Abstract：
BACKGROUND: Mucopolysaccharidoses (MPS) are rare metabolic diseases that impair respiratory function leading to respiratory failure. This study aimed to compare maximal inspiratory and expiratory pressures (MIP and MEP) obtained in children with MPS and compare with predicted values from previous studies involving healthy children.
METHODS: This is a cross-sectional study, in which the chest deformity was evaluated; MIP, MEP through digital manometer, and lung function through spirometry. MIP and MEP were compared with five different predict equations and with a control group of healthy children. Agreement between respiratory muscle weakness regarding absolute values of MIP and MEP in relation to predictive values by the equations included in the study were assessed by Kappa coefficient.
RESULTS: MPS group was composed of 22 subjects. 45.5% had pectus carinatum, 36.4% pectus excavatum, and presented lower MIP (37.14±36.23 cmH2 O) and MEP (60.09±22.3 cmH2 O) compared with control group (22 healthy subjects) (MIP: 91.45±35.60; MEP: 95.73±22.38). Only the MEP equations proposed by Tomalak et al. were close to those found in our MPS children (P=0.09). In the MPS group it was observed a weak agreement between inspiratory weakness through absolute and predicted values in only two equations: Tomalak et al. and Domenèch-Clar et al. (for both: k=0.35, P value =0.03); and for MEP a moderate agreement was found using all predictive equations.
CONCLUSIONS: In MPS children MRP data should not be normalized using the reference equations for healthy ones, is more coherent to longitudinally follow absolute pressures and lung volumes in this group.
METHODS: This is a cross-sectional study, in which the chest deformity was evaluated; MIP, MEP through digital manometer, and lung function through spirometry. MIP and MEP were compared with five different predict equations and with a control group of healthy children. Agreement between respiratory muscle weakness regarding absolute values of MIP and MEP in relation to predictive values by the equations included in the study were assessed by Kappa coefficient.
RESULTS: MPS group was composed of 22 subjects. 45.5% had pectus carinatum, 36.4% pectus excavatum, and presented lower MIP (37.14±36.23 cmH
CONCLUSIONS: In MPS children MRP data should not be normalized using the reference equations for healthy ones, is more coherent to longitudinally follow absolute pressures and lung volumes in this group.
摘要：
背景：粘多糖（MPS）是罕见的代谢性疾病，会损害呼吸功能，导致呼吸衰竭。这项研究旨在比较MPS儿童获得的最大吸气和呼气压力（MIP和MEP），并与先前涉及健康儿童的研究的预测值进行比较。
方法：这是一项横断面研究，其中评估了胸部畸形；MIP，MEP通过数字压力计，和肺功能通过肺活量测定。将MIP和MEP与五个不同的预测方程以及健康儿童的对照组进行比较。通过Kappa系数评估了呼吸肌无力关于MIP和MEP绝对值与预测值之间的一致性。
结果：MPS组由22名受试者组成。45.5%的人有胸骨，36.4%漏斗胸,与对照组（22名健康受试者）相比，MIP（37.14±36.23cmH2 O）和MEP（60.09±22.3cmH2 O）较低（MIP：91.45±35.60；MEP：95.73±22.38）。只有Tomalak等人提出的MEP方程。与我们的MPS儿童中发现的接近(P=0.09)。在MPS组中，仅在两个方程式中观察到通过绝对值和预测值的吸气无力之间的弱一致性：Tomalak等。和Domench-Clar等人。（对于两者：k=0.35，P值=0.03）；对于MEP，使用所有预测方程都发现了适度的一致性。
结论：在MPS儿童中，MRP数据不应使用健康参考方程进行归一化，在该组中，纵向跟随绝对压力和肺容量更一致。
方法：这是一项横断面研究，其中评估了胸部畸形；MIP，MEP通过数字压力计，和肺功能通过肺活量测定。将MIP和MEP与五个不同的预测方程以及健康儿童的对照组进行比较。通过Kappa系数评估了呼吸肌无力关于MIP和MEP绝对值与预测值之间的一致性。
结果：MPS组由22名受试者组成。45.5%的人有胸骨，36.4%漏斗胸,与对照组（22名健康受试者）相比，MIP（37.14±36.23cmH
结论：在MPS儿童中，MRP数据不应使用健康参考方程进行归一化，在该组中，纵向跟随绝对压力和肺容量更一致。
