involvement of monocytic b1 and b2 receptors in inflammation and chronic vascular disease in patients with hereditary bradykinetic angioedema

Conditions: Hereditary Bradykinetic Angioedema

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.


Eligibility criteria:
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.