Hemophagocytic Lymphystiocytosis is a rare and fatal complication of rheumatic diseases, particularly Adult Onset Still's Disease (AOSD). It may be precipitated with immunosuppressive drugs and with viral and bacterial infections. A diagnosis depends on a high index of suspicion associated to certain clinical manifestations (fever, rash, Splemomegaly, any cytology blood dyscrasia, hipertrigliceridemia, hiperfibrinogenemia, and others), as well as pathologic evidence of hemophagocitosis from bone marrow biopsy or tissue samples of affected organs. Therapy consists of high dose corticosteroids and immunosuppressive drugs. We present a 42 year old woman with AOSD in remission who developed HLH in spite of receiving therapy with high dose steroids and immunosuppressive drugs. She had 2 negative bone marrow aspirates. Evidence of Hemophagocytosis was detected in both bone marrow biopsies. Timely evaluation and recognition of the signs and symptoms of HLH is crucial for the prompt management and a decrease in the mortality associated with this disease. PMID:23875527
Benitez, Lourdes; Vila, Salvador; Mellado, Robert Hunter
Sixteen patients with adult onset Still's disease are reported and compared to 42 previously reported cases. The onset of this illness is sudden and is characterized by quotidian fever, evanescent rash, arthritis, leukocytosis and with variable frequency abnormalities of the liver function tests, adenopathy, splenomegaly and loss of weight. The response to anti-inflammatory therapy is satisfactory and the majority of patients will have a good functional prognosis even though they may require corticosteroids to suppress the signs and symptoms of the disease. It is stressed that Still's disease should be considered one of the diagnostic possibilities in cases of fever of unknown etiology and in cases of seronegative arthritis in adult patients. The pertinent clinical, laboratory, radiological and histological features of this illness are reviewed. PMID:555811
Jiménez Balderas, F J; Enriquez Casillas, R D; Robles Saavedra, E J; Mintz, G
First described in 1971, adult-onset Still's disease (AOSD) is a rare multisystemic disorder considered as a complex (multigenic) autoinflammatory syndrome. A genetic background would confer susceptibility to the development of autoinflammatory reactions to environmental triggers. Macrophage and neutrophil activation is a hallmark of AOSD which can lead to a reactive hemophagocytic lymphohistiocytosis. As in the latter disease, the cytotoxic function of natural killer cells is decreased in patients with active AOSD. IL-18 and IL-1?, two proinflammatory cytokines processed through the inflammasome machinery, are key factors in the pathogenesis of AOSD; they cause IL-6 and Th1 cytokine secretion as well as NK cell dysregulation leading to macrophage activation. The clinico-biological picture of AOSD usually includes high spiking fever with joint symptoms, evanescent skin rash, sore throat, striking neutrophilic leukocytosis, hyperferritinemia with collapsed glycosylated ferritin (<20%), and abnormal liver function tests. According to the clinical presentation of the disease at diagnosis, two AOSD phenotypes may be distinguished: i) a highly symptomatic, systemic and feverish one, which would evolve into a systemic (mono- or polycyclic) pattern; ii) a more indolent one with arthritis in the foreground and poor systemic symptomatology, which would evolve into a chronic articular pattern. Steroid- and methotrexate-refractory AOSD cases benefit now from recent insights into autoinflammatory disorders: anakinra seems to be an efficient, well tolerated, steroid-sparing treatment in systemic patterns; tocilizumab seems efficient in AOSD with active arthritis and systemic symptoms while TNF?-blockers could be interesting in chronic polyarticular refractory AOSD. PMID:24657513
Gerfaud-Valentin, Mathieu; Jamilloux, Yvan; Iwaz, Jean; Sève, Pascal
The prevalence of obesity is more than 20% in many developed countries and it increases in developing countries. Obesity is associated with metabolic disorders, cardiovascular diseases, pulmonary diseases, digestive diseases, and cancers. Although other specific treatments for these complications exist, weight loss is still an essential intervention in obesity and its complications. Therapeutic life change, behavior modification, pharmacotherapy, and surgery are major approaches to weight loss. In addition, medicine used in diabetes such as Glucagon-like peptide-1 analogues may be a new type of medicine for obesity, at least for those obese patients with diabetes.
Xiao, Jianzhong; Yang, Wenying
Adult-onset Still’s disease (AOSD) is a rare, systemic inflammatory disorder, characterized by spiking high fever, fever-associated\\u000a evanescent rash, arthritis, myalgia, serositis and hepatosplenomegaly. White blood cell count, neutrophilic cell count, and\\u000a serum ferritin level are markedly elevated in the active stage of the disease. Neurological complications of AOSD commonly\\u000a were cranial nerve palsies, seizures, aseptic meningoencephalitis, peripheral neuropathy and Miller–Fisher
Hong Zhao; Yun Yuan; Yue Li; Chong-Wen Si; Geng-Shan Tian; Gui-Qiang Wang; Xue-Dong Yang
AIM: To investigate recurrence rates, patterns and complications after nonoperatively managed complicated diverticulitis (CD). METHODS: A retrospective study of patients treated for CD was performed. CD was defined on computed tomography by the presence of a localized abscess, pelvic abscess or extraluminal air. For follow-up, patients were contacted by telephone. Numbers of elective surgeries, recurrences and abdominal pain were analyzed. RESULTS: A total of 114 patients (median age 57 years (range 29-97)), were admitted for CD. Nine patients required surgical intervention for failure of conservative therapy (Hartmann’s procedure: n = 6; resection and colorectal anastomosis: n = 3). Of the 105 remaining patients, 24 (22.9%) underwent elective sigmoid resection. The 81 (71%) non-operated patients were all contacted after a median follow-up of 32 mo (4-63). Among them, six had developed a recurrent episode of diverticulitis at a median follow-up of 12 mo (6-36); however, no patient required hospitalization. Sixty-eight patients (84%) were asymptomatic and 13 (16%) had recurrent abdominal pain. CONCLUSION: Conservative policy is feasible and safe in 71% of cases, with a low medium-term recurrence risk.
Bridoux, Valerie; Antor, Marlene; Schwarz, Lilian; Cahais, Julien; Khalil, Haitham; Michot, Francis; Tuech, Jean-Jacques
We describe two patients with Behcet's disease (BD) with cardiac complications and their response to treatment. This report adds to the evidence that cardiac involvement can be a feature of Behcet's disease and may be more common than previously thought. PMID:24526249
Jagadeesh, L Yalakki; Wajed, J; Sangle, S R; Carr-White, G; D'Cruz, D P
Hodgkin's disease (HD) in association with pregnancy is rarely reported. Thus, the data in the management of pregnancy complicated by HD is limited. We report here the management of advanced HD in pregnancy that was treated successfully with chemotherapy. PMID:17240590
Fadilah, S A W; Leong, C F; Jamil, M Y M A; Cheong, S K; Rozilaila, R
Ophthalmopathy, pretibial myxoedema and acropachy are progressively less common features of Graves' disease with unknown but probably related aetiologies (Table 1). Treatment for affected eyes and skin is unsatisfactory and will only improve when we understand the pathogenesis of these complications and their arcane relationship with thyroid autoimmunity. This brief review will highlight recent research which suggests that fibroblast activation is critical to the appearance of ophthalmopathy and dermopathy; more general overviews can be found elsewhere. PMID:7692461
Weetman, A P
A variety of neurological disorders have been reported in association with coeliac disease including epilepsy, ataxia, neuropathy, and myelopathy. The nature of this association is unclear and whether a specific neurological complication occurs in coeliac disease remains unproved. Malabsorption may lead to vitamin and trace element deficiencies. Therefore, patients who develop neurological dysfunction should be carefully screened for these. However, malabsorption does not satisfactorily explain the pathophysiology and clinical course of many of the associated neurological disorders. Other mechanisms proposed include altered autoimmunity, heredity, and gluten toxicity. This review attempts to summarise the literature and suggests directions for future research.
Pengiran, T; Wills, A; Holmes, G
During their disease course, the majority of Crohn's disease patients will develop a complicated disease which is characterized by the occurrence of fistulas and/or stenosis. Symptomatic, perianal fistulas should be surgically drained before anti-inflammatory therapy will be initiated. Antibiotics, such as metronidazole, improve disease symptomatic however, they are not sufficient to induce continuous fistula closure. For this purpose, azathioprine/6-mercaptopurine as well as anti-TNF antibodies are useful when administered continuously. Surgical options include seton drainage, fistula excision, fistula plugs and mucosa flaps. As ultima ratio, temporary ileostomy and proctectomy are to be discussed. Non-perianal fistulas often require surgical approaches. Symptomatic strictures or stenosis can be treated by anti-inflammatory medications (only if they are cause by inflammation), endoscopic balloon dilatation or surgery. PMID:24618311
Scharl, Michael; Barthel, Christiane; Rogler, Gerhard
Seventy-three patients were seen between 1970 and 1983 with complicated diverticular disease. There were only six hospital deaths (8%). Two out of 7 patients with faecal peritonitis died, 2 of 27 patients with purulent peritonitis died and there was one death each associated with an inflammatory mass and a peridiverticular abscess. Five of the six hospital deaths were from cardiorespiratory disease and only one was from sepsis. Three of the early deaths were in patients who were receiving steroid therapy. There were three late deaths: one from uncontrolled sepsis, one an anaesthetic death from coronary occlusion during revision of a Hartmann operation and the third was an incidental myocardial infarction. A very conservative surgical policy was adopted, primary resection only being used for an inflammatory mass and selectively for fistula and local purulent disease. Despite our apparent low hospital mortality there was a high incidence of complication; wound sepsis 29%, fistula after colostomy closure 12% and anastomotic dehiscence after primary or secondary reconstruction 12%. These findings indicate the need for a prospective audit which is now in progress.
Shephard, A. A.; Keighley, M. R.
Diverticular disease is more frequently seen in our practice. The aim of this retrospective study was to evaluate our experience with complicated diverticular disease in our surgical emergency unit. Between 2004-2007 46 cases with complicated diverticular disease were registered in Surgical Emergency Clinical Hospital of Bucharest. A male preponderance (sex ratio M: F 1:1.4) and a medium age of 62.9 +/- 15 years were recorded. Perforation was the main complication encountered and sigmoid colon was the most frequent involved segment (71.7%). Diagnostic procedures relayed especially on CT-scan. Three cases were operated laparoscopically. Failure of conservative measures was the main cause for interventions. A Hartmann procedure was performed in 7 patients (15.2%). Global mortality was 8.1% (4 cases). There were no significant differences among the surgical procedures employed. Diverticular disease is more frequently encountered in our practice. CT-scan is the most efficient diagnostic tool but there are limitations. Conservative measures are not always successful and urgent operative treatment is the only choice. Hartmann procedure is still frequently employed in our practice. PMID:19388565
Beuran, M; Iordache, F; Chiotoroiu, A L; Teleanu, G; Vartic, Mihaela; Turcule?, C; Surdeanu, D; Ro?u, Oana
: Adult-onset Still’s disease (AOSD) is a rare disorder of unknown aetiology, characterised by high spiking fever, an evanescent,\\u000a erythematous, maculopapular rash, arthralgia or arthritis, lymphadenopathy, hepatosplenomegaly, sore throat and serositis.\\u000a It is associated with marked leukocytosis, high erythrocyte sedimentation rate, increased level of serum ferritin and negative\\u000a rheumatoid factor and antinuclear antibody tests. Here we report a patient in
F. Salaffi; G. Filosa; L. Bugatti; M. D. Maestrini
A case of adult-onset Still’s disease complicated by hemophagocytic lymphohistiocytosis is reported. Its management is being\\u000a discussed on the background of the latest literature with special regard on the use of high-dose corticosteroids and immunosuppressive\\u000a agents.
Urs Bürgi; Adriana Mendez; Paul Hasler; Hans Dieter Hüllstrung
We report two cases of hydatic cyst with rupture into the aorta in two young patients cared for at the Ibn Sina hospital in Rabat, Morocco. In the first patient, a false hydatic aneurysm of the descending thoracic aorta was discovered at surgery performed for suspected hydatic cyst of the lower lobe of the left lung. Despite reconstruction with a prosthetic graft, the patient died peroperatively due to exsanguination via uncontrollable bleeding through the aneurysmal sac. The second case was a 20-year-old woman whose false hydatic aneurysm of the thoracoabdominal aorta was disclosed by embolic ischemia of the lower limbs. Aorto-aortic reconstruction was followed by medical treatment with albendazol. During follow-up, the patient developed a hydatic cyst of the kidney and a parietal cyst. Both were treated surgically. An analysis of these two cases and 9 others reported in the literature concerning arterial involvement in hydatic disease revealed the characteristic clinical, radiological and therapeutic features of this rare but serious complication. PMID:12015489
Mayoussi, C; El Mesnaoui, A; Lekehal, B; Sefiani, Y; Benosman, A; Bensaid, Y
Adult onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. Systemic onset juvenile idiopathic\\u000a arthritis (SoJIA) is the preferred nomenclature of Still’s disease. Strong association with so-called macrophage activation\\u000a syndrome (MAS) may provide a clue to the understanding of the distinctive pathogenetic features of SoJIA. MAS is a severe,\\u000a potentially life-threatening complication characterized by the excessive
Francisco Hélder C. Félix; Luzia Kalyne A. M. Leal; Juvenia Bezerra Fontenele
Hemophagocytic syndrome is a potentially fatal complication that rarely occurs after liver transplantation. We present a 25-year-old man with a history of Still’s disease who presented with fever, arthralgia, and elevated serum ferritin levels 6 months after undergoing liver transplantation for fulminant hepatic failure due to autoimmune hepatitis potentially triggered by infliximab therapy. Liver biopsy demonstrated features consistent with hemophagocytic syndrome. The patient was successfully treated with a course of high dose steroids and had complete resolution of his symptoms and normalization of liver chemistry test abnormalities. Patients with Still’s disease may rarely complicate with fulminant hepatic failure with infliximab therapy. Hemophagocytic syndrome a rare potentially life threatening condition may occur in such patients following liver transplantation.
Satapathy, Sanjaya K.; Isabel Fiel, M.; Del Rio Martin, Juan; Aloman, Costica
A 57 year old woman presented with clinical and biological features of adult onset Still's disease. A kidney biopsy was performed at the onset of the condition and was found to be normal. Four years later, in view of a nephrotic syndrome, histopathological examination of a new kidney biopsy specimen was made and showed typical amyloid deposition. Renal amyloidosis was suspected in five previously reported cases but demonstrated by kidney biopsy in only two of them. In the present case renal amyloidosis was recorded after disease of four years' duration. The normality of the first renal biopsy specimen suggests the possibility of a direct relation between amyloidosis and adult onset Still's disease. Images
Wendling, D; Humbert, P G; Billerey, C; Fest, T; Dupond, J L
We describe the case of a 43-year-old man recently diagnosed with ileal Crohn's disease complicated by a free peritoneal perforation of a Meckel's diverticulum and the presence of enteroliths in the intestinal lumen. The coexistence of Crohn?s disease, Meckel's diverticulum and enteroliths has rarely been reported. Meckel's diverticulum can hamper the management of Crohn's disease. PMID:22749502
Fernández Salazar, Luis; Alvarez-Quiñones Sanz, María; Sánchez Lite, Israel; Velayos Jiménez, Benito; Legido Morán, Patricia; Macho Conesa, Ana; González Hernández, José Manuel
Still’s disease is very rare in elderly patients. We report a case of Still’s disease in an elderly patient that had an atypical initial presentation. A 76-year-old woman developed unexplained chronic anemia and thrombocytosis. Three years later she had acute onset of high fever, arthritis, maculopapular rash, pleuritic chest pain, abdominal pain, lymphadenopathy and elevated erythrocyte sedimentation rate. Rheumatoid factor
Luis M. Vilá; María J. Molina
In the past several decades, Hodgkin's disease has been transformed from a uniformly fatal illness to one that can be treated with the expectation of long-term remission or cure in the majority of patients. Because patients now survive for long periods after curative intervention, various complications have been identified. The spectrum of complications following curative therapy is quite diverse and includes immunologic, cardiovascular, pulmonary, thyroid, and gonadal dysfunction. In addition, second malignant neoplasms in the form of acute leukemia as well as secondary solid tumors have now been documented to occur with increased frequency in patients cured of Hodgkin's disease. 80 references.
Young, R.C.; Bookman, M.A.; Longo, D.L. (Fox Chase Cancer Center, Philadelphia, PA (USA))
Hemophagocytic syndrome is a potentially fatal complication that rarely occurs after liver transplantation. We present a 25-year-old man with a history of Still's disease who presented with fever, arthralgia, and elevated serum ferritin levels 6 months after undergoing liver transplantation for fulminant hepatic failure due to autoimmune hepatitis potentially triggered by infliximab therapy. Liver biopsy demonstrated features consistent with hemophagocytic syndrome. The patient was successfully treated with a course of high dose steroids and had complete resolution of his symptoms and normalization of liver chemistry test abnormalities. Patients with Still's disease may rarely complicate with fulminant hepatic failure with infliximab therapy. Hemophagocytic syndrome a rare potentially life threatening condition may occur in such patients following liver transplantation. PMID:21442058
Satapathy, Sanjaya K; Isabel Fiel, M; Del Rio Martin, Juan; Aloman, Costica; Schiano, Thomas D
Diseases of the liver and the biliary tract are commonly observed in patients with inflammatory bowel diseases (IBD). Besides primary sclerosing cholangitis (PSC), drug-induced hepatotoxicity and non-alcoholic fatty liver disease (NAFLD) are the most frequent liver complications in IBD. PSC is a chronic inflammatory and commonly progressive disorder of unknown etiology associated with fibrosis and stricture development in the intrahepatic and extrahepatic biliary tree. Interestingly, this form of liver disease is mainly associated with ulcerative colitis. Development of PSC is highly relevant for IBD patients as cholestasis-associated problems increase over time resulting in biliary strictures, cholangitis, cholangiocarcinoma and importantly these patients also have a higher risk to develop colon cancer. Another major aspect regarding IBD and liver disease refers to drug-induced hepatotoxicity. Clinically, most relevant is liver toxicity caused by immunosuppressants such as azathioprine. Azathioprine and its derivate 6-mercaptopurine can cause a spectrum of liver injuries ranging from asymptomatic elevated liver enzymes to cholestasis and nodular regenerative hyperplasia. The third common IBD-associated liver disease is NAFLD, and first studies suggest that NAFLD might appear in IBD patients independent of classical risk factors such as obesity or insulin resistance. Overall, liver complications are observed in 10-20% of IBD patients, and therefore physicians have to be familiar with these complications to improve and to optimize patient care. PMID:24030232
Wieser, V; Gerner, R; Moschen, A R; Tilg, H
Iron is the second most abundant metal in the Earth’s crust. Despite being present in trace amounts, it is an essential trace element for the human body, although it can also be toxic due to oxidative stress generation by the Fenton reaction, causing organic biomolecule oxidation. This process is the basis of numerous pathologies, including cardiovascular diseases (CVD). The relationship between iron and cardiovascular disease was proposed in 1981 by Jerome Sullivan. Since then, numerous epidemiological studies have been conducted to test this hypothesis. The aim of this review is to present the main findings of the chief epidemiological studies published during the last 32 years, since Sullivan formulated his iron hypothesis, suggesting that this element might act as a risk factor for cardiovascular disease. We have analyzed 55 studies, of which 27 supported the iron hypothesis, 20 found no evidence to support it and eight were contrary to the iron hypothesis. Our results suggest that there is not a high level of evidence which supports the hypothesis that the iron may be associated with CVD. Despite the large number of studies published to date, the role of iron in cardiovascular disease still generates a fair amount of debate, due to a marked disparity in results.
Munoz-Bravo, Carlos; Gutierrez-Bedmar, Mario; Gomez-Aracena, Jorge; Garcia-Rodriguez, Antonio; Fernandez-Crehuet Navajas, Joaquin
There is growing interest in the pathogenesis, treatment, and prevention of long-term complications of HIV disease and its therapies. Specifically, studies focused on cardiovascular, renal, bone, and fat abnormalities were prominent at the 17th Conference on Retroviruses and Opportunistic Infections. Although enthusiasm about the effectiveness of current antiretroviral therapy remains strong, collectively, the ongoing work in the area of HIV disease and treatment complications appears to reflect concerns that these clinical problems will continue to remain important and possibly increase over time in the current therapeutic era. This year’s conference also highlighted important data on prevention and optimal treatment of common coinfections that occur in HIV-infected individuals, including tuberculosis, influenza, and viral hepatitis.
Luetkemeyer, Anne F.; Havlir, Diane V.; Currier, Judith S.
Sickle cell disease (SCD) is a common monogenetic disorder with high associated morbidity and mortality. The pulmonary complications of SCD are of particular importance, as acute chest syndrome and pulmonary hypertension have the highest associated mortality rates within this population. This article reviews the pathophysiology, diagnosis, and treatment of clinically significant pulmonary manifestations of SCD, including acute chest syndrome, asthma, and pulmonary hypertension in adult and pediatric patients. Clinicians should be vigilant in screening and treating such comorbidities to improve patient outcomes.
Miller, Andrew C.
Fabry disease is an X-linked ?-galactosidase A deficiency, resulting in accumulation of glycosphingolipids, especially globotriaosylceramide, in cells in different organs in the body. Renal failure is a serious complication of this disease. Fabry nephropathy lesions are present and progress in childhood while the disease commonly remains silent by routine clinical measures. Early and timely diagnosis of Fabry nephropathy is crucial since late initiation of enzyme replacement therapy may not halt progressive renal dysfunction. This may be challenging due to difficulties in diagnosis of Fabry disease in children and absence of a sensitive non-invasive biomarker of early Fabry nephropathy. Accurate measurement of glomerular filtration rate and regular assessment for proteinuria and microalbuminuria are useful, though not sensitive enough to detect early lesions in the kidney. Recent studies support the value of renal biopsy in providing histological information relevant to kidney function and prognosis and renal biopsy could potentially be used to guide treatment decisions in young Fabry patients. This review aims to provide an update of the current understanding, challenges and needs to better approach renal complications of Fabry disease in children.
Najafian, Behzad; Mauer, Michael; Hopkin, Robert J; Svarstad, Einar
A 24-year-old man presented to the emergency department with fever, maculopapular rash, myalgia and polyarthralgia, thoracic pain and dry cough, which had been present for 24?h. At the time of observation he had high fever (39°C), maculopapular rash on the torso, arms and legs proximally, axillary adenopathies and pharyngitis. Laboratorial data showed elevated inflammation markers (leukocytosis, C reactive protein of 44?mg/dL, erythrocyte sedimentation rate of 120?mm), elevated transaminases, lactate dehydrogenase, ferritin levels (>2000?ng/mL) and rising troponin. ECG had sinus rhythm and ST elevation in leads V1-V5. Thoracic radiography revealed bilateral interstitial infiltrate confirmed by CT scan. Echocardiographic findings included diffuse hypokinesia of the left ventricle and impaired systolic function. After the investigation of an infectious or autoimmune aetiology was negative, the diagnosis of adult-onset Still's disease was considered. The patient was put on a 60?mg/day prednisolone regimen with remission of symptoms and normalisation of systolic function and ECG. PMID:24898998
Gonzalez, Filipe André; Beirão, Pedro; Adrião, Joana; Coelho, Margarida Lopes
Chronic kidney disease (CKD) is a critical and rapidly growing global health problem. Neurological complications occur in almost all patients with severe CKD, potentially affecting all levels of the nervous system, from the CNS through to the PNS. Cognitive impairment, manifesting typically as a vascular dementia, develops in a considerable proportion of patients on dialysis, and improves with renal transplantation. Patients on dialysis are generally weaker, less active and have reduced exercise capacity compared with healthy individuals. Peripheral neuropathy manifests in almost all such patients, leading to weakness and disability. Better dialysis strategies and dietary modification could improve outcomes of transplantation if implemented before surgery. For patients with autonomic neuropathy, specific treatments, including sildenafil for impotence and midodrine for intradialytic hypotension, are effective and well tolerated. Exercise training programs and carnitine supplementation might be beneficial for neuromuscular complications, and restless legs syndrome in CKD responds to dopaminergic agonists and levodopa treatment. The present Review dissects the pathophysiology of neurological complications related to CKD and highlights the spectrum of therapies currently available. PMID:19724248
Krishnan, Arun V; Kiernan, Matthew C
Background: Cardiovascular diseases are the leading causes of death around the world. The coronary artery disease (CAD) is one of the most common diseases in this category, which can be the trigger to various psychosocial complications. We believe that inadequate attention has been paid to this issue. Objectives: The purpose of the present study was to explore the psychosocial complications of CAD from the Iranian patients’ perspective. Patients and Methods: A qualitative design based on the content analysis approach was used to collect the data and analyze the perspective of 18 Iranian patients suffered from CAD, chosen by a purposeful sampling strategy. Semi-structured interviews were held in order to collect the data. Sampling was continued until the data saturation. The data were analyzed using qualitative content analysis approach by MAXQUDA 2010 software. Results: This study revealed the theme of the patients’ challenges with CAD. This theme consisted of: "primary challenges," including doubting early diagnosis and treatment, and feeling being different from others; "psychological issues," including preoccupation, fear of death and surgical intervention, recurrence stress , anxiety and depression; "problems of life," including financial problems, work-related problems, and family-related problems; and "sociocultural issues," including change in perspective of people towards the patient, and cultural issues. Conclusions: Although the management of physical problems in patients with CAD is important, psychosocial effects of this disease is more important. Thus, health care personnel should pay ample attention to identify and resolve psychosocial problems of these patients. Results of this study can be used to empower these patients.
Karimi-Moonaghi, Hossein; Mojalli, Mohammad; Khosravan, Shahla
In the small intestine of celiac disease patients, dietary wheat gluten and similar proteins in barley and rye trigger an inflammatory response. While strict adherence to a gluten-free diet induces full recovery in most patients, a small percentage of patients fail to recover. In a subset of these refractory celiac disease patients, an (aberrant) oligoclonal intraepithelial lymphocyte population develops into overt lymphoma. Celiac disease is strongly associated with HLA-DQ2 and/or HLA-DQ8, as both genotypes predispose for disease development. This association can be explained by the fact that gluten peptides can be presented in HLA-DQ2 and HLA-DQ8 molecules on antigen presenting cells. Gluten-specific CD4+ T cells in the lamina propria respond to these peptides, and this likely enhances cytotoxicity of intraepithelial lymphocytes against the intestinal epithelium. We propose a threshold model for the development of celiac disease, in which the efficiency of gluten presentation to CD4+ T cells determines the likelihood of developing celiac disease and its complications. Key factors that influence the efficiency of gluten presentation include: (1) the level of gluten intake, (2) the enzyme tissue transglutaminase 2 which modifies gluten into high affinity binding peptides for HLA-DQ2 and HLA-DQ8, (3) the HLA-DQ type, as HLA-DQ2 binds a wider range of gluten peptides than HLA-DQ8, (4) the gene dose of HLA-DQ2 and HLA-DQ8, and finally,(5) additional genetic polymorphisms that may influence T cell reactivity. This threshold model might also help to understand the development of refractory celiac disease and lymphoma.
van Bergen, Jeroen; Koning, Frits
After more than 40 years of clinical use, levodopa (LD) remains the gold standard of symptomatic efficacy in the drug treatment of Parkinson’s disease (PD). Compared with other available dopaminergic therapies, dopamine replacement with LD is associated with the greatest improvement in motor function. Long-term treatment with LD is, however, often complicated by the development of various types of motor response oscillations over the day, as well as drug-induced dyskinesias. Motor fluctuations can be improved by the addition of drugs such as entacapone or monoamine oxidase inhibitors, which extend the half-life of levodopa or dopamine, respectively. However, dyskinesia control still represents a major challenge. As a result, many neurologists have become cautious when prescribing therapy with LD. This review summarizes the available evidence regarding the use of LD to treat PD and will also address the issue of LD delivery as a critical factor for the drug’s propensity to induce motor complications.
Poewe, Werner; Antonini, Angelo; Zijlmans, Jan CM; Burkhard, Pierre R; Vingerhoets, Francois
The association of chronic liver disease with respiratory symptoms and hypoxia is well recognized. Over the last century, three pulmonary complications specific to chronic liver disease have been characterized: hepatopulmonary syndrome (HPS), portopulmonary hypertension (POPH), and hepatic hydrothorax (HH). The development of portal hypertension is fundamental in the pathogenesis of each of these disorders. HPS is the most common condition, found in 5%-30% of cirrhosis patients, manifested by abnormal oxygenation due to the development of intrapulmonary vascular dilatations. The presence of HPS increases mortality and impairs quality of life, but is reversible with liver transplantation (LT). POPH is characterized by development of pulmonary arterial hypertension in the setting of portal hypertension, and is present in 5%-10% of cirrhosis patients evaluated for LT. Screening for POPH in cirrhosis patients eligible for LT is critical since severe POPH is a relative contraindication for LT. Patients with moderate POPH, who respond adequately to medical therapy, may benefit from LT, although sufficient controlled data are lacking. HH is a transudative pleural effusion seen in 5%-10% of cirrhosis patients, in the absence of cardiopulmonary disease. Diagnosis of HH should prompt consideration for LT, which is the ultimate treatment for HH. Conservative management includes salt restriction and diuretics, with thoracentesis and transjugular intrahepatic portosystemic shunt (TIPS) as second-line therapeutic options. PMID:24089295
Machicao, Victor I; Balakrishnan, Maya; Fallon, Michael B
Thromboembolic complications following splenectomy for hematologic diseases occur in up to 10% of patients and may range from portal vein thrombosis (PVT) to pulmonary embolism (PE) and deep vein thrombosis (DVT). Up to now there exist no recommendations for the duration and intensity of prophylactic anticoagulation, which usually follows local institutional protocols. We report on three consecutive patients with severe portal vein thrombosis and/or pulmonary embolism--one with fatal outcome--7 to 35 days after splenectomy for autoimmune hemolytic anemia, immunothrombocytopenia, and indolent lymphoma, respectively. Incidence and pathophysiology of thromboembolic events (TE) in this patient group as well as prophylactic anticoagulation will be discussed, including a review of the current literature on this topic. PMID:15164380
Mohren, Martin; Markmann, Ilka; Dworschak, Ulrike; Franke, Astrid; Maas, Christian; Mewes, Sabine; Weiss, Günter; Jentsch-Ullrich, Kathleen
Vertebral osteomyelitis complicating Crohn's disease is a rare occurrence and mostly occurred in patients with Crohn's disease complicated by an abscess or fistulising disease. We report a case of thoracic vertebral osteomyelitis, occurring in a refractory Crohn's disease without contiguous abscess or fistula with the bowel. PMID:24916975
Ajayi, Olushola; Mayooran, Nithiananthan; Iqbal, Nasir
Adult-onset Still's disease (AOSD), an autoinflammatory syndrome of unknown etiology, typically manifests with spiking fevers, polyarthritis, and characteristic evanescent rash. We describe a young woman with AOSD complicated by calf fasciitis that serendipitously responded to clarithromycin administered for another indication. Remarkable improvement followed rechallenges with clarithromycin for subsequent AOSD flares. In addition to their antibacterial actions, macrolides demonstrate immunomodulatory effects, including suppression of proinflammatory cytokine production and neutrophil action. Previous clinical trials provide promising preliminary evidence of a therapeutic effect of macrolides in chronic inflammatory diseases. Although AOSD pathogenesis remains unclear, a role for dysregulation of innate immunity is supported by recent literature. Based on this possible innate immune mechanism, we suspect that macrolides may have induced a therapeutic response in this patient with AOSD. A clinical trial is warranted to establish or refute their therapeutic efficacy. PMID:21946464
Thanou-Stavraki, Aikaterini; Aberle, Teresa; Aksentijevich, Ivona; Bane, Barbara L; Harley, John B
Adamantiades-Behcet's disease (ABD) is a chronic, relapsing, systemic vasculitis of unknown etiology. It is more prevalent in populations along the ancient Silk Road from Eastern Asia to the Mediterranean Basin, and most frequently affects young adults between the second and fourth decades of life. ABD-complicated gastroenteropathy is a significant cause of morbidity and mortality, with abdominal pain as the most common symptom. The ileocecal region is affected predominantly, with ulcerations that may lead to penetration and/or perforation, whereas other parts of the gastrointestinal system including the esophagus and stomach can also be affected. Endoscopy is useful to locate the site and extent of the lesions, and tissue biopsy is often warranted to examine the histopathology that is often suggestive of underlying vasculitis of small veins/venules or, alternatively in some cases, nonspecific inflammation. Bowel wall thickening is the most common finding on computed tomography scan. Treatment is largely empirical since well-controlled studies are difficult to conduct due to the heterogeneity of the disease, and the unpredictable course with exacerbation and remission. Corticosteroids with or without other immunosuppressive drugs, such as cyclophosphamide, azathioprine, sulfasalazine, tumor necrosis factor ? antagonist or thalidomide should be applied before surgery, except in emergency. PMID:22363131
Wu, Qing-Jun; Zhang, Feng-Chun; Zhang, Xuan
As part of a health technology assessment, economic evaluation of health technologies for rare diseases poses specific challenges given that such technologies are rarely cost-effective. Therefore, conventional economic evaluation techniques appear to be less relevant to health technologies for rare diseases. However, the definition of health technology assessment points to multi-criteria decision analysis by stating that a health technology needs to be assessed against multiple criteria in order to pronounce a judgement about the value of the health technology. Thus, this editorial argues that a full health technology assessment which uses a multi-criteria decision analysis framework to evaluate the value of a technology can be applied to health technologies for rare diseases. Past experiences demonstrate that the specific characteristics of health technologies for rare diseases can fit in the conventional health technology assessment paradigm by means of multi-criteria decision analysis. PMID:24702042
Little is known about the interactions between adult-onset Still's disease (AOSD) and pregnancy. In an attempt to clarify the link between these 2 conditions, we retrospectively analyzed patients registered as suffering from AOSD seen in our university hospital. A total of 57 patients, among them 30 women, were diagnosed. Ten pregnancies in 8 women were identified. Three cases manifested AOSD in their first trimester, all treated with prednisone. Premature births and flares occurred in 2 patients. One patient developed a monocyclic AOSD during her second pregnancy's postpartum. In the 4 other cases, AOSD was known and quiescent before pregnancy. One patient had 2 pregnancies without any flare or complication. One patient experienced her first pregnancy while under treatment and presented a late flare 8 months after delivery. The third patient developed exacerbation in the first trimester of her second pregnancy which was treated with IgIV alone. The last one presented her first pregnancy 7 years after diagnosis. A prednisone-treated systemic flare occurred during the first trimester without later complication. Based on our own experience and the analysis of only two series of the literature, including, respectively, 4 and 5 patients, we suggest that two settings could be distinguished. First, AOSD can occur during pregnancy and can be responsible for obstetrical complications. Then, in patients with known AOSD, the second trimester and postpartum appear to be periods exposing to disease recurrence. Thus, we recommend a close multidisciplinary monitoring by a rheumatologist and an obstetrician prior to, during and after pregnancies. PMID:23624554
Gerfaud-Valentin, M; Hot, A; Huissoud, C; Durieu, I; Broussolle, C; Seve, P
Introduction Lymphadenopathy is found in about 65% of patients with adult-onset Still’s disease and is histologically characterized by an intense, paracortical immunoblastic hyperplasia. Adult-onset Still’s disease has not been previously described as an etiology of suppurative necrotizing granulomatous lymphadenitis. Case presentation We describe a 27-year-old Greek man who manifested prolonged fever, abdominal pain, increased inflammatory markers, episodic skin rash and mesenteric lymphadenopathy histologically characterized by necrotizing granulomatous adenitis with central suppuration. Disease flares were characterized by systemic inflammatory response syndrome with immediate clinico-laboratory response to corticosteroids but the patient required prolonged administration of methylprednisolone at a dose of above 12mg/day for disease control. After an extensive diagnostic work-up, which ruled out any infectious, malignant, rheumatic or autoinflammatory disease the patient was diagnosed as having adult-onset Still’s disease. The patient is currently treated with 4mg of methylprednisolone, 100mg of anakinra daily and methotrexate 7.5mg for two consecutive days per week and exerts full disease remission for six months. Conclusion To the best of our knowledge this is the first report of suppurative necrotizing granulomatous lymphadenitis attributed to adult-onset Still’s disease. This case indicates that the finding of a suppurative necrotizing granulomatous lymphadenitis should not deter the consideration of adult-onset Still’s disease as a potential diagnosis in a compatible clinical context; however, the exclusion of other diagnoses is a prerequisite.
The complications of gastroesophageal reflux disease (GERD) include ulcers, strictures, Barrett's esophagus and carcinoma. Although the prevalence of GERD is very high, the development of complications remains quite rare and usually occurs in association with factors generally observed in more severe disease such as hiatal hernia, bile reflux or severely disturbed motility. Recent studies have emphasized the role of obesity and genetic factors as aggravating factors in the development of GERD complications. Barrett's esophagus is the most prevalent complication of GERD and seems to be associated with an increased mortality rate. However, cancer incidence is low and most patients die from other causes, especially cardiovascular disease. PMID:19439954
Belhocine, Kafia; Galmiche, Jean-Paul
We report a case of sudden thigh pain from spontaneous quadriceps necrosis, also known as diabetic myonecrosis, in a 28-year-old patient with poorly controlled diabetes mellitus. Diabetic muscle infarction is a rare end-organ complication seen in patients with poor glycemic control and advanced chronic microvascular complications. Proposed mechanisms involve atherosclerotic microvascular occlusion, ischemia-reperfusion related injury, vasculitis with microthrombi formation, and an acquired antiphospholipid syndrome. Diabetic myonecrosis most commonly presents as sudden thigh pain with swelling and should be considered in any patient who has poorly controlled diabetes mellitus.
Sran, Manpreet; Ferguson, Nicole
An overview on the diversity of neurologic complications of infections with different human herpes virus strains is given. Encephalitis, meningoencephalitis and the Guillain-Barré-syndrome are of major importance. Affection of single cranial nerves and mononeuropathies occur in a lesser frequency, while myelitis and isolated disturbances of the autonomic nervous system are rare complications. Therapeutically the application of acyclovir in herpes simplex and varicella zoster virus infections has given encouraging outlooks, whereas no convincing results exist with respect to cytomegalovirus and Epstein-Barr virus infections. PMID:3013738
Bleistein, J; Tackmann, W
Coeliac disease is a malabsorption syndrome in which dietary gluten damages the small bowel mucosa. Gluten contains gliadin, the primary toxic component that is primarily found in wheat, barley and rye products. The initial diagnosis of coeliac disease is usually made by endoscopic biopsy of the jejunum although sometimes imaging features can suggest the diagnosis. Once a diagnosis is made, patients need to be diet compliant and monitored for potential complications. Many complications are more common when dietary compliance is poor. Complications include intussusception (usually intermittent), ulcerative jejunitis, osteomalacia, cavitating lymph node syndrome and an increased risk of malignancies such as lymphoma, adenocarcinoma and squamous cell carcinoma. Radiological evaluation is central in the evaluation of these complications. Imaging may assist both in the diagnosis and staging of complications as well as enabling radiological guided percutaneous biopsy for complications of coeliac disease such as lymphoma. As coeliac disease is a relatively common disorder, it is likely that most radiologists will encounter the disease and its potential complications. The aim of this review article is to discuss and illustrate the role of modern radiology in evaluating the many presentations of this complex disease. PMID:17531415
Buckley, O; Brien, J O; Ward, E; Doody, O; Govender, P; Torreggiani, W C
Six patients with an aortoiliac vascular disease and a peripheral neurological deficit are presented. Clinical and electromyographic findings revealed lumbosacral plexus, sciatic and femoral nerve lesions. A correlation is made between the level of the vascular lesion (aortic, aortoiliac or distally) and the type of peripheral nerve deficit observed. In a patient complaining of pain, weakness, or numbness in a leg, the differential diagnosis should include aortoiliac vascular disease. The peripheral neurological symptoms may be the initial manifestation of the vascular disease or may appear in the early post-operative period. PMID:3038289
D'Amour, M L; Lebrun, L H; Rabbat, A; Trudel, J; Daneault, N
Neurologic complications of Hodgkin's disease (HD) include both metastatic and non-metastatic involvement of the nervous system. There are at least five paraneoplastic syndromes associated with HD but chorea has not been described. We report the first choreiform disorder as a paraneoplastic complication of HD and only the second case of paraneoplastic chorea in the literature.
T. T. Batchelor; M. Platten; D. E. Palmer-Toy; G. J. Hunter; M. H. Lev; J. Dalmau; F. H. Hochberg
Pulmonary hypertension (PH) may complicate parenchymal lung disease, specifically interstitial lung diseases and chronic obstructive pulmonary disease, and uniformly increases the mortality risk. The epidemiology and degree of PH is variable and unique to the underlying lung disease. The clinician should exercise a high index of suspicion for PH complicating parenchymal lung disease especially given the nonspecific symptomatology and the limitations of echocardiography in this patient population. In general, PH-specific therapies in this setting have been poorly studied, with concern for increased shunting and/or ventilation/perfusion (V/Q) mismatch and resultant hypoxemia. A better understanding of the mechanisms underlying PH related to parenchymal lung disease may lead to novel pharmacological targets to prevent or treat this serious complication. PMID:24037628
Shino, Michael Y; Lynch, Joseph P; Saggar, Rajeev; Abtin, Fereidoun; Belperio, John A; Saggar, Rajan
Patients with cirrhosis and portal hypertension exhibit characteristic cardiovascular and pulmonary hemodynamic changes. A vasodilatatory state and a hyperdynamic circulation affecting the cardiac and pulmonary functions dominate the circulation. The recently defined cirrhotic cardiomyopathy may affect systolic and diastolic functions, and imply electromechanical abnormalities. In addition, the baroreceptor function and regulation of the circulatory homoeostasis is impaired. Pulmonary dysfunction involves diffusing abnormalities with the development of the hepatopulmonary syndrome and portopulmonary hypertension in some patients. Recent research has focused on the assertion that the hemodynamic and neurohumoral dysregulation are of major importance for the development of the cardiovascular and pulmonary complications in cirrhosis. This aspect is important to take into account in the management of these patients.
M?ller, S?ren; Henriksen, Jens H
Chronic kidney disease (CKD) is recognized as a major health problem affecting approximately 13% of the US population (1). Numbers of preva- lent CKD patients will continue to rise, reflecting the growing elderly pop- ulation and increasing numbers of patients with diabetes and hypertension. As numbers of CKD patients increase, primary care practitioners will be confronted with management of the
Robert Thomas; Abbas Kanso; John R. Sedor
One of the highlights of the 16th International Prostate Cancer Update was a session on treatment- and disease-related complications of prostate disease. It began with presentation of a challenging case of rising prostate-specific antigen levels after radical prostatectomy, followed by an overview of the use of zoledronic acid in prostate cancer, a review of side effects of complementary medicines, an overview of complications of cryotherapy, an assessment of complications of brachytherapy and external beam radiation therapy, and a comparison of laparoscopy versus open prostatectomy.
Simoneau, Anne R
Still's disease is very rare in elderly patients. We report a case of Still's disease in an elderly patient that had an atypical initial presentation. A 76-year-old woman developed unexplained chronic anemia and thrombocytosis. Three years later she had acute onset of high fever, arthritis, maculopapular rash, pleuritic chest pain, abdominal pain, lymphadenopathy and elevated erythrocyte sedimentation rate. Rheumatoid factor and antinuclear antibodies were negative. She responded favorably to prednisone and methotrexate treatment. Anemia and thrombocytosis as well as Still's disease manifestations resolved. PMID:17510560
Vilá, Luis M; Molina, María J
Summary The Minnesota Multiphasic Personality Inventory (MMPI-2) is commonly used in chronic illness and chronic pain populations\\u000a to assess psychological functioning. We report the results of the first study employing the MMPI-2 to assess psychological\\u000a aspects of patients with Gaucher disease, type I (GD) is an inborn error of metabolism with unique features as a chronic illness:\\u000a the disorder often presents
W. Packman; T. Wilson Crosbie; A. Riesner; C. Fairley; S. Packman
Several hepatobiliary abnormalities have been described in association with inflammatory bowel disease (IBD), including primary\\u000a sclerosing cholangitis (PSC), small duct PSC, chronic hepatitis, cryptogenic cirrhosis, cholangiocarcinoma, and cholelithiasis.\\u000a PSC is the most common biliary condition in patients with IBD, with an incidence ranging from 2.5% to 7.5%. PSC usually progresses\\u000a insidiously and eventually leads to cirrhosis independent of inflammatory bowel
David R. Lichtenstein
Objective: To evaluate frequency of fibromyalgia in rheumatoid arthritis and its effect on disease activity score. Study Design: Cross-sectional study. Place and Duration of Study: The Indus Hospital, Karachi, from December 2010 to May 2011. Methodology: All adult patients of either gender diagnosed as rheumatoid arthritis on the basis of clinical, laboratory and X-ray criteria were included in the study. The sample data was separated into two groups depending on presence or absence of fibromyalgia and 28 joint disease activity score (DAS-28) value was evaluated. Results: There were 31 (25.83%) patients with rheumatoid arthritis and fibromyalgia (RAFM) out of the total 120. The median (IQR) age of patients was 40 (32 - 51) years. All were females. The overall female frequency was 79 (88.8%). The median (IQR) DAS-28 score in RA group was 4.9 (3.66 - 5.71), while the median (IQR) DAS-28 score in RAFM was 7.04 (6.62 - 7.64) [p < 0.0001]. The number of patient getting combination therapy of DMARD in RAFM group was 61.3% while in RA group was 42.7%. Conclusion: DAS-28 was found to be significantly higher in RAFM patients probably because of higher perception of pain. PMID:24953918
Abbasi, Lubna; Haidri, Fakhir Raza
To our knowledge, the possible unveiled interaction between adult-onset Still’s disease (AOSD) with autoimmune thyroid disease (AITD) has never been reported although it is well established that systemic autoimmune disease may usually occur in relation to AITD. As increasingly clear links of AITD with other autoimmune disease such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and primary Sjögren’s syndrome (pSS) have been reported, and the incidence of AOSD concurrent AITD draws our attention rapidly. In this study, we searched relevant literatures published in the past 30 years to explore that condition.
Hu, Yingchun; Wang, Han; Deng, Juelin
Coeliac disease is a common condition that is increasingly being recognised as a result of the development of sensitive and specific serology. The diagnosis of coeliac disease and its subsequent treatment with a gluten?free diet have implications for the patient, not just for symptom control but also for the possible effect on quality of life and risk of complications. Whether the mode of presentation of coeliac disease has an effect on survival or risk of complication is yet unclear. This article reviews the available evidence regarding these issues.
Goddard, C J R; Gillett, H R
Hypovitaminosis D and its consequences are significant complications in the course of untreated celiac disease (CD). We report a case that illustrates the natural evolution of osteomalacia in an adult patient with untreated CD and his response to vitamin D supplementation. In patients with complicated CD and hypovitaminosis D, vitamin D-2 replacement in high enough doses is important to improving functional performance and for the treatment of osteomalacia. PMID:20128172
Eid, Wael E
Subthalamic nucleus deep brain stimulation (STN-DBS) is effective for medically refractory Parkinson's disease. We retrospectively analyzed complications in 180 consecutive patients who underwent bilateral STN-DBS. Surgery-related complications were symptomatic intracerebral hemorrhage in 2, chronic subdural hematoma in 1, and transient deterioration of medication-induced psychosis in 2 patients. Device-related complications involved device infection in 5, skin erosion in 5, and implantable pulse generator malfunction in 2 patients. All of these patients required surgical repair. Surgery and device-related complications could be reduced with increased surgical experience and the introduction of new surgical equipment and technology. Treatment or stimulation-related complications were intractable dyskinesia/dystonia in 11, problematic dysarthria in 7, apraxia of eyelid opening (ALO) in 11, back pain in 10, and restless leg syndrome in 6 patients. Neuropsychiatric complications were transient mood changes in some, impulse control disorder in 2, severe depression related to excessive reduction of dopaminergic medications in 2, rapid progression of dementia in 1, and suicide attempts in 2 patients. Most complications were mild and transient. Dysarthria and ALO were the most frequent permanent sequelae after STN-DBS. Treatment-related adverse events may be caused not only by the effect of stimulation effect but also excessive reduction of dopaminergic medication, or progression of the disease. In conclusion, STN-DBS seems to be a relatively safe procedure. Although serious complications with permanent sequelae are rare, significant incidences of adverse effects occur. Physicians engaged in this treatment should have a comprehensive understanding of the probable complications and how to avoid them. PMID:22123476
Umemura, Atsushi; Oka, Yuichi; Yamamoto, Kenichi; Okita, Kenji; Matsukawa, Noriyuki; Yamada, Kazuo
The operative approach for discectomy in the treatment of thoracic disc disease has changed from standard laminectomy to a variety of dorsolateral and ventral approaches. The procedure-related complications have been reported in numerous clinical studies over the last seven decades: death, neurological deterioration, postoperative vertebral column instability, incomplete disc resection, cerebrospinal fluid leak and fistula, infection, misdiagnosis, pulmonary embolism, pneumonia, and intercostal neuralgia. The authors conducted a Medline search to identify series reporting clinical data related to thoracic discectomy. They analyzed the morbidity and mortality resulting from the various surgical approaches for thoracic disc disease, with special attention to the avoidance and management of surgery-related complications. PMID:16833243
McCormick, W E; Will, S F; Benzel, E C
Adult-onset Still’s disease (AOSD), as a category of connective tissue diseases, has about 5?9% of fever of unknown origin\\u000a (FUO) cases. Diagnosis of AOSD was challenging because of its nonspecific characteristics. The present study analyzed clinical\\u000a manifestations and laboratory findings in a series of patients with AOSD from eastern China. Medical records of 61 patients\\u000a admitted with FUO and with
Pei-Dong Chen; Sheng-Lei Yu; Shu Chen; Xin-Hua Weng
Diverticular disease of the colon in patients under the age of 40 years is uncommon. Between 1975 and 1985, 58 patients (31\\u000a men and 27 women) were admitted for pathologically or radiographically proven acute diverticulitis. Seventeen (29.3 percent)\\u000a were younger than 40 years. Fifteen of the 17 (88.2 percent) required urgent or emergent surgery for complications of diverticular\\u000a disease. This
Julie Freischlag; Robert S. Bennion; Jesse E. Thompson
Objective: To review autoimmune disease complicating therapy with type I interferons (IFNs), specifically in the setting of hepatitis C virus (HCV) infection. Methods: This study describes 13 reported cases of drug-induced systemic lupus erythematosus (SLE) associated with IFN therapy for the period reported during 1990-2002 by searching MEDLINE. In addition, 2 additional patients are presented, 1 with SLE and 1
Leslie E. Wilson; David Widman; Steven H. Dikman; Peter D. Gorevic
A sustained status of chronic inflammation is closely linked to several complications of chronic kidney disease (CKD), such as vascular degeneration, myocardial fibrosis, loss of appetite, insulin resistance, increased muscle catabolism and anemia. These consequences of a chronically activated immune system impact on the acceleration of atherosclerosis, vascular calcification and development of heart dysfunction. Recent evidence suggests that these immune-mediated
Andréa E. M. Stinghen; Sergio Bucharles; Miguel C. Riella; R. Pecoits-Filho
In this case report, the authors described the first case of a patient with adult-onset Still’s disease (AOSD) who presents advanced periodontal infection. AOSD is a rare systemic inflammatory disorder of unknown aetiology, characterised by spiking fever, usually exceeding 39°C, an evanescent salmon pink rash, arthritis and multiorgan involvement. Periodontal infection is a pathogen-induced oral inflammatory disease affecting the supporting tissues of teeth and is currently considered as a risk factor for cardiovascular disease. Several cytokines capable of inducing systemic effects are produced during the course of this infection and the values of serum markers of inflammation, such as C reactive protein (CRP), may significantly decrease after periodontal treatment. Although AOSD can produce elevations in CRP, similar increase may be produced by periodontal infection, suggesting the need for medical and dental diagnosis when evaluating the sources of acute-phase responses in systemic autoimmune disease patients.
