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101 GROWTH IN A GIRL WITH NESIDIOBLASTOSIS DURING AND AFTER TEATMENT WITH THE SOMATOSTATIN ANALOGUE: SMS 201–995

1988; Springer Nature; Volume: 24; Issue: 4 Linguagem: Inglês

10.1203/00006450-198810000-00122

1530-0447

Roelof J. Odink, Henriette A Delemarre-van de Wall,

Neuroblastoma Research and Treatments

Severe hyperinsulinism can be treated with somatostatin analogues. We studied growth and growth hormone(gh) in a patient with nesidioblastosis, treated with SMS 201-995. In a maroccan female with nesidioblastosis subtotal pancreatectomy was performed at an age of 4 weeks. Because of recurrent hypoglycaemia SMS 201-995 was instituted from an age of 11 months till 3.1 yrs.In a dose of 8 × 25 μg/day s.c. SMS 201-995 resulted in normoglycaemia(portable infusion pump, Ferring). The SMS treatment could be ceased at an age of 3.1 yrs. Growth velocity during the last 6 months of treatment was 5.2 cm/yr. In the year after discontinuation of therapy growth vel. increased to 12.2 cm yr.(CA:4.0 yr). During treatment SMC level was normal to slightly elevated(162 ng/mL); maximum gh level during sleep decreased with the treatment period and was 8.2 μg/L. just before discontinuation, 14 days off treatment max.gh had increased to 13 μg/L. Conclusion: Chronic subcutaneous therapy(2.2 yrs) of SMS 201-995 was succesfull in preventing hypoglycaemic periods in a girl with nesidioblastosis. Growth hormone secretion was gradually impaired during treatment. Catch up growth after discontinuation of therapy makes evident that growth was impaired during treatment with the somatostatin analogue SMS 201-995.