Table 2.

Biochemical analyses in plasma and urine from ZSD patients treated with CA (group 1: patients 1–15, group 2: patients 16–19)

CA treatment	0 weeks						4 weeks			12 weeks						36 weeks
Patient #	CA	DHCA	THCA	FGF19	C4	Urine#	CA	DHCA	THCA	CA	DHCA	THCA	FGF19	C4	Urine#	CA	DHCA	THCA	FGF19	C4	Urine#
1	0.5	0.2	0.6	0.999	30.6	present	3.0	<0.05	0.1	3.5	0.3	0.1	0.545	<1.0	nd	2.2	0.3	0.1	0.444	4.3	nd
2	0.5	<0.05	<0.05	0.413	24.3	nd	6.2	<0.05	<0.05	1.0	<0.05	<0.05	0.193	11.6	nd	2.6	<0.05	<0.05	0.507	12.7	nd
3	2.8	0.2	0.4	0.242	12.3	nd	10.0	0.1	0.2	2.2	<0.05	<0.05			nd	1.6	0.1	<0.05	0.286	7.2	nd
4	0.8	1.6	0.9	0.055	19.1	nd	7.7	0.4	<0.05	3.7	0.5	0.1	0.647	1.0	nd	2.7	<0.05	0.1	1.166	<1.0	nd
5	0.1	0.3	0.1	0.186	27.4	nd	3.4	<0.05	<0.05	0.4	<0.05	<0.05	0.601	<1.9	nd	0.2	<0.05	<0.05	0.387	4.4	nd
6	0.3	<0.05	<0.05	0.134	23.5	present	3.5	<0.05	<0.05	4.0	<0.05	<0.05	1.284	1.0	nd	0.8	<0.05	<0.05	0.356	<1.0	nd
7	0.6	<0.05	<0.05	0.449	28.7	nd	2.4	<0.05	<0.05	1.0	<0.05	<0.05	0.248	10.0	nd	1.0	<0.05	<0.05	0.390	17.5	nd
8	1.0	0.2	0.4	0.475	18.9	nd	2.4	<0.05	<0.05	9.1	<0.05	0.3	0.799	1.6	nd	2.6	0.2	0.1	0.885	<1.0	nd
9	0.4	<0.05	0.1	0.100	12.1	nd	8.3	<0.05	<0.05	5.5	<0.05	<0.05	0.914	1.2	nd	1.9	<0.05	0.1	0.453	1.4	nd
10	0.1	<0.05	<0.05			nd	1.3	<0.05	<0.05	1.1	<0.05	<0.05	0.847	11.7	nd	1.3	<0.05	<0.05	0.208	7.7	nd
11	1.0	0.2	<0.05	0.406	29.2	nd	1.6	<0.05	<0.05	4.0	<0.05	0.1	0.686	14.2	nd	6.5	<0.05	0.1	0.328	15.2	nd
12	1.4	2.8	7.2	0.181	11.3	present	15.1	1.0	2.4	21.6	1.3	1.4	0.319	1.7	nd	39.9	1.3	2.5	0.573	1.2	nd
13	0.6	0.5	2.0	0.142	<1.0	nd	7.2	0.4	0.1	10.1	0.4	<0.05	0.328	<1.0	nd	13.3	0.3	0.4	0.353	<1.0	nd
14	0.5	4.1	0.4	0.114	34.3	present	11.3	0.5	0.2	15.1	<0.05	<0.05	0.817	<1.0	nd	9.9	<0.05	0.1	0.422	<1.0	nd
15	3.3	4.4	1.7	0.177	8.4	nd	12.2	1.1	0.7	9.6	1.2	0.6	0.276	2.5	nd	13.0	0.8	0.8	0.240	3.6	nd
16	3.3	6.0	10.6	0.359	2.4	present	174.8	3.3	10.0	69.4	3.2	16.7	0.516	<1.0	present				excluded
17	47.4	6.0	18.5	0.254	1.7	present	116.6	1.0	1.5	123.2	1.6	1.7	0.483	<1.0	nd	251.8	1.5	3.7	0.815	<1.0	nd
18	11.0	10.8	32.4	0.423	1.8	no urine	92.4	3.8	8.9	175.8	3.9	9.0	1.053	<1.0	no urine	154.2	4.9	10.1	1.083	<1.0	no urine
19	2.4	15.5	12.0	0.094	1.1	present	60.0	9.3	6.2	20.6	8.3	3.6	0.460	<1.0	present	100.2	10.5	11.5	0.374	<1.0	nd
Median	0.8	0.3	0.4	0.2	15.6		7.7	0.1	0.1	5.5	0.0	0.1	0.6	1.1		2.7	0.1	0.1	0.4	1.3

Abbreviation: CA cholic acid, DHCA dihydroxycholestanoic acid, THCA trihydroxycholestanoic acid, FGF19 fibroblast growth factor 19, C4 7alpha-hydroxy-4-cholesten-3-one, nd not detected. # Urinary bile acids comprises C₂₇-bile acid intermediates, derivatives and bile alcohols. Plasma bile acids in μmol/L, FGF19 and C4 in μg/L; 10 mg/kg/day cholic acid doses are depicted in bold, 20 mg/kg/day cholic acid doses are depicted in italic. Due to problems with blood withdrawal or hemolysis of the samples, some data points are missing. Note that urinary bile acid were only measured qualitatively

Note: Significance of bold and italic - effect of different doses of cholic acid supplementation on different biochemical parameters