Apresentação atípica de síndrome de hiper-IgM ligada ao X simulando doença inflamatória intestinal / Atypical presentation of X-linked hyper-IgM syndrome simulating inflammatory bowel disease

Relatamos o caso de um paciente do sexo masculino, que iniciou quadro de úlceras em trato gastrointestinal, associado a febre recorrente e diarreia com muco e sangue aos 10 meses de vida, suspeitado inicialmente de doença inflamatória intestinal, no entanto, não apresentou melhora do quadro com terapia imunossupressora, sendo realizada investigação para erro inato da imunidade. Nos exames laboratoriais, apresentou níveis baixos de IgG e IgA e níveis elevados de IgM e neutropenia persistente. Diante disso, foi realizado teste genético que confirmou diagnóstico de síndrome de hiper-IgM ligada ao X. Os erros inatos da imunidade podem se manifestar com doenças do trato gastrointestinal, de forma relativamente frequente, devendo entrar como diagnóstico diferencial de diarreia crônica. Inclusa nesse grupo de doenças, as síndromes de hiper-IgM constituem um grupo heterogêneo de doenças, possuindo em comum níveis significativamente baixos ou ausentes de IgG e IgA e níveis normais ou elevados de IgM, o que predispõe a infecções e febre recorrente; além de outras alterações laboratoriais, como neutropenia, que pode estar associada a úlceras no trato gastrointestinal e proctite, simulando apresentação clínica de doença inflamatória intestinal. Para o paciente relatado, foi iniciada terapia com imunoglobulinas de forma periódica, além de antibioticoprofilaxia para infecções, evoluindo com resposta clínica satisfatória. O artigo possui objetivo principal de alertar para o diagnóstico diferencial de erros inatos da imunidade diante do quadro apresentado, visando o diagnóstico precoce e a instituição da terapia adequada.

We report the case of a male patient, who started with ulcers in the gastrointestinal tract, associated with recurrent fever and diarrhea with mucus and blood at 10 months of life, initially suspected of inflammatory bowel disease, however, he did not improve the condition with immunosuppressive therapy, being investigated for inborn error of immunity. In laboratory tests, he had low levels of IgG and IgA and high levels of IgM and persistent neutropenia. Therefore, a genetic test was performed and confirmed the diagnosis of X-linked hyper IgM syndrome. Inborn errors of immunity can manifest relatively frequently with diseases of the gastrointestinal tract, and should be included as a differential diagnosis of chronic diarrhea. Included in this group of diseases, hyper-IgM syndromes constitute a heterogeneous group of diseases, having in common significantly low or absent levels of IgG and IgA and normal or high levels of IgM, which predispose to infections and recurrent fever; in addition to other laboratory alterations, such as neutropenia, which may be associated with ulcers in the gastrointestinal tract and proctitis, simulating the clinical presentation of inflammatory bowel disease. For the reported patient, therapy with immunoglobulins was started periodically, in addition to antibiotic prophylaxis for infections, evolving with a satisfactory clinical response. The main objective of the article is to alert to the differential diagnosis of inborn errors of immunity in view of the presented condition, aiming at early diagnosis and the institution of adequate therapy.

O estado da arte das síndromes autoinflamatórias associadas à criopirina / The state of the art of cryopyrin-associated periodic syndromes

As síndromes autoinflamatórias associadas à criopirina (CAPS) compreendem um grupo espectral de doenças raras autoinflamatórias. Todas estas doenças estão relacionadas ao inflamassoma NLRP3, sendo que de 50-60% dos pacientes apresentam mutações ao longo do gene NLRP3. Clinicamente, febre recorrente associada à urticária neutrofílica e outros sintomas sistêmicos são o grande marco clínico, comum a todo o espectro. O bloqueio da interleucina-1 trouxe grande alívio ao tratamento destas desordens, mas variações na resposta clínica podem ser observadas, principalmente nos espectros mais graves. Neste trabalho os autores trazem uma revisão do estado da arte das doenças autoinflamatórias CAPS. Foi realizado levantamento de literatura e, ao final, 49 artigos restaram como base para construção do texto final. O trabalho traz de forma narrativa os principais pontos relacionados a imunofisiopatologia, manifestação clínica, diagnóstico, tratamento, complicações e novas armas diagnósticas, e terapia gênica.

