中山大学学报（医学科学版）2025，Vol.46Issue(1)：132-137,6.
儿童肝糖原贮积病血糖及生化指标的横断面调查
A Cross-sectional Study of Blood Glucose and Biochemical Indicators in Pediatric Patients with Hepatic Glycogen Storage Disease
摘要
Abstract
[Objective]Patients with hepatic glycogen storage disease(GSD)have recurrent episodes of hypoglycemia.This study aimed to investigate and analyze blood glucose and biochemical indicators in pediatric patients with hepatic GSD,thus provide data support for hypoglycemia prevention and its clinical management.[Methods]A cross-sectional field study was conducted among patients with hepatic GSD treated in the Department of Pediatrics of Guangdong Provincial People's Hospital on July 14,2024.We collected the peripheral blood samples of the patients and their healthy family controls on site,then analyzed and compared their blood glucose and biochemical indicators.[Results]Of the 44 patients with hepatic GSD,there were 34 males and 10 females,including GSD Ib(n=14),GSD Ia(n=15),GSD Ⅲ(n=2),GSD Ⅵ(n=7)and GSD Ⅸ(n=6).The average age was 7.60(5.08-11.98)years.All patients were on uncooked cornstarch(UCCS)therapy.Of the patients,77.3%(34/44)had hepatomegaly,61.4%(27/44)had recurrent hypoglycemia,61.4%(27/44)had blood glucose≤3.9 mmol/L,18.2%(8/44)had blood glucose≤2.8 mmol/L,and none of the 8 cases was GSD Ib.The lowest blood glucose level was 1.19 mmol/L and no episodes of hypoglycemia occurred.Of the family control subjects,65.9%(29/44)had blood glucose≤3.9 mmol/L.There was no significant difference in hypoglycemia prevalence between hepatic GSD group and control group(P=0.658).The hepatic GSD patients had hyperlactacemia,hyperuricemia and hypercholesterolemia prevalence rates of 65.9%,45.5%and 9.1%,respectively,as compared with 18.2%,43.2%and 15.9%,respectively,for the family control subjects.No significant difference was found in the prevalence rates of hyperuricemia and hypercholesterolemia between the two groups(P=0.830 and P=0.334,respectively).[Conclusions]Asymptomatic hypoglycemia is common in patients with hepatic GSD,especially in non-GSD-Ib patients.It is necessary to optimize the diet management of UCCS,conduct dynamic blood glucose monitoring and follow a light diet,so as to decrease hyperuricemia and hypercholesterolemia,avoid and reduce the serious adverse reactions and complications caused by severe hypoglycemia.关键词
肝糖原贮积病/低血糖/血生化指标/横断面调查/儿童/家庭对照Key words
hepatic glycogen storage disease/hypoglycemia/blood biochemical indicator/cross-sectional study/children/family control分类
医药卫生引用本文复制引用
马妮,吴昊天,王颖,杨静,梁丹霞,杨敏..儿童肝糖原贮积病血糖及生化指标的横断面调查[J].中山大学学报（医学科学版）,2025,46(1):132-137,6.基金项目
国家自然科学基金(82470545,82070537) （82470545,82070537）
