Cholic acid

What is Cholic acid?

Cholic acid (CHOLBAM) is a bile acid used as a treatment for children and adults with bile acid synthesis disorders due to single enzyme defects, and for peroxisomal disorders (such as Zellweger syndrome).

File:Cholic acid.png

What are the uses of this medicine?

This medicine is used for:

• Treatment of bile acid synthesis disorders due to single enzyme defects (SEDs).
• Adjunctive treatment of peroxisomal disorders (PDs) including Zellweger spectrum disorders in patients who exhibit manifestations of liver disease, steatorrhea or complications from decreased fat-soluble vitamin absorption.

How does this medicine work?

• Cholic acid is a primary bile acid synthesized from cholesterol in the liver.
• In bile acid synthesis disorders due to SEDs in the biosynthetic pathway, and in PDs including Zellweger spectrum disorders, deficiency of primary bile acids leads to unregulated accumulation of intermediate bile acids and cholestasis.
• Bile acids facilitate fat digestion and absorption by forming mixed micelles and facilitate absorption of fat-soluble vitamins in the intestine.
• Endogenous bile acids including cholic acid enhance bile flow and provide the physiologic feedback inhibition of bile acid synthesis.
• The mechanism of action of cholic acid has not been fully established; however, it is known that cholic acid and its conjugates are endogenous ligands of the nuclear receptor, farnesoid X receptor (FXR).
• FXR regulates enzymes and transporters that are involved in bile acid synthesis and in the enterohepatic circulation to maintain bile acid homeostasis under normal physiologic conditions.

Who Should Not Use this medicine ?

Limitations of use:

• The safety and effectiveness of CHOLBAM on extrahepatic manifestations of bile acid synthesis disorders due to SEDs or PDs including Zellweger spectrum disorders have not been established.

What drug interactions can this medicine cause?

• Avoid concomitant use of inhibitors of the bile salt efflux pump (BSEP) such as cyclosporine.
• Take CHOLBAM at least 1 hour before or 4 to 6 hours (or at as great an interval as possible) after a bile acid binding resin.
• Take CHOLBAM at least 1 hour before or 4 to 6 hours (or at as great an interval as possible) after an aluminum-based antacid.

Is this medicine FDA approved?

• It was approved for use in the United States in 2015.

How should this medicine be used?

Recommended Dosage:

• The recommended dosage of CHOLBAM is 10 to 15 mg/kg administered orally once daily, or in two divided doses, in pediatric patients and in adults.
• The recommended dosage in patients with concomitant familial hypertriglyceridemia is 11 to 17 mg/kg once daily or in two divided doses and is adjusted based on clinical response.
• Monitor AST, ALT, GGT, alkaline phosphatase, bilirubin and INR every month for the first 3 months, every 3 months for the next 9 months, every 6 months during the next three years and annually thereafter. Administer the lowest dose that effectively maintains liver function.
• Discontinue CHOLBAM if liver function does not improve within 3 months of starting treatment, if complete biliary obstruction develops, or if there are persistent clinical or laboratory indicators of worsening liver function or cholestasis; continue to monitor liver function and consider restarting at a lower dose when parameters return to baseline.

Administration

• Take CHOLBAM with food.
• Take CHOLBAM at least 1 hour before or 4 to 6 hours (or at as great an interval as possible) after a bile acid binding resin or aluminum-based antacid.
• Do not crush or chew the capsules.
• For patients unable to swallow the capsules, open the capsules and mix the contents with infant formula or expressed breast milk (for younger children), or soft food such as mashed potatoes or apple puree (for older children and adults) in order to mask any unpleasant taste:
• Hold the capsule over the prepared liquid/food, gently twist open, and allow the contents to fall into the liquid/food.
• Mix the entire capsule contents with one or two tablespoons (15 mL to 30 mL) of infant formula, expressed breast milk, or soft food such as mashed potatoes or apple puree.
• Stir for 30 seconds.
• The capsule contents will remain as fine granules in the milk or food and will not dissolve.
• Administer the mixture immediately

What are the dosage forms and brand names of this medicine?

This medicine is available in fallowing doasage form:

• As Capsules: 50 mg, 250 mg

This medicine is available in fallowing brand namesː

• CHOLBAM

What side effects can this medication cause?

The most common side effects of this medicine include:

• diarrhea
• reflux esophagitis
• malaise
• jaundice
• skin lesion
• nausea
• abdominal pain
• intestinal polyp
• urinary tract infection
• peripheral neuropathy

What special precautions should I follow?

• Monitor liver function and discontinue CHOLBAM in patients who develop worsening of liver function while on treatment.
• Discontinue treatment with CHOLBAM at any time if there are clinical or laboratory indicators of worsening liver function or cholestasis.

What to do in case of emergency/overdose?

• Symptoms of overdosage may include concurrent elevations of serum GGT and ALT.

Management for overdosage:

• If an overdose is suspected, discontinue CHOLBAM and treat symptoms.
• Continue to monitor laboratory parameters of liver function and consider restarting at a lower dose when the parameters return to baseline.

Can this medicine be used in pregnancy?

• No studies in pregnant women or animal reproduction studies have been conducted with CHOLBAM.

Can this medicine be used in children?

• The safety and effectiveness of CHOLBAM have been established in pediatric patients 3 weeks of age and older.

What are the active and inactive ingredients in this medicine?

Active ingredient: CHOLIC ACID

Inactive ingredients: MAGNESIUM STEARATE CELLULOSE, MICROCRYSTALLINE

Who manufactures and distributes this medicine?

Manufactured by: Patheon France SA 38300 Bourgoin-Jallieu, France

Manufactured for: Manchester Pharmaceuticals, Inc. a wholly owned subsidiary of Retrophin, Inc San Diego

What should I know about storage and disposal of this medication?

• Store at 20°C–25°C (69°F-77°F), excursions permitted between 15°C-30°C (59°F-86°F).

• Dailymed label info on Cholic acid
• FDA Cholic acid

Cholic acid Resources
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