation that did not differ, in terms of pD 2 and maximal relaxation, among the 3 groups studied. Treatment with ADMA (3 × 10 -4 mol/L) inhibited acetylcholine-induced relaxation in the 3 groups, but the inhibition was higher ( P < 0.05) in the BDL group compared with that for the Sham and PPVL groups. The mRNA and protein expression of DDAH-1 were similar in kidneys from the three groups. Conversely, DDAH-2 expression was increased ( P < 0.05) in PPVL and further enhanced ( P < 0.05) in the BDL group. However, renal DDAH activity was significantly decreased in the BDL group. Cirrhosis increased the inhibitory effect of ADMA on basal- and induced-release of NO in renal arteries, and decreased DDAH activity in the kidney.

Use of portal pressure studies in the management of variceal haemorrhage

PubMed Central

Addley, Jennifer; Tham, Tony CK; Cash, William Jonathan

2012-01-01

Portal hypertension occurs as a complication of liver cirrhosis and complications such as variceal bleeding lead to significant demands on resources. Endoscopy is the gold standard method for screening cirrhotic patients however universal endoscopic screening may mean a lot of unnecessary procedures as the presence of oesophageal varices is variable hence a large time and cost burden on endoscopy units to carry out both screening and subsequent follow up of variceal bleeds. A less invasive method to identify those at high risk of bleeding would allow earlier prophylactic measures to be applied. Hepatic venous pressure gradient (HVPG) is an acceptable indirect measurement of portal hypertension and predictor of the complications of portal hypertension in adult cirrhotics. Varices develop at a HVPG of 10-12 mmHg with the appearance of other complications with HPVG > 12 mmHg. Variceal bleeding does not occur in pressures under 12 mmHg. HPVG > 20 mmHg measured early after admission is a significant prognostic indicator of failure to control bleeding varices, indeed early transjugular intrahepatic portosystemic shunt (TIPS) in such circumstances reduces mortality significantly. HVPG can be used to identify responders to medical therapy. Patients who do not achieve the suggested reduction targets in HVPG have a high risk of rebleeding despite endoscopic ligation and may not derive significant overall mortality benefit from endoscopic intervention alone, ultimately requiring TIPS or liver transplantation. Early HVPG measurements following a variceal bleed can help to identify those at risk of treatment failure who may benefit from early intervention with TIPS. Therefore, we suggest using HVPG measurement as the investigation of choice in those with confirmed cirrhosis in place of endoscopy for intitial variceal screening and, where indicated, a trial of B-blockade, either intravenously during the initial pressure study with assessment of response or oral therapy with repeat HVPG six weeks later. In those with elevated pressures, primary medical prophylaxis could be commenced with subsequent close monitoring of HVPG thus negating the need for endoscopy at this point. All patients presenting with variceal haemorrhage should undergo HVPG measurement and those with a gradient greater than 20 mmHg should be considered for early TIPS. By introducing portal pressure studies into a management algorithm for variceal bleeding, the number of endoscopies required for further intervention and follow up can be reduced leading to significant savings in terms of cost and demand on resources. PMID:22816007

Abdominal ultrasound

MedlinePlus

... inflammation in pancreas) Spleen enlargement ( splenomegaly ) Portal hypertension Liver tumors Obstruction of bile ... Digestive system Kidney anatomy Ultrasound in pregnancy Kidney - blood and urine flow ...

Prevalence of schistosome antibodies with hepatosplenic signs and symptoms among patients from Kaoma, Western Province, Zambia.

PubMed

Payne, Lara; Turner-Moss, Eleanor; Mutengo, Mable; Asombang, Akwi W; Kelly, Paul

2013-08-30

Schistosomiasis is a major cause of morbidity and mortality, with over 200 million people infected worldwide. Eighty-five percent of cases are in Africa. The hepatosplenic form develops over time by an immune reaction to trapped Schistosoma mansoni eggs in the portal system leading to liver fibrosis, portal hypertension and oesophageal varices. Most patients presenting to the University Teaching Hospital in Lusaka with oesophageal varices, come from Western province, but no formal studies have been carried out in this area assessing the burden of hepatosplenic pathology. We aimed to define the extent of the problem in Kaoma district, western Zambia, and to correlate signs and symptoms with serology. A symptom questionnaire, demographic survey and physical examination was conducted amongst patients presenting to Kaoma district outpatient clinics. To assess the prevalence of Schistosoma mansoni infections, blood was collected and screened for the presence of Schistosoma antibodies using Enzyme linked immunosorbent assay (ELISA). Of the 110 patients screened, 97 (88%) were ELISA positive. Forty-six percent (51/110) reported haematochezia and 7% experienced haematemesis (8/110). On physical examination 27% (30/110) hepatomegaly and 17% (30/110) splenomegaly was observed amongst participants but there were few correlations between serology and signs/symptoms. On questioning 68% (75/110) of participants knew nothing about schistosomiasis transmission. Our serological and clinical data indicate a very heavy burden of schistosomiasis-related portal hypertension. Our evidence highlights a need for mass treatment in Kaoma to address and prevent extensive pathology of hepatosplenic schistosomiasis. Safe water and health education throughout Western Province are clearly also important.

Portopulmonary hypertension: state of the art.

PubMed

Porres-Aguilar, Mateo; Zuckerman, Marc J; Figueroa-Casas, Juan B; Krowka, Michael J

2008-01-01

Portopulmonary hypertension is an uncommon but treatable pulmonary vascular consequence of portal hypertension, which can lead to significant morbidity and mortality. Portopulmonary hypertension results from excessive pulmonary vasoconstriction and vascular remodeling that eventually leads to right-heart failure and death if left untreated. Although pulmonary vascular disease in these patients may be asymptomatic or associated with subtle and nonspecific symptoms (dyspnea, fatigue and lower extremity swelling), it should be looked for especially if patients are potential candidates for liver transplantation. Patients with clinical suspicion of portopulmonary hypertension should undergo screening testing, specifically echocardiography. Right heart catheterization remains the gold standard for the diagnosis. The existence of moderate to severe disease poses higher risks and challenges for liver transplantation. The disease has a substantial impact on survival and requires focused pharmacological therapy. New and evolving medical therapies, such as prostanoids (intravenous, inhaled or oral), endothelin receptors antagonists, phosphodiesterases inhibitors, combination therapy and other experimental drugs might change the natural course of the disease. Case reports and cases series have been published regarding the efficacy and safety of pharmacological therapy, but randomized, controlled multicenter trials are urgently needed. Liver transplantation is not the treatment of choice for portopulmonary hypertension, but after optimal hemodynamic and clinical improvement with medical therapy as a bridge, liver transplant can be considered an option in selected patients.

Droxidopa, an oral norepinephrine precursor, improves hemodynamic and renal alterations of portal hypertensive rats.

PubMed

Coll, Mar; Rodriguez, Sarai; Raurell, Imma; Ezkurdia, Nahia; Brull, Astrid; Augustin, Salvador; Guardia, Jaime; Esteban, Rafael; Martell, María; Genescà, Joan

2012-11-01

We aimed to evaluate the effects of droxidopa (an oral synthetic precursor of norepinephrine) on the hemodynamic and renal alterations of portal hypertensive rats. Sham, portal vein-ligated (PVL), and 4-week biliary duct-ligated (BDL) rats received a single oral dose of droxidopa (25-50 mg/kg) or vehicle and hemodynamic parameters were monitored for 2 hours. Two groups of BDL and cirrhotic rats induced by carbon tetrachloride (CCl(4) ) were treated for 5 days with droxidopa (15 mg/kg, twice daily, orally); hemodynamic parameters and blood and urinary parameters were assessed. The droxidopa effect on the Rho kinase (RhoK) / protein kinase B (AKT) / endothelial nitric oxide synthase (eNOS) pathways was analyzed by western blot in superior mesenteric artery (SMA). The acute administration of droxidopa in PVL and BDL rats caused a significant and maintained increase in arterial pressure and mesenteric arterial resistance, with a significant decrease of mesenteric arterial and portal blood flow, without changing portal pressure and renal blood flow. Two-hour diuresis greatly increased. Carbidopa (DOPA decarboxylase inhibitor) blunted all effects of droxidopa. Chronic droxidopa therapy in BDL rats produced the same beneficial hemodynamic effects observed in the acute study, did not alter liver function parameters, and caused a 50% increase in 24-hour diuresis volume (7.4 ± 0.9 mL/100g in BDL vehicle versus 11.8 ± 2.5 mL/100g in BDL droxidopa; P = 0.01). Droxidopa-treated rats also showed a decreased ratio of p-eNOS/eNOS and p-AKT/AKT and increased activity of RhoK in SMA. The same chronic treatment in CCl(4) rats caused similar hemodynamic effects and produced significant increases in diuresis volume and 24-hour natriuresis (0.08 ± 0.02 mmol/100g in CCl(4) vehicle versus 0.23 ± 0.03 mmol/100g in CCl(4) droxidopa; P = 0.014). Droxidopa might be an effective therapeutic agent for hemodynamic and renal alterations of liver cirrhosis and should be tested in cirrhosis patients. Copyright © 2012 American Association for the Study of Liver Diseases.

Glutamine prevents oxidative stress in a model of portal hypertension.

PubMed

Zabot, Gilmara Pandolfo; Carvalhal, Gustavo Franco; Marroni, Norma Possa; Licks, Francielli; Hartmann, Renata Minuzzo; da Silva, Vinícius Duval; Fillmann, Henrique Sarubbi

2017-07-07

To evaluate the protective effects of glutamine in a model of portal hypertension (PH) induced by partial portal vein ligation (PPVL). Male Wistar rats were housed in a controlled environment and were allowed access to food and water ad libitum . Twenty-four male Wistar rats were divided into four experimental groups: (1) control group (SO) - rats underwent exploratory laparotomy; (2) control + glutamine group (SO + G) - rats were subjected to laparotomy and were treated intraperitoneally with glutamine; (3) portal hypertension group (PPVL) - rats were subjected to PPVL; and (4) PPVL + glutamine group (PPVL + G) - rats were treated intraperitoneally with glutamine for seven days. Local injuries were determined by evaluating intestinal segments for oxidative stress using lipid peroxidation and the activities of glutathione peroxidase (GPx), endothelial nitric oxide synthase (eNOS) and inducible nitric oxide synthase (iNOS) after PPVL. Lipid peroxidation of the membrane was increased in the animals subjected to PH ( P < 0.01). However, the group that received glutamine for seven days after the PPVL procedure showed levels of lipid peroxidation similar to those of the control groups ( P > 0.05). The activity of the antioxidant enzyme GTx was decreased in the gut of animals subjected to PH compared with that in the control group of animals not subjected to PH ( P < 0.01). However, the group that received glutamine for seven days after the PPVL showed similar GTx activity to both the control groups not subjected to PH ( P > 0.05). At least 10 random, non-overlapping images of each histological slide with 200 × magnification (44 pixel = 1 μm) were captured. The sum means of all areas, of each group were calculated. The mean areas of eNOS staining for both of the control groups were similar. The PPVL group showed the largest area of staining for eNOS. The PPVL + G group had the second highest amount of staining, but the mean value was much lower than that of the PPVL group ( P < 0.01). For iNOS, the control (SO) and control + G (SO + G) groups showed similar areas of staining. The PPVL group contained the largest area of iNOS staining, followed by the PPVL + G group; however, this area was significantly smaller than that of the group that underwent PH without glutamine ( P < 0.01). Treatment with glutamine prevents gut mucosal injury after PH in rats.

The classification based on intrahepatic portal system for congenital portosystemic shunts.

PubMed

Kanazawa, Hiroyuki; Nosaka, Shunsuke; Miyazaki, Osamu; Sakamoto, Seisuke; Fukuda, Akinari; Shigeta, Takanobu; Nakazawa, Atsuko; Kasahara, Mureo

2015-04-01

Liver transplantation was previously indicated as a curative operation for congenital absence of portal vein. Recent advances in radiological interventional techniques can precisely visualize the architecture of the intrahepatic portal system (IHPS). Therefore, the therapeutic approach for congenital portosystemic shunt (CPS) needs to be reevaluated from a viewpoint of radiological appearances. The aim of this study was to propose the IHPS classification which could explain the pathophysiological characteristics and play a complementary role of a therapeutic approach and management for CPS. Nineteen patients with CPS were retrospectively reviewed. The median age at diagnosis was 6.8 years old. Eighteen of these patients underwent angiography with a shunt occlusion test and were classified based of the severity of the hypoplasia of IHPS. The eighteen cases who could undergo the shunt occlusion test were classified into mild (n=7), moderate (n=6) and severe types (n=5) according to the IHPS classification. The IHPS classification correlated with the portal venous pressure under shunt occlusion, the histopathological findings, postoperative portal venous flow and liver regeneration. Shunt closure resulted in dramatic improvement in the laboratory data and subclinical encephalopathy. Two patients with the severe type suffered from sepsis associated with portal hypertension after treatment, and from the portal flow steal phenomenon because of the development of unexpected collateral vessels. The patients with the severe type had a high risk of postoperative complications after shunt closure in one step, even if the PVP was relatively low during the shunt occlusion test. The IHPS could be visualized by the shunt occlusion test. The IHPS classification reflected the clinicopathological features of CPS, and was useful to determine the therapeutic approach and management for CPS. Copyright © 2015 Elsevier Inc. All rights reserved.

A Meta-analysis for the Diagnostic Performance of Transient Elastography for Clinically Significant Portal Hypertension.

PubMed

You, Myung-Won; Kim, Kyung Won; Pyo, Junhee; Huh, Jimi; Kim, Hyoung Jung; Lee, So Jung; Park, Seong Ho

2017-01-01

We aimed to evaluate the correlation between liver stiffness measurement using transient elastography (TE-LSM) and hepatic venous pressure gradient and the diagnostic performance of TE-LSM in assessing clinically significant portal hypertension through meta-analysis. Eleven studies were included from thorough literature research and selection processes. The summary correlation coefficient was 0.783 (95% confidence interval [CI], 0.737-0.823). Summary sensitivity, specificity and area under the hierarchical summary receiver operating characteristic curve (AUC) were 87.5% (95% CI, 75.8-93.9%), 85.3 % (95% CI, 76.9-90.9%) and 0.9, respectively. The subgroup with low cut-off values of 13.6-18 kPa had better summary estimates (sensitivity 91.2%, specificity 81.3% and partial AUC 0.921) than the subgroup with high cut-off values of 21-25 kPa (sensitivity 71.2%, specificity 90.9% and partial AUC 0.769). In summary, TE-LSM correlated well with hepatic venous pressure gradient and represented good diagnostic performance in diagnosing clinically significant portal hypertension. For use as a sensitive screening tool, we propose using low cut-off values of 13.6-18 kPa in TE-LSM. Copyright © 2016 World Federation for Ultrasound in Medicine & Biology. Published by Elsevier Inc. All rights reserved.

Jejunal permeability to water and electrolytes in patients with chronic intrahepatic hypertension: evidence for a role of aldosterone.

PubMed Central

Duclos, B; Bories, P; Mathieu-Daude, J C; Michel, H

1991-01-01

Acute prehepatic portal hypertension induces intestinal secretion in animal models. In the course of chronic liver disease, however, these changes are not observed, despite higher portal pressures than those found in experimental studies. Eight patients without diarrhoea and with chronic alcoholic liver disease were examined for evidence of increased jejunal secretion; their suprahepatic wedge pressure was raised from 21 to 45 mmHg (mean 34.6 mmHg). Jejunal perfusion with a triple lumen catheter and a proximal occluding balloon was used to study net flows of water and chloride as well as net and unidirectional flows of sodium and potassium. No statistical difference in intestinal flows of water and electrolytes was noted between cirrhotic patients and control subjects after infusion with a 30 mmol/l glucose solution. Infusion with a 30 mmol/l mannitol solution resulted in a lower absorption of water, Na, K, and Cl than with the glucose solution. A higher rate of Na secretion was observed in cirrhotic patients than control subjects after infusion with 30 mmol/l mannitol (p less than 0.01). In addition, the rate of Na secretion was higher in cirrhotic patients than in control subjects (p less than 0.05). There was no correlation between the net flow of Na and the suprahepatic wedge pressure. A second perfusion with a 30 mmol/l glucose solution was given 75 minutes after a bolus injection of spironolactone (400 mg). Net flows of Na and Cl were lower in cirrhotic patients than in control subjects (p less than 0.05) because of a lower absorption of Na. Patients with gradually developing portal hypertension have moderate jejunal secretions of H2O and electrolytes which we assume are partly masked by increased absorption resulting from hyperaldosteronism. In contrast to animal models, this mechanism may be part of the jejunal adaptation to permeability in acute portal hypertension. PMID:2060871

Soluble CD163, a marker of Kupffer cell activation, is related to portal hypertension in patients with liver cirrhosis.

PubMed

Grønbaek, H; Sandahl, T D; Mortensen, C; Vilstrup, H; Møller, H J; Møller, S

2012-07-01

Activation of Kupffer cells may be involved in the pathogenesis of portal hypertension by release of vasoconstrictive substances and fibrosis due to co-activation of hepatic stellate cells. To study soluble plasma (s) CD163, a specific marker of activated macrophages, as a biomarker for portal hypertension in patients with liver cirrhosis. We measured sCD163 concentration and the hepatic venous pressure gradient (HVPG) by liver vein catheterisation in 81 cirrhosis patients (Child-Pugh CP-A: n = 26, CP-B: n = 29, CP-C: n = 26) and 22 healthy subjects. We also measured their cardiac output (CO), cardiac index and systemic vascular resistance (SVR). Liver status was examined by Child-Pugh and MELD-score. In cirrhosis, sCD163 concentration was nearly three times higher than in controls (4.7 ± 2.5 vs. 1.6 ± 0.5 mg/L, P < 0.001). sCD163 was also higher, as measured in steps by CP-score (P < 0.001). The HVPG rose steeply to an asymptote of 22 mmHg with sCD163 up to about 5 mg/L and not to higher values with higher sCD163. In a multivariate analysis, sCD163 was the only independent predictor of the HVPG but did not predict any of the systemic circulatory findings. sCD163 > 3.95 mg/L (upper normal limit) predicted HVPG ≥ 10 mmHg with a positive predictive value of 0.99. Circulating sCD163 originating from activated Kupffer cells is increased in cirrhosis with increasing Child-Pugh score and with increasing HVPG, and it is an independent predictor for HVPG. These findings support a primary role of macrophage activation in portal hypertension, and may indicate a target for biological intervention. © 2012 Blackwell Publishing Ltd.

Invasive and non-invasive diagnosis of cirrhosis and portal hypertension

PubMed Central

Kim, Moon Young; Jeong, Woo Kyoung; Baik, Soon Koo

2014-01-01

With advances in the management and treatment of advanced liver disease, including the use of antiviral therapy, a simple, one stage description for advanced fibrotic liver disease has become inadequate. Although refining the diagnosis of cirrhosis to reflect disease heterogeneity is essential, current diagnostic tests have not kept pace with the progression of this new paradigm. Liver biopsy and hepatic venous pressure gradient measurement are the gold standards for the estimation of hepatic fibrosis and portal hypertension (PHT), respectively, and they have diagnostic and prognostic value. However, they are invasive and, as such, cannot be used repeatedly in clinical practice. The ideal noninvasive test should be safe, easy to perform, inexpensive, reproducible as well as to give numerical and accurate results in real time. It should be predictive of long term outcomes related with fibrosis and PHT to allow prognostic stratification. Recently, many types of noninvasive alternative tests have been developed and are under investigation. In particular, imaging and ultrasound based tests, such as transient elastography, have shown promising results. Although most of these noninvasive tests effectively identify severe fibrosis and PHT, the methods available for diagnosing moderate disease status are still insufficient, and further investigation is essential to predict outcomes and individualize therapy in this field. PMID:24764667

Portal vein thrombosis is a potentially preventable complication in clinical islet transplantation

PubMed Central

Kawahara, Toshiyasu; Kin, Tatsuya; Kashkoush, Samy; Gala-Lopez, Boris; Bigam, David L.; Kneteman, Norman M.; Koh, Angela; Senior, Peter A.; Shapiro, A.M. James

2011-01-01

Percutaneous transhepatic portal access avoids surgery, but is rarely associated with bleeding or portal venous thrombosis. We herein report our large, single-center experience of percutaneous islet implantation, and evaluate risk factors of portal vein thrombosis and graft function. Prospective data was collected on 268 intraportal islet transplants (122 subjects). A portal venous Doppler ultrasound was obtained on Days 1 and 7 days posttransplant. Therapeutic heparinization, complete ablation of the portal catheter tract with Avitene paste, and limiting packed cell volume to < 5 ml completely prevented any portal thrombosis in the most recent 101 islet transplant procedures over the past 5 years. In the previous cumulative experience, partial thrombosis did not affect islet function. Standard liver volume correlated negatively (r=−0.257, P<0.001), and packed cell volume correlated positively with portal pressure rise (r=0.463, P<0.001). Overall, partial portal thrombosis occurred after 10 procedures (overall incidence 3.7%, most recent 101 patient incidence 0%). There were no cases of complete thrombosis, and no patient developed sequelae of portal hypertension. In conclusion, portal thrombosis is a preventable complication in clinical islet transplantation, provided therapeutic anticoagulation is maintained, and packed cell volume is limited to <5 ml. PMID:21883914

[Correlation between the width of lingual vein and the changes of hemodynamics of portal system in patients with primary liver cancer].

PubMed

Yue, Xiao-Qiang; Gao, Jing-Dong; Zhai, Xiao-Feng; Liu, Qing; Jiang, Dong; Ling, Chang-Quan

2006-09-01

To explore the correlation between the width of lingual varix and changes of hemodynamics of portal system in patients with primary liver cancer so as to supply the data for the forecast of portal hypertension by observing lingual varix. The diameter of lingual vein (Dlv) was measured by vernier caliper as dependent variable, and the diameters and indexes of hemodynamics of portal vessels were measured by Doppler as independent variables, then a multipe stepwise analysis was performed. The diameters of portal vein (Dpv) and splenic vein (Dsv) entered the formula Dlv (mm) = 0.185 + 0.311 Dsv (mm) + 0.236 Dpv (mm) when the entry and removal values were alpha(in)=0.10 and alpha(out)=0.15, respectively. The width of lingual vein is closely correlated with the diameters of portal vein and splenic vein in patients with primary liver cancer.

Immediate effects of different schedules of somatostatin on portal pressure in patients with liver cirrhosis.

PubMed

Zhang, C; Xu, J-M; Kong, D-R; Min, X-K; Chen, R

2013-06-01

Somatostatin (SST) is used for the treatment of acute variceal bleeding based on its ability to decrease portal pressure and collateral blood flow. To date, no studies have focused on the immediate-early effects (between 1 and 30 min) of SST. The aim of this study was to compare the efficacy of different schedules of SST therapy with placebo on portal pressure in patients with portal hypertension treated with portal-azygous disconnection and to test whether an increase in bolus or infusion dose can improve the clinical efficacy of SST therapy.   Patients were treated with four different schedules: (a) standard dose (n = 11): one 250 μg bolus + a continuous infusion of 250 μg/h; (b) medium dose (n = 10): 500 μg bolus + a continuous infusion of 250 μg/h; (c) high dose (n = 10): 250 μg bolus + a continuous infusion of 500 μg/h; (d) control (n = 10): an injection of placebo (saline) followed by a placebo infusion. Following SST or placebo administration, portal pressure, central venous pressure (CVP), systemic blood pressure and heart rate (HR) were measured at 1, 3, 5, 7, 10 and 30 min.   The three schedules of SST induced a marked, rapid and highly significant decrease in portal pressure. The decline in portal pressure was moderate at 1 min (P < 0·040), achieved a peak effect at 5 min (P < 0·009) and remained decreased at 30 min. The effect of SST on portal pressure was significantly greater than placebo from 1 min after administration. There were no significant differences in portal pressure decrease between the three schedules of SST. The three schedules of SST and the placebo schedule did not induce significant changes in HR, systemic blood pressure and CVP.   This study shows that SST is effective in decreasing portal pressure within 30 min of administration in patients with liver cirrhosis. The clinical schedule used in this study was reasonable and safe. © 2013 Blackwell Publishing Ltd.

