Document Detail

Liver transplantation in a case of hypoproteinemia and coagulopathy.
MedLine Citation:
PMID:  9583213     Owner:  NLM     Status:  MEDLINE    
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'.
M Kawai; T Yorifuji; C Yamanaka; A Miyazaki; H Hattori; S Uemoto; Y Inomata; K Tanaka; K Furusho
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Acta paediatrica Japonica; Overseas edition     Volume:  40     ISSN:  0374-5600     ISO Abbreviation:  Acta Paediatr Jpn     Publication Date:  1998 Feb 
Date Detail:
Created Date:  1998-05-28     Completed Date:  1998-05-28     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0370357     Medline TA:  Acta Paediatr Jpn     Country:  AUSTRALIA    
Other Details:
Languages:  eng     Pagination:  96-8     Citation Subset:  IM    
Department of Pediatrics, Faculty of Medicine, Kyoto University, Japan.
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MeSH Terms
Disseminated Intravascular Coagulation / complications,  congenital*,  surgery*
Hypoproteinemia / complications,  congenital*,  surgery*
Infant, Newborn
Liver Transplantation / methods*
Living Donors*
Tyrosine / blood
Reg. No./Substance:

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