Longitudinal lung function and structural changes in children with primary ciliary dyskinesia
Pediatric Pulmonology,

Magnin ML et al. – In stable clinical condition, functional, and structural progressive impairments significantly correlated in children with primary ciliary dyskinesia (PCD). Further prospective studies, including large populations of patients with various levels of disease severity, are needed to confirm whether lung function follow–up can be used to adjust CT frequency and help at minimizing the radiation burden in children with a good life expectancy.

Methods
  • Data from children followed in the French National Center were retrospectively collected. Inclusion criteria were (i) definitive diagnosis of PCD, (ii) age less than 15 years at the beginning of follow-up, (iii) at least 8 years of follow-up, (iv) at least two couples of concurrent CT and LFT available in a phase of clinical stability of the lung disease without modification of the treatment regimen in the last 4 weeks.
  • Twenty children (median age at entry 4.6 years, median follow-up 15.4 years) were included. Concurrent LFT (blood gas and spirometry) and CT (score) results were recorded.

Results
  • LFT indices (PaO2 (n=210), FVC, FEV1, FEF2575% (n=195)) significantly decreased with age, and the mean annual decrease (z-score (% predicted)) was -0.17 (-0.49%), -0.09 (-0.50%), -0.10 (-0.89%), and -0.07 (-1.73%), respectively.
  • First CT (median age 8.7 years) revealed bronchiectasis (70%), mucous plugging (70%), peribronchial thickening (90%), parenchymal abnormalities (65%), and hyperinflation (50%). CT scores (n=74) significantly increased with age, and was negatively correlated to PaO2, FVC, FEV1, and FEF2575% longitudinal changes.

Please login or register to follow this author.
► Click here to access PubMed, Publisher and related articles...
<< Previous Article | Next Article >>

Your Unread Messages in Physician Assistant

See All >> Messages include industry-sponsored communications and special communications from MDLinx

Most Popular Physician Assistant Articles

1 Radiotherapy or surgery of the axilla after a positive sentinel node in breast cancer (EORTC 10981-22023 AMAROS): A randomised, multicentre, open-label, phase 3 non-inferiority trial The Lancet Oncology, October 22, 2014    Clinical Article

2 Dance for Parkinson's: A new framework for research on its physical, mental, emotional, and social benefits Complementary Therapies in Medicine, June 17, 2014    Review Article

3 Omega-3 supplements for patients in chemotherapy and/or radiotherapy: A systematic review Clinical Nutrition, November 21, 2014    Clinical Article

4 Global burden of cancer attributable to high body-mass index in 2012: A population-based study The Lancet Oncology, November 26, 2014    Clinical Article

5 Twelve or 30 months of dual antiplatelet therapy after drug-eluting stents New England Journal of Medicine, November 18, 2014    Evidence Based Medicine    Clinical Article

6 Nonobstructive coronary artery disease and risk of myocardial infarction JAMA, November 6, 2014    Evidence Based Medicine    Clinical Article

7 Treatment of syphilis: a systematic review JAMA, November 13, 2014    Review Article    Clinical Article

8 Mechanical versus manual chest compression for out-of-hospital cardiac arrest (PARAMEDIC): a pragmatic, cluster randomised controlled trial The Lancet, November 26, 2014    Evidence Based Medicine    Clinical Article

9 Effects of salt substitutes on blood pressure: A meta-analysis of randomized controlled trials American Journal of Clinical Nutrition, October 31, 2014    Evidence Based Medicine    Review Article    Clinical Article

10 Bipolar disorder, affective psychosis, and schizophrenia in pregnancy and the post-partum period The Lancet, November 19, 2014    Evidence Based Medicine

11 Standard versus atrial fibrillation-specific management strategy (SAFETY) to reduce recurrent admission and prolong survival: pragmatic, multicentre, randomised controlled trial The Lancet, November 26, 2014    Clinical Article

12 Initial choice of oral glucose-lowering medication for diabetes mellitus: a patient-centered comparative effectiveness study JAMA Internal Medicine, November 14, 2014    Evidence Based Medicine    Clinical Article

13 Optimization of rituximab for the treatment of diffuse large B-cell lymphoma (II): extended rituximab exposure time in the smarte-R-CHOP-14 trial of the German High-grade Non-hodgkin Lymphoma Study Group Journal of Clinical Oncology, November 26, 2014    Clinical Article

14 Early versus on-demand nasoenteric tube feeding in acute pancreatitis New England Journal of Medicine, November 26, 2014    Evidence Based Medicine    Clinical Article

15 Ventriculitis caused by primary T-cell CNS lymphoma in an immunocompetent patient Journal of Clinical Oncology, November 26, 2014

16 Long-term safety and efficacy of factor IX gene therapy in hemophilia B New England Journal of Medicine, November 26, 2014    Evidence Based Medicine    Clinical Article

17 Management of high-grade gliomas in the elderly Seminars in Radiation Oncology, October 3, 2014    Clinical Article

18 The diagnosis and management of hiatus hernia BMJ, November 14, 2014    Evidence Based Medicine    Review Article    Clinical Article

19 Statins in the elderly: an answered question Current Opinion in Cardiology, June 9, 2014    Evidence Based Medicine    Review Article    Clinical Article

20 Changes in bone mineral density at 3 years in postmenopausal women receiving anastrozole and risedronate in the IBIS-II bone substudy: An international, double-blind, randomised, placebo-controlled trial The Lancet Oncology, November 26, 2014    Clinical Article

Indexed Journals in Physician Assistant: Advance for Physicians Assistants, American Family Physicianmore