Periodic Fever, Aphthae, Pharyngitis and Cervical Adenitis. Our experience in the management of PFAPA syndrome

Detalhes bibliográficos
Autor(a) principal: Martín-Bailón, María
Data de Publicação: 2015
Outros Autores: García-Bastida, Clara, Faraldo-García, Ana
Tipo de documento: Artigo
Idioma: spa
Título da fonte: Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
Texto Completo: https://doi.org/10.34631/sporl.592
Resumo: Aim: To analyze our experience in the management of PFAPA syndrome (Periodic Fever, Adenopathy, Pharyngitis and Aphthae) and literature review. Patients and methods: observational, prospective, descriptive study of 8 patients younger than 15 years, diagnosed with PFAPA syndrome according to Thomas´ criteria, between August 2014 and January 2015. Epidemiological, clinical and laboratory findings are discussed. Results: Median age at onset of symptoms 3.4 years; mean lag time from onset of symptoms and diagnosis 3.1 years. 100% presented fever and pharyngitis, 87.5% cervical adenitis and 67.5% aphthous stomatitis. Mean symptom-free interval 27.1 days. Good response to single dose of corticosteroids in 100%. One patient undergoing tonsillectomy. Conclusions: PFAPA syndrome is a rare benign disease, whose knowledge allows early diagnosis and appropriate treatment, providing a better quality of life for patients and optimization of diagnostic and therapeutic resources.
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spelling Periodic Fever, Aphthae, Pharyngitis and Cervical Adenitis. Our experience in the management of PFAPA syndromeFiebre periódica, aftosis, faringitis y adenitis cervical. Nuestra experiencia en pacientes con síndrome PFAPAPFAPA syndromeMarshall´s syndromeperiodic fevertonsillectomycorticoid therapysíndrome PFAPAsíndrome Marshallfiebre periódicaamigdalectomíacorticoterapiaAim: To analyze our experience in the management of PFAPA syndrome (Periodic Fever, Adenopathy, Pharyngitis and Aphthae) and literature review. Patients and methods: observational, prospective, descriptive study of 8 patients younger than 15 years, diagnosed with PFAPA syndrome according to Thomas´ criteria, between August 2014 and January 2015. Epidemiological, clinical and laboratory findings are discussed. Results: Median age at onset of symptoms 3.4 years; mean lag time from onset of symptoms and diagnosis 3.1 years. 100% presented fever and pharyngitis, 87.5% cervical adenitis and 67.5% aphthous stomatitis. Mean symptom-free interval 27.1 days. Good response to single dose of corticosteroids in 100%. One patient undergoing tonsillectomy. Conclusions: PFAPA syndrome is a rare benign disease, whose knowledge allows early diagnosis and appropriate treatment, providing a better quality of life for patients and optimization of diagnostic and therapeutic resources.Objetivo: análisis de nuestra experiencia en el manejo de síndrome PFAPA (Periodic Fever, Adenopathy, Pharingitis and Afthae) y revisión bibliográfica.Material y métodos: estudio observacional, prospectivo, descriptivo, de 8 pacientes menores de 15 años, diagnosticados de síndrome PFAPA según los criterios de Thomas entre agosto de 2014 y enero de 2015. Se analizan variables epidemiológicas, clínicas y analíticas.Resultados: edad media al inicio de los síntomas de 3,4 años; retraso diagnóstico medio de 3,1 años. 100% presentaron fiebre y faringoamigdalitis, 87,5% adenitis cervical y 67,5% aftas orales. Promedio de tiempo libre de enfermedad de 27,1 días. Buena respuesta a dosis única de corticoides en el 100%. 1 paciente tratado con amigdalectomía.Conclusiones: El síndrome PFAPA es una patología benigna poco frecuente, cuyo conocimiento permite realizar un diagnóstico precoz y tratamiento adecuado, lo que implica una mejor calidad de vida para los pacientes y optimización de recursos diagnósticos y terapéuticos.Sociedade Portuguesa de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço2015-12-11info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttps://doi.org/10.34631/sporl.592https://doi.org/10.34631/sporl.592Portuguese Journal of Otorhinolaryngology and Head and Neck Surgery; Vol. 53 No. 3 (2015): Setembro; 149-153Revista Portuguesa de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço; Vol. 53 Núm. 3 (2015): Setembro; 149-153Revista Portuguesa de Otorrinolaringologia-Cirurgia de Cabeça e Pescoço; Vol. 53 N.º 3 (2015): Setembro; 149-1532184-6499reponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiainstacron:RCAAPspahttps://journalsporl.com/index.php/sporl/article/view/2765https://journalsporl.com/index.php/sporl/article/view/2765/767Martín-Bailón, MaríaGarcía-Bastida, ClaraFaraldo-García, Anainfo:eu-repo/semantics/openAccess2024-06-06T13:00:13Zoai:journalsporl.com:article/2765Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireinfo@rcaap.ptopendoar:https://opendoar.ac.uk/repository/71602025-05-28T17:54:24.487504Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) - FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiafalse
