Respiratory muscular strength in children with mucopolysacaridosis: comparison with predictive equations.

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 cmH2O) and MEP (60.09±22.3 cmH2O) 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.

Effects of 12 weeks of inspiratory muscle training and whole body vibration on the inflammatory profile, BDNF and muscular system in pre-frail elderly women: A randomized controlled trial.

AIM: to investigate the effects of the whole body vibration (WBV) and inspiratory muscle training (IMT) on the inflammatory profile and in muscle mass and strength in pre-frail older women. METHODS: this study was a randomized double-blind trial. Forty two older women aged 60-80 years were randomly allocated to IMT + WBV (G1), IMTsham + WBV (G2) or Sham groups (G3). During 12 weeks G1 received both trainings, whereas G2 received WBV alone and G3 received IMT with a low fixed load and were positioned at the vibratory platform without therapeutic effect. Participants were evaluated before and after the intervention for the following outcomes: Brain-derived neurotrophic factor (BDNF) and inflammatory biomarkers (IB), respiratory (RT) and quadriceps thickness (QT) and diaphragmatic mobility (DM) using muscle ultrasound, body composition (BC) using a bioelectrical impedance scale and inspiratory muscle strength (IMS). RESULTS: after the training, G1 (114.93 ± 21.29) improved IMS (p<0.005) compared with G2 (91.29 ± 23.10) and G3 (85.21 ± 27.02). There was also a significant improve on time of the DM (p<0.001) and RT (p=0.006) for G1 (8.59 ± 3.55 and 11.11 ± 12.66) compared with G2 (1.05 ± 3.09 and 1.10 ± 10.60) and G3 (0.40 ± 2.29 and -1.85 ± 7.45). BDNF, IB, QT and BC were similar between groups. CONCLUSIONS: IMT associated with WBV is effective to improve in increasing IMS, RT and DM in pre-frail older women. However, these interventions do not modify BDNF, IB, QT or BC in this population.