Growth and endocrine changes in the hepatic glycogenoses

The biochemical and endocrine responses of 13 patients with hepatic glycogen storage disease (HGSD) (type I-six patients, type Ib-two, type III-three, type IX-two patients) to an oral glucose load have been investigated. Longitudinal growth data was available in all patients. The height velocity standard deviation score (HVSDS) was positively correlated with the plasma somatomedin and inversely correlated with the glucose-insulin ratio, plasma cortisol and plasma growth hormone concentrations. There was no correlation between plasma glucagon and HVSDS with treatment was accompanied by a rise in plasma somatomedin and a fall in growth hormone and cortisol. In two patients the glucose-insulin ratio decreased. Growth retardation in HGSD can be explained as part of the adaptation to the inability to maintain normal glucose homeostasis.