Persistent hyperinsulinaemic hypoglycaemia of infancy: long-term treatment with the somatostatin analogue Sandostatin

Six infants with severe, persistent hyperinsulinaemic hypoglycaemia were treated with the long-acting somatostatin analogue SMS 201-995 (Sandostatin, Sandoz, Basle, Switzerland). Effective control of hypoglycaemia without the need for parenteral glucose was achieved in five of the six cases with doses ranging from 10 to 40 micrograms/kg day given either by four s.c. injections per day, or by continuous subcutaneous infusion (CSI). One has been well controlled on SMS 10 micrograms/kg day for 17 months as an out-patient without requiring surgery, while the five others underwent sub-total pancreatectomy after receiving short courses of the drug. In two patients where hypoglycaemia persisted after sub-total pancreatectomy SMS was effective in inhibiting insulin secretion and preventing hypoglycaemia. Plasma somatomedin concentrations and linear growth were not suppressed in any patient. It is concluded that Sandostatin is useful in the pre and post-operative management of most infants with this syndrome. In selected cases this analogue of somatostatin may also be a long-term treatment option in place of pancreatectomy.