PFAPA syndrome in a 2-year-old girl: a case report and literature review

PFAPA syndrome is the most common periodic fever syndrome in our geographic zone. It usually develops in children under 5 years of age and is classified as an autoinflammatory disease. PFAPA syndrome is characterised by episodes of high fever (>39°C) accompanied by aphthous stomatitis, pharyngi...

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Main Authors: Jakub Haracz, Monika Tadla, Grzegorz Fleszar, Jacek Postępski
Format: Article
Language:English
Published: Medical Communications Sp. z o.o. 2018-03-01
Series:Pediatria i Medycyna Rodzinna
Subjects:
Online Access:http://www.pimr.pl/index.php/issues/2018-vol-14-no-1/pfapa-syndrome-in-a-2-year-old-girl-a-case-report-and-literature-review?aid=1165
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author Jakub Haracz
Monika Tadla
Grzegorz Fleszar
Jacek Postępski
author_facet Jakub Haracz
Monika Tadla
Grzegorz Fleszar
Jacek Postępski
author_sort Jakub Haracz
collection DOAJ
description PFAPA syndrome is the most common periodic fever syndrome in our geographic zone. It usually develops in children under 5 years of age and is classified as an autoinflammatory disease. PFAPA syndrome is characterised by episodes of high fever (>39°C) accompanied by aphthous stomatitis, pharyngitis, and cervical lymphadenopathy, which occur cyclically at 25–35-day intervals and last between 3 and 6 days. Patients experience no symptoms between these episodes. The disease resolves with age. The cause of PFAPA and the predisposing factors remain unknown. The paper presents a clinical case of a 2-year-old girl diagnosed in the Department of Paediatric Pulmonology and Rheumatology of the Medical University of Lublin due to recurrent fever episodes. After exclusion of other causes, PFAPA syndrome was diagnosed. Corticosteroid treatment was used and good therapeutic response was achieved. The paper also presents a literature review on the current diagnosis and treatment modalities in PFAPA.
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spelling doaj.art-2574177749ab407f91efc8a02b0898772022-12-21T18:21:40ZengMedical Communications Sp. z o.o.Pediatria i Medycyna Rodzinna1734-15312451-07422018-03-01141889510.15557/PiMR.2018.0009PFAPA syndrome in a 2-year-old girl: a case report and literature reviewJakub Haracz0Monika Tadla1Grzegorz Fleszar2Jacek Postępski3Student’s Paediatric Scientific Association at the Department of Pulmonology and Rheumatology, Medical University of Lublin, Lublin, PolandStudent’s Paediatric Scientific Association at the Department of Pulmonology and Rheumatology, Medical University of Lublin, Lublin, PolandStudent’s Paediatric Scientific Association at the Department of Pulmonology and Rheumatology, Medical University of Lublin, Lublin, PolandDepartment of Paediatric Pulmonology and Rheumatology, Medical University of Lublin, Lublin, PolandPFAPA syndrome is the most common periodic fever syndrome in our geographic zone. It usually develops in children under 5 years of age and is classified as an autoinflammatory disease. PFAPA syndrome is characterised by episodes of high fever (>39°C) accompanied by aphthous stomatitis, pharyngitis, and cervical lymphadenopathy, which occur cyclically at 25–35-day intervals and last between 3 and 6 days. Patients experience no symptoms between these episodes. The disease resolves with age. The cause of PFAPA and the predisposing factors remain unknown. The paper presents a clinical case of a 2-year-old girl diagnosed in the Department of Paediatric Pulmonology and Rheumatology of the Medical University of Lublin due to recurrent fever episodes. After exclusion of other causes, PFAPA syndrome was diagnosed. Corticosteroid treatment was used and good therapeutic response was achieved. The paper also presents a literature review on the current diagnosis and treatment modalities in PFAPA.http://www.pimr.pl/index.php/issues/2018-vol-14-no-1/pfapa-syndrome-in-a-2-year-old-girl-a-case-report-and-literature-review?aid=1165paediatricsfeverpharyngitiscervical adenitisaphthous stomatitis
spellingShingle Jakub Haracz
Monika Tadla
Grzegorz Fleszar
Jacek Postępski
PFAPA syndrome in a 2-year-old girl: a case report and literature review
Pediatria i Medycyna Rodzinna
paediatrics
fever
pharyngitis
cervical adenitis
aphthous stomatitis
title PFAPA syndrome in a 2-year-old girl: a case report and literature review
title_full PFAPA syndrome in a 2-year-old girl: a case report and literature review
title_fullStr PFAPA syndrome in a 2-year-old girl: a case report and literature review
title_full_unstemmed PFAPA syndrome in a 2-year-old girl: a case report and literature review
title_short PFAPA syndrome in a 2-year-old girl: a case report and literature review
title_sort pfapa syndrome in a 2 year old girl a case report and literature review
topic paediatrics
fever
pharyngitis
cervical adenitis
aphthous stomatitis
url http://www.pimr.pl/index.php/issues/2018-vol-14-no-1/pfapa-syndrome-in-a-2-year-old-girl-a-case-report-and-literature-review?aid=1165
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AT monikatadla pfapasyndromeina2yearoldgirlacasereportandliteraturereview
AT grzegorzfleszar pfapasyndromeina2yearoldgirlacasereportandliteraturereview
AT jacekpostepski pfapasyndromeina2yearoldgirlacasereportandliteraturereview