患有粘膜多糖症的儿童的呼吸肌肉强度:与预测方程进行比较
Bárbara Bernardo Figueirêdo1,2, Cyda Reinaux1, Taylline G Oliveira1
1Department of Physical Therapy, Federal University of Pernambuco, Pernambuco, Brazil.
Minerva pediatrics
|July 8, 2024
概括
患有粘多糖症 (MPS) 的儿童的最大吸气和呼吸压力 (MIP和MEP) 比健康儿童要低得多. 健康儿童的参考方程不适合MPS患者;建议对绝对压力的纵向监测.
科学领域:
- 儿科肺病学 儿科肺病学
- 罕见疾病 罕见疾病
- 代谢障碍 代谢障碍 代谢障碍
背景情况:
- 粘多糖症 (MPS) 是一种罕见的遗传代谢疾病.
- 这些情况往往导致呼吸功能受损和潜在的呼吸衰竭.
- 最大吸入和呼吸压力 (MIP和MEP) 是呼吸肌肉强度的关键指标.
研究的目的:
- 将MPS儿童的MIP和MEP与健康儿童的预测值进行比较.
- 评估MPS人群中呼吸肌肉强度现有的预测方程的适用性.
- 在被诊断为MPS的儿童中评估呼吸道肌肉功能.
主要方法:
- 一项涉及22名MPS儿童和22名健康对照者的横截面研究.
- 胸部形,MIP和MEP的评估,使用数字压力计.
- 进行了螺旋计,以评估整体肺功能.
- 用五种不同的预测方程和一个健康的对照组比较测量的MIP和MEP.
- 卡帕系数被用来评估测量和预测的呼吸肌弱之间的一致性.
主要成果:
- 与健康对照组相比,患有MPS的儿童的MIP (37.14±36.23cmH2O) 和MEP (60.09±22.3cmH2O) 显著较低 (MIP: 91.45±35.60;MEP: 95.73±22.38).
- 在MPS组中,胸部变形,如胸部卡里纳图 (45.5%) 和胸部挖掘 (36.4%) 在MPS组中普遍存在.
- 只有MEP的预测方程由Tomalak等. 与MPS儿童MEP的亲密关系 (P=0.09) 的趋势不显著.
- 在使用Tomalak等的绝对值和预测值之间的启发性弱点中发现了较弱的一致性. 和多梅内奇-克拉尔等人. 的方程 (k=0.35).
- 对于使用MPS组中所有预测方程的MEP,观察到适度的协议.
结论:
- 健康儿童的标准参考方程不适合在患有MPS的儿童中规范MIP和MEP数据.
- 纵向监测绝对MIP和MEP值,以及肺体积,是管理MPS患者呼吸功能的更合适的方法.
- 这项研究强调了对儿科MPS人群呼吸肌肉强度的专业评估的需要.
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