Etiologies (Gastroenterology 2001;120:682)
• TIGARO classification
• Toxic/metabolic: EtOH, hypercalcemia, hyperlipidemia, organic acidemias, CRF
• Idiopathic
• Genetic (PMID: 22094894)
• PRSS 1 (24%) (serine protease 1); cationic trypsinogen; ↑ trypsin act/block inactivation
• SPINK 1 (27%) (serine protease inhib Kazal type 1); AR, blocks trypsin inhibition, early onset
• CFTR (48%); highest risk in compound heterozygotes
• Hereditary pancreatitis; (AD genetics), onset age 10–12, strong risk for pancreatic Ca, most w/ some genetic mutation (PRSS 1 most common)
• Autoimmune: Isolated autoimmune pancreatitis, Sjogren, IBD, PBC
• Recurrent acute
• Obstructive: W/ pancreatic divisum, choledochal cysts, stricture, trauma, idiopathic
Clinical Presentation
• Repeated episodes of acute pancreatitis
• Chronic abdominal pain: Generally w/ epigastric pain, radiating to back, +/− emesis
• Worse after meals (particularly fatty foods)
• Fluctuates and may improve as exocrine glands “burn out”
• Insidious onset often with less pain
• Malabsorption (protein and fat); w/ greasy, loose, foul-smelling stools, FTT
• Diabetes mellitus/glucose intolerance (high risk for hypoglycemia, rarely DKA)
Diagnosis
• Often clinical diagnosis with repeated episodes of acute pancreatitis
• Laboratory: ↑ amylase and lipase (can be nml early or late), hyperglycemia (late and w/ endocrine pancr damage), ADEK and Vit B12 def (late), ↑ fecal fat (72-hr collection vs. Sudan III stain spot test), ↓ fecal elastase, gene test PRSS I, SPINK I, CFTR
• Imaging: KUB (pancreatic calcifications), abd US (↑ pancr duct diameter, pseudocyst), abd CT (calcification, dilated ducts, atrophy), MRCP (sens ↑ w/ secretin), ERCP (therapeutic as well; stricture dilation, stone extraction, stents)
Treatment
• Treat flares as you would episodes of acute pancreatitis (see previous section)
• Special considerations
• Pain control w/ narcotic/non-narcotic analgesia, avoid triggers
• Nutrition; low-fat diet, pancreatic enzyme replacement, adequate protein, fat soluble vitamins, NJ/J-tube feeds if severe growth failure
• Islet cell autotransplantation: Total pancreatectomy followed by reimplantation of islet cells into liver; may improve glycemic control and pain