Hereditary pancreatitis appears in many different ways and in a variety of age groups, spanning both pediatric and adult medicine. The variable expression of hereditary pancreatitis is emphasized by the difficulty in diagnosing it in a patient obviously at risk because of a severely affected father and son. The morphine prostigmine test and hypotonic duodenogram were most helpful. Aminoaciduria previously associated with this disorder is coincidental or nonspecifically related to acute pancreatic inflammation. The increased risk for pancreatic carcinoma (about 20%) is emphasized by the concern for that complication in the proband's grandfather.
Riccardi VM, Shih VE, Holmes LB, Nardi GL. Hereditary PancreatitisNonspecificity of Aminoaciduria and Diagnosis of Occult Disease. Arch Intern Med. 1975;135(6):822-825. doi:10.1001/archinte.1975.00330060066009