Impaired Nitric Oxide Synthetase activity in Primary Ciliary Dyskinesia – data driven hypothesis - PCD-NO-Arginine
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 63
研究概览
简要总结
Results In both lung diseases sputum concentrations of L-Arg (PCD 28.78 vs. CF 36.54 vs. HC 11.59 µmol/mg, p < 0.001), ADMA (PCD 0.13 vs. CF 0.18 vs. HC 0.01 µmol/mg, p < 0.001), hArg and ornithine/citrulline ratio (indirectly indicating arginase/ NO synthase activity ratio) were elevated in comparison to HC. However, nitrite and nitrate were decreased in PCD vs. HC (nitrite 5.74 vs. 9.68 µmol/mg, p < 0.001; nitrate 18.14 vs. 36.51 µmol/mg, p < 0.001), but increased in CF (nitrite 16.64 µmol/mg, nitrate 63.31 µmol/mg), indicating a PCD disease-specific effect. Metabolite concentrations in urine and plasma were similar between the groups. Conclusion Our data suggest increased sputum arginase activity and ADMA in both lung diseases with neutrophilic inflammation. In contrast, reduced NO metabolites in sputum only in pwPCD indicate an impaired NO synthesis in PCD.
研究设计
- 研究类型
- Observational
入排标准
- 年龄范围
- 10 Years 至 one（—）
- 性别
- All
入选标准
- •PCD patients:
- •- Confirmed diagnosis of PCD according to the current diagnostic standard (at least 2 pathological findings typical of PCDs in different cilia examination methods, or 3 congruent abnormal results in 1 cilia examination technique, obtained from different samples, or proving PCD molecular genetics)
- •- Age> = 10 years
- •- Consent to study participation
- •CF patients:
- •- Patients with a confirmed diagnosis of CF according to current diagnostic criteria (2x positive sweat test, positive CF molecular genetics and / or CF-typical CFTR dysfunction in CF electrophysiology (ICM or nPD))
- •- Age> = 10 years
- •- Consent to study participation
- •Healthy controls:
- •- Free from chronic respiratory diseases (such as asthma, bronchiectasis, etc.), free from acute infectious or inflammatory conditions (infections, trauma, fever), free from any chronic underlying disease with a potential inflammatory reaction / component
- •- Age> = 10 years
- •- Consent to study participation
排除标准
- •- special diet in the last 2 weeks before the examination
- •- consumption of canned fish the day before
- •- for PCD and CF patients: acute pulmonary exacerbation according to modified Fuchs criteria
- •- for healthy controls: acute infection