Current role of liver transplantation for methylmalonic acidemia: A review of the literature

• Published in: Pediatric transplantation Vol. 10; no. 8; pp. 943 - 947
• Main Authors: Kasahara, Mureo, Horikawa, Reiko, Tagawa, Manabu, Uemoto, Shinji, Yokoyama, Satoshi, Shibata, Yumiko, Kawano, Takafumi, Kuroda, Tatsuo, Honna, Toshiro, Tanaka, Koichi, Saeki, Morihiro
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01.12.2006; Blackwell
• Subjects: Adolescent; Adult; Aspergillus; Biological and medical sciences; Child; Child, Preschool; Female; Humans; Infant; Liver Transplantation; living donor; Living Donors; Male; Medical sciences; metabolic; Metabolism, Inborn Errors - enzymology; Metabolism, Inborn Errors - surgery; Methylmalonic Acid - blood; Methylmalonyl-CoA Mutase; organic acidemia; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Treatment Outcome; Human; Pediatrics; Digestive system; Living donor; Liver; Transplantation; Homotransplantation; Metabolism; Acidemia; Treatment; Methylmalonic acid; Surgery; organic acidemia; Graft; Pharmacokinetics; Child; Bibliographic review; liver transplantation; metabolic
• ISSN: 1397-3142; 1399-3046
• DOI: 10.1111/j.1399-3046.2006.00585.x

:  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.