Complex glycemic instability in a patient with nesidioblastosis: diagnostic and therapeutic challenges

Abstract

Diffuse congenital hyperinsulinism (CH) can be resistant to medical therapy and may require total pancreatectomy. This procedure controls hypoglycemia but frequently results in pancreatogenic diabetes (type 3c) and persistent exocrine insufficiency, creating significant management challenges. A 14-year-old female with a family history of CH and recurrent hypoglycemia since infancy was initially treated with diazoxide (15 mg/kg/day) and high-carbohydrate intake, which failed. She underwent subtotal pancreatectomy at age 3, without improvement. A total pancreatectomy at age 5 led to insulin-dependent diabetes. She was managed with conventional insulin (1 U/kg/day), pancreatic enzyme replacement (Creon 7,500 U/kg/day), and fat-soluble vitamins (A, D, E, K). At age 14, she presented with poorly controlled diabetes (HbA1c 12.6%) characterized by unstable glycemia and frequent severe hypoglycemia despite insulin analogues. She also had steatorrhea (5 greasy stools/day; 13 g/24 h) without other signs of malabsorption. Laboratory results were within normal limits for calcium, potassium, and hemoglobin. No micro- or macrovascular complications were observed. Management included insulin dose adjustment, optimization of pancreatic enzymes, and continuation of vitamin supplementation. Pancreatogenic diabetes after total pancreatectomy for diffuse CH requires individualized, multidisciplinary management. Careful optimization of insulin therapy, pancreatic enzyme replacement, and vitamin supplementation is essential to achieve metabolic stability, prevent hypoglycemia, and maintain nutritional status.