From: Clinical profiles of treated and untreated adults with hypophosphatasia in the Global HPP Registry
Characteristic | Adults (overall)a N = 398 | Pediatric-onset HPP n = 213 | Adult-onset HPP n = 141 |
---|---|---|---|
Sex, n (%) | |||
Male | 109 (27.4) | 68 (31.9%) | 34 (24.1) |
Female | 289 (72.6) | 145 (68.1%) | 107 (75.9) |
Age at baseline, years | |||
Mean (SD) Median (minimum, maximum) | 47.5 (14.9) 48.1 (18.3, 81.2) | 44.8 (15.1) 43.8 (18.3, 77.9) | 51.4 (13.9) 51.9 (19.5, 79.8) |
Age at first HPP manifestation, years | |||
n Mean (SD) | 275 22.1 (20.9) | 161 8.6 (11.5) | 114 41.2 (15.6) |
Median (minimum, maximum) | 14.4 (0.0, 75.3) | 5.0 (0.0, 72.0) | 39.0 (18.0, 75.3) |
Age at HPP diagnosis, years | |||
n Mean (SD) Median (minimum, maximum) | 349 41.3 (19.3) 42.3 (0.0, 78.9) | 184 35.2 (21.0) 36.0 (0.0, 77.0) | 131 48.9 (13.9) 49.0 (18.7, 76.6) |
Received ERT, n (%) | 119 (29.9%) | 114 (53.5%) | 2 (1.4%) |
Baseline ALP, (U/L)b | |||
n Mean (SD) Median (minimum, maximum) | 344 26.4 (13.8) 24.0 (2.0, 98.0) | 176 25.1 (15.0) 22.5 (2.0, 98.0) | 128 27.5 (12.8) 25.0 (7.0, 98.0) |