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Rota Scalabrini Delia - - 2014
Vanishing bile duct syndrome has been associated with different pathologic conditions (adverse drug reactions, autoimmune diseases, graft versus host disease, and cancer). Though its causes are unknown, an immune-related pathogenesis is the most likely one. Vanishing bile duct syndrome can evolve to hepatic failure and, eventually, to death. The treatment ...
Kook P H PH Clinic for Small Animal Internal Medicine, Vetsuisse Faculty, University of Zurich, 8057, Zurich, - - 2014
A 12-month-old beagle presented for anorexia, pyrexia and vomiting. The dog had been treated intermittently with antibiotics and corticosteroids for inappetence and lethargy since five months of age. Previous laboratory abnormalities included macrocytosis and neutropenia. At presentation, the dog was lethargic, febrile and thin. Laboratory examination findings included anaemia, a ...
Floreani Annarosa A Department of Surgery, Oncology and Gastroenterology, University of Padova, Padova, - - 2014
In this article, the authors use the term "overlaps" to refer to the coexistence of primary biliary cirrhosis (PBC) with another autoimmune condition that involves the liver or extrahepatic organs. Diagnosing PBC-autoimmune hepatitis (PBC-AIH) overlap syndrome remains a challenge, especially because there is still no consensus on the most appropriate ...
Morgan Michael L ML Department of Ophthalmology (MLM, AEBP, US, SY, AGL), Houston Methodist Hospital, Houston, Texas; Baylor College of Medicine (NL, AGL), Houston, Texas; Departments of Ophthalmology, Neurology, and Neurosurgery (AGL), Weill Cornell Medical College, Houston, Texas; UTMB (AGL), Galveston, Texas and the UTMD Anderson Cancer Center (AGL), Houston, Texas; and University of Iowa Hospitals and Clinics (AGL), Iowa City, - - 2014
: We present a woman with acute onset of bilateral ophthalmoparesis and ataxia occurring 4 weeks after gastroenteritis. Serum antibody titers against asialo-GM1 and GD1a, typically associated with inflammatory axonal neuropathies, were elevated but titers against anti-GQ1b, the most commonly found antibody found in the Miller Fisher variant of Guillain-Barre ...
Trang N T NT Department of Veterinary Pathology, Faculty of Agriculture, University of Miyazaki, Miyazaki 889-2192, Japan; Department of Veterinary Public Health, Faculty of Veterinary Medicine, Hanoi University of Agriculture, Hanoi, - - 2014
A 2-month-old Japanese black calf was presented with a history of weight loss, exophthalmos and subcutaneous oedema of the brisket. Urinalysis and serum biochemistry showed proteinuria and hypoproteinaemia suggestive of nephrotic syndrome. Microscopically, lesions in the kidney were characterized by proliferation of mesangial cells and diffuse thickening of the glomerular ...
Latov Norman N Department of Neurology and Neuroscience, Weill Medical College of Cornell University, 1305 York Avenue, Suite 217, New York, NY 10021, - - 2014
Chronic neuropathies are operationally classified as primarily demyelinating or axonal, on the basis of electrodiagnostic or pathological criteria. Demyelinating neuropathies are further classified as hereditary or acquired-this distinction is important, because the acquired neuropathies are immune-mediated and, thus, amenable to treatment. The acquired chronic demyelinating neuropathies include chronic inflammatory demyelinating ...
Sanz-Bueno Jimena J Department of Dermatology, 12 de Octubre University Hospital, Madrid, - - 2014
We describe a patient with paraneoplastic autoimmune multiorgan syndrome (PAMS) secondary to a lymphoblastic T- cell lymphoma who presented with a lichenoid dermatitis and vitiligo, later developing bronchiolitis obliterans and autoimmune hepatitis. Notably, he had no detectable autoantibodies. The development of vitiligo and autoimmune hepatic involvement probably indicate a role ...
Sanada Yukihiro Y 1 Department of Surgery, Jichi Medical University, Shimotsuke, - - 2014
Abstract We report a 71-year-old man who had undergone pylorus-preserving pancreatoduodenectomy (PPPD) using PPPD-IV reconstruction for cholangiocarcinoma. For 6 years thereafter, he had suffered recurrent cholangitis, and also a right liver abscess (S5/8), which required percutaneous drainage at 9 years after PPPD. At 16 years after PPPD, he had been ...
Morgan Stuart A SA Centre for Endocrinology, Diabetes and Metabolism, School of Clinical and Experimental Medicine, Institute of Biomedical Research, University of Birmingham, Birmingham B15 2TT, United Kingdom; - - 2014
The adverse metabolic effects of prescribed and endogenous glucocorticoid (GC) excess, Cushing syndrome, create a significant health burden. We found that tissue regeneration of GCs by 11β-hydroxysteroid dehydrogenase type 1 (11β-HSD1), rather than circulating delivery, is critical to developing the phenotype of GC excess; 11β-HSD1 KO mice with circulating GC ...
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 ...
Choi Clark K - - 2014
Anesthetic management for massive blood loss in liver surgery concomitant with hemodynamic instability secondary to carcinoid crisis can be challenging in the perioperative setting. Hypotension, diarrhea, facial flushing, bronchospasm, and tricuspid and pulmonic valvular diseases are the common manifestations of carcinoid syndrome. This report illustrates the importance of early recognition ...
