RESUMO
Abstract Introduction: The role of tonsillectomy in the periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome, is controversial. Although some studies reported high success rates with tonsillectomy, further investigations are needed with larger numbers of patients. Objective: To seek the long-term outcomes of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome. Methods: Case series; multi-center study. The study comprised 23 patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome who underwent surgery (tonsillectomy with or without adenoidectomy) between January 2009 and November 2014. Results: 21 (91%) of 23 patients had complete resolution immediately after surgery. One patient had an attack 24 h after surgery, but has had no further attacks. One patient had three attacks with various intervals, and complete remission was observed after 3 months. Conclusions: Tonsillectomy is a good option for the treatment of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome.
Resumo Introdução: O papel da tonsilectomia na síndrome da febre periódica, estomatite aftosa, faringite e adenite é controverso. Embora alguns estudos tenham relatado altas taxas de sucesso com a tonsilectomia, são necessárias mais pesquisas com um número maior de pacientes. Objetivo: Avaliar os resultados em longo prazo da tonsilectomia na síndrome de febre periódica, estomatite aftosa, faringite e adenite. Método: Série de casos; estudo multicêntrico. O estudo avaliou 23 pacientes com síndrome de febre periódica, estomatite aftosa, faringite e adenite submetidos a cirurgia (tonsilectomia com ou sem adenoidectomia) entre janeiro de 2009 e novembro de 2014. Resultados: Dos 23 pacientes, 21 (91%) apresentaram resolução completa imediatamente após a cirurgia. Um paciente apresentou um episódio 24 horas após a cirurgia, mas sem recorrência posterior. Um paciente teve três episódios com vários intervalos e a remissão completa foi observada após 3 meses. Conclusões: A tonsilectomia é uma boa opção para o tratamento da síndrome de febre periódica, estomatite aftosa, faringite e adenite.
Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Estomatite Aftosa/cirurgia , Tonsilectomia/métodos , Faringite/cirurgia , Febre/cirurgia , Linfadenite/cirurgia , Síndrome , Adenoidectomia/métodos , Reprodutibilidade dos Testes , Seguimentos , Resultado do TratamentoRESUMO
INTRODUCTION: The role of tonsillectomy in the periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome, is controversial. Although some studies reported high success rates with tonsillectomy, further investigations are needed with larger numbers of patients. OBJECTIVE: To seek the long-term outcomes of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome. METHODS: Case series; multi-center study. The study comprised 23 patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome who underwent surgery (tonsillectomy with or without adenoidectomy) between January 2009 and November 2014. RESULTS: 21 (91%) of 23 patients had complete resolution immediately after surgery. One patient had an attack 24h after surgery, but has had no further attacks. One patient had three attacks with various intervals, and complete remission was observed after 3 months. CONCLUSIONS: Tonsillectomy is a good option for the treatment of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome.
Assuntos
Febre/cirurgia , Linfadenite/cirurgia , Faringite/cirurgia , Estomatite Aftosa/cirurgia , Tonsilectomia/métodos , Adenoidectomia/métodos , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Masculino , Reprodutibilidade dos Testes , Síndrome , Resultado do TratamentoRESUMO
We reviewed the medical records of patients with periodic fever, aphthosis, pharyngitis, and adenitis (PFAPA) to investigate the clinical course, treatment response, and association with MEFV gene mutation. Familial Mediterranean fever should be considered in patients with PFAPA who do not respond to adenotonsillectomy.
