Literature DB >> 11387003

Adult-onset nesidioblastosis causing hypoglycemia: an important clinical entity and continuing treatment dilemma.

R M Witteles1, F H Straus II, S L Sugg, M R Koka, E A Costa, E L Kaplan.   

Abstract

HYPOTHESIS: Nesidioblastosis is an important cause of adult hyperinsulinemic hypoglycemia, and control of this disorder can often be obtained with a 70% distal pancreatectomy.
DESIGN: The records of all adult patients operated on for hypoglycemia between 1974 and 1999 were reviewed retrospectively. Patients with the pathologic diagnosis of nesidioblastosis were contacted for follow-up (1.5-21 years) and are presented. Patients' results were compared with those of 36 other individuals with this disorder who were previously reported in the literature.
SETTING: The University of Chicago Medical Center (Chicago, Ill), a tertiary care facility. PATIENTS: A consecutive sample of all patients operated on for hypoglycemia.
INTERVENTIONS: Seventy percent distal pancreatectomy for all patients with nesidioblastosis, and maintenance therapy with verapamil hydrochloride for 2 patients. MAIN OUTCOME MEASURES: Achievement of normoglycemia with and without medication, development of insulin-dependent diabetes mellitus, pancreatic exocrine insufficiency, and need for reoperation.
RESULTS: Of 32 adult patients who underwent surgical exploration for hyperinsulinemic hypoglycemia at our institution, 27 (84%) were found to have 1 or more insulinomas, and 5 (16%) were diagnosed with nesidioblastosis. Each patient with nesidioblastosis underwent a 70% distal pancreatectomy. Follow-up duration for the 5 patients ranged from 1.5 to 21 years, with 3 patients (60%) asymptomatic and taking no medications, and 2 patients (40%) experiencing some recurrences of hypoglycemia. The 2 patients with recurrences are now successfully treated with a calcium channel blocker, an approach, to our knowledge, never before reported for adult-onset nesidioblastosis.
CONCLUSIONS: Nesidioblastosis is an uncommon but clinically important cause of hypoglycemia in the adult population, and must always be considered in a patient with a presumptive preoperative diagnosis of insulinoma. This study indicates that a 70% distal pancreatectomy is often successful in controlling hypoglycemia, and rarely results in diabetes mellitus. However, the optimal treatment of this disorder remains to be determined.

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Year:  2001        PMID: 11387003     DOI: 10.1001/archsurg.136.6.656

Source DB:  PubMed          Journal:  Arch Surg        ISSN: 0004-0010


  24 in total

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7.  Hyperinsulinemic hypoglycemia due to diffuse nesidioblastosis in adults: a case report.

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10.  Metastatic insulinoma in an adult patient with underlying nesidioblastosis.

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