ウエムラ　シユウイチロウ   UEMURA Shiyuuichirou   植村　修一郎    所属   医学部 医学科（東京女子医科大学病院）    職種   助教
論文種別	症例報告
言語種別	英語
査読の有無	査読なし
表題	Clinically Adult-Onset Nesidioblastosis with Repeated Severe Hypoglycemia, Successfully Treated by Two Times Pancreatectomies. A Rare Case Report
掲載誌名	正式名：Clinical and experimental gastroenterology 略　 称：Clin Exp Gastroenterol ISSNコード：11787023/11787023
掲載区分	国外
巻・号・頁	18,pp.163-170
著者・共著者	IZUMO Wataru†, HIGUCHI Ryota, UEMURA Shuichiro, YAZAWA Takehisa, TAKANO Noriyoshi, ICHIHARA Atsuhiro, FURUKAWA Toru, YAMAMOTO Masakazu, HONDA Goro
発行年月	2025/07
概要	Although nesidioblastosis is the most common cause of hyperinsulinemic hypoglycemia in infants, it is rare in adults. Nesidioblastosis is pathologically characterized by diffuse neoformation of the islets of Langerhans islets from the pancreatic ductal epithelium and is a disease that does not exhibit neoplastic proliferation, unlike insulinoma. Hence, we present a rare case of adult-onset nesidioblastosis that caused repeated severe hypoglycemic symptoms and was cured by pancreatic resection twice, resulting in total pancreatectomy. A 37-year-old woman with the Whipple's triad visited our institution. In the fasting test, the plasma glucose level decreased and immunoreactive insulin levels increased after 12 h. No tumor was identified in the pancreas by imaging. A selective arterial calcium injection test revealed that step-up was detected only in the gastroduodenal artery. The patient underwent pancreatoduodenectomy with a diagnosis of adult-onset nesidioblastosis, with the pancreatic head region as the culprit. Pathological examination revealed neither tumorous islet cells nor an obvious increase in the number of islets. However, there were some isolated single insulin-producing cells in the pancreatic parenchyma, which could cause hyperinsulinemia and hypoglycemia. This patient was diagnosed with adult-onset nesidioblastosis. After the operation, the hypoglycemic symptoms improved, but 1 year later, the same symptoms recurred. The patient underwent remnant pancreatectomy and had no hypoglycemic symptoms for > 5 years after the second surgery.
DOI	10.2147/CEG.S520986
PMID	40656591