USA
Register

Today

For Valuable Updates and Resources

  2. Home
  3. About HPP
  4. Systemic and functional consequences
  5. Skeletal

Skeletal

 
Clinical snapshot

Over time, systemic consequences can impact quality of life.1

86% (n=44) of pediatric patients reported pain.2,a
 
Pain impairs mobility, function, and activities of daily living.1-3
 

Skeletal manifestations can have devastating lifelong impacts for patients with hypophosphatasia (HPP).1,4-14

Patients with hypophosphatasia may experience4,6-15

Severe hypomineralization
Progressive skeletal demineralization
Hypophosphatasia-induced rickets
Bowing
Skeletal deformities
Bone pain
 
Osteomalacia
Frequent, nonhealing, nontraumatic, or recurrent fractures
Short stature
Multiple surgical interventions
 
 

Hypophosphatasia-induced rickets can have a lasting impact on mobility.4,7

Hypophosphatasia-induced rickets can lead to short stature, delayed walking, and waddling gait7,16,17
83% (5/6) of pediatric patients (5 to 15 years of age) had bowing of the long bones due to hypophosphatasia-induced rickets3,b
 

In patients with hypophosphatasia, the increasing number of recorded fractures over time suggests progressive morbidity as patients age.1,15,19

23% (7/30) of infants and young children experience fractures20,c
34% (11/32) of pediatric patients (5 to 15 years of age) experience fractures21,d
86% (108/125) of adult patients report fractures1,e
Adults with hypophosphatasia suffer from a large number of fractures (12.9 [mean] fractures [SD±19.0, range: 1-100])1,2,e

Devastating skeletal manifestations can progressively worsen across a lifetime in patients with hypophosphatasia.1,4,15,18

aThe HPP Impact Patient Survey (HIPS) was an Internet questionnaire that queried demographics, hypophosphatasia-related illness history, disease progression, and health-related quality of life. One hundred thirty-three patients participated.2

bData from a retrospective, noninterventional substudy of children (n=6) enrolled in a larger retrospective, multinational, noninterventional natural history study of childhood hypophosphatasia (N=32).3

cData from a noninterventional, retrospective chart review study designed to understand the natural history of 48 patients with severe perinatal- and infantile-onset hypophosphatasia ≤5 years of age.20

dData from a retrospective, multinational, noninterventional natural history study of childhood hypophosphatasia (N=32).21

eHIPS/HPP Outcomes Survey Telephone (HIPS/HOST) combined data from an Internet questionnaire and telephone survey that queried demographics, hypophosphatasia-related illness history, disease progression, and health-related quality of life. One hundred eighty-four patients participated (59 children, 125 adults).1,2

Keep Me Updated
I want to know the latest news and
information about Hypophosphatasia.

By clicking "SUBMIT" I agree to the Privacy Notice and Terms of Use.