Search

Purpose Long bone lengthening procedures are paramount in the treatment of limb length discrepancies. We witnessed a revolution in the treatment paradigm of this pathology with the development of expandable intramedullary rods. Endomedular nailing might be technically demanding and some steps are critical for success. The aim of authors is to describe a technical note of the PRECICE system that may ease femoral nailing and fixation: the nail can be advanced through the femur and proximal and distal fixation performed previous to complete femoral osteotomy. Materials and methods The authors present a case series of XX patients in which the limb lengthening has been performed with partial osteotomy with Gigli saw, nail advancement, proximal and distal fixation, and osteotomy completion at the end of the procedure. Results After 18 consecutive limb (femoral) lengthening operations with this technical variation in PRECICE, nail patients presented no significant lower limb length discrepancy. There were no records of rotational deformities, nonunion, or infection. Material failure was not reported. Conclusion This technical note is another positive variable that can help to ease the procedure, minimise possible complications, and confirm magnetic expandable nails as the gold standard technique in limb lengthening procedures and it might be applied to other nailing systems for limb lengthening procedures. How to cite this article Lopes M, Nunes B, Couto A, et al. Electromagnetic Rod in Lower Limb Lengthening: A Technical Note for Shaft Osteotomy. Strategies Trauma Limb Reconstr 2019;14(3):139–141.

In this observational study performed during 45 months we evaluated patients with chronic and recalcitrant diabetic ulcers who were treated with homologous platelet gel. Methods platelet gels were obtained from homologous platelet concentrates that were aliquoted and freezed, being then activated with calcium gluconate and applied in the ulcer after cleaning and debridement. We evaluated patient's comorbidities, wound characteristics (size, tissue, inflammatory signs, pain), number and time of treatment as well as outcome (classified as complete epithelialization; partial improvement- 50% reduction in wound size or pain relief; no evolution). Results Fifty-two patients (42 males, 10 females), with a median age of 65 years (range 43–85) were proposed for platelet gel. The following associated comorbidities were observed: hypertension (n = 41), dyslipidemia (n = 29), polyneuropathy (n = 30), peripheral arteriopathy (n = 32), retinopathy (n = 21), nephropathy (n = 15), cardiac ischemic disease (n = 14), obesity (n = 9). Thirty-eight patients presented with 3 or more associated comorbidities. The more frequent ulcer locations were sole of the foot (n = 13) and heel (n = 10). The median number of applications was 16, during 8.5 weeks. Nineteen patients (44%) achieved complete healing, 3 patients (7%) had a partial response and 21 (49%) had no progression. We did not observe a statistically significant relationship between patient age and response nor between number of comorbidities and response. We observed a more favorable evolution in patients with good compliance and good glycemic control. Conclusion Platelet gel is an effective therapeutic alternative, provided compliance and effective metabolic control are ensured.

Purpose The most significant advance in our time about limb lengthening is the magnetic lengthening nail, as the first reports appeared to show good results with accurate lengthening rates and good regenerate bone formation. The described complication rate is generally low. They avoid external fixation elements, and are activated transcutaneously, so the patient’s pain and discomfort are reduced and the rehabilitation is faster and more effective. The aim of authors is to describe a special technical issue of the PRECICE system: the nail can be extended inside the patient limb (after the osteotomy), but it also can be retracted inside the limb after achieving the bone union. Methods The authors present a case in which the limb lengthening has been performed in consecutive lengthening periods using the same nail. The nail was extended and retracted by altering the settings on the external remote control as well as accurately setting the rate of distraction. Results After two consecutive femoral lengthening with the same PRECICE nail, the patient no longer has a significant lower limb length discrepancy and patient satisfaction was high. During this clinical case, we were not confronted with any type of complications. Conclusion This technique utilizes the principles and advantages of lengthening over an magnetic lengthening nail, avoids the necessity of nail removal and minimizes the complication rates and the overall time for complete recovery. Level of evidence Level IV.

