Evaluating the enterohepatic cycle consisting of the biliary system, intestine, portal circulation, and hepatocytes
Supporting researchers in need of free and conjugated values of all 20 bile acid species as well as total bile acid
This is a serum test that measures all free and conjugated bile acids, including 20 individual species and total bile acids.
No interpretation is provided for this test that quantitates all free and conjugated bile acid species.
Liquid Chromatography Tandem Mass Spectrometry (LC-MS/MS)
Peroxisomal Disorder
Peroxisomal Biogenesis Disorder
Zellweger syndrome
D-bifunctional protein deficiency
Alpha methyl-CoA racemase deficiency
Cholic acid
Chenodeoxycholic acid
Deoxycholic acid
Ursodeoxycholic acid
Hyodeoxycholic acid
Lithocholic acid
Glycocholic acid
Glcyochenodeoxycholic acid
Glcyodeoxycholic acid
Glycoursodeoxycholic acid
Glycohyodeoxycholic acid
Glycolithocholic acid
Taurocholic acid
Taurochenodeoxycholic acid
Taurodeoxycholic acid
Tauroursodeoxycholic acid
Taurohyodeoxycholic acid
Taurolithodeoxycholic acid
Dihydroxycholestanoic acid
Trihydroxycholestanoic acid
Total bile acids
Serum
This test is intended for use by research scientists. Approval must be obtained before ordering.
Patient Preparation: Patient must be fasting for 12 to 14 hours.
Collection Container/Tube:
Preferred: Serum gel
Acceptable: Red top
Submission Container/Tube: Plastic vial
Specimen Volume: 0.5 mL
Collection Instructions: Centrifuge and aliquot serum into a plastic vial.
0.3 mL
Gross hemolysis | OK |
Gross lipemia | OK |
Specimen Type | Temperature | Time | Special Container |
---|---|---|---|
Serum | Refrigerated (preferred) | 90 days | |
Ambient | 90 days | ||
Frozen | 90 days |
Evaluating the enterohepatic cycle consisting of the biliary system, intestine, portal circulation, and hepatocytes
Supporting researchers in need of free and conjugated values of all 20 bile acid species as well as total bile acid
Bile acids are formed in the liver from cholesterol, conjugated primarily to glycine and taurine, stored and concentrated in the gallbladder, and secreted into the intestine after the ingestion of a meal. In the intestinal lumen, the bile acids serve to emulsify ingested fats and thereby promote digestion. During the absorptive phase of digestion, approximately 90% of the bile acids are reabsorbed.
The efficiency of the hepatic clearance of bile acids from portal blood maintains serum concentrations at low levels in normal persons. An elevated fasting level, due to impaired hepatic clearance, is a sensitive indicator of liver disease. Following meals, serum bile acid levels have been shown to increase only slightly in normal persons, but markedly in patients with various liver diseases, including cirrhosis, hepatitis, cholestasis, portal-vein thrombosis, Budd-Chiari syndrome, cholangitis, Wilson disease, and hemochromatosis. No increase in bile acids will be noted in patients with intestinal malabsorption. Metabolic hepatic disorders involving organic anions (eg, Gilbert disease, Crigler-Najjar syndrome, and Dubin-Johnson syndrome) do not cause abnormal serum bile acid concentrations.
The concentration of bile acids in serum is influenced by many different liver diseases due to the inability of the liver to efficiently extract circulating bile acids from portal blood.
In addition, bile acid levels are altered in several biochemical genetic conditions, such as peroxisomal biogenesis disorders (eg, Zellweger spectrum disorder) and disorders of bile acid synthesis (eg, D-bifunctional protein deficiency and alpha methyl-CoA racemase deficiency), due to the loss of specific enzymes important for bile acid metabolism.
This analysis includes a quantitative characterization of primary and secondary bile acids as well as 2 bile acid precursor species for the assessment of bile acid metabolism.
Chenodeoxycholic acid: < or =2.26 nmol/mL
Cholic acid: < or =2.74 nmol/mL
Deoxycholic acid: < or =2.84 nmol/mL
Dihydroxycholestanoic acid: < or =0.07 nmol/mL
Glycochenodeoxycholic acid: < or =5.14 nmol/mL
Glycocholic acid: < or =2.17 nmol/mL
Glycodeoxycholic acid: < or =3.88 nmol/mL
Glycohyodeoxycholic acid: < or =0.01 nmol/mL
Glycolithocholic acid: < or =0.11 nmol/mL
Glycoursodeoxycholic acid: < or =1.00 nmol/mL
Hyodeoxycholic acid: < or =0.12 nmol/mL
Lithocholic acid: < or =0.09 nmol/mL
Taurochenodeoxycholic acid: < or =0.80 nmol/mL
Taurocholic acid: < or =0.31 nmol/mL
Taurodeoxycholic acid: < or =0.78 nmol/mL
Taurohyodeoxycholic acid: < or =0.02 nmol/mL
Taurolithocholic acid: < or =0.04 nmol/mL
Tauroursodeoxycholic acid: < or =0.05 nmol/mL
Trihydroxycholestanoic acid: < or =1.73 nmol/mL
Ursodeoxycholic acid: < or =0.64 nmol/mL
Total bile acids: < or =19.00 nmol/mL
Total bile acids are metabolized in the liver and can serve as a marker for normal liver function. Increases in serum C27 bile acids are seen in patients with peroxisomal biogenesis disorders (eg, as Zellweger spectrum disorder) or single enzyme defects of bile acid synthesis (eg, D-bifunctional protein deficiency and alpha methyl CoA racemases).