Cryopyrin-associated periodic syndromes (CAPS) comprise a spectrum of rare autoinflammatory disorders. They are all related to the NLRP3 inflammasome, and 50-60% of the patients harbor mutations along the NLRP3 gene. Clinically, recurrent fever associated with neutrophilic urticaria and other systemic symptoms are a hallmark of all the disorders in the spectrum. Biologic drugs that can block interleukin-1 were a milestone for the treatment of such rare diseases, although variability in clinical response to this therapeutic intervention were observed, especially in those affected by severe phenotypes. In this paper, the authors provide a state-of-the-art review of CAPS. A literature search was performed and, finally, 49 articles remained for the construction of the final manuscript. The article presents a narrative review focused on the topics related to immune pathophysiology, clinical manifestations, diagnosis, treatment, complications and new therapeutic options, and gene therapy.

Síndrome de fiebre periódica, estomatitis aftosa, faringitis y adenitis / Periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome

Introducción: El síndrome de fiebre periódica, estomatitis aftosa, faringitis y adenitis es un cuadro relativamente frecuente de curso autolimitado y buen pronóstico. Es la más común de las enfermedades autoinflamatorias que presentan fiebre recurrente y su etiología y fisiopatogenia permanecen inciertas. Objetivo: Describir el caso clínico de un niño que consultó por fiebre recurrente y faringitis y se arribó al diagnóstico de síndrome de fiebre periódica, estomatitis aftosa, faringitis y adenitis. Presentación del caso: Se trata de un paciente de 5 años que consultó por un episodio febril acompañado de faringitis y adenitis, con antecedente de múltiples episodios previos. Conclusiones: El síndrome fiebre periódica, estomatitis aftosa, faringitis y adenitis, es una entidad de curso benigno y autolimitado que constituye la causa más común de fiebre recurrente con un gran impacto en la vida de los pacientes. La fisiopatogenia permanece incierta. Es destacable la sospecha clínica para arribar al diagnóstico ya que no existen en la actualidad exámenes complementarios específicos(AU)

Introduction: Periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome is a relatively common picture of self-limiting course and good prognosis. It is the most common of self-inflammatory diseases with recurrent fever and its etiology and physiopathology remain uncertain. Objective: Describe the clinical case of a boy who was checked due recurrent fever and pharyngitis and being diagnosed with Periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome. Case presentation: This is a 5-year-old patient who was consulted due to a febrile episode accompanied by pharyngitis and adenitis, with a history of multiple previous episodes. Conclusions: Periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome is a benign, self-limiting course entity that is the most common cause of recurrent fever with a large impact on patients' lives. Its physiopathology remains uncertain. Clinical suspicion for diagnosis is noteworthy as there are currently no specific complementary test for it(AU)

Caracterización de pacientes con fiebre de origen desconocido recurrente / Characterizing patients with recurrent fever of unknown origin

Introducción: La fiebre de origen desconocido es un síndrome que genera un gran desafío para el pensamiento clínico; el patrón recurrente es el subtipo más retador y de difícil diagnóstico etiológico. Objetivo: Caracterizar los pacientes con fiebre de origen desconocido con patrón recurrente. Método: Se realizó un estudio descriptivo de corte transversal en el periodo de enero de 2008 a diciembre de 2015. La muestra quedó constituida por 48 pacientes, los cuales fueron hospitalizados y se les realizó una evaluación clínica en el Hospital Clínico Quirúrgico Hermanos Amejeiras. Para el análisis de los datos se utilizaron medidas de resumen para las variables cualitativas y para las cuantitativas, media y desviación estándar. Resultados: La edad media fue 41,3 años, predominaron los hombres (62,5 por ciento), la duración media de la fiebre fue de 497 días y las pistas clínicas más importantes fueron los hallazgos de adenopatías (43,8 por ciento) y hepatomegalia (22,9 por ciento). Los casos sin diagnóstico fueron 45,8 por ciento. Conclusiones: La fiebre de origen desconocido con patrón recurrente es de difícil diagnóstico. A pesar de una evaluación clínica minuciosa, la mayoría de los pacientes quedan sin diagnóstico(AU)

