title: Two cases of persistent hyperinsulinemic hypoglycemia that showed spontaneous regression and maturation of the Langerhans islets.
abstract: Near-total pancreatectomy has been recommended as the treatment for persistent hyperinsulinemic hypoglycemia (PHH) in infants. However, recently there has been a report described that one third of 95% pancreatectomy failed to prevent hypoglycemia and more than two thirds had diabetes ultimately. The authors experienced 2 cases of PHH, which raise a query about the extensive pancreatectomy. Case 1: A female patient who manifested PHH shortly after birth underwent less extensive pancreatectomy twice at age 2 months and 8 years. After each operation, her clinical symptoms regressed, and she became free from the disease eventually. Histologic findings showed nesidioblastosis in which the islets clearly matured. Case 2: A male infant with PHH had an absolute indication for pancreatectomy. However, after a meticulous control of the blood glucose level with parenteral nutrition followed by continuous enteral nutrition feeding combined with medication, he became free from the disease. The current cases show there exist cases of PHH in which the islets mature and symptoms regress spontaneously. Therefore, we conclude near-total pancreatectomy is not always the treatment of choice. As an alternate strategy, long-term controlled feeding and medication combined with or without less extensive pancreatectomy should be considered with the expectation of spontaneous regression.
authors.affiliation: Department of Surgery II, Kinki University, Osaka, Japan.
authors.forename: A
authors.initials: A
authors.lastname: Kubota
authors.forename: T
authors.initials: T
authors.lastname: Yonekura
authors.forename: N
authors.initials: N
authors.lastname: Usui
authors.forename: T
authors.initials: T
authors.lastname: Kosumi
authors.forename: K
authors.initials: K
authors.lastname: Yamauchi
authors.forename: M
authors.initials: M
authors.lastname: Yamasaki
authors.forename: H
authors.initials: H
authors.lastname: Oyanagi
authors.forename: M
authors.initials: M
authors.lastname: Nakayama
authors.forename: S
authors.initials: S
authors.lastname: Ida
authors.forename: T
authors.initials: T
authors.lastname: Nakajima
mesh_terms: D007516:Adenoma, Islet Cell / Q000150:complications / Q000473:pathology / Q000601:surgery*; D002908:Chronic Disease; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006946:Hyperinsulinism / Q000175:diagnosis* / Q000209:etiology; D007003:Hypoglycemia / Q000175:diagnosis* / Q000209:etiology; D007231:Infant, Newborn; D007801:Langerhans Cells / Q000502:physiology*; D008297:Male; D010180:Pancreatectomy / Q000379:methods*; D010190:Pancreatic Neoplasms / Q000150:complications / Q000473:pathology / Q000601:surgery*; D012075:Remission, Spontaneous; D018570:Risk Assessment