Skip to main content

Pediatric primary sclerosing cholangitis

Summary

An 11-year-old male presented with abdominal pain and emesis. Serum chemistries revealed cholestasis and an ERCP demonstrated sclerosing cholangitis. Secondary causes of this disease process were excluded. Sclerosing cholangitis is distinctly uncommon in the pediatric age group, but it should be considered in evaluation of patients of any age with cholestasis. It appears in some cases that there may be subtle histologic and radiographic features which separate the pediatric and adult forms of the disease.

This is a preview of subscription content, access via your institution.

References

  1. Freese D, Latimer JS, Gilberstadt S, Kane W, Sharp HL: Sclerosing cholangitis associated with inflammatory bowel disease. Clin Pediatr 21:11–15, 1982

    Google Scholar 

  2. Werlin SL, Glicklich M, Jona J, Starshak RJ: Sclerosing cholangitis in childhood. J Pediatr 96:433–435, 1980

    Google Scholar 

  3. Naveh Y, Mendelson H, Spira G, Auslaender L, Mandel H, Berant M: Primary sclerosing cholangitis associated with immunodeficiency. Am J Dis Child 137:114–117, 1983

    Google Scholar 

  4. Record CO, Eddleston ALWF, Shilkim KB, Williams R: Intrahepatic sclerosing cholangitis associated with a familial immunodeficiency syndrome. Lancet 2:18–20, 1973

    Google Scholar 

  5. Thomas IT, Ochs HD, Wedgwood RJ: Liver disease and immunodeficiency syndromes. Lancet 1:311, 1974

    Google Scholar 

  6. Spivak W, Grand RJ, Eraklis A: A case of primary sclerosing cholangitis in childhood. Gastroenterology 82:129–132, 1982

    Google Scholar 

  7. Mowat AP: Biliary disorders in childhood. Semin Liver Dis 2:271–281, 1982

    Google Scholar 

  8. Wiesner RH, LaRusso NF: Clinicopathologic features of the syndrome of primary sclerosing cholangitis. Gastroenterology 79:200–206, 1980

    Google Scholar 

  9. Chapman RWG, Marborgh BAM, Rhodes JM, Summerfield JA, Dick R, Schever PJ, Sherlock S: Primary sclerosing cholangitis: A review of its clinical features, cholangiography, and hepatic histology. Gut 21:870–877, 1980

    Google Scholar 

  10. MacCarty RL, LaRusso NF, Wiesner RH, Ludwig J: Cholangiographic and pancreatographic features of primary sclerosing cholangitis. Radiology 149:39–44, 1983

    Google Scholar 

  11. Amedee-Manesme O, Brunnelle F, Hadchovel M, Bernard O, Alagille D: Sclerosing cholangitis in infancy. Hepatology [abst] 4:786, 1984.

    Google Scholar 

  12. Lefkowitch JH: Primary sclerosing cholangitis. Arch Intern Med 142:1157–1160, 1982

    Google Scholar 

  13. Williams LF, Schoetz DJ: Primary sclerosing cholangitis. Surg Clin North Am 61:951–962, 1981

    Google Scholar 

  14. Starzl TE, Iwatsuki S, Van Thiel DH, Gartner JC, Zitelli BJ, Malatack JJ, Schade RR, Shaw BW, Hakala TR, Rosenthal JT, Porter KA: Evolution of liver transplantation. Hepatology 2:614–636, 1982

    Google Scholar 

  15. Bartholomew LG, Cain JC, Woolner LB Sclerosing cholangitis: Its possible association with Reidel's stroma and retroperitoneal fibrosis-report of two cases. N Engl J Med 269:8–12, 1963

    Google Scholar 

  16. Wegner J, Gingrich GW, Medelhoff H: Sclerosing cholangitis—a manifestation of systemic disease. Arch Intern Med 116:509–514, 1965

    Google Scholar 

  17. Denzi JT, Makipour H, Farmer RG: Primary sclerosing cholangitis: A report of nine cases and clinical review. Am J Gstroenterol 65:109–116, 1976

    Google Scholar 

  18. Hellstrom HR, Perez-Stable EC: Retroperitoneal fibrosis with disseminated vasculitis and intrahepatic sclerosing cholangitis. Am J Med 40:184–187, 1966

    Google Scholar 

  19. Thompson HH, Pitt HA, Tompkins RK, Longmire WP: Primary sclerosing cholangitis—a heterogeneous disease. Ann Surg 196 (2):127–136, 1982

    Google Scholar 

  20. Waldram R, Tsantoulas D, Kopelman RP, Williams R: Chronic pancreatitis, sclerosing cholangitis and sicca complex in two siblings. Lancet 1:550–552, 1975

    Google Scholar 

  21. Viteri AL, Hardin WJ, Dyck WP: Peyronie's disease and sclerosing cholangitis in a patient with ulcerative colitis. Dig Dis Sci 24:490–491, 1979

    Google Scholar 

  22. Schwartz SI, Dale WA: Primary sclerosing cholangitis. Arch Surg 77:439–441, 1958

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Additional information

The opinions and assertions contained herein are the private ones of the authors and are not to be construed as official or reflecting the views of the Department of Defense, Department of the Navy or the Naval Service at large, or the Uniformed Services University of the Health Sciences.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Johnson, D.A., Cattau, E.L. & Hancock, J.E. Pediatric primary sclerosing cholangitis. Digest Dis Sci 31, 773–777 (1986). https://doi.org/10.1007/BF01296457

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01296457

Keywords

  • Public Health
  • Abdominal Pain
  • Disease Process
  • Cholangitis
  • Cholestasis