Introduction: Fever of unknown origin is a syndrome that generates great challenge for clinical thought; the recurrent pattern is the most challenging subtype and with difficult etiological diagnosis. Objective: To describe patients with fever of unknown origin with recurring pattern. Method: A descriptive cross-sectional study was carried out from January 2008 to December 2015. Forty-eight patients formed the sample. They were hospitalized and underwent clinical evaluation at Hermanos Amejeiras Surgical Clinical Hospital. For data analysis, summary measures were used for qualitative variables and mean and standard deviation variables were used for quantitative. Results: The mean age was 41.3 years, men predominated (62.5 percent), the mean duration of fever was 497 days, and the most important clinical signs were the findings of lymphadenopathy (43.8 percent) and hepatomegaly. (22.9 percent). Undiagnosed cases were 45.8 percent. Conclusions: Fever of unknown origin with recurring pattern is difficult to diagnose. Despite careful clinical evaluation, most patients remain undiagnosed(AU)

Síndrome PFAPA ­ A propósito de um caso clínico nos Cuidados Primários / PFAPA Syndrome - About a clinical case in Primary Health Care / Síndrome PFAPA - A propósito de un caso clínico en los Cuidados Primarios

Objetivo: Apresentar informações sobre o diagnóstico e tratamento da síndrome PFAPA na Atenção Primária à Saúde. Métodos: Revisão sistemática de literatura baseada na recomendação PRISMA e realizada nas bases de dados Scielo, Lilacs, Medline, IBECS e PubMed, incluindo estudos publicados no período de 2004 a 2018, além da consulta a outros documentos específicos da síndrome PFAPA. Resultados: Após busca e seleção, foram incluídos 31 artigos. Avaliação e Diagnóstico: A síndrome PFAPA acomete principalmente crianças, sendo caracterizada por febre periódica acompanhada por faringite, estomatite aftosa e/ou adenite cervical. Seu diagnóstico é clínico e por exclusão, baseado em critérios estabelecidos. Recomendações: Os episódios costumam responder a prednisona e, em graus variáveis, a cimetidina e colchicina. Casos refratários e acompanhados de hipertrofia tonsilar são candidatos a tonsilectomia, devendo ser encaminhados à avaliação otorrinolaringológica.

Síndrome PFAPA: Diagnóstico e Tratamento na Atenção Primária à Saúde / PFAPA Syndrome: Diagnosis and Treatment in Primary Health Care / Síndrome PFAPA: Diagnóstico y Tratamiento en la Atención Primaria a la Salud

Objetivo: Apresentar informações sobre o diagnóstico e tratamento da síndrome PFAPA na Atenção Primária à Saúde. Métodos: Revisão sistemática de literatura baseada na recomendação PRISMA e realizada nas bases de dados Scielo, Lilacs, Medline, IBECS e PubMed, incluindo estudos publicados no período de 2004 a 2018, além da consulta a outros documentos específicos da síndrome PFAPA. Resultados: Após busca e seleção, foram incluídos 31 artigos. Avaliação e Diagnóstico: A síndrome PFAPA acomete principalmente crianças, sendo caracterizada por febre periódica acompanhada por faringite, estomatite aftosa e/ou adenite cervical. Seu diagnóstico é clínico e por exclusão, baseado em critérios estabelecidos. Recomendações: Os episódios costumam responder a prednisona e, em graus variáveis, a cimetidina e colchicina. Casos refratários e acompanhados de hipertrofia tonsilar são candidatos a tonsilectomia, devendo ser encaminhados à avaliação otorrinolaringológica.

