This case report demonstrates the otologic complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis (SJS/TEN). A five year-old male was admitted to the burn unit with the diagnosis of SJS/TEN believed secondary to acetaminophen use. After resolution of the acute phase, the patient was left with a severe auricular deformity with scarring and complete occlusion of the right external auditory canal (EAC). The patient underwent meatoplasty, canaloplasty, and tympanoplasty with subsequent placement of a molded stent in order to maintain patency of his EAC. Four years later, the patient has a patent right EAC with adequate hearing. PMID:24933358
Hotaling, Jeffrey M; Hotaling, Andrew J
Non-steroidal anti-inflammatory drugs are commonly prescribed in dental practice after minor oral surgical procedures such as tooth extraction. Diclofenac sodium is one of the non-steroidal anti-inflammatory drugs widely used for pain relief in dentistry. Although adverse reactions to these drugs are rare, at times they can cause a life-threatening phenomenon. Stevens-Johnson syndrome is one such potentially lethal adverse drug reaction. Most reported cases of analgesic-induced Stevens-Johnson syndrome were due to oxicams or propionic acid derivatives. There are very few detailed reports of Stevens-Johnson syndrome due to use of diclofenac. We report here a case of Stevens-Johnson syndrome which occurred due to use of diclofenac sodium. The clinical features of this condition and multidisciplinary management of the patient are described in brief. PMID:20587964
Shetty, Shishir R; Chatra, Laxmikanth; Shenai, Prashanth; Rao, Prasanna K
Stevens-Johnson syndrome (SJS) is a severe form of erythema multiforme that primarily affects skin and mucous membranes. In Malawi, manifestations of SJS may become more common in human immunodeficiency virus–infected patients receiving nevirapine-based antiretroviral therapy (ART) because the CD4 cell threshold for starting ART has increased from 250 to 350 cells/?L. We describe a patient with severe ocular complications from SJS that developed soon after initiation of nevirapine-based ART and cotrimoxazole preventive treatment, which led to blindness. We draw attention to preventive measures that can potentially reduce permanent ocular damage from SJS.
Schulze Schwering, Markus; Kayange, Petros; van Oosterhout, Joep J.; Spitzer, Martin S.
Cushing's syndrome is a pathological condition associated with excessive cortisol production, the commonest etiology being Cushing's disease. Corticosteroids in high doses have been used in the management of Steven Johnson Syndrome (SJS) with favourable outcome. We describe a patient with Cushing's disease who developed SJS, one week after taking sperulina a product from sea-weed while waiting for transphenoidal surgery. PMID:20527277
Mustafa, N; Periyasamy, P; Kamaruddin, N
Phenobarbital, an antiepileptic agent has numerous adverse reactions including Stevens– Johnson syndrome (SJS), a rare medical emergency. A 12-year-old male epileptic child with phenobarbital-induced SJS was referred for the management of severe pain in relation to extensively decayed molar tooth and oral mucosal ulcerations. The patient was managed by withdrawal of phenobarbital and palliative treatment of the lesions.
Gaur, Sumit; Agnihotri, Rupali
The objective of this study was to demonstrate the clinical profiles of Stevens-Johnson syndrome (SJS) in Thai patients, and to compare those clinical features between younger and older patients. Medical records of all patients with SJS who were admitted to Srinagarind Hospital Medical School, Khon Kaen, Thailand, from January 2002 to December 2014 were reviewed. Epidemiological features, etiologies, treatment and clinical outcomes were collected. There were 45 patients with SJS during the 10-year period. Females were the majority (57.8%) and the median age was 49 years. Hepatitis was the most frequent complication (67.5%). Phenytoin (15.6%), sulfonamide drugs (15.6%) and allopurinol (13.3%) were implicated as leading causes of SJS. Steroids were prescribed in 37 cases (82.2%). The mortality rate was 4.4%. Comparing older patients to younger patients, allopurinol appeared to be the main instigating drug to develop SJS with an odds ratio of 5.6 (95% confidence interval, 2.8-10.6). In conclusion, clinical features of Thai patients with SJS were similar to other reports. Allopurinol had the strongest association with SJS in older patients as compared to the younger ones. PMID:24815085
Limpawattana, Panita; Choonhakarn, Charoen; Kongbunkiat, Kannikar
Toxic epidermal necrolysis and Stevens–Johnson syndrome are severe cutaneous drug reactions of unknown mechanism. Nitric oxide can cause apoptosis and necrosis. The inducible form of nitric oxide synthase generates large amounts of nitric oxide and has been described in human skin. We propose that a large burst of nitric oxide in toxic epidermal necrolysis and Stevens–Johnson syndrome may cause the
Lisa H. Lerner; Abrar A. Qureshi; Bhaskar V. Reddy; Ethan A. Lerner
Stevens-Johnson syndrome is an uncommon inflammatory skin disorder in which immune mechanisms, cytotoxic reactions, and delayed hypersensitivity seem to be involved. Herein, an unusual case with strange complaint of"bus allergy" is presented, suffering from severe generalized itching and skin lesions, followed by ulcers in his mouth and genitalia. The diagnosis of Stevens-Johnson syndrome was made and appropriate treatment was advised. However, detailed medical history revealed a completely different cause of his allergic reactions, as he had occasionally used dimenhydrinate because of motion sickness in his history. Therefore, adverse drug reaction to dimenhydrinate was considered as the main underlying cause of the disease. In conclusion, thorough medical history should be taken to make a definitive diagnosis and identify the underlying disease, since accurate diagnosis and appropriate treatment can prevent further complications. PMID:24558775
Mohammadzadeh, Iraj; Khaledi, Mojdeh; Rezaei, Nima
Toxic epidermal necrolysis (TEN) and Stevens Johnson Syndrome (SJS) are severe adverse cutaneous drug reactions that predominantly involve the skin and mucous membranes. Both are rare, with TEN and SJS affecting approximately 1or 2/1,000,000 annually, and are considered medical emergencies as they are potentially fatal. They are characterized by mucocutaneous tenderness and typically hemorrhagic erosions, erythema and more or less severe epidermal detachment presenting as blisters and areas of denuded skin. Currently, TEN and SJS are considered to be two ends of a spectrum of severe epidermolytic adverse cutaneous drug reactions, differing only by their extent of skin detachment. Drugs are assumed or identified as the main cause of SJS/TEN in most cases, but Mycoplasma pneumoniae and Herpes simplex virus infections are well documented causes alongside rare cases in which the aetiology remains unknown. Several drugs are at "high" risk of inducing TEN/SJS including: Allopurinol, Trimethoprim-sulfamethoxazole and other sulfonamide-antibiotics, aminopenicillins, cephalosporins, quinolones, carbamazepine, phenytoin, phenobarbital and NSAID's of the oxicam-type. Genetic susceptibility to SJS and TEN is likely as exemplified by the strong association observed in Han Chinese between a genetic marker, the human leukocyte antigen HLA-B*1502, and SJS induced by carbamazepine. Diagnosis relies mainly on clinical signs together with the histological analysis of a skin biopsy showing typical full-thickness epidermal necrolysis due to extensive keratinocyte apoptosis. Differential diagnosis includes linear IgA dermatosis and paraneoplastic pemphigus, pemphigus vulgaris and bullous pemphigoid, acute generalized exanthematous pustulosis (AGEP), disseminated fixed bullous drug eruption and staphyloccocal scalded skin syndrome (SSSS). Due to the high risk of mortality, management of patients with SJS/TEN requires rapid diagnosis, evaluation of the prognosis using SCORTEN, identification and interruption of the culprit drug, specialized supportive care ideally in an intensive care unit, and consideration of immunomodulating agents such as high-dose intravenous immunoglobulin therapy. SJS and TEN are severe and life-threatening. The average reported mortality rate of SJS is 1-5%, and of TEN is 25-35%; it can be even higher in elderly patients and those with a large surface area of epidermal detachment. More than 50% of patients surviving TEN suffer from long-term sequelae of the disease. PMID:21162721
Harr, Thomas; French, Lars E
Toxic epidermal necrolysis (TEN) and Stevens Johnson Syndrome (SJS) are severe adverse cutaneous drug reactions that predominantly involve the skin and mucous membranes. Both are rare, with TEN and SJS affecting approximately 1or 2/1,000,000 annually, and are considered medical emergencies as they are potentially fatal. They are characterized by mucocutaneous tenderness and typically hemorrhagic erosions, erythema and more or less severe epidermal detachment presenting as blisters and areas of denuded skin. Currently, TEN and SJS are considered to be two ends of a spectrum of severe epidermolytic adverse cutaneous drug reactions, differing only by their extent of skin detachment. Drugs are assumed or identified as the main cause of SJS/TEN in most cases, but Mycoplasma pneumoniae and Herpes simplex virus infections are well documented causes alongside rare cases in which the aetiology remains unknown. Several drugs are at "high" risk of inducing TEN/SJS including: Allopurinol, Trimethoprim-sulfamethoxazole and other sulfonamide-antibiotics, aminopenicillins, cephalosporins, quinolones, carbamazepine, phenytoin, phenobarbital and NSAID's of the oxicam-type. Genetic susceptibility to SJS and TEN is likely as exemplified by the strong association observed in Han Chinese between a genetic marker, the human leukocyte antigen HLA-B*1502, and SJS induced by carbamazepine. Diagnosis relies mainly on clinical signs together with the histological analysis of a skin biopsy showing typical full-thickness epidermal necrolysis due to extensive keratinocyte apoptosis. Differential diagnosis includes linear IgA dermatosis and paraneoplastic pemphigus, pemphigus vulgaris and bullous pemphigoid, acute generalized exanthematous pustulosis (AGEP), disseminated fixed bullous drug eruption and staphyloccocal scalded skin syndrome (SSSS). Due to the high risk of mortality, management of patients with SJS/TEN requires rapid diagnosis, evaluation of the prognosis using SCORTEN, identification and interruption of the culprit drug, specialized supportive care ideally in an intensive care unit, and consideration of immunomodulating agents such as high-dose intravenous immunoglobulin therapy. SJS and TEN are severe and life-threatening. The average reported mortality rate of SJS is 1-5%, and of TEN is 25-35%; it can be even higher in elderly patients and those with a large surface area of epidermal detachment. More than 50% of patients surviving TEN suffer from long-term sequelae of the disease.
Vulvovaginal sequelae of Stevens-Johnson syndrome and toxic epidermal necrolysis are well documented in the literature, although little consensus exists about effective prevention strategies. This review summarizes the available literature and offers expert opinion about how to minimize long-term vaginal impairment from these rare but often devastating illnesses.
Kaser, Daniel J; Reichman, David E; Laufer, Marc R
Stevens-Johnson syndrome (SJS) is a severe cutaneous eruption that most often appears as an adverse reaction to medication. In this report, we present two children with brain tumour who developed SJS while receiving cranial irradiation and anticonvulsant therapy with phenobarbital. Concomitant application of these two therapies may play an important role in the occurrence of the disease. PMID:17589805
Ruggiero, Antonio; Buonuomo, Paola Sabrina; Maurizi, Palma; Cefalo, Maria Giuseppina; Cefalo, Maria Pia; Corsello, Mirta; Riccardi, Riccardo
Stevens-Johnson syndrome (SJS) is a serious and potentially life-threatening disease. Vanishing bile duct syndrome (VBDS) is a rare cause of progressive cholestasis. Both syndromes are mostly related with drugs. We report a case of a patient with ciprofloxacin-induced SJS and acute onset of VBDS, and reviewed the related literature. It is the first case of ciprofloxacin-induced VBDS successfully treated with tacrolimus. This case reminds physicians of the importance of drug reactions, their severity, techniques for diagnosis and methods of management. PMID:18698687
Okan, Gokhan; Yaylaci, Serpil; Peker, Onder; Kaymakoglu, Sabahattin; Saruc, Murat
Stevens-Johnson syndrome (SJS) is a serious and potentially life-threatening disease. Vanishing bile duct syndrome (VBDS) is a rare cause of progressive cholestasis. Both syndromes are mostly related with drugs. We report a case of a patient with ciprofloxacin-induced SJS and acute onset of VBDS, and reviewed the related literature. It is the first case of ciprofloxacin-induced VBDS successfully treated with tacrolimus. This case reminds physicians of the importance of drug reactions, their severity, techniques for diagnosis and methods of management.
Okan, Gokhan; Yaylaci, Serpil; Peker, Onder; Kaymakoglu, Sabahattin; Saruc, Murat
Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN)-like lesions in acute cutaneous lupus erythematosus [LE]) are an unusual manifestation of systemic LE. We describe a patient with widespread vesiculobullous lesions diagnosed as SJS/TEN-like acute cutaneous LE as the initial presentation of systemic LE. Stevens-Johnson syndrome/TEN-like LE may be differentiated from other vesiculobullous lesions by factors including a history of recent LE exacerbation, photodistribution of lesions, lack of a precipitating infection or medication exposure, minimal mucosal involvement, a prolonged course, response steroid treatment, and histologic and immunofluorescence findings. It is paramount to identify SJS/TEN-like LE as this condition requires early and aggressive intervention. The optimal treatment approach for SJS/TEN-like LE is unclear, and although some case reports have shown glucocorticoids to be useful, there are also reports of cases in which additional measures, such as intravenous immunoglobulin and plasmapheresis, were required to achieve a response. Our patient's condition was refractory to high-dose corticosteroids and intravenous immunoglobulin but was successfully treated using plasma exchange. As such, this treatment may hold potential for improving the care of other patients with refractory SJS/TEN-like LE. PMID:24662561
Baker, Mary Grace; Cresce, Nicole D; Ameri, Mariam; Martin, Adam A; Patterson, James W; Kimpel, Donald L
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe adverse cutaneous reactions to drugs. We describe the case of a 19 year old patient with SJS/TEN overlap syndrome, who developed severe interstitial pneumonia after she had received antiepileptic drugs. A cytomegalovirus infection was diagnosed by Real Time Polymerase Chain Reaction (RT-PCR) detection on Bronchoalveolar lavage. Based on observations on biological data, temporal relationship, and clinical features, it could be inferred that the reactivation of cytomegalovirus with viral replication can predispose a person to TEN-SJS. We discuss here, in the light of the current literature, the probable association between drug-induced SJS-TEN and fulminant reactivation of cytomegalovirus.
Tagajdid, Mohamed Rida; Doblali, Taoufik; Elannaz, Hicham; Hammi, Salaheddine; Belfequih, Bouchra; Mrani, Saad
Stevens-Johnson syndrome (SJS) and Toxic epidermal necrolysis (TEN) are characterized by extensive blistering of the skin and mucosa; they are considered as one disease entity with varying severity. They are rare but potentially life-threatening and accompanied by high mortality. A clear clinical diagnosis is needed to direct specific therapy, but supportive therapy remains most important. In order to identify and withdraw the inducing drug, a very detailed and thorough medication history has to be obtained. Among the highly suspected (strongly associated) agents are allopurinol, antibacterial sulfonamides, non-steroidal anti-inflammatory drugs of the oxicam type, various anti-epileptics and nevaripine. Together they account for more than half of the cases of SJS/TEN. Although a drug is not always the cause, it is considered very like in approximately 75% of cases. Infections have also to be considered as etiologic factors. PMID:24820799
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare life-threatening conditions almost exclusively attributed to drugs. The incidence in children is lower than in adults and has a better outcome. Mycosplama pneumoniae infection may be involved in some cases of paediatric SJS. The main etiologic factors for both SSJ and TEN are sulphonamides and anticonvulsants, followed by penicillins and non-steroidal anti-inflammatory drugs. In rare instances, paracetamol is the only suspected drug. By contrast with adults, allopurinol, oxicams and nevirapine are not identified as causative agents in children, probably due to differences in drug prescriptions. The only aspects of treatment that have been proved to improve survival are the rapid withdrawal of the suspected offending drugs and an optimal supportive therapy with emphasis in nutritional support, accompanied by management of denuded skin areas. The use of specific therapies remains controversial. PMID:23873883
Ferrandiz-Pulido, Carla; Garcia-Patos, Vicente
The pathophysiology of erythema multiforme (EM), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN) is unclear. Whether autoantibodies against desmoplakin (Dp) I and II play a pathogenic role or result from an epitope spreading phenomenon is uncertain. Our aim was to characterize the keratinocyte antigens recognized in EM, TEN and SJS. Of 33 patients studied, 2 had TEN, 1 SJS, 9 EM major and 21 EM minor, according to Roujeau's criteria. All sera were studied by indirect immunofluorescence (IIF), immunoblotting and immunoprecipitation. Twenty normal sera were used as controls. 10/33 sera reacted with polypeptides of 215 and/or 250-kDa molecular mass, which co-migrate with Dp I and II as assessed by an anti-Dp I and II monoclonal antibody on IB. In IP, none of the anti-Dp I and -Dp II 10 patient sera immunoprecipitated Dp I and/or II from radiolabeled keratinocyte extracts. Two of 10 patient sera (SJS, EM minor) reacted with DpI and II when denaturated by the IB procedure. The reactivity against intracellular antigens DpI and II as denaturated proteins may result from the epidermal damage produced by aggressive autoreactive T cells, playing therefore only a secondary role in the pathogenesis of the disease. PMID:21233064
Cozzani, Emanuele; Di Zenzo, Giovanni; Calabresi, Valentina; Caproni, Marzia; Schena, Donatella; Quaglino, Pietro; Marzano, Angelo V; Fabbri, Paolo; Rebora, Alfredo; Parodi, Aurora
Stevens-Johnson syndrome (SJS) is a debilitating condition involving the skin and mucous membranes. It is commonly the result of adverse drug reactions but can also be caused by infections. A predisposition to recurrent viral infections, such as in the case of natural killer (NK) cell dysfunction, may manifest with virally induced SJS. We present this case to suggest a possible association between NK cell dysfunction and recurrent SJS. An 11-year-old girl presents with recurring erythema, erosion, and ulceration of oral and vaginal mucosa. Small fluid-filled bumps would appear, leading to blistering and later sloughing of mucosal tissue, and bleeding would ensue. Seven separate episodes have occurred over an 8-year period, with each episode being preceded by symptoms of an upper respiratory infection. NK cell function assays were performed and NK cell phenotyping was ordered. NK cell assays showed decreased NK cell cytotoxicity at all ratios of K562 target cells. NK cell surface expression evaluation showed an immature phenotype but normal overall numbers of NK cells. NK cells are a pivotal part of the innate immune system, and, among other things, provide defense of viral infection. This case represents the manifestation of recurrent SJS as a result the lack of protection from viral illness, usually provided by NK cells in the healthy immune system.
Venglarcik, John; Bludorn, Matt; Chernin, Leah; Swender, David; Tcheurekjian, Hiag; Hostoffer, Robert
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions (SCAR) to drugs which are associated with significant morbidity and mortality. High risk drugs in Asia are similar to those reported worldwide. Human leukocyte antigen (HLA)-related risk alleles for carbamazepine and allopurinol SCAR are unique to Asians. Although prognostic scoring systems like the SCORTEN have been used for more than a decade, pitfalls and caveats need to be recognized, in particular in patients with multiple medical co-morbidities and systemic features in SJS/TEN. In centres without a tertiary Burns Centre, SJS/TEN patients can still be managed successfully in general and dermatology wards with well-executed supportive/nursing care. Controversy remains regarding the effectiveness of immunomodulation in reducing SJS/TEN morbidity, mortality and hastening re-epithelialization. Despite paucity of robust evidence, intravenous immunoglobulins and ciclosporin remain the most commonly used modalities worldwide. Acute and long-term ocular effects are an important source of morbidity for which emerging ophthalmic therapies appear promising. Quality of life issues have now become an important outcome in patients with SJS/TEN as they often impact survivors' future attitudes towards pharmacotherapy. Even though pharmacogenetic testing for high-risk drugs appears to be the panacea for preventing carbamazepine- and allopurinol-induced SJS/TEN in ethnic Asians, many issues remain before health regulators in our region can conclusively determine whether testing should be made mandatory or highly recommended as standard of care. PMID:24260726
Thong, Bernard Yu-Hor
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions (SCAR) to drugs which are associated with significant morbidity and mortality. High risk drugs in Asia are similar to those reported worldwide. Human leukocyte antigen (HLA)-related risk alleles for carbamazepine and allopurinol SCAR are unique to Asians. Although prognostic scoring systems like the SCORTEN have been used for more than a decade, pitfalls and caveats need to be recognized, in particular in patients with multiple medical co-morbidities and systemic features in SJS/TEN. In centres without a tertiary Burns Centre, SJS/TEN patients can still be managed successfully in general and dermatology wards with well-executed supportive/nursing care. Controversy remains regarding the effectiveness of immunomodulation in reducing SJS/TEN morbidity, mortality and hastening re-epithelialization. Despite paucity of robust evidence, intravenous immunoglobulins and ciclosporin remain the most commonly used modalities worldwide. Acute and long-term ocular effects are an important source of morbidity for which emerging ophthalmic therapies appear promising. Quality of life issues have now become an important outcome in patients with SJS/TEN as they often impact survivors' future attitudes towards pharmacotherapy. Even though pharmacogenetic testing for high-risk drugs appears to be the panacea for preventing carbamazepine- and allopurinol-induced SJS/TEN in ethnic Asians, many issues remain before health regulators in our region can conclusively determine whether testing should be made mandatory or highly recommended as standard of care.
Objective Stevens-Johnson Syndrome (SJS) is one of the most severe muco-cutaneous diseases and its occurrence is often attributed to drug use. The aim of the present study is to quantify the risk of SJS in association with drug and vaccine use in children. Methods A multicenter surveillance of children hospitalized through the emergency departments for acute conditions of interest is currently ongoing in Italy. Cases with a diagnosis of SJS were retrieved from all admissions. Parents were interviewed on child’s use of drugs and vaccines preceding the onset of symptoms that led to the hospitalization. We compared the use of drugs and vaccines in cases with the corresponding use in a control group of children hospitalized for acute neurological conditions. Results Twenty-nine children with a diagnosis of SJS and 1,362 with neurological disorders were hospitalized between 1st November 1999 and 31st October 2012. Cases were more frequently exposed to drugs (79% vs 58% in the control group; adjusted OR 2.4; 95% CI 1.0–6.1). Anticonvulsants presented the highest adjusted OR: 26.8 (95% CI 8.4–86.0). Significantly elevated risks were also estimated for antibiotics use (adjusted OR 3.3; 95% CI 1.5–7.2), corticosteroids (adjusted OR 4.2; 95% CI 1.8–9.9) and paracetamol (adjusted OR 3.2; 95% CI 1.5–6.9). No increased risk was estimated for vaccines (adjusted OR: 0.9; 95% CI 0.3–2.8). Discussion Our study provides additional evidence on the etiologic role of drugs and vaccines in the occurrence of SJS in children.
Raucci, Umberto; Rossi, Rossella; Da Cas, Roberto; Rafaniello, Concita; Mores, Nadia; Bersani, Giulia; Reale, Antonino; Pirozzi, Nicola; Menniti-Ippolito, Francesca; Traversa, Giuseppe; in Drug and Children, Italian Multicenter Study Group for Vaccine Safety
Stevens–Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are closely related severe, acute mucocutaneous reactions usually caused by drugs. They are acute life-threatening conditions and cause widespread necrosis of the epithelium. There is persistence of a high risk of SJS or TEN in relation to human immunodeficiency virus (HIV) infection associated with exposure to nevirapine (NVP). In this article, we present nine cases of SJS and one case of TEN in HIV-seropositive individuals who developed cutaneous, oral, ocular and genital lesions while being treated with NVP.
Reddy, Ramana BV; Shekar, P Chandra; Chandra, K Lalith Prakash; Aravind, RS
Stevens-Johnson syndrome and its more severe variant, toxic epidermal necrolysis, have relatively low overall incidence; however, this disease presents with high morbidity and mortality. The majority of patients develop ocular inflammation and ulceration at the acute stage. Due to the hidden nature of these ocular lesions and the concentration of effort toward life-threatening issues, current acute management has not devised a strategy to preclude blinding cicatricial complications. This review summarizes recent literature data, showing how sight-threatening corneal complications can progressively develop from cicatricial pathologies of lid margin, tarsus, and fornix at the chronic stage. It illustrates how such pathologies can be prevented with the early intervention of cryopreserved amniotic membrane transplantation to suppress inflammation and promote epithelial healing at the acute stage. Significant dry eye problems and photophobia can also be avoided with this intervention. This new therapeutic strategy can avert the catastrophic ophthalmic sequelae of this rare but devastating disease.
Shay, Elizabeth; Kheirkhah, Ahmad; Liang, Lingyi; Sheha, Hossam; Gregory, Darren G.; Tseng, Scheffer C.G.
Objective: Cutaneous drug reactions are the most common type of adverse drug reactions. Adverse cutaneous drug reactions form 2-3% of the hospitalized patients. 2% of these are potentially serious. This study aims to detect the drugs commonly implicated in Steven Johnson Syndrome-Toxic Epidermal Necrosis (SJS-TEN). Materials and Methods: A retrospective analysis was done in all patients admitted in the last five years in SDM hospital with the diagnosis of SJS-TEN. Results: A total of 22 patients with SJS-TEN were studied. In 11 patients anti-epileptics was the causal drug and in 7, anti-microbials was the causal drug. Recovery was much faster in case of anti epileptics induced SJS-TEN as compared to that induced by ofloxacin. Conclusion: SJS-TEN induced by ofloxacin has a higher morbidity and mortality compared to anti convulsants.
Naveen, Kikkeri Narayanasetty; Pai, Varadraj V.; Rai, Vijetha; Athanikar, Sharatchandra B.
A 46 year old male patient with metastatic prostate cancer developed Stevens-Johnson syndrome (SJS), initially in three well-demarcated areas on his scalp, chest and back, corresponding to ports of radiation therapy while on phenytoin. The rash spread from these locations and became more generalized and associated with pain and sloughing in the mucous lining of the mouth. There is a documented association between phenytoin administration with concurrent cranial radiation therapy and development of SJS. Erythema multiforme (EM) associated with phenytoin and cranial radiation therapy (EMPACT) is the term that describes this reaction. However, this term may not cover the full spectrum of the disease since it describes EM associated with phenytoin and only cranial radiation therapy. This case report presents evidence that SJS may be induced by radiation to other parts of the body in addition to the cranium while phenytoin is administered concomitantly. With increasing evidence that phenytoin and levetiracetam are equally efficacious for seizure treatment and prophylaxis, and since there is no link identified so far of an association between levetiracetam and SJS, we believe that levetiracetam is a better option for patients who need anticonvulsant medication(s) while undergoing radiation therapy, especially cranial irradiation.
This study presents an extremely rare case of constrictive bronchiolitis obliterans (BO) associated with Stevens-Johnson Syndrome (SJS) provides the morphological and immunohistochemical features using histopathological bronchial reconstruction technique. A 27-year-old female developed progressive dyspnea after SJS induced by taking amoxicillin at the age of 10. Finally, she died of exacerbation of type II respiratory failure after 17 years from clinically diagnosed as having BO. Macroscopic bronchial reconstruction of the whole lungs at autopsy showed the beginning of bronchial obliterations was in the 4th to 5th branches, numbering from each segmental bronchus. Once they were obliterated, the distal and proximal bronchi were dilated. Microscopic bronchial reconstruction demonstrated the localization of obliteration was mainly from small bronchi to membranous bronchioli with intermittent airway luminal narrowing or obliteration. Moreover, CD3-, CD20-, and CD68-positive cells were found in the BO lesions. CD34- and D2-40-positive cells were mainly distributed in the peribronchiolar lesions and bronchiolar lumens, respectively. SMA- and TGF-?-positive cells were seen in the fibrous tissue of BO lesions. The virtual slides The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1071703140102601.
Introduction Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening drug reactions with a higher incidence in HIV-infected persons. SJS/TEN are associated with skin and mucosal failure, predisposing to systemic bacterial infection (BSI), a major cause of death. There are limited data on risk factors associated with BSI and and mortality in HIV-infected people with SJS/TEN. Methods We conducted a retrospective study of patients admitted to a university hospital with SJS/TEN over a 3 year period. We evaluated their underlying illnesses, eliciting drugs, predictive value of bacterial skin cultures and other factors associated with mortality and BSI in a predominantly HIV-infected population by comparing characteristics of the patients who demised and those who survived. Results We admitted 86 cases during the study period and 67/86(78%) were HIV-infected. Tuberculosis was the commonest co-morbidity, diagnosed in 12/86(14%) cases. Skin cultures correlated with BSI by the same organism in 7/64(11%) cases and 6/7 were Gram-negative. Two of the 8 cases of Gram-negative BSI had an associated Gram-negative skin culture, although not always the same organism. All 8 fatalities had >30% epidermal detachment, 7 were HIV-infected, 6 died of BSI and 6 had tuberculosis. Conclusions Having >30% epidermal detachment in SJS/TEN carries an increased risk of BSI and mortality. Tuberculosis and BSI are associated with higher risk of death in SJS/TEN. Our data suggests there may be an association between Gram-negative BSI and Gram-negative skin infection.
Knight, Lauren; Muloiwa, Rudzani; Dlamini, Sipho; Lehloenya, Rannakoe J.
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe drug hypersensitivities with high mortality. Typical over-the-counter drugs of cold medicines are suggested to be causative. As multiple ingredients are generally contained in cold medicines, it is of particular interest to investigate which ingredients are responsible for SJS/TEN. However, experimental examination of causal relationships between SJS/TEN and a particular drug molecule is not straightforward. Significant association between HLA-A*02:06 and SJS/TEN with severe ocular surface complications has been observed in the Japanese. In the present study, we have undertaken in silico docking simulations between various ingredients contained in cold medicines available in Japan and the HLA-A*02:06 molecule. We use the composite risk index (CRI) that is the absolute value of the binding affinity multiplied by the daily dose to assess the potential risk of the adverse reactions. The drugs which have been recognized as causative drugs of SJS/TEN in Japan have revealed relatively high CRI, and the association between SJS/TEN and HLA-A*02:06 has been qualitatively verified. The results have also shown that some drugs whose links to SJS/TEN have not been clinically recognized in Japan show the high CRI and suggested that attention should be paid to their adverse drug reactions.
Isogai, Hideto; Miyadera, Hiroko; Ueta, Mayumi; Sotozono, Chie; Kinoshita, Shigeru; Tokunaga, Katsushi
A 42-year-old man from Ghana presented with bilateral painful corneal perforations following ingestion of a sulphur-based antibiotic. Emergency bilateral penetrating keratoplasty was performed, with restoration of globe integrity. However, surgical complications arose such as non-healing epithelial defect, secondary infection, graft dehiscence, and mounting intraocular pressure. This case illustrates the challenges faced in managing corneal grafts in patients with already compromised ocular surfaces.
Md Noh, Umi Kalthum; Then, Kong Yong
Purpose. Ocular complications related to Stevens-Johnson Syndrome (SJS)–Toxic Epidermal Necrolysis (TEN) may persist and progress after resolution of systemic disease. This is thought to be related in part to persistent ocular innate-immune signaling. In this study, our aim was to characterize infiltrative conjunctival cellular profiles during acute (<12 months) and chronic (>12 months) disease. Methods. Consecutive patients presenting with SJS-TEN over a 12-month period were followed for 1 year. Detailed clinical examination and conjunctival impression cell recovery was analyzed by flow cytometry for the presence of intraepithelial leukocytes and compared with healthy controls (n = 21). Results. Ten patients were recruited of whom six had acute disease and five were classified as TEN (SCORTEN = 1, n = 4). Conjunctival inflammation was graded as absent/mild in a total of nine patients; but despite this, evidence of fornix shrinkage was observed in nine subjects. This inversely correlated with disease duration (P < 0.05). A reduction in percentage of CD8??+ T cells compared with controls (80% vs. 57%; P < 0.01) was associated with a corresponding increase in the number/percentage of CD45INTCD11b+CD16+CD14? neutrophils (186 vs. 3.4, P < 0.01, 31% vs. 0.8%, P < 0.001). Neutrophils inversely correlated with disease duration (r = ?0.71, P = 0.03), yet there was no absolute change in the CD8??+ or neutrophil populations during the study period (P = 1.0). Conclusions. These data highlight that a neutrophilic infiltrate is present in mildly inflamed or clinically quiescent conjunctival mucosa in patients with ocular SJS-TEN, where neutrophil numbers inversely correlate with disease duration. Neutrophil persistence endorses the hypothesis of an unresolved innate-inflammatory process that might account for disease progression.
Williams, Geraint P.; Tomlins, Paul J.; Denniston, Alastair K.; Southworth, H. Susan; Sreekantham, Sreekanth; Curnow, S. John; Rauz, Saaeha
Background. Stevens-Johnson syndrome (SJS) and/or toxic epidermal necrolysis (TEN) are uncommon and life-threatening drug reaction associated with a high morbidity and mortality. Objective. We studied SJS and/or TEN by conducting a retrospective analysis of 87 patients treated during a 10-year period. Methods. We conducted a retrospective review of the records of all patients with a diagnosis of SJS and/or TEN based on clinical features and histological confirmation of SJS and/or TEN was not available at the Department of Medicine, Vajira hospital, Bangkok, Thailand. The data were collected from two groups from 2003 to 2007 and 2008 to 2012. Results. A total of 87 cases of SJS and/or TEN were found, comprising 44 males and 43 females whose mean age was 46.5 years. The average length of stay was 17 days. Antibiotics, anticonvulsants, and allopurinol were the major culprit drugs in both groups. The mean SCORTEN on admission was 2.1 in first the group while 1.7 in second the group. From 2008 to 2012, thirty-nine patients (76.5%) were treated with corticosteroids while only eight patients (22.2%) were treated between 2003 and 2007. The mortality rate declined from 25% from the first group to 13.7% in the second group. Complications between first and second groups had no significant differences. Conclusions. Short-term corticosteroids may contribute to a reduced mortality rate in SJS and/or TEN without increasing secondary infection. Further well-designed studies are required to compare the effect of corticosteroids treatment for SJS and/or TEN.
Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are severe, cutaneous adverse drug reactions that are rare but life threatening. Genetic biomarkers for allopurinol-related SJS/TEN in Japanese were examined in a genome-wide association study in which Japanese patients (n=14) were compared with ethnically matched healthy controls (n=991). Associations between 890?321 single nucleotide polymorphisms and allopurinol-related SJS/TEN were analyzed by the Fisher's exact test (dominant genotype mode). A total of 21 polymorphisms on chromosome 6 were significantly associated with allopurinol-related SJS/TEN. The strongest association was found at rs2734583 in BAT1, rs3094011 in HCP5 and GA005234 in MICC (P=2.44 × 10(-8); odds ratio=66.8; 95% confidence interval, 19.8-225.0). rs9263726 in PSORS1C1, also significantly associated with allopurinol-related SJS/TEN, is in absolute linkage disequilibrium with human leukocyte antigen-B*5801, which is in strong association with allopurinol-induced SJS/TEN. The ease of typing rs9263726 makes it a useful biomarker for allopurinol-related SJS/TEN in Japanese. PMID:21912425
Tohkin, M; Kaniwa, N; Saito, Y; Sugiyama, E; Kurose, K; Nishikawa, J; Hasegawa, R; Aihara, M; Matsunaga, K; Abe, M; Furuya, H; Takahashi, Y; Ikeda, H; Muramatsu, M; Ueta, M; Sotozono, C; Kinoshita, S; Ikezawa, Z
Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are considered to be drug-induced diseases, and are characterized by extensive mucocutaneous disorder and epidermal necrosis which result in the detachment of the epidermis. Inactive and active forms of metalloproteinases (MMP2 and MMP9) secreted by skin explants maintained in organ culture for 72 h and in blister fluid from two TEN and three SJS patients were investigated. Interestingly, lesional skin from both the TEN and the SJS patients cultured for 3 days in conditioned medium showed high levels of both 72 kDa progelatinase A and 66 kDa activated gelatinase A, and the 66 kDa activated form was not observed in cultures of skin from control individuals. Furthermore, indirect immunodetection showed the presence of MMP2 and MMP9 in TEN and SJS patients' skin. Increased gelatinase activity in the culture medium of TEN and SJS skin maintained in organ culture and in blister fluid indicates that these gelatinases may be responsible for the detachment of the epidermis in these drug-induced necrolyses. PMID:15449075
Gaultier, F; Ejeil, A-L; Igondjo-Tchen, S; Dohan, D; Dridi, S M; Maman, L; Wierzba, C B; Stania, D; Pellat, B; Lafont, A; Godeau, G; Gogly, B
Epidermal necrolysis, whether involving limited, that is, Stevens–Johnson syndrome (SJS), or large, that is, toxic epidermal necrolysis (TEN), areas of the skin surface, is associated with high mortality. A specific prognosis score (SCORTEN) accurately predicts mortality. Management of SJS and TEN mainly relies on supportive care but several treatments have been proposed, particularly corticosteroids and intravenous immunoglobulins (IVIG), to block the progression of the disease. The aim of this study was a pooled analysis of published series addressing the question of treatment efficacy by comparing the mortality observed to the mortality predicted by the SCORTEN score. A literature search was carried out through PubMed from January 2001 to December 2009. It found 47 original series including at least 10 patients. Among these, 13 containing a clear description of treatment(s) and an evaluation of SCORTEN score were analyzed allowing the calculation of a mortality ratio (MR) for each series and a pooled MR with 95% confidence interval (CI) for each treatment. The analyzed series comprised a total of 439 patients. Supportive care was used only in 199 patients with a pooled MR of 0.89 (CI 0.67–1.16, p?=?0.43), corticosteroids were administered to 78 patients with a pooled MR of 0.92 (CI 0.53–1.48, p?=?0.84), and IVIG in 162 with a pooled MR of 0.82 (CI 0.58–1.12, p?=?0.23). In conclusion, even though this analysis had some limitations, it strongly suggested that neither corticosteroids nor IVIG provide any important reduction in the risk of dying from SJS and TEN.
Stevens-Johnson syndrome (SJS) and its severe variant, toxic epidermal necrolysis (TEN), are acute inflammatory vesiculobullous reactions of the skin and mucous membranes. Cold medicines including non-steroidal anti-inflammatory drugs (NSAIDs) and multi-ingredient cold medications are reported to be important inciting drugs. We used two sample sets of Japanese patients to investigate the association between HLA genotypes and cold medicine-related SJS/TEN (CM-SJS/TEN), including acetaminophen-related SJS/TEN (AR-SJS/TEN) with severe mucosal involvement such as severe ocular surface complications (SOC). HLA-A*02:06 was strongly associated with CM-SJS/TEN with SOC and AR-SJS/TEN with SOC. HLA-B*44:03 was also detected as an independent risk allele for CM-, including AR-SJS/TEN with SOC. Analyses using data obtained from CM-SJS/TEN patients without SOC and patients with CM-unrelated SJS/TEN with SOC suggested that these two susceptibility alleles are involved in the development of only CM-SJS/TEN with SOC patients. PMID:24781922
Ueta, Mayumi; Kaniwa, Nahoko; Sotozono, Chie; Tokunaga, Katsushi; Saito, Yoshiro; Sawai, Hiromi; Miyadera, Hiroko; Sugiyama, Emiko; Maekawa, Keiko; Nakamura, Ryosuke; Nagato, Masaki; Aihara, Michiko; Matsunaga, Kayoko; Takahashi, Yukitoshi; Furuya, Hirokazu; Muramatsu, Masaaki; Ikezawa, Zenrou; Kinoshita, Shigeru
Purpose This retrospective observational case series of fifty-one consecutive patients referred to the eye clinic with acute-stage Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN) from 1995 to 2011 examines the effect of early treatment with a systemic corticosteroid or intravenous immunoglobulin (IVIG) on the ocular outcomes in patients with SJS or TEN. Methods All patients were classified by age (?18 years vs. >18 years) and analyzed by treatment modality and early intervention with systemic corticosteroids (?5 days), IVIG (?6 days), or amniotic membrane graft transplantation (AMT) (?15 days). The main outcomes were best-corrected visual acuity (BCVA) in logarithm of the minimum angle of resolution (logMAR) and ocular involvement scores (OIS, 0-12), which were calculated based on the presence of superficial punctate keratitis, epithelial defect, conjunctivalization, neovascularization, corneal opacity, keratinization, hyperemia, symblepharon, trichiasis, mucocutaneous junction involvement, meibomian gland involvement, and punctal damage. Results The mean logMAR and OIS scores at the initial visit were not significantly different in the pediatric group (logMAR = 0.44, OIS = 2.76, n = 17) or the adult group (logMAR = 0.60, OIS = 2.21, n = 34). At the final follow-up, the logMAR and OIS had improved significantly in the adult group (p = 0.0002, p = 0.023, respectively), but not in the pediatric group. Early intervention with IVIG or corticosteroids significantly improved the mean BCVA and OIS in the adult group (p = 0.043 and p = 0.024, respectively for IVIG; p = 0.002 and p = 0.034, respectively for corticosteroid). AMT was found to be associated with a significantly improved BCVA or OIS in the late treatment group or the group with a better initial OIS (p = 0.043 and p = 0.043, respectively for BCVA; p = 0.042 and p = 0.041, respectively for OIS). Conclusions Our findings suggest that patients with SJS or TEN who are aged 18 years or less have poorer ocular outcomes than older patients and that early treatment with steroid or immunoglobulin therapy improves ocular outcomes.
