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. Author manuscript; available in PMC: 2016 Mar 1.
Published in final edited form as: J Pediatr Gastroenterol Nutr. 2015 Mar;60(3):368–374. doi: 10.1097/MPG.0000000000000630

Table 2.

Kinetics parameters for CA and CDCA acid as determined by stable isotope dilution

CA CDCA
Subject Diagnosis Fractional Turnover (d−1) Pool Size (mg/m2) Synthesis Rate (mg/d) Fractional Turnover (d−1) Pool Size (mg/m2) Synthesis Rate (mg/d)
PEBD subjects
1 PFIC1 0.93 1312 923 2.09 565 892
2 PFIC1 4.25 365 2401 2.65 418 1717
3 PFIC1 1.86 821 1081 1.9 119 160
4 PFIC-U 0.49 447 237 0.71 48 37
5 PFIC-U 1.50 1498 1463 4.48 77 224
LTX subjects
6 PFIC1 0.81 428 604 0.44 162 124
7 PFIC2 0.20 800 183 0.26 504 150
8 PFIC2 0.38 522 337 0.34 402 233
9 PFIC2 0.74 282 171 0.41 550 185
10 PFIC2 0.95 739 1071 0.29 780 345