Medication Summary

No specific medical therapy is available for congenital hepatic fibrosis (CHF). The child's condition is usually stable, with liver enzyme levels within the reference range.

Class Summary

Antibiotic therapy is indicated for acute and recurrent cholangitis and is based essentially on the results of culture.

Sulfamethoxazole and trimethoprim (Bactrim, Septra, Cotrim)

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Reported to be an effective therapy in cholangitis that complicates CHF. Efficacy is attributed to high concentration in bile and hepatic parenchyma. Also has good in vitro activity against Enterobacteriaceae.

Class Summary

These agents enhance bile salt–dependent biliary flow. These may prove to be a valuable addition to therapy in repeated and refractory cholangitis.

Ursodiol (Actigall, Urso)

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Also called ursodeoxycholic acid. Has been shown to promote bile flow in cholestatic conditions associated with a patent extrahepatic biliary system.

Class Summary

These agents are used in medical management of portal hypertension. They reduce portal pressure through vasoconstriction of the mesenteric arterioles and reduce inflow to the portal venous system and portosystemic collaterals.

Vasopressin (Pitressin)

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Decreases portal pressure in portal hypertension through vasoconstriction of the splanchnic arterioles thus controlling hemorrhage. Coronary artery disease is a notable undesirable effect. It may dispose patients with coronary artery disease to cardiac ischemia. This may be prevented with concurrent use of nitrates.

Has vasopressor and ADH activity. Increases water resorption at distal renal tubular epithelium (ADH effect) and promotes smooth muscle contraction throughout vascular bed of renal tubular epithelium.

Glypressin, triglycyl lysine vasopressin, can also be used in a dose of up to 2 mg IV q6h.

Somatostatin (Zecnil)

Diminishes blood flow to portal system because of vasoconstriction, thus decreasing variceal bleeding. Has similar effects as vasopressin but does not cause coronary vasoconstriction.

Propranolol (Inderal)

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Beta-blocker that lowers heart rate, myocardial contractility, cardiac output, and portal hypertension, thus reducing the risk of bleeding. Additionally, prevents increases in portal pressure (hepatic venous pressure gradient) during physical exertion.

Both propranolol and nadolol, beta-blockers, are effective in preventing first bleeding and reducing the mortality rate associated with bleeding.

Follow-up

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Media Gallery

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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Author

Hisham Nazer, MBBCh, FRCP, DTM&H Professor of Pediatrics, Consultant in Pediatric Gastroenterology, Hepatology and Clinical Nutrition, University of Jordan Faculty of Medicine, Jordan

Hisham Nazer, MBBCh, FRCP, DTM&H is a member of the following medical societies: American Association for Physician Leadership, Royal College of Paediatrics and Child Health, Royal College of Surgeons in Ireland, Royal Society of Tropical Medicine and Hygiene, Royal College of Physicians and Surgeons of the United Kingdom

Disclosure: Nothing to disclose.

Coauthor(s)

Dena Nazer, MD, FAAP Assistant Professor of Pediatrics, Wayne State University School of Medicine; Chief, Child Protection Team, Children's Hospital of Michigan

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

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

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, Royal College of Physicians and Surgeons of Canada

Disclosure: Received honoraria from Prometheus Laboratories for speaking and teaching; Received honoraria from Abbott Nutritionals for speaking and teaching. for: Abbott Nutritional, Abbvie, speakers' bureau.

Additional Contributors

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

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

Disclosure: Nothing to disclose.