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Long‐term results of living donor liver transplantation for glycogen storage disorders in children
Author(s) -
Iyer Shridhar G.,
Chen ChaoLong,
Wang ChihChi,
Wang ShihHo,
Concejero Allan M.,
Liu YuehWei,
Yang ChinHsiang,
Yong CheeChien,
Jawan Bruno,
Cheng YuFan,
Eng HockLiu
Publication year - 2007
Publication title -
liver transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.814
H-Index - 150
eISSN - 1527-6473
pISSN - 1527-6465
DOI - 10.1002/lt.21151
Subject(s) - medicine , surgery , liver transplantation , transplantation , metabolic acidosis , gastroenterology
Liver transplantation (LT) may be indicated in glycogen storage disorders (GSD) when medical treatment fails to control the metabolic problems or when hepatic adenomas develop. We present our institutional experience with living donor LT (LDLT) for children with GSD. A total of 244 patients underwent primary LDLT at our institution from June 1994 to December 2005. A total of 12 (5%) children (8 female and 4 male) were afflicted with GSD and were not responsive to medical treatment. Nine patients had GSD type I and 3 had GSD type III. The median age at the time of transplantation was 7.27 yr (range, 2.4‐15.7). All patients presented with metabolic abnormalities, including hypoglycemia, and lactic acidosis. In addition, 4 patients presented with growth retardation. A total of 11 patients received left lobe grafts and 1 received a right lobe graft. The mean graft‐to‐recipient weight ratio was 1.25 (range, 0.89‐1.61). Two patients had hepatic vein stenoses that were treated by balloon dilatation; 1 patient had bile leak, which settled spontaneously. The overall surgical morbidity rate was 25%. Three patients had hepatic adenomas in the explanted liver. There was a single mortality at 2 months posttransplantation due to acute pancreatitis and sepsis. The mean follow up was 47.45 months. The metabolic abnormalities were corrected and renal function remained normal. In patients with growth retardation, catch‐up growth was achieved posttransplantation. In conclusion, LDLT is a viable option to restore normal metabolic balance in patients with GSD when medical treatment fails. Long‐term follow‐up after LT for GSD shows excellent graft and patient survival. Liver Transpl 13:848–852, 2007. © 2007 AASLD.