Lung function in infants with cystic fibrosis.

Beardsmore, Caroline; Bar‐Yishay, Ephraim; Maayan, Ch.; Yahav, Yaacov; Katznelson, D; Godfrey, Simon

doi:10.1136/thx.43.7.545

Cited by 52 publications

(38 citation statements)

References 25 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The results of this trial will contribute valuable information regarding detectable treatment effects and feasibility of infant PFTs in the current era. Similar to our findings, an elevated FRC measured by plethysmography or gas dilution has been reported in infants with CF in several small studies (18,(34)(35)(36). Only one small prior study has evaluated fractional lung volumes in infants with CF; our findings are consistent with that study (18).…”

Section: Discussionsupporting

confidence: 83%

Multicenter Evaluation of Infant Lung Function Tests as Cystic Fibrosis Clinical Trial Endpoints

Davis¹,

Rosenfeld²,

Kerby³

et al. 2010

Am J Respir Crit Care Med

119

View full text Add to dashboard Cite

Rationale: The conducting of clinical trials in infants with cystic fibrosis (CF) has been hindered by lack of sensitive outcome measures. Objectives: To evaluate safety, feasibility, and ability to detect abnormalities in lung function of serial pulmonary function tests (PFTs) in infants with CF. Methods: Multicenter observational study using a commercial device, rigorous training, ongoing quality control, and over-reading of data by an independent panel. Raised volume rapid thoracoabdominal compression technique and plethysmography were performed at enrollment and at 6 and 12 months, with an additional 1-month reproducibility visit. Measurements and Main Results: A total of 342 procedures were performed in 100 infants with CF at 10 centers. FRC measurements were acceptable at a higher proportion of study visits (89%) than raised volume (72%) or fractional lung volume (68%) measurements. Average Z scores for many parameters differed significantly from historical control values. Mean (95% confidence interval) Z scores were: 20.52 (20.78 to 20.25) for forced expiratory flow at 75% (FEF 75 ) for FVC; 1.92 (1.39-2.45) for FRC; 1.22 (0.68-1.76) for residual volume; 0.87 (0.60-1.13) for FRC/total lung capacity; and 0.66 (0.27-1.06) for residual volume/total lung capacity. For future multicenter clinical trials using infant PFTs as primary endpoints, minimum detectable treatment effects are presented for several sample sizes. Conclusions: In this 10-center study, key PFT measures were significantly different in infants with CF than in historical control subjects. However, infant PFTs do not yet appear ready as primary efficacy endpoints for multicenter clinical trials, particularly at inexperienced sites, based on acceptability rates, variability, and potentially large sample sizes required to detect reasonable treatment effects.

show abstract

Section: Discussionsupporting

confidence: 83%

Multicenter Evaluation of Infant Lung Function Tests as Cystic Fibrosis Clinical Trial Endpoints

Davis¹,

Rosenfeld²,

Kerby³

et al. 2010

Am J Respir Crit Care Med

119

View full text Add to dashboard Cite

show abstract

“…A gasometria arterial conseguiu detectar alteração na função pulmonar em 81,25% dos indivíduos, e a espirometria, em56,25% (com 50% de sobreposição), ao contrário do que ocorre com as crianças com fibrose cística de até 1 ano de idade, que ainda possuem a função pulmonar preservada 12,13 . Contudo, ao passar de 5 anos de acompanhamento clínico desses pacientes, apenas ¼ deles não apresentam um declínio da função pulmonar 14 .…”

Section: Discussionunclassified

Study of lung involvement in patients with cystic fibrosis

Dornelas¹,

Fernandes²,

Galvão³

et al. 2000

J Pediatr (Rio J)

