Burnout syndrome is frequent among Brazilian cancer physicians and further studies should be conducted to evaluate its prevalence and prevention among other sub specialists.

Physicians had a tendency to order excessive laboratory tests, an action which can be related to lack of knowledge and to divergence among guidelines. A more intensive educational effort regarding cancer prevention, directed towards teaching physicians in training, seems to be warranted.

CONTEXT AND OBJECTIVE: Intravenous amphotericin B deoxycholate (AmB-D) infusions, usually given over 4 hours, frequently induce nephrotoxicity and undesirable infusion-related side effects such as rigors and chills. There is evidence in the literature that the use of AmB-D in the form of continuous 24-hour infusion is less toxic than the usual four-hour infusion of this drug. Our objective was to evaluate the efficacy and safety of continuous infusion of AmB-D for the treatment of persistent fever in neutropenic patients with hematological malignancies after chemotherapy. DESIGN AND SETTING: Observational retrospective analysis of our experience with continuous infusion of AmB-D, at Faculdade de Medicina da Fundação ABC and Hospital Estadual Mário Covas in Santo André. METHODS: From October 2003 to May 2004, 12 patients with hematological malignancies and chemotherapy-induced neutropenia received 13 cycles of continuous infusion of AmB-D. RESULTS: The median dose of AmB-D was 0.84 mg/kg/day (0.33 to 2.30 mg/kg/day). Concomitant use of nephrotoxic medications occurred in 92% of the cycles. Nephrotoxicity occurred in 30.76% of the cycles, hypokalemia in 16.67%, hepatotoxicity in 30% and adverse infusion-related events in 23%. All patients survived for at least seven days after starting continuous infusion of AmB-D, and clinical resolution occurred in 76% of the cycles. CONCLUSIONS: Continuous infusion of AmB-D can be used in our Institution as an alternative to the more toxic four-hour infusion of AmB-D and possibly also as an alternative to the more expensive liposomal formulations of the drug.

Introduction: Intrinsic limitations of glucocorticoid therapy in patients with congenital adrenal hyperplasia (CAH) determine frequent loss in final height. The association of secondary central precocious puberty and early epiphyseal fusion is also frequent. In these conditions, GnRHa treatment alone or in combination with GH has been indicated. Objectives: This is a retrospective study, describing the estatural findings of CAH patients with significant decrease in height prediction, who were submitted to combined GH plus GnRHa therapy up to near-final height. Subjects and methods: We studied 13 patients, eight females and five males, eight with the classical and five with the nonclassical form of the disorder. Treatment with hydrocortisone (10-20 mg/m 2 /day) or prednisolone (3-6 mg/kg/day) was associated with GnRHa (3.75 mg/months) for 4.0 (1.5) years, and GH (0.05 mg/kg/day) for 3.6 (1.4) years. Results: Stature standard deviation score for bone age improved significantly after GH treatment, becoming similar to target height at the end of the second year of GH treatment. Conclusion: We conclude that combined GH plus GnRHa therapy can be useful in a subset of CAH patients with significant reduction of predicted final height associated with poor hormonal control and central precocious puberty.

RESUMO:A Síndrome de Li-Fraumeni é uma síndrome de predisposição familiar ao câncer, caracterizada pela presença de múltiplos tumores, tais como sarcomas, carcinomas de mama, tumores cerebrais e leucemia. O caso relatado é de uma paciente feminina de 37 anos, que apresenta uma significativa história familiar de câncer, bem como história pessoal de seis diferentes tumores primários (um de cólon, um nevus displásico, um de ovário e três de mama). O seqüenciamento do gene supressor de tumor p53 em seus linfócitos presentes no sangue periférico revelou uma mutação do aminoácido triptofano (TGG) para um códon de parada prematuro (TAG), no nucleotídeo 437 do códon 146 do exon 5 deste gene. As implicações clínicas, preventivas e éticas deste caso são também abordadas. DESCRITORES:Neoplasias primárias múltiplas/genética; Proteína P53/genética; Mutação/genética. INTRODUÇÃOO câncer é uma patologia de etiologia multifatorial, resultante principalmente de alterações genéticas, de fatores ambientais e do estilo de vida 7 . O descobrimento de genes relacionados ao câncer humano -oncogenes -permitiu uma maior compreensão das alterações genéticas associadas a transformações malignas das células 17,19 . Tais alterações afetam principalmente três classes de genes reguladores: os promotores do crescimento (proto-oncogenes), os inibidores do crescimento celular (genes supressores de tumor) e os genes que regulam o programa de morte celular (apoptose) 4 . De fato, a principal alteração genética encontrada no câncer humano é a mutação do gene supressor de tumor p53 5,10,13,,21 , presente em pouco mais de 50% dos tumores 3 . O gene p53 localiza-se no braço curto do cromossomo humano 1712 , e codifica a síntese de uma fosfoproteína nuclear expressa em níveis basais em todas as células normais 11 . A proteína p53 desacelera a progressão do ciclo celular e permite, portanto, que a célula tenha mais tempo para reparar o DNA antes de entrar na fase S ou mitose. Entretanto, quando a reparação dos danos do DNA não for eficiente, o p53 pode estimular a morte celular programada (apoptose) da célula. Estes efeitos contribuem para a proteção do organismo contra o acúmulo de

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This is the first overview on resistant and multidrug resistant isolates of Mycobacterium tuberculosis circulating in the French Department of the Americas (Guadeloupe, Martinique, and French Guiana) over 17 years (January 1995-December 2011). A total of 1,239 cases were studied: 1,199 new cases (primary and multidrug resistance of 11.8 and 1.6% respectively), and 40 persistent (defined as cases with a previous history of positive culture over 6 months interval and whose spoligotypes remain unchanged), in which significantly higher proportions of resistance to at least isoniazid (22.5%, P = 0.002), rifampicin (20.0%, P < 0.001), and multidrug resistance (17.5%, P < 0.001) were observed as compared to new cases. The 281 spoligotypes obtained showed the presence of five major lineages, T (29.9%), LAM (23.9%), Haarlem (22.1%), EAI (7.1%), and X (6.7%). Two of these lineages, X and LAM, predominate among resistant and multidrug resistant isolates respectively (X: 10.5% of resistant isolates, P = 0.04; LAM: 42.3% of multidrug resistant isolates, P = 0.02). Four of the 19 major spoligo-profiles, corresponding to SIT 20, 64, 45, and 46, were significantly associated with drug resistance. Among them, genotype SIT 20, associated with monoresistance to isoniazid and multidrug resistance, would be actively and persistently in circulation, since 1999, in French Guiana, department in which one may also observe the presence of strains of M. tuberculosis phylogeographically associated to Guiana and Suriname (SIT 131 and SIT 1340).