Endoscopic Ultrasound for the Hepatologist: A Comprehensive Review.

PubMed

Rimbaş, Mihai; Di Maurizio, Luca; Rizzatti, Gianenrico; Gasbarrini, Antonio; Costamagna, Guido; Larghi, Alberto

2018-05-01

In the last few years, the diagnostic and therapeutic utilization of endoscopic ultrasound (EUS) for a variety of liver conditions has exponentially grown. We performed a thorough search for all available studies on the performance of diagnostic and therapeutic EUS in the field of hepatology. This article reviews the indication of EUS in the evaluation and treatment of portal hypertension, portal vein pressure measurement, focal liver lesions, and parenchymal liver diseases, and presents all the clinical evidences available so far in this regard. All the review data suggest that EUS is becoming an increasingly important tool in the armamentarium of the hepatologists for the management of certain liver-related conditions. Implementation in the education of the hepatologists of means to become more familiar with both diagnostic and therapeutic capabilities of EUS is warranted. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Methylene Blue Enteric Mapping for Intraoperative Localization in Obscure Small Bowel Hemorrhage: Report of a New Technique and Literature Review

DTIC Science & Technology

2012-07-12

Becker GJ, Park HM, O’Connor KW, Tarver RD, Scott JA, Jackson VP, Lappas JC, Broadie TA, Holden RW. Portal hypertension due to jejunal vascular...controlled hypertension and hyperlipidemia, a pri- or of laparoscopic cholecystectomy, and open small bowel resection. The patient reported melena and

Successful resolution of severe hepatopulmonary syndrome following liver transplantation.

PubMed

Asthana, Sonal; Maguire, Connor; Lou, Lawrence; Meier, Michael; Bain, Vincent; Townsend, Derek R; Townsend, Rex; Lien, Dale; Bigam, David; Kneteman, Norman; Shapiro, Andrew Mark James

2010-04-01

Hepatopulmonary syndrome (HPS) is a complication of portal hypertension, defined by the presence of liver disease, abnormal pulmonary gas exchange and evidence of intrapulmonary vascular dilatations producing a right-to-left intrapulmonary shunt. Liver transplantation (LT) is the treatment of choice; however, severe hypoxemia (PaO(2) < 50 mmHg on room air) is considered a contraindication to LT. This approach disadvantages some patients, particularly young patients with no intrinsic cardio-respiratory disease. We discuss one such patient who improved with LT despite having extremely severe HPS (PaO2 < 29 mmHg).

Two cases of esophageal cancer with portal hypertension: esophagectomy with venous shunt procedure.

PubMed

Kato, T; Motohara, T; Kaneko, Y; Shikishima, H; Okushiba, S; Kondo, S; Kato, H

2001-01-01

We performed venous shunt procedure in the reconstruction of the esophagus after esophagectomy using the gastric tube in two cases of esophageal cancer with portal hypertension due to liver cirrhosis. In both cases, the short-term postoperative course was uneventful, without congestion in the gastric tube. In Case 1 where the short gastric vein had been used as the shunt vein, the long-term postoperative course was also uneventful, without hepatic encephalopathy or hemorrhage from deterioration of the varices of the gastric tube. However, in Case 2 where the left gastroepiploic vein had been used, hepatic encephalopathy developed due to excessive shunt flow. These results suggested that appropriate shunt flow could be expected by using short gastric vein.

[Association of biliary calculosis and portal cavernomatosis].

PubMed

Crespi, C; De Giorgio, A M

1992-08-01

This paper reports the case of a woman, who underwent surgery because of cholelithiasis, with intraoperative finding of prehepatic portal hypertension from portal vein thrombosis ("portal cavernoma") with healthy liver, later confirmed by angiographic studies. This rare pathologic association carries a higher risk of major operative complications; therefore the Authors agree with the general belief that, for these cases, biliary tract surgery should be as simple and safe as possible. In the case of preoperative diagnosis of biliary disease associated with portal cavernoma, should a surgical approach on the biliary tract be required, we agree on the advisability of performing a shunting procedure before any kind of biliary surgery. In case of variceal bleeding endoscopic sclerotherapy will be the first choice; surgical procedures (shunting) should be seen as a second choice in case of rebleeding after sclerotherapy.

[Current status and perspectives of diagnosis and treatment of complications related to liver cirrhosis].

PubMed

Nan, Y M

2017-04-20

Liver cirrhosis is the severe period of chronic liver diseases, especially decompensated liver cirrhosis and its complications, such as ascites, esophagogastric variceal bleeding, hepatic encephalopathy, acute kidney injury, and hepatocellular carcinoma, which greatly affect patients' quality of life and even threaten their lives. Early prevention and treatment of the causes of development and progression and pathogenic mechanism may slow down or reverse liver cirrhosis and its severe complications. Once the disease progresses to portal hypertension and related complications, it is very important to select preventive measures for acute exacerbation of different complications, as well as the methods and timing for treatment in acute stage, which may help to save patients' lives and improve their prognosis.

Catheter interventional treatment of congenital portosystemic venous shunts in childhood.

PubMed

Knirsch, Walter; Benz, Dominik C; Bühr, Patrick; Quandt, Daniel; Weber, Roland; Kellenberger, Christian; Braegger, Christian P; Kretschmar, Oliver

2016-06-01

The study aimed on the catheter interventional treatment of congenital portosystemic venous shunt (CPSVS) in childhood and the impact on vascular growth of hypoplastic portal veins. Clinical course and follow up of partial or complete closure of CPSVS are described. CPSVS is a very rare vascular malformation of the portal venous drainage resulting in potentially life threatening abdominal, pulmonary, cerebral, and cardiac complications. The anatomic type, clinical course, and diagnosis must be determined for optimal management. Single centre case series. Eight (6 female) children with extrahepatic (n = 5) and intrahepatic (n = 3) CPSVS were diagnosed invasively by catheterization, including test balloon occlusion of the shunt and simultaneous retrograde angiography, and treated by catheter interventions (n = 5) with partial (n = 2) and complete (n = 3) occlusion of CPSVS at a median age of 3.9 years (range 0.7-21). Congenital heart disease (CHD) was the most frequent associated organ manifestation (n = 5) followed by mild to severe pulmonary arterial hypertension (n = 4), hepatopulmonary syndrome (n = 2), and portosystemic encephalopathy (n = 1). CHD was simple (n = 3) or complex type (n = 2). Three patients were untreated so far, because they were in excellent clinical condition at an age <1 year, refused treatment, or planned for later treatment. Accurate invasive diagnosis of CPSVS with test balloon occlusion of the shunt is mandatory to depict the anatomic situation. Catheter interventional treatment of CPSVS offers a feasible and safe approach with complete or partial closure of the vascular malformation inducing potentially significant vascular growth of a former hypoplastic portal venous system. © 2015 Wiley Periodicals, Inc. © 2015 Wiley Periodicals, Inc.

Rapid alternative absorption of dietary long-chain fatty acids with upregulation of intestinal glycosylated CD36 in liver cirrhosis.

PubMed

Yamamoto, Yasunori; Hiasa, Yoichi; Murakami, Hidehiro; Ikeda, Yoshio; Yamanishi, Hirofumi; Abe, Masanori; Matsuura, Bunzo; Onji, Morikazu

2012-07-01

Dietary long-chain fatty acid (LCFA) intake is an important risk factor for hepatic inflammation and hepatocarcinogenesis. An alternate route of dietary LCFA absorption has been suggested in patients with liver cirrhosis (LC). We aimed to determine this alternate route and to identify its mechanism. Twenty healthy control subjects and 47 patients with LC-n = 23 with portal hypertension [PH(+)LC] and 24 without portal hypertension [PH(-)LC)]-were enrolled. [¹³C]Palmitate (an LCFA) and octanoate (a medium-chain fatty acid [MCFA]) were administered by using gastrointestinal endoscopy. Breath ¹³CO₂ was measured to quantify metabolized fatty acids. We also examined intestinal specimens of patients in these groups. A more rapid increase in metabolized palmitate, which showed a pattern similar to that of octanoate metabolism, was observed in patients with LC than in healthy control subjects. The increase in the PH(-)LC group was higher than that in the PH(+)LC group. However, the concentration of metabolized palmitate increased with treatment of the PH(+)LC group with a portal-systemic shunt. Morphologic changes such as expanded lymph and blood vessels were present, and glycosylated CD36 increased in the jejunum of the PH(+)LC group. This group had high serum concentrations of glucagon-like peptide-2. These data suggest that dietary LCFAs, similar to MCFAs, are absorbed via blood vessels in patients with LC. Rapid absorption of LCFAs by an alternative method occurred in patients with LC. This altered LCFA processing is likely related to upregulation of intestinal glycosylated CD36 and could contribute to pathogenesis in patients with LC.

Hepatic vein transit time of second-generation ultrasound contrast agent: new tool in the assessment of portal hypertension.

PubMed

Luisa, Siciliani; Vitale, Giovanna; Sorbo, Anna Rita; Maurizio, Pompili; Lodovico, Rapaccini Gian

2017-03-01

It has been demonstrated that Doppler waveform of the hepatic vein (normally triphasic) is transformed into a biphasic or monophasic waveform in cirrhotic patients. The compressive mechanism of liver tissue has been considered up till now the cause of this change. Moreover, cirrhotics show, after USCA injection, a much earlier HVTT due to intrahepatic shunts. Our aim was to prospectively evaluate the correlation between Doppler pattern of hepatic vein and HVTT of a second-generation USCA; we also correlated HVTT with the most common indexes of portal hypertension. We enrolled 38 participants: 33 cirrhotics and 5 healthy controls. Doppler shift signals were obtained from the right hepatic vein. To characterize the hepatic vein pattern, we used the hepatic vein waveform index (HVWI). This index becomes >1 with the appearance of the triphasic waveform. We recorded a clip from 20 s before to 2 min after a peripheral intravenous bolus injection of 2.4 ml of USCA (sulfur hexafluoride).The time employed by USCA to cross the liver from the hepatic artery and portal vein to the hepatic vein was defined as HA-HVTT and PV-HVTT, respectively. Cirrhotics with low HVWI showed an earlier transit time; participants with higher HVWI had a longer transit time ( p  < 0.001). HVTT was earlier as MELD, Child-Pugh score and spleen diameter increased. Patients with ascites and varices of large size had significantly shorter transit times. Abnormal hepatic vein Doppler waveform in cirrhotic patients could be due to intrahepatic shunts. HVTT could be useful in the non-invasive evaluation of portal hypertension.

CT perfusion imaging of the liver and the spleen in patients with cirrhosis: Is there a correlation between perfusion and portal venous hypertension?

PubMed

Talakić, Emina; Schaffellner, Silvia; Kniepeiss, Daniela; Mueller, Helmut; Stauber, Rudolf; Quehenberger, Franz; Schoellnast, Helmut

2017-10-01

To correlate hepatic and splenic CT perfusion parameters with hepatic venous pressure gradient (HVPG) measurements in patients with cirrhosis. Twenty-one patients with cirrhosis (males, 17; females, 4; mean ± SD age, 57 ± 7 years) underwent hepatic and splenic perfusion CT on a 320-detector row volume scanner as well as invasive measurement of HVPG. Different CT perfusion algorithms (maximum slope analysis and Patlak plot) were used to measure hepatic arterial flow (HAF), portal venous flow (PVF), hepatic perfusion index (HPI), splenic arterial flow (SAF), splenic blood volume (SBV) and splenic clearance (SCL). Hepatic and splenic perfusion parameters were correlated with HVPG, and sensitivity and specificity for detection of severe portal hypertension (≥12 mmHg) were calculated. The Spearman correlation coefficient was -0.53 (p < 0.05) between SAF and HVPG, and -0.68 (p < 0.01) between HVPG and SCL. Using a cut-off value of 125 ml/min/100 ml for SCL, sensitivity for detection of a HVPG of ≥12 mmHg was 94%, and specificity 100%. There was no significant correlation between hepatic perfusion parameters and HVPG. CT perfusion in patients with cirrhosis showed a strong correlation between SCL and HVPG and may be used for detection of severe portal hypertension. • SAF and SCL are statistically significantly correlated with HVPG • SCL showed stronger correlation with HVPG than SAF • 125 ml/min/100 ml SCL-cut-off yielded 94 % sensitivity, 100 % specificity for severe PH • HAF, PVF and HPI showed no statistically significant correlation with HVPG.

Assessing the brain through the eye: New ways to explore hepatic encephalopathy.

PubMed

Arias, Natalia; Méndez, Marta; Alcalde, Ignacio; Íñigo-Portugués, Almudena; Merayo-Lloves, Jesús; Arias, Jaime; Arias, Jorge L

2017-05-01

Minimal hepatic encephalopathy (mHE) has been shown to affect daily functioning, quality of life, driving and overall mortality. However, little is known about treating or diagnosing early impairments in mHE. We studied one of its precipitating factors, portal hypertension which is driving the inflammatory process behind mHE. The purpose was to describe an indirect diagnostic method able to detect the pathology at early stages based on the study of the vascularization and mast cells conjunctival hyperplasia as secondary inflammatory response associated to portal hypertension. Finally, we correlated the presence of histological changes in the eye in mHE with deficits in behavioral task acquisition. Rats were trained on a stimulus-response task and a spatial working memory task using the Morris water maze. Two groups of animals were used: a SHAM (sham-operated) group (n=10) and a portal hypertension (HT) group (n=10). The triple portal vein ligation method was used to create an animal model of mHE. Latencies to reach the platform, number of glial fibrillary acidic protein-immunoreactive astrocytes (GFAP-IR), mast cell expression and presence/absence of blood and lymphatic vessels were examined. There were differences in stimulus-response behavioral performance, with a deficit in the acquisition in the HT group. However, no differences between groups were found on the spatial working memory task. At the same time, differences between groups were found in the GFAP-IR density, which was lower in the HT group, and in the number of mast cells and the presence of vessels, which were higher in the HT group. In this study, we provide the first preliminary insight into the validity of exploring the eye as a possible tool to assess the diagnosis of mHE conditions. Copyright © 2017 Elsevier Inc. All rights reserved.

A Combined Training Program for Veterans with Amnestic Mild Cognitive Impairment

DTIC Science & Technology

2013-10-01

severe liver disease (cirrhosis,  esophageal varices, ascites,  portal   hypertension , hepatic encephalopathy).  E05  Current severe cardiac disease (e.g...cognitive impairment such as hypertension , obesity, and type II diabetes mellitus [1, 2; 3; 4]. Hence, it may be that by improving cardiovascular health

Cavernous Transformation of Portal Vein Secondary to Portal Vein Thrombosis: A Case Report

PubMed Central

Ramos, Radhames; Park, Yoojin; Shazad, Ghulamullah; A.Garcia, Christine; Cohen, Ronny

2012-01-01

There are few reported cases of cavernous transformation of the portal vein (CTPV) in adults. We present a case of a 58 year-old male who was found to have this complication due to portal vein thrombosis (PVT). A 58-year old African American male with chronic alcohol and tobacco use presented with a 25-day history of weakness, generalized malaise, nausea and vomiting associated with progressively worsening anorexia and weight loss. The patient was admitted for severe anemia in conjunction with abnormal liver function tests and electrolyte abnormalities, and to rule out end stage liver disease or hepatic malignancy. The work-up for anemia showed no significant colon abnormalities, cholecystitis, liver cirrhosis, or liver abnormalities but could not rule out malignancy. An esophageogastroduodenoscopy (EGD) was suspicious for a mass compressing the stomach and small bowel. After further work-up, the hepatic mass has been diagnosed as a cavernous transformation of the portal vein (CTPV), a very rare complication of portal vein thrombosis (PVT). Cavernous Transformation of the Portal Vein (CTPV) is a rare and incurable complication of portal vein thrombosis (PVT) that should be considered as one of the differential diagnoses of a hepatic mass. Keywords Cavernous transformation of the portal vein; Portal vein thrombosis; Portal hypertension; Hyperbilirubinemia; Hepatic mass PMID:22383935

CT Demonstration of Caput Medusae

ERIC Educational Resources Information Center

Weber, Edward C.; Vilensky, Joel A.

2009-01-01

Maximum intensity and volume rendered CT displays of caput medusae are provided to demonstrate both the anatomy and physiology of this portosystemic shunt associated with portal hypertension. (Contains 2 figures.)

Jejunal variceal bleeding after esophageal transection in a patient with idiopathic portal hypertension.

PubMed

Migou, S; Hashizume, M; Tsugawa, K; Kishihara, F; Kawanaka, H; Ohta, M; Tanoue, K; Kuroiwa, T; Kawamoto, K; Sugimachi, K

1998-01-01

This report describes a 38-year-old man with massive gastrointestinal bleeding from jejunal varices. He had been previously diagnosed to have idiopathic portal hypertension and esophageal varices, and had undergone an esophageal transection 8 years earlier. The pre-operative diagnosis was a suspected hemorrhage from the small intestine as visualized by 99mTc-HSAD scintigraphy (technetium 99m-labeled human serum albumin D-type) and was not considered to be repeated massive lower GI tract bleeding. An exploratory laparotomy was performed, and intra-operative endoscopy revealed active bleeding from the jejunal varices. A partial resection of the small intestine resulted in a complete resolution of the bleeding. A review of the literature thereafter disclosed twelve previously reported cases of jejunal variceal bleeding.

N-acetylcysteine modulates angiogenesis and vasodilation in stomach such as DNA damage in blood of portal hypertensive rats.

PubMed

Licks, Francielli; Hartmann, Renata Minuzzo; Marques, Camila; Schemitt, Elizângela; Colares, Josieli Raskopf; Soares, Mariana do Couto; Reys, Juliana; Fisher, Camila; da Silva, Juliana; Marroni, Norma Possa

2015-11-21

To evaluate the antioxidant effect of N-acetylcysteine (NAC) on the stomach of rats with portal hypertension. Twenty-four male Wistar rats weighing ± 250 g were divided into four experimental groups (n = 6 each): Sham-operated (SO), SO + NAC, partial portal vein ligation (PPVL), and PPVL + NAC. Treatment with NAC in a dose of 10 mg/kg (i.p.) diluted in 0.6 mL of saline solution was administered daily for 7 d starting 8 d after the surgery. Animals from the PPVL and SO group received saline solution (0.6 mL) for the same period of time as the PPVL + NAC and SO + NAC group. On the 15(th) day the animals were anesthetized and we evaluated portal pressure by cannulating mesenteric artery. After, we removed the stomach for further analysis. We performed immunohistochemical analysis for endothelial nitric oxide synthase (eNOS), vascular endothelial growth factor (VEGF), and nitrotirosine (NTT) proteins in stomach. We also evaluated eNOS and VEGF by Western blot analysis and assessed DNA damage in blood samples by the comet assay. The portal hypertension group exhibited increases in portal pressure when compared to SO group (29.8 ± 1.8 vs 12.0 ± 0.3 mmHg) (P < 0.001). The same was observed when we compared the eNOS (56.8 ± 3.7 vs 13.46 ± 2.8 pixels) (P < 0.001), VEGF (34.9 ± 4.7 vs 17.46 ± 2.6 pixels) (P < 0.05), and NTT (39.01 ± 4.0 vs 12.77 ± 2.3 pixels) (P < 0.05) expression by immunohistochemistry of the PPVL animals with the SO group. The expression of eNOS (0.39 ± 0.03 vs 0.25 ± 0.03 a.μ) (P < 0.01) and VEGF (0.38 ± 0.04 vs 0.26 ± 0.04 a.μ) (P < 0.01) were also evaluated by Western blot analysis, and we observed an increase of both proteins on PPVL animals. We also evaluated the DNA damage by comet assay, and observed an increase on damage index and damage frequency on those animals. NAC decreased portal pressure values in PPVL + NAC animals (16.46 ± 2 vs 29.8 ± 1.8 mmHg) (P < 0.001) when compared to PPVL. The expression of eNOS (14.60 ± 4.1 vs 56.8 ± 3.7 pixels) (P < 0.001), VEGF (19.53 ± 3.2 vs 34.9 ± 4.7 pixels) (P < 0.05) and NTT (21.84 ± 0.7 vs 39.01 ± 4.0 pixels) (P < 0.05) evaluated by immunohistochemistry were also reduced in PPVL + NAC animals. Also, when evaluated by Western blot eNOS expression (0.32 ± 0.03 vs 0.39 ± 0.03 a.μ) (P < 0.05) and VEGF expression (0.31 ± 0.09 vs 0.38 ± 0.04 a.μ) (P < 0.01). Furthermore, NAC modulated DNA damage in PPVL + NAC animals. In view of these results, we believe NAC is able to protect the stomach from the alterations induced by the PPVL procedure.

[Hemodynamic changes in portal system after transjugular intrahepatic portosystemic shunts (TIPS)].

PubMed

Li, W; Xiao, Y; Xu, H

1995-08-01

In this study portal venous flow (PVF), splenic venous flow (SPVF), hepatic artery volume (HAV), and portal pressure were measured before and after TIPS in 11 patients with portal hypertension. The results were compared with those of normal controls. In the normal controls PVF averaged 947.2 +/- 133.4ml/min, SPVF 239.6 +/- 116.3ml/min, and HAV 241.6 +/- 78.8ml/min. In the TIPS group before and after TIPS, PVF was 883.2 +/- 233.4ml/min vs. 958.7 +/- 185.2ml/min; SPVF was 448.9 +/- 111.6ml/min vs. 333.1 +/- 101.5ml/min; HVP was 225.3 +/- 122.7ml/min vs. 249.3 +/- 103.8ml/min. Portal pressure dropped from 3.94kPa +/- 0.46 to 2.52 +/- 0.60kPa after TIPS. We conclude that in patients after TIPS portal pressure drops blood flow in the truck of the portal vein increases, flow of hepatic tissue decreases, and blood flow in splenic vein decreases. Blood flow in hepatic aftery was not significantly changed.

A Combined Training Program for Veterans with Amnestic Mild Cognitive Impairment

DTIC Science & Technology

2015-10-01

Acute illness or unstable chronic illness, e.g., history of severe liver disease (cirrhosis,  esophageal varices, ascites,  portal   hypertension ...impairment such as hypertension , obesity, and type II diabetes mellitus [1; 2; 3; 4]. Hence, it may be that by improving cardiovascular health and reducing

A Combined Training Program for Veterans with Amnestic Mild Cognitive Impairment

DTIC Science & Technology

2014-10-01

portal   hypertension , hepatic encephalopathy).  E05  Current severe cardiac disease (e.g., uncontrolled atrial fibrillation, defined as mean 24 hour  heart... hypertension , obesity, and type II diabetes mellitus [1, 2; 3; 4]. Hence, it may be that by improving cardiovascular health and reducing vascular risk factors

Use of concomitant variceal embolization and prophylactic antiplatelet/anticoagulative in transjugular intrahepatic portosystemic shunting: A retrospective study of 182 cirrhotic portal hypertension patients.

