A patient with a novel homozygous mutation in presented with autoimmune lymphoproliferative syndrome, and had impaired TCR-driven Fas ligand upregulation, providing a mechanism for the T cell lymphoproliferation.
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http://dx.doi.org/10.1016/j.jaci.2020.06.019 | DOI Listing |
J Neurol Sci
December 2024
Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.
Objectives: This study aimed to investigate cerebrospinal fluid (CSF) adenosine deaminase (ADA) levels in various neurological disorders and examine the relationships between CSF ADA levels and immunological parameters.
Methods: Overall, 276 patients whose CSF ADA levels were measured for suspected tuberculous meningitis (TBM) were evaluated. Data on baseline characteristics, final diagnoses, CSF ADA levels, and other laboratory parameters were collected.
Pediatr Allergy Immunol Pulmonol
January 2025
Clinical Immunology Unit, Faculty of Medicine and Health Sciences, Department of Paediatrics, Universiti Putra Malaysia, Selangor, Malaysia.
: RAS guanyl-releasing protein 1 (RASGRP1) deficiency is characterized by immune dysregulation and Epstein-Barr virus (EBV)-related lymphoproliferation. Diffuse mesangial sclerosis is one of the infrequent causes of infantile nephrotic syndrome. : Here, we described a 7-year-old girl who was diagnosed with diffuse mesangial sclerosis at 5 months old and subsequently developed chronic bilateral neck swelling at the age of 3 years.
View Article and Find Full Text PDFExpert Rev Anti Infect Ther
January 2025
Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Milan, Italy.
Introduction: Hepatitis C virus (HCV) infection represents a significant global health burden, particularly due to its extrahepatic immune-mediated manifestations, such as mixed cryoglobulinemia, associated vasculitis (CryoVas), and non-Hodgkin B-cell lymphoma (B-NHL), which pose significant challenges. The advent of direct-acting antiviral (DAA) has changed the therapeutic landscape for HCV-related complications.
Areas Covered: This review explores the evolving epidemiology and management of HCV extrahepatic manifestation and lymphoproliferative disorders in the era of DAAs.
Int J Biol Macromol
December 2024
State Key Laboratory of Biocatalysis and Enzyme Engineering, School of Life Sciences, Hubei University, Wuhan, Hubei 430062, China; National & Local Joint Engineering Research Center of High-throughput Drug Screening Technology, School of Life Sciences, Hubei University, Wuhan, Hubei 430062, China; Hubei Jiangxia Laboratory, Wuhan, Hubei 430200, China. Electronic address:
TNFα inhibitors have been successfully developed and used in the clinical treatment of autoimmune disorders. However, the use of TNFα blockade may be accompanied by side effects. The cases of bacterial and viral infections, lymphoproliferative disorders, and anti-TNFα-induced lupus, have been reported among the rheumatoid arthritis or Crohn's disease patients treated with TNFα blockers.
View Article and Find Full Text PDFImmunopharmacol Immunotoxicol
December 2024
Nursing Department, College of Nursing and Health Sciences, Jazan University, Jazan, Saudi Arabia.
Background: One of the common findings in systemic sclerosis (SSc) patients has been long-term exposure to environmental toxins such as pesticides. However, the data available shows an equivocal association between pesticide exposure and autoimmunity in SSc.
Methods: We investigated the levels of organochlorine pesticides (OCPs) in blood of 20 SSc patients and 17 healthy controls, and also studied their effect on T lymphocytes and their functional responses.
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