Assuntos
Adenoidectomia , Febre Familiar do Mediterrâneo/diagnóstico , Linfadenite/diagnóstico , Faringite/diagnóstico , Pirina/genética , Estomatite Aftosa/diagnóstico , Tonsilectomia , Criança , Pré-Escolar , Diagnóstico Diferencial , Doenças Endêmicas , Febre Familiar do Mediterrâneo/complicações , Febre Familiar do Mediterrâneo/epidemiologia , Febre Familiar do Mediterrâneo/genética , Feminino , Marcadores Genéticos , Heterozigoto , Humanos , Lactente , Linfadenite/complicações , Linfadenite/epidemiologia , Linfadenite/cirurgia , Masculino , Mutação , Faringite/complicações , Faringite/epidemiologia , Faringite/cirurgia , Estudos Retrospectivos , Estomatite Aftosa/complicações , Estomatite Aftosa/epidemiologia , Estomatite Aftosa/cirurgia , Síndrome , Resultado do Tratamento , Turquia/epidemiologiaRESUMO
OBJECTIVE: To compare the effectiveness of tonsillectomy and the long-term outcome of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome in patients fulfilling the classic diagnostic criteria and in those with regularly recurring fever as the only symptom or with onset of symptoms after age 5 years or both. STUDY DESIGN: We reviewed the medical records of 3852 children who underwent tonsillectomy between 1990 and 2007 and identified 108 children who did so because of regularly recurring fevers. The patients were invited to an outpatient visit and were classified into 2 groups: those who met (N = 58) and those who did not meet (N = 50) Thomas diagnostic criteria. We then compared the clinical profile and outcome of PFAPA symptoms after tonsillectomy between the 2 groups. RESULTS: In the group that met Thomas criteria, 97% (56/58) had complete resolution of fever episodes after tonsillectomy; in the group that did not meet Thomas criteria (50/50) had complete resolution of fever episodes after tonsillectomy (P = .25). The clinical profile of the periodic fevers and the occurrence of other illnesses during follow-up were similar in both groups. Thomas criteria identified 56 of 106 patients responding to tonsillectomy. CONCLUSIONS: Tonsillectomy was an effective treatment for patients with regularly recurring fever episodes who failed to meet the classic Thomas criteria. We suggest that PFAPA syndrome should be suspected and tonsillectomy considered in children with a late onset of symptoms (>5 years of age) or when fever is the only symptom during the episodes.
Assuntos
Febre/cirurgia , Linfadenite/cirurgia , Faringite/cirurgia , Estomatite Aftosa/cirurgia , Tonsilectomia , Adolescente , Criança , Pré-Escolar , Feminino , Febre/diagnóstico , Seguimentos , Humanos , Lactente , Linfadenite/diagnóstico , Masculino , Faringite/diagnóstico , Estudos Retrospectivos , Estomatite Aftosa/diagnóstico , Síndrome , Fatores de Tempo , Resultado do TratamentoRESUMO
OBJECTIVE: To establish guidelines based on scientific evidence for the management of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. DESCRIPTION OF THE EVIDENCE COLLECTION METHOD: The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. RESULTS: 806 articles were retrieved and evaluated by title and abstract; from these, 32 articles were selected to support the recommendations. RECOMMENDATIONS: 1. PFAPA is a diagnosis of exclusion established on clinical grounds, and one must suspect of this problem in children with recurrent and periodic febrile episodes of unknown origin, or with recurrent tonsillitis interspersed with asymptomatic periods, especially in children in good general condition and with preservation of weight and height development. 2. Laboratory findings are nonspecific. Additional tests do not reveal pathognomonic changes. 3. The evidence supporting an indication for surgical treatment (tonsillectomy with or without adenoidectomy), is based on two non-blinded randomized clinical trials with small numbers of patients. 4. The use of prednisone at the onset of fever in patients with PFAPA proved to be an effective strategy. There is still need for more qualified evidence to support its use in patients with PFAPA. 5. Despite promising results obtained in studies with IL-1ß inhibitors, such studies are limited to a few case reports.
