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Chronic Candidiasis in Children.

Laura Green1, William K Dolen2

  • 1From the Department of Pediatrics, Allergy-Immunology and Pediatric Rheumatology Division, Medical College of Georgia at Augusta University, 1120 15th Street, Augusta, GA, 30912, USA.

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|April 22, 2017
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Summary
This summary is machine-generated.

Chronic mucocutaneous candidiasis (CMC) in children is often caused by genetic defects affecting the dectin pathway and IL-17 immunity, rather than T cell deficiencies. Genetic testing is crucial for diagnosing these rare immune disorders.

Keywords:
AIREAutoimmune polyendocrinopathy-candidiasis-ectodermal dystrophyChronic mucocutaneous candidiasisDectinHyper-IgE syndromeInterleukin-17

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Area of Science:

  • Immunology
  • Genetics
  • Pediatrics

Background:

  • Chronic mucocutaneous candidiasis (CMC) involves persistent Candida infections, unusual in children post-newborn period.
  • CMC presents as a phenotype within immunologic disorders, sometimes with endocrine and autoimmune links.

Purpose of the Study:

  • To review the pathogenesis of CMC in children.
  • To discuss immunologic pathways involved in handling Candida infections.
  • To highlight recent insights into genetic causes and clinical features of CMC.

Main Methods:

  • Review of current understanding of CMC pathogenesis.
  • Discussion of immunologic pathways (dectin, IL-17).
  • Emphasis on genetic defects (AIRE) and clinical syndromes.

Main Results:

  • In children, CMC is primarily linked to inborn errors affecting the dectin pathway and IL-17 immunity.
  • Genetic causes are diverse, including AIRE gene defects.
  • Distinguishing clinical features and inheritance patterns of different CMC syndromes are noted.

Conclusions:

  • CMC in children has numerous underlying genetic causes.
  • Genetic testing is essential for accurate diagnosis of CMC.
  • Understanding genetic defects clarifies the immunologic basis of CMC.