Congenital Hepatic Fibrosis Differential Diagnoses

  • Author: Hisham Nazer, MB, BCh, FRCP, DCh, DTM&H; Chief Editor: Carmen Cuffari, MD   more...
 
Updated: Mar 8, 2010
 
 

Differential Diagnoses

Proceed to Workup
 
 
Contributor Information and Disclosures
Author

Hisham Nazer, MB, BCh, FRCP, DCh, DTM&H  Professor of Pediatrics, Consultant in Pediatric Gastroenterology, Hepatology and Clinical Nutrition, Bushnaq Medical Centre, University of Jordan

Hisham Nazer, MB, BCh, FRCP, DCh, DTM&H is a member of the following medical societies: Royal College of Paediatrics and Child Health, Royal College of Physicians, Royal College of Surgeons in Ireland, Royal College of Surgeons of Edinburgh, and Royal Society of Tropical Medicine and Hygiene

Disclosure: Nothing to disclose.

Coauthor(s)

Dena Nazer, MD  Medical Director, Child Protection Center, Children's Hospital of Michigan; Assistant Professor, Wayne State University

Dena Nazer, MD is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, American Professional Society on the Abuse of Children, and Helfer Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Chris A Liacouras, MD  Director of Pediatric Endoscopy, Department of Pediatrics, Division of Gastroenterology and Nutrition, Associate Professor, Children's Hospital of Philadelphia and University of Pennsylvania

Chris A Liacouras, MD is a member of the following medical societies: American Gastroenterological Association

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Steven M Schwarz, MD, FAAP, FACN, AGAF  Professor of Pediatrics, Children's Hospital at Downstate, SUNY-Downstate Medical Center

Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research

Disclosure: TAP Pharmaceuticals Honoraria Speaking and teaching; Curemark, LLC Consulting fee Board membership; Centocor, Inc. Grant/research funds Independent contractor; Johnson & Johnson, Inc. Grant/research funds Independent contractor

Chief Editor

Carmen Cuffari, MD  Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

References

  1. Lipschitz B, Berdon WE, Defelice AR, Levy J. Association of congenital hepatic fibrosis with autosomal dominant polycystic kidney disease. Report of a family with review of literature. Pediatr Radiol. 1993;23(2):131-3. [Medline].

  2. Bristowe F. Cystic disease of the liver associated with similar disease of the kidney. Trans Pathol Soc Lond. 1856;5:229.

  3. Kerr DN, Harrison CV, Sherlock S, Walker RM. Congenital hepatic fibrosis. Q J Med. Jan 1961;30:91-117. [Medline].

  4. Akhan O, Karaosmanoglu AD, Ergen B. Imaging findings in congenital hepatic fibrosis. Eur J Radiol. Jan 2007;61(1):18-24. [Medline].

  5. Desmet VJ. Congenital diseases of intrahepatic bile ducts: variations on the theme "ductal plate malformation". Hepatology. Oct 1992;16(4):1069-83. [Medline].

  6. Harris PC, Rossetti S. Molecular genetics of autosomal recessive polycystic kidney disease. Mol Genet Metab. Feb 2004;81(2):75-85. [Medline].

  7. Losekoot M, Haarloo C, Ruivenkamp C, et al. Analysis of missense variants in the PKHD1-gene in patients with autosomal recessive polycystic kidney disease (ARPKD). Hum Genet. Nov 2005;118(2):185-206. [Medline].

  8. Turkbey B, Ocak I, Daryanani K, Font-Montgomery E, Lukose L, Bryant J. Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF). Pediatr Radiol. Feb 2009;39(2):100-11. [Medline].

  9. El-Youssef M, Mu Y, Huang L, et al. Increased expression of transforming growth factor-beta1 and thrombospondin-1 in congenital hepatic fibrosis: possible role of the hepatic stellate cell. J Pediatr Gastroenterol Nutr. Apr 1999;28(4):386-92. [Medline].

  10. Ozaki S, Sato Y, Yasoshima M, et al. Diffuse expression of heparan sulfate proteoglycan and connective tissue growth factor in fibrous septa with many mast cells relate to unresolving hepatic fibrosis of congenital hepatic fibrosis. Liver Int. Aug 2005;25(4):817-28. [Medline].

  11. Abdullah AM, Nazer H, Atiyeh M, Ali MA. Congenital hepatic fibrosis in Saudi Arabia. J Trop Pediatr. Oct 1991;37(5):240-3. [Medline].

  12. Arikan C, Ozgenc F, Akman SA, et al. Impact of liver transplantation on renal function of patients with congenital hepatic fibrosis associated with autosomal recessive polycystic kidney disease. Pediatr Transplant. Dec 2004;8(6):558-60. [Medline].

  13. Dagli U, Atalay F, Sasmaz N, et al. Caroli's disease: 1977-1995 experiences. Eur J Gastroenterol Hepatol. Feb 1998;10(2):109-12. [Medline].

  14. Al-Bhalal L, Akhtar M. Molecular basis of autosomal recessive polycystic kidney disease (ARPKD). Adv Anat Pathol. Jan 2008;15(1):54-8. [Medline].

  15. Ananthakrishnan AN, Saeian K. Caroli's disease: identification and treatment strategy. Curr Gastroenterol Rep. Apr 2007;9(2):151-5. [Medline].

  16. Braga AC, Calheno A, Rocha H, Lourenco-Gomes J. Caroli's disease with congenital hepatic fibrosis and medullary sponge kidney. J Pediatr Gastroenterol Nutr. Nov 1994;19(4):464-7. [Medline].

  17. Brancatelli G, Federle MP, Vilgrain V, et al. Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. May-Jun 2005;25(3):659-70. [Medline].

  18. Gocmen R, Akhan O, Talim B. Congenital absence of the portal vein associated with congenital hepatic fibrosis. Pediatr Radiol. Sep 2007;37(9):920-4. [Medline].

  19. Ling SC. Congenital cholestatic syndromes: what happens when children grow up?. Can J Gastroenterol. Nov 2007;21(11):743-51. [Medline].

  20. McEvoy CF, Suchy FJ. Biliary tract disease in children. Pediatr Clin North Am. Feb 1996;43(1):75-98. [Medline].

  21. Miller WJ, Sechtin AG, Campbell WL, Pieters PC. Imaging findings in Caroli's disease. AJR Am J Roentgenol. Aug 1995;165(2):333-7. [Medline].

  22. Suchy FJ. Liver disease in children. In: Mosby Yearbook. St Louis, MO: Mosby Year Book Inc; 1994.

  23. Yonem O, Ozkayar N, Balkanci F, et al. Is congenital hepatic fibrosis a pure liver disease?. Am J Gastroenterol. Jun 2006;101(6):1253-9. [Medline].

 
Previous
Next
 
Histopathology of liver biopsy in congenital hepatic fibrosis, which shows a widened portal tract with bands of fibrous tissue that separate areas of normal hepatic parenchyma. Note the multiple irregularly shaped narrow and elongated bile ducts and the absent lobular and portal inflammation.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.