RESUMO
OBJECTIVE: To describe clinical, diagnostic and therapeutic characteristics of the periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. DATA SOURCE: Literature review in the PubMed database by using specific descriptors to identify all articles published in the English language in the last three years; 38 articles were found. After performing selection of titles and abstract analysis, 13 out of the 38 articles were fully read. Relevant studies found in the references of the reviewed articles were also included. DATA SYNTHESIS: The PFAPA syndrome (Periodic Fever, Aphthous Stomatitis, Pharyngitis and cervical Adenitis) is a medical condition grouped among the periodic fever syndromes. The etiology is uncertain, but possibly multifactorial, and its symptoms are accompanied by recurrent febrile episodes although weight and height development are preserved. It is a self-limiting disease of benign course with remission of two to three years without significant interference in the patient's overall development. Treatment consists of three pillars: interruption of febrile episodes, increase in the interval between episodes, and remission. CONCLUSIONS: Despite several attempts to establish more sensitive and specific criteria, the diagnosis of PFAPA syndrome is still clinical and reached by exclusion, based on the modified Marshall's criteria. The most common pharmacological options for treatment include prednisolone and betamethasone; colchicine may be used as prophylaxis, and surgical treatment with tonsillectomy can be considered in selected cases.
Assuntos
Linfadenite , Faringite , Estomatite Aftosa , Criança , Febre/diagnóstico , Febre/etiologia , Humanos , Linfadenite/complicações , Linfadenite/diagnóstico , Linfadenite/terapia , Faringite/complicações , Faringite/diagnóstico , Faringite/terapia , Estomatite Aftosa/complicações , Estomatite Aftosa/diagnóstico , Estomatite Aftosa/terapia , SíndromeRESUMO
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
Lymphadenitis caused by non-tuberculous mycobacteria is an uncommon manifestation in immunocompetent individuals. Here, we report a case of Mycobacterium fortuitum infection in a previously healthy 9-year-old patient who developed cervical lymphadenitis evolving to a suppurative ulcer associated with a varicella-zoster virus infection. We discuss the relationship between the varicella-zoster virus and the immune response of the host as an explanation for the unusual progression of the case.
Assuntos
Herpes Zoster/complicações , Linfadenite/microbiologia , Infecções por Mycobacterium não Tuberculosas/complicações , Mycobacterium fortuitum , Dermatopatias Bacterianas/complicações , Criança , Feminino , Herpesvirus Humano 3/isolamento & purificação , Humanos , Hospedeiro Imunocomprometido , Linfadenite/complicações , Mycobacterium fortuitum/isolamento & purificação , Dermatopatias Bacterianas/microbiologiaRESUMO
Lymphadenitis caused by non-tuberculous mycobacteria is an uncommon manifestation in immunocompetent individuals. Here, we report a case of Mycobacterium fortuitum infection in a previously healthy 9-year-old patient who developed cervical lymphadenitis evolving to a suppurative ulcer associated with a varicella-zoster virus infection. We discuss the relationship between the varicella-zoster virus and the immune response of the host as an explanation for the unusual progression of the case.
Assuntos
Criança , Feminino , Humanos , Herpes Zoster/complicações , Linfadenite/microbiologia , Mycobacterium fortuitum , Infecções por Mycobacterium não Tuberculosas/complicações , Dermatopatias Bacterianas/complicações , /isolamento & purificação , Hospedeiro Imunocomprometido , Linfadenite/complicações , Mycobacterium fortuitum/isolamento & purificação , Dermatopatias Bacterianas/microbiologiaRESUMO
We report three familial cases of periodic fever with aphthous stomatitis, pharyngitis, and cervical adenitis syndrome, including a pair of monozygotic twins and their mother. It suggests that periodic fever with aphthous stomatitis, pharyngitis, and cervical adenitis syndrome may have a certain monogenetic background.
Assuntos
Doenças Hereditárias Autoinflamatórias/genética , Linfadenite/genética , Faringite/genética , Estomatite Aftosa/genética , Adulto , Pré-Escolar , Feminino , Doenças Hereditárias Autoinflamatórias/complicações , Humanos , Lactente , Linfadenite/complicações , Pescoço , Faringite/complicações , Estomatite Aftosa/complicaçõesRESUMO
This case report concerns a 40-year-old patient with an unspecific abdominal pain, diarrhoea, a big axillary mass and a previous pulmonary infection. After biopsy of the axillary mass the diagnosis of lymphoma was excluded based on the presence of cells expressing polyclonal antibodies. Abdominal CT scans and angionuclear magnetic resonance showed an extensive intestinal venous thrombosis. The patient also presented positive results for C and S proteins, lupic anticoagulant factor and antiphospholipid antibodies (anticardiolipin antibodies - IgM and IgG). Treatment started with administration of recombinant tissue plasminogen activator and heparin which decreased the degree of thrombosis. Antibiotics were also administrated to treat pulmonary and abdominal infections. After 25 days, he was discharged with no signs of infection, no abdominal pain and reduction of the thrombosis. He was medicated with warfarin, hydroxichloroquine and clopidogrel. Forty-five days after discharge, abdominal CT scan showed a significant regression of thrombosis.
