A 54-year-old man was admitted to our hospital for evaluation of
hypoglycemia. He had frequent episodes of loss of concentration before dinner. The ratio of IRI to plasma
glucose (PG) was 0.8-1.0. Abdominal CT revealed no pancreatic
tumor, and angiography of splenic artery showed no definite
tumor stain within the pancreas. Based on the results of selective arterial
calcium stimulation and hepatic venous sampling (ASVS), the provisional diagnosis was a small
insulinoma in the pancreatic body. The patient underwent subtotal distal
pancreatectomy. However, histopathological and immunohistochemical examinations of the resected tissue showed
hypertrophy of islets of Langerhans islands and beta cells around pancreatic ducts. The final diagnosis was adult-onset
nesidioblastosis. Postoperatively, the patient continued to exhibit
hyperinsulinemia and nighttime
hypoglycemia.
Octreotide,
voglibose and
diet therapies failed to improve the nocturnal
hypoglycemia. However, treatment with
diazoxide at a starting dose of 200 mg/day resulted in immediate amelioration of nocturnal
hypoglycemia. This is the first Japanese adult case of
nesidioblastosis treated successfully with
diazoxide. This case report suggests that
diazoxide may be effective for adult-onset
nesidioblastosis in a manner similar to that described for pediatric cases.