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Table 5 Median age at first reported occurrence of manifestations/events considered to contribute to HPP clinical burden, by age at occurrence of first HPP manifestation

From: Frequency and age at occurrence of clinical manifestations of disease in patients with hypophosphatasia: a systematic literature review

Manifestation/event Median age at first manifestation of HPP, years (range)
In utero
n = 30
Infancy
(0 to < 2 years)
n = 102
Childhood
(2 to < 10 years)
n = 78
Adolescence
(10 to < 18 years)
n = 9
Adulthood
(≥ 18 years)
n = 46
Respiratory symptoms 0.1 (0–0.8) 0.4 (0–1) N/A N/A N/A
Cranial abnormalities 1 (0–4) 0.7 (0–12) 5 (1–9) N/A N/A
Pain 1 (1–1) 34 (0–50) 40 (3–56) 42 (10–57) 51 (20–64)
Surgery 2 (0–3) 2 (0.1–30) 59 (1–59) 44 (12–44) 70 (32–83)
Gross motor/ambulation difficulties 2 (0.5–5) 22 (0–54) 18 (1–55) 19 (19–19) 89 (30–90)
Premature loss of teeth 3 (1–4) 3 (0–24) 5 (2–21) 14 (13–17) 25 (6–60)
One or more fractures 15 (0–15) 38 (0–53) 35 (2–55) 53 (15–53) 50 (19–75)
  1. HPP-related manifestations/events considered to contribute to the clinical burden of HPP were identified by four clinical experts who had experience in treating patients with HPP. Only patients who had been followed longitudinally for at least 1 year were included in this analysis
  2. HPP hypophosphatasia