Papel da Linfadenectomia no Tumor Renal

Objetivos: Avaliar os benefícios e morbilidade da linfadenectomia (LND) como componente integral da nefrectomia radical. Material e Métodos: Efetuámos uma pesquisa bibliográfica exaustiva com recurso a vários motores de busca informática, incluindo MEDLINE, PUBMED, GOOGLE SEARCH e AUA MEDSEARCH, correspondendo o período de análise aos últimos 30 anos, entre 1980 e 2010, e utilizando como palavras-chave, exclusivamente em terminologia anglosaxónica, as seguintes: lymph node dissection, lymphadenectomy, renal cell carcinoma, renal tumor, kidneyneoplasms, radical nephrectomy, staging e prognosis. Para uma validação rigorosa das publicações relevantes, utilizámos a Tabela de Níveis de Evidência e Graus de Recomendação de Oxford publicada em Março de 2009. Com base nestas informações, tentámos analisar em detalhe o papel da linfadenectomia no tratamento do carcinoma de células renais e o seu impacto em termos prognósticos. Resultados: Em mais de 150 artigos, considerados pelos autores como relevantes e bem estruturados, apenas 1 era um estudo prospetivo e aleatorizado (Nível de Evidência 1/Grau de Recomendação A). Neste estudo de 772 doentes N0 M0, a taxa de LND positiva foi de 4,0% em status ganglionar clinicamente negativo, não tendo havido qualquer vantagem na sobrevivência e, por isso, não foi recomendada por rotina. Embora a informação obtida da maioria dos estudos seja contraditória, vários estudos retrospetivos recomendam a LND como potencialmente benéfica em casos selecionados de tumor agressivo e em contexto de nefrectomia citorredutora seguida de terapêutica adjuvante,realçando alguns autores a remoção total, sempre que possível, de quaisquer adenopatias palpáveis. Conclusão: A LND não está recomendada, por rotina, em tumores renais com gânglios linfáticos clinicamente negativos. Terá algum benefício potencial em casos selecionados de tumor agressivo e em contexto de cirurgia citorredutora seguida de terapêutica adjuvante, incentivando-se nestas situações a remoção total, se possível, das adenopatias palpáveis. Consequentemente, o tipo de LND deverá ser individualizada e dependente do estadio/extensão da doença neoplásica. Será, provavelmente, útil em futuros protocolos de terapêutica adjuvante desde que associados a inquestionável melhoria da sobrevivência.

Gravidez na Transplantação Renal

Com a Transplantação Renal regista-se recuperação da fertilidade. A exposição a fármacos imunossupressores, como a prednisona, a ciclosporina, a azatioprina e o tacrolimus não está associada com um aumento da incidência de malformações congénitas. A Imunossupressão, particularmente com a ciclosporina, está relacionada com recém-nascidos com baixo peso ao nascer. Doentes transplantados têm um risco aumentado de complicações infecciosas, algumas com implicações importantes para o feto, como as infecções por citomegalovírus, herpes simples e toxoplasmose. Esta população tem uma maior frequência de prematuridade, variando a percentagem de nados vivos entre 70 e 100%. Impõe-se a manutenção de esforços continuados para identificar os factores de risco pré-gestacionais, optimizando as estratégias de abordagem neonatal para uma gravidez bem-sucedida.

Oncocitoma Renal na Gravidez. Uma Forma Invulgar de Hipertensão Secundária

Introdução: O oncocitoma renal representa 5-7% das neoplasias primárias do rim, é diagnosticado em doentes assintomáticos e caracteriza-se por um comportamento benigno, sem invasão dos tecidos adjacentes ou metastização. O seu diagnóstico no decurso da gravidez é raro, havendo poucos casos descritos na literatura. Caso clínico: Os autores apresentam o caso de uma nulípara de 32 anos com hipertensão arterial de difícil controlo diagnosticada às sete semanas gestacionais com internamento às 24 semanas por quadro de hipertensão crónica agravada com pré-eclâmpsia sobreposta, edema agudo do pulmão e instabilidade hemodinâmica com necessidade de suporte ventilatório mecânico, restrição do crescimento fetal e morte fetal. O estudo etiológico do quadro hipertensivo efectuado no período pós-parto permitiu demonstrar a existência de um tumor renal-oncocitoma. Conclusão: O comportamento clínico do oncocitoma renal permanece mal caracterizado durante a gravidez, podendo associar-se, apesar do seu comportamento teoricamente benigno, a um desfecho materno e fetal adverso. É fundamental excluir uma possível causa secundária nos quadros hipertensivos de difícil controlo.