The Internet of Things (IoT) is an emerging technological paradigm, which is experiencing an exponential growth. It focus on distributed sensing to acquire knowledge about the real world, and distributed computing for its computational capabilities. An IoT node is composed of a microprocessor, a communication unit (RF radio), sensors or actuators and a power source. It tries to achieve extremely high battery life (years or decades), mainly through aggressive duty cycling. This way, the node spends most of its life in a sleep state (apart from some always-on components). The duty cycling comes with a disadvantage: all data must be stored in retentive memories (e.g. flash memory) or in always-on parts of the circuitry. Static Random Access Memory (SRAM) is a type of non-retentive memory that can hold its data indefinitely (while powered). It is used in small embedded devices and SoC, as well as in processors cache. By designing a SRAM module for the sensors data that focus on lowering the power consumption, the overall power consumption of the power node can be reduced. This work focus on designing a full-custom 180 nm CMOS SRAM for Ultra Low Power. The SRAM has 128 bytes (1024 bits) of storage, and works at 128 Hz clock frequency, while powered at 200 mV.

Background Immunophenotypic analysis of the bone marrow (BM) cells has proven to be helpful in the diagnosis of Myelodysplastic Syndromes (MDS). However, the usefulness of flow cytometry (FCM) for the detection of myelodysplasia in the peripheral blood (PB) still needs to be investigated. The aim of this pilot study was to evaluate the value of FCM-based PB neutrophil and monocyte immunophenotyping for the diagnosis of lower risk MDS (LR-MDS). Methods We evaluated by 8-color FCM the expression of multiple cell surface molecules (CD10, CD11b, CD11c, CD13, CD14, CD15, CD16, CD34, CD45, CD56, CD64 and HLA-DR) in PB neutrophils and monocytes from a series of 14 adult LR-MDS patients versus 14 normal individuals. Results Peripheral blood neutrophils from patients with LR-MDS frequently had low forward scatter (FSC) and side scatter (SSC) values and low levels of CD11b, CD11c, CD10, CD16, CD13 and CD45 expression, in that order, as compared to normal neutrophils. In addition, patients with LR-MDS commonly display a higher fraction of CD14+CD56+ and a lower fraction of CD14+CD16+ monocytes in the PB. Based on these results, we proposed an immunophenotyping score based on which PB samples from patients with LR-MDS could be distinguished from normal PB samples with a sensitivity 93% and a specificity of 100%. In addition, we used this score to construct the MDS Thermometer, a screening tool for detection and monitoring of MDS in the PB in clinical practice. Conclusions Peripheral blood neutrophil and monocyte immunophenotyping provide useful information for the diagnosis of LR-MDS, as a complement to cytomorphology. If validated by subsequent studies in larger series of MDS patients and extended to non-MDS patients with cytopenias, our findings may improve the diagnostic assessment and avoid invasive procedures in selected groups of MDS patients. Electronic supplementary material The online version of this article (10.1186/s12878-018-0101-8) contains supplementary material, which is available to authorized users.

A Urolitíase é uma afecção metabólica que pode ter diversas causas e frequentemente afeta os cães de diversas raças, idades e sexo. A dieta do animal pode interferir tanto no aparecimento quanto na prevenção de recidivas de urolitíase, por isso, é considerada de fundamental importância na prevenção da afecção do trato urinário, minimizando as chances de formação de cálculos. O diagnóstico é baseado na anamnese, sinais clínicos e exames complementares como a urinálise, ultrassonografia, a radiografia, entre outros. Os sinais clínicos mais comumente observados em animais acometidos são a hematúria, disúria e polaquiúria. O tratamento clínico da urolitíase tem por objetivo a promoção da dissolução e/ou interrupção do crescimento subsequente dos urólitos, podendo ser feito através de modificações na dieta ou, em alguns casos, é necessária a realização de cirurgia.

Primary liver lymphoma is extremely rare, in most of cases it is a B cell lymphoma. Usually the diagnosis is made in middle-aged individuals and most of them have a relatively short life expectancy. In this article, the authors present a case report of a 75-year-old woman with symptoms of three weeks of evolution of diffuse abdominal pain, asthenia and anorexia. The analysis showed cyto cholestasis and the radiological image with lush hepatomegaly because of a large hepatic mass. The patient started chemotherapy and actually is well twenty months after the end of the chemotherapy.

