Pediatric Caroli Disease Medication

  • Author: Joshua R Friedman, MD, PhD; Chief Editor: Carmen Cuffari, MD   more...
 
Updated: Apr 3, 2012
 

Medication Summary

Ursodiol can promote the dissolution of intrahepatic stones and promote bile flow in Caroli disease or Caroli syndrome. Broad-spectrum antibiotics are used in the treatment of cholangitis associated with Caroli disease or Caroli syndrome.

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Gallstone dissolution agents

Class Summary

These agents are used to prevent and possibly to dissolve gallbladder stones. They enhance bile salt–dependent biliary flow. They also act as choleretic agents and may prove to be a valuable addition to therapy in repeated and refractory cholangitis.

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Ursodiol (Urso, Actigall)

 

Also called ursodeoxycholic acid. Naturally occurring bile used to dissolve radiolucent gallstones. Suppresses hepatic cholesterol synthesis, cholesterol secretion, and inhibits cholesterol intestinal absorption. Little inhibitory effect on synthesis and secretion into bile of endogenous bile acids and does not appear to affect phospholipid secretion into bile. Alters bile composition from supersaturated to unsaturated. Ursodiol-rich bile solubilizes cholesterol by increasing the concentration level at which cholesterol saturation occurs. Promotes bile flow in cholestatic conditions associated with patent extrahepatic biliary system.

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Antibiotic agents

Class Summary

Broad-spectrum antibiotics are used to treat cholangitis. Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting.

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Ampicillin and sulbactam (Unasyn)

 

Drug combination of beta-lactamase inhibitor with ampicillin. Used to treat infections involving skin, enteric flora, and anaerobes. Provides broad gram-positive and anaerobic coverage. Should be combined with an agent with gram-negative coverage, such as aminoglycoside.

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Gentamicin (Garamycin)

 

Used to treat cholangitis. Aminoglycoside antibiotic for gram-negative coverage. Used in combination with an agent against gram-positive organisms and one that covers anaerobes.

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Contributor Information and Disclosures
Author

Joshua R Friedman, MD, PhD  Assistant Professor of Pediatrics, University of Pennsylvania School of Medicine; Attending Physician, Division of Gastroenterology, Hepatology, and Nutrition, Children's Hospital of Philadelphia

Joshua R Friedman, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association for the Study of Liver Diseases, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Pfizer, Inc. Ownership interest None; Johnson & Johnson Ownership interest None

Coauthor(s)

David A Piccoli, MD  Chief of Pediatric Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia; Professor, University of Pennsylvania School of Medicine

David A Piccoli, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Robert Baldassano, MD  Director, Center for Pediatric Inflammatory Bowel Disease, Children's Hospital of Philadelphia; Professor, Department of Pediatrics, Division of Gastroenterology and Nutrition, University of Pennsylvania School of Medicine

Robert Baldassano, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Abbott, Inc Consulting fee Consulting

Melissa Crawford Kennedy, MD  Fellow, Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Children's Hospital of Philadelphia

Disclosure: Nothing to disclose.

Specialty Editor Board

Robert Baldassano, MD  Director, Center for Pediatric Inflammatory Bowel Disease, Children's Hospital of Philadelphia; Professor, Department of Pediatrics, Division of Gastroenterology and Nutrition, University of Pennsylvania School of Medicine

Robert Baldassano, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Abbott, Inc Consulting fee Consulting

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.

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.

Steven M Schwarz, MD, FAAP, FACN, AGAF  Professor of Pediatrics, Children's Hospital at Downstate, State University of New York 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: 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.

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Hepatic ultrasonogram of a neonate with Caroli disease. Multiple dilated intrahepatic bile ducts are present. Courtesy of Richard Bellah, MD, The Children's Hospital of Philadelphia.
 
 
 
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