PURPOSE Adrenocortical carcinoma (ACC) is a rare aggressive pediatric malignancy with distinct biology. Its treatment follows the principles developed for adults; pediatric-specific studies are scarce. PATIENTS AND METHODS Prospective single-arm risk-stratified interventional study. Study objectives were (1) to describe the outcome of patients with stage I ACC treated with adrenalectomy alone; (2) to describe the outcome of stage II patients (completely resected > 200 cc or > 100 g) treated with adrenalectomy and retroperitoneal lymph node dissection; and (3) to describe the outcome of patients with stage III or IV treated with mitotane and chemotherapy. RESULTS Between September 2006 and May 2013, 78 patients (77 eligible, 51 females) were enrolled. The 5-year event-free survival estimates for stages I (24 patients), II (15 patients), III (24 patients), and IV (14 patients) were 86.2%, 53.3%, 81%, and 7.1%, respectively. The corresponding 5-year overall survival estimates were 95.2%, 78.8%, 94.7%, and 15.6%, respectively. On univariate analysis, age, stage, presence of virilization, Cushing syndrome, or hypertension, germline TP53 status, and presence of a somatic ATRX mutation were associated with outcome. On multivariable analysis, only stage and age were significantly associated with outcome. The probabilities of mitotane and chemotherapy feasibility events were 10.5% and 31.6%, respectively. CONCLUSION Outcome for children with stage I ACC is excellent with surgery. Outcome for patients with stage II disease is inferior despite retroperitoneal lymph node dissection. Patients with stage III ACC have an excellent outcome combining surgery and chemotherapy. Patients with stage IV ACC are older and have a poor outcome; new treatments should be explored for this high-risk group. The combination of mitotane and chemotherapy as prescribed in ARAR0332 resulted in significant toxicity; one third of patients with advanced disease could not complete the scheduled treatment.
JUSTIFICATIVAS E OBJETIVOS: A dor sempre foi uma das maiores preocupações do homem, entretanto, apesar dos progressos da ciência, ainda existem várias barreiras ao seu adequado tratamento, incluindo a falta de conhecimento por parte da equipe médica, sobre o mecanismo das diversas drogas e técnicas empregadas. O objetivo deste trabalho é abordar as principais drogas e técnicas empregadas no controle da dor pós-operatória, visando estimular o interesse sobre o assunto bem como aumentar a eficácia do tratamento dado aos pacientes. CONTEÚDO: Está ressaltada neste artigo, a importância da adequada analgesia pós-operatória, considerando as principais drogas e técnicas utilizadas no controle da dor, seus mecanismos de ação, posologias, vias de administração e efeitos colaterais, bem como a importância da integração de toda a equipe envolvida nos cuidados do paciente para o sucesso do tratamento. O tratamento inadequado da dor no pós-operatório não se justifica, pois há um arsenal considerável de drogas e técnicas analgésicas. O que se faz necessário, portanto, é que toda equipe, anestesistas, cirurgiões, e enfermeiros tenham conhecimento e estejam integrados na utilização deste arsenal.
Objective: To design and validate a surgical checklist to improve patient safety and prevent surgical site infection. Methods: This quantitative study was carried out to validate an instrument created and used for surgical safety. Seven experts validated the instrument. For agreement among experts, was used Kendall's concordance coefficient; if their opinions differed significantly, the Cochran's test was adopted. An instrument is validated when concordance among experts is achieved and its clarity is significant. Results: In the first assessment of the instrument, Kendall's concordance coefficients were 0.230 in terms of pertinence and 0.390 for clarity. These results cauded a reformulation in the checklist. After reformulation, an absolute concordance was achieved for pertinence and no significant difference was seen in terms of clarity. After instrument validation, was created an information system to input data collected. Conclusion: The instrument was validated. It can help improve patient safety and prevent surgical site infection. ResumoObjetivo: Construir e validar checklist cirúrgico para segurança do paciente e prevenção de infecção de sítio cirúrgico. Métodos: Pesquisa quantitativa realizada para validar instrumento criado e utilizado em cirurgia segura. O instrumento foi validado por sete peritos. Para concordância entre os juízes utilizou-se o coeficiente de concordância de Kendall e para verificar se a opinião dos juízes diferiu significativamente, o teste de Cochran. O instrumento é validado se houver concordância entre os juízes e a clareza for significante. Resultados: Na primeira avaliação do instrumento, obteve-se Kendall de 0,230 para pertinência e 0,390 para clareza, o que implicou em reformulação do checklist. Após a reformulação, obteve-se concordância absoluta para pertinência e não houve diferença significativa para clareza. Com o instrumento validado, foi criado um sistema informatizado para inserção dos dados coletados. Conclusão: O instrumento criado foi validado e pode auxiliar na segurança do paciente e prevenção de infecção de sítio cirúrgico.
OBJECTIVE:Here, we describe our experience with different therapeutic modalities used to treat cystic lymphangiomas in children in our hospital, including single therapy with OK-432, bleomycin and surgery, and a combination of the three modalities.METHODS:We performed a retrospective, cross-sectional study including patients treated from 1998 to 2011. The effects on macrocystic lymphangiomas and adverse reactions were evaluated. Twenty-nine children with cystic lymphangiomas without any previous treatment were included. Under general anesthesia, patients given sclerosing agents underwent puncture of the lesion (guided by ultrasound when necessary) and complete aspiration of the intralesional liquid. The patients were evaluated with ultrasound and clinical examinations for a maximum follow-up time of 4 years.RESULTS:The proportions of patients considered cured after the first therapeutic approach were 44% in the surgery group, 29% in the bleomycin group and 31% in the OK-432 group. These proportions were not significantly different. Sequential treatment increased the rates of curative results to 71%, 74% and 44%, respectively, after the final treatment, which in our case was approximately 1.5 applications per patient.CONCLUSION:The results of this study indicate that most patients with cystic lymphangiomas do not show complete resolution after the initial therapy, regardless of whether the therapy is surgical or involves the use of sclerosing agents. To achieve complete resolution of the lesions, either multiple operations or a combination of surgery and sclerotherapy must be used and should be tailored to the characteristics of each patient.
APM avoided unnecessary surgery in one third of the cases and was important to treatment decision in 100 %. We believe that this simple test is an excellent diagnostic tool when selectively applied mainly in the presence of functional deficit.
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