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. 2020 Mar;8(5):185. doi: 10.21037/atm.2020.01.103

Table S2. Concentrations of UDCAs, THBAs and some atypical bile acids (nM) in patients with ABCB11 mutations before and after biliary diversion.

Patient 1 1* 2 2* 2* 3 3* 3* HC (n=40), M (Q1, Q3) nM PFIC2 control (n=8), M (Q1, Q3)
Sampling age 5 m 7 m 2.6 y 4.0 y 4.3 y 7.5 y 8.8 y 9.5 y
tauro-THBA 227 232 12 1 0 32 0 26 0.8 (0, 1.4) 156.5 (14.1, 273.6)
glyco-UDCA-3-sulfate 5,194 8,045 14,895 11,517 8,790 11,714 13,250 34,294 90 (48.6, 143.1) 14,391.8 (5,136.6, 83,232.4)
tauro-UDCA-3-sulfate 4,171 5,337 19 13 9 11,961 7 19 0.3 (0.1, 14.5) 8,690 (2,070.5, 27,430.1)
glyco-CA-3-sulfate 17 4 233 76 19 1 47 223 0.8 (0.3, 1.8) 183.3 (2.2, 319.6)
tauro-dehydro-CA 0.4 0.5 0 0 0 0.2 0 0 0.2 (0, 0.2) 0.3 (0.2, 1)
tauro-ω-MCA 68.5 0 0 0 0 37.4 2.6 42.7 0.2 (0, 2) 137.9 (22.8, 476.7)
tauro-α-MCA 931 506 100 62 54 43 1 47 6.3 (0.5, 20.1) 730.6 (84.5, 1,711)
tauro-β-MCA 1,096 590 191 32 39 6 20 283 1.5 (0.2, 2.4) 396.8 (50, 1,865.3)
glyco-dehydro-CA 0.1 0 0 6.3 1.7 0 0 0 0 (0, 1.2) 0 (0, 2)
THBA 0.4 0.4 6.3 3.5 4.4 0.2 0 0.1 0.4 (0.3, 2.5) 1.8 (0.3, 33.5)
glyco-DCA-3-sulfate 0 0 2.3 0 0 0 1,293 1,417.9 50.1 (4.3, 85.8) 0.8 (0, 1.8)
tauro-CDCA-3-sulfate 27,854 16,699 16 16 8 5,292 0 0 0.7 (0.1, 90.3) 10,196 (4,111.4, 29,233.7)
tauro-HCA 3,837 2,852 419 330 172 198 48 648 8.6 (0, 21.6) 1,713.5 (1,273.9, 2,818)
CA-3-sulfate 54.9 110.5 20.8 10.9 3.4 6.9 7.4 24.7 0.8 (0.3, 1.8) 27.5 (18.7, 41.4)
tauro-DCA-3-sulfate 0 0 0 0.4 0 252.6 1 2.7 0.1 (0, 3.4) 0.2 (0, 3.3)
tauro-HDCA 19387 4286.2 1.7 0.3 0.2 61,032 4.7 4.2 0 (0, 14.1) 8,690 (2,070.5, 27,430.1)
tauro-UDCA 20,149 4,739 14,691 13,979 39,234 61,662 1,271 11,856 16.2 (4.8, 26.6) 10,812.1 (1,719.8, 32,152.5)
glyco-LCA-3-sulfate 120.7 0 24.9 40.4 28.1 123.7 1,398.6 1,762.5 50 (7.7, 140.7) 54.2 (39.1, 68.7)
UCA 2.1 2.3 7.8 5.3 1.9 2.2 0 0 2.6 (1.3, 4.7) 4.1 (2.1, 16.8)
7,12-keto-LCA 379.2 576.7 2.8 0.1 2.3 594.8 0 0 3.4 (0, 9.9) 28.4 (2, 151.4)
glyco-HCA 1,322 1,647 406 400 167 141 348 1,276 42.9 (30.3, 96.8) 1,156.3 (338.2, 1,515.1)
tauro-LCA-3-sulfate 91.4 12.4 0.2 0.1 0.1 0.7 1.4 0 0.1 (0, 0.7) 20.5 (4.1, 88.1)
