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Frieri Marianne M Department of Medicine, Division of Allergy Immunology, Nassau University Medical Center, 2201 Hempstead Turnpike, East Meadow, NY, 11554, USA, - - 2014
Good's syndrome and common variable immune deficiency (CVID) are associated with chronic rhinosinusitis. Good's syndrome is characterized by hypogammaglobulinemia, B-cell depletion, variable defects in cellular immunity and thymoma. Immunodeficiency and recurrent infections can initially present after thymectomy. The pathogenesis can involve cytokines from bone marrow along with genetic defects. Intravenous ...
Moreau Richard R Inserm, U1149, Centre de Recherche sur l'Inflammation (CRI),; UMR_S 1149, Université Paris Diderot, Faculté de Médecine, Département Hospitalo-Universitaire (DHU) UNITY, Service d'Hépatologie, Hôpital Beaujon, AP-HP, Clichy, Paris, - - 2014
Decompensated cirrhosis is diagnosed by the presence of signs such as ascites, gastrointestinal hemorrhage, hepatic encephalopathy, bacterial infection or any combination of these (1). Ascites is the most common sign of decompensated cirrhosis (1). Ascites is primarily related to an inability to excrete an adequate amount of sodium into urine, ...
Aziz Hafsa H aNuclear Medicine, Oncology and Radiotherapy Institute bMaroof International Hospital, Islamabad, - - 2014
Hepatitis C viral (HCV) infection is caused by an RNA virus. HCV infection is considered to induce systemic disease that causes steatosis, alters lipid metabolism, and results in metabolic syndrome. This study aimed to investigate the therapeutic outcome in HCV genotype 3 patients with metabolic syndrome. A total of 621 ...
Ozguler Yesim Y Istanbul University, Cerrahpasa Medical School, Department of Internal Medicine, Division of Rheumatology, Istanbul, - - 2014
There is agreement that the management of Behçet's syndrome has recently improved, especially with the wider use of biologics. The aim of this review is to summarize the current management strategies in Behçet's syndrome. There is growing experience with tumor necrosis factor-alpha antagonists in the management of eye gastrointestinal and ...
Wang Ji Yao JY Department of Gastroenterology, Zhongshan Hospital and Center of Evidence-Based Medicine, Fudan University, Shanghai, - - 2014
Hepatic sinusoidal obstruction syndrome (HSOS), characterized by hepatomegaly, ascites and hyperbilirubinemia, is caused by toxic injury to hepatic sinusoidal endothelial cells. One major etiology of HSOS in China is the intake of products containing pyrrolizidine alkaloids (PA) such as Tusanqi. The manifestations of patients with HSOS are usually non-specific, presenting ...
Wong G L-H GL Institute of Digestive Disease, The Chinese University of Hong Kong, Hong Kong SAR, China; Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Hong Kong SAR, China; State Key Laboratory of Digestive Disease, The Chinese University of Hong Kong, Hong Kong SAR, - - 2014
Metabolic syndrome is a known risk factor of cirrhosis in chronic hepatitis B (CHB). To investigate the effects of coincidental metabolic syndrome on liver fibrosis progression in treatment-naïve CHB patients. A total of 1466 CHB patients underwent liver stiffness measurement (LSM) by transient elastography in 2006-2008; 663 patients remained treatment-naïve ...
Umezawa Natsuka N Department of Medicine and Rheumatology, Graduate School of Medicine and Dental Science, Tokyo Medical and Dental University , Tokyo , - - 2014
Abstract A 44-year old woman with eosinophilic granulomatosis with polyangiitis (EGPA) developed sequential paralysis of different cranial nerves despite treatments including methylpredonisolone pulse therapy, intravenous immunoglobulins (IVIG), and cyclophosphamide. Infusions of rituximab ameliorated her neurological symptoms and serological inflammatory findings. Rituximab, a specific B cell-targeting therapy, might offer an alternative ...
Xie He-Ping HP Traditional Chinese Medicine & Acupuncture Department, The Third Affiliated Hospital of Sun Yet-sen University, Guangzhou, 510630, - - 2014
To explore Chinese medicine (CM) syndrome distribution of chronic hepatitis B virus (HBV) carriers in immunotolerant phase (ITP). One hundred and eighty-five chronic HBV carriers in ITP, seen in the Third Affiliated Hospital of Sun Yat-sen University from May 2009 to December 2010, were admitted in an observational study under ...
