Document Detail


Congenital hepatic fibrosis with congenital heart disease. A family study with ultrastructural features of the liver.
MedLine Citation:
PMID:  7429344     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
A family with congenital hepatic fibrosis (CHF) and congenital heart disease (CHD) is presented. The consanguineous healthy parents gave birth to 12 children of whom 10 survived. One son had CHF and CHD, one daughter had CHF and a second daughter had CHD. Three other siblings probably had small a ventricular septal defect and another one probably had mild pulmonary valve stenosis. Development of portal hypertension and hypersplenism necessitated performing shunt operation on both siblings suffering from congenital hepatic fibrosis. Ultrastructural findings were giant mitochondria with large laminar inclusions in hepatocytes, and excess of villi and whorls of membranes and collagen fibrils between hepatocytes.
Authors:
Y Naveh; N Roguin; R Ludatscher; L Auslaender; A Schramek; M Aharon
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Gut     Volume:  21     ISSN:  0017-5749     ISO Abbreviation:  Gut     Publication Date:  1980 Sep 
Date Detail:
Created Date:  1981-01-29     Completed Date:  1981-01-29     Revised Date:  2009-11-18    
Medline Journal Info:
Nlm Unique ID:  2985108R     Medline TA:  Gut     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  799-807     Citation Subset:  AIM; IM    
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Child
Female
Heart Defects, Congenital / genetics*
Humans
Infant
Liver / ultrastructure*
Liver Diseases / congenital,  genetics*,  pathology
Male
Microscopy, Electron
Pedigree
Comments/Corrections

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