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adult diffuse nesidioblastosis

Friday 16 May 2008

Persistent hyperinsulinemic hypoglycemia (PHH) in adults that is not caused by an insulinoma is a rare and not well-characterized disease that has been named nesidioblastosis.

Diffuse nesidioblastosis in adult patients with PHH resembles that seen in neonates suffering from PHH. The most important criterion for the diagnosis is the beta-cell hypertrophy.

As approximately 4% of adult patients with PHH are affected by diffuse nesidioblastosis, this disease is not as rare as it has been thought to be. Pathogenetically, the defective insulin secretion could be based on a molecular defect. (15767809)


- Klöppel G, Anlauf M, Raffel A, Perren A, Knoefel WT. Adult diffuse nesidioblastosis: genetically or environmentally induced? Hum Pathol. 2008 Jan;39(1):3-8. PMID: 18070631

- Coincidence of insulinoma with nesidioblastosis or false diagnosis in 2 of 15 patients? Goretzki PE, Starkel A. Am J Surg Pathol. 2006 Jul;30(7):919-20; author reply 920-1. PMID: 16819338

- Persistent hyperinsulinemic hypoglycemia in 15 adults with diffuse nesidioblastosis: diagnostic criteria, incidence, and characterization of beta-cell changes. Anlauf M, Wieben D, Perren A, Sipos B, Komminoth P, Raffel A, Kruse ML, Fottner C, Knoefel WT, Mönig H, Heitz PU, Klöppel G. Am J Surg Pathol. 2005 Apr;29(4):524-33. PMID: 15767809