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European Archives of Oto-Rhino-Laryngology

, Volume 265, Issue 10, pp 1289–1292 | Cite as

TAP deficiency syndrome: chronic rhinosinusitis and conductive hearing loss

  • Marco CaversaccioEmail author
  • Harald Marcel Bonél
  • Rachel Carter
  • Anthony P. Williams
  • Stephan D. Gadola
Case Report

Abstract

Nose–ear–throat manifestations of immunodeficiency disorders represent a diagnostic challenge for clinicians as these diseases often constitute the initial sign for connective disorders or autoimmune disease. The history of chronic rhinosinusitis and conductive hearing loss is often non specific. Therefore attention to an HLA class I deficiency must be considered if the disease has not been diagnosed on routine examination. One of the syndromes is due to a defective TAP complex, the peptide transporter complex associated with antigen presentation. Herein, we report two sisters with TAP-deficiency. The treatment of choice for TAP-deficient patients is conservative.

Keywords

Immunodeficiency TAP-deficiency Chronic rhinosinusitis Ear 

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Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  • Marco Caversaccio
    • 1
    Email author
  • Harald Marcel Bonél
    • 2
  • Rachel Carter
    • 5
  • Anthony P. Williams
    • 5
  • Stephan D. Gadola
    • 3
    • 4
  1. 1.Department of ENT, Head and Neck Surgery, InselspitalUniversity of BerneBerneSwitzerland
  2. 2.Institute of Diagnostic, Interventional and Pediatric Radiology, InselspitalUniversity of BerneBerneSwitzerland
  3. 3.Department of Rheumatology and Clinical Immunology, InselspitalUniversity of BerneBerneSwitzerland
  4. 4.Division of Infection, Inflammation and RepairSouthampton University School of MedicineSouthamptonUK
  5. 5.Division of Cancer SciencesSouthampton University School of MedicineSouthamptonUK

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