title: Pulmonary function in children and adolescents with sickle cell disease: have we paid proper attention to this problem?
abstract: To evaluate pulmonary function and functional capacity in children and adolescents with sickle cell disease.
  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.
  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.
  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.
  Avaliar a função pulmonar e a capacidade funcional em crianças e adolescentes com doença falciforme.
  Estudo transversal com 70 crianças e adolescentes com doença falciforme (8-15 anos), submetidos a testes de função respiratória (espirometria) e de capacidade funcional (teste de caminhada de seis minutos). Os resultados da avaliação da função pulmonar foram comparados com variáveis relacionadas à gravidade da doença falciforme e à presença de história de asma e de síndrome torácica aguda.
  Dos 64 pacientes submetidos à espirometria, 15 (23,4%) apresentaram resultados alterados: distúrbio ventilatório restritivo, em 8; (12,5%) e distúrbio respiratório 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. Não houve associações significativas entre função pulmonar alterada e as outras variáveis analisadas, exceto para hipoxemia e distúrbio ventilatório restritivo.
  Observou-se uma significativa prevalência de alterações na função pulmonar nesta amostra de crianças e adolescentes com doença falciforme. A elevada prevalência de distúrbios ventilatórios sugere a necessidade de um olhar mais atento à função pulmonar desde a infância nessa população.
authors.affiliation: . Fundação Hemominas, Belo Horizonte (MG) Brasil.
authors.forename: Ana Karine
authors.initials: AK
authors.lastname: Vieira
authors.affiliation: . Departamento de Pediatria, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte (MG) Brasil.
authors.forename: Cristina Gonçalves
authors.initials: CG
authors.lastname: Alvim
authors.affiliation: . Grupo de Pneumologia Pediátrica, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte (MG) Brasil.
authors.forename: Maria Cristina Marquez
authors.initials: MC
authors.lastname: Carneiro
authors.affiliation: . Departamento de Pediatria, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte (MG) Brasil.
authors.forename: Cássio da Cunha
authors.initials: CD
authors.lastname: Ibiapina
mesh_terms: D056586:Acute Chest Syndrome / Q000453:epidemiology / Q000503:physiopathology; D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000503:physiopathology*; D001249:Asthma / Q000453:epidemiology / Q000503:physiopathology; D001938:Brazil / Q000453:epidemiology; D002648:Child; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008168:Lung / Q000503:physiopathology*; D008297:Male; D015999:Multivariate Analysis; D012129:Respiratory Function Tests; D012140:Respiratory Tract Diseases / Q000453:epidemiology; D013147:Spirometry; D000070857:Walk Test