Kim, Kyeong Hwan; Park, Sung Wook; Wee, Won Ryang
Important role of prodromal viral infections responsible for inhibition of xenobiotic metabolizing enzymes in the pathomechanism of idiopathic Reye's syndrome, Stevens-Johnson syndrome, autoimmune hepatitis, and hepatotoxicity of the therapeutic doses of acetaminophen used in genetically predisposed persons.
Upper respiratory tract febrile illnesses caused by various viruses, mycoplasma, chlamydia infections, and/or inflammatory diseases are usually observed a few days to a few (several) weeks before the onset of Reye's syndrome, Stevens-Johnson syndrome, autoimmune hepatitis (hepatotropic virus infections), or hepatotoxicity associated with therapeutic administration of acetaminophen in persons with varying degrees of deficits of important enzymatic activity. Activation of systemic host defense mechanisms by inflammatory component(s) results in depression of various induced and constitutive isoforms of cytochrome P-450 mixed-function oxidase system superfamily enzymes in the liver and most other tissues of the body. Because several cytochrome P-450 enzymes activities important for biotransformation of many endogenous and egzogenous substances show considerable variability between individuals, in some genetically predisposed persons, even the administration of therapeutic doses of a drug may result in serious clinical mishaps, if an important concomitant risk factor (eg, acute viral infection) is involved. Several inflammatory cytokines, such as interleukins, transforming growth factor beta1, human hepatocyte growth factor, and lymphotoxin, downregulate gene expression of major cytochrome P-450 enzymes with the specific effects on mRNA levels, protein expression, and enzyme activity observed with a given cytokine varying for each P-450 studied, thus eventually leading to metabolite-mediated adverse drug reactions and immunometallic diseases which sometimes result in tissue injury beyond the site(s) where metabolic bioactivation takes place. On the other hand, it must be emphasized that inhibition of metabolism of several drugs, as well as influence on the concentration and/or ratio of various cytokines in inflamed tissues, may exert beneficial effects in patients with different diseases, thus opening new therapeutic possibilities. Clinically relevant interactions may be exemplified by the effects of some fluoroquinolone antibiotics, such as pefloxacin and ciprofloxacin, which probably have a steroid-sparing effect in some patients with frequently relapsing nephrotic syndrome, and an increased bioavailability of several drugs following concomitant intake with freshly pressed grapefruit juice, eventually caused by inhibition of their metabolism, mediated mainly by CYP3A and specifically inhibited by naturally occurring flavonoids. PMID:11897929
... 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 ...
A 25-year-old Caucasian heterosexual man with a recent history of unprotected sex in Vietnam while on a holiday was prescribed HIV postexposure prophylaxis by a local doctor; nevirapine, stavudine and lamivudine. He was subsequently admitted to a UK hospital with sore throat, bilateral conjunctivitis, genital ulceration and severe widespread maculo-papular rash. Extensive investigations for infective causes were negative and he was subsequently recovered with conservative therapy. PMID:23625676
Bannaga, Ayman; Rahama, Omar; Barlow, Gavin
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
A case report of 6-year-old boy with progeria syndrome, with marked cardiac complications is presented. The boy had cardiorespiratory failure. Discoloured purpuric skin patches, alopecia, prominent forehead, protuberant eyes, flattened nasal cartilage, malformed mandible, hypodentition, and deformed rigid fingers and toes were observed on examination. The boy was unable to speak. A sclerotic systolic murmur was audible over the mitral and aortic areas. Chest x-rays showed cardiac enlargement and the electrocardiogram (ECG) showed giant peaked P waves (right atrial hypertrophy) and right ventricular hypertrophy. Atherosclerotic dilated ascending aorta, thickened sclerotic aortic, mitral, and tricuspid valves with increased echo texture, left and right atrial and right ventricular dilatation, reduced left ventricular cavity, and thickened speckled atrial and ventricular septa were observed on echocardiography. PMID:24601202
Ilyas, Saadia; Ilyas, Hajira; Hameed, Abdul; Ilyas, Muhammad
Thromboembolic complications are among the most important extrarenal consequences of nephrotic syndrome (NS). In addition to deep vein thrombosis in the legs and pulmonary embolism, NS is very frequently accompanied by renal vein thrombosis. Due to enhanced procoagulatory and antifibrinolytic potential and reduced anticoagulatory potential, multifactor disruption of hemostatic equilibrium leads to hypercoagulability in NS patients, which is aggravated by an increase in blood viscosity and endothelial dysfunction. Circulating antibodies against ?-enolase, a plasmin(ogen)-binding protein, and the possibility of certain molecules being renally eliminated in specific manner are discussed as reasons for the particular frequency of thromboembolic complications in patients with idiopathic membranous nephropathy. Serum albumin concentration is an indicator for the risk of thrombosis in NS patients. When applying the current KDIGO (Kidney Disease: Improving Global Outcomes) clinical practice guideline for glomerulonephritis to NS patients with a serum albumin concentration of less than 25 g/l and at least one additional thrombogenic risk factor, primary prophylactic anticoagulation ("conditioned prophylaxis") with an orally administered vitamin K antagonist (target INR 2-3) is recommended as long as the serum albumin concentration is less than 30 g/l. PMID:23677507
The neurological complications of Behçet's syndrome have not been characterized with clarity. We present the clinical features, imaging characteristics and CSF findings of a series of 50 patients seen at the National Hospital for Neurology and Neurosurgery over the past 10 years. In this series, vascular complications had a low prevalence, whereas involvement of the brainstem was common; spinal cord lesions, hemisphere lesions and meningoencephalitis also occurred. Optic neuropathy, vestibulocochlear and peripheral nerve involvement occurred, but were rare. The prognosis for recovery was in general good, and the majority of those followed-up over a median of 3 years (range 1-19 years) had only single attacks. One-third of patients underwent further attacks, and four underwent progressive deterioration leading to disability. Factors suggesting a poor prognosis are repeated attacks, incomplete recovery, progressive disease course and a high level of CSF leucocytosis during acute attack. These data should be of help in the further definition of the clinical characteristics of this rare neurological disorder and in the planning of treatment trials. PMID:10545402
Kidd, D; Steuer, A; Denman, A M; Rudge, P
Summary A case of bilateral compartment syndrome after prolonged Lloyd-Davies lithotomy position is described. The diagnosis was made early, despite effective extradural bupivacaine- fentanyl analgesia. The aetiology, diagnosis, pathology and treatment of compartment syn- drome are described. Complications of the syndrome may be life-threatening and perma- nently disabling. The anaesthetist should be aware of the potential complications of the operative
Numerous oral manifestations associated with salivary gland dysfunction, and particularly Sjögren's syndrome, have been reported in the literature. This article discusses the evidence on a wide range of oral manifestations associated with Sjögren's syndrome. PMID:24287193
Napeñas, Joel J; Rouleau, Tanya S
Chemical Injuries; Unspecified Complication of Corneal Transplant; Autoimmune Diseases; Ocular Cicatricial Pemphigoid; Stevens Johnson Syndrome; Lupus Erythematosus, Systemic; Rheumatoid Arthritis; Other Autoimmune Diseases
Lemierre's syndrome, also known as postanginal sepsis, is a rare condition that presents as an increasing sore throat due to acute pharyngitis or tonsillitis and progresses to sepsis, due to suppurative thrombophlebitis of the internal jugular vein. We present an atypical case of Lemierre's syndrome complicated by carotid thrombosis. The etiological factors and the diagnostic and therapeutic measures are discussed. PMID:11917980
Maalikjy Akkawi, N; Borroni, B; Magoni, M; Vignolo, L A; Padovani, A
In addition to pure PD and pure dystonic syndromes, there are a group of disorders with overlapping features. The differential diagnosis of these dystonia parkinsonism syndromes can be complex. In view of the growing list of recognized disorders and recent advances in genetics, we review the autosomal recessive forms of dystonia parkinsonism, summarizing clinical presentations, results of investigations, and response to treatment of gene-proven cases. We concentrate on PANK2-, PLA2G6-, ATP13A2-, FBX07, TAF1-, and PRKRA-associated neurodegeneration. Parkin, PINK1, and DJ-1 are also briefly reviewed. PMID:19185014
Schneider, Susanne A; Bhatia, Kailash P; Hardy, John
This article reviews serious clinical complications related to assisted reproductive technology (ART) procedures (ovarian stimulation and oocyte aspiration), including ovarian hyperstimulation syndrome (OHSS), bleeding and infection. These complications are rare, but can be severe and even life-threatening. It is important that general practitioners and gynaecologists are aware of these complications, because they will often be the first to be contacted by patients. Similarly, patients should be counselled before starting ART procedures that iatrogenic complications can be associated with ovarian stimulation and/or oocyte aspiration. PMID:19632900
Vloeberghs, Veerle; Peeraer, Karen; Pexsters, Anne; D'Hooghe, Thomas
A 68-year-old man underwent a left upper lobectomy for squamous cell lung carcinoma. After 15 days, he was readmitted with fever, dyspnea, and a skin rash. Computed tomography showed a pleural effusion, which was drained. He was diagnosed with hemophagocytic syndrome in view of the fever, skin rash, bicytopenia, hypertriglyceridemia, high lactate dehydrogenase, and raised ferritin levels. His clinical condition deteriorated in spite of steroid therapy, and he died on the 23rd postoperative day. PMID:22718729
Jiwnani, Sabita; Karimundackal, George; Kulkarni, Atul; Pramesh, C S
. Lemierre's syndrome, also known as postanginal sepsis, is a rare condition that presents as an increasing sore throat due\\u000a to acute pharyngitis or tonsillitis and progresses to sepsis, due to suppurative thrombophlebitis of the internal jugular\\u000a vein. We present an atypical case of Lemierre's syndrome complicated by carotid thrombosis. The etiological factors and the\\u000a diagnostic and therapeutic measures are
N. Maalikjy Akkawi; B. Borroni; M. Magoni; L. A. Vignolo; A. Padovani
Neuroleptic malignant syndrome (NMS) is a rare but well described complication of the administration of antipsychotic agents. Compartment syndrome, with increased pressures within the confined space of fascial sheaths leading to compression damage of the contained tissue, similarly is well described. Brachial plexus injuries caused by patient malposition are also very rare but a few cases have been reported. We report a case where these three complications occurred together. This was attributable to the patient developing NMS whilst asleep in the prone position overnight.
Ahmad, A.; Harrison, C. A.; Davies, H. G.
Neuroleptic malignant syndrome is an uncommon but potentially fatal side effect of antipsychotic drug treatment. Several serious complications have been associated with neuroleptic malignant syndrome, such as acute renal failure, deep venous thrombosis, pulmonary embolism and aspiration pneumonia. Reports on infections other than aspiration pneumonia appear, from the literature, to be uncommon. Four cases of infection (three cases of upper respiratory tract infection and one case of urinary tract infection) which developed during the course of neuroleptic malignant syndrome are reported and pathophysiological mechanisms underlying their presentation are suggested. PMID:18609425
Bilanakis, Nikolaos; Peritogiannis, Vaios; Kalampokis, Georgios
Antiphospholipid syndrome (APS) is an autoimmune condition involving arterial and venous thrombosis. An unusual APS variant, catastrophic antiphospholipid syndrome (CAPS), includes rapid multi-organ failure from widespread small vessel thrombosis. Central nervous system complications arise in one-third of CAPS patients. In rare cases, CAPS co-manifests with cerebellar hemorrhage presenting a neurosurgical emergency. We present a 65-year-old woman with CAPS-related cerebellar hematoma, co-morbid idiopathic thrombocytopenic purpura, deep vein thrombosis and altered mental status, with treatment complicated by thrombocytopenia. The patient suddenly deteriorated, secondary to a cerebellar subdural hematoma, and underwent decompression and excision of the hematoma. After recovery in the intensive care unit, she developed a new spontaneous epidural hematoma requiring additional surgery. Management of these patients is hematologically complex and often requires a multi-disciplinary team of physicians. This patient provides an important learning point for clinicians - consider CAPS when hemorrhage and thrombosis are present. PMID:24269552
Drazin, Doniel; Westley Phillips, H; Shirzadi, Ali; Drazin, Noam; Schievink, Wouter
... page: About CDC.gov . Middle East Respiratory Syndrome (MERS) Facebook Reccomend Twitter Tweet Share Compartir Add this ... Bookmarks Symptoms & Complications Most people confirmed to have MERS-CoV infection have had severe acute respiratory illness ...
Walker-Warburg syndrome is a very rare autosomal recessive disorder with congenital muscular dystrophy, brain malformations on the basis of a neuronal migration defect and ocular abnormalities. We report our experience in treating two cases of Walker-Warburg syndrome complicated by hydrocephalus with shunting and endoscopic techniques. PMID:20516736
Preuss, M; Heckmann, M; Stein, M; Nestler, U
Grisel's syndrome is a nontraumatic atlantoaxial subluxation which is usually secondary of an infection or an inflammation at the head and neck region. It can be observed after surgery of head and neck region. Etiopathogenesis has not been clearly described yet, but increased looseness of paraspinal ligament is thought to be responsible. Patients typically present with painful torticollis. Diagnosis of Grisel's syndrome is largely based on suspicion of the patient who has recently underwent surgery or history of infection in head and neck region. Physical examination and imaging techniques assist in diagnosis. Therefore, clinicians should be aware of acute nontraumatic torticollis after recently applied the head and neck surgery or undergone upper respiratory tract infection. In this paper, a case of an eight-year-old male patient who had Grisel's syndrome after adenotonsillectomy is discussed with review of the literature.
Bucak, Abdulkadir; Ulu, Sahin; Aycicek, Abdullah; Kacar, Emre; Miman, Murat Cem
\\u000a \\u000a Zusammenfassung\\u000a Wir berichten über eine Patientin mit kongenitalem Sick-Sinus-Syndrom, das 23 Jahre nach Erstdiagnose durch Vorhofflimmern\\u000a und einen embolischen Schlaganfall bei der inzwischen 34jährigen kompliziert wurde. Eine Therapie mit Zweikammer-Schrittmacher\\u000a und oraler Antikoagulation wurde initiiert, die weitere Nachsorge war unauffällig, jedoch dokumentierte der Schrittmacher-Speicher\\u000a weiterhin asymptomatische Rezidive von paroxysmalem Vorhofflimmern.
R. Lehmann; G. Groenefeld; C. W. Israel
Isolated renal vein thrombosis is a rare entity. We present a patient whose complaint of flank pain led to the diagnosis of a renal vein thrombosis. In this case, abdominal computed tomography angiography was helpful in diagnosing the nutcracker syndrome complicated by the renal vein thrombosis. Anticoagulation was started and three weeks later, CTA showed complete disappearance of the renal vein thrombosis. To treat the Nutcracker syndrome, we proposed left renal vein transposition that the patient consented to. PMID:24349817
Mallat, Faouzi; Hmida, Wissem; Jaidane, Mehdi; Mama, Nadia; Mosbah, Faouzi
Isolated renal vein thrombosis is a rare entity. We present a patient whose complaint of flank pain led to the diagnosis of a renal vein thrombosis. In this case, abdominal computed tomography angiography was helpful in diagnosing the nutcracker syndrome complicated by the renal vein thrombosis. Anticoagulation was started and three weeks later, CTA showed complete disappearance of the renal vein thrombosis. To treat the Nutcracker syndrome, we proposed left renal vein transposition that the patient consented to.
Mallat, Faouzi; Hmida, Wissem; Jaidane, Mehdi; Mama, Nadia; Mosbah, Faouzi
We describe a case of pulmonary bleeding and subsequent acute respiratory distress syndrome (ARDS) in a 20-month-old female suffering from a typical postdiarrheal hemolytic-uremic syndrome (HUS). Acute renal failure was treated early by peritoneal dialysis. It is of interest to underline that thrombocytopenia or any coagulative impairment was absent when this complication occurred, and spontaneous diuresis recovery was ongoing. All
M. Piastra; A. Ruggiero; A. Langer; E. Caresta; A. Chiaretti; S. Pulitanò; G. Polidori; R. Riccardi
Guillain-Barre syndrome (GBS) is a heterogenous group of peripheral-nerve disorders with similar clinical presentation characterized by acute, self-limited, progressive, bilateral and relatively symmetric ascending flaccid paralysis, which peaks in 2-4 weeks and then subsides. The usual complications, which occur in a patient of GBS are pneumonia, sepsis, pulmonary embolism, respiratory insufficiency and cardiac arrest. The clinical course of GBS complicated by acute rhabdomyolysis is extremely rare. We present the case of GBS with marked elevation in serum creatine kinase, serum myoglobin levels and persistent hyperkalemia as a result of associated acute rhabdomyolysis. PMID:24872655
Saxena, Amrish; Singh, Vineeta; Verma, Nitin
Guillain-Barre syndrome (GBS) is a heterogenous group of peripheral-nerve disorders with similar clinical presentation characterized by acute, self-limited, progressive, bilateral and relatively symmetric ascending flaccid paralysis, which peaks in 2-4 weeks and then subsides. The usual complications, which occur in a patient of GBS are pneumonia, sepsis, pulmonary embolism, respiratory insufficiency and cardiac arrest. The clinical course of GBS complicated by acute rhabdomyolysis is extremely rare. We present the case of GBS with marked elevation in serum creatine kinase, serum myoglobin levels and persistent hyperkalemia as a result of associated acute rhabdomyolysis.
Saxena, Amrish; Singh, Vineeta; Verma, Nitin
Bleeding is the most important complication of treatment with intravenous tissue plasminogen activator for acute ischemic stroke. Neurologists are familiar with intracranial hemorrhage, the most feared site for bleeding following thrombolysis, but extracranial bleeding can also occur resulting in substantial morbidity and mortality. We describe an 88-year-old woman with an acute stroke who developed bleeding into the left arm complicated by hemodynamic instability and compartment syndrome following intravenous thrombolysis. The patient was treated conservatively in view of the risks associated with fasciotomy and her other medical comorbidities. PMID:24291484
Brownlee, Wallace J; Wu, Teddy Y; Van Dijck, Stephanie A; Snow, Barry J
A 5-year-old girl sought treatment for pyrexia of unknown origin. Despite prompt surgical drainage of a streptococcal septic arthritis of the ankle joint, her condition deteriorated. Multifocal pyomyositis was subsequently diagnosed. This was complicated by acute compartment syndrome in three extremities. With aggressive surgical and medical management, the child made a complete recovery. Orthopaedic clinicians in nontropical areas must familiarize themselves with this rare, potentially life-threatening, but eminently curable disease. PMID:11360777
Harrington, P; Scott, B; Chetcuti, P
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
Compartment syndrome (CS) is a common complication of crush injury but it is rare to find bilateral gluteal compartment syndrome (BGCS). Only six cases of BGCS have been reported in the literature. This syndrome has been reported after crush injury, drug overdose, surgical positioning, and vascular surgery. Apart from CS, crush injury is associated with multi-system adverse effects and these patients are at high risk for renal failure and sepsis. CS patients may present with dehydration; coagulation disorders; elevated creatine phosphokinase and myoglobin levels; hyperkalemia and hypocalcaemia, which may cause life-threatening arrhythmias and therefore need urgent and aggressive therapy. The early goal in these patients is prevention of acute renal failure with aggressive fluid therapy, alkalinization of urine, and forced diuresis. Early treatment of hyperkalemia, antibiotic therapy, immunoprophylaxis, and wound care will minimize the risk of arrhythmias and sepsis. CS must be considered when any patient is diagnosed with crush injury syndrome. CS is defined as elevation of interstitial/ intracompartmental pressure, leading to microvascular and myoneural dysfunction and secondary hypoxia; it may cause functional loss or even death if not detected early and treated properly. The increase in pressure in one or all compartments of the gluteal region causes CS with devastating effects on muscle and neurovascular bundles. CS is traditionally diagnosed on the basis of five 'p's: pain, pallor, paraesthesia, pulselessness and paralysis. Diagnosis of gluteal CS is difficult as the peripheral pulses are preserved and the condition is usually only diagnosed when neurological abnormality is noticed. Diagnosis of CS can be made by direct measurement of the compartment pressure and magnetic resonance imaging or computerized tomography. Gluteal CS is managed by fasciotomy and debridement of necrosed tissue, with secondary closure of fascia. A high index of suspicion is necessary for the early diagnosis of gluteal CS, and this will reduce the disability and complications as a consequence of this syndrome. The acute-care physician, the intensivist, and the trauma surgeon must be aware of this rare syndrome, as it can result in multiorgan dysfunction and death. Here we report a case of bilateral gluteal CS that was successfully treated in our trauma intensive care unit. PMID:20606796
The Ehlers-Danlos syndrome is a special challenge to the surgeon because of its relative infrequency and the late onset of overt symptoms, such as hyperelastic skin. We should consider this diagnosis in every child with recurrent inguinal herniae and search for its stigmata. A child with a known Ehlers-Danlos syndrome who requires an elective operation should be carefully studied for coagulation disorders. If there is a history of past injuries or operations in which there was poor healing, the benefits of the proposed operation must be carefully weighed against possible complications. Operations must be carried out with great care to avoid tearing the skin and fascia. Meticulous attention is given to hemostasis to prevent postoperative bleeding or hematomas. Inguinal hernias in these children resemble those seen in elderly patients. The transversalis fascia is thin and the internal ring is hugely dilated. An adult-type repair, with the use of mesh or felt may result in a lower incidence of recurrence. In addition, the Ehlers-Danlos syndrome may be yet another etiology for obscure gastrointestinal bleeding in children. Specific enzyme defects have been identified in 2 of the 7 subtypes of this disease. Further, biochemical study of connective tissue defects may contribute to our knowledge of normal collagen synthesis and wound healing. PMID:302328
McEntyre, R L; Raffensperger, J G
Summary Background: Pseudo-Bartter syndrome encompasses a heterogenous group of disorders similar to Bartter syndrome. Sometimes a few status may be nested, as in our case presented here. Case Report: An 8-month-old boy was referred to our hospital with of intractable diarrhea, polyuria, persistent hypokalemia, abdominal distension and failure to thrive. He was born in the 34 6/7 gestational week (GW) to consanguineous parents. In the 30th GW polyhydramnios was verified by ultrasonography. The laboratory results showed hypokalemic-hypochloremic metabolic alkalosis, hyponatremia, and increased urinary loss of chloride, potassium and calcium. An audiogram test revealed complete sensorineural deafness. Ultrasonography revealed medullary nephrocalcinosis in both kidneys. Elevated plasma renin activity and aldosterone were found and a provisional diagnosis of type-IV neonatal Bartter syndrome was made. Treatment with indomethacin, spironolactone and additional intake of NaCl/KCl was initiated. Despite these therapies, the child’s diarrhea persisted but serum potassium concentration normalized, and hypercalciuria and urine output reduced. After determining the high fecal chloride concentration, there was an immediate decompensation of the disease on indomethacin withdrawal, thus a diagnosis of type IV neonatal Bartter syndrome complicated with congenital chloride diarrhea was considered. Indomethacin, spironolactone and supplementary therapies with NaCl/KCl were continued, which resulted in the normalization of serum electrolytes as well as his physical development, but high contents of chloride in urine and faeces and nephrocalcinosis remains unchanged during 1-year follow-up. Conclusions: Because of the clinical and laboratory simulations between the various diseases that lead to hypokalemic-hypochloremic metabolic alkalosis, patients must be evaluated carefully.
Sakall?, Hale; Bucak, Hakan Ibrahim
: New concepts regarding the diagnosis and treatment of thoracic outlet syndrome, double crush syndrome related to thoracic\\u000a outlet syndrome, and minor traumatic dystrophy related to thoracic outlet syndrome are explained.
Masataka Abe; Katsuaki Ichinohe; Jun Nishida
Background. There are challenges in the clinical diagnosis of drug-induced injury and in obtaining information on the reactivation of human herpes viruses (HHV) during idiosyncratic adverse drug reactions. Objectives. (i) To develop a unified list of drugs incriminated in drug-induced hepatotoxicity and severe cutaneous reactions, in which drug hypersensitivity leads to HHV-6 reactivation and further complication of therapy and recovery and (ii) to supplement the already available data on reporting frequencies of liver- or skin-induced cases with knowledge of individual case reports, including HHV-6 reactivation and briefly introducing chromosomally integrated HHV-6. Data Sources and Extraction. Drugs identified as causes of (i) idiosyncratic reactions, (ii) drug-induced hypersensitivity, drug-induced hepatotoxicity, acute liver failure, and Stevens-Johnson syndrome, and (iii) human herpes virus reactivation in PubMed since 1997 have been collected and discussed. Results. Data presented in this paper show that HHV-6 reactivation is associated with more severe organ involvement and a prolonged course of disease. Conclusion. This analysis of HHV-6 reactivation associated with drug-induced severe cutaneous reactions and hepatotoxicity will aid in causality assessment and clinical diagnosis of possible life-threatening events and will provide a basis for further patient characterization and therapy.
Pritchett, Joshua C.; Nanau, Radu M.; Neuman, Manuela G.
Development of autoantibodies against coagulation factor VIII (FVIII) leads to a rare condition defined as acquired hemophilia (AH). If not diagnosed and treated early, AH may be associated with high mortality and morbidity. A 65-year-old woman presented with history of macrohematuria, acute renal failure, cardiogenic shock, and acute respiratory failure. Blood investigation revealed azotemia, prolonged activated partial thromboplastin time (aPTT), coagulation FVIII level of <1%, and presence of FVIII inhibitor. Echocardiography showed global hypokinesia and ultrasonography and computed tomography (CT) revealed bilateral hydroureteronephrosis. The final diagnosis was acquired hemophilia A, complicated by acute obstructive renal failure and cardiorenal syndrome (CRS) type 3. Patient was managed with mechanical ventilation, heparin-free hemodialysis, negative fluid balance, recombinant activated factor VII, and prednisolone. Hematuria was relieved, renal function improved, and cardiac function showed improvement on repeat echocardiography. Patient was discharged on prednisolone with subsequent follow ups.
Sharma, Rakesh; Dash, Sananta Kumar; Chawla, Rajesh; Kansal, Sudha; Agrawal, Devender Kumar; Dua, Harsh
Development of autoantibodies against coagulation factor VIII (FVIII) leads to a rare condition defined as acquired hemophilia (AH). If not diagnosed and treated early, AH may be associated with high mortality and morbidity. A 65-year-old woman presented with history of macrohematuria, acute renal failure, cardiogenic shock, and acute respiratory failure. Blood investigation revealed azotemia, prolonged activated partial thromboplastin time (aPTT), coagulation FVIII level of <1%, and presence of FVIII inhibitor. Echocardiography showed global hypokinesia and ultrasonography and computed tomography (CT) revealed bilateral hydroureteronephrosis. The final diagnosis was acquired hemophilia A, complicated by acute obstructive renal failure and cardiorenal syndrome (CRS) type 3. Patient was managed with mechanical ventilation, heparin-free hemodialysis, negative fluid balance, recombinant activated factor VII, and prednisolone. Hematuria was relieved, renal function improved, and cardiac function showed improvement on repeat echocardiography. Patient was discharged on prednisolone with subsequent follow ups. PMID:24501492
Sharma, Rakesh; Dash, Sananta Kumar; Chawla, Rajesh; Kansal, Sudha; Agrawal, Devender Kumar; Dua, Harsh
Hyperimmunoglobulinemia E (Job's) syndrome is characterized by markedly increased levels of immunoglobulin E, recurrent cutaneous and systemic pyogenic infections, atopic dermatitis, and peripheral eosinophilia. Although ocular involvement in Job's syndrome is rare, there are reports of keratoconus, staphylococcal chalazia with blepharitis, and Candida endophthalmitis by various authors. We present the first case report of retinal detachment with complicated cataract in Job's syndrome.
Arora, Vipul; Kim, Usha R; Khazei, Hadi M; Kusagur, Shivayogi
Dengue infection is now known to present with wide spectrum of complications. Isolated cases of acute pancreatitis complicating dengue haemorrhagic fever have been reported in literature. Here the authors report a case of dengue haemorrhagic fever that develops acute pancreatitis and presented with acute onset of breathlessness, which then progressed to full-blown acute respiratory distress syndrome. To our knowledge, this is the first reported case of dengue haemorrhagic fever complicated with acute pancreatitis and acute respiratory distress syndrome.
Agrawal, Avinash; Jain, Nirdesh; Gutch, Manish; Shankar, Amit
Background: The metabolic syndrome is associated with an increased risk of cardiovascular complications. Especially patients with evident cardiac pathology are at high risk for further complications. A sufficient weight reduction would improve the metabolic pathology and reduce the cardiovascular risk. Unfortunately, overweight and obese patients, even with complicated coronary heart disease, do not alter lifestyles regarding fat intake and physical
Giuseppe Cocco; Stefano Pandolfi; Valentin Rousson
Cardio-facio-cutaneous syndrome (CFC) is a multiple congenital anomaly disorder characterized by craniofacial dysmorphia, ectodermal abnormalities, congenital heart defects, and developmental and growth delay. Neurological complications associated with CFC remain to be clearly defined. Recent discovery of causative mutations in genes of the MAPK pathway (BRAF, MEK1, and MEK2) now permit accurate molecular diagnosis of CFC. The aim of the study was to characterize neurological features of participants with molecularly-confirmed CFC. Medical records, and laboratory and imaging data were reviewed for 39 mutation-positive individuals with CFC. Participants with a clinical diagnosis of CFC but a negative result on mutation screening of the BRAF, MEK1, and MEK2 genes were excluded from the study. Mean age of participants was 9 years 4 months (range 18 mo-24 y); there were 24 females and 15 males. Mutations in B RA F were present in 32 participants, MEK1 in five, and MEK2 in two participants. Hypotonia, motor delay, speech delay, and learning disability were universally present in this cohort. Macrocephaly was present in 13 participants, ptosis in 11, strabismus in 14, and nystagmus in 11 of the 22 participants who underwent a neurological exam. Corticospinal tract findings were present in seven participants. Ventriculomegaly or hydrocephalus was present in 14 of 32 participants who underwent brain imaging. Other findings on magnetic resonance imaging included prominent Virchow-Robin spaces (n=6), abnormal myelination (n=4), and structural anomalies (n=5). Seizures were present in 15 participants. No specific genotype-phenotype correlation was observed. PMID:18039235
Yoon, Grace; Rosenberg, Jodine; Blaser, Susan; Rauen, Katherine A
Harlequin syndrome is a rare neurological condition that results in unilateral facial flushing and sweating. Although the syndrome is generally a benign condition with complete resolution if appropriate treatment is initiated, unilateral facial flushing can be a sign of several serious conditions and should be thoroughly investigated. Sudden onset of facial flushing related to harlequin syndrome developed in a patient who had bilateral lung transplant with postoperative epidural anesthesia for pain control. Differential diagnosis includes neurovascular disease (acute stroke), malignant neoplasm of brain or lung, Horner syndrome, idiopathic hyperhidrosis, and Frey syndrome. Harlequin syndrome is often easily treated by discontinuing the anesthetic or adjusting placement of the epidural catheter. (Critical Care Nurse. 2014;34:57-61). PMID:24882829
Boling, Bryan; Key, Christopher; Wainscott, Justin; Rebel, Annette
A 33-year-old man who had received mediastinal radiation therapy for Hodgkin's disease 12 years earlier developed a symptomatic sick sinus syndrome requiring the implantation of a permanent pacemaker. The sick sinus syndrome and a finding of an occult constrictive pericarditis were considered to be due to the previous mediastinal irradiation. A ventricular pacemaker was chosen because mediastinal radiotherapy also increases
Sinikka Pohjola-Sintonen; K. J. K. Toetterman; Markku Kupari
Two patients with Behçet's syndrome are described. One patient developed spontaneous bilateral popliteal artery aneurysms and the other spontaneous thrombosis of the superior vena cava. These cases emphasize that primary inflammatory lesions of large vessels, with arterial aneurysm formation and thrombosis may be a feature of the syndrome. ImagesFig. 1Fig. 2Fig. 3Fig. 4Fig. 5Fig. 6Fig. 7
Enoch, B. Anthony; Khoo, T. C. L.; Castillo-Olivares, J. L.; Grainger, R. G.; Henry, L.
An 80-year-old man was admitted to our hospital because of pancytopenia. Bone marrow examination revealed an increase in the number of dysplastic cells indicating trilineage dysplasia. A 5q13q31 deletion was the only genetic abnormality found, and consequently, 5q deletion syndrome was diagnosed. Although lenalidomide therapy was initiated, it had to be discontinued because of Stevens-Johnson syndrome, which occurred during the second course of treatment. There was no discernible hematological improvement, and bone marrow aspiration showed transformation to refractory anemia with excess blasts-2(RAEB-2)after lenalidomide therapy. However, by changing the therapy to azacitidine, cytogenetic remission was achieved. PMID:24335378
Yamada, Michiko; Kuroda, Hiroyuki; Jomen, Wataru; Yoshida, Masahiro; Miura, Shogo; Abe, Tomoyuki; Sakurai, Tamaki; Fujii, Shigeyuki; Maeda, Masahiro; Fujita, Miri; Nagashima, Kazuo; Arihara, Yohei; Hirako, Tasuku; Kato, Junji
Spontaneous rupture of cystic mediastinal teratomas is rare but may cause serious complications. Here we report an unusual case of a cystic teratoma, which ruptured into the mediastinal and pleural cavities resulting in superior vena cava syndrome, acute mediastinitis, and pleural effusion. Early diagnosis and prompt surgical treatment of ruptured mediastinal teratomas are essential to preventing life-threatening complications. PMID:24484811
Liu, Chia-Hsin; Peng, Yi-Jen; Wang, Hong-Hau; Cheng, Yeung-Lung; Chen, Chien-Wen
Tonic dystonia is an underrecognized complication of reflex sympathetic dystrophy syndrome (RSDS) characterized by an increase in muscle tone at the site of injury.Case-reports.– We describe five cases of tonic dystonia complicating RSDS of the lower extremity. There were four women and one man, with a mean age of 52 years. In addition to the typical features of RSDS, the patients
Aude Morelet; Laurence Gagneux-Lemoussu; Pascal Brochot; Stanley Ackah-Miezan; Jean-François Colmet-Daage; François Gaillard; François Boyer; Jean-Paul Eschard; Jean-Claude Etienne
Prune belly syndrome (PBS) is a rare congenital malformation of unknown etiology characterized by congenital abnormalities including abdominal wall flaccidity, urinary tract alterations, and bilateral cryptorchidism. The incidence of the syndrome is between 1/35000 and 1/50000 live births and there is little information about the oral findings. The present case describes the oral manifestations of a 15-year-old boy diagnosed with PBS. The findings include enamel hypoplasia associated with generalized hypocalcemic dental lines. In the radiographic exam, pronounced demineralization of the trabecular bone of the jaws, loss of lamina dura in all the teeth, and discontinuity of the mandibular cortical bone were observed. Prune belly syndrome is a rare disease, whose clinical dental aspects are not pathognomonic of the syndrome. The comprehension of the systemic mechanism of PBS and its comorbidities enable an understanding of the systemic etiologic factors associated with oral manifestations. PMID:22901648
Pessoa, Larissa; Galvão, Virgilio
A 33-year-old man who had received mediastinal radiation therapy for Hodgkin's disease 12 years earlier developed a symptomatic sick sinus syndrome requiring the implantation of a permanent pacemaker. The sick sinus syndrome and a finding of an occult constrictive pericarditis were considered to be due to the previous mediastinal irradiation. A ventricular pacemaker was chosen because mediastinal radiotherapy also increases the risk of developing atrioventricular conduction defects.
Pohjola-Sintonen, S.; Toetterman, K.J.K.; Kupari, M. (Helsinki Univ. Central Hospital (Finland))
We report the case of a 24-year-old patient with known scimitar syndrome presenting with hemoptysis. Multidetector CT angiography showed the scimitar vein draining the right lung to the inferior vena cava associated with right lower lobe bronchopulmonary sequestration. The presence of hemoptysis associated with scimitar syndrome is likely secondary to an anomalous systemic arterial blood supply. We treated this patient successfully with transcatheter occlusion of the anomalous feeding arterial vessels.
Nedelcu, Cosmina; Carette, Marie-France [AP-HP, Tenon Hospital, Radiology Department (France); Parrot, Antoine [Respiratory Intensive Care Unit, AP-HP, Tenon Hospital (France); Hammoudi, Nadjib [AP-HP, Tenon Hospital, Cardiology Department (France); Marsault, Claude; Khalil, Antoine, E-mail: email@example.com [AP-HP, Tenon Hospital, Radiology Department (France)
We describe the clinical characteristics of 4 singleton cases, 3 males and 1 female, with Myhre Syndrome (OMIM 139210), who were born to non-consanguineous parents. Three cases had no family history of similarly affected individuals but 1 male’s mother had short stature, some facial features suggestive of Myhre syndrome and evidence of skewed X-chromosome inactivation in her blood DNA. Short
Ruth McGowan; Ramkumar Gulati; Pamela McHenry; Alexander Cooke; Sandra Butler; Wee Teik Keng; Victoria Murday; Margo Whiteford; Frederik G. Dikkers; Brigit Sikkema-Raddatz; Ton van Essen; John Tolmie
Scimitar syndrome (pulmonary venolobar syndrome) is a rare anomaly of venous return to the heart, most commonly consisting of partial or total anomalous pulmonary venous return from the right lung. This is the report of a case of a 29-year-old woman at 31 weeks of gestation of pregnancy who was previously diagnosed with scimitar syndrome. MR angiography and PET-CT results which were obtained before pregnancy demonstrated vascular malformation in the inferior part of the right lung. No specific treatment was planned throughout the pregnancy due to the absence of any symptoms. The patient's first physical examination was unremarkable except mild hypertension. In her follow-up, severe preeclampsia was developed and the patient had undergone a cesarean section of a live birth at 34 weeks and 2 days of gestation. This is the first case of scimitar syndrome with pregnancy in which the cardiac status of the patient deteriorated coincidentally due to the development of another manifestation such as severe preeclampsia besides the syndrome itself. PMID:24549272
Yuksel, Mehmet Aytac; Imamoglu, Metehan; Dincgez Cakmak, Burcu; Oncul, Mahmut; Madazli, Riza
We report a case of a child who presented cerebral and ocular congenital toxoplasmosis associated with West syndrome. He was seen and followed-up in the in patients pediatric and ophthalmologic units at the Gyneco-Obstetric and Pediatric Hospital of Yaoundé in Cameroon between July 2008 and February 2010.
Eballe, Andre Omgbwa; Ellong, Augustin; Zoua, Marie Evodie Akono; Bella, Lucienne Assumpta; Ngeufack, Seraphin; Kouam, Jeanne Mayouego; Melong, Justin
The carcinoid syndrome, associated with carcinoid tumors of the midgut, consists of symptoms such as diarrhea, flushing, wheezing and cardiovascular symptoms. This review focuses on these symptoms and discusses therapeutic options. The symptoms are caused by the secretion of biogenic amines, polypeptides and other factors of which serotonin is the most prominent. However, diarrhea is also due to factors such
Anouk N. A. van der Horst-Schrivers; A. N. Machteld Wymenga; Thera P. Links; Pax H. B. Willemse; Ido P. Kema; Elisabeth G. E. de Vries
Summary Background: Hyperimmunoglobulinemia E syndrome (hyper-IgE syndrome, Job syndrome, HIES) is a complex immune deficiency with multiorgan clinical manifestations and diverse genetic background. The clinical triad of symptoms observed in approximately 75% of patients with HIES includes: recurrent abscesses of staphylococcal etiology, recurrent respiratory infections and elevated immunoglobulin E in serum. Case Report: The paper discusses three cases of female patients presenting typical pulmonary complications of the hyper-Ig E syndrome. In the first case, the development of aspergilloma in a postinflamatory cyst was observed, in the other one, pneumonia with pleural effusion, and as a consequence of inflammatory infiltrations – fibrotic changes, giving rise to lobectomy, while in the last of these cases, the course of lung disease was complicated by formation of staphylococcal abscess. In one of the girls, bronchiectasis appeared at follow-up. Conclusions: Complications of pulmonary infections are the most common causes of death in hyper-Ig E syndrome. Late diagnosis significantly worsens the respiratory function and reduces the chance for normal development of a child. Introduction of comprehensive treatment, including prophylaxis, decreases the recurrences. Therefore, the important role is attributed to the radiologist in the multidisciplinary care of patients with this syndrome.
