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. 1975 Feb;16(2):81–88. doi: 10.1136/gut.16.2.81

A British family with herediatary pancreatitis.

J R Sibert
PMCID: PMC1410989  PMID: 1079196

Abstract

A family with hereditary pancreatitis is described. Nine family members definitely have had pancreatitis, whilst 15 more are suspected of having the disease. The condition presents as recurrent attacks of epigastric or central abdominal pain, sometimes radiating to the back, often associated with vomiting. The attacks of pain usually last three to four days. The inheritance fits well with an autosomal dominant pattern with limited penetrance, as it does in other families described in the literature. There is no aminoaciduria as has been described in some previously reported families. The attacks of pain start in childhood or young adult life (mean age of onset inthis family is 12-6 years) and appear to cease in this family by the age of 40 years. The diagnosis of pancreatitis in members of the family who have had confirmed pancreatitis was made by finding a raised serum amylase concentration in four cases, at laparotomy in four cases, and by pancreatic calcification seen on radiography in one case, The literature on the condition is reviewed, and it is speculated that the condition may have been underdiagnosed in Britain.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Adham N. F., Dyce B., Haverback B. J. Elevated serum trypsin binding activity in patients with hereditary pancreatitis. Am J Dig Dis. 1968 Jan;13(1):8–15. doi: 10.1007/BF02239206. [DOI] [PubMed] [Google Scholar]
  2. COMFORT M. W., STEINBERG A. G. Pedigree of a family with hereditary chronic relapsing pancreatitis. Gastroenterology. 1952 May;21(1):54–63. [PubMed] [Google Scholar]
  3. CORNET E., DUPON H., HARDY M., GORDEEF A. [Primary familial chronic pancreatitis with ectasia of the ducts (6 operated cases)]. J Chir (Paris) 1962 Dec;84:527–542. [PubMed] [Google Scholar]
  4. Carey M. C., Fitzgerald O. Hyperparathyroidism associated with chronic pancreatitis in a family. Gut. 1968 Dec;9(6):700–703. doi: 10.1136/gut.9.6.700. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Davidson P., Costanza D., Swieconek J. A., Harris J. B. Hereditary pancreatitis. A kindred without gross aminoaciduria. Ann Intern Med. 1968 Jan;68(1):88–96. doi: 10.7326/0003-4819-68-1-88. [DOI] [PubMed] [Google Scholar]
  6. GERBER B. C. Hereditary pancreatitis. The role of surgical intervention. Arch Surg. 1963 Jul;87:70–80. doi: 10.1001/archsurg.1963.01310130072010. [DOI] [PubMed] [Google Scholar]
  7. GROSS J. B., COMFORT M. W. Hereditary pancreatitis: report on two additional families. Gastroenterology. 1957 May;32(5):829–854. [PubMed] [Google Scholar]
  8. GROSS J. B., GAMBILL E. E., ULRICH J. A. Hereditary pancreatitis. Description of a fifth kindred and summary of clinical features. Am J Med. 1962 Sep;33:358–364. doi: 10.1016/0002-9343(62)90232-2. [DOI] [PubMed] [Google Scholar]
  9. GROSS J. B. Some recent developments pertaining to pancreatitis. Ann Intern Med. 1958 Oct;49(4):796–819. doi: 10.7326/0003-4819-49-4-796. [DOI] [PubMed] [Google Scholar]
  10. HENDREN W. H., GREEP J. M., PATTON A. S. PANCREATITIS IN CHILDHOOD: EXPERIENCE WITH 15 CASES. Arch Dis Child. 1965 Apr;40:132–145. doi: 10.1136/adc.40.210.132. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Kattwinkel J., Lapey A., Di Sant'Agnese P. A., Edwards W. A. Hereditary pancreatitis: three new kindreds and a critical review of the literature. Pediatrics. 1973 Jan;51(1):55–69. [PubMed] [Google Scholar]
  12. Logan A., Jr, Schlicke C. P., Manning G. B. Familial pancreatitis. Am J Surg. 1968 Jan;115(1):112–117. doi: 10.1016/0002-9610(68)90138-4. [DOI] [PubMed] [Google Scholar]
  13. McElroy R., Christiansen P. A. Hereditary pancreatitis in a kinship associated with portal vein thrombosis. Am J Med. 1972 Feb;52(2):228–241. doi: 10.1016/0002-9343(72)90072-1. [DOI] [PubMed] [Google Scholar]
  14. Nash F. W. Familial calcific pancreatitis: an acute episode with massive pleural effusion. Proc R Soc Med. 1971 Jan;64(1):17–18. [PMC free article] [PubMed] [Google Scholar]
  15. Robechek P. J. Hereditary chronic relapsing pancreatitis. A clue to pancreatitis in general? Am J Surg. 1967 Jun;113(6):819–824. doi: 10.1016/0002-9610(67)90354-6. [DOI] [PubMed] [Google Scholar]
  16. Sibert J. R. Hereditary pancreatitis in a Newcastle family. Arch Dis Child. 1973 Aug;48(8):618–621. doi: 10.1136/adc.48.8.618. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Whitten D. M., Feingold M., Eisenklam E. J. Hereditary pancreatitis. Am J Dis Child. 1968 Oct;116(4):426–428. doi: 10.1001/archpedi.1968.02100020430016. [DOI] [PubMed] [Google Scholar]

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