Objective: To present information about the diagnosis and treatment of PFAPA syndrome in Primary Health Care. Methods: Systematic review of literature based on the PRISMA recommendation and carried out in the Scielo, Lilacs, Medline, IBECS and PubMed databases, including studies published from 2004 to 2018, in addition to consulting other PFAPA syndrome specific documents. Results: After search and selection, 31 articles were included. Assessment and Diagnosis: PFAPA syndrome affects mainly children and is characterized by periodic fever accompanied by pharyngitis, aphthous stomatitis and/or cervical adenitis. Its diagnosis is clinical and by exclusion, based on established criteria. Recommendations: The episodes usually respond to prednisone and, in varying levels, cimetidine and colchicine. Refractory cases and accompanied by tonsillar hypertrophy are candidates for tonsillectomy, and should be referred to otorhinolaryngological evaluation.

Objetivo: Presentar informaciones sobre el diagnóstico y tratamiento del síndrome PFAPA en la Atención Primaria a la Salud. Métodos: Revisión sistemática de literatura basada en la recomendación PRISMA y realizada en las bases de datos Scielo, Lilacs, Medline, IBECS y PubMed, incluyendo estudios publicados en el período 2004 a 2018, además de la consulta a otros documentos específicos del síndrome PFAPA. Resultados: Después de la búsqueda y selección, se incluyeron 31 artículos. Evaluación y Diagnóstico: El síndrome PFAPA acomete principalmente niños, siendo caracterizada por fiebre periódica acompañada por faringitis, estomatitis aftosa y/o adenitis cervical. Su diagnóstico es clínico y por exclusión, basado en criterios establecidos. Recomendaciones: Los episodios suelen responder a la prednisona y, en grados variables, a la cimetidina y colchicina. Los casos refractarios y acompañados de hipertrofia tonsilar son candidatos a tonsilectomía, debiendo ser encaminados a la evaluación otorrinolaringológica.

Series de Pediatría Garrahan. El niño y las infecciones / Garrahan Pediatrics Series. The child and the infections

Las consultas pediátricas por temas infectológicos son frecuentes y relevantes durante toda la infancia, desde la etapa neonatal hasta la adolescencia. En este nuevo volumen de las Series Garrahan: El ñino y las infecciones, se han seleccionado temas específicos, sobre la base de la actualización del conocimiento, los cambios epidemiológicos y de las conductas clínicas ocurridos en los últimos años y la necesidad del manejo adecuado de estas afecciones, ya sea ambulatorio o durante la internación. Entre sus aspectos sobresalientes se incluyen: El estudio de temas destacados como el abordaje del niño febril; las infecciones de piel y partes blandas, incluidas las asociadas con mordeduras; las infecciones en el recién nacido; las infecciones respiratorias bajas, incluida la tuberculosis; y las infecciones osteoarticulares, del sistema nervioso central e intraabdominales. La inclusión de un capítulo especial sobre la prevención de infecciones para ayudar a reducir su incidencia. El enfoque práctico, con discusión de casos clínicos y definición de conductas, y ubicando al pediatra en un papel central como coordinador de la atención interdisciplinaria. Aspectos clave y lecturas recomendadas en el cierre de cada capítulo. Una obra actualizada que aporta información científica y la experiencia de los profesionales del Hospital Garrahan, dedicada a todos los miembros del equipo de salud que atienden y cuidan niños dondequiera que trabajen al servicio de la salud infantil

Algunas consideraciones diagnósticas y terapéuticas del paciente con síndrome febril en Colombia / Some considerations regarding diagnosis and therapeutics of patients with febrile syndrome in Colombia

Sr. Editor, hemos leído con gran interés la revisión realizada por Cortés et al. respecto al enfoque clínico del síndrome febril agudo en Colombia, recientemente publicada en la revista INFECTIO. A manera de contribución, en primer lugar, quisiéramos resaltar la importancia de incluir y considerar la enfermedad de Chagas (en su fase aguda) como otro de los diagnósticos diferenciales a tener en cuenta y de suma importancia, en el contexto del síndrome febril agudo en Colombia, particularmente en los últimos años.