Solitary Plasmacytoma (SP) is a rare plasma cell dyscrasia characterized by localized plasma cell infiltration. Radiotherapy is recommended as the treatment of choice. Epidemiological data is important to evaluate the effectiveness of treatment and the progression to Multiple Myeloma (MM). We described the clinical features and follow-up of 32 patients with SP treated with radiotherapy as primary treatment at our Department of Radiotherapy and we reviewed the literature on prognostic factors of progression to MM. Ninety percent of patients (71% complete response and 19% partial response) responded to treatment. Fourteen patients progressed to MM with a median time of progression of 16, 9 months. With a median follow-up of 40 months, the 5 and 10-year estimated Overall Survey (OS) was 62, 6% and 47, 7% respectively. Predictive factors of progression are still controversial. New factors are emerging in the fields of pathology, imagiology and immunology and thus we wait longer follow-up to confirm their predictive value. SP is highly radiosensitive and radiotherapy combines excellent control rates with minimal toxicity. However, it is a heterogeneous disease and approximately half of the patients will progress to MM. Identifying the patients more likely to progress would allow us to treat them in a different way.

A presente Dissertação de Mestrado tem por escopo o exame das nulidades no processo penal brasileiro. O estudo foca, em especial, o prejuízo, critério eleito pelo ordenamento jurídico brasileiro para verificar quando há, ou não, nulidade, tendo em vista a adoção da teoria da instrumentalidade das formas. Escolheu-se estudar o tema, em razão da falta de uniformidade no tratamento da matéria, tanto em nível doutrinário quanto jurisprudencial. Com efeito, é possível notar que são proferidas decisões divergentes para casos iguais, em curto espaço de tempo. Entre os fatores que podem, eventualmente, explicar essa carência de sistematização está o fato de não se conseguir especificar e/ou bem delimitar: (a) qual o conceito e o cerne da nulidade, sobretudo processual, (b) qual sua natureza jurídica, (c) como a nulidade pode ser diferenciada dos demais institutos jurídicos, haja vista existir muita confusão entre as nulidades e as demais invalidades, sem contar o instituto da inexistência jurídica. No que tange ao prejuízo, não há consenso a seu respeito, qual o seu conteúdo, bem como se dá a sua ocorrência no processo, se precisa ser efetivo (concreto), ou meramente potencial. Todas essas questões têm trazido muitas dúvidas tanto na teoria quanto na prática, havendo uma grande insegurança jurídica, o que pode levar a decisões casuísticas, acarretando tratamentos diferenciados para casos iguais: o que enseja, de um lado, impunidade (ou tratamento privilegiado) e, de outro, persecuções penais arbitrárias, vulnerando o sistema de direitos e garantias no Estado de Direito Democrático. O estudo é feito, ao final, à luz da jurisprudência do Supremo Tribunal Federal e do Superior Tribunal de Justiça, pois é somente a partir dessa análise que se conseguirá melhor esmiuçar quais critérios e balizas têm sido levados em conta para, em cada caso concreto, reconhecer-se o prejuízo e, por via reflexa, a nulidade. O presente estudo, ainda que de maneira audaciosa, tem a pretensão de propor uma maior sistematização da matéria, almejando a aplicação equânime e justa do direito. This Master\'sDissertation aims to analyze annulments in the Brazilian criminal proceeding. The instant Research focuses on the harm rule, the legal standard chosen by the Brazilian system to check whether there is a kind of nullity in the criminal proceeding, considering the instrumentality theory of legal forms. The subject was chosen due to the lack of uniformity: both in academic area (doctrinaire) and in the judicial practice. Indeed, there exist different kinds of decisions for similar cases, in short periods of time. Among the reasons that could possibly explain this lack of systematization or this difference of treatment is the fact that scholars and the justices are not able to specify and/or even to define properly: (a) the essence of the concept or the core of the proceeding invalidity and, particularly (b) its legal constitution as well as (c) how the nullity can be distinguished from other legal institutions that are alike, also considering that there is too much confusion between the nullity and the other kinds of invalidities, including a lot of misunderstanding between the invalidity and the legal non-existence theory. Regarding specifically the harm rule, the scholars and the Courts also do not clarify for instance, what it is and its content is, as well as how its occurrence takes in the criminal law proceeding: if it must be effective (concrete) or merely potentially. All these non-solution issues have brought many questions to both theory and practice, and there is considerable legal uncertainty, which may lead casuistic decisions, bringing different treatments which can lead impunity (or preferential treatment) and on to other hand arbitrary acts against the defendant, violating human rights. The study is done in light of the decisions of the Supreme Court and the Superior Court of Justice, because this analysis allows to elaborate the criteria and goals taken into account, to assess properly the harm rule and the nullity. The present study also dares to propose a better systematization of the issue, always taking into consideration the desired and the necessary fair enforcement of law.

