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Diseases

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
More Names
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Overview



What is hyper IgM syndrome?

How might hyper IgM syndrome be treated?


What is hyper IgM syndrome?

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

How might hyper IgM syndrome be treated?

The cornerstone of treatment for individuals with hyper IgM syndrome is regular injections of intravenous immunogloblulin (IVIG). This treatment not only supplies missing IgG antibodies, but also prompts a drop in IgM antibodies.[3][4] Patients with neutropenia can take granulocyte colony-stimulating factor (G-CSF). Antibiotics may also be prescribed to prevent the respiratory infection, pneumocystis carinii pneumonia.[4]

Most children with hyper-IgM syndrome respond well to treatment, become symptom-free and resume normal growth.[3]
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.
  3. Primary Immunodeficiency Diseases: Some Examples. National Institute of Child Health and Human Development (NICHD). April 7, 2008; http://www.nichd.nih.gov/publications/pubs/primary_immuno.cfm#PrimaryImmunoDiseases. Accessed 4/16/2008.
  4. Hyper IgM Immunodeficiency. Primary Immunodeficiency Resource Center. 2008; http://www.info4pi.org/aboutPI/index.cfm?section=aboutPI&content=syndromes&area=13&CFID=4740438&CFTOKEN=e428d93702237370-4B49B0F6-0023-0F1A-C289B29F1FE43C9A. Accessed 4/16/2008.