Management of supravalvular aortic stenosis with the Doty technique, in a South American pediatric center.

Supravalvular aortic stenosis (SVAS) is a congenital malformation associated with Williams-Beuren syndrome. It is characterized by severe stenosis of the sinotubular junction. The smooth muscle layer is thickened due to an alteration of the Elastin gene. The treatment is surgical and there are several techniques to relieve obstruction which range from the single-patch technique to the replacement of all three aortic sinuses with three patches. However, the optimal technique is still unclear. The objective of this article is to carry out a bibliographic review of surgical techniques for SVAS and the description of the application of the Doty technique for a 4-year-old infant at the Roberto Gilbert Elizalde Hospital.

La estenosis aórtica supravalvular es una malformación congénita asociada al síndrome de Williams-Beuren. Se caracteriza por una severa estenosis en la unión sinotubular. La capa de músculo liso se engrosa debido a una alteración en el gen Elastina. El tratamiento es quirúrgico y existen varias técnicas para aliviar la obstrucción, que van desde la técnica de un solo parche hasta el reemplazo de los tres senos aórticos con tres parches. Sin embargo, la técnica óptima aún no está clara. El objetivo de este artículo es realizar una revisión bibliográfica de las técnicas quirúrgicas para estenosis valvular aórtica supravalvular y describir la aplicación de la técnica de Doty en una niña de 4 años en el Hospital Roberto Gilbert Elizalde.

Access and adherence to isoniazid preventive therapy and occurrence of active TB in a cohort of people living with HIV: a retrospective cohort study in Sao Paulo, Brazil.

Tuberculosis (TB) is still a leading cause of morbidity and mortality among people living with HIV (PLHIV). The diagnosis of latent TB is required for the implementation of prophylactic therapy with isoniazid (PTI). However, low access to diagnosis of latent TB and non-adherence to PTI may hinder potential benefits of this essential intervention. In this study, we addressed the access and adherence to PTI in a cohort of PLHIV with positive tuberculin skin test (TST) in a reference HIV clinic in Sao Paulo, Brazil. We have also analyzed the occurrence of active TB over a median of 131 months after a positive TST among study participants. Our findings revealed that 88.3% of the 238 TST-positive patients had access to PTI, and 196 (93.3%) of those with access adhered to PTI. Active tuberculosis was diagnosed in three of the 196 TST-positive patients who adhered to PTI (1.5%; 95% confidence interval [CI] 0.3-4.4%), whereas seven cases were detected among 42 patients without access or who did not adhere to PTI (16.6%; 95% CI 7.0-31.3%). The apparent beneficial effect of PTI in our cohort is consistent with previous studies including PLHIV, and highlights the importance of reliably delivering each of the steps between screening for latent TB and provision of PTI.

Risk for cancer among people living with AIDS, 1997-2012: the São Paulo AIDS-cancer linkage study.

Previous studies have reported an increased risk for certain types of cancer in the HIV-infected population. The aim of this study was to assess the risk for cancer in people with AIDS (PWA) in comparison with the general population in São Paulo (Brazil), between 1997 and 2012. A population-based registry linkage study was carried out to assess the risk for cancer, using a standardized incidence ratio (SIR) approach. A total of 480 102 person-years, of which 337 941 (70.4%) person-years were men, were included in the analysis. Around 2074 cancer cases were diagnosed among PWA, of which 51.0% were non-AIDS-defining cancers (NADC). The risk for AIDS-defining cancers and NADC in the male population with AIDS was significantly higher than that in the general population (SIR=27.74 and 1.87, respectively), as it was in the female population with AIDS compared with the general population (SIR=8.71 and 1.44, respectively). Most virus-related NADC occurred at elevated rates among PWA: anal cancer (SIR=33.02 in men and 11.21 in women), liver (SIR=4.35 in men and 4.84 in women), vulva and vagina (SIR=6.78 in women) and Hodgkin lymphoma (SIR=5.84 in men and 2.71 in women). Lung (SIR=2.24 in men and 2.60 in women) and central nervous system (SIR=1.92 in men and 3.48 in women) cancers also occurred at increased rates. Cancer burden among PWA in São Paulo was similar to that described in high-income countries such as the USA and Italy following the introduction of the highly active antiretroviral therapy. As coinfection with oncogenic viruses disproportionally affects this population, virus-related cancers accounted for a great share of excessive cases.

