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Utility of maximum inspiratory and expiratory pressures as a screening method for respiratory insufficiency in slowly progressive neuromuscular disorders
Neuromuscular Disorders ( IF 2.7 ) Pub Date : 2020-08-01 , DOI: 10.1016/j.nmd.2020.06.009
Stephan Wenninger 1 , Kristina Stahl 1 , Corinna Wirner 1 , Krisztina Einvag 1 , Simone Thiele 1 , Maggie C Walter 1 , Benedikt Schoser 1
Affiliation  

The aim of this study was to assess whether different cut-offs of maximum inspiratory and/or expiratory pressure (MIP/MEP) are valuable screening parameters to detect restrictive respiratory insufficiency. Spirometry, MIP, MEP and capillary blood gas analysis were obtained from patients with confirmed neuromuscular disorders. We calculated regression analysis, sensitivity, specificity and predictive values. We enrolled 29 patients with myotonic dystrophy type 1 (DM1), 19 with late-onset Pompe disease (LOPD), and 24 with spinal muscular atrophy type 3. Moderate to high reduction in manometry was exclusively found in LOPD and DM1 patients. Significant associations were found between manometry and spirometry. Highest adjusted r2 was found for MIP % predicted and forced vital capacity (FVC) % predicted. Manometry predicted abnormal FVC and forced expiratory volume 1 s (FEV1). MEP > 80 cmH2O predicted normal FVC and FEV1, regardless of cut-off values. MIP and MEP did not positively predict alterations in capillary blood gas analysis. Disease-specific cut-offs of manometry did not increase the prediction rate of patients with abnormal FVC and FEV1. Predicted values should be calculated for a more comprehensive interpretation of manometry results. MIP and MEP can serve as a screening parameter for patients with neuromuscular disorders, but parallel testing of both MIP and MEP needs to be performed to increase the positive prediction probability across disease groups.

中文翻译:

使用最大吸气和呼气压力作为缓慢进展性神经肌肉疾病呼吸功能不全的筛查方法

本研究的目的是评估最大吸气和/或呼气压力 (MIP/MEP) 的不同临界值是否是检测限制性呼吸功能不全的有价值的筛选参数。肺活量测定法、MIP、MEP 和毛细血管血气分析来自确诊的神经肌肉疾病患者。我们计算了回归分析、敏感性、特异性和预测值。我们招募了 29 名患有 1 型肌强直性营养不良 (DM1)、19 名患有迟发性庞贝病 (LOPD) 和 24 名患有 3 型脊髓性肌萎缩症的患者。仅在 LOPD 和 DM1 患者中发现了中度至高度的测压降低。在测压法和肺活量测定法之间发现了显着的关联。发现 MIP % 预测值和用力肺活量 (FVC) % 预测值的最高调整 r2。测压预测异常 FVC 和用力呼气量 1 s (FEV1)。MEP > 80 cmH2O 预测正常 FVC 和 FEV1,无论临界值如何。MIP 和 MEP 没有积极预测毛细血管血气分析的变化。特定疾病的测压截止值并未增加 FVC 和 FEV1 异常患者的预测率。应计算预测值以更全面地解释测压结果。MIP 和 MEP 可以作为神经肌肉疾病患者的筛查参数,但需要对 MIP 和 MEP 进行平行测试,以增加跨疾病组的阳性预测概率。MIP 和 MEP 没有积极预测毛细血管血气分析的变化。特定疾病的测压截止值并未增加 FVC 和 FEV1 异常患者的预测率。应计算预测值以更全面地解释测压结果。MIP 和 MEP 可以作为神经肌肉疾病患者的筛查参数,但需要对 MIP 和 MEP 进行平行测试,以增加跨疾病组的阳性预测概率。MIP 和 MEP 没有积极预测毛细血管血气分析的变化。特定疾病的测压截止值并未增加 FVC 和 FEV1 异常患者的预测率。应计算预测值以更全面地解释测压结果。MIP 和 MEP 可以作为神经肌肉疾病患者的筛查参数,但需要对 MIP 和 MEP 进行平行测试,以增加跨疾病组的阳性预测概率。
更新日期:2020-08-01
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