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Table 3 History of manifestations of HPP in pediatric patients

From: Burden of disease in pediatric patients with hypophosphatasia: results from the HPP Impact Patient Survey and the HPP Outcomes Study Telephone interview

Manifestation

Patients, n (%)

Pain (any HPP-related pain)

51/ 59 (86.4)

 Joint paina

27/44 (61.4)

 Bone paina

23/44 (52.3)

 Muscle paina

9/44 (20.5)

Joint and muscle manifestations

 Muscle weakness

41/58 (70.7)

 Extremely flexible joints

21/57 (36.8)

Developmental manifestations

 Difficulty gaining weight

38/59 (64.4)

 Delayed walking

34/58 (58.6)

 Short stature

28/58 (48.3)

 Seizuresa

4/44 (9.1)

Skeletal abnormalities

 Bowing of legs or knock knees

34/59 (57.6)

 Abnormally shaped chest

32/59 (54.2)

 Abnormally shaped head

30/57 (52.6)

 Knock knees

23/59 (39.0)

 Bowing of legs

22/59 (37.3)

 Bowing of arms

12/59 (20.3)

Respiratory manifestations

 Pneumonia

16/59 (27.1)

 Difficulty breathinga

11/44 (25.0)

Fractures

 Non-vertebral fracturea

6/44 (13.6)

 Pseudofracturea

3/44 (6.8)

 Non-healing fracturea

1/44 (2.3)

  1. Data are expressed as the number of patients who reported a particular HPP manifestation divided by the total number of patients who responded to the question. All manifestations are captured by both the HIPS and HOST unless stated otherwise. Bold italicized numbers indicate the three most frequent symptoms
  2. a Assessed by HIPS only (n = 44)
  3. HIPS Hypophosphatasia Impact Patient Survey, HOST Hypophosphatasia Outcomes Study Telephone interview, HPP hypophosphatasia