Hemorrhagic Course In A Patient With Hereditary Pancreatitis

Case report. This case-report presents the clinical course in a 7 year old boy with hereditary pancreatitis who was suffering from melaena requiring frequent transfusions and severe abdominal pain correlated with slightly elevated serum lipase and amylase. The diagnosis hereditary pancreatitis was established by detection of the mutation R122H in the cationic trypsinogen gene. Later on, the family history could be completed and revealed a pancreatic carcinoma in a grand uncle of the patient. Although identification of the source of the gastrointestinal bleeding was inconclusive various signs and one occasion with observable bleeding from the pancreatic duct during duodenoscopy lead to the assumption of pancreatic bleeding. Following a period of continued seriousness in the patients condition over half-a-year a decision to carry out a longitudinal pancreaticojejunostomy was pre-empted by spontaneous remission with a complete absence of clinical symptoms. Conclusion. Hereditary pancreatitis may have a hemorrhagic course with gastrointestinal blood losses. Owing to the incomplete penetrance of the illness the differential diagnosis hereditary pancreatitis cannot be discounted even in a patient with a negative family history.