Lipid Status and Linear Relationship Between Total Cholesterol and Triglycerides in Glycogen Storage Disease Type Ia

Abstract

Background Glycogen storage disease type Ia(GSDIa) is a glucose metabolic disorder disease. GSDIa patients are characterized by hypoglycemia, hepatomegaly, hyperlipidemia, and hyperlactacidemia. This study aims to review the lipid status, explore lipid treatment targets, and assess preferable lipid-lowering drugs. Methods Clinical data on GSDIa patients’ characteristics were collected. Diet control and raw cornstarch treatment 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. Results A total of 163 GSDIa patients were enrolled in this study. The lipid level was assessed 773 times. After treatment with raw cornstarch, TG level has significantly decreased by 30 ± 50%(P < 0.001). There was no change in TC level. Thirty patients regularly took atorvastatin or fibrates for more than one year. The therapeutic effect of atorvastatin was better than fibrates. The total cholesterol(TC) was positively correlated with TG after treatment, resulting in the following regression equation: TG = 1.63*TC-2.86. Conclusion Patients with GSDIa have significant abnormalities in blood lipid metabolism. We found that there was a linear correlation between TC and TG, which was TG = 1.63*TC-2.86. Because the complications of hyperlipidemia are caused mainly by TC, thereby, as long as the TC remains normal, we could set a TG target to allow a certain degree of hypertriglyceridemia. The bound of Chinese Children's TC normal level (5.18 mmol/L) was used in the established regression equation to obtain TG = 5.58 mmol/L. We, therefore, targeted to keep TG level below 5.58 mmol/L. This study found that the therapeutic effect of atorvastatin was better than fibrates.

Relationships Between Age, Breed, Body Condition Score, Lipogram and Antioxidant Parameters in Female Dromedary Camels with Reproductive Abnormalities

“…It is well-established that an increase in TG storage is associated with a linear rise in cholesterol production, which, in turn, is related to an increase in cholesterol in bile and an elevated risk of illness (Zhang et al, 2020). However, the exact biochemical mechanisms underlying the association between overweight and the aforementioned lipid profiles have not been fully elucidated.…”