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Test ID: BAPS Bile Acid Profile, Serum


Ordering Guidance


This test is intended for use by research scientists. Approval must be obtained before ordering.



Specimen Required


Patient Preparation: Patient must be fasting for 12 to 14 hours.

Collection Container/Tube:

Preferred: Red top

Acceptable: Serum gel

Submission Container/Tube: Plastic vial

Specimen Volume: 0.5 mL


Useful For

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

Method Name

Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS)

Reporting Name

Bile Acid Profile, S

Specimen Type

Serum

Specimen Minimum Volume

0.3 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Serum Refrigerated (preferred) 90 days
  Ambient  90 days
  Frozen  90 days

Clinical Information

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 like Zellweger syndrome and disorders of bile acid synthesis such as 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.

Reference Values

Chenodeoxycholic acid: ≤2.26 nmol/mL Cholic acid: ≤2.74 nmol/mL Deoxycholic acid: ≤2.84 nmol/mL Dihydroxycholestanoic acid: ≤0.07 nmol/mL Glycochenodeoxycholic acid: ≤5.14 nmol/mL Glycocholic acid: ≤2.17 nmol/mL Glycodeoxycholic acid: ≤3.88 nmol/mL Glycohyodeoxycholic acid: ≤0.01 nmol/mL Glycolithocholic acid: ≤0.11 nmol/mL Glycoursodeoxycholic acid: ≤1.00 nmol/mL Hyodeoxycholic acid: ≤0.12 nmol/mL Lithocholic acid: ≤0.09 nmol/mL Taurochenodeoxycholic acid: ≤0.80 nmol/mL Taurocholic acid: ≤0.31 nmol/mL Taurodeoxycholic acid: ≤0.78 nmol/mL Taurohyodeoxycholic acid: ≤0.02 nmol/mL Taurolithocholic acid: ≤0.04 nmol/mL Tauroursodeoxycholic acid: ≤0.05 nmol/mL Trihydroxycholestanoic acid: ≤1.73 nmol/mL Ursodeoxycholic acid: ≤0.64 nmol/mL Total bile acids: ≤19.00 nmol/mL

Interpretation

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 such as Zellweger syndrome or single enzyme defects of bile acid synthesis such as D-bifunctional protein deficiency and alpha methyl CoA racemaces.

 

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.

Clinical Reference

1. Sundaram SS, Bove KE, Lovell MA, Sokol RJ: Mechanisms of disease: inborn errors of bile acid synthesis. Nat Clin Pract Gastroenterol Hepatol. 2008 Aug;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 Nov;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

Day(s) Performed

Monday through Friday

Report Available

2 to 5 days

Test Classification

This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the U.S. Food and Drug Administration.

CPT Code Information

82542

LOINC Code Information

Test ID Test Order Name Order LOINC Value
BAPS Bile Acid Profile, S 43130-4

 

Result ID Test Result Name Result LOINC Value
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

Forms

If not ordering electronically, complete, print, and send a Gastroenterology and Hepatology Client Test Request (T728) with the specimen.

Mayo Clinic Laboratories | Genetics and Pharmacogenomics Catalog Additional Information:

mml-biochemical