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Table 1 Baseline demographics and clinical characteristics of HPP in adults (aged ≥ 18 years)

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)

  1. ALP Alkaline phosphatase, ERT enzyme replacement therapy, HPP hypophosphatasia, SD standard deviation
  2. aIncludes 44 patients with missing or unknown age of onset
  3. bMeasures may have been obtained during adulthood (age ≥ 18 years) or childhood (age < 18 years)