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

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. Objetivo:: Avaliar a funcao pulmonar e a capacidade funcional em criancas e adolescentes com doenca falciforme. Metodos:: Estudo transversal com 70 criancas e adolescentes com doenca falciforme (8-15 anos), submetidos a testes de funcao respiratoria (espirometria) e de capacidade funcional (teste de caminhada de seis minutos). Os resultados da avaliacao da funcao pulmonar foram comparados com variaveis relacionadas a gravidade da doenca falciforme e a presenca de historia de asma e de sindrome toracica aguda. Resultados:: Dos 64 pacientes submetidos a espirometria, 15 (23,4%) apresentaram resultados alterados: disturbio ventilatorio restritivo, em 8; (12,5%) e disturbio respiratorio obstrutivo, em 7 (10,9%). Dos 69 pacientes submetidos ao teste de caminhada de seis minutos, 18 (26,1%) apresentaram resultados alterados na distância em % do previsto para a idade, e houve uma queda ≥ 3% na SpO2 em 36 (52,2%) dos pacientes. Nao houve associacoes significativas entre funcao pulmonar alterada e as outras variaveis analisadas, exceto para hipoxemia e disturbio ventilatorio restritivo. Conclusoes:: Observou-se uma significativa prevalencia de alteracoes na funcao pulmonar nesta amostra de criancas e adolescentes com doenca falciforme. A elevada prevalencia de disturbios ventilatorios sugere a necessidade de um olhar mais atento a funcao pulmonar desde a infância nessa populacao.