Pessoa, Larissa; Galvao, Virgilio; Ferreira, Clarissa; Neto, Leopoldo Santos
Adult onset Still's disease (AOSD) is an inflammatory disease of unknown etiology, characterized by spiking fever, evanescent\\u000a salmon pink maculopapular rash, arthritis, and leukocytosis with neutrophilia. Malignant lymphoma is one of the most important\\u000a differential diagnoses of AOSD. AOSD has been reported as one of paraneoplastic syndromes associated with breast cancer. We\\u000a report a rare case of occult papillary thyroid
Joong Kyong Ahn; Ji-Min Oh; Jaejoon Lee; Sun Wook Kim; Hoon-Suk Cha; Eun-Mi Koh
Histoplasmosis is caused by the dimorphic fungus Histoplasma capsulatum.Primary infection occurs through inhalation of spores from the air. Immunocompetent individuals are usually asymptomatic, but may develop pulmonary disease. Immunocompromised patients tend to present systemic histoplasmosis with cutaneous lesions occurring by secondary invasion. In this case report, we describe a probable primary cutaneous histoplasmosis (PCH) in a patient with adult onset Still's disease under immunosuppression. PMID:24937841
Lise, Michelle Larissa Zini; Godinho, Ronaldo Nunes; Brollo, Flávia Moojen; Staub, Henrique Luiz
Histoplasmosis is caused by the dimorphic fungus Histoplasma capsulatum.Primary infection occurs through inhalation of spores from the air. Immunocompetent individuals are usually asymptomatic, but may develop pulmonary disease. Immunocompromised patients tend to present systemic histoplasmosis with cutaneous lesions occurring by secondary invasion. In this case report, we describe a probable primary cutaneous histoplasmosis (PCH) in a patient with adult onset Still's disease under immunosuppression.
Lise, Michelle Larissa Zini; Godinho, Ronaldo Nunes; Brollo, Flavia Moojen; Staub, Henrique Luiz
Still's disease and chronic recurrent multifocal osteomyelitis (CRMO) are febrile rheumatic diseases of unknown etiology, which predominantly affect children but can also have their initial manifestation in adults. Both can present as intermittent, relapsing episodes and are considered potential candidates within the expanding spectrum of autoinflammatory disorders, although no genetic abnormalities have been described for either of them. Here, we describe a man with an initial manifestation of abacterial multifocal osteitis at the age of 41. During a relapsing-remitting course of his illness, he increasingly developed symptoms of adult-onset Still's disease (AOSD), and the diagnosis was established according to the Yamaguchi criteria. When treated with anakinra, not only the acute symptoms disappeared promptly, but also the osteitis went into complete remission. This is to our knowledge the first description of a simultaneous occurrence of these two manifestations of autoinflammation in adulthood. PMID:21769488
Rech, J; Manger, B; Lang, B; Schett, G; Wilhelm, M; Birkmann, J
Renal disease is one of the most frequent and severe complications experienced by patients with sickle cell disease; its prevalence is likely to increase as the patient population ages. We recommend regular monitoring for early signs of renal involvement and a low threshold for the use of hydroxyurea as preventative measures for end-stage renal disease. Once renal complications are detected, a careful assessment of the patient is required to rule out other causes of renal disease. Proteinuria and hypertension should be managed aggressively and the patient referred to a specialist nephrology center when progressive decline in renal function is noted. For the few patients who develop advanced chronic kidney disease, timely planning for dialysis and transplantation can significantly improve outcome, and we recommend an exchange blood transfusion policy for all patients on the transplant waiting list and for those with a functioning graft. Alongside the invasive treatment regimes, it is important to remember that renal failure in conjunction with sickle cell disease does carry a significant burden of morbidity and that focusing on symptom control has to be central to good patient care. PMID:24764565
Sharpe, Claire C; Thein, Swee Lay
Chronic graft-versus-host disease (cGVHD) is one of the most frequent and serious complications of allogeneic hematopoietic stem cell transplantation (HSCT). Nephrotic syndrome (NS) is an uncommon and underrecognized manifestation of cGVHD. We report a patient who developed NS 18 months after allogeneic bone marrow transplantation. The onset of NS was accompanied by active manifestations of cGVHD, and immunosuppressants had not
Hsin-Hui Wang; An-Hang Yang; Ling-Yu Yang; Giun-Yi Hung; Jei-Wen Chang; Chun-Kai Wang; Tzong-Yann Lee; Ren-Bin Tang
"The Still Divided Academy" is a wonderful examination of the academic community that shows their inner workings by addressing a broad range of issues including: academic politics, tenure, perceived and real political imbalance, academic freedom, and diversity. Administrators, professors, and students have very different priorities, values, and…
Rothman, Stanley; Kelly-Woessner, April; Woessner, Matthew
A 43-year-old woman was admitted for examination of fever, an elevated transaminase level, LDH, skin eruption, sore throat and bicytopenia. As bone marrow examination revealed an increased proportion of histiocytes and active phagocytosis, hemophagocytic syndrome (HPS) was diagnosed. After admission, the peripheral blood counts recovered spontaneously and the HPS subsided, but other symptoms persisted and the neutrophil count increased. At this time, we diagnosed the patient as having adult Still's disease. All the symptoms disappeared after administration of prednisolone. The markedly increased concentrations of TNF-alpha and IFN-gamma in the peripheral blood at the time of HPS declined gradually, and the IL-6 concentration increased at the time of diagnosis of Still's disease. However, all of these concentrations normalized after administration of prednisolone. As HPS and Still's disease have a common etiology, and each shows high concentrations of IFN-gamma, IL 6 and TNF-alpha, the symptoms are similar in both diseases. In particular, a relationship between HPS and high concentrations of TNF-alpha and IFN-gamma is suspected. PMID:11925884
Hirayama, Yasuo; Sakamaki, Sumio; Tsuji, Yasushi; Sagawa, Tamotsu; Nakajima, Takaharu; Matsunaga, Takuya; Kogawa, Katsuhisa; Kato, Junji; Niitsu, Yoshiro
Adult onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology characterized by spiking fever, evanescent skin rash, arthralgia or arthritis, involvement of various organs, and predominantly neutrophilic leucocytosis. AOSD, although uncommon, has a characteristic constellation of clinical and laboratory features and should be considered in the differential diagnosis of pyrexia of unknown origin associated with a rash and arthralgia. The diagnosis is one of clinical suspicion and it is essential that infections, malignancy, and other rheumatic diseases are excluded. We report a case which illustrates the typical features of AOSD that were treated with steroids and azathioprine.
Said, Noor Hanif; Wong, Siew Ngoh; Tan, Wei Hsia
Inflammatory bowel diseases (IBD) are a heterogeneous group of diseases, not always easy to diagnose, even more difficult to classify, and diagnostic criteria are not always uniform. Well done population-based studies are not abundant, and so comparisons among different geographical areas or populations are not always very reliable. In this article, we have reviewed epidemiological studies available on the world’s population while making a critical review of published data.
Gismera, Cristina Saro; Aladren, Beatriz Sicilia
Still's disease is a subset of juvenile idiopathic arthritis (JIA) that usually presents with intermittent fever, rash, and arthritis. Extra-articular flares can occur several years after disease onset. We report two cases of adult Still's disease with myocarditis after several years of being in remission. A 34-year-old Caucasian man with history of systemic juvenile arthritis in remission since age 13 was admitted in hospital with 10 days history of fever, odynophagia, and arthralgias. Chest X-ray and cardiac ultrasound showed cardiac enlargement. An endomyocardial biopsy revealed acute myocarditis. He was treated with methylprednisolone and intravenous gammaglobulin, with improvement of his general condition and cardiac parameters. A 16-year-old Caucasian male patient with history of systemic JIA in remission for the last 7 years was admitted with 7 days history of fever, odynophagia, arthralgias, and myalgias. Two days after admission, he developed chest pain and pericardial rubbing was found on examination. Cardiac ultrasound showed left ventricular dilatation with impaired systolic function, and posterior, inferior and apical-septal wall hypokinesia. Blood test showed elevated creatine phosphokinase levels. He was treated with IV methylprednisolone with normal follow-up cardiac ultrasound. Cardiac involvement in patients with systemic JIA can be the first symptom of disease reactivation, even after many years of disease remission. PMID:19579028
Cavallasca, Javier Alberto; Vigliano, Carlos A; Perandones, Carlos E; Tate, Guillermo A
Purpose Ependymoma is less commonly found in the supratentorial brain and has known clinical and molecular features that are unique. Our single institution series provides valuable information about disease control for supratentorial ependymoma and the complications of supratentorial irradiation in children. Methods and Materials A total of 50 children with newly diagnosed supratentorial ependymoma were treated with adjuvant radiation therapy (RT); 36 were using conformal methods after 1996. The median age at RT was 6.5 years (range, 1–18.9). The entire group was characterized according to sex (girls = 27), race (Caucasian = 43), extent of resection (gross-total = 46), and tumor grade (anaplastic = 28). The conformal RT group was prospectively evaluated for neurological, endocrine and cognitive effects. Results With a median follow-up of 9.1 years from the start of RT for survivors (range 0.2–23.2), the 10-year progression-free and overall survival were 73% + 7% and 76% + 6%, respectively. None of the evaluated factors was prognostic for disease control. Local and distant failures were evenly divided among the 16 patients who experienced progression. Eleven patients died from disease and one from CNS necrosis. Seizure disorders were present in 17 patients and 4 were considered to be clinically disabled. Clinically significant cognitive effects were limited to children with difficult to control seizures. Average values for IQ and academic achievement (reading, spelling, and math) were within the range of normal through 10 years of follow-up. Central hypothyroidism was the most commonly treated endocrinopathy. Conclusion RT may be administered with acceptable risks for complications in children with supratentorial ependymoma. These results suggest that outcomes for these children are improving and that complications may be limited by use of focal irradiation methods.
Landau, Efrat; Boop, Frederick A.; Conklin, Heather M.; Wu, Shengjie; Xiong, Xiaoping; Merchant, Thomas E.
Motor fluctuations and dyskinesias are common sequelae of Parkinson's disease (PD) that may limit function and quality of life. With disease progression, striatal dopamine concentration becomes closely linked to plasma levodopa levels, which vary considerably with standard oral regimens. Exposure of striatal dopamine receptors to wildly fluctuating transmitter levels is thought to contribute to the development of dyskinesias and motor fluctuations. Continuous dopaminergic delivery has been shown to reduce motor complications in advanced PD patients, and has been hypothesized to prevent their incidence when given as early therapy in mild PD. In this article, the authors outline the rationale for continuous dopaminergic delivery and review clinical strategies implementing the concept, including transdermal rotigotine, subcutaneous apomorphine infusion, intraduodenal infusion of levodopa gel and the investigational oral levodopa formulation IPX066. PMID:23739008
Wright, Brenton A; Waters, Cheryl H
Adenocarcinoma of the small intestine complicating coeliac disease is uncommon. Only 14 cases have been reported, and in only one of these was a jejunal biopsy carried out more than eight months before the diagnosis of malignancy. We describe four more patients with this association, all with long histories of coeliac disease, confirmed in three by jejunal biopsy over five years before the diagnosis of malignancy. Important presenting features of carcinoma were abdominal pain, anaemia, occult gastrointestinal bleeding, abdominal mass, and intestinal obstruction, and these were the main indications for operation. After resection of the tumour survival may be prolonged, as evidenced by one of our cases who remains well eight years after surgery.
Holmes, G K; Dunn, G I; Cockel, R; Brookes, V S
Metastatic spine disease accounts for 10% to 30% of new cancer diagnoses annually. The most frequent presentation is axial spinal pain. No treatment has been proven to increase the life expectancy of patients with spinal metastasis. The goals of therapy are pain control and functional preservation. The most important prognostic indicator for spinal metastases is the initial functional score. Treatment is multidisciplinary, and virtually all treatment is palliative. Management is guided by three key issues; neurologic compromise, spinal instability, and individual patient factors. Site-directed radiation, with or without chemotherapy is the most commonly used treatment modality for those patients presenting with spinal pain, causative by tumours which are not impinging on neural elements. Operative intervention has, until recently been advocated for establishing a tissue diagnosis, mechanical stabilization and for reduction of tumor burden but not for a curative approach. It is treatment of choice patients with diseaseadvancement despite radiotherapy and in those with known radiotherapy-resistant tumors. Vertebral resection and anterior stabilization with methacrylate or hardware (e.g., cages) has been advocated.Surgical decompression and stabilization, however, along with radiotherapy, may provide the most promising treatment. It stabilizes the metastatic deposited areaand allows ambulation with pain relief. In general, patients who are nonambulatory at diagnosis do poorly, as do patients in whom more than one vertebra is involved. Surgical intervention is indicated in patients with radiation-resistant tumors, spinal instability, spinal compression with bone or disk fragments, progressive neurologic deterioration, previous radiation exposure, and uncertain diagnosis that requires tissue diagnosis. The main goal in the management of spinal metastatic deposits is always palliative rather than curative, with the primary aim being pain relief and improved mobility. This however, does not come without complications, regardless of the surgical intervention technique used. These complication range from the general surgical complications of bleeding, infection, damage to surrounding structures and post operative DT/PE to spinal specific complications of persistent neurologic deficit and paralysis.
Dunning, Eilis Catherine; Butler, Joseph Simon; Morris, Seamus
Adult-onset Still disease (AOSD) is a systemic inflammatory disorder characterized clinically by high spiking fever, polyarthralgia/arthritis, a salmon-pink evanescent rash, predominantly neutrophilic leucocytosis, lymphadenopathy, liver dysfunction, and splenomegaly. Recently, a nonclassic, nonevanescent skin rash has been reported. We report a 27-year-old woman with AOSD showing persistent pruritic papular lesions. Histologically, dyskeratotic keratinocytes were seen in the upper epidermis. We describe this case in detail and review the previous literature. Nonclassic pruritic eruptions with characteristic dyskeratotic keratinocytes might provide an important clue for the diagnosis of AOSD. PMID:24773396
Yoshifuku, A; Kawai, K; Kanekura, T
An extensive series of experimental investigations has shown that both inherited and induced autoimmune diseases in laboratory animals may be transferred and, conversely, cured by stem cell transplantation. In man, the evidence is mainly anecdotal, originating both from the transmission of autoimmune conditions following allogeneic BMT from carrier donors to non-autoimmune recipients transplant-requiring diseases, and from the resolution of autoimmune diseases (mainly rheumatoid arthritis) of the recipients after allogeneic BMT from healthy donors. Will it be possible to cure severe autoimmune diseases with powerfully immunosuppressive conditioning regimens followed by the administration of hematopoietic stem cells? If the reconstitution of a naive immune system is necessary, allogeneic stem cells will be necessary, but the procedure is still saddled with its attending problems, with TRM in the foreground. When utilizing autologous stem cells in conjunction with TCD the patients' tolerance will be significantly better, but remissions are to be anticipated rather than cures. However, some special manipulations may be expected to ameliorate results in those selected autoimmune patients not or badly responding to conventional immunosuppressive therapy, for whom this type of treatment can be offered. PMID:8528163
Marmont, A M; Van Bekkum, D W
The term "complicated" diverticulitis is reserved for inflamed diverticular disease complicated by bleeding, abscess, peritonitis, fistula or bowel obstruction. Hemorrhage is best treated by angioembolization (interventional radiology). Treatment of infected diverticulitis has evolved enormously thanks to: 1) laparoscopic colonic resection followed or not (Hartmann's procedure) by restoration of intestinal continuity, 2) simple laparoscopic lavage (for peritonitis +/- resection). Diverticulitis (inflammation) may be treated with antibiotics alone, anti-inflammatory drugs, combined with bed rest and hygienic measures. Diverticular abscesses (Hinchey Grades I, II) may be initially treated by antibiotics alone and/or percutaneous drainage, depending on the size of the abscess. Generalized purulent peritonitis (Hinchey III) may be treated by the classic Hartmann procedure, or exteriorization of the perforation as a stoma, primary resection with or without anastomosis, with or without diversion, and last, simple laparoscopic lavage, usually even without drainage. Feculent peritonitis (Hinchey IV), a traditional indication for Hartmann's procedure, may also benefit from primary resection followed by anastomosis, with or without diversion, and even laparoscopic lavage. Acute obstruction (nearby inflammation, or adhesions, pseudotumoral formation, chronic strictures) and fistula are most often treated by resection, ideally laparoscopic. Minimal invasive therapeutic algorithms that, combined with less strict indications for radical surgery before a definite recurrence pattern is established, has definitely lead to fewer resections and/or stomas, reducing their attendant morbidity and mortality, improved post-interventional quality of life, and less costly therapeutic policies. PMID:22936232
Fingerhut, Abe; Veyrie, Nicolas
Extended field radiation therapy represents the main therapeutic option in early stage Hodgkin's disease with favorable prognostic features. Its role however has recently been criticized, mainly due to the high incidence of late complications in irradiated tissues. Furthermore, surgical staging, which in the opinion of many is mandatory for proper selection of patients for radiotherapy alone, has a well-known morbidity, and splenectomy has been associated with a high risk of secondary leukemias. Lastly, the failure rate after radiotherapy only is not negligible and second-line treatment is not always successful. A review of our experience and of the recent literature has allowed us to refute these objections. The results of radiotherapy, when properly performed, are highly reliable and have been reproducible in many Institutions. Chemotherapy alone cannot yet be regarded as an alternative to radiotherapy in these patients since data reported on this issue are conflicting. Present knowledge regarding the relationship between clinical features and the risk of occult subdiaphragmatic spread allows patients with localized disease to be selected without surgical staging; the results of radiotherapy in clinically staged patients confirm this statement. Concern for the late effects in irradiated tissues is justified, and future efforts should be directed at reducing the toxicity of this treatment. Associating a short chemotherapy course with low-dose radiotherapy to involved sites could help to achieve this goal. PMID:8641642
Frezza, G; Barbieri, E; Zinzani, P L; Babini, L; Tura, S
Background Over the last few years, accumulating data have implicated a role for ferritin as a signaling molecule and direct mediator of the immune system. Hyperferritinemia is associated with a multitude of clinical conditions and with worse prognosis in critically ill patients. Discussion There are four uncommon medical conditions characterized by high levels of ferritin, namely the macrophage activation syndrome (MAS), adult onset Still’s disease (AOSD), catastrophic antiphospholipid syndrome (cAPS) and septic shock, that share a similar clinical and laboratory features, and also respond to similar treatments, suggesting a common pathogenic mechanism. Ferritin is known to be a pro-inflammatory mediator inducing expression of pro-inflammatory molecules, yet it has opposing actions as a pro-inflammatory and as an immunosuppressant. We propose that the exceptionally high ferritin levels observed in these uncommon clinical conditions are not just the product of the inflammation but rather may contribute to the development of a cytokine storm. Summary Here we review and compare four clinical conditions and the role of ferritin as an immunomodulator. We would like to propose including these four conditions under a common syndrome entity termed “Hyperferritinemic Syndrome”.
Von Willebrand disease (vWD) is a common hereditary bleeding disorder resulting from a quantitative and/or qualitative deficiency of von Willebrand factor (vWF). We report two cases of peritonsillar abscess complicated by vWD. A 46-year-old Japanese man was intravenously administered factor VIII clotting antigen (500U×3 days)and platelet transfusion (10U), when before puncture was performed. After puncture, his symptoms promptly improved with the administration of the antibiotic doripenem (DRPM, 1.5g/day). He left our facility one week later and had no recurrence of symptoms. A 24-year-old Japanese woman was intravenously administered factor VIII clotting antigen (4500U×3 days) and desmopressin (DDAVP) before undergoing a puncture. Her symptoms promptly improved with DRPM treatment (1.5g/day). The patient left our facility one week later. However, the peritonsillar abscess recurred in three weeks. Afterwards, tonsillectomy was enforced three months later. Intravenous factor VIII clotting antigen (4500U×2 days) and platelet transfusion (10U×1 day) had been used before tonsillectomy. We therefore suggest that a peritonsillar abscess in patients with vWD can be safely treated by factor VIII clotting antigen and DDAVP at the appropriate disease stage and by performing paracentesis for the acute phase or tonsillectomy for the chronic phase. PMID:22075138
Nagano, Hiromi; Harada, Mizue; Umakoshi, Mizuo; Hayamizu, Yoshiko; Yoshifuku, Kosuke; Kurono, Yuichi
Adult-onset Still's disease (AOSD) is a systemic inflammatory autoimmune disorder of unknown etiology and pathogenesis. There are no specific laboratory tests for AOSD. To investigate the potential role of adenosine deaminase (ADA) in the diagnosis of AOSD and analyze the correlation among ADA, LDH and WBC (white blood cell count), the serum levels of ADA and LDH in 26 patients with active untreated AOSD, 40 patients with active systemic lupus erythematosus (SLE) as disease control and 48 healthy volunteers as healthy control were determined using automatic biochemical analyzer (Olympus AU2700, Japan). WBC was examined by automatic blood cell analyzer (Beckman Coulter Hmx, America). Significantly higher levels of serum ADA, LDH and WBC were found in active untreated AOSD patients than in active SLE patients and healthy volunteers (F = 27.823; P = 0.000; F = 28.458, P = 0.000; F = 51.929, P = 0.000). Serum ADA were related to LDH level in patients with AOSD patients (r = 0.786, P = 0.000 < 0.01). Both ADA and LDH were not related to WBC (r = 0.244, P = 0.229 > 0.01; r = 0.054, P = 0.794 > 0.01). This is the first study to show that serum ADA could play an important role in AOSD and may be an important biomarker for the diagnosis of AOSD. Serum ADA could be another diagnostic marker independent from whole blood WBC. PMID:23096558
Xun, Chunhua; Zhao, Yong; Hu, Zhi Jian
Introduction Adult-onset still's disease (AOSD) is a rare systemic inflammatory disorder in which abnormalities in inflammatory cytokines production appear to play a pathophysiological role. Our previous work has reported increased expression of macrophage migration inhibitory factor (MIF) and revealed its correlation with disease severity and activity in AOSD. A -173 G/C single nucleotide polymorphism (SNP) (rs755622) and a -794 CATT5-8 repeat (rs5844572) in the MIF promoter have been reported. In this study, we sought to explore the relationship between functional MIF promoter polymorphisms and MIF expression in AOSD. Methods 100 patients and 200 controls were recruited in the study. A polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) assay was utilized to analyze the -173 G/C SNP (rs755622) and PCR-based size discrimination assay was applied to detect the -794 CATT5-8 repeat (rs5844572) in the MIF promoter. Plasma MIF levels were measured by ELISA. MIF mRNA levels were quantified by real-time reverse transcription (RT)-PCR. Bisulfate genomic sequencing was employed to evaluate DNA methylation status within the MIF promoter. Results We identified that the frequencies of MIF -794 CATT5 (P = 0.001) allele and the expression of MIF (P <0.001) were increased in patients compared to healthy controls. Plasma levels of MIF in patients with CC genotype were higher than those of patients with GC or GG genotypes (P = 0.05). In patients with established AOSD, a higher frequency of -794 CATT7 containing MIF genotypes was observed in those with liver dysfunction (P = 0.009). Haplotype analysis revealed a higher representation of the MIF haplotype defined by -173*C/-794 CATT5 (C5) in AOSD patients (P = 0.001). Conclusion Functional promoter polymorphisms in the MIF gene influence plasma MIF levels in AOSD and may contribute to disease susceptibility or clinical presentation of AOSD.
Case. A 34-year-old African-American female with a history of adult-onset Still's disease presented to an outside hospital with oligoarthritis. She experienced a generalized tonic-clonic seizure en route via ambulance, was intubated upon arrival, and transferred to the intensive care unit for treatment of suspected pneumonia and sepsis. She subsequently developed generalized cutaneous desquamation that progressed despite the cessation of antibiotics and other potential offending drugs which required transfer to our hospital's burn unit. She was suspected to have reactive hemophagocytic syndrome based on her clinical presentation of fever, rash, polyarthritis, elevated liver enzymes, coagulopathy, splenomegaly, normocytic anemia, thrombocytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis visualized in bone marrow biopsy specimen. Magnetic resonance imaging demonstrated necrotic demyelination of the deep white matter and corona radiata. The patient developed multiorgan dysfunction and DIC without any other attributable etiology. Despite aggressive broad spectrum therapy and high dose of steroids she progressively deteriorated and eventually expired. Conclusion. Previous publications have highlighted the prevalence of necrotic leukoencephalopathy in children with familial hemophagocytic syndrome. Our patient demonstrated some uncommon features complicating her HLH including DIC and necrotic leukoencephalopathy, which are very rare entities in AOSD.
Nannapaneni, Naveen; Altinok, Gulcin; Levine, Miriam; Dhar, J. Patricia
Case. A 34-year-old African-American female with a history of adult-onset Still's disease presented to an outside hospital with oligoarthritis. She experienced a generalized tonic-clonic seizure en route via ambulance, was intubated upon arrival, and transferred to the intensive care unit for treatment of suspected pneumonia and sepsis. She subsequently developed generalized cutaneous desquamation that progressed despite the cessation of antibiotics and other potential offending drugs which required transfer to our hospital's burn unit. She was suspected to have reactive hemophagocytic syndrome based on her clinical presentation of fever, rash, polyarthritis, elevated liver enzymes, coagulopathy, splenomegaly, normocytic anemia, thrombocytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis visualized in bone marrow biopsy specimen. Magnetic resonance imaging demonstrated necrotic demyelination of the deep white matter and corona radiata. The patient developed multiorgan dysfunction and DIC without any other attributable etiology. Despite aggressive broad spectrum therapy and high dose of steroids she progressively deteriorated and eventually expired. Conclusion. Previous publications have highlighted the prevalence of necrotic leukoencephalopathy in children with familial hemophagocytic syndrome. Our patient demonstrated some uncommon features complicating her HLH including DIC and necrotic leukoencephalopathy, which are very rare entities in AOSD. PMID:24707428
Namas, Rajaie; Nannapaneni, Naveen; Venkatram, Malini; Altinok, Gulcin; Levine, Miriam; Dhar, J Patricia
Background: Consensus is lacking on treatment for corticosteroid resistant adult onset Still's disease (ASD). Objective: To assess anti-TNF? efficacy and tolerance in refractory ASD. Methods: All departments of rheumatology and internal medicine in France were contacted by mail to identify cases of refractory ASD for which anti-TNF? had been used. Medical information was collected using a standardised questionnaire. Results: Of 20 patients with mean age 40.7 years (range 18–74) at treatment start and mean disease duration 8.5 years (range 2–21), the clinical expression of ASD was predominantly systemic in five patients and polyarticular in 15. Response to corticosteroids and methotrexate had been considered inadequate in all patients. Infliximab was used to treat 15 patients, and etanercept used for 10; five had received both drugs consecutively. Steroids were concurrently used in 18 patients and an immunosuppressant in 17. At a mean (SD) follow up of 13 (14) months, complete remission had occurred in five cases (of 25 treatment sequences): one receiving etanercept and four infliximab. Partial response was observed in 16 cases (seven etanercept and nine infliximab). Treatment failed in four cases (two with each anti-TNF?). At the last visit, anti-TNF? therapy was discontinued in 17 cases, 11 times because of lack (or loss) of efficacy, four times because of a side effect, and twice for other reasons. Conclusion: Anti-TNF? therapy may be helpful for some patients with refractory ASD. However, most patients achieve only partial remission. Additional information is thus needed to evaluate more precisely the risk–benefit ratio of this treatment.
Fautrel, B; Sibilia, J; Mariette, X; Combe, B; the, C
Typical skin rash, which appears and disappears along with respective rise and fall of fever, is well-known, and included as one of the major criteria of adult-onset Still's disease (AOSD) (Yamaguchi's criteria). In addition, various skin lesions are occasionally observed in association with AOSD. Persistent pruritic eruptions present with some clinical features, such as urticarial erythema, flagellate erythema, erythematous, slightly scaly or crusted papules, and/or plaques on the trunk and extremities. These lesions show unique histological features such as dyskeratosis with a peculiar, distinctive distribution in the upper epidermis and cornified layers with focal hyperkeratosis. We describe herein six cases of AOSD, which presented with skin lesions of persistent pruritic papules and plaques. All six cases were female, and three of them were elderly women. The patients presented with linear erythematous streaks, scaly erythema, keratotic papules, infiltrative plaques and irregular coalesced erythemas. By contrast, histological features were characteristic, and dyskeratotic cells were found in the horny layers as well as in the upper layers of the epidermis. Persistent pruritic eruption is an important cutaneous sign for the diagnosis of AOSD. PMID:24628100
Kikuchi, Nobuyuki; Satoh, Masataka; Ohtsuka, Mikio; Yamamoto, Toshiyuki
Sickle-cell anaemia (SCA) is a multi-system disease, associated with episodes of acute illness and progressive organ damage. Disease severity shows substantial variation and it is often a burden for adolescents. Complications such as leg ulcer and priapism have a significant impact on quality of life. There are still no definitive treatment guidelines available. Considering the embarrassing nature of priapism and the dire consequences for erectile dysfunction, it is important to inform patients, parents and providers about the relationship of SCA to prolonged painful erections. This article will review the pathophysiology and treatment options of SCA focusing the complications of leg ulcers, priapism, cholelithiasis and retinopathy. The case study of a 14-year-old boy is used to present a management challenge of multiple SCA-related complications.
Vasconcelos, Alexandra; Prior, Ana Rita; Ferrao, Anabela; Morais, Anabela
Open surgery for thoracic aortic disease is associated with significant morbidity and the reported rates for paraplegia and stroke are 3%-19% and 6%-11%, respectively. Spinal cord ischemia and stroke have also been reported following endoluminal repair. This study reviews the incidence of paraplegia and stroke in a series of 186 patients treated with thoracic stent grafts. From July 1997 to September 2006, 186 patients (125 men) underwent endoluminal repair of thoracic aortic pathology. Mean age was 71 years (range, 17-90 years). One hundred twenty-eight patients were treated electively and 58 patients had urgent procedures. Anesthesia was epidural in 131, general in 50, and local in 5 patients. Seven patients developed paraplegia (3.8%; two urgent and five elective). All occurred in-hospital apart from one associated with severe hypotension after a myocardial infarction at 3 weeks. Four of these recovered with cerebrospinal fluid (CSF) drainage. One patient with paraplegia died and two had permanent neurological deficit. The rate of permanent paraplegia and death was 1.6%. There were seven strokes (3.8%; four urgent and three elective). Three patients made a complete recovery, one had permanent expressive dysphasia, and three died. The rate of permanent stroke and death was 2.1%. Endoluminal treatment of thoracic aortic disease is an attractive alternative to open surgery; however, there is still a risk of paraplegia and stroke. Permanent neurological deficits and death occurred in 3.7% of the patients in this series. We conclude that prompt recognition of paraplegia and immediate insertion of a CSF drain can be an effective way of recovering spinal cord function and improving the prognosis.
Morales, J. P.; Taylor, P. R.; Bell, R. E.; Chan, Y. C. [Guy's and St. Thomas' Foundation Hospital NHS Trust, Department of Vascular Surgery (United Kingdom); Sabharwal, T. [Guy's and St. Thomas' Foundation Hospital NHS Trust, Department of Interventional Radiology (United Kingdom); Carrell, T. W. G. [Guy's and St. Thomas' Foundation Hospital NHS Trust, Department of Vascular Surgery (United Kingdom); Reidy, J. F. [Guy's and St. Thomas' Foundation Hospital NHS Trust, Department of Interventional Radiology (United Kingdom)], E-mail: John.Reidy@gstt.nhs.uk
Sickle cell disease is the most common inherited blood disorder in the United States. This disorder of hemoglobin structure leads to a chronic hemolytic anemia and complex chronic disease manifested by sudden, severe, and life-threatening complications. These acute complications can occur in any organ system beginning in early childhood and lasting throughout life. The intermittent nature and acuity of these complications lend the emergency department to be an important site of care. The hallmark of sickle cell disease is the vasoocclusive painful event. Other more “typical” complications include fever, acute chest syndrome, priapism, and ischemic stroke. Children with sickle cell disease can also present with other “atypical” complications that can have devastating consequences if they are unrecognized. Detailed discussion of these “atypical” sickle cell disease complications, organized by organ system involved, will be the focus of this article.
Brandow, Amanda M.; Liem, Robert
The Kidney Disease Outcome and Quality Initiative (KDOQI) Group recommended guidelines for the monitoring and treatment of chronic kidney disease (CKD) in 2002. These recommendations were based on the prevalence of known complications as seen in adults. In children, the exact prevalence of these complications is unknown. We therefore conducted a cross-sectional study of 366 patients with CKD in a
H Wong; K Mylrea; J Feber; A Drukker; G Filler
Tissue granulomas formation in adult-onset Still’s disease (AOSD) is extremely rare. We describe a case of AOSD associated with formation of granulomatous lesions in lymph nodes, liver and presumably spleen. The high dose steroid-dependent nature of our patient’s illness, characterized by disease relapses when methylprednisolone dose was reduced below 10 mg/d, was overwhelmed with institution of anakinra (100 mg/d). The histologic finding of granulomas formation in lymph nodes, liver or spleen should not deter the consideration of AOSD as a potential diagnosis in a compatible clinical context; however, other more common etiologies of tissue granulomas formation should be first excluded.
Assimakopoulos, Stelios F.; Karamouzos, Vassilios; Papakonstantinou, Christos; Zolota, Vassiliki; Labropoulou-Karatza, Chryssoula; Gogos, Charalambos
Herpes simplex virus type 1 (HSV-1) is associated with a large spectrum of pathologies i.e. pulmonary diseases. Although it has often been isolated from the lower respiratory tract of immunocompetent or immunosuppressed patients undergoing prolonged mechanical ventilation (MV), its causative role in serious lung infections is still unclear. Here we report the case of a 44-year-old man presenting seizures that followed an acute respiratory illness that occurred during hospitalization. The detection of HSV-1 DNA in bronchoalveolar lavage (BAL), in spinal fluid, and in blood samples, supported the evidence of a disseminated viral infection that strengthens the hypothesis of herpetic pneumonia as a possible triggering cause of neurological complications and fatal outcome. This observation draws attention to the opportunity of introducing tests for the detection of HSV-1 into the diagnostic protocols for such patients. In fact, adequate diagnostic tools would favor early diagnosis and correct therapy to HSV-1 that could reduce the possibility of either encephalic complications or the rate of mortality in critical long-term patients affected by respiratory pathologies who need assisted ventilation. PMID:24326294
Astuto, Marinella; Palermo, Concetta Ilenia; Costanzo, Carmela Maria; Ettorre, Giovanni Carlo; Palmucci, Stefano; Franchina, Concetta; Russo, Raffaela; Valastro, Pietro; Timpanaro, Valentina; Scalia, Guido
... Prevention People at Risk History & Disease Patterns Fast Facts For Clinicans For Media Outbreaks For Health Departments Identify and Report Travel-Associated Legionnaires' Disease CSTE Position Statement Epidemiologic Investigation ...
A total of 590 patients with Stage IA-IIIB Hodgkin's disease received mantle irradiation at the Joint Center for Radiation Therapy between April 1969 and December 1984 as part of their initial treatment. Recurrence patterns as well as pulmonary, cardiac and thyroid complications were analyzed. Pulmonary recurrence was more frequently seen in patients with large mediastinal adenopathy (LMA); 11% of patients with LMA recurred in the lung in contrast to 3.1% with small or no mediastinal disease, p = 0.003. Hilar involvement, when corrected for size of mediastinal involvement, was not predictive of lung relapse. Patients with LMA also had a high rate of nodal relapse above the diaphragm (40%) following radiation therapy (RT) alone as compared to similarly treated patients with small or no mediastinal adenopathy (6.5%), p less than 0.0001. This risk of nodal recurrence was greatly reduced (4.7%) for LMA patients receiving combined radiation therapy and chemotherapy (CMT), p less than 0.0001. Sixty-seven patients (11%) with hilar or large mediastinal involvement received prophylactic, low dose, whole lung irradiation. No decrease in the frequency of lung recurrence was seen with the use of whole lung irradiation. Radiation pneumonitis was seen in 3% of patients receiving radiation therapy alone. In contrast, the use of whole lung irradiation was associated with a 15% risk of pneumonitis, p = 0.006. The risk of pneumonitis was also significantly increased with the use of chemotherapy (11%), p = 0.0001. Cardiac complications were uncommon with pericarditis being the most common complication (2.2%). Thyroid dysfunction was seen in 25% of patients and appeared to be age-related. These data suggest that the long-term complications of mantle irradiation are uncommon with the use of modern radiotherapeutic techniques.
Tarbell, N.J.; Thompson, L.; Mauch, P. (Harvard Medical School, Boston, MA (USA))
Adult onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. It is characterized by daily fevers, arthralgias or arthritis, typical skin rash, and leukocytosis. Hepatic involvement is frequently observed in the course of AOSD with mildly elevated transaminases and/or hepatomegaly. Fulminant hepatic failure, occasionally requiring urgent liver transplantation, is a rare manifestation of AOSD. Here, we present a case of 22-year-old woman with no significant medical history who initially came with fever, arthralgias, myalgias, generalized weakness, and sore throat. Laboratory data showed mildly elevated transaminases and markedly elevated ferritin levels. She was diagnosed with AOSD based on Yamaguchi diagnostic criteria and was started on prednisone. Three months later, while she was on tapering dose of steroid, she presented with fever, abdominal pain, jaundice, and markedly elevated transaminases. Extensive workup excluded all potential causes of liver failure. She was diagnosed with AOSD associated acute liver failure (ALF). Intravenous (IV) methylprednisolone pulse therapy was started, with dramatic improvement in liver function. Our case demonstrated that ALF can present as a complication of AOSD and IV mega dose pulse methylprednisolone therapy can be employed as a first-line treatment in AOSD associated ALF with favorable outcome.
Valluru, Nalini; Tammana, Venkata S.; Windham, Michael; Mekonen, Eyasu; Begum, Rehana; Sanderson, Andrew
INTRODUCTION: Macrophage activating syndrome is a severe, potentially life-threatening condition that may accompany Still's disease. It is characterized by fever, hepatosplenomegaly, lymphadenopathy, severe cytopenia, serious liver dysfunction, coagulopathy and neurologic involvement. The principal treatment for patients with this syndrome includes etoposide 150 mg\\/2 M twice a week for two weeks, dexamethasone 10 mg\\/2 M for two weeks and cyclosporine 3
Meir Mizrahi; Eldad Ben-Chetrit
Linear growth was studied in 20 children suffering from Still's disease on various treatment regimens, and their ability to secrete growth hormone and cortisol was investigated. Growth recovered on reducing daily corticosteroid therapy or on changing to an alternative regimen. Retardation of growth was not due to an absolute inability to secrete growth hormone. Basal plasma cortisol levels and the
R. A. Sturge; C. Beardwell; M. Hartog; D. Wright; B. M. Ansell
Traditionally, surgical diseases including emergency and injury care have garnered less attention and support internationally when compared to other medical specialties. Over the past decade however, healthcare professionals have increasingly advocated for the need to address the global burden of non-communicable diseases. Surgical disease, including traumatic injury, is among the top causes of death and disability worldwide and the subsequent economic burden is substantial, falling disproportionately on low- and middle-income countries (LMICs). The future of global health in these regions depends on a redirection of attention to diseases managed within surgical, anesthesia and emergency specialties. Increasing awareness of these disparities, as well as increasing focus in the realms of policy and advocacy, is crucial. While the barriers to providing quality trauma and emergency care worldwide are not insurmountable, we must work together across disciplines and across boundaries in order to negotiate change and reduce the global burden of surgical disease.
The incidence of complications associated with disease and treatment was compared in younger versus elderly patients with Parkinson's disease (PD). One hundred sixty-five patient records were divided according to patient age into two groups ("younger," 41 to 64, and "elderly," > or = 65 years) and reviewed for the incidence of dyskinesias, fluctuations, freezing, psychosis, dementia, depression, and insomnia. Younger patients had a greater incidence of chorea (75.8 percent vs 49.5 percent), dystonia (82.3 percent vs 49.0 percent), fluctuations (90.1 percent vs 68.1 percent), depression (73.2 percent vs 36.8 percent), and insomnia (57.9 percent vs 18.1 percent). There were no significant differences in the incidence of freezing, dementia, or psychosis. At the time of the first adverse event, there was no difference in patient characteristics such as gender, lag time from disease diagnosis to levodopa initiation, disease symptoms at the time of diagnosis, levodopa dose, or concomitant drug use despite the fact that the older group had a longer duration of disease, higher Hoehn and Yahr stage, an older age at onset of PD, and longer duration of levodopa use. Younger patients with PD experience a greater incidence of adverse effects than do elderly PD patients. The spectrum of adverse effects is comparable to those of young-onset (< or = 40 years) patients. PMID:8879356
Wagner, M L; Fedak, M N; Sage, J I; Mark, M H
In normal children, fractures of the femoral neck are uncommon and accompany severe trauma and multiple injuries elsewhere in the skeleton [16, 17]. In children with Gaucher disease, a rare hereditary disorder of lipid metabolism, midcervical or basicervical fractures can occur with minor or no trauma and without other injury to the skeleton. Three children with Gaucher disease who developed
Amy Beth Goldman; Bernard Jacobs
Fever of unknown origin, pleural and pericardial effusions can be caused by a variety of independent agents. On the other hand, we can identify a common causative condition in other cases. Infectious diseases, malignancies and autoimmune diseases are the most common etiological factors. Considering the pleural and pericardial effusion, we also have to think of cardiovascular and pulmonary diseases. The basis of every diagnostic process is thorough medical history and detailed clinical examination followed by laboratory and imaging methods. In spite of that, the right diagnosis sometimes stays long time hidden. One of such conditions is Adult-onset Stills disease (AOSD). It is a rare inflammatory, potentially life-threatening disease with unclear pathogenesis and heterogeneous symptoms. It has some features similar to systemic form of juvenile idiopathic arthritis. Diagnosis is established so called per exclusionem by fulfilling a set of clinical criteria and ruling out other diseases with similar symptomatology. In our article, we present an example of such an arduous diagnostic journey to final diagnosis. Key words: anakinra - arthralgia - evanescent rash - ferritin - fever of unknown origin - pericardial effusion - pleural effusion - procalcitonin - Stills disease. PMID:24974758
Malegová, Jana; Koten, Lukáš; Horák, Pavel
Although self-limited respiratory tract infections caused by Mycoplasma pneumoniae are well recognized in children and young adults, respiratory involvements and hepatic dysfunction may occur. The frequency and clinical features of these complications were investigated. Experimental studies with regard to bacterial superinfection were also carried out. The test animals which were first infected with Mycoplasma pneumoniae and then with Staphylococcus aureus showed more extensive bacteriological and pathological changes than those infected with Staphylococcus aureus only. Liver biopsies performed on three human patients showed hepatic dysfunction and the histological findings were diagnosed as non-specific reactive hepatitis in each case.
Suzuyama, Y.; Iwasaki, H.; Izumikawa, K.; Hara, K.
Postoperative cardiovascular complications are common, predictable, and typically treatable in geriatric patients who have sustained fractures. Although intervention-specific data are sparse, observational evidence from high-performing geriatric fracture centers coupled with an understanding of geriatric principles can serve as a basis for treatment guidelines. Many patients can be safely and effectively managed with close attention to intravascular volume status, heart rate control, and minimization of other physiologic stresses, including pain and delirium. Many chronic cardiovascular therapies may be harmful in the immediate postoperative period, and can usually be safely omitted or attenuated until hemodynamic stability and mobility have been restored. PMID:24721369
Nicholas, Joseph A
Atypical symptoms of celiac disease may cause a late diagnosis, revealed by the onset of complications, which may inaccurately be attributed to Crohn's disease, as manifestations frequently overlap. Assessing the entire small bowel mucosa, capsule endoscopy may be an accurate procedure in these challenging situations. We present four Crohn's disease patients diagnosed with ulcerative jejunoilieitis complicating celiac disease after capsule endoscopy procedure. In three of these patients, the ulcerative jejunoileitis led in time to stricture formation, suggesting Crohn's disease. Administration of non-steroidal anti-inflammatory drugs made the diagnosis even more difficult in one case. In patients with Crohn's disease not responding to immunosuppressive or biological treatment, complicated celiac disease should be considered and capsule endoscopy should be performed for reassessing the diagnosis. PMID:23539398
Ciobanu, Lidia; Pascu, Oliviu; Iobagiu, Silviu; Damian, Dana; Dumitru, Eugen; Tantau, Marcel
The number of adults with congenital heart disease (CHD) requiring diagnostic and/or therapeutic cardiac catheterization has been increasing. However, there have been few studies on the complications of performing cardiac catheterization in adults with CHD. The aim of this study was to determine the incidence of complications during congenital cardiac catheterization in both adults and pediatric patients. A total of 2134 consecutive cardiac catheterizations performed between 2003 and 2008 were prospectively analyzed. Complications were graded from 1 to 5 based on severity and these, with ? grade 3 being defined as major. During the study period, 576 procedures (393 diagnostic, 90 interventional, and 93 electrophysiological) were performed in adult patients (? 18 years). Complex heart disease was present in 435 of 576 procedures (75.6 %). A total of 65 complications (11.3 %) with 13 major complications including 1 death (2.3 %) were encountered. The most common complications were arrhythmias. The majority of complications were successfully treated or temporary, and all but one of the patients were without residua. Of the 1558 pediatric procedures performed during the same period, we found a total of 229 complications (14.7 %), of which 89 (5.7 %) were major complications including 5 deaths. The safety of performing cardiac catheterization for adult CHD appears to be similar to that for pediatric patients. The complication rates in adults with CHD are low, but not negligible. PMID:22457096
Mori, Yoshiki; Takahashi, Kazuhiro; Nakanishi, Toshio
We present a case series of three women of New Zealand M?ori ethnicity, who presented to the emergency department of a Brisbane hospital (Brisbane, Australia) with symptomatic cardiac complications of Graves disease requiring hospital admission. We raise the question as to whether individuals of M?ori ethnicity are genetically susceptible to cardiac complications of thyrotoxicosis. PMID:22282281
Nigam, Parul; Morton, Adam
Genome-wide association studies (GWAS) have identified several loci associated with many common, multifactorial diseases which have been recently used to market genetic testing directly to the consumers. We here addressed the clinical utility of such GWAS-derived genetic information in predicting type 2 diabetes mellitus (T2DM) and coronary artery disease (CAD) in diabetic patients. In addition, the development of new statistical approaches, novel technologies of genome sequencing and ethical, legal and social aspects related to genetic testing have been also addressed. Available data clearly show that, similarly to what reported for most common diseases, genetic testing offered today by commercial companies cannot be used as predicting tools for T2DM and CAD, both in the general and in the diabetic population. Further studies taking into account the complex interaction between genes as well as between genetic and non genetic factors, including age, obesity and glycemic control which seem to modify genetic effects on the risk of T2DM and CAD, might mitigate such negative conclusions. Also, addressing the role of relatively rare variants by next-generation sequencing may help identify novel and strong genetic markers with an important role in genetic prediction. Finally, statistical tools concentrated on reclassifying patients might be a useful application of genetic information for predicting many common diseases. By now, prediction of such diseases, including those of interest for the clinical diabetologist, have to be pursued by using traditional clinical markers which perform well and are not costly.
