A novel association of acquired ADAMTS13 inhibitor and acute dengue virus infection.

BACKGROUND: Dengue is a mosquito-borne viral disease with an increasing incidence worldwide. Thrombocytopenia is a common finding in dengue virus (DV) infection; however, the underlying mechanisms remain unknown. CASE REPORT: Here we provide the first evidence of a case of antibody formation against ADAMTS13 (ADAMTS13 inhibitor) in the course of a severe acute DV infection resulting in thrombotic microangiopathy (TMA). The patient presented with classical dengue symptoms (positive epidemiology, high fever, myalgia, predominantly in the lower limbs and lumbar region for 1 week) and, after 11 days of initial symptoms, developed TMA. Clinical and laboratorial investigation of dengue and TMA was performed. RESULTS: The patient presented with ADAMTS13 inhibitor (IgG) during the acute phase of the disease, without anti-platelet antibodies detectable. Dengue infection had laboratorial confirmation. There were excellent clinical and laboratory responses to 11 serial plasma exchanges. Anti-ADAMTS13 inhibitor disappeared after remission of TMA and dengue resolution. No recurrence of TMA symptoms was observed after 2-year follow-up. CONCLUSIONS: Although the real incidence of dengue-related TMA is unknown, this case provides the basis for future epidemiologic studies on acquired ADAMTS13 deficiency in DV infection. The prompt clinical recognition of this complication and early installment of specific therapy with plasma exchange are likely to improve the outcome of severe cases of dengue.

[Spotted fever in Campinas region, State of São Paulo, Brazil]. / Situação da febre maculosa na Região Administrativa de Campinas, São Paulo, Brasil.

Brazilian spotted fever was detected for the first time in the State of S o Paulo in 1929. However, there is no systematic reporting of the disease in the State. In 1985, three cases of the disease occurred in the municipality of Pedreira, located in the Campinas Region, belonging to the 5th Administrative Region, in the Northeast part of the State, including 88 municipalities. An investigation was conducted at the time, but the lack of case registry limited its scope. The present study was undertaken with the aim of recovering the history of the disease in the Region. Data recovered from several public health services for 1985-2000 were used to analyze incidence patterns. It was observed that the transmission area expanded and the number of suspected cases increased, especially after 1996, when mandatory reporting was established. Deaths due to spotted fever were observed in most of the years under study. The study concluded that spotted fever incidence is increasing in the Campinas Region. Complementary bio-ecological studies are currently under way to better understand the epidemiology of this disease, recognized worldwide as an emerging public health problem.

Situaçäo da febre maculosa na Regiäo Administrativa de Campinas, São Paulo, Brasil / Spotted fever in Campinas region, State of São Paulo, Brazil

A febre maculosa brasileira foi detectada pela primeira vez no Estado de São Paulo em 1929. No entanto, näo há registro sistemático de casos neste Estado. Em 1985 ocorreram três casos desta doença no Município de Pedreira, situado na regiäo de Campinas, que fica no nordeste do Estado de São Paulo, Brasil, correspondendo à 5ª Regiäo Administrativa, e compreendendo 88 municípios. Alguns estudos foram realizados no Município de Pedreira, mas a falta de registro de casos foi um obstáculo encontrado. Com a finalidade de recuperar o histórico da doença, resolveu-se pesquisar e registrar as ocorrências de febre maculosa na regiäo no período de 1985 a 2000 e analisar o seu comportamento. Foram recuperados todos os registros da doença nos diversos serviços de saúde pública. Observou-se uma ampliaçäo da área de transmissäo e a ocorrência de um aumento dos casos suspeitos a partir de 1996, ano em que a doença foi determinada como de notificaçäo compulsória na regiäo. Esta doença foi causa de óbito na maioria dos anos do período de estudo. Conclui-se que a febre maculosa está em ascensäo na regiäo e estudos bioecológicos complementares estäo sendo desenvolvidos para melhor compreensäo da epidemiologia dessa doença, que é mundialmente reconhecida como um problema emergente de saúde pública