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Table 3 Pubertal development in 13 patients and treatment for HH in seven patients

From: Clinical and genetic characteristics of 42 Chinese paediatric patients with X-linked adrenal hypoplasia congenita

Patient ID

age (year)

Height SDS

Testes (ml)

FSH (IU/L)

LH (IU/L)

Testosterone (nmol/L)

GnRH stimulation test

Treatment duration (month)

Height SDS

Testes (ml)

FSH (IU/L)

LH (IU/L)

Testosterone (nmol/L)

GnRH stimulation test

Peak of FSH (IU/L)

Peak of

Peak of

Peak of

LH

FSH

LH

(IU/L)

(IU/L)

(IU/L)

Pulsatile GnRH

16

14

0.43

3.5

1.58

 < 0.20

 < 0.35

4.99

0.59

1

0.38

4.5

6.57

1.77

1.43

6.32

1.94

20

14

 − 1.50

3

4.12

0.23

 < 0.35

6.27

1.35

3

0.19

4.5

8.37

0.95

1.12

9.31

0.99

32

14.5

 − 1.67

3

2.82

0.39

 < 0.35

1.93

1.69

4

 − 0.58

9

3.63

3.42

4.56

3.34

0.91

hCG (1000–2000 IU, twice weekly)

11

15.3

 − 3.24

3

5.73

0.55

 < 0.35

NE

NE

48

 − 0.54

4

5.57

1.4

0.92

  

14

14.1

 − 2.31

2.5

1.52

0.2

 < 0.35

1.54

0.64

5

 − 2.29

3

1.46

0.62

1.39

  

20

14

 − 1.50

3

4.12

0.23

 < 0.35

6.27

1.35

30

0.11

3.5

4.5

0.8

3.01

  

23

14.2

 − 2.76

2.5

1.5

 < 0.20

 < 0.35

2.18

0.21

6

 − 1.88

3

0.81

 < 0.20

1.14

  

32

14.5

 − 1.67

3

2.82

0.39

 < 0.35

1.93

1.69

6

 − 1.57

3

1.61

0.67

2.59

  

34

14

 − 1.71

2

1.25

0.13

0.62

1.41

0.16

7

 − 1.39

3.5

0.99

0.1

14.86

  

Spontaneous pubertal development

2

12.8

 − 1.05

6

9.21

1.17

5.79

–

–

–

–

–

–

–

–

  
 

16.9

 − 1.54

3.5

7.75

0.87

3.92

9.19

3.66

–

–

–

–

–

–

  
 

17.3

 − 0.40

3

4.77

0.42

0.49

NE

NE

NE

NE

NE

NE

NE

NE

  

9

12.2

0.43

5

11.9

1.23

2.95

–

–

–

–

–

–

–

–

  
 

14.5

 − 0.64

6

15.26

1.2

6.75

–

–

–

–

–

–

–

–

  

10

12.6

 − 1.14

5

10.38

0.53

3.06

–

–

–

–

–

–

–

–

  
 

15.5

 − 0.65

8

10.21

3.24

4.73

–

–

–

–

–

–

–

–

  

Othersa

3

14.1

 − 2.35

2

2.45

0.24

 < 0.35

1.77

 < 0.20

NE

NE

NE

NE

NE

NE

  

21

14

 − 1.86

2.5

2.48

0.22

 < 0.35

NE

NE

NE

NE

NE

NE

NE

NE

  

37

14.2

 − 1.89

2

2.68

0.24

 < 0.35

NE

NE

NE

NE

NE

NE

NE

NE

  
  1. hCG human chorionic gonadotropin; GnRH gonadotropin-releasing hormone; SDS standard deviation score
  2. aPatients without data on treatment outcomes; NE not evaluated; – not applicable
  3. Reference range: FSH (follicle-stimulating hormone): 14 years, 0.45–10.46 IU/L; 15 years, 0.43–18.54 IU/L; 16 years, 0.16–9.65 IU/L; 17–19 years, 2.12–14.15 IU/L; LH (luteinizing hormone): 14 years, 0.48–7.93 IU/L; 15 years, 0.50–10.73 IU/L; 16 years, 0.48–10.83 IU/L; 17–19 years, 1.20–5.49 IU/L; testosterone: 13–15 years, 0.1–17.6 nmol/L; 16–18 years, 4.0–24.0 nmol/L; normal pubertal response to GnRH stimulation test: the peak value of LH by 8.0 IU/L