C1 ESTERASE INHIBITOR DEFICIENCY

Course overview

C1 ESTERASE INHIBITOR DEFICIENCY, ALSO KNOWN AS HEREDITARY ANGIOEDEMA, RESULTS IN THE UNCHECKED PRODUCTION OF THE VASODILATOR BRADYKININ. THIS INCREASE IN BRADYKININ LEADS TO AN INCREASE IN SMOOTH MUSCLE RELAXATION IN THE WALLS OF BLOOD VESSELS AND RESULTANT EDEMA IN THE HANDS, FEET, GASTROINTESTINAL TRACT, AND IN SEVERE CASES, THE LARYNX. IN SEVERE CASES, THE AIRWAY CAN BE COMPROMISED AND EFFECTIVELY SWOLLEN CLOSED, PREVENTING AIR MOVEMENT INTO AND OUT OF THE LUNGS. THIS ACTIVITY REVIEWS THE PRESENTATION, EVALUATION, AND MANAGEMENT OF C1 ESTERASE INHIBITOR DEFICIENCY, AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN CARING FOR PATIENTS WITH THIS CONDITION.