Pancreatic and Biliary Disorders
Scope
Acute and chronic pancreatitis, pancreatic insufficiency (cystic fibrosis, Shwachman-Diamond), biliary atresia, choledochal cysts, and gallstone disease.
Concepts
- Acute pancreatitis: requires two of three-characteristic pain, lipase >3× upper limit, or imaging findings. Treat with fluids and early enteral feeding; avoid unnecessary antibiotics.
- Chronic pancreatitis: recurrent pain, calcifications, exocrine insufficiency; fecal elastase low.
- Cystic fibrosis pancreatic insufficiency: steatorrhea, fat-soluble vitamin deficiency; treat with pancreatic enzyme replacement and monitor growth.
- Biliary atresia: jaundice beyond 2 weeks with direct hyperbilirubinemia, acholic stools; Kasai portoenterostomy improves outcomes when performed early.
- Choledochal cyst: risk of cholangiocarcinoma; surgical excision.
Traps
Questions bait you into ordering lipase in a child with abdominal pain but normal values-remember the three-criteria rule. Another trap: missing biliary atresia because jaundice is attributed to breast milk. Always fractionate bilirubin in prolonged neonatal jaundice.
Worked example
A 5-week-old with jaundice, pale stools, and direct hyperbilirubinemia: urgent evaluation for biliary atresia with ultrasound and hepatobiliary scintigraphy or biopsy; early Kasai improves native liver survival.
Rule of thumb
Direct hyperbilirubinemia in a neonate = biliary atresia until excluded. Lipase >3× ULN supports pancreatitis.