Clinical and immunological features of 44 common variable immunodeficiency patients: The experience of a single center in Turkey

Basit Öğe Kaydı
dc.authoridSinem Fırtına / 0000-0002-3370-8545en_US
dc.authorscopusidSinem Fırtına / 16642650000
dc.authorwosidSinem Fırtına / X-8520-2018
dc.contributor.authorNepesov, Serdar
dc.contributor.authorAygün, Fatma Deniz
dc.contributor.authorFırtına, Sinem
dc.contributor.authorCokugras H.
dc.contributor.authorCamcıoğlu, Yıldız
dc.date.accessioned2020-08-30T20:01:35Z
dc.date.available2020-08-30T20:01:35Z
dc.date.issued2020
dc.departmentİstinye Üniversitesi, Mühendislik ve Doğa Bilimleri Fakültesi, Moleküler Biyoloji ve Genetik Bölümüen_US
dc.description.abstractIntroduction and objectives: Common variable immunodeficiency (CVID) is one of the most prevalent forms of primary immunodeficiency characterized by hypogammaglobinemia. Its heterogeneous clinical features include recurrent respiratory tract infections and other complications such as gastrointestinal, autoimmunity, and lymphoproliferative disorders. The aim of this article is to evaluate the general characteristics of CVID patients. Materials and methods: Clinical and immunological features of 44 CVID patients were evaluated retrospectively with long-term follow-up. Patients who participated in the study were diagnosed according to the criteria of the European Society for Immunodeficiency Diseases (ESID). Results: The median age at onset of symptoms was 2.75 years (range six months to 17 years), and the median age at diagnosis was 7.75 years (range 4–20 years). The average delay in diagnosis was 4.6 years (range 1–14 years). Positive family history was 18.2%. Before treatment, patients’ median total serum IgG was 271.5 mg/dL, median IgA was 7.5 mg/dL, and median IgM was 21 mg/dL. Infections were the most common clinical manifestation, and 63.6% of patients presented with sinopulmonary infection as the first manifestation. Bronchiectasis developed in 23 CVID subjects, while bronchiectasis was detected prior to CVID diagnosis in eight patients. All patients received immunoglobulin replacement therapy, and one patient died because of granulomatous lymphocytic interstitial lung disease (GLILD). Conclusions: CVID is a heterogeneous group of immunologic disorders with unknown etiology. There are significant differences in the clinical presentation and prevalence of CVID-related complications among countries. Local guidelines for diagnosis and clinical follow-up are needed. © 2020 SEICAPen_US
dc.identifier.citationNepesov, S., Aygun, F. D., Firtina, S., Cokugras, H., & Camcioglu, Y. (2020). Clinical and immunological features of 44 common variable immunodeficiency patients: The experience of a single center in Turkey. Allergologia et Immunopathologia.en_US
dc.identifier.doi10.1016/j.aller.2019.12.008en_US
dc.identifier.issn0301-0546en_US
dc.identifier.pmid32299645en_US
dc.identifier.urihttps://doi.org/10.1016/j.aller.2019.12.008
dc.identifier.urihttps://hdl.handle.net/20.500.12713/301
dc.identifier.wosWOS:000604402200022en_US
dc.identifier.wosqualityQ4en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakPubMeden_US
dc.institutionauthorFırtına, Sinemen_US
dc.language.isoenen_US
dc.publisherElsevier Doymaen_US
dc.relation.ispartofAllergologia et Immunopathologiaen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectCommon Variable Immunodeficiencyen_US
dc.subjectHypogammaglobulinemiaen_US
dc.subjectImmunoglobulin Therapyen_US
dc.titleClinical and immunological features of 44 common variable immunodeficiency patients: The experience of a single center in Turkeyen_US
dc.typeArticleen_US

Dosyalar

Orijinal paket
Listeleniyor 1 - 1 / 1
Küçük Resim Yok
İsim:
16.pdf
Boyut:
375 KB
Biçim:
Adobe Portable Document Format
Açıklama:
Tam Metin / Full Text
İndir

Koleksiyon

PubMed İndeksli Yayınlar Koleksiyonu
Moleküler Biyoloji ve Genetik Bölümü Makale Koleksiyonu
WoS İndeksli Yayınlar Koleksiyonu