Table 1 Statistics of subjects with inborn errors in bile acid metabolism.

Measurement	Subject
	A	B	C	D	E
Age at diagnosis	13 y	2 mo	2 y	3 mo	3 mo
Age at study	19 y	6 y	6 y	13 y	13 y
Sex	M	F	M	M	M
Diagnosis	3‐HSD	Racemase	3‐HSD	5β reductase	5β reductase
BA therapy	CA	CA	CA	UDCA	UDCA
ALT on therapy (U/l)*	<3	7	21	24	25
ALT off therapy(U/l)*	140	19	61	23	25
GGT on therapy(U/l)*	45	23	29	28	21
GGT off therapy(U/l)*	37	15	24	ND	21

BA, bile acid; 3‐HSD, 3β hydroxy‐C₂₇‐steroid dehydrogenase/isomerase; 5β reductase, Δ⁴‐3‐oxosteroid 5β reductase; racemase, 2‐methylacyl CoA racemase; CA, cholic acid; UDCA, ursodeoxycholic acid; ALT, alanine aminotransferase; GGT, gamma glutamyl transferase; ND, not determined.

*Age matched normal values for Children's Hospital Clinical Laboratory: ALT 10–45 U/l, GGT 11–34 U/l.