Longitudinal Analysis of Respiratory and Intestinal Microbiome in Cystic Fibrosis

Study Overview
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Brief Summary
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Respiratory und intestinal microbiome will bei analyzed during a period of 6 months. In a retrospective analysis it will be looked for correlations between microbiome and cf therapy (e.g. inhaled and systemic antibiotics, cftr modifiers, proton pump Inhibitors, enzymes, nutritional habits), clinical status and self reported outcome.

Detailed Description
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DNA will be isolated from sputum, throat swabs and stool samples and used for the generation of microbial profiles. Both bacterial and fungal profiles will be analyzed by next generation sequencing. Clinical outcome parameters (lung function test, weight, calprotectin, elastase, blood parameters, self reported outcome) will be recorded and analyzed along with medication.

Official Title
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Longitudinal Analysis of Respiratory and Intestinal Microbiome in Cystic Fibrosis

Conditions
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Cystic Fibrosis

Intervention / Treatment
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* Diagnostic Test: analysis of microbiome


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: subject has confirmed diagnosis of cystic fibrosis (sweat chloride >60mmol/l and/or 2 mutations in the cftr gene known to cause cystic fibrosis) subject is able to perform informed consent Exclusion Criteria: inability to give informed consent antibiotic therapy in the last 4 weeks prior to study start (exception: long term azithromycin therapy, long term antistaphylococcal therapy, long term inhaled antibiotics)

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| cf patients at the cf centre Kiel<br>microbiome of cf patients at the cf centre Kiel will be analyzed and correlated to standard cf care. | Diagnostic Test: analysis of microbiome<br>* microbial profiling by next generation sequencing<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| respiratory microbiome | abundance [operational taxonomic units] | 24 weeks | 
| intestinal microbiome | abundance [operational taxonomic units] | 24 weeks | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Forced Exspiratory Volume FEV1 [%pred] | performed by bodyplethmograph | 24 weeks | 
| weight (kg) | for children, height (cm) is also recorded | 24 weeks | 
| Quality of life [score] | Cystic Fibrosis Questionnaire Revised CFQ-R [score 0-100, higher scores indicating better Quality of Life] | 24 weeks | 
| Quality of life [score] | Kiel Questionnaire Kiel-Q [score 0-100, higher scores indicating hetter Quality of Life] | 24 weeks | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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diversity, respiratory and intestinal microbiome, antibiotic therapy, cftr modifier