Finkel Richard S, Weiner Daniel J, Mayer Oscar H, McDonough Joseph M, Panitch Howard B
Divisions of Neurology, Nemours Children's Hospital, Orlando, Florida.
Pediatr Pulmonol. 2014 Dec;49(12):1234-42. doi: 10.1002/ppul.22997. Epub 2014 Apr 29.
To determine the feasibility and safety of respiratory muscle function testing in weak infants with a progressive neuromuscular disorder.
Respiratory insufficiency is the major cause of morbidity and mortality in infants with spinal muscular atrophy type I (SMA-I).
Tests of respiratory muscle strength, endurance, and breathing patterns can be performed safely in SMA-I infants. Useful data can be collected which parallels the clinical course of pulmonary function in SMA-I.
An exploratory study of respiratory muscle function testing and breathing patterns in seven infants with SMA-I seen in our neuromuscular clinic. Measurements were made at initial study visit and, where possible, longitudinally over time.
We measured maximal inspiratory (MIP) and transdiaphragmatic pressures, mean transdiaphragmatic pressure, airway occlusion pressure at 100 msec of inspiration, inspiratory and total respiratory cycle time, and aspects of relative thoracoabdominal motion using respiratory inductive plethysmography (RIP). The tension time index of the diaphragm and of the respiratory muscles, phase angle (Φ), phase relation during the total breath, and labored breathing index were calculated.
Age at baseline study was 54-237 (median 131) days. Reliable data were obtained safely for MIP, phase angle, labored breathing index, and the invasive and non-invasive tension time indices, even in very weak infants. Data obtained corresponded to the clinical estimate of severity and predicted the need for respiratory support.
The testing employed was both safe and feasible. Measurements of MIP and RIP are easily performed tests that are well tolerated and provide clinically useful information for infants with SMA-I.
确定对患有进行性神经肌肉疾病的虚弱婴儿进行呼吸肌功能测试的可行性和安全性。
呼吸功能不全是Ⅰ型脊髓性肌萎缩症（SMA-Ⅰ）婴儿发病和死亡的主要原因。
可以在SMA-Ⅰ型婴儿中安全地进行呼吸肌力量、耐力和呼吸模式测试。可以收集到与SMA-Ⅰ型肺功能临床病程相似的有用数据。
对在我们神经肌肉诊所就诊的7名SMA-Ⅰ型婴儿的呼吸肌功能测试和呼吸模式进行探索性研究。在初次研究就诊时进行测量，并尽可能随时间纵向测量。
我们使用呼吸感应体积描记法（RIP）测量最大吸气压力（MIP）和跨膈压、平均跨膈压、吸气100毫秒时的气道阻塞压、吸气和总呼吸周期时间以及相对胸腹运动的各个方面。计算膈肌和呼吸肌的张力时间指数、相角（Φ）、整个呼吸过程中的相位关系以及呼吸费力指数。
基线研究时的年龄为54-237（中位数131）天。即使在非常虚弱的婴儿中，也能安全地获得关于MIP、相角、呼吸费力指数以及有创和无创张力时间指数的可靠数据。获得的数据与严重程度的临床评估相符，并预测了呼吸支持的需求。
所采用的测试既安全又可行。MIP和RIP测量是易于进行的测试，耐受性良好，并为SMA-Ⅰ型婴儿提供临床有用信息。
