[Nesidiodysplasia. A rare cause of hyperinsulinemia in an adult patient].
Adult hyperinsulinaemia is usually caused by benign insulinomas. These tumours are often small, and are associated with excellent outcome of surgical removal. In cases of negative outcome of surgical exploration, or of persistent hyperinsulinaemic hypoglycaemia, the possibility must be considered of some rare condition such as the presence of metastatic insulinoma, B-cell hyperplasia, multiple micro- or macro-adenomas, hyperinsulinaemia factitia, or nesidiodysplasia. The latter is the most common cause of hyperinsulinaemia in children but is rare in adults. It is defined as a maldistributed islet cell mass within the normal exocrine pancreas, with disturbed control of hormone synthesis/secretion resulting in hormone overproduction, usually hyperinsulinaemia. In persistent hyperinsulinaemic hypoglycaemia of infancy, inactivating mutations of the sulphonylurea receptor gene is one possible cause of nesidiodysplasia. Adult nesidiodysplasia is discussed in the article, and illustrated by the case of a patient undergoing distal pancreatectomy for hyperinsulinaemia, the diagnosis being subsequently established histologically. Following spleen- and duodenum-preserving subtotal (95%) pancreatectomy, the patient was symptom-free.
Wängberg
B
B
Sahlgrenska Universitetssjukhuset, Göteborg. bo.wangberg@medfak.gu.se
Nilsson
O
O
Grimelius
L
L
Lundell
L
L
Ahlman
H
H
D000328:Adult; D003937:Diagnosis, Differential; D006801:Humans; D006946:Hyperinsulinism / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D007150:Immunohistochemistry; D007515:Islets of Langerhans / Q000378:metabolism / Q000473:pathology*; D008297:Male; D010180:Pancreatectomy; D010190:Pancreatic Neoplasms / Q000150:complications / Q000473:pathology / Q000601:surgery
Nesidiodysplasi. Ovanlig orsak till hyperinsulinemi hos en vuxen patient.