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Discordant Phenotypes of Nephritis in Patients with X-linked Agammaglobulinemia.
J Clin Immunol
July 2024
Department of Child Health and Development, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), 1-3-45 Yushima, Bunkyo-ku, 113-8519, Tokyo, Japan.
Purpose: To define the clinical and histological characteristics of nephritis in patients with X-linked agammaglobulinemia (XLA) and their immunological profiles.
Methods: The clinical, immunological, and histological findings of nine patients with XLA and nephritis were retrospectively analyzed.
Results: Based on kidney histological findings, patients with XLA and nephritis could be divided into two groups, viz.
Membranoproliferative Glomerulonephritis Type I Associated with Intravenous Immunoglobulin Administration Arising in a Child with X-Linked Agammaglobulinemia: A Case Report and a Reappraisal.
Saudi J Kidney Dis Transpl
November 2023
Department of Nephrology, Armed Forces Hospitals Southern Region, Asir, Saudi Arabia.
In 1952, X-linked agammaglobulinemia (XLA) was discovered as a rare inherited disorder. It markedly compromises the ability of the body to combat infectious microorganisms. Membranoproliferative glomerulonephritis (MPGN) Type I is characterized by subendothelial immune complex deposits.
View Article and Find Full Text PDFIgA nephropathy in a child with X-linked agammaglobulinemia: a case report.
BMC Pediatr
April 2024
Department of Nephrology and Immunology, Qingdao Women and Children's Hospital, Qingdao, China.
Background: 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. However, renal involvement are rare in cases of XLA.
View Article and Find Full Text PDFRituximab-associated hypogammaglobulinemia in children with idiopathic nephrotic syndrome: results of an ESPN survey.
Pediatr Nephrol
September 2023
Division of Nephrology and Dialysis, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.
Background: There is paucity of information on rituximab-associated hypogammaglobulinemia (HGG) and its potential infectious consequences in children treated for idiopathic nephrotic syndrome (INS).
Methods: A survey was distributed by the European Society Pediatric Nephrology to its members. It addressed the screening and management practices of pediatric nephrology units for recognizing and treating RTX-associated HGG and its morbidity and mortality.
Targeting Multiple End Organs in Lupus and Other Systemic Rheumatic Diseases by Inhibiting Bruton's Tyrosine Kinase.
Front Immunol
July 2022
The Department of Biomedical Engineering, University of Houston, Houston, TX, United States.
Bruton tyrosine kinase (Btk) plays a vital role in activating and differentiating B-cells and regulating signaling in myeloid cells. Indeed, the potential use of Btk inhibitors in preventing lupus has been reported. Here, we extend these observations to 4 additional models of end-organ inflammation: (a) BWF1 lupus nephritis mice, (b) anti-GBM nephritis, (c) bleomycin-induced systemic sclerosis like skin disease, and (d) bleomycin-induced lung disease.
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