Results 21 to 30 of about 24,959 (186)
Congenital hepatic fibrosis complicated with Caroli’s disease: a case report [PDF]
Xin yixue, 2023 Congenital hepatic fibrosis （CHF） complicated with Caroli’s disease is a rare autosomal recessive genetic disease with non-specific clinical and imaging manifestations， mainly including portal hypertension and complications， which is likely to be missed ...
Yang Shuang, Liu Yaping, Gao Lili, Duan Xuefei
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Congenital hepatic fibrosis [PDF]
Serbian Journal of Experimental and Clinical Research, 2013 Congenital hepatic fi brosis (CHF) is a rare developmental disorder of the portobiliary system and most commonly associated with polycystic kidney disease. The pattern of inheritance of this disorder is autosomal recessive. The exact prevalence of CHF is unknown (estimated from 1:10000 to 1:20000).
Zorica Raskovic +3 more
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Archives of Surgery, 1965 CONGENITAL hepatic fibrosis is a rare cause of intrahepatic portal hypertension in children or young adults. Hepatic function is well preserved, as in extrahepatic obstruction, making these patients acceptable candidates for surgical decompression of their portal hypertension.
Laurence J. McCarthy +2 more
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Telmisartan ameliorates fibrocystic liver disease in an orthologous rat model of human autosomal recessive polycystic kidney disease. [PDF]
PLoS ONE, 2013 Human autosomal recessive polycystic kidney disease (ARPKD) produces kidneys which are massively enlarged due to multiple cysts, hypertension, and congenital hepatic fibrosis characterized by dilated bile ducts and portal hypertension.
Daisuke Yoshihara +8 more
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Caroli Syndrome in a Child: A Case Report
Case Reports in Clinical Practice, 2019 Introduction: Caroli syndrome is a congenital disorder characterized by multiple segmental or saccular dilatations of the intrahepatic bile ducts associated with congenital hepatic fibrosis.
Pantea Tajik, Amir Hossein Goudarzian
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Monolobar Caroli’s disease with renal cysts: Case report
The Egyptian Journal of Radiology and Nuclear Medicine, 2014 Caroli’s disease is autosomal recessive, non-obstructive dilatation of intrahepatic biliary ducts. The exact etiology is unclear. Two variants of Caroli’s disease are well known-simple; in which bile ducts are dilated without hepatic fibrosis and the ...
Shruti Thakur +5 more
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Medical Journal of Dr. D.Y. Patil Vidyapeeth, 2020 Hypoplasia of the right hepatic lobe is a rare and usually asymptomatic anomaly, which sometimes may be associated with portal hypertension but rarely with retroperitoneal fibrosis.
Sumit Verma +3 more
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Clinical and Paraclinical Findings in Children With Congenital Hepatic Fibrosis: A Single Center 10-Year Study [PDF]
Hospital Practices and Research, 2020 Background: Congenital hepatic fibrosis (CHF) is an autosomal hereditary disorder affecting the porto-biliary system. It is a rare hereditary disorder often presenting in childhood or adolescence with hepatomegaly, splenomegaly, and gastrointestinal ...
Seyed Mohsen Dehghani +8 more
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A preterm neonate with infantile liver failure syndrome 1 due to leucyl-tRNA synthetase 1 gene (<i>LARS1</i>) mutations with a histopathologic phenotype of neonatal hemochromatosis. [PDF]
JPGN RepAbstract We report a case of a premature, growth‐restricted female infant with feeding intolerance and coagulopathy, treated initially for sepsis, who progressed to neonatal acute liver failure and end‐stage hepatic encephalopathy after a prolonged hospitalization with extensive diagnostic evaluation, and was found by autopsy to have histopathologic ...
Bruder A +3 more
europepmc +2 more sources
Ciência Rural, 2022 : Caroli syndrome is characterized by a combination of intrahepatic biliary ductal ectasia and congenital ductal fibrosis due to the failure of involution of ductal plates and large intrahepatic ducts.
Bianca Santana de Cecco +7 more
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