OVERVIEW

PRODUCT INFORMATION

CHEMICAL FORMULA AND STRUCTURE

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DRUG	CAS REGISTRY NUMBER	MOLECULAR FORMULA	STRUCTURE
Cholic Acid	81-25-4	C24-H40-O5

ANNOTATED BIBLIOGRAPHY

References updated: 07 November 2016

• Zimmerman HJ. Bile acid derivatives. Miscellaneous drugs and diagnostic chemicals. In, Zimmerman, HJ. Hepatotoxicity: the adverse effects of drugs and other chemicals on the liver. 2nd ed. Philadelphia: Lippincott, 1999: pp. 721.

  (Expert review of hepatotoxicity published in 1999 before the availability of cholic acid).
• Sharkey KA, Wallace JL. Treatment of disorders of bowel motility and water flux: anti-emetics; agents used in biliary and pancreatic disease. In, Brunton LL, Chabner BA, Knollman BC, eds. Goodman & Gilman's the pharmacological basis of therapeutics. 12th ed. New York: McGraw-Hill, 2011, pp. 1323-50.

  (Textbook of pharmacology and therapeutics).
• Daugherty CC, Setchell KD, Heubi JE, Balistreri WF. Resolution of liver biopsy alterations in three siblings with bile acid treatment of an inborn error of bile acid metabolism (delta 4-3-oxosteroid 5 beta-reductase deficiency). Hepatology 1993; 18: 1096-101. [PubMed: 8225213]

  (Among 3 siblings with bile acid synthetic defects and severe liver disease, treatment with cholic acid and ursodiol was followed by resolution of jaundice and improvements in ALT, Alk P and liver histology).
• Bove KE, Heubi JE, Balistreri WF, Setchell KD. Bile acid synthetic defects and liver disease: a comprehensive review. Pediatr Dev Pathol 2004; 7: 315-34. [PubMed: 15383928]

  (Extensive review of the inherited forms of defective bile acid synthesis which are often accompanied by progressive cholestatic liver injury, some of which respond to treatment with oral bile acids, including 3β-OH steroid dehydrogenase, 5β-reductase, and mitochondrial C-27 hydroxylase deficiencies as well as several peroxisomal defects [Zellweger syndrome]).
• Woollett LA, Buckley DD, Yao L, Jones PJ, Granholm NA, Tolley EA, Tso P, Heubi JE. Cholic acid supplementation enhances cholesterol absorption in humans. Gastroenterology 2004; 126: 724-31. [PubMed: 14988826]

  (In a crossover design study in healthy volunteers, administration of cholic acid [15 mg/kg daily] resulted in an increase in cholesterol absorption).
• Chen J, Raymond K. Nuclear receptors, bile-acid detoxification, and cholestasis. Lancet 2006; 367(9509): 454-6. [PubMed: 16473109]

  (Commentary on the nuclear receptor FXR, which acts as a bile acid receptor and mediates reduction in bile acid synthesis and increase in uptake of bile acids from the circulation as well as secretion of bile acids from hepatocytes).
• Wang Y, Jones PJ, Woollett LA, Buckley DD, Yao L, Granholm NA, Tolley EA, Heubi JE. Effects of chenodeoxycholic acid and deoxycholic acid on cholesterol absorption and metabolism in humans. Transl Res 2006; 148: 37-45. [PubMed: 16887497]

  (In two cross over studies in adults, oral chenodiol had no effect of plasma lipid concentrations while it increased biliary chenodiol, but also increased biliary cholesterol concentrations).
• Heubi JE, Setchell KD, Bove KE. Inborn errors of bile acid metabolism. Semin Liver Dis 2007; 27: 282-94. [PubMed: 17682975]

  (Review of the rare inherited conditions marked by abnormal bile acid synthesis or metabolism, the diagnosis of which generally requires mass spectrometry techniques, and many of which are treatable using oral bile acids such as cholic acid, chenodiol, ursodiol and glycocholic acid).
• Chiang JY. Bile acids: regulation of synthesis. J Lipid Res 2009; 50: 1955-66. [PMC free article: PMC2739756] [PubMed: 19346330]

  (Review of the pathways of cholic and chenodeoxycholic acid synthesis and their regulation via FXR, FGF19, FGFR4 and CYP7A1).
• Gonzales E, Gerhardt MF, Fabre M, Setchell KD, Davit-Spraul A, Vincent I, Heubi JE, et al. Oral cholic acid for hereditary defects of primary bile acid synthesis: a safe and effective long-term therapy. Gastroenterology 2009; 137: 1310-1320. [PubMed: 19622360]

  (Among 15 patients with bile acid synthesis defects treated with oral bile acids [ursodiol and cholic acid initially, and eventually cholic acid alone for all except one patient: 3-9 mg/kg daily] for 5-15 years, all had lasting clinical improvements shown by repeat liver biopsies in 14; overdose of cholic acid was marked by diarrhea, pruritus and increases in GGT and ALT that responded to dose modification).
• Heubi JE, Setchell KD, Jha P, Buckley D, Zhang W, Rosenthal P, Potter C, et al. Treatment of bile acid amidation defects with glycocholic acid. Hepatology 2015; 61: 268-74. [PMC free article: PMC4280294] [PubMed: 25163551]

  (Treatment of 5 patients with bile acid amidation defects with glycine conjugated cholic acid [glycocholic acid; 15 mg/kg daily] resulted in improved absorption of fat soluble vitamins and growth improvement in all 3 prepubertal children with delayed growth).
• In brief: Cholic acid (Cholbam) for bile acid synthesis disorders. Med Lett Drugs Ther 2016; 58 (1493): 56. [PubMed: 27101212]

  (Short review of the efficacy, safety and costs of cholic acid, shortly after its approval as an orphan drug in the therapy of bile acid synthesis disorders, mentions that its most common adverse event is diarrhea).