Background Age is a negative prognostic factor in lymphomas, and elderly patients are often undertreated because of toxicity concerns. The pattern of treatment in elderly patients with diffuse large B-cell lymphoma (DLBCL) in Portugal has not been previously described. Patients and Methods We conducted a multicenter retrospective study including 378 elderly patients with DLBCL receiving alkylating agent–containing regimens between 2003 and 2010. We compared the outcome of patients aged 60 to 79 years with patients > 79 years and analyzed the second group according to treatment. Results R-CHOP (rituximab, cyclophosphamide, doxorubicin [hydroxydaunorubicin], vincristine [Oncovin], prednisolone) was prescribed in only 60% of patients and was prescribed significantly less in patients > 79 years, despite no significant differences being found in comorbidities between the 2 age groups. Similarly, dose reductions frequently were instituted because of chronologic age and not always because of toxicity. When different regimens were compared, multivariate analysis showed an independent beneficial effect of R-CHOP in treatment outcomes. Additionally, treatment with anthracyclines and rituximab predicted a better progression-free survival (PFS) and time to progression (TTP) in patients > 79 years. Conclusion This was the first characterization of the clinical care of elderly Portuguese patients with DLBCL. We showed that R-CHOP is effective even in patients > 79 years, emphasizing that treatment decisions based on age alone can compromise treatment efficacy and outcome in fit patients.

We report 12 cases of aggressive natural killer (NK) cell neoplasms diagnosed in Portugal, with emphasis on flow cytometry. Ten patients had extranodal NK/T cell lymphoma, nasal type and two had aggressive NK cell leukemia, and seven were men and five were women, with a median age of 50 years. NK cells brightly expressed the CD56 adhesion molecule and CD94 lectin type killer receptor and had an activation-related HLA-DR+ CD45RA+ CD45RO+ immunophenotype, in most cases. In contrast, dim CD16 expression was found in a minor proportion of cases, whereas CD57 and the CD158a and CD158e1 killer immunoglobulin-like receptors were negative. One-third of cases showed a hyperploid DNA content and nearly all had a very high S-phase proliferative rate. The phenotypic features of the neoplastic NK cells would suggest that they represent the transformed counterpart of the CD56 + bright NK cells that circulate in normal blood.

Introduction: Among patients receiving vitamin K antagonists (VKA) therapy, maintenance of an international normalized ratio (INR) in the therapeutic range is essential for treatment efficacy and safety. This requires regular monitoring and appropriate dose adjustment. It has been reported that anticoagulation clinics should aim for a time in therapeutic range (TTR) between 70-80% to optimize benefit and minimize the risk of adverse events. Previously (in a study between September 2006 and June 2012), we have reported that patients with longer INR recall interval (4-8 weeks) showed no decrease of monitoring quality and that it would be safe to increase time between measurements. Aim: Since actual recommendations for improving TTR include shortening INR recall interval (Lip et al. 2018) we aimed to evaluate the quality of anticoagulation monitoring after having increased time between measurements beyond the 4-8 weeks recall interval. Methodology: We retrospectively analyzed 37931 appointments of 6 consecutive years (July 2012 to July 2018) corresponding to 1587 patients that are regularly followed up at an outpatient Anticoagulation Clinic of a central hospital under anticoagulation for at least 8 weeks, using TTR determined by Rosendaal method. Patients were divided according to target INR in three groups: Group 1 with target INR 2-3, including 1430 patients corresponding to 30743 appointments with mean age 69±15 years (mean±SD), majority (46.4%) with atrial fibrillation (AF); Group 2 with target INR 2.5-3.5, including 125 patients corresponding to 5439 appointments with mean age 67±12 years, majority (85.6%) with mechanical heart valves; Group 3 with target INR 3-4, including 32 patients corresponding to 1749 appointments with mean age 62±14 years, majority (62.5%) with antiphospholipid syndrome. Descriptive statistics (mean, standard deviation, minimum, maximum, chi-square), inferential statistics (t-test, A-Nova and effect sizes) tests and correlations were performed. Results: The 1587 patient population, 50.5% male, mean age of 68±17 years and 90.1% in Group 1, showed a mortality of 18%. A point-biserial correlation was run to determine the relationship between mortality and gender, age, INR group and diagnostic. Mortality was correlated with diagnosis (57.2% with AF) (rpb = -.071, n = 1587, p = .004), male gender (60%) (rpb = -.089, n = 1587, p Conclusions and Discussion: More than 90% of the population under VKA treatment showed effective TTR which may infer safety in increasing INR recall interval. The TTR decrease with a smaller population may be explained by the introduction of direct oral anticoagulants in patients with less comorbidities. The increase of TTR in patients with higher INR target suggests a better management of patients under VKA therapy that is actually the only choice for challenging patients. Disclosures No relevant conflicts of interest to declare.

