Affiliation:
1. Seattle Children's Hospital/University of Washington School of Medicine, Department of Pediatrics, Seattle, USA
2. Laboratory of Host Defenses, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA
Abstract
DOCK8 immunodeficiency syndrome (DIDS) is a combined immunodeficiency characterized by recurrent viral infections, severe atopy, and early onset malignancy. Genetic studies revealed large, unique deletions in patients from different families and ethnic backgrounds. Clinical markers of DIDS include atopic dermatitis, allergies, cutaneous viral infections, recurrent respiratory tract infections, and malignancy. Immune assessments showed T cell lymphopenia, hyper-IgE, hypo-IgM, and eosinophilia. The impaired lymphocyte functions in DIDS patients appear central for disease pathogenesis.
Funder
National Institute of Allergy and Infectious Diseases
Subject
Biochemistry, medical,Clinical Biochemistry,Genetics,Molecular Biology,General Medicine
Cited by
57 articles.
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