Acil Serviste Herediter Anjioödem’e Yaklaşım

Öner Özdemir

doi:10.31832/smj.627591

Acil Serviste Herediter Anjioödem’e Yaklaşım

Abstract

Herediter anjioödem (HAÖ) nadir görülen, genelde C1 inhibitör (C1-INH) eksikliğine bağlı, tekrarlayan anjioödem ataklarıyla bilinen ölümcül olabilen otozomla dominant geçişli bir hastalıktır. Anjioödem atakları aniden oluşur, sıklıkla tahmin edilemez ve tipik olarak ekstremite, gövde, yüz, karın, genital organlar ve üst solunum yollarını tutan şişliklere yol açar. Herediter anjioödem hastalarında tipik olarak eşlik eden ürtiker olmaması sıklıkla nonhistaminerjik (bradikinerjik) anjioödem şeklinde düşünülmesine yol açar. Ataklar önemli derecede işlevsel bozukluğa, yaşam kalitesinin azalmasına ve larengeal atak olgularında ise ölüme yol açabilir. Herediter anjioödemin karın tutulumuyla giden atağı genelde tanıda gecikme ve uygunsuz tedaviye neden olan ailevi Akdeniz ateşi (FMF) ve apandisit gibi bazı akut batın durumlarını taklit eder. Kompleksliğinden dolayı HAÖ hastalarının bakımı bazen zorlayıcı olabilir. Herediter anjioödem tedavisi kısa ve uzun süreli profilaksi ile atak tedavilerini içerir. Acilde HAÖ bakımı yanlış tanı ve sınırlı sayıda tedavi seçeneği ile gecikebilir. Son iki dekattır HAÖ atak tedavisinde bazı tedavi seçenekleri mevcuttur. Bu seçeneklerin çoğu ABD, Avrupa, Birleşik Krallık ve Avustralya’da yaşayan HAÖ hastaları için günümüzde ulaşılabilir durumda olmasına rağmen, gelişmekte olan ülkelerde sınırlı sayıda tercihler hazırda bulunur. Dört tür akut/atak tedavi seçenekleri arasında, plazma kaynaklı C1-INH konsantresi, rekombinant C1-INH konsantresi, ecallantide ve ikatibant günümüzde kullanılanlardır.

Keywords

Herediter anjioödem,atak,acil servis

Supporting Institution

Yok

Project Number

Yok

Thanks

Yok

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Details

Primary Language

Turkish

Subjects

Health Care Administration

Journal Section

Review

Authors

Öner Özdemir ^*
0000-0002-5338-9561
Türkiye

Publication Date

March 16, 2020

Submission Date

October 1, 2019

Acceptance Date

February 9, 2020

Published in Issue

Year 2020 Volume: 10 Number: 1

DOI

https://doi.org/10.31832/smj.627591

IZ

https://izlik.org/JA43UH52PZ

Cite

RIS / Bibtex

APA

Özdemir, Ö. (2020). Acil Serviste Herediter Anjioödem’e Yaklaşım. Sakarya Medical Journal, 10(1), 152-161. https://doi.org/10.31832/smj.627591

AMA

1.Özdemir Ö. Acil Serviste Herediter Anjioödem’e Yaklaşım. Sakarya Medical Journal. 2020;10(1):152-161. doi:10.31832/smj.627591

Chicago

Özdemir, Öner. 2020. “Acil Serviste Herediter Anjioödem’e Yaklaşım”. Sakarya Medical Journal 10 (1): 152-61. https://doi.org/10.31832/smj.627591.

EndNote

Özdemir Ö (March 1, 2020) Acil Serviste Herediter Anjioödem’e Yaklaşım. Sakarya Medical Journal 10 1 152–161.

IEEE

[1]Ö. Özdemir, “Acil Serviste Herediter Anjioödem’e Yaklaşım”, Sakarya Medical Journal, vol. 10, no. 1, pp. 152–161, Mar. 2020, doi: 10.31832/smj.627591.

ISNAD

Özdemir, Öner. “Acil Serviste Herediter Anjioödem’e Yaklaşım”. Sakarya Medical Journal 10/1 (March 1, 2020): 152-161. https://doi.org/10.31832/smj.627591.

JAMA

1.Özdemir Ö. Acil Serviste Herediter Anjioödem’e Yaklaşım. Sakarya Medical Journal. 2020;10:152–161.

MLA

Özdemir, Öner. “Acil Serviste Herediter Anjioödem’e Yaklaşım”. Sakarya Medical Journal, vol. 10, no. 1, Mar. 2020, pp. 152-61, doi:10.31832/smj.627591.

Vancouver

1.Öner Özdemir. Acil Serviste Herediter Anjioödem’e Yaklaşım. Sakarya Medical Journal. 2020 Mar. 1;10(1):152-61. doi:10.31832/smj.627591

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