scholarly journals Nasal Nitric Oxide in Primary Immunodeficiency and Primary Ciliary Dyskinesia: Helping to Distinguish Between Clinically Similar Diseases

2019 ◽  
Vol 39 (2) ◽  
pp. 216-224 ◽  
Author(s):  
Zofia N. Zysman-Colman ◽  
Kimberley R. Kaspy ◽  
Reza Alizadehfar ◽  
Keith R. NyKamp ◽  
Maimoona A. Zariwala ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Primary Immunodeficiency ◽  
Primary Ciliary Dyskinesia ◽  
Nasal Nitric Oxide ◽  
Ciliary Dyskinesia

Use caution interpreting nasal nitric oxide: Overlap in primary ciliary dyskinesia and primary immunodeficiency

2021 ◽  
Author(s):  
Andrew T. Barber ◽  
Stephanie D. Davis ◽  
Hannah Boutros ◽  
Maimoona Zariwala ◽  
Michael R. Knowles ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Primary Immunodeficiency ◽  
Primary Ciliary Dyskinesia ◽  
Nasal Nitric Oxide ◽  
Ciliary Dyskinesia

scholarly journals Nasal nitric oxide and nitric oxide synthase expression in primary ciliary dyskinesia

2011 ◽  
Vol 37 (3) ◽  
pp. 572-577 ◽  
Author(s):  
M. Pifferi ◽  
A. Bush ◽  
F. Maggi ◽  
A. Michelucci ◽  
V. Ricci ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Nitric Oxide Synthase ◽  
Primary Ciliary Dyskinesia ◽  
Nasal Nitric Oxide ◽  
Oxide Synthase ◽  
Ciliary Dyskinesia

scholarly journals How to use nasal nitric oxide in a child with suspected primary ciliary dyskinesia

2017 ◽  
Vol 102 (6) ◽  
pp. 314-318 ◽  
Author(s):  
Kim Simpson ◽  
Malcolm Brodlie
Keyword(s):  
Nitric Oxide ◽  
Primary Ciliary Dyskinesia ◽  
Clinical Utility ◽  
Diagnostic Testing ◽  
Older Children ◽  
Tidal Breathing ◽  
Closure Technique ◽  
Nasal Nitric Oxide ◽  
Ciliary Function ◽  
Ciliary Dyskinesia

Measuring nasal nitric oxide (nNO) is increasingly used as part of testing for primary ciliary dyskinesia (PCD). The diagnosis of PCD is often delayed until after bronchiectasis is established and auditory damage has occurred. It is important that all paediatricians are aware of clinical features that are suggestive of PCD that should prompt diagnostic testing. nNO levels are recognised to be low in people with PCD and results generated by static chemiluminescence analysers using velum closure technique in older children have good sensitivity and specificity. However, to conclusively rule PCD in or out, further tests of ciliary function are required and assessment of cilia ultrastructure, immunohistochemistry studies and genotyping may also be indicated. These tests are more complex, invasive and expensive than nNO. nNO is less well studied in younger children where tidal breathing measurements are required. Portable nitric oxide analysers are also increasingly used in practice. This paper discusses when to consider PCD as a possible diagnosis in a child along with the indications, physiological and technical background and clinical utility of nNO as a test for PCD in children.

scholarly journals Nasal nitric oxide measurements for the screening of primary ciliary dyskinesia

2003 ◽  
Vol 21 (1) ◽  
pp. 43-47 ◽  
Author(s):  
T. Wodehouse ◽  
S.A. Kharitonov ◽  
I.S. Mackay ◽  
P.J. Barnes ◽  
R. Wilson ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Primary Ciliary Dyskinesia ◽  
Nasal Nitric Oxide ◽  
Ciliary Dyskinesia

Experience of measuring nasal nitric oxide (nNO) in a National Primary Ciliary Dyskinesia (PCD) Centre (2006-20)

2020 ◽  
Author(s):  
Amanda Harris ◽  
Hang Phan ◽  
Florina Borca ◽  
Samantha Packham ◽  
Amanda Friend ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Primary Ciliary Dyskinesia ◽  
Nasal Nitric Oxide ◽  
Ciliary Dyskinesia

scholarly journals Decreased nasal nitric oxide in children with isolated midline neuroanatomical defects: a possible indicator of ciliary dysfunction?

2015 ◽  
Vol 42 (S1) ◽  
pp. S22-S23 ◽  
Author(s):  
H Goez ◽  
O Scott ◽  
B Al-Jabri ◽  
M Prowse ◽  
W Beaudoin ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Spinal Cord ◽  
Longitudinal Studies ◽  
Screening Test ◽  
Primary Ciliary Dyskinesia ◽  
Normal Range ◽  
Normal Children ◽  
Nasal Nitric Oxide ◽  
Ciliary Dyskinesia ◽  
Effect Of Age

Background: Ciliary mutations cause multi-system disorders, often involving the CNS. We set to evaluate the prevalence of ciliary dysfunction in children with isolated neuroanatomical defects, by measuring nasal nitric oxide (nNO), a screening test for Primary Ciliary Dyskinesia (PCD). Study design: We measured nNO levels of 26 children with congenital midline CNS defects. We evaluated the effect of age, gender, and anomaly (brain, spinal cord, or combined) on measurements. We compared our results to the previously established normal range (153.6-509.9 nL/min), and to the cutoff for PCD (77 nL/min). Results: The range for nNO in our cohort was 56.5-334.7 nL/min, with age, gender, and anomaly not having a significant effect. The overall mean, 217.7 nL/min, was significantly lower than that of normal children, 314.51 nL/min (p<0.01). Four subjects (15.4%) had nNO levels below the lower end of normal, with two (7.7%) having values fitting the cutoff for PCD. Conclusions: We report an association between ciliary dysfunction and isolated midline neuroanatomical defects, not in context of any known syndrome. This suggests that genes causing isolated CNS defects, may be implied in the function of cilia. Longitudinal studies are required to investigate whether children with abnormal measurements suffer from any respiratory sequelae.

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