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Impairment of Nitric Oxide Output of Conducting Airways in Primary Ciliary Dyskinesia
Author(s) -
Mahut Bruno,
Escudier Estelle,
de Blic Jacques,
ZerahLancner Françoise,
Coste André,
Harf Alain,
Delclaux Christophe
Publication year - 2006
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.20329
Subject(s) - primary ciliary dyskinesia , medicine , nitric oxide , dyskinesia , cardiology , bronchiectasis , anesthesia , lung , disease , parkinson's disease
Nasal nitric oxide (NO) concentration is dramatically reduced in primary ciliary dyskinesia (PCD). The aims of this study were to apply a multiple‐flow NO analysis to investigate whether NO output from the bronchial tree was affected in a similar way to nasal NO output, and to search for a relationship between flow‐independent exchange parameters and airflow limitation. Multiple flow rate analysis of exhaled NO, allowing the calculation of maximum airway wall flux and alveolar NO concentration, was performed in 17 PCD patients (median age, 25–75th percentiles: 13.5, 12.1–17.6) with documented ultrastructural cilia abnormalities and 28 healthy subjects (16.0, 11.0–21.0). Median maximum airway wall flux and median alveolar NO concentration were significantly reduced in PCD patients compared to healthy subjects: 16.0, 7.5–29.5, vs. 25.0, 15.0–32.5 nl/min ( P < 0.05) and 2.5, 1.6–3.3, vs. 5.0, 3.6–6.5 ppb ( P < 0.01), respectively. Significant correlations between maximum airway wall flux and airflow limitation were found, i.e., resistance of respiratory system (rho = 0.74, P < 0.005), forced expiratory volume in one second (FEV 1 )/VC (rho = −0.61, P < 0.05), FEV 1 (rho = −0.52, P < 0.05), mid expiratory flow between 25 and 75% of forced vital capacity (MEF 25–75 ) (rho = −0.54, P < 0.05), and maximal instantaneous expiratory flow at 50% of the vital capacity (MEF 50 ) (rho = −0.55, P < 0.05). In conclusion, the impairment of NO output is less pronounced in the lower than in the upper (nasal) respiratory tract in PCD. A decrease in maximal NO output from conducting airways is associated with limited airflow impairment. Pediatr Pulmonol. © 2005 Wiley‐Liss, Inc.