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Diseases

Genetic and Rare Diseases Information Center (GARD)

Immunodeficiency with hyper IgM type 1


Other Names for this Disease
  • X-linked hyper IgM syndrome
  • Hyper IgM immunodeficiency, x-linked
  • Hyper IgM syndrome
  • Hyper IgM syndrome 1
  • XHIM
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Overview

Hyper IgM syndrome is a type of primary immunodeficiency syndrome. Primary immunodeficiency occurs when part of a person’s immune system is missing or does not work correctly. The bodies of people with primary immunodeficiency can’t get rid of germs or protect themselves from new germs as well as they should. Primary immunodeficiencies are inherited, meaning they are passed down from parents to children.[1]

Hyper IgM syndromes are characterized by normal or elevated serum immunoglobulin M levels with absence of immunoglobulin G, A, and E. Immunoglobulins are proteins found in the blood. Hyper IgM results in a susceptibility to bacterial infections and sometimes opportunistic infections. There are five different types of hyper IgM syndromes (types 1-5). The types are distinguished by the location of the gene mutation involved.[2]

Last updated: 8/1/2013

References

  1. Primary Immunodeficiency. National Institute of Child Health and Human Development (NICHD). April 7, 2008; http://www.nichd.nih.gov/publications/pubs/pages/primary_immuno.aspx#WhatisPrimaryImmunodeficiency.
  2. Hyper IgM Immunodeficiency Syndrome. MeSH. http://www.nlm.nih.gov/cgi/mesh/2008/MB_cgi?mode=&index=24001&field=all&HM=&II=&PA=&form=&input=. Accessed 4/16/2008.
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Basic Information

  • Genetics Home Reference (GHR) contains information on Immunodeficiency with hyper IgM type 1. This website is maintained by the National Library of Medicine.

In Depth Information

  • The Merck Manual for health care professionals provides information on Immunodeficiency with hyper IgM type 1.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
    The Online Mendelian Inheritance in Man (OMIM)
    The Online Mendelian Inheritance in Man (OMIM)
    The Online Mendelian Inheritance in Man (OMIM)
    The Online Mendelian Inheritance in Man (OMIM)
    The Online Mendelian Inheritance in Man (OMIM)
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Immunodeficiency with hyper IgM type 1. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • X-linked hyper IgM syndrome
  • Hyper IgM immunodeficiency, x-linked
  • Hyper IgM syndrome
  • Hyper IgM syndrome 1
  • XHIM
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.