Naïve Regulatory T Cell Subset Is Altered in X-Linked Agammaglobulinemia [PDF]
Frontiers in Immunology, 2021 The interplay between T- and B-cell compartments during naïve, effector and memory T cell maturation is critical for a balanced immune response. Primary B-cell immunodeficiency arising from X-linked agammaglobulinemia (XLA) offers a model to explore B ...
Pavel V. Shelyakin +24 more
doaj +2 more sources
Immune Thrombocytopenia in an Adult With X‐linked Agammaglobulinemia: A Case Report [PDF]
eJHaemIn patients with X‐linked agammaglobulinemia (XLA), serum immunoglobulins are almost completely lacking. The prevalence of autoimmune diseases is low in XLA compared with other primary immunodeficiency diseases because antibodies are absent in XLA ...
Takeaki Matsunaga +6 more
doaj +2 more sources
Clinical case of primary immunodeficiency: X-linked agammaglobulinemia
Медицинская иммунология, 2020 Aclinical case ofprimary immunodeficiency state (PIDS) is described: X-linked agammaglobulinemia in the boy of 8 years old. The results of molecular genetic studies: gene btk (ex 1-19) genome version GRCh38.p5, transcript version ENST00000308731 single ...
E. V. Kuvschinova +4 more
doaj +3 more sources
Clinical features and mutational analysis of X-linked agammaglobulinemia patients in Malaysia [PDF]
Frontiers in Immunology, 2023 BackgroundBruton’s tyrosine kinase (BTK) is a cytoplasmic protein involved in the B cell development. X-linked agammaglobulinemia (XLA) is caused by mutation in the BTK gene, which results in very low or absent B cells.
Chai Teng Chear +17 more
doaj +2 more sources
Recurrent pneumonia with mild hypogammaglobulinemia diagnosed as X-linked agammaglobulinemia in adults [PDF]
Respiratory Research, 2001 Background X-linked agammaglobulinemia (XLA) is a humoral immunodeficiency caused by disruption of the Bruton's tyrosine kinase (BTK) gene. Typical XLA patients suffer recurrent and severe bacterial infections in childhood.
Tsuchiya Shigeru +7 more
doaj +2 more sources
Hematopoietic stem cell gene therapy for the treatment of X-linked agammaglobulinemia [PDF]
Molecular Therapy: Methods & Clinical DevelopmentX-linked agammaglobulinemia (XLA) is a rare inborn error of immunity caused by loss-of-function mutations in the gene encoding Bruton’s tyrosine kinase (BTK).
Christopher R. Luthers +8 more
doaj +2 more sources
X-linked agammaglobulinemia (XLA): Phenotype, diagnosis, and therapeutic challenges around the world
World Allergy Organization Journal, 2019 Background: X-linked agammaglobulinemia is an inherited immunodeficiency recognized since 1952. In spite of seven decades of experience, there is still a limited understanding of regional differences in presentation and complications.
Zeinab Awad El-Sayed +2 more
exaly +3 more sources
PROTECTIVE LEVELS OF VARICELLA-ZOSTER ANTIBODY DID NOT EFFECTIVELY PREVENT CHICKENPOX IN AN X-LINKED AGAMMAGLOBULINEMIA PATIENT [PDF]
Revista do Instituto de Medicina Tropical de São Paulo, 2015 SUMMARY We describe the case of an eight-year-old boy with X-linked agammaglobulinemia who developed mild varicella despite regular intravenous immunoglobulin (IVIG) therapy.
Fernanda Aimée NOBRE +3 more
doaj +2 more sources
IgA nephropathy in a child with X-linked agammaglobulinemia: a case report [PDF]
BMC PediatricsBackground X-linked agammaglobulinemia (XLA) is a primary immunodeficiency disease caused by mutations in the Bruton tyrosine kinase (BTK) gene. Individuals diagnosed with XLA are at an increased risk of developing autoimmune diseases.
Yuanjin Song +4 more
doaj +2 more sources
Clinical and Genetic Study of X-linked Agammaglobulinemia Patients (The Benefit of Early Diagnosis)
Iranian Journal of Allergy, Asthma and Immunology, 2020 X-linked agammaglobulinemia (XLA) is a primary immunodeficiency caused by genetic defects in the Bruton tyrosine kinase (Btk) gene. XLA is characterized as an antibody deficiency by recurrent bacterial infections, the absence of peripheral B cells, and ...
Zahra Alizadeh +9 more
doaj +2 more sources