Abstract
A Maroccan newborn female was admitted to the hospital directly after birth with asphyxia and convulsions. Laboratory examination revealed hypoglycaemia with elevated insulin levels. Glucose infusions in combination with diazoxide treatment could not maintain normoglycaemia. At 4 weeks of age subtotal pancreatectomy was performed. Pathological examination of the specimen showed nesidioblastosis. At 8.5 months, after a period of normoglycaemia, there was a reoccurrence of hypoglycaemia. Frequent feedings, diazoxide or glucagon treatment failed. At age 11 months SMS 201-995 was started. The dose was gradually increased from 20 once to 25 μg 8 times per day (sc administration by a portable infusion pump, Zyklomat, Ferring, Kiel). Glucose levels were in the normal range, insulin levels declined. The patient was treated with SMS 201-995 for 17 months. After 9 months of treatment the dose could be decreased to 25 μg 6 times per day sc. Height and weight gain are normal. During treatment normal growth hormone levels are found during sleep. Psychomotor development is appropriate for age. Short term interruptions of treatment do not elicit a rebound phenomenon with hypoglycaemia, in contrast to SMS 201-995 withdrawal for several hours. In conclusion: the long-acting somato-statin analogue (SMS 201-995) may be an alternative therapeutic appraoch in the long term management of hyperinsulinism.
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Delemarre-van de Waal, H., Veldkamp, E. LONG TERM TREATMENT WITH A SOMATOSTATIN-ANALOGUE(SMS 201-995) IN A PATIENT WITH PERSISTENT HYPOGLYCAEMIA DUE TO NESIDIOBLASTOSIS. Pediatr Res 20, 1184 (1986). https://doi.org/10.1203/00006450-198611000-00065
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DOI: https://doi.org/10.1203/00006450-198611000-00065