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Respiratory

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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