Hereditary pancreatitis history and symptoms
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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
Patients with hereditary pancreatitis usually have a positive family history of recurrent acute pancreatitis or chronic pancreatitis occurring in two first degree relatives or three or more second degree relatives, in two or more generations in the absence of precipitating factors after negative work-up for known chronic pancreatitis etiology. The majority of patients with hereditary pancreatitis are asymptomatic. The most common presentation is recurrent acute pancreatitis.
History and Symptoms
Family history:
A positive family history is defined as recurrent acute pancreatitis or chronic pancreatitis occurring in two first degree relatives or three or more second degree relatives, in two or more generations in the absence of precipitating factors after negative work-up for known chronic pancreatitis etiology.
Symptoms:
- The majority of patients with hereditary pancreatitis are asymptomatic.
- The most common presentation is recurrent acute pancreatitis.[1][2][3][4][5]
- Presentation depends upon dysfunction in any one of the following:
- Biliary tract obstruction; presenting as jaundice
- Exocrine dysfunction; presenting as malabsorption
- Endocrine dysfunction; presenting as diabetes
Patients with chronic pancreatitis usually present with:
- Persistent abdominal pain with episodic flares may or may not be associated with food intake[6][7][8][9]. Typical features of pain are:
- Located in the epigastrium
- Radiating to the back
- May be associated with nausea and vomiting
- Usually worse 15-30min after meal
- Initially it occurs in discrete episodes but later on as the disease progresses, it changes to continuous-like pattern
- Steatorrhea resulting from malabsorption of the fats in food (greasy, loose, very bad smelling stools that are difficult to flush away).[10][11][12]
- Pancreatic diabetes[10][13][11]
- Nausea
- Weight loss
Less Common Symptoms
Less common symptoms of hereditary pancreatitis include
References
- ↑ 1.0 1.1 Howes N, Lerch MM, Greenhalf W, Stocken DD, Ellis I, Simon P, Truninger K, Ammann R, Cavallini G, Charnley RM, Uomo G, Delhaye M, Spicak J, Drumm B, Jansen J, Mountford R, Whitcomb DC, Neoptolemos JP (2004). "Clinical and genetic characteristics of hereditary pancreatitis in Europe". Clin. Gastroenterol. Hepatol. 2 (3): 252–61. PMID 15017610.
- ↑ 2.0 2.1 Rebours V, Boutron-Ruault MC, Schnee M, Férec C, Le Maréchal C, Hentic O, Maire F, Hammel P, Ruszniewski P, Lévy P (2009). "The natural history of hereditary pancreatitis: a national series". Gut. 58 (1): 97–103. doi:10.1136/gut.2008.149179. PMID 18755888.
- ↑ Sossenheimer MJ, Aston CE, Preston RA, Gates LK, Ulrich CD, Martin SP, Zhang Y, Gorry MC, Ehrlich GD, Whitcomb DC (1997). "Clinical characteristics of hereditary pancreatitis in a large family, based on high-risk haplotype. The Midwest Multicenter Pancreatic Study Group (MMPSG)". Am. J. Gastroenterol. 92 (7): 1113–6. PMID 9219780.
- ↑ Whitcomb DC (2000). "Genetic predispositions to acute and chronic pancreatitis". Med. Clin. North Am. 84 (3): 531–47, vii. PMID 10872412.
- ↑ Sibert JR (1978). "Hereditary pancreatitis in England and Wales". J. Med. Genet. 15 (3): 189–201. PMC 1013676. PMID 671483.
- ↑ Ammann RW, Akovbiantz A, Largiader F, Schueler G (1984). "Course and outcome of chronic pancreatitis. Longitudinal study of a mixed medical-surgical series of 245 patients". Gastroenterology. 86 (5 Pt 1): 820–8. PMID 6706066.
- ↑ Lankisch PG, Seidensticker F, Löhr-Happe A, Otto J, Creutzfeldt W (1995). "The course of pain is the same in alcohol- and nonalcohol-induced chronic pancreatitis". Pancreas. 10 (4): 338–41. PMID 7792289.
- ↑ Warshaw AL (1984). "Pain in chronic pancreatitis. Patients, patience, and the impatient surgeon". Gastroenterology. 86 (5 Pt 1): 987–9. PMID 6706079.
- ↑ Layer P, Yamamoto H, Kalthoff L, Clain JE, Bakken LJ, DiMagno EP (1994). "The different courses of early- and late-onset idiopathic and alcoholic chronic pancreatitis". Gastroenterology. 107 (5): 1481–7. PMID 7926511.
- ↑ 10.0 10.1 DiMagno EP, Go VL, Summerskill WH (1973). "Relations between pancreatic enzyme outputs and malabsorption in severe pancreatic insufficiency". N. Engl. J. Med. 288 (16): 813–5. doi:10.1056/NEJM197304192881603. PMID 4693931.
- ↑ 11.0 11.1 Mergener K, Baillie J (1997). "Chronic pancreatitis". Lancet. 350 (9088): 1379–85. doi:10.1016/S0140-6736(97)07332-7. PMID 9365465.
- ↑ Toskes PP, Hansell J, Cerda J, Deren JJ (1971). "Vitamin B 12 malabsorption in chronic pancreatic insufficiency". N. Engl. J. Med. 284 (12): 627–32. doi:10.1056/NEJM197103252841202. PMID 5547614.
- ↑ Malka D, Hammel P, Sauvanet A, Rufat P, O'Toole D, Bardet P, Belghiti J, Bernades P, Ruszniewski P, Lévy P (2000). "Risk factors for diabetes mellitus in chronic pancreatitis". Gastroenterology. 119 (5): 1324–32. PMID 11054391.
- ↑ Cui Y, Andersen DK (2011). "Pancreatogenic diabetes: special considerations for management". Pancreatology. 11 (3): 279–94. doi:10.1159/000329188. PMID 21757968.
- ↑ 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.
- ↑ Whitcomb DC, Applebaum S, Martin SP (1999). "Hereditary pancreatitis and pancreatic carcinoma". Ann. N. Y. Acad. Sci. 880: 201–9. PMID 10415865.