Cancer survival in people with AIDS: A population-based study from São Paulo, Brazil.

Cancer survival among people with AIDS (PWA) has been described in developed countries, but there is lack of data from developing countries. The aim of this study was to evaluate survival after cancer diagnosis in PWA and compare it with people without AIDS (non-PWA) in São Paulo, Brazil. A probabilistic record linkage was carried out between the databases of the Population-based Cancer Registry of São Paulo (PBCR-SP) and the AIDS registry of SP (SINAN) to identify PWA who developed cancer. For comparison, non-PWA were frequency matched from the PBCR-SP by cancer site/type, sex, age, and period. Hazard ratio (HR) stratified by matching variables was estimated using a Cox proportional hazards model. A total of 1,294 PWA (20 patients with two primary site tumors) were included in the site/type-specific analyses. AIDS-defining cancers (ADC) comprised 51.9% of cases assessed. The all-cancer 5-year overall survival in PWA was 49.4% versus 72.7% in non-PWA (HR = 2.64; 95%CI = 2.39-2.91). Survival was impaired in PWA for both ADC (HR = 2.93; 95%CI = 2.49-3.45) and non-ADC (HR = 2.51; 95%CI = 2.21-2.84), including bladder (HR = 8.11; 95% CI = 2.09-31.52), lung (HR = 2.93; 95%CI = 1.97-4.36) and anal cancer (HR = 2.53; 95%CI = 1.63-3.94). These disparities were seen mainly in the first year after cancer diagnosis. The overall survival was significantly lower in PWA in comparison with non-PWA in São Paulo, as seen in high-income countries. Efforts to enhance early diagnosis and ensure proper cancer treatment in PWA should be emphasized.

Trends in the incidence of AIDS-defining and non-AIDS-defining cancers in people living with AIDS: a population-based study from São Paulo, Brazil.

People living with AIDS are at increased risk of developing certain cancers. Since the introduction of the highly active antiretroviral therapy (HAART), the incidence of AIDS-defining cancers (ADCs) has decreased in high-income countries. The objective of this study was to analyse trends in ADCs and non-AIDS-defining cancers (NADCs) in HIV-positive people with a diagnosis of AIDS, in comparison to the general population, in São Paulo, Brazil. A probabilistic record linkage between the 'Population-based Cancer Registry of São Paulo' and the AIDS notification database (SINAN) was conducted. Cancer trends were assessed by annual per cent change (APC). In people with AIDS, 2074 cancers were diagnosed. Among men with AIDS, the most frequent cancer was Kaposi's sarcoma (469; 31.1%), followed by non-Hodgkin lymphoma (NHL; 304; 20.1%). A decline was seen for ADCs (APC = -14.1%). All NADCs have increased (APC = 7.4%/year) significantly since the mid-2000s driven by the significant upward trends of anal (APC = 24.6%/year) and lung cancers (APC = 15.9%/year). In contrast, in men from the general population, decreasing trends were observed for these cancers. For women with AIDS, the most frequent cancer was cervical (114; 20.2%), followed by NHL (96; 17.0%). Significant declining trends were seen for both ADCs (APC = -15.6%/year) and all NADCs (APC = -15.8%/year), a comparable pattern to that found for the general female population. Trends in cancers among people with AIDS in São Paulo showed similar patterns to those found in developed countries. Although ADCs have significantly decreased, probably due to the introduction of HAART, NADCs in men have shown an opposite upward trend.

Screening anal dysplasia in HIV-infected patients: is there an agreement between anal pap smear and high-resolution anoscopy-guided biopsy?

