Resumo No Brasil, os inquéritos sorológicos têm assinalado taxa de infecção pelo vírus do dengue de 25% a 56%, porém esses estudos foram realizados em populações de cidades de médio ou grande porte. No presente estudo, são descritas duas epidemias de febre clássica de dengue (DEN) no Estado da Bahia. A primeira, ocorrida em 1987 e causada pelo sorotipo DEN-1 em Ipupiara e, a segunda, causada pelo DEN-2, em Prado e que ocorreu em 1995. O diagnóstico laboratorial foi realizado utilizando o teste de inibição da hemaglutinação (IH). Em 1995, foram coletadas 461 amostras sorológicas de uma população de 3.868 habitantes em Ipupiara (região da Chapada Diamantina) e 228 de um total de 9.126 habitantes em Prado (Litoral Extremo Sul). A soro-positividade das amostras foi de 11,9% (55/461) em Ipupiara e 17,5% (40/228) em Prado. Não houve diferença, estatisticamente significante, quanto a idade e o gênero entre os indivíduos soro-positivos e negativos das duas cidades estudadas. Entretanto, em Ipupiara os soro-positivos (15,9% vs. 9,3%) relataram, mais freqüentemente (p < 0,03), residência ou viagens para outros Estados do Brasil. Com base nos dados, estimou-se a ocorrência de 460 e 1.597 casos da infecção em Ipupiara e Prado, respectivamente. Em conclusão, nas cidades de menor porte a dinâmica da infecção pelo vírus do dengue, provavelmente, tem características peculiares, porque nessas localidades a prevalência é menor em conseqüência das menores potencialidades de desenvolvimento do Aedes aegypti. Palavras-chaves: Dengue. Epidemia. Inquérito soro-epidemiológico. Bahia.Abstract Serologic studies in Brazil have indicated a 25% to 56% prevalence of dengue virus infections. However, these studies were carried out in populations of middle-sized and larger cities. The present study describes two epidemics of classic dengue fever in two small cities in the State of Bahia. The first occurred in 1987, in Ipupiara and was caused by dengue serotype-1 (DEN-1), the second occurred in 1995, in Prado and was caused by dengue serotype-2 (DEN-2). The laboratory diagnosis was made by the hemagglutination-inhibitation test. 461 serum samples were collected in 1995 in Ipupiara (district of Chapada Diamantina) out of a population of 3,868 and 228 samples out of a total of 9,126 inhabitants of Prado (in the Southernmost coastal region). The seropositivity of the samples was 11.9% (55/461) in Ipupiara and 17.5% (40/228) in Prado. These were no statistically significant differences as to age and sex between seropositive and seronegative individuals of the two cities studied. However, the seropositive cases in Ipupiara indicated a higher percentage (15.9% vs. 9.3%) of residency in or travel to other states of Brazil (p<0.03). Based on these data we estimate the occurrence of 460 and 1,597 cases of infection in Ipupiara and Prado, respectively. In conclusion, dengue virus infections in smaller cities probably have singular characteristics, since they exhibit a lesser prevalence of seropositivity caused by decreased breeding possibilities of Aedes...
Introduction: The hemoglobinopathies are the most common monogenic disorders known. A mutation in the gene for β globin gave origin to hemoglobin S, an abnormal hemoglobin originated in Africa. Sickle cell disease (SCD) is characterized by the presence of hemoglobin S, which results in vasoocclusion episodes and hemolytic anemia throughout patients life. Vascular occlusion leads to acute events and progressive disabling organ damage. Sickle cell anemia is the homozygous state SS, while hemoglobinopathy SC is a doubly heterozygous state, where hemoglobin S occurs in combination with hemoglobin C. Brazil has a prominent African ancestry and SCD is highly prevalent in some regions of the country. In Bahia State, for example, neonatal screening data have shown that, from every 650 children born alive, one has SCD, mostly homozygous SS. Among other therapeutic measures, packed red blood cells (RBC) play a prominent role in SCD management. In situations such as acute chest syndrome (ACS), primary and secondary prevention of stroke, splenic or hepatic sequestration crisis, severe anemia, complicated pregnancy, isquemic organ damages and others, the transfusions may save lives. Although RBC may contribute to reduce morbidity and improve quality of life in SCD patients, there still are risks. Among other risk categories, alloimmunization may result from transfusions and occurs in 5 % to 50 % of SCD patients. It is still not known whether allosensibilization significantly affects the clinical outcomes in SCD. Objecive: The purpose of this study was to compare the clinical profile of multitransfused adult SCD patients who developed alloantibodies (ALO) to patients with the same disease, coming from the same population who did not become alloimmunized (non-ALO). Methods: This is a cross sectional study where medical records of SCD patients, referred to a reference center of Salvador, the capital of Bahia State, Brazil, were reviewed. Only SCD patients 18 years of age or older were included. They had received at least 3 RBC transfusions from 2004 to 2007, or had any alloantibody identified during this period. Patient characteristics, clinical findings, number of transfusions, frequency and specificity of alloantibodies, laboratory data, and the main clinical outcomes were reviewed. Results: a hundred and eight patients were included: 105 SS and 3 SC. The pre-transfusional RBC matching was done to ABH, D,C,c,E,e and Kell antigens. 56 patients developed alloantibodies (53 SS and 3 SC). Anti-E, anti-K, and anti-C were the most prevalent alloantibodies identified (39,3 %, 21,4 % and 16,1 %, respectively). Among the variables addressed in this study, age (higher in non-ALO, .041) and antiglobulin test positivity, more prevalente in ALO (.0001), depicted statistically significant difference. A few patients developed immune hemolysis, controlled successfully with corticosteroids. Alloimmunization was more prevalent among women, although no statistically significant difference was reached between ALO and non-ALO Other variables such as number of transfusions, hematological profile, biochemical data and complications such as stroke, leg ulcers, osteonecrosis, renal disease, abnormal cardiac features, and pulmonary hypertension did not show significant difference between both groups. Conclusion: his study shows that, although alloimmunization is a potential dangerous consequence of RBC transfusions, it did not modify the clinical profile of SCD alloimmunized patients. The concomitance of allosensibilization and autoantibodies in SCD leads to additional difficulties in the RBC matching for transfusion and may exacerbate hemolysis. In order to address autoimmunity in SCD, prospective studies with larger samples are needed.
