Glucose Tolerance and Insulin Secretion in Parents and Children with Familial Dwarfism and High Plasma Immunoreactive Growth Hormone (IR-HGH) and Familial Isolated Growth Hormone Deficiency

Blood glucose and plasma insulin concentrations during an oral glucose tolerance test were measured in 13 patients with the syndrome of familial dwarfism and high plasma IR-HGH and in 12 of their parents, as well as in seven parents and five of their children with familial dwarfism due to isolated HGH deficiency. In the first group glucose intolerance was found in six patients and four parents. Subnormal insulin response was found in five patients and one parent and hyperinsulinemia was registered in three patients and four parents. One of the parents has overt diabetes mellitus requiring insulin. In the families with isolated HGH lack, both patients and parents had a normal glucose tolerance. All the patients had a normal insulin response, but in four of the parents hyperinsulinemia was found. It was concluded that in the syndrome of familial dwarfism and high plasma IR-HGH there is a remarkably high incidence of carbohydrate intolerance even in the heterozygous parents. It is possible that this disturbance is not only due to the prolonged absence of HGH activity but is an intrinsic part of this syndrome.