Cholbam (cholic acid)
Last reviewed · Source: First Databank (FDB) drug data
Overview
Cholic acid, a primary bile acid, is used as replacement therapy.
Cholic acid is a primary bile acid synthesized from cholesterol in the liver. Endogenous primary bile acids (i.e., cholic acid, chenodeoxycholic acid) improve the flow of bile and provide physiologic feedback inhibition of bile acid synthesis. The mechanism of action of cholic acid has not been fully established.
Cholic acid and its conjugates are endogenous ligands of the nuclear farnesoid X receptor (FXR), which regulates enzymes and transporters involved in bile acid synthesis and enterohepatic circulation to maintain bile acid homeostasis under normal physiologic conditions. Activation of the FXR through the binding of cholic acid results in the upregulation of the transcription of genes that code for hepatic conjugation enzymes, resulting in increased metabolic conjugation of bile acids and bile acid-dependent bile flow. The activated FXR also results in downregulation of cholesterol 7 alpha-hydroxylase encoded by the CYP7A1 gene, potentially resulting in a reduction in the de novo synthesis of primary bile acids; however, the clinical importance of such downregulation is not fully known.
Orally administered cholic acid is absorbed by passive diffusion along the length of the GI tract. Exogenously administered cholic acid is subject to the same metabolic pathway as endogenous cholic acid and undergoes conjugation with glycine or taurine in the liver to form bile salts. Cholic acid, mainly in conjugated forms, undergoes enterohepatic circulation.
Any conjugated cholic acid that is not absorbed in the colon is deconjugated and dehydroxylated to form cholic acid and deoxycholic acid, which may be reabsorbed in the colon or excreted in the feces. The elimination half life of cholic acid in healthy adults is approximately 3 hours.
Generic name: cholic acid
Reference product & biosimilars
No reference, biosimilar or interchangeable associated with this drug.
Today’s intelligence on Cholbam
Indications
The following indications are listed for Cholbam:
- Deficiency Of Bile Acids Due To Peroxisomal Disorder
- Deficiency Of Bile Acids In Bile Acid Synthesis Disorder
Also known as: Bile Acid Deficiency In Bile Acid Synthesis Disorder Due To Single Enzyme Defect, Deficiency Of Bile Acids Due To Basd, Deficiency Of Bile Acids Due To Pd.
Dosing
The recommended dosage of cholic acid for the management of adult and pediatric patients with bile acid synthesis disorders due to single enzyme defects or the adjunctive management of adult and pediatric patients with bile acid synthesis disorders due to peroxisomal disorders is 10-15 mg/kg daily given in 1 or 2 divided doses.
The recommended dosage of cholic acid for the management of bile acid synthesis disorders due to single enzyme defects or the adjunctive management of bile acid synthesis disorders due to peroxisomal disorders and concomitant familial hypertriglyceridemia is 11-17 mg/kg daily given in 1 or 2 divided doses. Patients with newly diagnosed familial hypertriglyceridemia or a family history of the condition may have poor absorption of cholic acid from the intestine and require a 10% increase in the usual recommended dosage to account for losses due to malabsorption. To assess whether the dosage is sufficient in such patients, the clinical response (e.g., presence of steatorrhea) and laboratory values (e.g., AST, ALT, and bilirubin concentrations; prothrombin time (PT)/INR) should be monitored.
The calculated dosage should be rounded to the nearest whole capsule strength available using the 50- or 250-mg capsules either alone or in combination. For more detailed information on selection of capsule strengths to provide a specific dosage of this drug, the dosage tables in the manufacturer's labeling should be consulted.
The lowest dosage required to effectively maintain liver function should be administered. Treatment with cholic acid should be discontinued if liver function does not improve within 3 months of initiation of therapy.
Cholic acid is administered orally with food. Capsules should be swallowed whole and should not be chewed or crushed. Doses should be taken at least 1 hour before or 4-6 hours (or as long an interval as possible) after administration of an aluminum-containing antacid or bile acid sequestrant. (See Drug Interactions: Aluminum-containing Antacids and Drug Interactions: Bile Acid Sequestrants.)
For patients unable to swallow the capsules whole, the capsules may be twisted open gently and the entire contents mixed with 15-30 mL of either infant formula or breast milk for younger children or soft foods (e.g., mashed potatoes, applesauce) for older children to mask any unpleasant taste. The mixture should be stirred for 30 seconds and administered immediately thereafter. The capsule contents will remain as fine granules in the mixture and will not dissolve.
No dosing information is available for this tab.
No dosing information is available for this tab.
Drug interactions
There is no enhanced messaging for this drug.
There are no contraindication interactions for this drug.
There are no severe interactions for this drug.
There are no moderate interactions for this drug.
Contraindications
The manufacturer states there are no known contraindications to the use of cholic acid.
There are no contraindication contraindications for this drug.
There are no severe contraindications for this drug.
There are no moderate contraindications for this drug.
Adverse reactions
Adverse effects reported in clinical studies in 1% or more of patients receiving cholic acid include diarrhea, reflux esophagitis, malaise, jaundice, skin lesion, nausea, abdominal pain, intestinal polyp, urinary tract infection, and peripheral neuropathy.
There are no severe adverse reactions for this drug.
There are no less severe adverse reactions for this drug.
Precautions
No enhanced messaging available.
Safety and efficacy of cholic acid have not been established in infants younger than 3 weeks of age.
There are no studies assessing associated risk of cholic acid therapy in pregnant women or animals. In a study of 15 patients with bile acid synthesis disorders due to single enzyme defects treated with cholic acid for a median of 12 years, 4 normal pregnancies in 2 patients resulted in the birth of 4 healthy infants; however, these limited data may not adequately predict the presence or absence of risk during pregnancy.
A pregnancy surveillance program (COCOA Registry (Cholbam(R): Child and Mother's Health)) that monitors pregnancy outcomes in women exposed to cholic acid during pregnancy has been established. Women who become pregnant while exposed to cholic acid during pregnancy are encouraged to enroll themselves in the pregnancy registry, or their clinicians may enroll them in the registry by calling 844-202-6262.
Endogenous cholic acid is distributed into human milk; there are no data in nursing women or lactating animals to determine whether exogenously administered cholic acid is distributed into milk. The effects of cholic acid on nursing infants or on milk production are unknown. The benefits of breast-feeding and the importance of cholic acid to the woman should be considered along with the potential adverse effects on the breast-fed child from the drug or the underlying maternal condition.
Clinical trials of cholic acid did not include sufficient numbers of patients 65 years of age or older to determine whether they respond differently than younger adults.
ICD codes
No ICD codes are available for this drug.
Formulary status
Search health-plan coverage for this drug by ZIP code.
Choose the first character of a health plan name.
Last reviewed · Source: First Databank (FDB) drug data
On this page
Actions
Sign in to save favorites
Saving this drug requires a free Physicians Office Resource account.