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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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Treatment


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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.[1][2] Patients with neutropenia can take granulocyte colony-stimulating factor (G-CSF). Antibiotics may also be prescribed to prevent the respiratory infection, pneumocystis carinii pneumonia.[2]

Most children with hyper-IgM syndrome respond well to treatment, become symptom-free and resume normal growth.[1]
Last updated: 8/1/2013

References
  1. 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.
  2. 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.


Management Guidelines

  • GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions. Click on the link to view the article on this topic.

Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Immunodeficiency with hyper IgM type 1. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.