Hereditäre chronisch-calcifizierende Pankreatitis

Abstract

Four family members covering three generations presented with chronic calcifying pancreatitis. A tumor at the pancreas head was detected intraoperatively in a fifth elderly member of the family. Two of the four cases of chronic pancreatitis were diagnosed in childhood and one in adolescence. The fourth patient had typical symptoms during adolescence but the disease was not recognized at that time. Hereditary chronic pancreatitis has an autosomal-dominant inheritance with incomplete penetrance. The pathogenesis is not known. The course of the disease differed between the family members. Duodenal stenosis with gastrointestinal bleeding was observed, but also a more mild development with recurrent pain and long complaint-free intervals. The occurrence of complications and pain appears to decrease with increasing age. The extent of calcification, widening of the duct, exocrine and endocrine pancreatic insufficiency varies. The question of conservative or operative therapy depends on the course of the disease. A Whipple operation was vitally necessary in one child at the age of six. A pancreo-jejunostomy had to be introduced in a further member of the family at the age of 20 years due to an occlusion of the duct. Two patients receive only conservative treatment.