Jonczyk-Potoczna, Katarzyna; Szczawinska-Poplonyk, Aleksandra; Warzywoda, Malgorzata; Breborowicz, Anna; Pawlak, Bogdan
Primary Sjögren's syndrome (pSS) is an autoimmune inflammatory disorder characterized by lymphocytic infiltration of exocrine glands, mainly the lacrimal and salivary glands leading to a chronic sicca syndrome. However, extraglandular organ systems may frequently be involved, including both central and peripheral nervous systems. Clinically significant neurologic manifestations affect approximately 20% of patients and may be the first manifestation of the disease in at least 25% of the cases. The spectrum of pSS-related neuropathies is wide including sensory neuropathies, neuronopathies, sensory-motor neuropathies, mononeuritis multiplex related to vasculitis… Central nervous system involvement is composed by multiple sclerosis-like manifestations including acute and chronic myelopathies and by more diffuse manifestations (cognitive dysfunction, subacute aseptic meningitis, encephalopathy, psychiatric symptoms, chorea, seizures…). The diagnosis and treatment of such pSS-related manifestations must be optimized in order to avoid severe disability. PMID:22840992
Fauchais, Anne-Laure; Magy, Laurent; Vidal, Elisabeth
The results of partial medial epicondylectomy for cubital tunnel syndrome were evaluated in 60 elbows of 54 patients. Preoperatively, 8 patients were grade I, 24 grade IIA, 16 grade IIB, and 12 grade III according to the modified McGowan score (Goldberg BJ et al. JHand Surg [Am] 1989;14:182-8). Mean follow-up was 38.8 months. Special emphasis was placed on evaluation of
Stÿn Muermans; Luc De Smet
Subcapsular liver hematoma (SLH) is a rare complication of severe preeclampsia and HELLP syndrome. These patients must be followed up in intensive care unit for advanced medical support with infused fluid, replacement of blood products, and treatment of underlying disorders. There are a lot of therapeutic options varying from conservative management to surgical treatment including hepatic resection, hepatic artery ligation, and liver transplantation. In this report we aimed to present a 26-year-old woman with SLH secondary to HELLP syndrome.
Karateke, Atilla; Silfeler, Dilek; Karateke, Faruk; Kurt, Raziye; Guler, Ayse; Kartal, Ismail
A case of a 29-year-old woman 18 days after delivery with catastrophic antiphospholipid syndrome secondary (CAPS) due to undiagnosed systemic lupus erythematosus, leading to cardiogenic shock is reported. Laboratory evaluation revealed increased anticardiolipin antibodies, lupus anticoagulant, antinuclear antibody and thrombocytopenia. Left ventricular ejection fraction was 20%, neurologic deficit and acute renal failure were also present. Cardiac involvement is common in CAPS, but cardiomyopathy due to microvascular thrombosis is rare. CAPS should be considered as a cause of acute heart failure in a women with systemic lupus erythematosus. In the presented case early therapy with anticoagulants, steroids, immunoglobulins and plasmaferesis was beneficial. PMID:19650000
Zieba, Bozena; Wegrzyn, Agnieszka; Mital, Andrzej; Szczepi?ska-Nowak, Maria; Lewicki, Lukasz; Chmielecki, Micha?; Puchalski, Wies?aw; Rynkiewicz, Andrzej
Prader-Willi syndrome in the newborn is essentially characterized by marked hypotonia, feeding difficulties, hypogonadism, and possible characteristic facial features. However, diagnosis at this age may be particularly difficult, and dysmorphic features may be subtle or absent. Prematurity can furthermore delay clinical features recognition and typical complications due to preterm birth may contribute to divert the diagnosis. We describe a preterm baby with a complicated perinatal course later diagnosed as PWS.
Ciana, G.; Fertz, M. C.; Pecile, V.; Demarini, S.
We describe a case of Ehlers-Danlos syndrome (EDS) type IV in a male in early half in his twenties, who experienced recurrent and eventually fatal pulmonary hemorrhage. EDS type IV is a rare disorder of type III collagen synthesis that is characterized by unusual facies, thin translucent skin with a venous vascular pattern, easy bruising, and hypermobility of the small joints. Autopsy findings showed hypermobility of the joints and distensibility of the skin. Microscopically, the abdominal skin showed substantially decreased dermal thickness. Moreover, the reticular dermis showed fine collagen bundles and large interstitial spaces compared with the skin from a normal control that showed large collagen bundles. Individual elastic fibers were also thicker than those observed in the skin of a normal control. The thoracic aorta showed thin adventitia and a relative increase in elastic fibers. The parenchyma of both the lungs showed markedly diffuse hemorrhage with hemosiderin-laden alveolar macrophages or old thrombi and organized thrombi in the small bronchi. Furthermore, both sections of the lung showed multiple fibrous nodules containing benign metaplastic bone. Vascular wall disruption and tearing of the vessel walls in the lung parenchyma were also observed. We concluded that EDS type IV led to the patient's death because of pulmonary hemorrhage. Because this syndrome resulted in the patient's death from arterial and bowel rupture, it is important to consider EDS as a potential cause of sudden death. PMID:22940417
Hatake, Katsuhiko; Morimura, Yoshifumi; Kudo, Risa; Kawashima, Wataru; Kasuda, Shogo; Kuniyasu, Hiroki
Analyzing a complex case and referring to the literature, the authors describe abdominal compartmental syndrome as a complication of Fournier's gangrene, stressing out the importance of an early diagnosis to perform prompt and effective treatment. The characteristic of this case is not represented only by the Fournier's gangrene rarity, but also by the appearance of an abdominal compartmental syndrome due to the gangrene extension from the scrotum to the abdominal wall and cavity through the spermatic funiculus. The treatment of the abdominal compartmental syndrome was the surgical toilette of the necrotic regions (scrotum, abdominal wall and cavity) together with an orchiectomy. PMID:19419615
Cattorini, L; Cirocchi, R; Spizzirri, A; Morelli, U; De Sol, A; Napolitano, V; La Mura, F; Locci, E; Coccetta, M; Mecarelli, V; Giustozzi, G; Sciannameo, F
Obstructive sleep apnoea syndrome (OSAS) is a highly prevalent disease and is recognised as a major public health burden. Large-scale epidemiological studies have demonstrated an independent relationship between OSAS and various cardiovascular disorders. The pathogenesis of cardiovascular complications in OSAS is not completely understood but a multifactorial aetiology is likely. Inflammatory processes have emerged as critical in the pathogenesis of
S Ryan; C T Taylor; W T McNicholas
A previously fit and healthy 8-year-old boy died following severe complications of influenza A. He developed lethargy and vomiting before presentation. On presentation to medical attention, on day 4 of his illness, he was in extremis and had extensive myositis, rhabdomyolysis, renal failure and compartment syndrome, which were resistant to supportive medical management.
Skellett, Sophie Clare; Dhesi, Rosepal
Budd-Chiari syndrome (BCS) and portal hypertension is an uncommon complication of hydatid cyst of the liver. Previous reports describe cyst excision or portosystemic shunt surgery for such patients. Here we present a case of hydatid cyst of the liver with BCS that was treated successfully with hepatic venoplasty and transjugular stent placement.
Sarawagi, Radha, E-mail: firstname.lastname@example.org; Keshava, Shyamkumar N., E-mail: email@example.com; Surendrababu, Narayanam R. S., E-mail: firstname.lastname@example.org [Christian Medical College, Department of Radiology (India); Zachariah, Uday G., E-mail: email@example.com; Eapen, Eapen C., E-mail: firstname.lastname@example.org [Christian Medical College, Department of Gastroenterology (India)
Background: The abdominal compartment syndrome (ACS) is a recognized complication of damage control surgery (DCS). The purposes of this study were to (1) determine the effect of ACS on outcome after DCS, (2) identify patients at high risk for the development of ACS, and (3) determine whether ACS can be prevented by preemptive intravenous bag closure during DCS.Methods: Patients requiring
Christopher D Raeburn; Ernest E Moore; Walter L Biffl; Jeffrey L Johnson; Daniel R Meldrum; Patrick J Offner; Reginald J Franciose; Jon M Burch
Oxidant stress plays a central role in mediating the macro- and microvascular complications of diabetes and metabolic syndrome. Radicals antagonize protective nitric oxide (NO) bioactivity, through direct quenching of NO and uncoupling of NO synthase, while promoting inflammation and fibrosis via activation of NF-kappaB and TGF-beta, respectively. Oxidants are key mediators of insulin resistance in hypertrophied adipocytes - which gives
Mark F. McCarty; Toyoshi Inoguchi
We present a case of symptomatic perihepatic adhesions, which developed after a pregnancy complicated by hemolysis, elevated liver enzymes and low platelet (HELLP) syndrome, in which a subcapsular liver hematoma occurred. Our patient presented with complaints of persistent, severe right-sided upper abdominal pain. The complaints developed gradually, one year after a pregnancy that had been complicated by HELLP syndrome with a subcapsular liver hematoma. The hematoma had resolved spontaneously. An upper-abdominal magnetic resonance imaging revealed a density between liver and diaphragm at the site of the former subcapsular hematoma, suspect of perihepatic adhesions. The presence of perihepatic adhesions was confirmed during a laparoscopy and treated by adhesiolysis in the same session. The adhesions may have developed in response to the degradation process of the subcapsular liver hematoma during conservative treatment. This case of perihepatic adhesions may therefor be the first presentation of a long term sequel of subcapsular liver hematoma in HELLP syndrome.
Koeneman, Margot M; Koek, Ger H; Bemelmans, Marc; Peeters, Louis L
Percutaneous endoscopic gastrostomy (PEG) has been used for providing enteral access to patients who require long-term enteral nutrition for years. Although generally considered safe, PEG tube placement can be associated with many immediate and delayed complications. Buried bumper syndrome (BBS) is one of the uncommon and late complications of percutaneous endoscopic gastrostomy (PEG) placement. It occurs when the internal bumper of the PEG tube erodes into the gastric wall and lodges itself between the gastric wall and skin. This can lead to a variety of additional complications such as wound infection, peritonitis, and necrotizing fasciitis. We present here a case of buried bumper syndrome which caused extensive necrosis of the anterior abdominal wall.
Biswas, Saptarshi; Dontukurthy, Sujana; Rosenzweig, Mathew G.; Kothuru, Ravi
Background: We experienced a case in which Cronkhite-Canada Syndrome presented with complications of multiple gastric cancers and multiple colon adenomas. Case Report: Our case is a 64-year-old male who visited a nearby hospital with diarrhea and weight loss. The patient was anemic and hypoproteinemic, with multiple polyps in the stomach, duodenum, and large intestine. He also presented with alopecia, onychatrophia, cutaneous pigmentation, and dysgeusia, and was diagnosed with Cronkhite-Canada Syndrome. Follow-up examinations found multiple gastric cancers and colon adenomas. We performed a total gastrectomy and a polypectomy of the large intestine lesions, revealing 4 well-differentiated adenocarcinomas in the resected stomach, and tubular adenomas in the large intestine lesions. Intraoperative findings included scattered melanoid pigmentation on the mesentery and the small intestinal wall. Tumor cells were positive for p53 and Ki67 and partially positive for MUC5AC and MUC2. Cronkhite-Canada Syndrome polyps are generally classified as juvenile type polyps, and these polyps rarely become cancerous. However, of the 383 cases of Cronkhite-Canada Syndrome reported in Japan, complications of gastric cancer were found in 39 cases (10.2%), and only 8 cases with multiple gastric cancer were reported in Japan. including the cases we have personally experienced. There were only two English literatures on Cronkhite-Canada Syndrome complicated with gastric cancer. So it is necessary to notify this information of Cronkhite-Canada Syndrome to the world. Conclusions: Close gastrointestinal examination and strict follow-up are believed to be essential for Cronkhite-Canada Syndrome patients.
Isobe, Taro; Kobayashi, Teppei; Hashimoto, Kousuke; Kizaki, Junya; Miyagi, Motoshi; Aoyagi, Keishiro; Koufuji, Kikuo; Shirouzu, Kazuo
Epidural steroid injections are well accepted as a treatment for radicular back pain in appropriate candidates. While overall incidence of systemic side effects has not been well established, at least five biochemically proven cases of iatrogenic Cushing's Syndrome have been reported as complications of epidural steroid treatment. We present an additional case of iatrogenic Cushing's Syndrome and adrenal suppression in a middle-aged woman who received three epidural steroid injections over a four-month period. We review this case in the context of previous cases and discuss diagnostic and management issues.
A 26-year-old man presented with ipsilateral femur and ankle fractures. The patient was treated with interlocking nail of his femur fracture, followed by open reduction and internal fixation of his ankle fracture under tourniquet control. Postoperatively, the patient developed compartment syndrome of his thigh with elevated pressures, requiring decompressive fasciotomies. This case illustrates the possible complication of treating a femur fracture with intramedullary nailing and then immediately applying a tourniquet to treat an ipsilateral extremity fracture. Because of the complication with this patient, we feel the procedure should be staged, or a tourniquet should be avoided if possible.
Moore, M. R.; Garfin, S. R.; Hargens, A. R.
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
The translational path from pharmacological insight to effective therapy can be a long one. We aim to describe the management of Marfan syndrome as a case-example of how pharmacological and genomic insights can contribute to improved therapy. We undertook a literature search for studies of Marfan syndrome, to identify milestones in description, understanding and therapy of the syndrome. From the studies retrieved we then weaved an evidence-based description of progress. Marfan syndrome shows considerable heterogeneity in clinical presentation. It relies on defined clinical criteria with confirmation based on FBN1 mutation testing. Surgical advances have prolonged life in Marfan syndrome. First-line prophylaxis of complications with ?-adrenoceptor blockers became established on the basis that reduction of aortic pressure and heart rate would help. Over-activity of proteinases, first suggested in 1980, has since been confirmed by evidence of over-expression of matrix metalloproteinases (MMP), notably MMP-2 and MMP-9. The search for MMP inhibitors led to the evaluation of doxycycline, and both animal studies and small trials, provided early evidence that this widely used antimicrobial agent was useful. Identification of the importance of TGF-? led to evaluation of angiotensin II type I receptor (AT1R) blockers with highly promising results. Combination prophylactic therapy would appear rational. Pharmacological and genomic research has provided good evidence that therapy with losartan and doxycycline would prevent the aortic complications of Marfan syndrome. If on-going well designed trials confirm their efficacy, the outlook for Marfan syndrome patients would be improved considerably.
Li-Wan-Po, Alain; Loeys, Bart; Farndon, Peter; Latham, David; Bradley, Caroline
The baseline characteristics, complications, and survival of 489 black and 6,890 non-black patients with acute coronary syndromes were studied. Important racial differences were observed in demographic features, atherosclerosis risk factors, and treatment strategies; however, despite these differences, no independent difference was observed in clinical outcomes according to race. The 1-year mortality rate was 2.9% for black patients and 2.5% for non-black patients (p = 0.93). PMID:15374792
Asher, Craig R; Moliterno, David J; Bhapkar, Manjushri V; McGuire, Darren K; Rao, Sunil V; Holmes, David R; Newby, L Kristin; Bates, Eric R; Topol, Eric J
Introduction A perforation occurring during colonoscopy is an extremely rare complication that may be difficult to diagnose. It can be responsible for acute abdominal compartment syndrome, a potentially lethal complex pathological state in which an acute increase in intra-abdominal pressure may provoke the failure of several organ systems. Case presentation We report a case of acute abdominal compartment syndrome after perforation of the bowel during a colonoscopy in a 60-year-old North African man with rectal cancer, resulting in respiratory distress, cyanosis and cardiac arrest. Our patient was treated by needle decompression after the failure of cardiopulmonary resuscitation. An emergency laparotomy with anterior resection, including the perforated sigmoid colon, was then performed followed by immediate anastomosis. Our patient remains alive and free of disease three years later. Conclusion Acute abdominal compartment syndrome is a rare disease that may occasionally occur after a colonoscopic perforation. It should be kept in mind during colonoscopy, especially considering its simple salvage treatment.
Quality of life were explored in an open prospective controlled study in 116 patients with complicated diabetic foot syndrome (experimental group) and 30 diabetics without this syndrome (control group) by questioning of quality of life (questionnaires SF-36, Functional Scale of Lower Extremity) in long terms (6-18 months) after surgical treatment. Advantages of foot "saving" resections in surgical treatment of complicated diabetic boot syndrome were detected: patients without amputations had the physical wellbeing score and the Functional Scale of Lower Extremity number, comparable with the group of diabetics without diabetic foot syndrome. The integral mental wellbeing scores were low in all observed groups of patients. PMID:20804022
Grachev, T V; Levchik, E Iu
The group of severe cutaneous drug reactions with systemic symptoms includes several syndromes: toxic epidermal necrolysis, Stevens-Johnson syndrome, acute generalized exanthematous pustulosis, and drug reaction with eosinophilia and systemic symptoms (DRESS). These reactions occur several days to six weeks after introducing the incriminating drug. The skin and internal organs (liver, kidneys, lungs, etc.) are usually involved. A great possibility of lethal outcome is a critical characteristic of these syndromes. A patient with pyelonephritis diagnosed during emergency room workup is described. Ciprofloxacin was prescribed and the patient was discharged. After ten days, the patient came back with worsening condition, general inflammatory response, skin changes, liver and kidney damage, and eosinophilia. DRESS syndrome was diagnosed based on clinical and other findings. The diagnosis and treatment of severe drug reactions with cutaneous and systemic symptoms pose a medical challenge. PMID:21830461
Artukovi?, Marinko; Kustelega, Josipa; Lugovi?-Mihi?, Liborija
Obstructive sleep apnea syndrome (OSAS) is a common sleep related breathing disorder. Its prevalence is estimated to be between 2% and 25% in the general population. However, the prevalence of sleep apnea is much higher in patients undergoing elective surgery. Sedation and anesthesia have been shown to increase the upper airway collapsibility and therefore increasing the risk of having postoperative complications in these patients. Furthermore, the majority of patients with sleep apnea are undiagnosed and therefore are at risk during the perioperative period. It is important to identify these patients so that appropriate actions can be taken in a timely fashion. In this review article, we will discuss the epidemiology of sleep apnea in the surgical population. We will also discuss why these patients are at a higher risk of having postoperative complications, with the special emphasis on the role of anesthesia, opioids, sedation, and the phenomenon of REM sleep rebound. We will also review how to identify these patients preoperatively and the steps that can be taken for their perioperative management. Citation: Vasu TS; Grewal R; Doghramji K. Obstructive sleep apnea syndrome and perioperative complications: a systematic review of the literature. J Clin Sleep Med 2012;8(2):199-207.
Vasu, Tajender S.; Grewal, Ritu; Doghramji, Karl
The main aim of this study was to assess the obstetric complications for those pregnancies that are complicated by ovarian hyperstimulation syndrome (OHSS) and continue beyond the first trimester. We checked also for other related serious events that occurred during the first trimester. METHODS: We included only patients whose preg- nancies continued beyond the first trimester and compared them with
A. Wiser; J. Levron; D. Kreizer; R. Achiron; A. Shrim; E. Schiff; J. Dor; A. Shulman
Organophosphates (OPs) are ubiquitous in the world as domestic and industrial agricultural insecticides. Intentional poisoning as suicides attempts are clinical phenomena seen in emergency departments and clinics in agricultural areas. Intermediate syndrome with the neurological complication of extra pyramidal symptoms following acute OP ingestion may occur in pediatric and adult cases. While death is the most serious consequence of toxic OP doses, low levels of exposure and nonfatal doses may disrupt the neurobehavioral development of fetuses and children in addition to bring linked to testicular cancer and male and female infertility. These are disturbing. Chronic and acute toxicity from OPs are barriers to the health of our present and future generations. Symptoms and treatment of acute and chronic OP exposure are briefly referenced with inclusion of the intermediate syndrome. Suggestions for local and systemic reduction of the acute and long term consequences of OP ingestion are opined. PMID:25002781
Detweiler, Mark B
Organophosphates (OPs) are ubiquitous in the world as domestic and industrial agricultural insecticides. Intentional poisoning as suicides attempts are clinical phenomena seen in emergency departments and clinics in agricultural areas. Intermediate syndrome with the neurological complication of extra pyramidal symptoms following acute OP ingestion may occur in pediatric and adult cases. While death is the most serious consequence of toxic OP doses, low levels of exposure and nonfatal doses may disrupt the neurobehavioral development of fetuses and children in addition to bring linked to testicular cancer and male and female infertility. These are disturbing. Chronic and acute toxicity from OPs are barriers to the health of our present and future generations. Symptoms and treatment of acute and chronic OP exposure are briefly referenced with inclusion of the intermediate syndrome. Suggestions for local and systemic reduction of the acute and long term consequences of OP ingestion are opined.
Detweiler, Mark B.
Adrenal necrosis, a rare life threatening complication of antiphospholipid syndrome, is difficult to diagnose during pregnancy. We report the case of a 33-year-old woman with bilateral adrenal necrosis which started during the third trimester of her second pregnancy. Antiphospholipid syndrome had been diagnosed few years ago, after a thrombotic event. The pregnancy was uneventful until 36 weeks plus five days, when the patient was admitted for bilateral back ache, initially considered as uterine contractions. Labour was induced because pain persisted and was associated with major thrombocytopenia. A healthy infant was delivered vaginally on the second day, adrenal failure was diagnosed based on intense asthenia, persistent severe lumbar pain, low blood sodium and cortisol. Bilateral adrenal oedema was documented by CT scan and MRI. Symptoms resolved following administration of hydrocortisone and fludrocortisone. This case illustrates the difficulty to diagnose adrenal necrosis in the third trimester of pregnancy. PMID:18420445
Legendre, G; Vauthier-Brouzes, D; Cornet, A; Al Hawari, M; Renard-Penna, R; Piette, J-C; Dommergues, M
The present study reports the case of a 71-year-old female with metastatic adenocarcinoma of the skin who developed tumor lysis syndrome (TLS) upon admittance to the First Affiliated Hospital of Liaoning Medical University (Jinzhou, China). The patient presented to the hospital due to multiple subcutaneous nodules, lethargy and weakness, but succumbed without any cancer therapy. Metastases to the skin from solid carcinomas are uncommon, and several studies have reported patients with minimal primary symptoms despite extensive metastatic skin disease. However, few cases were accompanied with spontaneous TLS at the time of presentation. TLS may be a severe complication during the therapy for hematological and oncological diseases. Although spontaneous TLS in internal tumors has been reported, it is extremely rare. The present study highlights the fact that multiple subcutaneous metastases may occur with the symptoms of spontaneous TLS, and may be key for the early recognition of this syndrome.
WANG, YU; YUAN, CAIJUN; LIU, XIAOMEI
A rare life-threatening complication during percutaneous cryoablation of an adrenal gland metastasis from a lung carcinoma is reported. The patient presented hypertensive crisis at the beginning of the thawing cycle, followed by electrocardiographic change which necessitated interruption of the procedure and his transfer to the intensive care unit with suspicion of heart infarct. There was a slight increase in cardiac enzyme levels, and ventricular angiography demonstrated transient hypokinesis-dyskinesis of the mid left ventricular segments without apical involvement, while the coronary arteries showed no significant stenosis on coronarography. These findings led to the diagnosis of Takotsubo cardiomyopathy left ventricular dysfunction syndrome. This is the first case of Takotsubo cardiomyopathy occurring as a complication during percutaneous ablation of an adrenal gland tumor.
Tsoumakidou, Georgia, E-mail: email@example.com; Buy, Xavier, E-mail: Xavier.firstname.lastname@example.org [University Hospital of Strasbourg, Department of Non Vascular Interventional Radiology (France); Zickler, Pierre, E-mail: email@example.com [University Hospital of Strasbourg, Department of Anaesthesiology (France); Zupan, Michel, E-mail: Michel.firstname.lastname@example.org; Douchet, Marie-Pierre, E-mail: Marie.email@example.com [University Hospital of Strasbourg, Department of Cardiology (France); Gangi, Afshin, E-mail: firstname.lastname@example.org [University Hospital of Strasbourg, Department of Non Vascular Interventional Radiology (France)
Cushing's syndrome (CS) is a clinical condition resulting from chronic exposure to glucocorticoid excess. As a consequence, hypercortisolism contributes significantly to the early development of systemic disorders by direct and/or indirect effects. Complications such as obesity, hypertension, diabetes, dyslipidemia, and hypercoagulability cause premature atherosclerosis and increase cardiovascular mortality. Impairment of the skeletal system is a relevant cause of morbidity and disability in these patients especially due to the high prevalence of vertebral fractures. In addition, muscle weakness, emotional lability, depression, and impairment of quality of life are very common. Clinical management of these patients is complex and should be particularly careful in identifying global cardiovascular risks and aim at controlling all complications. Although the primary goal in the prevention and treatment of complications is the correction of hypercortisolism, treatment does not completely eliminate these comorbidities. Given that cardiovascular risk and fracture risk can persist after cure, early detection of each morbidity could prevent the development of irreversible damage. In this review we present the various complications of CS and their pathogenetic mechanisms. We also suggest the clinical management of these patients based on our extensive clinical experience and on the available literature. PMID:22652826
Arnaldi, G; Mancini, T; Tirabassi, G; Trementino, L; Boscaro, M
Hemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in childhood and is primarily diagnosed in up to 4.5% of children who undergo chronic renal replacement therapy. Escherichia coli serotype O157:H7 is the predominant bacterial strain identified in patients with HUS; more than 100 types of Shiga toxin-producing enterohemorrhagic E. coli (EHEC) subtypes have also been isolated. The typical HUS manifestations are microangiopathic hemolytic anemia, thrombocytopenia, and renal insufficiency. In typical HUS cases, more serious EHEC manifestations include severe hemorrhagic colitis, bowel necrosis and perforation, rectal prolapse, peritonitis, and intussusceptions. Colonic perforation, which has an incidence of 1%-2%, can be a fatal complication. In this study, we report a typical Shiga toxin-associated HUS case complicated by small intestinal perforation with refractory peritonitis that was possibly because of ischemic enteritis. Although the degree of renal damage is the main concern in HUS, extrarenal complications should also be considered in severe cases, as presented in our case.
Chang, Hye Jin; Kim, Hwa Young; Choi, Jae Hong; Choi, Hyun Jin; Ko, Jae Sung; Ha, Il Soo; Cheong, Hae Il; Choi, Yong
Hemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in childhood and is primarily diagnosed in up to 4.5% of children who undergo chronic renal replacement therapy. Escherichia coli serotype O157:H7 is the predominant bacterial strain identified in patients with HUS; more than 100 types of Shiga toxin-producing enterohemorrhagic E. coli (EHEC) subtypes have also been isolated. The typical HUS manifestations are microangiopathic hemolytic anemia, thrombocytopenia, and renal insufficiency. In typical HUS cases, more serious EHEC manifestations include severe hemorrhagic colitis, bowel necrosis and perforation, rectal prolapse, peritonitis, and intussusceptions. Colonic perforation, which has an incidence of 1%-2%, can be a fatal complication. In this study, we report a typical Shiga toxin-associated HUS case complicated by small intestinal perforation with refractory peritonitis that was possibly because of ischemic enteritis. Although the degree of renal damage is the main concern in HUS, extrarenal complications should also be considered in severe cases, as presented in our case. PMID:24678335
Chang, Hye Jin; Kim, Hwa Young; Choi, Jae Hong; Choi, Hyun Jin; Ko, Jae Sung; Ha, Il Soo; Cheong, Hae Il; Choi, Yong; Kang, Hee Gyung
Thymomas can present with a variety of paraneoplastic manifestations, mostly of autoimmune origin, including Good's syndrome when there is associated hypogammaglobulinemia. Although pure red cell aplasia is a recognised complication of thymoma, selective white cell aplasia is very rare, particularly in Good's syndrome. Lethal opportunistic infections are a feature of Good's syndrome, usually occurring in those patients with associated severe T lymphocyte defects. Although the cryptococcus is a recognised fungal pathogen in patients with other causes of CD4+ T cell lymphopenia, surprisingly this complication has not been reported in patients with Good's syndrome. We now describe a 70 year old man with Good's syndrome and pure white cell aplasia who presented with disseminated cryptococcosis, and provide an up-to-date review of the relevant literature. Despite meningeal involvement our patient recovered after combined treatment with intravenous globulin, granulocyte stimulating growth, corticosteroids and antifungal therapy. PMID:24627080
Akinosoglou, K; Melachrinou, M; Siagris, D; Koletsis, E; Marangos, M; Gogos, C A; Solomou, E E
Venous thrombosis is a well-known complication of nephrotic syndrome (NS), while arterial thrombosis is rare. We know of no reports of children with this complication. Here we report a case of 14-year-old girl with NS, who complicated with renal and cerebral infarctions resulting from arterial thrombosis. Urinary examination showed heavy proteinuria. She had intravascular dehydration. Serum albumin was 0.9 g/dL. Contrast-enhanced computed tomography (CT) showed a low-attenuation area in the right kidney. Decreased blood flow in the right middle cerebral artery was observed on MRA and also on multi-detector-row head CT. Urokinase and heparin were given. Cerebral infarction was treated neuroprotectively by i.v. infusion of edaravone. Comprehensive assessment of intravascular dehydration and the coagulation-fibrinolysis system is needed to guide decisions concerning prophylactic anticoagulation therapy. Better understanding of NS and its risks, as well as the necessity of drug therapy, may help teenagers to accept and cooperate with treatment. PMID:22830545
Sugimoto, Keisuke; Iba, Yoshinori; Fujita, Shinsuke; Sakata, Naoki; Okada, Mitsuru; Takemura, Tsukasa
Background Polycystic ovary syndrome (PCOS) is a common endocrine disorder in women of childbearing age. The risk of pregnancy and neonatal complications in women with PCOS is debatable. In order to determine the risk of pregnancy and neonatal complications, evidence regarding these risks was examined. Methods Literature searches were performed in the electronic databases MEDLINE, EMBASE, and CENTRAL based on the established strategy and eligible tries were included according to inclusion and exclusion criteria. A systematic literature review looking at rates of gestational diabetes mellitus (GDM), pregnancy-induced hypertension (PIH), preeclampsia, premature delivery, neonatal birth weight, caesarean section and admission to a neonatal intensive care unit (NICU) was conducted in women with PCOS. Pregnancy outcomes between women with PCOS versus controls were included. Sensitivity analyses were performed to determine the reliability of the available evidence and to validate the results. The study was performed with the approval of the ethics committee of the First Affiliated Hospital of Guangxi Medical University. Results A total of 27studies, involving 4982 women with PCOS and 119692 controls were eligible for the meta-analysis. Women with PCOS demonstrated a significantly higher risk of developing GDM (OR3.43; 95% CI: 2.49–4.74), PIH (OR3.43; 95% CI: 2.49–4.74), preeclampsia (OR2.17; 95% CI: 1.91–2.46), preterm birth (OR1.93; 95%CI: 1.45–2.57), caesarean section (OR 1.74; 95% CI: 1.38–2.11) compared to controls. Their babies had a marginally significant lower birth weight (WMD ?0.11g; 95%CI: -0.19 – -0.03), and higher risk of admission to NICU (OR 2.32; 95% CI: 1.40–3.85) compared to controls. Conclusions Women with PCOS have increased risk of adverse pregnancy and neonatal complications. It is necessary to establish guidelines for supervision during pregnancy and parturition to prevent these complications.
Introduction Cantrell syndrome is a rare syndrome of congenital defects, which can be complicated by severe pulmonary hypertension and left ventricular diverticulum; it has proved difficult to treat in clinical practice. Case presentation A 6-month-old Han Chinese baby girl weighing 3.5kg was diagnosed, using ultrasonography and radiography, as having Cantrell syndrome complicated by severe pulmonary hypertension. For safety, we divided management into two stages. For the first stage, we dealt with the left ventricular diverticulum and pulmonary hypertension. Three months later, we performed diorthosis for an intracardiac malformation. Conclusions Cantrell syndrome with pulmonary hypertension may respond well to this novel two-stage operation, which needs more verification via clinical practice.
We report a young female patient with Henoch-Schönlein purpura (HSP) nephritis complicated by reversible posterior leukoencephalopathy syndrome (RPLS). The patient suddenly showed generalized seizures and cortical blindness with severe hypertension due to renal insufficiency approximately 1 year after cessation of corticosteroid treatment for HSP nephritis. Magnetic resonance imaging (MRI) demonstrated bilateral abnormal signals mainly in the cerebellum and white matter of the occipital lobe. Clinical symptoms quickly improved in conjunction with disappearance of abnormal signals on brain MRI after starting control of hypertension and continuous hemodiafiltration with steroid pulse therapy and plasmapheresis. RPLS may be caused by vasculitis and also by hemodynamic change due to severe hypertension in HSP, particularly in patients with nephropathy. In such cases intensive treatment should be performed as soon as possible to avoid neurological sequelae. PMID:17205213
Sasayama, Daimei; Shimojima, Yasuhiro; Gono, Takahisa; Kaneko, Kazuma; Matsuda, Masayuki; Ikeda, Shu-ichi
Bardet-Biedl syndrome (BBS) is a rare heterogeneous autosomal recessive disorder characterized by rod-cone dystrophy, postaxial polydactyly, truncal obesity, hypogonadism, learning disability, and renal anomaly that are caused by ciliary dysfunction. 16 genes have been associated with the BBS phenotype. Although recent pathophysiological studies using animal models have shown that ciliary dysfunction may induce hydrocephalus, there have been no reports of BBS with intracranial hypertension. We here describe a 9-year-old Japanese girl who was diagnosed as having BBS and later received renal transplantation due to chronic renal failure. She also exhibited intracranial hypertension, including papilledema and increased intrathecal pressure (260-300mmH2O), but her brain magnetic resonance imaging was normal. No genetic abnormalities were detected by DNA chip analysis or exome sequencing. Her papilledema improved following administration of acetazolamide. This is the first report of a case of BBS complicated with intracranial hypertension and its treatment. PMID:24290075
Saida, Ken; Inaba, Yuji; Hirano, Makito; Satake, Wataru; Toda, Tatsushi; Suzuki, Yutaka; Sudo, Asuka; Noda, Shunsuke; Hidaka, Yoshihiko; Hirabayashi, Kazutaka; Imai, Hiroki; Kurokawa, Toru; Koike, Kenichi
Posterior reversible encephalopathy syndrome (PRES) is an uncommon post-renal transplant complication. We report a 16-year-old boy who had an acute cellular rejection immediate post-transplant and was given intravenous methylprednisolone along with an increase in tacrolimus dose. He was diagnosed to have PRES based on clinical and radiological features within 6 h of intensified immunosuppression. This is an unusual case report of successfully managing PRES with continuation of the intensified immunosuppression as warranted by the clinical situation, along with aggressive blood pressure control. After 6 weeks, magnetic resonance imaging showed complete resolution of lesions. He has good graft function and no residual neurological deficits while on small doses of three antihypertensives, 12 months after transplantation.
Alexander, S.; David, V. G.; Varughese, S.; Tamilarasi, V.; Jacob, C. K.
Ovarian hyperstimulation syndrome (OHSS) is the most serious complication of sterility treatment. It is characterised by ovarian enlargement, ascites, electrolyte disturbance, hypovolaemia and haemoconcentration. A case of ischaemic stroke due to right middle cerebral artery (MCA) occlusion in a young female with OHSS after pharmacological treatment of sterility is reported. Left central hemiparesis occurred suddenly within a few days after the embryo transfer. Magnetic resonance imaging diffusion-weighted images showed infarction in the right basal ganglia and magnetic resonance angiography (MRA) revealed the occlusion of the M1 segment of the right MCA. The haemodilution and the anticoagulation therapy were effective. Twenty-four hours after the stroke onset, MRA showed MCA recanalisation. The neurological deficit resolved completely within 3 months. The patient delivered 2 healthy infants at term. This case emphasises that the recent advent of ovulation induction and reproductive techniques is a newly recognised cause of cerebral stroke in otherwise healthy females. PMID:18923808
Bartkova, Andrea; Sanak, Daniel; Dostal, Jiri; Herzig, Roman; Otruba, Pavel; Vlachova, Ivanka; Hlustik, Petr; Horak, David; Kanovsky, Petr
Gastrointestinal and feeding complications after the Norwood procedure in infants with hypoplastic left heart syndrome increases\\u000a morbidity and mortality. These problems are the result of intraoperative challenges, shunt-dependent physiology, and the absence\\u000a of best-practice guidelines. In response, a systematic review of feeding-related complications and management strategies was\\u000a performed. A literature search from 1950 to March 2010 identified 21 primary research
Jessica R. Golbus; Brandon M. Wojcik; John R. Charpie; Jennifer C. Hirsch
The short bowel syndrome is a multifactorial disease that requires an intensive treatment especially if complication develop. The authors report their experience in a patient who, affected by acute renal failure after extensive intestinal resection, underwent temporary jejunostomy. The treatment of choice in this patient was initially a careful hydroelectrolytic balance, as he was in good nutritional status but underwent a dialytic treatment and jejunostomy. Then we started the nutritional support and initially we adopted enteral nutrition in order to facilitate small bowel functional integrity, avoid gastric uremic lesions and bacterial translocation which could prelude to multiple organ failure. At surgery we tried to preserve the distal ileum which is very important in order to absorb biliary salts and nutritional mixtures. Cholecystectomy was associated due to gangrenous cholecystitis probably caused both by short bowel syndrome and prolonged artificial nutrition. Six months after surgery the patient underwent a decrease of the body weight and then authors verified the stabilization of the weight and general conditions. PMID:7849149
Li Destri, G; Greco, L; Gresta, S; Di Cataldo, A; Puleo, S
Patients developing the short bowel syndrome (SBS) are at risk for hepatobiliary disease, as are morbidly obese individuals. We hypothesized that morbidly obese SBS individuals would be at increased risk for developing hepatobiliary complications. We reviewed 79 patients with SBS, 53 patients with initial body mass index (BMI) < 35 were controls. Twenty-six patients with initial BMI > 35 were the obese group. Obese patients were more likely to be weaned off parenteral nutrition (PN) (58% vs. 21%). Pre-resection BMI was significantly lower in controls (26 vs. 41). BMI at 1, 2, and 5 years was decreased in controls but persistently increased in obese patients. Obese patients were more likely to undergo cholecystectomy prior to SBS (42% vs. 32%) and after SBS (80% vs. 39%, p < 0.05). Fatty liver was more frequent in the obese group prior to SBS (23% vs. 0%, p < 0.05) but was similar to controls after SBS (23% vs. 15%). Fibrosis (8% vs. 13%) and cirrhosis/portal hypertension (19% vs. 21%) were similar in obese and control groups. Overall, end stage liver disease (ESLD) was similar in obese and control groups (19% vs. 11%) but was significantly higher in obese patients receiving PN (45% vs. 14%, p < 0.05). Obese patients developing SBS are at increased risk of developing hepatobiliary complications. ESLD was similar in the two groups overall but occurs more frequently in obese patients maintained on chronic PN.
Thompson, Jon S.; Weseman, Rebecca A.; Rochling, Fedja A.; Grant, Wendy J.; Botha, Jean F.; Langnas, Alan N.; Mercer, David F.