Mr. Editor, we have read with great interest the review by Cortés et al. on the clinical approach to acute febrile syndrome in Colombia, recently published in the journal INFECTIO. As a contribution, first of all, we would like to highlight the importance of including and considering Chagas disease (in its acute phase) as another of the differential diagnoses to be taken into account and of utmost importance in the context of acute febrile syndrome in Colombia, particularly in recent years.

Respuesta a la carta de Faccini et al / Reply to Faccini et al

Sr. Editor, Agradecemos los aportes en relación con nuestra publicación. El objetivo del artículo "Enfoque clínico del síndrome febril agudo en Colombia" fue el de considerar los posibles diagnósticos etiológicos que se enmarquen dentro de la definición de la duración de la fiebre en el tiempo, definiendo como aguda aquella que tiene una duración de hasta 7 días. Sin embargo, el cuadro de Chagas agudo incluye en su definición de caso probable "Fiebre continua o prolongada mayor de 7 días, acompañado o no de alguno de los siguientes síntomas. . .". Este criterio ha sido utilizado en los estudios de brotes referenciados en Colombia. Por esta razón, no fue incluido como parte de la revisión

Mr. Editor, We appreciate the contributions in relation to our publication. The aim of the article "Clinical approach to acute febrile syndrome in Colombia" was to consider the possible etiological diagnoses that fall within the definition of the duration of fever over time, defining as acute that which has a duration of up to 7 days. However, acute Chagas disease includes in its probable case definition "Continuous or prolonged fever greater than 7 days, accompanied or not by any of the following symptoms . . .". This criterion has been used in outbreak studies referenced in Colombia. For this reason, it was not included as part of the review.

Síndrome PFAPA / PFAPA syndrome

Introducción: PFAPA es una enfermedad pediátrica caracterizada por fiebre recurrente asociada a síntomas de cabeza y cuello. La etiopatogenia es desconocida. Se han usado diferentes tratamientos, incluyendo corticoides, cimetidina y tonsilectomía. Objetivos: describir la epidemiología, presentación clínica, hallazgos de laboratorio, diagnóstico diferencial y tratamiento. Métodos: se realizó una revisión sistemática de reportes de casos y ensayos clínicos de PFAPA en las bases de datos Lilacs, Medline, Scielo y Biblioteca Cochrane. Fueron utilizados como descriptores DeCS/MeSH las palabras clave:PFAPA syndrome, periodic fever in children. Resultados: se revisaron 23 publicaciones con 379 casos. La edad promedio al inicio fue de 3.2 años (0.05 a 37) y habían transcurrido 3.5 años (0.08 a 20) desde el diagnóstico. Las crisis febriles duraban 4.4 días (2 a 14), con periodicidad de 29.5 días (7 a 70). Conclusión: el síndrome PFAPA es una entidad de descripción relativamente reciente, necesitándose mayor información para un diagnóstico rápido y un abordaje terapéutico.

Detection of Borrelia persica infection in Ornithodoros tholozani using PCR targeting rrs gene and xenodiagnosis

Relapsing fever caused by Borrelia persica, is an acute tick-borne disease which is transmitted by soft ticks of Ornithodoros tholozani to human. Value of PCR and xenodiagnosis for detection of B. persica in O. tholozani ticks was compared. Sixty-four Borrelia- free ticks were fed on infected guinea pigs and used for the experiments. For xenodiagnosis, a group of 32 ticks in subsequent blood meal were fed on sterile guinea pigs and the indication of B. persica in the animal blood was tested 5-14 days later by dark-field microscopy. For PCR, all 64 ticks were subjected to PCR against B. persica rrs gene [16S-rDNA]. Also sensitivity of PCR in terms of minimum detectable number of spirochetes as well as the effects of tick sex and post digestion was tested. PCR revealed B.persica DNA in 98.4% ticks, in which B. persica were found in 25.0% by xenodiagnosis. PCR was enough sensitive to give positive results for DNA of 1 spirochete. PCR success rates were similar for male or female ticks. Course of time did not affect the efficacy of PCR and similar results were observed for ticks of immediately fed, semior completely gravid or completely digested blood ones. Our results indicate that due to very low specificity and time consuming, xenodiagnosis is not a useful method whereas PCR method has advantages for study the Borrelia prevalence in ticks

A case report of relapsing fever.