Adedeji Adeyemi O AO Christopher Bond Life Sciences Center, University of Missouri, Columbia, MO 65211 Department of Molecular Microbiology & Immunology, University of Missouri School of Medicine, Columbia, MO - - 2014
We have previously shown that SSYA10-001 blocks Severe Acute Respiratory Syndrome Coronavirus (SARS-CoV) replication by inhibiting SARS-CoV helicase (nsp13). Here, we show that SSYA10-001 also inhibits replication of two other coronaviruses, Mouse Hepatitis Virus (MHV) and Middle Eastern Respiratory Syndrome Coronavirus (MERS-CoV). A putative binding pocket for SSYA10-001 was identified ...
Tzachanis Dimitrios D Cedars-Sinai Medical Center, Los Angeles, Calif., - - 2014
This is the case of a 79-year-old man with chronic lymphocytic leukemia who presented with Guillain-Barré syndrome with features overlapping with the Miller Fisher syndrome and Bickerstaff brainstem encephalitis and positive antiganglioside GQ1b antibody about 6 months after treatment with bendamustine and rituximab. His clinical and neurologic condition continued to ...
Belcastro Vincenzo V Neurology Unit, Department of Medicine, S. Anna Hospital, Como, Italy. Electronic address: - - 2014
Opsoclonus-myoclonus syndrome (OMS) is characterized by opsoclonus and arrhythmic-action myoclonus that predominantly involves the trunk, limbs, and head. Human herpes virus-6 (HHV-6) can rarely cause encephalitis in immunocompetent children and adults. Here we report on a case of OMS associated with HHV-6 rhomboencephalitis. HHV-6 infection should be considered in OMS ...
Davey Megan G MG Division of Developmental Biology; The Roslin Institute and R(D)SVS; University of Edinburgh; Midlothian, - - 2014
Sonic hedgehog plays an essential role in maintaining hepatoblasts in a proliferative non-differentiating state during embryogenesis. Transduction of the Hedgehog signaling pathway is dependent on the presence of functional primary cilia and hepatoblasts, therefore, must require primary cilia for normal function. In congenital syndromes in which cilia are absent or ...
Picque Marie M Laboratoire de biologie, GHI du Raincy Montfermeil, - - 2014
Hepatosplenic T-cell lymphoma (HSCTL) is rare and caracterised by gama/delta T-lymphocyte proliferation in the spleen, bone marrow and liver. Association between HSCTL and hemophagocytic syndrome is known, but association with chronic lymphoid leukaemia (CLL) has not been described to our knowledge. We report the case of a 76 year's old ...
Kistler C Andrew CA 1 Department of Internal Medicine, 2 Department of Neurology, 3 Department of Medicine, Division of Gastroenterology and Hepatology, Thomas Jefferson University Hospital, Philadelphia, PA, - - 2014
Posterior reversible encephalopathy syndrome (PRES) is a rare complication of transarterial chemoembolization (TACE) used to treat liver metastases and has never been reported in a patient with metastatic uveal melanoma (UM) to the liver. We report the first case of PRES secondary to TACE with drug eluting beads (DEBs) loaded ...
Karadas Sevdegul - - 2014
Alveolar echinococcosis of the liver is caused by the tapeworm Echinococcus multilocularis. Budd-Chiari Syndrome (BCS) is a hepatic venous outflow tract obstruction and involves abdominal pain, hepatomegaly and high-gradient ascites. A low-gradient ascites in connection with the Syndrome rarely occurs in case of alveolar echinococcosis of the liver. Here we ...
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 ...
Søreide Kjetil - - 2014
Budd-Chiari syndrome is an eponym for "hepatic venous outflow tract obstruction". BCS left untreated has a high mortality rate. Diagnosis can be difficult because of the wide spectrum of presentation of the disease and the varying severity of liver damage. The onset of disease may be insidious, with a chronic, ...
Li Ke - - 2014
Budd-Chiari syndrome (BCS) is a rare and life-threatening disorder secondary to hepatic venous outflow obstruction. How to manage this complex disease has haunted many surgeons. The aim of this study is to investigate the treatment of Budd-Chiari syndrome in our hospital. The clinical data of 280 BCS patients were analyzed ...
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 ...
Karateke Atilla A Department of Obstetric and Gynecology, School of Medicine, Mustafa Kemal University, 3100 Hatay, - - 2014
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 ...
Katkar A S AS Department of Radiology, University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900, - - 2014
Budd-Chiari syndrome refers to hepatic pathology secondary to diminished venous outflow, most commonly associated with venothrombotic disease. Clinically, patients with Budd-Chiari present with hepatomegaly, ascites, abdominal distension, and pain. On imaging, Budd-Chiari syndrome is hallmarked by occluded IVC and or hepatic veins, caudate lobe enlargement, heterogeneous liver enhancement, intrahepatic collaterals, ...
Ferreira Helena H 0000-0001-8578-4091 Departamento de Pediatria, Centro Hospitalar do Alto Ave, Rua dos Cutileiros, Creixomil, 4835-044 Guimarães, - - 2014
Chickenpox, resulting from primary infection by the varicella-zoster virus, is an exanthematous disease very common during childhood and with good prognosis. However, serious complications, namely, neurological syndromes, may develop during its course, especially in risk groups, including adolescents. Peripheral facial palsy is a rare neurologic complication that has been previously ...
Wang Lei L Institute of Digestive Diseases, Longhua Hospital, Shanghai University of Traditional Chinese Medicine, Shanghai 200032, China ; Department of Hepatology, Longhua Hospital, Shanghai University of Traditional Chinese Medicine, Shanghai 200032, - - 2014
Objective. To investigate the dendritic cells (DCs) maturity differences of HBeAg negative chronic hepatitis B (CHB) patients with different spleen deficiency (SD) syndromes and explore the role of syndrome differentiation in the therapeutic evaluation of Chinese medicine. Methods. 120 participants were recruited including three treatment groups in different SD syndrome ...
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 ...
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