Adult T-cell leukemia/lymphoma (ATL) is an aggressive type of leukemia/lymphoma associated with the human T-cell lymphotropic virus (HTLV-I). We describe an adult male patient clinically and pathologically diagnosed as mycosis fungoides and treated with chemotherapy after which complete involution of the lesions occurred. The disease relapsed with confluent dyshidrosis-like vesicles on the palmoplantar regions, followed by disseminated vesiculopapules and associated lymphocytosis. A serological test performed at this time revealed HTLV-I infection, and a diagnosis of chronic ATL was made. Monoclonal integration of HTLV-I was detected in peripheral blood mononuclear cells by inverse long polymerase chain reaction. A skin biopsy revealed spongiosis, Pautrier abscesses, and intraepidermal vesicles with atypical lymphocytes and an infiltration of small and atypical CD4 lymphocytes in the superficial dermis. Proliferative index (Ki-67) was 70%. This is the first reported vesicular cutaneous ATL with confirmation of HTLV-I proviral integration. The delay that occurred in diagnosing ATL was due to the fact that mycosis fungoides and ATL may present the same clinical, histopathological, and immunohistochemical features.
Background: ABO incompatibility occurs in up to 20–40% of HLA matched allogeneic hematopoietic stem cell transplantation (HSCT). Pure red cell aplasia (PRCA) following ABO-incompatible allogeneic HSCT is a rare complication associated with interaction of recipient anti-A or anti-B isoagglutinins, produced by residual B cells, with donor erythrocytes precursors expressing A and/or B antigens. The best treatment approach for PRCA is not established. Plasma exchange and immunoadsorption are regarded as a first-line treatment strategies. Other therapeutic approaches include: erythropoietn (EPO), donor-derived leukocyte infusion (DLI), steroids or induction of graft versus host disease (GVHD) by immunosuppression withdraw. Moreover, recovery or erythropoiesis is often incomplete and may induce futher complications. Recent reports describe successful treatment of PRCA and others cytopenias after HSCT using Rituximab (Mabthera®; Roche; Switzerland). This drug is a chimeric IgG1 monoclonal antibody directed against the CD20 surface antigen expressed by most human B lymphocytes. Its exact mechanism of action is not known. Case Report: We treat a 47-year-old woman with PRCA after a major ABO-mismatched (Donor A Rh-; Recipient O Rh-) allogeneic HSCT after a second remission of a acute myeloid leukemia. Allogeneic HSCT was performed in November 2005, from a HLA-matched older sister, using bone marrow graft (Mn cell dose 1,72x108/kg) following a myeloablative conditioning with busulfan (16mg/kg) and melfalan (140mg/m2). GVHD prophylaxis included cyclosporine A and methotrexate. Baseline anti-A isoagglutinin IgM titers of the paient before transplantation was 1:128 and after immunoadsorption with plasma A for 48 hours decreased to 1:32. One plasmapheresis session was performed to remove ABO antibodies from the patient and her anti-A IgM titer dropped to 1:4 before bone marrow infusion. We also removed red blood cells from the donor’s marrow product to prevent risk of hemolysis. After bone marrow transplantation, the patient developed a severe anemia that required weekly red blood cells transfusion over six months. Reticulocyte counts were extremely low during this period, bone marrow aspirate was normal, except for decreased erythrocyte precursors (only 1% of total cells). In August 2006, the diagnosis of PRCA was set after exclusion of red blood cells alloantibodies, hemolysis and viral or bacterial infection. Donor chimerism (STR) was totally completed and the recipient anti-A IgM titer was 1:16 and the patient showed no evidence of GVHD. In October 2006, she was treated with rituximab 375mg/m2 administered once weekly for four weeks. In the next two months, after the treatment with rituximab, her hemoglobin (Hb) increased from 5.5g/dl to 12g/dl. Up to her last medical avaluation (July 2006), she was in PRCA remission, her Hb was 12g/dl and she had no detectable anti-A titer. Conclusion: Rituximab can be a very effective treatment to PRCA secondary to major ABO-incompatible allogeneic stem cell transplantation. The best dose schedule and long term effects must be establish.
