ABSTRACT Introduction: Minimal residual disease is an important independent prognostic factor that can identify poor responders among patients with acute lymphoblastic leukemia. Objective: The aim of this study was to analyze minimal residual disease using immunoglobulin (Ig) and T-cell receptor (TCR) gene rearrangements by conventional polymerase chain reaction followed by homo-heteroduplex analysis and to compare this with real-time polymerase chain reaction at the end of the induction period in children with acute lymphoblastic leukemia. Methods: Seventy-four patients diagnosed with acute lymphoblastic leukemia were enrolled. Minimal residual disease was evaluated by qualitative polymerase chain reaction in 57 and by both tests in 44. The Kaplan-Meier and multivariate Cox methods and the log-rank test were used for statistical analysis. Results: Nine patients (15.8%) were positive for minimal residual disease by qualitative polymerase chain reaction and 11 (25%) by real-time polymerase chain reaction considering a cut-off point of 1 × 10−3 for precursor B-cell acute lymphoblastic leukemia and 1 × 10−2 for T-cell acute lymphoblastic leukemia. Using the qualitative method, the 3.5-year leukemia- free survival was significantly higher in children negative for minimal residual disease compared to those with positive results (84.1% ± 5.6% versus 41.7% ± 17.3%, respectively; p-value = 0.004). There was no significant association between leukemia-free survival and minimal residual disease by real-time polymerase chain reaction. Minimal residual disease by qualitative polymerase chain reaction was the only variable significantly correlated to leukemia-free survival. Conclusion: Given the difficulties in the implementation of minimal residual disease monitoring by real-time polymerase chain reaction in most treatment centers in Brazil, the qualitative polymerase chain reaction strategy may be a cost-effective alternative.

Acredita-se que haja uma associação entre síndrome da apneia obstrutiva do sono (SAOS) e refluxo laringofaríngeo (RLF). OBJETIVO: Estudar a prevalência de sintomas/sinais de refluxo em pacientes roncadores com suspeita de SAOS. MÉTODO: Estudo clínico corte transversal de 74 adultos Berlim positivo acompanhados em ambulatório de distúrbios do sono de centro universitário. As variáveis estudadas foram IMC, Epworth, índice de sintomas de refluxo (RSI) e escala de achados endolaríngeos de refluxo (RFS), bem como seus subdomínios que podem traduzir maior inflamação. Correlações entre questionários de sono e escalas de refluxo, bem como seus subdomínios, foram feitas com significância estabelecida em p < 0,05. RESULTADOS: Noventa e oito porcento dos pacientes apresentaram sintomas e sinais sugestivos de RLF, sendo esta prevalência significativamente maior nos obesos (p = 0,002). CONCLUSÃO: A diferença significativa nos sinais inflamatórios sugestivos de RLF entre obesos e não obesos com suspeita de SAOS sugere que a obesidade pode interferir nos achados inflamatórios na faringe e laringe. A altíssima prevalência destes sintomas e sinais de refluxo nos pacientes com suspeita de SAOS demonstra a importância de aprofundar estudos nesta área.

OBJECTIVE: To study the prevalence of signs and symptoms of reflux in snorers with suspected OSA. METHOD: This cross-sectional study enrolled 74 patients assessed positive for OSA with the Berlin questionnaire. The subjects were followed up at the sleep disorder ward of a university center. Studied variables included the BMI, the Epworth sleepiness scale, the reflux symptom index (RSI), the reflux finding score (RFS), and their subdomains related to increased inflammation. The correlations between sleep questionnaires, reflux scales, and their subdomains were deemed statistically significant when p < 0.05. RESULTS: Ninety-eight percent of the subjects had symptoms and signs suggestive of LPR; prevalence was significantly higher among obese individuals (p = 0.002). CONCLUSION: The significant difference seen in the prevalence of signs of inflammation suggestive of LPR when obese and non-obese subjects with suspected OSA were compared indicates that obesity may affect inflammatory findings of the pharynx and larynx. The high prevalence of symptoms and signs of reflux in patients with suspected OSA calls for more studies on the matter.

