Hereditary Pancreatitis: Report on Two Additional Families

Abstract

Summary The pedigrees of two additional, unrelated families in which the hereditary form of chronic relapsing pancreatitis and sequelae have occurred have been described. These additional observations tend to confirm the original impression that in certain families chronic relapsing pancreatitis is transmitted as an autosomal dominant gene. The hereditary form of the disease appears to resemble the sporadic form in most respects. However, the hereditary form of the disease does appear to begin earlier in life and appears so far to involve females predominantly, whereas males seem more often affected in the usual form of chronic pancreatitis. The cause and pathogenesis of the disease are not known. Alcohol and hyperlipemia appear to be unimportant factors in the etiology of the hereditary form of the disease, nor have gallstones been associated. It is suspected that the inherited defect predisposing to recurring bouts of pancreatitis and sequelae is metabolic in nature. It appears quite probable that the hereditary form of chronic relapsing pancreatitis occurs more frequently than has been realized in the past and that increasing awareness of the entity will result in the discovery of many more families so affected.