X-linked agammaglobulinemia (XLA): Phenotype, diagnosis, and therapeutic challenges around the world

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El-Sayed Z. A., Abramova I., Carlos Aldave J., Al-Herz W., Bezrodnik L., Boukari R., ...Daha Fazla

WORLD ALLERGY ORGANIZATION JOURNAL, cilt.12, sa.3, 2019 (SCI-Expanded, Scopus) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 12 Sayı: 3
  • Basım Tarihi: 2019
  • Doi Numarası: 10.1016/j.waojou.2019.100018
  • Dergi Adı: WORLD ALLERGY ORGANIZATION JOURNAL
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Anahtar Kelimeler: XLA, Agammaglobulinemia, Infection, Autoimmunity, Outcomes, Immunoglobulin, Therapy, PRIMARY IMMUNODEFICIENCY DISEASES, BRUTONS TYROSINE KINASE, PRIMARY HYPOGAMMAGLOBULINEMIA, INTRAVENOUS IMMUNOGLOBULIN, REPLACEMENT THERAPY, CLINICAL-FEATURES, NATIONAL-REGISTRY, BTK, EXPERIENCE, GENE
  • Açık Arşiv Koleksiyonu: AVESİS Açık Erişim Koleksiyonu
  • Ankara Üniversitesi Adresli: Evet

Özet

Background: X-linked agammaglobulinemia is an inherited immunodeficiency recognized since 1952. In spite of seven decades of experience, there is still a limited understanding of regional differences in presentation and complications. This study was designed by the Primary Immunodeficiencies Committee of the World Allergy Organization to better understand regional needs, challenges and unique patient features.