Diagnosis and treatment of congenital hyperinsulinism--to Paris at any price?
Søvik, O.; Njølstad, P.R.; Reigstad, H.; Brackman, D.; Teslo, I.; Brunvand, L.
Tidsskrift for den Norske Laegeforening Tidsskrift for Praktisk Medicin Ny Raekke 121(5): 612-614
2001
ISSN/ISBN: 0029-2001 PMID: 11301621 Document Number: 534900
Persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI) is a hyperfunctional disorder of pancreatic insulin-producing cells with hypertrophic beta-cells present either focally or diffusely. With an estimated frequency of 1:50,000 live births, Norway will on average have one case per year. It is clearly difficult to maintain expertise in diagnostics and treatment with such a low incidence. We report three Norwegian patients with PHHI who were successfully treated at Hôpital des Enfants Malades in Paris. Two patients were shown to have focal hyperinsulinism treated with partial pancreas resection. After follow-up of three and a half and two years respectively, these patients have normal glucose tolerance and exocrine pancreatic function. One patient with diffuse hyperinsulinism was operated with subtotal (90%) pancreatectomy. At 2.5-years follow-up this patient has slight glucose intolerance whereas her fasting blood glucose is low normal. The exocrine pancreatic function is normal. Patients with PHHI should be referred to a centre where the possibility of focal hyperinsulinism can be thoroughly explored.