Relapsing fever is an acute febrile illness caused by spirochetes of the genus Borrelia. The high fevers of presenting patients spontaneously abate and then recur. Here we report a 50-year-old woman having relapsing fever associated with thrombocytopenia. Giemsa staining of peripheral blood smear revealed spiral organisms morphologically resembling Borrelia. A rare case of relapsing fever which was successfully treated with doxycycline is discussed.

Caracterización epidemiológica, clínica y de algunos parámetros inmunológicos del síndrome de infección recurrente en niños y adolescentes desplazados a la ciudad de Cúcuta / Epidemiological, clinical and immunological characteristics of recurrent infection syndrome in individuals from low income neighborhoods from Cúcuta

Objetivo:Caracterizar desde el punto de vista epidemiológico, clínico e inmunológico el síndrome de infección recurrente en niños y adolescentes desplazados a cuatro barrios de Cúcuta. Metodología: Estudio descriptivo de corte transverso, de noviembre de 2003 a enero de 2004. Se realizó una encuesta a todos los individuos entre 0 y 15 años de edad y se clasificaron en síndrome de infección recurrente normal o en casos sospechosos de infección recurrente anormal de acuerdo con criterios previamente establecidos. Los pacientes con sospecha de síndrome de infección recurrente anormal fueron evaluados en consulta médica y con estudios básicos de laboratorio para definir su causa. Resultados: Se encuestaron 328 individuos, con edad promedio de 6,1 años; el 50,3 por ciento eran masculinos y 49,7 por ciento femeninos. Setenta (21,3 por ciento) fueron clasificados como síndrome de infección recurrente y 19 (5,8 por ciento) como sospechosos de síndrome de infección recurrente anormal. De estos úlimos doce presentaron síndrome de infección recurrente anormal no inmunológico (10 casos por alergias y dos por cardiopatía congénita), cinco, síndrome de infección recurrente anormal inmunológico, con un posible caso de deficiencia selectiva de lg A y dos fueron reclasificados como síndrome de infección recurrente normal después del análisis tanto clínico de laboratorio. Discusión: En la población desplazada estudiada la mayoría de los casos de síndrome de infección recurrente anormal son causados por enfermedades alérgicas. La encuesta probada fue útil para realizar tamización para la detección del síndrome de infección recurrente anormal.

Tick-borne relapsing fever, a neglected cause of fever in Fars province

Tick-borne relapsing fever is an endemic disease in some parts of Iran. Borellia persica, the most common cause of this disease in Iran, has a wide geographic distribution and is present in Alborz and Zagros mountain chains. Here we report a 16-year-old male patient who presented with two episodes of fever within 15 days. He had a history of few overnight stays in a cave of mountainous area in Fars province. Sites of tick bites were found on exposed areas of extremities. Spirochetes were detected in Wright-stained smears of the patient's peripheral blood. He was successfully treated with penicillin. To the best of our knowledge, there has been no reported case of tick-borne relapsing fever from Fars province in the literature during the last 28 years. Tick-borne relapsing fever should be considered in patients with recurrent fever and peripheral blood smear should be investigated for spirochetes. Further seroepidemiologic studies should be carried out to determine the prevalence of this disease in Fars province

Community Knowledge; Attitudes and Practices Related to Tick-Borne Relapsing Fever in Dodoma Rural District; Central Tanzania

