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Experience of a single center with congenital hepatic fibrosis:A review of the literature

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World Journal of Gastroenterology 2010年第6期16卷 683-690页

作者： Ali Shorbagi Yusuf Bayraktar Faculty of Medicine Department of Internal MedicineGastroenterology ClinicHacettepe University

congenital hepatic fibrosis(CHF) is an autosomal recessive inherited malformation defined pathologically by a variable degree of periportal fibrosis and irregularly shaped proliferating bile *** is one of the fibropolycystic diseases,which also include Caroli disease,autosomal dominant polycystic kidney disease,and autosomal recessive polycystic kidney disease. Clinically it is characterized by hepatic fibrosis,portal hypertension,and renal cystic *** is known to occur in association with a range of both inherited and non-inherited disorders,with multiorgan involvement,as a result of ductal plate *** of the similarities in the clinical picture,it is necessary to differentiate CHF from idiopathic portal hypertension and early liver cirrhosis,for which a liver biopsy is essential. Radiological tests are important for recognizing involvement of other organ *** regards to our experience at Hacettepe University,a total of 26 patients have been diagnosed and followed-up between 1974 and 2009 with a diagnosis of *** with Caroli syndrome was the most common diagnosis,with all such patients presenting with symptoms of recurrentcholangitis and symptoms related to portal hypertension. Although portal fibrosis is known to contribute to the ensuing portal hypertension,it is our belief that portal vein cavernous transformation also plays an important role in its *** all patients with CHF portal vein morphology should be evaluated by all means since portal vein involvement results in more severe and complicated portal *** associations include the Joubert and Bardet-Biedl syndromes.

关键词： congenital hepatic fibrosis Fibropolycystic disorders Portal hypertension Bardet Biedl syndrome

Is portal vein cavernous transformation a component of congenital hepatic fibrosis?

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World Journal of Gastroenterology 2007年第13期13卷 1928-1929页

作者： Ozlem Yonem Yusuf Bayraktar Hacettepe University Faculty of Medicine Department of Gastroenterology Sihhiye 06100 Ankara Turkey Hacettepe University Faculty of Medicine Department of Gastroenterology Sihhiye 06100 Ankara Turkey MD Series Editor

congenital hepatic fibrosis (CHF) is an autosomal recessive disorder that belongs to the family of fibropolycystic liver diseases. This family includes a spectrum of disorders which are usually found in combination with each other and are usually inherited. Clinically fibropolycystic diseases have three effects being present in different proportions, those of a space occupying lesion, of portal hypertension and of cholangitis. In most patients, the first manifestations of CHF are signs and symptoms related to portal hypertension such as splenomegaly and varices. Portal hypertension in these patients has been attributed to the hypoplasia or compression of the portal vein radicles in the fibrous bands. Cavernous transformation of the portal vein (CTPV) is a relatively rare condition resulting from extrahepatic portal vein obstruction with recanalization or collateral vein formation to bypass the obstruction. It has been found that patients with CHF having an accompanying CTPV have relatively large splenomegaly and suffers more frequent episodes of bleeding from esophageal *** believe that CTPV is a congenital component of CHF and also one of the important causative factors of portal hypertension in these patients.

关键词： congenital hepatic fibrosis Cavernoustransformation of portal vein Portal hypertension

Novel variant syndrome associated with congenital hepatic fibrosis

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World Journal of Clinical Cases 2015年第10期3卷 904-910页

作者： Yusuf Bayraktar Ozlem Yonem Kubilay Varl? Hande Taylan Ali Shorbagi Cenk Sokmensuer Department of Gastroenterology Faculty of MedicineHacettepe University Department of Gastroenterology Cumhuriyet University Department of Neurology Hacettepe University Department of Ophthalmology Hacettepe University Department of Pathology Hacettepe University

congenital hepatic fibrosis is part of many different malformation syndromes, of which oculo-encephalohepato-renal syndrome is the most common. These syndromes largely overlap, and so accurate classification of individual patients may be difficult. We present herein three syndromic siblings who were products of a consanguineous marriage. We investigated in detail at least six organ systems in these patients, namely the liver, brain, eye, kidneys, skeleton, and gonads. The common features observed in these three cases were congenital hepatic fibrosis, retinitis pigmentosa, truncal obesity, rotatory nystagmus, mental retardation, advanced myopia, and high-arched palate. The clinical dysmorphology in these patients was distinct and lacked the major features of the known syndromes associated with congenital hepatic fibrosis. Although some features of these presented cases are similar to those found in Bardet-Biedl syndrome(BBS), the absence of some major criteria of BBS(polydactyly, renal abnormality, and hypogonadism) suggests that this may be a new syndrome. All three patients remain under follow-up in the departments of Gastroenterology, Ophthalmology, and Neurology at Hacettepe University.

