STUDIES ON GROWTH HORMONE SECRETION IN A PATIENT WITH THE DIENCEPHALIC SYNDROME OF EMACIATION

A girl with a large optic glioma, and the typical features of diencephalic syndrome of emaciation (Russell) was followed up from ¹/₂ to 2 ¹/₂ years of age. She had very high levels of growth hormone (GH) in the plasma, which were not influenced by hyperglycaemia, by hypoglycaemia or by dexamethasone, reserpine or chlorpromazine. Interference by the glioma with GH releasing factor or more likely with GH inhibiting factor is suggested. In spite of the elevated plasma GH, linear growth was markedly retarded. Sulfation factor activity (Somatomedin) was low with no significant response to injections of HGH. The patho-genesis of the profound metabolic disturbances in this syndrome is not properly understood. The endogenous GH obviously exerts its full adipokinetic effect, whereas the synthesis of the sulfation factor (Somatomedin) must be defective. This case illustrates a type of retardation of linear growth, not seen in any other known syndrome.