tailieunhanh - Báo cáo y học: "Successful C1 inhibitor short-term prophylaxis during redo mitral valve replacement in a patient with hereditary angioedema"

Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học Wertheim cung cấp cho các bạn kiến thức về ngành y đề tài: Successful C1 inhibitor short-term prophylaxis during redo mitral valve replacement in a patient with hereditary angioedema. | Bernstein et al. Journal of Cardiothoracic Surgery 2010 5 86 http content 5 1 86 JOTS JOURNAL OF CARDIOTHORACIC SURGERY CASE REPORT Open Access Successful C1 inhibitor short-term prophylaxis during redo mitral valve replacement in a patient with hereditary angioedema Jonathan A Bernstein1 t Suzanne Coleman2t Arturo J Bonnin3t Abstract Hereditary angioedema is characterized by sudden episodes of nonpitting edema that cause discomfort and pain. Typically the extremities genitalia trunk gastrointestinal tract face and larynx are affected by attacks of swelling. Laryngeal swelling carries significant risk for asphyxiation. The disease results from mutations in the C1 esterase inhibitor gene that cause C1 esterase inhibitor deficiency. Attacks of hereditary angioedema result from contact complement and fibrinolytic plasma cascade activation where C1 esterase inhibitor irreversibly binds substrates. Patients with hereditary angioedema cannot replenish C1 esterase inhibitor levels on pace with its binding. When C1 esterase inhibitor is depleted in these patients vasoactive plasma cascade products cause swelling attacks. Trauma is a known trigger for hereditary angioedema attacks and patients have been denied surgical procedures because of this risk. However uncomplicated surgeries have been reported. Appropriate prophylaxis can reduce peri-operative morbidity in these patients despite proteolytic cascade and complement activation during surgical trauma. We report a case of successful short-term prophylaxis with C1 esterase inhibitor in a 51-year-old man with hereditary angioedema who underwent redo mitral valve reconstructive surgery. Background Attacks of hereditary angioedema HAE are characterized by sudden episodes of brawny nonpitting edema causing discomfort and pain 1 . Areas of the body typically affected include the extremities genitalia trunk gastrointestinal tract face and larynx. Untreated patients with HAE are at risk for deadly .