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

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 wer...

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Main Authors: Márcio V.F. Donadio, Guilherme C. de Souza, Gabriele Tiecher, João P. Heinzmann-Filho, Taísa F. Paim, Patrícia X. Hommerding, Paulo J.C. Marostica
Format: Article
Language:English
Published: Elsevier 2013-03-01
Series:Jornal de Pediatria
Online Access:http://www.sciencedirect.com/science/article/pii/S0021755713000259
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author Márcio V.F. Donadio
Guilherme C. de Souza
Gabriele Tiecher
João P. Heinzmann-Filho
Taísa F. Paim
Patrícia X. Hommerding
Paulo J.C. Marostica
author_facet Márcio V.F. Donadio
Guilherme C. de Souza
Gabriele Tiecher
João P. Heinzmann-Filho
Taísa F. Paim
Patrícia X. Hommerding
Paulo J.C. Marostica
author_sort Márcio V.F. Donadio
collection DOAJ
description 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 (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. 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. Resumo: 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 44,41 ± 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 (VEF) 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 DMO1 correlacionou-se de forma moderada com o VEF (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. Keywords: Bone mineral density, Cystic fibrosis, Pulmonary function, Palavras-chave: Densidade mineral óssea, Fibrose cística, Função pulmonar
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spelling doaj.art-7a1871011df7459f9291880a512cbcb42022-12-22T02:22:34ZengElsevierJornal de Pediatria0021-75572013-03-01892151157Bone mineral density, pulmonary function, chronological age, and age at diagnosis in children and adolescents with cystic fibrosisMárcio V.F. Donadio0Guilherme C. de Souza1Gabriele Tiecher2João P. Heinzmann-Filho3Taísa F. Paim4Patrícia X. Hommerding5Paulo J.C. Marostica6PhD in Physiology. Professor, Programa de Pós-graduação em Pediatria e Saúde da Criança, Faculdade de Enfermagem, Nutrição e Fisioterapia, Pontifícia Universidade Católica do Rio Grande do Sul (PUCRS), Porto Alegre, RS, Brazil; Corresponding author.Physiotherapist, Faculdade de Enfermagem, Nutrição e Fisioterapia, PUCRS, Porto Alegre, RS, BrazilPhysiotherapist, Faculdade de Enfermagem, Nutrição e Fisioterapia, PUCRS, Porto Alegre, RS, BrazilPhD Candidate. Professor, Programa de Pós-graduação em Pediatria e Saúde da Criança, PUCRS, Porto Alegre, RS, BrazilPneumopediatrician, PUCRS, Porto Alegre, RS, BrazilPhD in Child and Adolescent Health. Professor, Physiotherapy, Centro Universitário Franciscano (UNIFRA), Santa Maria, RS, BrazilPhD in Pneumology. Professor, Faculdade de Medicina, Universidade Federal do Rio Grande do Sul (UFGRS), Porto Alegre, RS, BrazilObjective: 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 (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. 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. Resumo: 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 44,41 ± 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 (VEF) 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 DMO1 correlacionou-se de forma moderada com o VEF (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. Keywords: Bone mineral density, Cystic fibrosis, Pulmonary function, Palavras-chave: Densidade mineral óssea, Fibrose cística, Função pulmonarhttp://www.sciencedirect.com/science/article/pii/S0021755713000259
spellingShingle Márcio V.F. Donadio
Guilherme C. de Souza
Gabriele Tiecher
João P. Heinzmann-Filho
Taísa F. Paim
Patrícia X. Hommerding
Paulo J.C. Marostica
Bone mineral density, pulmonary function, chronological age, and age at diagnosis in children and adolescents with cystic fibrosis
Jornal de Pediatria
title Bone mineral density, pulmonary function, chronological age, and age at diagnosis in children and adolescents with cystic fibrosis
title_full Bone mineral density, pulmonary function, chronological age, and age at diagnosis in children and adolescents with cystic fibrosis
title_fullStr Bone mineral density, pulmonary function, chronological age, and age at diagnosis in children and adolescents with cystic fibrosis
title_full_unstemmed Bone mineral density, pulmonary function, chronological age, and age at diagnosis in children and adolescents with cystic fibrosis
title_short Bone mineral density, pulmonary function, chronological age, and age at diagnosis in children and adolescents with cystic fibrosis
title_sort bone mineral density pulmonary function chronological age and age at diagnosis in children and adolescents with cystic fibrosis
url http://www.sciencedirect.com/science/article/pii/S0021755713000259
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