Assuntos
Febre/terapia , Linfadenite/terapia , Faringite/terapia , Guias de Prática Clínica como Assunto , Estomatite Aftosa/terapia , Adenoidectomia , Febre/diagnóstico , Febre/cirurgia , Humanos , Linfadenite/diagnóstico , Linfadenite/cirurgia , Faringite/diagnóstico , Faringite/cirurgia , Estomatite Aftosa/diagnóstico , Estomatite Aftosa/cirurgia , Síndrome , TonsilectomiaRESUMO
Resumo Objetivo: Estabelecer diretrizes baseadas em evidências científicas para manejo da síndrome de febre periódica, estomatite aftosa, faringite e adenite (PFAPA). Descrição do método de coleta de evidência: A Diretriz foi elaborada a partir de cinco questões clínicas que foram estruturadas por meio do Pico (Paciente, Intervenção ou Indicador, Comparação e Outcome), com busca nas principais bases primárias de informação científica. Após definir os estudos potenciais para sustento das recomendações, esses foram graduados pela força da evidência e pelo grau de recomendação. Resultados: Foram recuperados e avaliados pelo título e resumo 806 trabalhos e selecionados 32 artigos, para sustentar as recomendações. Recomendações: 1. O diagnóstico da PFAPA é clínico e de exclusão, deve a suspeita ser considerada em crianças que apresentam episódios febris de origem indeterminada recorrentes e periódicos ou amidalites de repetição, intercalados com períodos assintomáticos, sobretudo em crianças em bom estado geral e com desenvolvimento pondero-estatural mantido; 2. Os achados laboratoriais são inespecíficos. Não existem alterações patognomônicas nos exames complementares; 3. A evidência que sustenta a indicação do tratamento cirúrgico (tonsilectomia com ou sem adenoidectomia) é baseada em dois ensaios clínicos randomizados não cegos que incluíram pequeno número de pacientes; 4. O uso de prednisona no início do quadro febril em pacientes com PFAPA mostrou ser eficaz. Melhores evidências ainda são necessárias para apoiar seu uso na PFAPA; 5. Apesar de os resultados obtidos de estudos com inibidores de IL-1ß serem promissores, esses são limitados a poucos relatos de casos.
Abstract Objective: To establish guidelines based on scientific evidence for the management of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. Description of the evidence collection method: The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. Results: 806 articles were retrieved and evaluated by title and abstract; from these, 32 articles were selected to support the recommendations. Recommendations: 1. PFAPA is a diagnosis of exclusion established on clinical grounds, and one must suspect of this problem in children with recurrent and periodic febrile episodes of unknown origin, or with recurrent tonsillitis interspersed with asymptomatic periods, especially in children in good general condition and with preservation of weight and height development. 2. Laboratory findings are nonspecific. Additional tests do not reveal pathognomonic changes. 3. The evidence supporting an indication for surgical treatment (tonsillectomy with or without adenoidectomy), is based on two non-blinded randomized clinical trials with small numbers of patients. 4. The use of prednisone at the onset of fever in patients with PFAPA proved to be an effective strategy. There is still need for more qualified evidence to support its use in patients with PFAPA. 5. Despite promising results obtained in studies with IL-1β inhibitors, such studies are limited to a few case reports.
Assuntos
Humanos , Estomatite Aftosa/terapia , Faringite/terapia , Guias de Prática Clínica como Assunto , Febre/terapia , Linfadenite/terapia , Estomatite Aftosa/cirurgia , Estomatite Aftosa/diagnóstico , Síndrome , Tonsilectomia , Adenoidectomia , Faringite/cirurgia , Faringite/diagnóstico , Febre/cirurgia , Febre/diagnóstico , Linfadenite/cirurgia , Linfadenite/diagnósticoRESUMO
OBJECTIVE: To seek evidence supporting a role for tonsillectomy or adenotonsillectomy in the management of affected children with periodic fever with aphthous stomatitis, pharnygitis, and adenitis (PFAPA) syndrome. STUDY DESIGN: A comprehensive literature search was conducted to identify all published English-language observational and randomized studies evaluating the efficacy of tonsillectomy or adenotonsillectomy on PFAPA syndrome. A combination of keywords was used to identify relevant articles. RESULTS: A total of 15 studies including 149 treated children were found, including 13 observational noncomparative studies and 2 randomized controlled trials. The pooled rate of complete resolution emerging from the combined analysis of all treated children was 83% (95% CI, 77%-89%). A meta-analysis of the two randomized controlled trials showed homogeneity of the results (P=.37, Breslow-Day test) and a common odds ratio for complete resolution of 13 (95% CI, 4-43; P<.001). CONCLUSIONS: Surgery appears to be a possible option for management of PFAPA syndrome. Available evidence is limited, however, and the precise role of surgery remains to be clarified. We suggest considering this option when symptoms markedly interfere with the child's quality of life and medical treatment has failed.
