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Eur J Pediatr. 2002 Jul;161(7):377-9. Epub 2002 Jun 5.

Progressive neurologic disability in methylmalonic acidemia despite transplantation of the liver.

Author information

  • 1UCSD Pediatrics (0830), 9500 Gilman Drive, La Jolla, CA 92093-0830, USA. wnyhan@ucsd.edu

Abstract

Methylmalonic acidemia unresponsive to cobalamin is often fatal in infancy. Patients have been considered candidates for hepatic transplantation and experience has been that the procedure eliminates the life-threatening episodes of ketoacidosis that characterize this disease.

CONCLUSION:

experience with a 24-year-old patient treated with hepatic transplantation indicates that this procedure does not prevent progressive renal failure and neurologic dysfunction.

PMID:
12111189
[PubMed - indexed for MEDLINE]
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