Hyperinsulinemic Hypoglycemia - Causes

Causes

Hypoglycemia due to endogenous insulin can be congenital or acquired, apparent in the newborn period, or many years later. The hypoglycemia can be severe and life-threatening or a minor, occasional nuisance. By far the most common type of severe but transient hyperinsulinemic hypoglycemia occurs accidentally in persons with type 1 diabetes who take insulin.

• Hypoglycemia due to endogenous insulin
  • Congenital hyperinsulinism
    • Transient neonatal hyperinsulinism (mechanism not known)
    • Focal hyperinsulinism (K_ATP channel disorders)
      • Paternal SUR1 mutation with clonal loss of heterozygosity of 11p15
      • Paternal Kir6.2 mutation with clonal loss of heterozygosity of 11p15
    • Diffuse hyperinsulinism
      • K_ATP channel disorders
        • SUR1 mutations
        • Kir6.2 mutations
      • Glucokinase gain-of-function mutations
      • Hyperammonemic hyperinsulinism (glutamate dehydrogenase gain-of-function mutations)
      • Short chain acyl coenzyme A dehydrogenase deficiency
      • Carbohydrate-deficient glycoprotein syndrome (Jaeken's Disease)
      • Beckwith-Wiedemann syndrome(suspected due to hyperinsulinism but pathophysiology uncertain: 11p15 mutation or IGF2 excess)
  • Acquired forms of hyperinsulinism
  • Insulinomas (insulin-secreting tumors)
    • Islet cell adenoma or adenomatosis
    • Islet cell carcinoma
  • Adult nesidioblastosis
  • Autoimmune insulin syndrome
  • Noninsulinoma pancreatogenous hypoglycemia
  • Reactive hypoglycemia (also see idiopathic postprandial syndrome)
  • Gastric dumping syndrome

• Drug induced hyperinsulinism
  • Sulfonylurea
  • Aspirin
  • Pentamidine
  • Quinine
  • Disopyramide
  • Bordetella pertussis vaccine or infection

• Hypoglycemia due to exogenous (injected) insulin
  • Insulin self-injected for treatment of diabetes (i.e., diabetic hypoglycemia)
  • Insulin self-injected surreptitiously (e.g., Munchausen syndrome)
  • Various forms of diagnostic challenge or "tolerance tests"
    • Insulin tolerance test for pituitary or adrenergic response assessment
    • Protein challenge
    • Leucine challenge
    • Tolbutamide challenge
  • Insulin potentiation therapy
  • Insulin-induced coma for depression treatment