Prudente, Sabrina; Dallapiccola, Bruno; Pellegrini, Fabio; Doria, Alessandro; Trischitta, Vincenzo
Genome-wide association studies (GWAS) have identified several loci associated with many common, multifactorial diseases which have been recently used to market genetic testing directly to the consumers. We here addressed the clinical utility of such GWAS-derived genetic information in predicting type 2 diabetes mellitus (T2DM) and coronary artery disease (CAD) in diabetic patients. In addition, the development of new statistical approaches, novel technologies of genome sequencing and ethical, legal and social aspects related to genetic testing have been also addressed. Available data clearly show that, similarly to what reported for most common diseases, genetic testing offered today by commercial companies cannot be used as predicting tools for T2DM and CAD. Further studies taking into account the complex interaction between genes as well as between genetic and non-genetic factors, including age, obesity and glycemic control which seem to modify genetic effects on the risk of T2DM and CAD, might mitigate such negative conclusions. Also, addressing the role of relatively rare variants by next generation sequencing may help identify novel and strong genetic markers with an important role in genetic prediction. Finally, statistical tools concentrated on reclassifying patients might be a useful application of genetic information for predicting many common diseases. By now, prediction of such diseases, including those of interest for the clinical diabetologist, have to be pursued by using traditional clinical markers which perform well and are not costly. PMID:22819342
Prudente, S; Dallapiccola, B; Pellegrini, F; Doria, A; Trischitta, V
Eales disease, which is reported mainly in patients from the Indian subcontinent, is characterized by ophthalmic abnormalities that are sometimes followed by neurologic sequelae, and it is associated with previous Mycobacterium tuberculosis infection. We describe the first patient, to our knowledge, to receive a diagnosis of active tuberculosis and concurrent, severe neurological Eales disease, including peripheral neuropathy. The patient recovered completely after receiving steroid therapy. PMID:12355398
McCaughan, Frank; Holmes, Alison; Lynn, William A; Friedland, Jon S
Hyperbaric oxygen has been advocated in the treatment of many head and neck diseases. Reports of such treatments have described eustachian tube dysfunction as the only complication. A review of patients receiving hyperbaric oxygen for head and neck diseases at The Mount Sinai Medical Center revealed serious complications, which included seizure, stroke, and myocardial infarction. In addition, follow-up study of these patients demonstrated that 11 patients treated for radiation-induced necrosis had an undiagnosed recurrence of cancer.
Giebfried, J.W.; Lawson, W.; Biller, H.F.
Tissue granulomas formation in adult-onset Still's disease (AOSD) is extremely rare. We describe a case of AOSD associated with formation of granulomatous lesions in lymph nodes, liver and presumably spleen. The high dose steroid-dependent nature of our patient's illness, characterized by disease relapses when methylprednisolone dose was reduced below 10 mg/d, was overwhelmed with institution of anakinra (100 mg/d). The histologic finding of granulomas formation in lymph nodes, liver or spleen should not deter the consideration of AOSD as a potential diagnosis in a compatible clinical context; however, other more common etiologies of tissue granulomas formation should be first excluded. PMID:23519167
Assimakopoulos, Stelios F; Karamouzos, Vassilios; Papakonstantinou, Christos; Zolota, Vassiliki; Labropoulou-Karatza, Chryssoula; Gogos, Charalambos
Introduction Macrophage activating syndrome is a severe, potentially life-threatening condition that may accompany Still's disease. It is characterized by fever, hepatosplenomegaly, lymphadenopathy, severe cytopenia, serious liver dysfunction, coagulopathy and neurologic involvement. The principal treatment for patients with this syndrome includes etoposide 150 mg/2 M twice a week for two weeks, dexamethasone 10 mg/2 M for two weeks and cyclosporine 3 mg/kg to 5 mg/kg for a longer period. Cases of relapse of macrophage activating syndrome are relatively rare. Case presentation We describe the case of a 15-year-old Iraqi girl with Still's disease who developed macrophage activating syndrome with acute respiratory distress syndrome that required resuscitation and mechanical ventilation. Following intensive treatment, including high dose steroids and cyclosporine, the patient improved significantly. Two weeks after cyclosporine was discontinued, however, she was readmitted with an acute relapse of macrophage activating syndrome manifested by spiking fever, arthralgias, maculopapular rash and leukocytosis. This time the patient recovered following the reintroduction of treatment with cyclosporine and the addition of mycophenolate mofetil (Cellcept). Conclusion We believe that cyclosporine is a cornerstone for the treatment of Still's disease. We recommend continuing this medication for several weeks following the patient's clinical recovery in order to prevent macrophage activating syndrome relapses.
Irritable bowel syndrome (IBS) is the most frequently diagnosed functional gastrointestinal disorder in primary and secondary care. It is characterised by abdominal discomfort, pain and changes in bowel habits that can have a serious impact on the patient’s quality of life. The pathophysiology of IBS is not yet completely clear. Genetic, immune, environmental, inflammatory, neurological and psychological factors, in addition to visceral hypersensitivity, can all play an important role, one that most likely involves the complex interactions between the gut and the brain (gut-brain axis). The diagnosis of IBS can only be made on the basis of the symptoms of the Rome III criteria. Because the probability of organic disease in patients fulfilling the IBS criteria is very low, a careful medical history is critical and should pay particular attention to the possible comorbidities. Nevertheless, the severity of the patient’s symptoms or concerns sometimes compels the physician to perform useless and/or expensive diagnostic tests, transforming IBS into a diagnosis of exclusion. The presence of alarming symptoms (fever, weight loss, rectal bleeding, significant changes in blood chemistry), the presence of palpable abdominal masses, any recent onset of symptoms in patient aged over 50 years, the presence of symptoms at night, and a familial history of celiac disease, colorectal cancer and/or inflammatory bowel diseases all warrant investigation. Treatment strategies are based on the nature and severity of the symptoms, the degree of functional impairment of the bowel habits, and the presence of psychosocial disorders. This review examines and discusses the pathophysiological aspects and the diagnostic and therapeutic approaches available for patients with symptoms possibly related to IBS, pointing out controversial issues and the strengths and weaknesses of the current knowledge.
Bellini, Massimo; Gambaccini, Dario; Stasi, Cristina; Urbano, Maria Teresa; Marchi, Santino; Usai-Satta, Paolo
Familial dysautonomia (FD) is an autosomal recessive disorder characterized by autonomic and sensory neuropathy. Owing to pervasive dysfunction, the disease has protean clinical manifestations, affecting the ocular, gastrointestinal, pulmonary, orthopedic, vasomotor, and neurologic systems. The gastrointestinal perturbations, including dysphagia, gastroesophageal dysmotility, gastroesophageal reflux, and vomiting crises, are among the earliest signs. Here, we present the first 3 instances of gastric ulcers in patients with FD and discuss their common presenting features and the special management that was required. PMID:20930641
Wan, David W; Levy, Joseph; Ginsburg, Howard B; Kaufmann, Horacio; Axelrod, Felicia B
\\u000a Osteoporosis is the commonest condition to affect the bony skeleton producing considerable morbidity and mortality to those\\u000a who sustain fractures. The majority of those affected are post-menopausal women and elderly people of both sexes. Although\\u000a the majority of sufferers of osteoporosis are otherwise healthy people, a variety of diseases are associated with osteoporosis\\u000a and sometimes treatment instituted for a particular
Accurate knowledge of the composition and ecology of vaginal microbial environment of a healthy woman is necessary for the understanding of normal flora and how to reduce the risk for diseases. Vagina and its microflora form a balanced ecosystem in which dominated bacteria are vaginal lactobacilli. There are dynamic changes in this ecosystem having structure and composition depending on many factors. The term dysbacteriosis defines any movement outside the normal range for the given biotope of obligate and/or facultative microflora. Such a change in the quantity and quality of the respective microbial balance is fraught with danger and requires correction and recovery. The purpose of this overview is to examine obstetric and gynecological diseases that can cause vaginal impaired microbial balance. Vaginal dysbacteriosis is a cause, predecessor, and often also consequence of vaginal infections. In essence, any vaginal infection can be seen as dysbacteriosis, developed to the most severe extent. Here, there is a dominant microorganism other than lactic acid bacteria in the vagina (clinically manifested or not, respectively), depletion of defense mechanisms of the vagina associated with the shift of lactobacilli from their dominant role in the vaginal balance, decrease in their number and species diversity, and a resulting change in the healthy status of the vagina. Vaginal dysbacteriosis can be found in pathogenetic mechanism, whereby many obstetric and gynecological diseases develop. Most of these diseases lead directly to increased maternal and infant morbidity and mortality, so it is important to understand the reasons for them and the arrangements for their prevention. PMID:24711012
Kovachev, Stefan Miladinov
Ascorbic acid is one of the important water soluble vitamins. It is essential for collagen, carnitine and neurotransmitters biosynthesis. Most plants and animals synthesize ascorbic acid for their own requirement. However, apes and humans can not synthesize ascorbic acid due to lack of an enzyme gulonolactone oxidase. Hence, ascorbic acid has to be supplemented mainly through fruits, vegetables and tablets. The current US recommended daily allowance (RDA) for ascorbic acid ranges between 100–120 mg/per day for adults. Many health benefits have been attributed to ascorbic acid such as antioxidant, anti-atherogenic, anti-carcinogenic, immunomodulator and prevents cold etc. However, lately the health benefits of ascorbic acid has been the subject of debate and controversies viz., Danger of mega doses of ascorbic acid? Does ascorbic acid act as a antioxidant or pro-oxidant ? Does ascorbic acid cause cancer or may interfere with cancer therapy? However, the Panel on dietary antioxidants and related compounds stated that the in vivo data do not clearly show a relationship between excess ascorbic acid intake and kidney stone formation, pro-oxidant effects, excess iron absorption. A number of clinical and epidemiological studies on anti-carcinogenic effects of ascorbic acid in humans did not show any conclusive beneficial effects on various types of cancer except gastric cancer. Recently, a few derivatives of ascorbic acid were tested on cancer cells, among them ascorbic acid esters showed promising anticancer activity compared to ascorbic acid. Ascorbyl stearate was found to inhibit proliferation of human cancer cells by interfering with cell cycle progression, induced apoptosis by modulation of signal transduction pathways. However, more mechanistic and human in vivo studies are needed to understand and elucidate the molecular mechanism underlying the anti-carcinogenic property of ascorbic acid. Thus, though ascorbic acid was discovered in 17th century, the exact role of this vitamin/nutraceutical in human biology and health is still a mystery in view of many beneficial claims and controversies.
Naidu, K Akhilender
Over the last decade, there has been an increasing attention to the role played by emotional processes in Parkinson's disease (PD). However, most of what is known in this area is based on research conducted in laboratory or clinical settings. In this article, the authors underline the need to expand our current knowledge of the psychological correlates of PD by investigating patients' everyday emotions in natural contexts. Specifically, the authors illustrate new research avenues based on the implementation of experience sampling methods. It is argued that these methods could permit future researchers to ecologically assess the frequency and intensity with which parkinsonian patients experience specific emotions (either negative or positive) during their everyday life, providing at the same time precious information on what are the most typical situations in which these emotions occur and on how patients behave in these circumstances. Potential practical implications associated with investigating these issues are discussed.
Sotgiu, Igor; Rusconi, Maria L.
Nonalcoholic fatty liver disease (NAFLD) refers to a spectrum of liver damage ranging from simple steatosis to nonalcoholic steatohepatitis, advanced fibrosis, and rarely, progression to cirrhosis. The pathogenesis of NAFLD is thought to be related to insulin resistance and oxidant stress. Truncal obesity, dyslipidema, hypertension, and hyperglycemia are strongly associated with NAFLD; therefore, management of NAFLD entails identification and treatment of metabolic risk factors, improving insulin sensitivity, and increasing antioxidant defenses in the liver. This article briefly summarizes advances in our understanding of the relationship between NAFLD and the insulin resistance (metabolic) syndrome, its prevalence, natural history, and treatment. PMID:17964913
Abdelmalek, Manal F; Diehl, Anna Mae
Radiation-induced arterial disease is caused by significant atherosclerosis in the circumjacent vessels being irradiated. Even though this has been recognized as survival of cancer patients treated with radiotherapy improves, it is a problem that is often under-reported. We present a case of chronic thromboembolic occlusion of right common iliac artery in a 53-year-old woman who was treated with radiation therapy for cervical cancer 13 years ago. We initially performed percutaneous transluminal angioplasty with thrombolytic therapy, but had to cease thrombolytic therapy due to upper gastrointestinal bleeding of Dieulafoy's lesion, nevertheless, achieved good results after revascularization by Fogarty embolectomy.
Won, Ki-Bum; Kim, Byeong-Keuk; Ko, Young-Guk; Hong, Myeong-Ki; Jang, Yangsoo
There is abundant evidence that mast cells are active participants in events that mediate tissue damage in autoimmune disease. Disease-associated increases in mast cell numbers accompanied by mast cell degranulation and elaboration of numerous mast cell mediators at sites of inflammation are commonly observed in many human autoimmune diseases including multiple sclerosis, rheumatoid arthritis, and bullous pemphigoid. In animal models, treatment with mast cell stabilizing drugs or mast cell ablation can result in diminished disease. A variety of receptors including those engaged by antibody, complement, pathogens, and intrinsic danger signals are implicated in mast cell activation in disease. Similar to their role as first responders in infection settings, mast cells likely orchestrate early recruitment of immune cells, including neutrophils, to the sites of autoimmune destruction. This co-localization promotes cellular crosstalk and activation and results in the amplification of the local inflammatory response thereby promoting and sustaining tissue damage. Despite the evidence, there is still a debate regarding the relative role of mast cells in these processes. However, by definition, mast cells can only act as accessory cells to the self-reactive T and/or antibody driven autoimmune responses. Thus, when evaluating mast cell involvement using existing and somewhat imperfect animal models of disease, their importance is sometimes obscured. However, these potent immune cells are undoubtedly major contributors to autoimmunity and should be considered as important targets for therapeutic disease intervention.
Brown, Melissa A.; Hatfield, Julianne K.
BackgroundPrediction of adverse maternal and neonatal events in women with heart disease is not well established. We aimed to assess cardiac, obstetrical and neonatal complications in pregnant women with heart disease referred to our tertiary care center and validate a previously proposed risk index.
Nicole Jastrow; Philippe Meyer; Paul Khairy; Lise-Andrée Mercier; Annie Dore; François Marcotte; Line Leduc
A considerable proportion of patients with Wilson's disease (WD) experience neurologic symptoms that are functionally disabling. The most common neurologic problems in advanced WD include dystonia and tremor. Medically refractory idiopathic dystonia and essential tremor (ET) have been successfully treated with deep brain stimulation (DBS), functional surgical therapy targeting the globus pallidus pars interna (GPi), or the ventral intermediate (Vim) thalamic nucleus. Even though the pathophysiology of tremor is different in WD and ET, available experience supports DBS targeting the Vim for WD patients. Dystonia associated with WD is classified as secondary dystonia and GPi stimulation has yielded mixed results in these patients. The presence of structural changes in the basal ganglia may limit the therapeutic success of DBS for WD dystonia compared with idiopathic dystonia. In spite of these limitations, DBS in WD may be an effective approach to treat medically refractory residual neurologic symptoms in carefully selected patients. PMID:24547944
Sickle cell disease is an inherited hemoglobinopathy with multi system complications. It has been associated with multiple maternal complications. A retrospective review of 68 consecutive pregnant women with sickle cell disease, followed in a tertiary center, was conducted over 5 years, to estimate the incidence of different maternal complications and the impact of baseline characteristics. Sixty-eight patients were analyzed (mean age 30 years). Sixty-two patients had a Hb SS genotype. The initial mean hemoglobin (Hb) level was 9.5 g/dL. Twelve patients delivered by Cesarean section. Sixty-five patients required admission for sickle cell disease/pregnancy-related complications [96.0%; 95% confidence interval (95% CI) 91-100]. Infection was seen in 17 patients (25.0%, 95% CI 14-36). Blood transfusions were given to 61 patients (90.0%, 95% CI 82-97). Eight patients had gestational hypertension (18.0%, 95% CI 4-20), while five patients (7.0%, 95% CI 1-14) had pre term labor. One patient developed eclampsia and one had a uterine rupture. One patient died due to post partum hemorrhage. The multi variable logistic regression model on the impact on the major maternal complications revealed none of the baseline factors to be statistically significant. Sickle cell disease patients have low mortality and pregnancy-related morbidity but high sickle cell disease-related morbidity. Prospective studies are needed to confirm these results. PMID:23590330
Al-Farsi, Sharifa H; Al-Riyami, Nihal M; Al-Khabori, Murtadha K; Al-Hunaini, Mohammed N
We report a case of a 47-year-old man with multicentric Castleman's disease (MCD) and progressive renal dysfunction due to mesangial proliferative glomerulonephritis, possibly from IgA nephropathy. At age 36 years, he was referred to a hematologist due to hypergammaglobulinemia. Because of systemic lymph node swelling, he underwent right cervical lymph node biopsy at age 41 years and MCD (plasma cell type)was diagnosed. During this period, microscopic hematuria and persistent proteinuria occurred and his renal function deteriorated (serum creatinine (Cr) rising from 0.7 mg/dL to 1.4 mg/dL). Treatment with intravenous methylprednisolone at the dose of 1 g daily for 3 days followed by oral prednisolone at 20 mg daily reduced his lymphadenopathy and improved the renal function. However, his renal function deteriorated again, from Cr 0.8 mg/dL to 1.8 mg/dL over 6 years in line with gradual prednisolone tapering to 6 mg daily. At age 47 years, he was referred to our nephrology department and underwent a renal biopsy. The microscopic examination showed IgA nephropathy with crescent formation, accompanied by mild lymphoplasmacytic tubulointerstitial nephritis. Treatment with the same dose of intravenous methylprednisolone therapy followed by oral prednisolone at 40 mg daily, improved his proteinuria, hematuria and renal dysfunction. The coexistence of MCD and IgA nephropathy is a rare phenomenon. In addition, IL-6, overproduced by MCD might have influenced the mesangial cell proliferation and the activity of IgA nephropathy in the present case. PMID:21516705
Fujiwara, Hiroaki; Mise, Naobumi; Ishimoto, Yu; Kotera, Nagaaki; Tanaka, Mototsugu; Tanaka, Shinji; Kurita, Noriaki; Fujii, Akiko; Yamaguchi, Yutaka; Sugimoto, Tokuichiro
Bronchiolitis obliterans (BO) is a manifestation of chronic graft-versus-host disease (GVHD) after allogeneic haemopoietic stem cell transplantation. Complications associated with this include persistent air-leak syndromes such as pneumothorax. Many methods have been described for treating this condition, both surgical and nonsurgical. We describe an 8-year-old boy with acute lymphoblastic leukaemia complicated by chronic GVHD-related BO, and subsequent pneumothorax with persistent
C Chadwick; S M Marven; A J Vora
Pyogenic liver abscess is a rarely seen extraintestinal complication of Crohn's disease. It has different features from other liver abscesses. Its clinical and laboratory findings are not specific and mimic the reactivation of Crohn's disease and diagnosis can be delayed. The radiological methods are very useful in diagnosis and treatment of liver abscess. In this paper, we present a patient with pyogenic liver abscess which developed in the course of Crohn's disease. PMID:15264121
Karaca, Cetin; Pinarba?i, Binnur; Danalio?lu, Ahmet; Akyüz, Filiz; Kaymako?lu, Sabahattin; Ozdil, Sadakat; Bozta?, Güngör; Mungan, Zeynel
This cross-sectional study was carried out at Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka Medical College Hospital (DMCH), National Institute of Kidney Diseases & Urology (NIKDU) and Kidney Foundation from July 2005 to June 2007 to find the cardiovascular complications in end stage renal disease (ESRD) patients on maintenance haemodialysis. Patients of both sexes with age ranging from 18-59 years and getting at least 8 hours of haemodialysis per week for the last 3 months were enrolled in the study. A total of 126 such patients were included in the study. Among 126 patients 77(61.1%) developed some types of cardiovascular complications. In terms of type of complications 63.6% of the patients had LVH, 23.4% had ischemic heart disease (IHD) and 10.4% had congestive heart failure (CCF) and 2.6% cardiomyopathy. Over 96% patients were hypertensive, followed by 46.8% diabetics and 42.1% smokers. Presence of hypertension, diabetes, family history of diabetes and hypertension were observed to be significantly higher in patients who developed cardiovascular complications (p<0.05). It is deserved that cardiovascular complications (CVC) are very common in ESRD patients on maintenance haemodialysis (MHD). Poor control of blood pressure, low Haemoglobin level and poor glycaemic control are higher in ESRD patients on MHD and are possibly related to the development of cardiovascular complications. PMID:24858162
Sweety, S A; Arzu, J; Rahman, M; Salim, M A; Mahmood, M
The sickle hemoglobin is an abnormal hemoglobin due to point mutation (GAG ? GTG) in exon 1 of the ? globin gene resulting in the substitution of glutamic acid by valine at position 6 of the ? globin polypeptide chain. Although the molecular lesion is a single-point mutation, the sickle gene is pleiotropic in nature causing multiple phenotypic expressions that constitute the various complications of sickle cell disease in general and sickle cell anemia in particular. The disease itself is chronic in nature but many of its complications are acute such as the recurrent acute painful crises (its hallmark), acute chest syndrome, and priapism. These complications vary considerably among patients, in the same patient with time, among countries and with age and sex. To date, there is no well-established consensus among providers on the management of the complications of sickle cell disease due in part to lack of evidence and in part to differences in the experience of providers. It is the aim of this paper to review available current approaches to manage the major complications of sickle cell disease. We hope that this will establish another preliminary forum among providers that may eventually lead the way to better outcomes.
Ballas, Samir K.; Kesen, Muge R.; Goldberg, Morton F.; Lutty, Gerard A.; Dampier, Carlton; Osunkwo, Ifeyinwa; Wang, Winfred C.; Hoppe, Carolyn; Hagar, Ward; Darbari, Deepika S.; Malik, Punam
A patient with homozygous hemoglobin SS disease presented with an intracerebral hemorrhage complicating reversible posterior leucoencephalopathy syndrome (RPLS), secondary to hypertension associated with acute post-streptococcal glomerulonephritis (APSGN). Distinguishing potentially reversible causes of central nervous system events from primary cerebral infarction or hemorrhage in patients with sickle cell disease is important because the management and prognosis of these complications is very different. Similarly, because of the difference in prognosis between APSGN and other forms of sickle cell nephropathy, it is also important to differentiate these conditions. PMID:17973321
Pashankar, Farzana D; Ment, Laura R; Pearson, Howard A
Continuous infusion of intraduodenal levodopa/carbidopa is an effective treatment that improves the motor complications and the quality of life of patients in the advanced stages of Parkinson's disease. However, it is not free of complications. These may present in the post-operative period following surgery (gastrostomy) or in the long-term during the follow-up period and can be related with the medication (levodopa/carbidopa), the stoma, the gastrostomy or the device (pump, enteral tube, parts of the FREKA system). The aim of this review is to report on the management of the complications that can be observed in patients with advanced Parkinson's disease treated with continuous infusion of intraduodenal levodopa/carbidopa. PMID:24861226
Santos-Garcia, Diego; de Deus, Teresa; Lopez-Pazos, Elina; Macias-Arribi, Mercedes; Llaneza-Gonzalez, Miguel A; Echarri-Piudo, Ana; Carpintero, Pedro; de la Fuente-Fernandez, Raúl
Background. Chronic granulomatous disease (CGD) is a genetic disorder of the phagocyte NADPH oxidase, which predisposes patients to infections and inflammatory complications, including severe colitis. Management of CGD colitis is a challenge because standard immunosuppressive therapy increases the risk of infection in already immunocompromised hosts. Methods. We report the use of infliximab in 5 patients with CGD. Results. Infliximab administration predisposed patients to severe infections with typical CGD pathogens but not mycobacteria, as reported with infliximab in other conditions. In addition to infections, infliximab administration led to successful closure of fistulae, sometimes with other untoward consequences. Infliximab-associated complications were associated with 2 deaths. Conclusions. Infliximab use in the treatment of CGD inflammatory bowel disease requires aggressive antimicrobial prophylaxis, assiduous surveillance for infection, and vigilance for untoward gastrointestinal complications. This experience suggests that infliximab therapy is effective but has untoward consequences in patients with CGD.
Uzel, Gulbu; Orange, Jordan S.; Poliak, Nina; Marciano, Beatriz E.; Heller, Theo; Holland, Steven M.
Maternal predisposition to vascular and metabolic disease may underlie both vascular-related pregnancy complications, such as preeclampsia and intrauterine growth restriction, as well as future maternal cardiovascular disease. We aimed to substantiate this hypothesis with biochemical and anthropometric evidence by conducting an intergenerational case-control study in a Dutch isolated population including 106 women after preeclampsia or intrauterine growth restriction (median follow-up:
A. L. Berends; Groot de C. J. M; E. J. G. Sijbrands; M. P. S. Sie; S. H. Benneheij; R. Pal; R. Heydanus; B. A. Oostra; P. Tikka-Kleemola; R. P. M. Steegers-Theunissen
We have performed surgical treatment for Moyamoya disease using the superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis and encephalo-duro-arterio-myo-synangiosis (EDAMS). In this paper, the surgical technique of combined revascularization for Moyamoya disease as well as peri-operative complications are discussed. Craniotomy and dural opening were extensively carried out to expose the brain surface as widely as possible. Dissection of
Kiyohiro Houkin; Tatsuya Ishikawa; Tetsuyuki Yoshimoto; Hiroshi Abe
We report an unusual case of small bowel obstruction caused by an intestinal cast in an 8-year-old female who developed intestinal graft-versus-host disease (GVHD) following two unrelated bone marrow transplants for aplastic anemia, and highlight the pathophysiology, common presentations, and surgical complications of intestinal GVHD from the surgeons' perspective. PMID:24805116
Grant, Christa N; Nellis, Eric D; Chahine, A Alfred
Complications involving the central nervous system are among the most devastating manifestations of sickle cell disease. Although overt stroke occurs in 1 in 10 children with Hemoglobin SS, “silent cerebral infarcts” are even more frequent. Both are associated with significant neuropsychological deficits. The end result of these effects on the CNS often is diminished school performance. The use of transcranial
Winfred C. Wang
Resistance to the actions of insulin is strongly associated with the microvascular complications of diabetes. To the extent that insulin resistance leads to hyperglycemia, dyslipidemia and hypertension, this association is not surprising. It is now clear that insulin also has direct actions in the microvasculature that influence the development and progression of microvascular disease. In the healthy state, insulin appears
Carol Forsblom; Merlin C Thomas; Per-Henrik Groop
A survey of 186 pediatric patients with neoplastic disease at Riley Children's Hospital in Indiana was conducted to determine the distribution, frequency, and types of oral prob- lems encountered during their hospitalization. Thirty-one per cent of the patients had some form of oral complications during the course of their hospitalization. Ten per cent of the patient population had existing dental
Anthony A. Kamp
Background As diagnostic techniques advance and surgical outcomes improve, the rate of utilization of liver hemihepatectomy for various indications will continue to increase. Objectives To explore the preoperative predictors of liver hemihepatectomy postoperative complications. Patients and methods This study included retrospective analysis of the clinical data of patients who underwent either liver hemihepatectomy or extended hemihepatectomy at Georg August University Hospital-Goettingen for the period 2002–2012. The outcomes were either postoperative complications or death of the patient (within 3 months from the end of the operation). Modified classification of surgical complications was adopted in the current study. The preoperative model for end-stage liver disease (MELD) score, aspartate aminotransferase, creatinine, international normalized ratio, and bilirubin in addition to the demographic characteristics of the patients and intraoperative blood loss were analyzed as predictive for postliver hemihepatectomy complications. Results The study included 144 patients who underwent liver hemiheptectomy or extended hemihepatectomy through the study period (2002–2012). Postoperative complications were reported among patients out of 144 (52.1%). The most frequent complications were pleural effusion (26.7%), biliary leakage (21.3%), wound dehiscence (13.3%), ascites, and intra-abdominal abscess (6.7%). Death was reported among six patients of those who developed complications (8%). There were four cases of hepatic cirrhosis (one macroscopic and three microscopic). Two of the microscopic cases had no postoperative complications (grade 1), whereas one case had grade 3a and the macroscopic case had postoperative complication grade 1. Their MELD scores ranged between 6 and 10 preoperatively. The association between preoperative MELD score and development of posthemihepatetomy was statistically significant, P=0.002. Death was reported in six cases, yielding a mortality rate of 4.17%. MELD score preoperatively was the only significant predictor for postoperative complications. Conclusion The rate of complications following hemihepatectomy remains high, with 52.1% of the patients in the current study having at least one complication as all of our patients underwent either hemihepatectomy or extended hemihepatectomy. A 4.17% mortality rate has been reported. A higher preoperative MELD score is the only significant predictor for the development of posthemihepatectomy complications.
Abdel-Fattah, Muataz; Zautner, Andreas; Lorf, Thomas
The colon responds monomorphically to a variety of insults thus making it difficult to differentiate invasive amoebic colitis and inflammatory bowel disease (IBD). The authors present a case with chronic dysentery, haematochezia, anaemia and hypoproteinaemia. The endoscopic findings were suggestive of IBD. The stool examination was negative for trophozoites or cysts of parasites. The recto-colonic biopsy specimens showed mucosal inflammation with exudates containing amoebic trophozoites. The patient was successfully treated with metronidazole and iodoquinol. He recovered within two weeks and repeat colonoscopy four weeks after the treatment showed a normal rectum and colon. Clinicians should have a high level of suspicion for amoebic colitis in cases of colitis especially in regions where amoebiasis is still present. Efforts should be made to find the amoebic trophozoites in multiple stool and colonic biopsy specimens. PMID:16209229
Ibrahim, T M; Iheonunekwu, N; Gill, V; Vantapool, H
Motor fluctuations and dyskinesias are common motor complications that manifest within the first few years from the initiation of therapy in patients with Parkinson disease. These complications negatively affect the quality of life and represent an important source of disability. A growing number of therapeutic options including treatments aimed at prolonging the efficacy of levodopa (eg, selective monoamine oxidase-B inhibitors and catechol-O-methyltransferase inhibitors), administration of longer-acting dopamine agonists (eg, rotigotine, sustained-release ropinirole), and continuous administration of intraduodenal levodopa exist or will soon become available. Patients who maintain a good response to levodopa but continue to experience disabling motor complications despite the best medical management may benefit from a regimen of subcutaneous apomorphine, ideally delivered by a subcutaneous pump, or deep-brain stimulation of the subthalamic nucleus or internal portion of the pallidum. Emerging therapies for motor complications are expected to further enhance continuous (physiologic) delivery of dopaminergic drugs and extend the reach of therapies beyond the dopaminergic system to influence not only the motor but also the vast range of nonmotor complications of this multisystemic disease. PMID:20816270
Espay, Alberto J
There have been increasing concerns regarding the safety of perioperative anti-tumour necrosis factor (anti-TNF) ? agents. We performed a literature review to evaluate the post-operative complications associated with perioperative anti-TNF use in patients with inflammatory bowel disease. A comprehensive review was performed with a literature search utilizing Pub Med, Cochrane, OVID and EMBASE databases according to published guidelines. To date, there are only data for infliximab. There are three published studies which have assessed post-operative complications with perioperative infliximab use in patients with Crohn’s disease (CD), four studies in ulcerative colitis (UC) patients, and one study on both CD and UC patients. Two out of the three studies in CD patients showed no increased post-operative complications associated with perioperative infliximab. Two out of four studies in UC patients also did not show an increase in post-operative complications, and the combined study with CD and UC patients did not show an increased risk as well. Study results could not be combined secondary to significant differences in study designs, patient population and definition of their endpoints. There appears to be a risk of post-operative complications associated with TNF therapy in some patients. Based on these data, careful patient selection and prospective data collection should be performed.
Ali, Tauseef; Yun, Laura; Rubin, David T
Introduction The objective of this study was to investigate the potential role of the Toll-like receptor 7 (TLR7) signaling pathway in the pathogenesis of adult-onset Still's disease (AOSD). Methods Frequencies of TLR7-expressing precursor of myeloid dendritic cells (pre-mDCs) and mDCs in 28 AOSD patients, 28 patients with systemic lupus erythematosus (SLE) and 12 healthy controls (HC) were determined by flow cytometry analysis. Transcript and protein levels of TLR7 signaling molecules in peripheral blood mononuclear cells (PBMCs) were evaluated by quantitative PCR and western blotting respectively. Serum cytokines levels were measured by ELISA. Results Significantly higher median frequencies of TLR7-expressing pre-mDCs and mDCs were observed in AOSD patients (65.5% and 14.9%, respectively) and in SLE patients (60.3% and 14.4%, respectively) than in HC (42.8% and 8.8%, respectively; both P <0.001). Transcript and protein levels of TLR7-signaling molecules, including MyD88, TRAF6, IRAK4 and IFN-?, were upregulated in AOSD patients and SLE patients compared with those in HC. Disease activity scores were positively correlated with the frequencies of TLR7-expressing mDCs and expression levels of TLR7 signaling molecules in both AOSD and SLE patients. TLR7 ligand (imiquimod) stimulation of PBMCs resulted in significantly enhanced levels of interleukin (IL)-1?, IL-6, IL-18 and IFN-? in AOSD and SLE patients. Frequencies of TLR7-expressing mDCs and expression levels of TLR7 signaling molecules significantly decreased after effective therapy. Conclusions Elevated levels of TLR7 signaling molecules and their positive correlation with disease activity in AOSD patients suggest involvement of the TLR7 signaling pathway in the pathogenesis of this disease. The overexpression of TLR7 MyD88-dependent signaling molecules may be a common pathogenic mechanism for both AOSD and SLE.
Patients with rheumatic diseases have an increased risk of mortality by cardiovascular events. In fact, several rheumatic diseases such as rheumatoid arthritis, osteoarthritis, systemic lupus erythematosus, and ankylosing spondylitis are associated with a higher prevalence of cardiovascular diseases (CVDs). Although traditional cardiovascular risk factors have been involved in the pathogenesis of cardiovascular diseases in rheumatic patients, these alterations do not completely explain the enhanced cardiovascular risk in this population. Obesity and its pathologic alteration of fat mass and dysfunction, due to an altered pattern of secretion of proinflammatory adipokines, could be one of the links between cardiovascular and rheumatic diseases. Indeed, the incidence of CVDs is augmented in obese individuals with rheumatic disorders. Thus, in this paper we explore in detail the relationships among adipokines, rheumatic diseases, and cardiovascular complications by giving to the reader a holistic vision and several suggestions for future perspectives and potential clinical implications.
Scotece, Morena; Conde, Javier; Gomez, Rodolfo; Lopez, Veronica; Pino, Jesus; Gonzalez, Antonio; Lago, Francisca; Gomez-Reino, Juan J.; Gualillo, Oreste
Background We studied the distribution of causes of death in the CONTRAST cohort and compared the proportion of cardiovascular deaths with other populations to answer the question whether cardiovascular mortality is still the principal cause of death in end stage renal disease. In addition, we compared patients who died from the three most common death causes. Finally, we aimed to study factors related to dialysis withdrawal. Methods We used data from CONTRAST, a randomized controlled trial in 714 chronic hemodialysis patients comparing the effects of online hemodiafiltration versus low-flux hemodialysis. Causes of death were adjudicated. The distribution of causes of death was compared to that of the Dutch dialysis registry and of the Dutch general population. Results In CONTRAST, 231 patients died on treatment. 32% died from cardiovascular disease, 22% due to infection and 23% because of dialysis withdrawal. These proportions were similar to those in the Dutch dialysis registry and the proportional cardiovascular mortality was similar to that of the Dutch general population. cardiovascular death was more common in patients <60 years. Patients who withdrew were older, had more co-morbidity and a lower mental quality of life at baseline. Patients who withdrew had much co-morbidity. 46% died within 5 days after the last dialysis session. Conclusions Although the absolute risk of death is much higher, the proportion of cardiovascular deaths in a prevalent end stage renal disease population is similar to that of the general population. In older hemodialysis patients cardiovascular and non-cardiovascular death risk are equally important. Particularly the registration of dialysis withdrawal deserves attention. These findings may be partly limited to the Dutch population.
den Hoedt, Claire H.; Bots, Michiel L.; Grooteman, Muriel P. C.; Mazairac, Albert H. A.; Penne, E. Lars; van der Weerd, Neelke C.; ter Wee, Piet M.; Nube, Menso J.; Levesque, Renee; Blankestijn, Peter J.; van den Dorpel, Marinus A.
Allogenic peripheral blood stem cell transplantation (Allo-PBSCT) is being used to treat hematological malignancies with increasing frequency. Graft-versus-host disease (GvHD) is a complex complication of PBSCT. A 43-year-old woman came to the gynecology clinic for amenorrhea. She had been diagnosed with acute myeloid leukemia 2 years earlier and treated with induction and consolidation chemotherapy. After developing complete remission, she underwent Allo-PBSCT. When she started chemotherapy, her menstrual cycle completely disappeared. Fourteen months after menopausal hormone replacement therapy, it was discovered that her upper vaginal canal was completely obstructed. The lower vagina had an atrophic appearance. We report a rare case of partial vaginal obstruction as a complication of chronic GvHD and review the literature. We expect that this case report provides an opportunity to remind clinician of the gynecologic complications of GvHD.
Park, Junsik; Lee, Hae-Hyeog; Chung, Soo-Ho; Lee, Daegeun
The authors offer analysis of surgical treatment methods of patients suffering from complicated forms of gall-bladder and anhepatic bile-excreting ducts' diseases. In elaborating tactics for treatment of the above-mentioned pathology the presently existing technical and tactical approaches are considered and the most acceptable for receiving favourable results are chosen. In implementing the operation the authors gave utmost attention to the choice of the method of bile-duct drainage depending on the severity of the disease, the age and the concomitant diseases. Taking into consideration application of the most approved and effective methods of treatment of complicated forms of gall-bladder and bile-duct diseases, 191 patients were operated within the period of 1990-2008 years. The operation cholecystectomy together with drainage of the choledochus was carried out in 64 cases, choledochoduodenostomy - in 54 cases, choledochojejunostomy - in 59 cases, reconstruction of the choledochus over a T-tube Kerr's drainage - in 8 cases. The portion of the gall-duct retained after the first operation was removed to 3 patients. There were 18 (9,4%) cases of complications in post-operation period, 8 patients died, lethality was 4,2%. PMID:22870828
Archvadze, B K; Tedoradze, V O; Tsekhelashvili, L O; Berishvili, K L
The pathogenesis of moyamoya disease remains unknown. Examination of diseases concurrent with moyamoya disease may offer a clue to clarify the pathogenesis. Coexistence of moyamoya disease, Graves' disease, and diabetes mellitus is very rare. We present the first cases in the literature. A 38-year-old man with moyamoya disease and a 43-year-old woman with quasi-moyamoya disease, both concurrent with Graves' disease and type 2 diabetes mellitus, are presented. Both patients underwent antithyroid therapy and revascularization. After normalization of thyroid hormones level and blood glucose level followed by revascularization, the symptoms of cerebral ischemia were improved. The common etiological factors of these diseases are discussed. Genetic and autoimmune factor appeared to be involved in the pathogenesis of the three diseases, which may suggest that these factors play important roles in the pathogenesis of moyamoya disease. Further studies are required to define the pathogenesis of moyamoya disease, especially in cases with comorbidities as in the present patients. PMID:21215511
Suzuki, Sakiko; Mitsuyama, Tetsuryu; Horiba, Ayako; Fukushima, Sayaka; Hashimoto, Naotake; Kawamata, Takakazu
BackgroundThe contribution of hypercoagulability to the pathophysiology of sickle cell disease (SCD) remains poorly defined. We sought to evaluate the association of markers of coagulation and platelet activation with specific clinical complications and laboratory variables in patients with SCD.Design and MethodsPlasma markers of coagulation activation (D-dimer and TAT), platelet activation (soluble CD40 ligand), microparticle-associated tissue factor (MPTF) procoagulant activity and
Kenneth I. Ataga; Julia E. Brittain; Payal Desai; Ryan May; Susan Jones; John Delaney; Dell Strayhorn; Alan Hinderliter; Nigel S. Key
Humoral immune mechanisms may have a role in the neurological complications of celiac disease (CD). We assessed 71 CD patients for neurologic manifestations and presence of serum antibodies to neural antigens. Sixteen patients (22.5%) were found to have neurological deficits including headache, depression, entrapment syndromes, peripheral neuropathy, and epilepsy. Antibody reactivity to neural antigens was detected in 30\\/71 (42.2%) patients.
Chiara Briani; Gabriella Zara; Armin Alaedini; Francesca Grassivaro; Susanna Ruggero; Elisabetta Toffanin; Maria Paola Albergoni; Milena Luca; Bruno Giometto; Mario Ermani; Franca De Lazzari; Anna D'Odorico; Leontino Battistin
. We describe four patients with cerebrovascular complications from two unrelated Italian families with Anderson-Fabry disease.\\u000a Clinical examination, neuroimaging (MRI), biochemical and genetic analyses were carried out in all the patients. ?-Galactosidase\\u000a A activity was detected by fluorimetric assay and genetic analysis was performed by DNA sequencing. Family 1. A male patient presented recurrent strokes when he was 34 years
W. Borsini; G. Giuliacci; F. Torricelli; E. Pelo; F. Martinelli; M. R. Scordo
Many patients with chronic kidney disease (CKD) receive care from primary care physicians. Identification and management of CKD complications in primary care is suboptimal. It is not known if current residency curriculum adequately prepares a future internist in this aspect of CKD care. We performed an online questionnaire survey of internal medicine residents in the United States to determine knowledge of CKD complications and their management. Four hundred seventy-nine residents completed the survey with postgraduate year (PGY) distribution 166 PGY1, 187 PGY2, and 126 PGY3. Most of the residents correctly recognized anemia (91%) and bone disease (82%) as complications at estimated glomerular filtration rate less than 60 mL/min/1.73 m; however, only half of the residents identified coronary artery disease (54%) as a CKD complication. For a patient with estimated glomerular filtration rate less than 60 mL/min/1.73 m, two thirds of the residents would workup for anemia (62%), whereas half of them would check for mineral and bone disorder (56%). With regard to anemia of CKD, less than half of the residents knew the CKD goal hemoglobin level of 11 to 12 g/dL (44%); most would supplement iron stores (86%), whereas fewer would consider nephrology referral (28%). For mineral and bone disorders, many residents would recommend dietary phosphorus restriction (68%) and check 25-hydroxyvitamin D (62%); fewer residents would start 1,25-dihydroxyvitamin D (40%) or refer to the nephrologist (45%). Residents chose to discontinue angiotensin-converting enzyme inhibitor for medication-related complication of greater than 50% decline in estimated glomerular filtration rate (68%) and potassium greater than 5.5 mEq/L (93%). Mean performance score improved with increasing PGY (PGY1 59.4% ± 17.6%, PGY2 63.6% ± 15.6%, and PGY3 66.2% ± 16.5%; P = 0.002). Our study identified specific gaps in knowledge of CKD complications and management among internal medicine residents. Educational efforts such as instruction on use of CKD clinical practice guidelines may help raise awareness of CKD complications, benefits of early intervention, and improve CKD management. PMID:19918169
Agrawal, Varun; Agarwal, Mohit; Ghosh, Amit K; Barnes, Michael A; McCullough, Peter A
Purpose To investigate clinical implications of delta neutrophil index (DNI) to discriminate adult onset Still's disease (AOSD) from sepsis. Materials and Methods We reviewed the medical records of 13 patients with AOSD and 33 gender and age-matched patients with sepsis. In all subjects, microbial tests were performed to exclude or confirm sepsis. All laboratory data were measured two or three times during the first 3 days and represented by their mean levels. DNI was measured automatically by ADVIA 2120 for the first 3 days. Results There were no significant differences in white blood cell counts, neutrophil proportion, erythrocyte sedimentation rate and C-reactive protein between two groups. AOSD patients had notably lower DNI than sepsis patients regardless of the presence of bacteremia or not. However, both DNI and ferritin were not significant independent factors for predicting sepsis in the multivariate logistic regression analysis. Meanwhile, the area under the receiver operating characteristic curve (AUROC) of DNI was slightly higher than that of ferritin. When we set DNI of 2.75% as the cut-off value for predicting sepsis, 11 (84.6%) of AOSD patients had a DNI value below 2.75% and 2 (15.4%) of them had a DNI over 2.75% (relative risk for sepsis 176). Conclusion We suggest that DNI may be a useful marker for differential diagnosis of AOSD from sepsis in the early phase as supplementary to ferritin.
Park, Hee-Jin; Ha, You-Jung; Pyo, Jung-Yoon; Park, Yong-Beom; Lee, Soo-Kon
BackgroundWe evaluated the association between a low ankle-brachial index (ABI), chronic complications of diabetes, and the presence of traditional cardiovascular disease risk factors in subjects with type 2 diabetes but without known cardiovascular disease.
Jose M Mostaza; Carmen Suarez; Luis Manzano; Marc Cairols; Fernando López-Fernández; Isabel Aguilar; Fernando Diz Lois; Juan L. Sampedro; Herminia Sánchez-Huelva; Miguel A. Sanchez-Zamorano
Background Clinical features, complications and treatments of Gaucher’s disease (GD), a rare autosomal–recessive disorder due to a confirmed lysosomal enzyme (glucocerebrosidase) deficiency, are described. Methods All patients with known GD, living in France, with ?1 consultations (1980–2010), were included in the French GD registry, yielding the following 4 groups: the entire cohort, with clinical description; and its subgroups: patients with ?1 follow-up visits, to investigate complications; recently followed (2009–2010) patients; and patients treated during 2009–2010, to examine complications before and during treatment. Data are expressed as medians (range) for continuous variables and numbers (%) for categorical variables. Results Among the 562 registry patients, 265 (49.6%) were females; 454 (85.0%) had type 1, 22 (4.1%) type 2, 37 (6.9%) perinatal–lethal type and 21 (3.9%) type 3. Median ages at first GD symptoms and diagnosis, respectively, were 15 (0–77) and 22 (0–84) years for all types. The first symptom diagnosing GD was splenomegaly and/or thrombocytopenia (37.6% and 26.3%, respectively). Bone-marrow aspiration and/or biopsy yielded the diagnosis for 54.7% of the patients, with enzyme deficiency confirming GD for all patients. Birth incidence rate was estimated at 1/50,000 and prevalence at 1/136,000. For the 378 followed patients, median follow-up was 16.2 (0.1–67.6) years. Major clinical complications were bone events (BE; avascular necrosis, bone infarct or pathological fracture) for 109 patients, splenectomy for 104, and Parkinson’s disease for 14; 38 patients died (neurological complications for 15 type-2 and 3 type-3 patients, GD complications for 11 type-1 and another disease for 9 type-1 patients). Forty-six had monoclonal gammopathy. Among 283 recently followed patients, 36 were untreated and 247 had been treated during 2009–2010; 216 patients received treatment in December 2010 (126 with imiglucerase, 45 velaglucerase, 24 taliglucerase, 21 miglustat). BE occurred before (130 in 67 patients) and under treatment (60 in 41 patients) with respective estimated frequencies (95% CI) of first BE at 10 years of 20.3% (14.1%–26.5%) and 19.8% (13.5%–26.1%). Conclusion This registry enabled the epidemiological description of GD in France and showed that BE occur even during treatment.
Introduction While Dupuytren's disease can cause disabling contractures requiring open surgery, a less-invasive option using Clostridium Histolyticum collagenase (CHC) via percutaneous injection was recently reported. A recent prospective, randomized trial demonstrated few complications during 90 days follow-up, however did not assess any longer term follow-up for these patients. Long-term outcomes in this setting have not been adequately reported, and the current manuscript aims to identify late complications from the clinical use of percutaneous CHC. Methods The current manuscript reports an extended 12-month follow-up for a cohort of twelve of patients enrolled in the original prospective, randomized trial, treated at a single institution. An analysis of complications requiring surgical intervention was undertaken. Results Two of twelve patients reported debilitating pain and triggering requiring surgical intervention. Extensive deep-tissue scarring and adhesions were identified, providing the first visual and qualitative analysis of the pathologic effects of CHC. Conclusion Late complications from CHC use can and have occurred, outside the follow-up period of the initial phase III trials. Longer term follow-up of such patients is thus essential, and further investigation and characterization of the late effects of CHC use is warranted.
