Nasal Nitric Oxide Measurement in Primary Ciliary Dyskinesia. A Technical Paper on Standardized Testing Protocols

Shapiro, Adam J.; Dell, Sharon D.; Gaston, Benjamin; O'Connor, M.G.; Marozkina, Nadzeya; Manion, Michele; Hazucha, Milan J.; Leigh, Margaret W.

doi:10.1513/annalsats.201904-347ot

Cited by 72 publications

(86 citation statements)

References 54 publications

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“…Moreover, a reduced nNO level alone is not diagnostic for PCD. When using established, standardized protocols ( 5 ), meta-analysis shows that low nNO measurements are “comparable to that of TEM and/or genetic testing”; however, the guideline states that “patients should still progress to further corroborative PCD diagnostic studies” ( 2 ). This recommendation is underscored by recent observations that some patients with primary immunodeficiency, a heterogeneous group of disorders sharing clinical features with PCD, can also have reduced nNO levels ( 6 ).…”

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confidence: 99%

Limitations of Nasal Nitric Oxide Testing in Primary Ciliary Dyskinesia

Shapiro

Davis

Leigh

et al. 2020

Am J Respir Crit Care Med

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confidence: 99%

Limitations of Nasal Nitric Oxide Testing in Primary Ciliary Dyskinesia

Shapiro

Davis

Leigh

et al. 2020

Am J Respir Crit Care Med

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“…Nasal NO levels obtained by tidal breathing are potentially two thirds the nasal NO level obtained with the velum closure technique 34 . Three subjects performed nasal NO testing with the tidal breathing technique: one subject in group 1 (age 16; nNO 86.9 nL/minute), one subject in group 2 (age 10; nNO 66.8 nL/minute) and one subject in group 4 (age 8; nNO 89.0 nL/minute).…”

Section: Discussionmentioning

confidence: 99%

Phenotypic features of ciliary dyskinesia among patients with congenital cardiovascular malformations

Sherman

Wodrich

Zampi

et al. 2020

Pediatric Pulmonology

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Background Cilia are cell membrane‐bound organelles responsible for airway mucus clearance, establishment of left‐right organ asymmetry, cardiogenesis, and many other functions in utero. Phenotypic features suggestive of respiratory ciliary dyskinesia among patients with heterotaxy syndrome, defined as complex cardiovascular malformations (CVM) and situs ambiguus (SA), has not been adequately explored. Objectives We hypothesized that there is a greater incidence of phenotypic features consistent with ciliary dyskinesia among patients with heterotaxy syndrome compared to patients with other CVM and laterality defects without heterotaxy syndrome. Methods Thirty six subjects were identified by medical record search and divided into four groups based on situs status and type of CVM as follows: SA and complex CVM (group 1); SA and simple CVM (group 2); situs solitus and complex CVM (group 3); and situs solitus and simple CVM (group 4). Phenotype was assessed with a clinical questionnaire, nasal nitric oxide (NO) level, and pulmonary function testing. Those with complex CVM underwent additional testing for variants in genes involved in ciliary structure and function. Results The mean nasal NO level was significantly lower among all subjects with complex CVM regardless of situs anomalies (groups 1 and 3). There was no significant difference in respiratory symptoms or lung function among the four groups. No bi‐allelic genetic mutations were detected among patients with complex CVM. Conclusions This study identified a relatively lower mean nasal NO level, suggestive of relative ciliary dyskinesia, among subjects with complex CVM. Pulmonary function and clinical symptoms did not reflect significant pulmonary disease among those with complex CVM.

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“…A screening test for primary ciliary dyskinesis is measuring nitric oxide from the nose 32 . Primary ciliary dyskinesis is characterized by extremely low values of nasal nitric oxide.…”

Section: Diagnosing the Cause Of Chronic Cough In Childrenmentioning

confidence: 99%

Diagnosis and management of chronic cough: similarities and differences between children and adults

Weinberger

Hurvitz

2020

F1000Res

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Cough is a natural process that protects the airway. Cough can occur spontaneously or voluntarily. It is considered chronic when it is present for longer than 4 weeks in children or 8 weeks in adults. In both, chronic cough causes patient distress and increased healthcare utilization. Etiologies of pediatric chronic cough include asthma, protracted bacterial bronchitis, tracheomalacia, habit cough, and various systemic disorders. While some diagnoses are identifiable by careful history alone, others require testing guided by specific pointers. Flexible fiberoptic bronchoscopy has been an important tool to identify etiologies of chronic cough that were not otherwise apparent. In adults, asthma and bronchitis are well-defined etiologies of chronic cough, but much chronic cough in adults is largely a conundrum. Reviews of adult chronic cough report that at least 40% of adults with chronic cough have no medical explanation. Gastroesophageal reflux and upper airway cough syndrome (a.k.a. post-nasal drip) have been common diagnoses of chronic cough, but those diagnoses have no support from controlled clinical trials and have been subjected to multiple published critiques. Cough hypersensitivity is considered to be an explanation for chronic cough in adults who have no other confirmed diagnosis. Gabapentin, a neuromodulator, has been associated with a modest effect in adults, as has speech pathology. While habit cough has not generally been a diagnosis in adults, there is evidence for a behavioral component in adults with chronic cough. Treatment for a specific diagnosis provides a better outcome than trials of cough suppression in the absence of a specific diagnosis. More data are needed for chronic cough in adults to examine the hypothesized cough hypersensitivity and behavioral management. This article reviews etiologies and the treatment of chronic cough in children and the conundrum of diagnosing and treating chronic cough in adults.

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Nasal Nitric Oxide Measurement in Primary Ciliary Dyskinesia. A Technical Paper on Standardized Testing Protocols

Cited by 72 publications

References 54 publications

Limitations of Nasal Nitric Oxide Testing in Primary Ciliary Dyskinesia

Limitations of Nasal Nitric Oxide Testing in Primary Ciliary Dyskinesia

Phenotypic features of ciliary dyskinesia among patients with congenital cardiovascular malformations

Diagnosis and management of chronic cough: similarities and differences between children and adults

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