Assuntos
Febre/cirurgia , Linfadenite/cirurgia , Periodicidade , Faringite/cirurgia , Estomatite Aftosa/cirurgia , Tonsilectomia , Adenoidectomia , Criança , Humanos , Ensaios Clínicos Controlados Aleatórios como Assunto , SíndromeRESUMO
La amigdalectomía es la intervención quirúrgica más frecuente en otorrinolaringología y sus indicaciones son ampliamente conocidas en la especialidad. El avance en el conocimiento de sus funciones inmunológicas ha determinado su uso en el tratamiento de otras enfermedades tales como la nefropatía por IgA, síndromes neuropsiquiátricos, el síndrome de fiebre recurrente, algunas entidades dermatológicas, entre otras. Pretendemos así, revisar la literatura para evaluar la evidencia disponible que sustente lo que denominamos indicaciones no tradicionales. Al parecer, la evidencia a favor del uso de amigdalectomía en el tratamiento de algunas de estas enfermedades es sólido, mientras que para otras aún no supera la suposición teórica, pues sólo se cuenta con casos aislados. Consideramos necesario estudios más extensos, con mayor calidad metodológica para definir mejor la indicación de amigdalectomía. Esto, más la evaluación multidisciplinaria de cada caso nos debiera conducirá la mejor decisión.
Tonsillectomy is the most common surgical procedure in otorhinolaryngology and their indications are well known in the field. The advance in knowledge of their immune function has determined its use in the treatment of other diseases such as IgA nephropathy neuropsychiatric syndromes, periodic fever syndrome, some dermatologic entities, and others. We review the literature to evaluate the available evidence that supports what we cali nontraditional indications. Apparently the evidence for the use of tonsillectomy in the treatment of some diseases is solid, while others still not overcome the theoretical assumption, since there are only isolated cases. Larger studies are needed to consider, with higher methodological quality, to better define the indication for tonsillectomy. This and the multidisciplinary assessment of each case should lead us to the best decision.
Assuntos
Humanos , Estomatite Aftosa/cirurgia , Faringite/cirurgia , Glomerulonefrite por IGA/cirurgia , Tonsilectomia , Doenças Autoimunes do Sistema Nervoso/cirurgia , Dermatopatias/cirurgia , Febre , Infecções Estreptocócicas , Periodicidade , Seleção de Pacientes , Tomada de DecisõesRESUMO
OBJECTIVE: To evaluate whether adenotonsillectomy leads to complete resolution in children with PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome. STUDY DESIGN: Thirty-nine children with PFAPA syndrome were randomized to either adenotonsillectomy (surgery group; n = 19) or expectant management (control group; n = 20). All patients were then invited prospectively to record all PFAPA episodes, and were evaluated clinically every 3 months for 18 months after randomization. RESULTS: The proportion of patients experiencing complete resolution was 63% in the surgery group and 5% in the control group (P < .001). The mean (+/- standard deviation) number of episodes recorded during the study period was 0.7 +/- 1.2 in the surgery group and 8.1 +/- 3.9 in the control group (P < .001). The episodes were less severe in the surgery group. CONCLUSIONS: Adenotonsillectomy is an effective treatment strategy for children with PFAPA syndrome.