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Table 2 Results found in control and AIP patients for the hormones and metabolites determined

From: Adrenal hormonal imbalance in acute intermittent porphyria patients: results of a case control study

 

T + DHEA

17OHP

DOC

S

 

Control

AIP patients

Control

AIP patients

Control

AIP patients

Control

AIP patients

Parent

7.2

7.0

1.12

0.68

0.82

0.50

0.57

0.51

(5.5-16.4)

(5.3-10.3)

(1.09-1.69)

(0.65-1.16)**

(0.65-1.27)

(0.41-1.05)

(0.45-0.81)

(0.38-0.66)

u- Parent

n.d.

n.d.

0.77

0.58

n.d.

n.d.

n.d.

n.d.

(0.75-1.09)

(0.48-0.90)*

20β-red.

n.d.

n.d.

n.d.

n.d.

n.d.

n.d.

1.19

0.61

(1.06-1.49)

(0.56-1.0)**

u- 20α-red.

n.d.

n.d.

0.45

0.28

n.d.

n.d.

n.d.

n.d.

(0.39-0.57)

(0.20-0.42)**

5β-red. + 3α-red.

2924

1993

200

240

15.1

6.5

41.8

36.3

(2520–3636)

(1612–2642)**

(178–382)

(218–421)

(10.8-31.7)

(5.8-15.1)*

(40.8-71.4)

(33.8-50.2)

5α-red. + 3α-red.

3276

1262

n.d.

n.d.

2.4

0.1

n.d.

n.d.

(2756–3820)

(1135–1990)**

(2.7-7.6)

(0.22-1.27)**

5β-red. + 3α-red. + 20β-red.

n.d.

n.d.

983

1070

n.d.

n.d.

4.9

5.6

(905–1346)

(883–1493)

(4.7-11.7)

(3.9-8.4)

5β-red. + 3α-red. + 20α-red.

n.d.

n.d.

n.d.

n.d.

n.d.

n.d.

8.5

7.7

(7.0-12.5)

(6.6-10.5)

 

B

A

F

E

 

Control

AIP patients

Control

AIP patients

Control

AIP patients

Control

AIP patients

Parent

7.8

3.1

48.5

22.2

119

38.4

227

135

(7.0-14.6)

(2.7-6.9)**

(43.5-63.3)

(19.1-40.1)**

(91–153)

(32.0-52.6)**

(211–290)

(110–173)**

u- Parent

n.d.

n.d.

n.d.

n.d.

50.7

10.3

106

43

(42.2-86.5)

(7.4-13.0)**

(99–175)

(39–69)**

u- 6β-hydroxyl.

3.7

0

n.d.

n.d.

291

87

17.2

10.7

(3.1-6.0)

(0.2-0.6)**

(265–408)

(77–122)**

(14.3-21.4)

(9.1-13.9)**

20β-red.

6.8

3.2

7.7

4.7

103

45

26.1

18.5

(5.2-7.7)

(2.5-4.6)**

(7.1-9.9)

(3.4-6.9)**

(88–136)

(37–64)**

(23.4-31.7)

(17.0-25.7)*

u- 20β-red.

n.d.

n.d.

n.d.

n.d.

62.3

24.3

13.2

7.2

(55.5-99.3)

(18.1-33.0)**

(10.0-17.9)

(6.0-10.4)**

20α-red.

5.4

3.3

17.0

8.8

n.d.

n.d.

64.0

39.3

(56.6-78.4)

(30.5-53.6)**

(5.1-10.3)

(2.8-5.8)*

(14.5-20.7)

(6.7-15.7)**

u- 20α-red.

n.d.

n.d.

n.d.

n.d.

230

36

38.1

17.8

(198–363)

(36–85)**

(34.2-52.6)

(14.3-27.0)**

5β-red.

n.d.

n.d.

n.d.

n.d.

6.9

4.3

9.4

13.0

(5.6-8.1)

(3.2-4.9)**

(6.3-16.4)

(8.2-19.0)

5β-red. + 3α-red.

190

87

29.6

16.9

2009

1115

3665

2876

(172–271)

(69–141)**

(24.7-40.5)

(14.0-31.6)*

(1751–2404)

(1040–1672)**

(3059–4325)

(2552–3743)

5α-red. + 3α-red.

308

89

8.1

2.8

967

350

56.6

31.8

(308–470)

(89–211)**

(7.5-12.2)

(2.4-5.0)**

(912–1352)

(344–884)**

(53.6-89.7)

(25.9-61.7)*

5β-red. + 3α-red. + 20β-red.

16.2

9.1

61.8

31.6

111

81

476

575

(9.9-25.0)

(7.2-20.0)

(50.7-91.7)

(32.7-94.8)

(94–158)

(70–115)*

(401–597)

(512–798)

5α-red. + 3α-red. + 20β-red.

n.d.

n.d.

n.d.

n.d.

35.4

14.0

n.d.

n.d.

(26.4-41.3)

(13.1-26.7)**

5β-red. + 3α-red. + 20α-red.

n.d.

n.d.

n.d.

n.d.

40.4

16.9

979

967

(29.8-50.9)

(14.8-31.8)**

(830–1207)

(827–1264)

5α-red. + 3α-red. + 20α-red.

n.d.

n.d.

n.d.

n.d.

59.8

22.3

n.d.

n.d.

(47.3-77.9)

(22.0-51.3)**

  1. AIP patients exhibited a significant decrease for all cortisol metabolites whereas concentrations in the normal range were found for most the metabolites from cortisol precursors. Results are expressed as median (μg/g creatinine) and 95% confidence interval for mean (in brackets) *P < 0.05, **P < 0.01 vs. control, “u-“ metabolite excreted unconjugated.
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172