Ribcage deformity and the altered breathing pattern in children with osteogenesis imperfecta.

An altered breathing pattern in severe OI is present since childhood and it worsens with age. This is caused by the combination of pectus carinatum, brittle ribs and spinal deformity that put the ribcage muscles in mechanical disadvantage. These results suggest that in severe OI the assessment of the respiratory function should start in early childhood in order to try to reduce the incidence of premature death.