View full text Add to dashboard Cite

Objective: To characterize the involvement of the respiratory apparatus of patients with cystic fibrosis in order to obtain a comprehensive view of their pulmonary picture.Methods: Data were obtained retrospectively from the medical records of 16 patients with cystic fibrosis; arterial gas and spirometry data were obtained prospectively for the same patients, who were not in an acute pulmonary situation. The patients were subjects of both sexes aged 6 years or older who were followed up at the Pediatrics Outpatient Clinic of the University Hospital, Faculty of Medicine of Ribeirão Preto, USP.Results: Median patient age was 114 months (9 years and 6 months) ranging between 72 -360 months, and 68.75% were males. Productive cough was the most frequent symptom observed in 75% of the population studied. All patients had positive sputum culture obtained at least one year before, with Pseudomonas aeruginosa being detected in 81.25% of the cases. Arterial gases revealed some abnormalities in 81.25% of the patients and spirometry revealed abnormalities in 56.25%.Conclusion: All patients presented at least one type of pulmonary alteration. Measurement of arterial gases detected a larger number of patients with altered pulmonary function than did spirometry, but the two examinations complemented each other for a good evaluation of pulmonary function.J. pediatr. (Rio J.). 2000; 76(4): 295-299: cystic fibrosis, pulmonary function, arterial gas, spirometry. ResumoObjetivo: O trabalho teve por finalidade caracterizar o acometimento do aparelho respiratório de pacientes com fibrose cística a fim de termos um panorama do quadro pulmonar dos mesmos.Métodos: Avaliação de dados obtidos, retrospectivamente, de prontuários médicos e de resultados prospectivos de gasometria arterial e espirometria de 16 pacientes com fibrose cística fora do quadro de doença pulmonar agudizada, com idade maior ou igual a 6 anos, de ambos os sexos, seguidos no ambulatório de Pediatria do Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto -USP-.Resultados: A mediana de idade dos pacientes foi de 114 meses (9 anos e 6 meses) com variação de 72 -360 meses. 68,75% são do sexo masculino. A tosse produtiva foi o sintoma mais freqüente (75% da população estudada). 100% dos indivíduos possuem cultura de escarro positiva de longa data (pelo menos 1 ano), sendo 81,25% por Pseudomonas aeruginosa. A gasometria arterial revelou alguma anormalidade em 81,25% dos indivíduos e a espirometria em 56,25% dos indivíduos.Conclusão: Todos os pacientes analisados apresentam pelo menos um tipo de alteração pulmonar. A gasometria arterial detectou maior número de pacientes com alteração na função pulmonar do que a espirometria, porém os dois exames complementam-se para uma boa avaliação funcional pulmonar.

show abstract

“…In others, pulmonary function remains normal in the first year of life. (5) The clinical course of the pulmonary aspect of the disease is influenced by chronic inflammation of the airways and by bacterial infections, which predispose to air trapping and modify the respiratory system compliance, increasing respiratory effort and decreasing respiratory muscle strength. (4,6) This last factor may be evaluated through pressure manometry, a noninvasive method that is simple and practical.…”

Section: Introductionmentioning

confidence: 99%

Influência do método Reequilíbrio Toracoabdominal sobre a força muscular respiratória de pacientes com fibrose cística

et al. 2006

View full text Add to dashboard Cite

OBJETIVO: Avaliar o efeito do método Reequilíbrio Toracoabdominal na força dos músculos respiratórios de pacientes com fibrose cística, acompanhados no Ambulatório de Fibrose Cística da Universidade Católica de Brasília. MÉTODOS: A amostra, constituída de 29 fibrocísticos, foi caracterizada com base em dados antropométricos, genéticos e de colonização bacteriana. Espirometria, manovacuometria e antropometria foram realizadas antes e depois do tratamento fisioterapêutico, no qual se utilizou o método Reequilíbrio Toracoabdominal, duas vezes por semana, durante quatro meses. RESULTADOS: Houve aumento da pressão inspiratória máxima e da pressão expiratória máxima após o tratamento fisioterapêutico em todos os pacientes, naqueles sem distúrbio ventilatório obstrutivo e naqueles com distúrbio ventilatório obstrutivo leve (p < 0,05). Foi encontrada correlação positiva entre a idade e a pressão expiratória máxima para a maioria dos grupos. A pressão inspiratória máxima só apresentou correlação positiva com a idade no grupo com distúrbio ventilatório obstrutivo leve (p = 0,012; r = 0,817). Para o sexo feminino e para o grupo sem distúrbio ventilatório obstrutivo houve correlação negativa entre a pressão expiratória máxima e a colonização por Pseudomonas aeruginosa (p = 0,036; r = -0,585). CONCLUSÃO: Para os fibrocísticos avaliados, o método Reequilíbrio Toracoabdominal aumentou a força dos músculos respiratórios, o que reafirma a importância do tratamento fisioterapêutico para estes pacientes.

show abstract

Lung function in infants with cystic fibrosis.

Cited by 52 publications

References 25 publications

Multicenter Evaluation of Infant Lung Function Tests as Cystic Fibrosis Clinical Trial Endpoints

Multicenter Evaluation of Infant Lung Function Tests as Cystic Fibrosis Clinical Trial Endpoints

Study of lung involvement in patients with cystic fibrosis

Influência do método Reequilíbrio Toracoabdominal sobre a força muscular respiratória de pacientes com fibrose cística

Contact Info

Product

Resources

About