Sirolimus-Induced Drug Fever in a Renal Transplant Patient: a Case Report

Herein we have described the case of a male renal transplant recipient who developed drug fever apparently related to sirolimus. He had been stable under an immunosuppressive regimen of tacrolimus and mycophenolate mofetil, but developed acute cellular rejection at 5 years after transplantation due to noncompliance. Renal biopsy showed marked interstitial fibrosis, and immunosuppression was switched from mycophenolate to sirolimus, maintaining low tacrolimus levels. One month later he was admitted to our hospital for investigation of intermittently high fever, fatigue, myalgias, and diarrhea. Physical examination was unremarkable and drug levels were not increased. Lactic dehydrogenase and C-reactive protein were increased. The blood cell count and chest radiographic findings were normal. After extensive cultures, he was started on broad-spectrum antibiotics. Inflammatory markers and fever worsened, but diarrhea resolved. All serologic and imaging tests excluded infection, immune-mediated diseases, and malignancy. After 12 days antibiotics were stopped as no clinical improvement was achieved. Drug fever was suspected; sirolimus was replaced by mycophenolate mofetil. Fever and other symptoms disappeared after 24 hours; inflammatory markers normalized in a few days. After 1 month the patient was in good health with stable renal function. Although infrequent, the recognition of drug fever as a potential side effect of sirolimus may avoid unnecessary invasive diagnostic procedures. Nevertheless, exclusion of other common causes of fever is essential.

Specificity and Sensitivity of Screening for Anti-HLA Antibodies in Kidney Allograft Dysfunction

BACKGROUND: Prospective testing for posttransplant circulating anti-HLA antibodies seems to be a critical noninvasive tool, but confirmatory data are lacking. MATERIALS AND METHODS: Over the last 3 years, peritubular capillary (PTC) C4d deposition was prospectively sought by an immunofluorescence technique applied to frozen tissue in biopsies obtained for allograft dysfunction. Screening for circulating anti-HLA class I/II alloantibodies (AlloAb) by the flow cytometric test was performed simultaneously. RESULTS: We evaluated 132 sets of biopsies and simultaneous serum samples. PTC C4d deposition was demonstrated in 15.9% (21/132) of biopsies. Circulating anti-HLA I/II AlloAb were detected in 25% (33/132) of serum samples. Employing receiver-operator characteristic (ROC) curves for all C4d-positive biopsies, screening for AlloAb showed a global specificity of 82% and sensitivity of 61.9%. When this analysis was restricted to biopsies obtained in the first month posttransplantation, the sensitivity increased to 81.8%, but the specificity decreased to 76.9%. After the first month posttransplantation, we observed sensitivity of 40.0% and a specificity of 86.4%. In the first month posttransplantation, all patients with a diagnosis of acute antibody-mediated rejection displayed circulating anti-HLA class I/II, but not always at the same time as the C4d-positive biopsy. CONCLUSIONS: In the first month posttransplantation, prospective monitoring of anti-HLA antibodies may be useful. The high sensitivity allows the identification of patients at risk, affording an earlier diagnosis of antibody-mediated rejection. After the first month, the test can be used to evaluate allograft dysfunction episodes, since positivity is highly suggestive of an antibody-mediated process.

Zero-Time Kidney Histology Predicts Early Graft Function

Our purposes are to determine the impact of histological factors observed in zero-time biopsies on early post transplant kidney allograft function. We specifically want to compare the semi-quantitative Banff Classification of zero time biopsies with quantification of % cortical area fibrosis. Sixty three zero-time deceased donor allograft biopsies were retrospectively semiquantitatively scored using Banff classification. By adding the individual chronic parameters a Banff Chronic Sum (BCS) Score was generated. Percentage of cortical area Picro Sirius Red (%PSR) staining was assessed and calculated with a computer program. A negative linear regression between %PSR/ GFR at 3 year post-transplantation was established (Y=62.08 +-4.6412X; p=0.022). A significant negative correlation between arteriolar hyalinosis (rho=-0.375; p=0.005), chronic interstitial (rho=0.296; p=0.02) , chronic tubular ( rho=0.276; p=0.04) , chronic vascular (rho= -0.360;P=0.007), BCS (rho=-0.413; p=0.002) and GFR at 3 years were found. However, no correlation was found between % PSR, Ci, Ct or BCS. In multivariate linear regression the negative predictive factors of 3 years GFR were: BCS in histological model; donor kidney age, recipient age and black race in clinical model. The BCS seems a good and easy to perform tool, available to every pathologist, with significant predictive short-term value. The %PSR predicts short term kidney function in univariate study and involves extra-routine and expensive-time work. We think that %PSR must be regarded as a research instrument.

