Long-term results of living donor liver transplantation for glycogen storage disorders in children.
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.
Iyer
Shridhar G
SG
Liver Transplantation Program, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Kaohsiung, Taiwan.
Chen
Chao-Long
CL
Wang
Chih-Chi
CC
Wang
Shih-Ho
SH
Concejero
Allan M
AM
Liu
Yueh-Wei
YW
Yang
Chin-Hsiang
CH
Yong
Chee-Chien
CC
Jawan
Bruno
B
Cheng
Yu-Fan
YF
Eng
Hock-Liu
HL
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D005953:Glycogen Storage Disease Type I / Q000503:physiopathology / Q000601:surgery*; D006010:Glycogen Storage Disease Type III / Q000503:physiopathology / Q000601:surgery*; D006130:Growth Disorders / Q000209:etiology / Q000628:therapy; D006801:Humans; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male; D011183:Postoperative Complications; D016896:Treatment Outcome