ABSTRACT Congenital and gestational syphilis are increasingly prevalent multisystemic infections in Brazil. This study aimed to present a case series of three children diagnosed with congenital syphilis even though their mother had unreactive treponemal tests. The VDRL (Venereal Disease Research Laboratory) titers of a 22-year-old mother with three pregnancies decreased after treatment. The mother did not have a reactive treponemal test, but all the three children were diagnosed with early congenital syphilis. This case series highlights the difficulty in diagnosing gestational and congenital syphilis in Brazil.

Resumo A sarcoidose é uma doença granulomatosa multissistémica de etiologia desconhecida. Os pulmões representam o local de envolvimento primário; contudo, esta patologia pode afetar virtualmente qualquer órgão, resultando numa grande variedade de manifestações clínicas. As anormalidades hematológicas são incomuns, e mais frequentemente incluem a linfopenia e anemia. A trombocitopenia, por outro lado, é rara, e três mecanismos fisiopatológicos distintos foram descritos: destruição imune das plaquetas, esplenomegalia com hiperesplenismo, e infiltração da medula óssea. Apresentamos um caso de sarcoidose com envolvimento pulmonar e cutâneo, complicado com trombocitopenia aguda grave. O mielograma foi compatível com um processo imune, que foi assumido como a causa primária para a citopenia; adicionalmente, a biópsia óssea mostrou também múltiplos granulomas não caseosos, enquanto uma tomografia computorizada revelou esplenomegalia maciça. Não existem recomendações para a abordagem da trombocitopenia na sarcoidose. No nosso doente, o trata-mento com imunoglobulina endovenosa e corticoides resultou na normalização da contagem de plaquetas.

Abstract Sarcoidosis is a multisystemic granulomatous disorder of unknown aetiology. Although the lungs are the primary site of involvement, the disease can affect virtually any organ, leading to a wide variety of clinical manifestations. Haematological abnormalities are uncommon, and most frequently include lymphopenia and anaemia. Thrombocytopenia, however, is rare, and three distinct pathophysiological mechanisms have been described: immune platelet destruction, splenomegaly with hypersplenism, and bone marrow infiltration. We report a case of sarcoidosis presenting with pulmonary and cutaneous involvement, and severe acute thrombocytopenia. Bone marrow aspirate was consistent with an immune process, assumed as the primary cause for the cytopenia, but bone marrow biopsy also showed multiple noncaseating granulomas, and a CT scan revealed massive splenomegaly. There are no guidelines for the management of thrombocytopenia in sarcoidosis. In our patient, treatment with intravenous immune globulin and corticosteroids restored normal platelet count.

Resumo O tumor fibroso solitário (TFS) é um tumor benigno raro de células fusiformes de natureza mesenquimatosa que geralmente ocorre na pleura, mas pode existir noutras localizações, nomeadamente na região da cabeça e pescoço, podendo envolver a órbita, a cavidade oral e os seios perinasais. Na literatura apenas existem três casos reportados de TFS do canal auditivo externo. Descreve-se o quarto caso e realiza-se uma revisão da literatura. Uma doente de 27 anos apresentou-se com uma neoformação na parede posterior do canal auditivo externo, condicionando hipoacusia de condução de grau ligeiro e episódios de otorreia. O estudo imagiológico revelou margens regulares e ausência de invasão dos tecidos adjacentes. Foi realizada a excisão cirúrgica completa da lesão. Histologicamente era composta por células fusiformes, com áreas de hipo e hipercelularidade, tendo a análise revelado positividade para CD34, vimentina, CD99, BCL2 e STAT-6. Aos seis meses de seguimento, não apresentava sinais de recidiva.

