Miscellaneous gastrohepatic disorders:
Indications for: CHOLBAM
Bile acid synthesis due to single enzyme defects (SEDs). Adjunct in peroxisomal disorders (PDs) including Zellweger spectrum disorders in patients who exhibit manifestations of liver disease, steatorrhea or complications from decreased fat soluble vitamin absorption.
Limitations of Use:
Safety and efficacy on extrahepatic manifestations of bile acid synthesis disorders due to SEDs or PDs not established.
Adults and Children:
<3wks: not established. Take with food. Swallow whole. If unable to swallow caps, can open and mix contents with infant formula/breast milk or soft foods. ≥3wks: 10–15mg/kg once daily or in 2 divided doses. Concomitant familial hypertriglyceridemia: 11–17mg/kg once daily or in 2 divided doses.
Exacerbation of hepatic impairment. Discontinue if liver function does not improve within 3 months of treatment initiation, complete biliary obstruction develops, or if persistent clinical or lab indicators of worsening liver function or cholestasis present; monitor and consider restarting at lower dose after return to baseline. Monitor AST, ALT, GGT, ALP, bilirubin, INR monthly for the first 3 months, every 3 months for the next 9 months, every 6 months for the next 3 years, and yearly thereafter; more frequently during rapid growth, concomitant disease, pregnancy. Use lowest effective dose that maintains liver function. Elderly. Pregnancy. Nursing mothers.
Avoid concomitant bile salt efflux pump inhibitors (eg, cyclosporine); if concomitant use needed, monitor serum transaminases and bilirubin. Antagonized by bile acid binding resins (eg, cholestyramine, colestipol, colesevelam) or aluminum-based antacids. Separate cholic acid dosing by ≥1hr before or 4–6hrs after bile acid resins or aluminum-based antacids.
Diarrhea, reflux esophagitis, malaise, jaundice, skin lesion, nausea, abdominal pain, intestinal polyp, UTI, peripheral neuropathy.
Generic Drug Availability: