Table 2. Concentrations of main primary and secondary bile acids (nM) in patients with ABCB11 mutations before and after biliary diversion.
| 1 | 1* | 2 | 2* | 2* | 3 | 3* | 3* | HC (n=40), M (Q1, Q3) | 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 | ||
| CA | 14.8 | 45.7 | 25.2 | 26.8 | 24.9 | 22.2 | 107.5 | 56.7 | 41.3 (26.6, 69.3) | 32.2 (9.4, 43.6) |
| tauro-CA | 41,969.0 | 38,944.9 | 39,335.6 | 18,553.2 | 12,074.6 | 35,946.1 | 1,015.7 | 10,438.4 | 66.6 (33.7, 151.3) | 16,378 (12,118, 52,241.9) |
| glyco-CA | 29,657.2 | 40,692.9 | 70,335.0 | 46,591.8 | 34,995.3 | 2,427.4 | 7,589.2 | 46,937.7 | 442.4 (187.9, 745.6) | 40,021.6 (28,211.8, 65,710.1) |
| T:G (CA) | 1.42 | 0.96 | 0.56 | 0.40 | 0.35 | 14.81 | 0.13 | 0.22 | 0.2 (0.13, 0.4) | 0.41 (0.24, 0.72) |
| Total CA | 71,641 | 79,684 | 109,696 | 65,172 | 47,095 | 38,396 | 8,712 | 57,433 | 583 (299, 978.9) | 64,543.4 (43,812.9, 95,838) |
| Proportion (%) | 31.60 | 34.11 | 47.26 | 19.79 | 14.48 | 24.06 | 17.77 | 46.26 | 13.6 (9.41, 20.87) | 41.05 (32, 49.44) |
| CDCA | 35.6 | 44.8 | 16.0 | 91.4 | 196.2 | 28.2 | 123.7 | 37.5 | 156.4 (68.2, 279.8) | 51.1 (21.8, 99.9) |
| tauro-CDCA | 84,437 | 62,412 | 30,480 | 54,346 | 57,107 | 37,054 | 2,309 | 7,650 | 243.6 (113, 706.3) | 42,328.1 (31,922.6, 51,978.7) |
| glyco-CDCA | 70,515 | 91,373 | 91,785 | 209,509 | 220,558 | 83,477 | 21,881 | 49,158 | 2,179 (1,218.6, 4,136.5) | 60,232.8 (38,923.8, 117,941.9) |
| T:G (CDCA) | 1.20 | 0.68 | 0.33 | 0.26 | 0.26 | 0.44 | 0.11 | 0.16 | 0.14 (0.08, 0.18) | 0.53 (0.34, 0.76) |
| Total CDCA | 154,988 | 153,829 | 122,281 | 263,947 | 277,861 | 120,560 | 24,314 | 56,846 | 2,859.6 (1,598.2, 5,048.1) | 105,683.8 (82,402.4, 162,918.1) |
| Proportion (%) | 68.36 | 65.84 | 52.68 | 80.16 | 85.43 | 75.54 | 49.58 | 45.79 | 70.88 (64.17, 80.33) | 58.86 (50.54, 67.91) |
| DCA | 45.2 | 71.2 | 55.4 | 44.8 | 184.7 | 37.9 | 627.2 | 181.2 | 135.3 (81.9, 296) | 39.7 (27.4, 75.5) |
| tauro-DCA | 9.9 | 13.1 | 15.6 | 19.0 | 16.0 | 167.6 | 2,271.1 | 1,710.3 | 23.3 (1, 40.7) | 9.4 (4.8, 18.1) |
| glyco-DCA | 18.5 | 21.9 | 38.9 | 42.7 | 46.6 | 364.5 | 12,317.0 | 7,542.8 | 93.4 (3.4, 260.6) | 16.7 (11.6, 24.4) |
| T:G (DCA) | 0.54 | 0.60 | 0.40 | 0.45 | 0.34 | 0.46 | 0.18 | 0.23 | 0.25 (0.11, 0.37) | 0.56 (0.45, 0.9) |
| Total DCA | 73.7 | 106.1 | 109.9 | 106.6 | 247.3 | 570.0 | 15,215.3 | 9,434.3 | 351.2 (99.8, 594.5) | 60.2 (51, 100.1) |
| Proportion (%) | 0.03 | 0.05 | 0.05 | 0.03 | 0.08 | 0.36 | 31.03 | 7.60 | 9.82 (2.15, 20.25) | 0.04 (0.02, 0.08) |
| LCA | 1.7 | 1.2 | 0.3 | 0.2 | 0.0 | 2.2 | 39.9 | 13.0 | 2.2 (0.9, 6.2) | 2.1 (1.2, 5.5) |
| tauro-LCA | 7.0 | 3.2 | 0.2 | 4.5 | 4.2 | 15.2 | 210.9 | 39.8 | 0.5 (0.1, 1.4) | 2.8 (2, 3.9) |
| glyco-LCA | 26.0 | 14.8 | 14.9 | 43.7 | 47.1 | 45.9 | 548.2 | 383.8 | 5.8 (1.8, 19.1) | 14.2 (8.2, 22.8) |
| T: G (LCA) | 0.27 | 0.22 | 0.02 | 0.10 | 0.09 | 0.33 | 0.38 | 0.10 | 0.05 (0.03, 0.15) | 0.15 (0.11, 0.2) |
| Total LCA | 34.8 | 19.2 | 15.4 | 48.5 | 51.4 | 63.3 | 798.9 | 436.6 | 10.9 (3.5, 22.5) | 20.8 (14.5, 46.7) |
| Proportion (%) | 0.02 | 0.01 | 0.01 | 0.01 | 0.02 | 0.04 | 1.63 | 0.35 | 0.36 (0.06, 0.71) | 0.01 (0.01, 0.02) |
Main primary bile acids: CA, tauro-CA, glyco-CA, CDCA, tauro-CDCA, glyco-CDCA. Main secondary bile acids: DCA, tauro-DCA, glyco-DCA, LCA, tauro-LCA, glyco-LCA. Proportion %: proportion of calculated bile acids, e.g., proportion of total CA (total CA, tauro-CA, glyco-CA) in calculated main primary (CA, tauro-CA, glyco-CA, CDCA, tauro-CDCA, glyco-CDCA) and secondary bile acids (DCA, tauro-DCA, glyco-DCA, LCA, tauro-LCA, glyco-LCA). HC, healthy controls. *Bile acid profiles after PIBD.