关键词： congenital hepatic fibrosis Nystagmus Mental retardation Retinitis pigmentosa High-arched palate

Portal ductopathy:Clinical importance and nomenclature

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World Journal of Gastroenterology 2011年第11期17卷 1410-1415页

作者： Yusuf Bayraktar Department of Internal Medicine Gastroenterology ClinicHacettepe University HospitalsAnkara 06100Turkey

Non-cirrhotic portal hypertension(PHT)accounts for about 20%of all PHT cases,portal vein thrombosis(PVT) resulting in cavernous transformation being the most common *** known complications of PHT may be encountered in patients with chronic ***,the effect of this entity on the biliary tree and pancreatic duct has not yet been fully ***,a dispute remains regarding the nomenclature of common bile duct abnormalities which occur as a result of chronic *** many clinical reports have focused on biliary abnormalities,only a few have evaluated both the biliary and pancreatic ductal *** this review the relevant literature evaluating the effect of PVT on both ductal systems is discussed,and findings are considered with reference to results of a prominent center in Turkey,from which the term"portal ductopathy"has been put forth to replace"portal biliopathy".

关键词： Portal hypertension Portal vein thrombosis Portal vein cavernous transformation congenital hepatic fibrosis Non-cirrhotic portal hypertension Portal ductopathy Portal double ductopathy Portal biliopathy

Heritable Chronic Cholestatic Liver Diseases:A Review

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Journal of Clinical and Translational Hepatology 2024年第8期12卷 726-738页

作者： Jasmine Tidwell George Y.Wu Department of Medicine University of Connecticut Health CenterFarmingtonCTUSA Division of Gastroenterology-Hepatology University of Connecticut Health CenterFarmingtonCTUSA

Chronic cholestasis due to heritable causes is usually diagnosedin ***,many cases can present andsurvive into *** time course varies considerablydepending on the underlying *** data usuallyreveal elevated conjugated hyperbilirubinemia,alkalinephosphatase,and gamma-glutamyl *** be asymptomatic;however,when present,the typicalsymptoms are pruritus,jaundice,fatigue,and *** diagnostic methods and management requireddepend on the underlying *** development of genome-wide associated studies has allowed the identificationof specific genetic mutations related to the pathophysiologyof cholestatic liver *** aim of this review was tohighlight the genetics,clinical pathophysiology,presentation,diagnosis,and treatment of heritable etiologies of chroniccholestatic liver disease.

关键词： Chronic cholestasis Cholestatic liver disease congenital hepatic fibrosis Caroli disease Caroli syndrome Alagille syndrome Biliary atresia

Pediatric non-cirrhotic portal hypertension:Endoscopic outcome and perspectives from developing nations

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World Journal of Hepatology 2021年第10期13卷 1269-1288页

作者： Moinak Sen Sarma Jayendra Seetharaman Department of Pediatric Gastroenterology Sanjay Gandhi Postgraduate Institute of Medical SciencesLucknow 226014Uttar PradeshIndia

Non-cirrhotic portal hypertension(NCPH)forms an important subset of portal hypertension in *** bleed and splenomegaly are their predominant *** features show cytopenias(hypersplenism)and preserved hepatic synthetic *** sessions of endoscopic variceal ligation or endoscopic sclerotherapy eradicate esophageal varices in almost all *** variceal eradication,there is an increased risk of other complications like secondary gastric varices,cholangiopathy,colopathy,growth failure,especially in extra-hepatic portal vein obstruction(EHPVO).Massive splenomegaly-related pain and early satiety cause poor quality of life(QoL).Meso-Rex bypass is the definitive therapy when the procedure is anatomically feasible in *** portosystemic shunt surgeries with splenectomy are indicated when patients present late and spleen-related issues *** surgeries prevent rebleed,improve growth and ***-cirrhotic portal fibrosis(NCPF)is a less common cause of portal hypertension in children in developing *** in the second decade,massive splenomegaly and patent portal vein are discriminating features of *** surgery is required in severe cases when endotherapy is insufficient for the *** hepatic fibrosis(CHF)presents with firm palpable liver and *** plate malformation forms the histological hallmark of *** is commonly associated with Caroli’s disease,renal cysts,and syndromes associated with neurological *** CHF has a favourable prognosis requiring *** transplanta-tion is required when there is decompensation or recurrent cholangitis,especially in Caroli’s *** liver-kidney transplantation is indicated when both liver and renal issues are present.

关键词： Extrahepatic portal vein obstruction Non-cirrhotic portal fibrosis Portosystemic shunt surgery congenital hepatic fibrosis