Disorders of the endocrine pancreas.
Hyperinsulinemic hypoglycemia in the neonate or in early infancy may be caused by an islet cell adenoma or a diffuse organic lesion of the endocrine pancreas such as nesidioblastosis. The 2 disorders cannot be distinguished by biochemical means but only by immunohistochemical analysis after subtotal pancreatectomy. An aggressive therapeutic approach is mandatory to avoid permanent neurological damage. In nesidioblastosis, 20% of patients are healed by subtotal pancreatectomy and the remainder may be controlled by postoperative treatment with diazoxide and hydrochlorothiazide. Some patients may need total pancreatectomy which leads to permanent insulin-dependent diabetes in most of them. When hyperinsulinemic hypoglycemia first manifests after 1 year of age, it is always caused by an islet cell adenoma, which is cured by enucleation.
D007516:Adenoma, Islet Cell / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D006801:Humans; D007003:Hypoglycemia / Q000209:etiology; D007223:Infant; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D007328:Insulin / Q000097:blood; D007340:Insulinoma / Q000175:diagnosis* / Q000601:surgery; D008297:Male; D010180:Pancreatectomy; D010182:Pancreatic Diseases / Q000150:complications / Q000473:pathology / Q000628:therapy; D010190:Pancreatic Neoplasms / Q000175:diagnosis* / Q000601:surgery; D011379:Prognosis
D007328:Insulin