Biallelic mutations in FKBP14 cause a recessive form of Ehlers-Danlos syndrome (EDS) characterized by progressive kyphoscoliosis, myopathy, and hearing loss. To date, four children and one adult with this condition have been reported. We recently identified a 42-year-old man with severe kyphoscoliosis, restrictive/obstructive lung disease, short stature, mild hearing loss, decreased muscle mass, and a dissection of the celiac artery at age 41. He also had complete occlusion of the superior mesenteric artery with compensatory flow through an enlarged and tortuous inferior mesenteric artery. He was homozygous for a previously identified FKBP14 mutation, c.362dupC, p.(Glu122Argfs*7). He had no mutations in COL3A1, ACTA2, TGFBR1, TGFBR2, or SMAD3. The FKBP14 mutations in our patient occurred on the same haplotype as others with this same mutation. Although one family member in a previous report was thought to have early vascular complications, it could not be confirmed that she had biallelic mutations in FKBP14. This report expands the phenotype of FKBP14-related EDS to include risk for vascular complications and also raises the question of whether the shared haplotype represents a risk allele or founder mutation. © 2014 Wiley Periodicals, Inc. PMID:24677762
Murray, Mitzi L; Yang, Margaret; Fauth, Christine; Byers, Peter H
Polycystic ovarian syndrome (PCOS) is often accompanied by infertility that necessitates ovulation induction using clomiphene citrate, gonadotropins or even in vitro fertilization (IVF). These treatment methods are known to increase the incidence of multiple pregnancies as well as some negative consequences, including a rise in the risk for gestational diabetes mellitus, pre-eclampsia, etc., Furthermore, pregnancies established after IVF carry an increased risk for maternal complications. However, the increased risk of developing adverse obstetric complications has been suggested to occur independently of obesity as well as in populations without assisted reproductive techniques. Many studies have been performed to study the effect of PCOS on pregnancy and the effect of pregnancy on PCOS. The hormonal milieu that is exaggerated in PCOS women is quite well understood at the biochemical and genetic levels. The maternal and neonatal outcomes of PCOS women who have undergone in vitro fertilization-embryo transfer (IVF-ET) have not been widely studied till date. This review aims to evaluate the current evidence regarding adverse obstetric outcomes of PCOS women undergoing IVF-ET. The rationale of this review is to study whether the adverse obstetric outcomes are increased in PCOS women in general, or particularly in those PCOS women who are undergoing IVF-ET. It is also important to analyze via a literature review whether the increased adverse outcomes are due to infertility in general or PCOS per se. An attempt has been made to give evidence regarding preventive strategies for obstetric complications in PCOS women who have undergone IVF-ET. PMID:24829525
Tandulwadkar, Sunita R; Lodha, Pooja A; Mangeshikar, Nirzari T
Polycystic ovarian syndrome (PCOS) is often accompanied by infertility that necessitates ovulation induction using clomiphene citrate, gonadotropins or even in vitro fertilization (IVF). These treatment methods are known to increase the incidence of multiple pregnancies as well as some negative consequences, including a rise in the risk for gestational diabetes mellitus, pre-eclampsia, etc., Furthermore, pregnancies established after IVF carry an increased risk for maternal complications. However, the increased risk of developing adverse obstetric complications has been suggested to occur independently of obesity as well as in populations without assisted reproductive techniques. Many studies have been performed to study the effect of PCOS on pregnancy and the effect of pregnancy on PCOS. The hormonal milieu that is exaggerated in PCOS women is quite well understood at the biochemical and genetic levels. The maternal and neonatal outcomes of PCOS women who have undergone in vitro fertilization-embryo transfer (IVF-ET) have not been widely studied till date. This review aims to evaluate the current evidence regarding adverse obstetric outcomes of PCOS women undergoing IVF-ET. The rationale of this review is to study whether the adverse obstetric outcomes are increased in PCOS women in general, or particularly in those PCOS women who are undergoing IVF-ET. It is also important to analyze via a literature review whether the increased adverse outcomes are due to infertility in general or PCOS per se. An attempt has been made to give evidence regarding preventive strategies for obstetric complications in PCOS women who have undergone IVF-ET.
Tandulwadkar, Sunita R; Lodha, Pooja A; Mangeshikar, Nirzari T
BackgroundThough a severe left ventricular systolic dysfunction has been described in most patients with Takotsubo-like (or Apical Ballooning) syndrome, the occurrence of intra-cavitary thrombus formation seems to be such an exceptional finding. However, no large studies but single case descriptions of this complication are available over the last decade in the literature.
Cesare de Gregorio; Patrizia Grimaldi; Concetta Lentini
Post-traumatic undiagnosed disco-ligamentous and osseous lesions of the cervical spine may eventually result in irreducible extreme kyphosis. Correction of such consolidated deformities requires major surgery with a combined posterior and anterior approach, aiming to correct bony impingement on neural and vascular structures, reduce deformity and to attain circumferential instrumentation and fusion in physiological alignment. This can be achieved using either a single-staged or a two-staged procedure. Regardless, this type of major surgery entails considerable neurological risks. Therefore, thorough planning of the intervention and considerable surgical experience is needed. We present an elderly woman with gross restriction of forward gaze and intractable nuchal and radicular pain due to cervical spine deformity. Her cervical kyphosis was corrected using preoperative skeletal axial traction for four days and subsequent operative reduction with circumferential instrumentation and fusion. The post-operative course was complicated by a temporary anterior spinal artery syndrome despite normal intraoperative somatosensory evoked potentials (SSEP) and by a wound infection requiring removal of the implant. Nevertheless, segmental fusion in physiological alignment was successfully achieved and the patient fully recovered from the neurological deficit and infection. Quality of life was significantly improved. PMID:15749431
Wenger, Markus; Braun, Michel; Markwalder, Thomas-Marc
Few cases of Churg-Strauss syndrome (CSS) complicated by giant coronary aneurysms (CAs)have been reported thus far. We report a case of CSS in a 60-year-old man who underwent surgery for giant CAs, and was managed with anesthetics. The patient developed acute myocardial infarction, and was diagnosed with giant CAs in the right coronary artery (RCA, 11 cm) and circumflex artery (3 cm). The CA in RCA was communicating with the right ventricle. He had a history of pericardiectomy for pericarditis caused by the CSS and developed thrombocytopenia due to consumptive coagulopathy within the CAs. An operation, including ligation and excision of the CAs, and coronary artery bypass grafting was performed under general anesthesia and cardiopulmonary bypass. There was massive hemorrhage followed by hemodynamic instability while detaching the tight pericardial adhesion and fragile surface of the CAs. Massive transfusion was required along with inotropes administration and intraaortic balloon support. In this case, determination of the appropriate surgical timing was difficult because symptoms of the CSS became worse followed by rapid enlargement of the CAs, myocardial infarction, and thrombocytopenia. Steroids were administered for treating CSS, and the blood transfusion was sufficient. However, it was difficult to control the hemorrhage and maintain hemodynamic stability. PMID:24783616
Kido, Koji; Tokuda, Rui; Suzuki, Tomofumi; Hanashiro, Ako; Kobashigawa, Teruyo; Mayama, Takashi; Kamikawa, Michie
Extracorporeal life support (ECLS) is an incredible life-saving measure that is being used ever more frequently in the care of the critically ill. Management of these patients requires extreme vigilance on the part of the care providers in recognizing and addressing the complications and challenges that may arise. We present a case of overt abdominal compartment syndrome (ACS) in a previously well young male on ECLS with a history of trauma, submersion, hypothermia, and no intra-abdominal injuries. The patient developed ACS soon after ECLS was initiated which resulted in drastically compromised flow rates. Taking into account the patient's critical status, an emergent laparotomy was performed in the intensive care unit which successfully resolved the ACS and restored ECLS flow. The patient had an unremarkable course following and was weaned off ECLS but unfortunately died from his original anoxic injury. This case highlights several salient points: first, care of patients on ECLS is challenging and multiple etiologies can affect our ability to manage these patients; second, intra-abdominal pressures should be monitored liberally in the critically ill, especially in patients on ECLS; third, protocols for emergent operative treatment outside of traditional operating rooms should be established and care providers should be prepared for these situations.
Lee, Arthur J.; Wells, Bryan J.; Chun, Rosaleen; Ball, Chad G.; Kirkpatrick, Andrew. W.
A 22-year-old morbidly obese, nonpregnant woman presented with left ileofemoral deep vein thrombosis (DVT) presenting as low back pain and bilateral, left greater than right, leg swelling and pain for 2 days. While on heparin, she developed compartment syndrome in her left leg and had evidence of dead muscle tissue at the time of fasciotomy. Three options exist for treatment of ileofemoral DVT: catheter-directed thrombolysis (CDT), CDT plus pharmacomechanical thrombolysis or percutaneous mechanical thrombectomy, and surgical thrombectomy. Catheter-directed thrombolysis alone or in conjunction with pharmacomechanical thrombolysis in patients with low risk of bleeding has shown significant lysis of occlusion in 79% of patients with ileofemoral DVT with relatively low complication rates. Surgical thrombectomy and fasciotomy have not proven to be as effective but are appropriate alternatives if CDT is not available. Standard anticoagulation alone is likely not a sufficient treatment for ileofemoral DVT. Other therapies including CDT, CDT plus pharmacomechanical thrombolysis or percutaneous mechanical thrombectomy, or surgical thrombectomy to address lysis of the clot should be attempted first or in conjunction with anticoagulation for appropriate patients. Catheter-directed thrombolysis with or without pharmacomechanical thrombolysis is the preferred initial treatment. PMID:24091199
Lamborn, David R; Schranz, Craig
Primary hypothyroidism in the juvenile population generally leads to retardation of linear growth and delay or even arrest of puberty. However, in rare conditions, children with long-standing hypothyroidism present with signs of Van Wyk-Grumbach's syndrome (VWGS) which include juvenile hypothyroidism, delayed bone age, and pseudoprecocious puberty. We report a rare case of prepubertal male child from Asian origin, presented with long-standing untreated hypothyroidism complicated with VWGS and other complications including obesity, short stature, hepatomegaly, asymptomatic mild pericardial effusion, and pituitary hyperplasia.
Omran, Ahmed; Peng, Jing; Shrestha, Biswas; Ashhab, Muhammad Usman; Yin, Fei
Background Giant placental chorioangiomas have been associated with a number of severe fetal complications and high perinatal mortality. Case presentation We report a case of giant chorioangioma with fetal hydrops, additionally complicated by severe anemia, mild cardiomegaly with hyperdinamic heart circulation and maternal mirror syndrome. Intrauterine blood transfusion and amniodrainage was performed at 29?weeks. Worsening of the fetal and maternal condition prompted us to proceed with delivery at 29?+?5?weeks. The newborn died 3 hours later due to pulmonary hypoplasia and hemodynamic failure. Maternal course was favourable, mirror syndrome resolved in the second day and the patient was discharged four days following delivery. Conclusions In the case described here, fetal condition got worse despite of the anemia correction and amniodrainage. Our outcome raises the issue whether additional intrauterine clinical intervention, as intersticial laser, should have been performed to stop further deterioration of the fetal condition when progressive severe hydrops develops.
Silver syndrome (SS) is a complicated form of hereditary spastic paraplegia associated with distal wasting of the small muscles of the hands. We have previously described a large kindred with SS and mapped a genetic locus (SPG17) to chromosome 11q12-q14. In the current study we analyse the clinical phenotype and perform linkage analysis in three new SS families. In addition
Thomas T. Warner; Heema Patel; Christos Proukakis; Johanna A. Reed; Laura McKie; Adrian Wills; Michael A. Patton; Andrew H. Crosby
Haemolytic-uraemic syndrome (HUS) is a rare cause of insulin-dependent diabetes mellitus during the acute stage. We previously reported the case of a 3-year-old girl having presented with typical HUS with diarrhea, microangiopathic anaemia, thrombocytopenia and acute renal failure (17 days of anuria). Transient hyperglycaemia (highest level: 513 mg/dl) was observed, requiring continuous intravenous insulin infusion for 9 days. Subcutaneous insulin injections were stopped after 24 days. Oral glucose tolerance test performed 4 months after normalization of blood glucose was normal. HLA DQ genotype (DQA1-DQB1.AZH/DQA3-DQB3.1) was not at risk for type 1 diabetes and there were no auto-antibodies (ICA and IAA). The 3-years follow-up was marked by persistent arterial hypertension, proteinuria and slight renal insufficiency despite angiotensin-converting enzyme inhibitor treatment. Ten years after HUS occurred (the patient had been lost to follow-up for 7 years), she came back with complaints of headache but neither polyurodipsia nor weight loss. She was found to have arterial hypertension. Chronic renal impairment had moderately progressed with decreased glomerular filtration rate (63 ml/min/1.73 m2) and proteinuria (2 g/24 hours). Fasting blood glucose was 189 mg/dl and reached 315 mg/dl during an oral glucose tolerance test. HbA1c level was 8.2% (N<6.2%) and diabetes mellitus was diagnosed without any signs of autoimmunity (IAA, ICA, GADA and IA2B were negative). Good glycaemic control was obtained with 0.5 U/kg/day of insulin. In conclusion, transient beta-cell dysfunction complicating HUS acute stage may evolve to overt non-autoimmune diabetes mellitus (microangiopathic process?), even after a long free interval. This case emphasizes the need for a long-term follow-up of patients with HUS. PMID:16799406
Goffin, L; Lolin, K; Janssen, F; Schurmans, T; Dorchy, H
Background Myelodysplastic syndrome (MDS) is rare in people aged <50 years. Most patients with this disorder experience progressive worsening of blood cytopenias, with an increasing need for transfusion. The more advanced and severe the disorder, the greater the risk that it will progress to acute myeloid leukemia. Therapy is typically based on the patient's risk category, age, and performance status. Supportive care alone is a major option for lower-risk, older patients with MDS or those with comorbidities. The only potentially curative treatment option is hematopoietic stem-cell transplantation, which is typically used to treat high-risk, younger patients. Objective To describe and compare the hematologic complications, healthcare utilization, and costs of supportive care in patients with MDS aged <50 years and in older patients aged ?50 years. Methods Using the i3/Ingenix LabRx claims database, this retrospective study included patients who were continuously enrolled (ie, 6 months preindex through 1 year postindex) in the study and who had an initial claim of MDS (index date) between February 1, 2007, and July 31, 2008. Patients treated with hypomethylating agents or thalidomide analogues were excluded. Claims included information on office visits, medical procedures, hospitalizations, drug use, and tests performed. The hematologic complications, costs, and utilization analyses were stratified by age into 2 age-groups—patients aged <50 years and those aged ?50 years. The MDS-related diagnoses, utilization, and costs were analyzed postindex. The data used in this study spanned the period from August 1, 2006, to July 31, 2009. Results We identified 1133 newly diagnosed patients with MDS who received supportive care only during the study period; of these, 19.5% were younger than age 50 years. These younger patients included more females (62.0% vs 52.5%; P = .011) and had fewer comorbidities (mean Charlson comorbidy index, 1.2 vs 2.4; P <.001) and physician office visits than those aged ?50 years. Postindex, compared with the older patients, the younger patients had less use of erythropoietin therapy and fewer transfusions, anemia diagnoses, and potential complications of neutropenia and pneumonia diagnoses; however, more diagnoses of neutropenia and of decreased white blood cell counts were seen in the younger patients than in the older patients (P ?.034 for all comparisons). Furthermore, younger patients had fewer mean office visits in the postindex period than older patients (17.5 vs 24.2, respectively; P <.001) and fewer hospitalizations (32.1% vs 44.6%, respectively; P = .004), but they had a longer (although not statistically significant) mean length of hospital stay (21 vs 14 days, respectively; P = .131). Mean total healthcare charges were $96,277 (median, $21,287) in younger patients compared with $84,102 (median, $39,402) in older patients, although this difference, too, was not significant. Conclusions MDS is associated with frequent and prolonged hospitalizations, frequent outpatient visits, and high costs in younger and in older patients who are receiving supportive care. Although this study shows that younger patients aged <50 years do not have significantly higher costs overall, a small proportion may have a higher healthcare utilization and cost-related burden of MDS than patients aged ?50 years.
Powers, Annette; Faria, Claudio; Broder, Michael S.; Chang, Eunice; Cherepanov, Dasha
A 69-year-old woman complained of diplopia and truncal titubation after upper respiratory infection. She presented with mydriasis and external opthalmoplegia of bilateral eyes, ataxia, hyporeflexia and cervical-brachial muscle weakness. The protein abnormally increased (49 mg/dl) in the cerebrospinal fluid, and the serum anti-GQ1b and anti-GT1a IgG antibodies were positive. The blood sodium level was 128 mmol/l indicating hyponatremia. She had low plasma osmolarity (251 mOsm/kg), high urine osmolarity (357 mOsm/kg) and high urine sodium level (129 mmol/l), while the blood level of antidiuretic hormone was not able to be measured. She was diagnosed to have Fisher syndrome (FS), pharyngeal-cervical-brachial variant of Guillain-Barré syndrome (PCB) and syndrome of inappropriate secretion of antidiuretic hormone (SIADH). The hyponatremia improved with hyperosmotic saline infusion and restriction of water intake. Intravenous immunoglobulin therapy (IVIg) was effective only for ataxia, but the other symptoms mostly remained unchanged for a month. The serum anti-GQ1b IgG antibody was still positive even after one month. We performed high-dose intravenous steroid-pulse therapy. Then the mydriasis, external opthalmoplegia and cervical-brachial muscle weakness were immediately improved. This was a rare case of FS and PCB complicated with SIADH. IVIg, not steroid therapy, is generally chosen for FS since FS is considered as a variant of Guillain-Barré syndrome and steroid is not effective for Guillain-Barré syndrome as was proven by double-blind study. We suppose that the combined therapy of IVIg and steroid would be effective in patients with complicated symptoms and multiple antibodies. PMID:23603545
Murakami, Takenobu; Yoshihara, Akioh; Kikuchi, Saeko; Yasuda, Megumi; Hoshi, Akihiko; Ugawa, Yoshikazu
Antiphospholipid syndrome is a systemic autoimmune disease with thrombotic tendency. Consensus guidelines for pregnancy with antiphospholipid syndrome recommend low-dose aspirin combined with unfractionated or low-molecular-weight heparin because antiphospholipid syndrome causes habitual abortion. We report a 36-year-old pregnant woman diagnosed with antiphospholipid syndrome receiving anticoagulation treatment. The patient developed left abdominal pain and gross hematuria at week 20 of pregnancy. An initial diagnosis of left ureteral calculus was made. Subsequently abdominal-pelvic computed tomography was required for diagnosis because of the appearance of severe contralateral pain. Computed tomography revealed serious renal hemorrhage, and ureteral stent placement and pain control by patient-controlled analgesia were required. After treatment, continuance of pregnancy was possible and vaginal delivery was performed safely. This is the first case report of serious renal hemorrhage in a pregnant woman with antiphospholipid syndrome receiving anticoagulation treatment and is an instructive case for urological and obstetrical practitioners. PMID:21350603
Kawaguchi, Shohei; Izumi, Kouji; Nohara, Takahiro; Miyagi, Tohru; Konaka, Hiroyuki; Mizokami, Atsushi; Koh, Eitetsu; Namiki, Mikio
Chyloptysis, in the context of middle lobe syndrome, and chylothorax are rare clinical entities. They are reported in the medical literature mostly as case reports, but never together in the same patient. The present report describes the case of a 34-year-old woman who presented with chyloptysis associated with recurrent right middle lobe syndrome since she was 20 years of age, and eventually underwent right middle lobectomy. A few weeks postoperatively, she developed a right-sided chylothorax, which was refractory to medical therapy, and was successfully treated with thoracic duct ligation. She has been symptom-free for two years postsurgery.
Illamperuma, Chamani; Reid, John; Kanthan, Rani
Background In 1996, Holmium laser enucleation of the prostate was introduced and has been shown to be safe and highly effective. Case presentation We report a case of a rare complication that resulted in intra-abdominal compartment syndrome with prolonged intubation and intensive care, involving an 74-year-old male after holmium laser enucleation of prostate, with a massive irrigant fluid leakage into the retroperitoneal space. The elevated abdominal pressure was reduced by forced diuresis. The tracheal tube was removed 18 hours after the patient’s transfer to the ICU. The patient was discharged to home one week after the operation. Conclusion In rare cases when no obvious ruptures of the prostate capsule or the bladder occur during laser enucleation of prostate, knowledge regarding possible emersion of massive amounts of irrigant fluid into the retroperitoneal space leading to intra-abdominal compartment syndrome aids in the diagnosis and subsequent successful therapy of intra-abdominal hypertension.
Abstract In 2011, autochthonous Plasmodium vivax malaria emerged in a focal geographical area in Greece after importation by immigrants from the Indian subcontinent. We report the case of complicated P. vivax malaria in a previously healthy 42-year-old Greek female. The patient presented acute respiratory distress syndrome (ARDS), worsening jaundice, and thrombocytopenia after the administration of antimalarial treatment and despite a decreasing burden of parasitemia. She recovered fully after admission in the intensive care unit and support with mechanical ventilation. We discuss the risks potentially associated with the reappearance of P. vivax malaria in a previously malaria-free area. PMID:24745658
Gougoutsi, Alexandra; Karageorgopoulos, Drosos E; Dimitriadou, Areti; Melas, Nikolaos; Kranidiotis, Georgios; Voutsinas, Dionysios; Melidonis, Andreas
We observed a 63-year old male with cardiac amyloidosis who presented with the clinical symptoms of sick sinus syndrome and dyspnea and abnormal thickening of the right atrial wall, which extended to the junction of the superior vena cava. This may explain the relationship of abnormal thickening of the right atrium which extends to the junction of the superior vena cava and right atrium with amyloid deposits in the sinus node and occurrence of sick sinus syndrome. PMID:17067698
Narumi, Hiroya; Funabashi, Nobusada; Takano, Hiroyuki; Sekine, Tai; Ueda, Marehiko; Hori, Yasuhiko; Fukawa, Taisuke; Minamino, Tohru; Kobayashi, Yoshio; Komuro, Issei
We report the case of a 51-year-old woman who underwent excision of a left postero-inferior, para-vermian cerebellar hemangioblastoma and foramen magnum decompression for an associated acquired Chiari I malformation. Two weeks postoperatively she developed a pseudomeningocele, bilateral supra- and infratentorial subdural hygromata, and a clinical disorder compatible with the cerebellar cognitive affective syndrome. There was no response to drainage of the left supratentorial collection. CSF aspiration from a pseudomeningocele lead to resolution of her syndrome and the subdural effusions. The relationships between the subdural hygromas, pseudomeningocele, location of the lesion within the cerebellum, cerebellar oedema, and the cause of her cognitive-affective syndrome are discussed. PMID:18224531
Stavrinos, N G; Taylor, R; Rowe, A; Whittle, I R
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
In reviewing 750 cases of acute colonic pseudo-obstruction from the literature, the most commonly associated disorders are listed and the therapeutical management is critically discussed. The authors describe an additional case of a 77-year-old male with ogilvie's syndrome following isolated thoracic trauma. Ogilvie's syndrome has been used synonymously with acute colonic pseudo-obstruction (ACPO) of the colon, first defined by Sir Heneage Ogilvie in 1948. If inappropriately managed, the massive colonic dilatation may lead to caecal ischaemia and perforation with a high mortality rate. Aetiology and pathogenesis of the syndrome are still unknown, but mostly ACPO is associated with a wide variety of medical conditions. Often it appears to be a complication after abdominal or pelvic surgery or trauma. In few cases the pseudo-obstruction is isolated and therefore truly idiopathic. Conservative treatment is indicated if the caecum is less than 12 cm in diameter. If there is a progressive increase in diameter or no improvement is seen, the colon should be decompressed without further delay. The indications for surgery are failure of conservative treatment and colonoscopy, signs for ceacal ischaemia or perforation. The choice of procedure, coecostomy or resection, is dictated by the state of the caecum. Due to a high mortality rate (up to 50%) if the caecum is perforated, an aggressive therapeutical management should be applied. PMID:10967947
Meier, C; Di Lazzaro, M; Decurtins, M
We report here a sudden death case of a patient previously diagnosed as Marfan syndrome (MFS). The victim was dead on the wheel and the cause of death was diagnosed to be a rupture of the thoracic aorta by autopsy findings. MFS is an autosomal dominant disorder of the connective tissue and can be a cause of sudden death. Postmortem
Motonori Takahashi; Takako Sato; Minori Nishiguchi; Koichi Suzuki; Hajime Nishio
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 35-year-old-man presented with nephrotic syndrome due to mesangiocapillary glomerulonephritis; he later developed a potassium-losing state, generalized amino aciduria and glycosuria. Clinical and biochemical improvement occurred after steroid therapy. The possible pathophysiological mechanisms are discussed. Images Fig. 1 Fig. 4
Luder, A. S.; Cohen, S. L.; Fisher, C.
We observed a 63-year old male with cardiac amyloidosis who presented with the clinical symptoms of sick sinus syndrome and dyspnea and abnormal thickening of the right atrial wall, which extended to the junction of the superior vena cava. This may explain the relationship of abnormal thickening of the right atrium which extends to the junction of the superior vena
Hiroya Narumi; Nobusada Funabashi; Hiroyuki Takano; Tai Sekine; Marehiko Ueda; Yasuhiko Hori; Taisuke Fukawa; Tohru Minamino; Yoshio Kobayashi; Issei Komuro
Chronic myeloproliferative disorders (CMD) and Myelodisplastic Syndromes (MDS) represents a group of clonal pluripotent stem-cell pathologies. During their natural history, the clinical picture reveals both thrombosis and hemorrhage. The thrombosis could affect the microvessels, and also the large vessels, including even less usual territories (suprahepatic veins, porta vein, pulmonary vein). There are many factors contributing to thrombosis in myeloproliferative chronic disorders--the associated comorbidities, the numeric alterations of blood elements and also the disorders of the platelet's function. Thus, there were described quantitative and qualitative anomalies of platelet's receptors: GP Ib, GP IIb/IIIa, GP IV, GP VI, thrombopoietin receptor of the platelet cMPL, the increase of platelet activation; the increase of P selectin and thrombospondin and the increase on GP IIb/IIIa expression--they were all correlated with thrombosis. An important role has been attributed to JAK2 mutation, which affects the platelet receptor for thrombopoietin cMPL. Regarding the hemorrhage in chronic myeloproliferative syndrome, it is favored by many disorders in platelet's function, such as: the decrease of von Willebrand factor's receptor of the platelet, which leads to acquired Bernard Soulier syndrome; quantitative and qualitative disorders of dense granules of the platelet, decrease of the secretion and platelet aggregation after epinephrine, ADP and collagen stimulation. It was also described the acquired von Willebrand syndrome, most frequently type 2. PMID:21688554
Vl?d?reanu, Ana-Maria; Popov, Viola; Bumbea, H; Ciufu, Cristina; Vasilache, Veronica; Petre, Anca; Onisâi, Minodora
BackgroundThe term secondary abdominal compartment syndrome (ACS) has been applied to describe trauma patients who develop ACS but do not have abdominal injuries. The purpose of this study was to describe major trauma victims who developed secondary ACS during standardized shock resuscitation.
Zsolt Balogh; Bruce A McKinley; Christine S Cocanour; Rosemary A Kozar; John B Holcomb; Drue N Ware; Frederick A Moore
We investigated the prognosis of epilepsy in 54 patients with severe motor and intellectual disabilities syndrome (SMIDS). The prevalence of epilepsy was 75.7% in our institution for SMIDS. We assessed activities of daily living (ADL) of them at the onset of epilepsy and at present, according to the scores in locomotion, language, toileting, dressing and feeding. Among them, patients with
Mana Kurihara; Komei Kumagai; Yoko Noda; Magoe Watanabe; Masayuki Imai
Systemic lupus erythematosus affects mostly young women. Despite this, the interrelationship between SLE and pregnancy has remained poorly understood for years. Fear of maternal or fetal complications has often resulted in advice against pregnancy. Since the mid-seventies, an increasing number of authors are providing specific care for pregnant women with SLE. Their experience over the years has demonstrated that pregnancy
Guillermo Ruiz-Irastorza; Munther A. Khamashta; Graham R. V. Hughes
Internal jugular vein thrombosis occurs as an uncommon complication of oropharyngitis. The following case report describes a previously healthy adult woman who presented with sore throat, left ear pain, and fever. She was initially diagnosed with pharyngitis and inadvertently had blood cultures sent as part of her workup. She was then called back to the Emergency Department the following day
Sjögren’s syndrome (SS) is an autoimmune disorder, the principal ocular manifestation of which is decreased tear production\\u000a leading to chronic irritation and damage to the corneal and conjunctival epithelium. The most important advance in the treatment\\u000a of ocular manifestations of SS is the introduction of topical anti-inflammatory agents such as cyclosporine A, which increases\\u000a tear production and decreases symptoms without
Michael Samarkos; Haralampos M. Moutsopoulos
Zika fever, considered as an emerging disease of arboviral origin, because of its expanding geographic area, is known as a benign infection usually presenting as an influenza-like illness with cutaneous rash. So far, Zika virus infection has never led to hospitalisation. We describe the first case of Guillain-Barré syndrome (GBS) occurring immediately after a Zika virus infection, during the current Zika and type 1 and 3 dengue fever co-epidemics in French Polynesia. PMID:24626205
Oehler, E; Watrin, L; Larre, P; Leparc-Goffart, I; Lastere, S; Valour, F; Baudouin, L; Mallet, Hp; Musso, D; Ghawche, F
Prophylactic aortic root replacement is the main factor explaining the increase in median probability of survival in Marfan syndrome (MFS) patients. In women with MFS, earlier root replacement has been suggested as a strategy to facilitate safe pregnancy. However, in this case report a pregnant patient with MFS who sustained a Type B aortic dissection in her second trimester, with fatal outcome, despite having had a prophylactic aortic root replacement five months prior to conception is described. PMID:17175042
McDermott, Colleen D; Sermer, Mathew; Siu, Samuel C; David, Tirone E; Colman, Jack M
We present the case of a 45-year old female operated on for minimally invasive closure of patent foramen ovale, who suffered in the postoperative course of the Budd–Chiari syndrome caused by the thrombotic occlusion of the inferior vena cava. Medical treatment with oral anticoagulants and heparin was promptly established, avoiding a further increase of the thrombus that completely disappeared 3 months later.
Ceresa, Fabrizio; Sansone, Fabrizio; Anfuso, Carmelo; Patane, Francesco
Takotsubo syndrome (TTS)/cardiomyopathy is a syndrome that mimics acute myocardial infarction in the absence of coronary artery disease and is characterized by acute onset of chest pain, electrocardiographic abnormalities, and reversible left ventricular dysfunction. It is usually induced by emotional and physical stress. Fluorouracil is one of the most frequently used chemotherapy agents and a relatively common adverse reaction of fluorouracil is cardiotoxicity. Herein we describe a patient without a history of cardiovascular disorder who developed severe heart failure during infusion of fluorouracil for metastatic gastric cancer. Remarkably, the patient did not develop TTS during prior chemotherapy regimen, which also included fluorouracil. The patient's findings were consistent with the proposed TTS diagnostic criteria and coronary angiography was normal, without obstructive coronary artery disease. With supportive care, the patient's cardiac functions returned to normal. TTS is not a well known syndrome to clinicians and this condition appears to occur more frequently than previously thought. In addition to the presented case, a review of the clinical features and outcome of 10 reported cases of fluorouracil-induced TTS is presented. PMID:23249567
Ozturk, Mehmet A; Ozveren, Olcay; Cinar, Veysel; Erdik, Baran; Oyan, Basak
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
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
The authors describe a case of a patient with celiac disease in whom a secondary bacterial overgrowth had developed in the small intestine. Vitamin B12 and folic acid consumption deficiency resulted in a development of macrocytary anaemia and non-immune haemolysis. Subsequently, within a few weeks, a significant pulmonary hypertension has developed, the cause of which was first unclear. Haemolysis as well as pulmonary hypertension ceased following bacterial overgrowth treatment and B12 and, later on, folic acid substitution. Retrospectively, the authors infer that this could have been the haemolytic anaemia-associated pulmonary hypertension syndrome. PMID:20681463
Kojecký, V; Lukastíková, M
We here report two cases of spontaneous, familial, recurrent ovarian hyperstimulation syndrome ended in a successful pregnancy. First case was a 26-year-old woman, gravida 3 para 0 and two previous terminations of pregnancy due to spontaneous ovarian hyperstimulation syndrome (OHSS). During her pregnancy, patient was treated with IV fluid therapy, albumin and thromboembolic prophylaxis and required pleural and peritoneal drainage. She was referred to the hospital twice, at 8 and 28 weeks'. At 32 weeks due to worsening of clinical condition, decision was made for a caesarean section. A live, healthy preterm baby of 1950?g was delivered. Second case was a 27-year-old woman, gravida 2 para 0 with one previous termination of pregnancy due to spontaneous OHSS. During her pregnancy patient was treated with IV fluid therapy, albumin, and thromboembolic prophylaxis. She did not require any pleural or peritoneal drainage. She was referred to the hospital twice, at 8 and 30 weeks. At 37 weeks of gestation, due to worsening of clinical condition decision for a caesarean section was made. A live, healthy term baby of 2700?g was delivered. Our experience seems to confirm that management of spontaneous OHSS during pregnancy should be conservative and treatment tailored to severity of symptoms. PMID:23952048
Di Carlo, Costantino; Savoia, Fabiana; Gargano, Virginia; Sparice, Stefania; Bifulco, Giuseppe; Nappi, Carmine
An 81-year-old woman was admitted to our hospital for hyponatremia and impaired consciousness after unsuccessful antibiotic treatment for pneumonia-like symptoms by a previous doctor. A chest X-ray film revealed unilateral infiltration. Mycobacterium tuberculosis was detected on a sputum smear and pulmonary tuberculosis was diagnosed. Based on the diagnostic criteria, we believed that her hyponatremia a consequence of syndrome of inappropriate antidiuretic hormone secretion (SIADH) as a complication of pulmonary tuberculosis. Sodium loading and water restriction quickly improved her serum sodium level and impaired consciousness. Anti-tuberculosis therapy reduced the abnormal shadows noted on chest X-ray films, and the sputum smear became negative for Mycobacterium tuberculosis. Her serum sodium level remained normal after the discontinuation of sodium loading. Previous reports have associated SIADH with severe types of tuberculosis such as miliary tuberculosis, tuberculosis meningitis, and pulmonary tuberculosis with massive bacterial excretion. However, this complication can also occur in mild tuberculosis, as in this case, thus SIADH should also be considered in mild cases of tuberculosis. PMID:21894778
Minami, Takahiro; Wakamatsu, Kentarou; Kumazoe, Hiroyuki; Nagata, Nobuhiko; Kajiki, Akira; Kitahara, Yoshinari
Complicated malaria is mainly caused by Plasmodium falciparum, but, increasingly, Plasmodium vivax is also being reported as a cause. Since the reemergence of indigenous vivax malaria in 1993, cases of severe malaria have been steadily reported in Korea. Herein, we report a case of vivax malaria complicated by adult respiratory distress syndrome (ARDS) that was successfully managed with extracorporeal membrane oxygenation (ECMO). A 59-year-old man presented at our hospital with fever and abdominal pain, which had persisted for 10 days. On admission, the patient had impaired consciousness, shock, hypoxia and haziness in both lungs, jaundice, thrombocytopenia and disseminated intravascular coagulation, metabolic acidosis, and acute kidney injury. A peripheral blood smear and a rapid diagnostic test verified P. vivax mono-infection. Ten hours after admission, hypoxia became more severe, despite providing maximal ventilatory support. The administration of antimalarial agents, ECMO, and continuous venovenous hemofiltration resulted in an improvement of his vital signs and laboratory findings. He was discharged from the hospital 7 weeks later, without any sequelae.
Lee, Hyun-Jung; Baek, Ji-Hyeon; Chae, Myoung-Hun; Joo, Hoyeon; Chung, Moon-Hyun; Park, Yun-Kyu; Kim, Joung-Teak
Prevalence of metabolic syndrome (MetS) in type 2 diabetes and its association with vascular complications were studied in 637 Japanese type 2 diabetic patients. MetS was diagnosed using criteria proposed by the Japanese study group for the definition of MetS in 2005. The prevalence of MetS in patients studied was higher in males (45.9%) than females (28.0%). The prevalence of MetS was 53.0% in males and 35.4% in females in patients with duration of less than 10 years, and decreased with an increase in duration. Upon comparing patients groups complicated with and without MetS, we determined the MetS group had significantly higher levels of fasting serum C-peptide and high-sensitivity C-reactive protein, and a significantly lower level of serum adiponectin. However, the prevalence of coronary heart disease, brain infarction, or peripheral arterial disease was not significantly different between these groups. On the other hand, the prevalence of microangiopathy in the group with MetS was significantly higher than in that without MetS, and became significantly higher along with an increase in duration. This study clarifies the prevalence of MetS in Japanese type 2 diabetic patients, and suggests that MetS is associated with microangiopathy rather than macroangiopathy in Japanese type 2 diabetic patients. PMID:17933413
Shimajiri, Yoshinori; Tsunoda, Keiko; Furuta, Machi; Kadoya, Yoshiki; Yamada, Shyoichi; Nanjo, Kishio; Sanke, Tokio
Sticky platelet syndrome (SPS) leads to hyperaggregabilty of platelets in response to physiologic stimuli. In this report we describe three patients with clinical symptoms of SPS after renal transplantation. The first patient developed an infarction of her transplant kidney with additional, subsequent renal microinfarctions. The second patient suffered multiple strokes and deep vein thrombosis with episodes of pulmonary embolism and ischemic bowel disease due to colonic microinfarctions. The third patient experienced a long episode of unexplained respiratory and graft dysfunction immediately after transplantation until therapy for SPS was initiated, at which point symptoms resolved quickly. Kidney transplant recipients with SPS may be at increased risk of developing thrombosis, given that most immunosuppressive drugs are known to induce either endothelial cell damage or augment platelet aggregation. All patients awaiting renal transplantation should be screened for a history of thrombosis and, if appropriate, tested for SPS. Affected patients should receive dose-adjusted acetylsalicylic acid. PMID:17532753
Mühlfeld, A S; Ketteler, M; Schwamborn, K; Eitner, F; Schneider, B; Gladziwa, U; Knüchel, R; Floege, J
Sjögren's syndrome (SS) is an autoimmune disorder, the principal ocular manifestation of which is decreased tear production leading to chronic irritation and damage to the corneal and conjunctival epithelium. The most important advance in the treatment of ocular manifestations of SS is the introduction of topical anti-inflammatory agents such as cyclosporine A, which increases tear production and decreases symptoms without any significant side effect. Stimulators of tear secretion, both topical, such as diquafosol, and systemic, such as pilocarpine and cevimeline, are also effective, although they have been associated with frequent side effects. Topical use of autologous serum is another new and effective form of treatment, but problems in the preparations prevent their widespread use. Additionally, nonpharmacologic treatments, such as insertion of punctal plugs, are beneficial in the dry eye of SS, whereas several other modalities, such as anti-CD4 monoclonal antibody eye drops and gene transfer, are still in experimental phases. PMID:15967078
Samarkos, Michael; Moutsopoulos, Haralampos M
Peutz-Jeghers syndrome is an autosomal dominant inherited disorder characterized by intestinal hamartomatous polyps in association with mucocutaneous pigmentations. Here we present a case of a 30-year-old woman who was hospitalized and underwent diagnostic procedures because of crampy abdominal pain. Physical examination on admission revealed pigmented spots around lips and on the oral mucosa. Multiple polyps were found in stomach, small and large intestine, with signs of initial ileo-ileal intussusception. After endoscopic removal of achievable polyps, we applied gastroscope through laparotomy and enterotomy and removed total number of 34 polyps from small bowel. The polyps were found to be mostly hamartomatous at histological examination. This procedure can provide removal of the most polyps, which are potentially premalignant, also with less complicationes than after multiple intestinal resectiones. PMID:23697287
Perko, Zdravko; Krni?, Dragan; Pogoreli?, Zenon; Druzijani?, Nikica; Simuni?, Miroslav; Bilan, Kanito; Kraljevi?, Damir; Simuni?, Marina Maras
Background Patients with the Shwachman-Diamond syndrome often develop hematologic complications. No risk factors for these complications have so far been identified. The aim of this study was to classify the hematologic complications occurring in patients with Shwachman-Diamond syndrome and to investigate the risk factors for these complications. Design and Methods One hundred and two patients with Shwachman-Diamond syndrome, with a median follow-up of 11.6 years, were studied. Major hematologic complications were considered in the case of definitive severe cytopenia (i.e. anemia <7 g/dL or thrombocytopenia <20×109/L), classified as malignant (myelodysplasia/leukemia) according to the 2008 World Health Organization classification or as non-malignant. Results Severe cytopenia was observed in 21 patients and classified as malignant severe cytopenia (n=9), non-malignant severe cytopenia (n=9) and malignant severe cytopenia preceded by non-malignant severe cytopenia (n=3). The 20-year cumulative risk of severe cytopenia was 24.3% (95% confidence interval: 15.3%–38.5%). Young age at first symptoms (<3 months) and low hematologic parameters both at diagnosis of the disease and during the follow-up were associated with severe hematologic complications (P<0.001). Fifteen novel SBDS mutations were identified. Genotype analysis showed no discernible prognostic value. Conclusions Patients with Shwachman-Diamond syndrome with very early symptoms or cytopenia at diagnosis (even mild anemia or thrombocytopenia) should be considered at a high risk of severe hematologic complications, malignant or non-malignant. Transient severe cytopenia or an indolent cytogenetic clone had no deleterious value.
