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Respiratory muscle force and lung volume changes in a population of children with sickle cell disease

Bruce A Ong1, Jason Caboot, Abbas Jawad

  • 1Division of Pediatric Pulmonology, Tripler Army Medical Center, Honolulu, HI, USA.

British Journal of Haematology
|July 23, 2013
PubMed

Insights

Sickle cell disease (SCD) impacts respiratory function. This study found correlations between respiratory muscle strength and lung volumes in children with SCD, noting expiratory muscles may be more vulnerable to damage.

Area of Science:

  • Pulmonary Medicine
  • Pediatric Hematology
  • Respiratory Physiology

Background:

  • Sickle cell disease (SCD) is a genetic blood disorder that can affect multiple organ systems, including the respiratory system.
  • Respiratory complications are a significant cause of morbidity and mortality in pediatric SCD patients.
  • Understanding the relationship between respiratory muscle function and lung volumes is crucial for managing SCD.

Purpose of the Study:

  • To investigate the relationship between respiratory muscle force (maximal inspiratory pressure [MIP] and maximal expiratory pressure [MEP]) and lung volumes in children with sickle cell disease (SCD).
  • To compare inspiratory and expiratory muscle strength in this pediatric SCD population.
  • To explore potential mechanisms of respiratory compromise in SCD.

Main Methods:

  • Pulmonary function testing was conducted on 34 pediatric subjects with SCD (SCD-SS).
  • Measurements included MIP, MEP, spirometry (forced vital capacity [FVC]), and lung volumes (total lung capacity [TLC]).
  • Statistical analyses utilized Pearson's correlation coefficient and paired t-tests to assess relationships and differences.

Main Results:

  • MIP and MEP were significantly correlated with FVC and TLC in pediatric SCD patients.
  • Percent predicted MIP was significantly higher than percent predicted MEP (101.3% vs. 72.1%, P = 0.002).
  • A strong correlation was observed between MIP and MEP (r = 0.64, P < 0.0001).

Conclusions:

  • Respiratory muscle strength is directly related to lung volumes in children with SCD.
  • Expiratory muscles appear more susceptible to SCD-related damage than inspiratory muscles.
  • These findings suggest that respiratory muscle weakness may contribute to altered lung volumes in pediatric SCD.

Keywords:
muscle strengthpaediatricspulmonary functionsickle cell anaemia

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