R52 R43
Conditions
Interventions
Group 1: Group 1:
Examination of CF patients with and without modulator therapy.
Group 2: Group 2: Examination of subjects with PCD
Group 3: In a second step
age-matched healthy subjects (group 3) a
Sponsors
Klinik für Kinder- und Jugendmedizin am St. Josef Hospital
Eligibility
Sex/Gender
All
Age
8 Years to No maximum
Inclusion criteria
Inclusion criteria: Group 1: - 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 Group 2: - 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. Group 3: - 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
Exclusion criteria
Exclusion criteria: 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
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| The comparison of the QST results between 3 groups (CF, PCD and healthy people) is carried out. Our initial hypothesis is that patients with CF have a lowered pain threshold for thermal and mechanical stimuli due to an increased vulnerability, which can be measured by quantitative sensory testing (QST). | — |
Secondary
| Measure | Time frame |
|---|---|
| Electrophysiological examination of the sural and ulnar nerve bds. We have two hypotheses for the causality of this lowered pain threshold which we want to test: 1.) Decreased NO lowers the pain threshold: Measurement of NO metabolism parameters in blood and urine including measurement of exhaled and nasal NO. 2) The lowered pain threshold depends directly or indirectly (via peripheral neuropathies) on the extent of CFTR dysfunction: Perform iontophoresis as an indirect measure of CFTR function. | — |
Countries
Germany
Contacts
Public ContactAnna Teresa Hoffmann
Universitätsklinikum Bochum Katholisches Klinikum Bochum Studienzentrum Kinderklinik
Outcome results
None listed