Abstract A solitary fibrous tumour (SFT) is a rare benign spindle-cell neoplasm of mesenchymal origin that commonly arises in the pleura but can occur in other locations such as head and neck, in particular in the orbit, oral cavity and paranasal sinuses. In the literature, there are only three reported cases of SFT in the external auditory canal. We present the fourth case and review the literature. A 27-year-old female presented with a neoplasm on the posterior wall of the external auditory canal with mild conductive hearing loss and ear discharge episodes. Imaging studies revealed regular margins and no invasion of the surrounding tissues. A complete surgical excision was made. Histologically, the mass comprised spindle cells with areas of hypo and hypercellularity, and immunohistochemically, there was a positive reaction for CD34, vimentin, CD99, BCL2 and STAT-6. After 6 months of follow-up, there were no signs of recurrence.

ABSTRACT Introduction: Ammonia is a resultant molecule from the protein metabolism that can be neurotoxic when present in high concentrations, causing brain edema and encephalopathy. Extracorporeal blood purification technique (EBPT) can play a role in reducing both ammonia and urea blood levels. However, there are no specific guidelines for its use in hyperammonemia, due to its low incidence and scarce literature on this subject, particularly in adults. Case report: The authors describe a case of a 22‑year‑old previously healthy woman. She presented with polyuria, polydipsia, anorexia, vomiting and significant (>10%) weight loss over a month. Laboratory data revealed hypoglycemia and a high anion gap metabolic acidosis with severe acidemia with normal lactate, serum creatinine of 1.12 mg/dl and 260 (reference value 26‑74)umol/l of ammonia. Urine analysis showed ketone bodies. She evolved with progressive encephalopathy and neurological deterioration. The hypothesis of hyperammonemic encephalopathy secondary to a hereditary metabolic disease was suspected. Medical supportive therapy was initiated. An EBPT for ammonia removal and acid‑base correction was initiated using continuous venovenous hemodiafiltration (CVVHDF) with a drastic neurologic improvement. A fatty acid beta‑oxidation deficit was discovered. The patient was discharged with no neurological changes, with a strict diet free of fat, animal protein and high‑protein vegetables, plus supplementation with benzoate, carnitine, riboflavin, maltodextrine and essential amino acids. Discussion and conclusion: In adults, hyperammonemia related to metabolic disorders is rare, and in the presence of encephalopathy, ECDT should be considered. Ammonia is easily dialysable either by intermittent or continuous techniques. CVVHDF provides a continuous clearance with less rebound effect.

ABSTRACT Introduction Acute liver failure (ALF) due to diffuse infiltrating solid malignancy without any focal lesions on radiographic imaging is rare. Case report A 70-year-old man was admitted due to mental confusion, abdominal pain, and ALF. Three years before, he had undergone a left nephrectomy for urothelial carcinoma followed by adjuvant chemotherapy. The abdominal computed tomography (CT) showed hepatomegaly and ascites. Ascitic fluid had transudate characteristics, with no malignant cells. Percutaneous liver biopsy (LB) showed diffuse liver infiltration of metastatic urothelial carcinoma. The patient rapidly deteriorated and died in a week due to ALF. Discussion History of solid cancer and hepatomegaly and/or liver failure without other obvious explanation should encourage to perform LB. Conclusion LB is warranted to avoid misdiagnosis, prolonged hospital stays, and delay in palliative care.

Background: In December 2019, a new form of coronavirus was identified (COVID-19) and quickly became a worldwide pandemic. Previous data reported a more severe disease in older patients, with high comorbidities, which are common features in hemodialysis patients. Scarce data is available about the clinical presentation and outcomes in this population. For this reason, we aim to report the characteristics of COVID-19 disease in hemodialysis patients admitted to our center. Methods: We retrospectively reviewed all patients on chronic hemodialysis admitted with a laboratory-confirmed COVID-19 infection from March 2nd to May 20th of 2020. Baseline characteristics, clinical, laboratory and radiological presentation were registered and treatment and outcomes Results: Fourteen patients were included, 57% female, with a median age of 81 years old and a high number of comorbidities. Cough and fever were the most common symptoms and lymphocytopenia, increased inflammatory parameters and coagulation dysregulation were frequently registered. Ground-glass opacities were present in each thorax computerized tomography performed. Five patients developed respiratory insufficiency, but none needed invasive ventilation or ICU admission. In a significant proportion, discharge was postponed due to the impossibility of ensuring social isolation. Median viral shedding was 38 days. Discussion and conclusion: Hemodialysis patients who required hospitalization were very old and had multiple comorbidities. Even so, the impaired immune response of this population seems to cause a less severe course of the disease and a longer time of viral shedding. HD units have required a reformulation of their circuits in order to avoid disease spreading.

