Primer Siliyer Diskinezili, Kistik Fibrozisli ve Sağlıklı Çocuklarda Nıox-Mıno Yöntemiyle Nazal Nitrik Oksit Ölçümü

Abstract

Primary ciliary dyskinesia (PCD) is a rare, inherited disorder. The defects of ciliary structure and/or function in respiratory tract and in other areas of the body lead to impaired mucociliary clearance. Characterized by recurrent upper and lower respiratory tract infections and infertility. Since the common symptoms of PCD disease can be seen in many other diseases as well, the diagnosis of the disease is delayed. The measurement of nasal nitric oxide (nNO) with chemiluminescence method is an important test for the diagnosis of PCD. NIOX-MINO (Aerocrine AB, Solna, Sweeden) is a portable, non-invasive, easily operated fast response device that shows correlation to chemiluminescence methods. As yet, there is not any wide case series published about nNO levels in healthy children with NIOX MINO method. The first aim of this study is to investigate the place of NIOX-MINO method, which is an easily applicable way of measuring nNO, in the diagnosis of patients with PCD and to define diagnostic cut-off levels. Furthermore, the second aim of this study is to determine the normal limits of nNO in healthy children and to evaluate the children with cystic fibrosis (CF), which is an another disease that may cause low levels of nNO, with the same method and to compare to the PCD children. The children included in this study are between 5 to 18,5 years old, 46 of them have PCD, 44 have CF and 200 are healthy and all are followed in Hacettepe University İhsan Doğramacı Children Hospital Pulmonary Medicine. In order to reduce the effect of air pollution and the effect of NO in the air, nNO is measured starting from May 2012 and including spring, summer and autumn seasons in which the heating systems were not used. Inclusion criteria of the children were, during the study subjects had no cold, acute respiratory tract infection, asthma or allergic rhinitis and were not receiving any antibiotics, inhale and nazal steroids. Mean nNO levels are found as 10,4 ppb (2,5-42), 22,8 ppb (2,5-81,0) and 21,0 ppb (7,0-48,0) in PCD, CF and healthy children, respectively. The nNO levels were found significantly lower in PCD than children with CF and healthy control groups (p<0,05). In this study, the diagnostic nNO cut-off level of PCD is 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 be PCD and therefore, prevents co-morbidities and prolongs survival with early treatment.