The occurrence of cases of yellow fever (YF) and also the extensive distribution of A. aegypti in Brazil, inspired a study about the estimate of immunity against vaccinal virus (17D) among the residents at two cities of the Bahia State, Ipupiara (n = 461) and Prado (n = 228). At this non-endemic area of YF, the search for serology antibody against 17D (Ab17D) and 18 another arbovirus was made thereby hemagglutination inhibition (HI). Only 1.2% (8/689) showed Ab17D, six of those with monotypic sort. The heterotypic sort for flavivirus (FLV) was interpreted as associated to immunity against 17D too, being much frequent in Prado (30.3%) than in Ipupiara (23.2%). The age > or = 50 years and residence in another states were related to seropositive for FLV, the same way that vaccination's history (17D). However, the history of vaccination presented low percentages of sensibility (< or = 45.4%) and predictive-positive value (< or = 38.4%), but high specificity (> or = 70.8%) and predictive-negative value (> or = 78.8%). Therefore, the frequency of residents with Ab17D was low (1.2%), although the higher frequency (25.5%) of antibody FLV carrier's, what signifies that 26.7% of the studied population should present protection against the YF virus.
Background: Acquired inhibitors against factor VIII (FVIII), also termed Acquired Hemophilia A (AHA), occur rarely in the nonhemophilic population. Its incidence is approximately 1 to 4 per million/year. The incidence of AHA increases with age and a major peak is seeing in patients aged 68 to 80 years. These autoantibodies are associated with high rate of morbidity (90% of severe bleeding in affected patients) and mortality (8–22% of the cases). The diagnosis of AHA is based on the demonstration of an isolated prolongation of the activated partial thromboplastin time (APTT), not corrected by incubating the patient’s plasma with equal volumes of normal plasma (mixing study), associated with FVIII reduced levels and formal evidence of a FVIII inhibitor in a patient with no previous personal or family history of bleeding. In approximately half of affected patients there is no identification of relevant concomitant diseases. The bleeding pattern in AHA is characterized by hemorrhages into the skin, muscles or soft tissues, and mucous membranes. Rituximab (Mabthera®; Roche; Switzerland) is a chimeric monoclonal antibody against the pan B-cell antigen CD20 that induces a rapid in vivo depletion of normal B lymphocytes. Primarily developed to treat B-cell non Hodgkin Lymphomas, more recently, Rituximab has demonstrated effectiveness in a number of autoantibody-mediated diseases including AHA. Case Report: We treated a 69-year-old diabetic and hypertensive patient with AHA. In March 2007, the diagnosis was made by a prolonged APTT (ratio 2.74 sec), not corrected by the mixing study, and FVIII inhibitor 320 Bethesda units. The patient had no evidence of malignancy, autoimmune disease or use of drugs. His bleeding manifestations were large ecchymosis of upper and lower extremities for almost a year, and at admission to the hospital, a hematoma of left calf and gengival bleeding. First line therapy consisted of steroids and cyclophosphamide (CFM). The steroids had to be withdraw after one week of use, because of very difficult glycemic control. The CFM were use in the dose of 50mg/day adding an total accumulative dose of 3g. This protocol were used considering the incapacity of the patient to tolerate higher dose of CFM due to leukopenia. After treatment, the patient was evaluated and neither normalization of APTT nor improvement of clinical findings happened. Therefore, considering all the above, it was decided to use Rituximab 375mg/m2, weekly, for four weeks. In June 2007, the patient received and tolereted very well all the infusions. In August 2007, he had no bleeding symptoms and his APTT was normal. At his laterest visit to the clinic in July 2008, an year after finishing his treatment, he was still very well and his bleeding tests were normal. Conclusion: Rituximab should be considered a treatment option for AHA.
Objetivo: Analisar a judicialização do cinacalcete no estado do Rio de Janeiro e estimar o seu impacto no orçamento do estado do Rio de Janeiro no ano de 2015. Métodos: Estudo transversal descritivo que analisou os pareceres técnicos emitidos pelo Núcleo de Assessoria Técnica em Ações de Saúde do Tribunal de Justiça do Rio de Janeiro entre 2009 e 2016. Realizou-se uma busca no banco de licitações da Secretaria de Estado de Saúde do Rio de Janeiro para encontrar o valor pago por esse medicamento em 2015. Resultados: Entre 2009 e 2015, esse núcleo elaborou 23.852 pareceres, com 1.553 relacionados ao cinacalcete, sendo 359 em 2015. Entre os autores, 88% residiam na capital deste estado, 50,4% eram mulheres, 46% tinham renda entre 1 e 3 salários mínimos. A decisão judicial foi favorável ao autor em 100% dos processos. Conclusão: Em 2015, foram gastos cerca de 3,7 milhões de reais para compra desse medicamento, o que equivale a 2,5% dos recursos destinados à assistência farmacêutica do estado do Rio de Janeiro neste ano, caracterizando um elevado impacto no orçamento da saúde
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