Hereditary pancreatitis
Last revised by Jeremy Jones on 20 Sep 2021
Citation, DOI, disclosures and article data
Citation:
Di Muzio B, Hacking C, St-Amant M, Hereditary pancreatitis. Reference article, Radiopaedia.org (Accessed on 19 Apr 2024) https://doi.org/10.53347/rID-50170
rID:
50170
Article created:
Disclosures:
At the time the article was created Bruno Di Muzio had no recorded disclosures.
View Bruno Di Muzio's current disclosures
Last revised:
20 Sep 2021,
Jeremy Jones ◉
Disclosures:
At the time the article was last revised Jeremy Jones had no recorded disclosures.
View Jeremy Jones's current disclosures
Revisions:
4 times, by
3 contributors -
see full revision history and disclosures
Systems:
Hereditary pancreatitis is a rare subtype of chronic pancreatitis that has an autosomal dominant inheritance and is one of the main causes of pancreatitis in childhood.
Clinical presentation
As a congenital condition, a presentation with acute pancreatitis attacks typically occurs in childhood, and features of chronic pancreatitis with parenchymal and ductal calcifications and atrophy are commonly seen when patients become adolescents.
The diagnosis is clinically considered when dealing with idiopathic chronic pancreatitis in young patients, especially when family history is present.
The condition has been reported with a 50-70-fold increased risk of pancreatic cancer 1.
References
- 1. Shanbhogue AK, Fasih N, Surabhi VR, Doherty GP, Shanbhogue DK, Sethi SK. A clinical and radiologic review of uncommon types and causes of pancreatitis. Radiographics : a review publication of the Radiological Society of North America, Inc. 29 (4): 1003-26. doi:10.1148/rg.294085748 - Pubmed
- 2. Rebours V, Lévy P, Ruszniewski P. An overview of hereditary pancreatitis. Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver. 44 (1): 8-15. doi:10.1016/j.dld.2011.08.003 - Pubmed
- 3. Kara L Raphael, Field F Willingham. Hereditary pancreatitis: current perspectives. (2016) Clinical and Experimental Gastroenterology. 9: 197. doi:10.2147/CEG.S84358 - Pubmed
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