Published January 1, 2019
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Abatacept as a Long-Term Targeted Therapy for LRBA Deficiency
Creators
- Kiykim, Ayca1
- Ogulur, Ismail1
- Dursun, Esra1
- Charbonnier, Louis Marie
- Nain, Ercan1
- Cekic, Sukru2
- Dogruel, Dilek3
- Karaca, Neslihan Edeer4
- Cogurlu, Mujde Tuba5
- Bilir, Ozlem Arman6
- Cansever, Murat7
- Kapakli, Hasan8
- Baser, Dilek1
- Kasap, Nurhan1
- Kutlug, Seyhan9
- Altintas, Derya Ufuk3
- Al-Shaibi, Ahmad10
- Agrebi, Nourhen10
- Kara, Manolya11
- Guven, Ayla12
- Guven, Ayla12
- 1. Marmara Univ, Sch Med, Div Pediat Allergy & Immunol, Istanbul, Turkey
- 2. Uludag Univ, Div Pediat Allergy & Immunol, Fac Med, Bursa, Turkey
- 3. Cukurova Univ, Fac Med, Div Pediat Allergy Immunol, Adana, Turkey
- 4. Ege Univ, Fac Med, Div Pediat Allergy & Immunol, Izmir, Turkey
- 5. Kocaeli Univ, Fac Med, Div Pediat Allergy & Immunol, Kocaeli, Turkey
- 6. Ankara Childrens Hematol Oncol Training & Res Hos, Div Pediat Hematol, Ankara, Turkey
- 7. Erciyes Univ, Fac Med, Div Pediat Immunol, Kayseri, Turkey
- 8. Necmettin Erbakan Univ, Fac Med, Div Pediat Allergy & Immunol, Konya, Turkey
- 9. Ondokuz Mayis Univ, Fac Med, Div Pediat Allergy & Immunol, Samsun, Turkey
- 10. Sidra Med, Res Branch, Div Translat Med, Doha, Qatar
- 11. Istanbul Univ, Istanbul Med Fac, Div Pediat Infect Dis, Istanbul, Turkey
- 12. Saglik Bilimleri Univ, Zeynep Kamil Women & Children Hosp, Med Fac, Div Pediat Endocrinol Clin, Istanbul, Turkey
Description
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.
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