Chronic pancreatitis

Question 1

Chronic pancreatitis - Pathology:

Answer 1

• Progressive inflammatory disease of the pancreas.
• The Pancreas has a large reserve capacity, thus is asymptomatic until loss of over 90 percent of pancreatic exocrine function

Question 2

Chronic pancreatitis - Causes:

Answer 2

• Toxic/metabolic
• Idiopathic
• Genetic
• Autoimmune Pancreatitis
• Recurrent acute
• Obstructive

Question 3

Chronic pancreatitis - Genetic causes:

Answer 3

• Cystic Fibrosis (CFTR)
• SPINK1 (serine protease inhibitor kazal type 1) - found in 25% of children/adolescents previously considered to have idiopathic disease
• Hereditary Pancreatitis
• PRSS1 (cationic trypsinogen (serine Protease 1) mutations
  
  • Autosomal dominant
  • Affects children
  • Risk factor for pancreatic cancer


Question 4

Chronic pancreatitis - Autoimmune pancreatitis - Type 1:

Answer 4

• Adults > Children
• Sometimes associated with a multi organ autoimmune disorder
• Mild symptoms + elevated serum IgG4

Question 5

Chronic pancreatitis - Autoimmune pancreatitis - Type 2:

Answer 5

• Disease of the pancreatic duct
• Low IgG4
• High GEL
• Associated with IBD

Question 6

Chronic pancreatitis - clinical features:

Answer 6

• Recurrent abdominal pain (15-30 mins after eating) -> persistent pain
• Typically epigastric -> radiates to back
• nausea/ vomiting - relieved by sitting forward
• Diabetes mellitus
• Fat and protein malabsorption

Question 7

Chronic pancreatitis - Diagnostics:

Answer 7

• Recurrent upper abdomen pain and/or pancreatic insufficiency +
• Calcifications on CT/xRay
• Increased diameter of the pancreatic duct
• Tests

Question 8

Chronic pancreatitis - Diagnostics - Tests:

Answer 8

• CBC, Electrolytes, Serum, serum glucose, LFTs, pancreatic enzymes
• Pancreatic insufficiency test
  
  • Fecal elastase-1 (Sudan III stain)
    ^ Decreased levels also seen in children with other causes of diarrhea e.g. villous atrophy or bacterial/ viral gastroenteritis
  • Stool chymotrypsin

Question 9

Chronic pancreatitis - Treatment:

Answer 9

• fat soluble vitamins (A, D, E, K) replacement
• Pain management
• Exocrine supplementation
• Endocrine supplementation
• ERCP- in symptomatic patients with stenosis/calcifications/ calculi and prestenotic dilatation of PD.

Question 10

Chronic pancreatitis - Differentials - exocrine insufficiency:

Answer 10

• pancreatic agenesis,
• hypoplasia or resection,
• cystic fibrosis,
• Shwachman-Diamond syndrome (SBDS gene),
• isolated enzyme deficiencies,
• malnutrition,
• primary sclerosing cholangitis,
• and Alagille syndrome ( includes abnormalities of bile ducts).

Question 11

Chronic pancreatitis - Complications:

Answer 11

• Pancreatic pseudocysts - encapsulated collection of pancreatic fluid which develops 4 weeks after an acute attack of pancreatitis; can occur in both acute and chronic pancreatitis
• Splenic vein thrombosis
• Pancreatic ascites
• Others

Question 12

Chronic pancreatitis - Complications - Others:

Answer 12

• Pancreatic abscess
• Portal vein thrombosis
• Pancreatic diabetes
• Pancreatic cancer (especially in patients with hereditary pancreatitis)