Öz
Herediter anjioödem C1 inhibitor eksikliği veya disfonksiyonuna bağlı cilt ve mukoza ödemi ile karakterizedir. Herediter anjioödem hastalığının küratif tedavisi yoktur. Hamilelik boyunca hastalığın atak sıklığı artar. Tedavi anlamında temel yaklaşımlar atakları önleyici profilaktik uygulamalar ve akut atak tedavilerinden ibarettir. Bu yazıda; hamilelikte C1 inhibitör konsantresini, profilaksi ve atak tedavisi şeklinde kullanarak başarılı bir doğum yapan herediter anjioödem tanılı iki hasta sunulmuştur
Anahtar Kelimeler
Kaynakça
- Khan DA. Hereditary angioedema: Historical aspects, clas- sification, pathophysiology, clinical presentation, and labo- ratory diagnosis. Allergy Asthma Proc 2011;32:1-10.
- Frank MM. Update on preventive therapy (prophylaxis) for hereditary angioedema. Immunol Allergy Clin North Am 2013;33:495-503.
- Xu YY, Buyantseva LV, Agarwal NS et al. Update on tre- atment of hereditary angioedema. Clin Exp Allergy 2013;43:395-405.
- Gower RG. HAE update: determining optimal patient spe- cific therapy. Allergy Asthma Proc 2013;34:7-12.
- Czaller I, Visy B, Csuka D et al. The natural history of here- ditary angioedema and the impact of treatment with human C1-inhibitor concentrate during pregnancy: a long-term survey. Eur J Obstet Gynecol Reprod Biol 2010;152:44-9.
- Bork K, Staubach P, Eckardt AJ, Hardt J. Symptoms, course, and complications of abdominal attacks in hereditary angi- oedema due to C1 inhibitor deficiency. Am J Gastroenterol 2006;101:619.
- Caballero T, Farkas H, Bouillet L et al. International con- sensus and practical guidelines on the gynecologic and obs- tetric management of female patients with hereditary an- gioedema caused by C1 inhibitor deficiency. J Allergy Clin Immunol 2012;129:308-20.
- Chan W, Berlin N, Sussman GL. Management of hereditary angioedema with C1-inhibitor concentrate during two suc- cessive pregnancies. Int J Gynaecol Obstet 2013;120:189-90.
Öz
Hereditary angioedema is characterized by skin and mucosal edema due to C1 inhibitor deficiency or dysfunction. There is no curative treatment of the illness. Attack frequency increases in most of the cases during pregnancy. In the context of treatments, the basic approaches consist of preventive prophylactic applications and acute attack treatments. In this paper, we presented two hereditary angioedema patients with successful delivery using C1 inhibitor concentrate as prophylaxis and demand therapy
Anahtar Kelimeler
Kaynakça
- Khan DA. Hereditary angioedema: Historical aspects, clas- sification, pathophysiology, clinical presentation, and labo- ratory diagnosis. Allergy Asthma Proc 2011;32:1-10.
- Frank MM. Update on preventive therapy (prophylaxis) for hereditary angioedema. Immunol Allergy Clin North Am 2013;33:495-503.
- Xu YY, Buyantseva LV, Agarwal NS et al. Update on tre- atment of hereditary angioedema. Clin Exp Allergy 2013;43:395-405.
- Gower RG. HAE update: determining optimal patient spe- cific therapy. Allergy Asthma Proc 2013;34:7-12.
- Czaller I, Visy B, Csuka D et al. The natural history of here- ditary angioedema and the impact of treatment with human C1-inhibitor concentrate during pregnancy: a long-term survey. Eur J Obstet Gynecol Reprod Biol 2010;152:44-9.
- Bork K, Staubach P, Eckardt AJ, Hardt J. Symptoms, course, and complications of abdominal attacks in hereditary angi- oedema due to C1 inhibitor deficiency. Am J Gastroenterol 2006;101:619.
- Caballero T, Farkas H, Bouillet L et al. International con- sensus and practical guidelines on the gynecologic and obs- tetric management of female patients with hereditary an- gioedema caused by C1 inhibitor deficiency. J Allergy Clin Immunol 2012;129:308-20.
- Chan W, Berlin N, Sussman GL. Management of hereditary angioedema with C1-inhibitor concentrate during two suc- cessive pregnancies. Int J Gynaecol Obstet 2013;120:189-90.
Ayrıntılar
| Birincil Dil | Türkçe |
|---|---|
| Bölüm | Case Report |
| Yazarlar | |
| Yayımlanma Tarihi | 1 Aralık 2016 |
| Yayımlandığı Sayı | Yıl 2016 Cilt: 26 Sayı: 4 |
Genel Tıp Dergisi Creative Commons Atıf-GayriTicari 4.0 Uluslararası Lisansı (CC BY NC) ile lisanslanmıştır.