Nasal nitric oxide levels in primary ciliary dyskinesia, cystic fibrosis and healthy children

Abstract

Primary ciliary dyskinesia (PCD) is a rare, inherited disorder characterized by recurrent respiratory tract infections. The measurement of nasal nitric oxide (nNO) is an important test for the diagnosis of PCD. In this study, we aim to evaluate NIOX-MINO Ò , which is an easily applicable method for measuring nNO, in the diagnosis of patients with PCD and define diagnostic cut-off levels. Furthermore, determining the normal limits of nNO in healthy children and investigating nNO levels of children with cystic fibrosis (CF) are the other aims of this study. The children included in this study were 5 to 18.5 years old, 46 of them had PCD, 44 had CF and 200 were healthy children. To our knowledge, this work contains the widest population compared to previous studies. Subjects receiving steroids or antibiotics or those with any acute respiratory tract infection, asthma or allergic rhinitis were not included in the study. Mean nNO levels were found as 10.4, 22.8 and 21.0 ppb in PCD, CF and healthy children, respectively. The nNO levels for PCD patients were found significantly lower than children with CF and the control groups (p<0.05). In this study, the diagnostic nNO cut-off level between PCD and the other two groups was determined to be <11.5 ppb with %83.6 specificity and %67.4 sensitivity. The screening of nNO with NIOX-MINO method provides early diagnose before mucosal biopsy of patients who are suspected to have PCD and therefore, prevents co-morbidities and prolongs survival with early treatment.

Oxidative Stress and Endoplasmic Reticulum Stress in Rare Respiratory Diseases

“…Altered RNS has been reported in PCD. Nasal NO (nNO) measurement is recommended as the initial test in patients with clinical suspicion of PCD since diminished nNO values are characteristic of patients with PCD [172]. However, despite its good sensitivity, there are cases of PCD with normal values of nNO, so even if the nNO is normal-if the symptoms are consistent-the disease should not be ruled out [173,174].…”

Nasal Nitric Oxide in Children: A Review of Current Outreach in Pediatric Respiratory Medicine

“…They observed that the mean nNO values were 10.4 ± 8.3 ppb, 22.8 ± 18.7 ppb, and 21 ± 8.9 ppb, respectively. Notably, nNO levels in children with PCD were found to be significantly lower than those in children with CF and the healthy control group (p < 0.05) [52].…”

Role of Nasal Nitric Oxide in Primary Ciliary Dyskinesia and Other Respiratory Conditions in Children

“…While NO concentration in airways is usually high in chronic inflammatory diseases (allergic rhinitis, bronchiectasis, etc.) because of the induction of NO synthase by inflammatory cytokines such as TNF-a, IL-1b, and interferon-c [48], cystic fibrosis (CF) represents an exception since CF patients usually have normal or even reduced nNO levels [49] (Table 1).…”