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

Pediatric Pulmonology
A LoMauroA Aliverti

Abstract

Osteogenesis Imperfecta (OI) is a genetic disease characterized by bones fragility and progressive deformity. Life expectancy is reduced in the non-lethal most severe type III form before the age of 10 years. The main cause of death in OI is respiratory insufficiency resulting from impaired thoracic function worsened by ribcage deformity and scoliosis. We used opto-electronic plethysmography to study chest geometry, the ventilatory, and the thoraco-abdominal pattern at rest in supine position in children younger than 10 years. Radiographic measurements were used to describe spinal deformity. Eight severe OI (sOI), seven affected by other moderate forms (mOI), and nine healthy controls (CTR) were analyzed. sOI were characterized by Pectus carinatum (sternal angle: 165.2°, CTR: 183.1°; P < 0.01), rapid and shallow breathing (RSBi: 267.4 L-1 min-1 , CTR: 150.7 L-1 min-1 ; P < 0.05) and reduced pulmonary rib cage contribution to tidal volume (5.1%, CTR: 14.6%; P < 0.001) that evolved with age approaching the paradoxical inspiratory inward movement previously found in adults. mOI showed almost normal ventilatory pattern (RSBi: 189.2-1 min-1 ) and absence of sternal deformity (sternal angle: 176.8°). Platyspondyly and kyphosis were c...Continue Reading

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Citations

Jul 31, 2019·Current Opinion in Pediatrics·Vittoria RossiRonit Marom
Feb 6, 2020·American Journal of Physiology. Lung Cellular and Molecular Physiology·Milena DimoriRoy Morello
Jan 19, 2019·The Journal of Asthma : Official Journal of the Association for the Care of Asthma·Priscila Figueiredo Dos Santos SilvaEmanuel Sávio Cavalcanti Sarinho
Nov 7, 2019·Current Opinion in Pediatrics·Vittoria RossiRonit Marom

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