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Figure 1.

Thoraco-abdominal volume variations during spontaneous breathing.

Time courses of the volumes of the rib cage (sum of the pulmonary and abdominal rib cage), abdomen and total chest wall during ten seconds of consecutive breaths at rest in supine position in a representative OI type III patient (left panes) and a representative healthy control subject (right panels). The thick lines highlight a single breath.

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Figure 1 Expand

Figure 2.

Assessment of chest wall geometry and rib cage deformity by markers' projections.

Experimental set-up for the analysis of chest wall volumes via optoelectronic plethysmography in supine (a) and seated (b) position on a representative OI type III patient. c: schematic view of the markers on the transversal plane at the xiphosternal level, in which medio-lateral diameter (distance between markers A and B), antero-posterior diameter (distance between markers C and D), area (grey area) and transversal angle (angle formed between lines CE and CF) are shown; d: schematic view of the markers on the sagittal plane in which trunk height (distance between markers G and H), and sagittal sternal angle (enclosed within lines CL and CM) are shown.

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Figure 3.

Transversal and sagittal sections of OI type III, OI type IV and controls.

Markers' projection on the transversal (left) and sagittal (right) views of the markers (at xiphoid and sternal level, respectively) on three representative subjects: OI type III (top), OI type IV (middle) and healthy control (bottom).

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Table 1.

Osteogenesis imperfecta (OI) patients' and healthy controls characteristics.

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Table 2.

Osteogenesis imperfecta (OI) patients' pulmonary function test.

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Table 3.

Ventilatory pattern of osteogenesis imperfecta (OI) patients' and controls in seated and supine positions.

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Figure 4.

Thoraco-abdominal contribution to tidal volume.

Average values ± SE of pulmonary rib cage (top panels), abdominal rib cage (middle panels) and abdominal (bottom panels) percentage contribution to tidal volume in OI type III patients (black bars), OI type IV (grey bars) and control group (white bars) in supine (left panels) and seated (right panels) position. °°, °°° : p<0.01, p<0.001 (vs control); *, **, ***: p<0.05, p<0.01, p<0.001 (vs OI type IV); •,••,•••: p<0.01, p<0.001 (vs seated).

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Figure 5.

Thoraco-abdominal asynchronies.

Average values ± SE of phase angle ΦTA between pulmonary rib cage and abdomen (top panels), phase angle ΦRC between pulmonary rib cage and abdominal rib cage (middle panels) and labored breathing index (bottom panels) in OI type III patients (black bars), OI type IV (grey bars) and control group (white bars) in supine (left panels) and seated (right panels) position. °, °°, °°°: p<0.05, p<0.01, p<0.001 (vs control); *, **, ***: p<0.05, p<0.01, p<0.001 (vs OI type IV); ••,•••: p<0.01, p<0.001 (vs seated).

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