What is Hyper-IgM Syndrome?

Hyper-IgM Syndrome: A rare inherited immunodeficiency disorder which causes frequent infections involving the ears, eyes, sinuses, lungs, skin, respiratory tract and other areas of the body.

Ophanet, a consortium of European partners, currently defines a condition rare when it affects 1 person per 2,000. They list Hyper-IgM Syndrome as a "rare disease".
Source - Orphanet

Hyper-IgM Syndrome: Introduction

Types of Hyper-IgM Syndrome:

Broader types of Hyper-IgM Syndrome:

Who gets Hyper-IgM Syndrome?

Patient Profile for Hyper-IgM Syndrome: Infants. Symptoms usually within first year.

How serious is Hyper-IgM Syndrome?

Complications of Hyper-IgM Syndrome: see complications of Hyper-IgM Syndrome

What causes Hyper-IgM Syndrome?

Causes of Hyper-IgM Syndrome: see causes of Hyper-IgM Syndrome
Causes of Hyper-IgM Syndrome: In a normal immune response to a new antigen, B cells first produce IgM antibody. Later, the B cells switch to produce IgG, IgA and IgE, antibodies that protect tissues and mucosal surfaces more effectively. (Source: Genes and Disease by the National Center for Biotechnology) ... A flawed gene (or genes) in T cells is responsible for hyper IgM syndrome. The faulty T cells do not give B cells a signal they need to switch from making IgM to IgA and IgG. Most cases of hyper-IgM syndrome are linked to the X chromosome. Because males do not have a second, healthy, X-chromosome to offset the disease, boys far outnumber girls with this disease. (Source: excerpt from Primary Immune Deficiency, NIAID Fact Sheet: NIAID)

What are the symptoms of Hyper-IgM Syndrome?

Symptoms of Hyper-IgM Syndrome: see symptoms of Hyper-IgM Syndrome

Complications of Hyper-IgM Syndrome: see complications of Hyper-IgM Syndrome

Can anyone else get Hyper-IgM Syndrome?

Contagion of genetic conditions: generally not; see details in contagion of genetic diseases.
Inheritance: see inheritance of Hyper-IgM Syndrome

Inheritance of genetic conditions: see details in inheritance of genetic diseases.

Hyper-IgM Syndrome: Testing

Diagnostic testing: see tests for Hyper-IgM Syndrome.

Misdiagnosis: see misdiagnosis and Hyper-IgM Syndrome.

How is it treated?

Treatments for Hyper-IgM Syndrome: see treatments for Hyper-IgM Syndrome
Research for Hyper-IgM Syndrome: see research for Hyper-IgM Syndrome

Organs Affected by Hyper-IgM Syndrome:

Organs and body systems related to Hyper-IgM Syndrome include:

Immune system

Name and Aliases of Hyper-IgM Syndrome

Main name of condition: Hyper-IgM Syndrome

Class of Condition for Hyper-IgM Syndrome: genetic

Other names or spellings for Hyper-IgM Syndrome:

Immunodeficiency with hyper-IgM, Immunodeficiency with increased IgM, hyperimmunoglobulin M immunodeficiency, hypergammaglobulinemia IgM, dysgammaglobulinemia IgM, IgM hyperglobulinemia, Dysgammaglobulinemia type 1, IHIS, IMD3, Immunodeficiency-3

Research the causes of these diseases that are similar to, or related to, Hyper-IgM Syndrome:

» Next page: Online Medical Textbooks for Hyper-IgM Syndrome

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What is Hyper-IgM Syndrome?