Assuntos
Trombose Venosa/diagnóstico , Abdome , Adulto , Anticorpos Antifosfolipídeos/sangue , Axila , Humanos , Lúpus Eritematoso Sistêmico/complicações , Linfadenite/complicações , Masculino , Trombose Venosa/sangue , Trombose Venosa/complicaçõesAssuntos
Febre de Causa Desconhecida/terapia , Glucocorticoides/administração & dosagem , Linfadenite/terapia , Faringite/terapia , Ensaios Clínicos Controlados Aleatórios como Assunto , Estomatite Aftosa/terapia , Tonsilectomia/métodos , Administração Oral , Criança , Cimetidina/administração & dosagem , Febre de Causa Desconhecida/complicações , Seguimentos , Antagonistas dos Receptores H2 da Histamina/uso terapêutico , Humanos , Linfadenite/complicações , Faringite/complicações , Estomatite Aftosa/complicações , Fatores de Tempo , Resultado do TratamentoRESUMO
La linfadenitis supurada es una complicación poco frecuente que sigue a la vacunación con bacilo de Calmette-Guerin. Se describen los casos de dos niños con reacciones adversas graves inducidas por esta vacuna, en ambos casos, linfadenitis regional supurada y abscedada, un mes después de nacidos. Después de cursos infructuosos de cirugía y quimioterapia, ambos recibieron interferón gamma recombinante por vía intramuscular, en una dosis inicial de 50 000 UI/kg (máximo: 1 000 000 UI), diariamente durante las primeras 4 semanas, y se disminuyó luego la frecuencia de administración. Esta citoquina fue bien tolerada, solo se presentaron complicaciones con fiebre, que fueron controladas bien con antipiréticos. El interferón gamma recombinante puede constituir una nueva y efectiva alternativa terapéutica para el tratamiento de la linfadenitis supurada causada por este bacilo.
Suppurative lymphadenitis is a non frequent complication following Bacillus Calmette-Guerin (BCG) vaccination. Two paediatric patients with adverse reactions induced by the BCG vaccine are presented, both with suppurative and abscessed regional lymphadenitis, one month after birth. After failed courses of surgery and chemotherapy, they were treated with 50 000 IU/Kg (maximum: 1 000 000 IU) of recombinant interferon (IFN) gamma, intramuscularly, daily during 4 weeks and 3 or 2 tpw afterwards. The first case, a nursing girl with family history of tuberculosis, had a rapid involution of the lesions since the first month of treatment, with drainage ceasing and gradual disappearance of the inflammatory signs. At the end of the 6 months of treatment, residues of the lesions were imperceptible and new adenopathies or relapses were not detected during 4 years of follow up. The second case, a boy without family history of tuberculosis, presented more lesions. The signs of marked improvement were observed in the whole affected region one year after IFN gamma started. Their treatment was extended for almost 2 years, when the scars took the normal skin pigmentation. The cytokine was well tolerated; few febrile events were recorded, well-controlled with antipyretics. We can conclude that IFN gamma could be a new effective therapeutic alternative for the treatment of the suppurated lymphadenitis caused by BCG vaccination.
Assuntos
Humanos , Criança , Interferon gama/uso terapêutico , Linfadenite/complicações , Vacina BCG/efeitos adversos , Relatos de CasosRESUMO
La tuberculosis, tanto en su forma pulmonar como extrapulmonar constituye una enfermedad reemergente al nivel mundial, asociada con insuficiencias de los programas de control sanitario o con el síndrome de inmunodeficiencia adquirida. El modo de presentación de las formas extrapulmonares se relaciona con síntomas constitucionales, que incluyen la fiebre y con signos dependientes del órgano afectado. Se presentó el caso de una adenitis granulomatosa en etapa o categoría 3, grupo integrado por aquellos casos nuevos de formas menos grave de tuberculosis extrapulmonar (TBe) con confirmación histopatológica del bacilo. Se enfatizó sobre la presentación a forma de fiebre de origen desconocido (FOD), al reunir los criterios necesarios por haber estado ingresada en otro centro hospitalario por más de 1 mes, sin haber llegado al diagnóstico a pesar de haber sido estudiada, y se resaltó la necesidad imperiosa de la búsqueda de este diagnóstico ante todo paciente con cuadro adénico febril e historia familiar de TB.
Tuberculosis, both in its pulmonary and extrapulmonary form is a reemerging disease in the world associated with insufficiencies of the health control programs or with the acquired immunodeficiency syndrome. The mode of presentation of the extrapulmonary forms is related to constitutional symptoms that include fever, and to signs depending on the affected organ. The case of a stage III granulomatous adenitis, a group composed of those new cases of less severe forms of extrapulmonary tuberculosis with histopathological confirmation of the bacillus, was presented. Emphasis was made on the presentation as a FUO, on having the necessary criteria for having been admitted in another hospital for more than a month without a definitive diagnosis despite having been studied. The pressing need to search this diagnosis in every patient with febrile adenic picture and family history of TB was stressed.
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Febre de Causa Desconhecida/complicações , Linfadenite/complicações , Tuberculose dos Linfonodos/diagnósticoRESUMO
OBJECTIVE: We carried out a prospective, randomized, controlled trial to clarify the effect of tonsillectomy on the clinical course of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome. STUDY DESIGN: Twenty-six consecutive children (mean age 4.1 years) with at least 5 PFAPA attacks were recruited from 3 tertiary care pediatric hospitals during 1999-2003 and randomly allocated to tonsillectomy or follow-up alone. They were all followed up with symptom diaries for 12 months. Tonsillectomy was allowed after 6 months in the control group if the attacks recurred. RESULTS: Six months after randomization all 14 children in the tonsillectomy group and 6/12 children in the control group (50%) were free of symptoms (difference 50%, 95% confidence interval 23% to 75%, P < .001). Tonsillectomy was performed on 5/6 of the patients in the control group who still had symptoms after 6 months. The remaining unoperated child in the control group had recurrences of the fever episodes throughout the follow-up, but the symptoms became less severe, and the parents did not choose tonsillectomy. CONCLUSION: Tonsillectomy appeared to be effective for treating PFAPA syndrome. The fever episodes ceased without any intervention in half of the control subjects. We conclude that although the mechanisms behind this syndrome are unknown, tonsillectomy can be offered as an effective intervention for children with PFAPA.