Hereditary pancreatitis overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Iqra Qamar M.D.[2]
Overview
Historical Perspective
In 1642, Johannes Wirsung of Padua first described the pancreatic duct and the concept of the pancreas as a secretory organ. In 1952, Comfort and Steinberg , were the first one to identify a genetic background associated with hereditary pancreatitis and they found hereditary pancreatitis in six family members spanning 3 generations. In 1996, a gene for hereditary chronic pancreatitis was mapped to chromosome 7.
Classification
Hereditary pancreatitis may be classified on the basis of mode of inheritance into autosomal dominant, autosomal recessive and hereditary pancreatitis with complex genetics.
Pathophysiology
Causes
Hereditary pancreatitis may be caused by mutation in any one of the following genes PRSS1, SPINK1, CFTR and CTRC gene.
Differentiating Hereditary pancreatitis from Other Diseases
Hereditary pancreatitis needs to be differentiated from other diseases presenting with similar complaints such as abdominal pain, diarrhea and weight loss.