Overall AHP Population | Type | Matched Population | ||||
---|---|---|---|---|---|---|
Acute Intermittent Porphyria | Hereditary Coproporphyria | Variegate Porphyria | ||||
Patients, n | 391 | 120 | 46 | 11 | 3910 | |
Sex, n (%) | Male | 177 (45.3) | 43 (35.8) | 27 (58.7) | 6 (54.5) | 1770 (45.3) |
Female | 214 (54.7) | 77 (64.2) | 19 (41.3) | 5 (45.5) | 2140 (54.7) | |
Age, years | Mean (SD) | 44.4 (23.1) | 42.6 (19.0) | 34.4 (27.1) | 56.1 (15.1) | 44.8 (23.4) |
Median | 44.0 | 39.5 | 25.5 | 57.0 | 45.0 | |
Min–max | 0–94 | 11–94 | 0–88 | 31–73 | 0–98 | |
Observation period (first visit~last visit), months | Mean (SD) | 35.2 (31.5) | 33.0 (31.1) | 36.1 (40.9) | 41.1 (41.1) | 38.3 (34.2) |
Median | 27.0 | 20.5 | 20.5 | 34.0 | 32.0 | |
Q1–Q3 | 5.0–59.0 | 5.0–54.5 | 3.0–54.0 | 2.0–72.0 | 4.0–64.0 | |
Min–max | 1–145 | 1–135 | 1–145 | 1–126 | 1–147 | |
Patients who had an acute attack that led to an ED visit or admission, n (%) | ED visit | 24 (6.1) | 15 (12.5) | 0 (0.0) | 0 (0.0) | — |
ED admission | 100 (25.6) | 34 (28.3) | 11 (23.9) | 2 (18.2) | — | |
Patients who experienced an acute attack, n (%) | Total | 112 (28.6) | 44 (36.7) | 11 (23.9) | 2 (18.2) | — |
1 | 71 (18.2) | 23 (19.2) | 10 (21.7) | 2 (18.2) | — | |
≥ 2 | 41 (10.5) | 21 (17.5) | 1 (2.2) | 0 (0.0) | — | |
Cumulative total | 207 | 94 | 12 | 2 | — | |
Mean (SD) | 0.5 (1.2) | 0.8 (1.6) | 0.3 (0.5) | 0.2 (0.4) | — | |
Min–max | 0–11 | 0–11 | 0–2 | 0–1 | — | |
Days to acute attack in patients with ≥ 2 acute attacks | n | 95 | 50 | 1 | 0 | — |
Mean (SD) | 281.3 (339.4) | 317.2 (337.7) | 21.0 (—) | — | — | |
Median | 183.0 | 250.0 | 21.0 | — | — | |
Q1–Q3 | 34.0–439.0 | 45.0–470.0 | 21.0–21.0 | — | — | |
Min–max | 1–1810 | 1–1499 | 21–21 | — | — |