Totals of the free and conjugated bile acid species for all 20 bile acids in addition to total bile acids will be reported. No interpretive report will be provided.
Bile acid concentrations in serum may be elevated post meal or due to bile acid therapy, such as cholic acid, deoxycholic acid, or ursodeoxycholic acid.
Do not use for assessment of general liver dysfunction in adults or diagnosis or monitoring of intrahepatic cholestasis of pregnancy.
1. Sundaram SS, Bove KE, Lovell MA, Sokol RJ. Mechanisms of disease: inborn errors of bile acid synthesis. Nat Clin Pract Gastroenterol Hepatol. 2008;5(8):456-468
2. Wanders RJA, Rizzo WB. Inborn errors of peroxisome biogenesis and function. In: Sarafoglou K, Hoffmann GF, Roth KS, eds. Pediatric Endocrinology and Inborn Errors of Metabolism. McGraw-Hill Medical Division. 2nd ed. 2017:427-446
3. Ducroq DH, Morton MS, Shadi N, et al. Analysis of serum bile acids by isotope dilution-mass spectrometry to assess the performance of routine total bile acid methods. Ann Clin Biochem. 2010;47(Pt 6):535-540
4. Fischler B, Eggersten G, Bjorkhem I. Genetic defects in synthesis and transport of bile acids. In: Sarafoglou K, Hoffmann GF, Roth KS, eds. Pediatric Endocrinology and Inborn Errors of Metabolism. McGraw-Hill Medical Division; 2017:447-460
5. Society for Maternal-Fetal Medicine (SMFM). Lee RH, Mara Greenberg, Metz TD, Pettker CM. Society for Maternal-Fetal Medicine Consult Series #53: Intrahepatic cholestasis of pregnancy: replaces consult #13, April 2011. Am J Obstet Gynecol. 2021;224(2):B2-B9. doi:10.1016/j.ajog.2020.11.002
Bile acid concentrations in serum are measured by liquid chromatography tandem mass spectrometry stable isotope dilution analysis. Serum is mixed with isotopically labeled internal standards of selected bile acids and then subjected to protein precipitation. Sample preparation is semiautomated using a liquid handler. Reverse-phase liquid chromatography is performed to separate free bile acids, their respective tauro- and glyco-conjugates, and 2 bile acid precursors.(Unpublished Mayo method)
This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.
82542
Test Id | Test Order Name | Order LOINC Value |
---|---|---|
BAPS | Bile Acid Profile, S | 43130-4 |
Result Id | Test Result Name |
Result LOINC Value
Applies only to results expressed in units of measure originally reported by the performing laboratory. These values do not apply to results that are converted to other units of measure.
|
---|---|---|
35802 | Chenodeoxycholic acid | 30519-3 |
35801 | Cholic acid | 30518-5 |
35803 | Deoxycholic acid | 30520-1 |
35819 | Dihydroxycholestanoic acid | 53479-2 |
35808 | Glycochenodeoxycholic acid | 93335-8 |
35807 | Glycocholic acid | 93334-1 |
35809 | Glycodeoxycholic acid | 93333-3 |
35811 | Glycohyodeoxycholic acid | 93332-5 |
35812 | Glycolithocholic acid | 93331-7 |
35810 | Glycoursodeoxycholic acid | 93330-9 |
35805 | Hyodeoxycholic acid | 93329-1 |
35806 | Lithocholic acid | 74897-0 |
35814 | Taurochenodeoxycholic acid | 93328-3 |
35813 | Taurocholic acid | 93327-5 |
35815 | Taurodeoxycholic acid | 93326-7 |
35817 | Taurohyodeoxycholic acid | 93325-9 |
35818 | Taurolithocholic acid | 93324-2 |
35816 | Tauroursodeoxycholic acid | 93323-4 |
35820 | Trihydroxycholestanoic acid | 38188-9 |
35804 | Ursodeoxycholic acid | 55159-8 |
35821 | Total bile acids | 14628-2 |