Pulmonary function in children and adolescents with sickle cell disease: have we paid proper attention to this problem?

Abstract

Objective:To evaluate pulmonary function and functional capacity in children and adolescents with sickle cell disease. Methods:This was a cross-sectional study involving 70 children and adolescents (8-15 years of age) with sickle cell disease who underwent pulmonary function tests (spirometry) and functional capacity testing (six-minute walk test). The results of the pulmonary function tests were compared with variables related to the severity of sickle cell disease and history of asthma and of acute chest syndrome. Results:Of the 64 patients who underwent spirometry, 15 (23.4%) showed abnormal results: restrictive lung disease, in 8 (12.5%); and obstructive lung disease, in 7 (10.9%). Of the 69 patients who underwent the six-minute walk test, 18 (26.1%) showed abnormal results regarding the six-minute walk distance as a percentage of the predicted value for age, and there was a ≥ 3% decrease in SpO2 in 36 patients (52.2%). Abnormal pulmonary function was not significantly associated with any of the other variables studied, except for hypoxemia and restrictive lung disease. Conclusions:In this sample of children and adolescents with sickle cell disease, there was a significant prevalence of abnormal pulmonary function. The high prevalence of respiratory disorders suggests the need for a closer look at the lung function of this population, in childhood and thereafter.

Surfactant protein D as a marker for pulmonary complications in pediatric patients with sickle cell disease: Relation to lung function tests

“…These findings underscore the need for the recognition of asthma, as well as the importance of longitudinal follow‐up of pulmonary function, in this population . This may also indicate that several inflammatory mechanisms are involved in the genesis of airway obstruction in SCD …”

“…Recently, Vieira et al evaluated pulmonary function in 70 children and adolescents with SCD. Of the 64 patients who underwent spirometry, 8 (12.5%) showed restrictive lung disease and 7 (10.9%) had OLD.…”

Avaliação da função pulmonar em crianças e adolescentes com doenças reumáticas autoimunes

“…Complementando o estudo 5, a pesquisa de número 11 identifica um prejuízo na ventilação pulmonar nas crianças portadoras de asma, quando comparadas com crianças sem a enfermidade, o achado se concretiza através de exames de imagem como ressonância magnética de gás hiperpolarizado, estando o comprometimento associado a gravidade da asma (de caminhada de 6 minutos, foi utilizado nesta pesquisa, buscando correlacionar a dessaturação como diagnóstico precoce também para hipertensão pulmonar. O estudo identificou ainda que há relação entre anormalidade da função pulmonar e os valores de saturação, podendo indicar doença pulmonar crônica na presença de doença falciforme (Vieira et al, 2016).…”

How can anemia negatively influence gas exchange?

“… 8 It was also demonstrated that patients with PH (including those with postcapillary PH) have reduced exercise tolerance, despite having preserved or even increased cardiac output. In the study conducted by Vieira et al, 7 the lack of echocardiographic evaluation constitutes a limitation that precludes the identification of other factors associated with desaturation. In patients with high-output PH, increased pulmonary flow can lead to diffusion/perfusion mismatch enhanced by a rightward shift of the hemoglobin dissociation curve and associated V• //Q mismatch ( Figure 1 ).…”

“…In the previous issue of the JBP, Vieira et al 7 evaluated children and adolescents with sickle cell disease using spirometry and the six-minute walk test. It is of note that changes in gas exchange during exercise were common in the study sample: 52% had a significant decrease in SaO 2 as assessed by pulse oximetry (SpO 2 ) at the end of the six-minute walk test.…”