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Farmer J., Foldvari Z., Ujhazi B., De R., Chen K., Bleesing J., ...More
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE, vol.7, no.6, pp.1970-1989, 2019 (SCI-Expanded)
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Publication Type:
Article / Article
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Volume:
7
Issue:
6
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Publication Date:
2019
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Doi Number:
10.1016/j.jaip.2019.02.038
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Journal Name:
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE
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Journal Indexes:
Science Citation Index Expanded (SCI-EXPANDED), Scopus
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Page Numbers:
pp.1970-1989
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Keywords:
Recombination activating gene (RAG), Severe combined immunodeficiency (SCID), Immune dysregulation, Autoimmune cytopenias, Hematopoietic stem cell transplantation (HSCT), SEVERE COMBINED IMMUNODEFICIENCY, RECOMBINATION ACTIVITY, GRANULOMATOUS-DISEASE, CLINICAL PHENOTYPES, VACCINE-STRAIN, OMENN SYNDROME, MUTATIONS, CYTOPENIAS, RITUXIMAB, GENE
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Erciyes University Affiliated:
Yes
Abstract
BACKGROUND: Although autoimmunity and hyperinflammation secondary to recombination activating gene (RAG) deficiency have been associated with delayed diagnosis and even death, our current understanding is limited primarily to small case series.