Em 1938, o médico português Fernando Bissaya Barreto promoveu a construção de um 'internato de leprosos' no centro de Portugal, afastado dos grandes centros populacionais, mas de fácil acesso a todo o país. Inaugurado em 1947, o Hospital-Colônia Rovisco Pais segue o modelo de um hospital-asilo-colônia, sendo simetricamente segmentado por pavilhões iguais em número e características para os dois sexos. Seguindo uma lógica disciplinar e não de exclusão, o plano urbanístico, o desenho dos edifícios e o próprio mobiliário e decoração são configurados, sob a influência direta de Bissaya Barreto, como instrumentos de intervenção sobre o corpo físico e moral dos doentes de Hansen, mas também, e em outra escala, de controle e modificação da sociedade portuguesa no seu conjunto.

In 1938, Portuguese physician Fernando Bissaya Barreto spearheaded the creation of a 'nursing home for lepers' in the center of Portugal, away from big towns and cities, but still accessible from any part of the country. Opened in 1947, Hospital-Colônia Rovisco Pais followed the model of a colony/hospital/hospice, and was divided symmetrically into buildings of equal features and numbers for both sexes. According to a disciplinary, non-exclusionary rationale, the urban design, building design and furniture and fittings were conceived, under the direct influence of Bissaya Barreto, as instruments for intervention in the physical and moral bodies of the patients, and also, on a different scale, for the control and modification of Portuguese society as a whole.

OBJECTIVE To detect markers for minimal residual disease monitoring based on conventional polymerase chain reaction for immunoglobulin, T-cell receptor rearrangements and the Sil-Tal1 deletion in patients with acute lymphocytic leukemia. METHODS Fifty-nine children with acute lymphocytic leukemia from three institutions in Minas Gerais, Brazil, were prospectively studied. Clonal rearrangements were detected by polymerase chain reaction followed by homo/heteroduplex clonality analysis in DNA samples from diagnostic bone marrow. Follow-up samples were collected on Days 14 and 28-35 of the induction phase. The Kaplan-Meier and multivariate Cox methods were used for survival analysis. RESULTS Immunoglobulin/T-cell receptor rearrangements were not detected in 5/55 children screened (9.0%). For precursor-B acute lymphocytic leukemia, the most frequent rearrangement was IgH (72.7%), then TCRG (61.4%), and TCRD and IgK (47.7%); for T-acute lymphocytic leukemia, TCRG (80.0%), and TCRD and Sil-Tal deletion (20.0%) were the most common. Minimal residual disease was detected in 35% of the cases on Day 14 and in 22.5% on Day 28-35. Minimal residual disease on Day 28-35, T-acute lymphocytic leukemia, and leukocyte count above 50 x 109/L at diagnosis were bad prognostic factors for leukemia-free survival in univariate analysis. Relapse risk for minimal residual disease positive relative to minimal residual disease negative children was 8.5 times higher (95% confidence interval: 1.02-70.7). CONCLUSION Immunoglobulin/T-cell receptor rearrangement frequencies were similar to those reported before. Minimal residual disease is an independent prognostic factor for leukemia-free survival, even when based on a non-quantitative technique, but longer follow-ups are needed.

