| Congenital hepatic fibrosis with congenital heart disease. A family study with ultrastructural features of the liver. | |
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MedLine Citation:
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PMID: 7429344 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Y Naveh; N Roguin; R Ludatscher; L Auslaender; A Schramek; M Aharon |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Gut Volume: 21 ISSN: 0017-5749 ISO Abbreviation: Gut Publication Date: 1980 Sep |
Date Detail:
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Created Date: 1981-01-29 Completed Date: 1981-01-29 Revised Date: 2009-11-18 |
Medline Journal Info:
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Nlm Unique ID: 2985108R Medline TA: Gut Country: ENGLAND |
Other Details:
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Languages: eng Pagination: 799-807 Citation Subset: AIM; IM |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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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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