Submitted by deniseoa@stj.jus.br (deniseoa@stj.jus.br) on 2018-01-11T16:24:07Z No. of bitstreams: 2 importancia_conservacao_local_paschoal.pdf: 622478 bytes, checksum: e72cb953771cfd8c93b91b6e1a357ec9 (MD5) license.txt: 1239 bytes, checksum: c9b4c351324448672315a00808efb725 (MD5) Approved for entry into archive by allanr@stj.jus.br (allanr@stj.jus.br) on 2018-01-31T16:33:30Z (GMT) No. of bitstreams: 2 importancia_conservacao_local_paschoal.pdf: 622478 bytes, checksum: e72cb953771cfd8c93b91b6e1a357ec9 (MD5) license.txt: 1239 bytes, checksum: c9b4c351324448672315a00808efb725 (MD5) Made available in DSpace on 2018-01-31T16:33:30Z (GMT). No. of bitstreams: 2 importancia_conservacao_local_paschoal.pdf: 622478 bytes, checksum: e72cb953771cfd8c93b91b6e1a357ec9 (MD5) license.txt: 1239 bytes, checksum: c9b4c351324448672315a00808efb725 (MD5) Previous issue date: 2017

Background Red cell distribution width (RDW), a classical parameter used in the differential diagnosis of anemia, has recently been recognized as a marker of chronic inflammation and high levels of oxidative stress (OS). Fanconi anemia (FA) is a genetic disorder associated to redox imbalance and dysfunctional response to OS. Clinically, it is characterized by progressive bone marrow failure, which remains the primary cause of morbidity and mortality. Macrocytosis and increased fetal hemoglobin, two indicators of bone marrow stress erythropoiesis, are generally the first hematological manifestations to appear in FA. However, the significance of RDW and its possible relation to stress erythropoiesis have never been explored in FA. In the present study we analyzed routine complete blood counts from 34 FA patients and evaluated RDW, correlating with the hematological parameters most consistently associated with the FA phenotype. Results We showed, for the first time, that RDW is significantly increased in FA. We also showed that increased RDW is correlated with thrombocytopenia, neutropenia and, most importantly, highly correlated with anemia. Analyzing sequential hemograms from 3 FA patients with different clinical outcomes, during 10 years follow-up, we confirmed a consistent association between increased RDW and decreased hemoglobin, which supports the postulated importance of RDW in the evaluation of hematological disease progression. Conclusions This study shows, for the first time, that RDW is significantly increased in FA, and this increment is correlated with neutropenia, thrombocytopenia, and highly correlated with anemia. According to the present results, it is suggested that increased RDW can be a novel marker of stress erythropoiesis in FA. Electronic supplementary material The online version of this article (doi:10.1186/s13023-016-0485-0) contains supplementary material, which is available to authorized users.