Donadieu, Jean; Fenneteau, Odile; Beaupain, Blandine; Beaufils, Sandrine; Bellanger, Florence; Mahlaoui, Nizar; Lambilliotte, Anne; Aladjidi, Nathalie; Bertrand, Yves; Mialou, Valerie; Perot, Christine; Michel, Gerard; Fouyssac, Fanny; Paillard, Catherine; Gandemer, Virginie; Boutard, Patrick; Schmitz, Jacques; Morali, Alain; Leblanc, Thierry; Bellanne-Chantelot, Christine
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
A 69-year-old man developed stromal edema and a pocket of fluid in the laser in situ keratomileusis (LASIK) interface wound in the left eye after acute endothelial cell loss from complicated trabeculectomy. He eventually required penetrating keratoplasty along with cataract surgery. Histologic examination of the corneal button showed an edematous 720 microm central residual stromal bed, a 54 microm empty space at the level of the central interface wound, and a 154 microm LASIK flap. The endothelial cell count was 0 to 2 cells per high-power field, corresponding to a cell density of 450 to 500 cells/mm(2). Four years after LASIK, the central interface wound was susceptible to forming a pocket of serous fluid after the corneal endothelial function was compromised. PMID:16931273
Kang, Shin J; Dawson, Daniel G; Hopp, Lawrence M; Schmack, Ingo; Grossniklaus, Hans E; Edelhauser, Henry F
Hemophagocytic syndrome (HPS) arises secondary to genetic, rheumatologic, neoplastic, and infectious causes. We discuss a patient whose presentation was consistent with systemic infection but was discovered to have HPS of unknown etiology. The presenting symptoms, as well as unremarkable malignancy and rheumatologic workups, led to the pursuit of an infectious cause, but the patient was ultimately discovered to have an occult anaplastic large-cell lymphoma (ALCL). This case demonstrates the diagnostic challenges that result from infectious mimicry in the context of HPS—first, in distinguishing noninfectious HPS from the systemic inflammation that can result from a widespread infectious process, second, in the identification of the precipitating cause of HPS. While evidence of these challenges has been suggested by the limited literature on HPS and ALCL, our case illustrates the diagnostic dilemma that arises when tissue biopsy does not quickly reveal an etiology. It is important that all physicians be aware that HPS can mimic infection and be prepared to redirect the workup when an infectious etiology for HPS cannot be identified.
Peluso, Michael J.; Chia, David; Sheen, Whitney; Hutchinson, Christoph; Barakat, Lydia
We describe 5 children from 2 families with mutations in the CD40 ligand (CD40L) gene leading to absent expression of CD40L on activated CD4 cells. All subjects presented with interstitial pneumonia with low serum IgG and normal serum IgM. One child had normal and one child had elevated serum IgA. Four had confirmed Pneumocystis carinii pneumonia. In spite of intravenous immunoglobulin treatment yielding therapeutic serum immunoglobulin levels, 3 children had enteroviral encephalitis. When assessed by flow cytometry, the 3 surviving affected male children had absent CD40L expression on activated CD4(+) T cells. The affected children from both families were shown to have the same single nucleotide insertion (codon 131) resulting in frameshift and early termination within exon 4 (extracellular domain). This observation demonstrates that persistent enteroviral infection is not only observed in X-linked agammaglobulinemia but may also occur in patients with X-linked hyper IgM syndrome. PMID:10228294
Cunningham, C K; Bonville, C A; Ochs, H D; Seyama, K; John, P A; Rotbart, H A; Weiner, L B
Abdominal Compartment Syndrome (ACS) is characterized by intra-abdominal hypertension (IAH), elevation and splinting of the diaphragm, high pleural pressure, and poor venous return to the heart, producing low cardiac output and shock which, in turn, results in poor venous return across the capillaries to set in a vicious cycle. Unless the Intra-abdominal pressure is reduced quickly by urgent surgical or medical interventions, death is inevitable. We report a case of ACS resulting from an unrecognized slow but massive intra-abdominal bleeding caused by a ruptured ectopic pregnancy (REP) in an Arab woman. Due to the unusual nature of the presentation of the REP, the diagnosis proved elusive for over a week until the patient succumbed to hypovolemic shock after losing about 4.2 l inside the peritoneal space. The fruitless effort at aggressive fluid resuscitation was at operation found not due to hypovolemia per se but due to IAH causing ACS. The lessons learned from this case emphasize the need for awareness about atypical presentations of REP and the need for quick intervention to terminate the vicious cycle of ACS. PMID:21912014
Sanda, Robert B; Aziz, Rafeeqa; Bhutto, Abdulrazaque; Seliem, Salah I
An 88-year-old male patient with macroglobulinemia was admitted to our hospital because of severe hyponatremia and unconsciousness. Laboratory findings showed decreased inhibition of antidiuretic hormone (ADH) and he was diagnosed with syndrome of inappropriate secretion of ADH (SIADH). Hyponatremia improved with only limitation of water intake and the patient was followed up on a continuing outpatient basis. However, soon after discharge from hospital, his legs started swelling with edema and hyponatremia worsened. He was re-admitted due to a fall at home. Hyponatremia was observed at re-admission. A CRH challenge test showed partial dysfunction of ACTH secretion. Corticosteroid therapy was performed, but the patient subsequently died from pneumonia. Pathological findings at autopsy revealed invasion of plasma cells and amyloid depositions in multiple organs, including the pituitary, adrenal cortex, heart, liver, kidney, lymph nodes and bone marrow. Consistent with these results, fibrosis was observed in the anterior lobe of the pituitary, suggesting that the autopsy findings were related to the clinical observations and diagnosis. This is the first reported case of macroglobulinemia complicated with multiple hormone dysfunction. PMID:24521938
Yamada, Chika; Yoneda, Chihiro; Ogino, Jun; Fukushima, Sayaka; Kodama, Shoko; Asano, Chihiro; Masuda, Michihiko; Horie-Tajima, Kanako; Toyonaga, Aiko; Hiroshima, Kenzo; Kawamura, Shunji; Hashimoto, Naotake
\\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
The patient was a 70-year-old man who had been given a diagnosis of remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome and had been placed on low-dose steroid therapy in the Department of Orthopedics. During treatment, sudden fever, hypoxemia and chest radiography-confirmed interstitial shadows throughout the lung fields were noted, and the patient was referred to the Department of Internal Medicine. RS3PE complicated by interstitial pneumonia was diagnosed, and steroid pulse therapy and immunosuppressant therapy were initiated. In the present case, soluble interleukin-2 receptor (sIL-2R) proved useful for assessing symptoms. To the best of our knowledge, RS3PE syndrome complicated by pulmonary lesions and accompanied by severe acute respiratory failure requiring noninvasive positive-pressure ventilation has not previously been reported, and this rare case is discussed with reference to the literature. PMID:19637808
Okuda, Miyuki; Kashio, Makoto; Aitani, Masakazu; Nakanishi, Daisuke; Tanaka, Nobuya; Kimura, Kentaro
We described a patient with secondary Sjögren's syndrome suspected showing multiple cranial neuropathies at the different time. A 68-year old woman has been treated as rheumatoid arthritis since 1963. In 2001, she was also diagnosed with Sjögren's syndrome suspected. Afterward, abducent and trigeminal neuropathies and uveites occurred. This case is important to insight into the cranial neuropathy with Sjögren's syndrome. PMID:23124085
Nanke, Yuki; Kobashigawa, Tsuyoshi; Yago, Toru; Yamanaka, Hisashi; Kotake, Shigeru
Primary bacterial peritonitis is a rare complication of idiopathic nephrotic syndrome (INS) in children, found in 1.5-3.7% cases. The 10-year-old girl was admitted with INS relapse: generalized edema, proteinuria 630 mg/kg/24 h, hypoalbuminemia 1.8 g/dL, hypogammaglobulinemia 74.0 mg/dL (n: 618-1537 mg/dL), GFR 71.6 mL/min/1.73 m2. She was treated with prednisone 60 mg/24 h. On 5th day severe pain, fever, CRP (15.5 mg/dL) and leukocytosis (19.5 tys/mm3) rise occurred. On 6th day due to suspicion of peritonitis, laparotomy was performed and 400 mL of suppurative exudate was evacuated (Streptococcus pneumoniae was cultured). Postoperative course was complicated with acute kidney injury (GFR 47.7 mL/min/ 1.73 m2), lung edema, arterial hypertension, and separation of the layers of a surgical wound. The patient was treated with: imipenem (9 days), vancomycine i.v. (4 days)/p.o. (11 days) (Clostridium difficile toxin present in stool), fluconazole (14 days), 20% albumins, furosemide, labetalole, cyclosporine A (started on 56th day after the operation due to secondary steroid-resistance of INS). The remission was achieved after 7 days of cyclosporine A treatment. Authors suggest that children with nephrotic syndrome belong to high-risk group of invasive pneumococcal disease, therefore they require careful implementation of mandatory immunization schedule. Peritonitis is a rare and still dangerous infectious complication of nephrotic syndrome in children. PMID:24490466
Skrzypczyk, Piotr; Szymanik-Grzelak, Hanna; Pa?czyk-Tomaszewska, Ma?gorzata; Szmigielska, Agnieszka; Roszkowska-Blaim, Maria
Scrub typhus is an acute infectious illness, distributed throughout the Asia Pacific rim. In India, it has been reported from northern, eastern, and southern India. However, cases of scrub typhus have not been well-documented from Vidarbha, an eastern region of Maharashtra state in central India. We report two cases of complicated scrub typhus from Vidarbha region. These cases admitted in unconscious state with 8-10 days history of fever, body ache, cough, and progressive breathlessness. The diagnosis in both cases was based on presence of eschar, a positive Weil-Felix test, and a positive rapid diagnostic test (immunochromatographic assay). Both cases were complicated by acute respiratory distress syndrome (ARDS) and multiorgan failure. Both of them presented in their 2nd week of illness and died during the hospital course in spite of intensive supportive care. The main cause of mortality was delayed referral leading to delay in diagnosis and treatment.
Saxena, Amrish; Khiangte, Benjamine; Tiewsoh, Iadarilang
Until recently, loosening, infection and fractures of the prosthesis have been considered the only major complications of otherwise good initial results with total endoprosthetic replacement of the knee joint; however, another serious long term complication has become apparent. Severe degenerative changes of the retropatellar cartilage are being observed, which seem to be caused by a possible axial malalignment of the knee, progression of a preexisting retropatellar arthrosis, and additional metallosis induced by the prosthesis. The resulting symptoms and complaints, in accordance with Debeyre, are summarized by the term Patellar Syndrome. Our studies indicate that these symptoms develop in 50% of all patients with a total knee endoprosthesis within three years after surgery. In 10% of the cases these symptoms are severe enough to require additional surgical intervention. PMID:24823189
Kohler, G; Richter, R; Coldewey, J F
We report the successful treatment of two patients with aneurismal subarachnoid hemorrhage complicated by severe respiratory failure and refractory septic shock using simultaneous prone position ventilation and high-volume hemofiltration. These rescue therapies allowed the patients to overcome the critical situation without associated complications and with no detrimental effects on the intracranial and cerebral perfusion pressures. Prone position ventilation is now an accepted therapy for severe acute respiratory distress syndrome, and high-volume hemofiltration is a non-conventional hemodynamic support that has several potential mechanisms for improving septic shock. In this manuscript, we briefly review these therapies and the related evidence. When other conventional treatments are insufficient for providing safe limits of oxygenation and perfusion as part of basic neuroprotective care in subarachnoid hemorrhage patients, these rescue therapies should be considered on a case-by-case basis by an experienced critical care team.
Cornejo, Rodrigo; Romero, Carlos; Ugalde, Diego; Bustos, Patricio; Diaz, Gonzalo; Galvez, Ricardo; Llanos, Osvaldo; Tobar, Eduardo
Recently, we experienced a rare case of Budd-Chiari Syndrome. The case was a 57-year-old female patient. Venous return had been severely disturbed by the membranous occlusion of the IVC and a giant floating thrombus. After catheter directed thrombolysis combined with stepwise percutaneous angioplasty, the IVC was recanalized sufficiently and the thrombus was completely resolved.
INTRODUCTION Median arcuate ligament (MAL) malposition is a rare cause of celiac axis compression syndrome (CACS) or Dunbar syndrome. PRESENTATION OF CASE A 26-year-old female presented with severe postprandial epigastric pain, weight loss, heartburn and regurgitation unresponsive to medical therapy. CT angiography and duplex ultrasound demonstrated the MAL crossing anterior to the celiac artery (CA). Reconstructions demonstrated CA compression, while the superior mesenteric artery (SMA) was normal. The MAL was laparoscopically divided, releasing the celiac axis. A concomitant Nissen fundoplication was performed. At 3-months follow-up, the CT-scan demonstrated no evidence of CACS with complete symptom resolution. DISCUSSION Dunbar's syndrome can be treated with endovascular surgery, laparoscopic MAL division or vascular surgery.Six anatomical and morphologic variations of aortic and esophageal hiatus are described. The result of the analysis of these anatomical data leads to the conclusion that hiatus hernia, Dunbar's syndrome and GERD have a common etiopathogenesis and physiopathology. CONCLUSION Laparoscopic treatment is useful and feasible in centers with experience in majorlaparoscopic surgery with reduced invasiveness, better cosmetic effect and shorter postoperative course.
di Libero, Lorenzo; Varricchio, Antonio; Tartaglia, Ernesto; Iazzetta, Igino; Tartaglia, Alberto; Bernardo, Antonella; Bernardo, Rosanna; Triscino, Giovangiuseppe; Conte, Domenico Lo
Recently, we experienced a rare case of Budd-Chiari Syndrome. The case was a 57-year-old female patient. Venous return had been severely disturbed by the membranous occlusion of the IVC and a giant floating thrombus. After catheter directed thrombolysis combined with stepwise percutaneous angioplasty, the IVC was recanalized sufficiently and the thrombus was completely resolved. PMID:24995066
Mitsuoka, Hiroshi; Saito, Takaaki; Higashi, Shigeki
Background Compartment syndrome of any extremity is a limb-threatening emergency requiring an emergent surgical management. Thus, ruling out compartment syndrome is often high on the list of priorities when treating high-energy injuries and fractures. However, even in the most seemingly benign injuries, this dangerous diagnosis must always remain on the differential and suspicion must remain high. Case presentation 23-year-old factory worker presents after a low energy entrapment injury to his left forearm. Initial work-up and evaluation noted an isolated radial head dislocation with a normal physical motor and sensory exam. However, maintaining high suspicion for compartment syndrome despite serial normal physical exams, led objective compartment pressure measurement leading to definitive diagnosis. Emergent surgical intervention via compartment fasciotomies was performed, along with closed reduction and ligament repair. At 1 year follow-up, the patient was well-healed, back to work with full range of motion and not activity limitations. Conclusion Despite a seemingly benign injury pattern, and a relatively low energy mechanism, vigilant concern for compartment syndrome following any kind of entrapment injury should initiate serial examinations and compartment pressure measurements especially in circumstances with continued swelling and inability to perform an accurate clinical assessment due to an obtunded or medicated patient.
Bilateral adrenal hemorrhage can complicate severe sepsis in the neonate and is most commonly attributed to meningococcal disease; however, it can be caused by other etiologic agents as well. We report herein a fatal case of Enterobacter cloacae sepsis in a preterm infant, resulting in massive adrenal hemorrhages. This is the first documented case of adrenal hemorrhage following infection with this pathogen. PMID:24328998
Pode-Shakked, Ben; Sadeh-Vered, Tal; Kidron, Dvora; Kuint, Jacob; Strauss, Tzipora; Leibovitch, Leah
We investigated the effects of individual (IC) and group (GC) diet and exercise counseling in men with metabolic syndrome. Participants received exercise instruction and exercise load was monitored. IC participants received individual diet counseling sessions and general consultations at baseline and monthly. GC participants received a group diet counseling session at baseline and general consultations at baseline and monthly. In the IC group, body mass index (BMI) percent body fat, waist circumference, diastolic blood pressure, low-density lipoprotein cholesterol, glycosylated hemoglobin A1c, and liver function levels were reduced significantly after 3 months, whereas in the GC group, waist circumference and levels of liver function were reduced. Exercise load was negatively correlated with change in BMI and waist circumference in the IC group, and positively correlated with changes in high-density lipoprotein cholesterol levels in all subjects and in the GC group. Diet and exercise counseling, especially IC, may benefit patients with metabolic syndrome.
Morita-Suzuki, S.; Fujioka, Y.; Mitsuoka, H.; Tashiro, M.; Harada, M.
The identification of Lynch syndrome has been greatly assisted by the advent of tumour immunohistochemistry (IHC) for mismatch\\u000a repair (MMR) proteins, and by the recognition of the role of acquired somatic BRAF mutation in sporadic MMR-deficient colorectal cancer (CRC). However, somatic BRAF mutation may also be present in the tumours in families with a predisposition to develop serrated polyps in
Michael D. Walsh; Daniel D. Buchanan; Rhiannon Walters; Aedan Roberts; Sven Arnold; Diane McKeone; Mark Clendenning; Andrew R. Ruszkiewicz; Mark A. Jenkins; John L. Hopper; Jack Goldblatt; Jillian George; Graeme K. Suthers; Kerry Phillips; Graeme P. Young; Finlay Macrae; Musa Drini; Michael O. Woods; Susan Parry; Jeremy R. Jass; Joanne P. Young
Pseudo-Meigs' syndrome accompanied by massive ascites in uterine leiomyoma is rare. We encountered a rare case of a 37-year-old, nulliparous woman with a lower abdominal tumor and severe abdominal distention due to massive ascites. Serum cancer antigen 125 and vascular endothelial growth factor levels were elevated to 1007.9?U/mL and 103?pg/mL, respectively. She was tentatively diagnosed with ovarian cancer. Emergency concentrated ascites re-infusion therapy was performed to improve dyspnea, abdominal pain, and her preoperative respiratory condition. Concentrated ascites re-infusion therapy eliminated dyspnea and abdominal discomfort without decreasing serum albumin levels. The patient underwent laparotomy, which revealed a fist-sized pedunculated uterine leiomyoma arising from the right uterine fundus. Myomectomy was performed. Pseudo-Meigs' syndrome mimics advanced ovarian cancer due to massive ascites and markedly elevated serum cancer antigen 125 and vascular endothelial growth factor levels. Concentrated ascites re-infusion therapy was effective in improving the subjective symptoms of pseudo-Meigs' syndrome and the patient's preoperative condition. PMID:25056475
Yonehara, Yukie; Yanazume, Shintaro; Kamio, Masaki; Togami, Shinichi; Tasaki, Takashi; Douchi, Tsutomu
Primary angiosarcoma of the breast (PAB) accounts for 0.04% of all breast malignant tumors. It affects young women usually at third or fourth decades of life. PAB clinically manifests as a painless, movable mass with sharp limits. A bluish red discoloration of the overlying skin is often observed. Enlargement of axillary lymph nodes generally does not occur. Angiosarcoma of the breast has a very poor prognosis due to the tendency to metastasize haematogenously and high frequency of local recurrence. Mastectomy and chemotherapy are preferable treatment choices. This paper presents a case of primary angiosarcoma of the breast with a syndrome of disseminated intravascular coagulation (DIC). PMID:24936339
Alexandrova, Elena; Sergieva, Sonya; Mihaylova, Iglika; Zarkova, Antoaneta
Dermatological emergencies include a number of clinical conditions usually accompanied by systemic symptoms that can lead to life-threatening complications. From the broad spectrum of life-threatening dermatoses, three cases are presented: a case of febrile ulceronecrotic Mucha-Habermann disease (FUMHD), a case of pemphigus vulgaris mimicking Stevens-Johnson syndrome (SJS), and a case of toxic epidermal necrolysis (TEN). Those cases were considered extreme, and presented to illustrate the positive outcome of timely intensive dermatological care. An interdisciplinary approach is essential in the diagnosis, treatment, management, and follow up of patients with life-threatening dermatoses. PMID:24813841
Maria S?l?v?stru, Carmen; Severin, Elena; Moisa, Mihaela; Fritz, Klaus; Tiplica, Sorin
A 17-year-old girl with multiple areas of skin hemangiomas that had been present since birth was referred to our institution complaining of sudden onset of dyspnea. Enhanced CT demonstrated a pulmonary thromboembolism and transthoracic echocardiogram showed a thrombus-like echo in the right ventricle. CT further revealed thrombi in the inferior vena cava (IVC) and peripheral vein. The thrombi, especially those in the RV, were highly life-threatening; therefore, immediate thrombectomy was performed and an IVC filter was placed. Because no major complications occurred, the patient was discharged 34 days after admission. In such young women, carefully using anticoagulation therapy and planning pregnancy are recommended. PMID:23774543
Yamada, Toshimasa; Ohba, Takayoshi; Yamamoto, Teppei; Kimata, Nakahisa; Inami, Toru; Munakata, Ryo; Murakami, Daisuke; Maruyama, Mitsunori; Takano, Masamichi; Ibuki, Chikao; Hata, Noritake; Seino, Yoshihiko; Mizuno, Kyoichi
We report here a 47-year-old male with the diagnosis of high-grade B-cell lymphoma and hemophagocytosis accompanying disseminated intravascular coagulation (DIC). Lymphoma-associated hemophagocytic syndrome (LAHS) is a life-threatening disorder, and LAHS secondary to B-cell lymphoma is relatively rare compared to that secondary to T- or NK/T-cell lymphoma in Western countries. T- or NK/T-cell LAHS is sometimes combined with DIC, which makes patients' outcomes even worse, but few reports of B-cell LAHS accompanying DIC has been published so far. We successfully treated a patient with this condition with recombinant thrombomodulin (rTM), a novel agent for DIC. We believe that rTM is a therapeutic option in cases with B-cell LAHS accompanying DIC. PMID:23696942
Uni, Masahiro; Yoshimi, Akihide; Maki, Hiroaki; Maeda, Daichi; Nakazaki, Kumi; Nakamura, Fumihiko; Fukayama, Masashi; Kurokawa, Mineo
We report here a 47-year-old male with the diagnosis of high-grade B-cell lymphoma and hemophagocytosis accompanying disseminated intravascular coagulation (DIC). Lymphoma-associated hemophagocytic syndrome (LAHS) is a life-threatening disorder, and LAHS secondary to B-cell lymphoma is relatively rare compared to that secondary to T- or NK/T-cell lymphoma in Western countries. T- or NK/T-cell LAHS is sometimes combined with DIC, which makes patients’ outcomes even worse, but few reports of B-cell LAHS accompanying DIC has been published so far. We successfully treated a patient with this condition with recombinant thrombomodulin (rTM), a novel agent for DIC. We believe that rTM is a therapeutic option in cases with B-cell LAHS accompanying DIC.
Uni, Masahiro; Yoshimi, Akihide; Maki, Hiroaki; Maeda, Daichi; Nakazaki, Kumi; Nakamura, Fumihiko; Fukayama, Masashi; Kurokawa, Mineo
Introduction Dermatomyositis is a chronic inflammatory disorder characterized by muscular and dermatologic symptoms with variable internal organ involvement. This is the first report on a patient with acute dermatomyositis and fulminant systemic capillary leak syndrome. Case presentation A 69-year-old Caucasian woman with chronic dermatomyositis presented with clinical signs of severe hypovolemic shock and pronounced hemoconcentration (hematocrit, 69%). Her colloid osmotic pressure was 4.6mmHg. Following a bolus dose of prednisolone (500mg), fluid resuscitation was initiated. During volume loading, anasarca and acute respiratory distress rapidly developed. Echocardiography revealed an underfilled, hypokinetic, diastolic dysfunctional left ventricle with pericardial effusion but no signs of tamponade. Despite continued fluid resuscitation and high-dosed catecholamine therapy, the patient died from refractory shock 12 hours after intensive care unit admission. A laboratory analysis of her complement system suggested the presence of C1 inhibitor deficiency as the cause for systemic capillary leakage. The post-mortem examination revealed bilateral pleural, pericardial and peritoneal effusions as well as left ventricular hypertrophy with patchy myocardial fibrosis. Different patterns of endomysial/perimysial lymphocytic infiltrations adjacent to degenerated cardiomyocytes in her myocardium and necrotic muscle fibers in her right psoas major muscle were found in the histological examination. Conclusions This case report indicates that acute exacerbation of chronic dermatomyositis can result in a fulminant systemic capillary leak syndrome with intense hemoconcentration, hypovolemic shock and acute heart failure. In the presented patient, the cause for diffuse capillary leakage was most probably acquired angioedema, a condition that has been associated with both lymphoproliferative and autoimmunologic disorders.
Prevalence of Metabolic Syndrome and its influence on microvascular complications in the Indian population with Type 2 Diabetes Mellitus. Sankara Nethralaya Diabetic Retinopathy Epidemiology And Molecular Genetic Study (SN-DREAMS, report 14)
BACKGROUND: The Metabolic syndrome (MS) consists of central obesity, glucose intolerance, hyperinsulinemia, low high density lipoproteins, high triglycerides and hypertension. Different studies have observed that MS causes microvascular complications in patients with type 2 diabetes. The aim of the study was to find out the prevalence of MS in the Indian population with type 2 diabetes mellitus in relation to
Rajiv Raman; Aditi Gupta; Swakshyar S Pal; Suganeswari Ganesan; Kadri Venkatesh; Vaitheeswaran Kulothungan; Tarun Sharma
Leukemia affects both the central and peripheral nervous systems. Neurological complications are a consequence of both direct\\u000a leukemic infiltration, as occurs with leukemic meningitis, and complications of either antileukemic treatment (e.g., thrombocytopenic\\u000a or DIC-related intracranial hemorrhage, steroid myopathy, vinca alkaloid peripheral neuropathy, posterior reversible encephalopathy\\u000a syndrome, multifocal necrotizing leuko-encephalopathy) or immune compromise (e.g., Herpes zoster shingles or Aspergillus infection).
Marc C. Chamberlain
Endometrial ablation as a treatment for abnormal uterine bleeding has evolved considerably over the past several decades. Postoperative complications include the following: (1) pregnancy after endometrial ablation; (2) pain-related obstructed menses (hematometra, postablation tubal sterilization syndrome); (3) failure to control menses (repeat ablation, hysterectomy); (4) risk from preexisting conditions (endometrial neoplasia, cesarean section); and (5) infection. Physicians performing endometrial ablation should be aware of postoperative complications and be able to diagnose and provide treatment for these conditions. PMID:22541856
Sharp, Howard T
I report here a case of a posterior horn syndrome presumably due to sacral cord injury during the procedure of epidural anesthesia. A 43-year-old female underwent hysterectomy for myoma uteri. The operation was initially planned to be performed under epidural anesthesia. When a needle was inserted at the level of Th 12/L 1, she felt severe pain in the medial aspect of the left thigh. After the operation under general anesthesia, intolerable pain continued in the buttocks, the medial aspect of the left thigh, and the posterolateral aspect of the left lower leg. Lumbar MR images demonstrated slight disc hernia but no intra- and extramedullary hematomas. Seven weeks after the operation, she was referred to my clinic for neurologic evaluation. Motor functions were intact. Knee jerks on both sides were hyperactive; other tendon reflexes were normal. Plantar reflexes were indifferent. Thermal and pin-prick sensations were lost in the left S1 dermatome, and moderately impaired in the left S2 dermatome. Light touch and vibration sense showed no remarkable changes. Her sensory disturbance could have been caused by a lesion involving the left posterior horn and lateral spinothalamic tract within the S1 segment of the spinal cord. PMID:10402822
We report on a triplet pregnancy of consanguineous parents with one fetus being affected by recurrent Johanson-Blizzard syndrome (JBS). At autopsy in the 35th gestational week, the affected triplet presented with an especially severe and lethal manifestation of the disorder as compared to his elder affected brother and to cases in the literature, thus exemplifying great interfamilial and intrafamilial phenotypic variability. Arhinencephaly and cystic renal dysplasia associated with urethral obstruction sequence were features not described previously in the literature. In addition to the lack of exocrine acini as the characteristic feature of JBS, the pancreas revealed a resorptive inflammatory reaction with infiltration by eosinophilic granulocytes that focally dispersed onto islets of Langerhans, thus favoring a progressive destructive rather than primary dysplastic process and possibly explaining the occurrence of diabetes mellitus in later life. JBS maps to chromosome 15q15-q21.1 and is associated with mutations in the UBR1 gene. Testing the fetus and the affected sibling revealed a homozygous truncating mutation in UBR1. The resulting absence of the UBR1 protein was confirmed by Western blot. Immunohistochemical staining using a commercial anti-UBR1 antibody demonstrated staining, presumably artifactual. This finding suggests that, until an appropriately validated antibody has been identified, this modality should not be utilized for diagnosis or confirmation of this disorder. PMID:21711208
Schoner, Katharina; Fritz, Barbara; Huelskamp, Georg; Louwen, Frank; Zenker, Martin; Moll, Roland; Rehder, Helga
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.
Background A comparative retrospective study was performed to compare the distribution of risk factors and complications in patients with acute coronary syndrome (ACS) at high-altitude vs low-altitude areas in Yemen. Methods The records of 768 patients from Sana'a (high altitude) and Aden (low altitude) were reviewed. Risk factors assessed were age, hypertension, diabetes mellitus, hyperlipidemia, cigarette smoking, and reported history and family history of coronary artery disease (CAD). Complications of ACS of interest were heart failure, arrhythmias, cerebrovascular accident (CVA), and death. Results The mean age of ACS patients at high altitude was significantly lower than those at low altitude (55.3 years [SD = 8.2] vs 56.8 years [SD = 7.1]; P = .007). History of hyperlipidemia was significantly higher in high-altitude patients than in low-altitude patients (49.2% vs 38.3%; odds ratio [OR] = 1.563; P = .002). Reported history of CAD was also significantly higher at higher altitudes (16.7% vs 9.4%; OR = 1.933; P = .003). Previous history of diabetes mellitus and tobacco smoking was slightly higher with borderline significance. Hypertension and reported family history of CAD were comparable among high- and low-altitude patients. In terms of in-hospital complications, CVAs were significantly higher in high-altitude patients than in low-altitude patients (7.8% vs 4.4%; P = .0001). Heart failure, arrhythmias, and death rates were comparable in both groups of patients. Wall motion abnormalities were comparable, whereas the ejection fraction was lower in the high-altitude patients (49.8% [SD = 16.08] vs 54.8% [SD = 16.23]; P = .0001). Conclusions ACS occurs at a younger age at high altitudes. Patients who live in high-altitude regions are also more likely to have hyperlipidemia and a previous history of CAD. Stroke and reduced left ventricular ejection fraction (LVEF) occur more commonly in high-altitude ACS patients. High altitude may generally be a risk factor for ACS.
Al-Huthi, Mohamed Ali; Ahmed Raja'a, Yahia; Al-Noami, Mohammed; Rahman, Abdul Rashid Abdul
Design and rationale of the AngioSeal versus the Radial approach In acute coronary SyndromE (ARISE) trial: a randomized comparison of a vascular closure device versus the radial approach to prevent vascular access site complications in non-ST-segment elevation acute coronary syndrome patients
Background Arterial access is a major site of bleeding complications after invasive coronary procedures. Among strategies to decrease vascular complications, the radial approach is an established one. Vascular closure devices provide more comfort to patients and decrease hemostasis and need for bed rest. However, the inconsistency of data proving their safety limits their routine adoption as a strategy to prevent vascular complications, requiring evidence through adequately designed randomized trials. The aim of this study is to compare the radial versus femoral approach using a vascular closure device for the incidence of arterial puncture site vascular complications among non-ST-segment elevation acute coronary syndrome patients submitted to an early invasive strategy. Methods ARISE is a national, multicenter, non-inferiority randomized clinical trial. Two hundred patients with non-ST-segment elevation acute coronary syndrome will be randomized to either radial or femoral access using a vascular closure device. The primary outcome is the occurrence of vascular complications at an arterial puncture site 30 days after the procedure, including major bleeding, retroperitoneal hematoma, compartment syndrome, hematoma???5 cm, pseudoaneurysm, arterio-venous fistula, infection, limb ischemia, arterial occlusion, adjacent nerve injury or the need for vascular surgical repair. Results Enrollment was initiated in September 2012, and until October 2013 91 patients were included. The inclusion phase is expected to last until the second half of 2014. Conclusions The ARISE trial will help define the role of a vascular closure device as a bleeding avoidance strategy in patients with NSTEACS. Trial registration ClinicalTrials.gov identifier: NCT01653587
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
Recently it has been reported that low serum IL-10 levels are associated with an increased susceptibility for metabolic syndrome and type 2 diabetes mellitus (T2DM). We investigated whether the -1087G/A (rs1800896), -824C/T (rs1800871), -597C/A (rs1800872) IL-10 polymorphisms were associated with type 2 diabetes in a study on a cohort of Italian Caucasians comprising 490 type 2 diabetic and 349 control subjects. Stratifying the data according to IL-10 genotypes, trends for the progressive increase of glucose and neutrophil levels were observed in -1087GG vs. -1087GA vs. -1087AA positive diabetic patients (-1087GG<-1087GA<-1087AA). In addition, evaluating the laboratory parameters according to the -597/-824/-1087 derived haplotypes a significant increase of neutrophils was found in diabetic vs. non-diabetic -597A/ -824T/-1087A positive subjects (Student t test = 3.707, p<0.01). In an attempt to integrate clinical laboratory and immunogenetic data to determine whether these factors taken together define sufficient risk sets for type 2 diabetes we performed the grade-of-membership analysis (GoM). GoM allowed to identify a population of subjects negative for IL-10 -824T allele, 74.4% of which were diabetic patients characterised by vascular damages (Chronic kidney failure and/or Myocardial Infarction), reduction of haematocrit, increase of blood urea nitrogen, creatinin and monocyte levels. These data seem to suggest that -597A/-824T/-1087A negative subjects are more prone to the major type 2 diabetic vascular damages and allow to hypothesise that the contemporary evaluation of some simple hematochemical parameters and IL-10 SNPs may allow identifying diabetic patients with the worse prognostic profile, needing both better complication prevention planning and therapeutic strategies. PMID:20388104
Forte, G I; Pilato, G; Vaccarino, L; Sanacore, M; Candore, G; Romano, G C; Testa, R; Franceschi, C; Capri, M; Marra, M; Bonfigli, A R; Caruso, C; Scola, L; Lio, D
Thyroid storm (TS) is a life-threatening endocrine emergency. However, the pathogenesis of TS is poorly understood. A 40-year-old man was admitted to a nearby hospital with body weight loss and jaundice. Five days after a contrasted abdominal computerized tomography (CT) scan, he exhibited high fever and disturbance of consciousness. He was diagnosed with TS originating from untreated Graves' disease and was transferred to the intensive care unit (ICU) of our hospital. The patient exhibited impaired consciousness (E4V1M4 in Glasgow coma scale), high fever (39.3°C), and atrial flutter with a pulse rate 162/min, and was complicated by heart failure, acute hepatic failure, and disseminated intravascular coagulation syndrome (DIC). His circulating level of soluble interleukin-2 receptor (sIL-2R), a serum marker of an activated immune response, was highly elevated (7,416 U/mL, reference range: 135-483). Multiple organ failure (MOF) and DIC were successfully managed by multimodality treatments using inorganized iodide, glucocorticoids, anti-thyroid drugs, beta-blockers, and diuretics as well as an anticoagulant agent and the transfusion of platelet concentrate and fresh frozen plasma. sIL-2R levels gradually decreased during the initial treatment, but were still above the reference range even after thyroidectomy. Mild elevations in serum levels of sIL-2R have previously been correlated with thyroid hormone levels in non-storm Graves' disease. The present study demonstrated, for the first time, that circulating sIL-2R levels could be markedly elevated in TS. The marked increase in sIL-2R levels was speculated to represent an inappropriate generalized immune response that plays an unknown role in the pathogenesis of TS. PMID:24748455
Shimoda, Yoko; Satoh, Tetsurou; Takahashi, Hiroki; Katano-Toki, Akiko; Ozawa, Atsushi; Tomaru, Takuya; Horiguchi, Norio; Kaira, Kyoichi; Nishioka, Masaki; Shibusawa, Nobuyuki; Hashimoto, Koshi; Wakino, Shu; Mori, Masatomo; Yamada, Masanobu
... disorders. Complications of Marfan Syndrome Heart and Blood Vessel Complications The most serious complications of Marfan syndrome involve the heart and blood vessels. Marfan syndrome can affect the aorta, the main ...
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
[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
Bronchiolitis Obliterans Syndrome (BOS), Bronchiolitis Obliterans Organizing Pneumonia (BOOP), and Other Late-Onset Noninfectious Pulmonary Complications following Allogeneic Hematopoietic Stem Cell Transplantation
Pulmonary dysfunction is a significant complication following allogeneic hematopoietic stem cell transplantation (HSCT), and is associated with significant morbidity and mortality. Effective antimicrobial prophylaxis and treatment strategies have increased the incidence of noninfectious lung injury, which can occur in the early posttransplant period or in the months and years that follow. Late-onset noninfectious pulmonary complications are frequently encountered, but diagnostic
Satoshi Yoshihara; Gregory Yanik; Kenneth R. Cooke; Shin Mineishi
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
BACKGROUND: The Iraq war has vividly brought the problem of traumatic brain injury to the foreground. The costs of death and morbidity in lost wages, lost taxes, and rehabilitative costs, let alone the emotional costs, are enormous. Military personnel with traumatic brain injury and acute respiratory distress syndrome may represent a substantial problem. Each of these entities, in and of
Thomas J Papadimos
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.
\\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
We herein present a case of unresectable giant hepatic hemangiomas with Kasabach-Merritt syndrome which was successfully treated\\u000a by living donor liver transplantation using a left lobe graft. The patient was a 45-year-old woman who complained of abdominal\\u000a distension. Two sessions of transarterial embolization were performed, but failed to reduce the size of the tumor. The hepatic\\u000a tumors were thus judged
Makoto Meguro; Yuji Soejima; Akinobu Taketomi; Toru Ikegami; Yo-ichi Yamashita; Noboru Harada; Shinji Itoh; Koichi Hirata; Yoshihiko Maehara
\\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
Paroxysmal Nocturnal Hemoglobinuria Following Alemtuzumab Immunosuppressive Therapy for Myelodysplastic Syndrome and Complicated by Recurrent Life-Threatening Thrombosis Despite Anticoagulation: Successful Intervention with Eculizumab and Fondaparinux
The pathogenesis of paroxysmal nocturnal hemoglobinuria (PNH) is not fully understood. We report a patient with myelodysplastic syndrome who developed symptomatic PNH following treatment with alemtuzumab. A small PNH clone, identified prior to alemtuzumab, expanded resulting in hemolytic anemia and recurrent CNS thromboses despite anticoagulation. Remission was achieved with eculizumab and fondaparinux therapy. Alemtuzumab has been associated with the development of glycosylphosphotidylinositol negative cells, but its clinical significance has been unclear. Our case emphasizes its potential clinical importance. Future studies are necessary to expand our understanding of this rare disease entity and improve its management.
Cheng, Kit L.; Brody, Judith; Warshall, Craig E.; Sloand, Elaine M.; Allen, Steven L.
Exogenous toxins and chronic hypoxia have been implicated in the etiopathogenesis of a variety of neurological disorders, but it is not always possible to establish a cause-effect relationships. We describe a patient who presented with an unusual gait disorder and progressive motor neuron disease after residing for 2 years within Biosphere 2, a scientifically engineered dome, tightly sealed to allow miniscule exchange of air between its atmosphere and the earth's atmosphere. We postulate that this unusual syndrome resulted from chronic hypoxia possibly coupled with carbon monoxide and nitrous oxide exposure. PMID:15077246
Lassinger, Brian K; Kwak, Carolyn; Walford, Roy L; Jankovic, Joseph
Hamartomatous polyposis syndromes are a diverse group of inherited conditions grouped together because they exhibit hamartomatous rather than epithelial polyp histology. Each syndrome exhibits characteristic polyp histology, gastrointestinal polyp distribution, gastrointestinal cancer risks, extra-intestinal benign findings and often extra-intestinal cancer risks. Identifying individuals at risk for these syndromes and accurately defining the precise diagnosis is necessary for planning surveillance and management in order to prevent the benign and malignant complications. Characteristic syndrome features including gastrointestinal findings, pathology, genetics, and management options for the three most common hamartomatous polyposis syndromes, Peutz-Jeghers syndrome, PTEN hamartoma tumor syndrome, and juvenile polyposis will be presented in this review.
