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Introduction
Acute pancreatitis is an inflammatory condition that usually affects a previously unaltered pancreas [1, 2]. In Western countries, common bile duct stone migration and alcohol abuse account for most cases of acute pancreatitis. Once the attack has occurred and the cause is treated, the gland will usually completely recover. On the other hand, recurrent attacks of pancreatitis or chronic pancreatitis may progressively alter the pancreatic parenchyma [3, 4]. Chronic pancreatitis is characterized by progressive loss of exocrine and endocrine functions that will ultimately lead to pancreas insufficiency with steatorrhea, weight loss, and diabetes [5]. Alcohol abuse, smoking, and autoimmune pancreatitis are the leading causes of chronic pancreatitis. In 1996, hereditary pancreatitis was found to be caused by a mutation in the cationic trypsinogen gene PRSS1 [6, 7]. However, PRSS1 mutations explain only part of genetically transmitted disease, suggesting that other genes were involved in the pathogenesis of chronic pancreatitis. In 2000, the serine protease inhibitor Kazal type 1 (SPINK1) was identified as a second pancreatitis gene. The SPINK1 protein is a potent antiprotease localized in the cytoplasm of acinar cells. Its function is to inactivate any intrapancreatic trypsin activity that would otherwise induce autodigestion of the gland [8]. The SPINK1 mutation c.101A>G (p.Asn34Ser, commonly called N34S) has been recognized to be associated with chronic pancreatitis in patients without a family history of pancreatitis. N34S is a rare polymorphism, with a worldwide carrier frequency of 1–3%, and is a known predisposing or risk factor for chronic pancreatitis, although only a minority of carriers develop disease. Reports claim that 15–40% of patients with so-called idiopathic pancreatitis indeed carry N34S on one allele or on both alleles, and that little or no phenotypic differences between heterozygous and homozygous N34S patients have been detected so far [9]. A number of rarer SPINK1