Non-insulinoma pancreatogenous hypoglycaemia syndrome

NIPHS

Epidemiology

• Usually a disease of infancy, but in rare cases identified in adults

Pathophysiology

• Excessive pancreatic B cell function, with associated pathologic changes including pancreatic islet cell hyperplasia and dysplasia, and histologic identification of B cells budding from and in apposition to pancreatic ductal structures
• A recognised complication of bariatric surgery, especially RYGB

Presentation

• Hallmark is post-prandial hypoglycaemia and associated neuroglycopaenic symptoms, within 4 hours of a meal, which does not happen with insulinoma
• Diagnosis is based on exclusion of insulinoma

Treatment

• Pancreatectomy - 95% distal pancreatectomy
• Can be treated with dietary control and medical therapy with diazoxide and somatostatin analogues
• If occurring post-RYGB, first-line is dietary modification, glucose monitoring, and maybe medical management. Pancreatic resection is common where surgical intervention is required. Controversial area.