Liao Bing B Department of Neuro-Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (B.L., S.S., C.K.-M., S.T.); Department of Neurology, The University of Texas Medical Branch, Galveston, Texas (B.L., S.S., - - 2014
BackgroundIpilimumab is a novel FDA-approved recombinant human monoclonal antibody that blocks cytotoxic T-lymphocyte antigen-4 and has been used to treat patients with metastatic melanoma. Immune-related neurological adverse effects include inflammatory myopathy, aseptic meningitis, posterior reversible encephalopathy syndrome, Guillain-Barré syndrome, myasthenia gravis-type syndrome, sensorimotor neuropathy, and inflammatory enteric neuropathy. To date, ...
Clave Emmanuel E Departement d'Immunologie, INSERM UMRS-940, Paris, France; Université Paris Diderot, Sorbonne Paris Cité, Paris, France, Paris, - - 2014
Although hypereosinophilia associated with chronic graft-versus-host disease (GvHD) has long been recognized, biological data on this phenomenon are scarce. In this paper, we describe hypereosinophilia (HE) together with a clonal T-cell expansion in patients with chronic GvHD, versus control patients with acute or chronic GvHD, but without HE. These clonal ...
Zhao Dongying D XinHua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, - - 2014
Early detection is the most effective way to improve the clinical outcome of biliary atresia (BA). Emerging metabolomics provides a powerful platform for discovering novel biomarkers and biochemical pathways to improve early diagnosis. The aim of this study is to find the potential biomarkers to distinguish BA from neonatal hepatitis ...
Ahmad Delshad D Department of Internal Medicine, Michigan State University, Flint, - - 2014
Essential mixed cryoglobulinaemia or type II cryoglobulinaemia is an important extrahepatic manifestation of chronic hepatitis C. Cryoglobulinaemia results in the deposition of immune complexes in small or medium-sized blood vessels leading to palpable purpura, arthralgia, renal disease and peripheral neuropathy. Posterior reversible encephalopathy syndrome (PRES) is a distinct phenomenon characterised ...
Sarkhy Ahmed Al - - 2014
Prader-Willi syndrome (PWS) is a rare genetic disorder caused by deletion or unexpression of the chromosome 15 (q 11-13). Symptomatologies include hypotonia, hyperphagia, cognitive impairment, and characteristic dysmorphic profile. Here, we report a 4-year-old boy with PWS who presented with complications of congenital hepatic fibrosis. The uniparental heterodisomy makes it ...
von Linstow Marie-Louise ML 0000-0002-9956-7941 Department of Paediatrics, Hvidovre University Hospital, Kettegaard Alle 30, 2650 Hvidovre, - - 2014
Hyper IgD syndrome (HIDS) is a rare metabolic autoinflammatory syndrome characterised by recurrent febrile episodes, accompanied by various inflammatory symptoms. We present a case of severe HIDS in a young girl, whose symptoms started in the neonatal period with hepatomegaly, hepatitis, thrombocytopenia, and conjugated hyperbilirubinemia. From the age of five ...
Jedraszak Guillaume G Medical Genetics Unit, Centre Hospitalier Universitaire d'Amiens, Amiens, - - 2013
Simpson-Golabi-Behmel syndrome type 1 (SGBS1) -OMIM 312870- is a rare X-linked inherited overgrowth syndrome caused by a loss of function mutation in the GPC3 gene. Affected patients present a variable phenotype with pre- and post-natal macrosomia, distinctive facial dysmophism, organomegaly, and multiple congenital anomalies. Intellectual disability is not constant. About ...
Wu Di-Jiong - - 2013
To explore differences in bone marrow angiogenesis seen in aplastic anemia (AA) patients presenting with differential Chinese medicine (CM) syndrome, and to correlate these differences with clinical pathology. Thirty-five patients were enrolled, including 18 with "yang deficiency syndrome" and 17 with "yin deficiency syndrome." Bone marrow biopsies and serum were ...
