Goldston syndrome with congenital hepatic fibrosis: A rare cause of neonatal cholestasis
نویسندگان
چکیده
منابع مشابه
Congenital Hepatic Fibrosis: An Uncommon Cause of Chronic Renal Failure
Congenital Hepatic Fibrosis (CHF) is a rare disease that affects both the liver and kidneys. Congenital hepatic fibrosis (CHF) is an autosomal recessive inherited malformation defined pathologically by a variable degree of periportal fibrosis and irregularly shaped proliferating bile ducts. Affected individuals also have impaired renal function, usually caused, in children and teenagers, by an...
متن کاملcongenital hepatic fibrosis: an uncommon cause of chronic renal failure
congenital hepatic fibrosis (chf) is a rare disease that affects both the liver and kidneys. congenital hepatic fibrosis (chf) is an autosomal recessive inherited malformation defined pathologically by a variable degree of periportal fibrosis and irregularly shaped proliferating bile ducts. affected individuals also have impaired renal function, usually caused, in children and teenagers, by an...
متن کاملCongenital hepatic fibrosis in an aborted calf
An aborted female Holstein foetus with marked generalized anasarca was referred to the Excellence Centre for Ruminant Abortion and Neonatal Mortality, Ferdowsi University of Mashhad. On postmortem examination, red-tinged ascites, pale and firm liver with extreme irregularity and numerous round to oval slightly raised foci on the capsular surface were seen. Histological examination revealed wide...
متن کاملArthrogryposis-renal tubular dysfunction-cholestasis syndrome: a cause of neonatal cholestasis. Case report.
Arthrogryposis-renal dysfunction-cholestasis syndrome is a rare lethal disorder that involves multipl organ system. It is inherited autosomal recessive and caused by defects in the VPS33B and VIPAR genes. Three cardinal findings of this syndrome are arthrogryposis, renal tubular dysfunction and cholestasis.The other organ involvements including ichthyosis, central nervous system malformation, p...
متن کاملManagement of Hepatic Cholestasis
Hepatic cholestasis is characterized by elevated alkaline phosphatase and gama - glutamyl transpeptidase levels which is then followed by conjugated hyperbilirubinemia. It is classified into intrahepatic and extrahepatic cholestasis. Intrahepatic cholestasis indicates hepatocellular dysfunction or the presence of an obstructive lesion in intrahepatic bile ducts distal to biliary canalicular sy...
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ژورنال
عنوان ژورنال: Intractable & Rare Diseases Research
سال: 2019
ISSN: 2186-361X,2186-3644
DOI: 10.5582/irdr.2019.01017