Pancreatic Disorders

Acute Pancreatitis - Fiery Flare-Up

• Sudden pancreatic inflammation. Key symptoms: severe epigastric pain (often radiating to back), nausea, vomiting.
• 📌 Pediatric Causes: Trauma, Drugs (valproate, L-asparaginase, steroids), Viral (Mumps, Coxsackie B), Systemic (CF, Kawasaki, HUS, IBD), Biliary/Anatomic (choledochal cyst, pancreas divisum), Idiopathic/Inherited (PRSS1, SPINK1).
• Diagnosis (≥2 of 3 criteria):
  • Characteristic acute onset, persistent, severe epigastric abdominal pain.
  • Serum lipase or amylase activity ≥3x ULN (Upper Limit of Normal).
  • Characteristic findings on abdominal imaging (USG, CECT, or MRI).
• Investigations: Serum lipase (preferred), amylase. USG abdomen (initial). CECT if diagnosis uncertain or for complications (e.g., necrosis) after 48-72 hrs.
• Management:
  • Supportive care: Nil Per Oral (NPO) initially, transitioning to early enteral nutrition if tolerated. Aggressive IV fluid resuscitation. Analgesia (opioids).
  • Monitor: Vital signs, urine output, electrolytes.
  • Treat underlying cause (e.g., ERCP for choledocholithiasis).
• Complications: Pancreatic pseudocyst, necrosis, abscess; Systemic: SIRS, ARDS, renal failure.

⭐ Serum lipase is preferred over amylase for diagnosing acute pancreatitis in children due to its higher sensitivity and specificity, and longer half-life.

Chronic Pancreatitis & EPI - Lasting Ache & Leak

• Chronic Pancreatitis (CP): Irreversible pancreatic damage, fibrosis, and ductal stricturing.
  • Causes: Alcohol (adults), tropical pancreatitis (common in India, early onset, large duct stones), genetic (CFTR, SPINK1, PRSS1), autoimmune.

  ⭐ Cystic fibrosis is the leading genetic cause of chronic pancreatitis and exocrine pancreatic insufficiency in children.

  • Symptoms: Persistent/recurrent epigastric pain (radiates to back), malabsorption (steatorrhea, weight loss), diabetes mellitus.
  • Diagnosis: Imaging shows calcifications (X-ray, CT), ductal dilation/strictures (MRCP - "chain of lakes" appearance), pancreatic atrophy.
• Exocrine Pancreatic Insufficiency (EPI): Reduced pancreatic enzyme secretion leading to maldigestion.
  • Symptoms: Steatorrhea (bulky, foul-smelling, greasy stools), weight loss, fat-soluble vitamin (A, D, E, K) deficiencies.
  • Diagnosis: Fecal elastase-1 < 200 µg/g stool (severe EPI < 100 µg/g).
  • Management: Pancreatic Enzyme Replacement Therapy (PERT), vitamin supplementation.
  • 📌 Mnemonic (EPI symptoms): Steatorrhea, Abdominal pain/distension, Decreased weight/vitamin deficiency (SAD).

Congenital & Other Issues - Twisted Tubes, Rare Lumps

• Annular Pancreas
  • Embryology: Ventral bud malrotation; encircles 2nd part of duodenum.
  • Sx: Neonatal duodenal obstruction, bilious/non-bilious vomiting.
  • Dx: X-ray ('double bubble' sign).
  • Tx: Surgical bypass (duodenoduodenostomy).
• Pancreas Divisum
  • Most common; failed fusion of ventral/dorsal ducts.
  • Often asymptomatic; can cause recurrent pancreatitis.
  • Dx: MRCP, ERCP (gold standard). Tx: Symptomatic - endoscopic sphincterotomy.
• Ectopic Pancreas
  • Pancreatic tissue in stomach, duodenum, Meckel's. Usually asymptomatic.
• Rare Tumors
  • Pancreatoblastoma: Most common malignant tumor in childhood (< 10 yrs); ↑ AFP.
  • Solid Pseudopapillary Neoplasm (SPN): Adolescent females; low malignant potential.

⭐ Annular pancreas typically presents in neonates with duodenal obstruction, vomiting (often bilious), and a characteristic 'double bubble' sign on abdominal X-ray.

Diagnostic & Management Pearls - Pancreas Pointers

• Key Labs:
  • Serum Lipase: ↑ in 4-8h, peaks 24h, normalizes 8-14d. More specific than amylase.
  • Serum Amylase: ↑ in 6-12h, normalizes 3-5d.
  • Trypsinogen (IRT): Newborn screen for Cystic Fibrosis.
• Exocrine Function:
  • Fecal Elastase-1: Measures enzyme output.

  ⭐ Fecal elastase-1 is a highly sensitive and specific non-invasive test for assessing exocrine pancreatic function, particularly useful in children with suspected malabsorption.

• Imaging:
  • USG: Initial; gallstones, pseudocysts.
  • CT/MRI/MRCP: Severity, complications, ductal anatomy.
• Management Core:
  • Acute: IV fluids, analgesia, NPO (brief), early enteral nutrition.
  • Chronic: Pain control, PERT, fat-soluble vitamins.
  • Address underlying cause.

High‑Yield Points - ⚡ Biggest Takeaways

• Cystic fibrosis: leading cause of exocrine pancreatic insufficiency in children.
• Acute pancreatitis in children: often due to trauma, viral infections (mumps), or medications.
• Recurrent/chronic pancreatitis: consider genetic mutations (PRSS1, CFTR).
• Pancreatic pseudocysts: common after acute pancreatitis or abdominal trauma.
• Serum lipase: more specific than amylase for diagnosing acute pancreatitis.
• Shwachman-Diamond syndrome: features pancreatic insufficiency, neutropenia, skeletal abnormalities.