Tropical pancreatitis :
is a disease of young individuals stemming from endemic regions such as southern India and portions of South America.
The disease typically presents at a young age. Affected individuals most commonly present with profound weight loss and malnutrition, often accompanied by abdominal pain.
The cause of malnutrition is typically pancreatic exocrine dysfunction and is the dominant cause of mortality in endemic areas.
Endocrine deficiency with difficult-to-control diabetes ensues in the majority of affected individuals. Secondary complications of diabetes are common.
Imaging demonstrates pancreatic fibrosis, a dilated pancreatic duct with large intraductal calcifications.
The pathogenesis of disease is poorly defined but has been linked to ingestion of local fruits (e.g., cassava), although more reliably to genetic factors such as SPINK-1.
There is no strong association with protein-calorie malnutrition or cassava ingestion and no association with APO C-II deficiency.
Hereditary pancreatitis:
is recognized as an autosomal-dominant disorder caused by a genetic defect identified in the members of a family affected with pancreatitis.
Several large kindreds were identified during the past 50 years, demonstrating that disease susceptibility is transmitted in an autosomal-dominant pattern, with high penetrance (80% of gene mutation carriers affected) and variable expression.
These large kindreds all proved to have a pancreatitis disease gene locus on chromosome 7.
The disease gene was identified as the cationic trypsinogen gene (PRSS1). Several common mutations in the cationic trypsinogen gene are now known to be associated with hereditary pancreatitis.
In addition, mutations in the SPINK1/PSTIgene have recently been associated with familial pancreatitis and with idiopathic chronic pancreatitis.
Growing experience with genetic testing reveals that family history alone is not an accurate predictor of detecting or excluding specific genes or mutations that predispose to pancreatitis. Thus, family history serves as an important clue to a genetic predisposition, but the final determination continues to require genetic testing.
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