dehydro-CA 5 3.5 1.4 1.8 0 2 3.7 14.7 10.2 (6.7, 18.6) 2.3 (0.6, 10.3)
ω-MCA 0.3 0.3 3.5 9.9 18.2 0.5 8 22.2 2.9 (0.3, 15.9) 2.8 (1.5, 3.9)
glyco-UDCA 30,655 25,544 56,811 70,169 141,921 156,371 17,697 123,036 293.5 (87.8, 531.2) 53,559.2 (25,854.3, 99,666.6)
glyco-HDCA 0 0 19.1 28.5 27 0 19.3 0 2 (0, 4.5) 16.2 (0, 18,612.7)
α-MCA 0.5 0.7 30.8 29.1 1.8 0.9 1.9 4.1 4.9 (0.4, 15.1) 6.6 (2.9, 7.5)
nor-CA 32 28.2 50.6 60.3 27.4 28.8 29.5 38.5 21.9 (12.9, 33.7) 36 (14.7, 46.9)
nor-UDCA 30.4 3.5 2.6 4.7 1.5 14.4 63.4 17.9 4.4 (2.3, 10.5) 29.9 (19.2, 31.3)
7-keto-DCA 1.3 1.9 0.3 8.5 7.5 1.7 8.9 5.2 2.3 (1.2, 4.1) 0.9 (0.1, 4.8)
DCA-3-sulfate 0 1.2 0.1 0.1 0.1 0.6 12 26.1 0.7 (0.3, 1.4) 0.2 (0.1, 0.5)
β-MCA 0.3 2 0.5 0 0.4 0.4 3.5 0.9 1 (0.3, 4.1) 2.8 (1, 11)
12-keto-CDCA 877 1114 0 78 3 920 0 2 2.9 (0, 24.3) 154.9 (12.2, 644.9)
HCA 5.1 7.5 0 0 0 0.7 28.5 14.2 12.3 (8, 28) 4.3 (1.6, 19.3)
MCA 1 0.4 0 0 0 0.4 0 0 0.5 (0, 1.8) 0.5 (0.2, 2.4)
3-keto-CA 3.3 1.7 0.2 0 1.3 0.5 3.3 3.9 0.2 (0, 1) 1.7 (0.9, 4.4)
alloCA 1.1 0.9 0.6 0.8 0 0 5.9 6.5 10.9 (5.3, 45.1) 2.1 (1.3, 8.4)
UDCA 148 39 119 116 818 411 575 3,522 58.6 (22.7, 148.6) 1,064.1 (320.2, 6,419.4)
HDCA 1.5 0.2 0 0 0 1.2 0 0 0 (0, 1.1) 2.5 (0, 4.5)
7-keto-LCA 1 1.3 4 6 23.8 4.3 20.8 3.1 5.7 (0.9, 11.9) 5.7 (4, 8.3)
6,7-keto-LCA 0 0 1 12.4 16.8 0.8 6.8 9.3 0.5 (0.2, 3.6) 0.2 (0.1, 9.3)
nor-DCA 1.5 7.6 0 0 0.4 0 0 0 0.4 (0, 1.3) 2.2 (1.1, 5.9)
12-keto-LCA 0.3 0.2 3.2 0.8 1.9 0.1 32.1 4.1 0.2 (0, 4.1) 0.2 (0.1, 1.5)
apoCA 0.6 0.2 0 0 0 0.1 9.5 0 0 (0, 0.5) 0 (0, 0.1)
alloisoLCA 0.5 0.6 2.1 2.3 2.7 0.8 0.3 0.2 0.9 (0.6, 1.6) 0.8 (0.3, 3.9)
isoLCA 0.8 1 0 0 0 1 7.1 0 0 (0, 1.8) 0.2 (0, 1.1)
isoDCA 0.2 0.2 0 0 0 0.1 1.8 0 0 (0, 0.1) 0.3 (0.1, 3.3)
dehydro-LCA 1.8 0.9 0 0 0 2.3 0 0 0.2 (0, 1.1) 0.1 (0, 1.2)
TBA 343,407 306,040 320,255 426,327 516,707 470,453 85,289 302,824 6,128.4 (3,250, 8,364) 335,544 (216,087, 612,401)

HC, healthy controls. PFIC2 control: PFIC2 patients who were underwent UDCA therapy with no biliary diversion procedure. *, Bile acid profiles after PIBD. TBA, total bile acids (60 bile acids listed in Table 2 and Table S2).