Advertisement

European Journal of Pediatrics

, Volume 139, Issue 3, pp 203–205 | Cite as

Latent chronic cholangitis in congenital hepatic fibrosis

  • F. Alvarez
  • M. Hadchouel
  • O. Bernard
Case Reports

Abstract

In a five-year-old girl with congenital hepatic fibrosis raised erythrocyte sedimentation rate and serum gammaglobulins were noted for 3 years. Needle liver biopsy showed histological features of cholangitis andKlebsiella oxytoca was cultured from a liver biopsy specimen. Antibiotic treatment resulted in normalization of erythrocyte sedimentation rate and serum gammaglobulins. This suggest that cholangitis can be clinically latent in congenital hepatic fibrosis and should be investigated and treated when unexplained inflammatory signs are present.

Key words

Cholangitis Child Liver disease 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Alvarez F, Bernard O, Brunelle F, Hadchouel M, Leblanc A Odièvre M, Alagille D (1981) Congenital hepatic fibrosis in children. J Pediatr 99:370–375PubMedGoogle Scholar
  2. 2.
    Auvert J, Weisgerber G (1973) La fibroadénomatose biliaire intrahépatique. Med Chir Dig 2:363–373PubMedGoogle Scholar
  3. 3.
    Blyth H, Ockenden G (1971) Polycystic disease of kidney and liver presenting in childhood. J Med Genet 8:257–284PubMedGoogle Scholar
  4. 4.
    Dusol M, Levi JU, Glasser K, Schiff ER (1976) Congenital hepatic fibrosis with dilatation of intrahepatic bile ducts. A therapeutic approach. Gastroenterology 71:839–843PubMedGoogle Scholar
  5. 5.
    Erlinger S, Sakellaridis DA, Maillard JN, Benhamou JP (1969) Les formes angiocholitiques de la fibrose hépatique congénitale. Presse Méd 77:1189–1191Google Scholar
  6. 6.
    Keighley MRB (1977) Micro-organism in the bile. A preventable cause of sepsis after biliary surgery. Ann R Coll Surg Engl 59:328–334PubMedGoogle Scholar
  7. 7.
    Kerr DNS, Harrison CV, Sherlock S, Milnes Walker R (1961) Congenital hepatic fibrosis. Q J Med 30:91–117PubMedGoogle Scholar
  8. 8.
    Kerr DNS, Okonkwo S, Choa RG (1978) Congenital hepatic fibrosis: The long term prognosis. Gut 19:514–520PubMedGoogle Scholar
  9. 9.
    Lieberman E, Salinas-Madrigal L, Gwinn J, Brennan L, Fine R, Landing BV (1971) Infantile polycystic disease of the kidneys and liver. Clinical, pathological and radiological correlations and comparison with congenital hepatic fibrosis. Medicine 50: 277–318PubMedGoogle Scholar
  10. 10.
    Murray Lyon I, Shilkin K, Laws J, Illing R, Williams R (1971) Non-obstructive dilatation of the intrahepatic biliary tree with cholangitis. Q J Med 41:477–489Google Scholar
  11. 11.
    Odièvre M, Chaumont P, Montagne J Ph, Alagille D (1977) Anomalies of the intrahepatic portal venous system in congenital hepatic fibrosis. Radiology 122: 427–430PubMedGoogle Scholar
  12. 12.
    Rogers Ch, Isenberg JN, Leonard A, Sharp H (1976) Ascending cholangitis diagnosed by percutaneous hepatic aspiration. J Pediatr 88:83–86PubMedGoogle Scholar
  13. 13.
    Waldram R, Vahrman J, Williams R (1975) Salmonella heildeberg infection in Caroli's syndrome. Gastroenterology 68: 151–153PubMedGoogle Scholar
  14. 14.
    Williams R, Scheuer P, Heard B (1964) Congenital hepatic fibrosis with an unusual pulmonary lesion. J Clin Pathol 17:135–142PubMedGoogle Scholar

Copyright information

© Springer-Verlag 1982

Authors and Affiliations

  • F. Alvarez
    • 1
    • 2
  • M. Hadchouel
    • 1
    • 2
  • O. Bernard
    • 1
    • 2
  1. 1.Unité de Recherche d'Hépatologie InfantileI.N.S.E.R.M.U 56BicetreFrance
  2. 2.Clinique de PédiatrieUniversité Paris-Sud, Hôpital d'EnfantsBicetreFrance

Personalised recommendations