Hereditary pancreatitis natural history, complications and prognosis
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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
The symptoms of hereditary pancreatitis usually develop in the first or second decade of life. Patients with hereditary pancreatitis usually present with recurrent episodes of acute pancreatitis and may develop exocrine and endocrine insufficiency. If left untreated, patients with hereditary pancreatitis may progress to develop pancreatitis, biliary or pancreatic ductal dilatation, jaundice, biliary obstruction, pancreatic duct stone or stricture, pancreatic pseudocysts, and pancreatic cancer. Mortality rate is found to be increased in patients who develop pancreatic cancer.
Natural History, Complications, and Prognosis
Natural History
- The symptoms of hereditary pancreatitis usually develop in the first or second decade of life.
- Patients with hereditary pancreatitis usually present with recurrent episodes of acute pancreatitis and may develop exocrine and endocrine insufficiency.
- If left untreated, patients with hereditary pancreatitis may progress to develop
- Pancreatitis
- Biliary or pancreatic ductal dilatation
- Jaundice
- Biliary obstruction
- Pancreatic duct stone or stricture
- Pancreatic pseudocysts
- Pancreatic cancer.
Complications
- Diabetes[1]
- Pseudocysts
- Bile duct or duodenal obstruction
- Pancreatic ascites[2][3]
- Pleural effusion
- Splenic vein thrombosis[4][5][6]
- Pseudoaneurysm of splenic artery[7][8][9]
- Pancreatic cancer[10]
Prognosis
- Mortality rate is found to be increased in patients who develop pancreatic cancer.[11]
- The overall mortality is not increased in patients with hereditary pancreatitis who do not develop pancreatic cancer compared with the general population.
- The median survival age is 74.[11]
References
- ↑ Rickels MR, Bellin M, Toledo FG, Robertson RP, Andersen DK, Chari ST, Brand R, Frulloni L, Anderson MA, Whitcomb DC (2013). "Detection, evaluation and treatment of diabetes mellitus in chronic pancreatitis: recommendations from PancreasFest 2012". Pancreatology. 13 (4): 336–42. doi:10.1016/j.pan.2013.05.002. PMC 3830751. PMID 23890130.
- ↑ Runyon BA (1987). "Amylase levels in ascitic fluid". J. Clin. Gastroenterol. 9 (2): 172–4. PMID 2437177.
- ↑ Gómez-Cerezo J, Barbado Cano A, Suárez I, Soto A, Ríos JJ, Vázquez JJ (2003). "Pancreatic ascites: study of therapeutic options by analysis of case reports and case series between the years 1975 and 2000". Am. J. Gastroenterol. 98 (3): 568–77. PMID 12650789.
- ↑ Forsmark CE, Wilcox CM, Grendell JH (1992). "Endoscopy-negative upper gastrointestinal bleeding in a patient with chronic pancreatitis". Gastroenterology. 102 (1): 320–9. PMID 1727767.
- ↑ Sakorafas GH, Sarr MG, Farley DR, Farnell MB (2000). "The significance of sinistral portal hypertension complicating chronic pancreatitis". Am. J. Surg. 179 (2): 129–33. PMID 10773149.
- ↑ Bernades P, Baetz A, Lévy P, Belghiti J, Menu Y, Fékété F (1992). "Splenic and portal venous obstruction in chronic pancreatitis. A prospective longitudinal study of a medical-surgical series of 266 patients". Dig. Dis. Sci. 37 (3): 340–6. PMID 1735356.
- ↑ Beattie GC, Hardman JG, Redhead D, Siriwardena AK (2003). "Evidence for a central role for selective mesenteric angiography in the management of the major vascular complications of pancreatitis". Am. J. Surg. 185 (2): 96–102. PMID 12559436.
- ↑ Arnaud JP, Bergamaschi R, Serra-Maudet V, Casa C (1994). "Pancreatoduodenectomy for hemosuccus pancreaticus in silent chronic pancreatitis". Arch Surg. 129 (3): 333–4. PMID 8129612.
- ↑ Wagner WH, Cossman DV, Treiman RL, Foran RF, Levin PM, Cohen JL (1994). "Hemosuccus pancreaticus from intraductal rupture of a primary splenic artery aneurysm". J. Vasc. Surg. 19 (1): 158–64. PMID 8301728.
- ↑ Lowenfels AB, Maisonneuve P, DiMagno EP, Elitsur Y, Gates LK, Perrault J, Whitcomb DC (1997). "Hereditary pancreatitis and the risk of pancreatic cancer. International Hereditary Pancreatitis Study Group". J. Natl. Cancer Inst. 89 (6): 442–6. PMID 9091646.
- ↑ 11.0 11.1 Rebours V, Boutron-Ruault MC, Jooste V, Bouvier AM, Hammel P, Ruszniewski P, Lévy P (2009). "Mortality rate and risk factors in patients with hereditary pancreatitis: uni- and multidimensional analyses". Am. J. Gastroenterol. 104 (9): 2312–7. doi:10.1038/ajg.2009.363. PMID 19550412.