نتایج جستجو برای: biliary disorder
تعداد نتایج: 624634 فیلتر نتایج به سال:
Background and purpose: Evaluation of suspected biliary obstruction is performed by common old methods such as Ultrasound, CT, and invasive cholangiography. These techniques have limitations due to the poor visualization of intraductal stones (US, CT) and the need for an invasive procedure (ERCP, PTC). Magnetic resonance cholangiography (MRC) is noninvasive imaging modality that provides good...
A 56-year-old woman who underwent living liver transplantation for primary biliary cirrhosis seven years previously presented with symptoms of repetitive cholangitis. She had been suffering from a wet cough for the past three months and had also been treated for bacterial pneumonia, which tended to easily flare up. When referred to our hospital, computed tomography showed consolidation with an ...
BACKGROUND Immunoglobulin (Ig) G4-associated cholangitis (IAC) is an inflammatory disorder of the biliary tract displaying characteristic features of IgG4-related disease (IgG4-RD): elevation of IgG4 serum levels, infiltration of IgG4+ plasma cells in the affected tissue, and good response to immunosuppressive treatment. METHODS AND RESULTS The clinical presentation of IAC is often misleading...
Hypercholesterolemia is a common disorder in adult population, but total cholesterol concentrations beyond 1000 mg/dl occur rarely, and are found in patients with homozygous familial hypercholesterolemia and familial lecithin-cholesterol acyltransferase deficiency, in chronic graft-versus-host disease of the liver, after intravenous infusion of fat emulsion (intralipid), in newborn infants with...
Autosomal recessive polycystic kidney disease (ARPKD) is a developmental disorder that mainly affects the kidneys and the biliary tract. Affected patients often have massively enlarged cystic kidneys as well as congenital hepatic fibrosis (CHF) characterized by dilated bile ducts and associated peribiliary fibrosis. This review will examine what is known about ARPKD-associated liver disease and...
We describe a neonatal patient with biliary ductopenia featuring duplication of exon 6 of the JAG1 gene. Facial alterations were observed, consisting of a prominent forehead, sunken eyes, upward slanting palpebral fissures, hypertelorism, flat nasal root and prominent chin. From birth, these were accompanied by the development of haematuria and renal failure and by renal Doppler findings indica...
Inherited copper toxic disease, Wilson's disease, is an autosomal recessive disorder arising from a defect in biliary copper excretion. Although there are several pathognomonic clinical features, such a multisystem disease can be difficult to diagnose, particularly in the early stages of copper toxicity. Even measurements of serum copper and caeruloplasmin, the major copper-transporting protein...
BACKGROUND Mutations in either of two genes comprising the STSL locus, ATP-binding cassette (ABC)-transporters ABCG5 (encoding sterolin-1) and ABCG8 (encoding sterolin-2), result in sitosterolemia, a rare autosomal recessive disorder of sterol trafficking characterized by increased plasma plant sterol levels. Based upon the genetics of sitosterolemia, ABCG5/sterolin-1 and ABCG8/sterolin-2 are h...
Aviation-related mechanism may exist in the post-cholecystectomy syndrome (PCS) of aircrew patients. The aim of this study was to test this hypothesis on vivo rabbit model and to explore the mechanism by using a novel telemetric method. We constructed a bile duct-to-intestinal bridge bypass on 30 rabbits, with a telemetry implant attached to the Oddi's sphincter. Then a telemetric recording sys...
A 63-year-old woman suffering from progressive systemic sclerosis for about 20 years disclosed symptoms of liver disease within the last three years. Diagnosis of biliary cirrhosis was established on the basis of clinical picture, pathological examination of the hepatic tissue sample, immunological tests, and x-ray studies. Association of systemic sclerosis with primary biliary cirrhosis is...
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