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Clinical Trials/DRKS00032870
DRKS00032870
Recruiting
未知

Radiological and biochemical effects in children, adolescents and young adults under supplementation with the dietary supplement choline on cystic fibrosis-associated liver disease. - SHIELD (Substitution in choline defcient CFALD)

niversitätsklinik Tübingen0 sites50 target enrollmentOctober 16, 2023

Overview

Phase
未知
Intervention
Not specified
Conditions
Cystic fibrosis associated liver disease
Sponsor
niversitätsklinik Tübingen
Enrollment
50
Status
Recruiting
Last Updated
last year

Overview

Brief Summary

No summary available.

Registry
who.int
Start Date
October 16, 2023
End Date
TBD
Last Updated
last year
Study Type
Interventional
Sex
All

Investigators

Sponsor
niversitätsklinik Tübingen

Eligibility Criteria

Inclusion Criteria

  • Patients aged 6 years and older up to the age of 25 years with a diagnosis of cystic fibrosis.
  • \- Detection of the CFTR genotype
  • \- Exocrine pancreatic insufficiency
  • \- Presence of CF\-associated hepatopathy (steatosis, elevation of hepatocellular enzymes, liver parenchymal changes in the sense of fibrosis).
  • \- Laboratory evidence of at least once decreased plasma concentration of choline (\<8\.1µmol/l) with recommendation for supplementation with a choline\-containing food supplement.
  • \- Written informed consent and agreement to the data protection declaration

Exclusion Criteria

  • \- Lack of informed consent
  • \- Patients who want to implement the recommendation for choline supplementation in any case, i.e., cannot be randomized
  • \- Chronic alcohol consumption
  • \- Concomitant liver disease apart from CFALD, such as infectious or congenital or autoimmune liver disease, Meulengracht disease, or Wilson disease
  • \- Irreversible cirrhotic liver remodeling
  • \- Patients in whom there is no indication for blood sampling outside of the trial
  • \- Critically ill patients who cannot be expected to undergo additional sonography or MRI examinations or who have a contraindication to MRI examinations
  • \- Patients who require sedation/anesthesia to undergo sonography or MRI examination.
  • \- Participation in another interventional study or planned start of another interventional study within the study period planned here, for example with a disease modifier (modulator).

Outcomes

Primary Outcomes

Not specified

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