Spontaneous Breathing Pattern as Respiratory Functional Outcome in Children with Spinal Muscular Atrophy (SMA).
Spontaneous Breathing Pattern as Respiratory Functional Outcome in Children with Spinal Muscular Atrophy (SMA).
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DOI：
10.1371/journal.pone.0165818
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发表时间：
2016
期刊：
影响因子：
3.7
通讯作者：
Baranello G
中科院分区：
文献类型：
--
作者：
LoMauro A;Aliverti A;Mastella C;Arnoldi MT;Banfi P;Baranello G
SMA的特征是进行性运动和呼吸肌无力。我们的目的是验证SMA儿童1）每种形式的特征是否为安静呼吸（QB）期间的特定呼吸和胸腹模式（VTA p）; 2）VTA p是否受沙丁胺醇治疗（目前建议作为标准治疗）或SMA自然史（NH）的影响; 3）整体运动障碍的严重程度与VTA p线性相关。采用光电体积描记法分析了32例SMA I型（SMA 1，最严重的形式）、51例II型（SMA 2，中度）、8例III型（SMA 3，最轻度）和20例健康（HC）患者的VTA p。在一个亚组的患者中测量肺量测定、咳嗽和运动功能。在SMA 1中，在仰卧位通过快速和浅呼吸以及反常的胸廓运动获得正常通气。在SMA 2中，由于呼吸频率增加（p<0.05），潮气量减少（p <0.05），继发于肺胸腔的不良贡献（%ΔVRC，P，p<0.001），坐位通气量在正常范围内。沙丁胺醇治疗对QB期间的VTA p无影响（p>0.05），而I型NH发生呼吸急促。运动功能量表与VTA p之间存在线性相关（p<0.001）。阴性或降低的%ΔVRC，P表示胸廓肌无力，是SMA 1和SMA 2自婴儿期起的显著特征。它的定量评估代表了一个非侵入性的，非意志性的指数，可以在所有的儿童，甚至uncollaborative，并提供有用的信息的行动，胸廓肌肉是已知的受疾病的影响。运动功能量表的低值表明运动障碍，但也呼吸功能。
SMA is characterised by progressive motor and respiratory muscle weakness. We aimed to verify if in SMA children 1)each form is characterized by specific ventilatory and thoraco-abdominal pattern(VTAp) during quiet breathing(QB); 2)VTAp is affected by salbutamol therapy, currently suggested as standard treatment, or by the natural history(NH) of SMA; 3)the severity of global motor impairment linearly correlates with VTAp. VTAp was analysed on 32 SMA type I (SMA1,the most severe form), 51 type II (SMA2,the moderate), 8 type III (SMA3,the mildest) and 20 healthy (HC) using opto-electronic plethysmography. Spirometry, cough and motor function were measured in a subgroup of patients. In SMA1, a normal ventilation is obtained in supine position by rapid and shallow breathing with paradoxical ribcage motion. In SMA2, ventilation is within a normal range in seated position due to an increased respiratory rate(p<0.05) with reduced tidal volume(p<0.05) secondary to a poor contribution of pulmonary ribcage(%ΔVRC,P, p<0.001). Salbutamol therapy had no effect on VTAp during QB(p>0.05) while tachypnea occurred in type I NH. A linear correlation(p<0.001) was found between motor function scales and VTAp. A negative or reduced %ΔVRC,P, indicative of ribcage muscle weakness, is a distinctive feature of SMA1 and SMA2 since infancy. Its quantitative assessment represents a non-invasive, non-volitional index that can be obtained in all children, even uncollaborative, and provides useful information on the action of ribcage muscles that are known to be affected by the disease.Low values of motor function scales indicate impairment of motor but also of respiratory function.
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影响因子：
1.7
作者：
Chng, SY;Wong, YQ;Goh, DY
通讯作者：
Goh, DY
影响因子：
9.6
作者：
Fauroux, Brigitte;Guilleinot, Nathahe;Lofaso, Frederic
通讯作者：
Lofaso, Frederic
影响因子：
3.3
作者：
Cala, SJ;Kenyon, CM;Rochester, DF
通讯作者：
Rochester, DF
影响因子：
2.8
作者：
Glanzman, A. M.;Mazzone, E.;Finkel, R. S.
通讯作者：
Finkel, R. S.
DOI：
10.1097/phm.0b013e31804a8505
发表时间：
2007-05-01
影响因子：
3
作者：
Bach, John R.;Saltstein, Katherine;Komaroff, Eugene
通讯作者：
Komaroff, Eugene