RESUMO Objetivo: Os autores pretendem fazer uma tradução minuciosa e culturalmente adaptada para a língua portuguesa do escore da Síndrome de Ressecção Anterior Baixa (Low Anterior Resection Syndrome [LARS]), um instrumento de desfecho relatado pelo paciente, na população portuguesa com câncer retal após proctectomia com anastomose. Métodos: De acordo com as recomendações internacionais atuais, o estudo foi projetado abrangendo três fases principais: (i) validação cultural e linguística para o português europeu; (ii) testes de viabilidade e confiabilidade da versão obtida na fase anterior; e (iii) testes de validade para produzir a versão final. O questionário foi preenchido por 154 pacientes de seis unidades portuguesas de câncer colorretal e 58 pacientes completaram duas vezes. Resultados: A versão em português do escore LARS mostrou alta validade de construto. A correlação intra-classe global apresentou confiabilidade muito forte no teste-reteste. Considerando-se todos os cinco itens, apenas os itens 3 e 5 apresentam uma correlação moderada. O escore LARS foi capaz de discriminar sintomas com pior qualidade de vida em pacientes submetidos a radio- e quimioterapia pré-operatória. Conclusões: O questionário LARS foi traduzido corretamente para o português europeu, demonstrando alta validade de construto e confiabilidade. Trata-se de uma ferramenta precisa, reproduzível, simples, clara e fácil de usar para avaliar a função intestinal em pacientes com câncer retal após operações poupando o esfíncter.

ABSTRACT Objective: The authors aim to perform a thorough translation with cultural adaptation of the patient reported outcome tool, Low Anterior Resection Syndrome (LARS) Score, to the Portuguese language (LARS-PT) in the Portuguese population with rectal cancer, after proctectomy with anastomosis. Methods: According to the current international recommendations, we designed this study encompassing three main phases: (i) cultural and linguistic validation to European Portuguese; (ii) feasibility and reliability tests of the version obtained in the previous phase; and (iii) validity tests to produce a final version. The questionnaire was completed by 154 patients from six Portuguese Colorectal Cancer Units, and 58 completed it twice. Results: Portuguese version of LARS score showed high construct validity. Regarding the test-retest, the global Intraclass Correlation showed very strong test-retest reliability. Looking at all five items, only items 3 and 5 present a moderate correlation. LARS score was able to discriminate symptoms showing worse quality of life, in patients submitted to preoperative radio and chemotherapy. Conclusions: LARS questionnaire has been properly translated into European Portuguese, demonstrating high construct validity and reliability. This is a precise, reproducible, simple, clear and user-friendly tool for evaluating bowel function in rectal cancer patients after sphincter saving operation.

