ABSTRACT Purpose: To evaluate the role of ARDT after surgical resection of ACC. Materials and Methods: Records of patients from our institutional ACC database were retrospectively assessed. A paired comparison analysis was used to evaluate the oncological outcomes between patients treated with surgery followed by ARDT or surgery only (control). The endpoints were LRFS, RFS, and OS. A systematic review of the literature and meta-analysis was also performed to evaluate local recurrence of ACC when ARDT was used. Results: Ten patients were included in each Group. The median follow-up times were 32 months and 35 months for the ARDT and control Groups, respectively. The results for LRFS (p=0.11), RFS (p=0.92), and OS (p=0.47) were similar among subsets. The mean time to present with local recurrence was significantly longer in the ARDT group compared with the control Group (419±206 days vs. 181±86 days, respectively; p=0.03). ARDT was well tolerated by the patients; there were no reports of late toxicity. The meta-analysis, which included four retrospective series, revealed that ARDT had a protective effect on LRFS (HR=0.4; CI=0.17-0.94). Conclusions: ARDT may reduce the chance and prolong the time to ACC local recurrence. However, there were no benefits for disease recurrence control or overall survival for patients who underwent this complementary therapy.

A doença falciforme caracteriza-se por uma alteração genética que resulta na produção da hemoglobina S. A triagem neonatal para a HbS é de fundamental importância para o diagnóstico da anemia falciforme, possibilitando a instituição de tratamento preventivo, no sentido de diminuir a morbidade e mortalidade na infância. Embora o diagnóstico precoce, através do "Teste do Pezinho", esteja ocorrendo praticamente em todo o país, que medidas estão sendo tomadas após a confirmação do diagnóstico? Realizamos um estudo transversal e retrospectivo em 22.785 (96,97%) de 23.497 nascidos vivos que foram submetidos ao exame de triagem neonatal. Todos os exames foram realizados no primeiro mês de vida. Foram diagnosticados quatro recém-nascidos com o padrão HbSS e dois HbSC. A primeira avaliação especializada ocorreu de 2 a 9 meses após o diagnóstico. Em apenas um caso houve a administração adequada das imunizações e da antibioticoterapia profilática. O tempo de realização do teste de triagem e a chegada dos resultados na Unidade coletora da amostra estão geralmente adequados. Levando-se em consideração a necessidade da instituição de um tratamento profilático em um centro especializado, é fundamental elaborar um fluxo que melhor articule todos os atores envolvidos, visando amenizar as prováveis consequências e sequelas desta doença.

Sickle disease is characterized by a genetic alteration which produces hemoglobin S. Neonatal screening for HbS has a major importance in the diagnosis of sickle anemia, thereby enabling preventive treatment in order to reduce childhood morbidity and mortality. Although early diagnosis by neonatal screening is being performed in practically the entire country, what measures are being taken after diagnostic confirmation? We carried out a cross-sectional retrospective study of 22,785 (96.97%) of 23,595 live births submitted to neonatal screening. All tests were performed within the first month of life. Four newborns with HbSS and two with HbSC were diagnosed. The first specialized evaluation occurred between 2 and 9 months after the disease had been identified. Only one case received correct immunization and prophylactic antibiotic therapy. The time between the screening test and the samples arriving at the collecting units was appropriate. Taking into account the necessity of establishing prophylactic treatment at specialized centers, effective communication between healthcare services is essential to safeguard against possible consequences and sequelae related to the disease.