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Table 3 Proportion of patients with manifestations/events considered to contribute to the clinical burden of HPP

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

 

Overall

n (%)

n = 265

In utero

n (%)

n = 30

Infancy

n (%)

n = 102

Childhood

n (%)

n = 78

Adolescence

n (%)

n = 9

Adulthood

n (%)

n = 46

Patients with at least one manifestation/event

248 (94)

25 (83)

96 (94)

78 (100)

9 (100)

40 (87)

Manifestation/event

Premature loss of teeth

142 (53.5)

3 (10.0)

63 (61.8)

58 (74.4)

3 (33.3)

14 (30.4)

One or more fractures

95 (35.8)

12 (40.0)

19 (18.8)

27 (34.6)

3 (33.3)

34 (73.9)

Multiple fracturesa

60 (22.6)

2 (6.7)

13 (12.7)

19 (24.4)

2 (22.2)

24 (52.2)

Pain

89 (33.6)

1 (3.3)

25 (24.5)

32 (41.0)

6 (66.7)

25 (54.3)

Gross motor/ambulation difficulties

82 (30.9)

7 (23.3)

41 (40.2)

18 (23.1)

1 (11.0)

15 (32.6)

Cranial abnormalities

66 (24.9)

12 (40.0)

44 (43.1)

10 (12.8)

0 (0.0)

0 (0.0)

Surgery

64 (24.2)

4 (13.3)

30 (29.4)

8 (10.1)

3 (33.3)

17 (37.0)

Dental otherb

37 (14.0)

0 (0.0)

23 (22.5)

11 (14.1)

0 (0.0)

3 (6.5)

Hospitalizationsc

33 (12.5)

1 (3.3)

18 (17.6)

6 (7.7)

2 (22.2)

6 (13.0)

Respiratory symptomsc

19 (7.2)

5 (16.7)

14 (13.7)

0 (0.0)

0 (0.0)

0 (0.0)

Nephrocalcinosis

11 (4.2)

2 (6.7)

8 (7.8)

1 (1.3)

0 (0.0)

0 (0.0)

Seizures

7 (2.6)

3 (10.0)

3 (2.9)

0 (0.0)

0 (0.0)

1 (2.2)

Renal insufficiency

4 (1.5)

0 (0.0)

1 (1.0)

3 (3.8)

0 (0.0)

0 (0.0)

Psychological

3 (1.1)

0 (0.0)

0 (0.0)

1 (1.3)

0 (0.0)

2 (4.3)

  1. HPP-related manifestations and events considered to contribute to the clinical burden of HPP were identified by four clinical experts who had experience in treating patients with HPP. Bold italicized numbers indicate the three most frequent symptoms for each age group. Only patients who had been followed longitudinally for at least 1 year were included in this analysis
  2. aMultiple fractures defined as three or more. bDental other includes delayed eruption of teeth, abnormality of the dentition, severe periodontitis, and atrophy of alveolar ridges. cOnly patients who survived at least 1 year were included in this analysis
  3. HPP hypophosphatasia