Liver Transplantation in a Patient With CD40 Ligand Deficiency and Hyper-IgM Syndrome: Clinical and Immunological Assessments
نویسندگان
چکیده
منابع مشابه
Morbidity and Mortality of Iranian Patients with Hyper IgM Syndrome: a Clinical Analysis
Background: Defects in B cell class switch recombination (CSR) are a heterogeneous and yet very uncommon group of disorders which all have a genetic basis uniformly leading to hyper IgM (HIgM) syndrome. Due to the rare frequency of these conditions, a very small number of case series have been conducted on the affected patients. Objective: To shed some light on the morbidity and mortality regar...
متن کاملCLINICAL TRIALS AND OBSERVATIONS Partial immune reconstitution of X-linked hyper IgM syndrome with recombinant CD40 ligand
1Laboratory of Host Defenses, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH), Bethesda, MD; 2Department of Critical Care Medicine, NIH Clinical Center, Bethesda, MD; 3Metabolism Branch, National Cancer Institute (NCI), Bethesda, MD; 4Laboratory of Immunoregulation, NIAID, NIH, Bethesda, MD; 5Laboratory of Pathology, NCI, Bethesda, MD; 6Amgen, ...
متن کاملPartial immune reconstitution of X-linked hyper IgM syndrome with recombinant CD40 ligand.
X-linked hyper IgM syndrome (XHM) is a combined immune deficiency disorder caused by genetic alterations in CD40 ligand. The purpose of this study was to investigate the safety and efficacy of recombinant CD40 ligand (rCD40L) in the treatment of the disease. Three children were administered rCD40L subcutaneously 3 times per week at 0.03 mg/kg for 22 weeks, and after a 12-week drug-free interval...
متن کاملA novel mutation in the CD40 ligand gene in a Chinese boy with X-linked hyper-IgM syndrome.
X-linked hyper-IgM Syndrome (XHIGM) is caused by a mutation of CD40 ligand (CD40L), which is normally expressed on activated CD4+ T cells and is responsible for immunoglobulin class switching. A 7-year-old boy with recurrent sino-pulmonary infections since the age of 3 months had normal CD3+, CD4+, CD8+T lymphocytes, and CD19+B lymphocytes and NK cells, but significantly elevated IgM and extrem...
متن کاملCD40 ligand-deficient T cells from X-linked hyper-IgM syndrome carriers have intrinsic priming capability.
Deficiency in CD40 ligand (CD40L) expression is associated with impaired T cell immunity in mouse models and in humans. Previous studies have indicated that this is due to the failure of induction of extrinsic costimulatory molecules. However, other studies have suggested that CD40L is an intrinsic costimulatory molecule. The X-linked hyper-IgM syndrome (XHIM) is a primary immunodeficiency caus...
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ژورنال
عنوان ژورنال: American Journal of Transplantation
سال: 2016
ISSN: 1600-6135
DOI: 10.1111/ajt.13580