Chronic myeloproliferative diseases without the Philadelphia chromosome marker (Ph-), although first described 60 years ago, only became the subject of interest after the turn of the millennium. In 2001, the World Health Organization (WHO) defined the classification of this group of diseases and in 2008 they were renamed myeloproliferative neoplasms based on morphological, cytogenetic and molecular features. In 2005, the identification of a recurrent molecular abnormality characterized by a gain of function with a mutation in the gene encoding Janus kinase 2 (JAK2) paved the way for greater knowledge of the pathophysiology of myeloproliferative neoplasms. The JAK2 mutation is found in 90-98% of polycythemia vera and in about 50% essential thrombocytosis and primary myelofibrosis. In addition to the JAK2 mutation, other mutations involving TET2 (ten-eleven translocation), LNK (a membrane-bound adaptor protein); IDH1/2 (isocitrate dehydrogenase 1/2 enzyme); ASXL1 (additional sex combs-like 1) genes were found in myeloproliferative neoplasms thus showing the importance of identifying molecular genetic alterations to confirm diagnosis, guide treatment and improve our understanding of the biology of these diseases. Currently, polycythemia vera, essential thrombocytosis, myelofibrosis, chronic neutrophilic leukemia, chronic eosinophilic leukemia and mastocytosis are included in this group of myeloproliferative neoplasms, but are considered different situations with individualized diagnostic methods and treatment. This review updates pathogenic aspects, molecular genetic alterations, the fundamental criteria for diagnosis and the best approach for each of these entities.

A infecção do papilomavírus humano (HPV) é uma das mais freqüentes doenças sexualmente transmissíveis em todo o mundo. A relação entre o HPV genital e oral permanece incerta, assim como o seu papel na carcinogênese oral. O objetivo deste estudo foi verificar a presença do DNA do HPV na mucosa oral e genital de mulheres com infecção genital por HPV, pela técnica de reação em cadeia de polimerase (PCR). FORMA DE ESTUDO: Coorte transversal. MATERIAL E MÉTODO: Trata-se de um estudo piloto, prospectivo, com 30 mulheres, idade de 14 a 51 anos, portadoras de infecção genital por HPV confirmada pelo exame de histopatológico. Todas as pacientes foram submetidas a exame e coleta por raspagem da cavidade oral e genital para pesquisa do DNA do HPV pela técnica PCR. RESULTADOS: Nenhuma das amostras da cavidade oral foi positiva para HPV, enquanto no genital, o HPV foi detectado em 17 (57%) das 30 pacientes, principalmente o HPV 6b e 16. CONCLUSÃO: Os resultados mostraram maior porcentagem do HPV genital em relação à cavidade oral, e sugerem que o HPV genital não parece ser fator predisponente para a infecção oral no mesmo paciente.

Infection by the Human Papilloma Virus (HPV) is one of the most frequent sexually transmitted diseases all over the world. The relationship between oral and genital HPV remains uncertain, as it is with its role on oral carcinogenesis. The goal of the present investigation was to check for the presence of HPV DNA in the oral and genital mucosas of women with HPV genital infection, using the polymerase chain reaction (PCR). STUDY METHOD: Cross-sectional cohort. MATERIALS AND METHODS: this is a pilot and prospective study involving 30 women, aged between 14 and 51 years, with HPV genital infection, confirmed by histopathology. All the patients were submitted to the exam and sample collection by swabbing the oral and genital mucosas in order to test for HPV DNA through the PCR technique. RESULTS: none of the oral cavity samples were positive for HPV, while in the genital tract, HPV was detected in 17 (57%) of the 30 patients, especially HPVs 6b and 16. CONCLUSION: Results show a higher percentage of genital HPV in comparison to the oral cavity, and suggest that genital HPV does not seem to be a predisposing factor for the oral infection in the same patient.

Procurarei pensar os projectos e o processo de trabalho do arquitecto paisagista João Gomes da Silva através do conceito de objectificação da cultura de Richard Handler. João Gomes da Silva segue a escola de arquitectura paisagista fundada por Francisco Caldeira Cabral e inclui frequentemente tipologias rurais, como elementos representativos da identidade do lugar, em jardins públicos e privados, em espaços públicos e urbanos. Influenciado pelo arquitecto Álvaro Siza Vieira e, através dele, pelo Inquérito à Arquitectura Popular em Portugal, também dedica particular atenção aos modos populares de construção da paisagem. Estes surgem nos seus projectos como modos de construção que marcam a cultura e a identidade de um território sendo, como tal, esteticamente valorizados.

