Defects of T-cell effector function and post-thymic maturation in X-linked hyper-IgM syndrome

A Jain, TP Atkinson, PE Lipsky… - The Journal of …, 1999 - Am Soc Clin Investig
X-linked hyper-IgM syndrome (XHIM) results from mutations in the gene encoding for CD40
ligand (CD154). Patients with the syndrome suffer from infections with opportunistic …

Correction of neutropenia and hypogammaglobulinemia in X-linked hyper-IgM syndrome by allogeneic bone marrow transplantation

PR Scholl, MRG O'Gorman, LM Pachman… - Bone marrow …, 1998 - nature.com
X-linked hyper-IgM (X-HIM) syndrome is a primary immunodeficiency disease characterized
by defects in both cellular and humoral immunity. X-HIM is caused by mutations in the gene …

Clinical and molecular characterization of X‐linked hyper‐IgM syndrome patients in China

Y An, J Xiao, L Jiang, X Yang, J Yu… - Scandinavian journal of …, 2010 - Wiley Online Library
X‐linked Hyper‐IgM syndrome (XHIM) is caused by mutations of CD154 gene also known
as CD40 ligand (CD40L). CD40L is expressed in activated T cells and interacts with CD40 …

Partial immune reconstitution of X-linked hyper IgM syndrome with recombinant CD40 ligand

A Jain, JA Kovacs, DL Nelson… - Blood, The Journal …, 2011 - ashpublications.org
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 …

The X-linked hyper-IgM syndrome: clinical and immunologic features of 79 patients

JA Winkelstein, MC Marino, H Ochs, R Fuleihan… - Medicine, 2003 - journals.lww.com
The X-linked hyper-IgM (XHIGM) syndrome is an uncommon primary immunodeficiency
disease caused by mutations in the gene for CD40 ligand and characterized by normal or …

Immunologic reconstitution following bone marrow transplantation for X-linked hyper IgM syndrome

JE Duplantier, K Seyama, NK Day, R Hitchcock… - Clinical …, 2001 - Elsevier
X-linked hyper IgM syndrome (XHIM), caused by mutations of the CD40 ligand (CD40L)
gene, is characterized by recurrent bacterial and opportunistic infections, an increased …

X-linked hyper IgM syndrome: clinical, immunological and molecular features in patients from India

M Madkaikar, M Gupta, S Chavan, K Italia… - Blood Cells, Molecules …, 2014 - Elsevier
Background X-linked hyper-IgM (XHIM) is a primary immunodeficiency disorder
characterized by recurrent infections, low serum IgG and IgA and normal or elevated IgM. It …

Clinical features and genetic analysis of 20 Chinese patients with X‐linked hyper‐IgM syndrome

LL Wang, W Zhou, W Zhao, ZQ Tian… - Journal of …, 2014 - Wiley Online Library
X‐linked hyper‐IgM syndrome (XHIGM) is one type of primary immunodeficiency diseases,
resulting from defects in the CD40 ligand/CD40 signaling pathways. We retrospectively …

Development of a rapid whole blood flow cytometry procedure for the diagnosis of X-linked hyper-IgM syndrome patients and carriers

MRG O'Gorman, D Zaas, M Paniagua… - Clinical immunology and …, 1997 - Elsevier
The CD40 ligand expressed on activated T cells plays a pivotal role in B cell proliferation
and differentiation. Mutations in the CD40 ligand gene, which alter its expression on the …

X-linked hyper IgM syndrome

LC Schneider - Clinical reviews in allergy & immunology, 2000 - Springer
X-linked hyper IgM syndrome is largely caused by defects in the CD40L (CD154). The
patients have low levels of IgG and have difficulty with respiratory infections as well as …