An observational study in Hereditary Hemorrhagic Telangiectasia, sponsored by Centre Hospitalier Universitaire Dijon. Completed at 1 site in France. Open to participants aged 18 Years and older, including healthy volunteers. Per ClinicalTrials.gov, last updated 2020-11-20.
Sponsored by Centre Hospitalier Universitaire Dijon · Observational
Hereditary hemorrhagic telangiectasia (HHT) results from genetic deregulation of angiogenesis. It is characterized by mucocutaneous telangiectasia responsible for recurrent epistaxis affecting quality of life (anaemia, iron deficiency, social distress). More rarely, HHT is complicated by the appearance of pulmonary, hepatic or cerebral arteriovenous malformations that can lead to serious complications: cerebrovascular accidents, cerebral abscesses, high output heart failure, and massive hemoptysis (1). The intensity of symptoms increases with age but with significant individual variability, even for the same mutation in the same family. Thus, while the mutations responsible for the disease have been identified, the pathophysiology is not fully understood because these mutations do not explain the great diversity of clinical presentations. Other factors not yet identified probably play an important role. Angiogenic T cells (TANG) are a newly individualized T cell population, defined by a CD4+CXCR4+CD31+ phenotype, which plays a key role in differentiating endothelial progenitors (2).
In an earlier study, the investigators showed that patients with HHT had a decrease in CD4+ and CD8+ LT compared to a cohort of healthy subjects (3).
They hypothesize that the lymphopenia mainly involves TANG, whose quantification could make it possible to assess the individual level of angiogenesis during HHT. The evaluation of the TANG levels could thus make it possible to personalize HHT management.
153 studies on the registry are indexed under Telangiectasis; 16 are open to participants now.
This study's enrollment of 60 is below the median of 100 across 37 observational studies indexed under Telangiectasis.
Browse Telangiectasis studies →Centre Hospitalier Universitaire Dijon is the lead sponsor of 495 studies on the registry; 105 are open to participants now.
Counted across the registry records on this site, refreshed daily.
outpatient
"Patient" group:
"Control" group :
Exclusion Criteria:
Treatment in progress or stopped less than 6 months ago or to be introduced within the next 3 months of the following medications:
Hereditary hemorrhagic telangiectasia patients
Biological: Blood samples · Other: Epistaxis charts
Matched for age (+/- 5 ans) and sex.
Biological: Blood samples
* 5 mL dry tube to separate serum * Two 6 mL EDTA tubes for plasma separation * Eight 6 mL heparinized tubes for flow cytometry (quantification of TANG such as CD3+CD31+CXCR4+ and CEC) and quantification of angiogenesis markers.
Three monthly epistaxis charts to be completed
Average monthly duration (in minutes) of epistaxis over the 3 months following inclusion
Time frame: Through study completion, an average of 3 months
Number/mm3 of circulating TANG (CD3+CXCR4+CD31+) at inclusion.
Time frame: At inclusion
This study is completed, as verified in Nov 2019. You cannot join it, but the record below documents what was studied.
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Centre Hospitalier Universitaire Dijon