Reduced-size liver transplantation for glycogen storage disease.
BACKGROUND
Glycogen storage disease (GSD) is an inherited metabolic disorder in which the concentration and/or structure of glycogen in tissues is abnormal. Essentially, abnormalities in all known enzymes involved in the synthesis or degradation of glycogen and glucose have been found to cause some type of GSD. Liver and muscle have abundant quantities of glycogen and are the most common and seriously affected tissues. This study was to assess reduced-size liver transplantation for the treatment of GSD.


METHODS
The clinical data from one case of GSD type I with hepatic adenoma was retrospectively analyzed. The clinical manifestations were hepatomegaly, delayed puberty, growth retardation, sexual immaturity, hypoglycemia, and lactic acidosis, which made the young female patient eligible for reduced-size liver transplantation.


RESULTS
The patient recovered uneventfully with satisfactory outcome, including 12 cm growth in height and 5 kg increase in weight during 16 months after successful reduced-size liver transplantation. She has been living a normal life for 4 years so far.


CONCLUSIONS
Reduced-size liver transplantation is an effective treatment for GSD with hepatomegaly and hepatic adenoma. Delayed puberty, growth retardation, hypoglycemia and lactic acidosis can be cured by surgery.
Ji
Hao-Feng
HF
Department of Hepatobiliary Pancreatic Surgery, First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China.
Wang
Wei-Lin
WL
Shen
Yan
Y
Zhang
Min
M
Liang
Ting-Bo
TB
Wu
Jian
J
Xu
Xiao
X
Yan
Sheng
S
Zheng
Shu-Sen
SS
D018248:Adenoma, Liver Cell / Q000150:complications / Q000601:surgery*; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000150:complications / Q000601:surgery*; D006529:Hepatomegaly / Q000209:etiology / Q000601:surgery; D006801:Humans; D008113:Liver Neoplasms / Q000150:complications / Q000601:surgery*; D016031:Liver Transplantation / Q000379:methods*; D009929:Organ Size; D055815:Young Adult