Abstract INTRODUCTION: Immunological control of Mycobacterium tuberculosis infection is dependent on the cellular immune response, mediated predominantly by Th1 type CD4+ T cells. Polarization of the immune response to Th2 can inhibit the host immune protection against pathogens. Patients with tuberculosis coinfected with helminths demonstrate more severe pulmonary symptoms, a deficiency in the immune response against tuberculosis, and an impaired response to anti-tuberculosis therapy. METHODS: We evaluated the cellular immune response and the impact of the presence of Ascaris lumbricoides on the immune and clinical response in pulmonary tuberculosis patients. Ninety-one individuals were included in the study: 38 tuberculosis patients, 11 tuberculosis patients coinfected with Ascaris lumbricoides and other helminths, 10 Ascaris lumbricoides patients, and 34 non-infected control individuals. Clinical evolution of pulmonary tuberculosis was studied on 0, 30, 60, and 90 days post-diagnosis of Mycobacterium tuberculosis and Ascaris lumbricoides. Furthermore, immune cells and plasma cytokine profiles were examined in mono/coinfection by Mycobacterium tuberculosis and Ascaris lumbricoides using flow cytometry. RESULTS: There were no statistical differences in any of the evaluated parameters and the results indicated that Ascaris lumbricoides infection does not lead to significant clinical repercussions in the presentation and evolution of pulmonary tuberculosis. CONCLUSIONS: The association with Ascaris lumbricoides did not influence the Th1, Th2, and Th17 type responses, or the proportions of T lymphocyte subpopulations. However, higher serum levels of IL-6 in tuberculosis patients may explain the pulmonary parenchymal damage.

Abstract INTRODUCTION This study characterized the clinico-epidemiological profile of American cutaneous leishmaniasis (ACL) cases in Barbalha, Ceará State, Brazil. METHODS Medical records of 363 patients visiting Federal University of Cariri between 2009 and 2014 were analyzed. RESULTS ACL was more prevalent in men with low education level from rural zones. The main presentation was a single ulcer, mainly in the lower limbs, and 49.8% also presented lymphadenomegaly. The annual incidence ranged from 2.83 to 22.60 per 10,000 inhabitants. CONCLUSIONS: The rates observed in this study indicate the importance of additional research to contribute to the control of this endemic disease.

O trabalho objetivou avaliar comparativamente o crescimento e a expressão do vigor de sementes de feijão provenientes de plantas submetidas à aplicação de ácido giberélico. Os tratamentos consistiram na aplicação de ácido giberélico nas concentrações de zero; 50; 100 e 200 mg.L-1 no estádio V2. As coletas para análise de crescimento foram realizadas em intervalos regulares de sete dias após a semeadura, até as plantas atingirem o estádio R9. Em cada coleta, as plantas foram separadas em órgãos, para a determinação do crescimento e da partição de matéria seca. Além disso, determinou-se o índice de colheita, massa de mil sementes, número de sementes por planta, inserção da primeira vagem, comprimento e matéria seca total de plântulas. A matéria seca total, as taxas de produção de matéria seca e a eficiência de conversão de energia solar são superiores em plantas submetidas à aplicação exógena de ácido giberélico. O índice de colheita bem como o número de sementes por planta apresentam uma tendência de redução com a elevação da concentração de ácido giberélico. Contudo, a massa de mil sementes tende a ser superior na dose de 50 mg.L-1. O vigor de sementes de feijão, avaliado pelo teste de comprimento de plântulas, é alterado com aplicação de ácido giberélico.

The objective of this work was to evaluate the growth and vigor expression of bean seeds from plants submitted to the application of gibberellic acid. The treatments consisted of the application of gibberellic acid in concentrations of zero; 50; 100 and 200 mg.L-1 in the V2 stage. The samples for growth analysis were performed at regular intervals of seven days after sowing, until the plants reached the R9 stage. At each collection, the plants were separated into organs for determination of growth and dry matter partition. In addition, the harvest index, mass of one thousand seeds, number of seeds per plant, insertion of the first pod, length and total dry matter of seedlings were determined. Total dry matter, dry matter production rates and solar energy conversion efficiency are higher in plants submitted to the exogenous application of gibberellic acid. The harvest index as well as the number of seeds per plant present a tendency of reduction with the increase of the gibberellic acid concentration. However, the mass of one thousand seeds tends to be higher in the dose of 50 mg.L-1. The vigor of bean seeds, evaluated by the test of seedling length, is altered with application of gibberellic acid.

