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Yayınlanmış 1 Ocak 2020 | Sürüm v1
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Long-term outcome of LRBA deficiency in 76 patients after various treatment modalities as evaluated by the immune deficiency and dysregulation activity (IDDA) score

Yazarlar/Oluşturanlar

  • 1. Med Univ Graz, Dept Pediat & Adolescent Med, Div Gen Pediat, Graz, Austria
  • 2. Belarusian Res Ctr Pediat Oncol Hematol & Immunol, Bone Marrow Transplantat Unit, Minsk, BELARUS
  • 3. Belarusian Res Ctr Pediat Oncol Hematol & Immunol, Res Dept, Minsk, BELARUS
  • 4. Hosp 12 Octubre, Res Inst, Immunodeficiencies Unit, Madrid, Spain
  • 5. Hosp Univ Virgen Nieves, Serv Pediat, Infect Dis & Immunodeficiencies Unit, Granada, Spain
  • 6. Dmitry Rogachev Natl Med Ctr Pediat Hematol Oncol, Immunol, Moscow, Russia
  • 7. Univ Tehran Med Sci, Pediat Ctr Excellence, Res Ctr Immunodeficiencies, Childrens Med Ctr, Tehran, Iran
  • 8. Karolinska Univ, Huddinge Hosp, Karolinska Inst, Div Clin Immunol,Dept Lab Med, Stockholm, Sweden
  • 9. Ankara Univ, Dept Pediat Immunol & Allergy, Sch Med, Ankara, Turkey

Açıklama

Background: Recent findings strongly support hematopoietic stem cell transplantation (HSCT) in patients with severe presentation of LPS-responsive beige-like anchor protein (LRBA) deficiency, but long-term follow-up and survival data beyond previous patient reports or meta-reviews are scarce for those patients who do not receive a transplant.

Dosyalar

bib-b15329c6-7de1-418c-9107-ebd285670865.txt

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md5:5199018ba48160a8ac59452d6c556c87
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