Decreased Exhaled Nitric Oxide Levels in Patients with Mitochondrial Disorders

Ricardo A. Mosquera*, 1, Cheryl L. Samuels2, Tomika S. Harris2, Aravind Yadav1, S. Shahrukh Hashmi3, Melissa S. Knight 4, Mary Kay Koenig4
1 University of Texas Health Science Center at Houston (UTHealth), School of Medicine, Department of Pediatric Pulmonary, Houston, Texas, USA
2 UTHealth School of Medicine, Pediatric Comprehensive Care, Houston, Texas, USA
3 UTHealth School of Medicine, Pediatric Research Center, Department of Pediatrics, Houston, Texas, USA
4 UTHealth School of Medicine, Department of Pediatric Neurology, Houston, Texas, US.

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© Mosquera et al.; Licensee Bentham Open.

open-access license: This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

* Address correspondence to this author at the 6431 Fannin Street, MBS 3.226A, Houston, Texas 77030, USA; Tel: 713-500-5650; E-mail:



Nitric oxide (NO) deficiency may occur in mitochondrial disorders (MD) and can contribute to the pathogenesis of the disease. It is difficult and invasive to measure systemic nitric oxide. NO is formed in the lungs and can be detected in expired air. Currently, hand-held fractional exhaled nitric oxide (FeNO) measurement devices are available enabling a fast in-office analysis of this non-invasive test. It was postulated that FeNO levels might be reduced in MD.


Sixteen subjects with definite MD by modified Walker criteria (4 to 30 years of age) and sixteen healthy control subjects of similar age, race and body mass index (BMI) underwent measurement of FeNO in accordance with the American Thoracic Society guidelines.


Sixteen patient-control pairs were recruited. The median FeNO level was 6.5 ppm (IQR: 4-9.5) and 10.5 ppm (IQR: 8-20.5) in the MD and control groups, respectively. In 13 pairs (81%), the FeNO levels were lower in the MD cases than in the matched controls (p=0.021). Eleven (69%) cases had very low FeNO levels (≤7ppm) compared to only 1 control (p=0.001). All cases with enzymatic deficiencies in complex I had FeNO ≤7ppm.


Single-breath exhaled nitric oxide recordings were decreased in patients with MD. This pilot study suggests that hand-held FeNO measurements could be an attractive non-invasive indicator of MD. In addition, measurement of FeNO could be used as a parameter to monitor therapeutic response in this population.

Keywords: : Mitochondrial disorder, exhaled nitric oxide, FeNO, walker criteria, modified walker criteria, NIOX MINO..