Existencias: Clinical, Immunologic, and Molecular Spectrum of Patients with LPS-Responsive Beige-Like Anchor Protein Deficiency: A Systematic Review

Clinical, Immunologic, and Molecular Spectrum of Patients with LPS-Responsive Beige-Like Anchor Protein Deficiency: A Systematic Review

Background: LPS-responsive beige-like anchor protein (LRBA) deficiency is a primary immunodeficiency and immune dysregulation syndrome caused by biallelic mutations in the LRBA gene. These mutations usually abrogate the protein expression of LRBA, leading to a broad spectrum of clinical phenotypes i...

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Detalles Bibliográficos
Autores Principales:	Habibi S., Zaki-Dizaji M., Rafiemanesh H., Lo B., Jamee M., Gámez-Díaz L., Salami F., Kamali A.N., Mohammadi H., Abolhassani H., Yazdani R., Aghamohammadi A., Anaya, Juan-Manuel, Azizi G.
Formato:	Artículo (Article)
Lenguaje:	Inglés (English)
Publicado:	American Academy of Allergy, Asthma and Immunology 2019
Materias:	ADP ribosyl cyclase/cyclic ADP ribose hydrolase 1 Azathioprine CD19 antigen CD27 antigen CD45RA antigen CD45RO antigen Complement component C3d receptor Corticosteroid Cyclosporine Immunoglobulin Immunoglobulin A Immunoglobulin D Immunoglobulin M Mycophenolate mofetil Rapamycin Salazosulfapyridine Steroid Tacrolimus Arab Article Autoimmune disease B lymphocyte subpopulation CD4 lymphocyte count Chronic diarrhea Clinical evaluation Clinical feature Delayed diagnosis Drug megadose Enteropathy Gastrectomy Gene Gene mutation Genetic screening Genotype phenotype correlation Hematopoietic stem cell transplantation Hormone substitution Human Idiopathic thrombocytopenic purpura Immune deficiency Immune dysregulation Immunoglobulin blood level Immunoglobulin deficiency Inflammation Iranian people Low drug dose LPS responsive and beige like anchor protein deficiency LRBA gene Multiple organ failure Nephroblastoma Pancytopenia Patient care planning Pneumonia Protein deficiency Protein expression Recurrent infection Regulatory T lymphocyte Respiratory failure Sepsis Septic shock Splenomegaly Stomach adenocarcinoma Systematic review Systemic inflammatory response syndrome Turk (people) Autoimmunity LRBA deficiency Polyautoimmunity Regulatory T cell
Acceso en línea:	https://repository.urosario.edu.co/handle/10336/22225 https://doi.org/10.1016/j.jaip.2019.04.011

Internet

https://repository.urosario.edu.co/handle/10336/22225
https://doi.org/10.1016/j.jaip.2019.04.011

Clinical, Immunologic, and Molecular Spectrum of Patients with LPS-Responsive Beige-Like Anchor Protein Deficiency: A Systematic Review

Internet

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