Bıyık Murat M Department of Gastroenterology Konya University, School of Medicine, Konya, - - 2013
Alström syndrome is a rare autosomal recessive genetic disorder characterized by cone-rod dystrophy, hearing loss, childhood truncal obesity, insulin resistance and hyperinsulinemia, type 2 diabetes, hypertriglyceridemia, short stature in adulthood, cardiomyopathy, and progressive pulmonary, hepatic, and renal dysfunction. Alström syndrome is a very rare cause of liver cirrhosis. Postmortem biopsies ...
Mittal Dinesh D Animal Disease Research Center, College of Veterinary Sciences, Guru Angad Dev Veterinary and Animal Science University, Ludhiana, - - 2014
The present study was carried out for detection and molecular characterization of fowl adenoviruses (FAdVs) associated with hydropericardium syndrome or inclusion body hepatitis in commercial broiler chickens. The FAdVs were detected in liver samples from 33 commercial broiler chicken flocks by polymerase chain reaction (PCR) using hexon gene specific primers. ...
Cho Bum-Joo - - 2013
A 55-year-old woman presented with diplopia following painful skin eruptions on the right upper extremity. On presentation, she was found to have 35 prism diopters of esotropia and an abduction limitation in the left eye. Two weeks later, she developed blepharoptosis and anisocoria with a smaller pupil in the right ...
Mena Alvaro - - 2013
There are few data of fibrosis development in chronic hepatitis B (CHB) patients classified as inactive carriers. The aim of this study is to determinate the prevalence of significant fibrosis and probable cirrhosis measured by FibroScan in real inactive CHB carriers and investigate the relationship with virological, epidemiological and metabolic ...
Adel A A Department of Surgery, Universiti Kebangsaan Malaysia Medical Centre (UKMMC), Jalan Yaakob Latif, 56000 Bandar Tun Razak, Cheras, Kuala Lumpur, - - 2013
Primary Biliary Cirrhosis (PBC) and Auto Immune Hepatitis (AIH) are autoimmune diseases of the liver which highlighted with slow destructive process of intra hepatic small bile ducts. As a result of these damages cholestasis and over the time tissue damage will happen, which leads to scarring, fibrosis and finally cirrhosis. ...
Gargouri L - - 2013
Overlap syndrome of autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) is considered when the patient presents with the diagnostic criteria of both diseases at some stage of the medical history, either simultaneously or consecutively. To report on a new case of overlap syndrome and describe the clinical presentation, progression, ...
Trott K A - - 2013
Fatty liver hemorrhagic syndrome, characterized by sudden death in overconditioned hens due to hepatic rupture and hemorrhage, is one of the leading noninfectious idiopathic causes of mortality in backyard chickens. Nutritional, genetic, environmental, and hormonal factors, or combinations of these, have been proposed yet not proven as the underlying cause. ...
Oh Won Sup - - 2013
Severe fever with thrombocytopenia syndrome (SFTS) is an emerging tick-borne disease caused by a novel bunyavirus. Although the increasing numbers of cases and deaths is of great concern, an effective treatment strategy for SFTS has not been established. We present the cases of two patients with rapidly progressing SFTS who ...
Buğdaycı Mehmet Sait MS Konya Meram Education and Research Hospital, Gastroenterohepatology Clinic, Konya, - - 2013
Dear editor, Overlap syndrome is used to define the variant forms of autoimmune hepatitis (AIH) which presents with characteristics of AIH and two cholestatic syndromes, i.e. primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC). The AIH-PBC overlap syndrome is the most common form, affecting 5% of patients with AIH ...
Singh Ankur - - 2013
Johanson-Blizzard syndrome (JBS); (OMIM: 243800) presents with features of malabsorption and dysmorphic features with onset of symptoms in infantile age group. The disorder was first described in the year 1971 with report of the first Indian case in 2004. We discuss two rare phenotypes (hepatitis and anemia) in a molecularly ...
Hazard Florette Kimberly - - 2013
Abstract Mitochondrial DNA (mtDNA) depletion syndrome is a rare cause of hepatic dysfunction in the pediatric population. It is caused by mutations in either mitochondrial or nuclear DNA (nDNA) that result in a quantitative reduction in mtDNA and, in turn, dysfunctional oxidative phosphorylation. In infants, it results in the hepatocerebral ...