Tick-borne Relapsing Fever (TBRF) is a vector-borne disease of humans which causes serious illness; primarily for children under five years old and pregnant women. Understanding people's knowledge; attitude and practices on the disease is important in designing appropriate interventions. This study was conducted to explore community knowledge; attitudes and practices regarding TBRF transmission and control to provide baseline data for the planned scaling up of intervention in Dodoma rural District in central Tanzania. A total of 198 heads of households were interviewed using a semi-structured questionnaire. Of these; 94.5were aware of TBRF. Fever of unknown origin (69.5); body pain (8.5); headache (8.5); chills (4.5) and vomiting (3.5) were the most commonly mentioned symptoms. The domestic tick-infestation and tickbites was known to 82.8. High domestic tick infestation reported to occur during dry season (85.4). The majority believed that the disease spreads through tick-bites (85.9). Regular plastering of house floors and walls was the most common method used by the community to control domestic tick infestation (96.5). Majority (84.3) of the respondents preferred public healthcare facilities for treatment. Only a small proportion (15.7) preferred using traditional medicines. Poor knowledge of the study subjects about the disease and its control underscores the need for health educational campaigns if any control and/or elimination programme is to succeed

Distribution of soft ticks and their natural infection with Borrelia in a focus of relapsing fever in Iran

Tick-borne diseases such as relapsing fever and Crimean-Congo Hemorrhagic Fever [CCHF] are of public health importance in Iran. There are 471 reported cases of relapsing fever in 2003, according to the Ministry of Health of Iran. The number of cases has been increased in recent years. Its distribution is more or less prevalent in different parts of Iran. The aim of this study was to find out the fauna and natural infection of soft ticks with Borrelia in Qazvin Province, during their seasonal activity. The province covers 15821 km[2] between 48-45 to 50-50 east of Greenwich Meridian of longitude and 35-37 to 36-45 north latitude of the equator. For this purpose a field study was carried out in the region. A total of 54 villages from 19 districts were selected randomly and ticks were collected from their habitats according to the standard method. A total of 3197 Argasidae ticks were collected from human dwellings, poultry and animal shelters. They belonged to Argas and Ornithodoros genera which 36.8% were Argas persicus, 4% A. reflexus, 6.4% O. canestrini, 45.5% O. lahorensis and 7.3% O. tholozani. It should be noted that 12 ticks of O. erraticus were collected from 12 rodents borrows. We found that 8.82% of O. tholozani ticks were infected with Borrelia persica and half of the O. erraticus were infected with Borrelia microti. All the people who are involved with veterinary activities should be aware of disease transmission by the ticks. In the endemic area of the disease tick control is recommended

Aspectos de interesse clínico sobre a síndrome PFAPA (febre periódica, estomatite aftosa faringite a adenite / Clinical aspects of PFAPA syndrome (periodic fever, aphthous somatitis, pharyngitis and adenitis

O objetivo do a artigo é apresentar uma revisão da literatura sobre síndrome PFAPA, enfocando suas características clínicas, métodos e diagnósticos e tratamento. Foram consultados artigos indexados no Medline, publicados no período de 1984 a 2003. A síndrome PFAPA é uma enfermidade benigna, que se caracteriza por episódios recorrentes de febre alta, estomatite aftosa, faringite e adenite cervical em crianças de faixa etária inferior a cinco anos. As crianças apresenta-se saudáveis entre episódios febris, cujo o interval é, classicamente, de 28 dias. A prednisona aborta os sinais e sintomas atenuam-se espontaneamente com o avanço da idade não são descritas sequelas a longo prazo. Após a análise da literatura, fica clara a importância do correto diagnóstico diferencial com a síndrome de Bechet, síndrome da imonuglobulinemiaD, febre familiar do mediterrâneo e neutropenia cíclica. Uma vez confirmado o diagnóstico, o profissional deve tranquilizar e orientar os familiares, esclarecendo-ossobre a diminuição da recorrência dos quadros com o avanço da idade ou mesmo completa remissão com a chegada da puberdade.