La Revue de médecine interne
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Rare thrombophilic states are mostly associated with recurrent venous thrombosis or severe thrombosis such as neonatal purpura fulminans. We review here the various causes of rare thrombophilic states. ⋯ These uncommon thrombophilic states require a treatment in a specialized department.
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A man with no atopic antecedent, but who was being treated with a Angiotensin-converting enzyme inhibitor (ACE), was admitted to hospital for an edema affecting the face and tongue. The symptoms included dyspnea and dysphagia but not pruritus or dermal erythema. ⋯ Treatment with C1 inhibitor concentrate (1000u) made the clinical symptoms disappear within 20 minutes. The resulting angioedema induced seems to be linked to the bradykinin metabolism, which would not be any better served by the angiotensin-converting enzyme, which normally inactivates about 75% of it.