The hyper IgM syndromes: Epidemiology, pathogenesis, clinical manifestations, diagnosis and management

R Yazdani, S Fekrvand, S Shahkarami, G Azizi… - Clinical …, 2019 - Elsevier
Abstract Hyper Immunoglobulin M syndrome (HIGM) is a rare primary immunodeficiency
disorder characterized by low or absent levels of serum IgG, IgA, IgE and normal or …

Hyper IgM syndrome: a report from the USIDNET registry

EA Leven, P Maffucci, HD Ochs, PR Scholl… - Journal of clinical …, 2016 - Springer
Abstract Purpose The United States Immunodeficiency Network (USIDNET) patient registry
was used to characterize the presentation, genetics, phenotypes, and treatment of patients …

The hyper IgM syndromes

N Qamar, RL Fuleihan - Clinical reviews in allergy & immunology, 2014 - Springer
The hyper IgM syndromes are a group of rare inherited immune deficiency disorders
characterized by impairment of immunoglobulin isotype switching resulting from defects in …

Autoimmunity in hyper-IgM syndrome

AA Jesus, AJS Duarte, JB Oliveira - Journal of clinical immunology, 2008 - Springer
Abstract Introduction Immunodeficiency with hyper-IgM (HIGM) results from genetic defects
in the CD40–CD40 ligand (CD40L) pathway or in the enzymes required for immunoglobulin …

First report of the Hyper-IgM syndrome Registry of the Latin American Society for Immunodeficiencies: novel mutations, unique infections, and outcomes

O Cabral-Marques, S Klaver, LF Schimke… - Journal of clinical …, 2014 - Springer
Hyper-IgM (HIGM) syndrome is a heterogeneous group of disorders characterized by normal
or elevated serum IgM levels associated with absent or decreased IgG, IgA and IgE. Here …

Clinical and Laboratory Findings in Hyper-IgM Syndrome with Novel CD40L and AICDA Mutations

A Aghamohammadi, N Parvaneh, N Rezaei… - Journal of clinical …, 2009 - Springer
Abstract Background Hyper-immunoglobulin M (HIGM) syndromes are a heterogeneous
group of primary immunodeficiency disorders, characterized by recurrent infections …

Immunodeficiency with hyper-IgM (HIM).

LD Notarangelo, M Duse, AG Ugazio - Immunodeficiency reviews, 1992 - europepmc.org
Immunodeficiency with hyper-IgM (HIM) is a rare disorder characterized by recurrent
infections associated with low IgG and IgA, and normal to increased IgM serum levels. Both …

Clinical phenotypes of hyper-IgM syndromes

MT de la Morena - The Journal of Allergy and Clinical Immunology: In …, 2016 - Elsevier
The primary immunodeficiency (PID) diseases comprise a heterogeneous group of inherited
disorders of immune function. Technical advancements in whole-genome, whole-exome …

Allogeneic hematopoietic stem cell transplantation for seven children with X‐linked hyper‐IgM syndrome: A single center experience

D Tomizawa, K Imai, S Ito, M Kajiwara… - American journal of …, 2004 - Wiley Online Library
X‐linked hyper‐IgM syndrome (XHIM), or hyper‐IgM syndrome type 1 (HIGM1), is a rare
primary immunodeficiency disorder susceptible to recurrent bacterial infection and …

Clinical, immunological, and molecular characterization of hyper-IgM syndrome due to CD40 deficiency in eleven patients

BK Al-Saud, Z Al-Sum, H Alassiri… - Journal of clinical …, 2013 - Springer
Abstract Purpose Hyper-IgM syndrome due to CD40 deficiency (HIGM3) is a rare form of
primary immunodeficiency with few reported cases. In this study, we further characterize the …