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Hyperimmunoglobulin E syndrome

Related Terms

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Background

  • Hyperimmunoglobulin E syndrome (HIES), also called Job syndrome, is an inherited immunodeficiency that is characterized by recurrent bacterial infections, skin abscesses, and high levels of immunoglobulin E (IgE). Immunoglobulin (Ig), also called antibodies, is secreted by immune system cells to detect antigens (foreign substances like bacteria and viruses that enter the body). Once the antibodies attach to the antigen, white blood cells destroy the antigen.
  • Even though HIES patients have high levels of IgE in their blood, they are vulnerable to infection and disease because other important immune cells do not function properly. HIES patients are born with abnormal T-cells (type of white blood cells) that are unable to produce enough interferon-gamma, which stimulates white blood cells called macrophages to engulf foreign invaders. Consequently, the immune system's response to antigens is delayed.
  • Studies suggest that HIES is a genetic disorder that can be inherited as either an autosomal dominant (AD-HIES) or autosomal recessive (AR-HIES) trait. However, the specific gene involved remains unidentified.
  • HIES is an extremely rare disease, with only 250 cases ever reported internationally. Most individuals are not diagnosed until childhood, or sometimes adulthood.
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Causes

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Symptoms

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Diagnosis

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Treatment

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Integrative Therapies

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Prevention

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Author Information

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References

Natural Standard developed the above evidence-based information based on a thorough systematic review of the available scientific articles. For comprehensive information about alternative and complementary therapies on the professional level, go to www.naturalstandard.com. Selected references are listed below.

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The information in this monograph is intended for informational purposes only, and is meant to help users better understand health concerns. Information is based on review of scientific research data, historical practice patterns, and clinical experience. This information should not be interpreted as specific medical advice. Users should consult with a qualified healthcare provider for specific questions regarding therapies, diagnosis and/or health conditions, prior to making therapeutic decisions.