Peak nasal inspiratory flow in children and adolescents with sickle cell disease: a case-control study

SUMMARY OBJECTIVE: Sickle cell disease is the most frequent of the hereditary hemoglobinopathies and it presents multisystemic effects. A manifestation that is commonly found in sickle cell disease is upper airway obstruction, particularly adenotonsillar hypertrophy. This study aims to evaluate the peak nasal inspiratory flow measurements of children and adolescents with sickle cell disease. METHODS: This is a case-control study on children aged between 8 and 15 years who were diagnosed with sickle cell disease. Peak nasal inspiratory flow measurements were obtained from patients. RESULTS: A total of 279 patients were enrolled in this study, with 93 in the case group and 186 in the control group. The case group had an 82.83% chance of having lower peak nasal inspiratory flow values than the control group. In the case group, 75% of the peak nasal inspiratory flow values were in the lower standards, whereas in the control group, only 25% were in the lower standards. CONCLUSION: This study showed a high prevalence of reduced peak nasal inspiratory flow values in children with sickle cell disease and could certainly be incorporated into the day-to-day clinical evaluation of patients as a screening instrument.

Pulmonary function in children and adolescents with sickle cell disease: have we paid proper attention to this problem? / Função pulmonar em crianças e adolescentes com doença falciforme: temos dado atenção adequada a esse problema?

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.

RESUMO Objetivo: Avaliar a função pulmonar e a capacidade funcional em crianças e adolescentes com doença falciforme. Métodos: 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. Resultados: 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. Conclusões: 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.

Anemia falciforme e suas manifestações respiratórias / Pulmonary manifestations of sickle cell disease

A doença falciforme é a enfermidade monogênica mais comum no Brasil, sendo uma afecção sistêmica que potencialmente pode afetar vários órgãos e sistemas. O pulmão é um dos órgãos mais acometidos e as complicações na doença falciforme resultam em significante morbimortalidade na faixa pediátrica. Nesse contexto, o presente artigoapresenta as principais manifestações respiratórias da doença falciforme, em virtude da importância do diagnóstico precoce e da abordagem inicial por parte dos pediatras,especialmente da síndrome torácica aguda (STA), da hipertensão arterial pulmonar e da associação com a asma brônquica. O conhecimento por parte dos pediatras da abordagem adequada das manifestações respiratórias citadas no presente artigo de revisão é fundamental para o sucesso do tratamento, sendo também relevante o atendimento inicial adequado e o manejo do procedimento...

The Sickle Cell Disease is the most common inherited genetic disorder in Brazil being asystemic disease that can powerfully affect several organs and systems. The lungs are oneof the most affected and the consequences of the Sickle Cell Disease result in a significant morbid-mortality in pediatric patients and, in this context, the present article presents the main pulmonary manifestations of the stated disease. The article also considers the importance of an early diagnosis and the initial pediatric approach to these manifestations especiallyin: the Acute Chest Syndrome, Pulmonary Hypertension and the association of SickleCell Disease with Asthma. The acknowledgment by the Pediatricians of these respiratory manifestations is fundamental for the success of the treatment, and the initial assessment and adequate management of the procedure are also extremely relevant...