Respiratory pattern in an adult population of dystrophic patients
Respiratory pattern in an adult population of dystrophic patients
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DOI：
10.1016/j.jns.2011.03.045
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发表时间：
2011-07-15
影响因子：
4.4
通讯作者：
Aliverti, A.
中科院分区：
文献类型：
--
作者：
D'Angelo, M. G.;Romei, M.;Aliverti, A.
我们研究了一大批患有慢性肌营养不良症如肢带型肌营养不良症的成年患者的呼吸功能和胸壁运动学（LGMD，n = 38），Becker肌营养不良症（BMD，n = 20）和面肩肱营养不良（FSHD，n = 30），通过标准肺量测定法和光电体积描记法，测量安静呼吸和缓慢肺活量操作期间的胸腹运动。在以LGMD和FSHD为特征的限制性肺综合征中，与健康受试者相比，在疾病的更晚期阶段存在几种不同的胸腹模式。在执行最大动作（如慢肺活量）期间，这些差异存在于坐位。一个全球性的呼吸（包括吸气和呼气）肌肉参与更明显的LGMD和FSHD比BMD患者，并观察到一个显着减少腹部的贡献在轮椅上的患者。结论：OEP技术能够显示FSHD和LGMD患者呼吸肌的轻度初始改变，这可能有助于功能和新的治疗策略的评估。(C)2011爱思唯尔有限公司版权所有。
We studied respiratory function and Chest Wall kinematics in a large population of adult patients affected by slow course muscular dystrophies such as Limb-Girdle Muscular Dystrophy (LGMD, n = 38), Becker Muscular Dystrophy (BMD, n = 20) and Facio-Scapulo Humeral Dystrophy (FSHD, n = 30), through standard spirometry and through the Optoelectronic Plethysmography, to measure the thoraco-abdominal motion during Quiet Breathing and Slow Vital Capacity maneuvers. Within the restrictive pulmonary syndrome characterizing LGMD and FSHD, several different thoraco-abdominal patterns compared to those of healthy subjects were present in the more advanced stages of the disease. These differences were present in the seated position, during the execution of a maximal maneuver such as Slow Vital Capacity. A global respiratory (both inspiratory and expiratory) muscle involvement was more pronounced in the LGMD and FSHD than in the BMD patients, and a significant reduction of abdominal contribution in wheelchair bound patients was observed. In conclusion, OEP technique is able to reveal mild initial modifications in the respiratory muscles in FSHD and LGMD patients, which could be helpful for functional and new therapeutic strategy evaluation. (C) 2011 Elsevier B.V. All rights reserved.