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Severe hypomineralization of the rib cage can lead to fatal respiratory manifestations.1,2

Defective bone mineralization in hypophosphatasia (HPP) can cause rachitic chest, resulting in decreased thoracic volume and pulmonary hypoplasia.1,3-5

Severe respiratory manifestations of hypophosphatasia range from progressive respiratory insufficiency to respiratory failure3-5
  • Respiratory failure is the most common cause of death in infants with hypophosphatasia1,3

In patients with hypophosphatasia, the need for invasive respiratory support usually results in a fatal outcome.8

Patients with hypophosphatasia with respiratory insufficiency often require intubation, mechanical ventilation, and/or oxygen support.8

In a natural history study of patients ≤5 years of age with hypophosphatasia (N=48)8,b:

64% (29/45) of patients with hypophosphatasia required respiratory support, most commonly invasive ventilation (mechanical ventilation via intubation or tracheostomy)
  • 95% (18/19) of patients who required invasive ventilation died

aRadiographs are from different patients with hypophosphatasia.

bData 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.8

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