International Journal of Pancreatology

, Volume 4, Issue 2, pp 221–228 | Cite as

Relapsing pancreatitis as initial manifestation of cystic fibrosis in a young man without pulmonary disease

  • Volker Gross
  • Juergen Schoelmerich
  • Klaus Denzel
  • Wolfgang Gerok
Case Report


In young adults with acute pancreatitis a wide etiologic spectrum has to be considered. Among the possible causes, cystic fibrosis is rare. Besides the typical clinical triad of pancreatic exocrine insufficiency, chronic obstrue-tive pulmonary disease, and elevated sweat chloride levels, there is a wide spectrum of variants of the cystic fibrosis syndrome. Especially mild manifestations of the disease may, therefore, escape proper identification. Here we describe a young man who presented initially with recurrent acute pancreatitis without pulmonary disease and without a family history of cystic fibrosis, in whom the diagnosis of cystic fibrosis was established by slightly elevated sweat chloride levels and obstructive azoospermia. Five years after the first attack of pancreatitis the patient developed pancreatic exocrine insufficiency.

Key Words

Cystic fibrosis pancreatitis azoospermia pulmonary function sweat testing 


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  1. 1.
    Stafford RJ, Grand RJ. Hereditary disease of the exocrine panreas. Clin. Gastroenterol 1982; 11: 141–170.PubMedGoogle Scholar
  2. 2.
    Shwachman H. Cystic fibrosis. Curr. Probl. Ped. 1978; 8: 1–72.CrossRefGoogle Scholar
  3. 3.
    Shwachman H, Lucas L, Kulczycki, Kon-Taik Khaw. Studies in cystic fibrosis. A report on sixty-five patients over 17 years of age. Pediatrics 1965; 36: 689–699.PubMedGoogle Scholar
  4. 4.
    Di Sant’Agnese PA, Darling RC, Perera GA, Shea E. Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas. Pediatrics 1953; 12: 549–563.Google Scholar
  5. 5.
    Shwachman H, Leubner H, Catzell P. Mucoviscidosis. Adv. Pediat. 1955; 7: 249.Google Scholar
  6. 6.
    Shwachman H, Kowalski M, Khaw KT. Cystic fibrosis: a new outlook; 70 patients above 25 years of age. Medicine 1977; 56: 129–149.PubMedCrossRefGoogle Scholar
  7. 7.
    Shwachman H, Lebenthal E, Khaw KT. Recurrent acute pancreatitis in patients with cystic fibrosis with normal pancreatic enzymes. Pediatrics 1975; 55: 86–95.PubMedGoogle Scholar
  8. 8.
    Bluestein PK, Gaskin K, Filler R, Ho CS, Connon J. Endoscopie retrograde cholangio-pancreatography in pancreatitis in children and adolescents. Pediatrics 1981; 68: 387–393.Google Scholar
  9. 9.
    Stern R, Boat TF, Doershuk CF. Obstructive azoospermia as a diagnostic criterion for the cystic fibrosis syndrome. Lancet I 1982; 1401–1404.CrossRefGoogle Scholar
  10. 10.
    Masaryk TJ, Achkar E. Pancreatitis as initial presentation of cystic fibrosis in young adults. Dig. Dis. Sci. 1983; 28: 874–878.PubMedCrossRefGoogle Scholar
  11. 11.
    BIythe SA, Farrell PM. Advances in the diagnosis and management of cystic fibrosis. Clin. Biochem. 1984; 17: 277–283.CrossRefGoogle Scholar
  12. 12.
    Jordan SC, Ament ME. Pancreatitis in children and adolescents. Pediatrics 1977; 91: 211–216.CrossRefGoogle Scholar
  13. 13.
    Riccardi VM. Pancreatitis in children, Pediatrics 1987; 92: 685–686.Google Scholar
  14. 14.
    Hadorn B, Zoppi G, Shmerling DH, Prader A, Mclntyre I, Anderson CM. Quantitative assessment of exocrine pancreatic function in infants and children. J. Pediatr. 1968; 73: 39–50.PubMedCrossRefGoogle Scholar