In this article, I will focus on the landscape architecture projects of João Gomes da Silva using Richard Handler’s concept of cultural objectification. João Gomes da Silva is a follower of the landscape architecture school of Francisco Caldeira Cabral and uses rural typologies as elements representing the identity of the place in public and private gardens, and in public and urban spaces. He is also influenced by the architect Álvaro Siza Vieira and by the Inquérito à Arquitectura Popular em Portugal, paying particular attention to the folk modes of constructing the landscape. In his projects, these folk modes are shown as representative elements of the culture of the place and they are aesthetically valued as such.

O papilomavírus humano (HPV) é universalmente aceito como agente causal do câncer de colo uterino e, recentemente, vem se especulando sobre sua possível relação com câncer oral e de orofaringe. O carcinoma espinocelular (CEC) oral representa 90% de todos os tumores malignos que afetam a cavidade bucal. Estudos sobre a prevalência de HPV em pacientes com CEC variam de 0 a 100%. O efeito citopático viral mais conhecido é a coilocitose, considerado "critério maior" na infecção pelo HPV do ponto de vista histopatológico. OBJETIVO: O objetivo deste estudo foi verificar a prevalência de achados sugestivos de HPV - coilocitose - em CEC oral e de orofaringe. FORMA DE ESTUDO: coorte transversal. MATERIAL E MÉTODO: Foram examinadas no microscópio 20 lâminas com o diagnóstico de CEC de cavidade oral ou orofaringe sendo que em 15 delas foi encontrada coilocitose, correspondendo a 75%. RESULTADO: Apesar de termos conhecimento que o método com maior sensibilidade atual para pesquisa de HPV ser a reação de polimerase em cadeia (PCR), iniciamos esta pesquisa com a investigação de coilocitose, o que é muito sugestivo de infecção por HPV. CONCLUSÃO: O estudo em questão trata-se de um projeto-piloto pois será dada continuidade a esta pesquisa através da realização de PCR a fim de confirmar a alta prevalência de infecção por HPV em CEC oral e de orofaringe.

Human papillomavirus (HPV) is considered to be an etiologic agent of cervical cancer and, recently its relation to oral and oropharyngeal cancer has also been investigated. Oral squamous cell carcinoma (SCC) represents 90% of all malignant tumors that affect the oral cavity. The prevalence of HPV in patients with SCC ranges from 0 to 100%. The most known viral cytopathic effect is koilocytosis, considered to be a major characteristic of HPV infection. AIM: The aim of this study was to verify the prevalence of some peculiar characteristics of HPV - koilocytosis - in oral and oropharyngeal SCC. STUDY DESIGN: transversal cohort. MATERIAL AND METHOD: Twenty slides with oral and/or oropharyngeal SCC were examined under microscopy. RESULTS: in 15 of them, koilocytosis was found, amounting to 75%. Although we know that polymerase chain reaction (PCR) is the method with the best sensitivity for HPV detection, we began this research looking for koilocytosis, which is highly suggestive of HPV infection. CONCLUSION: This study is a trial project and we will continue this research with PCR measures to confirm this high prevalence of HPV infection in oral and oropharyngeal SCC.

Amiloidose é caracterizada por deposição extracelular anormal de amilóide em diferentes tecidos e órgãos, geralmente associada com disfunção do tecido ou órgão envolvido, cuja causa não é ainda conhecida. Pode ser dividida em amiloidose sistêmica e localizada. A forma sistêmica é dividida em: (1) primária; (2) amiloidose associada ao mieloma múltiplo; (3) secundária; (4) amiloidose heredofamiliar. Há uma considerável diferença de sobrevida entre os pacientes com forma localizada e sistêmica de amiloidose, e também com mieloma múltiplo. Não há tratamento satisfatório para amiloidose sistêmica. O presente estudo relata um caso de amiloidose sistêmica primária, com revisão de literatura. O diagnóstico de amiloidose foi conseguido com biópsia de língua e o envolvimento sistêmico confirmado através de aspirado de gordura abdominal. Apesar de a amiloidose não ser a principal hipótese diagnóstica que deva ser aventada quando nos deparamos com um paciente com macroglossia, esta doença não pode ser esquecida, uma vez que o suporte clínico é essencial para o controle das doenças que podem estar associadas, como falência renal, cardíaca e o mieloma múltiplo.

