Treatment of Hereditary Angioedema items that need to be addressed in practice parameter 英文参考文献.docVIP

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Treatment of Hereditary Angioedema items that need to be addressed in practice parameter 英文参考文献.doc

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Treatment of Hereditary Angioedema items that need to be addressed in practice parameter 英文参考文献

Dagen and Craig Allergy, Asthma Clinical Immunology 2010, 6:11 /content/6/1/11 ALLERGY, ASTHMA CLINICAL IMMUNOLOGY REVIEW Open Access Treatment of Hereditary Angioedema: items that Review need to be addressed in practice parameter Callie Dagen1 and Timothy J Craig*2 Abstract Background: Hereditary Angioedema (HAE) is a rare, autosomal dominant (AD) disorder caused by a C1 esterase inhibitor (C1-inh) deficiency or qualitative defect. Treatment of HAE in many parts of the world fall short and certain items need to be addressed in future guidelines. Objective: To identify those individuals who should be on long-term prophylaxis for HAE. Additionally, to determine if prodromal symptoms are sensitive and specific enough to start treatment with C-1 INH and possibly other newly approved therapies. Also, to discuss who is appropriate to self-administer medications at home and to discuss training of such patients. Methods: A literature review (PubMed and Google) was performed and articles published in peer-reviewed journals, which addressed HAE prophylaxis, current HAE treatments, prodromal symptoms of HAE and self-administration of injected home medications were selected, reviewed and summarized. Results: Individuals whom have a significant decrease in QOL or have frequent or severe attacks and who fail or are intolerant to androgens should be considered for long-term prophylaxis with C1INH. Prodromal symptoms are sensitive, but non-specific, and precede acute HAE attacks in the majority of patients. Although the treatment of prodromal symptoms could lead to occasional overtreatment, it could be a viable option for those patients able to adequately predict their attacks. Finally, self-administration, has been shown to be feasible, safe and effective for patients who require IV therapy for multiple other diseases to include, but not limited to, hemophilia. Conclusions: Prophylactic therapy, treatment at the time of prodromal symptoms and self-administration a