Rozen, Warren M.; Edirisinghe, Yasith; Crock, John
We report on a small bowel transplant patient, donor/recipient seropositive (D+/R+) for cytomegalovirus (CMV), with a clinical course complicated by CMV disease. Anti-CMV prophylaxis was given for 100 days. Immunosuppression consisted of alemtuzumab, tacrolimus, mycophenolate mofetil and prednisolone. Five months posttransplant, CMV tissue invasive disease of the upper gastrointestinal tract was evident without the presence of viremia, tested by quantitative polymerase chain reaction (PCR). Complete viral load suppression was achieved with intravenous ganciclovir, followed by valganciclovir for secondary prophylaxis. Mycophenolate mofetil and prednisolone were discontinued. Shortly thereafter the patient presented with recurrent CMV and candida esophagitis. While on ganciclovir and caspofungin, the patient developed CMV tissue invasive disease of the ileal graft, with persistent absence of viremia. Foscarnet and CMV immunoglobulin were added. Viral load declined to undetectable levels; however, clinical improvement did not occur due to occurrence of graft rejection. Despite infliximab and high dose prednisolone, graft rejection was progressive, requiring surgical explantation of the graft. This case highlights the importance of additional diagnostic tools such as endoscopy including PCR analysis of tissue samples. Extension of primary antiviral prophylaxis interval up to 6 months and prolonged retreatment for recurrent CMV disease may be useful to avoid severe CMV-related complications. PMID:24703746
Avsar, Yesim; Cicinnati, Vito R; Kabar, Iyad; Wolters, Heiner; Anthoni, Christoph; Schmidt, Hartmut H J; Beckebaum, Susanne
Leptin is a small peptide hormone (16 kDa), a product of the obesity gene (Ob), and is mainly synthesized and secreted by adipocytes. It is removed from the blood by the kidneys. The kidney is not only a site of leptin clearance, but also a target organ for its action in different pathophysiological states. Several studies have documented a strong relationship between chronic kidney disease (CKD) and accelerated cardiovascular disease (CVD) defined as a cardiorenal syndrome. Patients with stage 3 and 4 CKD develop cardiovascular complications and are at increased risk of death from CVD. Renal dysfunction promotes several mechanisms responsible for exacerbation of cardiovascular disease. These include activation of the renin-angiotensin system, oxidative stress, elevated asymmetric dimethylarginine (ADMA), low-grade inflammation with increased circulating cytokines, and dyslipidemia. Recently, it has been observed that plasma leptin level is elevated in patients with cardiorenal syndrome. In obesity, hyperleptinemia combined with selective leptin resistance appear to have a critical role in the development and progression of kidney disease, CVD and metabolic syndrome. This has clinical implications for the treatment of obesity-related hypertension and kidney disease. In this paper the role of leptin in chronic kidney disease and accelerated cardiovascular disease is out lined. The link between hyperleptinemia and development and progression of morphologic changes that effect kidney in obese patients is also discussed. PMID:23688007
Korolczuk, Agnieszka; Dudka, Jaroslaw
Complications of the central nervous system (CNS) are common in systemic autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus (SLE) and primary Sjögren's syndrome. Specific diagnostic tests are lacking and early intervention with immunosuppressive therapy is frequently necessary. Therefore knowledge of these CNS complications is essential for early diagnosis and treatment. Residual cognitive effects were observed in some but not in all tests after prolonged heavy cannabis use. The effects were mostly mild. The relationship of cannabis use, psychotic effects and schizophrenia was unclear; the cannabis conceivably gave relief, but it also appeared that cannabis caused schizophrenia in young people and (or) enhanced the symptoms, especially in young people poorly able to cope with stress or in whom the antipsychotic therapy was unsuccessful. PMID:9623096
Markusse, H M; van den Bent, M J; Vecht, C J
Rituximab (RTX) is a chimeric anti-CD20 antibody, approved for rheumatoid arthritis (RA) patients who failed anti-Tumor Necrosis Factor therapy. It has been used occasionally for life-threatening autoimmune diseases (AID). We report our center experience in the use of RTX in life-threatening complications or refractory AID. Clinical charts of patients treated with RTX at our center were reviewed, cases treated for life-threatening complications or refractory AID were analyzed. Acute damage to vital organs such as lung, heart, kidney, nervous system with severe functional impairment were defined as life-threatening complications; treatment failure with high-dose corticosteroids, cyclophosphamide, IVIG, plasmapheresis was defined as refractory autoimmune disease. During the years 2003-2009, 117 patients were treated with RTX, most of them for RA. Nine patients (6 females, mean age 51.5 years, mean disease duration 6.3 years) answered the criteria. The indications were as follows: pulmonary hemorrhage (1 patient with cryoglobulinemic vasculitis, 1 with systemic sclerosis, 1 with ANCA-associated vasculitis), catastrophic anti-phospholipid syndrome (2 SLE patients), non-bacterial endocarditis and pulmonary hypertension (1 patient with mixed connective tissue disease), vasculitis and feet necrosis (1 patient with systemic lupus erythematosus), severe lupus demyelinative neuropathy and acute renal failure (1 patient), and severe rheumatoid lung disease with recurrent empyema and pneumothorax (1 patient). B cell depletion was achieved in all patients. The median time since starting of complications to RTX administration was 3 weeks (range 2-15 weeks). Complete remission (suppression of the hazardous situation and return to previous stable state) was seen in 7 out of 9 patients. Partial remission (significant improvement) was achieved in the remained. The median time to response was 3 weeks (range 1-8 weeks), mean follow-up 47.2 months (range 6-60 months). A rapid tapering off of steroids was achieved in all patients. Two patients relapsed and were successfully retreated with RTX: the patient with severe RA lung relapsed after 3 years, one of the patients with ANCA-associated pulmonary alveolar hemorrhage relapsed after 10 months. There were no side effects during RTX infusion. Two episodes of serious infections were registered: fatal Gram-negative sepsis 6 months after RTX treatment, and septic discitis 4 months after receiving RTX. RTX serves as a safe, efficient, and prompt rescue therapy in certain life-threatening conditions and resistant to aggressive immunosuppression AID. RTX when administrated at an earlier stage, prevented irreversible vital organ damage, and allowed rapid steroid tapering off in already severe immunodepressed patients. PMID:23239037
Braun-Moscovici, Yolanda; Butbul-Aviel, Yonatan; Guralnik, Ludmila; Toledano, Kochava; Markovits, Doron; Rozin, Alexander; Nahir, Menahem A; Balbir-Gurman, Alexandra
BACKGROUND/AIMS—Proper management of enterocutaneous fistulas complicating Crohn's disease largely depends on the anatomical characteristics of the sinus tracks as well as the coexistence of complications such as abscesses and distal bowel stenosis. The aim of this prospective study was to evaluate the accuracy of a new technique (hydrogen peroxide enhanced ultrasound (US)-fistulography) compared with conventional x ray fistulogram and/or surgical findings in the detection of Crohn's disease associated enterocutaneous fistulas.?METHODS—Patients with known Crohn's disease and a suspicion of enterocutaneous fistulas were prospectively studied with this novel technique, conventional x ray fistulogram, and barium radiography as well as with computed tomography whenever an abdominal abscess was suspected at US. In those undergoing surgery, intraoperative findings were also compared.?RESULTS—Seventeen of 502 (3.4%) consecutive patients with Crohn's disease seen over a ten month period had associated enterocutaneous fistulas and were enrolled. Hydrogen peroxide enhanced US-fistulography visualised the extent and configuration of fistula in all cases: 13 patients had a fistula arising from the ileum and two from the sigmoid colon, whereas in two there was no evidence of communication with intestinal loops; in contrast, conventional x ray fistulography missed a correct definition of the fistulous branches or communication with intestinal loops in 50% (4/8) and 36% (4/11) of patients respectively; barium radiography showed fistulas in two cases only. The presence of abscesses along or close to the sinus track, as well as the coexistence of intestinal stenosis, was correctly detected at US in all patients.?CONCLUSIONS—Hydrogen peroxide enhanced US-fistulography could be considered the diagnostic procedure of choice in Crohn's disease associated enterocutaneous fistulas, as it is at least as accurate, simple, and safe as conventional x ray fistulogram, does not miss coexisting abdominal complications, and also provides information on the diseased bowel segments. In addition, it can be easily repeated over time in order to monitor the course of fistulas undergoing conservative treatment.???Keywords: enterocutaneous fistulas; Crohn's disease; x ray fistulogram; hydrogen peroxide enhanced US-fistulography
Maconi, G; Parente, F; Porro, G
Patients who suffer from Crohn’s disease are prone not only to the complications of a relapsing, unpredictable disease, but also to feelings of stigmatization; depression; and increased risk of suicidal ideation, suicide, or drug and alcohol abuse. Cases performed at the Jackson County Medical Examiner’s Office from 2008 to 2010 were reviewed. Autopsy findings, investigator reports, toxicology results, medical records, and interviews with survivors were analyzed. Twelve cases of Crohn’s disease were recovered. In 10 of these cases, inflammatory bowel disease was not the cause of death. Instead, psychosocial consequences of the disease had significant implications in the deaths. The mean age of decedents was 45 years, with a female predominance. In eight cases, the decedents lived alone. Five patients had issues of acute or chronic drug or alcohol use. Five patients committed suicide. These cases underscore the role of psychosocial factors that can contribute significantly to the cause of death in patients with Crohn’s disease. PMID:24749148
Carson, Henry J; Dudley, Mary H; Knight, Laura D; Lingamfelter, Daniel
Radiotracer imaging (RTI) techniques such as positron emission tomography (PET) allow the in vivo assessment of nigrostriatal DA function in Parkinson's disease and have provided valuable insights into the mechanisms of nigrostriatal degeneration and the consequent compensatory changes. Moreover, functional imaging serves as an excellent tool in the assessment of the progression of PD and the evolution of treatment-related complications. However, various studies have shown discordance between clinical progression of PD and nigrostriatal degeneration estimated by PET or SPECT, and no RTI technique can be reliably used as a biomarker for progression of PD. Presynaptic dopaminergic imaging has consistently demonstrated an anterior-posterior gradient of dopaminergic dysfunction predominantly affecting the putamen, with side-to-side asymmetry in tracer binding. Dopaminergic hypofunction in the striatum follows a negative exponential pattern with the fastest rate of decline in early disease. Evaluation of central pharmacokinetics of levodopa action by PET has demonstrated the role of increased synaptic dopamine turnover and downregulation of the dopamine transporter in the pathophysiology of levodopa-induced dyskinesias. In PD with behavioral complications such as impulse control disorders, increased levels of dopamine release have been observed in the ventral striatum during performance of a positive reward task, as well as loss of deactivation in orbitofrontal cortex in response to negative reward prediction errors. This suggests that there is a pathologically heightened "reward" response in the ventral striatum together with loss of the capacity to respond to negative outcomes. Overall, functional imaging with PET is an excellent tool for understanding the disease and its complications; however, caution must be applied in interpretation of the results. PMID:20887875
Kuriakose, Renju; Stoessl, A Jon
Levodopa is the most effective treatment for Parkinson's disease (PD) signs and symptoms, and patients invariably will require it during the course of the disease. It also provides benefits in activities of daily living, quality of life and life expectancy. However, after a few years of levodopa treatment the majority of patients will experience motor fluctuations and dyskinesia. Initial use of a dopamine receptor agonist may delay the emergence of motor fluctuations but at the cost of reduced symptomatic control compared with the use of levodopa in some cases. Adequate management of motor fluctuations and dyskinesia is essential to maintaining satisfactory quality of life at the advanced stage of disease. Various levodopa-based strategies are currently available that aim to control motor complications (wearing-off and dyskinesia) in PD and each approach has its own unique benefit and risk profile. Strategies such as dose fragmentation (smaller, more frequent dosing) or the use of orally administered, liquid levodopa formulations or melevodopa can reduce off-time intervals or facilitate absorption. More recently introduced, continuous delivery of dopaminergic medications may represent a more effective approach to treat motor complications in advanced PD and its effect can be perceived from improvement in clinical scales, as well as in health-related items. Indeed, continuous levodopa delivery by duodenal infusion may stabilize and significantly improve motor function as well as patients' quality of life. We propose a treatment algorithm that takes into account all currently available levodopa-based treatment strategies for motor complications in patients with PD. PMID:20088619
Antonini, Angelo; Chaudhuri, K Ray; Martinez-Martin, Pablo; Odin, Per
Background Germinal center kinase-like kinase (GLK, also termed MAP4K3), a member of the MAP4K family, may regulate gene transcription, apoptosis and immune inflammation in response to extracellular signals. The enhanced expression of GLK has been shown to correspond with disease severity in patients with systemic lupus erythematosus. We investigated the role of GLK in the pathogenesis of adult-onset Still's disease, which shares some similar clinical characteristics with systemic lupus erythematosus. Methods The frequencies of circulating GLK-expressing T-cells in 24 patients with active adult-onset Still's disease and 12 healthy controls were determined by flow cytometry analysis. The expression levels of GLK proteins and transcripts were evaluated in peripheral blood mononuclear cells by immunoblotting and quantitative PCR. Serum levels of T helper (Th)17-related cytokines, including IL-1?, IL-6, IL-17 and TNF-?, were measured by ELISA. Results Significantly higher median frequencies of circulating GLK-expressing T-cells were observed in patients with adult-onset Still's disease (31.85%) than in healthy volunteers (8.93%, P <0.001). The relative expression levels of GLK proteins and transcripts were also significantly higher in patients with adult-onset Still's disease (median, 1.74 and 2.35, respectively) compared with those in healthy controls (0.66 and 0.92, respectively, both P <0.001). The disease activity scores were positively correlated with the frequencies of circulating GLK-expressing T-cells (r = 0.599, P <0.005) and the levels of GLK proteins (r = 0.435, P <0.05) or GLK transcripts (r = 0.452, P <0.05) in patients with adult-onset Still's disease. Among the examined Th17-related cytokines, elevated levels of serum IL-6 and IL-17 were positively correlated with the frequencies of circulating GLK-expressing T-cells and the levels of GLK proteins as well as transcripts in patients with adult-onset Still's disease. GLK expression levels decreased significantly after effective therapy in these patients. Conclusions Elevated expression levels of GLK and their positive correlation with disease activity in patients with adult-onset Still's disease indicate that GLK may be involved in the pathogenesis and act as a novel activity biomarker of this disease.
Microvillus inclusion disease (MVID), a rare severe congenital enteropathy characterized by intracytoplasmic microvillous inclusions and variable brush border atrophy on intestinal epithelial cells histology, is associated with defective synthesis or abnormal function of the motor protein myosin Vb encoded by the MYO5B gene. Although MYO5B gene is expressed in all epithelial tissues, it is unclear so far whether organs other than intestine are affected in MVID patients. We report a case of an infant with MVID who presented liver dysfunction, hematuria, and Pneumocystis jiroveci pneumonia during the course of the disease. It is discussed whether extraintestinal manifestations in this patient are secondary consequences of MVID or might be features of the disease associated with altered MYO5B function. Conclusions: MVID is classically included in the differential diagnosis of congenital diarrhea of secretory type. Recent advances in our knowledge regarding the role of myosin Vb in the pathophysiology of MVID is expected to clarify the clinical spectrum of the disease and the possible primary involvement of organs other than intestine. PMID:23354788
Siahanidou, Tania; Koutsounaki, Eirini; Skiathitou, Anna-Venetia; Stefanaki, Kalliopi; Marinos, Evangelos; Panajiotou, Ioanna; Chouliaras, Giorgos
Humoral immune mechanisms may have a role in the neurological complications of celiac disease (CD). We assessed 71 CD patients for neurologic manifestations and presence of serum antibodies to neural antigens. Sixteen patients (22.5%) were found to have neurological deficits including headache, depression, entrapment syndromes, peripheral neuropathy, and epilepsy. Antibody reactivity to neural antigens was detected in 30/71 (42.2%) patients. There was no clear correlation between anti-neural reactivity and neurologic dysfunction. Follow-up of 62 patients did not reveal change in electrophysiology or antibodies, regardless of diet. However, in 2 patients with neuropathy, symptoms improved or worsened depending on the diet. PMID:18343508
Briani, Chiara; Zara, Gabriella; Alaedini, Armin; Grassivaro, Francesca; Ruggero, Susanna; Toffanin, Elisabetta; Albergoni, Maria Paola; Luca, Milena; Giometto, Bruno; Ermani, Mario; De Lazzari, Franca; D'Odorico, Anna; Battistin, Leontino
This paper describes a case of a patient suffering from Hodgkin's disease and treated by means of minimally invasive surgery: laparoscopic splenectomy. The performance of laparoscopic splenectomy led to a complication in the form of a pancreatic cyst. The cyst was subjected to endoscopic procedures (endoscopic retrograde cholangiopancreatography, with a shaft to the pancreatic tract) and percutaneous drainage. The application of the above methods allowed for a quick introduction of causative treatment. Because in the case in question open surgery methods would impede the introduction of systemic treatment, minimally invasive surgery techniques (laparoscopy and endoscopy) were applied and are discussed below. PMID:24501607
Krasowski, Grzegorz; Budzynski, Andrzej; Miodo?ski, Maciej; Seweryn-Serkis, Katarzyna; Janus, Werner
This paper describes a case of a patient suffering from Hodgkin's disease and treated by means of minimally invasive surgery: laparoscopic splenectomy. The performance of laparoscopic splenectomy led to a complication in the form of a pancreatic cyst. The cyst was subjected to endoscopic procedures (endoscopic retrograde cholangiopancreatography, with a shaft to the pancreatic tract) and percutaneous drainage. The application of the above methods allowed for a quick introduction of causative treatment. Because in the case in question open surgery methods would impede the introduction of systemic treatment, minimally invasive surgery techniques (laparoscopy and endoscopy) were applied and are discussed below.
Budzynski, Andrzej; Miodonski, Maciej; Seweryn-Serkis, Katarzyna; Janus, Werner
The treatment of peritonitis complicating diverticular disease of the colon is yet to be universally regarded as established practice and major differences in management are to be noted in the various surgical institutions. In the emergency setting, the minimally invasive approach is used by few surgeons and the most frequent therapeutic options are sigmoid resection with primary anastomosis (with or without a diverting stoma) and Hartmann's procedure. The Authors report their preliminary experience (13 cases) with laparoscopic lavage and drainage without colonic resection in diverticulitis complicated by peritonitis and describe the technical details of the surgical procedure. They conduct a systematic review of the literature and, on the basis of their latest experience, compare the results of the traditional resective operations (resection with primary anastomosis and Hartmann's procedure) with those of laparoscopic conservative and non-resective treatment. Laparoscopic non-resective procedures reduce the frequency and severity of the surgical complications, as well as the hospital stay and costs of treatment. In conclusion, laparoscopic lavage and drainage can be used in the majority of patients, with excellent prospects of recovery, without disabling stomas, in a single operative stage. PMID:19845268
Lippi, Carlo Ettore; Beatini, Luca; Cervia, Silvio; Fabbricotti, Alaido; Miaruelli, Piero Antonio; Spessa, Elisabetta; Sturlese, Ivarco; Braini, Andrea
The burden of diseases caused by food-borne pathogens remains largely unknown. Importantly data indicating trends in food-borne infectious intestinal disease is limited to a few industrialised countries, and even fewer pathogens. It has been predicted that the importance of diarrhoeal disease, mainly due to contaminated food and water, as a cause of death will decline worldwide. Evidence for such a
Diane G. Newell; Marion Koopmans; Linda Verhoef; Erwin Duizer; Awa Aidara-Kane; Hein Sprong; Marieke Opsteegh; Merel Langelaar; John Threfall; Flemming Scheutz; Joke van der Giessen; Hilde Kruse
Simple renal cysts are the most common renal masses, accounting for roughly 65 to 70% of cases. They most often occur in patients over the age of 50 as determined from post-mortem examination or renal ultrasonography. The major concern with simple renal cysts is differentiating them from more serious disorders, such as polycystic kidney disease and solid masses such as a renal carcinoma or abscess. Renal arteriovenous malformations may present with ultrasound picture mimicking simple parapelvic cyst. Ultrasound, doppler ultrasound, computed tomography and magnetic resonance imaging are effective in documenting the underlying lesions non-invasively. Arteriography may be useful to characterise vascular lesion. We report here the spectrum of cystic kidney disease in adulthood in a group of patient with different disorders. The differential diagnosis, complications and associated process are discussed. PMID:12891942
Peces, R; Costero, O
A 24-year-old Chinese woman with Graves' disease presented with myositis two months after treatment with carbimazole. The patient's myositis resolved with hydration and cessation of carbimazole. No other causes of myositis were found, and a change in the medication to propylthiouracil was uneventful. Review of the literature suggests a possible genetic susceptibility, as the majority of reported cases are Asian in origin, similar to patients who present with thyroid periodic paralysis. Changing the antithyroid drugs (ATDs) administered, decreasing the dose of pre-existing ATDs in the treatment regimen or addition of levothyroxine has been shown to result in clinical improvement of this complication. These observations suggest various mechanisms of carbimazole-induced myositis in the treatment of Graves' disease, including the direct effect of ATDs on myocytes, immune-related responses secondary to ATDs and rapid decrements in thyroid hormone with ensuing myositis. PMID:23900475
Lim, Adoree Yi Ying; Kek, Peng Chin; Soh, Abel Wah Ek
Opportunistic viral infections are a well-recognized complication of anti-tumor necrosis factor (TNF) therapy for inflammatory bowel disease (IBD). Cases of severe or atypical varicella zoster virus infection, both primary and latent reactivation, have been described in association with immunosuppression of Crohn's disease (CD) patients. However, central nervous system varicella zoster virus infections have been rarely described, and there are no previous reports of varicella zoster virus meningitis associated with anti-TNF therapy among the CD population. Here, we present the case of a 40-year-old male with severe ileocecal-CD who developed a reactivation of dermatomal herpes zoster after treatment with prednisone and adalimumab. The reactivation presented as debilitating varicella zoster virus meningitis, which was not completely resolved despite aggressive antiviral therapy with prolonged intravenous acyclovir and subsequent oral valacyclovir. This is the first reported case of opportunistic central nervous system varicella zoster infection complicating anti-TNF therapy in the CD population. This paper also reviews the literature on varicella zoster virus infections of immunosuppressed IBD patients and the importance of vaccination prior to initiation of anti-TNF therapy. PMID:23745038
Ma, Christopher; Walters, Brennan; Fedorak, Richard N
Opportunistic viral infections are a well-recognized complication of anti-tumor necrosis factor (TNF) therapy for inflammatory bowel disease (IBD). Cases of severe or atypical varicella zoster virus infection, both primary and latent reactivation, have been described in association with immunosuppression of Crohn’s disease (CD) patients. However, central nervous system varicella zoster virus infections have been rarely described, and there are no previous reports of varicella zoster virus meningitis associated with anti-TNF therapy among the CD population. Here, we present the case of a 40-year-old male with severe ileocecal-CD who developed a reactivation of dermatomal herpes zoster after treatment with prednisone and adalimumab. The reactivation presented as debilitating varicella zoster virus meningitis, which was not completely resolved despite aggressive antiviral therapy with prolonged intravenous acyclovir and subsequent oral valacyclovir. This is the first reported case of opportunistic central nervous system varicella zoster infection complicating anti-TNF therapy in the CD population. This paper also reviews the literature on varicella zoster virus infections of immunosuppressed IBD patients and the importance of vaccination prior to initiation of anti-TNF therapy.
Ma, Christopher; Walters, Brennan; Fedorak, Richard N
Angiogenesis is believed to be implicated in the pathogenesis of alcoholic liver disease (ALD). We aimed to explore the usefulness and accuracy of plasma angiogenic biomarkers for noninvasive evaluation of the severity of liver failure and ALD outcome. One hundred and forty-seven patients with ALD were prospectively enrolled and assessed based on their (1) gender, (2) age, (3) severity of liver dysfunction according to the Child-Turcotte-Pugh and MELD scores, and (4) the presence of ALD complications. Plasma levels of vascular endothelial growth factor (VEGF-A) and angiopoietins 1 and 2 (Ang1 and Ang2) were investigated using ELISAs. Multivariable logistic regression was applied in order to select independent predictors of advanced liver dysfunction and the disease complications. Significantly higher concentrations of Ang2 and VEGF-A in ALD patients as compared to controls were found. There was no difference in Ang1 levels in both groups. A positive correlation of Ang2 levels with INR (Rho 0.66; P < 0.0001) and its inverse correlation with plasma albumin levels (Rho –0.62; P < 0.0001) were found. High Ang2 concentrations turned out to be an independent predictor of severe liver dysfunction, as well as hepatic encephalopathy and renal impairment. Ang2 possessed the highest diagnostic and prognostic potential among three studied angiogenesis-related molecules.
We report a 31-year-old woman with Crohn’s disease complicated by multiple stenoses and internal fistulas clinically misdiagnosed as small bowell endometriosis, due to the patient’s perimenstrual symptoms of mechanical subileus for 3 years; at first monthly, but later continuous, and gradually increasing in severity. We performed an exploratory laparotomy for small bowel obstruction, and found multiple ileal strictures and internal enteric fistulas. Because intraoperative findings were thought to indicate Crohn’s disease, a right hemicolectomy and partial distal ileum resection were performed for obstructive Crohn’s ileitis. Histopathology of the resected specimen revealed Crohn’s disease without endometrial tissue. The patient made an uneventful recovery from this procedure and was discharged home 10 d post-operatively. The differential diagnosis of Crohn’s diease with intestinal endometriosis may be difficult pre-operatively. The two entities share many overlapping clinical, radiological and pathological features. Nevertheless, when it is difficult to identify the cause of intestinal obstruction in a woman of child-bearing age with cyclical symptoms suggestive of small bowel endometriosis, Crohn’s disease should be included in the differential diagnosis.
Teke, Zafer; Aytekin, Faruk Onder; Atalay, Ali Ozgur; Demirkan, Nese Calli
The Multi-Societal Database Committee for Pediatric and Congenital Heart Disease was established in 2005 with the goal of providing the infrastructure, spanning geographical and subspecialty boundaries, for collaboration between health care professionals interested in the analysis of outcomes of treatments provided to patients with congenital cardiac disease, with the ultimate aim of improvement in the quality of care provided to these patients. The purpose of these collaborative efforts is to promote the highest quality comprehensive cardiac care to all patients with congenital heart disease, from the fetus to the adult, regardless of the patient's economic means, with an emphasis on excellence in teaching, research and community service. This manuscript provides the Introduction to the 2008 Supplement to Cardiology in the Young titled: "Databases and The Assessment of Complications associated with the Treatment of Patients with Congenital Cardiac Disease". This Supplement was prepared by The Multi-Societal Database Committee for Pediatric and Congenital Heart Disease. The Multi-Societal Database Committee for Pediatric and Congenital Heart Disease offers the following definition of the term "Complication": "A complication is an event or occurrence that is associated with a disease or a healthcare intervention, is a departure from the desired course of events, and may cause, or be associated with, suboptimal outcome. A complication does not necessarily represent a breech in the standard of care that constitutes medical negligence or medical malpractice. An operative or procedural complication is any complication, regardless of cause, occurring (1) within 30 days after surgery or intervention in or out of the hospital, or (2) after 30 days during the same hospitalization subsequent to the operation or intervention. Operative and procedural complications include both intraoperative/intraprocedural complications and postoperative/postprocedural complications in this time interval." The Multi-Societal Database Committee for Pediatric and Congenital Heart Disease offers the following definition of the term "Adverse Event": "An adverse event is a complication that is associated with a healthcare intervention and is associated with suboptimal outcome. Adverse events represent a subset of complications. Not all medical errors result in an adverse event; the administration of an incorrect dose of a medication is a medical error, but it does not always result in an adverse event. Similarly, not all adverse events are the result of medical error. A child may develop pneumonia after an atrial septal defect repair despite intra- and peri-operative management that is free of error. Complications of the underlying disease state, which are not related to a medical intervention, are not adverse events. For example, a patient who presents for medical care with metastatic lung cancer has already developed a complication (Metastatic spread) of the primary lung cancer without any healthcare intervention. Furthermore, complications not associated with suboptimal outcome or harm are not adverse events and are known as no harm events. The patient who receives an incorrect dose of a medication without harm has experienced a no harm event, but not an adverse event." Based on the above definitions, it is apparent that The Multi-Societal Database Committee for Pediatric and Congenital Heart Disease has taken an inclusive approach to defining the universe of complications. Complications may or may not be associated with healthcare intervention and may or may not be associated with suboptimal outcome. Meanwhile, adverse events must be associated with healthcare intervention and must be associated with suboptimal outcome. PMID:19063774
Jacobs, Jeffrey P
The hematopoietic colony-stimulating factors have been introduced into clinical practice as additional supportive measures that can reduce, but not eliminate infectious complications associated with therapy-induced neutropenia. Over the past decade, we have begun to appreciate the subtler aspects of the proper use of G-CSF and GM-CSF, identifying appropriate indications and contraindications. In the course of evaluating the multitude of studies, a set of formal recommendations have been propagated for the judicious use of these expensive growth factors. To prevent serious infection, the use of G- or GM-CSF is recommended in a subset of pediatric cancer patients shortly after receiving chemotherapy or a marrow transplant. Children with intensive chemotherapy (e.g., children with high risk ALL, NHL or metastatic neuroblastoma) seem to benefit from hematopoietic growth factors whereas it is not clear that this applies to children undergoing therapy for solid tumors such as rhabdomyosarcoma or Ewing's sarcoma. An exciting development is the use of G-CSF and GM-CSF to mobilize peripheral-blood progenitor cells. Future studies in pediatric cancer patients are clearly warranted to address several issues. Prospective clinical trials are still needed to define specific treatment groups who can benefit from growth factor support. In this regard, efforts must be directed at better defining the endpoints and in particular, assigning value to reduction in treatment of possible infectious complications, such as days in hospital, antibiotic usage and costs. In addition, randomized studies are required to evaluate the proper dosage and duration of therapy, which most likely will vary between groups, depending upon underlying malignancy and therapy given. In addition, combinations of different growth factors have to be tested, particularly if ex vivo expansion and the storage of hematopoietic stem cells are to be utilized in a wider spectrum of patients. PMID:11577368
Introduction Fistula formation in patients with Crohn’s disease is a common complication during the course of the disease. Perianal and enteroenteric are the most common forms of fistulas, whereas the involvement of the upper gastrointestinal tract with gastrocolic and duodenocolic fistulas represents an extremely unusual condition. Moreover, hyperthyroidism in association with Crohn’s disease has been rarely described. Case presentation We present here a rare case of a 25-year-old male with simultaneous onset of hyperthyroidism and fistulizing Crohn’s disease. Crohn’s disease was complicated with intra-abdominal fistulas involving the upper gastrointestinal tract (duodenocolic, gastrocolic) and an intra-peritoneal abscess formation in the lesser sac. We describe the clinical presentation and therapeutic management of the patient including both medical treatment and surgical intervention. Despite intense medical treatment with total parenteral nutrition, antibiotics, aminosalicylates and corticosteroids the clinical course of the disease was suboptimal. Finally, the patient underwent laparotomy and right hemi-colectomy with ileo-transverse anastomosis performed, with simultaneous drainage of the abdominal abscess and primary closure of the upper gastrointestinal tract openings (gastric, duodenal and jejunal) at one stage operation. Although the surgical approach definitively cured the perforating complications of the disease (fistulas and abscess), the luminal disease in the colon remnant was still active and steroid-refractory. The subsequent successful treatment with infliximab, azathioprine and mesalazine resulted in the induction and maintenance of the disease remission. Thyrotoxicosis was successfully treated with methimazole and the hyperthyroidism has definitely subsided. Conclusion The management of intra-abdominal fistulas in Crohn’s disease is a complex issue, requiring a multi-disciplinary approach and ‘tailoring’ of the treatment to the individual patient’s needs. Probably, a sensible approach involves early surgical intervention with prior optimization of the patient’s general condition when feasible. Common autoimmune mechanisms are probably involved in thyroid dysfunction associated with Crohn’s disease. Moreover, diagnosis and treatment of coexisting thyroid disorder in patients with Crohn’s disease has a favorable impact in disease prognosis.
Pachiadakis, Ioannis; Nakos, Andreas; Tatsi, Presvia; Moschos, John; Milias, Stefanos; Nikolopoulos, Panagiotis; Balaris, Christos; Apostolidis, Dimosthenis
The most important extra-renal manifestation of autosomal dominant polycystic kidney disease (ADPKD) in terms of debilitating injury and premature death is the development of intracranial aneurysms (IAs) and other vascular complications, resulting in subarachnoid hemorrhage (SAH). IAs are found at a rate approximately five times higher in ADPKD patients than in the general population and in patients with a family history of SAH/IAs the frequency is elevated further three to five times, indicating the importance of genetic factors in its etiology. Expression of the ADPKD gene products, polycystin-1 (PKD1) and polycystin-2 (PKD2), in vascular smooth muscle and the endothelium, and evidence that reduced levels of these proteins leads to IA development in mouse models, suggests a direct role of these proteins in the vascular disease. PKD1 and PKD2 patients seem equally likely to develop IAs, while patients with mutations to the 5’ half of PKD1 may more likely have vascular complications. Genome wide association and candidate studies of multiplex families with IAs without ADPKD have identified a number of genes/proteins that may be risk factors for the development of IAs. These candidate proteins largely have roles in the maintenance and remodeling of the arterial wall of small brain arteries. The development of the genetic methodologies of massively parallel sequencing mean it is now possible to test these and other candidates in ADPKD families with multiplex and singleton IA cases. Identifying strong modifiers of this phenotype will be important for prioritizing patients for presymptomatic screening and interventions.
Rossetti, Sandro; Harris, Peter C.
BACKGROUND/OBJECTIVES Significant controversy exists as to the meaning of a low glomerular filtration rate (GFR) in the elderly. The goal of the study was to evaluate whether elderly patients with low GFR are at risk for anemia, hyperkalemia, acidosis, and hyperphosphatemia. DESIGN Retrospective study SETTING Veterans Affairs Medical Center PARTICIPANTS All patients over 65 years of age with chronic kidney disease (CKD) and a GFR between 15 and 60 mL/min/1.73m2. MEASUREMENTS Anemia was defined as a hemoglobin <10g/dL, hyperkalemia as a potassium >5.5mEq/L, acidosis as a bicarbonate <21mEq/L, and hyperphosphatemia as a phosphorus >4.6mg/dL. Multivariable logistic regression was used to evaluate whether age modifies the effect of low GFR on metabolic complications by including an interaction term between age and GFR in each model. RESULTS 13874 veterans were included in the study. The average age was 79, the average GFR was 46.5; 3.1% had anemia, 2.5% hyperkalemia, 2.3% acidosis, and 4.4% had hyperphosphatemia. Lower GFR was associated with increased rates of metabolic complications across all age groups (odds ratio per 5mL/min/1.73m2 decrease in GFR in multivariable models was 1.21 for anemia, 1.26 for hyperkalemia, 1.45 for acidosis, and 1.72 for hyperphosphatemia). There was no significant interaction between age and GFR in models including only age and GFR or in multivariable models (p values for the age X GFR interaction term: 0.66 for anemia, 0.19 for hyperkalemia, 0.54 for acidosis, and 0.22 for hyperphosphatemia). CONCLUSION Elderly patients with CKD are at risk for anemia, hyperkalemia, acidosis, and hyperphosphatemia; age does not modify the relationship between GFR and development of metabolic complications. Elderly patients with low GFR should be monitored for metabolic complications, regardless of age.
Drawz, Paul E.; Babineau, Denise C.; Rahman, Mahboob
For almost a century, epidemiologists have stratified age-specific disease rates by year of birth to better understand the distribution of a disease in a population and its evolution across time. In the present article, I review the contributions of John Brownlee, Kristian Feyer Andvord, and Wade Hampton Frost and, to accentuate the similarities of their approaches, redraw their original graphs of age-specific death rates of tuberculosis organized either by year of death or year of birth. In addition, this article reports on an apparently universally forgotten publication in the American Journal of Hygiene published in 1929, which both upsets the conventional history of the earliest reports of disease rates stratified by birth cohorts and challenges the theory that Frost discovered cohort analysis independently and gave it its name. PMID:24920785
Background: Coronary heart disease (CHD) is the leading cause of death in women aged over 40 years in the United States, for whom it conveys a worse prognosis than for men. Recently, psychosocial factors have been understood to represent significant risk factors for developing CHD, as well as having a worse outcome with established CHD. However, these factors are often
Bettina Bankier; Andrew B. Littman
Background: The objective of this study was to compare the performance of the Mini-Mental State Examination (MMSE) total score as well as item scores in separating 4 groups of elderly (55–85 years of age) subjects—normal controls, subjects with mild cognitive impairment (MCI), subjects with mild Alzheimer's disease, and subjects with depression. Method: The MMSE scores of 86 subjects (25 normal elderly controls, 26 subjects with MCI, 10 subjects with mild Alzheimer's disease, and 25 subjects with depression) were analyzed. Statistically significant differences between groups in both overall MMSE score and individual item scores were documented. Receiver operating characteristic curves were constructed to yield further data. Results: The overall MMSE scores of the mild Alzheimer's disease group were significantly below those of subjects in the control, MCI, and depression groups (p < .001). The overall MMSE scores of MCI subjects were significantly lower than those of control subjects (p = .005) but not different from those of subjects with depression. Furthermore, individual item responses were not significantly different between MCI subjects and controls. The delayed recall item scores were statistically lower in the mild Alzheimer's disease group versus the other 3 groups but did not separate the control, MCI, and depression groups from each other. Conclusion: The MMSE effectively separates those with mild Alzheimer's disease from the other 3 groups and MCI from normal aging, but it is relatively ineffective in separating normal elderly individuals from those with depression and individuals with MCI from those with depression. Measures other than the MMSE may need to be implemented to evaluate mental status to more effectively separate MCI from depression and depression from normal aging.
Benson, Aaron D.; Slavin, Melissa J.; Tran, Thanh-Thu; Petrella, Jeffrey R.; Doraiswamy, P. Murali
Background The presence of “mechanic’s hands” is one of the clinical clues for collagen vascular diseases. However, the exact relevance of “mechanic’s hands” in collagen vascular diseases has not been well documented. The aim of this study was to clarify the relevance of “mechanic’s hands” to collagen vascular diseases including various skin lesions and interstitial pneumonia. Methods A retrospective review of the medical records of patients with “mechanic’s hands” at our hospital between April 2011 and December 2012 was conducted. A PubMed search was also conducted using the term “mechanic’s hands”. Results Four patients in our institution and 40 patients obtained from PubMed who had “mechanic’s hands” were identified. The most frequent diseases were DM/amyopathic DM (n = 24, 54.5%) and anti-ARS syndrome (n = 17, 38.6%). In these patients, the major skin lesions associated with “mechanic’s hands” were periungual erythema (n = 23, 52.3%), Gottron’s sign (n = 17, 38.6%), heliotrope rash (n = 10, 22.7%), Raynaud’s phenomenon (n = 9, 20.5%), and anti-ARS syndrome (n = 17, 38.6%). Six cases (2 DM, 4 anti-ARS syndrome) had only “mechanic’s hands”. Antibodies to anti-ARS (n = 24) were Jo-1 (n = 19), PL-7 (n = 3), OJ (n = 1), and PL-12 (n = 1). Conclusion The presence of “mechanic’s hands” together with diverse skin lesions could be a clinical clue to the diagnosis of lung involvement associated with collagen vascular diseases, especially in anti-ARS syndrome or DM/amyopathic DM.
Autologous graft versus host disease (autoGVHD) is a rare transplant complication with significant morbidity and mortality. It has been hypothesized that patients with multiple myeloma might be predisposed to autoGVHD through dysregulation of the immune response resulting from either their disease, the immunomodulatory agents (IMiDs) used to treat it, or transplant conditioning regimen. Hematopoietic progenitor cell (HPC) products were available from 8 multiple myeloma patients with biopsy-proven autoGVHD, 16 matched multiple myeloma patients who did not develop autoGVHD, and 7 healthy research donors. The data on number of transplants prior to developing autoGVHD, mobilization regimens, exposure to proteasome inhibitors, use of IMiDs, and class I human leukocyte antigen types (HLA A and B) were collected. The HPC products were analyzed by flow cytometry for expression of CD3, CD4, CD8, CD25, CD56, and FoxP3. CD3+ cell number was significantly lower in autoGVHD patients compared to unaffected controls (P = 0.047). On subset analysis of CD3+ cells, CD8+ cells (but not CD4+ cells) were found to be significantly lower in patients with autoGVHD (P = 0.038). HLA-B55 expression was significantly associated with development of autoGVHD (P = 0.032). Lower percentages of CD3+ and CD8+ T-cells and HLA-B55 expression may be predisposing factors for developing autoGVHD in myeloma.
Batra, Anu; Cottler-Fox, Michele; Harville, Terry; Rhodes-Clark, Bobbie S.; Makhoul, Issam; Nakagawa, Mayumi
The remarkable advances in interferon-sparing, all-oral hepatitis C virus (HCV) treatment were a highlight of the 2014 Conference on Retroviruses and Opportunistic Infections (CROI). The backbone of the nucleotide inhibitor sofosbuvir and the nonstructural protein 5A (NS5A) inhibitor ledipasvir with an additional third agent (HCV protease inhibitor or HCV nonnucleoside reverse transcriptase inhibitor) led to a sustained virologic response (SVR) rate 12 weeks after cessation of treatment of 95% to 100% after only 6 weeks of treatment. These results demonstrate the potential of combination directacting antiviral (DAA) therapy for abbreviated, well-tolerated, and highly effective HCV treatment. Two triple-drug regimens that comprised 12 weeks of an NS5A inhibitor, an HCV protease inhibitor, and a nonnucleoside inhibitor also resulted in SVRs of more than 90% in patients with HCV genotype 1. HIV coinfection does not appear to negatively impact response to DAA-based HCV therapy, as evidenced by similar response rates in HIV/HCV-coinfected patients compared with HCV-monoinfected patients receiving interferonsparing or -containing regimens. There was continued emphasis at CROI 2014 on non-AIDS complications of HIV infection, specifically cardiovascular disease, renal insufficiency, and bone and endocrine disorders that persist among patients with treated HIV disease and contribute to morbidity and mortality. Finally, new data on novel drugs and combinations for treatment of tuberculosis (TB), patient outcomes using new rapid TB diagnostics, and a short-course TB prevention strategy were presented. PMID:24901886
Luetkemeyer, Anne F; Havlir, Diane V; Currier, Judith S
Background Alterations in extracellular vesicles (EVs), including exosomes and microparticles, contribute to cardiovascular disease. We hypothesized that obesity could favour enhanced release of EVs from adipose tissue, and thereby contribute to cardiovascular risk via obesity-induced metabolic complications. The objectives of this study were: 1) to investigate the relation between the quantity, distribution and (dys) function of adipose tissue and plasma concentrations of atherothrombotic EV-markers; 2) to determine the relation between these EV-markers and the prevalence of the metabolic syndrome; and 3) to assess the contribution of EV markers to the risk of incident type 2 diabetes. Methods In 1012 patients with clinically manifest vascular disease, subcutaneous and visceral fat thickness was measured ultrasonographically. Plasma EVs were isolated and levels of cystatin C, serpin G1, serpin F2 and CD14 were measured, as well as fasting metabolic parameters, hsCRP and adiponectin. The association between adiposity, EV-markers, and metabolic syndrome was tested by multivariable linear and logistic regression analyses. As sex influences body fat distribution, sex-stratified analyses between adipose tissue distribution and EV-markers were performed. The relation between EV-markers and type 2 diabetes was assessed with Cox regression analyses. Results Higher levels of hsCRP (? 5.59; 95% CI 3.00–8.18) and lower HDL-cholesterol levels (?-11.26; 95% CI ?18.39 – -4.13) were related to increased EV-cystatin C levels, and EV-cystatin C levels were associated with a 57% higher odds of having the metabolic syndrome (OR 1.57; 95% CI 1.19–2.27). HDL-cholesterol levels were positively related to EV-CD14 levels (? 5.04; 95% CI 0.07–10.0), and EV-CD14 levels were associated with a relative risk reduction of 16% for development of type 2 diabetes (HR 0.84, 95% CI 0.75–0.94), during a median follow up of 6.5 years in which 42 patients developed type 2 diabetes. Conclusions In patients with clinically manifest vascular disease, EV-cystatin C levels were positively related, and EV-CD14 levels were negatively related to metabolic complications of obesity.
Accumulating evidence indicates that obesity is closely associated with an increased risk of metabolic diseases such as insulin resistance, type 2 diabetes, dyslipidemia and nonalcoholic fatty liver disease. Obesity results from an imbalance between food intake and energy expenditure, which leads to an excessive accumulation of adipose tissue. Adipose tissue is now recognized not only as a main site of storage of excess energy derived from food intake but also as an endocrine organ. The expansion of adipose tissue produces a number of bioactive substances, known as adipocytokines or adipokines, which trigger chronic low-grade inflammation and interact with a range of processes in many different organs. Although the precise mechanisms are still unclear, dysregulated production or secretion of these adipokines caused by excess adipose tissue and adipose tissue dysfunction can contribute to the development of obesity-related metabolic diseases. In this review, we focus on the role of several adipokines associated with obesity and the potential impact on obesity-related metabolic diseases. Multiple lines evidence provides valuable insights into the roles of adipokines in the development of obesity and its metabolic complications. Further research is still required to fully understand the mechanisms underlying the metabolic actions of a few newly identified adipokines.
Jung, Un Ju; Choi, Myung-Sook
Background: The objective of this study was to compare the performance of the Mini-Mental State Examination (MMSE) total score as well as item scores in separating 4 groups of elderly (55-85 years of age) subjects—normal controls, subjects with mild cognitive impairment (MCI), subjects with mild Alzheimer's disease, and sub- jects with depression. Method: The MMSE scores of 86 subjects (25
Aaron D. Benson; Melissa J. Slavin; Thanh-Thu Tran; Jeffrey R. Petrella; P. Murali Doraiswamy
Background Transthyretin amyloidosis is a systemic disorder caused by amyloid deposits formed by misfolded transthyretin monomers. Two main forms exist: hereditary and wild-type transthyretin amyloidosis, the former associated with transthyretin gene mutations. There are several disease manifestations; however, gastrointestinal complications are common in the hereditary form. The aim of this study was to explore the prevalence and distribution of gastrointestinal manifestations in transthyretin amyloidosis and to evaluate their impact on the patients’ nutritional status and health-related quality of life (HRQoL). Methods The Transthyretin Amyloidosis Outcomes Survey (THAOS) is the first global, multicenter, longitudinal, observational survey that collects data on patients with transthyretin amyloidosis and the registry is sponsored by Pfizer Inc. This study presents baseline data from patients enrolled in THAOS as of June 2013. The modified body mass index (mBMI), in which BMI is multiplied with serum albumin, was used to assess the nutritional status and the EQ-5D Index was used to assess HRQoL. Results Data from 1579 patients with hereditary transthyretin amyloidosis and 160 patients with wild-type transthyretin amyloidosis were analyzed. Sixty-three percent of those with the hereditary form and 15% of those with the wild-type form reported gastrointestinal symptoms at enrollment. Unintentional weight loss and early satiety were the most frequent symptoms, reported by 32% and 26% of those with transthyretin gene mutations, respectively. Early-onset patients (<50 years) reported gastrointestinal complaints more frequently than those with a late onset (p?0.001) and gastrointestinal symptoms were more common in patients with the V30M mutation than in those with other mutations (p?0.001). For patients with predominantly cardiac complications, the prevalence of gastrointestinal manifestations was not evidently higher than that expected in the general population. Both upper and lower gastrointestinal symptoms were significant negative predictors of mBMI and the EQ-5D Index Score (p?0.001 for all). Conclusions Gastrointestinal symptoms were common in patients with hereditary transthyretin amyloidosis and had a significant negative impact on their nutritional status and HRQoL. However, patients with wild-type transthyretin amyloidosis or transthyretin mutations associated with predominantly cardiac complications did not show an increased prevalence of gastrointestinal disturbances.
Orphan medicinal products (OMPs) are targeted at the diagnosis, prevention or treatment of rare diseases and have a special status in European law. This status brings incentives for pharmaceutical companies to invest in OMP development. The goal of the legislation is to encourage the development of more treatments for life-threatening rare disorders, but increased availability of OMPs raises important issues surrounding the public funding of very expensive treatments by national health services. In this article we review OMPs and the incentives for their development and discuss the challenges presented by funding these treatments.