Abstract i* models have been used for requirements specification in many domains, such as healthcare, telecommunication, and air traffic control. Managing the scalability and the complexity of such models is an important challenge in Requirements Engineering (RE). Scalability is also one of the most intractable issues in the design of visual notations in general: a well-known problem with visual representations is that they do not scale well. This issue has led us to investigate scalability in i* models and its variants by means of a systematic mapping study. This paper is an extended version of a previous paper on the scalability of i* including papers indicated by specialists. Moreover, we also discuss the challenges and open issues regarding scalability of i* models and its variants. A total of 126 papers were analyzed in order to understand: how the RE community perceives scalability; and which proposals have considered this topic. We found that scalability issues are indeed perceived as relevant and that further work is still required, even though many potential solutions have already been proposed. This study can be a starting point for researchers aiming to further advance the treatment of scalability in i* models.

Resumo Introdução: O envelhecimento é compreendido como a soma de todas as alterações biológicas, psicológicas e sociais que ocorrem com o passar dos anos. Associadas ao envelhecimento elencam-se as alterações de ordem morfológica e funcional do sistema imunológico: Imunossenescência. Objetivo: Caracterizar o efeito do condicionamento físico breve sobre o perfil de citocinas e células mononucleares do sangue periférico de idosos na cidade de Manaus, AM. Materiais e métodos: Doze indivíduos com idade média de 66,8±3,7 anos realizaram 3 sessões semanais de exercícios físicos por 16 semanas e sete indivíduos com idade média de 66,1±6,7 anos, praticantes de atividades lúdicas, formaram um grupo controle. Os níveis séricos de IL-2, IL-4, IL-6, IL-10, IL-17, TNF-α e INF-γ foram medidos pela técnica CBA (Cytometric Bead Array) e as contagens de subpopulações de linfócitos B, B1, T/CD4, T/CD8, TReg, NK e NKT foram realizadas por citometria de fluxo. Resultados: Observou-se que, após a atividade física, houve aumento significativo (p < 0,05) no número de linfócitos B, T/CD4 + e NKT (CD3 + /CD16 + /CD56 + ), quando comparados à fase pré-treinamento e ao grupo controle. Em outro modelo de análise, qualificando-se cada indivíduo do grupo teste como alto produtor ou baixo produtor das citocinas, observaram-se padrões na fase pós-treinamento que permitiram definir conjuntos ("assinaturas") de citocinas que se expressam associadas ao exercício. Conclusão: Nosso trabalho evidenciou que o exercício induz alterações na contagem de células imunes, o que nos permite inferir que pode ser usado como alternativa para reverter ou atenuar as implicações da imunossenescência.

Abstract Introduction: Aging is understood as the sum of all biological, psychological and social changes that occur over the years. Associated with aging we list up the changes of morphological and functional order of the immune system: Immunosenescence. Objective This study's objective was to characterize the effect of a brief exercise program on the profile of cytokines and peripheral blood mononuclear cells of elderly individuals in Manaus, AM, Brazil. Materials and methods: Twelve subjects aged 66.8 (± 3.7) years old on average engaged in three weekly sessions of exercises for 16 weeks and, seven subjects aged 66.1 (± 6.7) years on average, who practiced only recreational activities, composed the control group. Serum levels of IL-2, IL-4, IL-6, IL-10, IL-17, TNF-α and INF-γ were measured using the CBA technique (cytometric Bead Array) and the count of subpopulations of lymphocytes - B, B1, T/CD4, T/CD8, Treg, NK and NKT - was performed using flow cytometry. Results: The relative number of B lymphocytes, T/CD4+ and NKT (CD3+/CD16 +/CD56+) increased significantly (p <0.05) after physical activity, compared to the pre-exercise phase and the control group. In another analysis, each individual in the test group was classified either as major or minor producer of each cytokine; i.e., their values were above or below the cut-off point defined by the median of all measurements of that cytokine. Patterns of cytokine production were observed in the post-exercise group, which allowed defining sets ("signatures") of cytokines that were associated with the practice of short-term physical exercises. Conclusion: Our work showed that exercise induces changes in the count of immune cells, which allows us to infer that it can be used as an alternative to reverse or mitigate the implications of immunosenescence.