  15. 15.
    Lapey A, Kattwinkel J, Di Sant’Agnese P, Laster L. Steatorrhea and azotorrhea and their relation to growth and nutrition in adolescents and young adults with cystic fibrosis. J. Pediatr. 1974; 84: 328–334.PubMedCrossRefGoogle Scholar
  16. 16.
    Johansen PG, Anderson CM, Hadorn B. Cystic fibrosis of the pancreas. A generalized disturbance of water and electrolyte movement in exocrine tissues. Lancet 1968; I: 455–457.CrossRefGoogle Scholar
  17. 17.
    Hadorn B, Johansen PG, and Anderson CM. Pancreozymin secretin test of exocrine pancreatic function of cystic fibrosis and the significance of the results for the pathogenesis of the disease. Can. Med. Assoc. J. 1968; 98: 377–382.PubMedGoogle Scholar
  18. 18.
    Wong LTK, Turtle S, Davidson AGF. Secretin pancreozymin stimulation test and confirmation of the diagnosis of cystic fibrosis. Gut 1982; 23: 744–750.PubMedCrossRefGoogle Scholar
  19. 19.
    Adelson JW. Pathophysiology of the pancreas in cystic fibrosis. J. Ped. GastroenteroL Nutr. 1984; 3 (Suppl. 1): 74–78.CrossRefGoogle Scholar
  20. 20.
    Rick W. Pancreatic exocrine functions in cystic fibrosis of the pancreas. Med. Welt. 1963; 42: 2158.Google Scholar
  21. 21.
    Cogswell JJ, Risdon RA, Taylor B. Chronic suppurative lung disease in sisters mimicking cystic fibrosis. Arch. Dis. Child 1974; 49: 520–524.PubMedGoogle Scholar
  22. 22.
    Sarsfield JK, Davies JM. Negative sweat tests and cystic fibrosis. Arch. Dis. Child 1975; 50: 463–466.PubMedCrossRefGoogle Scholar
  23. 23.
    Huff DS Huang NN, Arey JB. Atypical cystic fibrosis of the pancreas with normal levels of sweat chloride and minimal pancreatic lesions. J. Ped. 1979; 94: 237–239.CrossRefGoogle Scholar
  24. 24.
    Davis PB, Hubbard VS, Di Sant’Agnese PA. Low sweat electrolytes in a patient with cystic fibrosis. Am. J. Med. 1980; 69: 643–646.PubMedCrossRefGoogle Scholar
  25. 25.
    Shwachman H, Mahmoodian A, Neff RK. The sweat test: Sodium and chloride values. Ped. 1981; 98: 576–578.CrossRefGoogle Scholar
  26. 26.
    Davis P, de Sant’Agnese PA. Diagnosis and treatment of cystic fibrosis. An update. Chest 1984; 85: 802–809.PubMedCrossRefGoogle Scholar
  27. 27.
    Davis PB, Del Rios S, Muntz JA, Dieckman L. Sweat chlorides in adults with pulmonary disease. Am. Rev. Respir. Dis. 1983; 128: 34–37.PubMedGoogle Scholar
  28. 28.
    Canciani M, Forno S, Mastella G. Borderline sweat test: criteria for cystic fibrosis (CF) diagnosis 15th annual meeting European working group for cystic fibrosis. Oslo/Norway, 1987: 29.Google Scholar
  29. 29.
    Denning CR, Sommers SC, Quigley, Jr HJ. Infertility in male patients with cystic fibrosis. Pediat. 1968; 41: 7–17.Google Scholar
  30. 30.
    Kaplan E, Shwachman H, Perlmutter AD, Rule A, Khaw KT, Holsclaw DS. Reproductive failure in males with cystic fibrosis. N. Engl. J. Med. 1968; 279: 65–69.PubMedGoogle Scholar
  31. 31.
    Oppenheimer EH, Esterly JR. Observations on cystic fibrosis of the pancreas. V. Developmental changes in the male genital system. J. Pediatr. 1969; 75: 806–811.PubMedCrossRefGoogle Scholar

Copyright information

© Humana Press Inc. 1989

Authors and Affiliations

  • Volker Gross
    • 1
  • Juergen Schoelmerich
    • 1
  • Klaus Denzel
    • 1
  • Wolfgang Gerok
    • 1
  1. 1.Medizinische Universitaetsklinik, Albert-Ludwigs-UniversitaetFreiburgFRG

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