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

Bárbara Bernardo Figueirêdo1,2, Cyda Reinaux1, Taylline G Oliveira1

  • 1Department of Physical Therapy, Federal University of Pernambuco, Pernambuco, Brazil.

Minerva Pediatrics
|July 8, 2024
PubMed

Insights

Children with mucopolysaccharidoses (MPS) have significantly lower maximal inspiratory and expiratory pressures (MIP and MEP) than healthy children. Reference equations for healthy children are not suitable for MPS patients; longitudinal monitoring of absolute pressures is recommended.

Area of Science:

  • Pediatric Pulmonology
  • Rare Diseases
  • Metabolic Disorders

Background:

  • Mucopolysaccharidoses (MPS) are rare genetic metabolic disorders.
  • These conditions often lead to impaired respiratory function and potential respiratory failure.
  • Maximal inspiratory and expiratory pressures (MIP and MEP) are key indicators of respiratory muscle strength.

Purpose of the Study:

  • To compare MIP and MEP in children with MPS against predicted values for healthy children.
  • To evaluate the applicability of existing predictive equations for respiratory muscle strength in the MPS population.
  • To assess respiratory muscle function in children diagnosed with MPS.

Main Methods:

  • A cross-sectional study involving 22 children with MPS and 22 healthy controls.
  • Evaluation of chest deformity, MIP, and MEP using a digital manometer.
  • Spirometry was performed to assess overall lung function.
  • Comparison of measured MIP and MEP with five different predictive equations and a healthy control group.
  • Kappa coefficient was used to assess agreement between measured and predicted respiratory muscle weakness.

Main Results:

  • Children with MPS exhibited significantly lower MIP (37.14±36.23 cmH2O) and MEP (60.09±22.3 cmH2O) compared to healthy controls (MIP: 91.45±35.60; MEP: 95.73±22.38).
  • Chest deformities such as pectus carinatum (45.5%) and pectus excavatum (36.4%) were prevalent in the MPS group.
  • Only MEP predictive equations by Tomalak et al. showed a non-significant trend towards closeness (P=0.09) with MPS children's MEP.
  • Weak agreement was found for inspiratory weakness between absolute and predicted values using Tomalak et al. and Domenèch-Clar et al. equations (k=0.35).
  • Moderate agreement was observed for MEP using all predictive equations in the MPS group.

Conclusions:

  • Standard reference equations for healthy children are inappropriate for normalizing MIP and MEP data in children with MPS.
  • Longitudinal monitoring of absolute MIP and MEP values, alongside lung volumes, is a more appropriate approach for managing respiratory function in MPS patients.
  • This study highlights the need for specialized assessment of respiratory muscle strength in pediatric MPS populations.
Abstract

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