Dear, James W; Lilitkarntakul, Pajaree; Webb, David J
The burden of diseases caused by food-borne pathogens remains largely unknown. Importantly data indicating trends in food-borne infectious intestinal disease is limited to a few industrialised countries, and even fewer pathogens. It has been predicted that the importance of diarrhoeal disease, mainly due to contaminated food and water, as a cause of death will decline worldwide. Evidence for such a downward trend is limited. This prediction presumes that improvements in the production and retail of microbiologically safe food will be sustained in the developed world and, moreover, will be rolled out to those countries of the developing world increasingly producing food for a global market. In this review evidence is presented to indicate that the microbiological safety of food remains a dynamic situation heavily influenced by multiple factors along the food chain from farm to fork. Sustaining food safety standards will depend on constant vigilance maintained by monitoring and surveillance but, with the rising importance of other food-related issues, such as food security, obesity and climate change, competition for resources in the future to enable this may be fierce. In addition the pathogen populations relevant to food safety are not static. Food is an excellent vehicle by which many pathogens (bacteria, viruses/prions and parasites) can reach an appropriate colonisation site in a new host. Although food production practices change, the well-recognised food-borne pathogens, such as Salmonella spp. and Escherichia coli, seem able to evolve to exploit novel opportunities, for example fresh produce, and even generate new public health challenges, for example antimicrobial resistance. In addition, previously unknown food-borne pathogens, many of which are zoonotic, are constantly emerging. Current understanding of the trends in food-borne diseases for bacterial, viral and parasitic pathogens has been reviewed. The bacterial pathogens are exemplified by those well-recognized by policy makers; i.e. Salmonella, Campylobacter, E. coli and Listeria monocytogenes. Antimicrobial resistance in several bacterial food-borne pathogens (Salmonella, Campylobacter, Shigella and Vibrio spp., methicillin resistant Staphylcoccus aureas, E. coli and Enterococci) has been discussed as a separate topic because of its relative importance to policy issues. Awareness and surveillance of viral food-borne pathogens is generally poor but emphasis is placed on Norovirus, Hepatitis A, rotaviruses and newly emerging viruses such as SARS. Many food-borne parasitic pathogens are known (for example Ascaris, Cryptosporidia and Trichinella) but few of these are effectively monitored in foods, livestock and wildlife and their epidemiology through the food-chain is poorly understood. The lessons learned and future challenges in each topic are debated. It is clear that one overall challenge is the generation and maintenance of constructive dialogue and collaboration between public health, veterinary and food safety experts, bringing together multidisciplinary skills and multi-pathogen expertise. Such collaboration is essential to monitor changing trends in the well-recognised diseases and detect emerging pathogens. It will also be necessary understand the multiple interactions these pathogens have with their environments during transmission along the food chain in order to develop effective prevention and control strategies. PMID:20153070
Newell, Diane G; Koopmans, Marion; Verhoef, Linda; Duizer, Erwin; Aidara-Kane, Awa; Sprong, Hein; Opsteegh, Marieke; Langelaar, Merel; Threfall, John; Scheutz, Flemming; van der Giessen, Joke; Kruse, Hilde
The beta amyloid (A beta) protein is a key molecule in the pathogenesis of Alzheimer's disease (AD). The tendency of the A beta peptide to aggregate, its reported neurotoxicity, and genetic linkage studies, have led to a hypothesis of AD pathogenesis that many AD researchers term the amyloid cascade hypothesis. In this hypothesis, an increased production of A beta results in neurodegeneration and ultimately dementia through a cascade of events. In the past 15 years, debate amongst AD researchers has arisen as to whether A beta is a cause or an effect of the pathogenic process. Recent in vitro and in vivo research has consolidated the theory that A beta is the primary cause, initiating secondary events, culminating in the neuropathological hallmarks associated with AD. This research has led to the development of therapeutic agents, currently in human clinical trials, which target A beta. PMID:15304237
Verdile, Giuseppe; Fuller, Stephanie; Atwood, Craig S; Laws, Simon M; Gandy, Samuel E; Martins, Ralph N
Background Liver stiffness measurement (LSM) by transient elastography (TE, FibroScan) is a validated method for noninvasively staging liver fibrosis. Most hepatic complications occur in patients with advanced fibrosis. Our objective was to determine the ability of LSM by TE to predict hepatic complications and mortality in a large cohort of patients with chronic liver disease. Methods In consecutive adults who underwent LSM by TE between July 2008 and June 2011, we used Cox regression to determine the independent association between liver stiffness and death or hepatic complications (decompensation, hepatocellular carcinoma, and liver transplantation). The performance of LSM to predict complications was determined using the c-statistic. Results Among 2,052 patients (median age 51 years, 65% with hepatitis B or C), 87 patients (4.2%) died or developed a hepatic complication during a median follow-up period of 15.6 months (interquartile range, 11.0–23.5 months). Patients with complications had higher median liver stiffness than those without complications (13.5 vs. 6.0 kPa; P<0.00005). The 2-year incidence rates of death or hepatic complications were 2.6%, 9%, 19%, and 34% in patients with liver stiffness <10, 10–19.9, 20–39.9, and ?40 kPa, respectively (P<0.00005). After adjustment for potential confounders, liver stiffness by TE was an independent predictor of complications (hazard ratio [HR] 1.05 per kPa; 95% confidence interval [CI] 1.03–1.06). The c-statistic of liver-stiffness for predicting complications was 0.80 (95% CI 0.75–0.85). A liver stiffness below 20 kPa effectively excluded complications (specificity 93%, negative predictive value 97%); however, the positive predictive value of higher results was sub-optimal (20%). Conclusions Liver stiffness by TE accurately predicts the risk of death or hepatic complications in patients with chronic liver disease. TE may facilitate the estimation of prognosis and guide management of these patients.
Pang, Jack X. Q.; Zimmer, Scott; Niu, Sophia; Crotty, Pam; Tracey, Jenna; Pradhan, Faruq; Shaheen, Abdel Aziz M.; Coffin, Carla S.; Heitman, Steven J.; Kaplan, Gilaad G.; Swain, Mark G.; Myers, Robert P.
In ?-thalassemia, unequal production of ?- and ?-globin chains in erythroid precursors causes apoptosis and inhibition of late-stage erythroid differentiation, leading to anemia, ineffective erythropoiesis (IE), and dysregulated iron homeostasis. Here we used a murine model of ?-thalassemia intermedia (Hbbth1/th1 mice) to investigate effects of a modified activin receptor type IIB (ActRIIB) ligand trap (RAP-536) that inhibits Smad2/3 signaling. In Hbbth1/th1 mice, treatment with RAP-536 reduced overactivation of Smad2/3 in splenic erythroid precursors. In addition, treatment of Hbbth1/th1 mice with RAP-536 reduced ?-globin aggregates in peripheral red cells, decreased the elevated reactive oxygen species present in erythroid precursors and peripheral red cells, and alleviated anemia by promoting differentiation of late-stage erythroid precursors and reducing hemolysis. Notably, RAP-536 treatment mitigated disease complications of IE, including iron overload, splenomegaly, and bone pathology, while reducing erythropoietin levels, improving erythrocyte morphology, and extending erythrocyte life span. These results implicate signaling by the transforming growth factor-? superfamily in late-stage erythropoiesis and reveal potential of a modified ActRIIB ligand trap as a novel therapeutic agent for thalassemia syndrome and other red cell disorders characterized by IE.
Suragani, Rajasekhar N. V. S.; Cawley, Sharon M.; Li, Robert; Wallner, Samantha; Alexander, Mark J.; Mulivor, Aaron W.; Gardenghi, Sara; Rivella, Stefano; Grinberg, Asya V.; Pearsall, R. Scott
Obesity increases the risk for developing type 2 diabetes mellitus (T2DM) and this in turn correlates with an elevated probability of long-term diabetes complications once diabetes is established. Interventions aimed at lowering weight via changes in diet and lifestyle have repeatedly been shown to improve glycemic control in patients with T2DM and even to reverse early disease. Weight gain, a potential side effect of treatment for patients with T2DM, is also an important concern, and it has been noted that weight increases associated with antidiabetes therapy may blunt cardiovascular risk reductions achieved by decreasing blood glucose. Among older agents, metformin and acarbose have the lowest risk for weight gain, while sulfonylureas, meglitinides, and thiazolidinediones are all associated with weight increases. Clinical trial results have also consistently demonstrated that treatment with glucagon-like peptide-1 receptor agonists and amylin lowers weight, and that dipeptidyl peptidase-4 inhibitors are weight neutral in patients with T2DM. Conventional human insulin formulations are known to increase weight in patients with T2DM. However, some insulin analogs, particularly insulin detemir, have lower liability for this adverse event. The use of both pharmacologic and surgical therapies aimed at treating obesity rather than lowering blood glucose have the potential to improve glycemic control and even resolve T2DM in some patients.
Apovian, Caroline M
In ?-thalassemia, unequal production of ?- and ?-globin chains in erythroid precursors causes apoptosis and inhibition of late-stage erythroid differentiation, leading to anemia, ineffective erythropoiesis (IE), and dysregulated iron homeostasis. Here we used a murine model of ?-thalassemia intermedia (Hbb(th1/th1) mice) to investigate effects of a modified activin receptor type IIB (ActRIIB) ligand trap (RAP-536) that inhibits Smad2/3 signaling. In Hbb(th1/th1) mice, treatment with RAP-536 reduced overactivation of Smad2/3 in splenic erythroid precursors. In addition, treatment of Hbb(th1/th1) mice with RAP-536 reduced ?-globin aggregates in peripheral red cells, decreased the elevated reactive oxygen species present in erythroid precursors and peripheral red cells, and alleviated anemia by promoting differentiation of late-stage erythroid precursors and reducing hemolysis. Notably, RAP-536 treatment mitigated disease complications of IE, including iron overload, splenomegaly, and bone pathology, while reducing erythropoietin levels, improving erythrocyte morphology, and extending erythrocyte life span. These results implicate signaling by the transforming growth factor-? superfamily in late-stage erythropoiesis and reveal potential of a modified ActRIIB ligand trap as a novel therapeutic agent for thalassemia syndrome and other red cell disorders characterized by IE. PMID:24795345
Suragani, Rajasekhar N V S; Cawley, Sharon M; Li, Robert; Wallner, Samantha; Alexander, Mark J; Mulivor, Aaron W; Gardenghi, Sara; Rivella, Stefano; Grinberg, Asya V; Pearsall, R Scott; Kumar, Ravindra
In 2009, the first outbreak of hand, foot and mouth disease (HFMD) or herpangina (HP) caused by enterovirus 71 occurred in the Republic of Korea. This study inquired into risk factors associated with complications of HFMD or HP. A retrospective medical records review was conducted on HFMD or HP patients for whom etiologic viruses had been verified in 2009. One hundred sixty-eight patients were examined for this investigation. Eighty patients were without complications while 88 were accompanied by complications, and 2 had expired. Enterovirus 71 subgenotype C4a was the most prevalent in number with 67 cases (54.9%). In the univariate analysis, the disease patterns of HFMD rather than HP, fever longer than 4 days, peak body temperature over 39?, vomiting, headache, neurologic signs, serum glucose over 100 mg/dL, and having an enterovirus 71 as a causative virus were significant risk factors of the complications. After multiple logistic analysis, headache (Odds ratio [OR], 10.75; P < 0.001) and neurologic signs (OR, 42.76; P < 0.001) were found to be the most significant factors. Early detection and proper management of patients with aforementioned risk factors would be necessary in order to attain a better clinical outcome.
Kim, Seong Joon; Kang, Jin-Han; Kim, Dong Soo; Kim, Ki Hwan; Kim, Kyung-Hyo; Kim, Young-Hoon; Chung, Ju-Young; Bin, Joong Hyun; Jung, Da Eun; Kim, Ji Hong; Kim, Hwang Min; Cheon, Doo-Sung; Kang, Byung Hak; Seo, Soon Young
In 2009, the first outbreak of hand, foot and mouth disease (HFMD) or herpangina (HP) caused by enterovirus 71 occurred in the Republic of Korea. This study inquired into risk factors associated with complications of HFMD or HP. A retrospective medical records review was conducted on HFMD or HP patients for whom etiologic viruses had been verified in 2009. One hundred sixty-eight patients were examined for this investigation. Eighty patients were without complications while 88 were accompanied by complications, and 2 had expired. Enterovirus 71 subgenotype C4a was the most prevalent in number with 67 cases (54.9%). In the univariate analysis, the disease patterns of HFMD rather than HP, fever longer than 4 days, peak body temperature over 39?, vomiting, headache, neurologic signs, serum glucose over 100 mg/dL, and having an enterovirus 71 as a causative virus were significant risk factors of the complications. After multiple logistic analysis, headache (Odds ratio [OR], 10.75; P < 0.001) and neurologic signs (OR, 42.76; P < 0.001) were found to be the most significant factors. Early detection and proper management of patients with aforementioned risk factors would be necessary in order to attain a better clinical outcome. PMID:23341722
Kim, Seong Joon; Kim, Jong-Hyun; Kang, Jin-Han; Kim, Dong Soo; Kim, Ki Hwan; Kim, Kyung-Hyo; Kim, Young-Hoon; Chung, Ju-Young; Bin, Joong Hyun; Jung, Da Eun; Kim, Ji Hong; Kim, Hwang Min; Cheon, Doo-Sung; Kang, Byung Hak; Seo, Soon Young
Background Celiac disease is a gluten-induced disease of global malabsorption. There is a subset of patients with celiac disease who are free of major symptoms but who have typical damage to the intestinal mucosa (silent disease). We present the case of a 50-year-old white woman with no clinical symptoms of celiac disease who developed diarrhea and weight loss 12 weeks
Aljamir D. Chedid; Cleber R. P. Kruel; Marcio F. Chedid; Ronaldo J. S. Torresini; Geraldo R. Geyer
Background Amyloid A amyloidosis is an obstinate disease complication in chronic inflammatory disease, and there are few effective therapies.\\u000a The objective of this study was to investigate the effect of oral dimethyl sulfoxide (DMSO) on amyloid A amyloidosis.\\u000a \\u000a \\u000a \\u000a Methods Fifteen secondary amyloid A amyloidosis patients (4 men, 11 women; age, 23–70 years) were treated with DMSO between 1995 and\\u000a 2003. DMSO was
Sadahiro Amemori; Ryuichi Iwakiri; Hiroyoshi Endo; Akifumi Ootani; Shinichi Ogata; Takahiro Noda; Seiji Tsunada; Hiroyuki Sakata; Hisashi Matsunaga; Masanobu Mizuguchi; Yuji Ikeda; Kazuma Fujimoto
Background Albuminuria is strongly associated with future risk for cardiovascular and kidney outcomes, and has been proposed to be included in the classification of chronic kidney disease (CKD) along with glomerular filtration rate (GFR). Few data are available on whether albuminuria is associated with concurrent complications of CKD. Study Design Cross-Sectional Setting & Participants The Modification of Diet in Renal Disease (MDRD) Study examined the effects of dietary protein restriction and blood pressure control on the progression of kidney disease. This analysis includes 1665 participants screened for the MDRD Study. Predictors Urine albumin-creatinine ratio (ACR) and measured GFR using urinary clearance of iothalamate. Outcomes & Measurements Associations between ACR categories and GFR categories with anemia, acidosis, hyperphosphatemia and hypertension were evaluated using log-binomial regression. Results Mean GFR (±SD) was 39 ml/min/1.73m2 (± 21) and the median (25th–75th percentile) ACR was 161 (38–680) mg/g. In multivariable models adjusted for age, sex, race, kidney disease etiology and GFR, higher ACR levels were not associated with any complication. For example, comparing ACR > 300 mg/g versus < 30 mg/g, the prevalence ratio (95% CI) for anemia was 0.98 (0.81–1.20), acidosis 1.13 (0.86–1.48), hyperphosphatemia 1.69 (0.91–3.17) and hypertension 1.04 (0.97–1.12). Lower levels of GFR were associated with all complications. For example, GFR levels < 30 ml/min/1.73m2 versus GFR levels 60–89 ml/min/1.73m2 were associated with prevalence ratios (95% CI) of anemia 4.35 (3.18–5.96), acidosis 5.31 (3.41–8.29), hyperphosphatemia 23.8 (7.71–73.6) and hypertension 1.21 (1.10–1.32). Limitations Limited generalizability; lack of data on other complications that may be related to CKD. Conclusions Albuminuria is not associated with complications after controlling for GFR and thus would not affect clinical action plans for decisions regarding evaluation and treatment of complications.
Viswanathan, Gautham; Sarnak, Mark J.; Tighiouart, Hocine; Muntner, Paul; Inker, Lesley A.
Introduction. Mondor's disease of the penis is an uncommon condition characterized by thrombosis or thrombophlebitis involving the superficial dorsal veins. An accompanied lymphangitis is discussed. There is typical self-limiting clinical course. Case Presentation. This paper firstly reports a secondary abscess formation of the preputial leaf two weeks after penile Mondor's disease and subcutaneous lymphangitis as complication of excessive sexual intercourse of a 44-year-old man. Sexual transmitted diseases could be excluded. Lesions healed up completely under abscess drainage, antibiotic, and anti-inflammatory medication. Conclusion. Previous reports in the literature include several entities of the penile Mondor's disease. Our patient is very unusual in that he presented with a secondary preputial abscess formation due to superficial thrombophlebitis, subcutaneous lymphangitis, and local bacterial colonisation. Abscess drainage plus antiphlogistic and antibiotic medication is the treatment of choice.
Wendler, Johann Jakob; Schindele, Daniel; Liehr, Uwe-Bernd; Porsch, Markus; Schostak, Martin
The aim of this study was to analyze ocular manifestations, their complications, and treatment in a sample of 40 patients with confirmed Behcet's disease. Results. Serofibrinous iridocyclitis was the most common form of uveitis (60%). Retinal periphlebitis manifested in 92.5% of cases, and periphlebitis in conjunction with periarteritis was diagnosed in 72.5% of cases. Macular edema was the most frequent complication on the posterior segment (60%) and it correlated with periphlebitis (P = 0.45) and periphlebitis associated with periarteritis (P = 0.51). Cyclosporine A and corticosteroids were used in the majority of cases (67%). Following six months of therapy, a significant improvement of visual acuity occurred in patients with initial visual acuity >0.5 on both eyes. Level of visual acuity before and after treatment had a strong significant correlation coefficient with various ocular complications. Previously proven significant increase of visual acuity in patients with macular edema depicts effectiveness of treatment in these types of ocular manifestations of Behcet's disease. Conclusions. Significant improvement of visual acuity occurred in patients with initial visual acuity >0.5 on both eyes. The highest increase in visual acuity was achieved by laser photocoagulation in combination with triamcinolone acetonide P = 0.038 < 0.050.
Leucine-rich repeat kinase 2 (LRRK2) is linked to various diseases, including Parkinson’s disease, cancer, and leprosy. Data from LRRK2 knockout mice has highlighted a possible role for LRRK2 in regulating signaling pathways that are linked to the pathogenesis of Crohn’s disease. Here, we examine how LRRK2’s role as a signaling hub in the cell could lead to diverse pathologies.
Patrick A. Lewis (University College London;UCL Institute of Neurology REV); Claudia Manzoni (University College London;UCL Institute of Neurology REV)
In this activity, learners work in groups to build simple solar stills filled with salt water. After the stills are complete, learners observe what happens when they place the stills in the sun. Learners taste the water they collect and discuss what has happened in their stills.
Schleith, Susan; Hall, Penny
Calciphylaxis presents like subcutaneous lesions with livedo reticularis leading to necrotic and painful ulcers, predominantly in the lower limbs and the abdomen. They initially simulate dermohypodermitis. Biology reveals secondary hyperparathyroidism, phosphocalcic metabolism abnormalities and state of hypercoagulability. Histological signs are constant: calcifications in the media of small and sub-cutaneous arteries, intimal hyperplasia and intravascular thrombosis. This complication occurs in 4% of end-stage renal disease patients. Its prognostic is awful with a rate of mortality of 60% due to sepsis. Treatment is based upon the normalization of phosphocalcic rates and local debridement. PMID:16243422
Trost, O; Kadlub, N; Trouilloud, P; Malka, G; Danino, A
Case Presentation Spontaneous mesenteric haematoma is a rare condition that occurs due to localized bleeding in the mesenteric vascular tree of a bowel segment in the absence of an identifiable cause. Here we report a case of spontaneous mesenteric haematoma during an inflammatory exacerbation of Crohn’s disease. The patient underwent surgical management for small bowel obstruction secondary to Crohn’s disease, however the concurrent presence of a spontaneous mesenteric haematoma in the mid-jejunal mesentery was successfully managed conservatively. Conclusion This case identifies the first association of spontaneous mesenteric haematoma with an exacerbation of Crohn’s disease and highlights the need to consider rare differential diagnoses such as SMH when performing radiological assessment of unexplained symptoms in inflammatory bowel disease patients.
BACKGROUND Sickle cell disease is recognized as a hypercoagulable state; however the frequency and characteristics of venous thromboembolism in sickle cell patients have not been well-defined. PURPOSE To establish the prevalence and risk factors for venous thromboembolism in a large cohort of patients with sickle cell disease and determine the relationship between venous thromboembolism and mortality. METHODS We performed a cross-sectional study of 404 sickle cell disease patients cared for at the Sickle Cell Center for Adults at Johns Hopkins. Demographic, sickle cell disease-specific comorbidity, and venous thromboembolism data were collected on all patients. RESULTS 101 patients (25%) had a history of venous thromboembolism with a median age at diagnosis of 29.9 years. A history of non-catheter-related venous thromboembolism was found in 18.8% of patients. Sickle variant genotypes conferred a higher risk of non-catheter-related venous thromboembolism compared to sickle cell anemia genotypes (SS/S?0) (relative risk (RR) 1.77, 95% confidence interval (CI) 1.18–2.66). Tricuspid regurgitant jet velocity ? 2.5 m/s was also associated with non-catheter-related venous thromboembolism (RR 1.65, CI 1.12–2.45). Thirty patients (7.4%) died during the study period. Adjusting for all variables, non-catheter-related venous thromboembolism was independently correlated with death (RR 3.63, CI 1.66–7.92). CONCLUSION Venous thromboembolism is common in adults with sickle cell disease. Sickle variant genotypes and tricuspid regurgitant jet velocity ? 2.5m/s are associated with non-catheter-related venous thromboembolism. In addition, non-catheter-related venous thromboembolism appears to be an independent risk factor for death in our cohort. These results suggest that disease-specific prophylaxis and treatment strategies for venous thromboembolism should be investigated in sickle cell disease patients.
Naik, Rakhi P.; Streiff, Michael B.; Haywood, Carlton; Nelson, Julie A.; Lanzkron, Sophie
Background and aims. There is growing evidence that white adipose tissue is an important contributor in the pathogenesis of alcoholic liver disease (ALD). We investigated serum concentrations of total adiponectin (Acrp30), leptin, and resistin in patients with chronic alcohol abuse and different grades of liver dysfunction, as well as ALD complications. Materials and Methods. One hundred forty-seven consecutive inpatients with ALD were prospectively recruited. The evaluation of plasma adipokine levels was performed using immunoenzymatic ELISA tests. Multivariable logistic regression was applied in order to select independent predictors of advanced liver dysfunction and the disease complications. Results. Acrp30 and resistin levels were significantly higher in patients with ALD than in controls. Lower leptin levels in females with ALD compared to controls, but no significant differences in leptin concentrations in males, were found. High serum Acrp30 level revealed an independent association with advanced liver dysfunction, as well as the development of ALD complications, that is, ascites and hepatic encephalopathy. Conclusion. Gender-related differences in serum leptin concentrations may influence the ALD course, different in females compared with males. Serum Acrp30 level may serve as a potential prognostic indicator for patients with ALD.
Surdacka, Agata; Smolen, Agata
A 28-year-old man treated with the antitumour necrosis factor ? (TNF?) monoclonal antibody infliximab for Crohn's disease developed pulmonary tuberculosis (TB), despite testing negative for latent TB prior to treatment. On starting anti-TB treatment and withdrawal of the anti-TNF? therapy, he deteriorated both clinically and radiologically. He was diagnosed with a flare of Crohn's disease, and immune reconstitution inflammatory syndrome (IRIS) in his right upper lobe and mediastinal lymph nodes, and commenced on oral prednisolone. Anti-TNF? therapy was re-introduced, and prednisolone weaned, following 4 months of anti-TB treatment without complication. He made a full recovery from TB, although his Crohn's symptoms continue to be troublesome. There has been no reactivation of TB to date, after 2 years follow-up.
O'Dowd, Caroline; Kewin, Peter; Morris, John; Cotton, Mark
Immunity to malaria can be acquired but only after repeat exposures to polymorphic Plasmodium. However, the development of clinical outcomes during P. falciparum infection is not clearly understood. This study elucidated whether monocytes, neutrophils and pro/anti-inflammatory cytokines were associated with clinical outcomes in single infection and prior repeated malaria infections. Two hundred and seventy-nine patients with complicated and uncomplicated malaria were investigated. Peripheral blood IFN-gamma, TNF-alpha and IL-10 levels were measured by ELISA, and monocytes and neutrophils by an automated cell counter. On admission, in patients with uncomplicated malaria prior repeated infections, absolute neutrophil counts were positively and monocyte to neutrophil ratio negatively correlated significantly with parasitemia (r = 0.358, p = 0.000; r = -0.356, p = 0.000, respectively), while those with single infection absolute monocyte counts and monocyte to neutrophil ratio were significantly correlated negatively with IFN-gamma (r = -0.381, p = 0.001; r = -0.393, p = 0.000, respectively), and positively with TNF-alpha levels (r = 0.310, p = 0.007; r = 0.227, p = 0.017, respectively). In sharp contrast, in complicated malaria with single infection extremely high IFN-gamma and IL-10 levels but significantly low percent monocyte counts and monocyte to neutrophil ratio were seen. After 7 days of treatment, absolute monocyte counts and monocyte to neutrophil ratio were significantly increased, while absolute neutrophil counts significantly decreased (p = 0.000, 0.000, and 0.001, respectively), similarly after 28 days of treatment (p = 0.008, 0.000 and 0.000, respectively). These results suggest different functions of monocytes, neutrophils and pro/anti-inflammatory cytokines in complicated and uncomplicated malaria with single P. falciparum infection or prior repeated infections in the context of disease severity. Low monocyte to neutrophil ratio may be regarded as a risk factor in developing complication in primary malaria infection. PMID:24974635
Tangteerawatana, Piyatida; Krudsood, Srivicha; Kanchanakhan, Naowarat; Troye-Blomberg, Marita; Khusmith, Srisin
Cytomegalovirus (CMV) affects the gastrointestinal tract commonly; however CMV colitis is uncommon in patients who are not severely immunocompromised. The author reports a 51-year-old Caucasian female who was admitted to the hospital with exacerbation of her chronic diarrhoea. CAT scan of the abdomen and pelvis showed thickening of the wall of the terminal ileum with inflammatory stranding and follow-up colonoscopy showed extensive circumferential ulceration in the terminal ileum. Biopsies confirmed diagnosis of CMV infection and the patient was started on appropriate antimicrobial agents. CMV viremia showed response to the medication; however the patient’s intestinal symptoms failed to improve. Nodular lesions consistent with erythema nodusum were noticed and promethus test was consistent with Crohn’s disease. The patient showed good response to immunosuppressive therapy. CMV infections are known to exacerbate symptoms of inflammatory bowel disease and hence Crohn’s disease should be suspected in an immunocompetent patient presenting with CMV ileitis.
Chronic Granulomatous Disease is the most commonly encountered immunodeficiency involving the phagocyte, and is characterized by repeated infections with bacterial and fungal pathogens, as well as the formation of granulomas in tissue. The disease is the result of a disorder of the NADPH oxidase system, culminating in an inability of the phagocyte to generate superoxide, leading to the defective killing of pathogenic organisms. This can lead to infections with Staphylococcus aureus, Psedomonas species, Nocardia species, and fungi (such as Aspergillus species and Candida albicans). Involvement of vital or large organs can contribute to morbidity and/or mortality in the affected patients. Major advances have occurred in the diagnosis and treatment of this disease, with the potential for gene therapy or stem cell transplantation looming on the horizon.
Behçet's disease is a chronic, relapsing multisystemic inflammatory disorder characterized by recurrent orogenital aphthous ulcers, uveitis, and skin lesions. Vascular involvement occurs in up to 38% of these patients. Herein, we report a 19-year-old male who initially presented with an isolated celiac artery aneurysm that was treated with open surgical repair. The patient was subsequently diagnosed with Behçet's disease after the development of oral aphthous ulcers and multiple recurrent postoperative deep venous thromboses and thoracoabdominal arterial aneurysms. Ultimately, a hybrid approach was undertaken. This is the fifth celiac artery aneurysm ever reported in this patient group and the first to present with an isolated celiac artery aneurysm as the initial manifestation of Behçet's disease. PMID:20089553
Ullery, Brant W; Pochettino, Alberto; Wang, Grace J; Jackson, Benjamin M; Fairman, Ronald M; Woo, Edward Y
Castleman's disease is a rare atypical lymphoproliferative disorder. Renal manifestations, such as proteinuria, hematuria, and renal dysfunction, are common in Castleman's disease; however, a nephrotic syndrome rarely occurs. We have encountered an unusual case of Castleman's disease of the plasma cell type characterized by nephrotic syndrome because of glomerulopathy mimicking membranoproliferative glomerulonephritis. Our patient showed higher levels of circulating cytokines (interleukin-6/vascular endothelial cell-derived growth factor), the glomerular lesions not associated with immunocomplex deposition, and the resolution of nephrotic syndrome after successful corticosteroids therapy resulting in a decline in cytokines levels, thereby implicating a cytokine-induced glomerular cell injury/activation as a possible cause of the glomerular pathological changes in this case. PMID:18552583
Sugimoto, Toshiro; Ito, Jun; Takeda, Naoko; Gasyu, Ippei; Okazaki, Tsutomu; Sakaguchi, Masayoshi; Osawa, Norihisa; Tanaka, Yuki; Oka, Kazumasa; Uzu, Takashi; Kashiwagi, Atsunori
A 21-year-old man with a history of sudden rectal hemorrhage was referred to our hospital. Examination disclosed thrombocytopenia and hepatosplenomegaly. A liver biopsy specimen demonstrated Gaucher cells in Glisson's capsule. Additional investigations revealed a low level of leukocyte ?-glucosidase activity and common mutations of the glucocerebrosidase gene, L444P/D409H. We diagnosed the patient with Gaucher disease type 1. He underwent enzyme replacement therapy. Thrombocytopenia and hepatosplenomegaly improved at a rate of approximately 50 and 20%, respectively, within 6 months. This case suggests that we must pay attention to adult Gaucher disease as a differential diagnosis for cryptogenic thrombocytopenia.
Ito, Junitsu; Saito, Takafumi; Numakura, Chikahiko; Iwaba, Akiko; Sugahara, Shinpei; Ishii, Rika; Sato, Chikako; Haga, Hiroaki; Okumoto, Kazuo; Nishise, Yuko; Watanabe, Hisayoshi; Ida, Hiroyuki; Hayasaka, Kiyoshi; Togashi, Hitoshi; Kawata, Sumio; Ueno, Yoshiyuki
Background & Aims Although percutaneous liver biopsy is a standard diagnostic procedure, it has drawbacks, including risk of serious complications. It is not known whether persons with advanced chronic liver disease have a greater risk of complications from liver biopsy than patients with more mild, chronic liver disease. The safety and complications of liver biopsy were examined in patients with hepatitis C-related bridging fibrosis or cirrhosis that were enrolled in the Hepatitis C Antiviral Long-Term Treatment against Cirrhosis (HALT-C) Trial. Methods Standard case report forms from 2,740 liver biopsies performed at 10 study sites between 2000 and 2006 were reviewed for serious adverse events, along with information from questionnaires completed by investigators about details of biopsy techniques used at each hospital. Results There were 29 serious adverse events (1.1%); the most common was bleeding (16 cases, 0.6%). There were no biopsy-related deaths. The bleeding rate was higher among patients with platelet counts ?60,000/mm3 and among those with an international normalized ratio (INR) ?1.3, although none of the patients with an INR >1.5 bled. Excluding subjects with a platelet count ?60,000/mm3 would have reduced the bleeding rate by 25% (4/16), eliminating only 2.8% (77/2740) of biopsies. Operator experience, the type of needle used, or the performance of the biopsy under ultrasound guidance did not influence the frequencies of adverse events. Conclusions Approximately 0.5% of persons with hepatitis C and advanced fibrosis experienced potentially serious bleeding after liver biopsy; risk increased significantly in patients with platelet counts ?60,000/mm3.(K2).
Seeff, Leonard B.; Everson, Gregory T.; Morgan, Timothy R.; Curto, Teresa M.; Lee, William M.; Ghany, Marc G.; Shiffman, Mitchell L.; Fontana, Robert J.; Di Bisceglie, Adrian M.; Bonkovsky, Herbert L.; Dienstag, Jules L.
Purpose: To evaluate the safety and feasibility of percutaneous transsplenic access to the portal vein for management of vascular complication in patients with chronic liver diseases. Methods: Between Sept 2009 and April 2011, percutaneous transsplenic access to the portal vein was attempted in nine patients with chronic liver disease. Splenic vein puncture was performed under ultrasonographic guidance with a Chiba needle, followed by introduction of a 4 to 9F sheath. Four patients with hematemesis or hematochezia underwent variceal embolization. Another two patients underwent portosystemic shunt embolization in order to improve portal venous blood flow. Portal vein recanalization was attempted in three patients with a transplanted liver. The percutaneous transsplenic access site was closed using coils and glue. Results: Percutaneous transsplenic splenic vein catheterization was performed successfully in all patients. Gastric or jejunal varix embolization with glue and lipiodol mixture was performed successfully in four patients. In two patients with a massive portosystemic shunt, embolization of the shunting vessel with a vascular plug, microcoils, glue, and lipiodol mixture was achieved successfully. Portal vein recanalization was attempted in three patients with a transplanted liver; however, only one patient was treated successfully. Complete closure of the percutaneous transsplenic tract was achieved using coils and glue without bleeding complication in all patients. Conclusion: Percutaneous transsplenic access to the portal vein can be an alternative route for portography and further endovascular management in patients for whom conventional approaches are difficult or impossible.
Chu, Hee Ho; Kim, Hyo-Cheol, E-mail: firstname.lastname@example.org; Jae, Hwan Jun [Seoul National University College of Medicine, Seoul National University Medical Research Center, Seoul National University Hospital, Department of Radiology, Institute of Radiation Medicine, Clinical Research Institute (Korea, Republic of); Yi, Nam-Joon; Lee, Kwang-Woong; Suh, Kyung-Suk [Seoul National University College of Medicine and Seoul National University Hospital, Department of Surgery (Korea, Republic of); Chung, Jin Wook; Park, Jae Hyung [Seoul National University College of Medicine, Seoul National University Medical Research Center, Seoul National University Hospital, Department of Radiology, Institute of Radiation Medicine, Clinical Research Institute (Korea, Republic of)
Pediatric neuromuscular diseases such as Duchenne muscular dystrophy and spinal muscular atrophy cause pulmonary compromise. In severely affected patients, upper respiratory tract infections exacerbate lower respiratory tract secretion retention, with the potential for pneumonia, pulmonary atelectasis, and respiratory failure. In the pediatric intensive care unit, effective treatment includes noninvasive positive pressure ventilation and manual and mechanical mucus clearance techniques. A
David J. Birnkrant; John F. Pope; Robert M. Eiben
Cerebral venous thrombosis is an uncommon and diverse entity accounting for less than 1% of strokes. It can present with a variety of clinical symptoms ranging from isolated headaches to deep coma making the clinical diagnosis difficult. We present a rare case of cerebral venous thrombosis secondary to dehydration and inflammatory bowel disease. PMID:18629575
Soong, M M; Carroll, A
Review of the literature reveals that postradiation chondrosarcoma is a rare secondary malignant bone tumor. This case report demonstrates a Grade 1 chondrosarcoma of the proximal right clavicle in a 17-year-old boy, eight years after extensive chemotherapy and radiation therapy for a Stage IIB Hodgkin's disease. PMID:3731593
Aprin, H; Calandra, J; Mir, R; Lee, J Y
Background & Aims Celiac crisis is a life-threatening syndrome in which patients with celiac disease have profuse diarrhea and severe metabolic disturbances. Celiac crisis is rare among adults and not well documented. To improve awareness of this condition and to facilitate diagnosis, we reviewed cases of celiac crisis to identify presenting features, formulate diagnostic criteria, and develop treatment strategies. Methods Cases of biopsy-proven celiac disease were reviewed. Celiac crisis was defined as acute onset or rapid progression of gastrointestinal symptoms that could be attributed to celiac disease and required hospitalization and/or parenteral nutrition, along with signs or symptoms of dehydration or malnutrition. Results Twelve patients met preset criteria for celiac crisis; 11 developed celiac crisis before they were diagnosed with celiac disease. Eleven patients had increased titres of tTG and 1 had immunoglobulin A deficiency. Results of biopsy analyses of duodenum samples from all patients were consistent with a Marsh 3 score (33% with total villous atrophy). Patients presented with severe dehydration, renal dysfunction, and electrolyte disturbances. All patients required hospitalization and intravenous fluids, 6 required corticosteroids, and 5 required parenteral nutrition. All patients eventually had a full response to a gluten-free diet. Conclusion Celiac crisis has a high morbidity and, although rarely described, occurs in adults and often has a clear precipitating factor. Patients that present with severe unexplained diarrhea and malabsorption should be tested for celiac disease; treatment with systemic steroids or oral budesonide should be considered. Nutritional support is often required in the short term but most patients ultimately respond to gluten avoidance.
Jamma, Shailaja; Rubio-Tapia, Alberto; Kelly, Ciaran P.; Murray, Joseph; Sheth, Sunil; Schuppan, Detlef; Dennis, Melinda; Leffler, Daniel A.
Osteoporosis affects all bones, including those of the facial skeleton. To date the facial bones have not drawn much attention due to the minimal probability of morbid fractures. Hearing and dentition loss due to osteoporosis has been reported. New research findings suggest that radiologic examination of the facial skeleton can be a cost-effective adjunct to complement the early diagnosis and the follow up of osteoporosis patients. Bone-mass preservation treatments have been associated with osteomyelitis of the jawbones, a condition commonly described as osteonecrosis of the jaws (ONJ). The facial skeleton, where alimentary tract mucosa attaches directly to periosteum and teeth which lie in their sockets of alveolar bone, is an area unique for the early detection of osteoporosis but also for the prevention of treatment-associated complications. We review facial bone involvement in patients with osteoporosis and we present data that make the multidisciplinary approach of these patients more appealing for both practitioners and dentists. With regard to ONJ, a tabular summary with currently available evidence is provided to facilitate multidisciplinary practice coordination for the treatment of patients receiving bisphosphonates.
Kyrgidis, Athanassios; Tzellos, Thrasivoulos-George; Toulis, Konstantinos; Antoniades, Konstantinos
We report the case of a 25-year-old male with Neurofibromatosis type?I?(NF-1), who presented at the time of admission with clinical findings of an acute abdomen caused by a mechanical obstruction. Computerized tomography showed a volvulus of the terminal ileum with mesenteric swirling as the cause of the patient's symptoms. Consecutive exploratory laparotomy confirmed the diagnosis and 70 cm of the small intestine was resected due to an affection of the mesentery by multiple neurofibromas. The gastrointestinal tract is affected in approximately 10% of patients with NF-1, however the mesentery is almost always spared. Here we describe the unique case of a patient with a volvulus caused by mesenteric manifestation of von Recklinghausen's disease, emphasizing the role of surgery in a team of multidisciplinary specialists to treat this multiorganic disease. PMID:24976735
Werner, Thomas Artur; Kröpil, Feride; Schoppe, Martin Olaf; Kröpil, Patric; Knoefel, Wolfram Trudo; Krieg, Andreas
Kawasaki disease is a generalized systemic vasculitis of unknown etiology involving medium and small size blood vessels throughout the body, virtually always involving the coronaries. In many part of this world, it is more common than rheumatic fever and viral myocarditis. Here, we are reporting 5 cases with history suggestive of Kawasaki disease in the early life, presenting with different coronary artery abnormalities. One of the patients had left main coronary artery cut-off, and the remaining 4 had some form of coronary artery aneurysms. 3 patients were given medical management, 1 patient had coronary bypass grafting, and the remaining 1 was planned for surgical correction. All the patients were stable on subsequent follow-ups.
Mandal, Saroj; Pande, Arindam; Mandal, Debosmita; Sarkar, Achyut; Kahali, Dhiman; Panja, Manotosh
We report the case of a 25-year-old male with Neurofibromatosis type?I?(NF-1), who presented at the time of admission with clinical findings of an acute abdomen caused by a mechanical obstruction. Computerized tomography showed a volvulus of the terminal ileum with mesenteric swirling as the cause of the patient’s symptoms. Consecutive exploratory laparotomy confirmed the diagnosis and 70 cm of the small intestine was resected due to an affection of the mesentery by multiple neurofibromas. The gastrointestinal tract is affected in approximately 10% of patients with NF-1, however the mesentery is almost always spared. Here we describe the unique case of a patient with a volvulus caused by mesenteric manifestation of von Recklinghausen’s disease, emphasizing the role of surgery in a team of multidisciplinary specialists to treat this multiorganic disease.
Werner, Thomas Artur; Kropil, Feride; Schoppe, Martin Olaf; Kropil, Patric; Knoefel, Wolfram Trudo; Krieg, Andreas
A seventy one year-old woman, who had an arch and thoracoabdominal aortic aneurysm (type II according to Crawford classification)\\u000a with ischemic heart disease, underwent a separate operation using the elephant trunk method. At first, she underwent the ascending\\u000a aorta and arch replacement the with elephant trunk technique, and, underwent CABG simultaneously utilizing the separate extra\\u000a corporeal circulation and hypothermic circulatory
Satoshi Yamashiro; Ryuzo Sakata; Yoshihiro Nakayama; Masashi Ura; Yoshio Arai; Akihiro Sugimoto
OBJECTIVE--To determine the incidence of and risk factors for the development of secondary acute leukaemia and myelodysplasia in patients treated in British National Lymphoma Investigation's studies of Hodgkin's disease since 1970. PATIENTS--2676 Patients entered into Hodgkin's disease studies between February 1970 and November 1986. Data accrued up to November 1988 were analysed, ensuring a minimum follow up period of two years. DESIGN--Retrospective analysis of multicentre trial data by case-control and life table methods. RESULTS--17 Cases of secondary leukaemia were recorded in this group of 2676 patients, giving an overall risk at 15 years of 1.7%. The risks of leukaemia after chemotherapy alone and chemotherapy with radiotherapy were not significantly different. The risk of leukaemia increased sharply with the amount of treatment given as measured by the number of attempts at treatment. The 15 year risks of leukaemia were 0.2%, 2.3%, and 8.1% for patients receiving one, two, or three or more attempts at treatment. The highest risk, 22.8% at 15 years, was observed in patients treated with lomustine (CCNU), and a case-control study suggested that this was an independent risk factor. The risk of secondary leukaemia was largely related to the overall quantity of treatment, although exposure to lomustine seemed to be an important risk factor. Treatment with both drugs and radiation was not more leukaemogenic than treatment with drugs alone. The greatest risk of secondary leukaemia was seen in multiply treated patients who were unlikely to be cured of Hodgkin's disease. CONCLUSIONS--Avoidance of secondary leukaemia should be a minor factor in the choice of treatment for Hodgkin's disease.
Devereux, S; Selassie, T G; Vaughan Hudson, G; Vaughan Hudson, B; Linch, D C
Enterovesical fistula (EVF) is a relatively uncommon condition that is associated with severe morbidity. Minimally invasive and organ-preserving surgery should be performed in the case of EVF caused by benign diseases. We applied laparoscopic bladder-preserving surgery (LBPS) for EVF caused by benign gastrointestinal disease. Here, we report a surgical technique for LBPS. Patient and instrument port positioning are similar to those used in laparoscopic colorectal surgery. Dissection around the fistula is performed along the intestine as distant from the bladder as possible. If there is sufficient area around the intestinal portion of the fistula, it is isolated and resected using a linear stapler. If this approach is not possible, the intestinal fistula is sharply dissected as far away from the bladder as possible. LBPS for EVF was performed in 4 patients and included 3 direct sharp dissections and 1 stapling dissection. Three of the 4 patients did not require any further treatment for the bladder, and all procedures were feasibly accomplished under laparoscopic conditions. In conclusion, LBPS is feasible in cases of EVF caused by benign gastrointestinal disease, and we suggest that it should be the first choice of intervention in such cases.
Mizushima, Tsunekazu; Ikeda, Masataka; Sekimoto, Mitsugu; Yamamoto, Hirofumi; Doki, Yuichiro; Mori, Masaki
Summary Background: Pneumatosis cystoides intestinalis (PCI) is a rare disorder characterized by the presence of multiple gas collections in the subserosal or submucosal intestinal wall of the large or small intestine. We report two cases of PCI in the course of chronic graft-versus-host disease. Case Report: A 5-year-old girl was treated for acute lymphoblastic leukemia. Twenty-four months after the hematopoietic stem cell transplantation, in the course of graft-versus-host disease, she developed subcutaneous emphysema of the right inguinal and pudendal region. PCI was diagnosed based on a CT examination. A 3-year-old boy was treated for juvenile myelomonocytic leukemia. Fourteen months after the hematopoietic stem cell transplantation he presented with an increased severity of intestinal symptoms, including intermittent bleeding from large intestine. PCI was diagnosed based on a CT exam and was confirmed by a colonoscopy. Conclusions: Pneumatosis cystoides interstitialis in the course of chronic graft-versus-host disease has a heterogeneous clinical presentation that does not correlate with results of imaging. CT is a method of choice to diagnose PCI. In patients with PCI, the presence of free air in the peritoneal cavity does not confirm an intestinal perforation.
Laskowska, Katarzyna; Burzynska-Makuch, Malgorzata; Krenska, Anna; Koltan, Sylwia; Chrupek, Malgorzata; Nawrocka, Elzbieta; Lasek, Wladyslaw; Serafin, Zbigniew
Benign anorectal disease comprises a broad group of processes with very diverse origins; these processes may be congenital or acquired as well as inflammatory or tumor related. However, benign anorectal disease has received less attention in the scientific literature than malignant disease. In this second part of this image-based review of benign anorectal disease, we describe the most common inflammatory and fistulous diseases, the postsurgical anatomy, and complications that can occur after surgical treatment or radiotherapy for anorectal disease. PMID:23102780
Herráiz Hidalgo, L; Cano Alonso, R; Carrascoso Arranz, J; Alvarez Moreno, E; Martínez de Vega Fernández, V
Background Although patients with systemic lupus erythematosus (SLE) may experience various gastrointestinal disorders, SLE and Crohn’s disease (CD) rarely coexist. The diseases may have gastrointestinal (GI) manifestations, laboratory results, and radiographic findings that appear similar and consequently differentiating between GI involvement in CD and in SLE may be difficult. We present the case of a patient with SLE and CD who developed continuous GI bleeding and diarrhea that was initially treated as SLE-related colitis to little effect. Case presentation A 55-year-old Japanese woman with systemic lupus erythematosus (SLE) developed continuous gastrointestinal bleeding and diarrhea since the patient was aged 30 years that was initially treated as SLE-related colitis. Although a longitudinal ulcer and aphthous ulcers in the colon were observed every examination, biopsy showed only mild inflammation and revealed neither granuloma nor crypt abscess. The patient underwent surgery for anal fistulas twice at 50 and 54 years of age and her symptoms were atypical of lupus enteritis. Colonoscopy was performed again when the patient was 55 years of age because we suspected she had some type of inflammatory bowel disease (IBD). Cobblestone-like inflammatory polyps and many longitudinal ulcers were detected between the descending colon and the cecum. Macroscopic examination strongly suggested CD. Histopathological examination revealed non-caseating granuloma and no evidence of vasculitis, consistent with CD. Introduction of infliximab dramatically relieved the patient’s melena and abdominal symptoms. Conclusion Diagnostic criteria for CD and SLE overlap, making them difficult to diagnose correctly. It is important to consider CD for patients who have SLE with gastrointestinal manifestations. The pathology of lupus enteritis should be clarified through the accumulation of cases of SLE combined with CD.
Background/Aims Glycogen storage disease III (GSD III) is caused by a deficiency of glycogen-debranching enzyme which causes an incomplete glycogenolysis resulting in glycogen accumulation with abnormal structure (short outer chains resembling limit dextrin) in liver and muscle. Hepatic involvement is considered mild, self-limiting and improves with age. With increased survival, a few cases of liver cirrhosis and hepatocellular carcinoma (HCC) have been reported. Methods A systematic review of 45 cases of GSD III at our center (20 months to 67 years of age) was reviewed for HCC, 2 patients were identified. A literature review of HCC in GSD III was performed and findings compared to our patients. Conclusions GSD III patients are at risk for developing HCC. Cirrhosis was present in all cases and appears to be responsible for HCC transformation There are no reliable biomarkers to monitor for HCC in GSD III. Systematic evaluation of liver disease needs be continued in all patients, despite lack of symptoms. Development of guidelines to allow for systematic review and microarray studies are needed to better delineate the etiology of the hepatocellular carcinoma in patients with GSD III.
Demo, Erin; Frush, Donald; Gottfried, Marcia; Koepke, John; Boney, Anne; Bali, Deeksha; Chen, Y.T.; Kishnani, Priya S.
We report the case of a woman with a history of chronic alcohol abuse who was hospitalized with diarrhea, severe hypokalemia refractory to potassium infusion, nausea, vomiting, abdominal pain, alternations of high blood pressure with phases of hypotension, irritability and increased urinary 5-hydroxyindoleacetic acid and cortisol. Although carcinoid syndrome was hypothesized, abdominal computed tomography and colonoscopy showed non-specific inflammatory bowel disease with severe colic wall thickening, and multiple colic biopsies confirmed non-specific inflammation with no evidence of carcinoid cells. During the following days diarrhea slowly decreased and the patient's condition progressively improved. One year after stopping alcohol consumption, the patient was asymptomatic and serum potassium was normal. Chronic alcohol exposure is known to have several deleterious effects on the intestinal mucosa and can favor and sustain local inflammation. Chronic alcohol intake may also be associated with high blood pressure, behavior disorders, abnormalities in blood pressure regulation with episodes of hypotension during hospitalization due to impaired baroreflex sensitivity in the context of an alcohol withdrawal syndrome, increased urinary 5-hydroxyindoleacetic acid as a result of malabsorption syndrome, and increased urinary cortisol as a result of hypothalamic-pituitary-adrenal axis dysregulation. These considerations, together with the regression of symptoms and normalization of potassium levels after stopping alcohol consumption, suggest the intriguing possibility of a alcohol-related acute inflammatory bowel disease mimicking carcinoid syndrome.