PubMed

Tang, Yingmei; Zheng, Sheng; Yang, Jinhui; Bao, Weimin; Yang, Lihong; Li, Yingchun; Xu, Ying; Yang, Jing; Tong, Yuyun; Gao, Jinhang; Tang, Chengwei

2017-12-01

Transjugular intrahepatic portosystemic shunting (TIPS) is an effective treatment modality for refractory variceal bleeding and ascites in patients with cirrhotic portal hypertension (CPH). Variceal rebleeding and shunt dysfunction are major post-TIPS morbidities. This study aimed to retrospectively evaluate the effectiveness and safety of use of concomitant variceal embolization and prophylactic antiplatelet/anticoagulative in patients with CPH undergoing TIPS. Between October 2006 and October 2011, 182 patients with CPH were retrospectively and consecutively hospitalized for elective TIPS with Fluency stenting. Concomitant variceal embolization was given after establishing the shunt. Subcutaneous heparin was given after TIPS and replaced by oral clopidogrel, aspirin, or warfarin for at least 6 months. Main outcome measures included shunt patency rate, recurrence of CPH (rebleeding and/or refractory ascites), hepatic encephalopathy (HE) frequency, and post-TIPS survival. The cumulative primary patency rate was 96%, 94%, 90%, 88%, and 88% at 6, 12, 24, 36, and 48 months, respectively. Shunt stenosis occurred in 16 (9%) patients, gastrointestinal (GI) rebleeding in 32 (17.5%) patients, recurrence of refractory ascites 44 (48%) patients, HE in 42 (23%) patients, and death in 36 (20%) patients during the follow-up period. Use of concomitant variceal embolization and prophylactic antiplatelet/anticoagulative was associated with a favorable shunt patency and a low risk of GI rebleeding.

Current concepts in congenital portosystemic shunts.

PubMed

Mankin, Kelley M Thieman

2015-05-01

Congenital portosystemic shunts (CPSS) are vascular abnormalities that allow portal blood to bypass the liver and join systemic circulation. Laboratory and imaging studies are performed preoperatively to diagnose CPSS and hopefully identify an anatomic location of the shunt. CPSS can be found in different locations in both small and large breed dogs. Most CPSS are best managed surgically. The goal of surgical management of CPSS is to slowly redirect blood from the shunting vessel through the portal vasculature while avoiding portal hypertension. Many surgical management methods are available, including open and less invasive procedures, such as laparoscopy and embolization. Copyright © 2015 Elsevier Inc. All rights reserved.

Telesonography Adaptation and Use to Improve the Standard of Patient Care Within a Dominican Community

ClinicalTrials.gov

2009-05-06

Ascites; Blunt Abdominal Trauma; Cholelithiasis; Cholecystitis; Cholangitis; Pancreatitis; Hydronephrosis; Abdominal Aortic Aneurysm; Hepatitis; Portal Hypertension; Urolithiasis; Abnormal Uterine Bleeding; Ovarian Mass; Ovarian Torsion

Nadolol reduces insulin sensitivity in liver cirrhosis: a randomized double-blind crossover trial.

PubMed

Lee, Wai Gin; Murphy, Rinki; McCall, John L; Gane, Edward J; Soop, Mattias; Tura, Andrea; Plank, Lindsay D

2017-03-01

Liver cirrhosis is frequently complicated by portal hypertension leading to increased mortality from variceal bleeding and hepatic decompensation. Noncardioselective β-blockers not only reduce portal hypertension and prevent variceal bleeding in cirrhosis but also impair glucose tolerance and insulin sensitivity in other settings. This study aimed to determine whether nonselective β-blockade with nadolol impairs glucose metabolism in liver cirrhosis. A randomized, double-blind, placebo-controlled crossover trial of nadolol in cirrhotic patients examined insulin sensitivity, disposition index, and glucose tolerance. Stable cirrhotic patients of mixed etiology underwent an intravenous glucose tolerance test and hyperinsulinemic-euglycemic clamp for the measurement of insulin secretion and insulin sensitivity (n = 16) and a 75-g oral glucose tolerance test (n = 17). These measurements were conducted twice (after 3 months of treatment with nadolol or placebo and, after a 1-month washout period, after 3 months on the alternative treatment). Total body fat and plasma catecholamines were measured at the end of each 3-month treatment. Compared with placebo, nadolol treatment reduced insulin sensitivity (79.7 ± 10.1 vs 99.6 ± 10.3 μL/kg fat-free mass·min -1 ·(mU/L) -1 , P = .005). Insulin secretion was unchanged (P = .24), yielding a lower disposition index with nadolol (6083 ± 2007 vs 8692 ± 2036, P = .050). There was no change in total body fat or plasma catecholamines. A 2-hour plasma glucose concentration from the oral glucose tolerance test was higher on nadolol than placebo (10.8 ± 0.9 vs 9.9 ± 0.9 mmol/L, P = .035). Nadolol significantly worsened insulin sensitivity, glycemia, and disposition index in patients with liver cirrhosis. These findings may have significant clinical implications because cirrhosis is already associated with an increased prevalence of diabetes. Copyright © 2016 John Wiley & Sons, Ltd.

Exercise and Pulmonary Hypertension (PH)

MedlinePlus

... with PAH Consensus Statement Issued by the Scientific Leadership Council DISCLAIMER: This information is for general information ... on our new PHPN/PHCR or Support Group Leadership Institute portal? Reset your password here . Login Username ...

Cirrhosis and Portal Hypertension

MedlinePlus

... Kids and Teens Pregnancy and Childbirth Women Men Seniors Your Health Resources Healthcare Management End-of-Life ... familydoctor.org editorial staff Categories: Family Health, Men, Seniors, WomenTags: adult, alcoholic cirrhosis, elderly, jaundice, liver disease, ...

Splenophrenic portosystemic shunt in dogs with and without portal hypertension: can acquired and congenital porto-caval connections coexist?

PubMed Central

Ricciardi, M.

2016-01-01

The possible existence of the same pattern of porto-caval connection in dogs having a single congenital portosystemic shunt (CPSS) and in dogs having multiple acquired portosystemic shunt (MAPSS) secondary to portal hypertension (PH) was evaluated. Retrospective evaluation of all CT examinations of patients having portosystemic shunt (PSS) was performed in a 4-year time period. All anomalous porto-caval connections were assessed for anatomical pattern and compared with published veterinary literature. Records of 25 dogs were reviewed. 16 dogs had a single CPSS (CPSS group), and 9 dogs had multiple acquired PSS secondary to PH (APSS group). The splenophrenic shunt pattern was found in 3 dogs of the CPSS group as a single congenital anomaly without PH and in 2 dogs of the APSS group associated with MAPSS and ascites due to different hepatic diseases causing PH. These findings corroborate two hypotheses: 1) Splenophrenic PSS should be considered as a classical CPSS but if this is not sufficient to alleviate a PH developed after birth because of eventual hepatic or portal diseases, in this case ascites and acquired portal collaterals may develop. In this case, MAPSS and CPSS may coexist. 2) The pattern of splenophrenic PSS, classically described among CPSS, may develop as acquired portal collateral in dogs with PH and it should also be included in the category of APSS. These preliminary findings may be helpful in reconsidering the classical haemodynamics of porto-caval diseases, enrich the classification of APSS in dogs and refine the imaging evaluation of patients with PH. PMID:27882305

Transjugular intrahepatic porto-systemic shunt in the elderly: Palliation for complications of portal hypertension.

PubMed

Syed, Mubin I; Karsan, Hetal; Ferral, Hector; Shaikh, Azim; Waheed, Uzma; Akhter, Talal; Gabbard, Alan; Morar, Kamal; Tyrrell, Robert

2012-02-27

To present a dedicated series of transjugular intrahepatic porto-systemic shunts (TIPS) in the elderly since data is sparse on this population group. A retrospective review was performed of patients at least 65 years of age who underwent TIPS at our institutions between 1997 and 2010. Twenty-five patients were referred for TIPS. We deemed that 2 patients were not considered appropriate candidates due to their markedly advanced liver disease. Of the 23 patients suitable for TIPS, the indications for TIPS placement was portal hypertension complicated by refractory ascites alone (n = 9), hepatic hydrothorax alone (n = 2), refractory ascites and hydrothorax (n = 1), gastrointestinal bleeding alone (n = 8), gastrointestinal bleeding and ascites (n = 3). Of these 23 attempted TIPS procedure patients, 21 patients had technically successful TIPS procedures. A total of 29 out of 32 TIPS procedures including revisions were successful in 21 patients with a mean age of 72.1 years (range 65-82 years). Three of the procedures were unsuccessful attempts at TIPS and 8 procedures were successful revisions of our existing TIPS. Sixteen of 21 patients who underwent successful TIPS (excluding 5 patients lost to follow-up) were followed for a mean of 14.7 mo. Ascites and/or hydrothorax was controlled following technically successful procedures in 12 of 13 patients. Bleeding was controlled following technically successful procedures in 10 out of 11 patients. We have demonstrated that TIPS is an effective procedure to control refractory complications of portal hypertension in elderly patients.

Thalidomide ameliorates portal hypertension via nitric oxide synthase independent reduced systolic blood pressure

PubMed Central

Theodorakis, Nicholas G; Wang, Yining N; Korshunov, Vyacheslav A; Maluccio, Mary A; Skill, Nicholas J

2015-01-01

AIM: Portal hypertension is a common complication of liver cirrhosis and significantly increases mortality and morbidity. Previous reports have suggested that the compound thalidomide attenuates portal hypertension (PHT). However, the mechanism for this action is not fully elucidated. One hypothesis is that thalidomide destabilizes tumor necrosis factor α (TNFα) mRNA and therefore diminishes TNFα induction of nitric oxide synthase (NOS) and the production of nitric oxide (NO). To examine this hypothesis, we utilized the murine partial portal vein ligation (PVL) PHT model in combination with endothelial or inducible NOS isoform gene knockout mice. METHODS: Wild type, inducible nitric oxide synthase (iNOS)-/- and endothelial nitric oxide synthase (eNOS)-/- mice received either PVL or sham surgery and were given either thalidomide or vehicle. Serum nitrate (total nitrate, NOx) was measured daily for 7 d as a surrogate of NO synthesis. Serum TNFα level was quantified by enzyme-linked immunosorbent assay. TNFα mRNA was quantified in liver and aorta tissue by reverse transcription-polymerase chain reaction. PHT was determined by recording splenic pulp pressure (SPP) and abdominal aortic flow after 0-7 d. Response to thalidomide was determined by measurement of SPP and mean arterial pressure (MAP). RESULTS: SPP, abdominal aortic flow (Qao) and plasma NOx were increased in wild type and iNOS-/- PVL mice when compared to sham operated control mice. In contrast, SPP, Qao and plasma NOx were not increased in eNOS-/- PVL mice when compared to sham controls. Serum TNFα level in both sham and PVL mice was below the detection limit of the commercial ELISA used. Therefore, the effect of thalidomide on serum TNFα levels was undetermined in wild type, eNOS-/- or iNOS-/- mice. Thalidomide acutely increased plasma NOx in wild type and eNOS-/- mice but not iNOS-/- mice. Moreover, thalidomide temporarily (0-90 min) decreased mean arterial pressure, SPP and Qao in wild type, eNOS-/- and iNOS-/- PVL mice, after which time levels returned to the respective baseline. CONCLUSION: Thalidomide does not reduce portal pressure in the murine PVL model by modulation of NO biosynthesis. Rather, thalidomide reduces PHT by decreasing MAP by an undetermined mechanism. PMID:25892862

Thalidomide ameliorates portal hypertension via nitric oxide synthase independent reduced systolic blood pressure.

PubMed

Theodorakis, Nicholas G; Wang, Yining N; Korshunov, Vyacheslav A; Maluccio, Mary A; Skill, Nicholas J

2015-04-14

Portal hypertension is a common complication of liver cirrhosis and significantly increases mortality and morbidity. Previous reports have suggested that the compound thalidomide attenuates portal hypertension (PHT). However, the mechanism for this action is not fully elucidated. One hypothesis is that thalidomide destabilizes tumor necrosis factor α (TNFα) mRNA and therefore diminishes TNFα induction of nitric oxide synthase (NOS) and the production of nitric oxide (NO). To examine this hypothesis, we utilized the murine partial portal vein ligation (PVL) PHT model in combination with endothelial or inducible NOS isoform gene knockout mice. Wild type, inducible nitric oxide synthase (iNOS)(-/-) and endothelial nitric oxide synthase (eNOS)(-/-) mice received either PVL or sham surgery and were given either thalidomide or vehicle. Serum nitrate (total nitrate, NOx) was measured daily for 7 d as a surrogate of NO synthesis. Serum TNFα level was quantified by enzyme-linked immunosorbent assay. TNFα mRNA was quantified in liver and aorta tissue by reverse transcription-polymerase chain reaction. PHT was determined by recording splenic pulp pressure (SPP) and abdominal aortic flow after 0-7 d. Response to thalidomide was determined by measurement of SPP and mean arterial pressure (MAP). SPP, abdominal aortic flow (Qao) and plasma NOx were increased in wild type and iNOS(-/-) PVL mice when compared to sham operated control mice. In contrast, SPP, Qao and plasma NOx were not increased in eNOS(-/-) PVL mice when compared to sham controls. Serum TNFα level in both sham and PVL mice was below the detection limit of the commercial ELISA used. Therefore, the effect of thalidomide on serum TNFα levels was undetermined in wild type, eNOS(-/-) or iNOS(-/-) mice. Thalidomide acutely increased plasma NOx in wild type and eNOS(-/-) mice but not iNOS(-/-) mice. Moreover, thalidomide temporarily (0-90 min) decreased mean arterial pressure, SPP and Qao in wild type, eNOS(-/-) and iNOS(-/-) PVL mice, after which time levels returned to the respective baseline. Thalidomide does not reduce portal pressure in the murine PVL model by modulation of NO biosynthesis. Rather, thalidomide reduces PHT by decreasing MAP by an undetermined mechanism.

Transjugular local thrombolysis with/without TIPS in patients with acute non-cirrhotic, non-malignant portal vein thrombosis.

PubMed

Klinger, Christoph; Riecken, Bettina; Schmidt, Arthur; De Gottardi, Andrea; Meier, Benjamin; Bosch, Jaime; Caca, Karel

2017-12-01

Therapeutic anticoagulation is the standard treatment in patients with acute non-cirrhotic portal vein thrombosis (PVT). In critically ill patients, anticoagulation only may not suffice to achive rapid and stable recanalization. This study evaluates efficacy and safety of transjugular interventional therapy in acute non-cirrhotic PVT. This retrospective study includes 17 consecutive patients with acute noncirrhotic, non-malignant PVT. Main indication for interventional therapy was imminent intestinal infarction (n=10). Treatment consisted of a combination of transjugular thrombectomy, local fibrinolysis and - depending on thrombus resolution - transjugular intrahepatic portosystemic shunt. Recanalization was successful in 94.1%. One- and two-year secondary PV patency rates were 88.2%. Major complications (n=3) resolved spontaneously in all but one patient (heparin induced thrombocytopenia type 2 with intestinal infarction). Symptoms improved in all patients. However, segmental bowel resection had to be performed in two (11.8%). During a median follow-up of 28.6 months, no patient experienced portal hypertensive complications. Presence of JAK2 V617F mutation predicted both short-term and long-term technical success. Transjugular recanalization is safe and effective in patients with acute non-cirrhotic, non-malignant PVT. It should be considered especially in patients with imminent bowel infarction and low likelihood of recanalization following therapeutic anticoagulation. Patients with JAK2 mutation ought to be followed meticulously. Copyright © 2017 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

[Clinical and laboratory characteristics of patients with pulmonary hypertension and pulmonary vascular complications hospitalized at the Instituto Nacional de Salud del Niño].

PubMed

Ormeño Julca, Alexis Jose; Alvarez Murillo, Carlos Melchor; Amoretti Alvino, Pedro Miguel; Florian Florian, Angel Aladino; Castro Johanson, Rosa Aurora; Celi Perez, Maria Danisa; Huamán Prado, Olga Rocío

2017-01-01

The hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHN) are distinct pulmonary vascular complications of portal hypertension (PHT) and are associated with increased morbidity and mortality. To describe the clinical and laboratory characteristics of patients with pulmonary hypertension and pulmonary vascular complications hospitalized at the Instituto Nacional de Salud del Niño. We included patients with HTP hospitalized from January 2012 to June 2013 and that during its evolution progressed with SHP or HTPP. For analysis, they were divided into a first group of patients with liver cirrhosis and a second group with extrahepatic portal vein obstruction. Of 22 patients with HPT 45.5% were male and the age range was between 1 month and 17 years. The etiology in the group of cirrhosis (n=14) was: autoimmune hepatitis (35.7%), cryptogenic cirrhosis (35.7%), inborn error of metabolism (14.3%), chronic viral hepatitis C (7.15%) virus and atresia extra-hepatic bile ducts (7.15%). Pulmonary vascular complications more frequently occurred in patients with liver cirrhosis (1 case of HPS and a case of PPHTN). They most often dyspnea, asthenia, edema, malnutrition, ascites, hypersplenism and gastrointestinal bleeding from esophageal varices was found. Also, they had elevated ALT values, alkaline phosphatase and serum albumin values decreased. In children with pulmonary hypertension, pulmonary vascular complications are rare. In the evaluation of these patients pulse oximetry should be included to detect hypoxemia and ubsequently a Doppler echocardiography and contrast echocardiography necessary. Dueto the finding of systolic pulmonary hypertension it is necessary to perform right heart catheterization.

Complex bile duct injuries: management

PubMed Central

Ardiles, V.; Pekolj, J.

2008-01-01

Background. Laparoscopic cholecystectomy is the present treatment of choice for patients with gallbladder stones, despite its being associated with a higher incidence of biliary injuries compared with the open procedure. Injuries occurring during the laparoscopic approach seem to be more complex. A complex biliary injury is a disease that is difficult to diagnose and treat. We considered complex injuries: 1) injuries that involve the confluence; 2) injuries in which repair attempts have failed; 3) any bile duct injury associated with a vascular injury; 4) or any biliary injury in association with portal hypertension or secondary biliary cirrhosis. The present review is an evaluation of our experience in the treatment of these complex biliary injuries and an analysis of the international literature on the management of patients. PMID:18695753

Laparoendoscopic transgastric histoacryl injection of gastric varices: a new surgical approach.

PubMed

Kassem, Mohamed I; El-Haddad, Hani M; El-Bahrawi, Hassan A

2013-02-01

Gastric varices (GVs) are a common finding in Egyptian patients with portal hypertension due to cirrhosis or schistosomal hepatic fibrosis. These patients present with an acute attack or history of hematemesis. Endoscopic histoacryl injection is the standard treatment in Egypt; however, because of technical difficulties it is possible to inject only a little amount of this material, as it may endanger the channels of the flexible endoscope. We thought of a new surgical laparoendoscopic technique to obviate the need for repeated endoscopies and complete obliteration of GVs. This study was conducted on 20 patients with portal hypertension and GVs. After the patient was placed under general anesthesia, a small gastrostomy was done in the anterior gastric wall through which a 10-mm trocar was inserted for the laparoscopic camera. Injection of GVs was done via a spinal needle or a central venous line needle inserted directly. Injection of an adequate amount of histoacryl was done under direct vision. This study was conducted from July 2009 to August 2011 on 20 patients with GVs. The age range was from 22 to 56 years, with a mean age of 39.8±7.85 years. There were 14 men (70%) and 6 women (30%). Fourteen patients (70%) showed complete obliteration of GVs after one session of treatment, whereas 6 patients (30%) had unsatisfactory results and were subjected to another session. GVs were completely obliterated after the second session in 4 patients. Two cases of recurrence of GVs were operated on. This new technique enabled us to inject GVs with a suitable amount of glue material under direct vision without harming the endoscope. Use of this procedure is recommended in patients fit for surgery and those who had failed endoscopic injection sclerotherapy.

[Portal thrombosis, common bile duct varices and cholestasis].

PubMed

Agüera Arroyo, B; Pérez Durán, M A; Montero Alvarez, J L; Navarro Jarabo, J M; Calero Ayala, B; Miño Fugarolas, G

1996-03-01

A case of cholestasis in a young patient with portal cavernomatosis is reported. This clinical picture is very infrequent and appears as a consequence of extrinsic compression on the common bile duct due to which the derivative venous collaterals. There does not appear to be any relationship between the intensity of the morphologic alteration of the biliary tract and the level of portal hypertension and the degree of extrahepatic obstruction. Diagnosis was fundamentally achieved by arteriography and retrograde cholangiography with differential diagnosis with the previously mentioned diseases being required. Chronic cholestasis advises derivative surgery in which difficulties may be found due to the presence of thick collaterals in the hepatic pedicle as occurred in this patient.

Medical Surveillance Monthly Report. Volume 21, Number 5

DTIC Science & Technology

2014-05-01

11.5 1,495 81.1 722 Intervertebral disc disorders 1,832 11.4 976 53.3 401 Essential hypertension 1,808 11.2 704 38.9 723 Other disorders of cervical...Accessed 14 May 2014. 5. Department of the Navy. Marine Corps Order 1500.54A. Subject: Marine Corps Martial Arts Program. http://www.marines.mil/ Portals ...Arts Program. 2011. Washington, DC: Headquarters, U.S. Marine Corps. http://www. marines.mil/ Portals /59/Publications/MCRP%20 3-02B%20PT%201.pdf

Extension of a Computer Assisted Decision Support (CADS) Study to Improve Outcomes in Patients with Type 2 DM Treated by Primary Care Providers (short title, CADS-X). Addendum

DTIC Science & Technology

2014-12-01

Hypertension is one of the most common co-morbidities associated with DM and substantially contributes to the macrovascular disease that occurs in...After Numera terminated the contract to provide glucometer download support Estenda activated the patient portal , Diabetes Mellitus Everywhere...several patients. Problem areas include having to download JAVA with first upload and accessing the DME portal . d. PO has downloaded glucose

[Congenital portosystemic shunt. The Abernethy malformation].

PubMed

Avila, L F; Luis, A L; Encinas, J L; Hernández, F; Olivares, P; Fernández Cuadrado, J; Hierro, L; Jara, P; López Santamaría, M; Tovar, J A

2006-10-01

Congenital portosystemic shunt (CEPS) is a rare condition that was first reported by John Abernethy in 1793. Two types of CEPS are described: type I (side to end anastomosis) or congenital absence of the portal vein, and type II (side to side anastomosis) with portal vein supply partially conserved. Type I CEPS is usually seen in girls and associates multiple malformations as polysplenia, malrotation, and cardiac anomalies. Type II is even rarer with no sex preference and no malformations associated. Hepatic encephalopathy is a common complication of both types in adulthood. Liver transplantation is the only effective treatment for symptomatic type I CEPS. A therapeutic approach for type II could be surgical closure of the shunt. To analyse our experience in diagnosis and management of portosystemic shunts. We report 4 cases of CEPS (3 type I and 1 type II) diagnosed between January-1997 and March-2005 in our department. We present 4 patients with ages at diagnosis ranging from 0 to 28 months, 3 type I CEPS (2 boys and 1 girl) and 1 boy type II. The type I girl was prenatally diagnosed at 12 weeks of gestation. Initial clinical signs in type 1 boys were splenomegaly and hypersplenism, both with normal pondo-statural growth. No polysplenia or cardiac anomalies were assessed. One of them presented mild developmental delay, dismorphic features and facial telangiectasias. He had normal coagulation tests with chronic hepatic dysfunction (high transaminases) and regenerative nodular lesions were seen by imaging techniques. The other type I patient had hypoprothrombinemia, tendency to capillary bleeding (haematomas and epistaxis) with preserved liver function. Both patients have developed mild portal hypertension and present steatosis signs at liver biopsy. The type I girl presents a 21 trisomy and associates a cardiac anomaly (interauricular communication). Her hepatic function test are normal but liver calcifications can be seen by ultrasound. Type II child associates hypospadias but he has no clinical sigh or symptom related to the shunt. In our three cases diagnosis was suggested by conventional and Doppler ultrasound and confirmed by angio-resonance imaging. All our patients are included in a meticulous clinical and radiological follow-up with no need of surgical treatment for the shunt until now. Although diagnosis of these malformations could be casual we have to think about CEPS in children presenting unspecific liver disease. Magnetic angio-resonance imaging is actually the best diagnosis methods for CEPS. These patients have a high risk for developing hepatic encephalopathy and portal hypertension, so a careful follow-up is required although surgery is not usually needed until adulthood.

