[Hereditary chronic relapsing calcifying pancreatitis].
The clinical picture of the hereditary chronic relapsing calcifying pancreatitis is demonstrated on the basis of a family. In 7 members of the family the disease could be ascertained, in other 3 members it is probably existing. Main symptom in all patients are severe relapsing epigastric pains with manifestations already in childhood or time of youth. Calcifications of the pancreas were always found and except one female patient an exocrine insufficiency of the pancreas with superficial steatorrhoea. A diabetes mellitus became manifest only in three of the seven patients. A larger attention is to be paid to the diagnosis of this special form of the chronic relapsing pancreatitis, in order to avoid long diagnostic wrong ways and non-indicated laparotomies in etiologically not clarified epigastric complaints. The therapy of the hereditary pancreatitis is conservative. Only in complications or in a not controllable so-called syndrome of pain it is recommended to decide on an operative approach, as in the family demonstrated in two female patients.
D000328:Adult; D000368:Aged; D002114:Calcinosis / Q000150:complications*; D002908:Chronic Disease; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010195:Pancreatitis / Q000150:complications / Q000000981:diagnostic imaging / Q000235:genetics*; D010375:Pedigree; D011859:Radiography; D012008:Recurrence
Die hereditäre chronisch-rezidivierende kalzifizierende Pankreatitis.