Abstract
Background: Mucopolysaccharidosis type VI (MPS VI) is considered a rare disease in the world which presents impairment of respiratory muscle strength. So far, it is unknown predictive values of inspiratory muscle pressure (PImax) in this specific population.
Aim: To compare standardization measures of PImax using four predictive equations in children with MPS type VI.
Method: This cross-sectional study included 10 children with MPS VI. PImax was acquired according ATS recommendations, measured by the digital manuvacuometer (MV300-Globalmed, Brazil). Standardization of the results was made using predicted values from the following equations: Wilson et al. 1984, United Kingdom (W); Tomalak et al. 2002, Poland (T); Domenèch-Clar et al. 2003, Spain (D) and Heinzmann et al. 2012, Brazil(H). Repeated measures ANOVA with Bonferroni post hoc test was used to compare observed and derived PImax from predictive equations.
Results: 10 children with MPS VI were studied, aged 10±3.86 years old, with a prevalence of males (58.33%). Pimax was -33.1±6.09cmH2O and significantly differed lower than the predict values for healthy children (F= 25.36; p-value <0.01). The result of the equations consisted: Tomalak: -65.33 ± 4.00cmH2O; Wilson: -60.37±2.22cmH2O; Domenèch-Clar: -60.39 ±3.41cmH2O; and Heinzmann: -66.72 ± 3.64cmH2O. Considering the use of these predict values, our sample was 51.12% ±21.54, 45.55% ± 31.22, 47.03% ±23.83 and 50.76%±28.15 bellow of the predict for T, W, D and H, respectively.
Conclusion: The standardization measures of PImax using predictive values from four equations for healthy children tends to overestimate inspiratory muscle strength for MPS VI.
Footnotes
Cite this article as: European Respiratory Journal 2019; 54: Suppl. 63, PA913.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2019