Distinctive aspects of peptic ulcer disease, Dieulafoy's lesion, and Mallory-Weiss syndrome in patients with advanced alcoholic liver disease or cirrhosis

PubMed Central

Nojkov, Borko; Cappell, Mitchell S

2016-01-01

AIM: To systematically review the data on distinctive aspects of peptic ulcer disease (PUD), Dieulafoy’s lesion (DL), and Mallory-Weiss syndrome (MWS) in patients with advanced alcoholic liver disease (aALD), including alcoholic hepatitis or alcoholic cirrhosis. METHODS: Computerized literature search performed via PubMed using the following medical subject heading terms and keywords: “alcoholic liver disease”, “alcoholic hepatitis”,“ alcoholic cirrhosis”, “cirrhosis”, “liver disease”, “upper gastrointestinal bleeding”, “non-variceal upper gastrointestinal bleeding”, “PUD”, ‘‘DL’’, ‘‘Mallory-Weiss tear”, and “MWS’’. RESULTS: While the majority of acute gastrointestinal (GI) bleeding with aALD is related to portal hypertension, about 30%-40% of acute GI bleeding in patients with aALD is unrelated to portal hypertension. Such bleeding constitutes an important complication of aALD because of its frequency, severity, and associated mortality. Patients with cirrhosis have a markedly increased risk of PUD, which further increases with the progression of cirrhosis. Patients with cirrhosis or aALD and peptic ulcer bleeding (PUB) have worse clinical outcomes than other patients with PUB, including uncontrolled bleeding, rebleeding, and mortality. Alcohol consumption, nonsteroidal anti-inflammatory drug use, and portal hypertension may have a pathogenic role in the development of PUD in patients with aALD. Limited data suggest that Helicobacter pylori does not play a significant role in the pathogenesis of PUD in most cirrhotic patients. The frequency of bleeding from DL appears to be increased in patients with aALD. DL may be associated with an especially high mortality in these patients. MWS is strongly associated with heavy alcohol consumption from binge drinking or chronic alcoholism, and is associated with aALD. Patients with aALD have more severe MWS bleeding and are more likely to rebleed when compared to non-cirrhotics. Pre-endoscopic management of acute GI bleeding in patients with aALD unrelated to portal hypertension is similar to the management of aALD patients with GI bleeding from portal hypertension, because clinical distinction before endoscopy is difficult. Most patients require intensive care unit admission and attention to avoid over-transfusion, to correct electrolyte abnormalities and coagulopathies, and to administer antibiotic prophylaxis. Alcoholics should receive thiamine and be closely monitored for symptoms of alcohol withdrawal. Prompt endoscopy, after initial resuscitation, is essential to diagnose and appropriately treat these patients. Generally, the same endoscopic hemostatic techniques are used in patients bleeding from PUD, DL, or MWS in patients with aALD as in the general population. CONCLUSION: Nonvariceal upper GI bleeding in patients with aALD has clinically important differences from that in the general population without aALD, including: more frequent and more severe bleeding from PUD, DL, or MWS. PMID:26755890

Circulating CO3-610, a degradation product of collagen III, closely reflects liver collagen and portal pressure in rats with fibrosis

PubMed Central

2011-01-01

Background Hepatic fibrosis is characterized by intense tissue remodeling, mainly driven by matrix metalloproteinases. We previously identified CO3-610, a type III collagen neoepitope generated by matrix metalloproteinase (MMP)-9, and tested its performance as a fibrosis marker in rats with bile-duct ligation. In this study, we assessed whether CO3-610 could be used as a surrogate biomarker of liver fibrosis and portal hypertension in carbon tetrachloride-induced experimental fibrosis. Results For this study, 68 Wistar rats were used. Serum CO3-610 was measured by ELISA. Liver fibrosis was quantified by Sirius red staining. Serum hyaluronic acid (HA) was measured with a binding-protein assay. Gene expression of collagens I and III, Mmp2 and Mmp9, and tissue inhibitors of matrix metalloproteinase 1 (Timp1) and 2(Timp2) was quantified by PCR. Hemodynamic measurements were taken in a subgroup of animals. A close direct relationship was found between serum CO3-610 and hepatic collagen content (r = 0.78; P < 0.001), superior to that found for serum HA (r = 0.49; P < 0.05). CO3-610 levels in rats with severe fibrosis (43.5 ± 3.3 ng/mL, P < 0.001) and cirrhosis (60.6 ± 4.3 ng/mL, P < 0.001) were significantly higher than those in control animals (26.6 ± 1.3 ng/mL). Importantly, a highly significant relationship was found between serum CO3-610 and portal hypertension (r = 0.84; P < 0.001). Liver Mmp9 expression increased significantly in fibrotic animals but decreased to control levels in cirrhotic ones. Conclusions Circulating CO3-610 behaves as a reliable indicator of hepatic remodeling and portal hypertension in experimental fibrosis. This peptide could ultimately be a useful marker for the management of liver disease in patients. PMID:21813019

Portal Hypertension

MedlinePlus

... Pill Identifier Commonly searched drugs Aspirin Metformin Warfarin Tramadol Lactulose Ranitidine News & Commentary Recent News Afib and Weight AHA: Using Electricity to Test Your Risk for Heart Failure Close Siblings Can Ease the Pain of Family Conflict Don't Turn Into a ...

Anatomy of spontaneous splenorenal and gastrorenal venous anastomoses. Review of the literature.

PubMed

Wind, P; Alves, A; Chevallier, J M; Gillot, C; Sales, J P; Sauvanet, A; Cuénod, C A; Vilgrain, V; Cugnenc, P H; Delmas, V

1998-01-01

Portal hypertension is characterised by the development of a collateral portocaval circulation. Among these venous reroutings, some are situated posteriorly in the left subphrenic compartment. These are the spontaneous splenorenal and gastrorenal anastomoses. Their incidence is estimated at around 16%. On the one hand, there are the direct shunts, which anastomose the spelling v. to the left renal v., of an anecdotal nature, and on the other the spontaneous indirect splenorenal shunts, characterised by the presence of a complete neurovascular pedicle traversing the gastrophrenic ligament. This relates to the gastric collateral v., which is connected to the left renal v. via the inferior v. of the left crus of the diaphragm and the middle capsular v., hence the name "gastro-phreno-capsulo-renal shunt". At an advanced stage of portal hypertension these splenorenal shunts may acquire a major caliber and behave like actual surgical shunts.

Medical and Endoscopic Management of Gastric Varices

PubMed Central

Al-Osaimi, Abdullah M. S.; Caldwell, Stephen H.

2011-01-01

In the past 20 years, our understanding of the pathophysiology and management options among patients with gastric varices (GV) has changed significantly. GV are the most common cause of upper gastrointestinal bleeding in patients with portal hypertension after esophageal varices (EV) and generally have more severe bleeding than EV. In the United States, the majority of GV patients have underlying portal hypertension rather than splenic vein thrombosis. The widely used classifications are the Sarin Endoscopic Classification and the Japanese Vascular Classifications. The former is based on the endoscopic appearance and location of the varices, while the Japanese classification is based on the underlying vascular anatomy. In this article, the authors address the current concepts of classification, epidemiology, pathophysiology, and emerging management options of gastric varices. They describe the stepwise approach to patients with gastric varices, including the different available modalities, and the pearls, pitfalls, and stop-gap measures useful in managing patients with gastric variceal bleed. PMID:22942544

[Glucose tolerance and insulinemia in patients with hepatic cirrhosis and portal hypertension treated by portacaval anastomosis].

PubMed

Postiglione, A; Casaretti, B; Masciariello, S; Riccardi, G; Perrotti, N; Lamenza, F; Porcellini, M; Mattioli, P L

1979-02-28

Development of diabetes mellitus is a common complication of side to side porta-caval anastomosis (PCA). Five patients with liver cirrhosis and portal hypertension have been studied with intravehous (IVGTT, 0,5 g/Kg B.W.) and oral (OGTT, 1 g/Kg B.W.) glucose tolerance tests before and three weeks after PCA. Fasting plasma glucose was 84 +/- 7 before and 87 +/- 3 mg/dl after PCA. Fasting IRI increased from 17 +/- 3 to 31 +/- 6 microU/ml. The pattern of plasma glucose and IRI response to IVGTT did not change after PCA. Plasma glucose resonse to OGTT after PCA showed only an earlier rise at 60 instead of 90 minutes, whereas IRI resonse (area under the insulin curve) was significantly enhanced (from 12.4 to 19.8 U/l, p < 0.05). These data suggest a role of gut polipeptides in determining hyperinsulinemia and insulin resistence in PCA patients.

Bleeding due to ectopic varices in a urinary diversion: A multidisciplinary diagnostic and therapeutic challenge

PubMed Central

Acosta, Eduardo Mariano Albers; Reyes, Alfonsi Friera; Menéndez, Ricardo Brime

2015-01-01

The ectopic varices in patients with portal hypertension are those that occur at any level of the gastrointestinal (GI) tract, regardless of the varices that occur at the esophageal level. These ectopic varices account for 2–5% of the causes of GI bleeding varices. The risk of bleeding is quadrupled compared to the esophagogastric area, with a mortality of up to 40%. The transjugular intrahepatic portosystemic shunt, should be considered in cases secondary to recurrent bleeding varices. We present a case report of an urological emergency of bleeding in a urinary diversion secondary to ectopic varices successfully treated through the placement of transjugular intrahepatic portosystemic shunt. The condition described here is rare, but important, as it can be a life-threatening complication of portal hypertension. This kind of complication should be known by urologic surgeons managing patients with urinary diversions. PMID:26834901

Transient elastography for the diagnosis of liver fibrosis.

PubMed

de Lédinghen, Victor; Vergniol, Julien

2010-11-01

Transient elastography (FibroScan(®)) is a noninvasive method proposed for the assessment of liver fibrosis in patients with chronic liver diseases by measuring liver stiffness. It can be easily performed at the bedside or in the outpatient clinic with immediate results and good reproducibility. FibroScan is validated for the diagnosis of significant fibrosis and cirrhosis in chronic hepatitis C, in recurrence of hepatitis C after liver transplantation, in co-infected HIV-HCV patients, in chronic hepatitis B, in chronic cholestatic diseases, in alcoholic disease and in nonalcoholic fatty liver disease. FibroScan is an excellent tool for the early detection of cirrhosis and for the evaluation of portal hypertension, and may have prognostic value in this setting. FibroScan evaluates liver stiffness, which is related to fibrosis, but also inflammation and portal hypertension. Therefore, FibroScan values have to be interpreted according to clinical, biological and morphological data.

Cardiovascular effects of Helichrysum ceres S Moore [Asteraceae] ethanolic leaf extract in some experimental animal paradigms.

PubMed

Musabayane, Cephas T; Kamadyaapa, Dave R; Gondwe, Mavuto; Moodley, Kogi; Ojewole, John A O

2008-01-01

The aim of this study was to examine some in vivo and in vitro cardiovascular effects of Helichrysum ceres leaf ethanolic extract (HCE) in experimental animal paradigms. The acute effects of HCE on blood pressure were studied in anaesthetised normotensive male Wistar rats challenged with intravenous hypotonic saline infusion after a 3.5-hour equilibration for four hours of one-hour control, 1.5-hour treatment and 1.5-hour recovery periods. HCE was added to the infusate during the treatment period. Sub-chronic hypotensive effects of HCE were examined in weanling Dahl saltsensitive (DSS) genetically hypertensive rats, which progressively develop hypertension with age, treated with HCE (80 mg/kg) every third consecutive day for seven weeks. Isolated atrial muscle strips, portal veins and descending thoracic aortic rings of healthy normotensive Wistar rats were used to investigate the vascular effects of HCE. Acute HCE administration caused a significant (p < 0.05) fall in blood pressure in the normotensive anaesthetised Wistar rats. DSS hypertensive rats treated with HCE displayed low arterial blood pressure and heart rate values from weeks five to seven. HCE produced concentrationdependent negative inotropic and chronotropic effects on rat isolated electrically driven left, and spontaneously beating right atrial muscle preparations, respectively. HCE also evoked concentration-dependent relaxation responses of endothelium-intact aortic rings and portal veins isolated from healthy normotensive Wistar rats. The vasorelaxant effects of HCE in intact aortic rings were significantly reduced, but not completely abolished by adding endothelial- derived factor (EDRF) inhibitor, L-NAME, suggesting that the vasorelaxant effect of the extract is mediated via EDRF-dependent and independent mechanisms. The results of the study suggest that the hypotensive action of HCE is elicited, in part, directly by decreasing myocardial contractile performance and total peripheral vascular resistance due to its negative inotropic and chronotropic effects on rat isolated atrial muscle strips; and vasorelaxant effects on isolated vascular smooth muscles. The observed cardiovascular effects of HCE partly support the basis for its use in the management of high blood pressure in folkloric medicine.

Cardiovascular effects of Helichrysum ceres S Moore [Asteraceae] ethanolic leaf extract in some experimental animal paradigms

PubMed Central

Musabayane, Cephas T; Kamadyaapa, Dave R; Gondwe, Mavuto; Moodley, Kogi; Ojewole, John AO

2008-01-01

Summary Summary The aim of this study was to examine some in vivo and in vitro cardiovascular effects of Helichrysum ceres leaf ethanolic extract (HCE) in experimental animal paradigms. The acute effects of HCE on blood pressure were studied in anaesthetised normotensive male Wistar rats challenged with intravenous hypotonic saline infusion after a 3.5-hour equilibration for four hours of one-hour control, 1.5-hour treatment and 1.5-hour recovery periods. HCE was added to the infusate during the treatment period. Sub-chronic hypotensive effects of HCE were examined in weanling Dahl salt-sensitive (DSS) genetically hypertensive rats, which progressively develop hypertension with age, treated with HCE (80 mg/kg) every third consecutive day for seven weeks. Isolated atrial muscle strips, portal veins and descending thoracic aortic rings of healthy normotensive Wistar rats were used to investigate the vascular effects of HCE. Acute HCE administration caused a significant (p < 0.05) fall in blood pressure in the normotensive anaesthetised Wistar rats. DSS hypertensive rats treated with HCE displayed low arterial blood pressure and heart rate values from weeks five to seven. HCE produced concentration-dependent negative inotropic and chronotropic effects on rat isolated electrically driven left, and spontaneously beating right atrial muscle preparations, respectively. HCE also evoked concentration-dependent relaxation responses of endothelium-intact aortic rings and portal veins isolated from healthy normotensive Wistar rats. The vasorelaxant effects of HCE in intact aortic rings were significantly reduced, but not completely abolished by adding endothelial-derived factor (EDRF) inhibitor, L-NAME, suggesting that the vasorelaxant effect of the extract is mediated via EDRF-dependent and independent mechanisms. The results of the study suggest that the hypotensive action of HCE is elicited, in part, directly by decreasing myocardial contractile performance and total peripheral vascular resistance due to its negative inotropic and chronotropic effects on rat isolated atrial muscle strips; and vasorelaxant effects on isolated vascular smooth muscles. The observed cardiovascular effects of HCE partly support the basis for its use in the management of high blood pressure in folkloric medicine. PMID:18997985

Angiostrongylus vasorum Causing Severe Granulomatous Hepatitis with Concurrent Multiple Acquired PSS.

PubMed

Cook, Simon; Priestnall, Simon L; Blake, Damer; Meeson, Richard L

2015-01-01

A 14 mo old female Jack Russell terrier presented with a 12 hr history of vomiting and inappetence. She was subsequently diagnosed with multiple acquired portosystemic shunts during an exploratory celiotomy. Gross and histopathological hepatic abnormalities were consistent with chronic disease, including features suggestive of portal hypertension that was potentially caused by migrating and resident Angiostrongylus vasorum larvae. Fecal analysis and polymerase chain reaction of hepatic tissue confirmed the presence of Angiostrongylus vasorum . The dog recovered clinically following empirical treatment and supportive care. A lack of parasite burden was confirmed 9 wk postdiagnosis; however, serum biochemical analysis at that time was suggestive of ongoing hepatic dysfunction.

Insight into congenital absence of the portal vein: is it rare?

PubMed

Hu, Guo-Hua; Shen, Lai-Gen; Yang, Jin; Mei, Jin-Hua; Zhu, Yue-Feng

2008-10-21

Congenital absence of portal vein (CAPV) was a rare event in the past. However, the number of detected CAPV cases has increased in recent years because of advances in imaging techniques. Patients with CAPV present with portal hypertension (PH) or portosystemic encephalopathy (PSE), but these conditions rarely occur until the patients grow up or become old. The patients usually visit doctors for the complications of venous shunts, hepatic or cardiac abnormalities detected by ultrasonography (US), computed tomography (CT) and magnetic resonance imaging (MRI). The etiology of this disease is not clear, but most investigators consider that it is associated with abnormal embryologic development of the portal vein. Usually, surgical intervention can relieve the symptoms and prevent occurrence of complications in CAPV patients. Moreover, its management should be stressed on a case-by-case basis, depending on the type or anatomy of the disease, as well as the symptoms and clinical conditions of the patient.

A Case of Extrahepatic Portal Vein Aneurysm Complicated by Acute Thrombosis.

PubMed

Kim, Hye Jin; Ha, Tae-Yong; Ko, Gi-Young; Noh, Minsu; Kwon, Tae-Won; Cho, Yong-Pil; Lee, Sung-Gyu

2017-08-01

Portal vein (PV) aneurysm is a rare disease entity, and the optimal strategy for its management remains unclear. We describe the case of a 34-year-old woman who was incidentally diagnosed with an asymptomatic extrahepatic PV aneurysm. Although expectant management with regular follow-up and surveillance imaging was adopted, the PV aneurysm progressed into a symptomatic type, accompanied by complications of acute thrombosis. Hence, an aneurysm excision with interposition bypass was performed. Her postoperative recovery was rapid and uneventful, with liver function test results within normal ranges and normal portal flow on color Doppler ultrasonography and contrast-enhanced computed tomography. The incidence of thromboses among the reported PV aneurysm cases may be markedly high, and early surgical intervention for low-risk patients may therefore be required to prevent the development of portal hypertension with clinically severe consequences. Copyright © 2017 Elsevier Inc. All rights reserved.

Intrapulmonary vascular dilatation evaluated by 99mTc-MAA scintigraphy and its association with portal hypertension in schistosomiasis.

PubMed

Queirós, Andréa Simone Siqueira de; Brandão, Simone Cristina Soares; Domingues, Ana Lúcia Coutinho; Macedo, Liana Gonçalves; Ourem, Maira Souto; Lopes, Edmundo Pessoa Almeida

2014-06-01

Portal hypertension is responsible for various complications in patients with schistosomiasis, among them intrapulmonary vascular dilations (IPVD). In cirrhotic patients the presence of IPVD is a sign of poor prognosis, but in patients with hepatosplenic schistosomiasis (HSS) there are no studies assessing the significance of this change. The aim of this study was to evaluate the occurrence of IPVD through 99mTc-MAA scintigraphy in patients with HSS and its relationship with clinical, laboratory, endoscopic and ultrasound parameters. Cross-sectional study evaluating 51 patients with HSS. Patients were diagnosed with IPVD when the brain uptake of 99mTc-MAA was higher than 6%. Subsequently, they were divided according to presence (G1) or absence (G2) of IPVD and variables were compared between groups. Overall, 51 patients with mean age of 56±12 years were assessed. IPVD was observed in 31 patients (60%). There was no statistically significant differences between groups when clinical, laboratory and endoscopic parameters were compared. Regarding ultrasound parameters, the splenic vein diameter was smaller in G1 (0.9 ± 0.3 cm) compared to G2 (1.2 ± 0.4 cm), p=0.029. In patients with HSS, the occurrence of IPVD by 99mTc-MAA scintigraphy was high and was associated with lower splenic vein diameter, which can be a mechanism of vascular protection against portal hypertension. However, more studies are needed to determine the clinical significance of the early diagnosis and natural evolution of IPVD in this population.

Transjugular intrahepatic porto-systemic shunt in the elderly: Palliation for complications of portal hypertension

PubMed Central

Syed, Mubin I; Karsan, Hetal; Ferral, Hector; Shaikh, Azim; Waheed, Uzma; Akhter, Talal; Gabbard, Alan; Morar, Kamal; Tyrrell, Robert

2012-01-01

AIM: To present a dedicated series of transjugular intrahepatic porto-systemic shunts (TIPS) in the elderly since data is sparse on this population group. METHODS: A retrospective review was performed of patients at least 65 years of age who underwent TIPS at our institutions between 1997 and 2010. Twenty-five patients were referred for TIPS. We deemed that 2 patients were not considered appropriate candidates due to their markedly advanced liver disease. Of the 23 patients suitable for TIPS, the indications for TIPS placement was portal hypertension complicated by refractory ascites alone (n = 9), hepatic hydrothorax alone (n = 2), refractory ascites and hydrothorax (n = 1), gastrointestinal bleeding alone (n = 8), gastrointestinal bleeding and ascites (n = 3). RESULTS: Of these 23 attempted TIPS procedure patients, 21 patients had technically successful TIPS procedures. A total of 29 out of 32 TIPS procedures including revisions were successful in 21 patients with a mean age of 72.1 years (range 65-82 years). Three of the procedures were unsuccessful attempts at TIPS and 8 procedures were successful revisions of our existing TIPS. Sixteen of 21 patients who underwent successful TIPS (excluding 5 patients lost to follow-up) were followed for a mean of 14.7 mo. Ascites and/or hydrothorax was controlled following technically successful procedures in 12 of 13 patients. Bleeding was controlled following technically successful procedures in 10 out of 11 patients. CONCLUSION: We have demonstrated that TIPS is an effective procedure to control refractory complications of portal hypertension in elderly patients. PMID:22400084

Percutaneous transhepatic embolization of gastroesophageal varices combined with partial splenic embolization for the treatment of variceal bleeding and hypersplenism

PubMed Central

Gong, Wei-Dong; Xue, Ke; Chu, Yuan-Kui; Wang, Qing; Yang, Wei; Quan, Hui; Yang, Peng; Wang, Zhi-Min; Wu, Zhi-Qun

2015-01-01

This study aims to evaluate the therapeutic results of percutaneous transhepatic embolization of gastroesophageal varices combined with partial splenic embolization in patients with liver cirrhosis, and to explore the role of this minimally invasive treatment as an alternative to surgery. 25 patients with liver cirrhosis were received percutaneous transhepatic embolization of gastroesophageal varices combined with partial splenic embolization. Another 25 patients with liver cirrhosis underwent Hassab’s operation. They were followed up, and received endoscopy, B ultrasound, liver function and hematologic examination at 24 months after the therapy. In minimal invasive group, before treatment and after 24 month following up after treatment, improved varices, improved portal hypertension and improved hypersplenism were showed comparing with the surgery group, and that they were measured by endoscopic visualization, ultrasound and blood counts. the white blood cell and platelet count were 2.33±0.65 (109/L) and 3.63±1.05 (1010/L), 7.98±3.0 (109/L) and 16.3±9.10 (1010/L) (P<0.05); the diameter of the portal vein were 1.47±0.25 cm, 1.31±0.23 cm (P<0.05). Esophageal varices passed from grade III to lower grade II in 11 patients, and from grade II to lower grade I in 6 patients at 24 month following up. In surgical group, the white blood cell and platelet count were 2.2±0.60 (109/L), 4.1±1.25 (1010/L) before treatment; 9.3±2.56 (109/L), 32.1±12.47 (1010/L) after the treatment at 24 month following up (P<0.05). The diameter of the portal vein were 1.43±0.22 cm before the treatment and 1.28±0.18 cm after the treatment (P<0.05). Esophageal varices passed from grade III to lower grade II in 13 patients, and from grade II to lower grade I in 7 patients. The combination of PGEV and PSE can be considered as an option for the treatment of variceal bleeding with hypersplenism. PMID:26770628

Ascites: A Common Problem in People with Cirrhosis

MedlinePlus

... liver (portal hypertension) and a decrease in liver function caused by scarring of the liver, i.e., cirrhosis. Symptoms Most patients who develop ascites notice abdominal distension and rapid weight gain. Some people also develop swelling of ankles and ...

