Diagnosis And Treatment Experience Of Reduce-size Liver Transplantation For Glycogen Storage Disease And Hepatic Adenoma And A Case Report.

Background:Glycogen storage disease (GSD), inherited disorders with the abnormal concentration and/or structure of glycogen in body tissue, can be associated with severe liver disease. All the known enzymes involved in the synthesis or degradation of glycogen and glucose may cause some type of GSD. The GSDs affecting the major organ are type I (glucose 6-phosphatase), type III (debrancher), type IV (brancher), type VI (liverphosphorylase) and type IX (phosphorylase b kinase), as well as glycogen synthase deficiency. GSD type I, or von Gierke's disease, is one of the most common types of liver glycogenosis. The clinical manifestations are growth retardation, hepatomegaly, hypoglycemia, lactic acidemia, hyperuricemia, and hyperlipidemia. Long-term complications include gout, short stature, osteoporosis, renal disease, pulmonary hypertension, and hepatic adenomas. Among these complications, hepatic adenomas, which are in up to 50% of patients, develop during the second decade of life, especially when glucose metabolism is poorly balanced. Although most of hepatic adenomas remain benign, some cases of hepatoadenocarcinomas have been reported. Multiple adenomas with the risk of malignant transformation, severe metabolic imbalance, and poor growth retardation make the patient as a candidate for the liver transplantation. The aim of this study was to assess reduce-size liver transplantation (RLT) for the treatment of GSD.Objective:To summarize the experience of diagnosis and treatment of GSD and hepatic adenoma with RLT and assess its value in RLT treatment forGSD with hepatic adenoma.Methods:A 21-year-old girl was referred to reduce-size liver transplantation for GSD type I with hepatic adenoma. GSD had been diagnosed when she was only 5 months old, notice of hepatomegaly. Later, she had showed significant pubertal, growth retardation, and sexual immaturity. At the age of 21 years, she was hospitalized at our center. The patient presented a growth profile and osseous age corresponding to that of a 10-year-old child (126 cm