OBJETIVO: relatar a ocorrência de uma deficiência funcional de neutrófilos rara, com quadro clínico e laboratorial semelhante ao da doença granulomatosa crônica. MÉTODOS: relato de caso de paciente com deficiência acentuada da glicose-6-fosfato desidrogenase e infecções de repetição. Realizada pesquisa bibliográfica utilizando as bases de dados Medline e Lilacs, abrangendo o período de 1972 a 2000. RESULTADOS: paciente com nível da glicose-6-fosfato desidrogenase extremamente reduzido e quadro de infeções graves com melhora clínica após uso de cotrimoxazol contínuo. Os leucócitos do paciente apresentam defeito no metabolismo oxidativo, similar ao da doença granulomatosa crônica. CONCLUSÕES: o diagnóstico da deficiência da glicose-6-fosfato desidrogenase em neutrófilos deve ser considerado em qualquer paciente com anemia hemolítica não esferocítica congênita no qual o nível da glicose-6-fosfato desidrogenase esteja anormalmente baixo ou apresente infeções de repetição. É diagnóstico diferencial da doença granulomatosa crônica.

OBJECTIVE: To report a case of rare neutrophil functional disorder with clinical and laboratory findings similar to those of chronic granulomatous disease. METHODS: Patient with extremely reduced level of glucose-6-phosphate dehydrogenase and recurrent infections that improved after continuous use of cotrimoxazole. The patient presented leukocytes with defective respiratory burst, similar to what occurs in chronic granulomatous disease. COMMENTS: The diagnosis of glucose-6-phosphate dehydrogenase deficiency in neutrophils should be considered in any patient with hemolytic anemia whose level of G6PD is extremely low or in any patient that presents recurrent infections as differential diagnosis of chronic granulomatous disease.

OBJECTIVES: To determine the HIV vertical transmission rate (VTR) and associated risk factors by use of zidovudine and infant care education in Brazil. METHODS: Since 1995, a prospective cohort of HIV infected pregnant women has been followed at the Federal University of Rio de Janeiro. A multidisciplinary team was established to implement the best available strategy to prevent maternal-infant HIV transmission. Patients with AIDS or low CD4 and high viral load received anti-retroviral drugs in addition to zidovudine. Children were considered infected if they had 2 positive PCR-RNA tests between 1 and 4 months of age, or were HIV antibody positive after 18 months. Education regarding infant treatment and use of formula instead of breast feeding was provided. RESULTS: Between 1995 and August, 2000, HIV status was determined for 145 infants. Compliance with intra-partum treatment, infant treatment and use of formula was 88.2%. Intra-partum zidovudine treatment was completed in 134/145 (92.6%) of patients; 88.1% had rupture of membranes < 4 hours; 85.4% of mothers were asymptomatic. The mean CD4 count was 428.4 cells and mean viral load 39,050 copies. HIV vertical transmission rate was 4/145 (2.75%; CI: 0.1%-5.4%). The only risk factor significantly associated with transmission was a failure to use zidovudine intra-partum in 2 of the 4 mothers (50% versus 6.4% in non-transmitting mothers). A trend toward low CD4 and high viral load at entry, and rupture of membranes > 4 hours were associated with increased HIV transmission. CONCLUSION: HIV vertical transmission in Brazil was reduced to a level similar to other countries with the most effective prevention programs using a multidisciplinary team approach. A high level of compliance for use of anti-retroviral drugs, the provision of health education to mothers, and use of formula for all exposed infants.

Esta série de casos foi conduzida com o objetivo de descrever a apresentação clínica, as características epidemiológicas, os exames laboratoriais e a evolução dos casos de angiomatose bacilar (e/ou peliose parenquimatosa bacilar) ocorridos em cinco hospitais públicos do Rio de Janeiro, no período de 1990 a 1997, comparando a casuística encontrada com a relatada na literatura médica. Foram incluídos 13 pacientes com mediana de idade de 39 anos, sendo todos pertencentes ao sexo masculino. Todos os pacientes apresentaram infecções oportunistas e/ou neoplasias associadas ao vírus da imunodeficiência humana (HIV-1) anteriores (ou concomitantes) ao diagnóstico de angiomatose bacilar. A mediana da contagem de linfócitos T Helper encontrada foi de 96 células por mm³. O acometimento cutâneo foi a apresentação clínica de angiomatose bacilar mais freqüente deste estudo. A freqüência de remissão após tratamento específico foi discretamente maior do que a relatada na literatura médica, enquanto a de recidiva foi semelhante. A freqüência de angiomatose bacilar entre indivíduos infectados pelo HIV de dois hospitais do Rio de Janeiro foi de 1,42 casos por 1.000 pacientes, semelhante ao relatado na literatura médica e sugestivo de que a angiomatose bacilar é relativamente incomum em nosso meio.

