| Liver disease in children with alpha 1-antitrypsin deficiency without neonatal cholestasis. | |
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MedLine Citation:
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PMID: 6984276 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Thirteen children with alpha 1-antitrypsin deficiency (8 PiZ and 5 PiSZ) were investigated at ages ranging from 4 to 6. None had had neonatal cholestasis. Nine, mainly the PiZ individuals, had increased serum concentration of transaminases. Liver biopsy was performed in 7 patients with increased serum levels of transaminases. One of these patients had cirrhosis and 4 had moderate to severe fibrosis. The results indicate that alpha 1-antitrypsin deficient individuals, also without neonatal cholestasis syndrome run a high risk of developing serious liver disease, already in childhood. The cirrhotic patient was the only one who had increased excretion of bile acids in urine. |
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Authors:
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A Nemeth; B Strandvik |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Acta paediatrica Scandinavica Volume: 71 ISSN: 0001-656X ISO Abbreviation: Acta Paediatr Scand Publication Date: 1982 Nov |
Date Detail:
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Created Date: 1983-03-17 Completed Date: 1983-03-17 Revised Date: 2009-11-11 |
Medline Journal Info:
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Nlm Unique ID: 0000211 Medline TA: Acta Paediatr Scand Country: SWEDEN |
Other Details:
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Languages: eng Pagination: 1001-5 Citation Subset: IM |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Bile Acids and Salts
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urine Child Child, Preschool Cholestasis / metabolism Humans Infant, Newborn Infant, Newborn, Diseases / metabolism Liver / pathology Liver Cirrhosis / metabolism* Liver Function Tests Male Risk Transaminases / blood alpha 1-Antitrypsin Deficiency* |
| Chemical | |
Reg. No./Substance:
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0/Bile Acids and Salts; EC 2.6.1.-/Transaminases |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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