[Diagnostic of ascites due to portal hypertension: accuracy of the serum-ascites albumin gradient and protein analises in ascitic fluid].

PubMed

Rodríguez Vargas, Brainy Omar; Monge Salgado, Eduardo; Montes Teves, Pedro; Salazar Ventura, Sonia; Guzmán Calderón, Edson

2014-01-01

To evaluate the diagnostic accuracy of the Serum-Ascites Albumin Gradient (GASA), Protein Concentration in the Ascitic Fluid (PTLA), Albumin Concentration in the ascitic fluid (CAA) and the Protein Ascites/Serum Ratio (IPAS) for the diagnosis of ascites due to portal hypertension. it was an observational and retrospective study of validation of diagnostic tests. The study population was patients from a National Public Health Hospital Daniel Alcides Carrion of Callao, Peru, during the period January to December of 2012, patients over 15 years old with a diagnosis of ascites which samples were taken for study by paracentesis with an standard technique, it was analyzed total protein and albumin, as well as study of total protein and albumin in blood. We obtained the diagnostic accuracy, sensitivity, specificity, PPV and NPV of the Serum-Ascites Albumin Gradient (GASA), Protein Concentration in the Ascitic Fluid (PTLA), Albumin Concentration in the ascitic fluid (CAA) and the Protein Ascites/Serum Ratio (IPAS) for the diagnosis of ascites due to portal hypertension. To determine ascites by HTP as diagnostic tests we took into account: GASA >= 1.1, PTLA <2.5, CAA <1.1 or IPAS< 0.5. There were 126 patients diagnosed with ascites, 10 patients was excluded for having incomplete data. Of the 116 patients, the average age was 53.03 +/- 15.73 years old, male 65 (56%) and female 51 (44%). 61 (52%) had ascites due to portal hypertension from liver cirrhosis, and 55 (48%) of ascites due to NO HTP. The sensitivity and specificity for GASA was 93% and 47% respectively, for PTLA was 80% and 89% respectively, for CAA was 85% and 87% respectively and for the IPAS was 83% and 80% respectively. The área under the ROC curve for GASA was 0.70, ATPL was 0.84, IPAS was 0.81 and CAA was 0.86, we found statistically significant differences between GASA compared to the other three parameters (p<0.01 ). The diagnostic accuracy of CAA, ATPL and IPAS is higher than the GASA to discriminate between ascites due to HTP or NO HTP, so that they could be used in clinical practice alone or together to achieve a diagnostic approach more successful.

Pulmonary complications of cirrhosis.

PubMed

Singh, C; Sager, J S

2009-07-01

Advanced liver disease and portal hypertension produce various intrathoracic complications that involve the pleural space, the lung parenchyma, and the pulmonary circulation. Dyspnea and arterial hypoxemia are the most common symptoms and signs in patients with such complications. This article focuses on the diagnosis and management of hepatopulmonary syndrome, portopulmonary hypertension, and hepatic hydrothorax. All are pulmonary processes associated with end-stage liver disease that lead to significant morbidity and affect the quality of life of patients who are suffering from liver cirrhosis.

[Elephantiasis nostras verrucosa in a patient with major depressive disorder].

PubMed

Simón Llanes, J; Coll Vilar, I; Tamarit Francés, C; Niubó de Castro, I

2012-01-01

Elephantiasis nostras verrucosa is a rare condition characterised by papules, verrucous lesions, fibrosis and deformity of the affected area. It is caused by chronic lymphedema that could be congenital or produced by a non-associated infection (such as tuberculosis, mycotic infection, syphilis), surgery, radiotherapy, trauma, neoplastic obstruction, obesity, portal hypertension, or congestive heart failure. There is no standard treatment for this rare skin disorder. Depending on the cause and the severity, the treatment can be medical or surgical. We report the case of a man seen in our hospital with a major depression and elephantiasis nostras verrucosa skin lesions on both legs, who was successfully treated with surgical debridement and conservative measures. Copyright © 2011 Elsevier España, S.L. y SEMERGEN. All rights reserved.

Mean Normal Portal Vein Diameter Using Sonography among Clients Coming to Radiology Department of Jimma University Hospital, Southwest Ethiopia.

PubMed

Geleto, Gemechu; Getnet, Wondim; Tewelde, Tsegaye

2016-05-01

Mean portal vein diameter is considered as the best indicator for portal hypertension. However, the cutoff point differs from study to study (above 10-15 mm) despite the existence of normal mean portal vein diameter between 10-15 mm in different settings.This implies the existence of limited evidence on normal portal vein diameter for all populations in all countries prior to setting the cutoff points. Therefore, the aim of this study was sonographic assessment of normal mean portal vein diameter among patients referred to The Department of Radiology in Jimma University Hospital. A facility based cross-sectional study was conducted from November to December 2014 at Jimma University Hospital on a total of 195 clients. Data about portal vein diameter for eligible clients were collected by radiologists using Sonography. Data were edited manually, entered and analyzed using SPSS version 16. Data were collected from a total of 195 participants. Among these, 121(62.1%) were males and the median age of the participants was 35 years. The study revealed a normal mean portal vein diameter of 10.6 mm ±1.8 SD with a respirophasic variation of 25.6%. Likewise, the normal mean portal vein diameter seemed to have varied significantly by age and sex. The study revealed a normal mean portal vein diameter ranging below 13 mm. Hence, decisions made in clinical settings should base on these findings. Besides, there is a need for large scale study to determine portal vein diameter variation by age and sex, controlling other confounders.

Extrahepatic Portal Vein Obstruction in Children: Role of Preoperative Imaging.

PubMed

Achar, Shashidhar; Dutta, Hemonta Kumar; Gogoi, Rudra Kanta

2017-01-01

Extrahepatic portal vein obstruction (EHPVO) is characterized by features of recent thrombosis or portal hypertension with portal cavernoma as a sequel of portal vein obstruction. Imaging of spleno-portal axis is the mainstay for the diagnosis of EHPVO. The aim of this study is to analyze the role of imaging in the preoperative assessment of the portal venous system in children with EHPVO. A hospital-based cross-sectional study was conducted on twenty children with EHPVO aged between 1 and 18 years over a period of 1 year. The children were evaluated clinically, followed by upper gastrointestinal endoscopy. Radiological assessment included imaging of the main portal vein, its right and left branches, splenic vein, and superior mesenteric vein using color Doppler ultrasonography (CDUSG) and magnetic resonance portovenogram (MRP). Evidence of portal biliopathy, status of collaterals, and possible sites for portosystemic shunt surgery were also examined. All the patients presented in chronic stage with portal cavernoma and only one patient (5%) had bland thrombus associated with cavernoma. The CDUSG and MRPs had a sensitivity of 66.6-90% and 96.7% and specificity of 91.5% and 98.3% respectively with regard to the assessment of the extent of thrombus formation and flow in the portal venous system. Both the modalities were found to be complementary to each other in preoperative assessment of EHPVO. However, the sensitivity of MRP was slightly superior to CDUSG in detecting occlusion and identifying portosystemic collaterals and dilated intrahepatic biliary radicals. Results of the present study indicate that MRP is well suited and superior to CDUSG in the preoperative imaging of patients with EHPVO.

Percutaneous transsplenic portal vein catheterization: technical procedures, safety, and clinical applications.

PubMed

Zhu, Kangshun; Meng, Xiaochun; Zhou, Bin; Qian, Jiesheng; Huang, Wensou; Deng, Meihai; Shan, Hong

2013-04-01

To evaluate the safety and feasibility of percutaneous transsplenic portal vein catheterization (PTSPC) by retrospective review of its use in patients with portal vein (PV) occlusion. From July 2004 to December 2010, 46 patients with a history of uncontrolled gastroesophageal variceal bleeding secondary to portal hypertension underwent endovascular PV interventions via a percutaneous transsplenic approach. All patients had occlusion of the main PV or central intrahepatic PV branches, which prevented the performance of a transhepatic approach. A vein within the splenic parenchyma was punctured under fluoroscopic guidance by referencing preoperative computed tomography images. PTSPC-related complications and clinical applications were analyzed. PTSPC was successfully performed in 44 of 46 patients (96%); two failures were caused by inaccessible small intrasplenic veins. PTSPC-related major bleeding complications occurred in three patients (6.5%), including large intraperitoneal hemorrhage in one patient and large splenic subcapsular hemorrhage in two patients. Two of the three patients developed hypotension, and one developed severe anemia. All three of the patients required blood transfusions. PTSPC-related minor bleeding complications occurred in six patients (13%) as a result of a small splenic subcapsular hemorrhage. In addition, three patients exhibited mild left pleural effusion, which subsided spontaneously 1 week later. All 44 patients successfully treated via PTSPC received gastroesophageal variceal embolization. Eight patients received PV stents, five for treatment of PV occlusion and three during transjugular intrahepatic portosystemic shunt placement. PTSPC is a safe and effective access for endovascular PV interventions in patients without a transhepatic window. Copyright © 2013 SIR. Published by Elsevier Inc. All rights reserved.

Hepatic Histology and Morphometric Measurements in Idiopathic Extrahepatic Portal Vein Thrombosis in Children, Correlated to Clinical Outcome of Meso-Rex Bypass.

PubMed

Tantemsapya, Niramol; Superina, Riccardo; Wang, Deli; Kronauer, Grace; Whitington, Peter F; Melin-Aldana, Hector

2018-06-01

The aim of this study was to correlate clinical, histologic, and morphometric features of the liver in children with extrahepatic portal vein thrombosis (EHPVT), with surgical outcome after Meso-Rex bypass (MRB). Idiopathic EHPVT, a significant cause of portal hypertension, is surgically corrected by MRB. Correlation of histologic and morphometric features of the liver with outcome has not been reported in children. We retrospectively reviewed clinical and intraoperative data of 45 children with idiopathic EHPVT. Liver samples were obtained at the time of MRB. Morphometric measurements of portal tract structures were performed and correlated with surgical outcome. Median follow-up was 3.65 years after surgery (range 1.5 to 10 years). Thirty-seven (82.2%) children had successful MRB. There was no association between age, sex, and suture material with surgical outcome. Average patient age was higher in patients with postoperative complications (P = NS). Portal fibrosis, bridging, parenchymal nodules, portal inflammation, hepatocellular swelling, steatosis, dilatation of portal lymphatics, and periductal fibrosis did not show a significant difference between the 2 groups. Portal vein and bile duct area index were significantly smaller in the unsuccessful group (P = 0.004 and 0.003, respectively). A portal vein area index <0.08 had a lower chance of successful surgical outcome. Hepatic artery area index was not significantly different. Measured intraoperative portal blood inflow was the only significant clinical factor affecting surgical outcome (P = 0.0003). Low portal vein area index and intraoperative portal blood inflow may be negative prognostic factors for MRB outcome in children with idiopathic EHPVT. Average patient age was higher, although not statistically significant, in patients with postoperative complications.

Testicular Cancer Presenting as Gastric Variceal Hemorrhage.

PubMed

Salazar-Mejía, Carlos Eduardo; Hernández-Barajas, David; Llerena-Hernández, Edio; González-Vela, José Luis; Contreras-Salcido, María Inés; González-Gutiérrez, Adriana; Borjas-Almaguer, Omar David; Pérez-Arredondo, Luis Alberto; Wimer-Castillo, Blanca Otilia

2017-01-01

Testicular cancer is the most common solid malignancy affecting males between the ages of 15 and 35. The symptomatology caused by this tumor varies according to the site of metastasis. We present the case of a 26-year-old male who arrived to the emergency department with hematemesis. He had no previous medical history. On arrival, we noted enlargement of the left scrotal sac. There was also a mass in the left scrotum which provoked displacement of the penis and right testis. The serum alpha-fetoprotein level was 17,090 ng/mL, lactate dehydrogenase was 1480 U/L, and human chorionic gonadotropin was 287.4 IU/mL. Upper endoscopy revealed a type 1 isolated gastric varix, treated with cyanoacrylate. A CT scan showed extrinsic compression of the portal vein by lymphadenopathy along with splenic vein partial thrombosis, which caused left-sided portal hypertension. Neoadjuvant chemotherapy was started with etoposide and cisplatin, and seven days later the patient underwent left radical orchiectomy. A postoperative biopsy revealed a pure testicular teratoma. Noncirrhotic left portal hypertension with bleeding from an isolated gastric varix secondary to metastasic testicular cancer has not been described before. Clinicians must consider the possibility of malignancy in the differential diagnosis of a young man presenting with unexplained gastrointestinal bleeding.

[Effect of a serotonin S2 receptor antagonist on portal hypertension due to cirrhosis. Preliminary results of a heart and liver hemodynamic study].

PubMed

Deflandre, J; Pirotte, J; Etienne, M; Carlier, J

1988-01-01

In 10 cirrhotic patients, with two balloon catheters introduced in the right internal jugular vein, the following parameters were measured before and after injection of ketanserine (0.1 mg/kg): cardiac output using the thermo-dilution method, free supra-hepatic pressure, wedged supra-hepatic pressure at rest and during cough, right atrial pressure, pulmonary capillary and arterial pressures. After 30 minutes, the following modifications were recorded: the cardiac output goes from 8.0 +/- 2.4 l/min to 8.7 +/- 2.5 l/min (p less than 0.05); the mean arterial pressure goes from 107.0 +/- 18.8 mmHg to 94.7 +/- 25.9 mmHg (p less than 0.02); the wedged supra-hepatic pressure, during coughing goes from 85.2 +/- 36.1 mmHg to 64.6 +/- 23.1 mmHg (p less than 0.005). As in non-cirrhotic patients, ketanserine causes a drop in the mean arterial pressure and a transient elevation of the cardiac output. Ketanserine is able to lower portal pressure of cirrhotic patients at rest as well as during coughing. These results seem to indicate that the activation of serotonin S2 receptors may play a role in determining the portal hypertension in cirrhotic patients.

Imaging Diagnosis of Splanchnic Venous Thrombosis

PubMed Central

Rajesh, S.; Mukund, Amar

2015-01-01

Splanchnic vein thrombosis (SVT) is a broad term that includes Budd-Chiari syndrome and occlusion of veins that constitute the portal venous system. Due to the common risk factors involved in the pathogenesis of these clinically distinct disorders, concurrent involvement of two different regions is quite common. In acute and subacute SVT, the symptoms may overlap with a variety of other abdominal emergencies while in chronic SVT, the extent of portal hypertension and its attendant complications determine the clinical course. As a result, clinical diagnosis is often difficult and is frequently reliant on imaging. Tremendous improvements in vascular imaging in recent years have ensured that this once rare entity is being increasingly detected. Treatment of acute SVT requires immediate anticoagulation. Transcatheter thrombolysis or transjugular intrahepatic portosystemic shunt is used in the event of clinical deterioration. In cases with peritonitis, immediate laparotomy and bowel resection may be required for irreversible bowel ischemia. In chronic SVT, the underlying cause should be identified and treated. The imaging manifestations of the clinical syndromes resulting from SVT are comprehensively discussed here along with a brief review of the relevant clinical features and therapeutic approach. PMID:26600801

Role of D-dimer in the Development of Portal Vein Thrombosis in Liver Cirrhosis: A Meta-analysis

PubMed Central

Dai, Junna; Qi, Xingshun; Li, Hongyu; Guo, Xiaozhong

2015-01-01

Background and Aims: A meta-analysis was performed to explore the role of the D-dimer in the development of portal vein thrombosis (PVT) in liver cirrhosis. Methods: All papers were searched via PubMed, EMBASE, China National Knowledge Infrastructure, Wan Fang, and VIP databases. A standardized mean difference (SMD) with 95% confidence interval (CI) was pooled. Results: Overall, 284 studies were initially identified, of which 21 were included. Cirrhotic patients with PVT had a significantly higher D-dimer concentration than those without PVT (pooled SMD = 1.249, 95%CI = 0.740–1.758). After the portal hypertension-related surgery, cirrhotic patients with PVT had a similar preoperative D-dimer concentration to those without PVT (pooled SMD = 0.820, 95%CI = −0.122–0.286), but a higher postoperative value of D-dimer concentration than those without PVT (pooled SMD = 2.505, 95%CI = 0.975–4.036). Notably, the D-dimer concentration at the 1st postoperative day was similar between cirrhotic patients with and without PVT (pooled SMD = 0.137, 95%CI = −0.827–1.101), but that at the 7th post-operative day was higher in cirrhotic patients with PVT than in those without PVT (pooled SMD = 1.224, 95%CI = 0.277–2.171). Conclusion: D-dimer might be regarded as a diagnostic marker for PVT in liver cirrhosis. In addition, postoperative D-dimer testing is worthwhile for the diagnosis of PVT after portal hypertension-related surgery. PMID:26021776

Etiological and Endoscopic Profile of Middle Aged and Elderly Patients with Upper Gastrointestinal Bleeding in a Tertiary Care Hospital in North India: A Retrospective Analysis.

PubMed

Mahajan, Pranav; Chandail, Vijant Singh

2017-01-01

Upper gastrointestinal (GI) bleeding is a common medical emergency associated with significant morbidity and mortality. The clinical presentation depends on the amount and location of hemorrhage and the endoscopic profile varies according to different etiology. At present, there are limited epidemiological data on upper GI bleed and associated mortality from India, especially in the middle and elderly age group, which has a higher incidence and mortality from this disease. This study aims to study the clinical and endoscopic profile of middle aged and elderly patients suffering from upper GI bleed to know the etiology of the disease and outcome of the intervention. Out of a total of 1790 patients who presented to the hospital from May 2015 to August 2017 with upper GI bleed, and underwent upper GI endoscopy, data of 1270 patients, aged 40 years and above, was compiled and analyzed retrospectively. All the patients included in the study were above 40 years of age. Majority of the patients were males, with a male to female ratio of 1.6:1. The most common causes of upper GI bleed in these patients were portal hypertension-related (esophageal, gastric and duodenal varices, portal hypertensive gastropathy, and gastric antral vascular ectasia GAVE), seen in 53.62% of patients, followed by peptic ulcer disease (gastric and duodenal ulcers) seen in 17.56% of patients. Gastric erosions/gastritis accounted for 15.20%, and duodenal erosions were seen in 5.8% of upper GI bleeds. The in-hospital mortality rate in our study population was 5.83%. The present study reported portal hypertension as the most common cause of upper GI bleeding, while the most common endoscopic lesions reported were esophageal varices, followed by gastric erosion/gastritis, and duodenal ulcer.

Feasibility of transnasal endoscopy in screening for esophageal and gastric varices in patients with chronic liver disease

PubMed Central

de Faria, Anderson Antônio; Dias, Carlos Alberto Freitas; Dias Moetzsohn, Luciana; de Castro Carvalho, Silas; Ferrari, Tereza Abreu; Nunes Arantes, Vitor

2017-01-01

Background and study aims  Screening for esophageal and gastric varices is indicated for patients with portal hypertension or cirrhosis. Typically, conventional endoscopy is used; however, the need for sedation increases the costs and risks, especially in cirrhotic patients. Use of transnasal endoscopy with an ultrathin endoscope enables study of the upper gastrointestinal tract without the need for sedation. The objective of this study is to evaluate the feasibility of transnasal endoscopy in screening for esophageal and gastric varices in patients with chronic liver disease. Patients and methods  This was a prospective study in which transnasal endoscopy was carried out in patients with cirrhosis or portal hypertension who had indications for screening of esophageal and gastric varices. The following variables were evaluated: demographical data, duration of procedure, patient tolerance and acceptance, adverse events (AEs), endoscopic findings and interobserver agreement related to portal hypertension alterations ( kappa index). Results  A total of 50 patients entered the study. The most common cause of liver disease was chronic viral hepatitis (66 %). Among the cirrhotic patients, most of the patients were Child-Pugh A (74 %). In 5 patients (10 %), nasal intubation was not possible. Two patients (4 %) experienced minor epistaxis. Tolerance was excellent or good in 92 % according with a visual analogic scale. In 16 patients (32 %), esophageal varices were detected and in 2 patients (4 %) gastric varices were detected. The mean duration of the procedure was 7 minutes. Conclusions  Transnasal endoscopy is feasible, effective and well tolerated for screening of esophageal and gastric varices in patients with chronic liver disease. It can be performed in outpatient clinics safely and without the use of sedation. PMID:28691048

[Congenital intrahepatic venous shunt as a cause of hepatic encephalopathy].

PubMed

Cosme Jiménez, A; Bujanda Fernández de Piérola, L; Poch Zapiraín, M; Orcolaga Alba, R; Ojeda Pérez, E; Arenas Miravé, J I

1995-11-01

Macroscopic intrahepatic portosystemic shunts are extremely rare and may be due to liver injury, congenital vascular malformations or pathologic collaterals secondary to portal hypertension. Forty-eight cases have been reported in the literature up to 1994 with 50-60% presenting cerebral manifestations and 40% being associated with cirrhosis. The case of a patient without cirrhosis who was admitted for upper digestive hemorrhage secondary to gastroduodenal ulcer is described. At 48 hours the patient had an episode of hepatic encephalopathy coinciding with bleeding reactivation. Abdominal echography suggested communication between the right portal and suprahepatic veins and posterior angiography confirmed the diagnosis. Color Doppler echography determined shunt and portal vein blood flow. No case of intrahepatic portosystemic venous shunt as a cause of encephalopathy was found to have been reported in the Spanish literature.