Ballo, Piercarlo; Dattolo, Pietro; Mangialavori, Giuseppe; Ferro, Giuseppe; Fusco, Francesca; Consalvo, Matteo; Chiodi, Leandro; Pizzarelli, Francesco; Zuppiroli, Alfredo
A total of 764 patients with Hodgkin's disease treated with radiotherapy (RT) or chemotherapy or both were reviewed 3-186 months (median 43 months) after initial treatment to assess the incidence of second malignancies. Incidence of solid tumours and acute non-lymphoblastic leukaemia (ANLL) were calculated by a life-table method and percentages of patients affected derived from life-table plots. Within 10 years after initial treatment the overall incidence of second solid tumours was 7.3%, and over a comparable period 2.4% of patients developed ANLL. Solid tumours occurred only in patients given RT with or without adjuvant chemotherapy, and ANLL occurred only after treatment with MOPP (mustine, vincristine, procarbazine, and prednisolone) or modified MOPP regimens. Neither solid tumours nor ANLL occurred in patients given ABVD (adriamycin, bleomycin, vinblastine, and dacarbazine). The highest incidence of leukaemia (5.4%) occurred after treatment with extensive RT plus (5.4%) occurred after treatment with extensive RT plus MOPP; hence the benefits of this approach in Hodgkin's disease must be weighed against its carcinogenic potential.
Valagussa, P; Santoro, A; Kenda, R; Fossati Bellani, F; Franchi, F; Banfi, A; Rilke, F; Bonadonna, G
We describe a 29-old-year Japanese man with autosomal recessive polycystic kidney disease who was frequently hypoglycemic. Insulinoma as a cause of hypoglycemia was denied because the ratio of plasma immunoreactive insulin to glucose was low. Adrenal insufficiency was diagnosed because of the low urinary excretion of 17-hydroxycorticosteroids, and both blunted responses of plasma cortisol to an intravenous injection of adrenocorticotropin and of plasma adrenocorticotropin to an intravenous injection of human corticotropin releasing hormone were observed, although basal plasma concentrations of cortisol and adrenocorticotropin were normal. The elusion profile of plasma sample from our patient chromatographed on a Sephadex G-75 column showed two peaks of (1-39)-ACTH and beta-lipotropin, with no evidence of high molecular weight form of ACTH. The plasma concentrations of thyroid stimulating hormone and growth hormone were within the normal range. These findings indicated that this patient with autosomal recessive polycystic kidney disease was associated with adrenal insufficiency due to isolated adrenocorticotropin deficiency. PMID:12803513
Yonemura, Katushiko; Yasuda, Hideo; Fujigaki, Yoshihide; Oki, Yutaka; Hishida, Akira
Background Thrombophlebitis of the cavernous sinus is a rare but serious disease that most often affects young adults and children. It is associated with significant morbidity or mortality and is often related to local infections of the head. The diagnosis is based on clinical findings and is confirmed by imaging. Case Report: We report the case of a 17-year-old male with a history of recurrent sinusitis, who presented general signs of infection, orbital symptoms, and meningeal involvement. CT and MRI showed thrombosis of the cavernous sinus associated with cerebral ischemic damage. The therapeutic management included empiric antibiotic therapy, drainage of an orbital collection, and anticoagulation. The patient died later secondary to septic shock. Conclusions: Although thrombophlebitis of the cavernous sinus is increasingly rare, it remains a lethal complication of sinusitis, and mortality is still high. The course of this disease can be dramatic due to infectious or vascular neurological complications. Early diagnosis and appropriate treatment are crucial.
Berdai, Adnane Mohamed; Shimi, Abdelkarim; Khatouf, Mohammed
Objective. This systematic review aims to evaluate the benefit and side effect of Xuezhikang for coronary heart disease (CHD) complicated by dyslipidemia. Methods. All randomized clinical trials (RCTs) with Xuezhikang as a treatment for CHD combined with dyslipidemia were considered for inclusion. Data extraction and analyses and quality assessment were conducted according to the Cochrane standards. Results. We included 22 randomized trials. Xuezhikang showed significant benefit on the incidence of all-cause deaths, CHD deaths, myocardial infarction, and revascularization as compared with placebo based on conventional treatment for CHD. It remarkably lowered total cholesterol (TC), triglyceride (TG), and low-density lipoprotein-cholesterol (LDL-C) as compared with the placebo or inositol nicotinate group, which was similar to statins group. Xuezhikang also raised high-density lipoprotein cholesterol (HDL-C) compared to placebo or no intervention, which was similar to Inositol nicotinate and slightly inferior to statins. The incidence of adverse events did not differ between the Xuezhikang and control group. Conclusions. Xuezhikang showed a comprehensive lipid-regulating effect and was safe and effective in reducing cardiovascular events in CHD patients complicated by dyslipidemia. However, more rigorous trials with high quality are needed to give high level of evidence.
Shang, Qinghua; Liu, Zhaolan; Chen, Keji; Xu, Hao; Liu, Jianping
Nearly two-thirds of patients with Parkinson's disease (PD) use vitamins or nutritional supplements, and many more may use other complementary therapies, yet <50% of patients have discussed the use of these complementary therapies with a healthcare professional. Physicians should be aware of the complementary therapies their patients with PD are using, and the possible effects of these therapies on motor and non-motor symptoms. Complementary therapies, such as altered diet, dietary supplements, vitamin therapy, herbal supplements, caffeine, nicotine, exercise, physical therapy, massage therapy, melatonin, bright-light therapy and acupuncture, may all influence the symptoms of PD and/or the effectiveness of dopaminergic therapy. Preliminary evidence suggests complementary therapy also may influence non-motor symptoms of PD, such as respiratory disorders, gastrointestinal disorders, mood disorders, sleep and orthostatic hypotension. Whenever possible, clinicians should ensure that complementary therapy is used appropriately in PD patients without reducing the benefits of dopaminergic therapy. PMID:19739693
Zesiewicz, Theresa A; Evatt, Marian L
For treating end-stage renal disease-associated anemia, various strategies to achieve optimal hemoglobin levels with lower erythropoiesis stimulating agent doses are being tried. One of these involves the use of a high dose [transferrin saturation (TSAT) >30%] of intravenous (IV) iron supplementation. However, due to in vitro effects of iron on stimulating bacterial growth, there are concerns of increased risk of infection. The safety of higher iron targets with respect to infectious complications (bacteremias, pneumonias, soft tissue infections, and osteomyelitis) is unknown. This was a retrospective study of patients on maintenance hemodialysis from a single, urban dialysis center to assess the long-term impact of the higher cumulative use of IV iron, on the incidence of clinically important infections. Our iron protocol was modified in June 2010 to aim for TSAT >30% unless serum ferritin levels were >1200 ng/mL. Data from only those patients who had been on dialysis for the whole duration between June 2009 and May 2011 were included. A total of 140 patients with end-stage renal disease on hemodialysis patients were found to be eligible for the study. There was a statistically significant increase in the mean TSAT and mean serum ferritin with the new anemia management protocol with a significant decrease in the mean erythropoiesis stimulating agent dose requirement. There was no statistically significant increase in the incidence of infectious complications. Although in vitro effects of iron are known to stimulate bacterial growth, a higher IV dose of iron may not increase the risk of infection in such patients. PMID:22832501
Bansal, Anip; Sandhu, Gagangeet; Gupta, Isha; Kalahalli, Shriharsha; Nayak, Rushi; Zouain, Eduardo; Chitale, Rohit A; Meisels, Ira; Chan, Germaine
Rationale Hyperglycemia and reduced insulin actions affect many biological processes. One theory is that aberrant metabolism of glucose via several pathways including the polyol pathway causes cellular toxicity. Aldose reductase (AR) is a multifunctional enzyme that reduces aldehydes. Under diabetic conditions AR converts glucose into sorbitol, which is then converted to fructose. Objective This article reviews the biology and pathobiology of AR actions. Results AR expression varies considerably among species. In humans and rats, the higher level of AR expression is associated with toxicity. Flux via AR is increased by ischemia and its inhibition during ischemia reperfusion reduces injury. However, similar pharmacologic effects are not observed in mice unless they express a human AR transgene. This is because mice have much lower levels of AR expression, probably insufficient to generate toxic byproducts. Human AR expression in LDL receptor knockout mice exacerbates vascular disease, but only under diabetic conditions. In contrast, a recent report suggests that genetic ablation of AR increased atherosclerosis and increased hydroxynonenal in arteries. It was hypothesized that AR knockout prevented reduction of toxic aldehydes. Conclusion Like many in vivo effects found in genetically manipulated animals, interpretation requires the reproduction of human-like physiology. For AR, this will require tissue specific expression of AR in sites and at levels that approximate those in humans.
Ramasamy, Ravichandran; Goldberg, Ira J.
Dietary fatty acids play significant roles in the cause and prevention of cardiovascular disease (CVD). Trans fatty acids from partially hydrogenated vegetable oils have well-established adverse effects and should be eliminated from the human diet. CVD risk can be modestly reduced by decreasing saturated fatty acids (SFA) and replacing it by a combination of polyunsaturated fatty acids (PUFA) and monounsaturated fatty acids (MUFA). Although the ideal type of unsaturated fat for this replacement is unclear, the benefits of PUFA appear strongest. Both n-6 and n-3 PUFA are essential and reduce CVD risk. However, additional research is needed to better define the optimal amounts of both and to discern the patients and/or general population that would benefit from supplemental n-3 fatty acid intake. Furthermore, consumption of animal products, per se, is not necessarily associated with increased CVD risk, whereas nut and olive oil intake is associated with reduced CVD risk. In conclusion, the total matrix of a food is more important than just its fatty acid content in predicting the effect of a food on CVD risk, and a healthy diet should be the cornerstone of CVD prevention. PMID:24727233
Michas, George; Micha, Renata; Zampelas, Antonis
Purpose of review We have reviewed the recent literature to describe the potential medication errors and adverse drug events (ADEs) associated with antihypertensives among older adults with chronic kidney disease (CKD). Recent findings Overall, few studies have been published describing ADEs in older adults with CKD. Several examined hyperkalemia associated with angiotensin-converting enzyme (ACE)-inhibitor/angiotensin II receptor blocker (ARB), diuretic (potassium-sparing), and ?-blocker use. Additional studies described acute kidney injury (AKI) most commonly with ACE-inhibitor/ARB therapy. Finally, orthostatic hypotension was evaluated in those taking ACE-inhibitor/ARB, ?-blocker, or calcium-channel blocker therapy. In the absence of robust literature examining these events in this understudied population, one must consider age-related antihypertensive pharmacokinetic/pharmacodynamic profiles concomitantly with the patient’s comorbidities and other medications in order to minimize the risk for potential medication errors, drug–drug interactions, and ADEs. Summary Some of the most common ADEs associated with antihypertensive use in older adults with CKD include hyperkalemia, AKI, and orthostatic hypotension. Diligent monitoring of laboratory data, vital signs, and potential drug–drug interactions may mitigate serious ADEs caused by antihypertensives in this high-risk patient population.
Marcum, Zachary A.; Fried, Linda F.
The effort to develop vaginal contraceptives began in the distant past and is still underway today. 1000 years ago, South American Indians inserted into the vagina bark strips impregnated with quinine. In medieval times women used vaginal inserts of cloth soaked in honey or vinegar. Quinine pessaries were introduced into Europe in the late 1800s, and in the early 1900s investigators began to study the effects of various chemicals on sperm motility. Following World War II, surfactant spermicides which disrupt the sperm membrane were developed and marketed. Many of these preparations contained nonoxynol-9. Currently, the D-isomer of propranolol is being examined as a spermicidal contraceptive, and several bacteriocides, e.g., benzalkonium and chlorhexidine, are being developed as spermicides which reduce the penetrability of cervical mucus. Other chemicals being investigated act by inhibiting the acrosome reaction. Advantages of vaginal contraceptives are that they are inexpensive, reversible, and relatively safe and easy to use. Generally they require no medical intervention or supervision. In addition, spermicides may kill or inhibit the growth of organisms responsible for sexually transmitted diseases. Disadvantages of spermicides are that they are generally less effective than many other methods, some interfere with sexual spontaneity, they may cause local irritations, and some women find them messy to use. Recently, concerns were expressed about the possible teratogenic effects of sperimicides. Most of these concerns proved to be unfounded. Given the many new avenues of research, the major disadvantage of sperimicides, i.e., their high failure rates, may be minimized in the near future. PMID:12340621
Pearson, R M
Background Pediatric venous thromboembolism (VTE) is an increasingly common problem. We hypothesized that VTE occurs most commonly in tertiary care settings and that the pattern of associated illnesses may have changed from earlier reports. Methods The Kids’ Inpatient Database 2006 was utilized to identify children ?18 years old with in-hospital VTE. Children were identified by the presence of thrombosis specific ICD-9-CM diagnosis or procedure codes. Remaining ICD-9-CM codes were utilized to categorize patients by acute or chronic illness. The incidence of in-hospital VTE by hospital type, age, gender, race, and disposition were estimated. Results Over 4,500 children met the inclusion criteria (188/100,000 discharges). Most VTE discharges (67.5%) were from children’s hospitals (RR 5.09; 95% CI 4.76; 5.44). Underlying chronic illnesses were associated with most VTE (76.2%), most commonly: cardiovascular (18.4%), malignancy (15.7%), and neuromuscular disease (9.9%). VTE not associated with chronic illness were most often idiopathic (12.6%), followed by infections (9.5%) and trauma (9.1%). The greatest proportions of children with VTE were infants (23.1%) and adolescents (37.8%). However, when standardized against the entire database of discharges, infants were least likely to develop VTE (RR 0.48; 95% CI 0.43; 0.52), while adolescents were at highest risk (RR 1.89; 95% CI 1.73; 2.07). Hospitalizations ending with death were more likely to include VTE (RR 6.16; 95% CI 5.32; 7.13). Conclusions Pediatric VTE is most commonly seen in tertiary care. Adolescents are at greatest risk to develop in-hospital VTE. Patients whose hospitalization ended with death are at much greater risk to develop VTE.
Setty, Bhuvana A.; O'Brien, Sarah H.; Kerlin, Bryce A.
A 59-year-old man, who was diagnosed as having Parkinson's disease and depression seven years ago and was on oral antiparkinsonian agents, antianxiety agents, and antidepressants, developed a high fever, disturbed consciousness, and marked muscle rigidity after discontinuation of etizolam and amitriptyline. He was admitted to a nearby hospital. Hypothyroidism had been noted two months before admission. Marked muscle rigidity and increased serum CK were observed. Since discontinuation of benzodiazepine has been known to rarely trigger a neuroleptic malignant syndrome (NMS), he was diagnosed as having NMS. After receiving dantrolene and bromocriptine, these symptoms temporarily improved but he again developed consciousness disturbance, and convulsive seizures associated with an elevated serum CK. He was transferred to our hospital. On admission, the CK level was normal at 168 IU/l, while free T4 was 0.6 ng/dl (normal range, 0.9-2.3) and TSH was 108.7 mU/ml (normal range, 0.2-4.2) in serum, indicating the presence of primary hypothyroidism. As an increase in thyroid hormone dosage improved the thyroid function to normal level, his disturbed consciousness and muscle rigidity gradually improved. Convulsive seizure and recurrence of NMS in a short interval are unusual in neuroleptic malignant syndrome. In this patient, hypothyroidism may have contributed to the development of malignant syndrome through metabolic changes of the central dopaminergic system, and discontinuation of etizolam, a kind of benzodiazepine, may have triggered NMS, since there has not been reported that discontinuation of antidepressants including amitriptyline triggers NMS. PMID:12424963
Kawajiri, Masakazu; Ohyagi, Yasumasa; Furuya, Hirokazu; Araki, Takehisa; Inoue, Naohide; Esaki, Shigemitsu; Yamada, Takeshi; Kira, Junichi
Background Low serum magnesium concentrations have been associated with cardiovascular disease risk and outcomes in some general population studies but there are no equivalent studies in diabetes. Metformin may have cardiovascular benefits beyond blood glucose lowering in type 2 diabetes but its association with hypomagnesemia appears paradoxical. The aim of this study was to examine relationships between metformin therapy, magnesium homoeostasis and cardiovascular disease in well-characterized type 2 patients from the community. Methods and Findings We studied 940 non-insulin-treated patients (mean±SD age 63.4±11.6 years, 49.0% males) from the longitudinal observational Fremantle Diabetes Study Phase I (FDS1) who were followed for 12.3±5.3 years. Baseline serum magnesium was measured using stored sera. Multivariate methods were used to determine associates of prevalent and incident coronary heart disease (CHD) and cerebrovascular disease (CVD) as ascertained from self-report and linked morbidity/mortality databases. 19% of patients were hypomagnesemic (serum magnesium <0.70 mmol/L). Patients on metformin, alone or combined with a sulfonylurea, had lower serum magnesium concentrations than those on diet alone (P<0.05). There were no independent associations between serum magnesium or metformin therapy and either CHD or CVD at baseline. Incident CVD, but not CHD, was independently and inversely associated with serum magnesium (hazard ratio (95% CI) 0.28 (0.11–0.74); P?=?0.010), but metformin therapy was not a significant variable in these models. Conclusions Since hypomagnesemia appears to be an independent risk factor for CVD complicating type 2 diabetes, the value of replacement therapy should be investigated further, especially in patients at high CVD risk.
Peters, Kirsten E.; Chubb, S. A. Paul; Davis, Wendy A.; Davis, Timothy M. E.
\\u000a Infections are the most frequently occurring complications of hematopoietic stem cell transplantation (HSCT). Myelosuppressive\\u000a medications, the conditioning regimen (chemotherapy, radiation therapy), mucosal damage, type of transplant, immune-suppressive\\u000a therapy, and graft-versus-host disease (GvHD) all predispose the HSCT patient to life-threatening infections. Abnormal B-\\u000a and T-lymphocyte function results in impaired cellular and humoral immune function. Infections that can occur in the setting
Since the 1990s numerous international experts have reported about the somatic complications of eating disorders including those having a dental and stomatological nature. Several reports emphasised that deformations in the oral cavity resulting from this grave nutritional disease typical of the young generation could already appear in the early stage and, therefore, dentists are among the first to diagnose them. Dentists are still often unaware of the importance of their role in multidisciplinary treatment. Even if they knew what the disease was about and recognised it on the basis of deformations in the oral cavity in time, their advice that their patients should brush their teeth more often would fail to eliminate the root cause of the problem. Not only the earliest possible treatment of the complications of the bingeing-purging mechanism and the maintenance of oral hygiene are important, but controlling and curing pathological habits with active participation of psychiatrists are also required to ensure full recovery. Due to the multidisciplinary nature of the disease, manifold communication is required. For this reason, publishing the dental ramifications of organic and systemic diseases at dental conferences and in technical journals, as well as providing information about oral complications of eating disorders for general practitioners and specialists are particularly important. PMID:23123325
Resch, Mária; Nagy, Agnes
Acute rehabilitation refers to the multidisciplinary rehabilitative treatment of patients in continuing need of integrated acute and rehabilitative longterm care. As a result of the advances in acute rheumatology and improved emergency services, an increasing number of patients survive episodes of severe disease and complications of immunosuppressive therapy. These patients require not only treatment of their acute medical problems but also specialized multidisciplinary acute rehabilitation starting as early as possible during their hospital stay. We describe 4 typical cases from the major fields of rheumatology. (1) Acute rehabilitation of a 63-year-old woman with rheumatoid arthritis after replacement of both preexisting knee endoprostheses in one session and removal of the left hip endoprosthesis due to infection and sepsis. (2) Rehabilitation of a 29-year-old man with a 7-year history of ankylosing spondylitis who lived in an adjustable easy chair for 2 years due to severe pain prior to admission. (3) A 61-year-old woman with active refractory Wegener's granulomatosis who developed respiratory insufficiency due to aspergillus and pseudomonas pneumonia. (4) The acute rehabilitation of a 21-year-old woman with systemic lupus erythematosus and a history of 14 laparotomies due to severe acute pancreatitis and multiple gut perforations. Acute rehabilitation was complicated by a large defect of the abdominal wall and significant critical illness polyneuropathy. Our report points out differences between acute, postacute, and longterm rehabilitation, describes the mobilization of patients in acute rheumatology units, and defines specific problems encountered in acute hospital-based rehabilitation of rheumatological patients. PMID:19531761
Kujath, Kerstin; Hermann, Kay-Geert A; Mathiske-Schmidt, Kirsten; Schwedtke, Christine; Hiepe, Falk; Burmester, Gerd-Rüdiger; Reisshauer, Anett
Non-alcoholic fatty liver disease (NAFLD) is currently the most common liver disease worldwide, both in adults and children. It is characterized by an aberrant lipid storage in hepatocytes, named hepatic steatosis. Simple steatosis remains a benign process in most affected patients, while some of them develop superimposed necroinflammatory activity with a non-specific inflammatory infiltrate and a progression to non-alcoholic steatohepatitis with or without fibrosis. Deep similarity and interconnections between innate immune cells and those of liver parenchyma have been highlighted and showed to play a key role in the development of chronic liver disease. The liver can be considered as an “immune organ” because it hosts non-lymphoid cells, such as macrophage Kupffer cells, stellate and dendritic cells, and lymphoid cells. Many of these cells are components of the classic innate immune system, enabling the liver to play a major role in response to pathogens. Although the liver provides a “tolerogenic” environment, aberrant activation of innate immune signaling may trigger “harmful” inflammation that contributes to tissue injury, fibrosis, and carcinogenesis. Pathogen recognition receptors, such as toll-like receptors and nucleotide oligomerization domain-like receptors, are responsible for the recognition of immunogenic signals, and represent the major conduit for sensing hepatic and non-hepatic noxious stimuli. A pivotal role in liver inflammation is also played by cytokines, which can initiate or have a part in immune response, triggering hepatic intracellular signaling pathways. The sum of inflammatory signals and deranged substrate handling induce most of the metabolic alteration traits: insulin resistance, obesity, diabetes, hyperlipidemia, and their compounded combined effects. In this review, we discuss the relevant role of innate immune cell activation in relation to NAFLD, the metabolic complications associated to this pathology, and the possible pharmacological tools.
Meli, Rosaria; Mattace Raso, Giuseppina; Calignano, Antonio
Background: We encountered a patient who developed serous retinal detachment in one eye first, subsequently showed multiple small subretinal punctated opacity looking like acute posterior multifocal placoid pigment epitheliopathy (APMPPE) in both eyes, and had repeated recurrence.Case: A 28-year-old female presented with serous retinal detachment accompanied by multifocal small subretinal white punctated opacity at the posterior pole of the fundus in the left eye. Fluorescein angiography (FAG) revealed many small punctated hypofluorescent lesions scattered all over the eye in the early stage and multiple subretinal spotty leakage of fluorescein, subretinal accumulation of dye, and leakage of fluorescein from the optic disc in the late stage. Indocyanine green angiography (ICG) revealed small punctated hypofluorescent lesions from the early to late stage in both eyes. The disease recurred five and a half months after, and there appeared many yellowish white round opaque parts and serous retinal detachment at the posterior pole in both eyes. FAG showed a reversal phenomenon of fluorescein in both eyes. On ICG many small punctated hypofluorescent lesions were noted and in the late stage insular faint hypofluorescence consistent with serous retinal detachment, light tissue stain and fluorescent leakage in the center of the hypofluorescent lesion were also found. The disease recurred three times thereafter. At present, about two years after the first medical examination, the fundus does not show any sunset glow-like fundus, and vision of 1.2 is maintained on the right and left.Conclusion: A rare case of Harada disease complicated with APMPPE-like recurrence is presented. PMID:11341909
Furusho, F; Imaizumi, H; Takeda, M
Nephrotic syndrome (NS) is one of the most common glomerular diseases that affect children. Renal histology reveals the presence of minimal change nephrotic syndrome (MCNS) in more than 80% of these patients. Most patients with MCNS have favorable outcomes without complications. However, a few of these children have lesions of focal segmental glomerulosclerosis, suffer from severe and prolonged proteinuria, and are at high risk for complications. Complications of NS are divided into two categories: disease-associated and drug-related complications. Disease-associated complications include infections (e.g., peritonitis, sepsis, cellulitis, and chicken pox), thromboembolism (e.g., venous thromboembolism and pulmonary embolism), hypovolemic crisis (e.g., abdominal pain, tachycardia, and hypotension), cardiovascular problems (e.g., hyperlipidemia), acute renal failure, anemia, and others (e.g., hypothyroidism, hypocalcemia, bone disease, and intussusception). The main pathomechanism of disease-associated complications originates from the large loss of plasma proteins in the urine of nephrotic children. The majority of children with MCNS who respond to treatment with corticosteroids or cytotoxic agents have smaller and milder complications than those with steroid-resistant NS. Corticosteroids, alkylating agents, cyclosporin A, and mycophenolate mofetil have often been used to treat NS, and these drugs have treatment-related complications. Early detection and appropriate treatment of these complications will improve outcomes for patients with NS.
Park, Se Jin
Transcutaneous electrical nerve stimulation (TENS) is used to alleviate muscle pain, and there is some evidence it may affect healing in chronic wounds. An 80-year-old male patient with a chronic left lower extremity wound and a history of peripheral arterial disease, type 2 diabetes, hypertension, chronic obstructive pulmonary disease, and lung cancer presented for treatment. Previous protocols of care, mainly consisting of sharp debridement and daily dressing changes, had not resulted in a decrease in wound size. The patient had right and left iliac artery stenosis - not amenable to surgical intervention - and an ankle brachial index (ABI) of 0.63 on the left and 0.59 on the right lower extremities. On presentation, the wound measured 3.0 cm x 2.0 cm with a depth of 0.3 cm and a 0.5-cm tract at the 5 o'clock position. Treatment was changed to application of an ionic silver-containing Hydrofiber™ dressing and low-frequency TENS. Electrodes were applied 2 cm superior and inferior to the wound margin at a frequency of 2 Hz with a pulse width of 250 microseconds and amplitude of 33 mA. Treatment time was 45 minutes, twice daily, for 3 months, performed at home by the patient and his caregiver. After 4 weeks, wound dimensions decreased by 1.51% per day, and the wound was completely healed (100% epithelialized) after 12 weeks. At that time, the ABI of the left (treated) leg had increased to 0.71. Research is needed to determine the efficacy and effectiveness of low-frequency TENS to help clinicians provide evidenced-based treatment for wounds complicated by decreased blood flow. PMID:25019248
Yarboro, Douglas D; Smith, Robert
INTRODUCTION. Establishing protocols of the best candidates for deep brain stimulation in patients with Parkinson's disease and a greater knowledge of the technique have increased its safety profile. Yet, the complications related with implanted devices still occur with a far-from-negligible frequency and have both an economic and clinical impact. AIM. From a broad series of patients undergoing deep brain stimulation included consecutively for the treatment of their Parkinson's disease, data concerning the complications related with implanted devices were gathered and compared with those in the literature. PATIENTS AND METHODS. Altogether 124 patients with a total of 242 implanted electrodes and 252 generator replacements were included in the study. Mean follow-up time was 8.4 years (range: 3-16 years). Data on all the complications related with implanted devices were collected retrospectively. RESULTS. Findings showed that 23 implanted device-related complications occurred (17.7% of the patients): 12 (9.6%) had culture-positive ulcers, five (4%) had culture-negative ulcers, four (3.2%) were left with infections following generator replacement, one (0.8%) had a generator malfunction, and electrode migration took place in one (0.8%). Significant differences were observed as regards the effectiveness of the treatment involving surgical revision of the ulcers, which suggests that the culture-negative ulcers responded to the surgical revision better than the culture-positive ulcers (80% healing versus 16.6%; p = 0.028). CONCLUSIONS. The results observed in the series were comparable to those in the existing literature. The presence of culture-positivity in the ulcers is a factor forecasting surgical revision. PMID:25005315
de Quintana-Schmidt, C; Pascual-Sedano, B; Alvarez-Holzapfel, M J; Gironell, A; Leidinger, A; Benito, N; Rodriguez-Rodriguez, R; Molet-Teixido, J
Immunoglobulin G4-related disease (IgG4-RD) is a systemic disorder characterized by multiorgan fibrosis with IgG4-producing plasma cells, increased IgG4 serum concentration, and responsiveness to steroid therapy. Involvement of the pancreas, salivary glands, orbit, aorta, and other sites has been well documented in the literature; however, there have been limited reports of cases involving the coronary arteries. We report the case of a 53-year-old Hispanic man who was brought to the emergency center and diagnosed with sudden cardiac death. Autopsy was subsequently performed, revealing multiorgan involvement by IgG4-RD, including involvement of the coronary arteries. The inflammation and fibrosis, in combination with concomitant atherosclerotic disease, resulted in severe stenosis of the coronary arteries. Two of the coronary arteries were further occluded by thrombosis. These factors led to cardiac hypoperfusion, myocardial infarction and, ultimately, sudden cardiac death. Fatal involvement of the coronary arteries has not been previously reported, raising a new concern for a severe complication of IgG4-RD. PMID:24878025
Patel, Nimesh R; Anzalone, Mary L; Buja, L Maximilian; Elghetany, M Tarek
The 10 major articles in this special journal issue deal with literary works designated by individual educators as "still worth reading." The works discussed are (1) "Madeline" by L. Bemelmans; (2) "The Assistant" by B. Malamud; (3) "The Pitfalls for Readers of Fiction" by H. Sample, the first of the pamphlet publications by the National Council…
McLeod, Alan M., Ed.
The discovery of new infectious agents often overshadows the continuing impact and importance of well-established organisms. In the case of diarrheal disease, Entamoeba histolytica and its complications remain a major cause of morbidity and mortality in developing countries. Although it is also present in developing countries, Giardia lamblia is a primary cause of waterborne outbreaks of diarrhea in developed countries. Persons at risk of developing giardiasis in these countries include backpackers and campers who drink untreated stream water or persons who drink improperly treated municipal water containing infective cysts. Investigators have recently identified the mechanisms used by these organisms to colonize the intestinal tract and to cause disease. New methods of identification using immunologic principles have been added to the traditional microscopic methods of identification. PMID:10177206
Smith, L A
The prognostic power of myocardial perfusion imaging in pa- tients with ischemic heart disease (IHD) has been demonstrated since planar imaging. We aimed to investigate whether gated SPECT retains this value in current cardiology if compared with a complete diagnostic work-up and with more recent prognostic indicators. Methods: We selected from our database a cohort of 676 consecutive inpatients who
Alessia Gimelli; Giuseppe Rossi; Patrizia Landi; Paolo Marzullo; Giorgio Iervasi; Antonio L'Abbate
Here are some things we know: The pathogen that causes Lyme disease is Borrelia burgdorferi and it is a highly motile spirochete that belongs to a genus of bacteria that are notorious for giving rise to variant strains. Borrelia are bacteria that are associated with dozens of tick and louse-borne Relapsing Fevers that are found throughout the world. These related
Tom Grier; Lyme Writer
Review of the therapeutic management of Parkinson's disease. Report of a joint task force of the European Federation of Neurological Societies (EFNS) and the Movement Disorder Society-European Section (MDS-ES). Part II: late (complicated) Parkinson's disease.
To provide evidence-based recommendations for the management of late (complicated) Parkinson's disease (PD), based on a review of the literature. Complicated PD refers to patients suffering from the classical motor syndrome of PD along with other motor or non-motor complications, either disease-related (e.g. freezing) or treatment-related (e.g. dyskinesias or hallucinations). MEDLINE, Cochrane Library and INAHTA database literature searches were conducted. National guidelines were requested from all EFNS societies. Non-European guidelines were searched for using MEDLINE. Part II of the guidelines deals with treatment of motor and neuropsychiatric complications and autonomic disturbances. For each topic, a list of therapeutic interventions is provided, including classification of evidence. Following this, recommendations for management are given, alongside ratings of efficacy. Classifications of evidence and ratings of efficacy are made according to EFNS guidance. In cases where there is insufficient scientific evidence, a consensus statement ('good practice point') is made. PMID:17038032
Horstink, M; Tolosa, E; Bonuccelli, U; Deuschl, G; Friedman, A; Kanovsky, P; Larsen, J P; Lees, A; Oertel, W; Poewe, W; Rascol, O; Sampaio, C
The de Bruijn diagram describing those decompositions of the neighborhoods of a one dimensional cellular automaton which conform to predetermined requirements of periodicity and translational symmetry shows how to construct extended configurations satisfying the same requirements. Similar diagrams, formed by stages, describe higher dimensional automata, although they become more laborious to compute with increasing neighborhood size. The procedure is illustrated by computing some still lifes for Conway's game of Life, a widely known two dimensional cellular automaton. This paper is written in September 10, 1988.
McIntosh, Harold V.
A diagnosis of asbestosis, lung fibrosis due to asbestos exposure, was proposed in 2003 in a 64-year-old woman on the basis of the history, computed tomography appearances, lung function studies, and biometric data. This diagnosis was confirmed by the pathological examination of a lung lobe resected surgically for bronchial carcinoma in 2010. The diagnosis of asbestosis is now rarely made as a result of a substantial decrease in dust exposure over the past decades and mainly because of the interdiction of asbestos use in western countries. Currently, the most frequent thoracic manifestations of asbestos exposure are benign pleural lesions and mesothelioma. It has also become exceptional to have pathological confirmation of the diagnosis, obtained in this woman thanks to the surgical treatment of another complication of her occupational exposure. PMID:23200587
De Vuyst, P; Remmelink, M; Mekinda, Z; Thimpont, J; Dumortier, P; Gevenois, P-A
Patients with end-stage liver disease frequently require invasive cardiac procedures in preparation for liver transplantation. Because of the impaired hepatic function, these patients often have a prolonged prothrombin time and elevated international normalized ratio (INR). To determine whether an abnormal prothrombin time/INR is predictive of bleeding complications from invasive cardiac procedures, we retrospectively reviewed, for bleeding complications, the databases and case records of our series of patients with advanced cirrhosis who underwent cardiac catheterization. A total of 157 patients underwent isolated right-sided heart catheterization, and 83 underwent left-sided heart catheterization or combined left- and right-sided heart catheterization. The INR ranged from 0.93 to 2.35. No major procedure-related complications occurred. Several patients in each group required a blood transfusion for gastrointestinal bleeding but not for procedure-related bleeding. No significant change was found in the hemoglobin after right-sided or left-sided heart catheterization, and no correlation was found between the preprocedure INR and the change in postprocedure hemoglobin. When comparing patients with a normal (?1.5) and elevated (>1.5) INR, no significant difference in hemoglobin after the procedure was found in either group. In conclusion, despite an elevated INR, patients with end-stage liver disease can safely undergo invasive cardiac procedures. An elevated INR does not predict catheterization-related bleeding complications in this patient population. PMID:22728001
Townsend, Jacob C; Heard, Richard; Powers, Eric R; Reuben, Adrian
Liver cirrhosis (LC) is the final evaluative stage of chronic liver diseases with dynamic progressive process to multiple complications especially splenomegaly and esophageal varices(EV). Efforts have been made to develop non-invasive predictive models that may correlate with LC and EV. The role of liver stiffness measurement (LSM)- and spleen stiffness measurement (SSM) by transient elastography (TE) in the diagnosis of LC and prediction of EV was studied on 90 subjects selected from the outpatient clinics of Bakhash Hospitals. They were classified into three groups: GI included 10 healthy volunteers as a control group, GII included 20 chronic hepatitis (CH) patients and GIII included 60 cirrhotic patients. Patients in GIII were further subdivided equally into two subgroups A & B according to presence or absence of EV. GIII patients were evaluated by gastroscope for screening and grading of EV. All groups were subjected to complete blood picture, liver and kidney function testes and abdominal ultrasonography as well as LSM (right lobe) and SSM by using fibroscan. LS were significantly higher in LC patients as compared with CH patients and controls. At a cutoff value of 9.8 kPa, sensitivity, specificity, positive predictive values (PPV) and negative predictive values (NPV) for LC were 90%, 73%, 55% &78% respectively. At a cutoff value of 17.75 kPa (no varices vs. varices at any grade), sensitivity, specificity, PPV & NPV for prediction of EV were 92%, 46.2%, 73% & 67.2% respectively. LS at cut off values of 14.4 KPa predicted the splenomegaly. SSM at 50.4 KPa being the best cut-off value for prediction of varices with sensitivity, specificity, PPV, NPV & accuracy were 81.2%, 73.2%, 88.7%, 48.8% & 79.4% respectively. Combination of LS >17 KPa & SS > 52, predicted EV with 87.6% diagnostic accuracy. As regard EV prediction, combination of LSM & SSM had a highest diagnostic accuracy than PSR (76.9% vs. 87.6). PMID:22662598
This paper reports that reports of damage caused by Hurricane Andrew were leveling off last week at the U.S. Minerals Management Service as Gulf of Mexico operators pressed ahead with repairs. The hurricane struck South Florida Aug. 4, churned west into the gulf, then swung north and hit the South Louisiana coast Aug. 5. By the close of business Sept. 8 MMS had received damage reports covering 83 pipeline segments and 193 platforms and satellite installations. MMS last week estimated about 500 MMcfd of gas production had been restored in the gulf and 100,000-150,000 b/d of oil. Production still lost as a result of Andrew was estimated at 2-2.5 bcfd of gas and 90,000-120 b/d of oil. MMS estimates Gulf of Mexico wells before the storm were producing about 12.5-13 bcfd of gas and 750,000 b/d of oil.
Low-density lipoprotein (LDL) oxidation, the immune response it provokes, and lipoprotein subclasses measured by nuclear magnetic resonance (NMR) spectroscopy have explained some of the enhanced coronary artery disease (CAD) risks in Type 1 diabetes. We examined whether cellular adhesion molecules further improve CAD prediction.Participants were identified from the Epidemiology of Diabetes Complications (EDC) cohort, a 10-year prospective study of childhood-onset
Tina Costacou; Maria F. Lopes-Virella; Janice C. Zgibor; Gabriel Virella; Jim Otvos; Michael Walsh; Trevor J. Orchard
This study examined the association between limited joint mobility (LJM) and diabetic control, atherosclerotic vascular disease and other diabetic complications in non–insulin-dependent diabetic (NIDDM) patients. LJM was studied in 139 [age (mean ± SD) 61.3 ± 12.3 years] NIDDM patients. Limitation of several joints was examined with a goniometer and LJM was classified by the Rosenbloom method. The NIDDM patients
Perttu E. T. Arkkila; Ilkka M. Kantola; Jorma S. A. Viikari
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis has been recently recognized in Graves' disease patients treated with propylthiouracil. We have experienced three adult cases of Graves' disease with main features being renal derangements. All three patients, who were between the ages of 22 and 82 years, had been treated with propylthiouracil for 2 to 5 years after a diagnosis of Graves' disease.
Masayuki Tanemoto; Hiroshi Miyakawa; Junichi Hanai; Masako Yago; Masafumi Kitaoka; Shunya Uchida
Disseminated blastomycosis can be a devastating disease, affecting multiple organ systems, including the musculoskeletal system. Osteomyelitis from disseminated disease can be difficult to eradicate but is particularly important to successfully manage in the load-bearing bones of the lower extremity. We present a staged protocol for salvage of blastomycotic calcaneal osteomyelitis in the presence of disseminated disease. PMID:24726794
Bibbo, Christopher; Spellman, Jeanne
Pregnancy induces a number of physiologic changes that affect the hematologic indices, either directly or indirectly. Recognizing and treating hematologic disorders that occur during pregnancy is difficult owing to the paucity of evidence available to guide consultants. This paper specifically reviews the diagnosis and management of benign hematologic disorders occurring during pregnancy. Anemia secondary to iron deficiency is the most frequent hematologic complication and is easily treated with oral iron formulations,; however care must be taken not to miss other causes of anemia, such as sickle cell disease. Thrombocytopenia is also a common reason for consulting the hematologist and distinguishing gestational thrombocytopenia from immune thrombocytopenia (ITP), preeclampsia, HELLP syndrome, or thrombotic thrombocytopenic purpura (TTP) is essential since the treatment differs widely. Occasionally the management of mother and infant involves the expeditious recognition of neonatal alloimmune thrombocytopenia (NAIT), a condition that is responsible for severe life-threatening bleeding of the newborn. Additionally, inherited and acquired bleeding disorders affect pregnant women disproportionately and often require careful monitoring of coagulation parameters in order to prevent bleeding in the puerperium. Finally, venous thromboembolism (VTE) during pregnancy is still largely responsible for mortality during pregnancy and the diagnosis, treatment options and guidelines for prevention of VTE during pregnancy are explored.
Townsley, Danielle M.
Background Few studies have evaluated the association between incident chronic kidney disease (CKD) and related complications, especially in elderly population. We attempted to verify the association between GFR and concurrent CKD complications and elucidate the temporal relationship between incident CKD and new CKD complications in a community-based prospective elderly cohort. Method We analyzed the available data from 984 participants in the Korean Longitudinal Study on Health and Aging. Participants were categorized into 6 groups according to eGFR at baseline examination (?90, 75–89, 60–74, 45–59, 30–44, and <30 ml/min/1.73 m2). Result The mean age of study population was 76 ± 9.1 years and mean eGFR was 72.3 ± 17.0 ml/min/1.73 m2. Compared to eGFR group 1, the odds ratio (OR) for hypertension was 2.363 (95% CI, 1.299-4.298) in group 4, 5.191 (2.074-12.995) in group 5, and 13.675 (1.611-115.806) in group 6; for anemia, 7.842 (2.265-27.153) in group 5 and 13.019 (2.920-58.047) in group 6; for acidosis, 69.580 (6.770-715.147) in group 6; and for hyperkalemia, 19.177 (1.798-204.474) in group 6. Over a 5-year observational period, CKD developed in 34 (9.6%) among 354 participants with GFR ? 60 ml/min/1.73 m2 at basal examination. The estimated mean number of new complications according to analysis of co-variance was 0.52 (95% CI, 0.35–0.68) in subjects with incident CKD and 0.24 (0.19–0.29) in subjects without CKD (p = 0.002). Subjects with incident CKD had a 2.792-fold higher risk of developing new CKD complications. A GFR level of 52.4 ml/min/1.73 m2 (p = 0.032) predicted the development of a new CKD complication with a 90% sensitivity. Conclusion In an elderly prospective cohort, CKD diagnosed by current criteria is related to an increase in the number of concurrent CKD complications and the development of new CKD complications.
Ahn, Shin Young; Ryu, Jiwon; Baek, Seon Ha; Kim, Sejoong; Na, Ki Young; Kim, Ki Woong; Chae, Dong-Wan; Chin, Ho Jun
Although it has been more than a year since the FDA approved the use of olestra in certain foods, this fat substitute, a mixture of sucrose polyesters, is still controversial. It would seem that a fat substitute that is heat stable and has an acceptable flavor and texture would be welcomed enthusiastically in a country where increasing numbers of people, young and old, exceed their ideal body weight. Obesity and diets containing high levels of fat have been linked to numerous health problems, including cardiovascular diseases, certain types of cancer, and adult-onset diabetes; they may also exacerbate some chronic problems such as arthritis in joints of the lower extremities. Nevertheless, some scientists and consumer groups question olestra's safety and usefulness.
The oldest isolated pulsar ever detected in X-rays has been found with NASA's Chandra X-ray Observatory. This very old and exotic object turns out to be surprisingly active. The pulsar, PSR J0108-1431 (J0108 for short) is about 200 million years old. Among isolated pulsars -- ones that have not been spun-up in a binary system -- it is over 10 times older than the previous record holder with an X-ray detection. At a distance of 770 light years, it is one of the nearest pulsars known. Pulsars are born when stars that are much more massive than the Sun collapse in supernova explosions, leaving behind a small, incredibly weighty core, known as a neutron star. At birth, these neutron stars, which contain the densest material known in the Universe, are spinning rapidly, up to a hundred revolutions per second. As the rotating beams of their radiation are seen as pulses by distant observers, similar to a lighthouse beam, astronomers call them "pulsars". Astronomers observe a gradual slowing of the rotation of the pulsars as they radiate energy away. Radio observations of J0108 show it to be one of the oldest and faintest pulsars known, spinning only slightly faster than one revolution per second. The surprise came when a team of astronomers led by George Pavlov of Penn State University observed J0108 in X-rays with Chandra. They found that it glows much brighter in X-rays than was expected for a pulsar of such advanced years. People Who Read This Also Read... Chandra Data Reveal Rapidly Whirling Black Holes Milky Way’s Giant Black Hole Awoke from Slumber 300 Years Ago Erratic Black Hole Regulates Itself Celebrate the International Year of Astronomy Some of the energy that J0108 is losing as it spins more slowly is converted into X-ray radiation. The efficiency of this process for J0108 is found to be higher than for any other known pulsar. "This pulsar is pumping out high-energy radiation much more efficiently than its younger cousins," said Pavlov. "So, although it's clearly fading as it ages, it is still more than holding its own with the younger generations." It's likely that two forms of X-ray emission are produced in J0108: emission from particles spiraling around magnetic fields, and emission from heated areas around the neutron star's magnetic poles. Measuring the temperature and size of these heated regions can provide valuable insight into the extraordinary properties of the neutron star surface and the process by which charged particles are accelerated by the pulsar. The younger, bright pulsars commonly detected by radio and X-ray telescopes are not representative of the full population of objects, so observing objects like J0108 helps astronomers see a more complete range of behavior. At its advanced age, J0108 is close to the so-called "pulsar death line," where its pulsed radiation is expected to switch off and it will become much harder, if not impossible, to observe. "We can now explore the properties of this pulsar in a regime where no other pulsar has been detected outside the radio range," said co-author Oleg Kargaltsev of the University of Florida. "To understand the properties of 'dying pulsars,' it is important to study their radiation in X-rays. Our finding that a very old pulsar can be such an efficient X-ray emitter gives us hope to discover new nearby pulsars of this class via their X-ray emission." The Chandra observations were reported by Pavlov and colleagues in the January 20, 2009, issue of The Astrophysical Journal. However, the extreme nature of J0108 was not fully apparent until a new distance to it was reported on February 6 in the PhD thesis of Adam Deller from Swinburne University in Australia. The new distance is both larger and more accurate than the distance used in the Chandra paper, showing that J0108 was brighter in X-rays than previously thought. "Suddenly this pulsar became the record holder for its ability to make X-rays," said Pavlov, "and our result became even more interesting without us doing much extra w
Background Cytokine-mediated endothelial activation pathway is a known mechanism of pathogenesis employed by Plasmodium falciparum to induce severe disease symptoms in human host. Though considered benign, complicated cases of Plasmodium vivax are being reported worldwide and from Pakistan. It has been hypothesized that P.vivax utilizes similar mechanism of pathogenesis, as that of P.falciparum for manifestations of severe malaria. Therefore, the main objective of this study was to characterize the role of cytokines and endothelial activation markers in complicated Plasmodium vivax isolates from Pakistan. Methods and Principle Findings A case control study using plasma samples from well-characterized groups suffering from P.vivax infection including uncomplicated cases (n=100), complicated cases (n=82) and healthy controls (n=100) were investigated. Base line levels of Tumor necrosis factor-? (TNF-?), Interleukin-6 (IL-6), Interleukin-10 (IL-10), Intercellular adhesion molecule-1 (ICAM-1), Vascular adhesion molecule-1(VCAM-1) and E-selectin were measured by ELISA. Correlation of cytokines and endothelial activation markers was done using Spearman’s correlation analysis. Furthermore, significance of these biomarkers as indicators of disease severity was also analyzed. The results showed that TNF-?, IL-10, ICAM-1and VCAM-1 were 3-fold, 3.7 fold and 2 fold increased between uncomplicated and complicated cases. Comparison of healthy controls with uncomplicated cases showed no significant difference in TNF-? concentrations while IL-6, IL-10, ICAM-1, VCAM-1 and E-selectin were found to be elevated respectively. In addition, significant positive correlation was observed between TNF-? and IL-10/ ICAM-1, IL-6 and IL-10, ICAM-1 and VCAM-1.A Receiver operating curve (ROC) was generated which showed that TNF-?, IL-10, ICAM-1 and VCAM-1 were the best individual predictors of complicated P.vivax malaria. Conclusion The results suggest that though endothelial adhesion molecules are inducible by pro-inflammatory cytokine TNF-?, however, cytokine-mediated endothelial activation pathway is not clearly demonstrated as a mechanism of pathogenesis in complicated P.vivax malaria cases from Pakistan.
