Abstract
Persistent hyperinsulinemic hypoglycemia (nesidioblastosis) not caused by an insulinoma is rare in adults. Morphologically no insulin secreting tumor is present. Keystones of diagnosis are not only low glucose levels but to maintain normoglycemia by use of intravenous glucose and the presence of high insulin and C-peptide levels. Noninvasive and invasive diagnostic techniques are required to rule out a hormone secreting tumor. Both conservative and/or surgical therapy are suggested to prevent damaging effects of repeated hypoglycemia. Two patients with frequent and serious episodes of hypoglycemia are reported. In the 34-year-old female symptoms appeared with sweating, dizziness, trembling, nervousness and serious neuroglycopenic signs. In the 22-year-old male the main complaint was tympany, a rare and unusual sign of hypoglycemia, and intense feeling of esurience. The 24-hour fasting test was positive in both cases, i.e. it had to be stopped because of symptomatic hypoglycemia. No insulinoma could be localized, despite extensive search, therefore in both cases the diagnosis of adult-onset nesidioblastosis was set up, despite lack of histological confirmation. Diazoxide therapy resulted in symptom-free life for both patients. S...Continue Reading
References
Oct 1, 1979·Cancer·M T HullJ T Higgins
Oct 1, 1992·Archives of Internal Medicine·W J BurmanM Bornemann
Jul 1, 1968·The American Journal of Digestive Diseases·R E Brown, W J Still
May 1, 1983·Clinical Endocrinology·M H OliverW Van't Hoff
Jan 1, 1983·American Journal of Clinical Pathology·K M WeidenheimW G Campbell
Aug 15, 1995·Annals of Internal Medicine·J L DoppmanP Gorden
Jun 1, 1995·Diabetic Medicine : a Journal of the British Diabetic Association·D WalmsleyJ S Bevan
Jun 1, 1994·Nature Genetics·B GlaserC A Stanley
Dec 1, 1996·The Journal of Clinical Endocrinology and Metabolism·Y FukunagaH Koshiyama
Aug 15, 1997·The Journal of Clinical Investigation·P de LonlayC Junien
Jan 22, 1998·The New England Journal of Medicine·B GlaserK C Herold
May 8, 1998·The New England Journal of Medicine·C A StanleyM Poncz
May 14, 1999·The Journal of Clinical Endocrinology and Metabolism·F J ServiceR V Lloyd
Feb 24, 2000·Archives of Disease in Childhood. Fetal and Neonatal Edition·A Aynsley-GreenK J Lindley
Jun 11, 2003·European Journal of Clinical Investigation·K KaczirekB Niederle
Nov 2, 2004·World Journal of Surgery·Klaus Kaczirek, Bruno Niederle
Dec 8, 2004·European Journal of Internal Medicine·A L Jabri, C Bayard
Sep 15, 2005·Internal Medicine·Motoyoshi TsujinoYukio Hirata
Oct 29, 2005·Best Practice & Research. Clinical Gastroenterology·Clive S Grant
Feb 16, 2006·Current Medicinal Chemistry·John Bondo Hansen
Mar 18, 2006·Endocrine Journal·Tadashi AraoYoshiya Tanaka
Oct 24, 2006·Clinical Endocrinology·Justin G S WonAaron Vinik
Apr 17, 2007·Arquivos brasileiros de endocrinologia e metabologia·Raquel R CostaLevimar R Araújo
Jul 5, 2007·European Journal of Endocrinology·D VezzosiP Caron
Dec 12, 2007·Human Pathology·Günter KlöppelWolfram Trudo Knoefel
Jan 8, 2008·World Journal of Gastroenterology : WJG·Ran HongSung-Chul Lim
Mar 6, 2008·The Surgeon : Journal of the Royal Colleges of Surgeons of Edinburgh and Ireland·M GeraghtyM J Cullen