| Liver transplantation in a case of hypoproteinemia and coagulopathy. | |
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MedLine Citation:
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PMID: 9583213 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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A female infant with hypoproteinemia and coagulopathy associated with hypertyrosinemia was successfully treated with living-related liver transplantation (LRLT). On the 12th day of life plasma amino acid analysis revealed a marked elevation of tyrosine, so the patient was fed on a low-tyrosine and low-phenylalanine diet. However, hepatosplenomegaly, hypotonia, alopecia, eczema and psychomotor delay did not improve and recurrent episodes of disseminated intravascular coagulation (DIC) caused her condition to deteriorate. Liver biopsy on the 230th day revealed marked fatty change accompanied by mild to moderate cholestasis. Therefore, LRLT from her father was performed on the 286th day resulting in improvement of all the aforementioned signs and symptoms. Despite a thorough examination, no diagnosis of a known disorder could be established. However, her elder brother had also been born with severe hypoproteinemia and coagulopathy, and died of DIC on the second day of life. Thus, the disorder is designated as a new entity, namely 'congenital hypoproteinemia and coagulopathy associated with hypertyrosinemia'. |
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Authors:
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M Kawai; T Yorifuji; C Yamanaka; A Miyazaki; H Hattori; S Uemoto; Y Inomata; K Tanaka; K Furusho |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Acta paediatrica Japonica; Overseas edition Volume: 40 ISSN: 0374-5600 ISO Abbreviation: Acta Paediatr Jpn Publication Date: 1998 Feb |
Date Detail:
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Created Date: 1998-05-28 Completed Date: 1998-05-28 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 0370357 Medline TA: Acta Paediatr Jpn Country: AUSTRALIA |
Other Details:
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Languages: eng Pagination: 96-8 Citation Subset: IM |
Affiliation:
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Department of Pediatrics, Faculty of Medicine, Kyoto University, Japan. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Disseminated Intravascular Coagulation
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complications,
congenital*,
surgery* Female Humans Hypoproteinemia / complications, congenital*, surgery* Infant, Newborn Liver Transplantation / methods* Living Donors* Tyrosine / blood |
| Chemical | |
Reg. No./Substance:
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55520-40-6/Tyrosine |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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