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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.