Amyloidosis is characterized by the abnormal, extracelular deposition of one of a family of unrelated proteins, amyloid, in different tissues or organs; it is usually associated with tissue or organ disfunction. The cause is not yet known. Amyloidosis can be divided in systemic or localized form. The sistemic form can be divided in: (1) primary; (2) amyloidosis associated with multiple myeloma; (3) secondary; (4) heredofamilial amyloidosis. There is a considerable difference in the survival of patients with localized and systemic amyloidosis, and also with or without multiple myeloma. Satisfactory treatment for systemic amyloidosis does not exist. The present study was undertaken in order to report a case of primary sistemic amyloidosis as well as literature review. The amyloidosis diagnosis was achieved by tongue biopsy and its sistemic involvement by fat abdominal aspirate. Even amyloidosis should not be the first diagnostic hypothesis when macroglossia is present, this disease can not be forgotten since clinical attendance is essential to control the associated disorders as renal, cardiac failure and myeloma multiple.

As sinusites fúngicas são divididas em 2 grandes grupos: (1) Forma invasiva, que se divide em aguda e crônica; (2) Forma não-invasiva, que consta da bola fúngica (micetoma) e a sinusite fúngica alérgica. O desenvolvimento das diferentes formas de sinusite fúngica depende do estado imunológico do paciente, sendo que a forma invasiva aguda ocorre na grande maioria das vezes em imunodeprimidos. Os autores apresentam um caso de uma paciente do sexo feminino, diabética que iniciou quadro clínico com as características de sinusite fúngica invasiva, com febre alta, comprometimento ocular que evoluiu para amaurose à esquerda. Submetida à cirurgia, não apresentou boa evolução, com a manutenção de fístula nasocutânea. Após a introdução de medicação antifúngica (anfotericina B), seu quadro clínico estabilizou-se, com cessação da rinorréia fétida, febre e sinais flogísticos periorbitais. No exame anatomo-patológico foi encontrado mucosa revestida por epitélio respiratório com processo inflamatório tendo em meio hifas septadas com diagnóstico final de zigomicose. Após um ano de total desaparecimento dos sintomas, a paciente foi encaminhada ao Serviço de Cirurgia Plástica onde foi realizada cirurgia estética na região naso orbitária esquerda, estando a paciente atualmente satisfeita com aspecto facial.

Fungal sinusitis are divided in two big groups: (1) Invasive form that is subdivided in acute and chronic; (2) Noninvasive form that is subdivided in mycetoma form (fungal ball) and allergic form. The development of one of these forms depends on the immunologic relationship of the fungus to the host, as invasive form occurs in most of the cases in immunocompromised hosts. It is reported a diabetic female, with suspected fungi sinusitis with clinical invasive features at its very beginning displaying high fever and left eye involvement that lead to amaurosis. A left orbital surgical drainage followed by sinusectomy resulted in a nasocutaneous fistula, that stabilized when antifungal treatment was instituted, with no more rinorrhea, fever and periorbital inflammatory signs. As improvement progressed the nasocutaneous fistula was closed under general anesthesia. On histopatology, respiratory epithelium with inflammatory process and septated hyphae was found, that disclosed zygomycosis. After one year of no patient's symptoms, she started to be followed by facial surgeons who did a naso-orbital esthetics surgery with a high patient's satisfaction.