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成骨不全症患者肋骨形状与肺功能之间的关联。

Association between ribs shape and pulmonary function in patients with Osteogenesis Imperfecta.

作者信息

Sanchis-Gimeno Juan A, Lois-Zlolniski Stephanie, María González-Ruiz José, Palancar Carlos A, Torres-Tamayo Nicole, García-Martínez Daniel, Aparicio Luis, Perez-Bermejo Marcelino, Blanco-Perez Esther, Mata-Escolano Federico, Llidó Susanna, Torres-Sanchez Isabel, García-Río Francisco, Bastir Markus

机构信息

Giaval Research Group, Department of Anatomy and Human Embryology, Faculty of Medicine, University of Valencia, Av. Blasco Ibanez, 15, 46010 Valencia, Spain.

Departamento de Paleobiología, Museo Nacional de Ciencias Naturales (CSIC), José Gutiérrez Abascal 2, 28006 Madrid, Spain.

出版信息

J Adv Res. 2019 Oct 22;21:177-185. doi: 10.1016/j.jare.2019.10.007. eCollection 2020 Jan.

DOI:10.1016/j.jare.2019.10.007
PMID:32071786
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7015465/
Abstract

The aim of the present study was to test the hypothesis that ribs shape changes in patients with OI are more relevant for respiratory function than thoracic spine shape. We used 3D geometric morphometrics to quantify rib cage morphology in OI patients and controls, and to investigate its relationship with forced vital capacity (FVC) and forced expiratory volume in 1 s (FEV1), expressed as absolute value and as percentage of predicted value (% pred). Regression analyses on the full sample showed a significant relation between rib shape and FEV1, FVC and FVC % pred whereas thoracic spine shape was not related to any parameter. Subsequent regression analyses on OI patients confirmed significant relations between dynamic lung volumes and rib shape changes. Lower FVC and FEV1 values are identified in OI patients that present more horizontally aligned ribs, a greater antero-posterior depth due to extreme transverse curve at rib angles and a strong spine invagination, greater asymmetry, and a vertically short, thoraco-lumbar spine, which is relatively straight in at levels 1-8 and shows a marked kyphosis in the thoraco-lumbar transition. Our research seems to support that ribs shape is more relevant for ventilator mechanics in OI patients than the spine shape.

摘要

本研究的目的是验证以下假设

与胸椎形状相比,成骨不全症(OI)患者的肋骨形状变化对呼吸功能更为重要。我们使用三维几何形态测量学来量化OI患者和对照组的胸廓形态,并研究其与用力肺活量(FVC)和第1秒用力呼气量(FEV1)的关系,以绝对值和预测值百分比(%pred)表示。对全部样本的回归分析显示,肋骨形状与FEV1、FVC及FVC%pred之间存在显著关系,而胸椎形状与任何参数均无关联。随后对OI患者进行的回归分析证实,动态肺容量与肋骨形状变化之间存在显著关系。在肋骨更水平排列、因肋骨角处极端横向弯曲导致前后径更大、脊柱明显内陷、不对称性更大以及胸腰椎垂直较短(在第1至8节相对较直,在胸腰段过渡处出现明显驼背)的OI患者中,FVC和FEV1值较低。我们的研究似乎支持,在OI患者中,肋骨形状对通气力学的影响比脊柱形状更为重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/895b/7015465/d70818111073/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/895b/7015465/84863c39aa7c/ga1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/895b/7015465/c1e09fbdacce/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/895b/7015465/caf16fdcd027/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/895b/7015465/18739d6ac07a/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/895b/7015465/5649c8b43fdb/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/895b/7015465/d70818111073/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/895b/7015465/84863c39aa7c/ga1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/895b/7015465/c1e09fbdacce/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/895b/7015465/caf16fdcd027/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/895b/7015465/18739d6ac07a/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/895b/7015465/5649c8b43fdb/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/895b/7015465/d70818111073/gr5.jpg

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本文引用的文献

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Osteogenesis Imperfecta: A Pediatric Orthopedic Perspective.成骨不全症:小儿骨科视角
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A multicenter study to evaluate pulmonary function in osteogenesis imperfecta.一项评估成骨不全症肺功能的多中心研究。
成骨不全成年患者的肺功能:一项队列研究。
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Respiratory function of children and adolescents with osteogenesis imperfecta: respiratory muscle strength, forced vital capacity, and peak expiratory flow.成骨不全症患儿和青少年的呼吸功能:呼吸肌力量、用力肺活量和呼气峰流量。
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Lung and chest wall volume during vital capacity manoeuvre in Osteogenesis Imperfecta.成骨不全症患者肺活量动作时的肺和胸壁容量。
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