Raza, Afsheen; Ghanchi, Najia K.; Sarwar Zubairi, Ali bin; Raheem, Ahmed; Nizami, Sobia; Beg, Mohammad Asim
People with type 2 diabetes are at increased risk of many complications, which are mainly due to complex and interconnected mechanisms such as hyperglycemia, insulino-resistance, low-grade inflammation and accelerated atherogenesis. Cardi-cerebrovascular disease are frequently associated to type 2 diabetes and may become life threatening, particularly coronaropathy, stroke and heart failure. Their clinical picture are sometimes atypical and silencious for a long time. Type 2 diabetes must be considered as an independent cardiovascular risk factor. Nephropathy is frequent in type 2 diabetes but has a mixed origin. Now it is the highest cause of end-stage renal disease. Better metabolic and blood pressure control and an improved management of microalbuminuria are able to slowdown the course of the disease. Retinopathy which is paradoxically slightly progressive must however be screened and treated in these rather old patients which are globally at high ophthalmologic risk. Diabetic foot is a severe complication secondary to microangiopathy, microangiopathy and neuropathy. It may be considered as a super-complication of several complications. Its screening must be done on a routine basis. Some cancer may be considered as an emerging complication of type 2 diabetes as well as cognitive decline, sleep apnea syndrome, mood disorders and bone metabolism impairments. Most of the type 2 diabetes complications may be prevented by a strategy combining a systematic screening and multi-interventional therapies. PMID:23528336
Type 1 Gaucher disease (GD1) is an inherited lysosomal storage disease, which is often managed by enzyme replacement therapy\\u000a (ERT). The bone response to ERT is usually slower than visceral and hematological responses. There is uncertainty as to whether\\u000a an increase in the dosage of ERT has a beneficial effect. The aim of our study was to determine whether or
Siavash Piran; Dominick Amato
We report on a 32-year-old man with a history of chronic lower abdominal pain and urogenital symptoms, leading to the diagnosis of coloseminal fistula complicating diverticular disease. We reviewed the literature on this rare clinical entity and would like to stress the role of pelvic imaging with rectal contrast to investigate complicated forms of diverticular disease. PMID:24457980
Barret, Maximilien; Cuenod, Charles-André; Jian, Raymond; Cellier, Christophe; Berger, Anne
Background and aims: Over the past two decades in Taiwan, pyogenic liver abscess has usually been caused by a single microorganism, Klebsiella pneumoniae, and is frequently associated with the serious complication of endophthalmitis, especially in diabetic patients. However, the relationship between the clinical presentation and bacterial factors remains unclear. The aim of this study was to investigate the clinical features of patients and the serotype and ribotype of K pneumoniae liver abscess. Methods: From July 1991 to June 1998, a total of 134 cases of K pneumoniae liver abscess with 248 K pneumoniae isolates from the same patients were collected from two large medical centres in northern Taiwan. Clinical data were collected from medical records. Serotyping and ribotyping were performed using the countercurrent immunoelectrophoresis method and automated Riboprinter. Results: Serotyping revealed that the most common serotypes were K1 (63.4%) and K2 (14.2%). K1 isolates occurred at a significantly higher frequency (p<0.01) than all other serotypes. Among 134 patients, 105 (78.4%) had suffered from diabetes mellitus for 3–15 years. Fourteen patients (10.4%) had metastatic infection to the eye causing septic endophthalmitis. Liver aspirates, and blood and vitreous pus cultures yielded the same serotype of K pneumoniae in all patients. Among patients with septic endophthalmitis, 92.3% (13/14) were diabetic, and 85.7% (12/14) of the isolates belonged to serotype K1. For molecular typing, different degrees of genetic polymorphism among isolates with the same K1 serotype suggested no particular prevalence of any one strain in K pneumoniae liver abscess. Conclusion: K pneumoniae serotype K1 was significantly associated with liver abscess and the complication of endophthalmitis, especially in diabetic patients. Physicians should request an immediate report of serotyping and susceptibility test results simultaneously if a diagnosis of pyogenic liver abscess has been made so that early and appropriate management for possible complications will not be delayed. The use of ceftriaxone because of its higher concentration in the aqueous humor is suggested to decrease the chance of septic endophthalmitis.
Fung, C-P; Chang, F-Y; Lee, S-C; Hu, B-S; Kuo, B I-T; Liu, C-Y; Ho, M; Siu, L K
The poor clinical conditions associated with end-stage cirrhosis, pre-existing pulmonary abnormalities, and high comorbidity rates in patients with high Model for End-Stage Liver Disease scores are all well-recognized factors that increase the risk of pulmonary complications after orthotopic liver transplantation (OLT) surgery. Many intraoperative and postoperative events, such as fluid overload, massive transfusion of blood products, hemodynamic instability, unexpected coagulation abnormalities, renal dysfunction, and serious adverse effects of reperfusion syndrome, are other factors that predispose an individual to postoperative respiratory disorders. Despite advances in surgical techniques and anesthesiological management, the lung may still suffer throughout the perioperative period from various types of injury and ventilatory impairment, with different clinical outcomes. Pulmonary complications after OLT can be classified as infectious or non-infectious. Pleural effusion, atelectasis, pulmonary edema, respiratory distress syndrome, and pneumonia may contribute considerably to early morbidity and mortality in liver transplant patients. It is of paramount importance to accurately identify lung disorders because infectious pulmonary complications warrant speedy and aggressive treatment to prevent diffuse lung injury and the risk of evolution into multisystem organ failure. This review discusses the most common perioperative factors that predispose an individual to postoperative pulmonary complications and these complications’ early clinical manifestations after OLT and influence on patient outcome.
Feltracco, Paolo; Carollo, Cristiana; Barbieri, Stefania; Pettenuzzo, Tommaso; Ori, Carlo
Dermatomyositis (DM) is occasionally complicated by interstitial lung disease. Acute/subacute interstitial pneumonia (A/SIP) with DM is intractable and life threatening. Clinically amyopathic dermatomyositis (C-ADM) is also reported to be complicated with A/SIP, especially in those patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody. In the present cases, we indicate that serum ferritin level correlated with activity of A/SIP with DM. Two patients, a 65-year-old woman and a 30-year-old woman, were diagnosed with anti-MDA5 antibody-associated A/SIP with DM. Serum ferritin was high, 1600 and 770 mg/dl, respectively, on admission. Immunosuppressive therapy ameliorated A/SIP in both cases. Similarly, serum ferritin was also decreasing. However, A/SIP was recurrent and progressive, and serum ferritin was also increasing again in one case. In conclusion, serum ferritin correlates with disease activity of anti-MDA5 antibody-associated A/SIP with DM. Intensity of treatment may be decided according to serum ferritin level. PMID:21052763
Gono, Takahisa; Kawaguchi, Yasushi; Ozeki, Eri; Ota, Yuko; Satoh, Takashi; Kuwana, Masataka; Hara, Masako; Yamanaka, Hisashi
C-reactive protein (CRP), fibrinogen, and interleukin-6 (IL-6) are associated with cardiovascular disease (CVD) and death in general populations. However, studies of these factors in type 2 diabetes are limited. We studied their associations with the risk of major macrovascular events, microvascular complications, and mortality in patients with type 2 diabetes who participated in the Action in Diabetes and Vascular Disease: Preterax and Diamicron Modified Release Controlled Evaluation (ADVANCE) Study. Plasma CRP, fibrinogen, and IL-6 levels were determined in a case-cohort study (n = 3,865) nested within the 11,140 men and women with type 2 diabetes and baseline CVD or risk factors in the ADVANCE Study. All three biomarkers of inflammation were associated with an increased risk of macrovascular events and death in analyses adjusted for age, sex, and treatment groups. After further adjustment, only IL-6 was an independent predictor of macrovascular events (hazard ratio per SD increase 1.37 [95% CI 1.24-1.51]) and death (1.35 [1.23-1.49]). IL-6 significantly improved the prediction of macrovascular events and death. After adjustment, none of the markers predicted microvascular complications. We conclude that IL-6 levels, but not CRP or fibrinogen levels, add significantly to the prediction of macrovascular events and mortality in individuals with type 2 diabetes who have baseline CVD or risk factors. PMID:24222348
Lowe, Gordon; Woodward, Mark; Hillis, Graham; Rumley, Ann; Li, Qiang; Harrap, Stephen; Marre, Michel; Hamet, Pavel; Patel, Anushka; Poulter, Neil; Chalmers, John
Advances in surgical techniques and immunosuppression have made orthotopic liver transplantation a first-line treatment for many patients with end-stage liver disease. The early detection and treatment of postoperative complications has contributed significantly to improved graft and patient survival with imaging playing a critical role in detection. Complications that can lead to graft failure or patient mortality include vascular abnormalities, biliary abnormalities, allograft rejection, and recurrent or post-transplant malignancy. Vascular abnormalities include stenosis and thrombosis of the hepatic artery, portal vein, and inferior vena cava, as well as hepatic artery pseudoaneurysm, arteriovenous fistula, and celiac stenosis. Biliary abnormalities include strictures, bile leak, obstruction, recurrent disease, and infection. While imaging is not used to diagnose allograft rejection, it plays an important role in identifying complications that can mimic rejection. Ultrasound is routinely performed as the initial imaging modality for the detection and follow-up of both early and delayed complications. Cholangiography and magnetic resonance cholangiopancreatography are used to characterize biliary complications and computed tomography is used to confirm abnormal findings on ultrasound or for the evaluation of postoperative collections. The purpose of this article is to describe and illustrate the imaging appearances and management of complications associated with liver transplantation. PMID:23644931
Itri, Jason N; Heller, Matthew T; Tublin, Mitchell E
The prevalence of obesity and diabetes is increasing dramatically. Currently, 800,000 patients are suffering from diabetes mellitus in Austria. Chronic hyperglycemia results in micro- and macrovascular complications, which reduce life expectancy up to 8 years. Furthermore, diabetes is among the most important risk factors for premature atherosclerosis and coronary artery disease. The incidence of coronary artery disease in diabetics is relatively high with about 146 cases per 10,000 patient years. Apart, it could be demonstrated that the presence of diabetes mellitus worsens the prognosis after an acute coronary syndrome. Considering ischemic stroke, the situation is nearly the same, as it is known that diabetes mellitus increases the risk for ischemic stroke events up to 5 times. Beside the macrovascular complications, microvascular complications like diabetic retinopathy, diabetic nephropathy and diabetic neuropathy also play a critical role. Retinopathy can be detected in nearly every patient after a diabetes duration of 20 years. Diabetic nephropathy, which is a major complication of diabetes mellitus, accounts for 19% of end stage renal disease. Microalbuminuria, which is an early marker of diabetic nephropathy, can be found in 30% of the patients after 10 years of diabetes. Due to the severity of the diabetic complications an early intensified antidiabetic treatment is highly important for the prevention of micro- and macrovascular events. PMID:20229155
Resl, Michael; Clodi, Martin
Type 1 and Type 2 diabetes are complex diseases associated with multiple complications, and both genetic and environmental factors have been implicated in these pathologies. While numerous studies have provided a wealth of knowledge regarding the genetics of diabetes, the mechanistic pathways leading to diabetes and its complications remain only partly understood. Studying the role of epigenetics in diabetic complications can provide valuable new insights to clarify the interplay between genes and the environment. DNA methylation and histone modifications in nuclear chromatin can generate epigenetic information as another layer of gene transcriptional regulation sensitive to environmental signals. Recent evidence shows that key biochemical pathways and epigenetic chromatin histone methylation patterns are altered in target cells under diabetic conditions and might also be involved in the metabolic memory phenomenon noted in clinical trials and animal studies. New therapeutic targets and treatment options could be uncovered from an in-depth study of the epigenetic mechanisms that might perpetuate diabetic complications despite glycemic control.
Villeneuve, Louisa M; Natarajan, Rama
Liposuction is the most frequent aesthetic procedure worldwide for adipose tissue reduction and treatment of lipedema. It is being employed with increasing frequency. In 2010, in the USA more than 200.000 liposuctions were performed. Apart from aesthetic indications, liposuction also is suitable for treatment of benign adipose tissue diseases. This intervention is not a simple procedure but requires extensive knowledge and experience to prevent irreversible medical or aesthetic complications. Severe complications including necrotizing fasciitis, toxic shock syndrome, hemorrhage, perforation of inner organs und pulmonary embolism - some even with lethal outcome - occasionally have been reported. These complications were mostly due to inadequate hygiene measures, inappropriate patient selection, use of excessive local anesthesia during mega-liposuction (tumescent technique) and inadequate post-operative surveillance. The complication rate usually reflects a lack of medical experience as well as technical inadequacies. PMID:23494094
Sattler, G; Eichner, S
In the United States and many other developed countries, active immunization of children has virtually eliminated poliomyelitis, measles, rubella, tetanus, and other diseases, such as disease due to Haemophilus influenzae type b. Individual vaccines can produce systemic or neurologic reactions ranging from minor events, such as pain and erythema at the injection site, to major complications, such as seizures, shock, encephalopathy, or death. Immunization programs have also generated considerable controversy, as witnessed by recent concerns regarding the relationship between vaccines or their constituents and autism or multiple sclerosis. This review summarizes current information regarding vaccines, the diseases that they prevent, and the potential relationships between vaccines and neurologic disease. PMID:15446387
Bale, James F
AIM—To investigate gastric pacemaker activity in gastro-oesophageal reflux disease using the electrogastrogram.?PATIENTS—Forty patients with gastro-oesophageal reflux disease (20 with acid reflux, 20 with the additional symptom of food regurgitation) and 30 asymptomatic controls.?METHODS—Patients were studied using an electrogastrogram, oesophageal manometry, and 24 hour ambulatory oesophageal pH analysis.?RESULTS—An abnormal electrogastrogram was recorded in two (7%) controls, two (10%) patients with acid reflux, and 10 (50%) patients with food regurgitation. Food regurgitators had significantly more gastric dysrhythmias (tachygastrias) both before (p<0.02) and after (p<0.01) a test meal. Gastric pacemaker activity was also significantly less stable following the test meal in food regurgitators (p<0.003). Patients with food regurgitation and an abnormal electrogastrogram had higher oesophageal acid exposure than those with a normal electrogastrogram (p<0.05).?CONCLUSIONS—The electrogastrogram is usually normal in gastro-oesophageal reflux disease but an abnormal rhythm occurred in half of our patients with the additional symptom of food regurgitation. Furthermore, an abnormal electrogastrogram is associated with increased oesophageal acid exposure.???Keywords: gastro-oesophageal reflux; gastrointestinal motility; electrogastrogram
Leahy, A; Besherdas, K; Clayman, C; Mason, I; Epstein, O
Infective endocarditis (IE) is a lethal disease if not promptly treated with antibiotics, either in association with surgery or not. The incidence of disease has not decreased over the last decades due to the change of risk conditions. Complications of IE may involve cardiac structures when the infection spreads within the heart, or extra cardiac ones when the cause is usually from embolic origin; they may also be due to medical treatment or to the septic condition itself. A variety of complications may occur in most of patients. The literature reports one complication of IE in 57%, two in 26% and three or more in about 14% of patients examined. The frequency of specific complications depends on variables as the infecting pathogen, duration of disease before therapy and type of treatment. However it is often difficult to assess the true incidence of complications because the published reviews in literature are frequently based on retrospective chart reviews and different diagnostic criteria are used. The decision over either indication or timing of surgery should be individualized and based on a multidisciplinary approach involving at least cardiologists and cardiac surgeons. Congestive heart failure (CHF) is the most important complication of IE, which has the greatest impact on prognosis. Periannular abscesses are a relatively common complication of IE (42% to 85% of cases during surgery or at autopsy respectively), associated with a higher morbidity and mortality. Systemic embolization occurs in 22% to 50% of cases; emboli may involve major arteries, mostly affecting the central nervous system, but also other organs. Splenic abscess is a rare complication of IE, due to direct seeding of spleen by an embolus or bacterial seeding of a bland infarction. Neurological complications develop in 20% to 40% of patients with IE and represent a dangerous subset of complications. Mycotic aneurysms are rare, resulting from diffusion of infection to the vessel wall. Actually the clinical profile, the best treatment (medical or surgical approach) and outcome of complicated IE are not well defined. Changing trends in aetiology of IE with emerging infections from Staphylococci, bacteria of the HACEK group and Fungi have resulted in an increased frequency of culture negative IE. Sepsis or persistent fever despite appropriate antimicrobial therapy, recurrent emboli, heart failure or new pathologic murmurs suggest haemodynamic impairment and/or infection extending beyond the valve leaflet or prosthetic valvular annulus. The course of the disease will consequently get worse with an increasing need of surgery. Patients who develop abscesses are more likely to undergo surgery than those who do not (84-91% vs 36%), and also their in-hospital mortality rate is higher (19% vs 11%). A prompt detection of complications often allows an earlier surgical treatment which represents the best way to improve the outcome. The introduction of molecular methods techniques has increased the ability to identify the causal agents of IE, mostly in cases of culture negative endocarditis. Echocardiography, mainly from transesophageal (TEE) approach, has significantly improved the evaluation of IE allowing to detect the specific signs of the disease as vegetations, abscesses, valve insufficiency, prosthetic valve dehiscence, fistulas. In our 3rd referral Hospital (Lancisi Heart Hospital, Ancona, Italy) we performed a follow-up (mean 8.26 years) of 15 patients with periannular complications associated with IE. The long term follow-up showed low mortality rate, high incidence of reintervention, improved New York Heart Association (NYHA) class in survivors and no changes of the lesions at the echocardiographic examination, suggesting that periannular complications have not significantly influenced the overall survival in our patients at the follow-up. PMID:19751182
Mocchegiani, R; Nataloni, M
Neuromyelitis optica (NMO) is usually a relapsing demyelinating disease of the central nervous system associated with optic neuritis, transverse myelitis involving three or more contiguous spinal cord segments, and seropositivity for NMO-IgG antibody. NMO is often mistaken for multiple sclerosis and there are relatively sporadic publications about NMO and overlapping systemic or organ-specific autoimmune diseases, such as systemic lupus erythematosus (SLE). We described a unique case of a 25-year-old Arab young woman who was diagnosed with SLE, depending on clinical, laboratory investigations and after she had fulfilled the diagnostic criteria for SLE and had presented the following findings: constitutional findings (fatigue, fever, and arthralgia); dermatologic finding (photosensitivity and butterfly rash); chronic renal failure (proteinuria up to 400 mg in 24 hours); hematologic and antinuclear antibodies (positivity for antinuclear factor (ANF), anti-double-stranded DNA antibodies, direct Coombs, ANA and anti-DNA, low C4 and C3, aCL by IgG and IgM). Recently, she presented with several episodes of transverse myelitis and optic neuritis. Clinical, radiological, and laboratory findings especially seropositivity for NMO-IgG were compatible with NMO. Accurate diagnosis is critical to facilitate initiation of immunosuppressive therapy for attack prevention. This case illustrates that NMO may be associated with SLE.
Adawi, Mohammad; Bisharat, Bishara; Bowirrat, Abdalla
Purpose The aim of this study was to compare two different treatment methods for pilonidal sinus with respect to complications, recurrence, and patient quality of life. Methods Five hundred forty-nine patients who underwent surgery for pilonidal sinus between January 2007 and August 2012 were included in this study. The patients were classified into group I (excision and primary closure) and group II (Limberg flap). Results There was no significant difference in the mean age and gender of the patients between groups I and II (P = 0.512 and P = 0.472). The duration of surgical operation was lower in group I (P < 0.001). There was no significant difference in hospitalization time after surgery, minor complications, and recurrence between the groups (P = 0.674, P = 1.000, and P = 1.000, respectively). The time required for pain-free walking, urinating, and returning to work was significantly lower in group II (P < 0.001, P < 0.001, and P < 0.001, respectively). The patients in group I stated that they were more satisfied in terms of aesthetics (P < 0.001). Conclusion According to the results of this study, Limberg flap method has better outcomes compared with excision and primary closure. Therefore, we recommend Limberg flap for treatment of pilonidal sinus disease.
Karaca, Ahmet Serdar; Ali, R?dvan; Capar, Muzaffer
\\u000a After hematopoietic stem cell transplant (HSCT), up to 60% of patients develop pulmonary complications. In spite of antibacterial,\\u000a antiviral, and antifungal prophylaxis, reduced host defenses render the HSCT patient vulnerable to pulmonary and other infections\\u000a in the early weeks and even months post-transplantation. This chapter will suggest an integrative approach followed by a description\\u000a of the most common pulmonary syndromes
Tarek Eid; Alan F. Barker
\\u000a Gastrointestinal and hepatic complications are common in the hematopoietic stem cell transplant (HSCT) patient. The agents\\u000a used in the conditioning regimen induce direct disruption of the intestinal barrier as well as indirect damage from cytokine\\u000a release and generalized inflammatory state. These events lead to permeation of bacteria and endotoxins through the bowel wall,\\u000a with subsequent organ damage and increased risk
Sickle cell disease (SCD) is characterized by a complex vasculopathy, consisting of endothelial dysfunction and increased arterial stiffness, with a global effect on cardiovascular function. The hypercoagulable state may result from chronic hemolysis and circulating cell-derived microparticles (MPs) originating mainly from activated platelets and erythrocytes. We measured the levels of platelet and erythrocyte-derived MPs (PMPs and ErMPs) in 50 young SCD patients compared with 40 age- and sex-matched healthy controls and assessed their relation to clinicopathological characteristics and aortic elastic properties. Patients were studied stressing on the occurrence of sickling crisis, transfusion history, hydroxyurea therapy, hematological, and coagulation profile as well as flow cytometric expression of PMPs (CD41b(+)) and ErMPs (glycophorin A(+)). Echocardiography was performed to assess aortic stiffness and distensibility, left ventricular function and pulmonary artery pressure. Both PMPs and ErMPs were significantly elevated in SCD patients compared with control group (p?0.001). SCD patients had significantly elevated d-dimer and von Willebrand factor antigen (vWF Ag) levels with lower antithrombin III compared with controls (p?0.001). Aortic stiffness index and pulmonary artery pressure were significantly higher in SCD (p?0.001), whereas aortic strain and aortic distensibility were significantly lower (p?0.001) compared with controls. MPs levels were significantly increased in SCD patients with pulmonary hypertension, acute chest syndrome, and stroke as well as those who had history of thrombosis or splenectomy (p?0.001). Also, patients in sickling crisis during the study had higher PMPs and ErMPs levels than those in steady state (p?0.001). Patients on hydroxyurea therapy had lower MPs levels than untreated patients (p?0.001). PMPs and ErMPs were positively correlated with disease duration, transfusion index, white blood cell count, HbS, markers of hemolysis, serum ferritin, D-dimer, and vWF Ag, whereas negatively correlated with hemoglobin and HbF levels (p?0.05). Both PMPs and ErMPs levels were positively correlated with aortic stiffness, pulmonary artery pressure, and tricuspid regurgitant velocity (p?0.05) while negatively correlated with aortic distensibility. We suggest that PMPs and ErMPs overproduction may be considered a potential biological marker for vascular dysfunction and disease severity in SCD and may be implicated in the pathogenesis of coagulation abnormalities encountered in those patients. Their levels are closely related to sickling crisis, pulmonary hypertension, markers of hemolysis, fibrinolysis, and iron overload. Therefore, quantification of MPs in SCD may provide utility for identifying patients who are at increased risk of thrombotic events or cardiovascular abnormalities and would help to monitor response to hydroxyurea therapy. PMID:23249216
Tantawy, Azza Abdel Gawad; Adly, Amira Abdel Moneam; Ismail, Eman Abdel Rahman; Habeeb, Nevin Mamdouh; Farouk, Amal
Gastrointestinal complications are common in patients undergoing various forms of cancer treatment, including chemotherapy, radiation therapy, and molecular-targeted therapies. Many of these complications are life-threatening and require prompt diagnosis and treatment. Complications of oncologic therapy can occur in the esophagus (esophagitis, strictures, bacterial, viral and fungal infections), upper gastrointestinal tract (mucositis, bleeding, nausea and vomiting), colon (diarrhea, graft–versus–host disease, colitis
Marta Davila; Robert S Bresalier
Background: Bleeding peptic ulcer (BPU) frequently occurs in the absence of preceding dyspeptic symptoms. We have observed that patients with BPU had a diminished symptom response to nutrient challenge test compared to uncomplicated peptic ulcer disease (uPUD). We postulated that more symptoms are manifest in patients with uPUD than BPU because there are greater derangements in gastric motor function. Aim: To assess gastric emptying in patients with BPU, uPUD and healthy controls (HC). Methods: We studied 17 patients with BPU, 10 with uPUD, and 15 HC. After an 8-hour fast, subjects ingested 200 ml of an enteral feeding solution, containing 5 MBq (99m)Tc-rhenium sulphide colloid, every 5 min up to a cumulative volume of 800 ml. Gastric emptying was measured by scintigraphy for the total, proximal and distal stomach. Results: Patients with uPUD had significantly higher gastric retention in the proximal and total stomach at 100 min than HC and BPU, while BPU had similar percent retention to HC. Patients with uPUD had significantly higher cumulative symptom response to the nutrient challenge than did HC and BPU, while BPU had similar symptom responses to HC. Conclusions: Patients with uPUD have significantly delayed gastric emptying compared to HC and BPU. Data suggest that in addition to alterations of visceral sensory function, altered gastric motor function occurs during a nutrient challenge in uPUD but not BPU. Gastric motor function may contribute to the manifestation of dyspeptic symptoms in PUD. © 2014 S. Karger AG, Basel. PMID:24903331
Gururatsakul, Montri; Holloway, Richard H; Bellon, Max; Bartholomeusz, Dylan; Talley, Nicholas J; Holtmann, Gerald J
Biliary tract complications are the most common complications after liver transplantation. These complications are encountered more commonly as a result of increased number of liver transplantations and the prolonged survival of transplant patients. Biliary complications remain a major source of morbidity in liver transplant patients, with an incidence of 5%-32%. Post liver transplantation biliary complications include strictures (anastomotic and non-anastomotic), leaks, stones, sphincter of Oddi dysfunction, and recurrence of primary biliary disease such as primary sclerosing cholangitis and primary biliary cirrhosis. The risk of occurrence of a specific biliary complication is related to the type of biliary reconstruction performed at the time of liver transplantation. In this article we seek to review the major biliary complications and their relation to the type of biliary reconstruction performed at the time of liver tranplantation.
Kochhar, Gursimran; Parungao, Jose Mari; Hanouneh, Ibrahim A; Parsi, Mansour A
Biliary tract complications are the most common complications after liver transplantation. These complications are encountered more commonly as a result of increased number of liver transplantations and the prolonged survival of transplant patients. Biliary complications remain a major source of morbidity in liver transplant patients, with an incidence of 5%-32%. Post liver transplantation biliary complications include strictures (anastomotic and non-anastomotic), leaks, stones, sphincter of Oddi dysfunction, and recurrence of primary biliary disease such as primary sclerosing cholangitis and primary biliary cirrhosis. The risk of occurrence of a specific biliary complication is related to the type of biliary reconstruction performed at the time of liver transplantation. In this article we seek to review the major biliary complications and their relation to the type of biliary reconstruction performed at the time of liver tranplantation. PMID:23704818
Kochhar, Gursimran; Parungao, Jose Mari; Hanouneh, Ibrahim A; Parsi, Mansour A
Background Individuals with peripheral arterial disease are at higher risk for cardiovascular events than the general population. While supplementation with omega-3 polyunsaturated fatty acids (PUFA) has been shown to improve vascular function, it remains unclear if supplementation decreases serious clinical outcomes. We conducted a systematic review and meta-analysis to determine whether omega-3 PUFA supplementation reduces the incidence of cardiovascular events and complications in adults with peripheral arterial disease. Methods We searched five electronic databases (MEDLINE, EMBASE, CENTRAL, Scopus and the International Clinical Trials Registry Platform) from inception to 6 December 2013 to identify randomized trials of omega-3 PUFA supplementation (from fish or plant oils) that lasted ?12 weeks in adults with peripheral arterial disease. No language filters were applied. Data on trial design, population characteristics, and health outcomes were extracted. The primary outcome was major adverse cardiac events; secondary outcomes included myocardial infarction, cardiovascular death, stroke, angina, amputation, revascularization procedures, maximum and pain-free walking distance, adverse effects of the intervention, and quality of life. Trial quality was assessed using the Cochrane Risk of Bias tool. Results Of 741 citations reviewed, we included five trials enrolling 396 individuals. All included trials were of unclear or high risk of bias. There was no evidence of a protective association of omega-3 PUFA supplementation against major adverse cardiac events (pooled risk ratio 0.73, 95% CI 0.22 to 2.41, I 2 75%, 2 trials, 288 individuals) or other serious clinical outcomes. Adverse events and compliance were poorly reported. Conclusions Our results showed that insufficient evidence exists to suggest a beneficial effect of omega-3 PUFA supplementation in adults with peripheral arterial disease with regard to cardiovascular events and other serious clinical outcomes.
Although online training is causing restructuring in some companies, content is still predominant and can be delivered without the Internet, for example, through self-study books. Companies should deliver training in the best format for their employees. (JOW)
In this video segment, adapted from a ZOOM television broadcast, cast members repeat an experiment designed to separate fresh water from a liquid solution, this time using colored sugar water in one solar still and orange juice in another. The basic still design they employ traps water vapor that is created when the Sun's heat causes water to evaporate. As the vapor cools, it condenses and trickles down to a collection container. The segment is three minutes fifty-five seconds in length.
In our university cardiac center, the incidence of a cohort of children with acute neurologic complications resulting from cardiac catheterization performed for acyanotic or cyanotic congenital heart disease is 0.38% (14 children of a total of 3,648 catheterization procedures). Neurologic complications consisted of convulsion (n = 10), stroke (n = 6), intracranial hemorrhage (n = 2), extrapyramidal features (n =
Xiao-Yan Liu; Virginia Wong; Maurice Leung
Autosomal dominant polycystic kidney disease (ADPKD) is a common cause of renal failure that is due to mutations in two genes, PKD1 and PKD2. Vascular complications, including aneurysms, are a well recognized feature of ADPKD, and a subgroup of families exhibits traits reminiscent of Marfan syndrome (MFS). MFS is caused by mutations in fibrillin-1 (FBN1), which encodes an extracellular matrix protein with homology to latent TGF-? binding proteins. It was recently demonstrated that fibrillin-1 deficiency is associated with upregulation of TGF-? signaling. We investigated the overlap between ADPKD and MFS by breeding mice with targeted mutations in Pkd1 and Fbn1. Double heterozygotes displayed an exacerbation of the typical Fbn1 heterozygous aortic phenotype. We show that the basis of this genetic interaction results from further upregulation of TGF-? signaling caused by Pkd1 haploinsufficiency. In addition, we demonstrate that loss of PKD1 alone is sufficient to induce a heightened responsiveness to TGF-?. Our data link the interaction of two important diseases to a fundamental signaling pathway. PMID:24071006
Liu, Dongyan; Wang, Connie J; Judge, Daniel P; Halushka, Marc K; Ni, Jie; Habashi, Jennifer P; Moslehi, Javid; Bedja, Djahida; Gabrielson, Kathleen L; Xu, Hangxue; Qian, Feng; Huso, David; Dietz, Harry C; Germino, Gregory G; Watnick, Terry
Vitamin D as a topical treatment has become one of the mainstays for treatment of psoriasis vulgaris. Oral vitamin D on the other hand has for the most part become a forgotten option. But a review of the literature on oral vitamin D as a treatment for psoriasis reveals that this treatment is efficacious. The main side effect of this therapy is hypercalcemia, which appears to be easily monitored and avoidable with proper dosing and monitoring. The literature also suggests a correlation between low levels of serum vitamin D in this patient population associated with increased severity of disease involvement. In addition, oral vitamin D improves psoriatic arthropathy. Moreover, vitamin D has been proven to have many health benefits such as prevention of cancer, improved cardiovascular health among many others. Psoriatic patients as a population are at increased risk of developing adverse health complications such as cardiovascular disease, and oral vitamin D may prove to be of benefit in this population. Oral vitamin D is inexpensive and easily available. It is still a viable option and should not be forgotten as a possible treatment for psoriasis. PMID:22103655
Kamangar, Faranak; Koo, John; Heller, Misha; Lee, Eric; Bhutani, Tina
Abstract Objectives. While there are a few reports describing 18F-fluoro-dexoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) findings in patients with adult-onset Still's disease (AOSD), no summary report has yet been published. In this study, we evaluated the usefulness of FDG-PET/CT for diagnosis and activity evaluation in patients with AOSD by summarizing the findings of our patients and those reported in the literature. Methods. Seven consecutive AOSD patients who had undergone PET/CT at our department between 2007 and 2012 were included. We evaluated FDG uptake for characteristic findings in patients with AOSD. In addition, we reviewed the literature on seven previously reported AOSD patients who had undergone PET/CT. Results. FDG accumulation was positive mainly in the bone marrow (100%), spleen (90.9%), lymph nodes (80.0%) and joints (75.0%). In addition, FDG uptake was positive in the pericardium, pleura, salivary glands, eyelids, muscle and major blood vessels. Six patients underwent follow-up FDG PET/CT for evaluation of treatment efficacy. Follow-up PET/CT showed diminished FDG accumulation in the bone marrow, spleen and lymph nodes, with maximum standardized uptake value (SUVmax) being substantially reduced from 4.03 ± 0.95 to 2.20 ± 0.75 (p = 0.04), 4.04 ± 1.10 to 2.55 ± 1.13 (p = 0.04) and 5.63 ± 4.99 to 2.10 ± 1.91 (p = 0.11), respectively. No significant correlation was found between SUVmax in each lesion and the laboratory data, except for a significant correlation between lactate dehydrogenase (LDH) and spleen SUV. Conclusions. FDG-PET/CT is useful for long-term assessments of AOSD activity in individual patients. However, PET/CT findings alone are not sufficient to make a differential diagnosis of AOSD versus malignant lymphoma. PMID:24252024
Yamashita, Hiroyuki; Kubota, Kazuo; Takahashi, Yuko; Minamimoto, Ryogo; Morooka, Miyako; Kaneko, Hiroshi; Kano, Toshikazu; Mimori, Akio
It is important to recognize the complications of cholestasis in patients with chronic cholestatic liver disease because of\\u000a their prevalence and their pre- and post-transplant implications. Understanding and treating these conditions can result in\\u000a a significant impact on morbidity and quality of life in this group of patients. Most of what is known is based on small studies\\u000a of patients
Abhitabh Patil; Marlyn J. Mayo
HIV affects many organs of the body, including the nervous system. As a result, a series of neurologic complications have\\u000a created challenges for scientists and clinicians alike. Among these, HIV-associated neuropathy and myopathy may occur at all\\u000a stages of the disease process. Of the neuropathies, distal symmetrical polyneuropathy is the most common form. The pathogenesis\\u000a of primary HIV neuropathy is
Susama Verma; Elena Micsa; Lydia Estanislao; David Simpson
Micro- and macro-vascular complications are the leading causes of morbidity and mortality in type 1 and type 2 diabetic patients. Despite the vast clinical experience linking diabetic metabolic abnormalities to cardiovascular lesions, the molecular basis of individual susceptibility to diabetic cardiovascular injury is still largely unknown. Significant advances in this area may come from studies on suitable animal models. Although no animal model can accurately reproduce the human disease, experimental studies in animals have the great advantage to eliminate factors such as ethnicity, economic and geographic variables, drug interactions, diet, gender and age differences that importantly limit clinical studies. Indeed, appropriate animal models have provided important information on genetic and environmental risks of diabetes, and helped to dissect molecular mechanisms underlying the development, progression and therapeutic control of this disease. Unfortunately, none of the diabetic models presently available fully mimics the human syndrome. Therefore, the current knowledge on the pathogenesis of cardiovascular complications relies on the evaluation of distinct phenotypes from various diabetic models. In addition to strains prone to diabetes, this disease can be induced by surgical, pharmacological or genetic manipulation in several animal species. Rodents are the most used, although some studies are still performed in larger animals as rabbits, cats, pigs or monkeys. Far from being exhaustive, this work should serve as a general overview of the most relevant clues provided by major species and models for the overall comprehension of cardiovascular complications in type 1 and type 2 diabetes. PMID:21466471
Potenza, M A; Nacci, C; Gagliardi, S; Montagnani, M
Fournier’s gangrene (FG) is a rare but severe necrotizing fasciitis of the external genitalia that may complicate the clinical course of hematologic malignancies and sometimes may be the first sign of the disease. The clinical course of FG is very aggressive and the mortality is still high despite the improvement in its management. Early recognition of FG and prompt appropriate treatment with surgical debridement and administration of antibiotics are the cornerstone of the management of this very severe disease. A review of the scientific literature focusing on the topic of FG complicating hematologic disorders is reported
D'Arena, Giovanni; Pietrantuono, Giuseppe; Buccino, Emilio; Pacifico, Giancarlo; Musto, Pellegrino
Vascular involvement is a common complication of Behçet's disease (BD) and affects up to 40% of BD patients. These complications worsen the prognosis of BD. The concept of vasculo-Behçet has been adopted for cases in which vascular complications dominate the clinical features. Vascular manifestations affect particularly young men, during the first years following onset of the disease. Venous complications are the most frequent vascular complications, affecting 14 to 40% of BD patients. Superficial and deep lower limb thrombosis is the most frequent venous complications but one third of venous thrombosis concern large vessels (such as cerebral venous thrombosis, pulmonary embolism, and inferior or superior vena cava, etc.). Budd-Chiari syndrome is the worst prognostic factor increasing mortality by 9 times. Arterial complications (2 to 17% of BD patients) include aneurysms and occlusions/stenosis. Main locations of arterial lesions are aortic (abdominal and thoracic), femoral, pulmonary and iliac arteries. Aneurysms are the most severe arterial complications, particularly pulmonary aneurysms associated with a high risk of massive bleeding. Cardiac complications (up to 6% of BD patients) include pericarditis, endocardial lesions (aortic regurgitation and less often mitral insufficiency), myocardial lesions (myocardial infarction, myocarditis and endomyocardial fibrosis) and intracardiac thrombosis (right ventricle and atrium). Coronary lesions complicated to myocardial infarction are the most severe cardiac complications. Treatment is based on corticosteroids and immunosuppressive drugs. The use of anticoagulation in venous thrombosis is still controversial. PMID:24434015
Desbois, A-C; Wechsler, B; Cluzel, P; Helft, G; Boutin, D; Piette, J-C; Cacoub, P; Saadoun, D
Determination of serum soluble transferrin receptor (sTfR) has been proposed to identify iron-deficiency anemia (IDA) in patients affected by concurrent inflammatory disease that may spuriously increase ferritin concentration. The aim of this study was to critically review the available literature to assess the diagnostic efficacy of sTfR in complicated anemia. The criteria for study selection were: enrolment of patients with complicated anemia; bone marrow examination used as diagnostic gold standard for IDA; evaluation of sTfR vs. ferritin and binary data presentation. Six published studies met the criteria. However, the small size and wide heterogeneity of the studies did not allow us to conduct a meta-analysis. sTfR was overall more sensitive, even though it was evident that the ferritin sensitivity was influenced by selected cut-offs. Well-designed studies are still needed to define the added value, if any, of sTfR to ferritin for IDA detection in complicated anemia. PMID:24525213
Braga, Federica; Infusino, Ilenia; Dolci, Alberto; Panteghini, Mauro
Hypoglycemia is an important complication of glucose-lowering therapy in patients with diabetes mellitus. Attempts made at intensive glycemic control invariably increases the risk of hypoglycemia. A six-fold increase in deaths due to diabetes has been attributed to patients experiencing severe hypoglycemia in comparison to those not experiencing severe hypoglycemia Repeated episodes of hypoglycemia can lead to impairment of the counter-regulatory system with the potential for development of hypoglycemia unawareness. The short- and long-term complications of diabetes related hypoglycemia include precipitation of acute cerebrovascular disease, myocardial infarction, neurocognitive dysfunction, retinal cell death and loss of vision in addition to health-related quality of life issues pertaining to sleep, driving, employment, recreational activities involving exercise and travel. There is an urgent need to examine the clinical spectrum and burden of hypoglycemia so that adequate control measures can be implemented against this neglected life-threatening complication. Early recognition of hypoglycemia risk factors, self-monitoring of blood glucose, selection of appropriate treatment regimens with minimal or no risk of hypoglycemia and appropriate educational programs for healthcare professionals and patients with diabetes are the major ways forward to maintain good glycemic control, minimize the risk of hypoglycemia and thereby prevent long-term complications.
Kalra, Sanjay; Mukherjee, Jagat Jyoti; Venkataraman, Subramanium; Bantwal, Ganapathi; Shaikh, Shehla; Saboo, Banshi; Das, Ashok Kumar; Ramachandran, Ambady
Hematopoietic cell transplantation (HCT) is the preferred treatment for an expanding range of neoplastic and nonmalignant conditions. Increasing numbers of solid organ transplantations (SOTs) add an additional population of immunosuppressed patients with multiple potential neurological problems. While the spectrum of neurological complications varies with conditioning procedure and hematopoietic cell or solid organ source, major neurological complications occur with all transplantation procedures. This 2 part review emphasizes a practical consultative approach to central and peripheral nervous system problems related to HCT or SOT with clinical and neuroimaging examples from the authors’ institutional experience with the following conditions: the diversity of manifestations of common infections such as varicella zoster virus, Aspergillus, and progressive multifocal leukoencephalopathy (PML), drug therapy-related complications, stroke mechanisms, the spectrum of graft versus host disease (GVHD), and neurologically important syndromes of immune reconstitution inflammatory syndrome (IRIS), posterior reversible encephalopathy syndrome (PRES), and posttransplantation lymphoproliferative disorder (PTLD). These complications preferentially occur at specific intervals after HCT and SOT, and neurological consultants must recognize an extensive spectrum of syndromes in order to effect timely diagnosis and expedite appropriate treatment.
Pruitt, Amy A.; Graus, Francesc; Rosenfeld, Myrna R.
Respiratory failure is still an important complication of chronic obstructive pulmonary disease (COPD) and hospitalisation with an acute episode being a poor prognostic marker. However, other comorbid conditions, especially cardiovascular disease, are equally powerful predictors of mortality. The physiological basis of acute respiratory failure in COPD is now clear. Significant ventilation\\/perfusion mismatching with a relative increase in the physiolo- gical
P. M. A. Calverley
... in the community. Home > Pregnancy > Pregnancy Complications > Gonorrhea Pregnancy complications Pregnancy complications may need special medical care. ... the United States. Can gonorrhea cause complications during pregnancy and for your baby? Yes. Gonorrhea can lead ...
Basic concepts of using desalinated water for agricultural purposes are outlined. A mathematical model describing heat and mass transfer in a system combining a solar still with a greenhouse, its solution, and test results of a small-scale unit built at the Middle East Technical University, Ankara, Turkey, are discussed. The unit was employed to demonstrate the technical feasibility of the system. Further development and modifications are necessary for larger-scale operations. The basis of an optimization study which is underway at the Brace Research Institute of McGill University in Montreal, Canada, aimed at finding the best combination of design and operation parameters is also presented.
Selcuk, M. K.; Tran, V. V.
Babesiosis (piroplasmosis) is a tick-borne disease with a symptoms of hemolytic anemia. For the first time babesiosis was described in dogs in United States in 1934. The etiological factor of this disease in Poland is protozoa Babesia canis, and its vector--Dermacentor-tick. The most common symptoms of babesiosis are: icterus, hemoglobinuria, occasionally vomits and diarrhea. The biochemical examination of blood serum from sick animals can reveal the increase of activity of AST, ALT, the increase of total bilirubine, urea and creatynine concentrations. The results of hematological examinations can show anemia, leucopenia and thrombocytopenia. The diagnosis of babesiosis bases on anamnesis, clinical examinations of dogs, microscopical examinations of blood smears from sick animals; IF-assay and PCR can also be helpful for the diagnosis of babesiosis. Till now does not exist the effective immunoprophylaxis against this disease. Babesiosis is well-known disease, however there are still problems with therapy of infected animals. Most effective drug in therapy of dog piroplasmosis is imidocarb, but sometimes can be observed side effects after it application. It is possible that the genetically differences which are detected in subspecies may have an influence on the severity of disease and the effectiveness of therapy. PMID:18702315
Adaszek, ?ukasz; Winiarczyk, Stanis?aw
Thyroid diseases are common in women of childbearing age and it is well known that untreated thyroid disturbances result in an increased rate of adverse events, particularly miscarriage, preterm birth and gestational hypertension. Furthermore, thyroid autoimmunity per se seems to be associated with complications such as miscarriage and preterm delivery. While strong evidence clearly demonstrates that overt dysfunctions (hyper- or hypothyroidism) have deleterious effects on pregnancy, subclinical disease, namely subclinical hypothyroidism, has still to be conclusively defined as a risk factor for adverse outcomes. Additionally, other conditions, such as isolated hypothyroxinemia and thyroid autoimmunity in euthyroidism, are still clouded with uncertainty regarding the need for substitutive treatment. PMID:22115167
Negro, Roberto; Mestman, Jorge Hector
Amitraz is a triazapentadiene, an ?2 adrenergic agonist and a member of the amidine chemical family. A limited number of human intoxication cases have been published in the literature. Lack of a clear and specific protocol for the therapy of amitraz intoxication may make its successfully managed case reports useful and valuable for other clinical practitioners in poisoning departments. The case is about a 22 years old female, single, university student, ingested a glass of amitraz poison (about 100 mL of a 20% solution) as a suicidal attempt on 11:30 am which was about 3.5 h before her hospital admission. She found nausea, vomiting, and dizziness. Immediately, her family took her to a clinic near their house. At that clinic (13:30 pm) she had miosis and they did gastric lavage , one adult dose of activated charcoal (50 g) and referred her to our Poisoning Emergency Department, where she was managed supportively and successfully. Amitraz is a poisonous chemical which may cause central nervous system depression and also respiratory/cardiovascular symptoms as well. Several studies reported that using atropine for those amitraz poisoned patients with both miosis and bradycardia resolved the problem and recommend it as the first line of drug therapy when bradycardia occurs from vagal stimulation and atrioventricular block. Management of amitraz poisoning is still considered to be supportive and symptomatic. Although the effects of activated charcoal and cathartics have not been studied, they may still be considered for treatment. PMID:24363695
Eizadi-Mood, Nastaran; Sabzghabaee, Ali Mohammad; Gheshlaghi, Farzad; Yaraghi, Ahmad
Despite the new and ever expanding array of medications for the treatment of inflammatory bowel disease (IBD), there are still clear indications for operative management of IBD and its complications. We present an overview of indications, procedures, considerations, and controversies in the surgical therapy of IBD.
Hwang, John M; Varma, Madhulika G
Fractionated CO2 laser technology has allowed physicians to resurface patients with a lower rate of complications than nonfractionated ablative laser treatment. Unfortunately, adverse effects can still occur even with the best technology and physician care. Complication prevention, detection, and treatment are an important part of a physician's ability to provide the best result when treating a patient with fractionated CO2 resurfacing.
Ramsdell, William M.
The role of biofilm in implant infections and the genetic control of its production are still giving rise to great interest and controversial new results are continuing to emerge from widespread investigations. Bacteria embedded in biofilms are more resistant to most antibiotics. Hypotheses regarding the mechanisms of antibiotic multi-resistance in biofilm are brought to light, including the onset of persister cells during the attachment of bacteria to implant surfaces.This 2008 special issue ''Focus on Implant Infections'', presents new strategies for combating biofilm infections,such as the use of staphylococcal quorum-sensing inhibitors or the ''bioelectric effect'': the phenomenon by which electric current can enhance the activity of some antimicrobial agents against bacteria in biofilms. PMID:18924085
Arciola, C R
Four months and three work stoppages into fiscal 1996, whole departments and agencies of the United States federal government remain in budgetary limbo. Five annual spending bills still await approval, and parts of nine federal departments and several agencies face the possibility of yet another shutdown, as the current continuing resolution for temporary funding expires on March 15.In the wake of the recent three-week shutdown of the federal government, congressional leaders worked in January to ease future political pain by funding a list of “essential services” for the remainder of the fiscal year. Deemed essential were government programs with the most immediate and conspicuous public impact, such as the National Parks Service and the Passport Services Office. Included on that list of essential services was the National Institutes of Health (NIH), which not only received full funding for the entire fiscal year but also got a 5.7% increase over its 1995 budget.