Comparatively to open repair, endovascular aneurysm repair has reduced transfusion rates but thereâs no recommendation about number of red blood cells units to be crossmatched preoperatively. Our aim is contribute to the analysis of red blood cells units needs in endovascular and hybrid aortic aneurysm repair and developing a protocol for maximum surgical blood orders schedule.We retrospectively analyzed our prospective database of elective endovascular aneurysm repair from 2001 to 2012. We analyzed patients' age, gender, ASA classification, maximum surgical blood orders schedule, red blood cells units transfused and timings, types of endoprosthesis, red blood cells units consumption/endoprosthesis' type ratio, crossmatch to transfusion ratio, conversion to open repair, hemoglobin concentrations before surgery and discharge.We selected 187 patients, 90% men, mean age 73.1, ASA mode III. The endoprosthesis were aorto-bi-iliac in 71%, aorto-uni-iliac in 23% and thoracic in 6%. Of these, 72,6% of the patients did not require blood transfusion. We transfused 171 red blood cells units. Crossmatch to transfusion ratio was 10.1 until 2010 and 7.3 after. The ratio of red blood cells units consumption/endoprosthesis in the first 24 hours was 0.21 red blood cells units/aorto-bi-iliac, 0.46 red blood cells units/aorto-uni-iliac, 0.8 red blood cells units/thoracic, 1.3 red blood cells units/hybrid-thoracic and 2 red blood cells units/hybrid-aorto-bi-iliac. A statistical correlation was observed between red blood cells units transfused postoperatively and type of endoprosthesis (p0.001) and between ASA classification and red blood cells units transfused after 24 hours (p0.01).Guidelines from the British Society of Haematology are based on a crossmatch to transfusion ratio of 2:1. Our crossmatch to transfusion ratio was 10.1 until 2010 and 7.3 from 2011 to 2012.These results changed our policy of maximum surgical blood orders schedule for endovascular aneurysm repair. We now type and screen aorto-bi-iliac and aorto-uni-iliac. We crossmatch two red blood cells units for thoracic, three red blood cells units for hybrid thoracic and four red blood cells units for hybrid abdominal procedures. This may lead to financial savings, improved efficiency and reduce workload in hematology department.Introdução: O tratamento endovascular dos aneurismas da aorta reduziu as taxas de transfusão. Não existem recomendações relativamente às unidades de glóbulos vermelhos a serem tipadas por crossmatch pré-operatoriamente. O nosso objetivo é contribuir para a análise das unidades de glóbulos vermelhos necessárias no tratamento endovascular dos aneurismas da aorta e na cirurgia híbrida de aneurismas da aorta e definir um protocolo do Maximum Surgical Blood Orders Schedule. Material e Métodos: Analisámos retrospetivamente a base de dados prospectiva dos doentes tratados eletivamente por tratamento endovascular dos aneurismas da aorta, entre 2001 e 2012. Analisamos idade, género, classe ASA, Maximum Surgical Blood Orders Schedule, unidades de glóbulos vermelhos transfundidas e tempos, endoprótese, relação unidades de glóbulos vermelhos consumidas/endoprótese, relação unidades crossmatched/transfundidas, conversão para cirurgia convencional, hemoglobina pré-operatória e na alta. Resultados: Selecionamos para análise 187 doentes, 90% homens, idade média 73,1, classe ASA III mais frequente. Usamos próteses aorto-bi-iliaca em 71%, aorto-uni-iliaca 23% e torácica 6%. Não necessitaram transfusão 72,6%. Transfundimos 171 unidades de glóbulos vermelhos. A relação unidades crossmatched/transfundidas foi 10,1 até 2010 e 7,3 após. A relação unidades de glóbulos vermelhos consumidas/endoprótese nas primeiras 24 horas foi 0,21 unidades de glóbulos vermelhos/aorto-bi-iliaca, 0,46 unidades de glóbulos vermelhos/aorto-uni-iliaca, 0,8 unidades de glóbulos vermelhos/torácica, 1,3 unidades de glóbulos vermelhos/híbrida-torácica e duas unidades de glóbulos vermelhos/híbrida-aorto-bi-iliaca. Obtivemos relação estatisticamente significativa entre unidades de glóbulos vermelhos transfundidas/endoprótese (p0,001) e entre classificação ASA e unidades de glóbulos vermelhos transfundidas após 24 h (p0,01). Discussão: As recomendações da British Society of Haematology baseiam-se numa relação unidades crossmatched/transfundidas de 2:1. Na nossa instituição, a relação unidades crossmatched/transfundidas foi de 10,1 até 2010 e 7,3 de 2011 a 2012. Conclusões: Os resultados mudaram o nosso Maximum Surgical Blood Orders Schedule para o tratamento endovascular dosaneurismas da aorta. Atualmente procedemos ao teste 'type and screen' para próteses aorto-bi-iliaca e aorto-uni-iliaca e a testes de crossmatch de duas unidades de glóbulos vermelhos para torácica, três para híbrida-torácica e quatro para híbrida-abdominal. Isto pode levar a menos gastos, aumento de eficácia e reduzir a sobrecarga no departamento de hematologia.

