Pediatric Caroli Disease Medication

Updated: Oct 20, 2017
  • Author: Mutaz I Sultan, MBChB, MD; Chief Editor: Carmen Cuffari, MD  more...
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Medication

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.

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.

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.

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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