Opinion statement \\u000a \\u000a \\u000a \\u000a \\u000a – \\u000a \\u000a The optimal “treatment” of postfundoplication complications is preoperative prevention of them. Nonreflux causes of the symptom\\u000a prompting surgery should be carefully eliminated preoperatively. Failure to respond to optimal powerful antireflux pharmacotherapy\\u000a suggests that GERD was not the cause of symptoms. Neurologic or respiratory disease, delayed gastric emptying or retching,\\u000a short esophagus, and esophageal dysmotility may predispose patients
Susan R. Orenstein; Carlo Di Lorenzo
The most frequent and acute complications of subclavian vein catheterization are arterial puncture to the subclavian artery and pneumothorax. We report an arterial puncture directly to the pulmonary artery in infant during subclavian vein catheterization. PMID:18502370
Lu, Wen-Hsien; Huang, Ta-Cheng; Pan, Jun-Yen; Wang, Hsiao-Ping; Lin, Chu-Chuan; Chen, Ying-Yao; Hsieh, Kai-Sheng
Chronic Chagas' myocarditis can alter the myocardial substrate in a way that facilitates the emergence of fatal VT in a way similar to the long-term consequences of myocardial infarction. Post-myocardial infarction and Chagas' VT share many similarities: they are both macroreentrant circuits, entrainable, involving any wall segment from the endocardium to the epicardium. However, as compared to patients with post-MI VT, Chagasic patients tend to be younger and have a higher left ventricular ejection fraction. It is assumed, therefore, that their prognosis is closely related to VT treatment rather than the progression of the myocardial damage caused by the disease itself. Although sudden death is a rare event in patients in NYHA functional class I and II treated with amiodarone, VT recurrence rate is 30% a year. Drug therapy is ineffective for patients with advanced heart failure (100% recurrence rate/40% mortality in 1 year). Open-chest surgery is effective but requires very specialized centers and great expertise making its widespread use unrealistic. The results of combining RF endo/epicardial catheter ablation are still disappointing. Thus, research protocols on the search for new ablation technologies may greatly impact overall mortality in this subset of patients. This review will focus on the limitations of the current catheter-based ablation technology and suggest that an alternative approach is urgently needed. Experimental evidence of the efficacy of near infrared Lasers for catheter ablation will be reported along with investigations of the optical properties of the chagasic myocardium in the near infrared region to indicate that it might be not only feasible but also an appropriate choice to treat these patients. PMID:12391418
d'Avila, André; Splinter, Robert; Svenson, Robert H; Scanavacca, Mauricio; Pruitt, Ernest; Kasell, Jackie; Sosa, Eduardo
Sulfur mustard (SM) is an alkylating chemical warfare agent with high military significance due to its high toxicity, resistance and availability. SM was widely used in military conflicts, the last being the Iran-Iraq war with more than 100,000 Iranians exposed, one-third of whom are still suffering from late effects. The intensity of the delayed complications correlates to the extent, the area and the route of exposure. The clinical manifestations most commonly involve respiratory, ocular and dermal effects. Respiratory complications include dyspnea, cough and expectorations and various obstructive and restrictive lung diseases. Dermal complications are itching, burning sensation, blisters, dry skin, dermatitis and pigmentary changes. Ocular complications include photophobia, red eye, tearing, corneal ulcers and blindness. Although the picture remains incomplete the major mechanisms responsible for the clinical and pathological effects of SM are: DNA alkylation and cross-linking, protein modification and membrane damage in addition to induction of inflammatory mediators in the target tissues causing extensive necrosis, apoptosis and loss of tissue structure. The current report reviews long-term complications of SM exposure, focusing on new treatments tested in clinical trials conducted on humans. Such treatments include: N-acetyl cysteine, bronchodilators, corticosteroids, Interferon-gamma, furosemide and morphine for the respiratory complications. Ocular complications may entail: Invasive procedures treating corneal complication, limbal ischemia and stem cell deficiency. Treatment for dermatological complications include: anti-depressants, pimercrolimus, Unna's boot, capsaicin, phenol and menthol, Aloe vera and olive oil, curcumin and Interferon-gamma. PMID:24791566
Shiyovich, Arthur; Rosman, Yossi; Krivoy, Amir; Statlender, Liran; Kassirer, Michael; Shrot, Shai
A prospective pediatric survey on the incidence of central venous catheter (CVC) complications was performed aimed at identifying risk factors of premature CVC removal. The study comprised 129 Broviac-Hickman CVCs inserted during a 13-month period in 112 children. The total number of CVC days was 19,328 (median: 122 days, range: 1–385). The overall rate of complications was 6.2\\/1000 CVC days, i.e.,
Simone Cesaro; Roberta Corrò; Anna Pelosin; Piergiorgio Gamba; Nicola Zadra; Fabio Fusaro; Marta Pillon; Riccardo Cusinato; Chiara Zampieri; Laura Magagna; Mara Cavaliere; Gloria Tridello; Gianfranco Zanon; Luigi Zanesco
Pancreatitis, in common with many other upper abdominal diseases, often leads to pleuropulmonary complications. Radiological evidence of pleuropulmonary abnormality was found in 55% of 58 cases examined retrospectively. The majority of such abnormalities are not specific for pancreatitis; but a particular category of pleural effusions, rich in pancreatic enzymes, is a notable exception. A patient with this type of effusion, complicated by a spontaneous bronchopleural fistula and then by an empyema, is reported. The literature relating to pancreatic enzyme-rich pleural effusions (pathognomonic of pancreatitis) is reviewed. Of several possible mechanisms involved in pathogenesis, transdiaphragmatic lymphatic transfer of pancreatic enzymes, intrapleural rupture of mediastinal extensions of pseudocysts, and diaphragmatic perforation are the most important. The measurement of pleural fluid amylase, at present little employed in this country, has considerable diagnostic value. Enzyme-rich effusions are more commonly left-sided, are often blood-stained, are frequently associated with pancreatic pseudocysts, and—if long standing—may be complicated by a bronchopleural fistula. Images
Kaye, Michael D.
Kidney transplantation is now recognized as the treatment of choice for patients with chronic renal failure. Despite the extension of indications to patients suffering severe hypertension, ischemic heart disease, and chronic heart failure, the worldwide results are superb. However, perioperative cardiac complications occur in 6% to 10% of transplanted patients. Aggressive intraoperative volume expansion is still recommended to maximize graft functional recovery (up to 30 mL/kg/h, central venous pressure [CVP] > 15 mm Hg), but patients with preexistent cardiac disease or poor myocardial function are exposed to the risk of fluid overload, acute respiratory failure, and prolonged ventilation. Among the last 90 cases performed at our institution, good functional recovery of the graft was present in 94% of the patients within 2 weeks, despite a much more conservative intraoperative hydration policy (crystalloids 2400 +/- 1000 mL, 15 mL/kg/h, CVP 7-9 mm Hg). Graft failure which occurred in 5 patients was significantly correlated only with donor age, while perioperative cardiovascular complications had been present in 9 cases (10%) who were coronary artery disease patients (55%). Age above 50 years was the only significant risk factor. Supranormal volume loading is probably not always warranted in kidney transplantation. PMID:16647477
De Gasperi, A; Narcisi, S; Mazza, E; Bettinelli, L; Pavani, M; Perrone, L; Grugni, C; Corti, A
Infectious complications in chronic graft-versus-host disease: a retrospective study of 145 recipients of allogeneic hematopoietic stem cell transplantation with reduced- and conventional-intensity conditioning regimens
To assess infectious complications associated with chronic graft-versus-host disease (cGVHD) after allogeneic hematopoietic stem cell transplantation (HSCT) with reduced- and conventional-intensity conditioning regimens (RIC, n 5 91; CIC, n 5 54, respectively), we retrospectively analyzed data from 145 consecutive patients with cGVHD after allogeneic HSCT from a human leukocyte antigen- matched related or unrelated donor. In the present retrospective analysis,
S. Yamasaki; Y. Heike; S. Mori; T. Fukuda; D. Maruyama; R. Kato; E. Usui; K. Koido; S. Kim; R. Tanosaki; K. Tobinai; T. Teshima; Y. Takaue
... the community. Home > Pregnancy > Pregnancy Complications > Placenta previa Pregnancy complications Pregnancy complications may need special medical care. ... to get pregnant again. Are gallstones common during pregnancy? Not common, but they do happen. Elevated hormones ...
... the community. Home > Pregnancy > Pregnancy Complications > HELLP syndrome Pregnancy complications Pregnancy complications may need special medical care. ... to get pregnant again. Are gallstones common during pregnancy? Not common, but they do happen. Elevated hormones ...
... in the community. Home > Pregnancy > Pregnancy Complications > Chlamydia Pregnancy complications Pregnancy complications may need special medical care. ... younger than 25. Can chlamydia cause problems during pregnancy? Yes. If you get it before or during ...
... the community. Home > Pregnancy > Pregnancy Complications > Genital herpes Pregnancy complications Pregnancy complications may need special medical care. ... to get pregnant again. Are gallstones common during pregnancy? Not common, but they do happen. Elevated hormones ...
[Bone Metabolism and Cardiovascular Function Update. ?-Klotho/FGF23 system ; A new insight into the field of mineral homeostasis and the pathogeneses of aging-associated syndromes and the complications of chronic kidney disease].
?-klotho (?-kl) was first identified as an aging gene and was later shown to be a regulator of mineral homeostasis. ?-kl (- / -) mice display multiple aging related phenotypes including atherosclerosis, cardiovascular/soft tissue calcifications, pulmonary emphysema, osteopenia, and senile atrophy of skin ; such age-related organ pathologies are associated with biochemical changes in blood, including severe hyperphosphatemia, elevated serum FGF23 and1,25 (OH) 2 Vitamin D levels. Of significance, advanced stage patients suffering chronic kidney disease (CKD) develop multiple complications quite resembling phenotypes observed in ?-kl (- / -) mice, and high serum phosphate, the major cause of abnormalities of ?-kl (- / -) mice, has been reported to be closely associated with high levels of cardiovascular disease morbidity and mortality in patients with CKD, particularly in patients with end-stage renal disease. In addition, the expressions of ?-kl mRNA and ?-Kl protein were severely reduced in these patients. These results suggest the involvement of ?-Kl and FGF23 in the pathogeneses of not only aging-associated syndromes but also the complications of CKD. Here, the unveiling of the molecular functions of ?-Klotho and FGF23 has recently given new insight into the field of mineral homeostasis and the pathogeneses of aging-associated syndromes and the complications of CKD. PMID:24976054
Nabeshima, Yo Ichi
Diabetes is increasing at an alarming rate and, despite anti-hypertensive and insulin therapies, diabetic patients are still at risk of developing complications such as chronic kidney disease, cardiovascular disease, and retinopathy. There is therefore an urgent need for more effective therapies to prevent the development and progression of diabetic complications. Oxidative stress is a major player in the aetiology of diabetic complications. However, results from clinical trials thus far using general antioxidants have been disappointing. Mechanism-based antioxidants have gained considerable attention due to their more targeted approach at reducing oxidative stress and associated complications in diabetes. The transcription factor, NFE2-related factor 2 (Nrf2), is a master regulator of redox homeostasis and the cellular detoxification response. Instead of relying on a single antioxidant, activation of Nrf2 results in the concerted upregulation of several antioxidant enzymes and cytoprotective genes, making it an attractive therapeutic target for diabetic complications. Several Nrf2 activators have been discovered and have proven effective at activating Nrf2 signalling through different mechanisms in both in vitro and in vivo models of diabetes. This review will address some of the most promising and well-known Nrf2 activators and their roles in preventing the development and progression of diabetic complications. Challenges facing the advancement of this drug class into the clinic will be discussed, as will be the future of Nrf2 activation as a therapeutic strategy in preventing the development of diabetic complications. PMID:24559141
Tan, Sih Min; de Haan, Judy B
Today, human behavior drives many extinctions and preserves some species. To help understand such behavior, we published a book in 1999 that viewed selected endangered species through the eyes of those who have watched them decline and, in some cases, vanish from the wild. Here we revisit those stories to document what has happened in the interim 10 years, a period that is very short in evolutionary time but that has proven decisive for some endangered species. One species is now extinct in the wild; others have been devastated or scattered; several are on Ã¢ÂÂlife support.Ã¢ÂÂ Some things have not changed: Too many people consume too much, and disagreements among conservationists still impede progress. Some issues have become more prominent, such as emerging diseases and global economic crises. Although there have been some striking successes, it is very clear that more species now depend on human support for their survival than may be sustainable.
Beverly Peterson Stearns (freelance writer;); Stephen Stearns (Yale University;Ecology and Evolutionary Biology)
Plastic surgery covers a broad spectrum of diseases and conditions in the areas of reconstructive surgery, hand, burn and aesthetic surgery. Besides acquired defects or malformations an increasing number of patients are being treated for surgical or multimodal complications. In a considerable number of patients plastic and reconstructive surgery remains the only therapeutic alternative after other therapy has failed. Therefore complication management in plastic surgery is of utmost importance for a successful outcome. In addition patient expectations in the results of plastic surgery as a discipline of invention and problem solving are steadily increasing. This challenge is reflected in clinical patient management by intensive research in tissue engineering and regenerative medicine. Patients in plastic surgery are recruited from all age groups of either gender, involving traumatic and oncologic as well as congenital and aesthetic disorders. The demographics of aging, multimorbidity and obesity pose new challenges to plastic surgery. Although age over 70 years is not an independent risk factor per se for complications in plastic surgery, e.g. for complex free flap transfer, medical problems are present at a higher rate, which is to be expected in this age group. Risk factors such as alcoholism and coronary heart diseases seem to be independent predictors of perioperative complications. Therefore older patients can also benefit from plastic surgery and recurrent operations by the corresponding risk and complication management. Complication management necessitates careful patient selection, estimation of operative risks and patient-adapted selection of procedures. In addition to expertise in plastic surgery a thorough knowledge of non-surgical and surgical back-up procedures for technical incidents as well as vascular circulatory and wound healing disorders is required to deal successfully with complications in plastic surgery. This article presents these specific aspects of postoperative complication management in plastic surgery. PMID:19669715
Vogt, P M
Objective: To estimate direct medical costs and average lifetime cost per case of pelvic inflammatory disease (PID).Methods: We estimated the direct medical expenditures for PID and its three major sequelae (chronic pelvic pain, ectopic pregnancy, and infertility) and determined the average lifetime cost of a case of PID and its sequelae. We analyzed 3 years of claims data of privately
David B Rein; William J Kassler; Kathleen L Irwin; Lara Rabiee
Abstract Cancer patients tend to have a higher incidence of herpes zoster (HZ), but little is known about their risk of HZ complications. We conducted a retrospective study of 424 newly diagnosed hematologic (HM, n = 140) and solid tumor malignancy (STM, n = 284) patients who developed HZ between January 2001 and December 2006 to measure the frequency and identify risk factors of HZ complications. Patients were adult members of Kaiser Permanente Northern California. HZ diagnosis and complications were confirmed by medical chart review. HM patients with HZ tended to have more HZ complications than STM patients (34% vs 23%, p = 0.02), largely due to more frequent non-pain complications. On multivariate analysis, older age and being male were associated with a higher risk of HZ complications in HM patients; more advanced cancer stage was associated with HZ complications in STM patients. HZ complications are frequent and can present extra disease burden in cancer patients who develop HZ. PMID:24796470
Tran, Trung N; Ray, G Thomas; Horberg, Michael A; Yawn, Barbara P; Castillo, Adrienne L; Saddier, Patricia; Habel, Laurel A
Non-infectious lung complications (NILC) are frequent, influencing morbidity and mortality of patients after allogeneic BMT. Although the term NILC encompasses a number of different entities, an association with GVHD has been noted for almost all of them. Our study was directed towards assessing the incidence and risk factors for developing NILC, as well as the response to treatment and long-term
C Duncker; D Dohr; S von Harsdorf; J Duyster; M Stefanic; C Martini; M Treiber; B Hertenstein; J Novotny; R Arnold; H Heimpel; L Bergmann; D Bunjes
TIPS is a new and exciting modality for treatment of portal hypertension and its complications. Indications for TIPS remain to be better defined in terms of efficacy and cost-benefit in relationship to other established modes of therapy of portal hypertension. Prospective, randomized controlled trials are needed for these comparisons. Until the role of TIPS in the routine management of the
Mitchell L. Shiffman; Lennox Jeffers; Jay H. Hoofnagle; Tommie Sue Tralka
I Cell Disease; Fucosidosis; Globoid Cell Leukodystrophy; Adrenoleukodystrophy; Mannosidosis; Niemann-Pick Disease; Pulmonary Complications; Mucopolysaccharidosis I; Mucopolysaccharidosis VI; Metachromatic Leukodystrophy; Gaucher's Disease; Wolman Disease
Contents: What Are the Symptoms; What Causes Crohn's Disease and Who Gets It; How Does Crohn's Disease Affect Children; How is Crohn's Disease Diagnosed; What Is the Treatment; Can Diet Control Crohn's Disease; What Are the Complications of Crohn's Diseas...
Diabetes is a chronic and complex multifactorial disease caused by persistent hyperglycemia and for which underlying pathogenesis is still not completely understood. The mathematical modeling of glucose homeostasis, diabetic condition, and its associated complications is rapidly growing and provides new insights into the underlying mechanisms involved. Here, we discuss contributions to the diabetes modeling field over the past five decades, highlighting the areas where more focused research is required.
Ajmera, I; Swat, M; Laibe, C; Novere, N Le; Chelliah, V
Diabetes is a major concern among medical practitioners, with the annual mortality rate increasing up to 26.9% in a person aged 65 years or older and 11.3% in the adult. There are many serious complications associated with diabetes, particularly cardiovascular complications due to microvascular diseases. A prerequisite to reduce the risk of microvascular and neurologic complications of type 1 diabetes is normoglycemia. Insulin therapy is the most common treatment used nowadays in type 1 diabetes. However, this method still has many disadvantages such as increased episode of severe hypoglycemia, hypoglycemia unawareness, increased weight gain, transient exacerbation of pre-existing retinopathy, etc. Using insulin pump (the insulin pump is a medical device used for continuous subcutaneous insulin infusion to manage the insulin level in the treatment of diabetes mellitus), is associated with known disadvantages including increased ketoacidosis, infection at the infusion site, and the treatment being less suitable in young children (less than 7 years of age). Therefore, alternative treatment for diabetes is still in great demand. We took the approach of traditional Chinese medicine to discuss this matter. Sesame (?? Zh? Má), a herb, has been used medicinally for thousands of years in almost all the countries in the world. The beneficial effects of sesame in remediating diabetes, such as hypoglycemic effects, antioxidant, anti-inflammatory, and hypolipidemic effects, improving fat metabolism, and reducing cholesterol, have been demonstrated in many studies,. However, reports on the effects of sesame in remediating cardiovascular complications in diabetic patients are limited, which necessitates further studies on the effects of sesame on cardiovascular complications.
Lin, Yen-Chang; Thuy, Tran Duong; Wang, Shu-Yin; Huang, Pung-Ling
Diabetes is a major concern among medical practitioners, with the annual mortality rate increasing up to 26.9% in a person aged 65 years or older and 11.3% in the adult. There are many serious complications associated with diabetes, particularly cardiovascular complications due to microvascular diseases. A prerequisite to reduce the risk of microvascular and neurologic complications of type 1 diabetes is normoglycemia. Insulin therapy is the most common treatment used nowadays in type 1 diabetes. However, this method still has many disadvantages such as increased episode of severe hypoglycemia, hypoglycemia unawareness, increased weight gain, transient exacerbation of pre-existing retinopathy, etc. Using insulin pump (the insulin pump is a medical device used for continuous subcutaneous insulin infusion to manage the insulin level in the treatment of diabetes mellitus), is associated with known disadvantages including increased ketoacidosis, infection at the infusion site, and the treatment being less suitable in young children (less than 7 years of age). Therefore, alternative treatment for diabetes is still in great demand. We took the approach of traditional Chinese medicine to discuss this matter. Sesame ( Zh? Má), a herb, has been used medicinally for thousands of years in almost all the countries in the world. The beneficial effects of sesame in remediating diabetes, such as hypoglycemic effects, antioxidant, anti-inflammatory, and hypolipidemic effects, improving fat metabolism, and reducing cholesterol, have been demonstrated in many studies,. However, reports on the effects of sesame in remediating cardiovascular complications in diabetic patients are limited, which necessitates further studies on the effects of sesame on cardiovascular complications. PMID:24872931
Lin, Yen-Chang; Thùy, Tr?n D Ng; Wang, Shu-Yin; Huang, Pung-Ling
Osteopenia and osteoporosis are well-known complications detected in celiac disease patients with still obscure pathogenesis. In the present study we investigated the presence of circulating anti-bone autoantibodies in patients with celiac disease and explored their role in the associated bone disease. We evaluated serum samples from 33 patients at the time of diagnosis and from 20 of them after treatment.
Emilia Sugai; Alejandra Cherñavsky; Silvia Pedreira; Edgardo Smecuol; Horacio Vazquez; Sonia Niveloni; Roberto Mazure; Eduardo Mauriño; Gabriel A. Rabinovich; Julio C. Bai
Human serum albumin (HSA) is the most abundant circulating protein and accounts for about 70% of the plasma colloid osmotic pressure. Beside the well known capacity to act as plasma-expander, HSA is provided of many other properties which are unrelated to the regulation of fluid compartmentalization, including binding and transport of many endogenous and exogenous substances, antioxidant function, immuno-modulation, anti-inflammatory activity, and endothelial stabilization. Treatment (hepatorenal syndrome) or prevention (renal failure after spontaneous bacterial peritonitis and post-paracentesis circulatory dysfunction after large volume paracentesis) of severe clinical complications in patients with cirrhosis and fluid resuscitation in critically ill patients, when crystalloids and non-proteic colloids are not effective or contra-indicated, represents the major evidence-based clinical indications for HSA administration. However, a large proportion of HSA prescription is inappropriate. Despite the existence of solid data against a real benefit, HSA is still given for nutritional interventions or for correcting hypoalbuminemia per se (without hypovolemia). Other clinical uses for HSA administration not supported by definitive scientific evidence are long-term treatment of ascites, nephrotic syndrome, pancreatitis, abdominal surgery, acute distress respiratory syndrome, cerebral ischemia, and enteric diseases. HSA prescription should be not uncritically restricted. Enforcement of clinical practice recommendations has been shown to allow a more liberal use for indications supported by strong scientific data and to avoid the futile administration in settings where there is a lack of clinical evidence of efficacy. As a result, a more appropriate HSA use can be achieved maintaining the health care expenditure under control. PMID:23790570
Caraceni, Paolo; Domenicali, Marco; Tovoli, Alessandra; Napoli, Lucia; Ricci, Carmen Serena; Tufoni, Manuel; Bernardi, Mauro
Morcellation at laparoscopy is a commonly used minimally invasive method to extract bulky tissue from the abdomen without extending abdominal incisions. Despite widespread use of morcellation, complications still remain underreported and poorly understood. We performed a systematic review of surgical centers in the United States to identify, collate and update the morcellator-related injuries and near misses associated with powered tissue removal. We searched articles on morcellator-related injuries published from 1993 through June 2013. In addition, all cases reported to MedSun and the FDA device database (MAUDE) were evaluated for inclusion. We used the search terms "morcellation," "morcellator," "parasitic," and "retained" and model name keywords "Morcellex," "MOREsolution," "PlasmaSORD," "Powerplus," "Rotocut," "SAWALHE," "Steiner," and "X-Tract." During the past 15 years, 55 complications were identified. Injuries involved the small and large bowels (n = 31), vascular system (n = 27), kidney (n = 3), ureter (n = 3), bladder (n = 1), and diaphragm (n = 1). Of these injuries, 11 involved more than 1 organ. Complications were identified intraoperatively in most patients (n = 37 [66%]); however, the remainder were not identified until up to 10 days postoperatively. Surgeon inexperience was a contributing factor in most cases in which a cause was ascribed. Six deaths were attributed to morcellator-related complications. Nearly all major complications were identified from the FDA device database and not from the published literature. The laparoscopic morcellator has substantially expanded our ability to complete procedures using minimally invasive techniques. Associated with this opportunity have been increasing reports of major and minor intraoperative complications. These complications are largely unreported, likely because of publication bias associated with catastrophic events. Surgeon experience likely confers some protection against these injuries. Understanding and implementing safe practices associated with the use of the laparoscopic morcellator will reduce these iatrogenic injuries. PMID:24333632
Milad, Magdy P; Milad, Elizabeth A
Empyemas are usually of infectious origin. Noninfectious causes of empyema may be a considerable diagnostic challenge, as exemplified in the present case report. A 21-year-old male presented with high fever, sore throat and myalgias of 1 week duration. In the following days, bilateral pleural effusions developed, with cellular counts in the pleural fluid up to 117 × 109\\/liter (98% neutrophils).
M. Mattiuzzo; M. Del Forno; B. Branca; V. Moretti; M. Pirisi
Dunnigan-type familial partial lipodystrophy (FPLD) is a rare monogenic adipose tissue disorder in which the affected subjects\\u000a have increased predisposition to insulin resistance and related metabolic complications, such as glucose intolerance, diabetes,\\u000a dyslipidemia, and hepatic steatosis. Our patient was a 35-year-old female who had been receiving insulin injection therapy for diabetes mellitus and was transferred\\u000a to our hospital. She was
Hitomi Imachi; Koji Murao; Shouji Ohtsuka; Mako Fujiwara; Tomie Muraoka; Hitoshi Hosokawa; Toshihiko Ishida
Infections with gastrointestinal parasitic helminths indigenous to Japan and their treatment historically studied in an attempt to control the diseases in countries where they are still rampant: (1) the Jomon to Edo periods.
Infections with gastrointestinal parasitic helminthes were historically surveyed from the Jomon period to the end of the Edo period in Japan. The parasitic helminthes whose eggs or symptoms were shown in the remains and bibliographies are the roundworm, whipworm, liver fluke, Yokogawa's fluke and the cestode, Diphyllobothrium sp. The first two are soil-transmitted nematodes and the other three parasitic helminths are those with which people are infected following eating raw fish. The infection routes provide valuable information on the environments, life-style and customs in those days. The eggs of the soil-transmitted parasites have the thick shells resistant to the environments. Humans are infected with the parasites after the eggs are orally ingested with soil, dust, vegetables grown with night soil or manure. When the custom of the night soil was started in the history of Japan was discussed with this infection route. In ancient times, feces are thought to have been discarded. In the Medieval Period, they were started to be used as a fertilizer. No mature types of manure were used until the modern times (already in the Edo period). To our idea, no recoveries of eggs of hookworms causing severe anemia do not necessarily mean that people were not infected with the parasites in those days because the eggs are covered with thin shells liable to rupture. The latter fact of the eggs of the platihelminths, C. sinensis, M. yokogawai and D. latum has something to do with Japanese traditional eating customs, unequivocally demonstrating that they ate raw fish from the Nara Period, at latest, until today. Whether eggs of the cestode (D. latum) are found in Jomon remains, Momijiyama Iseki, Hokkaido should be investigated. If no eggs of the cestode are found in their toilet site or elsewhere, it could be concluded that they did not have the custom of eating raw salmon. Such a conclusion would be itself a new fact. One of the effective treatments for the cestode (D. latum) sometimes still carried out in the 21st century in Japan, is binding worms from the anus using chopsticks. This method can be traced back to the Edo period as far as this investigation is concerned. Though the historical studies on anthelmintics are in progress, there seem to be no effective anthelmintics leaving nothing to be desired to the present authors' knowledge. PMID:20527291
Maki, Jun; Sakagami, Hiroshi; Kuwada, Masahiro; Caceres, Armando; Sekiya, Hiroshi; Tamai, Eiji
Wound botulism is a rare infectious disease that is becoming a frequent complication of parental drug use. Diagnosis is often difficult and based on clinical suspicion. We report the first Italian case of wound botulism due to intramuscular heroin injection in a 48-year-old man with an acute onset of slurred speech and dysphagia. The most considerable finding of electrophysiological study was the reduction in amplitude of compound muscle action potential which should be considered a useful initial electrodiagnostic sign in the clinical context of botulism. Alerting clinicians to botulism is crucial for a rapid diagnosis and appropriate treatment and thus decreasing mortality and complications. PMID:20552239
Rodolico, C; Barca, E; Fenicia, L; Anniballi, F; Sinardi, A U; Girlanda, P
Early complications can be defined as those occurring within 100 days after transplant. Both epithelial and endothelial damage represent the pathogenetic basis for the onset of the most frequent complications. Clinical features related to endothelial damage depend on the involved district or on the grade and type of general distribution. Veno-occlusive disease (VOD) most often occurs within the first 20
M Miano; M Faraci; G Dini; P Bordigoni
Lipoprotein subclass measurements by nuclear magnetic resonance spectroscopy improve the prediction of coronary artery disease in Type 1 Diabetes. A prospective report from the Pittsburgh Epidemiology of Diabetes Complications Study
\\u000a \\u000a Aim\\/hypothesis. To examine whether nuclear magnetic resonance lipoprotein spectroscopy improves the prediction of coronary artery disease\\u000a in patients with Type 1 diabetes, independently of conventional lipid and other risk factors.\\u000a \\u000a \\u000a \\u000a \\u000a Methods. A prospective nested case-control design of subjects with childhood onset Type 1 diabetes from the Pittsburgh Epidemiology\\u000a of Diabetes Complications Study was used. 59 controls were age-, sex-
S. S. Soedamah-Muthu; Y.-F. Chang; J. Otvos; R. W. Evans; T. J. Orchard
Arthritis in the small joints of the hand can be treated with arthrodesis or arthroplasty. Arthrodesis has known risks of infection, pain, and nonunion. Distal interphalangeal (DIP) arthroplasty has been successful in preserving motion and alleviating pain for distal DIP, proximal interphalangeal, and metacarpophalangeal joints. Unfortunately, complications arise that limit the success of surgery. Silicone implants have been reliable for many years but still present with the risks of infection, implant breakage, stiffness, and pain. Newer implant designs may limit some of these complications, but present with unique problems such as dislocations and loosening. It is not yet clear as to which type of implant provides the most reliable results, although implant arthroplasty appears to give better function than arthrodesis. Silicone arthroplasty does not lead to silicone synovitis and is a reliable procedure. Pyrocarbon implants are showing some promise, particularly in the osteoarthritic patient. PMID:20494746
Drake, Matthew L; Segalman, Keith A
A five year study on the ocular complications of diabetes compared results of eye examinations and biochemical analyses of diabetics with eye disease to nondiabetics with the same prognoses. For the same two populations, results of cataract surgery and po...
T. H. Kirmani
Objective To compare the effects of piglitazone and metformin on retinol-binding protein-4(RBP-4)and adiponcetin(APN)in patients with type 2 diabetes mellitus(T2DM)complicated with Non alcohol fatty acid liver disease(NAFLD). Methods Totally 60 T2DM patients complicated with NAFLD were equally and randomly divided into pioglitazone group and metform group. The levels of biochemical indicators including body mass index(BMI),glucose hemoglobin A1C(GHbA1C),insulin resistance(HOMA-IR),fasting blood glucose(FBG),fasting insulin(FIns),and serum triglycerides(TG)as well as serum RBP-4 and APN level were measured pre-treatment and 12 weeks after treatments. Results After 12 weeks of treaments,BMI,FBG,HOMA-IR,GHbA1C,FIns,and TG decreased(all P<0.05)in both piglitazone group and metform group. APN increased(all P<0.05)in both groups. RBP-4 decreased(P<0.05)in piglitazone group. Compare with the metform group,the levels of RBP-4,FIns,and HOMA-IR decreased and BMI increased in piglitazone group(P<0.05). Conclusion Piglitazone is superior to metoform in decreasing RBP-4 level and HOMA-IR in patients with T2DM complicated with NAFLD. PMID:24997826
Lu, Yang; Mei-Qing, Song; Qiu-Ling, Zhang; Lan, Shou; Shu-Fei, Zang; Yan-Li, Yang
Unusual Development of Iatrogenic Complex, Mixed Biliary and Duodenal Fistulas Complicating Roux-en-Y Antrectomy for Stenotic Peptic Disease of the Supraampullary Duodenum Requiring Whipple Procedure: An Uncommon Clinical Dilemma
Complex fistulas of the duodenum and biliary tree are severe complications of gastric surgery. The association of duodenal and major biliary fistulas occurs rarely and is a major challenge for treatment. They may occur during virtually any kind of operation, but they are more frequent in cases complicated by the presence of difficult duodenal ulcers or cancer, with a mortality rate of up to 35%. Options for treatment are many and range from simple drainage to extended resections and difficult reconstructions. Conservative treatment is the choice for well-drained fistulas, but some cases require reoperation. Very little is known about reoperation techniques and technical selection of the right patients. We present the case of a complex iatrogenic duodenal and biliary fistula. A 42-year-old Caucasian man with a diagnosis of postoperative peritonitis had been operated on 3 days earlier; an antrectomy with a Roux-en-Y reconstruction for stenotic peptic disease was performed. Conservative treatment was attempted with mixed results. Two more operations were required to achieve a definitive resolution of the fistula and related local complications. The decision was made to perform a pancreatoduodenectomy with subsequent reconstruction on a double jejunal loop. The patient did well and was discharged on postoperative day 17. In our experience pancreaticoduodenectomy may be an effective treatment of refractory and complex iatrogenic fistulas involving both the duodenum and the biliary tree.
Polistina, Francesco A.; Costantin, Giorgio; Settin, Alessandro; Lumachi, Franco; Ambrosino, Giovanni
Percutaneous and surgical ablation procedures are flourishing, in large part because of the relative paucity and the acceptability\\u000a of complications. However, serious and fatal complications have occurred, albeit rarely, with ablation. This chapter identifies\\u000a those complications, describes their underlying cause, and, when possible, discusses strategies to avoid them.
Lawrence Cheung; Tito Livraghi; Luigi Solbiati; Gerald D. Dodd; Eric vanSonnenberg
Whole organ vascularized pancreatic transplant is a recognized treatment for diabetes and is increasingly being performed worldwide. The procedure itself is complex and is associated with significant mortality and morbidity. Despite improvements in surgical techniques, postoperative complications of pancreatic transplantation are still common and include graft rejection, pancreatitis, peripancreatic fluid collections, exocrine leaks, vascular thrombosis, and hemorrhage. In this pictorial essay, we review clinical presentation and imaging features of these complications. We also briefly discuss technique and complications of islet cell transplants. PMID:20563577
Liong, S Y; Dixon, R E; Chalmers, N; Tavakoli, A; Augustine, T; O'Shea, S
Shoulder arthroscopy is generally a safe and effective method for treating a wide variety of shoulder pathology. Fortunately, complications following shoulder arthroscopy are rare, with reported rates between 4.6% and 10.6%.¹?? These rates may be underestimated, as underreporting of complications and varying definitions of the term complication are likely. During shoulder arthroscopy, complications may occur at numerous points. The surgeon must be aware of potential problems and take necessary measures to prevent them. This article describes common complications after arthroscopic shoulder surgery. Although failure of treatment and postoperative stiffness are undesirable outcomes, they are not described. PMID:20608603
Marecek, Geoffrey S; Saltzman, Matthew D
Diabetic encephalopathies are now accepted complications of diabetes. They appear to differ in type 1 and type 2 diabetes\\u000a as to underlying mechanisms and the nature of resulting cognitive deficits. The increased incidence of Alzheimer’s disease\\u000a in type 2 diabetes is associated with insulin resistance, hyperinsulinemia and hyperglycemia, and commonly accompanying attributes\\u000a such as hypercholesterolemia, hypertension and obesity. The relevance
Anders A. F. Sima
Over the last decade, the laparoscopic approach to antireflux surgery has been widely applied, resulting in improved early\\u000a outcomes and greater patient acceptance of surgery for gastroesophageal reflux disease. However, although short-term outcomes\\u000a are probably better overall than those following open surgery, it has become apparent that the laparoscopic approach is associated\\u000a with an increased risk of some complications, and
D. I. Watson; A. C. de Beaux
One hundred forty patients who had complicated diverticular disease were identified in a retrospective review at the Lahey\\u000a Clinic between 1967 and 1982. Of these patients, 86 underwent resection with primary anastomosis with a 1 percent mortality\\u000a rate and an 18 percent morbidity rate; 13 had resection with anastomosis and creation of a proximal colostomy with no death\\u000a and a
Alan W. Hackford; David J. Schoetz; John A. Coller; Malcolm C. Veidenheimer
Laparoscopic techniques performed in the urologic setting have received great attention in the past decade. With the development of improved laparoscopic instrumentation, approaches to gonadal, renal, prostate, and bladder diseases have been successfully performed. A discussion of urologic laparoscopy (UL) with particular attention to potential complications and limitations is presented. Awareness of these evolving technologies remains critical to all surgeons with an interest in laparoscopy. PMID:15630945
Madeb, Ralph; Koniaris, Leonidas G; Patel, Hitendra R H; Dana, James F; Nativ, Ofer; Moskovitz, Boaz; Erturk, Erdal; Joseph, Jean V
We describe the case of a male patient suffering from long-lasting Crohn's disease of the small bowel who developed thyroiditis Hassimoto, Raynaud's phenomenon, and primary biliary cirrhosis, during the course of the underlying bowel disease. It is not clear whether these co-morbidities appeared coincidentally, or because they share some common immunopathogenetic mechanisms. In this patient, Crohn's disease favorably responded to the treatment with an anti-TNF-? agent (adalimumab). The serum titers of antimitochondrial antibodyies and cholestatic enzymes considerably reduced during the 3-year treatment with the biologic agent. Raynaud's phenomenon, also, completely disappeared. Bearing in mind the possible involvement of TNF-? in the pathogenesis of primary biliary cirrhosis, it could be argued that the clinical and laboratory improvement of liver disease, as well as the reduction in serum titers of antimitochondrial antibodies, might be due to the anti-TNF-? action of adalimumab. We suggest that it would be worth further investigating the role of biologic agents in the treatment of patients with primary biliary cirrhosis.
Durakis, Spyros; Merikas, Emmanuel
A 48 year old man was transferred to our department with cardiogenic shock, pyrexia, a high white cell count and significant serum troponin T level. Clinical evaluation revealed severe mitral regurgitation secondary to a flail of both mitral valve leaflets. An emergency cardiac catheterisation did not reveal any significant coronary artery disease. Left ventricular angiogram and echocardiography demonstrated a good
Federico Bizzarri; Consalvo Mattia; Massimo Ricci; Flaminia Coluzzi; Vincenzo Petrozza; Giacomo Frati; Giuseppe Pugliese; Luigi Muzzi
We report a rare case of orbital apex syndrome following epidural steroid injections of the lumbar spine in an immunocompetent individual with osteomyelitis and discitis caused by Aspergillus fumigatus. We suspect that the craniospinal venous system, also known as the Batson's plexus, was the main route for steroid-facilitated disease propagation from the spine to intracranial structures.
Camargo, Jose F.; Seriburi, Vimon; Tenner, Michael; El Khoury, Marc Y.
Diabetes mellitus affects virtually every organ system in the body and the degree of organ involvement depends on the duration and severity of the disease, and other co-morbidities. Gastrointestinal (GI) involvement can present with esophageal dysmotility, gastro-esophageal reflux disease (GERD), gastroparesis, enteropathy, non alcoholic fatty liver disease (NAFLD) and glycogenic hepatopathy. Severity of GERD is inversely related to glycemic control and management is with prokinetics and proton pump inhibitors. Diabetic gastroparesis manifests as early satiety, bloating, vomiting, abdominal pain and erratic glycemic control. Gastric emptying scintigraphy is considered the gold standard test for diagnosis. Management includes dietary modifications, maintaining euglycemia, prokinetics, endoscopic and surgical treatments. Diabetic enteropathy is also common and management involves glycemic control and symptomatic measures. NAFLD is considered a hepatic manifestation of metabolic syndrome and treatment is mainly lifestyle measures, with diabetes and dyslipidemia management when coexistent. Glycogenic hepatopathy is a manifestation of poorly controlled type 1 diabetes and is managed by prompt insulin treatment. Though GI complications of diabetes are relatively common, awareness about its manifestations and treatment options are low among physicians. Optimal management of GI complications is important for appropriate metabolic control of diabetes and improvement in quality of life of the patient. This review is an update on the GI complications of diabetes, their pathophysiology, diagnostic evaluation and management. PMID:23772273
Krishnan, Babu; Babu, Shithu; Walker, Jessica; Walker, Adrian B; Pappachan, Joseph M
Bronchio biliary fistula in adults is a rare event defined by the passage of bile into the bronchus and the sputum (biloptysis).Typically these lesions occur in the congenital form, as a result of thoracoabdominal trauma, or in rare instances as a result of iatrogenic injury or long-standing biliary tract disease and obstruction. In this paper, we report a novel case of a fatal bronchobiliary fistula that developed in a 67-year-old Chinese male with Oriental cholangiohepatitis. To our knowledge, this is the first case report of a bronchobiliary fistula complicating the clinical management of a patient with this disease.
Howman, SF; Feng, TL; Chamberlain, RS; Blumgart, LH
Dermatomyositis is an inflammatory disease of unclear etiology with characteristic cutaneous and musculoskeletal findings. Amyopathic dermatomyositis is a subtype without musculoskeletal involvement. Many cases of dermatomyositis are associated with underlying malignancy, but pulmonary manifestations can also be seen, the most common of which is interstitial lung disease. Pneumomediastinum is a rare complication that is important for clinicians to recognize, as it may be fatal if left untreated. The sudden onset of facial edema and shortness of breath in the setting of dermatomyositis should raise the suspicion of this condition.
Tang, Randy; Green, Justin J.
Gibson, G. J. and Davis, P. (1974).Thorax, 29, 726-731. Respiratory complications of relapsing polychondritis. The respiratory function of a patient with relapsing polychondritis is described. He had severe airflow obstruction due to disease of both the extra and intrathoracic large airways. Evidence of small airways disease was lacking. The airflow obstruction was probably due to a combination of structural narrowing and an enhanced dynamic effect. Despite the severity of his disease the patient's exercise capacity was only slightly reduced but he developed carbon dioxide retention on exercise. Involvement of the airways is a common feature of this rare disease and demands full physiological and radiographic assessment if tracheostomy or other surgical procedure is contemplated. Images
Gibson, G. J.; Davis, P.
Wound botulism is a rare infectious disease that is becoming a frequent complication of parental drug use. Diagnosis is often\\u000a difficult and based on clinical suspicion. We report the first Italian case of wound botulism due to intramuscular heroin\\u000a injection in a 48-year-old man with an acute onset of slurred speech and dysphagia. The most considerable finding of electrophysiological\\u000a study
C. Rodolico; E. Barca; L. Fenicia; F. Anniballi; A. U. Sinardi; P. Girlanda
We analysed the outcome and hospitalization requirements of the first 100 patients (Hodgkin's disease (HD), N=13; multiple myeloma (MM), N=14; CLL, N=12; non-Hodgkin's lymphoma (NHL), N=17; myelodysplastic syndrome (MDS), N=18; AML, N=24 and CML, N=2) treated in Denmark with haematopoietic cell transplantation after non-myeloablative conditioning with TBI 2 Gy±fludarabine. The cumulative incidence of acute GVHD grade II–IV and extensive chronic
B Kornblit; T Masmas; H O Madsen; L P Ryder; A Svejgaard; B Jakobsen; H Sengeløv; G Olesen; C Heilmann; E Dickmeiss; S L Petersen; L Vindeløv
A 48-year-old woman was diagnosed and treated for Graves' disease (GD) in 1999 but she discontinued treatment at her own discretion. In 2011, she was admitted to a local hospital for management of thyrotoxic crisis. Treatment with propylthiouracil, iodide potassium (KI), and prednisolone (PSL) was started, which resulted in improvement of the general condition. PSL and KI were discontinued before she was transferred to our hospital. At the local hospital, fasting plasma glucose (FPG) was 212 mg/dL and hemoglobin A1c concentration was 11.2%; intensive insulin therapy had been instituted. Upon admission to our hospital, FPG level was 122 mg/dL, but insulin secretion was compromised, suggesting aggravation of thyroid function and deterioration of glycemic control. The FPG level increased to 173 mg/dL; continuous glucose monitoring (CGM) identified dawn phenomenon at approximately 0400 h. Resumption of KI resulted in improvement of FPG and disappearance of the dawn phenomenon, as assessed by CGM. These results indicate that in patients with compromised insulin secretion, hyperthyroidism can induce elevation of not only postprandial blood glucose, but also FPG level due to the dawn phenomenon and that the dawn phenomenon can be alleviated with improvement in thyroid function. To our knowledge, no studies have assessed glucose variability by CGM before and after treatment of Graves' disease. The observations made in this case shed light on the understanding of abnormal glucose metabolism associated with Graves' disease. PMID:24420389
Torimoto, Keiichi; Okada, Yosuke; Arao, Tadashi; Mori, Hiroko; Yamamoto, Sunao; Narisawa, Manabu; Kurozumi, Akira; Tanaka, Yoshiya
cute complications such as veno-occlusive disease of the liver, acute and chronic graft- vs-host disease (GVHD), and infectious conditions remain major obstacles for the suc- cess of allogeneic hematopoietic stem cell transplantation (HSCT). Progress in allo- geneic HSCT depends on several factors, including the adequate prevention and management of associated complications, advances in the conventional management of diseases currently treated
Imad A. Tabbara; Kathy Zimmerman; Connie Morgan; Zeina Nahleh
Interaction between a jet and its neighbors in a still ambient fluid is dealt with. A model for the merging of an infinite line of equally spaced identical jets in a still ambient fluid is developed. The velocity and tracer distributions in the model are determined based on the summation of momentum fluxes and tracer fluxes, respectively. Laser-induced fluorescence experiments
H. J. Wang; M. J. Davidson
This four-volume student text is designed for use by Air Force personnel enrolled in a self-study extension course for apprentice still photographic specialists. Covered in the individual volumes are general subjects (career ladder progression, security, photographic safety, and photographic laboratory administration); still photographic…
Air Univ., Gunter AFS, Ala. Extension Course Inst.
...2013-07-01 2013-07-01 false Still photography. 705.10 Section 705.10 National...AFFAIRS REGULATIONS Â§ 705.10 Still photography. (a) Policy and procedures on...are set forth in the Manual of Naval Photography, OPNAVINST 3150.6D. (c)...
...2009-07-01 2009-07-01 false Still photography. 705.10 Section 705.10 National...AFFAIRS REGULATIONS Â§ 705.10 Still photography. (a) Policy and procedures on...are set forth in the Manual of Naval Photography, OPNAVINST 3150.6D. (c)...
...2010-07-01 2010-07-01 false Still photography. 705.10 Section 705.10 National...AFFAIRS REGULATIONS Â§ 705.10 Still photography. (a) Policy and procedures on...are set forth in the Manual of Naval Photography, OPNAVINST 3150.6D. (c)...
Since the causative role of Helicobacter pylori in peptic ulcer and gastritis was established, a number of advances have been made. Helicobacter virulence factors have been identified, the changes it causes in gastric acid secretion has been elucidated, and the entire genome of H. pylori has been mapped. Multiple lines of evidence indicate a strong link between the bacterium and noncardia gastric cancer. The infection can be confidently diagnosed by noninvasive serologic tests and the urea breath test. Triple therapy is almost always curative, and the infection almost never recurs in Canadian adults, but eradicating the bacteria in the absence of peptic ulcer only rarely leads to resolution of dyspepsia. New studies suggest that treating Helicobacter may increase the risk of peptic esophagitis and adenocarcinoma of the esophagus and cardia.
Complications associated with hip arthroscopy occur between 1.6% and 5%. Fortunately, with the greater understanding of the causes and advancements in techniques and equipment, the incidence is declining. Most of the complications were transient neuropraxias and fluid extravasations resulting in no permanent damage. Severe scuffing of two femoral heads and one case of avascular necrosis were considered serious and permanent, thereby resulting in a 0.5% rate in our series for significant complications. PMID:11675890
Sampson, T G
As posttransplant longevity has increased, nonimmune complications related to the transplant and posttransplant course have emerged as important factors in defining long-term outcomes. The incidence of, and risk factors for these complications may vary by transplanted organ based on immunosuppressive protocols and preexisting risk factors. This article discusses the relevant nonimmune complications associated with posttransplant care, with a focus on risk factors and management strategies. PMID:20371049
Choquette, Monique; Goebel, Jens W; Campbell, Kathleen M
Sixty patients with chloroquine induced psychiatric complications are reported in the present series. Psychosis was the most common complication followed by anxiety state and seizures. The maximum number of patients were between the ages of 6 and 10 years and females (70.0%) outnumbered the males (30.0%). Headache and sleeplessness were found to be more common amongpatients developing psychiatric complications of chloroquine. The symptoms disappeared within 2 to 21 days after the discontinuation of chloroquine.
Bhatia, M.S.; Malik, S.C.
Neurologic complications are relatively common after solid organ transplantation and affect 15%-30% of liver transplant recipients. Etiology is often related to immunosuppressant neurotoxicity and opportunistic infections. Most common complications include seizures and encephalopathy, and occurrence of central pontine myelinolysis is relatively specific for liver transplant recipients. Delayed allograft function may precipitate hepatic encephalopathy and neurotoxicity of calcineurin inhibitors typically manifests with tremor, headaches and encephalopathy. Reduction of neurotoxic immunosuppressants or conversion to an alternative medication usually result in clinical improvement. Standard preventive and diagnostic protocols have helped to reduce the prevalence of opportunistic central nervous system (CNS) infections, but viral and fungal CNS infections still affect 1% of liver transplant recipients, and the morbidity and mortality in the affected patients remain fairly high. Critical illness myopathy may also affect up to 7% of liver transplant recipients. Liver insufficiency is also associated with various neurologic disorders which may improve or resolve after successful liver transplantation. Accurate diagnosis and timely intervention are essential to improve outcomes, while advances in clinical management and extended post-transplant survival are increasingly shifting the focus to chronic post-transplant complications which are often encountered in a community hospital and an outpatient setting.
Zivkovic, Sasa A
The trend of body piercing has grown in popularity in the past decade within the general population and especially among young adults. Complications of body piercing include local inflammation and infections, but severe complications are also possible and largely underestimated. People are usually not aware of the risks before making a piercing, and their medical history, medication and comorbidities are largely neglected by the people who realise the piercing. This article presents a review of the complications that a primary care physician may observe, for a patient who wishes to make a piercing, or presents complications due to the implementation of such a device. PMID:24734366
Friedrich, L; Madrid, C; Odman-Jaques, M; Yersin, B; Carnon, P N
Dermatomyositis (DM) and polymyosits (PM) are systemic autoimmune diseases whose pathogeneses remain unclear. Neutrophil extracellular traps (NETs) are reputed to play an important role in the pathogenesis of autoimmune diseases. This study tests the hypothesis that NETs may be pathogenic in DM/PM. Plasma samples from 97 DM/PM patients (72 DM, 25 PM) and 54 healthy controls were tested for the capacities to induce and degrade NETs. Plasma DNase I activity was tested to further explore possible reasons for the incomplete degradation of NETs. Results from 35 DM patients and seven PM patients with interstitial lung disease (ILD) were compared with results from DM/PM patients without ILD. Compared with control subjects, DM/PM patients exhibited a significantly enhanced capacity for inducing NETs, which was supported by elevated levels of plasma LL-37 and circulating cell-free DNA (cfDNA) in DM/PM. NETs degradation and DNase I activity were also decreased significantly in DM/PM patients and were correlated positively. Moreover, DM/PM patients with ILD exhibited the lowest NETs degradation in vitro due to the decrease in DNase I activity. DNase I activity in patients with anti-Jo-1 antibodies was significantly lower than in patients without. Glucocorticoid therapy seems to improve DNase I activity. Our findings demonstrate that excessively formed NETs cannot be degraded completely because of decreased DNase I activity in DM/PM patients, especially in patients with ILD, suggesting that abnormal regulation of NETs may be involved in the pathogenesis of DM/PM and could be one of the factors that initiate and aggravate ILD. PMID:24611519
Zhang, S; Shu, X; Tian, X; Chen, F; Lu, X; Wang, G