Document Detail


The effects of early treatment of hereditary tyrosinemia type I in infancy by orthotopic liver transplantation.
MedLine Citation:
PMID:  2336709     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Two infants with hereditary tyrosinemia secondary to fumarylacetoacetate hydrolase (FAH) deficiency underwent orthotopic liver transplantation at 14 and 16 weeks of age due to poor clinical and biochemical response to medical therapy. Prompt clearance of abnormal metabolites with improved mental alertness and appetite occurred with minimal perioperative complications. Both infants tolerated rapid institution of normal diets and have shown progressive growth and development in the first 36 months after transplantation. Early liver transplantation should be considered as an option for infants with certain inherited metabolic disorders with poor prognosis, such as tyrosinemia type I, who fail to respond to medical therapy.
Authors:
M W Flye; C A Riely; B E Hainline; S Sassa; R J Gusberg; K J Blakemore; K W Barwick; A L Horwich
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Transplantation     Volume:  49     ISSN:  0041-1337     ISO Abbreviation:  Transplantation     Publication Date:  1990 May 
Date Detail:
Created Date:  1990-06-13     Completed Date:  1990-06-13     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0132144     Medline TA:  Transplantation     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  916-21     Citation Subset:  IM    
Affiliation:
Department of Surgery, Washington University School of Medicine, St. Louis, Missouri 63110.
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MeSH Terms
Descriptor/Qualifier:
Amino Acid Metabolism, Inborn Errors / surgery*
Humans
Hydrolases / deficiency*
Infant
Liver Transplantation / methods*
Porphobilinogen Synthase / antagonists & inhibitors
Time Factors
Tyrosine / blood*
Chemical
Reg. No./Substance:
55520-40-6/Tyrosine; EC 3.-/Hydrolases; EC 3.7.1.2/fumarylacetoacetase; EC 4.2.1.24/Porphobilinogen Synthase

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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