Phillip S Adams1, Xin Tian2, Maliha Zahid3, Omar Khalifa4, Linda Leatherbury5, Cecilia W Lo6. 1. Division of Pediatric Anesthesiology, Department of Anesthesiology, University of Pittsburgh School of Medicine, 4401 Penn Ave, Pittsburgh, PA, 15224, USA. Electronic address: adamsp@upmc.edu. 2. Office of Biostatistics Research, National Heart, Lung and Blood Institute, 6701 Rockledge Dr MSC 7913, Bethesda, MD, 20892, USA. Electronic address: tianx@nhlbi.nih.gov. 3. Department of Developmental Biology, University of Pittsburgh School of Medicine, 4401 Penn Ave, 8120 Rangos Research Center, Pittsburgh, PA, 15201, USA. Electronic address: maz7@pitt.edu. 4. Department of Developmental Biology, University of Pittsburgh School of Medicine, 4401 Penn Ave, 8120 Rangos Research Center, Pittsburgh, PA, 15201, USA. Electronic address: omk4@pitt.edu. 5. Division of Cardiology, Department of Pediatrics, Children's National Medical Center, 111 Michigan Ave NW #200, Washington, DC, 20310, USA. Electronic address: lleather@childrensnational.org. 6. Department of Developmental Biology, University of Pittsburgh School of Medicine, 4401 Penn Ave, 8120 Rangos Research Center, Pittsburgh, PA, 15201, USA. Electronic address: cel36@pitt.edu.
Abstract
INTRODUCTION: Primary ciliary dyskinesia (PCD), a disease of impaired respiratory cilia motility, is often difficult to diagnose. Recent studies show low nasal nitric oxide (nNO) is closely linked to PCD, allowing the use of nNO measurement for PCD assessments. Nasal NO cutoff values for PCD are stratified by age, given nNO levels normally increase with age. However, normative values for nNO have not been established for infants less than 1 year old. In this study, we aim to establish normative values for nNO in infants and determine their utility in guiding infant PCD assessment. METHODS AND RESULTS: We obtained 42 nNO values from infants less than 1 year old without a history of PCD or recurrent sinopulmonary disease. Using regression analysis, we estimated the mean age-adjusted nNO values and established a 95% prediction interval (PI) for normal nNO. Using these findings, we were able to show 14 of 15 infant PCD patients had abnormally low nNO with values below the 95% PI. CONCLUSIONS: In this study we determined a regression model that best fits normative nNO values for infants less than 1 year old. This model identified the majority of PCD infants as having abnormally low nNO. These findings suggest nNO measurement can help guide PCD assessment in infants, and perhaps other pulmonary diseases with a link to low nNO. With early assessments, earlier clinical intervention may be possible to slow disease progression and help reduce pulmonary morbidity.
INTRODUCTION:Primary ciliary dyskinesia (PCD), a disease of impaired respiratory cilia motility, is often difficult to diagnose. Recent studies show low nasal nitric oxide (nNO) is closely linked to PCD, allowing the use of nNO measurement for PCD assessments. Nasal NO cutoff values for PCD are stratified by age, given nNO levels normally increase with age. However, normative values for nNO have not been established for infants less than 1 year old. In this study, we aim to establish normative values for nNO in infants and determine their utility in guiding infantPCD assessment. METHODS AND RESULTS: We obtained 42 nNO values from infants less than 1 year old without a history of PCD or recurrent sinopulmonary disease. Using regression analysis, we estimated the mean age-adjusted nNO values and established a 95% prediction interval (PI) for normal nNO. Using these findings, we were able to show 14 of 15 infantPCDpatients had abnormally low nNO with values below the 95% PI. CONCLUSIONS: In this study we determined a regression model that best fits normative nNO values for infants less than 1 year old. This model identified the majority of PCDinfants as having abnormally low nNO. These findings suggest nNO measurement can help guide PCD assessment in infants, and perhaps other pulmonary diseases with a link to low nNO. With early assessments, earlier clinical intervention may be possible to slow disease progression and help reduce pulmonary morbidity.
Authors: Adam J Shapiro; Sharon D Dell; Benjamin Gaston; Michael O'Connor; Nadzeya Marozkina; Michele Manion; Milan J Hazucha; Margaret W Leigh Journal: Ann Am Thorac Soc Date: 2020-02
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Authors: Maliha Zahid; Abha Bais; Xin Tian; William Devine; Dong Ming Lee; Cyrus Yau; Daniel Sonnenberg; Lee Beerman; Omar Khalifa; Cecilia W Lo Journal: PLoS One Date: 2018-02-14 Impact factor: 3.240
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Authors: Nicole Beydon; Thomas Ferkol; Amanda Lea Harris; Murielle Colas; Stephanie D Davis; Eric Haarman; Claire Hogg; Emma Kilbride; Panayotis Kouis; Claudia E Kuehni; Philipp Latzin; Diana Marangu; June Marthin; Kim G Nielsen; Phil Robinson; Nisreen Rumman; Matthew Rutter; Woolf Walker; Jane S Lucas Journal: ERJ Open Res Date: 2022-04-04