PURPOSE: The purpose of this study was to analyze the agreement between anal Pap smear and high-resolution anoscopy-guided biopsy in diagnosing anal dysplasia in HIV-infected patients. METHODS: We conducted cross-sectional analysis of HIV-infected patients receiving anal dysplasia screening as part of routine care. Agreement between measures was estimated by weighted kappa statistics, using a three-tiered cytologic and histologic grading system (normal, low-grade dysplasia, and high-grade dysplasia). Estimates of sensitivity, specificity, and predictive values were calculated using a two-tiered cytologic and histologic grading system ("without dysplasia" and "with dysplasia of any grade"). Estimates were also calculated for the detection of high-grade dysplasia. RESULTS: During a one-year period, 222 patients underwent 330 anal Pap smears followed by high-resolution anoscopy-guided biopsies. There were 311 satisfactory Pap smears with concurrent biopsies. Considering histology the standard, the frequency of anal dysplasia was 46%. Kappa agreement between anal Pap smear and biopsy was 0.20. For detection of anal dysplasia of any grade, anal Pap smear showed sensitivity of 61%, specificity of 60%, positive predictive value of 56%, and negative predictive value of 64%. For high-grade dysplasia, anal Pap smear showed sensitivity of 16% and specificity of 97%. CONCLUSION: Anal Pap smears alone were not sensitive enough to rule out anal dysplasia. We recommend that high-resolution anoscopy-guided biopsy be incorporated as a complementary screening test for anal dysplasia in high-risk patients. Following baseline high-resolution anoscopy, these individuals could be followed with serial anal cytology to dictate the need for future high-resolution anoscopy-guided biopsies.

Aspectos sócio-econômicos dos indivíduos com malária importada na regiäo metropolitana de Säo Paulo, Brasil. I. Caracterizaçäo da populaçäo e conhecimento sobre a doença / Socioeconomic aspects of subjects with imported malaria in the metropolitan region of, Säo Paulo, about Brazil. I. Characterization of the population and knowledge of the disease

Com o objetivo de conhecer o perfil sócio-econômico dos indivíduos que se deslocaram das áreas endêmicas de malária do país, foram estudadas 566 pessoas com suspeita de malária que procuraram a confirmaçäo diagnòstica no Laboratòrio de Malária da Regiäo Metropolitana de Säo Paulo da Superintendência de Controle de Endemias (SUCEN). As informaçöes foram obtidas através da aplicaçäo de formulário, no período de novembro de 1986 a junho de 1987. Da populaçäo estudada, 345 (61,0 por cento) residiam na área endêmica, 479 (84,6 por cento) eram do sexo masculino, 513 (90,7 por cento)estavam na faixa etária de 15 a 55 anos e 307 (54,2 por cento) apresentaram hemoscopia positiva para plasmòdio. Com relaçäo à ocupaçäo na área de transmissäo, observou-se que 109 (19,3 por cento) estavam ligados a atividade de extraçäo de minerais, 74(13,2 por cento) à agricultura e 46(8,1 por cento) à atividade de transporte. A análise da escolaridade mostrou que 486 (85,9 por cento) tinham 1§ ou 2§ grau. Quanto ao conhecimento sobre a doença, 384 (67,8 por cento) declararam pelo menos uma malária anterior e 491(86,8 por cento) associavam à doença e presença do vetor. Dentre os 221 indivíduos residentes em Säo Paulo, 207(93,7 por cento) conheciam o risco de contrair malária por ocasiäo do deslocamento para área de transmissäo. Daqueles residentes na área endêmica, 336(97,4 por cento) tinham conhecimento do risco de contrair a doença naquela regiäo. O intervalo transcorrido entre os primeiros sitomas e a procura de atendimento médico em 386 (68,2 por cento) indivíduos variou de 0 a 3 dias. As frequências das variáveis estudadas mostraram de acordo com o resultado hemoscòpico e o local de residência, diferenças estatísticas relevantes