Pancreatic Insufficiency in Infants: Insulin & Enzyme Replacement

An infant born without a pancreas (pancreatic agenesis) or with complete pancreatic failure faces a life-threatening dual deficit: loss of both endocrine (insulin, glucagon) and exocrine (digestive enzymes) function. Lifelong, meticulous replacement is essential.

Endocrine Replacement

• Insulin: Continuous subcutaneous insulin infusion (pump) is preferred for precise, weight-based dosing; multiple daily injections are an alternative. Continuous glucose monitoring (CGM) is standard of care.
• Glucagon: Emergency kit (injection or nasal) for severe hypoglycemia; caregivers must be trained.

Exocrine Replacement

• Pancreatic Enzyme Replacement Therapy (PERT): Enteric-coated microspheres (lipase, protease, amylase) with every feed (breast milk, formula, solids).
• Dosing: 2,000–4,000 lipase units/kg/meal; titrated to growth, stool pattern (frequency, consistency, steatorrhea), and fat-soluble vitamin levels.
• Fat-Soluble Vitamins: Supplementation of vitamins A, D, E, and K is mandatory.

Multidisciplinary Care

Coordinated follow-up with pediatric endocrinology, gastroenterology, nutrition, and genetics is critical. Genetic testing (e.g., PDX1, PTF1A) informs recurrence risk counseling and may guide future therapies.