Clinical Outcome and Morphologic Analysis after Endovascular Aneurysm Repair Using the Excluder Endograft

OBJECTIVE: Long-term follow-up after endovascular aneurysm repair (EVAR) is very scarce, and doubt remains regarding the durability of these procedures. We designed a retrospective cohort study to assess long-term clinical outcome and morphologic changes in patients with abdominal aortic aneurysms (AAAs) treated by EVAR using the Excluder endoprosthesis (W. L. Gore and Associates, Flagstaff, Ariz). METHODS: From 2000 to 2007, 179 patients underwent EVAR in a tertiary institution. Clinical data were retrieved from a prospective database. All patients treated with the Excluder endoprosthesis were included. Computed tomography angiography (CTA) scans were retrospectively analyzed preoperatively, at 30 days, and at the last follow-up using dedicated tridimensional reconstruction software. For patients with complications, all remaining CTAs were also analyzed. The primary end point was clinical success. Secondary end points were freedom from reintervention, sac growth, types I and III endoleak, migration, conversion to open repair, and AAA-related death or rupture. Neck dilatation, renal function, and overall survival were also analyzed. RESULTS: Included were 144 patients (88.2% men; mean age, 71.6 years). Aneurysms were ruptured in 4.9%. American Society of Anesthesiologists classification was III/IV in 61.8%. No patients were lost during a median follow-up of 5.0 years (interquartile range, 3.1-6.4; maximum, 11.2 years). Two patients died of medical complications ≤ 30 days after EVAR. The estimated primary clinical success rates at 5 and 10 years were 63.5% and 41.1%, and secondary clinical success rates were 78.3% and 58.3%, respectively. Sac growth was observed in 37 of 142 patients (26.1%). Cox regression showed type I endoleak during follow-up (hazard ratio, 3.74; P = .008), original design model (hazard ratio, 3.85; P = .001), and preoperative neck diameter (1.27 per mm increase, P = .006) were determinants of sac growth. Secondary interventions were required in 32 patients (22.5%). The estimated 10-year rate of AAA-related death or rupture was 2.1%. Overall life expectancy after AAA repair was 6.8 years. CONCLUSIONS: EVAR using the Excluder endoprosthesis provides a safe and lasting treatment for AAA, despite the need for maintained surveillance and secondary interventions. At up to 11 years, the risk of AAA-related death or postimplantation rupture is remarkably low. The incidences of postimplantation sac growth and secondary intervention were greatly reduced after the introduction of the low-permeability design in 2004.

Pilosidade Púbica na Criança e Hiperplasia Congénita Supra-Renal Não Clássica

Antes dos 8 anos de idade no sexo feminino e dos 9 no masculino, o aparecimento de pelos púbicos, pode ser a primeira manifestação de doença das gónadas ou das supra-renais. Para reconhecimento da Hiperplasia congénita supra-renal de início tardio, em situações de Pubarca prematura, realizámos a Prova curta de estimulação da supra-renal com Synacthen, em trinta crianças. Cinco meninas e um rapaz revelaram um défice de 21-hidroxilase, demonstrado pelo valores da 17-OH Progesterona sérica aos 60 minutos;28,8 +_ 15,2ng/ml, sendo num grupo de controle 2,30 ng/ml (p<0,01). A incidência de 20% encontrada, justifica a realização desta prova em todas as Pubarcas prematuras.

Diagnóstico Pré-Natal de Uropatia: Importância do Desenvolvimento Embriológico Renal

As uropatias malformativas constituem a principalcausa de anomalia neonatal nao letal. Estas anomalias do desenvolvimento devem ser interpretadas com base no conhecimento da morbilidade e, em alguns casos, a mortalidade associada a este tipo de patologia 1,2. As malformações congénitas devem ser pensadas com base no conhecimento da dinâmica do normal desenvolvimento embrionário e dos múltiplos factores reguladores intervenientes, so assim será possivel estabelecer estratégias eficazes de investigação e terapêutica.