Niemeyer Daniela - - 2013
Middle East respiratory syndrome coronavirus (MERS-CoV) causes severe acute respiratory infection with as yet unclear epidemiology. We previously showed that MERS-CoV counteracts parts of the innate immune response in human bronchiolar cells. Here we analyzed accessory proteins 3, 4a, 4b and 5 for their abilities to inhibit the type I ...
Nir Vered - - 2013
Gorham-Stout syndrome is a very rare syndrome characterized by progressive angiomatosis and lymphangiomas involving multiple organs. We describe herein a girl with progressive Gorham-Stout syndrome since the age of 13 years. Her disease involved the mediastinum, pericardium, vertebrae, ribs, and skull and did not respond to interferon and bisphosphonates. Propranolol ...
Hall Daniel John - - 2013
We describe the case of an 85-year-old gentleman admitted with bilateral ptosis and complete bilateral ocular paralysis. Initial differential diagnoses included myasthenia gravis, diabetic cranial neuropathy, an ischaemic event and possible occult neoplasm. Investigations did not support any of the differentials and Miller Fisher syndrome (MFS) was considered. Anti-GQ1b IgG ...
Iwasaki Eisuke - - 2013
A 42-year-old man was admitted to our hospital with a lower extremity rash, general fatigue, and abdominal discomfort. Laboratory findings revealed elevated serum transaminases as well as positivity for the hepatitis B surface antigen and the immunoglobulin M type anti-hepatitis B core antibody. He was diagnosed with a rash typical ...
Lieberman Scott M SM Division of Rheumatology, The Children's Hospital of Philadelphia and Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA. - - 2013
Sjögren syndrome is a chronic autoimmune disease affecting lacrimal and salivary glands that often is accompanied by extraglandular disease manifestations. Although common in adults, the prevalence and prognosis of childhood Sjögren syndrome are unknown, in part due to lack of child-specific diagnostic and classification criteria. This review discusses difficulties in ...
Perme Tina - - 2013
Systemic capillary leak syndrome is a rare condition, characterized by hypotension, edema, hemoconcentration and hypoalbuminemia. We describe two episodes of systemic capillary leak syndrome, following influenza A virus infection, occurring during two subsequent influenza seasons, in an eight year-old boy.
Dimitrijevic-Sreckovic Vesna - - 2013
Abstract Objective: The aim of this study was to investigate the association between metabolic syndrome and liver enzymes in overweight and obese adolescents and young adults. Methods: A total of 126 overweight and obese adolescents and young adults (age, 15-26 years), 55 (43.6%) with metabolic syndrome and 71 (56.4%) without ...
Tan Ene-Choo - - 2013
Van der Woude syndrome is the most common among syndromes which include cleft lip and/or cleft palate as one of the presentations. It is usually caused by mutations in the interferon regulatory factor 6 (IRF6) gene. We previously reported on a patient with suspected deletion of the IRF6 gene. Using ...
Sarig Ofer - - 2013
3-Methylglutaconic aciduria (3-MGCA) type IV is defined as a heterogeneous group of inborn errors featuring in common 3-MGCA and associated with primary mitochondrial dysfunction leading to a spectrum of multisystem conditions. We studied four patients who presented at birth with a clinical picture simulating a primary mitochondrial hepatic disorder consistent ...
De Santis Maria - - 2013
The liver is a lymphoid organ involved in the immune response and in the maintenance of tolerance to self molecules, but it is also a target of autoimmune reactions, as observed in primary liver autoimmune diseases (AILD) such as autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis. Further, the ...
Drain Paul K - - 2013
We describe an HIV-infected South African male who experienced two distinct episodes of disseminated giant molluscum contagiosum immune reconstitution inflammatory syndrome over a six-year period. The first episode of molluscum contagiosum immune reconstitution inflammatory syndrome occurred with rapid virologic suppression following antiretroviral therapy initiation. The second episode occurred during a ...
