X-Linked Agammaglobulinemia (XLA) Research This is an RSS file. You can use it to subscribe to this data in your favourite RSS reader or to display this data on your own website or blog.

Keratoconjunctivitis as a Single Entity in X-linked Agammaglobulinemia?
CONCLUSION: Whereas steroid eye drops like dexamethasone could control the symptoms and the corneal scarring progression as short time therapy options, ciclosporin A eye drops showed problems in therapy adherence in long-time use. Antibiotic eye drops supported the anti-inflammatory therapy effects, but no typical pathogen was detected. Antineovascular subconjunctival application did not show any relevant effect in one-time use. Artificial tears were needed as basic therapy.PMID:35404736 | DOI:10.1080/09273948.2022.2042824 (Source: Ocular Immunology and Inflammation)
Source: Ocular Immunology and Inflammation - April 11, 2022 Category: Allergy & Immunology Authors: Stefan Mielke Bastian Grundel Sebastian M Schmidt Frank Tost Source Type: research

Improved HUMARA for the Detection of X-Linked Agammaglobulinemia Carriers
Genetic Testing and Molecular Biomarkers, Ahead of Print. (Source: Genetic Testing and Molecular Biomarkers)
Source: Genetic Testing and Molecular Biomarkers - April 8, 2022 Category: Genetics & Stem Cells Authors: Eduardo Carrillo-Tapia Sara E. Espinosa-Padilla Daniela Perez-Perez Maria E. Gonzalez-Serrano Laura Berron-Ruiz Francisco J. Espinosa-Rosales Juan C. Rodriguez-Alba Fabiola M újica-Guzman Emiy Yokoyama-Rebollar Jose R. Garc ía-Flores Norma E. Herrera-Go Source Type: research

Clinical and genetic findings in two siblings with X-Linked agammaglobulinemia and bronchiolitis obliterans: a case report
X-linked agammaglobulinemia (XLA) is an Inborn Errors of Immunity (IEI) characterized by pan-hypogammaglobulinemia and low numbers of B lymphocytes due to mutations in BTK gene. Usually, XLA patients are not susc... (Source: BMC Pediatrics)
Source: BMC Pediatrics - April 5, 2022 Category: Pediatrics Authors: Ronaldo da Silva Francisco Junior, Guilherme Loss de Morais, Joseane Biso de Carvalho, Cristina dos Santos Ferreira, Alexandra Lehmkuhl Gerber, Ana Paula de C Guimar ães, Flávia Anisio Amendola, Fernanda Pinto-Mariz, Zilton Farias Meira de Vasconcelos, Tags: Case report Source Type: research

SARS-CoV-2-Specific and Functional Cytotoxic CD8 Cells in Primary Antibody Deficiency: Natural Infection and Response to Vaccine
ConclusionsCVID is associated with reduced SARS-CoV-2-specific CD8 T cells and functional CTLs in both natural SARS-CoV-2 infection and in response to SARS-CoV-2 mRNA vaccine, whereas natural infection in XLA is associated with a robust SARS-CoV-2-specific CD8 and functional CTL responses. (Source: Journal of Clinical Immunology)
Source: Journal of Clinical Immunology - April 2, 2022 Category: Allergy & Immunology Source Type: research

Ecthyma gangrenosum as a presenting feature of X-linked agammaglobulinemia: A case study
(Source: Pediatrics and Neonatology)
Source: Pediatrics and Neonatology - March 25, 2022 Category: Perinatology & Neonatology Authors: Intan Hakimah Ismail, Julia Adam Tags: Images Source Type: research

Responses to SARS-CoV-2 Vaccines of Patients with Common Variable Immune Deficiencies and X-linked Agammaglobulinemia
(Source: Journal of Clinical Immunology)
Source: Journal of Clinical Immunology - March 24, 2022 Category: Allergy & Immunology Source Type: research

Chronic immune thrombocytopenia in a child with X-linked agammaglobulinemia-an uncommon phenotype
In this study, we detailly described a surprising autoimmune phenomenon, chronic ITP, in a small boy diagnosed with XLA. This is an interesting phenotype found in XLA, and it is helpful to understand the immune pathogenesis of autoimmunity in patients with XLA.PMID:35296220 | DOI:10.1080/09537104.2022.2053090 (Source: Platelets)
Source: Platelets - March 17, 2022 Category: Hematology Authors: Jing Yin Jijun Ma Xiaoxue Liu Jingyue Xia Qi Ai Chongwei Li Source Type: research

X-Linked Agammaglobulinemia with Kawasaki Disease
(Source: Indian Journal of Pediatrics)
Source: Indian Journal of Pediatrics - March 17, 2022 Category: Pediatrics Source Type: research

X-Linked Agammaglobulinemia: Infection Frequency and Infection-Related Mortality in the USIDNET Registry
AbstractX-linked agammaglobulinemia (XLA) is a primary immunodeficiency disorder caused by mutations in the Bruton tyrosine kinase (BTK) gene leading to B lymphocyte deficiency and susceptibility to infection. A potential benefit of earlier diagnosis and treatment initiation on morbidity and mortality in XLA is incompletely understood. In the USIDNET Registry, we describe infection frequency and infection-related mortality in patients with XLA and their relationship to age of diagnosis and treatment initiation. Among the 231 XLA patients enrolled in the Registry, respiratory infections (N = 203, 88%) were the most comm...
Source: Journal of Clinical Immunology - March 15, 2022 Category: Allergy & Immunology Source Type: research

Health-Related Quality of Life in 91 Patients with X-Linked Agammaglobulinemia
ConclusionOur study provides further insight into what factors impact both physical and mental domains of health amongst patients with XLA. Early detection to prevent the development of associated morbidity, as well as vigilant care to prevent hospitalizations and infections, can limit the impact this disease may have on the overall well-being of XLA patients. (Source: Journal of Clinical Immunology)
Source: Journal of Clinical Immunology - March 14, 2022 Category: Allergy & Immunology Source Type: research

B cell repertoire in patients with a novel BTK mutation: expanding the spectrum of atypical X-linked agammaglobulinemia
This report sheds further light on atypical cases of XLA. (Source: Immunologic Research)
Source: Immunologic Research - March 13, 2022 Category: Allergy & Immunology Source Type: research