Gammon, Amanda; Jasperson, Kory; Kohlmann, Wendy
Abernethy malformation type II with nephrotic syndrome and other multisystemic presentation: an illustrative case for understanding pathogenesis of extrahepatic complication of congenital portosystemic shunt.
Abernethy malformation, an extrahepatic congenital portosystemic shunt, is more often diagnosed based on associated cardiac or pulmonary malformation. Although predominately a pediatric diagnosis, "late diagnoses" in adulthood have been reported especially in type II malformations that involve only a partial shunt of portal circulation directly into the inferior vena cava. Aside from the cardiac-related presentation, Abernethy malformation is also associated with multiple liver nodules, either benign or malignant, and pulmonary hypertension. In this report, we present immunoglobulin A glomerulonephritis with nephrotic syndrome as a hitherto unrecognized manifestation of this malformation outside the pediatric population, in a patient who also had pulmonary hypertension and multiple liver tumors. We also propose a pathogenetic basis for this multisystemic presentation that includes release into the systemic circulation of unfiltered bacteria, vasoactive substances, and immunoglobulin A-antigen complexes. PMID:23245671
Schaeffer, David F; Laiq, Simin; Jang, Hyun-Jung; John, Rohan; Adeyi, Oyedele A
Although most cases of craniosynostosis are nonsyndromic, craniosynostosis is known to occur in conjunction with other anomalies in well-defined patterns that make up clinically recognized syndromes. Patients with syndromic craniosynostoses are much more complicated to care for, requiring a multidisciplinary approach to address all of their needs effectively. This review describes the most common craniosynostosis syndromes, their characteristic features and syndrome-specific functional issues, and new modalities utilized in their management. General principles including skull development, the risk of developing increased intracranial pressure in craniosynostosis syndromes, and techniques to measure intracranial pressure are discussed. Evolving techniques of the established operative management of craniosynostosis are discussed together with more recent techniques including spring cranioplasty and posterior cranial vault distraction osteogenesis.
Derderian, Christopher; Seaward, James
INTRODUCTION Bochdalek's diaphragmatic hernia (BDH) rarely developed symptomatic in adulthood but mostly required an operation. In adult BDH cases, long-term residing of the massive intraabdominal organs in the thoracic cavity passively causes loss of domain for abdominal organs (LOD). PRESENTATION OF CASE A 63-year-old man presented at our institution complaining of sudden left upper quadrant abdominal pain. Chest radiography showed a hyperdense lesion containing bowel gas in the left pleural space. Computed tomography revealed a dilated bowel above the diaphragm and intestinal obstruction suggestive of gangrenous changes. These findings were consistent with the diagnosis of incarcerated BDH and an emergency laparotomy was performed. Operative findings revealed the hypoplastic lung, lack of hernia sac, and location of the diaphragmatic defect, which indicated that his hernia was true congenital. Organs were reduced into the abdominal cavity, and large defect of the diaphragm was repaired with combination of direct vascular closure and intraperitoneal onlay mesh reinforcement using with expanded polytetrafluoroethylene (ePTFE) mesh. On the postoperative day 1, the patient fell into the shock and was diagnosed to have abdominal compartment syndrome (ACS). Conservative therapies were administered, but resulted in gastropleural fistula and pleural empyema, which required an emergency surgery. Mesh extraction and fistulectomy were performed. DISCUSSION A PubMed search for the case of ACS after repair of the adult BDH revealed only three cases, making this very rare condition. CONCLUSION In dealing with adult BDH, possible post-repair ACS should be considered.
Suzuki, Toshiaki; Okamoto, Tomoyoshi; Hanyu, Ken; Suwa, Katsuhito; Ashizuka, Shuichi; Yanaga, Katsuhiko
Atypical form of tako-tsubo cardiomyopathy (TTC) is associated with regional wall motion abnormalities in basal and/or middle segments or only middle segments with sparing of apical segments or apical and basal segments. We described a case of47-year-old female with atypical form of TTC due to fast atrial fibrillation that converted into ventricular fibrillation in WPW syndrome. The echocardiogram made after direct current cardioversion revealed decreased left ventricular ejection fraction (LVEF 35%) with akinesis of inferior and posterior walls and anterior part of interventricular septum in the middle and the basal segments with hyperkinesis of apical segments. The biochemistry blood samples revealed elevated both troponin T- 0.35 ng/mL and NT-proBNP - 3550 pg/mL plasma level. The ECG showed sinus rhythm 62 bpm, shortened PQ interval 100 ms, widened QRS duration - 115 ms with delta wave, prolonged QT interval - 520 ms, QS in leads: II, III, aVF. NegativeT waves in leads: I, aVL and positive, symmetrical T waves in leads V1-V6. The coronarography revealed normal coronaryarteries. The control echocardiography after 10 days showed normal LVEF 70%, without any wall motion abnormalities. TTC was recognised based on: history of sudden stress situation before, ischaemic ECG changes, positive markers of myocardial injury, transient segmental wall motion abnormalities and normal coronary arteries. The ablation of right postero-septal accessory pathway was successfully performed. PMID:24049031
Kukla, Piotr; Stec, Sebastian; Karbarz, Dariusz; Wrzosek, Bo?ena; Jastrz?bski, Marek; Kluczewski, Maciej; Kurdzielewicz, Wojciech
Background Palsies involving the anterior interosseous nerve (AIN) comprise less than 1% of all upper extremity nerve palsies. Objectives This case highlights the potential vascular and neurological hazards of minimal penetrating injury of the proximal forearm and emphasizes the phenomenon of delayed presentation of vascular injuries following seemingly obscure penetrating wounds. Case Report We report a case of a 22-year-old male admitted for a minimal penetrating trauma of the proximal forearm that, some days later, developed an anterior interosseous syndrome. A Duplex study performed immediately after the trauma was normal. Further radiologic investigations i.e. a computer-tomographic-angiography (CTA) revealed a false aneurysm of the proximal portion of the interosseous artery (IA). Endovascular management was proposed but a spontaneous rupture dictated surgical revision with simple excision. Complete neurological recovery was documented at 4 months postoperatively. Conclusions/Summary After every penetrating injury of the proximal forearm we propose routinely a detailed neurological and vascular status and a CTA if Duplex evaluation is negative.
... 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 ...
The cases are reported of five children with chronic renal failure who underwent gastrocystoplasty for a variety of urological disorders. Gastrocystoplasty comprises the transplantation of a vascularised segment of stomach to the bladder to form an augmented neobladder. The patients had gastrointestinal complications after the operation, including considerable weight loss in all five patients, accompanied by marked failure to thrive in four of the five patients, and food aversion, feeding intolerance, dumping syndrome, delayed gastric emptying, and oesophagitis in two patients. Three of the five patients developed severe abdominal pain and haemorrhagic cystitis secondary to gastric acid secretion in the neobladder from the transplanted gastric pedicle. Nutritional and pharmacological interventions were used to manage the gastrointestinal problems. Explanations are offered for the pathophysiology of the observed complications of gastrocystoplasty. It is believed that the use of this procedure in infants and children, particularly those with chronic renal failure and uraemia, warrants caution until successful long term follow up and experience with this procedure have been reported.
Gold, B D; Bhoopalam, P S; Reifen, R M; Harvey, E; Marcon, M A
An arrhythmia is defined as an abnormal heart rhythm. Certain arrhythmias have much higher rates of neurologic complications, including stroke, cognitive impairment, and dementia. The purpose of this paper is to review both the direct and indirect causes of neurologic problems caused by arrhythmias. Direct complications from arrhythmia can include stroke, transient ischemic attack (TIA), and varying disorders of cognition. According to the Stroke Data Bank, which divided potential direct causes of cardioembolic stroke into strong and weak sources, both atrial fibrillation and sick-sinus syndrome were considered to be strong sources of this type of ischemic stroke. Indirect causes of neurologic complications include cardiopulmonary resuscitation, medications, cardiology procedures, and cardiac surgical procedures used to treat arrhythmias. PMID:24365293
Leary, Megan C; Veluz, Jeffrey S; Caplan, Louis R
Esophagectomy can be used to treat several esophageal diseases; it is most commonly used for treatment of esophageal cancer. Esophagectomy is a major procedure that may result in various complications. This article reviews only the important complications resulting from esophageal resection, which are anastomotic complications after esophageal reconstruction (leakage and stricture), delayed emptying or dumping syndrome, reflux, and chylothorax. PMID:24876942
Esophagectomy can be used to treat several esophageal diseases; it is most commonly used for treatment of esophageal cancer. Esophagectomy is a major procedure that may result in various complications. This article reviews only the important complications resulting from esophageal resection, which are anastomotic complications after esophageal reconstruction (leakage and stricture), delayed emptying or dumping syndrome, reflux, and chylothorax.
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.
In this review, we analyse the role of the endothelium in the development of several complications that appear soon after haematopoietic SCT (HSCT). Once it had been demonstrated that sinusoidal damage is the initiating event of the sinusoidal obstruction syndrome, it was considered that other short-term complications with overlapping clinical manifestations, such as capillary leak syndrome, engraftment syndrome, transplant-associated microangiopathy,
E Carreras; M Diaz-Ricart
... 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 ...
... 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 ...
Five cases with end-stage dry eye (four with pemphigoid, one with Stevens-Johnson syndrome) have been operated with a keratoprosthesis and have been followed for followed for 6 to 22 months. Two patients have been failures, three still have good vision. PMID:8030536
Dohlman, C; Doane, M
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.
Tonsillectomy is one of the most common operations performed by otolaryngologists. Some extraordinary complications of this surgery are massive bleeding, taste perception disorders due to glossopharyngeal nerve damage, hematoma of the mouth floor, jugular vein thrombosis, Grisel syndrome, cervical osteomyelitis, nasopharyngeal stenosis, pulmonary edema, infection, and lingual artery pseudoaneurysm. In this clinical report, an 8-year-old girl presented with a posterior plica perforation after adenotonsillectomy performed 1 week previously and this complication has led to velopharyngeal insufficiency. This rare complication may result from traumatic damage or excessive cauterization of the posterior plica. PMID:25006950
Yildirim, Yavuz Selim; Senturk, Erol; Ozturan, Orhan
In last decade, dengue has emerged as one of the most important vector born disease. With increasing cases, uncommon presentations and complications are now commonly recognized. Here, we report two cases of rare pattern of respiratory involvement in dengue: acute respiratory distress syndrome and bronchiolitis with respiratory failure. PMID:23870477
Kumar, Naveen; Gadpayle, A K; Trisal, Deepshikha
The most important issues in perioperative complications of oesophagectomy are prevention, early detection and appropriate management. Anastomotic leakage is the most frequent technical surgical complication. Prevention comprises avoidance of tension or impaired vascularization of the conduit and meticulous suture technique. Management includes early diagnosis, conservative treatment or endoscopic stenting of contained leakage, and re-operation of non-contained insufficiency. All other surgical complications - such as bleeding, tracheobronchial lesions or chylothorax - are rare and warrant special therapeutic modalities. The main general non-surgical complication is postoperative pneumonia, which should be prevented by effective pain control (especially peridural catheter) and appropriate techniques of artificial respiration. Special attention should be offered to postoperative tachyarrhythmias and alcohol withdrawal syndrome. Prevention of complications also includes exclusion of patients with high operative risk based on scores and specific preoperative treatment of risk factors. PMID:16997169
Hölscher, Arnulf H; Vallböhmer, Daniel; Brabender, Jan
Nine cases of Olmsted syndrome have been reported in the world literature. In this syndrome, keratoderma usually starts during infancy on the palms and soles when the baby starts to use the feet for walking and the hands for grasping. Within weeks or months, there is progressive spread of solid, symmetrical, thick hyperkeratotic keratoderma to both palms and soles, surrounded by erythematous margins. Contraction of fingers and deep fissuring of the feet are common complications. Symmetrical, yellow-brown hyperkeratotic plaques and papules are also observed around body orifices such as the mouth, nares, inguinal region, and perianal and gluteal areas. Other clinical manifestations have been reported, including diffuse alopecia, thin nails, leukokeratosis of the oral mucosa, onychodystrophy, hyperkeratotic linear streaks, exaggerated keratosis pilaris, and large verrucous plaques in the axillae. In the differential diagnosis, other keratoderma and hyperkeratotic syndromes should be considered. PMID:7640195
Perry, H O; Su, W P
Neuroacanthocytosis (NA) syndromes are a group of genetically defined diseases characterized by the association of red blood cell acanthocytosis and progressive degeneration of the basal ganglia. NA syndromes are exceptionally rare with an estimated prevalence of less than 1 to 5 per 1'000'000 inhabitants for each disorder. The core NA syndromes include autosomal recessive chorea-acanthocytosis and X-linked McLeod syndrome which have a Huntington´s disease-like phenotype consisting of a choreatic movement disorder, psychiatric manifestations and cognitive decline, and additional multi-system features including myopathy and axonal neuropathy. In addition, cardiomyopathy may occur in McLeod syndrome. Acanthocytes are also found in a proportion of patients with autosomal dominant Huntington's disease-like 2, autosomal recessive pantothenate kinase-associated neurodegeneration and several inherited disorders of lipoprotein metabolism, namely abetalipoproteinemia (Bassen-Kornzweig syndrome) and hypobetalipoproteinemia leading to vitamin E malabsorption. The latter disorders are characterized by a peripheral neuropathy and sensory ataxia due to dorsal column degeneration, but movement disorders and cognitive impairment are not present. NA syndromes are caused by disease-specific genetic mutations. The mechanism by which these mutations cause neurodegeneration is not known. The association of the acanthocytic membrane abnormality with selective degeneration of the basal ganglia, however, suggests a common pathogenetic pathway. Laboratory tests include blood smears to detect acanthocytosis and determination of serum creatine kinase. Cerebral magnetic resonance imaging may demonstrate striatal atrophy. Kell and Kx blood group antigens are reduced or absent in McLeod syndrome. Western blot for chorein demonstrates absence of this protein in red blood cells of chorea-acanthocytosis patients. Specific genetic testing is possible in all NA syndromes. Differential diagnoses include Huntington disease and other causes of progressive hyperkinetic movement disorders. There are no curative therapies for NA syndromes. Regular cardiologic studies and avoidance of transfusion complications are mandatory in McLeod syndrome. The hyperkinetic movement disorder may be treated as in Huntington disease. Other symptoms including psychiatric manifestations should be managed in a symptom-oriented manner. NA syndromes have a relentlessly progressive course usually over two to three decades.
Craniovertebral dislocation is uncommon, but its diagnosis is important taking into account the potential severity of the neurologic complications. A number of causes are known; the most common are Down syndrome, rheumatoid arthritis, Paget's disease, other metabolic bone diseases, and craniocervical trauma. Down's syndrome is a relatively common clinical condition but craniovertebral subluxation is only observed in a small percentage of patients. About half of all cervical spine injuries affect the atlanto-occipital region and C2 vertebra. In rheumatoid arthritis, craniocervical dislocation occurs in up to 40% of patients with severe disease. In Paget's disease, involvement of the craniovertebral region occurs in about 30% of all cases. The clinical neurologic syndrome is characterized by local pain, features of upper spinal cord and medullary compression, positive Lhermitte phenomenon, syncope associated with neck flexion, vertebral artery obstruction or dissection leading to stroke, and asymmetrical lower cranial nerve palsies. Neuroimaging is essential to confirm the clinical diagnosis and to categorize severity. The treatment of this disorder is usually surgical, but traction and external immobilization is relevant in some cases. Specific conditions may require additional treatments such as radiotherapy, antibiotics, or chemotherapy. PMID:24365311
de Carvalho, Mamede; Swash, Michael
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
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
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
Gastrointestinal complications of pre-eclampsia can occur and have the risk of being life-threatening for the mother and fetus. Hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome has been recognized as a complication of pre-eclampsia for decades. Pregnancies complicated by this syndrome require a well-formulated management plan, including assessing and stabilizing the maternal condition as well as evaluating fetal well-being. Patients with HELLP syndrome should receive anti-seizure prophylaxis with magnesium sulfate, treatment for severe hypertension, and correction of coagulopathy, if present. The potential benefits of expectant management of HELLP syndrome in those remote from term and the use of corticosteroids to improve maternal outcome remain experimental. Computed tomography or ultrasound of the abdomen should be performed if a subcapsular hematoma of the liver is suspected. If a ruptured hematoma is confirmed, massive transfusions and laparotomy are indicated. Ischemia associated with pre-eclampsia cannot only damage the liver but also the pancreas and gallbladder. PMID:19464509
Barton, John R; Sibai, Baha M
Cirrhosis results from progressive fibrosis and is the final outcome of all chronic liver disease. It is among the ten leading causes of death in United States. Cirrhosis can result in portal hypertension and/or hepatic dysfunction. Both of these either alone or in combination can lead to many complications, including ascites, varices, hepatic encephalopathy, hepatocellular carcinoma, hepatopulmonary syndrome, and coagulation disorders. Cirrhosis and its complications not only impair quality of life but also decrease survival. Managing patients with cirrhosis can be a challenge and requires an organized and systematic approach. Increasing physicians' knowledge about prevention and treatment of these potential complications is important to improve patient outcomes. A literature search of the published data was performed to provide a comprehensive review regarding the management of cirrhosis and its complications. PMID:24833875
Nusrat, Salman; Khan, Muhammad S; Fazili, Javid; Madhoun, Mohammad F
Cirrhosis results from progressive fibrosis and is the final outcome of all chronic liver disease. It is among the ten leading causes of death in United States. Cirrhosis can result in portal hypertension and/or hepatic dysfunction. Both of these either alone or in combination can lead to many complications, including ascites, varices, hepatic encephalopathy, hepatocellular carcinoma, hepatopulmonary syndrome, and coagulation disorders. Cirrhosis and its complications not only impair quality of life but also decrease survival. Managing patients with cirrhosis can be a challenge and requires an organized and systematic approach. Increasing physicians’ knowledge about prevention and treatment of these potential complications is important to improve patient outcomes. A literature search of the published data was performed to provide a comprehensive review regarding the management of cirrhosis and its complications.
Nusrat, Salman; Khan, Muhammad S; Fazili, Javid; Madhoun, Mohammad F
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
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
Abstract Oncology patients often experience urgent or emergent medical complications that are a direct or indirect result of the underlying malignant condition and are first identified or clarified on radiologic imaging studies. The aim of this review is to identify, discuss, and illustrate some of the major thoracic complications in patients with primary intrathoracic or extrathoracic neoplasms; particular focus is placed on issues in which radiologic imaging may have a significant impact on patient management, including superior vena cava (SVC) syndrome, post-obstructive pneumonia, diaphragmatic paralysis, pleural effusions, pericardial disease, tracheo-esophageal fistula, deep venous thrombosis, and pulmonary embolism.
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
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.
Sotos syndrome is an overgrowth condition characterized by cardinal features including excessive growth during childhood, macrocephaly, distinctive facial gestalt and various degrees of learning difficulty, and associated with variable minor features. The exact prevalence remains unknown but hundreds of cases have been reported. The diagnosis is usually suspected after birth because of excessive height and occipitofrontal circumference (OFC), advanced bone age, neonatal complications including hypotonia and feeding difficulties, and facial gestalt. Other inconstant clinical abnormalities include scoliosis, cardiac and genitourinary anomalies, seizures and brisk deep tendon reflexes. Variable delays in cognitive and motor development are also observed. The syndrome may also be associated with an increased risk of tumors. Mutations and deletions of the NSD1 gene (located at chromosome 5q35 and coding for a histone methyltransferase implicated in transcriptional regulation) are responsible for more than 75% of cases. FISH analysis, MLPA or multiplex quantitative PCR allow the detection of total/partial NSD1 deletions, and direct sequencing allows detection of NSD1 mutations. The large majority of NSD1 abnormalities occur de novo and there are very few familial cases. Although most cases are sporadic, several reports of autosomal dominant inheritance have been described. Germline mosaicism has never been reported and the recurrence risk for normal parents is very low (<1%). The main differential diagnoses are Weaver syndrome, Beckwith-Wiedeman syndrome, Fragile X syndrome, Simpson-Golabi-Behmel syndrome and 22qter deletion syndrome. Management is multidisciplinary. During the neonatal period, therapies are mostly symptomatic, including phototherapy in case of jaundice, treatment of the feeding difficulties and gastroesophageal reflux, and detection and treatment of hypoglycemia. General pediatric follow-up is important during the first years of life to allow detection and management of clinical complications such as scoliosis and febrile seizures. An adequate psychological and educational program with speech therapy and motor stimulation plays an important role in the global development of the patients. Final body height is difficult to predict but growth tends to normalize after puberty. PMID:17825104
Baujat, Geneviève; Cormier-Daire, Valérie
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
Bloom Syndrome (BS, MIM #210900) is an autosomal recessive genetic disorder caused by a mutation in the BLM gene, which codes for the DNA repair enzyme RecQL3 helicase. Without proper DNA repair mechanisms, abnormal DNA exchange takes place between sister chromatids and results in genetic instability that may lead to cancer, especially lymphoma and acute myelogenous leukemia, lower and upper gastrointestinal tract neoplasias, cutaneous tumors, and neoplasias in the genitalia and urinary tract. BS patients are usually of Ashkenazi Jewish descent and exhibit narrow facial features, elongated limbs, and several dermatologic complications including photosensitivity, poikiloderma, and telangiectatic erythema. The most concerning manifestation of BS is multiple malignancies, which require frequent screenings and strict vigilance by the physician. Therefore, distinguishing between BS and other dermatologic syndromes of similar presentation such as Rothmund-Thomson Syndrome, Erythropoietic Protoporphyria, and Cockayne Syndrome is paramount to disease management and to prolonging life. BS can be diagnosed through a variety of DNA sequencing methods, and genetic testing is available for high-risk populations. This review consolidates several sources on BS sequelae and aims to suggest the importance of differentiating BS from other dermatologic conditions. This paper also elucidates the recently discovered BRAFT and FANCM protein complexes that link BS and Fanconi anemia. PMID:24602044
Arora, Harleen; Chacon, Anna H; Choudhary, Sonal; McLeod, Michael P; Meshkov, Lauren; Nouri, Keyvan; Izakovic, Jan
Hepatorenal syndrome is a severe complication of end-stage liver disease. The pathophysiological hallmark is severe renal vasoconstriction, resulting from peripheral and splanchnic vasodilation as well as activation of renal vasoconstrictor molecules, which induce the effective arterial volume reduction and the functional renal failure. The diagnosis of hepatorenal syndrome is currently based on the exclusion of other causes of renal failure (especially prerenal). Spontaneous bacterial peritonitis is one of the triggering factors and should be sought in all patients with severe liver disease and acute renal failure. Quickly treating patients with parental antibiotics and albumin infusion significantly decreases the risk. The combined use of intravenous albumin, splanchnic and peripheral vasoconstrictor and/or renal replacement therapy sometimes enables a delay until liver transplantation (or combined liver-kidney in selected patients). Transplantation is in fact the only way to improve the long-term prognosis. PMID:24388293
Surgery of the upper airway is performed in dogs for the correction of brachycephalic airway syndrome and laryngeal paralysis and for temporary or permanent tracheostomy. Although technically simple to perform, upper airway surgeries can lead to the development of significant postoperative complications. This article reviews complications associated with common surgical conditions of the upper airway. It involves a discussion of brachycephalic airway syndrome and associated respiratory and gastrointestinal complications. It also covers laryngeal paralysis with a focus on unilateral arytenoid lateralization and the complication of aspiration pneumonia. The condition of acquired laryngeal webbing/stenosis and potential treatment options is also discussed. Finally, tracheostomies and associated complications in dogs and cats are reviewed. PMID:21889695
The hepatopulmonary syndrome (HPS) is a pulmonary complication of cirrhosis and/or portal hypertension whereby patients develop hypoxemia as a result of alterations in pulmonary microvascular tone and architecture. HPS occurs in up to 30% of patients with cirrhosis. Although the degree of hypoxemia does not reliably correlate with the severity of liver disease, patients with HPS have a higher mortality than do patients with cirrhosis without the disorder. There has been progress into defining the mechanisms that lead to hypoxemia in HPS, but to date there are no therapeutic options for HPS aside from liver transplantation. PMID:24679503
Koch, David G; Fallon, Michael B
Neuroendoscopy is rapidly becoming an essential part of the neurosurgeon's repertoire. Currently, very few studies have identified the complications of this new technique, yet many have warned of the steep learning curve associated with its practice. We have reviewed the last 173 neuroendoscopic procedures performed by one surgeon and identified two distinct groups of complications: those that have clinically significant
Charles Teo; Salim Rahman; Frederick A. Boop; Bruce Cherny
Pneumomediastinum and subcutaneous emphysema is a rare complication of the second stage of labor. Although this entity runs a benign course, fatalities have been reported. Clinical findings of subcutaneous emphysema necessitate a chest X-ray to document pneumomediastinum. If pneumomediastinum is present, rapid delivery via elective forceps or even surgery may be considered to prevent serious complications. PMID:1167973
Bard, R; Hassini, N
Rhinoplasty presents a unique set of challenges for the cosmetic surgeon. Complications may arise from inadequate diagnosis, errors in surgical technique, or variations in the patient's anatomy or healing response. Complications as a result of overly aggressive surgery may also have functional consequences and be harder to correct. PMID:19185797
Harsha, Brian C
PURPOSE: Dynamic graciloplasty can improve continence in patients with severe refractory fecal incontinence, but associated morbidity is high. The purpose of this study was to identify complications associated with dynamic graciloplasty and to characterize their treatment and impact on patient outcome. METHODS: In 121 patients enrolled in a prospective trial of 20 centers and eligible for safety analysis, all complications
Klaus E. Matzel; Robert D. Madoff; Laura J. LaFontaine; Cor G. M. I. Baeten; W. Donald Buie; John Christiansen; Steven Wexner
The purpose of this study was to retrospectively evaluate the complications of 58 patients who underwent surgery for mandibular condylar process fractures. Data were collected from patients during a 10-year period (1999-2009). The data recorded included demographic data, etiology, diagnosis, type of condylar fracture, surgical approaches, and postoperative complications. A total of 58 underwent surgery for reduction of the condylar fractures. There were 22 patients with bilateral condyle fractures and 36 patients with unilateral condyle fractures, accounting for 65 surgeries. In 8 fractures, a preauricular approach was performed to access the fractures condyle, whereas the retromandibular approach was performed in 57 fractures. There were 2 temporary facial palsies, 1 permanent facial palsy, and 1 sialocele. There were no cases of hypertrophic scar, Frey syndrome, or salivary fistula. In conclusion, permanent deformities after surgical complications were unusual, and the results are acceptably safe. PMID:21778851
Lima, Sergio Monteiro; Asprino, Luciana; Moreira, Roger Willian Fernandes; de Moraes, Márcio
This is a case-report about a 4 year old boy with "cherry red" epiglottis, purulent meningitis and pleuropneumonia. Purulent meningitis and pleuropneumonia are not complications of treatment of "cherry red" epiglottis but an entity caused by Hemophilus influenzae-infection and is called Kleinschmidt's syndrome (Hemophilus influenzae type B-infection-syndrome). This severe illness is successfully treated if recognized early enough. At present, chloramphenicol is the therapy of choice. Intubation or tracheotomy are important but supportive measures. PMID:988430
Freudenberg, V; Wolf, H
Hay-Wells syndrome or AEC (Ankyloblepharon, Ectodermal dysplasia and Cleft lip and palate syndrome) is a rare ectodermal disorder. The treatment is aimed to prevent clinical complications. We describe the case of a four-month old male patient with erosions on the scalp, trunk and arms, trachyonychia, deformity of the ears, micropenis, cleft palate, decreased eyebrow and eyelash hairs, in addition to antecedents of surgical correction of ankyloblepharon. The importance of the correct diagnosis is emphasized, besides the investigation of the associated diseases, treatment of complications and genetic counseling of the parents.
Tonolli, Vanessa Mello; Stolf, Hamilton Ometto; Tonello, Claudio Sampieri; Pires, Rafaelle Batistella; Abbade, Luciana Patricia Fernandes
Hay-Wells syndrome or AEC (Ankyloblepharon, Ectodermal dysplasia and Cleft lip and palate syndrome) is a rare ectodermal disorder. The treatment is aimed to prevent clinical complications. We describe the case of a four-month old male patient with erosions on the scalp, trunk and arms, trachyonychia, deformity of the ears, micropenis, cleft palate, decreased eyebrow and eyelash hairs, in addition to antecedents of surgical correction of ankyloblepharon. The importance of the correct diagnosis is emphasized, besides the investigation of the associated diseases, treatment of complications and genetic counseling of the parents. PMID:24770526
Tonolli, Vanessa Mello; Stolf, Hamilton Ometto; Tonello, Cláudio Sampieri; Pires, Rafaelle Batistella; Abbade, Luciana Patricia Fernandes
The mainstream use of dental implants has allowed millions of patients to benefit from the predictability of dental implant therapy and, in many instances, dental implants have become the standard of care. Even though success rates in implant dentistry are well above 90 percent, complications do occur. Most complications are preventable with proper planning and execution. Others are inherent to the risks of surgery and may require intervention. The purpose of this paper is to classify the possible complications that may occur and to discuss their prevention and management. PMID:24881442
Acute hematometra, also termed the postabortal syndrome or redo syndrome, is a rare immediate complication of suction curettage characterized by severe lower abdominal cramping in association with an enlarged and markedly tender uterus. We describe the transvaginal sonographic features of this syndrome. PMID:21337586
Sherer, David M; Salame, Ghadir; Shah, Tana; Lee, Yi-Chun; Serur, Eli; Economos, Katherine; Gorelick, Constantine; Abulafia, Ovadia
Hamartomatous Polyposis Syndromes (HPS) are genetic syndromes, which include Peutz-Jeghers syndrome, Juvenile polyposis syndrome, PTEN hamartoma tumour syndrome (Cowden Syndrom, Bannayan-Riley-Ruvalcaba and Proteus Syndrome) as well as hereditary mixed polyposis syndrome. Other syndromes such as Gorlin Syndrome and multiple endocrine neoplasia syndrome 2B are sometimes referred to as HPS. HPS is characterized by the development of hamartomatous polyps in the gastrointestinal tract as well as several extra-intestinal findings such as dermatological and dysmorphic features or extra-intestinal cancer. The syndromes are rare and inherited in an autosomal dominant manner. The diagnosis of HPS has traditionally been based on clinical criteria, but can sometimes be difficult as the severity of symptoms range considerably from only a few symptoms to very severe cases - even within the same family. De novo cases are also frequent. However, because of the discovery of several associated germline-mutations as well as the rapid development in genetics it is now possible to use genetic testing more often in the diagnostic process. Management of the syndromes is different for each syndrome as extra-intestinal symptoms and types of cancers differs. Clinical awareness and early diagnosis of HPS is important, as affected patients and at-risk family members should be offered genetic counselling and surveillance. Surveillance in children with HPS might prevent or detect intestinal or extra-intestinal complications, whereas in adulthood surveillance is recommended due to an increased risk of cancer e.g. intestinal cancer or breast cancer.
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
Laparoscopic hernioplasty is a technique which can present a number of specific complications. This paper reviews the complications that can occur during laparoscopic hernia repair and ways to avoid them; it also describes the surgical technique used successfully in over 1000 cases. Initial experience suggests that complications can be avoided with adequate knowledge, attention to surgical anatomy and the proper technique of laparoscopic hernioplasty. Early recurrences are rare and invariably result from inadequate surgical technique. Inadequate fixation of the mesh, inadequate mesh size, and failure to cover unidentified wall defects (hernias which have never been repaired), are the main causes of early recurrence of hernia. Experience, knowledge of complications and how to avoid them, adequate training and attention to the anatomy of the inguinal region are the most important factors in correcting inguinal hernia successfully by laparoscopy.
Reusch, Marcus; daRosa, Andre L. M.; Carlos, Jose Roberto B.
Certain dermatoses may be complicated by systemic amyloidosis. The underlying disorders in eight of 100 patients with secondary systemic amyloidosis studied at autopsy were: hidradenitis suppurativa, stasis ulcers, psoriatic arthritis, basal cell carcinom...
M. H. Brownstein E. B. Helwig
In the United States, circumcision is a commonly performed procedure. It is a relatively safe procedure with a low overall complication rate. Most complications are minor and can be managed easily. Though uncommon, complications of circumcision do represent a significant percentage of cases seen by pediatric urologists. Often they require surgical correction that results in a significant cost to the health care system. Severe complications are quite rare, but death has been reported as a result in some cases. A thorough and complete preoperative evaluation, focusing on bleeding history and birth history, is imperative. Proper selection of patients based on age and anatomic considerations as well as proper sterile surgical technique are critical to prevent future circumcision-related adverse events. PMID:22235177
Krill, Aaron J; Palmer, Lane S; Palmer, Jeffrey S
... ALL current lymph- edema treatment modalities (including manual lymphatic drainage, bandaging, and compression garments). The antibiotics of ... complications, but in rare situations a form of lymphatic cancer called lymphangiosarcoma may develop. Symptoms include a ...
Complications of shoulder dystocia are divided into fetal and maternal. Fetal brachial plexus injury (BPI) is the most common fetal complication occurring in 4-40% of cases. BPI has also been reported in abdominal deliveries and in deliveries not complicated by shoulder dystocia. Fractures of the fetal humerus and clavicle occur in about 10.6% of cases of shoulder dystocia and usually heal with no sequel. Hypoxic ischemic brain injury is reported in 0.5-23% of cases of shoulder dystocia. The risk correlates with the duration of head-to-body delivery and is especially increased when the duration is >5min. Fetal death is rare and is reported in 0.4% of cases. Maternal complications of shoulder dystocia include post-partum hemorrhage, vaginal lacerations, anal tears, and uterine rupture. The psychological stress impact of shoulder dystocia is under-recognized and deserves counseling prior to home discharge. PMID:24863025
Dajani, Nafisa K; Magann, Everett F
The received account of whistleblowing, developed over the last quarter century, is identified with the work of Norman Bowie\\u000a and Richard DeGeorge. Michael Davis has detailed three anomalies for the received view: the paradoxes of burden, missing harm\\u000a and failure. In addition, he has proposed an alternative account of whistleblowing, viz., the Complicity Theory. This paper\\u000a examines the Complicity Theory.
A. David Kline
The received account of whistleblowing, developed over the last quarter century, is identified with the work of Norman Bowie and Richard DeGeorge. Michael Davis has detailed three anomalies for the received view: the paradoxes of burden, missing harm and failure. In addition, he has proposed an alternative account of whistleblowing, viz., the Complicity Theory. This paper examines the Complicity Theory. The supposed anomalies rest on misunderstandings of the received view or misreadings of model cases of whistleblowing, for example, the Challenger disaster and the Ford Pinto. Nevertheless, the Complicity Theory is important for as in science the contrast with alternative competing accounts often helps us better understand the received view. Several aspects of the received view are reviewed and strengthened through comparison with Complicity Theory, including why whistleblowing needs moral justification. Complicity Theory is also critiqued. The fundamental failure of Complicity Theory is its failure to explain why government and the public encourage and protect whistleblowers despite the possibility of considerable harm to the relevant company in reputation, lost jobs, and lost shareholder value. PMID:16609713
Kline, A David
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
Mirizzi syndrome is an important complication of gallstone disease. If not recognized preoperatively, it can result in significant morbidity and mortality. Preoperative diagnosis may be difficult despite the availability of multiple imaging modalities. Ultrasonography (US), CT, and magnetic resonance cholangiopancreatography (MRCP) are common initial tests for suspected Mirizzi syndrome. Typical findings on US suggestive of Mirizzi syndrome are a shrunken gallbladder, impacted stone(s) in the cystic duct, a dilated intrahepatic tree, and common hepatic duct with a normal-sized common bile duct. The main role of CT is to differentiate Mirizzi syndrome from a malignancy in the area of porta hepatis or in the liver. MRI and MRCP are increasingly playing an important role and have the additional advantage of showing the extent of inflammation around the gallbladder that can help in the differentiation of Mirizzi syndrome from other gallbladder pathologies such as gallbladder malignancy. Endoscopic retrograde cholangiopancreatography (ERCP) is the gold standard in the diagnosis of Mirizzi syndrome. It delineates the cause, level, and extent of biliary obstruction, as well as ductal abnormalities, including fistula. ERCP also offers a variety of therapeutic options, such as stone extraction and biliary stent placement. Percutaneous cholangiogram can provide information similar to ERCP; however, ERCP has an additional advantage of identifying a low-lying cystic duct that may be missed on percutaneous cholangiogram. Wire-guided intraductal US can provide high-resolution images of the biliary tract and adjacent structures. Treatment is primarily surgical. Open surgery is the current standard for managing patients with Mirizzi syndrome. Good short- and long-term results with low mortality and morbidity have been reported with open surgical management. Laparoscopic management is contraindicated in many patients because of the increased risk of morbidity and mortality associated with this approach. Endoscopic treatment may serve as an alternative in patients who are poor surgical candidates, such as elderly patients or those with multiple comorbidities. Endoscopic treatment also can serve as a temporizing measure to provide biliary drainage in preparation for an elective surgery. PMID:17391625
Ahlawat, Sushil K; Singhania, Rohit; Al-Kawas, Firas H
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
Allopurinol, a commonly prescribed medication for gout and hyperuricemia, is a frequent cause of severe cutaneous adverse reactions (SCAR), which include the drug hypersensitivity syndrome, Stevens-Johnson syndrome, and toxic epidermal necrolysis. The adverse events are unpredictable and carry significant morbidity and mortality. To identify genetic markers for allopurinol-SCAR, we carried out a case-control association study. We enrolled 51 patients with
Shuen-Iu Hung; Wen-Hung Chung; Lieh-Bang Liou; Chen-Chung Chu; Marie Lin; Hsien-Ping Huang; Yen-Ling Lin; Joung-Liang Lan; Li-Cheng Yang; Hong-Shang Hong; Ming-Jing Chen; Ping-Chin Lai; Mai-Szu Wu; Chia-Yu Chu; Kuo-Hsien Wang; Chien-Hsiun Chen; Cathy S. J. Fann; Jer-Yuarn Wu; Yuan-Tsong Chen
Acute and chronic pancreatitis may lead to complications that prior the recent advancement of therapeutic endoscopy had to be treated with conventional surgery. The current techniques in interventional endoscopy allow us to access the pancreas and treat some of the complications in a minimally invasive way leading to less morbidity in our patients. Approximately 90% of acute pancreatitis episodes are edematous with early resolution of clinical symptoms and rarely leading to major complications. Patients with necrotizing pancreatitis are susceptible to local and systemic complications particularly in the setting of organ failure. On the other hand chronic pancreatitis is as a chronic inflammatory process that leads to fibrosis of the gland and in occasions to exocrine and/or endocrine insufficiency. The main objective of therapeutic endoscopy in chronic pancreatitis is to relief the obstruction and decreases the pressure in the pancreatic duct with the intention to alleviate abdominal pain. In this review we will address the indications and endoscopic techniques to treat the different complications of pancreatitis such as pseudo cyst drainage, walled off necrosis debridement, disconnected duct syndrome and different options for endoscopic therapy in chronic pancreatitis. PMID:24108377
Coronel, Emmanuel; Czul, Frank; Gelrud, Andres
Interscalene blocks of the brachial plexus are used for surgery of the shoulder and are frequently associated with complications\\u000a such as temporary phrenic block, Horner syndrome or hematoma. To minimize the risk of these complications, we developed an\\u000a approach that avoids medially directed needle advancement and favors spread to lateral regions only: the supraomohyoidal block.\\u000a We tested this procedure in
G. Feigl; A. Fuchs; M. Gries; Q. H. Hogan; B. Weninger; W. Rosmarin
We present a series of 31 patients treated for complications following open carpal tunnel syndrome surgery over a time period\\u000a of 10 years. The most frequent complications encountered were major nerve lacerations at the wrist and thenar followed by\\u000a persistent and recurrent symptoms, neuroma formation and wound infection. All patients had primary treatment by a different\\u000a approach, by different spectrum of
Andreas F. Mavrogenis; Panayiotis J. Papagelopoulos; Ioannis A. Ignatiadis; Sarantis G. Spyridonos; Dimitrios G. Efstathopoulos
Down syndrome, the most common chromosomal abnormality, may be associated with various neurologic complications such as moyamoya syndrome, cervical spinal cord compression due to atlantoaxial subluxation, and basal ganglia damage, as well as epileptic seizures and stroke. Many cases of Down syndrome accompanied by isolated neurologic manifestations have been reported in children; however, Down syndrome with multiple neurologic conditions is rare. Here, we have reported a case of Down syndrome in a 10-year-old girl who presented with asymptomatic moyamoya syndrome, atlantoaxial subluxation with spinal cord compression, and basal ganglia calcification. To the best of our knowledge, this is the first report of Down syndrome, in a child, which was accompanied by these 3 neurologic complications simultaneously. As seen in this case, patients with Down syndrome may have neurologic conditions without any obvious neurologic symptoms; hence, patients with Down syndrome should be carefully examined for the presence of neurologic conditions.
