[Swiss family with chronic relapsing calcifying pancreatitis]

Abstract

A further Swiss family with hereditary chronic relapsing pancreatitis is reported. Five members definitely have the disease, while in 4 subjects the condition is suspected. The five proven cases are a mother and her four children. All suffer from recurrent attacks of abdominal pain which started between the 8th and 20th year of life, and in all of them exocrine pancreatic insufficiency and pancreatic calcifications can be demonstrated. The mother has had diabetes mellitus since the age of 40 and 3 of the 4 children also have the disease, with age of onset between 15 and 29. In 3 patients insignificant traces of aminoacids could be detected in the urine. At 16 years one male patient had a pseudocyst of the pancreas surgically removed. All the others are treated conservatively. Reports on approximately 25 families with this disease have been published so far. The condition is inherited as an autosomal dominant disorder. Typically, the clinical symptoms begin during childhood or early adolescent life. The pathogenesis is unknown. It is possible that an abnormal pancreatic juice is produced which causes the intracanalicular calcifications. The treatment is identical to that in the non-hereditary forms, i.e. generally conservative. Recently some authors have favored a more aggressive approach consisting of pancreatico-jejunostomy or partial resection of the pancreas.