Immune Dysregulation in the Tonsillar Microenvironment of Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA) Syndrome
| Autor: | Jack A. Gilbert, Wen Jiang, Shelby C. Leuin, Daniela Carvalho, Marisela Guaderrama, Anukriti Sharma, Anthony E. Magit, Robert O. Newbury, Donald B. Kearns, Nathan Page, Javan Nation, Irene Luu, Matthew T. Brigger, Marcella Bothwell, Michelle Peru, Seth M. Pransky, Lori Broderick, Morgan Bliss |
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| Rok vydání: | 2019 |
| Předmět: |
0301 basic medicine
Male PFAPA syndrome Adolescent Fever Immunology Population Palatine Tonsil CD8-Positive T-Lymphocytes medicine.disease_cause Article Cell Line 03 medical and health sciences 0302 clinical medicine Lymphadenitis medicine Immunology and Allergy Humans Leukocytosis education Child Stomatitis Tonsillectomy Inflammation education.field_of_study business.industry Infant Pharyngitis Adenitis Syndrome Immune dysregulation medicine.disease 030104 developmental biology medicine.anatomical_structure Cellular Microenvironment Tonsil Child Preschool Female Stomatitis Aphthous medicine.symptom business 030215 immunology |
| Zdroj: | J Clin Immunol |
| ISSN: | 1573-2592 |
| Popis: | Periodic Fever, Aphthous stomatitis, Pharyngitis and Adenitis (PFAPA) syndrome is an inflammatory disorder of childhood classically characterized by recurrent fevers, pharyngitis, stomatitis, cervical adenitis, and leukocytosis. While the mechanism is unclear, previous studies have shown that tonsillectomy can be a therapeutic option with improvement in quality of life in many patients with PFAPA, but the mechanisms behind surgical success remain unknown. In addition, long-term clinical follow-up is lacking. In our tertiary care center cohort, 62 patients with PFAPA syndrome had complete resolution of symptoms after surgery (95.3%). Flow cytometric evaluation demonstrates an inflammatory cell population, distinct from patients with infectious pharyngitis, with increased numbers of CD8+ T cells (5.9% vs. 3.8%, p |
| Databáze: | OpenAIRE |
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