The aim of this case series was to describe the clinical, laboratory and epidemiological characteristics and the presentation of bacillary angiomatosis cases (and/or parenchymal bacillary peliosis) that were identified in five public hospitals of Rio de Janeiro state between 1990 and 1997; these cases were compared with those previously described in the medical literature. Thirteen case-patients were enrolled in the study; the median age was 39 years and all patients were male. All patients were human immunodeficiency virus type 1 (HIV-1) infected and they had previous or concomitant HIV-associated opportunistic infections or malignancies diagnosed at the time bacillary angiomatosis was diagnosed. Median T4 helper lymphocyte counts of patients was 96 cells per mm³. Cutaneous involvement was the most common clinical manifestation of bacillary angiomatosis in this study. Clinical remission following appropriate treatment was more common in our case series than that reported in the medical literature, while the incidence of relapse was similar. The frequency of bacillary angiomatosis in HIV patients calculated from two of the hospitals included in our study was 1.42 cases per 1000 patients, similar to the frequencies reported in the medical literature. Bacillary angiomatosis is an unusual opportunistic pathogen in our setting.

Aspectos epidemiológicos da doença meningocócica registrados a partir da segunda metade da década de 80 impulsionaram as autoridades de saúde pública a discutirem as medidas de controle disponíveis. A ocorrência da doença entre adolescentes e o registro de surtos envolvendo escolas são os dois pontos que mais pressionaram uma revisão das medidas de controle disponíveis. O uso das vacinas antimeningocócicas polissacarídeas e as recomendações para o controle de surtos localizados (clusters) são outros aspectos que mereceram atenção recentemente. Objetivou, assim, apresentar um panorama atual de alguns aspectos da epidemiologia e do controle dos casos secundários da doença meningocócica.

Epidemiological features of meningococcal disease described as from the second half of the 80's inclusive, have motivated a revision of current guidelines for sporadic disease and outbreak control. The increase of disease among teenagers and linked cases involving schools are the two most significant aspects that have prompted the revision of control measures. Vaccination routines and advice for the disease management of clusters are also relevant features recently revised. This present paper describes the management and some epidemiological features of secondary cases.

Aspectos epidemiológicos da doença meningocócica registrados a partir da segunda metade da década de 80 impulsionaram as autoridades de saúde pública a discutirem as medidas de controle disponíveis. A ocorrência da doença entre adolescentes e o registro de surtos envolvendo escolas são os dois pontos que mais pressionaram uma revisão das medidas de controle disponíveis. O uso das vacinas antimeningocócicas polissacarídeas e as recomendações para o controle de surtos localizados (clusters) são outros aspectos que mereceram atenção recentemente. Objetivou, assim, apresentar um panorama atual de alguns aspectos da epidemiologia e do controle dos casos secundários da doença meningocócica.

Epidemiological features of meningococcal disease described as from the second half of the 80's inclusive, have motivated a revision of current guidelines for sporadic disease and outbreak control. The increase of disease among teenagers and linked cases involving schools are the two most significant aspects that have prompted the revision of control measures. Vaccination routines and advice for the disease management of clusters are also relevant features recently revised. This present paper describes the management and some epidemiological features of secondary cases.

Apresentação de um caso de paracoccidioidomicose com comprometimento do sistema nervoso central, manifestando-se por movimento involuntário do tipo tremor rubral. São discutidas, ainda, as outras formas de apresentação desta afecção relatadas na literatura.

A case of paracoccidioidomycoses of the central nervous system and movement disorders is reported in this paper, and neurological aspects are discussed.