Current role of liver transplantation for methylmalonic acidemia: a review of the literature.
Pediatr Transplant
; 10(8): 943-7, 2006 Dec.
Article
in En
| MEDLINE
| ID: mdl-17096763
ABSTRACT
To evaluate the current role of liver transplantation (LT) for methylmalonic acidemia (MMA), we reviewed the literature on outcomes of this treatment, and describe three of our own cases of living-donor liver transplantation (LDLT). The total number of LT cases identified was 18. Transplantation mode was deceased donor LT in 12, including five combined liver-kidney transplantations (CLKT) from deceased donors, and LDLT in six. Three hospital mortalities were noted, because of metabolic decompensation, sepsis and aspergillosis. Although mean postoperative serum MMA level decreased to 13.8% +/- 9.2% (range 1.25-26.1%) of preoperative levels, four patients (22.2%) had renal insufficiency after isolated LT and three (16.7%) had postoperative neurological disability. Continuing metabolic damage to the kidney and brain may occur even after successful LT. Further evaluation is required to determine the long-term suitability of this treatment modality.
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Collection:
01-internacional
Database:
MEDLINE
Main subject:
Liver Transplantation
/
Metabolism, Inborn Errors
/
Methylmalonic Acid
Limits:
Adolescent
/
Adult
/
Child
/
Child, preschool
/
Female
/
Humans
/
Infant
/
Male
Language:
En
Journal:
Pediatr Transplant
Journal subject:
PEDIATRIA
/
TRANSPLANTE
Year:
2006
Document type:
Article
Affiliation country:
Japan