Life-Threatening Pneumopathy and U urealyticum in a STAT3-Deficient Hyper-IgE Syndrome Patient.

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  • Additional Information
    • Source:
      Publisher: American Academy of Pediatrics Country of Publication: United States NLM ID: 0376422 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1098-4275 (Electronic) Linking ISSN: 00314005 NLM ISO Abbreviation: Pediatrics Subsets: MEDLINE
    • Publication Information:
      Publication: Elk Grove Village Il : American Academy of Pediatrics
      Original Publication: Springfield, Ill., Thomas.
    • Subject Terms:
      Job Syndrome/*complications ; Lung Diseases/*complications ; STAT3 Transcription Factor/*deficiency ; Ureaplasma Infections/*complications ; Ureaplasma urealyticum/*isolation & purification; Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Lung Diseases/microbiology ; Mutation
    • Abstract:
      A deficiency in signal transducer and activator of transcription 3 (STAT3) is responsible for autosomal dominant hyperimmunoglobulin E syndrome, an immunodeficiency syndrome causing Staphylococcus aureus , Streptococcus pneumonia , Haemophilus influenzae , and, rarely, Pseudomonas aeruginosa and Aspergillus sp infections. Currently, intracellular pathogens are not targeted in the management of severe infections. The pathophysiologic mechanism of hyperimmunoglobulin E syndrome immunodeficiency has recently been linked to a disorder in the T helper 17 pathway and disruption of the interleukin -23/interleukin-17 axis. We report an unusual case of severe pleuropneumopathy by Ureaplasma urealyticum in a teenage girl with STAT3-deficient hyperimmunoglobulin E syndrome (STAT3 HIES). A previous case of severe lung infection by Mycoplasma pneumoniae has already been described in a STAT3-deficient patient, but U urealyticum has never been reported in patients with STAT3 HIES. After a review of the literature, it seems that the specific immunodeficiency pathway of STAT3 HIES exposes STAT3 HIES patients to Ureaplasma lung infections because the pathophysiology of STAT3 HIES and Ureaplasma is based on STAT3 and T helper 17 cells.
      Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.
      (Copyright © 2017 by the American Academy of Pediatrics.)
    • Accession Number:
      0 (STAT3 Transcription Factor)
      0 (STAT3 protein, human)
    • Publication Date:
      Date Created: 20170601 Date Completed: 20170907 Latest Revision: 20170907
    • Publication Date:
      20250114
    • Accession Number:
      10.1542/peds.2016-0845
    • Accession Number:
      28562253