Gracey David M - - 2013
Tenofovir disoproxil fumarate (TDF) is increasingly used in patients with chronic hepatitis B (CHB) infection. Although associated with renal toxicity in HIV-infected patients, renal dysfunction has been reported rarely in the monoinfected CHB population. To date, TDF-associated Fanconi Syndrome has not been reported. Here we present two cases of TDF-associated ...
Igoe Ann - - 2013
To summarize the recent developments concerning the potential viral pathomechanisms and involvement of viruses in Sjögren's syndrome, and to highlight the areas for future research and therapies. Activated IFN-1 pathway plays an important part in the autoimmune disease process of Sjögren's syndrome; therefore, several therapies aiming to reduce or inhibit ...
Morimoto Noriko - - 2013
Gorham-Stout syndrome is a rare disorder characterized by progressive osteolysis that leads to the disappearance of bone. Lymphvascular proliferation causes the local destruction of bony tissue. Owing to the low incidence of this syndrome, little is known about its etiology or treatment. We present an 11-year-old girl with Gorham-Stout syndrome ...
Czaja Albert J AJ Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA. - - 2013
Autoimmune hepatitis may have cholestatic features that are outside the classical phenotype and that resemble findings in other immune-mediated liver diseases. These cholestatic phenotypes have been designated 'overlap syndromes'. To recognize the overlap syndromes in adults and manage them appropriately. The MEDLINE database was reviewed for published experiences from 1984 ...
Descamps Vincent - - 2013
Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome, initially recognized as a serious form of cutaneous drug adverse reaction, is now viewed as a drug-related syndrome that can cause life-threatening organ dysfunctions. Characteristic features include a long time interval from first drug exposure to symptom onset and a prolonged ...
Riggio Oliviero - - 2013
Budd-Chiari syndrome is a rare disorder characterized by hepatic venous outflow obstruction at any level from the small hepatic veins to the atrio-caval junction, in the absence of heart failure or constrictive pericarditis. Various imaging modalities are available for investigating the gross hepatic vascular anatomy but there are rare forms ...
Yoon Harry - - 2013
Metabolic syndrome, comprising diabetes, hypertension, central obesity, and dyslipidemia, is increasingly prevalent worldwide. We aimed to study the relationship between metabolic syndrome and the risk of liver fibrosis in patients with chronic hepatitis B (CHB) and chronic hepatitis C (CHC). In total, 954 patients (CHB, 850; CHC, 104 patients) with ...
Hung Po-Cheng - - 2013
Stevens-Johnson syndrome (SJS) is a severe idiosyncratic reaction, most commonly triggered by medications, which is characterized by fever and mucocutaneous lesions, leading to necrosis and sloughing of the epidermis. Aside from skin and mucosal manifestations, SJS may also compromise heart, liver, kidney, lung, and gastrointestinal tract. Although cholestatic liver disease ...
Du Xin-Liang - - 2013
OBJECTIVE: To explore the pathological mechanisms of Guizhi Decoction () syndrome and the therapeutic molecular mechanisms of the Guizhi Decoction, Mahuang Decoction (), Sangju Decoction () and Yinqiao Powder (), as well as the potentially biological basis that Guizhi Decoction is most effective only for the patients with Guizhi Decoction ...
Asakawa Midori G - - 2013
BACKGROUND: In humans, necrolytic migratory erythema (NME) is a syndrome with a characteristic skin rash that is associated most often with a pancreatic glucagonoma and is recognized as part of the glucagonoma syndrome. In veterinary medicine, NME (also called as superficial necrolytic dermatitis, hepatocutaneous syndrome or metabolic epidermal necrosis) has ...
Malik Saira - - 2013
The role of interferon regulatory factor 6 (IRF6) gene mutations in causing Van der Woude syndrome (VWS) and poplyteal pterygium syndrome has been described in different populations worldwide. The former is one of the major syndromes of cleft lip and/or cleft palate with the distinct phenotype of presence of pits ...
Prunotto Giulia - - 2013
Wolf-Hirschhorn syndrome (WHS) is a rare microdeletion syndrome associated with a characteristic facial appearance, failure to thrive, psychomotor delays, and various major malformations of internal organs; many medical complications have been described (feeding difficulties, epilepsy, hearing problems). Benign or malignant oncologic problems are not a typical feature of the natural ...
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