Massive Hemorrhage from Ectopic Duodenal Varices: Importance of a Multidisciplinary Approach

PubMed Central

House, Tyler; Webb, Patrick; Baarson, Chad

2017-01-01

Duodenal variceal bleeding is an uncommon complication of portal hypertension that can easily go unrecognized and reach mortality rates as high as 40%. Cirrhosis is the most common cause of duodenal varices. In most cases, duodenal varices occur concomitantly with esophageal varices, further complicating identification with initial endoscopy. Although many modalities have been explored with respect to management and treatment approaches, guidelines have yet to be established owing to the infrequency in which bleeding occurs from ectopic duodenal varices. We present a case of massive duodenal variceal hemorrhage that highlights the complexity of initial diagnosis and ultimately required a transesophageal intrahepatic portosystemic shunt with coil embolization for control of bleeding. PMID:28203136

Liver transplantation for severe hepatic trauma: Experience from a single center

PubMed Central

Delis, Spiros G; Bakoyiannis, Andreas; Selvaggi, Gennaro; Weppler, Debbie; Levi, David; Tzakis, Andreas G

2009-01-01

Liver transplantation has been reported in the literature as an extreme intervention in cases of severe and complicated hepatic trauma. The main indications for liver transplant in such cases were uncontrollable bleeding and postoperative hepatic insufficiency. We here describe four cases of orthotopic liver transplantation after penetrating or blunt liver trauma. The indications were liver failure, extended liver necrosis, liver gangrene and multiple episodes of gastrointestinal bleeding related to portal hypertension, respectively. One patient died due to postoperative cerebral edema. The other three patients recovered well and remain on immunosuppression. Liver transplantation should be considered as a saving procedure in severe hepatic trauma, when all other treatment modalities fail. PMID:19340909

Telerehabilitation for OIF/OEF Returnees with Combat-Related Traumatic Brain Injury

DTIC Science & Technology

2013-06-01

VA has no national program for providing individualized care coordination for veterans via telemedicine. It does have an e-health portal where...Heart Failure, diabetes, hypertension , COPD and mental illness. However, this technology does not allow for the posting of individualized questions

[Clinical dilemma: an unusual case of ascites].

PubMed

Romi, Hila; Hausman, Michael; Kachko, Leonid; Emanuel, Sikuler

2012-12-01

Cirrhotic portal hypertension is the major cause of ascites. Ascites is the most common expression of decompensated liver disease. However, other etiologies may occur and may pose a diagnostic and therapeutic challenge. A patient with chronic hepatitis C and an unusual cause of ascites is presented.

To screen or not to screen? Celiac antibodies in liver diseases

PubMed Central

Narciso-Schiavon, Janaína Luz; Schiavon, Leonardo Lucca

2017-01-01

Celiac disease (CD) is a systemic immune-mediated disorder triggered by dietary gluten in genetically predisposed individuals. The typical symptoms are anemia, diarrhea, fatigue, weight loss, and abdominal pain. CD has been reported in patients with primary sclerosing cholangitis, primary biliary cholangitis, autoimmune hepatitis, aminotransferase elevations, nonalcoholic fatty liver disease, hepatitis B, hepatitis C, portal hypertension and liver cirrhosis. We evaluate recommendations for active screening for CD in patients with liver diseases, and the effect of a gluten-free diet in these different settings. Active screening for CD is recommended in patients with liver diseases, particularly in those with autoimmune disorders, steatosis in the absence of metabolic syndrome, noncirrhotic intrahepatic portal hypertension, cryptogenic cirrhosis, and in the context of liver transplantation. In hepatitis C, diagnosis of CD can be important as a relative contraindication to interferon use. Gluten-free diet ameliorates the symptoms associated with CD; however, the associated liver disease may improve, remain the same, or progress. PMID:28223722

Elastography in Chronic Liver Disease: Modalities, Techniques, Limitations, and Future Directions

PubMed Central

Srinivasa Babu, Aparna; Wells, Michael L.; Teytelboym, Oleg M.; Mackey, Justin E.; Miller, Frank H.; Yeh, Benjamin M.; Ehman, Richard L.

2016-01-01

Chronic liver disease has multiple causes, many of which are increasing in prevalence. The final common pathway of chronic liver disease is tissue destruction and attempted regeneration, a pathway that triggers fibrosis and eventual cirrhosis. Assessment of fibrosis is important not only for diagnosis but also for management, prognostic evaluation, and follow-up of patients with chronic liver disease. Although liver biopsy has traditionally been considered the reference standard for assessment of liver fibrosis, noninvasive techniques are the emerging focus in this field. Ultrasound-based elastography and magnetic resonance (MR) elastography are gaining popularity as the modalities of choice for quantifying hepatic fibrosis. These techniques have been proven superior to conventional cross-sectional imaging for evaluation of fibrosis, especially in the precirrhotic stages. Moreover, elastography has added utility in the follow-up of previously diagnosed fibrosis, the assessment of treatment response, evaluation for the presence of portal hypertension (spleen elastography), and evaluation of patients with unexplained portal hypertension. In this article, a brief overview is provided of chronic liver disease and the tools used for its diagnosis. Ultrasound-based elastography and MR elastography are explored in depth, including a brief glimpse into the evolution of elastography. Elastography is based on the principle of measuring tissue response to a known mechanical stimulus. Specific elastographic techniques used to exploit this principle include MR elastography and ultrasonography-based static or quasistatic strain imaging, one-dimensional transient elastography, point shear-wave elastography, and supersonic shear-wave elastography. The advantages, limitations, and pitfalls of each modality are emphasized. ©RSNA, 2016 PMID:27689833

Plug-Assisted Retrograde Transvenous Obliteration for the Treatment of Gastric Variceal Hemorrhage.

PubMed

Chang, Min-Yung; Kim, Man-Deuk; Kim, Taehwan; Shin, Wonseon; Shin, Minwoo; Kim, Gyoung Min; Won, Jong Yun; Park, Sung Il; Lee, Do Yun

2016-01-01

To evaluate the feasibility, safety, and clinical outcomes of plug-assisted retrograde transvenous obliteration (PARTO) to treat gastric variceal hemorrhage in patients with portal hypertension. From May 2012 to June 2014, 19 patients (11 men and 8 women, median age; 61, with history of gastric variceal hemorrhage; 17, active bleeding; 2) who underwent PARTO using a vascular plug and a gelfoam pledget were retrospectively analyzed. Clinical and laboratory data were examined to evaluate primary (technical and clinical success, complications) and secondary (worsening of esophageal varix [EV], change in liver function) end points. Median follow-up duration was 11 months, from 6.5 to 18 months. The Wilcoxon signed-rank test was used to compare laboratory data before and after the procedure. Technical success (complete occlusion of the efferent shunt and complete filling of gastric varix [GV] with a gelfoam slurry) was achieved in 18 of 19 (94.7%) patients. The embolic materials could not reach the GV in 1 patient who had endoscopic glue injection before our procedure. The clinical success rate (no recurrence of gastric variceal bleeding) was the same because the technically failed patient showed recurrent bleeding later. Acute complications included fever (n = 2), fever and hypotension (n = 2; one diagnosed adrenal insufficiency), and transient microscopic hematuria (n = 3). Ten patients underwent follow-up endoscopy; all exhibited GV improvement, except 2 without endoscopic change. Five patients exhibited aggravated EV, and 2 of them had a bleeding event. Laboratory findings were significantly improved after PARTO. PARTO is technically feasible, safe, and effective for gastric variceal hemorrhage in patients with portal hypertension.

Insight into congenital absence of the portal vein: Is it rare?

PubMed Central

Shen, Guo-Hua Hu‚ Lai-Gen; Zhu, Jin Yang‚ Jin-Hua Mei‚ Yue-Feng

2008-01-01

Congenital absence of portal vein (CAPV) was a rare event in the past. However, the number of detected CAPV cases has increased in recent years because of advances in imaging techniques. Patients with CAPV present with portal hypertension (PH) or porto-systemic encephalopathy (PSE), but these conditions rarely occur until the patients grow up or become old. The patients usually visit doctors for the complications of venous shunts, hepatic or cardiac abnormalities detected by ultrasonography (US), computed tomography (CT) and magnetic resonance imaging (MRI). The etiology of this disease is not clear, but most investigators consider that it is associated with abnormal embryologic development of the portal vein. Usually, surgical intervention can relieve the symptoms and prevent occurrence of complications in CAPV patients. Moreover, its management should be stressed on a case-by-case basis, depending on the type or anatomy of the disease, as well as the symptoms and clinical conditions of the patient. PMID:18932274

[Portal perfusion with right gastroepiploic vein flow in liver transplant].

PubMed

Mendoza-Sánchez, Federico; Javier-Haro, Francisco; Mendoza-Medina, Diego Federico; González-Ojeda, Alejandro; Cortés-Lares, José Antonio; Fuentes-Orozco, Clotilde

Liver transplantation in patients with liver cirrhosis, portal vein thrombosis, and cavernous transformation of the portal vein, is a complex procedure with high possibility of liver graft dysfunction. It is performed in 2-19% of all liver transplants, and has a significantly high mortality rate in the post-operative period. Other procedures to maintain portal perfusion have been described, however there are no reports of liver graft perfusion using right gastroepiploic vein. A 20 year-old female diagnosed with cryptogenic cirrhosis, with a Child-Pugh score of 7 points (class "B"), and MELD score of 14 points, with thrombosis and cavernous transformation of the portal vein, severe portal hypertension, splenomegaly, a history of upper gastrointestinal bleeding due to oesophageal varices, and left renal agenesis. The preoperative evaluation for liver transplantation was completed, and the right gastroepiploic vein of 1-cm diameter was observed draining to the infrahepatic inferior vena cava and right suprarenal vein. An orthotopic liver transplantation was performed from a non-living donor (deceased on January 30, 2005) using the Piggy-Back technique. Portal vein perfusion was maintained using the right gastroepiploic vein, and the outcome was satisfactory. The patient was discharged 13 days after surgery. Liver transplantation was performed satisfactorily, obtaining an acceptable outcome. In this case, the portal perfusion had adequate blood flow through the right gastroepiploic vein. Copyright © 2015 Academia Mexicana de Cirugía A.C. Publicado por Masson Doyma México S.A. All rights reserved.

Imaging and radiological interventions in extra-hepatic portal vein obstruction

PubMed Central

Pargewar, Sudheer S; Desai, Saloni N; Rajesh, S; Singh, Vaibhav P; Arora, Ankur; Mukund, Amar

2016-01-01

Extrahepatic portal vein obstruction (EHPVO) is a primary vascular condition characterized by chronic long standing blockage and cavernous transformation of portal vein with or without additional involvement of intrahepatic branches, splenic or superior mesenteric vein. Patients generally present in childhood with multiple episodes of variceal bleed and EHPVO is the predominant cause of paediatric portal hypertension (PHT) in developing countries. It is a pre-hepatic type of PHT in which liver functions and morphology are preserved till late. Characteristic imaging findings include multiple parabiliary venous collaterals which form to bypass the obstructed portal vein with resultant changes in biliary tree termed portal biliopathy or portal cavernoma cholangiopathy. Ultrasound with Doppler, computed tomography, magnetic resonance cholangiography and magnetic resonance portovenography are non-invasive techniques which can provide a comprehensive analysis of degree and extent of EHPVO, collaterals and bile duct abnormalities. These can also be used to assess in surgical planning as well screening for shunt patency in post-operative patients. The multitude of changes and complications seen in EHPVO can be addressed by various radiological interventional procedures. The myriad of symptoms arising secondary to vascular, biliary, visceral and neurocognitive changes in EHPVO can be managed by various radiological interventions like transjugular intra-hepatic portosystemic shunt, percutaneous transhepatic biliary drainage, partial splenic embolization, balloon occluded retrograde obliteration of portosystemic shunt (PSS) and revision of PSS. PMID:27358683

Portal inflow and pressure changes in right liver living donor liver transplantation including the middle hepatic vein.

PubMed

Chan, See Ching; Lo, Chung Mau; Ng, Kelvin K C; Ng, Irene O L; Yong, Boon Hun; Fan, Sheung Tat

2011-02-01

The middle hepatic vein may be included in right liver living donor liver transplantation (LDLT) to optimize hepatic venous outflow. We studied the graft's ability to relieve portal hypertension and accommodate portal hyperperfusion with portal manometry and ultrasonic flowmetry. Surgical outcomes with respect to portal hemodynamometry were also investigated. The ages of the recipients and donors for 46 consecutive LDLT procedures were 50 (range, 16-66 years) and 31 years (range, 18-54 years), respectively. The graft to standard liver volume ratio was 47.4% (range, 32.4%-69.0%). The hospital mortality rate was 4.4% as 2 recipients died from a subarachnoid hemorrhage and sepsis. The portal pressure dropped by 8 mm Hg (range, -7 to 19 mm Hg) from 23 (range, 8-37 mm Hg) to 14 mm Hg (range, 10-26 mm Hg) after graft implantation. The portal inflow positively correlated with the portal pressure before native liver hepatectomy (R(2) = 0.305, P = 0.001) and not with the graft size. The portal inflow increased from 81 mL/minute/100 g (range, 35-210 mL/minute/100 g) before donor right hepatectomy to 318 mL/minute/100 g (range, 102-754 mL/minute/100 g) after graft implantation. The graft portal inflow had a positive linear correlation with the recipient portal pressure before native liver total hepatectomy (R(2) = 0.261, P = 0.001) but not after graft implantation, and it had a negative correlation with the graft to standard liver volume ratio (R(2) = 0.247, P = 0.001). Only 1 of the graft biopsies showed moderate sinusoidal congestion. Twelve recipients had Clavien grade 2+ complications that were not related to the portal inflow and pressure or graft size. Right liver LDLT including the middle hepatic vein effectively lowered the recipient portal pressure by allowing unimpeded venous outflow. Copyright © 2011 American Association for the Study of Liver Diseases.

Orthotopic liver transplantation for portosystemic encephalopathy in an adult with congenital absence of the portal vein.

PubMed

Wojcicki, Maciej; Haagsma, Elizabeth B; Gouw, Annette S H; Slooff, Maarten J H; Porte, Robert J

2004-09-01

Congenital absence of the portal vein (CAPV) is a very rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. There is no portal perfusion of the liver and no portal hypertension. This abnormality is usually coincidentally discovered in children, the majority of whom have no signs of encephalopathy and only slightly abnormal liver function tests. Additional anomalies common in CAPV are cardiovascular abnormalities and hepatic tumors. To date, only 5 adult patients (>18 years) with CAPV have been described, none of whom underwent liver transplantation. We describe a 45-year-old man with CAPV and end-stage renal insufficiency due to focal segmental glomerulopathy, who developed therapy-resistant encephalopathy with intermittently high ammonia levels. The patient underwent a combined liver and kidney transplantation and is doing well at 2.5 years of follow-up. Histopathological examination of the native liver showed no portal vein branches in the portal tracts. In conclusion, our experience suggests that, although children with CAPV usually have no symptoms of encephalopathy, this may still develop at a later stage in adult life. When encephalopathy becomes refractory to medical therapy, orthotopic liver transplantation (OLT) can be successfully performed with restoration of normal cerebral function.

The Intersection of Pulmonary Hypertension and Solid Organ Transplantation.

PubMed

Frost, Adaani E

2016-01-01

Pulmonary hypertension (PH) is a complication and marker of disease severity in many parenchymal lung diseases. It also is a frequent complication of portal hypertension and negatively impacts survival with liver transplant. Pulmonary hypertension is frequently diagnosed in patients with end-stage renal disease who are undergoing dialysis, and it has recently been demonstrated to adversely affect posttransplant outcome in this patient population even though the mechanism of PH is substantially different from that associated with liver disease. The presence of PH in patients with heart failure is frequent, and the necessity for PH therapy prior to heart transplant has evolved in the last decade. We review the frequency of and risk factors for PH in recipients of and candidates for lung, liver, heart, and renal transplants as well as the impact of this diagnosis on posttransplant outcomes.

Comparative randomized study on efficacy of losartan versus propranolol in lowering portal pressure in decompensated chronic liver disease.

PubMed

Agasti, Ananta Kumar; Mahajan, Ajay U; Phadke, Aniruddha Y; Nathani, Pratap J; Sawant, Prabha

2013-05-01

This study aimed to compare the efficacy of losartan, an angiotensin II receptor antagonist, with propranolol on portal hypertension in patients with decompensated chronic liver disease. In all, 30 patients with Child-Pugh B cirrhosis and large varices without any prior therapy for portal hypertension were randomized to either losartan (n = 15) or propranolol (n = 15). Clinical, biochemical and hemodynamic parameters including hepatic venous pressure gradient (HVPG), wedged hepatic venous pressure (WHVP), mean arterial blood pressure (MABP) and free hepatic venous pressure (FHVP) were measured at baseline and after 4-week therapy. Patients with HVPG < 12 mmHg were regarded as responders. An equal number of responders were seen in both groups (6/15, 40.0%). The reduction of WHVP and HVPG was greater in the losartan group than in the propranolol group, although no significant differences between them were found. Heart rate decreased more in the propranolol arm than in the losartan arm (P < 0.01); however, no correlation between the decrease of heart rate and the reduction of HVPG was observed. One patient in the losartan group, although a responder, had gastrointestinal bleeding 2 months after the drug administration, but the varices were small under endoscopy and did not require definitive therapy. The fall of MABP was greater with losartan, with no statistical difference between the two groups. The effect of losartan was comparable to propranolol in reducing portal pressure in decompensated Child-Pugh B chronic liver disease. © 2012 The Authors. Journal of Digestive Diseases © 2012 Chinese Medical Association Shanghai Branch, Chinese Society of Gastroenterology, Renji Hospital Affiliated to Shanghai Jiaotong University School of Medicine and Wiley Publishing Asia Pty Ltd.

Portal hypertension associated with sickle cell disease.

PubMed

Kumar, Sunil; Joshi, R; Jain, A P

2007-01-01

We report a 12-year-old girl with sickle cell disease who presented with pain in abdomen, fever, joint pain and hematemesis. On examination she had mild jaundice and splenomegaly. Upper GI endoscopy showed esophageal varices. She was treated with variceal band ligation and is well on folic acid supplements and propranolol.

Telerehabilitation for OIF/OEF Returnees with Combat-Related Traumatic Brain Injury

DTIC Science & Technology

2012-06-01

coordination for veterans via telemedicine. It does have an e-health portal where veterans may submit and track vital signs such as BP readings and...of its program to support patients with chronic conditions such as Congestive Heart Failure, diabetes, hypertension , COPD and mental illness. However

Diagnosis and treatment of portal vein thrombosis following splenectomy.

PubMed

van't Riet, M; Burger, J W; van Muiswinkel, J M; Kazemier, G; Schipperus, M R; Bonjer, H J

2000-09-01

Portal vein thrombosis is a rare but potentially fatal complication of splenectomy. The aim of this study was to assess the incidence, risk factors, treatment and outcome of portal vein thrombosis after splenectomy in a large series of patients. All patients who had undergone a splenectomy in the University Hospital, Rotterdam, between 1984 and 1997 were reviewed retrospectively. Splenectomy that was followed by symptomatic portal vein thrombosis was selected for analysis. Risk factors for portal vein thrombosis were sought. Of 563 splenectomies, nine (2 per cent) were complicated by symptomatic portal vein thrombosis. All these patients had either fever or abdominal pain. Two of 16 patients with a myeloproliferative disorder developed portal vein thrombosis after splenectomy (P = 0.03), and four of 49 patients with haemolytic anaemia (P = 0.005). Treatment within 10 days after splenectomy was successful in all patients, while delayed treatment was ineffective. Portal vein thrombosis should be suspected in a patient with fever or abdominal pain after splenectomy. Patients with a myeloproliferative disorder or haemolytic anaemia are at higher risk; they might benefit from early detection and could have routine Doppler ultrasonography after splenectomy.

Acute mesenteric ischemia: a vascular emergency.

PubMed

Klar, Ernst; Rahmanian, Parwis B; Bücker, Arno; Hauenstein, Karlheinz; Jauch, Karl-Walter; Luther, Bernd

2012-04-01

Acute mesenteric ischemia is still fatal in 50% to 70% of cases. This consensus paper was written with the participation of physicians from all of the involved specialties for the purpose of improving outcomes. Mesenteric ischemia must be recognized as a vascular emergency requiring rapid and efficient clinical evaluation and treatment. We reviewed pertinent literature that was retrieved by a PubMed search on the terms "mesenteric ischemia" AND "arterial" OR "venous" OR "clinical presentation" OR "diagnosis" OR "therapy" OR "surgery" OR " interventional radiology." Our review also took account of the existing guidelines of the American College of Cardiology/American Heart Association. Intensive discussions among the participating physicians, representing all of the specialties involved in the management of mesenteric ischemia, led to the creation of this interdisciplinary paper. Biphasic contrast-enhanced computerized tomography is the diagnostic tool of choice for the detection of arterial or venous occlusion. If non-occlusive mesenteric ischemia is suspected, angiography should be performed, with the option of intraarterial pharmacotherapy to induce local vasodilation. Endovascular techniques have become increasingly important in the treatment of arterial occlusion. Embolic central mesenteric artery occlusion requires surgical treatment; surgery is also needed in case of peritonitis. Portal-vein thrombosis can be treated by local thrombolysis through a transhepatically placed catheter. This should be done within 3 to 4 weeks of the event to prevent later complications of portal hypertension. Rapid diagnosis (within 4 to 6 hours of symptom onset) and interdisciplinary cooperation in the provision of treatment are required if the poor outcome of this condition is to be improved.

Washout of hepatocellular carcinoma on portal venous phase of multidetector computed tomography in a pre-transplant population.

PubMed

Kitzing, Yu Xuan; Ng, Bernard H K; Kitzing, Bjoern; Waugh, Richard; Kench, James G; Strasser, Simone I; McCormack, Samuel

2015-12-01

Washout is an important diagnostic imaging feature of hepatocellular carcinoma (HCC) on computed tomography (CT). The primary aim of this study is to evaluate the prevalence and the interobserver variation in the detection of portal venous phase (PVP) washout of HCCs using CT in a transplant population. The secondary aim is to evaluate factors influencing the detection of PVP washout. Forty-five patients who underwent CT liver imaging within the 60 days before transplantation had viable HCCs confirmed on pathology. Two radiologists retrospectively reviewed the images for HCCs including features of arterial enhancement and PVP washout. Clinical data, peak kilovoltage, imaging features of portal hypertension, region of interest attenuation measurements of the individual lesions, background liver parenchyma and portal vein were obtained. Liver to lesion attenuation ratio was also calculated. Statistical analysis was performed. The two readers identified 50 arterially enhancing HCCs in 45 patients. In consensus, the two readers identified washout in 60% of the HCCs with a substantial interobserver agreement (kappa = 0.633). PVP washout was associated with larger lesion size, increased background liver parenchyma attenuation, increased liver to lesion attenuation ratio, increased portal vein attenuation and hepatitis B viral status (P = 0.027, 0.008, 0.014, 0.017 and 0.037 respectively). In our transplant population, portal venous phase washout was seen in 60% of the hypervascular HCCs. Factors influencing the presence of PVP washout include lesion size as well as the liver and portal vein attenuation reflective of the portal haemodynamics. © 2015 The Royal Australian and New Zealand College of Radiologists.

eHealth Literacy: Patient Engagement in Identifying Strategies to Encourage Use of Patient Portals Among Older Adults.

PubMed

Price-Haywood, Eboni G; Harden-Barrios, Jewel; Ulep, Robin; Luo, Qingyang

2017-12-01

Innovations in chronic disease management are growing rapidly as advancements in technology broaden the scope of tools. Older adults are less likely to be willing or able to use patient portals or smartphone apps for health-related tasks. The authors conducted a cross-sectional survey of older adults (ages ≥50) with hypertension or diabetes to examine relationships between portal usage, interest in health-tracking tools, and eHealth literacy, and to solicit practical solutions to encourage technology adoption. Among 247 patients surveyed in a large integrated delivery health system between August 2015 and January 2016, eHealth literacy was positively associated with portal usage (OR [95% CI]: 1.3 [1.2-1.5]) and interest in health-tracking tools (1.2 [1.1-1.3]). Portal users compared to nonusers (N = 137 vs.110) had higher rates of interest in using websites/smartphone apps to track blood pressure (55% vs. 36%), weight (53% vs. 35%), exercise (53% vs. 32%), or medication (46% vs 33%, all P < 0.05). Portal users noted cumbersome processes for accessing portals and variations in provider availability for online scheduling and response times to messages. Portal nonusers expressed concerns about data security, lack of personalization, and limited perceived value of using portals. Both groups noted the importance of computer literacy and technical support. Patient stakeholders recommended marketing initiatives that capture patient stories demonstrating real-life applications of what patients can do with digital technology, how to use it, and why it may be useful. Health systems also must screen for eHealth literacy, provide training, promote proxy users, and institute quality assurance that ensures patients' experiences will not vary across the system.

