Glycogen storage disease type I(GSDI) is a glucose metabolic disorder caused by glucose-6-phosphatase deficiency. GSDIa patients are characterized by hypoglycemia, hepatomegaly, hyperlipidemia and lactic acidemia. This study aims to review the lipid status in patients with GSDIa, explores the lipid treatment targets and preferable lipid-lowering drugs.
The clinical data of GSDIa patients characteristics were collected. Diet control, raw cornstarch were used to maintain normal blood glucose and lipid level. Some patients were given lipid-lowering drugs. We compared the lipid levels before and after each treatment.
A total of 163 GSDIa patients were enrolled in this study and 773 times of lipid levels were detected. TG was normal in only 13 times of detection from 10 patients(2%). There were 26 patients who took atorvastatin or fibrates (fenofibrate or gemfibrozil) regularly for more than one year. The lipid levels could be improved after lipid-lowering drugs treatment. The therapeutic effect of atorvastatin was better than fibrates. The total cholesterol(TC) was positively correlated with total triglycerides(TG) after treatment. A regression equation was established for TC and TG, TG=-2.86 + 1.63TC. The complication of hyperlipidemia was mainly caused by TC. As long as TC maintained normal, TG did not have to be maintained normal and a certain degree of hypertriglyceridemia can be accepted. The lower bound of China Children's TC abnormal level (5.18 mmol/L) was substituted into the established regression equation to obtain TG = 5.58 mmol/L. We set a target that when the TG level was lower than 5.58 mmol / L after treatment, TC could maintain normally. There was no need for further increasing the dosage of lipid-lowering drug.
Patients with GSDIa had significant abnormalities in blood lipid metabolism. We found that there was a linear correlation between TC and TG, which was TG=-2.86 + 1.63*TC. We made a point that as long as the TC remained normal, a certain degree of hypertriglyceridemia could be accepted. We set a target that when the TG level was lower than 5.58 mmol / L after treatment, TC could maintain normally. This study found that the therapeutic effect of atorvastatin was better than fibrates.