Involvement of Monocytic B1 and B2 Receptors in Inflammation and Chronic Vascular Disease in Patients With Hereditary Bradykinetic Angioedema

Study Overview
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Brief Summary
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The objective is to compare the gene expression of B1 and / or B2 monocyte receptors between patients with hereditary bradykinetic angioedema and control subjects.

Official Title
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Involvement of Monocytic B1 and B2 Receptors in Inflammation and Chronic Vascular Disease in Patients With Hereditary Bradykinetic Angioedema

Conditions
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Hereditary Bradykinetic Angioedema

Intervention / Treatment
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* Other: Explorations
* Other: Blood samples


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria (patients): Patient with inherited bradykinic angioedema due to quantitative or qualitative C1-inhibitor deficiencies, regardless of the frequency or severity of seizures and regardless of their background treatment, with the following diagnostic criteria: C1-inhibitor rate <50% of normal Repeated episodes characteristic of bradykinic angioedema Hereditary nature of the disease. Person who read and understood the newsletter and signed the consent form Person affiliated with a social security scheme Effective contraception in women of childbearing age (negative pregnancy test). For postmenopausal women, a confirmatory diagnosis should be obtained (amenorrhea for at least 12 months before the inclusion visit). Inclusion Criteria (volunteers): Person affiliated with a social security scheme Person who read and understood the newsletter and signed the consent form Effective contraception in women of childbearing age (negative pregnancy test). For postmenopausal women, a confirmation diagnosis should be obtained (amenorrhea for at least 12 months before the inclusion visit) Exclusion Criteria (patients): Angioedema crisis less than 1 month old Chronic inflammatory disease such as rheumatoid arthritis or Crohn's disease. Acute infection in progress, with or without anti-infectious treatment Contraindication to the use of trinitrin: Hypersensitivity to nitrates or to any of the excipients shock, severe hypotension, obstructive cardiomyopathy, inferior court inferior myocardial infarction with right ventricular extension, except in case of evidence of left ventricular failure, intracranial hypertension, patient treated with sildenafil Pregnant or parturient or breastfeeding woman or lack of proven contraception Person deprived of liberty by an administrative or judicial decision or person placed under the protection of justice / sub-tutorship or curatorship Patient participating or having participated in another therapeutic trial within one week. Exclusion Criteria (volunteers) Chronic inflammatory disease such as rheumatoid arthritis or Crohn's disease. Acute infection in progress, with or without anti-infectious treatment Contraindication to the use of trinitrin: Hypersensitivity to nitrates or to any of the excipients shock, severe hypotension, obstructive cardiomyopathy, inferior court inferior myocardial infarction with right ventricular extension, except in case of evidence of left ventricular failure, intracranial hypertension, patient treated with sildenafil Pregnant or parturient or breastfeeding woman or lack of proven contraception Person deprived of liberty by an administrative or judicial decision or person placed under the protection of justice / sub-tutorship or curatorship Person participating or having participated in another therapeutic trial within one week.

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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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Other
Allocation: Non-Randomized
Intervention Model: Parallel Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Other: Patients hereditary bradykinetic angioedema<br> | Other: Explorations<br>* On Day 1 vascular fonctions explorations performed<br>Other: Blood samples<br>* On Day 1 35ml taken<br>|
| Other: healthy volunteers<br> | Other: Explorations<br>* On Day 1 vascular fonctions explorations performed<br>Other: Blood samples<br>* On Day 1 35ml taken<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| compare the gene expression of B1 and / or B2 monocyte receptors | compare between patients with hereditary bradykinetic angioedema and control subjects. | Day 1 |