Abstract
Abstract
Hereditary Angioedema is a hereditary disease caused by deficiency or loss of C1 inhibitor (C1-INH), with an autosomal dominant inheritance pattern and which may be potentially life threatening. The prevalence of the disease is considerably low. In this case report, a 31 years old woman who has applied to the hospital with an angioedema attack after odontotherapy and who had angioedema attacks for many years with abdominal symptoms in the forefront and as a consequence to whom laparotomy was applied 3 times, is discussed together with literature. Hereditary Angioedema must certainly be considered for patients who apply to hospital with localized angioedema and especially with symptoms relating to upper airway tract and treatment strategy must be determined urgently, because delay in treatment increases morbidity and affects life quality.
Keywords: Hereditary, angioedema, type ΙΙ
Özet
Herediter Anjioödem, C1 İnhibitör (C1-INH) fonksiyon kaybı veya eksikliği ile ortaya çıkan, otozomal dominant geçişli ve potansiyel olarak yaşamı tehdit edebilen kalıtsal bir hastalıktır. Hastalığın prevelansı oldukça düşüktür. Bu olgu sunumunda uzun yıllardır abdominal semptomların ön planda olduğu, anjioödem atakları geçiren ve bu nedenle 3 kez laparotomi uygulanan, diş tedavisi sonrası anjioödem atağı ile başvuran 31 yaşında kadın hasta literatür eşliğinde tartışıldı. Lokalize anjioödemle ve özellikle üst havayolunu ilgilendiren semptomlarla başvuran hastalarda herediter anjioödem mutlaka akılda bulundurulmalı, acil tedavi stratejisi belirlenmelidir. Çünkü tedavideki gecikme morbiditeyi arttırır ve yaşam kalitesini olumsuz etkiler.
Anahtar sözcükler: Herediter, anjioödem, tip ΙΙ
Keywords
References
- Giavina-Bianchi P, França AT, Grumach AS, Motta AA, Fernandes FR, Campos RA, Valle SO, Rosário NA, Sole D. Brazilian Guidelines for the Diagnosis and Treatment of Hereditary Angioedema. Clinics 2011; 66: 1627-36.
- Kasamatsu Y, Yoshinoya K, Kasamatsu Y, Yamamoto T, Horiuchi T, Kadoya M.A case of hereditary angioedema involving recurrent abdominal attacks. Intern Med 2011; 50: 2911-4.
- Zurow BL. HAE Therapies: Past Present and Future. Allergy Asthma Clin Immunol 2010; 6: 23.
- Zuraw BL, Christiansen SC. Pathophysiology of Hereditary Angioedema. Am J Rhinol Allergy 2011; 25: 373-8.
- Bork K, Meng G, Staubach P, Hardt J. Hereditary angioedema: new findings concerning symptoms, affected organs, and course. Am J Med 2006; 119: 267- 74.
- Bork K. Recurrent angioedema and the threat of asphyxiation. Dtsch Arztebl Int 2010; 107: 408-14.
- Locascio EJ, Mahler SA, Arnold TC. Intestinal Angioedema Misdiagnosed as Recurrent Episodes of Gastroenteritis. West J Emerg Med 2010; 11: 391-4.
- Church JA. Oxandrolone Treatment of Chilhood Hereditary Angioedema. Ann Allergy Asthma Immunol 2004; 92: 377-8.
- Sheffer AL, Fearon DT, Austen KF, Rosen FS. Tranexamic Acid: Preoperative Prophylactic Therapy for Patients with Hereditary Angioneurotic Edema. J Allergy Clin Immunol 1977; 60: 38-40.
- Lyseng-Williamson KA. Nanofiltreted Human C1 Inhibitor Concentrate (Cinryze): In Hereditary Angioedema. Biodrugs 2011; 25: 317-27.
- Gompels MM, Lock RJ, Morgan JE, Osborne J, Brown A, Virgo PF. A multicentre evaluation of the diagnostic efficiency of serological investigations for C1 inhibitor deficiency. J Clin Pathol 2002; 55: 145-7.
Abstract
Herediter Anjioödem, C1 İnhibitör (C1-INH) fonksiyon kaybı veya eksikliği ile ortaya çıkan, otozomal dominant geçişli ve potansiyel olarak yaşamı tehdit edebilen kalıtsal bir hastalıktır. Hastalığın prevelansı oldukça düşüktür. Bu olgu sunumunda uzun yıllardır abdominal semptomların ön planda olduğu, anjioödem atakları geçiren ve bu nedenle 3 kez laparotomi uygulanan, diş tedavisi sonrası anjioödem atağı ile başvuran 31 yaşında kadın hasta literatür eşliğinde tartışıldı. Lokalize anjioödemle ve özellikle üst havayolunu ilgilendiren semptomlarla başvuran hastalarda herediter anjioödem mutlaka akılda bulundurulmalı, acil tedavi stratejisi belirlenmelidir. Çünkü tedavideki gecikme morbiditeyi arttırır ve yaşam kalitesini olumsuz etkiler
References
- Giavina-Bianchi P, França AT, Grumach AS, Motta AA, Fernandes FR, Campos RA, Valle SO, Rosário NA, Sole D. Brazilian Guidelines for the Diagnosis and Treatment of Hereditary Angioedema. Clinics 2011; 66: 1627-36.
- Kasamatsu Y, Yoshinoya K, Kasamatsu Y, Yamamoto T, Horiuchi T, Kadoya M.A case of hereditary angioedema involving recurrent abdominal attacks. Intern Med 2011; 50: 2911-4.
- Zurow BL. HAE Therapies: Past Present and Future. Allergy Asthma Clin Immunol 2010; 6: 23.
- Zuraw BL, Christiansen SC. Pathophysiology of Hereditary Angioedema. Am J Rhinol Allergy 2011; 25: 373-8.
- Bork K, Meng G, Staubach P, Hardt J. Hereditary angioedema: new findings concerning symptoms, affected organs, and course. Am J Med 2006; 119: 267- 74.
- Bork K. Recurrent angioedema and the threat of asphyxiation. Dtsch Arztebl Int 2010; 107: 408-14.
- Locascio EJ, Mahler SA, Arnold TC. Intestinal Angioedema Misdiagnosed as Recurrent Episodes of Gastroenteritis. West J Emerg Med 2010; 11: 391-4.
- Church JA. Oxandrolone Treatment of Chilhood Hereditary Angioedema. Ann Allergy Asthma Immunol 2004; 92: 377-8.
- Sheffer AL, Fearon DT, Austen KF, Rosen FS. Tranexamic Acid: Preoperative Prophylactic Therapy for Patients with Hereditary Angioneurotic Edema. J Allergy Clin Immunol 1977; 60: 38-40.
- Lyseng-Williamson KA. Nanofiltreted Human C1 Inhibitor Concentrate (Cinryze): In Hereditary Angioedema. Biodrugs 2011; 25: 317-27.
- Gompels MM, Lock RJ, Morgan JE, Osborne J, Brown A, Virgo PF. A multicentre evaluation of the diagnostic efficiency of serological investigations for C1 inhibitor deficiency. J Clin Pathol 2002; 55: 145-7.
Details
| Primary Language | English |
|---|---|
| Journal Section | Case Reports |
| Authors | |
| Publication Date | March 11, 2012 |
| Published in Issue | Year 2012Volume: 34 Issue: 4 |