Abatacept as a Long-Term Targeted Therapy for LRBA Deficiency

Kiykim, Ayca; ÖĞÜLÜR, İSMAİL; Dursun, Esra; Charbonnier, Louis; Nain, Ercan; Cekic, Sukru; DOĞRUEL, DİLEK; Karaca, Neslihan; ÇÖĞÜRLÜ, MÜJDE; BILIR, Ozlem; Cansever, Murat; Kapakli, Hasan; Baser, Dilek; Kasap, Nurhan; Kutlug, Seyhan; Altintas, Derya; AL-SHAIBI, Ahmad; AGREBI, Nourhen; Kara, Manolya; Guven, Ayla; Somer, Ayper; AYDOGMUS, Cigdem; AYAZ, Nuray; METIN, Ayse; Aydogan, Metin; Uncuoglu, Aysen; Patiroglu, Turkan; Yildiran, Alisan; Guner, Sukru; Keles, Sevgi; Reisli, Ismail; Aksu, Guzide; Kutukculer, Necil; Kilic, Sara; Yilmaz, Mustafa; Karakoc-Aydiner, Elif; LO, Bernice; Ozen, Ahmet; Chatila, Talal; Baris, Safa

doi:10.1016/j.jaip.2019.06.011

Abatacept as a Long-Term Targeted Therapy for LRBA Deficiency

Atıf İçin Kopyala

Kiykim A., ÖĞÜLÜR İ., Dursun E., Charbonnier L. M., Nain E., Cekic S., ...Daha Fazla

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE, cilt.7, sa.8, ss.2790-2815, 2019 (SCI-Expanded)

Yayın Türü: Makale / Tam Makale
Cilt numarası: 7 Sayı: 8
Basım Tarihi: 2019
Doi Numarası: 10.1016/j.jaip.2019.06.011
Dergi Adı: JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE
Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
Sayfa Sayıları: ss.2790-2815
Anahtar Kelimeler: LPS-responsive beige-like anchor, Immune dysregulation, Abatacept, T follicular helper cells, Autoimmunity, IMMUNE DYSREGULATION, CTLA-4 CHECKPOINT, MUTATIONS, POLYENDOCRINOPATHY, ENTEROPATHY, DISEASE
İstanbul Üniversitesi Adresli: Evet

Özet

BACKGROUND: LPS-responsive beige-like anchor (LRBA) deficiency presents with susceptibility to infections, autoimmunity, and lymphoproliferation. The long-term efficacy of cytotoxic T-lymphocyte-associated antigen 4-immunoglobulin (abatacept) as targeted therapy for its immune dysregulatory features remains to be established.