Brolatti Noemi, Trucco Federica, Ferretti Marta, Avanti Chiara, Tacchetti Paola, Panicucci Chiara, Striano Pasquale, Minetti Carlo, Bruno Claudio, Pedemonte Marina
Paediatric Neurology and Muscle Disease Unit, IRCCS Istituto Giannina Gaslini, 16147 Genova, Italy.
Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Care, University of Genova, 16132 Genova, Italy.
J Clin Med. 2023 Dec 7;12(24):7553. doi: 10.3390/jcm12247553.
Spinal muscular atrophy (SMA) type 1 is a severe condition leading to early respiratory failure. Treatment options have become available, yet respiratory outcome measures in SMA type 1 are limited. The aim of this study was to assess the respiratory pattern in SMA type 1 patients via structured light plethysmography (SLP). SLP measures the thoraco-abdominal movements by projecting a light grid onto the anterior thoraco-abdominal surface.
Cross-sectional study of consecutive children with SMA type 1. All children underwent motor assessment (CHOP-INTEND) and one-minute tidal breathing recording by SLP in supine position while self-ventilating in room air. The , the abdominal vs. chest contribution to breath (, ) and the severity of thoraco-abdominal paradox () were acquired.
Nineteen patients were included, median (IQR) age 2.3 years (1.4-7.9). Their respiratory pattern captured via SLP showed a raised median (IQR) respiratory rate per age of 33.5 bpm (26.6-41.7), a prevalent abdominal contribution to tidal breathing with median (IQR) 77% (68-90) vs. 23% (10-32). Thoracoabdominal paradox was detected (median 48.70°) and its severity correlated negatively with CHOP-INTEND (r -0.8, < 0.01).
SLP captured and quantified the respiratory features of infants and children with SMA type 1.
1型脊髓性肌萎缩症(SMA)是一种严重疾病,可导致早期呼吸衰竭。目前已有治疗方案,但1型SMA的呼吸结局指标有限。本研究旨在通过结构光体积描记法(SLP)评估1型SMA患者的呼吸模式。SLP通过将光栅投射到胸腹前表面来测量胸腹运动。
对连续的1型SMA儿童进行横断面研究。所有儿童均接受运动评估(CHOP-INTEND),并在室内空气中自主呼吸时,于仰卧位通过SLP记录一分钟的潮气呼吸。获取呼吸频率、腹部与胸部对呼吸的贡献(%、%)以及胸腹矛盾运动的严重程度()。
纳入19例患者,中位(IQR)年龄2.3岁(1.4 - 7.9岁)。通过SLP捕捉到的呼吸模式显示,按年龄计算的中位(IQR)呼吸频率升高,为33.5次/分钟(26.6 - 41.7),潮气呼吸中腹部贡献普遍,中位(IQR)为77%(68 - 90),而胸部为23%(10 - 32)。检测到胸腹矛盾运动(中位值为48.70°),其严重程度与CHOP-INTEND呈负相关(r = -0.8,<0.01)。
SLP捕捉并量化了1型SMA婴幼儿和儿童的呼吸特征。