A history of pulmonary insults in pediatric sickle cell patients was not significantly associated with reduced maximal inspiratory pressure (78.7 vs 89.9 cm H2O; p=0.4913) or expiratory pressure.
Observational (n=11)
Does a history of pulmonary insults correlate with reduced respiratory muscle strength and exercise capacity in pediatric patients with sickle cell disease?
In a small pilot study of pediatric sickle cell patients, respiratory muscle strength and exercise capacity did not significantly correlate with a history of pulmonary insults, though maximal expiratory pressure was lower than normative values.
Tasa de eventos absoluta: 78.7% vs 89.9%
valor p: p=0.4913
Abstract Rationale Pulmonary complications in sickle cell disease (SCD) significantly contribute to morbidity and impact respiratory muscle strength. Few studies have explored the impact of recurrent insults on respiratory muscle performance and functional capacity. Previous research showed that submaximal exercise capacity, measured by the six-minute walk test (6MWT), tends to be lower in pediatric SCD patients compared with age-matched healthy peers. Methods This pilot study aims to investigate the correlation between respiratory muscle strength (RMS) and submaximal exercise capacity in pediatric patients with SCD, with and without a history of pulmonary insults. Eleven pediatric patients were enrolled, including 8 with a history of pulmonary insults and 3 without. Results The median IQR age was 13 12,15 years, and 55% were male. The median IQR six-minute walk distance (6MWD) was 435.0 420.0, 450.0 m. FVC was abnormal in 22% of patients; FEV1 was abnormal in 44% of patients. Median IQR maximal inspiratory pressure (MIP) was 83.0 69.5, 102.3 cm H2O which was not found to differ from normative values (p = 0.5918, Wilcoxon signed rank test). The median IQR MIP was 78.7 61.4, 97.1 cm H2O in patients with pulmonary insults compared to 89.9 78.4, 102.3 cm H2O in those without (p = 0.4913, Wilcoxon rank sum test). Median IQR maximal expiratory pressure (MEP) was 67.8 54.7, 74.9 cm H2O which was significantly lower than normative values (p = 0.0068), Wilcoxon signed rank test). The median IQR MEP was 66.8 54.6,71.5 cm H2O in patients with pulmonary insults compared to 74.9 54.7, 108.8 cm H2O in those without (p =0.3799, Wilcoxon rank sum test). No significant correlations were found among MIP (Figure 1), MEP and 6MWD (all p 0.05) (Figure 2). Similarly, 6MWD was not found to correlate with FVC or FEV1. The direction and magnitude of the mean differences, particularly in MIP, MEP, and 6MWD, suggest a potential trend toward reduced inspiratory muscle strength and functional capacity among participants with prior pulmonary insults. Conclusion While these findings did not reach statistical significance in this small pilot cohort, they suggest that clinically meaningful differences may emerge with a larger sample size. These preliminary results underscore the potential value of incorporating RMS and 6MWT assessments into routine evaluation of pediatric SCD patients. Adequately powered studies are warranted to confirm these associations and determine whether early identification of respiratory muscle weakness can inform interventions aimed at improving long-term pulmonary and functional outcomes. This abstract is funded by: None
Garcia et al. (Fri,) conducted a observational in Pediatric sickle cell disease (n=11). History of pulmonary insults vs. No history of pulmonary insults was evaluated on Maximal inspiratory pressure (MIP) (p=0.4913). A history of pulmonary insults in pediatric sickle cell patients was not significantly associated with reduced maximal inspiratory pressure (78.7 vs 89.9 cm H2O; p=0.4913) or expiratory pressure.