32 research outputs found

    The Accuracy Of (99m)tc-dtpa Scintigraphy In The Evaluation Of Acute Renal Graft Complications.

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    Renal scintigraphy has been used for many years in the evaluation of renal transplants and can help in the diagnosis of graft complications, leading to prompt clinical management and preventing further deterioration of renal function. The purpose of this study was to evaluate the overall accuracy of renal scintigraphy with (99m)Tc-DTPA in the diagnosis of acute renal graft complications. Seventy-six scintigraphic studies performed in 55 patients (ages ranging from 6 to 65 years), were reviewed. Scintigraphy results were compared to biopsies performed within 5 days of imaging. (99m)Tc-DTPA study was performed within a mean time of 19 days after kidney transplants. Dynamic images were performed in the anterior position of the abdomen and pelvis every 2 seconds for 80 seconds (flow phase) and every 15 seconds for 30 minutes (functional phase), after an intravenous injection of 370 MBq (10 mCi) of (99m)Tc-DTPA. The scintigraphic results were concordant with the biopsies in 86% of the cases studied. The sensitivities of renal scintigraphy for detection of acute tubular necrosis (ATN), acute rejection (AR) and cortical necrosis (CN) were 98%, 87% and 100%, respectively. Specificities and accuracies for detection of ATN, AR and CN were 89%, 86% and 100%, and 95%, 87% and 100%, respectively. Renal scintigraphy with (99m)Tc-DTPA showed a good overall accuracy in the detection of acute renal graft complications. It can be used as a reliable tool in the routine evaluation of these patients.29507-1

    Bioinorganic Chemistry of Alzheimer’s Disease

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    Several Opportunistic Infections In A Young Patient With Chronic Lymphocytic Leukemia Treated With Cladribine [múltiplas Infecções Oportunistas Em Um Paciente Com Leucemia Linfocítica Crônica Tratado Com Cladribina]

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    Hypogamaglobulinemia is frequently associated with B-CLL rendering the patients prone to bacterial infections. The use of purine analogs that cause depletion of T lymphocytes in the treatment of this disease, increases the spectrum of susceptibility of these patients to oportunistic agents. We report a case of a young B-CLL patient treated with 2-CdA, who presented multiple infectious complications during the course of his disease associated with a severe cellular immune deficiency.223420423Lorand-Metze, I., Leucemias linfoides crônicas Diagnóstico, estadiamento, fatores prognósticos e tratamento (1999) Ser. Monogr. Esc. Bras. Hematol., 6, pp. 18-22Keating, M.J., (1999) Translational Research in Chronic Lymphocytic Leukemia, pp. 249-254. , American Society of HematologyCheson, B.D., Infectious and immunossupressive complications of purine analog therapy (1995) J Clin Oncol, 12 (9), pp. 2431-2448O'Brien, S., (1999) Infectious Complications of Nucleoside Analogs, pp. 536-542. , American Society of HematologyMauro, F.R., Foa, R., Giannarelli, D., Cordone, I., Clinical characteristics and outcome of young chronic lymphocytic leukemia patients: A single institution study of 204 cases (1999) Blood, 94 (2), pp. 448-454Ikpeazu, E.V., Kaplon, M., Cryptococcal meningitis occurring at 19 months after cladribine therapy for hairy cell leukemia (1998) Eur J Haematol, 61 (4), pp. 286-287Van Den Neste, E., Delannoy, A., Vandercam, B., Infectious complications after 2-chlorodeoxyadenosine therapy (1996) Eur J Haematol, 56, pp. 235-240Broady, R., Roberts, S., Hawkins, T., Mycobacterium avium-intracellulare complex infection following 2-cholodeoxyadenosine therapy for hairy cell leukemia (2000) Leuk Lymphoma, 36, pp. 639-642Montserrat, E., Lopez-Lorenzo, J.L., Manso, F., Fludarabine in resistant or relapsing B-cell chronic lymphocitic leukemia. The spanish group experience (1996) Leuk Lymphoma, 21, p. 46

    Osteolytic Lesions As A Presenting Sign Of Acute Myeloid Leukemia.

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    Osteolytic lesions rarely occur in acute myeloid leukemia (AML). We reported an atypical form of the disease, with marrow fibrosis and osteolytic lesions, in a 17-year-old patient, whose main symptom was lumbar pain. Diagnosis of AML was established by bone marrow and lymph node histological analysis. Computed tomography (CT) scan and 99mTc-MDP bone scintyscan revealed osteolytic lesions. After remission-induction, bone marrow aspirate and biopsy showed no evidence of leukemic infiltration, nevertheless bone abnormalities persisted on 99mTc-MDP bone scintyscan, suggesting residual disease. Suspect bone areas were irradiated with symptomatic improvement and 99mTc-MDP bone scintyscan showed the appearance of more condensed bone compared with the pre-radiotherapy pattern. Twelve months later he was readmitted to the hospital due to relapse of AML and died of sepsis within a few weeks. This report illustrates the usefulness of histological studies to establish diagnosis of AML in atypical cases, as well as the importance of CT scan and bone scintigraphy scan for the identification of osteolytic lesions. It also provides additional data as evidence that although osteolytic lesions indicate an adverse prognosis in AML, local irradiation results symptomatic relief.30325-3

    Adrenocortical tumors associated with the TP53 p.R337H germline mutation can be identified during child-care consultations

