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January 1983

Idiopathic Hereditary Pancreatitis

Author Affiliations

Rotterdam, Holland

Arch Surg. 1983;118(1):129. doi:10.1001/archsurg.1983.01390010095026

To the Editor.—I read with interest the article by Williams et al (Archives 1982;117:408-412) and I have some complementary remarks. In addition to the mentioned countries in Europe, kindreds also have been described in the Netherlands,1 Scandinavia, and Switzerland. The pathogenesis of the disease is unknown. Although the existence of an anatomic structural defect in the pancreatic ducts would be an attractive causative theory for hereditary pancreatitis, the evidence in the literature has been conflicting and inconclusive. Some patients showed no duct abnormalities. Structural changes in the pancreatic ducts may well have been secondary to the pancreatitis and not the cause of it. There is experimental evidence that blockage of the pancreatic ducts will not cause pancreatitis. It seems more likely that a structural defect in the protein synthesis of the pancreatic juice will be responsible in most of the patients. The role of elective surgery in hereditary

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