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 autosomal recessive polycystic kidney disease (ARPKD). Impaired renal function associated with CHF in adults is caused by an autosomal dominant polycystic kidney disease (ADPKD). Case presentation: We report the case of a 8-year-old Iranian girlwas admitted to our hospital for evaluation ofrenal failure. In patient hepatomegaly was noted incidentally on a routine physical examination and then kidney biopsy showed global sclerosis and A liver biopsy revealed proliferation of collagen fibres surrounding the portal area, a finding that was compatible with congenital hepatic fibrosisand our patient was scheduled for kidney and liver transplantation. Conclusion: The relationship of ARPKD to CHF is the subject of substantial controversy. Some clinicians suggest that the two conditions represent one disorder with a range of clinical/pathological presentations Key word: Congenital Hepatic Fibrosis Polycystic Kidney Disease, CRF.
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 کامل
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 کامل
BACKGROUND Karyomegalic nephropathy, first identified in 1974, represents an increasingly recognized, but perhaps underdiagnosed condition associated with interstitial nephritis. It undoubtedly leads to end-stage renal disease requiring renal support. METHODS AND RESULTS We present a series of six cases of karyomegalic nephropathy. The age at diagnosis was 9-51 years, median 33 years. Impaire...متن کامل
Background: Adrenal hematoma is a very rare acquired cause of neonatal hypertension. In this study, we report a case of neonatal hypertension associated with adrenal hematoma. A male neonate was immediately transferred to our neonatal intensive care unit after delivery due to meconium aspiration. He needed to be on mechanical ventilation support. During his hospital stay, hospital records showe...متن کامل
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 کامل
To cite: Alvarez-Acosta L, Rodriguez-Esteban M, Hernández Afonso J. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2013202881 DESCRIPTION An 83-year-old woman with a suspected coronary atherosclerotic disease in the setting of severe left ventricular dysfunction underwent a coronary angiography at our institution. Prior to catheterisation, Allen test was performe...متن کامل
ذخیره در منابع من
دوره 2 شماره 2.1
صفحات 40- 40
تاریخ انتشار 2014-04-01
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