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Genetic and Rare Diseases Information Center (GARD)

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Immunodeficiency with hyper IgM type 1


Other Names for this Disease

  • HIGM
  • HIGM1
  • Hyper IgM immunodeficiency, x-linked
  • Hyper IgM syndrome
  • Hyper IgM syndrome 1
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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/primary_immuno.cfm#WhatisPrimaryImmunodeficiency. Accessed 10/12/2010.
  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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In Depth Information

  • The 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. 
  • 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
  • HIGM
  • HIGM1
  • Hyper IgM immunodeficiency, x-linked
  • Hyper IgM syndrome
  • Hyper IgM syndrome 1
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.