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Key Features

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  • Chronic pancreatitis is differentiated from acute pancreatitis in that the pancreas remains structurally or functionally abnormal after an attack

  • Causes are multiple and can be divided into

    • Toxic-metabolic (eg, alcohol, chronic kidney disease, hypercalcemia)

    • Idiopathic

    • Genetic (increasingly recognized in children and adolescents)

    • Autoimmune

    • Recurrent and severe acute pancreatitis

    • Obstructive pancreatitis (eg, pancreas divisum, choledochal cyst)

  • Diagnosis often is delayed by the nonspecificity of symptoms and the lack of persistent laboratory abnormalities

  • There is usually a prolonged history of recurrent upper abdominal pain of variable severity

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Clinical Findings

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  • Radiation of the pain into the back is a frequent complaint

  • Fever and vomiting are rare

  • Diarrhea, due to steatorrhea, and symptoms of diabetes may develop later in the course

  • Malnutrition due to acquired exocrine pancreatic insufficiency may also occur

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Diagnosis

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  • Serum amylase and lipase levels are usually elevated during early acute attacks, but are often normal in the chronic phase

  • Pancreatic insufficiency may be diagnosed by demonstration of a low fecal pancreatic elastase 1

  • Mutations of the cationic trypsinogen gene, the pancreatic secretory trypsin inhibitor, the cystic fibrosis transmembrane conductance regulator (CFTR), carboxypeptidase A1 and chymotrypsin C are associated with recurrent acute and chronic pancreatitis

  • Elevated blood glucose and glycohemoglobin levels and glycosuria frequently occur in protracted disease

  • Radiographs of the abdomen may show pancreatic calcifications in up to 30% of patients

  • Ultrasound or CT examination demonstrates an abnormal gland (enlargement or atrophy), ductal dilation, and calculi in up to 80%

  • CT is the initial imaging procedure of choice

  • MRCP or ERCP can show ductal dilation, stones, strictures, or stenotic segments

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Treatment

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  • If ductal obstruction is strongly suspected, endoscopic therapy (balloon dilation, stenting, stone removal, or sphincterotomy) should be pursued

  • Pancreatic enzyme therapy should be used in patients with pancreatic insufficiency

  • Lateral pancreaticojejunostomy or the Frey procedure can reduce pain in pediatric patients with a dilated pancreatic duct and may prevent or delay progression of functional pancreatic impairment

  • Pancreatectomy and islet cell autotransplantation has been used in selected cases

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