RESUMO O cumaru (Amburana cearensis (Allem.) A. C. Smith) é uma espécie nativa do semiárido brasileiro que apresenta múltiplas utilidades como madeira de boa qualidade e contém princípio ativo que pode ser utilizado nas indústrias alimentícias, de perfume e na produção de medicamentos. É, também, planta forrageira e melífera. As respostas do cumaru à adubação orgânica e cobertura morta do solo, insumos oriundos e ciclados dentro do próprio agroecossistema, são ainda pouco conhecidas. Para isto, um experimento foi montado visando estudar o comportamento das mudas em um sistema agroflorestal (em consórcio com milho e feijão), testando-se diferentes níveis de matéria orgânica (0, 15 e 30 L de esterco na cova), com e sem cobertura morta no solo. Avaliou-se a altura de plantas, diâmetro caulinar, número de folhas, área foliar e sobrevivência das plantas no campo após um ano de plantio. O cumaru, no sistema agroflorestal estudado, mostrou-se indiferente ao uso de cobertura morta e adaptado às condições de baixa matéria orgânica no solo, obtendo-se o maior crescimento na ausência de matéria orgânica (esterco).

ABSTRACT Amburana cearensis (Allem.) A. C. Smith is a Brazilian semiarid region native plant. It presents good quality wood, contains an active ingredient that can be used for food, perfume and medicine production and it is also a forage and a melifera plant. The responses of the studied species to organic fertilizer and mulch, inputs produced and cycled within its ecosystem are still unknown. For this, an experiment was conducted to study the behavior of seedlings in an agroforestry system intercropped with maize and beans, testing different levels of organic matter (0, 15 and 30 L of manure) with and without soil mulch. It was evaluated the plant height, stem diameter, leaf number, leaf area and the survival of plants in the field one year after planting. The performance of A. cearensis was indifferent to the use of mulch and well adapted to low soil organic matter conditions, obtaining the largest growth in the absence of organic matter.

Foram examinadas quatro estratégias (Tris / EDTA, dodecilsulfato de sódio, resina Chelex 100 e brometo de cetiltrimetilamónio -CTAB-) para a extração de ácido nucleico (DNA) das comunidades de nematóides. Os nematóides foram isolados a partir de uma área agrícola sob manejo diferente num experimento de rotação de culturas de longo prazo, durante três temporadas na Argentina. Após a extração de DNA, a reação em cadeia da polimerase e amplificações foram realizadas e consideradas como indicadoras da extração de DNA com sucesso. O CTAB combinada com proteinase K e álcool fenol-clorofórmio-isoamílico foi o único método bem sucedido porque as amplificações positivas foram obtidas utilizando ambos os primers específicos para os eucariótas e nematóides. Este trabalho poderá contribuir para o estudo da biodiversidade de nematóides em agroecossistemas.

We examined four strategies (Tris/EDTA, sodium dodecyl sulfate, Chelex 100 resin and cetyltrimethylammonium bromide -CTAB-) for extracting nucleic acid (DNA) from communities of nematodes. Nematodes were isolated from an agricultural area under different management of long-term crop rotation experiment from Argentina during three seasons. After DNA extraction, Polymerase Chain Reaction-amplifications were performed and considered as indicators of successful DNA extraction. The CTAB combined with proteinase K and phenol-chloroform-isoamyl alcohol was the unique successful method because positive amplifications were obtained by using both eukaryotic and nematode specific primers. This work could contribute to biodiversity studies of nematodes on agroecosystems.