Congenital Hepatic Fibrosis Follow-up

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

Further Inpatient Care

  • Recurrent episodes of GI bleeding, recurrent cholangitis, and the extent of renal impairment largely influence the course of the disease.
  • Congenital hepatic fibrosis (CHF) is also associated with varied clinical conditions that require consultations, resulting in further inpatient care and management (see Consultations).
  • With severe bleeding varices, the child may require admission to the intensive care unit.
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Further Outpatient Care

  • Patients with congenital hepatic fibrosis are usually seen regularly at pediatric gastroenterology, hepatology, and nephrology clinics.
  • In complicated cases, other disciplines are involved for regular follow-up assessment, including pediatric infectious disease, vascular surgery, and transplant surgery.
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Inpatient & Outpatient Medications

  • No specific medication is available for congenital hepatic fibrosis.
  • Medication therapy is usually directed at treatment of complications, such as recurrent cholangitis, sepsis, or renal impairment.
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Transfer

  • A pediatric gastroenterologist or hepatologist usually provides follow-up care to the child with CHF, in collaboration with a pediatric nephrologist in cases with renal involvement (eg, ARPKD).
  • Transfer to other services is indicated only in the presence of complications, especially cholangitis, and particularly with recurrent cholangitis that does not adequately respond to medical management.
  • In complicated cases, transfer to a tertiary care center is recommended to facilitate the consultation and contribution of other services, such as pediatric surgery, vascular surgery, and transplant surgery.
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Complications

  • Complications in congenital hepatic fibrosis are mainly related to its association with autosomal recessive polycystic kidney disease (ARPKD), resulting in renal impairment, bleeding varices, and recurrent cholangitis.
  • Recognition of cholangitis and prevention of its recurrence by appropriate surgical procedures are important. Transhepatic cholangiography is a safe and direct means of identifying this entity.
  • Cholangiocarcinoma and amyloidosis have been reported as late sequelae of congenital hepatic fibrosis.
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Prognosis

  • Most patients do well. If bleeding from varices can be controlled and renal failure does not occur, the prognosis in congenital hepatic fibrosis is expected to be favorable. Respiratory insufficiency in the first month of life and renal insufficiency are primary determinant factors of mortality.
  • As many as 25% of patients may eventually succumb to renal failure.
  • Renal involvement in neonates and young infants with congenital hepatic fibrosis carries a worse prognosis, with most patients dying of renal failure within the first year of life.
  • Other major causes of death include sepsis with ascending cholangitis and hepatic failure.
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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

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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.
 
 
 
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