Table 3 HPP-related disease history
n/N (%) | ||
|---|---|---|
Categorya: Symptom/sign | Children (n = 121) | Adults (n = 148) |
Rheumatic | 25/119 (21.0) | 115/137 (83.8) |
Calcific periarthritis | 0 | 9 (6.6) |
Chondrocalcinosis | 0 | 6 (4.4) |
Fibromyalgia | 2 (1.7) | 13 (9.5) |
Painb | 23 (19.3) | 102 (74.5) |
Pseudogout | 2 (1.7) | 8 (5.8) |
Skeletal | 53/120 (44.2) | 66/137 (48.2) |
Bone deformity | 39 (32.5) | 18 (13.1) |
Pseudofractures | 1 (0.8) | 9 (6.6) |
Recurrent and poorly healing fractures | 5 (4.2) | 50 (36.5) |
Rickets-like changes (by radiograph) | 22 (18.3) | 8 (5.8) |
Orthopedic procedures and therapies | 26/120 (21.7) | 62/139 (44.6) |
Muscular | 23/119 (19.3) | 51/137 (37.2) |
Abnormal gait | 17/91c (18.7) | 28/137 (20.4) |
Weakness | 15/119 (12.6) | 42/137 (30.7) |
Premature loss of deciduous teeth | 53/110d (48.2) | 34/137 (24.8) |
Renal/metabolic | 28/119 (23.5) | 22/137 (16.1) |
Hypercalcemia | 15 (12.6) | 6 (4.4) |
Hyperphosphatemia | 7 (5.9) | 6 (4.4) |
Kidney stones | 2 (1.7) | 7 (5.1) |
Nephrocalcinosis | 11 (9.2) | 5 (3.6) |
Neurologic | 32/120 (26.7) | 16/137 (11.7) |
Craniosynostosis | 12 (10.0) | 4 (2.9) |
Developmental delay | 16 (13.3) | 3 (2.2) |
Increased intracranial pressure | 2 (1.7) | 1 (0.7) |
Seizures | 11 (9.2) | 12 (8.8) |
Failure to thrive | 32/120 (26.7) | 7/137 (5.1) |
Respiratory support | 22/118 (18.6) | 6/138 (4.3) |
Invasive ventilation | 18 (15.3) | 0 |
CPAP/BiPAP | 7 (5.9) | 5 (3.6) |
Supplemental oxygen | 11 (9.3) | 2 (1.4) |