Table 2 Baseline characteristics of acute hepatic porphyria sub-groups compared to the reference population (1992 to 2017, 18–67 years of age)
From: Sick leave, disability, and mortality in acute hepatic porphyria: a nationwide cohort study
Characteristics | Hospitalised (n = 42) | Non-hospitalised (n = 104) | Asymptomatic (n = 101) | Unclassified (n = 46) | Reference population (n = 5,021,163) | |||||
|---|---|---|---|---|---|---|---|---|---|---|
n/ mean | %/(95% CI) | n / mean | %/(95% CI) | n / mean | %/(95% CI) | n / mean | %/(95% CI) | n / mean | %/(95% CI) | |
Sex – female | 29 | 69.1 | 63 | 60.6 | 48 | 47.2 | 25 | 54.4 | 2,427,746 | 48.4 |
Age in years (study start) | 29.0 | (24.9, 33.0) | 34.7 | (32.2, 37.2) | 27.0 | (24.6, 29.4) | 32.8 | (28.7, 36.9) | 29.0 | (29.0, 29.0) |
Education attainment | ||||||||||
Unspecified | 0 | 0.0 | 0 | 0.0 | 0 | 0.0 | 0 | 0.0 | 636,494 | 12.7 |
Primary/middle edu (1–10 yrs) | 8 | 19.1 | 22 | 21.2 | 20 | 19.8 | 17 | 37.0 | 1,113,270 | 22.2 |
Intermediate edu (11–13 yrs) | 18 | 42.9 | 42 | 40.4 | 44 | 43.6 | 19 | 41.3 | 1,827,443 | 36.4 |
Tertiary edu (14+ yrs) | 16 | 38.1 | 40 | 38.5 | 37 | 36.6 | 10 | 21.7 | 1,443,956 | 28.8 |
Biochemical characteristics | ||||||||||
PBG | 24.5 | (14.4, 34.5) | 11.8 | (8.9, 14.7) | 4.1 | (2.6, 5.5) | 7.8 | (3.9, 11.7) | ||
ALA | 24.5 | (11.3, 37.7) | 10.9 | (8.6, 13.3) | 4.9 | (4.0, 5.9) | 6.8 | (4.0, 9.9) | ||
PBG > 4*upper reference limit | 33 | 78.6 | 68 | 65.4 | 24 | 23.8 | 15 | 32.6 | ||
PBG > 10*upper reference limit | 26 | 61.9 | 39 | 37.5 | 18 | 17.8 | 13 | 28.3 | ||
Smoking | ||||||||||
Never / have quit | 24 | 57.1 | 66 | 63.5 | 74 | 73.3 | ||||
Occasionally / daily | 16 | 38.1 | 35 | 33.7 | 20 | 19.8 | ||||
Not specified | 2 | 4.8 | 3 | 2.9 | 7 | 6.9 | ||||
Drinks alcohol > 3 / week | ||||||||||
Yes | 1 | 2.4 | 1 | 1.0 | 2 | 2.0 | ||||
No | 26 | 61.9 | 77 | 74.0 | 61 | 60.4 | ||||
Not specified | 15 | 35.7 | 26 | 25.0 | 38 | 37.6 | ||||
Body mass index (BMI) | 23.7 | (22.5, 24.9) | 25.7 | (24.8, 26.6) | 23.4 | (22.5, 24.4) | ||||