Lee, Kun-Soo; Weon, Young Cheol
Purpose: To compare the incidence of intraoperative and early postoperative complications (IEPC), visual outcomes, and change in intraocular pressure (IOP) between eyes with and without pseudoexfoliation syndrome (PEX) having cataract extraction by phacoemulsification. Material and Method: 800 eyes with PEX and 1,600 eyes without PEX having cataract extraction by phacoemulsification were included in this retrospective study. Evaluated parameters were incidence
Arsen Akinci; Cosar Batman; Orhan Zilelioglu
Endoscopic retrograde cholangiopancreatography (ERCP) is an invasive procedure which carries a complication rate of 5-10%. Splenic injury is a very rare and potentially lethal complication following ERCP. We report a case of a 64-year-old man with a mass at the pancreatic head and obstructive jaundice, who sustained a splenic injury following ERCP. Six hours after the procedure, the patient presented with epigastric pain and hypotension. The abdominal CT scan revealed splenic hematoma. He was offered surgical treatment. Splenectomy was performed with enterogastrostomy.
Grammatopoulos, Anastasios; Moschou, Maria; Rigopoulou, Efrossyni; Katsoras, George
Objective. Drugs can induce almost the entire spectrum of hematologic disorders, affecting white cells, red cells, platelets, and the coagulation system. This paper aims to emphasize the broad range of drug-induced hematological syndromes and to highlight some of the newer drugs and syndromes. Methods. Medline literature on drug-induced hematologic syndromes was reviewed. Most reports and reviews focus on individual drugs or cytopenias. Results. Drug-induced syndromes include hemolytic anemias, methemoglobinemia, red cell aplasia, sideroblastic anemia, megaloblastic anemia, polycythemia, aplastic anemia, leukocytosis, neutropenia, eosinophilia, immune thrombocytopenia, microangiopathic syndromes, hypercoagulability, hypoprothrombinemia, circulating anticoagulants, myelodysplasia, and acute leukemia. Some of the classic drugs known to cause hematologic abnormalities have been replaced by newer drugs, including biologics, accompanied by their own syndromes and unintended side effects. Conclusions. Drugs can induce toxicities spanning many hematologic syndromes, mediated by a variety of mechanisms. Physicians need to be alert to the potential for iatrogenic drug-induced hematologic complications.
Mintzer, David M.; Billet, Shira N.; Chmielewski, Lauren
REVERSIBLE posterior leukoencephalopathy syndrome (RPLS) is a rare neurological syndrome characterized by headache, altered mental status, seizures, and visual disturbance, associated with reversible white matter changes.1 It has been commonly reported in patients with severe hypertension and pre-eclampsia. Here we report a case with nephrotic syndrome complicated by RPLS. PMID:24698681
Liu, Sheng-da; Shen, Qing-min; Lv, Chun-feng
NINDS Asperger Syndrome Information Page Condensed from Asperger Syndrome Fact Sheet Table of Contents (click to jump to sections) ... Trials Organizations Additional resources from MedlinePlus What is Asperger Syndrome? Asperger syndrome (AS) is a developmental disorder. ...
NINDS Cushing's Syndrome Information Page Synonym(s): Hypercortisolism Table of Contents (click to jump to sections) What is Cushing's Syndrome? Is ... is being done? Clinical Trials Organizations What is Cushing's Syndrome? Cushing's syndrome, also called hypercortisolism , is a rare ...
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
Following the death of a loved one, a small group of grievers develop an abnormal grieving style, termed complicated or prolonged grief. In the effort to establish complicated grief as a disorder in DSM and ICD, several attempts have been made over the past two decades to establish symptom criteria for this form of grieving. Complicated grief is different from depression and PTSD yet often comorbid with other psychological disorders. Meta-analyses of grief interventions show small to medium effect sizes, with only few studies yielding large effect sizes. In this article, an integrative cognitive behavioral treatment manual for complicated grief disorder (CG-CBT) of 25 individual sessions is described. Three treatment phases, each entailing several treatment strategies, allow patients to stabilize, explore, and confront the most painful aspects of the loss, and finally to integrate and transform their grief. Core aspects are cognitive restructuring and confrontation. Special attention is given to practical exercises. This article includes the case report of a woman whose daughter committed suicide.
Rosner, Rita; Pfoh, Gabriele; Kotoucova, Michaela
Pulmonary complications are prevalent in the critically ill neurological population. Respiratory failure, pneumonia, acute lung injury and the acute respiratory distress syndrome (ALI/ARDS), pulmonary edema, pulmonary contusions and pneumo/hemothorax, and pulmonary embolism are frequently encountered in the setting of severe brain injury. Direct brain injury, depressed level of consciousness and inability to protect the airway, disruption of natural defense barriers, decreased mobility, and secondary neurological insults inherent to severe brain injury are the main cause of pulmonary complications in critically ill neurological patients. Prevention strategies and current and future therapies need to be implemented to avoid and treat the development of these life-threatening medical complications.
Lee, Kiwon; Rincon, Fred
The metabolic syndrome is defined as a constellation of metabolic abnormalities, including glucose intolerance, obesity, dyslipidaemia, and hypertension. It is associated with an elevated risk for cardiovascular disease and type 2 diabetes. Over the past 20 years, a worldwide increase of the metabolic syndrome has taken place. In the USA, 21% to 39% of the general population suffer from metabolic syndrome; in Europe prevalence rates are lower. At the same time, depressive disorders, which are associated with a 1.5 to 2.5 fold risk for cardiovascular events, are increasing. The combination of metabolic syndrome and depression multiplies the risk for severe complications: Due to behavioural, physiological, genetic, and treatment-induced factors, depression stimulates the development of a metabolic syndrome. In reverse, the metabolic syndrome favours development and maintenance of the metabolic syndrome. Consequently, in patients suffering from metabolic syndrome and depression, both disorders must be treated simultaneously. The treatment plan includes weight reduction, physical activity, psychoeducation, family interventions, the patient's subjective explanatory and treatment models, cognitive techniques, and problem solving techniques. Weight, blood pressure, fastening glucose, haemoglobin A1c, and lipids must be controlled in order to monitor treatment progress. Additional drug treatment can be appropriate for hypertension, dyslipidaemia, hyperglycaemia, and depression. Under these conditions, sustained improvements of individual well-being, psychosocial risk-factors, functional limitations, glycemic control, hypertension, and obesity are possible. PMID:16941396
Löwe, B; Hochlehnert, A; Nikendei, C
Dettol is involved in 10% of self-poisoning-related hospital admissions in Hong Kong. Although serious poisonings and even deaths after ingesting this common household disinfectant have been reported, the frequency with which these complications may occur is not known. In a retrospective study of 67 cases of Dettol poisoning, we found that serious complications were relatively common (8%) and these included aspiration of Dettol with gastric contents resulting in pneumonia, cardiopulmonary arrest, bronchospasm, adult respiratory distress syndrome, and severe laryngeal oedema with upper airways obstruction. It is particularly important that the airways are adequately protected before the patient is lavaged following Dettol poisoning, and the immediate recognition of any upper airway obstruction requiring intubation will then be life-saving. PMID:8265774
Chan, T Y; Lau, M S; Critchley, J A
Infection, graft-versus-host disease (GVHD), and to a lesser extent sinusoidal obstructive syndrome (SOS) represent the major causes of morbidity and mortality in patients undergoing allogeneic hematopoietic stem cell transplantation (AHSCT). During the last decade, progress in prevention and treatment of these complications led to improvement in the outcome of these patients. Despite the fact that nonmyeloablative regimens have been increasingly used in elderly patients and in patients with co-morbidities, the nonrelapse related mortality remains a challenge and long-term follow-up is required. The objective of this manuscript is to provide an updated concise review of the complications of AHSCT and of the available treatment interventions. PMID:24902046
Arnaout, Karim; Patel, Nihar; Jain, Maneesh; El-Amm, Joelle; Amro, Farah; Tabbara, Imad A
Primary infection with varicella zoster is characterized by a generalized vesicular rash usually without significant systemic\\u000a illness. Encephalitis, pneumonitis, pancreatitis, nephritis, Reye and Guillan-Barre syndrome transvers myelitis, myocarditis\\u000a have been reported before, but there is not any case having all these system to be involved during the same infection in a\\u000a sequential manner ending up with multiorgan failure. We wanted
Kivilcim Gticiiyenel; Elvan Çaglar Çitak; Murat Elli; Ayse Serdaroglu; Funda Erkasar Çitak
Primary infection with varicella zoster is characterzed by a generalized vesicular rash usually without significant systemic illness. Encephalitis, pneumonitis, pancreatitis, nephritis, Reye and Guillan-Barre syndrome transvers myelitis, myocarditis have been reported before, but there is not any case having all these system to be involved during the same infection in a sequential manner ending up with multiorgan failure. We wanted to represent 21-month-old boy had a multiorgan failure due to varicella zoster infection. PMID:11929039
Gücüyener, Kivilcim; Citak, Elvan Ca?lar; Elli, Murat; Serdaro?lu, Ayse; Citak, Funda Erkasar
In children with medically refractory gastroesophageal reflux disease (GERD), fundoplication is effective and safe. However,\\u000a in a subset of patients, gastrointestinal dysfunction occurs postoperatively. Symptoms include chest pain, persistent dysphagia\\u000a in 5%, gas bloat in 2% to 4%, diarrhea in up to 20%, and dumping syndrome in up to 30%. Symptoms are often nonspecific, arising\\u000a from recurrent or persistent GERD,
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
Silicone oil tamponade is used for the management of complicated retinal detachment. Patients should be closely monitored as many complications may occur with intraocular silicone oil. Short-term complications include temporarily increased intraocular pressure and anterior segment inflammation. Long-term complications include cataract, emulsification, ocular hyper- or hypotension, keratopathy and retinal redetachment associated with proliferative vitreoretinopathy. PMID:24559527
Baillif, S; Gastaud, P
Bariatric surgical procedures are increasingly common. In this review, we characterize the neurologic complications of such procedures, including their mechanisms, frequency, and prognosis. Literature review yielded 50 case reports of 96 patients with neurologic symptoms after bariatric procedures. The most common presentations were peripheral neuropathy in 60 (62%) and encephalopathy in 30 (31%). Among the 60 patients with peripheral neuropathy, 40 (67%) had a polyneuropathy and 18 (30%) had mononeuropathies, which included 17 (94%) with meralgia paresthetica and 1 with foot drop. Neurologic emergencies including Wernicke's encephalopathy, rhabdomyolysis, and Guillain-Barré syndrome were also reported. In 18 surgical series reported between 1976 and 2004, 133 of 9996 patients (1.3%) were recognized to have neurologic complications (range: 0.08-16%). The only prospective study reported a neurologic complication rate of 4.6%, and a controlled retrospective study identified 16% of patients with peripheral neuropathy. There is evidence to suggest a role for inflammation or an immunologic mechanism in neuropathy after gastric bypass. Micronutrient deficiencies following gastric bypass were evaluated in 957 patients in 8 reports. A total of 236 (25%) had vitamin B(12) deficiency and 11 (1%) had thiamine deficiency. Routine monitoring of micronutrient levels and prompt recognition of neurological complications can reduce morbidity associated with these procedures. PMID:15973660
Koffman, Boyd M; Greenfield, L John; Ali, Imran I; Pirzada, Noor A
The central nervous system (CNS) and peripheral nervous system (PNS) are very susceptible to cancer and its treatment. The most direct involvement of the nervous system manifests in the development of primary brain and spinal cord tumors. Many cancers exhibit a propensity toward spread to the CNS, and brain metastases are common problems seen in malignancies such as lung, breast, and melanoma. Such spread may involve the brain or spine parenchyma or the subarachnoid space. In the PNS, spread is usually through direct infiltration of nerve roots, plexi, or muscle by neighboring malignancies. In some cases, cancer has sudden, devastating effects on the nervous system: epidural spinal cord compression or cord transection from pathologic fractures of vertebra involved by cancer; increased intracranial pressure from intracranial mass lesion growth and edema; and uncontrolled seizure activity as a result of intracranial tumors (status epilepticus), which are neuro-oncologic emergencies. The best known indirect or remote effects of cancer on the nervous system are the neurologic paraneoplastic syndromes. Cancer can also result in a hypercoagulable state causing cerebrovascular complications. Treatment of cancer can have neurologic complications. The commonest of these complications are radiation-induced injury to the brain, spine, and peripheral nerves and chemotherapy-induced peripheral neuropathy. The suppressant effect of cancer and its treatment on the body’s immune system can result in infectious complications within the nervous system.
Nine patients are described in whom psychopharmacotherapy was associated with the development of rare complications in the form of the syndrome of cortical disturbances, psychosensory disorders and toxic polyneuropathy. All patients presented residual organic insufficiency of the CNS, with some of them exhibiting endocrine-metabolic disorders as well. PMID:2992192
Niss, A I; Trubkovich, M Ia
Myhre syndrome (MS) is a developmental disorder characterized by typical facial dysmorphism, thickened skin, joint limitation and muscular pseudohypertrophy. Other features include brachydactyly, short stature, intellectual deficiency with behavioral problems and deafness. We identified SMAD4 as the gene responsible for MS. The identification of SMAD4 mutations in Laryngotracheal stenosis, Arthropathy, Prognathism and Short stature (LAPS) cases supports that LAPS and MS are a unique entity. The long-term follow up of patients shows that these conditions are progressive with life threatening complications. All mutations are de novo and changing in the majority of cases Ile500, located in the MH2 domain involved in transcriptional activation. We further showed an impairment of the transcriptional regulation via TGF? target genes in patient fibroblasts. Finally, the absence of SMAD4 mutations in three MS cases may support genetic heterogeneity. PMID:24580733
Le Goff, C; Michot, C; Cormier-Daire, V
Hypospadias is the most common congenital abnormality of the lower urinary tract affecting one of 300 male newborns. More than 300 different surgical hypospadias repair techniques have been described. Currently, tubularized incised plate and meatal advancement and glansplasty integrated repair are the preferred techniques for distal hypospadias, whereas two-staged procedures are most frequently used in proximal forms. Success rates are high in the hands of dedicated surgeons, although studies on long-term results are sparse. The most frequent complications of hypospadias repairs include urethrocutaneous fistulas, meatal stenosis, and urethral strictures. Urological follow-up into puberty is warranted, as well as further studies with standardized reporting of long-term results and complications. PMID:25023236
Soave, A; Riechardt, S; Engel, O; Rink, M; Fisch, M
OBJECTIVE: Outpatient drug complications have not been well studied. We sought to assess the incidence and characteristics of outpatient\\u000a drug complications, identify their clinical and nonclinical correlates, and evaluate their impact on patient satisfaction.\\u000a \\u000a \\u000a DESIGN: Retrospective chart reviews and patient surveys.\\u000a \\u000a \\u000a \\u000a \\u000a SETTING: Eleven Boston-area ambulatory clinics.\\u000a \\u000a \\u000a \\u000a \\u000a PATIENTS: We randomly selected 2,248 outpatients, 20 to 75 years old.\\u000a \\u000a \\u000a \\u000a \\u000a MEASUREMENTS AND MAIN
Tejal K. Gandhi; Helen R. Burstin; E. Francis Cook; Ann L. Puopolo; Jennifer S. Haas; Troyen A. Brennan; David W. Bates
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
Neuropathy as a complication of diabetes is common and presents in a wide variety of clinical scenarios. Often the work-up is one of exclusion tempered with monitoring the response of symptoms to treatment options. The collaboration of a neurologist is often crucial to determining the best course of action for the patient. This review will address proposed pathogenic mechanisms and potential therapeutic interventions, both pharmacologic and nonpharmacologic. PMID:24817097
Charnogursky, Gerald A; Emanuele, Nicholas V; Emanuele, Mary Ann
This study was undertaken to assess the appropriate management of patients with diverticulitis complicated by fistula formation.\\u000a A retrospective chart review was conducted on patients with symptoms of a fistula who presented between 1975 to 1995. There\\u000a were 42 patients (32 women, 76%; 10 men, 24%) who ranged in age from 46 to 89 years (mean 69.8±9.8). Six patients had
C.-A. Vasilevsky; P. Belliveau; J. L. Trudel; B. L. Stein; P. H. Gordon
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.
Respiratory complications of blood transfusion have several possible causes. Transfusion-Associated Circulatory Overload (TACO) is often the first mentioned. Transfusion-Related Acute Lung Injury (TRALI), better defined since the consensus conference of Toronto in 2004, is rarely mentioned. French incidence is low. Non-hemolytic febrile reactions, allergies, infections and pulmonary embolism are also reported. The objective of this work was to determine the statistical importance of the different respiratory complications of blood transfusion. This work was conducted retrospectively on transfusion accidents in six health centers in Champagne-Ardenne, reported to Hemovigilance between 2000 and 2009 and having respiratory symptoms. The analysis of data was conducted by an expert committee. Eighty-three cases of respiratory complications are found (316,864 blood products). We have counted 26 TACO, 12 TRALI (only 6 cases were identified in the original investigation of Hemovigilance), 18 non-hemolytic febrile reactions, 16 cases of allergies, 5 transfusions transmitted bacterial infections and 2 pulmonary embolisms. Six new TRALI were diagnosed previously labeled TACO for 2 of them, allergy and infection in 2 other cases and diagnosis considered unknown for the last 2. Our study found an incidence of TRALI 2 times higher than that reported previously. Interpretation of the data by a multidisciplinary committee amended 20% of diagnoses. This study shows the imperfections of our system for reporting accidents of blood transfusion when a single observer analyses the medical records. PMID:24814817
Bernasinski, M; Mertes, P-M; Carlier, M; Dupont, H; Girard, M; Gette, S; Just, B; Malinovsky, J-M
Facial beauty depends on shape, proportion, and harmony between the facial thirds. The chin is one of the most important components of the inferior third and has an important role on the definition of facial aesthetic and harmony in both frontal and lateral views. There are 2 principal therapeutic approaches that one can choose to treat mental deformities, alloplastic implants, and mental basilar ostectomy, also known as genioplasty. The latest is more commonly used because of great versatility in the correction of three-dimensional deformities of the chin and smaller taxes of postoperative complications. Possible transoperative and postoperative complications of genioplasty include mental nerve lesion, bleeding, damage to tooth roots, bone resorption of the mobilized segment, mandibular fracture, ptosis of the lower lip, and failure to stabilize the ostectomized segment. The study presents 2 cases of displacement of the osteotomized segment after genioplasty associated with facial trauma during postoperative orthognathic surgery followed by rare complications with no reports in the literature. PMID:24621765
Avelar, Rafael Linard; Sá, Carlos Diego Lopes; Esses, Diego Felipe Silveira; Becker, Otávio Emmel; Soares, Eduardo Costa Studart; de Oliveira, Rogerio Belle
Cutaneous drug reactions are frequent in hospitalized patients and vary from simple manifestations like rash and erythema to severe life threatening conditions like angio-oedema, erythroderma, Stevens-Johnson syndrome and toxic epidermal necrolysis. However drug eruptions with antitubercular drugs are largely unknown except few case reports. We highlight here one similar case which presented with pleomorphic cutaneous manifestations after taking anti tubercular therapy and closely mimicked vasculitis. But when the offending drugs were stopped the lesions disappeared and the patient improved.
Aggarwal, Ramesh; Dwivedi, Shridhar; Aggarwal, Meenakshi
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
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
Hepatic hydrothorax secondary to transdiaphragmatic spread of peritoneal fluid can cause respiratory discomfort to the patient. Draining of hydrothorax helps relieve these symptoms. Pleurovenous shunt (Denver shunt) is a relatively non-invasive method of shunting the pleural fluid to the central venous system. Reported complications of pleurovenous shunts are shunt failure, pulmonary edema, post shunt coagulopathy, deep vein thrombosis, and infection. We report a rare case of a leak at the venous end of the catheter that was placed within the right internal jugular vein, resulting in a large collection in the neck.
Perera, Eranga; Bhatt, Shweta; Dogra, Vikram S.
Brain death is associated with complex hemodynamic, endocrine, and metabolic dysfunction that can lead to major complications with the potential donor. Untreated, this can progress to cardiovascular collapse with loss of valuable organs for transplantation. We hypothesized that brain death-related complications would have no effect on the number of organs donated if an aggressive donor management protocol was in place. We identified all successful organ donations between January 2000 and December 2003 and evaluated them for brain death-associated complications (defined as vasopressor requirement, coagulopathy, diabetes insipidus, cardiac ischemia, lactic acidosis, renal failure, and acute respiratory distress syndrome) and donated organs per donor. Sixty-nine organ donors were identified. Complications identified were as follows: intravenous vasopressor requirement in 97.1 per cent, coagulopathy in 55.1 per cent, thrombocytopenia in 53.6 per cent, diabetes insipidus in 46.4 per cent, cardiac ischemia in 30.4 per cent, lactic acidosis in 24.6 per cent, renal failure in 20.3 per cent, and acute respiratory distress syndrome in 13 per cent. There was no significant effect of complications on the average number of organs harvested, with the exception of an increase in organs harvested in the presence of diabetes insipidus. With the implementation of an aggressive organ donor management protocol, these complications can be effectively managed with no impact on the number of organs harvested for transplant. PMID:16719188
Salim, Ali; Martin, Matthew; Brown, Carlos; Belzberg, Howard; Rhee, Peter; Demetriades, Demetrios
Guillain-Barré syndrome (GBS) represents a diverse spectrum of diseases, with variable pathophysiological mechanisms, clinical manifestation, presentation pattern, and degree of severity. We report a rare case of fatal fulminant GBS complicated with myocarditis during the course of illness.
Mishra, Ajay; Dave, Nikhil; Mehta, Manan
As hip arthroscopy becomes a more common procedure, more complications may occur. We present a case of abdominal compartment syndrome resulting from fluid extravasation in a 42-year-old man who underwent routine hip arthroscopy for femoral acetabular impi...
B. D. Owens J. Fowler
Infectious dental complications are quite frequent given the high incidence of tooth decay. Other pathologies not related to dental decay can cause mouth infectious such as periodontal infections, alveolitis, peri-coronitis of impacted wisdom tooth and secondary infection of dental fragments. Dental infection can be localised to apex (top of the root) or progress to soft and bony tissues surrounding the teeth. The most frequent germs involved in these infections are generally the same as saprophyte buccal flora but are often associated to other anaerobic germs. The following complications will be described in what concerns their etiology, pathology and treatment: abcess, fistula, phlegmon and cellulitis, odontogenic cysts, actinomycosis, craniofacial thrombophlebitis, osteitis and osteomyelitis, maxillary sinusitis, septicemia and local odontogenic infections. The consequences of these infections can vary according to immunologic resistance of the patient as well as the resistance of some germs to the most common antibiotics. Several factors should be taken in consideration in the treatment; patient history factors, germ virulence, maintaining or suppression of etiologic factors and drainage possibilities. PMID:11680190
Slit ventricle syndrome is well known as a complication in the treatment of hydrocephalus by shunting. It is generally considered to be a chronic (but not acute) complication, occurring years after the shunt procedure; there has been no report of this syndrome occurring before 1 year of age. The authors present infantile cases that developed a severe form of this
Shizuo Oi; Satoshi Matsumoto
Chemosis is a rare and reversible complication of nephrotic syndrome in children. However, it is not a threatening condition\\u000a as there are no complications of its own. We report a 15-year-old girl with steroid-resistant nephrotic syndrome with bilateral\\u000a chemosis recovering along with the disappearance of the anasarca.
Augustina Jankauskiene; Inga Buteikiene
Ovarian hyperstimulation syndrome (OHSS) is a rare iatrogenic complication of ovarian stimulation occurring during the luteal phase or during early pregnancy. Fortunately, the reported prevalence of the severe form of OHSS is small, ranging from 0.5 to 5%. Nevertheless, as this is an iatrogenic complication of a non-vital treatment with a potentially fatal outcome, the syndrome remains a serious problem
Annick Delvigne; Serge Rozenberg
Jacobsen syndrome is a MCA/MR contiguous gene syndrome caused by partial deletion of the long arm of chromosome 11. To date, over 200 cases have been reported. The prevalence has been estimated at 1/100,000 births, with a female/male ratio 2:1. The most common clinical features include pre- and postnatal physical growth retardation, psychomotor retardation, and characteristic facial dysmorphism (skull deformities, hypertelorism, ptosis, coloboma, downslanting palpebral fissures, epicanthal folds, broad nasal bridge, short nose, v-shaped mouth, small ears, low set posteriorly rotated ears). Abnormal platelet function, thrombocytopenia or pancytopenia are usually present at birth. Patients commonly have malformations of the heart, kidney, gastrointestinal tract, genitalia, central nervous system and skeleton. Ocular, hearing, immunological and hormonal problems may be also present. The deletion size ranges from approximately 7 to 20 Mb, with the proximal breakpoint within or telomeric to subband 11q23.3 and the deletion extending usually to the telomere. The deletion is de novo in 85% of reported cases, and in 15% of cases it results from an unbalanced segregation of a familial balanced translocation or from other chromosome rearrangements. In a minority of cases the breakpoint is at the FRA11B fragile site. Diagnosis is based on clinical findings (intellectual deficit, facial dysmorphic features and thrombocytopenia) and confirmed by cytogenetics analysis. Differential diagnoses include Turner and Noonan syndromes, and acquired thrombocytopenia due to sepsis. Prenatal diagnosis of 11q deletion is possible by amniocentesis or chorionic villus sampling and cytogenetic analysis. Management is multi-disciplinary and requires evaluation by general pediatrician, pediatric cardiologist, neurologist, ophthalmologist. Auditory tests, blood tests, endocrine and immunological assessment and follow-up should be offered to all patients. Cardiac malformations can be very severe and require heart surgery in the neonatal period. Newborns with Jacobsen syndrome may have difficulties in feeding and tube feeding may be necessary. Special attention should be devoted due to hematological problems. About 20% of children die during the first two years of life, most commonly related to complications from congenital heart disease, and less commonly from bleeding. For patients who survive the neonatal period and infancy, the life expectancy remains unknown. PMID:19267933
Mattina, Teresa; Perrotta, Concetta Simona; Grossfeld, Paul
The aim of this study was to investigate the complications following surgical treatment of thyroid cancer and the association between the extent of surgery and complication rates. A total of 2,636 patients who underwent surgery due to thyroid cancer were retrospectively reviewed to identify surgical complications. Complication rates were assessed according to the extent of surgery, which was classified as follows; less-than-total thyroidectomy with central compartment node dissection (CCND) (Group I, n=636), total thyroidectomy with CCND (Group II, n=1,390), total thyroidectomy plus ipsilateral neck dissection (Group III, n=513), and total thyroidectomy plus bilateral neck dissection (Group IV, n=97). The most common surgical complication was symptomatic hypoparathyroidism, of which 28.4% of cases were transient and 0.3% permanent. The other surgical complications included vocal cord palsy (0.7% transient, and 0.2% permanent), hematoma (0.5%), seroma (4.7%), chyle fistula (1.8%), and Horner's syndrome (0.2%). The complication rates increased significantly with increasing the extent of surgery from Group I to Group IV. The more extensive surgery makes more complications, such as hypoparathyroidism, seroma, and others.
Lee, Yong Sang; Nam, Kee-Hyun; Chung, Woong Youn; Park, Cheong Soo
Tonic dystonia is an underrecognized complication of reflex sympathetic dystrophy syndrome (RSDS) characterized by an increase in muscle tone at the site of injury.Case-reports. – We describe five cases of tonic dystonia complicating RSDS of the lower extremity. There were four women and one man, with a mean age of 52 years. In addition to the typical features of RSDS, the
Aude Morelet; Laurence Gagneux-Lemoussu; Pascal Brochot; Stanley Ackah-Miezan; Jean-François Colmet-Daage; François Gaillard; François Boyer; Jean-Paul Eschard; Jean-Claude Etienne
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
Conclusion: The rate of severe complications was low and cochlear implantation is a relatively safe procedure. Standardization is crucial when reporting on cochlear implant complications to ensure comparability between studies. A consensus on the reporting of complications proposed by a Danish team of researchers was applied, evaluated and found beneficial. Objectives: To report the surgical complications following cochlear implantation at our centre, applying and evaluating a proposed reporting consensus. Methods: A retrospective file review of 308 consecutive adult implantations in 269 patients between 1994 and 2010 at Odense University Hospital was performed. Results: The three most common major complications were wound infection (1.6%), permanent chorda tympani syndrome (1.6%) and electrode migration/misplacement/accidental removal (1.3%). Permanent facial nerve paresis occurred following one implantation (0.3%). Transient chorda tympani syndrome (30.8%), vertigo/dizziness (29.5%) and tinnitus (4.9%) were the most frequent minor complications.
Faber, Christian Emil
The myelodysplastic syndromes represent a heterogeneous series of clonal hematologic neoplasms characterized by morphologic dysplasia, aberrant hematopoiesis and a variable risk of progression to acute myeloid leukemia. These syndromes have a complex pathobiology, and ineffective hematopoiesis is a well-recognized feature of all of them. Normal blood cell maturation, differentiation, function, and survival are impaired, and these abnormalities contribute to the development of peripheral blood pancytopenia. The majority of patients succumb to complications of either bone marrow failure or leukemic progression. The fact that the majority of patients are elderly and have other comorbidities complicates therapeutic decision making and necessitates the development of individualized treatment strategies. PMID:21717901
Although endotracheal intubation is commonly performed in the hospital setting, it is not without risk. In this article, we review the impact of endotracheal intubation on airway injury by describing the acute and long-term sequelae of each of the most commonly injured anatomic sites along the respiratory tract, including the nasal cavity, oral cavity, oropharynx, larynx, and trachea. Injuries covered include nasoseptal injury, tongue injury, dental injury, mucosal lacerations, vocal cord immobility, and laryngotracheal stenosis, as well as tracheomalacia, tracheoinnominate, and tracheoesophageal fistulas. We discuss the proposed mechanisms of tissue damage that relate to each and present their most common clinical manifestations, along with their respective diagnostic and management options. This article also includes a review of complications of airway management pertaining to video laryngoscopy and supraglottic airway devices. Finally, potential strategies to prevent intubation-associated injuries are outlined. PMID:24891204
Pacheco-Lopez, Paulette C; Berkow, Lauren C; Hillel, Alexander T; Akst, Lee M
Opinion statement The treatment of severe, complicated pancreatitis requires:\\u000a \\u000a \\u000a \\u000a – \\u000a \\u000a Rapid assessment and recognition of severity, determined by using APACHE or equivalent scoring systems.\\u000a \\u000a \\u000a \\u000a \\u000a – \\u000a \\u000a Aggressive resuscitation with crystalloid and colloid solutions using central venous monitoring for guidance, and to help\\u000a identify and treat early organ failure.\\u000a \\u000a \\u000a \\u000a \\u000a – \\u000a \\u000a Nutritional support including total parenteral feeding, which can assist in maintaining nutrition during
Stephen P. Martin; Charles D. Ulrich
Recognition and management of gastrointestinal and hepatic complications of hematopoietic stem cell transplantation has gained increasing importance as indications and techniques of transplantation have expanded in the last few years. The transplant recipient is at risk for several complications including conditioning chemotherapy related toxicities, infections, bleeding, sinusoidal obstruction syndrome, acute and chronic graft-versus-host disease (GVHD) as well as other long-term problems. The severity and the incidence of many complications have improved in the past several years as the intensity of conditioning regimens has diminished and better supportive care and GVHD prevention strategies have been implemented. Transplant clinicians, however, continue to be challenged with problems arising from human leukocyte antigen-mismatched and unrelated donor transplants, expanding transplant indications and age-limit. This review describes the most commonly seen transplant related complications, focusing on their pathogenesis, differential diagnosis and management.
Tuncer, Hande H; Rana, Naveed; Milani, Cannon; Darko, Angela; Al-Homsi, Samer A
Abstract Background and Purpose: Because of recent advances in minimally invasive surgical techniques, robot-assisted radical prostatectomy (RARP) has become the primary treatment option in prostate cancer. RARP, however, necessitates patients to be placed in a steep Trendelenberg position, which presents multiple opportunities for complications relating to the positioning of the patient. Our study aims to study the prevalence and demographic predictors of these positioning complications and assess their impacts on length of stay (LOS) and total cost. Patients and Methods: We included patients who underwent RP from 2008 to 2009 using data extracted from the Nationwide Inpatient Sample database. Positioning complications (eye, nerve, compartment syndrome/rhabdomyolysis) were identified using patient-level diagnosis and procedural International Classification of Disease, 9th edition, Clinical Modification codes. Logistic regression models assessed relationships between demographic factors and occurrence of complications and the effects of them on prolonged LOS and total inpatient cost. Results: Positioning complications occurred in 0.4% of cases with eye complications contributing the most to this frequency. Laparoscopic RP procedure (odds ratio [OR]=2.88, P<0.01) and comorbidities (OR=2.34, P<0.01) were highly associated with increased odds of positioning complication occurrence, whereas RARP procedures (OR=0.93, P>0.4) were not associated with positioning complications. Having positioning complications increased a patient's odds of having increased inpatient costs and extended LOS by almost 400% and 300%, respectively. Conclusion: The steep Trendelenberg position used in RARP was not shown to be associated with patient positioning-related complications in this sample. The occurrence of positioning-related complications, however, places huge burdens on total inpatient costs and LOS. PMID:24428586
Wen, Timothy; Deibert, Christopher M; Siringo, Frank S; Spencer, Benjamin A
Background\\/Aims: Tumor lysis syndrome is a challenging complication of cancer therapy. This review focuses on the risk factors and pathologies of patients at risk for hyperuricemic complications. Methods: A review of the literature was performed that included original articles and related reviews from MEDLINE (PubMed) and published abstracts of meeting presentations. Results: Both host-related and tumor-related factors predispose cancer patients
A. Tsimberidou; M. J Keating
The Marfan syndrome is an autosomal dominant disorder of connective tissue, caused by mutations in the FBN1 gene on chromosome 15. More than 500 mutations have been identified and almost all are unique to an affected individual or family. Genotype–phenotype correlations in the Marfan syndrome have been complicated by the large number of unique mutations reported, as well as by
G. J. Nollen; B. J. M. Mulder
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
Objective: To assess the incidence and type of laparoscopic complications. Methods: A series of 2140 operative laparoscopies were reviewed in a retrospective study of patient records. The setting was a tertiary-care university hospital. Operative laparoscopy included minor procedures (minimal adhesiolysis, destruction of minimal endometriosis foci, ovarian biopsy, ovarian puncture, tubal sterilization), major laparoscopic surgery (extended adhesiolysis, tuboplasties, uterine suspension, treatment for ectopic pregnancy, salpingitis, ovarian cyst, moderate and severe endometriosis), and advanced laparoscopic surgery (hysterectomy, myomectomy, bladder neck suspension). Results: Two major vascular complications, 3 intestinal injuries, 1 anesthesiological complication, and 4 urinary tract injuries occurred. Two minor and 5 postoperative complications were noted. The overall complication rate was 17/2140 (0.79%). The major complication rate was 10/2140 (0.46%). Conclusions: This review is useful for helping surgeons reduce the risk of injuries and to inform patients about potential complications. These rates are similar to those that have been previously reported.
Carvajal, Antonio R.
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
Post-transplant hemolytic uremic syndrome characterized by microangiopathic hemolysis, thrombocytopenia, and renal failure is an infrequent but potentially serious complication in organ transplant recipients. Hemolytic uremic syndrome developed in 2% (2\\/100) of our consecutive liver transplants. We report our patients and review a total of 91 cases of hemolytic uremic syndrome in adult solid organ transplant recipients reported in the literature.
Nina Singh; Timothy Gayowski; Ignazio R. Marino
Obesity is a health problem of considerable magnitude in the Western world. Dermatological changes have been reported in patients with obesity, including: acanthosis nigricans and skin tags (due to insulin resistance); hyperandrogenism; striae due to over extension; stasis pigmentation due to peripheral vascular disease; lymphedema; pathologies associated with augmented folds; morphologic changes in the foot anatomy due to excess load; and complications that may arise from hospitalization. Acanthosis nigricans plaques can be managed by improved control of hyperinsulinemia; the vitamin D3 analog calcipitriol has also been shown to be effective. Skin tags can be removed by snipping with curved scissors, by cryotherapy or by electrodesiccation. Hyperandrogenism, a result of increased production of endogenous androgens due to increased volumes of adipose tissue (which synthesizes testosterone) and hyperinsulinemia (which increases the production of ovarian androgens) needs to be carefully assessed to ensure disorders such as virilizing tumors and congenital adrenal hyperplasia are treated appropriately. Treatment of hyperandrogenism should be centred on controlling insulin levels; weight loss, oral contraceptive and antiandrogenic therapies are also possible treatment options. The etiology of striae distensae, also known as stretch marks, is yet to be defined and treatment options are unsatisfactory at present; striae rubra and alba have been treated with a pulsed dye laser with marginal success. The relationship between obesity and varicose veins is controversial; symptoms are best prevented by the use of elastic stockings. Itching and inflammation associated with stasis pigmentation, the result of red blood cells escaping into the tissues, can be treated with corticosteroids. Lymphedema is associated with dilatation of tissue channels, reduced tissue oxygenation and provides a culture medium for bacterial growth. Lymphedema treatment is directed towards reducing the limb girth and weight, and the prevention of infection. Intertrigo is caused by friction between skin surfaces, combined with moisture and warmth, resulting in infection. This infection, most commonly candidiasis, is best treated with topical antifungal agents; systemic antifungal therapy may be required in some patients. Excess load on the feet can result in morphological changes that require careful diagnosis; insoles may offer some symptom relief while control of obesity is achieved. Obesity-related dermatoses associated with hospitalization, such as pressure ulcers, diminished wound healing, dermatoses secondary to respiratory conditions, and incontinence, must all be carefully managed with an emphasis on prevention where possible. Recognition and control of the dermatological complications of obesity play an important role in diminishing the morbidity of obesity. PMID:12180897
García Hidalgo, Linda
Pathologic consequence of tattooing is relatively rare, but in light of the great number of persons in military service, where tattooing seems to be popular, consideration of the complications seems worth while. Four unusual cases of patients with cutaneous lesions in tattoos are reported. One patient had Boeck's sarcoid, in which the cutaneous eruption was limited solely to the tattooed areas on the body and involved all the various colors of the tattoo. The cutaneous eruption was only a single manifestation of generalized sarcoidosis. Another patient had secondary syphilis with the cutaneous lesions involving multi-colored tattoos, including the red areas. These tattoos were applied in Hawaii, and presumably the red dye was not the usual cinnabar (mercuric sulfide). As a general rule, the cutaneous lesions of secondary syphilis do not involve the red areas because of the treponomicidal action of mercuric sulfide. In the third case the characteristic eruption of discoid lupus erythematosus was present. It began over the red areas of a tattoo on the patient's forearm. It has been stated that mercuric sulfide is a photo-sensitizing agent, and it is believed that this explains the localization of the eruption in this case. The fourth patient had eruption, caused by mercury sensitivity, in an area of tattoo. The eruption was noted soon after the tattooing was done, and it persisted for seven years.
Lubeck, Glenn; Epstein, Ervin
Intravenous immunoglobulin [IVIg] is useful for treating several clinical conditions and is largely considered safe, without major adverse events. Here we report a case of acute ST elevation myocardial infarction associated with high dose IVIg administration in a previously healthy 69-year-old male patient of Guillain Barre syndrome. The case is being reported to emphasize the need for treating physicians to be aware of thrombotic complications associated with IVIg. The thrombotic complications associated with IVIg are reviewed in brief, and the measures to reduce them are discussed.