Total fibrous obliteration of main portal vein and portal foam cell venopathy in chronic hepatic allograft rejection.

PubMed

Jain, Dhanpat; Robert, Marie E; Navarro, Victor; Friedman, Amy L; Crawford, James M

2004-01-01

Chronic hepatic allograft rejection is characterized by arteriopathy and bile duct loss. Pathology of the portal vein or its branches is not considered to play a major role in chronic rejection. A recent case of chronic rejection with total fibrous obliteration of the portal vein at the hilum and graft loss prompted us to retrospectively analyze cases of failed allografts for portal vein changes. Six cases of failed hepatic allograft recorded in our files from 1994 to 1998 were selected for the study. For comparison, 4 cases of hepatitis C cirrhosis were included. Clinical features, including arteriograms or Doppler studies, were reviewed whenever available. Sections taken from the hilum and random parenchyma stained with routine hematoxylin-eosin, elastic van Gieson, and Masson trichrome were examined by 3 experienced liver pathologists in a randomized, blinded fashion. Significant hepatic artery occlusion with foam cell change and bile duct loss was seen in all cases of chronic rejection (3/3), but not in the other cases. Foam cell change in the portal vein at the hilum (3/3) and occasionally into the distal branches (2/3) with variable occlusion of the lumen was seen only in cases of chronic hepatic allograft rejection. Mild luminal narrowing was observed in all the cases of cirrhosis (4/4) as a result of phlebosclerosis, most likely representing a change secondary to portal hypertension. Total obliteration of the portal vein at the hilum was seen in the index case (case 1) only. Portal venopathy can be a significant finding in chronic hepatic allograft rejection and may contribute to graft dysfunction or failure. Two-vessel disease must be considered in cases of chronic hepatic allograft rejection, and pathologists should thoroughly examine the hilum in explanted hepatic allografts.

Complications of congenital portosystemic shunts in children: therapeutic options and outcomes.

PubMed

Franchi-Abella, Stéphanie; Branchereau, Sophie; Lambert, Virginie; Fabre, Monique; Steimberg, Clarisa; Losay, Jean; Riou, Jean-Yves; Pariente, Danièle; Gauthier, Frédéric; Jacquemin, Emmanuel; Bernard, Olivier

2010-09-01

Congenital portosystemic shunts are rare vascular malformations that lead to severe complications. Their management is controversial. The aim of this study was to propose a clear definition of the risks and management of congenital portosystemic shunts in children according to our experience and a review of the literature. Twenty-two children with a complicated congenital portosystemic shunt were studied in our institution. When necessary, management included portal pressure measurement and portal vein angiography during an occlusion test and closure of the shunt by surgical and/or endovascular methods. Five neonates with intrahepatic shunts presented with cholestasis that resolved spontaneously, and 17 older children presented with liver tumors (13) and/or hepatopulmonary syndrome (2), pulmonary artery hypertension (3), portosystemic encephalopathy (3), heart failure (1), and glomerulonephritis (1). The portosystemic shunt was extrahepatic (11) or intrahepatic (6). Portosystemic shunts were closed by endovascular methods in 5 children and surgically in 10, 4 of whom had portal pressure during occlusion above 35 mmHg and extremely hypoplastic or undetectable portal veins requiring banding of the fistula before closure. Shunt closure resulted in restoration of intrahepatic portal flow in all, with complete or partial regression of benign liver masses, and regression or stabilization of pulmonary, cardiac, neurological, and renal complications. Congenital portosystemic shunt carries risks of severe complications in children. Closure of a shunt persisting after age 2 years should be considered preventively. Intrahepatic portal flux restoration can be expected, even when intrahepatic portal veins are extremely hypoplastic or undetectable.

Short-Term Outcome of Patients with Cirrhosis and Concurrent Portal Cavernoma Presenting with Acute Variceal Bleeding.

PubMed

Luo, Xuefeng; Wang, Wanqin; Fan, Xiaoli; Zhao, Ying; Wang, Xiaoze; Yang, Jinlin; Yang, Li

2018-01-01

The outcome of cirrhotic patients with main portal vein occlusion and portal cavernoma after the first episode of acute variceal bleeding (AVB) is unknown. We compared short-term outcomes after AVB in cirrhotic patients with and without portal cavernoma. Between January 2009 and September 2014, 28 patients with cirrhosis and portal cavernoma presenting with the first occurrence of AVB and 56 age-, sex-, and Child-Pugh score-matched cirrhotic patients without portal cavernoma were included. The primary endpoints were 5-day treatment failure and 6-week mortality. The 5-day treatment failure rate was higher in the cavernoma group than in the control group (32.1% versus 12.5%; p = 0.031). The 6-week mortality rate did not differ between the cavernoma and control group (25% versus 12.5%, p = 0.137). Multivariable Cox proportional hazard regression analyses revealed that 5-day treatment failure (HR = 1.223, 95% CI = 1.082 to 1.384; p = 0.001) independently predicted 6-week mortality. Cirrhotic patients with AVB and portal cavernoma have worse short-term prognosis than patients without portal cavernoma. The 5-day treatment failure was an independent risk factor for 6-week mortality in patients with cirrhosis and portal cavernoma.

[Effects of malnutrition on infective morbidity in the surgical treatment of portal hypertension (prospective evaluation)].

PubMed

Herrera, M F; Hoyos, C; Prado, E; Orozco, H

1991-01-01

With the aim of investigating the preoperative frequency of undernutrition and its impact on the infectious morbidity in patients without malignant disease, we studied prospectively 41 patients operated because of portal hypertension between 1987 and 1989 at the Instituto Nacional de la Nutrición. All patients were evaluated through anthropometric analysis and biochemical markers one week before the surgical procedure. A standard scheme of antibiotic profilaxis was used during surgery and the preoperative complications were registered up to discharge from the hospital. Undernutrition was considered when the serum albumin was less than 3 mg/dL or the total lymphocyte count was under 900, associated with a 10% weight loss in six months and reduction of one or more anthropometric parameters below the 30th percentile of the normal value. The group consisted of 17 males and 24 females with a mean age of 48 +/- 14 years old. 35 were Child A, four Child B and two Child C. Ten patients had a distal splenorenal shunt, seven esophageal devascularization and 24 gastric desvascularization with splenectomy. Twenty eight patients were well nourished and 13 undernourished. The two groups were comparable in all parameters except for the nutritional status. In the first group seven patients developed 10 complications and in the undernourished group eight patients had 14 complications (p less than 0.05 chi 2). There was no significant difference in the mortality rate. Infections occurred more frequently in: urinary tract, surgical wound, lung and pleura, and esophageal fistulae was an additional complication. The univariate analysis of the anthropometric parameters did not show significant differences between both groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Plug-Assisted Retrograde Transvenous Obliteration for the Treatment of Gastric Variceal Hemorrhage

PubMed Central

Chang, Min-Yung; Kim, Taehwan; Shin, Wonseon; Shin, Minwoo; Kim, Gyoung Min; Won, Jong Yun; Park, Sung Il; Lee, Do Yun

2016-01-01

Objective To evaluate the feasibility, safety, and clinical outcomes of plug-assisted retrograde transvenous obliteration (PARTO) to treat gastric variceal hemorrhage in patients with portal hypertension. Materials and Methods From May 2012 to June 2014, 19 patients (11 men and 8 women, median age; 61, with history of gastric variceal hemorrhage; 17, active bleeding; 2) who underwent PARTO using a vascular plug and a gelfoam pledget were retrospectively analyzed. Clinical and laboratory data were examined to evaluate primary (technical and clinical success, complications) and secondary (worsening of esophageal varix [EV], change in liver function) end points. Median follow-up duration was 11 months, from 6.5 to 18 months. The Wilcoxon signed-rank test was used to compare laboratory data before and after the procedure. Results Technical success (complete occlusion of the efferent shunt and complete filling of gastric varix [GV] with a gelfoam slurry) was achieved in 18 of 19 (94.7%) patients. The embolic materials could not reach the GV in 1 patient who had endoscopic glue injection before our procedure. The clinical success rate (no recurrence of gastric variceal bleeding) was the same because the technically failed patient showed recurrent bleeding later. Acute complications included fever (n = 2), fever and hypotension (n = 2; one diagnosed adrenal insufficiency), and transient microscopic hematuria (n = 3). Ten patients underwent follow-up endoscopy; all exhibited GV improvement, except 2 without endoscopic change. Five patients exhibited aggravated EV, and 2 of them had a bleeding event. Laboratory findings were significantly improved after PARTO. Conclusion PARTO is technically feasible, safe, and effective for gastric variceal hemorrhage in patients with portal hypertension. PMID:26957908

Hepatic manifestations of telomere biology disorders.

PubMed

Patnaik, Mrinal M; Kamath, Patrick S; Simonetto, Douglas A

2018-06-07

A 51-year-old Caucasian male was referred for evaluation of variceal bleeding. Laboratory tests were remarkable for mild thrombocytopenia and moderate alkaline phosphatase elevation. Synthetic liver function was well preserved. Abdominal computed tomography scan revealed moderate splenomegaly, gastric varices, and normal hepatic contour. A transjugular liver biopsy was performed revealing findings of nodular regenerative hyperplasia with no significant fibrosis or necroinflammatory activity. Hepatic venous pressure gradient was elevated at 31 mmHg, consistent with clinically significant portal hypertension. The clinical course was complicated by refractory gastric variceal bleeding requiring a surgical portosystemic shunt. Approximately seven years after the initial presentation, the patient developed progressive dyspnoea and a diagnosis of idiopathic pulmonary fibrosis was made. Contrast-enhanced echocardiogram was not suggestive of hepatopulmonary syndrome or portopulmonary hypertension. Given this new diagnosis a telomere biology disorder was suspected. A flow-fluorescence in situ hybridisation analysis for telomere length assessment revealed telomere lengths below the first percentile in both lymphocytes and granulocytes. Next generation sequencing analysis identified a heterozygous mutation involving the hTERT gene (Histidine983Threonine). The lung disease unfortunately progressed in the subsequent two years, leading to the patient's death nine years after his initial presentation with portal hypertension. During those nine years two brothers also developed idiopathic pulmonary fibrosis. The questions that arise from this case include. Copyright © 2018 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.

Use of Statins in Patients with Chronic Liver Disease and Cirrhosis: Current Views and Prospects.

PubMed

Vargas, Jose Ignacio; Arrese, Marco; Shah, Vijay H; Arab, Juan Pablo

2017-09-01

The purpose of this study is to analyze the current evidence regarding the use of statins in patients with chronic liver disease and cirrhosis. Chronic liver disease (CLD), cirrhosis, and its complications, including hepatocellular carcinoma (HCC), are significant public health problems. The use of statins in patients with CLD has been a matter of concern, and physicians are often reluctant to its prescription in these patients. This mainly relates to the potential occurrence of drug-induced liver injury. However, newer evidence from pre-clinical and clinical research has shown that statins are drugs with a potentially beneficial impact on the natural history of cirrhosis, on portal hypertension, and in HCC prevention. In this review, we summarize current evidence regarding the influence of statins in endothelial dysfunction in CLD, their ability to modulate hepatic fibrogenesis, and their vasoprotective effects in portal hypertension; we also focus on existing data about the impact of statins in cirrhosis development, progression, and complications and critically assess the current concerns about its use in patients with CLD.

Plasma Prothrombin Time and Esophageal Varices in Patients with Cirrhosis of Liver.

PubMed

Islam, Md Nasirul; Khan, Mobin; Ahmad, Nooruddin; Mamun-Al-Mahtab; Karim, Md Fazal

2016-01-01

Cirrhosis of the liver is a common complication of chronic liver disease and is associated with portal hypertension and esophageal varices. In this study, we checked the implication of prothrombin time, if any, in the genesis of esophageal varices. Sixty patients with cirrhosis of the liver were randomly assigned into two groups: Group I - 30 cirrhotic patients with esophageal varices, and group II - 30 cirrhotic patients without esophageal varices. The prothrombin time was checked for both groups. A positive correlation was found between the prolonged plasma prothrombin time (> 4 seconds) and esophageal varices with a sensitivity of 56.67% and specificity of 73.33%. The Child-Pugh score showed a correlation; however, the size of varices did not exhibit any such relation. Prothrombin time may be cautiously used to assess portal hypertension in a field level and rural setting where endoscopy is not available or feasible. Islam MN, Khan M, Ahmad N, Al-Mahtab M, Karim MF. Plasma Prothrombin Time and Esophageal Varices in Patients with Cirrhosis of Liver. Euroasian J Hepato-Gastroenterol 2016;6(1):10-12.

The clinical expression of primary biliary cirrhosis.

PubMed

Heathcote, J

1997-02-01

Primary biliary cirrhosis (PBC) is likely an autoimmune disease that destroys the interlobular bile ducts. Although the term PBC implies cirrhosis, this is not always present. The condition may be entirely silent clinically, save for the hallmark mitochondrial antibodies in serum. The clinical spectrum of PBC ranges from asymptomatic anicteric cholestasis with or without extrahepatic manifestations to severe cholestasis with decompensated cirrhosis. It is uncertain whether or not the course of this disease is universally fatal. Currently, no specific features have been identified which predict progression from asymptomatic to symptomatic disease, although once hyperbilirubinemia is present, a rising level indicates a poor prognosis. The liver-specific complications include pruritus, abdominal pain, xantholasma, and portal hypertension. The latter is often an early feature, as the portal hypertension is presinusoidal in nature and, when present, does not always reflect the presence of cirrhosis. There are many extrahepatic features of PBC, the most common being metabolic, chiefly hypothyroidism and metabolic bone disease. Other common associations are rheumatologic, renal, pulmonary, neuromuscular, and dermatologic. The non-specific yet distressing symptom of fatigue affects up to two-thirds of PBC subjects, but its etiology remains obscure.

Exploring Determinants of Patient Adherence to a Portal-Supported Oncology Rehabilitation Program: Interview and Data Log Analyses

PubMed Central

Tabak, Monique; van Velsen, Lex; van der Geest, Thea; Hermens, Hermie

2017-01-01

Background Telemedicine applications often do not live up to their expectations and often fail once they have reached the operational phase. Objective The objective of this study was to explore the determinants of patient adherence to a blended care rehabilitation program, which includes a Web portal, from a patient’s perspective. Methods Patients were enrolled in a 12-week oncology rehabilitation treatment supported by a Web portal that was developed in cooperation with patients and care professionals. Semistructured interviews were used to analyze thought processes and behavior concerning patient adherence and portal use. Interviews were conducted with patients close to the start and the end of the treatment. Besides, usage data from the portal were analyzed to gain insights into actual usage of the portal. Results A total of 12 patients participated in the first interview, whereas 10 participated in the second round of interviews. Furthermore, portal usage of 31 patients was monitored. On average, 11 persons used the portal each week, with a maximum of 20 in the seventh week and a drop toward just one person in the weeks in the follow-up period of the treatment. From the interviews, it was derived that patients’ behavior in the treatment and use of the portal was primarily determined by extrinsic motivation cues (eg, stimulation by care professionals and patient group), perceived severity of the disease (eg, physical and mental condition), perceived ease of use (eg, accessibility of the portal and the ease with which information is found), and perceived usefulness (eg, fit with the treatment). Conclusions The results emphasized the impact that care professionals and fellow patients have on patient adherence and portal usage. For this reason, the success of blended care telemedicine interventions seems highly dependent on the willingness of care professionals to include the technology in their treatment and stimulate usage among patients. PMID:29242173

A clinical hepatologist's predictions about non-absorbed carbohydrates for the early twenty-first century.

PubMed

Conn, H O

1997-01-01

To put these predictions into perspective, the primary indication for lactulose therapy in the treatment of HE and SHE is presented and discussed. Six secondary indications for lactulose therapy are also listed and briefly commented upon. A dozen predictions about the status of lactulose are presented and briefly discussed. A. Lactulose will be the treatment of choice for HE.B. TIPS will be the most common cause of HE.C. Lactulose will not be approved in Mexico. D. Lactulose plus anti-diarrheal drugs will be agents for treatment of HE. E. Lactulose will not be the treatment of choice for constipation. F. Lactulose will not be used for Salmonella or Shigella carrier states. G. Lactulose will be routinely administered prophylactically after TIPS. H. Lactulose will be administered prophylactically to cirrhotic patients with portal hypertension. I. Lactulose plus anti-diarrheal drugs will be used for a variety of diverse purposes: (i) Suppression of bacterial growth; (ii) prevention of bacteriuria; (iii) diminution of cholesterol saturation of bile; (iv) adjunct treatment of gallstones with ursodeoxycholic acid; (v) Prevention of colon carcinoma.

Spontaneous Intrahepatic Portal Venous Shunt: Presentation and Endovascular Treatment.

PubMed

Sheth, Nakul; Sabbah, Nathanael; Contractor, Sohail

2016-07-01

Spontaneous intrahepatic portal venous shunts are rare with only few case reports published. Treatments using various endovascular techniques have been described, although no single technique has been shown to be preferred. We present a patient who was referred for treatment of a spontaneous portal venous shunt and describe our treatment approach and present a review on previously reported cases. © The Author(s) 2016.

The Impact of PNPLA3 rs738409 SNP on Liver Fibrosis Progression, Portal Hypertension and Hepatic Steatosis in HIV/HCV Coinfection

PubMed Central

Scheiner, Bernhard; Mandorfer, Mattias; Schwabl, Philipp; Payer, Berit Anna; Bucsics, Theresa; Bota, Simona; Aichelburg, Maximilian C.; Grabmeier-Pfistershammer, Katharina; Stättermayer, Albert; Ferenci, Peter; Trauner, Michael; Peck-Radosavljevic, Markus; Reiberger, Thomas

2015-01-01

Background Faster fibrosis progression and hepatic steatosis are hallmarks of HIV/HCV coinfection. A single nucleotide polymorphism (SNP) of the PNPLA3-gene is associated with development of non-alcoholic steatohepatitis and a worse outcome in alcoholic liver disease. However, the role of PNPLA3 rs738409 SNP on liver fibrosis and steatosis, portal hypertension, and virological response in HIV/HCV coinfection remains unclear. Methods In this cross-sectional study PNPLA3 (rs738409) and IL28B (rs12979860) SNPs were determined in 177 HIV/HCV coinfected patients. Liver fibrosis and steatosis—staged by liver biopsy and transient elastography using the Controlled Attenuation Parameter (CAP)–and portal hypertension (hepatic venous pressure gradient, HVPG) were compared across PNPLA3 genotypes. Results 75 (42.4%) patients tested positive for a PNPLA3 minor/major risk allele (G/C:66; G/G:9) showed comparable fibrosis stages (median F2 vs. F2; p = 0.292) and similar amounts of hepatic steatosis (CAP: 203.5±41.9 vs. 215.5±59.7dB/m; p = 0.563) as compared to patients without a PNPLA3 risk allele. Advanced liver fibrosis was neither associated with PNPLA3 (p = 0.253) nor IL28B-genotype (p = 0.628), but with HCV-GT3 (p = 0.003), higher BMI (p = 0.008) and higher age (p = 0.007). Fibrosis progression rate (0.27±0.41 vs. 0.20±0.26 units/year; p = 0.984) and HVPG (3.9±2.6 vs. 4.4±3.0 mmHg; p = 0.472) were similar in patients with and without PNPLA3 risk alleles. SVR rates to PEGIFN/RBV therapy were similar across PNPLA3 genotypes. Conclusions The presence of a PNPLA3 risk allele had no independent impact on liver disease or virological response rates to PEGIFN/RBV therapy in our cohort of HIV/HCV coinfected patients. PMID:26599080

The Impact of PNPLA3 rs738409 SNP on Liver Fibrosis Progression, Portal Hypertension and Hepatic Steatosis in HIV/HCV Coinfection.

PubMed

Scheiner, Bernhard; Mandorfer, Mattias; Schwabl, Philipp; Payer, Berit Anna; Bucsics, Theresa; Bota, Simona; Aichelburg, Maximilian C; Grabmeier-Pfistershammer, Katharina; Stättermayer, Albert; Ferenci, Peter; Trauner, Michael; Peck-Radosavljevic, Markus; Reiberger, Thomas

2015-01-01

Faster fibrosis progression and hepatic steatosis are hallmarks of HIV/HCV coinfection. A single nucleotide polymorphism (SNP) of the PNPLA3-gene is associated with development of non-alcoholic steatohepatitis and a worse outcome in alcoholic liver disease. However, the role of PNPLA3 rs738409 SNP on liver fibrosis and steatosis, portal hypertension, and virological response in HIV/HCV coinfection remains unclear. In this cross-sectional study PNPLA3 (rs738409) and IL28B (rs12979860) SNPs were determined in 177 HIV/HCV coinfected patients. Liver fibrosis and steatosis-staged by liver biopsy and transient elastography using the Controlled Attenuation Parameter (CAP)-and portal hypertension (hepatic venous pressure gradient, HVPG) were compared across PNPLA3 genotypes. 75 (42.4%) patients tested positive for a PNPLA3 minor/major risk allele (G/C:66; G/G:9) showed comparable fibrosis stages (median F2 vs. F2; p = 0.292) and similar amounts of hepatic steatosis (CAP: 203.5 ± 41.9 vs. 215.5 ± 59.7 dB/m; p = 0.563) as compared to patients without a PNPLA3 risk allele. Advanced liver fibrosis was neither associated with PNPLA3 (p = 0.253) nor IL28B-genotype (p = 0.628), but with HCV-GT3 (p = 0.003), higher BMI (p = 0.008) and higher age (p = 0.007). Fibrosis progression rate (0.27 ± 0.41 vs. 0.20 ± 0.26 units/year; p = 0.984) and HVPG (3.9 ± 2.6 vs. 4.4 ± 3.0 mmHg; p = 0.472) were similar in patients with and without PNPLA3 risk alleles. SVR rates to PEGIFN/RBV therapy were similar across PNPLA3 genotypes. The presence of a PNPLA3 risk allele had no independent impact on liver disease or virological response rates to PEGIFN/RBV therapy in our cohort of HIV/HCV coinfected patients.

Transient elastography for diagnosis of advanced fibrosis and portal hypertension in patients with hepatitis C recurrence after liver transplantation.

PubMed

Carrión, Jose A; Navasa, Miquel; Bosch, Jaume; Bruguera, Miquel; Gilabert, Rosa; Forns, Xavier

2006-12-01

Recurrence of hepatitis C after liver transplantation (LT) is the main cause of graft loss and retransplantation. Frequent liver biopsies are essential to follow-up hepatitis C virus (HCV)-induced liver damage. However, liver biopsy is an invasive and expensive procedure. We evaluated prospectively the diagnostic accuracy of noninvasive measurement of liver stiffness (by transient elastography) to assess the severity of hepatitis C recurrence after LT. For this purpose, we included 124 HCV-infected liver transplant recipients who underwent 169 liver biopsies and 129 hepatic hemodynamic studies with determination of hepatic venous pressure gradient (HVPG). Simultaneously, patients underwent measurement of liver stiffness. Liver fibrosis was mild (F0-F1) in 96 cases (57%) and significant (F2-F4) in 73 (43%). HVPG was normal (<6 mm Hg) in 69 cases (54%) and elevated (>or=6 mm Hg) in 60 (46%). Using a liver stiffness cutoff value of 8.5 kilopascals, the sensitivity, specificity, negative predictive value, and positive predictive value for diagnosis of fibrosis >or=F2 were 90%, 81%, 79%, and 92%, respectively. The area under the curve (AUC) for diagnosis of fibrosis >or=F2, >or=F3 and F4 were 0.90, 0.93, and 0.98, respectively. There was a close direct correlation between liver stiffness and HVPG (Pearson coefficient, 0.84; P < 0.001) and the AUC for diagnosis of portal hypertension (HVPG >or=6 mm Hg) was 0.93. Importantly, none of the individuals with liver stiffness below the cutoff value had either bridging fibrosis (F3) or cirrhosis (F4) or significant portal hypertension (HVPG >or=10 mm Hg). In conclusion, determination of liver stiffness is an extremely valuable tool to assess the severity of HCV recurrence after LT and in reducing the need of follow-up liver biopsies.

