Chest
Original ResearchHigh-Resolution CTStructural and Functional Lung Disease in Primary Ciliary Dyskinesia
Section snippets
Patients
Twenty patients (13 male patients; median age, 14.3 years; age range, 4.6 to 27.5 years) with PCD attending the departments of Pediatric Pulmonology at Federico II University (Naples, Italy) or Erasmus MC-Sophia Children's Hospital (Rotterdam, the Netherlands) were enrolled into the study. The patients from Rotterdam (n = 9) were retrospectively enrolled. CT scans were obtained to establish the severity of lung disease at some time point during follow-up. The patients from Naples (n = 11)
Results
The prevalences of HRCT scan abnormalities and HRCT scan scores of the PCD study population are shown in Table 2. Bronchiectasis, mucous plugging, and peribronchial thickening were the most frequent lung changes and showed the highest scores in the entire PCD study population, and in affected children and adults. Fifty percent of the patients showed central mucous plugging, while 70% had peripheral mucous plugging. The median scores of central and peripheral mucous plugging were 1.4% (range, 0
Discussion
This study of children and adults with PCD describes a composite HRCT scan scoring system that evaluates the extension and severity of all common structural lung changes associated with PCD. The following are the main findings. There is reasonable agreement between observers for the presented HRCT scan composite score and the bronchiectasis score in PCD patients. The HRCT scan score was able to demonstrate the prevalence and lobar distribution of the disease. The HRCT scan score increased with
Conclusions
This study on PCD pulmonary disease assessed by the modified CF composite HRCT scan score demonstrates that PCD patients show significantly milder lung damage than CF patients. We found a reasonable agreement between observers for the total CT score, and for the bronchiectasis and parenchyma scores. The total CT scan score and the CT scan bronchiectasis score correlate with lung function parameters. The CT scan score can be used in future longitudinal studies to better define the role of HRCT
Acknowledgment
The authors are indebted to Dr. Margherita De Santi, who conducted the electron microscopy ultrastructural analysis of nasal cilia from Italian PCD patients, and to Dr. Anders Lindblad, who kindly gave us permission to use clinical, functional, and CT scan data from Swedish CF patients.
References (32)
A human syndrome caused by immotile cilia
Science
(1976)Cilia-related diseases
J Pathol
(2004)Genetics and pulmonary medicine: 6. Immotile cilia syndrome: past, present, and prospects for the future
Thorax
(1998)- et al.
Primary ciliary dyskinesia: diagnosis and standards of care
Eur Respir J
(1998) - et al.
Primary ciliary dyskinesia
Arch Dis Child
(2007) - et al.
Primary ciliary dyskinesia in the paediatric population: range and severity of radiological findings in a cohort of patients receiving tertiary care
Clin Radiol
(2007) - et al.
Classifying severity of cystic fibrosis lung disease using longitudinal pulmonary function data
Am J Respir Crit Care Med
(2006) - et al.
Pulmonary disease assessment in cystic fibrosis: comparison of CT scoring systems and value of bronchial and arterial dimension measurements
Radiology
(2004) - et al.
Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis
Eur Respir J
(2004) - et al.
Computed tomography in the evaluation of cystic fibrosis lung disease
Am J Respir Crit Care Med
(2005)
Computed tomography and cystic fibrosis: promises and problems
Thorax
Imaging of the chest in cystic fibrosis
Clin Chest Med
High-resolution CT of patients with primary ciliary dyskinesia
AJR Am J Roentgenol
High-resolution computed tomography in young patients with cystic fibrosis: distribution of abnormalities and correlation with pulmonary function tests
J Pediatr
Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis
Thorax
Multiple-breath inert gas washout and spirometry vs. structural lung disease in cystic fibrosis
Thorax
Cited by (87)
Radiology of Bronchiectasis
2022, Clinics in Chest MedicineClinical and molecular characteristics of primary ciliary dyskinesia: A tertiary care centre experience
2021, International Journal of Pediatrics and Adolescent MedicineCitation Excerpt :A high-resolution chest CT scan might be helpful in making a PCD diagnosis by allowing for the detection of SI with bronchiectasis, and CT scan findings are useful for determining disease severity, which is correlated with FEV1 [35]. Santamaria et al. reported the presence of bronchiectasis in 71% of paediatric patients [36]. However, in our study, 81% of patients had bronchiectasis, with an average age of 8.2 years.
Non-cystic fibrosis bronchiectasis in children and adolescents: Neglected and emerging issues
2020, Pediatrics and NeonatologyPrimary ciliary dyskinesia in the genomics age
2020, The Lancet Respiratory MedicineCitation Excerpt :Lung disease, in particular bronchiectasis, predominantly occurs in the middle and lower lobes, with relative sparing of the upper lobes.115,128–129 As expected, structural changes increase with age and are associated with lung function.128,129 There are no scoring systems specific for primary ciliary dyskinesia.
Diagnostic evaluation of bronchiectasis
2019, Respiratory Medicine: X71 - Primary Ciliary Dyskinesia
2019, Kendig's Disorders of the Respiratory Tract in Children
The work was performed at the Department of Pediatrics, Federico II University, Naples, Italy, and at Erasmus MC-Sophia Children's Hospital, Rotterdam, the Netherlands.
The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.
Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/misc/reprints.shtml).