Lung function and nutritional status in children with cystic fibrosis and primary ciliary dyskinesia

Walker, Woolf T.; Harris, Amanda; Rubbo, Bruna; Keenan, Victoria; Friend, Amanda; Payne, Sarah; Maddison, J.; Yonge, C.; Crocker, Catherine L.; McGinnity, Tricia; Curbishley, T.; Gove, Kerry; Phillips, Sian; Legg, Julian; Evans, Hazel; Lucas, Jane S.; Connett, Gary

doi:10.1183/13993003.congress-2016.pa3128

Cited by 3 publications

(3 citation statements)

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“…Can early diagnosis prevent disease progression? This has been suggested by previous studies, [14,[28][29][30] while the Danish study and ours found no association. [8] As we still lack robust evidence for the best treatments for PCD, management is based on experience from other lung diseases such as CF.…”

Section: Discussionsupporting

confidence: 67%

Lung function from school age to adulthood in primary ciliary dyskinesia

Halbeisen

Goutaki

Pedersen

et al. 2021

Preprint

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Primary ciliary dyskinesia (PCD) presents with symptoms early in life and the disease course may be progressive, but longitudinal data on lung function are scarce. This multinational cohort study describes lung function trajectories in children, adolescents, and young adults with PCD. We analysed data from 486 patients with repeated lung function measurements obtained between the age of 6 and 24 years from the international PCD Cohort (iPCD) and calculated z-scores for forced expiratory volume in the first second (FEV1) and forced vital capacity (FVC) using the Global Lung Function Initiative 2012 references. We described baseline lung function and change of lung function over time and described their associations with possible determinants in mixed-effects linear regression models. Overall, FEV1 and FVC z-scores declined over time (average crude annual FEV1 decline was -0.07 z-scores) but not at the same rate for all patients. FEV1 z-scores improved over time in 21% of patients, remained stable in 40% and declined in 39%. Low BMI was associated with poor baseline lung function and with further decline. Results differed by country and ultrastructural defect, but we found no evidence of differences by sex, calendar year of diagnosis, age at diagnosis, diagnostic certainty, or laterality defect. Our study shows that on average lung function in PCD declines throughout the entire period of lung growth, from childhood to young adult age, even among patients treated in specialized centres. It is essential to develop strategies to reverse this tendency and improve prognosis.Take home messageLung function in children with PCD is reduced by age 6 years and further declines during the growth period. It is essential to develop strategies to improve prognosis.

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Section: Discussionsupporting

confidence: 67%

Lung function from school age to adulthood in primary ciliary dyskinesia

Halbeisen

Goutaki

Pedersen

et al. 2021

Preprint

View full text Add to dashboard Cite

show abstract

“…Can early diagnosis prevent disease progression? This has been suggested by previous studies, [14,[27][28][29] but the Danish study and ours found no association. [8] As we still lack robust evidence for the best treatments for PCD, management is based on experience from other lung diseases such as CF.…”

Section: Discussionsupporting

confidence: 63%

Lung function from school age to adulthood in primary ciliary dyskinesia

et al. 2022

View full text Add to dashboard Cite

Primary ciliary dyskinesia (PCD) presents with symptoms early in life and the disease course may be progressive, but longitudinal data on lung function are scarce. This multinational cohort study describes lung function trajectories in children, adolescents, and young adults with PCD. We analysed data from 486 patients with repeated lung function measurements obtained between the age of 6 and 24 years from the international PCD Cohort (iPCD) and calculated z-scores for forced expiratory volume in the first second (FEV1), forced vital capacity (FVC), and FEV1/FVC ratio using the Global Lung Function Initiative 2012 references. We described baseline lung function and change of lung function over time and described their associations with possible determinants in mixed-effects linear regression models. Overall, FEV1, FVC, and FEV1/FVC z-scores declined over time (average crude annual FEV1 decline was −0.07 z-scores) but not at the same rate for all patients. FEV1 z-scores improved over time in 21% of patients, remained stable in 40% and declined in 39%. Low BMI was associated with poor baseline lung function and with further decline. Results differed by country and ultrastructural defect, but we found no evidence of differences by sex, calendar year of diagnosis, age at diagnosis, diagnostic certainty, or laterality defect. Our study shows that on average lung function in PCD declines throughout the entire period of lung growth, from childhood to young adult age, even among patients treated in specialised centres. It is essential to develop strategies to reverse this tendency and improve prognosis.

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“…Comparisons of lung function between PCD and CF are rare and the results have been unclear. Some studies concluded that FEV1 was similar in PCD and CF patients [31,40,41], while another reported that it was lower in PCD children compared to pancreatic-sufficient and -insufficient CF children [42]. Yet another study found that children with PCD had better FVC, but similar FEV1 to children with CF [43].…”

Section: Discussionmentioning

confidence: 99%

Lung function in patients with primary ciliary dyskinesia: an iPCD Cohort study

et al. 2018

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Primary ciliary dyskinesia (PCD) has been considered a relatively mild disease, especially compared to cystic fibrosis (CF), but studies on lung function in PCD patients have been few and small.This study compared lung function from spirometry of PCD patients to normal reference values and to published data from CF patients. We calculated z-scores and % predicted values for forced expiratory volume in 1 s (FEV) and forced vital capacity (FVC) using the Global Lung Function Initiative 2012 values for 991 patients from the international PCD Cohort. We then assessed associations with age, sex, country, diagnostic certainty, organ laterality, body mass index and age at diagnosis in linear regression models. Lung function in PCD patients was reduced compared to reference values in both sexes and all age groups. Children aged 6-9 years had the smallest impairment (FEV z-score -0.84 (-1.03 to -0.65), FVC z-score -0.31 (-0.51 to -0.11)). Compared to CF patients, FEV was similarly reduced in children (age 6-9 years PCD 91% (88-93%); CF 90% (88-91%)), but less impaired in young adults (age 18-21 years PCD 79% (76-82%); CF 66% (65-68%)). The results suggest that PCD affects lung function from early in life, which emphasises the importance of early standardised care for all patients.

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Lung function and nutritional status in children with cystic fibrosis and primary ciliary dyskinesia

Cited by 3 publications

References 0 publications

Lung function from school age to adulthood in primary ciliary dyskinesia

Lung function from school age to adulthood in primary ciliary dyskinesia

Lung function from school age to adulthood in primary ciliary dyskinesia

Lung function in patients with primary ciliary dyskinesia: an iPCD Cohort study

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