Skip to main content

Table 1 Demographic, biochemical characteristics and HRQoL scores of SA-AIP and L-AIP patients

From: Health impact of acute intermittent porphyria in latent and non-recurrent attacks patients

 

SA-AIP

L-AIP

p value

N (%)

27 (49.1 %)

28 (50.9 %)

Age (mean ± SD)

47.5 ± 15.6

41.3 ± 15.7

Age range (years)

[28–77]

[16–75]

Females, N (%)

20 (74.1 %)

12 (42.8 %)

Males, N (%)

7 (25.9 %)

18 (57.1 %)

c.669_698del30 carriersa, N (%)

20 (74.1 %)

24 (85.7 %)

BMI (mean ± SD)

26.93 ± 4.4

28.17 ± 4.93

0.3436

Biochemical assessments (mean ± SD)

   

eGFR (ml/min per 1.73m2)

73.16 ± 27.3

105.07 ± 19.2

< 0.0001*

AST (U/L)

28.19 ± 6.5

22.31 ± 8.6

0.0082*

ALT (U/L)

27.04 ± 14.4

25.3 ± 10.8

0.6119

GGT (U/L)

20.24 ± 18.5

26.52 ± 17.6

0.3047

Hb in males (g/dl)

13.54 ± 1.73

14.4 ± 1.36

0.0563

Ferritin in males (ng/ml)

57.59 ± 64.26

80.46 ± 65.08

0.2373

Hb in females (g/dl)

12.64 ± 0.89

13.34 ± 0.95

0.1270

Ferritin in females (ng/ml)

53.44 ± 37.5

43.73 ± 68.7

0.6513

U-PBG/Crb (µmol /mmol)

17.09 ± 15.93

1.32 ± 2.29

0.0001*

U-ALA/Crb (µmol /mmol)

9.86 ± 6.76

6.06 ± 4.35

0.1333

HRQoL scores (mean ± SD)

   

EQ-5D-5L index

0.809 ± 0.16

0.926 ± 0.14

0.0497*

EQ-5D-5L VAS

75.44 ± 16.16

86.78 ± 15.78

0.1353

  1. SA-AIP sporadic attack AIP, L-AIP latent AIP, N total number of participants, BMI body mass index, eGFR estimated glomerular filtrate rate, AST amino aspartate transferase, ALT alanine aminotransferase, GGT gamma glutamyl transferase, Hb haemoglobin; ferritin; U-PBG/Cr urinary porphobilinogen/creatinine ratio, U-ALA/Cr urinary aminolevulinic acid/creatinine ratio, VAS visual analogue scale
  2. *P value ≤ 0.05 adjusted by age and gender. Laboratory reference ranges: eGFR > 60mL/min/1.73m2; AST 5–35 U/L; ALT 10–40 U/L; GGT 7–50 U/L; Hb 12–16 g/dL; Ferritin 12–300 ng/mL; U-PBG/Cr < 1.5 µmol /mmol; U-ALA/Cr < 3.8 µmol /mmol
  3. aFounder mutation in the Region of Murcia. bOnly evaluated in 51 participants