dc.title.none.fl_str_mv Periodic Fever, Aphthae, Pharyngitis and Cervical Adenitis. Our experience in the management of PFAPA syndrome
Fiebre periódica, aftosis, faringitis y adenitis cervical. Nuestra experiencia en pacientes con síndrome PFAPA
title Periodic Fever, Aphthae, Pharyngitis and Cervical Adenitis. Our experience in the management of PFAPA syndrome
spellingShingle Periodic Fever, Aphthae, Pharyngitis and Cervical Adenitis. Our experience in the management of PFAPA syndrome
Martín-Bailón, María
PFAPA syndrome
Marshall´s syndrome
periodic fever
tonsillectomy
corticoid therapy
síndrome PFAPA
síndrome Marshall
fiebre periódica
amigdalectomía
corticoterapia
title_short Periodic Fever, Aphthae, Pharyngitis and Cervical Adenitis. Our experience in the management of PFAPA syndrome
title_full Periodic Fever, Aphthae, Pharyngitis and Cervical Adenitis. Our experience in the management of PFAPA syndrome
title_fullStr Periodic Fever, Aphthae, Pharyngitis and Cervical Adenitis. Our experience in the management of PFAPA syndrome
title_full_unstemmed Periodic Fever, Aphthae, Pharyngitis and Cervical Adenitis. Our experience in the management of PFAPA syndrome
title_sort Periodic Fever, Aphthae, Pharyngitis and Cervical Adenitis. Our experience in the management of PFAPA syndrome
author Martín-Bailón, María
author_facet Martín-Bailón, María
García-Bastida, Clara
Faraldo-García, Ana
author_role author
author2 García-Bastida, Clara
Faraldo-García, Ana
author2_role author
author
dc.contributor.author.fl_str_mv Martín-Bailón, María
García-Bastida, Clara
Faraldo-García, Ana
dc.subject.por.fl_str_mv PFAPA syndrome
Marshall´s syndrome
periodic fever
tonsillectomy
corticoid therapy
síndrome PFAPA
síndrome Marshall
fiebre periódica
amigdalectomía
corticoterapia
topic PFAPA syndrome
Marshall´s syndrome
periodic fever
tonsillectomy
corticoid therapy
síndrome PFAPA
síndrome Marshall
fiebre periódica
amigdalectomía
corticoterapia
description Aim: To analyze our experience in the management of PFAPA syndrome (Periodic Fever, Adenopathy, Pharyngitis and Aphthae) and literature review. Patients and methods: observational, prospective, descriptive study of 8 patients younger than 15 years, diagnosed with PFAPA syndrome according to Thomas´ criteria, between August 2014 and January 2015. Epidemiological, clinical and laboratory findings are discussed. Results: Median age at onset of symptoms 3.4 years; mean lag time from onset of symptoms and diagnosis 3.1 years. 100% presented fever and pharyngitis, 87.5% cervical adenitis and 67.5% aphthous stomatitis. Mean symptom-free interval 27.1 days. Good response to single dose of corticosteroids in 100%. One patient undergoing tonsillectomy. Conclusions: PFAPA syndrome is a rare benign disease, whose knowledge allows early diagnosis and appropriate treatment, providing a better quality of life for patients and optimization of diagnostic and therapeutic resources.
publishDate 2015
dc.date.none.fl_str_mv 2015-12-11
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv https://doi.org/10.34631/sporl.592
https://doi.org/10.34631/sporl.592
url https://doi.org/10.34631/sporl.592
dc.language.iso.fl_str_mv spa
language spa
dc.relation.none.fl_str_mv https://journalsporl.com/index.php/sporl/article/view/2765
https://journalsporl.com/index.php/sporl/article/view/2765/767
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Sociedade Portuguesa de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço
publisher.none.fl_str_mv Sociedade Portuguesa de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço
dc.source.none.fl_str_mv Portuguese Journal of Otorhinolaryngology and Head and Neck Surgery; Vol. 53 No. 3 (2015): Setembro; 149-153
Revista Portuguesa de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço; Vol. 53 Núm. 3 (2015): Setembro; 149-153
Revista Portuguesa de Otorrinolaringologia-Cirurgia de Cabeça e Pescoço; Vol. 53 N.º 3 (2015): Setembro; 149-153
2184-6499
reponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia
instacron:RCAAP
instname_str FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia
instacron_str RCAAP
institution RCAAP
reponame_str Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
collection Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
repository.name.fl_str_mv Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) - FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia
repository.mail.fl_str_mv info@rcaap.pt
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