Partial external biliary diversion in bile salt export pump deficiency: Association between outcome and mutation

Table 1 Concentrations of bile acids in serum and bile

	Serum			Bile
	T919del	G1032R	Controls (n = 33)	T919del	G1032R	Controls (n = 6)	Cholestasis (n = 6)
CA			0.15 ± 0.09		2232	15 ± 9	88 ± 72
Tauro-CA	5.4	7.2	0.15 ± 0.17	85.7	549	3721 ± 2597	754 ± 405
Glyco-CA	25	39	0.28 ± 0.31	274.5	552	8189 ± 5978	1133 ± 662
CDCA			0.13 ± 0.12		118		120 ± 48
Tauro-CDCA	5.2	6.2	0.17 ± 0.17	54.2		3840 ± 2005	646 ± 396
Glyco-CDCA	26	18	0.49 ± 0.60	185.7		8285 ± 8306	1100 ± 811
DCA			0.25 ± 0.29				35 ± 19
Tauro-DCA			0.11 ± 0.08			3028 ± 2562	351 ± 328
Glyco-DCA			0.24 ± 0.25			5777 ± 4287	537 ± 674
UDCA	0.01		0.11 ± 0.04				146
Tauro-UDCA	7.5			77.4		357 ± 218	384 ± 591
Glyco-UDCA	81		0.36 ± 0.18	853.2		861 ± 635	1403 ± 1877
total	150.1	70.4	1.37 ± 1.61	1531	3451	33603 ± 23354	5965 ± 4447
% CDCAderivatives	21	34	36 ± 16	40	3	38 ± 8	44 ± 5
bile/serum (μmol/L/μmol/L)				10.2	49	ca. 24500

Bile salt derivatives in bile of the female patient (p.T919del) and the male patient (p.G1032R), in six non-cholestatic patients and in six patients with obstructive cholestasis of different causes. Serum control values are from 33 non-cholestatic patients. Values are given in μmol/L. CA: Cholic acid; UDCA: Ursodeoxycholic acid.