US EPA

2263055 

Journal Article 

Abstract 

A multi-center, longitudinal study of nasal nitric oxide in children with primary ciliary dyskinesia 

Chawla, KK; Hazucha, MJ; Dell, SD; Ferkol, TW; Sagel, SD; Rosenfeld, M; Baker, B; Davis, SD; Knowles, MR; Leigh, MW; Genet Dis Mucociliary Clearance Co 

2010 

Yes 

American Journal of Respiratory and Critical Care Medicine
ISSN: 1073-449X
EISSN: 1535-4970 

181 

A6726 

English 

10.1164/ajrccm-conference.2010.181.1_MeetingAbstracts.A6726 

WOS:000208771005345 

is part of a larger document 3452678 Proceedings of the American Thoracic Society 2010 International Conference, May 14-19, 2010, New Orleans

RATIONALE: Nasal nitric oxide (nNO) is very low in patients with Primary Ciliary Dyskinesia (PCD) and thus can serve as a screening test for PCD in patients who can cooperate with the maneuver. The purpose of this study is to assess year to year variability of nNO measurements in children with PCD.

METHOD: Five sites enrolled children (5-18 years of age) with definite PCD (clinical phenotype + PCD-specific defect in cilia and/or 2 known PCD gene mutations) or probable PCD (same phenotype + nNO < 100 nl/min, but no cilia defect or known gene mutations) into our longitudinal study. Participants were evaluated at yearly intervals using IRB approved protocols and standard operating procedures, including ATS guidelines for nNO measurement. All nNO measurements were made during velum closure (while blowing into a resistor or party favor).

RESULTS: Seventy-six participants (53 definite PCD and 23 probable PCD) had yearly nNO measurements. Three outliers (2 definite PCD and 1 probable PCD) were identified and were not included in group analyses. Age at enrollment for the definite PCD group (10.2 ± 3.8 years) was not different from the probable PCD group (10.6 ± 3.0 years). Initial nNO for definite PCD (15.3 ± 8.7, range 0.7 – 40.4 nl/min) and probable PCD (19.0 ± 17.2, range 1.1 – 69.7 nl/min) were not different. Delta sum (net sum of positive and negative differences between nNO measurements in consecutive years for each individual) was close to zero for definite PCD (1.3 ± 10.3, range -20.5 – 23.9 nl/min) suggesting that the variability does not reflect a consistent age-related trend, but not as close to zero for probable PCD (6.53 ± 14.6, range -31.9 – 42.9 nl/min). The peak difference (highest nNO-lowest nNO for each individual) was relatively small for the definite PCD group (9.81 ± 8.5, range 0.0 – 25.7 nl/min) and the probable PCD group (11.5 ± 12.3, range 0.0 – 42.9 nl/min) compared to the 3 outliers (137.7, 62.3, and 101.4 nl/min).

CONCLUSION: Nasal NO values remain very low (less than 60 nl/min) in most children 5-18 years old with definite PCD or probable PCD. The 3 outliers with a large increase in nNO at later measurements may have mild variant PCD vs. incorrect diagnosis vs. spurious measurements and will undergo continued yearly evaluations to define how they differ from other participants. 

American Thoracic Society 2010 International Conference 

New Orleans, LA 

May 14-19, 2010