Bone mineral density, pulmonary function, chronological age, and age at diagnosis in children and adolescents with cystic fibrosis

AbstractObjectiveTo assess bone mineral density in patients with cystic fibrosis (CF), and to correlate it with possible intervening variables.MethodsChildren and adolescents diagnosed with CF, aged 6 to 18 years, followed at the outpatient clinic were included in the study. First, demographic data were collected and, subsequently, patients underwent a spirometric test. All patients answered the Cystic Fibrosis Quality of Life Questionnaire (CFQ) and underwent the six-minute walk test (6MWT) and bone densitometry (DXA).ResultsA total of 25 CF patients were included, of which 56% were males. The mean age was 12.3±3.4 years; mean height was 149.2±14.4 cm; and mean weight was 44.4±13.9 kg. Most results on pulmonary function and bone mineral density (BMD) were within normal limits. The mean forced expiratory volume in one second (FEV) was 92.5±23.6 (% of predicted), mean forced vital capacity (FVC) was 104.4±21.3 (% of predicted), and1 mean BMD z-score was 0.1±1.0. BMD was moderately correlated with FEV (r = 0.43, p = 0.03) and FVC (r = 0.57, p = 0.003). Regarding chronological age and age at diagnosis, a moderate and inverse correlation was also found (r = −0.55, p = 0.004; r = −0.57, p = 0.003, respectively). However, no significant correlations were found with the data from CFQ, 6MWT, and body mass index.ConclusionMost patients had BMD within normal limits and presented a positive correlation with pulmonary function, as well as a negative correlation with chronological age and age at diagnosis

Densidade mineral óssea, função pulmonar, idade cronológica e idade de diagnóstico em crianças e adolescentes com fibrose cística Bone mineral density, pulmonary function, chronological age, and age at diagnosis in children and adolescents with cystic fibrosis

OBJETIVO: Avaliar a densidade mineral óssea de pacientes com fibrose cística (FC) e correlacioná-la com possíveis variáveis intervenientes. MÉTODOS: Foram incluídos crianças e adolescentes com diagnóstico clínico de FC, idade entre seis e dezoito anos, e em acompanhamento ambulatorial. Primeiramente, foram coletados os dados demográficos, para posterior realização do teste espirométrico. Todos os pacientes responderam ao questionário de qualidade de vida em FC (QFC) e realizaram o teste de caminhada dos seis minutos (TC6) e o exame de densitometria óssea (DXA). RESULTADOS: Foram incluídos 25 pacientes fibrocísticos, sendo 56% do sexo masculino. A média de idade foi de 12,3 ± 3,4 anos, altura de 149,2 ± 14,4 cm e peso de 44,4 ± 13,9 kg. A maioria dos dados de função pulmonar e de densidade mineral óssea (DMO) encontrou-se dentro dos limites de normalidade. A média do volume expiratório forçado no primeiro segundo (VEF1) foi de 92,5 ± 23,6 (% do previsto), capacidade vital forçada (CVF) de 104,4 ± 21,3 (% do previsto) e o escore z da DMO de 0,1 ± 1,0. A DMO correlacionou-se de forma moderada com o VEF1 (r = 0,43; p = 0,03) e com a CVF (r = 0,57; p = 0,003). Em relação à idade cronológica e à idade de diagnóstico, também foi encontrada uma correlação moderada e inversa (r = -0,55; p = 0,004 /r = -0,57; p = 0,003, respectivamente). Entretanto, não foram encontradas correlações significativas com os dados do QFC, TC6 e índice de massa corporal. CONCLUSÃO: A maioria dos pacientes avaliados apresenta DMO dentro dos limites de normalidade e possui correlação positiva com a função pulmonar e negativa com a idade cronológica e a idade de diagnóstico.<br>OBJECTIVE: To assess bone mineral density in patients with cystic fibrosis (CF), and to correlate it with possible intervening variables. METHODS: Children and adolescents diagnosed with CF, aged 6 to 18 years, followed at the outpatient clinic were included in the study. First, demographic data were collected and, subsequently, patients underwent a spirometric test. All patients answered the Cystic Fibrosis Quality of Life Questionnaire (CFQ) and underwent the six-minute walk test (6MWT) and bone densitometry (DXA). RESULTS: A total of 25 CF patients were included, of which 56% were males. The mean age was 12.3±3.4 years; mean height was 149.2±14.4 cm; and mean weight was 44.4±13.9 kg. Most results on pulmonary function and bone mineral density (BMD) were within normal limits. The mean forced expiratory volume in one second (FEV1) was 92.5±23.6 (% of predicted), mean forced vital capacity (FVC) was 104.4±21.3 (% of predicted), and mean BMD z-score was 0.1±1.0. BMD was moderately correlated with FEV1 (r = 0.43, p = 0.03) and FVC (r = 0.57, p = 0.003). Regarding chronological age and age at diagnosis, a moderate and inverse correlation was also found (r = -0.55, p = 0.004 ; r = -0.57, p = 0.003, respectively). However, no significant correlations were found with the data from CFQ, 6MWT, and body mass index. CONCLUSION: Most patients had BMD within normal limits and presented a positive correlation with pulmonary function, as well as a negative correlation with chronological age and age at diagnosis