Hereditary Bradykinetic Angioedema
Conditions
Brief summary
The objective is to compare the gene expression of B1 and / or B2 monocyte receptors between patients with hereditary bradykinetic angioedema and control subjects.
Interventions
OTHERExplorations
On Day 1 vascular fonctions explorations performed
OTHERBlood samples
On Day 1 35ml taken
Sponsors
University Hospital, Rouen
Study design
Allocation
NON_RANDOMIZED
Intervention model
PARALLEL
Primary purpose
OTHER
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
Yes
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.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| compare the gene expression of B1 and / or B2 monocyte receptors | Day 1 | compare between patients with hereditary bradykinetic angioedema and control subjects. |
Countries
France
Outcome results
None listed