Investigation of the relevance of impaired nitric oxide (NO) expression and CFTR dysfunction for the pathogenesis of neurological complications in patients with cystic fibrosis compared to other chronic lung diseases - NO Chloride Project

Klinik für Kinder- und Jugendmedizin am St. Josef Hospital0 个研究点目标入组 90 人开始时间: 2022年10月5日最近更新: 2024年8月19日

适应症

试验速览

暂无简介。

•- Patients with 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 (intestinal short-current measurement (ICM) or nasal potential difference (nPD)).
•- Age = 8 years
•- Consent to the study
•- No febrile infection or otherwise reliably diagnosed exacerbation in the last 4 weeks
•- The group will be split into 2 subgroups depending on the status of modulator therapy:
•Group 1.1: No modulator therapy prior to baseline measurement.
•Control measurement at the earliest 3 months after initiation of modulator therapy
•Group 1.2: Modulator therapy for at least 3 months
•- Patients with confirmed diagnosis of PCD (genetically confirmed or at least highly likely according to currently valid ERS criteria; ERS Guideline 2016).
•- Age = 8 years
•- Consent to the study
•- No febrile infection or otherwise reliably diagnosed exacerbation in the last 4 weeks.
•- Age = 8 years
•- Consent to the study
•- No febrile infection in the last 4 weeks
•- Neither CF, PCD, bronchial asthma nor other underlying disease associated with increased inflammation

•1) Age < 8 years
•2) Pre-existing or family history of hereditary neuromuscular disease.
•3) Skin disease, inflammation or injury in the area of measurement during QST.
•4) Local treatment in the area of measurement with topical local anaesthetics (= 7 days in the last 6 weeks or = 7 days in the last 4 months) or with capsaicin (in the last 6 months) for QST
•5) Pronounced hypersensitivity/allodynia.
•6) Lack of consent from the guardian or subject.
•7) Consumption of a special diet in the last 2 weeks prior to the time of the study.
•8) Consumption of canned fish on the previous day
•9) acute pulmonary exacerbation according to modified Fuchs criteria in the last 4 weeks
•10) Acute infection with SARS-CoV2 in the last 3 months or persistent symptoms after a SARS-CoV2 infection in the sense of a Long-CoViD

发起方

Klinik für Kinder- und Jugendmedizin am St. Josef Hospital