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    Objective: To evaluate the clinical features associated with adrenocortical hormone overexpression and familial cancer profiling as potential markers for early detection of adrenocortical tumors in children from South and Southeast Brazil. Methods: The clinical manifestations and anthropometric measurements of 103 children diagnosed with adrenocortical tumors were analyzed. Results: Between 1982 and 2011, 69 girls and 34 boys diagnosed with adrenocortical tumors were followed-up for a median time of 9.0 years (0–34 years). Signs of androgen overproduction alone (n = 75) or associated with cortisol (n = 18) were present in 90.3%. TP53 p.R337H mutation was found in 90.5% of patients. Stages I, II, III, and IV were observed in 45.6%, 27.2%, 19.4%, and 7.8% of patients, respectively. At diagnosis, there were no significant differences in height (p = 0.92) and weight (p = 0.22) among children with adrenocortical tumors, but children with virilization alone had significantly higher height-for-age Z-scores (0.92 ± 1.4) than children with hypercortisolism alone or combined (−0.32 ± 1,8; p = 0.03). The five-year overall survival was 76.7% (SD ± 4.2). Patients with advanced-stage disease had a significantly worse prognosis than those with limited disease (p < 0.001). During follow-up, ten of 55 p.R337H carrier parents developed cancer, whereas none of the 55 non-carriers did. Conclusions: Signs of adrenocortical hormone overproduction appear early, even in cases with early-stage. These signs can be identified at the physical examination and anthropometric measurements. In southern Brazil, pediatric adrenocortical tumor is a sentinel cancer for detecting families with germline p.R337H mutation in TP53 gene. Resumo: Objetivo: Avaliar as manifestações clínicas da hiperexpressão de hormônios do córtex da adrenal e câncer familiar como marcadores para a detecção precoce de tumores adrenocorticais em crianças do Sul e Sudeste do Brasil. Pacientes e métodos: Foram analisadas as manifestações clínicas e antropométricas de 103 crianças diagnosticadas com tumores adrenocorticais. Resultados: Entre 1982 e 2011, 69 meninas e 34 meninos diagnosticados com tumores adrenocorticais foram acompanhados por um tempo mediano de nove anos (0-34). Ao diagnóstico, sinais de virilização isolada (n = 75) ou associada ao cortisol (n = 18) estavam presentes em 90,3% dos pacientes; a mutação do gene TP53 p.R337H foi identificada em 90,5% dos pacientes. Os pacientes foram classificados em estádio I (45,6%), II (27,2%), III (19,4%) e IV (7,8%). Ao diagnóstico, não houve diferença significativa para as medidas de altura (p = 0,92) e de peso (p = 0,22) entre as crianças com tumores adrenocorticais, mas crianças com virilização tiveram escore-Z mais elevado para a idade (0,92 ± 1,4) do que aquelas com hipercortisolismo isolado ou combinado (−0,32 ± 1,8; p = 0,03). A sobrevida global de cinco anos foi de 76,7% (DP ± 4,2). Pacientes com estádios avançados tiveram pior prognóstico (p < 0,001). Durante o seguimento, 10 dos 55 genitores portadores da p.R337H desenvolveram câncer, enquanto que nenhum caso ocorreu entre os 55 não portadores. Conclusões: Os sinais de hiperprodução de hormônios adrenocorticais aparecem precocemente no desenvolvimento do tumor e podem ser identificados pelo exame físico e medidas antropométricas na consulta pediátrica de rotina. O tumor adrenocortical pediátrico é sentinela para a detecção de câncer em famílias que segregam a mutação germinativa p.R337H do gene TP53. Keywords: Adrenocortical tumor, TP53 p.R337H, Germinal mutation, Early diagnosis, Corticosteroid, Growth, Palavras-chave: Tumor adrenocortical, TP53 p.R337H, Mutação germinativa, Diagnóstico precoce, Corticosteroide, Cresciment

    Spindle Assembly Checkpoint Gene Expression in Childhood Adrenocortical Tumors (ACT): Overexpression of Aurora Kinases A and B Is Associated With a Poor Prognosis

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    Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)BackgroundPediatric adrenocortical tumors (ACT) are rare malignancies and treatment has a small impact on survival in advanced disease and the discovery of potential target genes could be important in new therapeutic approaches. MethodsThe mRNA expression levels of spindle checkpoint genes AURKA, AURKB, BUB, and BUBR1 were analyzed in 60 children with ACT by quantitative real time PCR. The anticancer effect of ZM447439, an experimental AURK inhibitor, was analyzed in a primary childhood ACT culture carrying the TP53 p.R337H mutation. ResultsA significant association was observed between malignancy as defined by Weiss score 3 and higher AURKA (2.0-fold, P=0.01), AURKB (7.0-fold, P=0.007), and BUBR1 (5.8-fold, P=0.007) gene expression, and between unfavorable event (death or relapse) and higher expression of AURKA (6.0-fold, P=0.034) and AURKB (17-fold, P=0.013). Overexpression of AURKA and AURKB was associated with lower event-free survival in uni- (P<0.001 and P=0.006, respectively) and multivariate (P=0.002 and P=0.03, respectively) analysis. Significant lower Event free survival (EFS) was also observed in patients with moderate/strong immunostaining to AURKA (P=0.012) and AURKB (P=0.045). ZM447439 was able to induce inhibition of proliferation and colony formation in a primary childhood ACT culture carrying the TP53 p.R337H mutation. ConclusionOur results suggest that AURKA and AURKB overexpression in pediatric ACT may be related to more aggressive disease and the inhibition of these proteins could be an interesting approach for the treatment of these tumors. Pediatr Blood Cancer 2013;60:1809-1816. (c) 2013 Wiley Periodicals, Inc.601118091816Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)FAPESP [2010/7020-9, 2010/08699-5
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