Different Levels of Exhaled Nasal Nitric Oxide in Patients Diagnosed with Primary Dyskinesia

Journal Title: Archives of Pulmonology and Respiratory Care - Year 2015, Vol 1, Issue 1

Abstract

Background: Primary ciliary dyskinesia (PCD) is a genetic disease characterized by abnormally beating cilia. In these patients levels of nasal nitric oxide (nNO) are lower than those observed in healthy subjects. Objectives: We recorded the nNO levels in PCD patients in order to use those nNO measurements in the screening and identification of patients with symptoms suggestive of disease PCD disease. Methods: We measured nasal NO in 36 PCD patients (3 uncooperative younger children and 33 cooperative adult patients) and did a nNO re-evaluation after 12 months in patients with higher levels of nNO. Results: Twenty-seven PCD patients showed very low nNO levels (29.1 ppb) and nine cooperative patients had high nNO levels (583.3 ppb, p<0.001) (T0); the PCD patients with high nNO levels were re-evaluated after 12 months (T1). The median T0 and T1 nNO values of the seven PCD patients were 360 ppb and 324 ppb (p=0.0180), respectively; in 6 patients with high levels of nNO the diagnosis of PCD was confirmed by electron microscopy, and in one subject the diagnosis was confirmed for secondary ciliary dyskinesia. Conclusions: Low levels of nNO remain indicative of PCD disease; high levels of nNO are supportive of PCD, but cannot be used to exclude diagnosis. These results suggest that repeated measures are warranted when nNO is occasionally high in patients with symptoms suggestive of PCD disease, and at present electron microscopy is still the only valid evaluation tool in unclear cases of PCD.

Authors and Affiliations

Bodini A, Peroni D, Tenero L, Sandri M, Brunelli M, Inzalaco G, Pradal U, Piazza M, Boner AL, Piacentini GL

Keywords

Related Articles

Impact of Calorie Intakes on the Risk of Bronchopulmonary Dysplasia in Extremely Preterm Infants

Aim: To examine whether caloric intake during the first week of age influences the risk of Bronchopulmonary Dysplasia (BPD) in extremely preterm infants. Methods: In this retrospective cohort study, all infants born with...

An atypical Klippel-Treanunay-Weber syndrome

A 44 year old female was referred for hyperbaric oxygen treatment of leg ulcers. The patient had port-wine stain, hand vitiligo, hypertrophy of one extremity, collateral abdominal, and varicose leg veins. Calcium was 12...

Timing and Efficacy of VATS Debridement in the Treatment of Parapneumonic Empyema

Objectives: Empyema thoracis is a common thoracic disease seen in both developing and developed countries. Despite modern management techniques, this condition is still associated with significant morbidity and mortality...

Inhaled GM-CSF in a Pulmonary Alveolar Proteinosis Patient Refractory to Plasmapheresis Combined with Multiple Whole Lung Lavages

A autoimmune Pulmonary Alveolar Proteinosis (PAP) patient with persistent disease underwent 3 Whole Lung Lavages (WLLs), 10 plasmapheresis sessions and further 3 WLL, from October 2004 to May 2007. Nevertheless HRTC and...

Different Levels of Exhaled Nasal Nitric Oxide in Patients Diagnosed with Primary Dyskinesia

Background: Primary ciliary dyskinesia (PCD) is a genetic disease characterized by abnormally beating cilia. In these patients levels of nasal nitric oxide (nNO) are lower than those observed in healthy subjects. Obje...

Download PDF file
  • EP ID EP340294
  • DOI 10.17352/aprc.000004
  • Views 105
  • Downloads 0

How To Cite

Bodini A, Peroni D, Tenero L, Sandri M, Brunelli M, Inzalaco G, Pradal U, Piazza M, Boner AL, Piacentini GL (2015). Different Levels of Exhaled Nasal Nitric Oxide in Patients Diagnosed with Primary Dyskinesia. Archives of Pulmonology and Respiratory Care, 1(1), 14-17. https://www.europub.co.uk/articles/-A-340294