Vinod, Kolar Vishwanath; Kumar, Mritunjai; Nisar, Kare Kadavath
Intravenous immunoglobulin [IVIg] is useful for treating several clinical conditions and is largely considered safe, without major adverse events. Here we report a case of acute ST elevation myocardial infarction associated with high dose IVIg administration in a previously healthy 69-year-old male patient of Guillain Barre syndrome. The case is being reported to emphasize the need for treating physicians to be aware of thrombotic complications associated with IVIg. The thrombotic complications associated with IVIg are reviewed in brief, and the measures to reduce them are discussed. PMID:24872657
Vinod, Kolar Vishwanath; Kumar, Mritunjai; Nisar, Kare Kadavath
Fractures of the distal radius and the scaphoid are common injuries in adults. In recent years, surgical fixation of these types of fractures has increased in response to improved patient outcomes and evolving fixation techniques. Potential soft-tissue, neurovascular, or osseous complications, including tendon injuries, carpal tunnel syndrome, loss of fracture reduction, and osteonecrosis, can increase the time the patient requires immobilization and can lead to poor patient outcomes. Prompt recognition and diagnosis of these complications may improve patient outcomes and satisfaction. PMID:24720299
Meyer, Carissa; Chang, James; Stern, Peter J; Osterman, A Lee; Abzug, Joshua M
Introduction Among the various drug induced dermatological entities toxic epidermal necrolysis and Stevens-Johnson’s syndrome occupy a primary place in terms of mortality. Prompt recognition of these conditions, immediate drug withdrawal and institution of appropriate treatment plays a vital role in reducing mortality. Drugs are by far the most common cause of toxic epidermal necrolysis, in which large sheets of skin are lost from the body surface making redundant the barrier function of the skin, with its resultant complications. The use of systemic corticosteroids in the treatment of toxic epidermal necrolysis has always been controversial, some consider corticosteroids life-saving while others believe that they increase mortality. Case presentation We describe two cases of drug-induced toxic epidermal necrolysis, a male and a female, both caucasoids of Pakistani origin, one treated without any steroids and the other with them, who made complete recovery without any major complications or sequelae. Conclusion The administration of systemic corticosteriods did not cause any major changes in outcome in our cases.
Raza, Naeem; Qadir, Fozi
The aim of this retrospective study was to describe the oral procedures used in osteo-odonto-keratoprosthesis (OOKP) surgery, and the demographics and oral findings of candidate patients in Singapore. The OOKP procedure utilizes an autologous tooth-bone complex to mount a poly-methylmethacrylate optical cylinder, as an artificial cornea, stabilized by an overlying autologous buccal mucosal graft. Consecutive patients referred over 3 years for dental evaluation prior to OOKP surgery were included. A total of 21 patients underwent oral clinical and radiographic evaluation. The aetiology of blindness included Stevens-Johnson's syndrome (11 cases), chemical burns (9 cases) and multiple failed corneal grafts (1 case). Evaluation revealed that 12 patients were suitable for OOKP surgery, 8 were at risk of complication or failure and 1 had no usable teeth. Fourteen patients have undergone unilateral OOKP Stage 1 surgery successfully. Complications included fracture of a tooth from its lingual bone necessitating the harvesting of a second tooth (1 case), oronasal perforation (1 case), exposure of adjacent roots (5 teeth), lower lip paresthesia (2 cases) and submucosal scar band formation in the buccal mucosal graft donor site (10 cases). Thirteen patients have completed Stage 2 surgery, with attainment of their best possible visual potential following OOKP surgery. PMID:17630251
Tay, A B G; Tan, D T H; Lye, K W; Theng, J; Parthasarathy, A; Por, Y-M
Metabolic syndrome affects one in four women in the USA, and the incidence is rising every year. Metabolic syndrome is strongly associated with development of coronary artery disease and diabetes. Women of reproductive age are not spared from the complications of metabolic syndrome, which overlaps with obesity and polycystic ovary syndrome (PCOS), both of which are linked to infertility and poor reproductive outcome. Therefore, the relationship between the metabolic syndrome and reproductive dysfunction is an active area of study. In this review, we discuss the animal and human data available to determine if the abnormality is at the level of the ovary and/or endometrium, and discuss the underlying mechanisms causing the associated poor reproductive outcomes. PMID:21277789
Cardozo, Eden; Pavone, Mary Ellen; Hirshfeld-Cytron, Jennifer E
... Duane syndrome? Duane syndrome (DS) is a rare, congenital (present from birth) eye movement disorder. Most patients ... or DR syndrome), Eye Retraction Syndrome, Retraction Syndrome, Congenital retraction syndrome and Stilling-Turk-Duane Syndrome. In ...
Volar locking plates are an increasingly popular treatment for distal radius fractures. We reviewed complications observed after volar locking plate fixation in a busy teaching hospital. The purpose of the study was to assess whether complication rates after volar locking plate use in general, routine trauma practice were higher than published literature from expert users. A retrospective review was carried out of patients treated with a volar locking plate between January 2009 and December 2010. The series included 206 procedures in 204 patients (77 males and 127 females) with mean age of 55 years (range 16-94). Surgery was performed by 18 different consultant surgeons and 11 registrars. A total of 22 complications were observed in 20 patients with an overall complication rate of 9.7%. Seven (3.4%) patients developed tendon problems including four (1.9%) tendon ruptures. Four (1.9%) patients required re-operation for metalwork problems; four patients developed complex regional pain syndrome (CRPS). Three fracture reduction problems were noted. A total of 16 further operations were carried out for complications. The overall complication rate was low even when surgery was done by many surgeons, suggesting that this is a safe and reproducible technique. This study provides information which can be used to counsel patients about risks, including those of tendon and metalwork problems. This allows patients to make an informed decision. Surgeons must have specific strategies to avoid these complications and remain vigilant so that these can be identified and managed early. PMID:24176679
Johnson, N A; Cutler, L; Dias, J J; Ullah, A S; Wildin, C J; Bhowal, B
Lemierre syndrome is a rare condition arising from an invasive oropharyngeal infection, which leads to septic thrombophlebitis of the internal jugular vein and multi-organ septic embolization. Intracranial complications are rare but serious, including subdural empyema, cavernous sinus thrombosis, and internal carotid artery aneurysms. We report a patient with Lemierre syndrome with multiple intracranial complications despite aggressive antimicrobial therapy. The patient eventually required transsphenoidal endoscopic drainage of the sphenoid sinus to help eradicate the infectious source. We postulate that in patients with Lemierre syndrome with evidence of infection in the paranasal sinuses, endoscopic sinus drainage can be an adjunct to antimicrobial therapy in achieving infection control. PMID:24060623
Golan, E; Wong, K; Alahmadi, H; Agid, R F; Morris, A; Sharkawy, A; Zadeh, G
Fregoli syndrome is the delusional belief that one or more familiar persons, usually persecutors following the patient, repeatedly change their appearance. This syndrome has often been discussed as a variant of the Capgras syndrome in the literature, but these two syndromes have different phenomenological structures and age and sex distributions. The author presents a review of 34 cases of Fregoli syndrome in the English and French language literature, discussing the syndrome's definition, aetiology and course. It is suggested that although an organic substrate may be found in some cases, it is the dominant psychotic theme which determines the content of the syndrome. PMID:7893241
PurposeThe post-vasectomy pain syndrome is a rare but troublesome complication of vasectomy. We report our experience with 32 patients who underwent vasectomy reversal for relief of the post-vasectomy pain syndrome.
Stanley A. Myers; Christopher E. Mershon; Eugene F. Fuchs
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
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
Implantable peritoneovenous shunts have facilitated the management of patients with medically intractable ascites. The more commonly reported complications occur with an incidence of 20-50%. Rare and problematic complications are becoming increasingly frequent. Two unusual complications, 1) progressive respiratory insufficiency and death secondary to pulmonary embolization of ascites fluid containing cholesterol crystals, and 2) shunt malfunction caused by the formation of a fibrous envelope around the venous catheter are presented. Pathogenesis, diagnosis and therapy of each of these unusual complications is discussed. Images Fig. 1a. Fig. 1b. Fig. 2a.
Eckhauser, F E; Strodel, W E; Girardy, J W; Turcotte, J G
Augmentation enterocystoplasty has been extensively used to attain high-capacity low-pressure urinary reservoirs in patients with neuropathic bladder, exstrophy-epispadias complex, valve bladder syndrome, and contracted bladder. Enterovesical fistula might occur as an early complication after enterocystoplasty. We report the case of a 16-year-old boy, who presented with chronic watery diarrhea 7 years after augmentation ileocystoplasty. A colovesical fistula was diagnosed. We discuss the clinical presentation, management plan, and operative findings. PMID:23830082
Abdelhalim, Ahmed; Hafez, Ashraf T
We report the case of a patient who presented with right sciatic pain as a manifestation of piriformis muscle syndrome 1 month after a clandestine- induced abortion. MRI revealed the presence of a piriformis abscess. Staphylococcus aureus was isolated from blood cultures and from the purulent material obtained by CT-guided aspiration. Piriformis pyomyositis should be recognized as a potential complication of "unsafe" abortions that if left untreated could progress to sepsis and death. PMID:17414537
Colmegna, Ines; Justiniano, Maria; Espinoza, Luis R; Gimenez, Carlos R
Polycystic ovary syndrome (POS) is one of the most common endocrine abnormalities affecting women of reproductive age. It is a cause of significant social embarrassment and emotional distress. The pathogenesis of the disease is not yet fully understood, but it is thought to be a complex multigenic disorder, including abnormalities in the hypothalamic-pituitary axis, steroidogenesis, and insulin resistance. The main diagnostic findings of the syndrome are: hyperandrogenism, chronic anovulation and polycystic ovarian morphology seen on ultrasound. Hyperandrogenism is generally manifested as hirsutism, acne, seborrhea, androgenic alopecia and, in severe cases, signs of virilization. Treatment may improve the clinical manifestations of excess androgen production, normalize menses and ameliorate metabolic syndrome and cardiovascular complications. This article reviews the diagnosis, clinical manifestations, metabolic complications, and treatment of the syndrome. Early diagnosis and the consequent early treatment may prevent metabolic complications and emotional distress that negatively impact the patients' quality of life. PMID:21437531
Moura, Heloisa Helena Gonçalves de; Costa, Dailana Louvain Marinho; Bagatin, Ediléia; Sodré, Celso Tavares; Manela-Azulay, Mônica
The aim of this study is the evaluation of laparoscopic treatment in abdominal complications following ventriculoperitoneal (VP) shunt. Methods: We report a retrospective study including 17 patients with abdominal complications secondary to VP shunt for hydrocephalus, laparoscopically treated in our department, between 2000 and 2007. Results: Patients' age ranged from 1 to 72 years old (mean age 25.8 years old). Male: female ratio was 1.4. Abdominal complications encountered were: shunt disconnection with intraperitoneal distal catheter migration 47.05% (8/17), infections 23.52% (4/17) such as abscesses and peritonitis, pseudocysts 11.76% (2/17), CSF ascites 5.88% (1/17), inguinal hernia 5.88% (1/17), and shunt malfunction due to excessive length of intraperitoneal tube 5.88% (1/17). Free–disease interval varies from 1 day to 21 years, depending on the type of complication, short in peritoneal irritation syndrome and abscesses (days) and long in ascites, pseudocysts(months– years). Laparoscopic treatment was: extraction of the foreign body in shunt disconnection with intraperitoneal distal catheter migration, evacuation, debridement, lavage and drainage for pseudocysts, abscess and peritonitis, shortening of the tube in shunt malfunction due to excessive length of intraperitoneal tube a nd hernioraphy. One diagnostic laparoscopy was performed in a peritoneal irritation syndrome, which found only CSF ascites. There were no conversions to open surgery. The overall mortality was of 5.88% and postoperative morbidity was of 11.76%. In 7 patients operated for abscesses, peritonitis, pseudocysts, and CSF ascites the shunting system was converted in to a ventriculocardiac shunt. Conclusions: Abdominal complication following VP shunt can be successfully performed laparoscopically. Abdominal surgery required, in selected cases, the repositioning of the distal catheter, frequently as a ventriculocardiac shunt. There are abdominal complications with no indication of surgery, like peritoneal irritation syndrome and CSF ascites. Free– disease interval varies from days (peritoneal irritation syndrome, abscesses) to month–years (pseudocyst, ascites), according to type of complication.
Grigorean, VT; Onose, G; Popescu, M; Strambu, V; Sandu, AM
Cauda equina syndrome is an uncommon complication of ankylosing spondylitis characterized by the slow and insidious development of severe neurologic impairment related to dural ectasia. This report describes a unique case of cauda equina syndrome in a patient with ankylosing spondylitis after hip revision surgery. A 70-year-old man with long-standing ankylosing spondylitis underwent standard hip revision surgery; combined spinal and general anesthesia was administered. Pain was controlled with intravenous opioids postoperatively (patient-controlled analgesia). As per routine protocol, on the first postoperative day, the patient remained supine on a hip abduction pillow; mobilization was initiated on the second postoperative day. On postoperative day 1, the patient had severe low back pain that was controlled with patient-controlled analgesia. On postoperative day 2, the Foley catheter was removed and the patient sat and dangled. Back pain persisted while supine; in addition, the patient noticed involuntary loss of urine. On postoperative day 3, the patient had below-the-knee numbness that progressed to saddle anesthesia and foot flexor and extensor weakness. An epidural hematoma was suspected and urgent magnetic resonance imaging was performed, which showed severe degenerative stenosis at the L4-L5 level (mainly by dense ligamentum flavum). An L4-L5 decompression and instrumented fusion was performed; intraoperatively, L4-L5 was found to be the sole mobile segment. The extension of the spine in the supine position that completely obliterated the spinal canal was considered the mechanism of cauda equina syndrome. The intensity of back pain is a good indicator of a severe spinal lesion; however, pain can be dampened by intravenous opioids. High suspicion is required in patients with preexisting spinal pathology, such as ankylosing spondylitis. PMID:24972447
Sakellariou, Vasileios I; Papadopoulos, Elias C; Babis, George C
We report a case of complicated pansinusitis presenting with thunderclap headache. The patient quickly developed left ophthalmoplegia and imaging demonstrated extension of inflammation from the sphenoid sinus into the sellar region. Thunderclap headache is well known to occur from a variety of intracranial events, but complicated sinusitis presenting this way is seldom described. The patient recovered completely with appropriate treatment. PMID:16618275
McGeeney, Brian E; Barest, Glenn; Grillone, Gregory
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
Findings are presented in 36 children, seen in the past 11 years, who are believed to have suffered from neurological complications of pertussis inoculation (given as triple vaccine). The clustering of complications in the first 24 hours after inoculation suggests a causal rather than a coincidental relation. Possible contributory factors were present in one-third of patients studied and support the
M. Kulenkampff; J. S. Schwartzman; J. Wilson
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
PURPOSE: Colonic perforations in renal transplant recipients have historically been associated with mortality rates as high as 50 to 100 percent. However, these previous series generally predate the use of cyclosporine-based immuno-suppressive protocols. METHODS: We retrospectively reviewed all patients who had undergone renal transplant from our institution and who developed complicated diverticulitis. Complicated diverticulitis was defined as diverticulitis involving free
Eric D. Lederman; David J. Conti; Neil Lempert; T. Paul Singh; Edward C. Lee
Hematopoietic stem cell transplantation (HSCT) is the only effective therapeutic modality for patients affected by major hemoglobinopathies such as thalassemia major and sickle cell anemia. Although improvements in transplant technology a substantial group of patients continue to have post transplant complications which increase morbidity and mortality. Optimizing outcomes through prevention or early detection of post transplant complications and mitigation of
Javid Gaziev; Policlinico Tor Vergata
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
Mirror syndrome is a preeclampsia-like disease first described in a case of severe hydrops fetalis caused by rhesus isoimmunization, later reported in some cases of nonimmunological fetal hydrops. Twin-twin transfusion syndrome (TTTS) is a severe complication associated with monochorionic pregnancies, in particular, severe TTTS with one hydropic fetus leading to a poor prognosis. We report here a case of mirror syndrome that occurred after selective fetoscopic laser photocoagulation in severe TTTS at 24 weeks' gestation. PMID:16354975
Hayashi, Satoshi; Sago, Haruhiko; Hayashi, Reiko; Nakagawa, Satoshi; Kitagawa, Michihiro; Miyasaka, Katsuyuki; Chiba, Toshio; Natori, Michiya
Background To examine the association between patient complications and admission to level 1 trauma centers (TC) compared to non-trauma centers (NTC). Study Design A retrospective cohort study of data derived from the National Study on the Costs and Outcomes of Trauma (NSCOT). Patients were recruited from 18 level 1 TC and 51 NTC in 15 regions encompassing 14 states. Trained study nurses, using standardized forms, abstracted the medical records of the patients. The overall number of complications per patient was identified as well as the presence or absence of 13 specific complications. Results Patients treated in TC were more likely to have any complication compared to NTC with an adjusted relative risk (RR) of 1.34 (95% CI 1.03, 1.74). For individual complications, only urinary tract infection RR 1.94 (95% CI 1.07, 3.17) was significantly higher in TC. TC patients were more likely to have three or more complications, RR 1.83 (95% CI 1.16, 2.90). Treatment variables that are surrogates for markers of injury severity, such as use of pulmonary artery catheters, multiple operations, massive transfusions (> 2,500mL packed red blood cells), and invasive brain catheters, occurred significantly more often in TC. Conclusions Trauma centers have a slightly higher incidence rate of complications even after adjusting for patient case mix. Aggressive treatment may account for a significant portion of TC-associated complications. PA catheter use and intubation had the most influence on overall TC complication rates. Further study is needed to provide accurate benchmark measures of complication rates and to determine their causes.
Ang, Darwin N; Rivara, Frederick P; Nathens, Avery; Jurkovich, Gregory J; Maier, Ronald V; Wang, Jin; MacKenzie, Ellen J
The pericardium is a sac containing the heart and proximal parts of the great vessels [1, 2]. It consists of two layers:\\u000a \\u000a \\u000a \\u000a \\u000a \\u000a \\u000a • \\u000a \\u000a The visceral pericardium is a single-layer serous membrane that refl ects back near the origins\\/insertions of the great vessels and becomes\\u000a continuous with and forming the inner layer of the parietal pericardium.\\u000a \\u000a \\u000a \\u000a • \\u000a \\u000a The parietal pericardium is
Seth Uretsky; Dan L. Musat; Mark V. Sherrid; Eyal Herzog
Aspergillus fumigatus is a filamentous mold that causes infections in patients who are inmmunocompromised. We report a case of Aspergillus tracheobronchitis in fulminant systemic lupus erythematosus case. Diagnosis with more invasive diagnostic procedures & aggressive antifungal therapy is indicated at early stage.
Yegneswaran Prakash, Peralam; Pandit, Vinay; Rao, Sugandhi P.
Pelvic floor disorders affect the quality of life of millions of women worldwide. Many options exist for the treatment of pelvic organ prolapse and urinary incontinence, surgery being one of the main strategies in the management of these conditions. Even though uncommon, all surgery has complications which can cause morbidity and rarely mortality. These complications can impair quality of life in the long-term and be a financial burden on both the patient and the health care system. Pelvic floor reconstructive surgery includes perioperative complications such as injury to neighboring organs, hemorrhage and infection. Recently the International Urogynecology Association and the International Continence Society have proposed a terminology and classification of complications related to female pelvic floor surgery, both using native tissue and synthetic implants to improve surgical audit and aid comparison between studies on pelvic floor procedures. Long-term complications such as pelvic pain and dyspareunia may be as high as 25%. Prolapse surgery associated with mesh may result in better anatomical outcomes but this is offset by the high complication rate, particularly that of mesh exposure which has been reported to be between 3-15%. Minimally invasive anti-incontinence procedures are associated with less morbidity than their abdominal predecessors but they are not free of complications. Complications of mid-urethral slings include those of mesh exposure (0.3%), voiding dysfunction (7%) and de novo urgency (25%). The risk and severity of complications varies depending on the procedure performed and on patient characteristics and, therefore, patients need to be informed of these risks or clinicians will be held responsible. This has never been more true than now with the debate regarding the value of transvaginal mesh and laparoscopic procedures for prolapse, their risks and potential benefits, and the associated medico-legal sequelae. PMID:23412020
Alvarez, J; Cvach, K; Dwyer, P
In this paper the results of investigations of the mechanism and kinetics of reactions of formation of fine crystalline complicated\\u000a oxides in thermovaporous and solvothermal conditions are presented. The processes of formation of complicated oxides, magnesian\\u000a spinel (MgAl2O4), aluminate of lanthanum (LaAlO3), gahnite (ZnAl2O4), barium hexaferrite (BaFe12O19), lithium metaniobate (LiNbO3), and yttrium-aluminum garnet (Y3Al5O12) were studied. Synthesis of complicated oxides
M. N. Danchevskaya; Yu. D. Ivakin; S. N. Torbin; G. P. Muravieva; O. G. Ovchinnikova
Cocaine use has increased considerably during the last twenty years and several related complications can be identified. Clinical features of cocaine intoxication are variable, but predominantly involve cardiovascular events. Chest pain is the most main complaint; myocardial ischemia must be ruled out. Other cardiovascular manifestations are left ventricular dysfunction, arrhythmia, endocarditis and aortic dissection. Non-cardiac complications include neurological (seizures, stroke, cerebral hemorrhage), respiratory (asthma, interstitial pneumonitis, pulmonary edema), renal (acute renal failure, rhabdomyolysis) and obstetrical disorders. Detection of cocaine in the urine provides the diagnosis. Symptomatic treatment is generally given, combining conventional treatment of the complication and broad use of benzodiazepines. PMID:12218880
Guerot, Emmanuel; Sanchez, Olivier; Diehl, Jean-Luc; Fagon, Jean-Yves
Trichorhinophalangeal syndrome type 1 is an autosomal dominant variety of peripheral dysostosis. I have reviewed nine cases of this syndrome and have described two of these cases. I have also presented an extensive review of the literature. PMID:1925732
Burgess, R C
The HELLP syndrome is a serious complication of pregnancy characterized by hemolysis (H), elevated liver (EL) enzymes, and low platelet (LP) count that occurs in 0.2-0.6% of all pregnancies and in 10-20% of cases with severe preeclampsia and frequently leads to adverse maternal and perinatal outcome. The exact pathobiology of HELLP syndrome has not been clearly defined. As it is considered a form or a complication of severe preeclampsia, it likely has its origin in aberrant placental development and function resulting in ischemia-producing oxidative stress. However, there is still a debate on whether HELLP must be considered a severe form of preeclampsia or a separate disease entity. It can be described as a placenta-induced disease, as is preeclampsia itself, but with a more acute and predominant inflammatory process typically targeting the liver and with a greater activation of the coagulation system. This occurs during a disordered immunologic process and may be due to a genetic predisposition. In this review, we discuss the main biochemical characteristics of HELLP syndrome, particularly focusing on molecular aspects of placental involvement and maternal systemic responses. PMID:21404915
Benedetto, Chiara; Marozio, Luca; Tancredi, Annalisa; Picardo, Elisa; Nardolillo, Paola; Tavella, Anna Maria; Salton, Loredana
Aims The long term results of the Legeais BioKpro III keratoprosthesis are presented for seven patients with severe corneal scarring. Methods The study took place at Moorfields Eye Hospital, London. Patients had either end stage ocular surface disease or corneal opacification after multiple failed graft surgery, with the potential for significant visual improvement. After insertion the device was covered with a conjunctival flap or buccal mucous membrane graft, which was later opened to expose the optic. The outcome measures were vision, complications, and retention of the device. Results The BioKpro III was inserted into seven patients with severe corneal scarring: ocular cicatricial pemphigoid, measles keratitis, thermal injury, Stevens?Johnson syndrome, aniridia, chemical injury, and congenital rubella. The follow up was 18–48?months. The keratoprosthesis failed in six, because of extrusion occurring 2–28?months postoperatively. Retroprosthetic membranes occurred in three patients, and endophthalmitis in one. Vision improved from hand movements to 6/12 in the only patient who retained the KPro; however he was troubled by mucus accumulation on the optic. Conclusions The one success has been in a patient with thermal burns. The remaining results have been poor, with the KPro extruding in six of the seven patients.
Hollick, E J; Watson, S L; Dart, J K G; Luthert, P J; Allan, B D S
Background.?Systemic antibiotics are a major cause of severe cutaneous adverse reactions (SCARs). The selection of alternative antibiotics and management for SCARs patients with underlying infections can be challenging. Methods.?We retrospectively analyzed 74 cases of SCARs, including Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), drug rash with eosinophilia and systemic symptoms (DRESS), and acute generalized exanthematous pustulosis (AGEP), related to use of systemic antibiotics in Taiwan from January 2006 to January 2012. We analyzed the causative antibiotics, clinical features, organ involvements, and mortality. We also assessed patient tolerability to alternative antibiotics after the development of antibiotic-related SCARs. Results.?The most common causes of SCARs were penicillins and cephalosporins for SJS/TEN and AGEP; glycopeptides for DRESS. Fatality was more frequent in the SJS/TEN group. In patients with SJS/TEN, higher mortality was associated with old age and underlying sepsis before the development of SCARs. The majority of patients with penicillin- or cephalosporin-related SCARs were able to tolerate quinolones, glycopeptides, and carbapenems. Conclusions.?Complicated underlying conditions and infections may increase mortality in patients with antibiotic-related SCARs. The selection of structurally different alternative drugs is important to avoid recurrence. PMID:24599767
Lin, Ying-Fang; Yang, Chih-Hsun; Sindy, Hu; Lin, Jing-Yi; Rosaline Hui, Chung-Yee; Tsai, Yun-Chen; Wu, Ting-Shu; Huang, Ching-Tai; Kao, Kuo-Chin; Hu, Han-Chung; Chiu, Cheng-Hsun; Hung, Shuen-Iu; Chung, Wen-Hung
Background/aims: To determine outcomes of transplants of cultivated autologous oral epithelial cells in patients with severe ocular surface disorders. Methods: The eyes (n?=?6) of four patients with Stevens-Johnson syndrome (three eyes) or chemical burns (three eyes) were studied. Autologous oral epithelial cells, grown for 2–3 weeks on a denuded amniotic membrane carrier in the presence of 3T3 fibroblasts, were air lifted. The resultant sheet was transplanted onto the damaged eye, and acceptance of the sheet by the corneal surface was confirmed 48 hours after surgery. The success of ocular surface reconstruction, graft survival, changes in visual acuity, and postoperative complications were assessed and the quality of the cultivated oral epithelial sheet was evaluated histologically. Results: At 48 hours after transplant, the entire corneal surface of all six eyes was free of epithelial defects indicating complete survival of the transplanted oral epithelium. Visual acuity was improved in all eyes. During follow up (mean 13.8 (SD 2.9) months), the corneal surface remained stable, although all eyes manifested mild peripheral neovascularisation. Conclusions: Autologous oral epithelial cells grown on denuded amniotic membrane can be transplanted to treat severe ocular surface disorders.
Nakamura, T; Inatomi, T; Sotozono, C; Amemiya, T; Kanamura, N; Kinoshita, S
Mycoplasma pneumoniae-associated mucositis is a rarely described complication of M. pneumoniae infection presenting with ocular, oral, and genital involvement but without the typical skin lesions seen in Stevens-Johnson syndrome. A 27-year-old man with a past history of asthma presented at the emergency room with a 1-week history of cough (initially non-productive but subsequently associated with non-bloody mucopurulent sputum), fever, myalgias, headache, and progressive dyspnea. Two days before admission he had commenced amoxicillin/clavulanic acid with no improvement. The patient reported bilateral conjunctival injection and hemorrhagic ulcers on the lips commencing the day prior to admission. Physical examination revealed fever (39 degrees C), bilateral exudative conjunctivitis, painful hemorrhagic ulcers on the lips, tongue, and oral mucosa, small scrotal erosions, erythema of the penile meatus, and small erythematous bullae on the dorsum of each hand; subsequently, the patient developed bullae at the venipuncture site on his right arm. Laboratory tests revealed positive IgM serology for M. pneumoniae, with titer elevation. The patient was successfully treated with levofloxacin and prednisolone. Our case appears to be the first adult patient described with M. pneumoniae-associated mucositis, which has previously been reported only in pediatric patients. This is also the first reported instance of a case of M. pneumoniae-associated mucositis treated with levofloxacin and prednisolone. M. pneumoniae infection should be considered in all cases of mucositis, and treatment of this condition with levofloxacin and prednisolone seems to be effective. PMID:18973408
Figueira-Coelho, João; Lourenço, Sofia; Pires, Ana Cristina; Mendonça, Paula; Malhado, José António
Velocardiofacial syndrome (VCFS), also known as DiGeorge, conotruncal anomaly face, and Cayler syndromes, is caused by a microdeletion in the long arm of Chromosome 22. We review the history of the syndrome from the first clinical reports almost half a century ago to the current intriguing molecular findings associating genes from the…
Gothelf, Doron; Frisch, Amos; Michaelovsky, Elena; Weizman, Abraham; Shprintzen, Robert J.
In recent years, a number of comprehensive reviews have been written on inherited intestinal polyposis syndromes (1–7), but none has dealt specifically with Gardner's syndrome and none has focused on basic research being carried out in an attempt to under-stand this syndrome and to improve the medical management of affected patients. A better understanding of this rare genetic disorder is
Edwin W. Naylor; Emanuel Lebenthal
Velocardiofacial syndrome (VCFS), also known as DiGeorge, conotruncal anomaly face, and Cayler syndromes, is caused by a microdeletion in the long arm of Chromosome 22. We review the history of the syndrome from the first clinical reports almost half a century ago to the current intriguing molecular findings associating genes from the microdeletion region and the physical and neuropsychiatric phenotype
Doron Gothelf; Amos Frisch; Elena Michaelovsky; Abraham Weizman; Robert J. Shprintzen
Interest in the diagnosis and treatment of common disorders of the carpus has intensified in recent years. Although newer nonoperative and surgical procedures have developed to improve outcome, complications and their treatment remain a challenging problem. To address complications of the more common carpal injuries, we have chosen fractures of the scaphoid, scapholunate ligament injuries, carpal fracture dislocations, and fractures of the hook of the hamate for discussion. PMID:8188775
Culp, R W; Lemel, M; Taras, J S
\\u000a Surgical complications following renal transplan-tation can occur at any time, and are predominantly related to the transplant\\u000a wound, vascular anastomoses, or other urologic problems. Surgical complications continue to occur in about 10–20% of transplant\\u000a recipients, but fortunately, are rarely the cause of allograft loss. Improvements in surgical technique and meticulous attention\\u000a to both the donor and recipient operations have led
Stuart M. Flechner
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
Purpose Percutaneous nephrolithotomy (PCNL) has been the preferred procedure for the removal of large renal stones in Iran since 1990. Recently, we encountered a series of devastating neurologic complications during PCNL, including paraplegia and hemiplegia. There are several reports of neurologic complications following PCNL owing to paradoxical air emboli, but there are no reports of paraplegia following PCNL. Materials and Methods We retrospectively reviewed the medical records of patients who had undergone PCNL in 13 different endourologic centers and retrieved data related to neurologic complications after PCNL, including coma, paraplegia, hemiplegia, and quadriplegia. Results The total number of PCNL procedures in these 13 centers was 30,666. Among these procedures, 11 cases were complicated by neurologic events, and four of these cases experienced paraplegia. All events happened with the patient in the prone position with the use of general anesthesia and in the presence of air injection. There were no reports of neurologic complications in PCNL procedures performed with the patient under general anesthesia and in the prone position and with contrast injection. Conclusions It can be assumed that using room air to opacify the collecting system played a major role in the occurrence of these complications. Likewise, the prone position and general anesthesia may predispose to these events in the presence of air injection.
Basiri, Abbas; Soltani, Mohammad Hossein; Kamranmanesh, Mohammadreza; Tabibi, Ali; Mohsen Ziaee, Seyed Amir; Nouralizadeh, Akbar; Sharifiaghdas, Farzaneh; Poorzamani, Mahtab; Gharaei, Babak; Ozhand, Ardalan; Lashay, Alireza; Ahanian, Ali; Aminsharifi, Alireza; Sichani, Mehrdad Mohammadi; Asl-Zare, Mohammad; Ali Beigi, Faramarz Mohammad; Najjaran, Vahid; Abedinzadeh, Mehdi
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
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
Secondary Sjögren's syndrome is due to another disease. When it develops in connective tissue diseases, their causative role is unchallenged. In AIDS or hepatitis C, exocrine involvement is virus related. Whether or not it qualifies for Sjögren's syndrome is debated. Amyloidosis and sarcoidosis do not produce direct, autoimmune lesions of the glands, hence their exocrine involvements are considered as differential diagnoses. The most common Sjögren's syndrome is found in rheumatoid arthritis. When it appears, the arthritis has been evolving for years, and has reached its typical, seropositive and erosive stage. Accordingly, dryness is not a major concern and should be sought for by proper questioning, specially on eye dryness. When a secondary Sjögren's syndrome is an early complication of rheumatoid arthritis, it could be confused with a primary syndrome with prominent joint involvement. In systemic lupus erythematosus, secondary Sjögren's syndrome develops rarely in the first years of evolution but later in life, when the patient becomes menopausal. In systemic sclerosis, especially in CREST, secondary syndrome can lead to the discovery of the unsuspected connective tissue disease thanks to mouth dryness. It can reveal primary biliary cirrhosis or auto-immune hepatitis. Often precede a true primary Sjögren dysfunctions of the thyroid. PMID:11252943
Sauvezie, B; Tournadre, A; Chamard, C; Dubost, J J
Sticky platelet syndrome (SPS) is a thrombophilic thrombocytopathy with familial occurrence and autosomal dominant trait, characterized by an increased in vitro platelet aggregation in response to low concentrations of adenosine diphosphate (ADP) and/or epinephrine (EPI). According to aggregation pattern, three types of the syndrome can be identified (hyperresponse after both reagents, Type I; EPI alone, Type II; ADP alone, Type III). Clinically, the syndrome is associated with both venous and arterial thrombosis. In pregnant women, complications such as fetal growth retardation and fetal loss have been reported. The first thrombotic event usually occurs before 40 years of age and without prominent acquired risk factors. Antiplatelet drugs generally represent adequate treatment. The use of other antithrombotics is usually ineffective and may result in the recurrence of thrombosis. In most patients, low doses of antiplatelet drugs (acetylsalicylic acid, 80-100 mg/d) lead to normalization of hyperaggregability. Combination of SPS with other thrombophilic disorders has been described. Despite several studies investigating platelet glycoproteins' role in platelets' activation and aggregation, the precise defect responsible for the syndrome remains unknown. The aim of this review is to summarize authors' own experience about SPS and the clinical data indexed in selected databases of medical literature (PubMed and Scopus). PMID:23934738
Kubisz, Peter; Stasko, Jan; Holly, Pavol
The exophthalmos, myxedema, acropachy (EMA) syndrome is a rare extrathyroid syndrome, interesting about 1% of the patients affected by extrathyroid complications of Graves' disease. The ratio female/male is 3.4:1 and this case report is very rare. The patient, a 52-year-old man, presented a serious ophthalmopathy with pretibial myxedema, acropachy with joint pain. The triad manifested itself after some ophthalmopathy treatments, i.e. total thyroidectomy, steroidal retrobulbar therapy and radiotherapy. The patient received T4 therapy and the thyroid function status was normal. The appearance of the EMA syndrome coincided with the fast worsening of the ophthalmopathy. This case report confirms previous observations regarding the chronological sequence of presentations of extrathyroidal manifestations of autoimmune thyroid disease. The thyroid disease develops first, followed by ophthalmopathy, then dermopathy, and finally, acropachy. The thyroid acropachy shows some differences between pulmonary and paraneoplastic osteoarthropathy, due to the presence of thyroid dermopathy and ophtalmopathy (EMA) and to the different subperiosteal proliferation. Steroidal therapy improved the ophthalmopathy, the pretibial myxedema and the acropachy. The improvement obtained has been faster as regards the exophtalmos and myxedema, slower as regards the acropachy, but of the same importance. In conclusion, acropachy is the latest manifestation of EMA and coincides with the worsening of ophthalmopathy. The traditional steroidal therapy is effective to improve the syndrome. PMID:15257258
Artuso, V; Roiter, I
Lance-Adams syndrome (LAS) is a rare complication of successful cardiopulmonary resuscitation and is often accompanied by action myoclonus. LAS is seen in patients who have undergone a cardiorespiratory arrest, later regained consciousness, and then developed myoclonus days or weeks after the event. Less than 150 cases of LAS have been reported in the worldwide medical literature. Here, we present a 32-year-old man who suffered from myoclonus after hypoxic brain damage due to hanging himself. This case was diagnosed as Lance-Adams syndrome according to a history of hypoxic brain damage, the clinical features, and the neuroimages such as brain SPECT. Making an early diagnosis and properly managing LAS is positively related to improving the patient's functional outcome. If patients have posthypoxic myoclonus after successful cardiopulmonary resuscitation, we should consider the diagnosis of LAS and initiate a proper rehabilitation program.
Lee, Ha Lim
Delleman Oorthuys syndrome (oculocerebrocutaneous syndrome) is a rare, congenital sporadic disorder affecting the skin and central nervous system. We present the case of a one-month-old male who presented with an orbital cyst in the left eye since birth along with other manifestations of this syndrome. The manifestations of this syndrome resemble other developmental disorders like Goldenhar and Goltz syndrome. Conservative management of the orbital cyst in these cases have been described. The need to diagnose this rare congenital anomaly with cerebral malformations as a separate entity is crucial in the management of these children.
Arora, Vipul; Kim, Usha R; Khazei, Hadi M
Antiphospholipid syndrome (APS) is associated with a risk of obstetrical complications, affecting both the mother and the fetus. Obstetrical APS is defined by a history of three consecutive spontaneous miscarriages before 10 weeks of gestation (WG), an intra-uterine fetal death after 10 WG, or a premature birth before 34 WG because of severe pre-eclampsia, eclampsia or placental adverse outcomes (intrauterine growth retardation, oligohydramnios). Pregnancy in women with a diagnosis of obstetric APS is at increased risk for placental abruption, HELLP (Hemolysis, Elevated Liver enzymes, Low Platelet count) syndrome and thrombosis that may be part of a catastrophic antiphospholipid syndrome (CAPS). A previous thrombosis and the presence of a lupus anticoagulant are risk factors for pregnancy failure. A multidisciplinary approach, associating the internist, the anesthesiologist and the obstetrician, is recommended for these high-risk pregnancies. Preconception counseling is proposed to identify pregnancy contraindications, and to define and adapt the treatment prior and during the upcoming pregnancy. Heparin and low-dose aspirin are the main treatments. The choice between therapeutic or prophylactic doses of heparin will depend on the patient's medical history. The anticoagulant therapeutic window for delivery should be as narrow as possible and adapted to maternal thrombotic risk. There is a persistent maternal risk in the postpartum period (thrombosis, HELLP syndrome, CAPS) justifying an antithrombotic coverage during this period. We suggest a monthly clinical and biological monitoring which can be more frequent towards the end of pregnancy. The persistence of notches at the Doppler-ultrasound evaluation seems to be the best predictor for a higher risk of placental vascular complications. Treatment optimization and multidisciplinary antenatal care improve the prognosis of pregnancies in women with obstetric APS, leading to a favorable outcome most of the time. PMID:22341691
Costedoat-Chalumeau, N; Guettrot-Imbert, G; Leguern, V; Leroux, G; Le Thi Huong, D; Wechsler, B; Morel, N; Vauthier-Brouzes, D; Dommergues, M; Cornet, A; Aumaître, O; Pourrat, O; Piette, J-C; Nizard, J
Pulmonary complications develop in 30–60% of hematopoietic stem cell transplants (HSCT). The main, late onset, non-infectious complications include Bronchiolitis obliterans (BO), Bronchiolitis obliterans organizing pneumonia (BOOP), and idiopathic pneumonia syndrome (IPS). BO and BOOP occur almost exclusively in allogeneic HSCT, and have 61% and 21% mortality rates, respectively. BOOP responds favorably to corticosteroids. IPS has less than 15% 1-year survival.
B Afessa; A Tefferi
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
Polycystic ovary syndrome (PCOS), a heterogeneous and chronic condition, today affects about 5% of women of reproductive age. PCOS is strongly associated with states of insulin resistance and hyperinsulinemia. Risk factors include genetics, metabolic profiles, and the in utero environment. Long-term consequences of PCOS include metabolic complications such as diabetes, obesity, and cardiovascular disease. Dysregulation of insulin action is closely linked to the pathogenesis of PCOS. However, whether insulin resistance is the causative factor in the development of PCOS remains to be ascertained. Moreover, the mechanism by which insulin resistance may lead to reproductive dysfunction requires further elucidation. PMID:24582095
Nandi, Anindita; Chen, Zijian; Patel, Ronak; Poretsky, Leonid