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Hereditary pancreatitis-a report on two kindreds
Insights
Hereditary pancreatitis in two Swedish families presented in early childhood. Surgical intervention, pancreatico-jejunostomy, yielded excellent results for severe pain, suggesting its use in hereditary cases.
Area of Science:
- Gastroenterology
- Medical Genetics
Background:
- Hereditary pancreatitis is a rare genetic disorder.
- Understanding its clinical course and treatment options is crucial.
Observation:
- Two Swedish families with hereditary pancreatitis were studied.
- Symptoms manifested in early childhood.
- Clinical presentation mimicked non-hereditary forms, excluding common causes like alcohol abuse or hyperlipidemia.
Findings:
- Three patients underwent pancreatico-jejunostomy for intractable pain.
- The surgical procedure resulted in excellent outcomes.
- Observation periods ranged from 0.5 to 4 years.
Implications:
- Pancreatico-jejunostomy may be a viable surgical option for hereditary pancreatitis.
- This intervention can effectively manage severe pain and frequent exacerbations in hereditary pancreatitis patients.
Abstract:
Two Swedish kindreds with hereditary pancreatitis are reported. The onset of symptoms was in early childhood. Otherwise the clinical course did not differ from the non-hereditary form. In no patients were found signs of alcohol-overconsumption, hyperparathyreoidism, hyperlipidemia or mucoviscoidosis. Three patients with intractable pain and frequent hospitalization were operated on with pancreatico-jejunostomy as described by Puestow-Gillesby, with excellent results. Although the observation periods are short (0.5, 2 and 4 years, respectively) it seems legitimate to recommend the operation also for the hereditary form of pancreatitis if intractable pain or frequent exacerbations are present.