Noninsulinoma pancreatogenous hypoglycemia syndrome
- F John Service, MD, PhD
F John Service, MD, PhD
- Emeritus Professor of Medicine
- Mayo Clinic College of Medicine
- Adrian Vella, MD
Adrian Vella, MD
- Professor of Medicine
- Mayo Clinic
The noninsulinoma pancreatogenous hypoglycemia syndrome (NIPHS) identifies a group of hyperinsulinemic hypoglycemic patients with unique clinical, diagnostic, surgical, and pathologic features [1,2]. These patients experience predominantly postprandial hypoglycemia and have nesidioblastosis with islet cell hypertrophy, findings different from those in patients with insulinomas.
The clinical manifestations, pathological findings, diagnosis, and treatment of NIPHS will be reviewed here. Other causes of hypoglycemia and how to differentiate among these causes are reviewed elsewhere. (See "Hypoglycemia in adults: Clinical manifestations, definition, and causes" and "Hypoglycemia in adults without diabetes mellitus: Diagnostic approach".)
Noninsulinoma pancreatogenous hypoglycemia syndrome (NIPHS) is a rare syndrome characterized by endogenous hyperinsulinemic hypoglycemia that is not caused by an insulinoma. Pancreatic specimens from such patients show beta cell hypertrophy, islets with enlarged and hyperchromatic nuclei, and increased islets budding from periductular epithelium [1-3]. These histologic findings are characteristic of nesidioblastosis, a term that refers to neoformation of islets of Langerhans from pancreatic duct epithelium . The pathologic findings are similar to those seen in neonates and infants with persistent hyperinsulinemic hypoglycemia. (See "Pathogenesis, clinical features, and diagnosis of persistent hyperinsulinemic hypoglycemia of infancy", section on 'Pathology of the beta cell'.)
Formal epidemiologic studies have not been conducted for NIPHS; however, the condition appears to be rarer than insulinoma. For example, during the period from 1996 to 2004, 20 patients with NIPHS (16 male, 4 female) and 118 patients with insulinoma (51 male, 67 female) were surgically confirmed at the Mayo Clinic [1,2,5].
Although the underlying pathologic features of NIPHS and post-gastric bypass hypoglycemia are similar in most instances, post-gastric bypass hypoglycemia is considered a separate clinical entity. (See 'Nesidioblastosis after Roux-en-Y gastric bypass surgery' below.)
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- CLINICAL FEATURES
- Biochemical findings
- DIFFERENTIAL DIAGNOSIS
- Nesidioblastosis after Roux-en-Y gastric bypass surgery
- - Dumping syndrome
- DIAGNOSTIC EVALUATION
- Laboratory evaluation
- Localization studies
- Mild to moderate symptoms
- Severe or refractory symptoms
- Recurrent symptoms
- SOCIETY GUIDELINE LINKS
- SUMMARY AND RECOMMENDATIONS