Gastric Drip Feeding in Patients with Glycogen Storage Disease Type I: Its Effects on Growth and Plasma Lipids and Apolipoproteins

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Abstract
In 19 patients with a deficiency of glucose-6-phosphatase and 1 patient with a deficiency of glucose-6-phosphate translocase, the effect of nocturnal gastric drip feeding (GDF) on growth and plasma lipids and apolipoproteins was studied. The effect on growth was estimated by determining the height standard deviation score (SDS) of the patients and comparing its changes (ΔSDS) over 4-, 2-, and 1-y periods before and 1-, 2-, 5-, and 8-y periods after the institution of GDF. The effect of GDF on plasma lipids and apolipoproteins was investigated by following the concentrations of triglycerides, cholesterol, and apolipoproteins A-I, A-II, B, C-I, C-II, C-III, and E. Growth caught up significantly or remained in the normal range in 14 patients. They were defined as responders to GDF. In the other six patients, growth caught up insufficiently or showed a further deceleration. They were defined as nonresponders to GDF. GDF had only a temporary and marginal effect on plasma lipids and apolipoproteins, but after 5–8 y, the levels of plasma triglycerides, cholesterol, apolipoprotein, B, C-I, C-II, C-III, and E increased further in both responders and nonreponders, whereas apolipoproteins A-I and A-II decreased in nonresponders. There were minor differences in the levels of lipids and apolipoproteins between responders and nonresponders without any discernible trends during the first years of GDF. After 5–8 y, the levels of cholesterol, triglycerides and apolipoproteins B, C-I, C-II, C-III, and E seemed to be higher, and apolipoproteins A-I and A-II lower, in nonresponders than in responders, but the differences were not significant.