| Nodular regenerative hyperplasia and severe portal hypertension in cystinosis. | |
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MedLine Citation:
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PMID: 16527704 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND & AIMS: Cystinosis is a rare autosomal-recessive disorder characterized by the intralysosomal accumulation of cystine, which is responsible for widespread tissue destruction. Liver biopsy specimens of patients with cystinosis show cystine crystal formation in Kupffer cells. However, significant liver disease and portal hypertension is not a common complication of cystinosis. We report the case histories of 2 young men with poorly treated nephropathic cystinosis who developed noncirrhotic portal hypertension with evidence of nodular regenerative hyperplasia (NRH). METHODS: Liver biopsy examinations, upper and lower endoscopy with biopsy examination, imaging studies, venous pressure measurements, and laboratory investigations were used to evaluate the causes of the liver disease and portal hypertension. RESULTS: Histologic examination of liver biopsy specimens from both patients showed changes characteristic of NRH with portal hypertension documented by measurement of pressure gradients. In addition, endoscopy in the first patient showed varices and portal hypertensive gastropathy. CONCLUSIONS: NRH was confirmed by histologic examination of the liver in both patients and is the likely cause of their portal hypertension. NRH may represent a rare, late complication of cystinosis, although the mechanism remains undefined. |
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Authors:
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Kevin O'Brien; Nadeem Hussain; Bradley A Warady; David E Kleiner; Robert Kleta; Isa Bernardini; Theo Heller; William A Gahl |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, N.I.H., Intramural |
Journal Detail:
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Title: Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association Volume: 4 ISSN: 1542-3565 ISO Abbreviation: Clin. Gastroenterol. Hepatol. Publication Date: 2006 Mar |
Date Detail:
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Created Date: 2006-03-10 Completed Date: 2006-06-06 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 101160775 Medline TA: Clin Gastroenterol Hepatol Country: United States |
Other Details:
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Languages: eng Pagination: 387-94 Citation Subset: IM |
Affiliation:
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Section on Human Biochemical Genetics, Medical Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland 20892-1851, USA. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Cystinosis / complications*, diagnosis, physiopathology Humans Hyperplasia / etiology Hypertension, Portal / etiology*, pathology, physiopathology Liver / pathology* Male |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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