Use of Nasal Nitric Oxide Testing in Improving Primary Ciliary Dyskinesia Clinical Care
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 入组人数
- 150
- 试验地点
- 1
- 主要终点
- Measuring nasal nitric oxide in comparison to ciliary biopsy and genetic testing for the diagnosis of PCD
研究概览
简要总结
this study is aiming at learning more about primary ciliary dyskinesia (PCD) and tests that are used to diagnose this condition. One purpose of this study is to measure the level of nitric oxide in the nasal passages and examine how often the results correlate with other tests currently done to make the diagnosis.
详细描述
This will be an observational study. Participants who are referred by their clinician for nasal NO testing and meet the inclusion and exclusion criteria will be allowed to undergo testing. Informed consent will be completed prior to testing. The participant and/or guardian will be informed that this testing is not a clinically approved test at this time and thus it cannot confirm diagnosis of PCD. This test is to correlate with other diagnostic tests so that in the future it can be an approved diagnostic test. Data will be collected from the electronic medical records regarding participants.
The data collected will be stored on a REDCap (Research Electronic Data Capture) registry. The registry will contain participants' direct identifiers (like medical record). De-identified data will be used from the registry for monitoring, further analysis and presentations. The results of the testing may be included in the medical chart of each participant as part of the diagnosis discussion with family.
Consent forms will be obtained from participants >18 years of age or who are emancipated minors, or guardian for patients <18 years of age. Guardian refers to parent, guardian, or legally authorized personnel throughout this document.
The data collected will be used to refine and improve the diagnostic testing for PCD at Arkansas Children's Hospital. This will also involve collaboration with other sub-specialties like otolaryngology and allergy/immunology. With continued research regarding nasal nitric oxide testing, this may involve the use of this test as a screening tool for PCD in the right clinical setting. This will involve analyzing the association of nasal nitric oxide levels with approved diagnostic tests (ciliary biopsy and genetic testing).
Testing will be completed using (Eco-Physics Analyzer CLD 88 sp with DENOX 88). this testing will be done in the Arkansas Children's hospital pulmonary function laboratory, once all training is completed.
研究设计
- 研究类型
- Observational
- 观察模型
- Other
- 时间视角
- Prospective
入排标准
- 年龄范围
- 2 Years 至 99 Years（Child, Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Individuals who are diagnosed with Primary Ciliary Dyskinesia OR
- •Individuals with abnormal PCD diagnostics (abnormal ciliary biopsy or PCD genetics) OR
- •Individuals with unexplained bronchiectasis OR
- •Individuals undergoing PCD diagnostic testing (ciliary biopsy, PCD genetic testing) or concern based on clinical symptoms (at least two of the following):
- •Neonatal respiratory distress
- •Organ laterality defects
- •Year-round cough starting in first year of life or bronchiectasis on chest CT
- •Year-round nasal congestion starting in first year of life or pansinusitis
- •Multiple ear infections in the first two years of life with sequelae (e.g. ear tubes, chronic effusion, abnormal audiological exam)
- •History of recurrent pneumonias (at least 2 in one year or more than 3 at any time)
- •Ability to provide consent for participation in study by the participants or guardian
- •Ability to perform the test
- •Age >= 2 years of age
排除标准
- •Individuals who are unable to understand the requirements of the study.
- •Individuals (or guardians) who are unwilling to provide consent.
- •Individuals who are unable to complete the testing
- •Recent history of sinus surgery (within four weeks) or bloody nose (within one week) of testing (they can be included at a later date)
- •Patients who are currently being treated (within one week) with antibiotics for sinusitis or respiratory symptoms (they can be included at a later date)
- •Age <2 years of age
结局指标
主要结局
Measuring nasal nitric oxide in comparison to ciliary biopsy and genetic testing for the diagnosis of PCD
时间窗: throughout the study, an average of 3 years
Individuals with PCD have characteristically low values of nNO; values below an established cut-off of 77nL/min are strongly associated with PCD in compatible clinical settings. These values are not well established for individuals who are in 2-5 years of age. nNO measurements (in nL/min) in 2-5 years of age will be compared to the diagnosis of PCD and assessed to establish diagnostic cut-offs.
次要结局
- Comparing tidal breathing to resistor method(throughout the study, an average of 3 years)