Hereditary Pancreatitis

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Abstract
The 7th kindred with hereditary pancreatitis is described. Of the 71 members, data were obtained on 55, and 18 of the 55 were examined by the authors. Six members had definite pancreatitis and 5 had suspected pancreatitis. The mode of transmission is non-sex-linked and appears to be autosomal dominant. The disease is characterized by onset in childhood of recurrent episodes of abdominal pain and is frequently accompanied by pancreatic calcifications, diabetes mellitus and steatorrhea. Comparison with 6 kindreds reported by others reveals some differences. In a series of 5 kindreds with hereditary pancreatitis reported from the Mayo Clinic, aminoaciduria of the incompletely recessive form of cystinuria was associated whereas urinary levels of amino acids, measured by alpha-amino N, were within the normal range in the present kindred. In the 6th kindred, reported from France, there was a high incidence of hemorrhagic pleural and peritoneal effusions, dilatation of the ducts of Wirsung and excellent results from surgical procedures, designed to drain the pancreatic ducts and cysts, in contrast to the findings in the present kindred.

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