Table 2 Median, range and 95 ^th centile for each urinary steroid metabolite ratio analysed for the whole group of controls as well as categorised by broad age groups

Enzyme defect

Median age

10 years

2 months

5 years

13 years

(Range)

(11d-18 yrs)

(11 d-5 m)

(6 m-9 yrs)

(10–18 yrs)

M:F ratio

115:137

7:5

47:59

61:73

21-Hydroxylase def

17HP/(THE + THF + 5αTHF)

Median (range)

0.01 (0–0.25)

0.01 (0.01-0.11)

0.01 (0–0.25)

0.01 (0–0.11)

95% centile

0.04

0.1

0.03

0.04

21-Hydroxylase def

PT/(THE + THF + 5αTHF)

Median (range)

0.03 (0–0.48)

0.02 (0.01-0.03)

0.02 (0–0.28)

0.06 (0.01-0.48)

95% centile

0.13

0.03

0.09

0.15

21-Hydroxylase def

PTONE/(THE + THF + 5αTHF)

Median (range)

0 (0–0.29)

0.01 (0–0.14)

0 (0–0.29)

0 (0–0.04)

95% centile

0.01

0.11

0.02

0.01

11β-Hydroxylase def

THS/(THE + THF + 5αTHF)

Median (range)

0.01 (0–0.09)

0 (0–0.04)

0.01 (0–0.09)

0 (0–0.02)

95% centile

0.02

0.03

0.03

0.01

3β-Hydroxysteroid dehydrogenase def

DHEA/(THE + THF + 5αTHF)

Median (range)

0 (0–1.25)

0.01 (0–0.1)

0 (0–1.25)

0 (0–0.31)

95% centile

0.12

0.1

0.03

0.14

P450 oxidoreductase def

(17HP + PT)/(An + Et)

Median (range)

0.38 (0.1-12.5)

1.95 (0.5-5)

0.75 (0.17-12.5)

0.23 (0.1-0.75)

95% centile

3.18

4.52

3.93

0.46

Apparent mineralocorticoid excess

(THF + 5αTHF)/THE

Median (range)

0.72 (0.01-7)

0.04 (0.01-0.35)

0.8 (0.15-7)

0.68 (0.25-.3.43)

95% centile

1.97

0.33

3.01

1.3

Apparent cortisone reductase deficiency

THE/(THF + 5αTHF)

Median (range)

1.4 (0.16-88.69)

22.82 (2.87-88.69)

1.25 (0.16-20.9)

1.49 (0.29-20.7)

95% centile

3.84

86.58

2.39

3

17β-Hydroxysteroid dehydrogenase deficiency

(An + Et)/(THE + THF + 5αTHF)

Median (range)

0.11 (0–1.65)

0.01 (0.01-0.09)

0.03 (0–0.75)

0.3 (0.04-1.65)

95% centile

0.7

0.07

0.23

0.9

5α-Reductase deficiency

THF/5αTHF

Median (range)

0.73 (0.08-25.25)

0.96 (0.19-5)

0.68 (0.08-25.25)

0.83 (0.22-2)

95% centile

1.75

4.19

1.93

1.71

5α-Reductase deficiency

THB/5αTHB

Median (range)

0.28 (0–68)

20.1 (0.33-68)

0.27 (0.02-47.64)

0.39 (0–2.05)

95% centile

3.89

53.06

1.97

1.24

5α-Reductase deficiency

Et/An

Median (range)

0.59 (0.03-4.86)

0.13 (0.03-1)

0.56 (0.06-4.86)

0.64 (0.26-2.1)

95% centile

1.7

0.69

2

1.5