Liver stiffness identifies two different patterns of fibrosis progression in patients with hepatitis C virus recurrence after liver transplantation.

PubMed

Carrión, José A; Torres, Ferran; Crespo, Gonzalo; Miquel, Rosa; García-Valdecasas, Juan-Carlos; Navasa, Miquel; Forns, Xavier

2010-01-01

Significant liver fibrosis (F >or= 2) and portal hypertension (hepatic venous pressure gradient [HVPG] >or= 6 mmHg) at 1 year after liver transplantation (LT) identify patients with severe hepatitis C recurrence. We evaluated whether repeated liver stiffness measurements (LSM) following LT can discriminate between slow and rapid "fibrosers" (fibrosis stage F2-F4 at 1 year after LT). Eighty-four patients who had undergone LT and who were infected with hepatitis C virus (HCV) and 19 LT controls who were not infected with HCV underwent LSM at 3, 6, 9, and 12 months after LT. All HCV-infected patients underwent liver biopsy 12 months after LT (paired HVPG measurements in 74); 31 (37%) were rapid fibrosers. Median LSM (in kilopascal) at months 6, 9, and 12 were significantly higher in rapid fibrosers (9.9, 9.5, 12.1) than in slow fibrosers (6.9, 7.5, 6.6) (P < 0.01 all time points). The slope of liver stiffness progression (kPa x month) in rapid fibrosers (0.42) was significantly greater than in slow fibrosers (0.05) (P < 0.001), suggesting two different speeds of liver fibrosis progression. Figures were almost identical for patients with HVPG >or= 6 mmHg or HVPG < 6 mmHg at 1 year after LT. Multivariate analysis identified donor age, bilirubin level, and LSM as independent predictors of fibrosis progression and portal hypertension in the estimation group (n = 50) and were validated in a second group of 34 patients. The areas under the receiver operating characteristic curve that could identify rapid fibrosers and patients with portal hypertension as early as 6 months after LT were 0.83 and 0.87, respectively, in the estimation group and 0.75 and 0.80, respectively, in the validation group. Early and repeated LSM following hepatitis C recurrence in combination with clinical variables discriminates between rapid and slow fibrosers after LT.

Portal Vein Stenting for Portal Biliopathy with Jaundice

DOE Office of Scientific and Technical Information (OSTI.GOV)

Hyun, Dongho, E-mail: mesentery@naver.com; Park, Kwang Bo, E-mail: kbjh.park@samsung.com; Lim, Seong Joo

2016-04-15

Portal biliopathy refers to obstruction of the bile duct by dilated peri- or para-ductal collateral channels following the main portal vein occlusion from various causes. Surgical shunt operation or endoscopic treatment has been reported. Herein, we report a case of portal biliopathy that was successfully treated by interventional portal vein recanalization.

Caval Subtraction 2D Phase-Contrast MRI to Measure Total Liver and Hepatic Arterial Blood Flow: Proof-of-Principle, Correlation With Portal Hypertension Severity and Validation in Patients With Chronic Liver Disease.

PubMed

Chouhan, Manil D; Mookerjee, Rajeshwar P; Bainbridge, Alan; Punwani, Shonit; Jones, Helen; Davies, Nathan; Walker-Samuel, Simon; Patch, David; Jalan, Rajiv; Halligan, Steve; Lythgoe, Mark F; Taylor, Stuart A

2017-03-01

Caval subtraction phase-contrast magnetic resonance imaging (PCMRI) noninvasive measurements of total liver blood flow (TLBF) and hepatic arterial (HA) flow have been validated in animal models and translated into normal volunteers, but not patients. This study aims to demonstrate its use in patients with liver cirrhosis, evaluate measurement consistency, correlate measurements with portal hypertension severity, and invasively validate TLBF measurements. Local research ethics committee approval was obtained. Twelve patients (mean, 50.8 ± 3.1 years; 10 men) with histologically confirmed cirrhosis were recruited prospectively, undergoing 2-dimensional PCMRI of the portal vein (PV) and the infrahepatic and suprahepatic inferior vena cava. Total liver blood flow and HA flow were estimated by subtracting infrahepatic from suprahepatic inferior vena cava flow and PV flow from estimated TLBF, respectively. Invasive hepatic venous pressure gradient (HVPG) and indocyanine green (ICG) clearance TLBF were measured within 7 days of PCMRI. Bland-Altman (BA) analysis of agreement, coefficients of variation, and Pearson correlation coefficients were calculated for comparisons with direct inflow PCMRI, HVPG, and ICG clearance. The mean difference between caval subtraction TLBF and direct inflow PCMRI was 6.3 ± 4.2 mL/min/100 g (BA 95% limits of agreement, ±28.7 mL/min/100 g). Significant positive correlations were observed between HVPG and caval subtraction HA fraction (r = 0.780, P = 0.014), but not for HA flow (r = 0.625, P = 0.053), PV flow (r = 0.244, P = 0.469), or caval subtraction TLBF (r = 0.473, P = 0.141). Caval subtraction and ICG TLBF agreement was modest (mean difference, -32.6 ± 16.6 mL/min/100 g; BA 95% limits of agreement, ±79.7 mL/min/100 g), but coefficients of variation were not different (65.7% vs 48.1%, P = 0.28). In this proof-of-principle study, caval subtraction PCMRI measurements are consistent with direct inflow PCMRI, correlate with portal hypertension severity, and demonstrate modest agreement with invasive TLBF measurements. Larger studies investigating the clinical role of TLBF and HA flow measurement in patients with liver disease are justified.

Association of Oesophageal Varices and Splanchnic Vein Thromboses in Patients with JAK2-Positive Myeloproliferative Neoplasms: Presentation of Two Cases and Data from a Retrospective Analysis

PubMed Central

Link, Cornelia S.; Platzbecker, Uwe; Kroschinsky, Frank; Pannach, Sven; Thiede, Christian; Platzek, Ivan; Ehninger, Gerhard; Schuler, Markus K.

2013-01-01

Background Oesophageal varices and gastrointestinal bleeding are common complications of liver cirrhosis. More rarely, oesophageal varices occur in patients with non-cirrhotic portal hypertension that results from thromboses of portal or splanchnic veins. Case Report We describe 2 young men who initially presented with varices as a result of portal vein thromboses. In the clinical follow-up, both were tested positive for a JAK2 mutation and consequently diagnosed with myeloproliferative neoplasms (MPNs). In an attempt to characterise the frequency of gastrointestinal complications in patients with JAK2-positive MPNs, we retrospectively analysed all known affected patients from our clinic for the diagnosis of portal vein thromboses and oesophageal varices. Strikingly, 48% of those who had received an oesophagogastroduodenoscopy had detectable oesophageal or gastric varices, and 82% of those suffered from portal or splanchnic vein thromboses. Conclusion While the association between JAK2, myeloproliferative disease and thrombotic events is well established, patients with idiopathic oesophageal varices are not regularly tested for JAK2 mutations. However, the occurrence of oesophageal varices may be the first presenting symptom of a MPN with a JAK2 mutation, and affected patients may profit from a close haematological monitoring to assure the early detection of developing MPN. PMID:23898274

PECULIARITIES OF THE CLINICAL COURSE OF NON-ALCOHOLIC STEATOHEPATITIS AGAINST THE BACKGROUND OF THE CHRONIC KIDNEY DISEASE OF THE I-III STAGE WITH SECONDARY ARTERIAL HYPERTENSION.

PubMed

Hukhlina, O; Antoniv, A; Dudka, I; Dudka, T; Mandryk, O

2017-09-01

The article addresses the theoretical generalization of the clinical study of non-alcoholic steatohepatitis peculiarities in comorbidity with obesity and chronic kidney disease of the І-ІІІ stage, characterized by higher frequency and intensity of clinical and biochemical syndromes, the manifestation of which is likely to increase the occurrence of secondary arterial hypertension (portal hypertension syndromes, cholestasis, mesenchymal inflammation). Comorbid course of non-alcoholic steatohepatitis with chronic kidney disease is characterized by higher degree of liver steatosis compared to the patients with only non-alcoholic steatohepatitis (p<0.05), and a higher diagnostic threshold of the hepatorenal index values, which correlates with the Steato-test index (p<0.001) with strong interdependence.

Current management of primary pulmonary hypertension.

PubMed

Klings, E S; Farber, H W

2001-01-01

Primary pulmonary hypertension (PPH) is a rare disorder with an annual incidence of 1 to 2 per million people. The aetiology of this disorder is unknown, but it appears to result from an abnormal interaction of environmental and genetic factors leading to a vasculopathy. The pulmonary arteries in these patients exhibit a spectrum of pathological lesions ranging from the early medial hypertrophy to the end-stage fibrotic plexiform lesions. This characteristic pathology is also observed in pulmonary hypertension resulting from connective tissue disease (particularly systemic sclerosis), HIV infection, portal hypertension and certain toxins. PPH is a condition that is difficult to diagnose and treat, with a median survival of 2.8 years in historical studies. One of the difficulties in treating patients with PHH is that the subacute nature of disease presentation often prevents an accurate diagnosis during the early stages of the illness. Progressive dyspnoea on exertion is the most common presenting symptom. Diagnostic evaluation should include electrocardiography, chest radiograph and echocardiography, and laboratory and other studies to evaluate for secondary causes (e.g. pulmonary function tests, chest computed tomography and ventilation/perfusion scans, pulmonary arteriogram, cardiopulmonary testing, right heart catherisation). PHH is a disorder for which there is no known cure. Current medical and surgical treatment options for patients with PHH include anticoagulation, vasodilators and transplantation. Calcium channel antagonists are currently the oral drugs of choice for the treatment of patients with New York Heart Association (NYHA) Class II disease. These agents, in particular the dihydropyridine compounds, have beneficial effects on haemodynamics and right ventricular function, and possibly increased survival. Epoprostenol is administered by intravenous infusion, and studies have demonstrated short- and long-term improvements in symptoms, haemodynamics and survival. It is well tolerated and has become the treatment of choice for patients with NYHA Class III and IV disease. Inotropic agents are used as a bridge to transplant, which is indicated in patients who do not respond to maximal medical therapy. Experience has shown that single lung, double lung and heart-lung transplantation are approximately of equal efficacy. Currently, single lung transplant appears to be the procedure of choice. Newer agents, such as sildenafil, beraprost and bosentan, are presently being evaluated for the treatment of this disorder. Future study should include elucidation of the pathogenic mechanisms in the development of this vasculopathy, which will hopefully lead to the development of improved treatment options for patients with PHH.

Budd-Chiari Syndrome Complicating Hydatid Cyst of the Liver Managed by Venoplasty and Stenting

DOE Office of Scientific and Technical Information (OSTI.GOV)

Sarawagi, Radha, E-mail: sarawagi_r@yahoo.co.uk; Keshava, Shyamkumar N., E-mail: aparna_shyam@yahoo.com; Surendrababu, Narayanam R. S., E-mail: nrssbabu@yahoo.com

Budd-Chiari syndrome (BCS) and portal hypertension is an uncommon complication of hydatid cyst of the liver. Previous reports describe cyst excision or portosystemic shunt surgery for such patients. Here we present a case of hydatid cyst of the liver with BCS that was treated successfully with hepatic venoplasty and transjugular stent placement.

[Treatment of nontumoral portal vein thrombosis in cirrhosis].

PubMed

Bañares, Rafael; Catalina, María-Vega

2014-07-01

Portal vein thrombosis in cirrhosis is a relatively common complication associated with the presence of an accompanying prothrombotic phenotype of advanced cirrhosis. The consequences of portal vein thrombosis are relevant because it can be associated with impaired hepatic function, might contraindicate hepatic transplantation and could increase morbidity in the surgical procedure. There is controversy concerning the most effective treatment of portal vein thrombosis, which is based on information that is seldom robust and whose primary objective is to achieve a return to vessel patency. Various studies have suggested that starting anticoagulation therapy early is associated with portal vein repatency more frequently than without treatment and has a low rate of complications. There are no proven data on the type of anticoagulant (low-molecular-weight heparins or dicoumarin agents) and the treatment duration. The implementation of TIPS is technically feasible in thrombosis without cavernous transformation and is associated with portal vein recanalization in a significant proportion of cases. Thrombolytic therapy does not appear to present an adequate balance between efficacy and safety; its use is therefore not supported for this indication. The proper definition of treatment for portal vein thrombosis requires properly designed studies to delimit the efficacy and safety of the various alternatives. Copyright © 2014 Elsevier España, S.L. All rights reserved.

Congenital portosystemic venous shunt.

PubMed

Papamichail, M; Pizanias, M; Heaton, N

2018-03-01

Congenital portosystemic venous shunts are rare developmental anomalies resulting in diversion of portal flow to the systemic circulation and have been divided into extra- and intrahepatic shunts. They occur during liver and systemic venous vascular embryogenesis and are associated with other congenital abnormalities. They carry a higher risk of benign and malignant liver tumors and, if left untreated, can result in significant medical complications including systemic encephalopathy and pulmonary hypertension. This article reviews the various types of congenital portosystemic shunts and their anatomy, pathogenesis, symptomatology, and timing and options of treatment. What is Known: • The natural history and basic management of this rare congenital anomaly are presented. What is New: • This paper is a comprehensive review; highlights important topics in pathogenesis, clinical symptomatology, and treatment options; and proposes an algorithm in the management of congenital portosystemic shunt disease in order to provide a clear idea to a pediatrician. An effort has been made to emphasize the indications for treatment in the children population and link to the adult group by discussing the consequences of lack of treatment or delayed diagnosis.

Clinical Application of Vibration Controlled Transient Elastography in Patients with Chronic Hepatitis B

PubMed Central

Liang, Xie-Er; Chen, Yong-Peng

2017-01-01

Abstract Evaluation of the extent and progression of liver fibrosis and cirrhosis is of critical importance in the management and prognosis of patients with chronic hepatitis B. Due to the limitation of liver biopsy, non-invasive methods, especially liver stiffness measurement (LSM) by vibration controlled transient elastography, have been developed and widely applied for liver fibrosis assessment. LSM aims to reduce, but not to substitute, the need for liver biopsy for fibrosis/cirrhosis diagnosis. While LSM may have potential utility in monitoring treatment response, its applications in prediction of liver complications in terms of portal hypertension and esophageal varices, as well as disease prognosis, have been gradually validated. Here, we review the latest clinical applications of LSM in patients with chronic hepatitis B. PMID:29226103

Transjugular intrahepatic portosystemic shunt with accidental diagnosis of persistence of the left superior vena cava.

PubMed

Petridis, Ioannis; Miraglia, Roberto; Marrone, Gianluca; Gruttadauria, Salvatore; Luca, Angelo; Vizzini, Giovanni Battista; Gridelli, Bruno

2010-03-07

Transjugular intrahepatic portosystemic shunt (TIPSS) is considered a valid therapeutic option for the treatment of portal hypertension and its complications. The guidelines for this procedure have already been established on the basis of the normal vascular anatomy and of various technical radiological aspects. In some few rare cases, diagnosis of a congenital vascular anomaly can be made accidentally by interventional radiologists, making the procedure of the TIPSS placement extremely difficult or in some cases technically impossible. This report describes a rare vascular malformation characterized by the absence of the right superior vena cava and persistence of the left superior vena cava in a patient with a diagnosis of advanced liver cirrhosis who needed a TIPSS placement in order to control refractory ascites.

The spleen in liver cirrhosis: revisiting an old enemy with novel targets.

PubMed

Li, Liang; Duan, Mubing; Chen, Weisan; Jiang, An; Li, Xiaoming; Yang, Jun; Li, Zongfang

2017-05-23

The spleen is a secondary lymphoid organ which can influence the progression of multiple diseases, notably liver cirrhosis. In chronic liver diseases, splenomegaly and hypersplenism can manifest following the development of portal hypertension. These splenic abnormalities correlate with and have been postulated to facilitate the progression of liver fibrosis to cirrhosis, although precise mechanisms remain poorly understood. In this review, we summarize the literature to highlight the mechanistic contributions of splenomegaly and hypersplenism to the development of liver cirrhosis, focusing on three key aspects: hepatic fibrogenesis, hepatic immune microenvironment dysregulation and liver regeneration. We conclude with a discussion of the possible therapeutic strategies for modulating splenic abnormalities, including the novel potential usage of nanomedicine in non-surgically targetting splenic disorders for the treatment of liver cirrhosis.

Stomal construction: Technical tricks for difficult situations, prevention and treatment of post-operative complications.

PubMed

Sabbagh, C; Rebibo, L; Hariz, H; Regimbeau, J M

2018-02-01

The creation of a digestive stoma, whether it is a lateral stoma or a terminal stoma, is an essential gesture in colorectal surgery, but that may result in post-operative complications in 35% of patients. Surgeons are aware of the situations at the origin of complications, although there is little factual data in the literature to discriminate them. They are related to patient-specific factors (obesity, cirrhosis, portal hypertension) or to the underlying pathology (colon obstruction) or the conditions under which the intervention is performed (emergency). The aim of this review is to describe these different situations and the data from the literature that may allow reduction of the risk of an unsatisfactory or even complicated stoma. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

 Hepatoportal sclerosis related to the use of herbals and nutritional supplements. Causality or coincidence?

PubMed

Rios, Fernanda Ferreira; Rodrigues de Freitas, Luiz Antônio; Codes, Liana; Santos Junior, Genario Oliveira; Schinoni, Maria Isabel; Paraná, Raymundo

 Introduction and aim. Non-cirrhotic idiopathic portal hypertension (NCIPH), also known as hepatoportal sclerosis (HPS) is a disease of uncertain etiology. However, various pathophysiological mechanisms has been postulated, including chronic or recurrent infections and exposure to drugs or toxins. In this context, it appears to be of multifactorial etiology or resulting from a portal vascular endothelium aggression. It is important to consider whether the use of dietary supplements and herbs can trigger or contribute to the occurance of HPS. We report a possible association of HPS with the consumption of herbals and / or dietary supplements. We describe two cases of HPS in patients without known etiology causes associated with this disease. Both patients were females who were diagnosed with HPS following the consumption of Herbalife® products and putative anorexigenic agents in the form herbals infusions. Image-based analysis and the assessment of the histopathological alterations found in the livers confirmed the diagnosis. The histopatological analysis of liver samples from both patients showed portal tracts enlarged by fibrosis with disappearance or reduction in the diameter of the portal vein branches. In many portal tracts, portal veins branches were replaced by aberrant thin-walled fendiforme vessels. The bile ducts and branches of the hepatic artery show normal aspects. After the exclusion of other etiologic factors and a comprehensive analysis of clinical history, consumption of Herbalife® products and anorexigenic agents was pointed-out as a puttative predisposing factor for the development of the disease.

Advanced non-alcoholic steatohepatitis cirrhosis: A high-risk population for pre-liver transplant portal vein thrombosis.

PubMed

Stine, Jonathan G; Argo, Curtis K; Pelletier, Shawn J; Maluf, Daniel G; Caldwell, Stephen H; Northup, Patrick G

2017-01-28

To examine if liver transplant recipients with high-risk non-alcoholic steatohepatitis (NASH) are at increased risk for pre-transplant portal venous thrombosis. Data on all liver transplants in the United States from February 2002 through September 2014 were analyzed. Recipients were sorted into three distinct groups: High-risk (age > 60, body mass index > 30 kg/m 2 , hypertension and diabetes), low-risk and non-NASH cirrhosis. Multivariable logistic regression models were constructed. Thirty-five thousand and seventy-two candidates underwent liver transplantation and of those organ recipients, 465 were transplanted for high-risk and 2775 for low-risk NASH. Two thousand six hundred and twenty-six (7.5%) recipients had pre-transplant portal vein thrombosis; 66 (14.2%) of the high-risk NASH group had portal vein thrombosis vs 328 (11.8%) of the low-risk NASH group. In general, all NASH recipients were less likely to be male or African American and more likely to be obese. In adjusted multivariable regression analyses, high-risk recipients had the greatest risk of pre-transplant portal vein thrombosis with OR = 2.11 (95%CI: 1.60-2.76, P < 0.001) when referenced to the non-NASH group. Liver transplant candidates with high-risk NASH are at the greatest risk for portal vein thrombosis development prior to transplantation. These candidates may benefit from interventions to decrease their likelihood of clot formation and resultant downstream hepatic decompensating events. Prospective study is needed.

Portal Vein Stenting for Delayed Jejunal Varix Bleeding Associated with Portal Venous Occlusion after Hepatobiliary and Pancreatic Surgery

PubMed Central

Hyun, Dongho; Cho, Sung Ki; Park, Hong Suk; Shin, Sung Wook; Choo, Sung Wook; Do, Young Soo; Choo, In Wook; Choi, Dong Wook

2017-01-01

Objective The study aimed to describe portal stenting for postoperative portal occlusion with delayed (≥ 3 months) variceal bleeding in the afferent jejunal loop. Materials and Methods Eleven consecutive patients (age range, 2–79 years; eight men and three women) who underwent portal stenting between April 2009 and December 2015 were included in the study. Preoperative medical history and the postoperative clinical course were reviewed. Characteristics of portal occlusion and details of procedures were also investigated. Technical success, treatment efficacy (defined as disappearance of jejunal varix on follow-up CT), and clinical success were analyzed. Primary stent patency rate was plotted using the Kaplan-Meier method. Results All patients underwent hepatobiliary-pancreatic cancer surgery except two children with liver transplantation for biliary atresia. Portal occlusion was caused by benign postoperative change (n = 6) and local tumor recurrence (n = 5). Variceal bleeding occurred at 27 months (4 to 72 months) and portal stenting was performed at 37 months (4 to 121 months), on average, postoperatively. Technical success, treatment efficacy, and clinical success rates were 90.9, 100, and 81.8%, respectively. The primary patency rate of portal stent was 88.9% during the mean follow-up period of 9 months. Neither procedure-related complication nor mortality occurred. Conclusion Interventional portal stenting is an effective treatment for delayed jejunal variceal bleeding due to portal occlusion after hepatobiliary-pancreatic surgery. PMID:28860900

Mechanical thrombolysis as an adjunct therapy to management of portal vein thrombosis following Radio Frequency Ablation.

PubMed

Hairol, A O; Affirul, C A; Azlanudin, A; Zamri, Z; Razman, J; Choi, S Y

2017-01-01

Radiofrequency ablation (RFA) has evolved to become the treatment of choice for non-resectable recurrent colorectal liver metastasis. It is however, not without complications. Portal vein thrombosis following RFA is rare but can be fatal to the outcome of the patient. Here, we present a case of a 66-year-old man who developed portal vein thrombosis following RFA. CT scan revealed a left portal vein thrombosis. This case report highlights the challenges and multimodal treatment of portal vein thrombosis following Radiofrequency ablation (RFA) in a cirrhotic patient.