Tese de doutoramento em Ciências da Comunicação, no ramo de Estudos de Jornalismo, apresentada à Faculdade de Letras da Universidade de Coimbra Os contornos de um mundo em mudança e a evolução do pensamento há muito que se afastaram dos conceitos de identidades unas e imutáveis. A própria aceitação da mutabilidade traduz-se também nos cenários das vastas possibilidades para os fenómenos identitários à escala global. Também por isso, e mais do que nunca, se revela preponderante a reflexão sobre tais fenómenos identitários e a compreensão da sua intervenção e pertinência nas constantes reconfigurações sociais da atualidade. Esse será o universo do presente trabalho, onde serão auscultadas atuais conceções identitárias e o seu lugar no caso português. Este estudo configura-se, portanto, a partir de objetivos centrais bem delimitados: identificar e caracterizar a eventual existência de identidades regionais em Portugal, no caso particular dos territórios associados a Viseu. Este ponto de partida surge da análise da frequente cartografia das identidades territoriais na realidade portuguesa, bem como da continuidade de anteriores estudos do autor. Com efeito, verifica-se que frequentemente se definem duas grandes tipologias identitárias no que concerne à relação com o território: a local e a nacional. Tal posição surge em aberta contradição com os discursos mediáticos recorrentes onde a constante presença de um discurso de matriz regional obriga à dúvida inevitável. Nesse sentido, falando-se de região, foi necessário percorrer os conceitos de limite e de fronteira que a delimitam e lhe conferem sentido, indagando-se sobre o lugar da sua criação e da sua vivência. Assim, o trabalho orientou-se em dois grandes sentidos iniciais: o do levantamento da ação delimitadora do Estado, tido como autor das demarcações regionais em Portugal e o da auscultação da produção discursiva dos media regionais, agentes e expressão da identidade regional. A segunda dimensão obrigaria a um estudo aturado sobre a imprensa regional, objeto essencial do presente trabalho e a partir do qual será possível alcançar conclusões validáveis para o período entre 1959 e 2011. É então pelo cruzamento das duas dimensões referidas que se surpreende a existência de traços identitários regionais bem vincados, profundamente arreigados ao discurso do Estado Novo, raramente correspondendo aos intentos de delimitação das sucessivas iniciativas governamentais posteriores que, desse modo, revelam também um profundo distanciamento desse território que insistentemente vão dividindo. Entre o discurso eivado de simbolismo – embora centralista – do Estado Novo e as denominações técnicas – embora com o propósito de promover a descentralização do poder – da democracia, o discurso identitário da imprensa regional de Viseu alimenta-se ainda hoje do primeiro. Não porque vise os seus objetivos, mas porque é o que melhor caracteriza uma identidade relativamente pacificada: sempre pronta a recorrer ao passado beirão para afirmar as suas diferenças, sem no entanto pôr em causa o espírito da Nação; sempre pronta a denunciar o centralismo de Lisboa, esquecendo embora que foi esse centralismo que outrora lhe atribuiu a característica da genuinidade de, o que quer que seja, ser português. The shapes of a changing world and the evolution of thought have long moved away from the concepts of singular and immutable identities. The acceptance of the mutability also finds expression in scenarios of vast possibilities for identity phenomena on a global scale. More than ever, the reflection about such identity phenomena, the understanding of their intervention and relevance in today's constant social reconfigurations is paramount. This will be the focus of this study where current conceptions of identity and its place in the Portuguese case will be closely examined. This study sets well-defined core objectives: to identify and characterize the possible existence of regional identities in Portugal, in particular the territories associated to Viseu. This starting point emerges from the analysis of frequent cartography of the territorial identities in the Portuguese reality as well as the continuity of previous studies by the author. Furthermore, it often appears that there are defined two major types of identities in regards to the relationship with the territory: local and national. Such position arises in open contradiction to the reoccurring speeches in the limelight, where a constant presence of a speech of regional character brings inevitable doubt. It was necessary to follow through the concepts of limits and boundaries, which confine and give it a meaning, inquiring about the place of their creation and experience. Therefore, this work followed two prominent directions: the study of the confining action of the state, which is seen as the author of the regional boundaries in Portugal and the study of the discursive production of regional media, agents and expression of regional identity. The second dimension would require a rigorous study on the regional press, essential object of this work and from which it is possible to achieve valid conclusions for the period between 1959 and 2011. The crossing of these two dimensions allowed us to understand the existence of regional identity features being sharply enforced and deeply entrenched to the Estado Novo speech. These features rarely did correspond to the delimitate attempts of the subsequent successive government initiatives and thus reveal their profound detachment of that territory, which is continually being divided by them. Between the riddled with symbolism – although centralist – Estado Novo speech and the technical denominations - albeit with the aim of promoting the power decentralization – of democracy, the identity speech in today's Viseu regional press still feeds on the first. Not because they share the same goals, but because it is what best characterizes a relatively pacified identity: always ready to use the "beirão" heritage to state their differences, although the spirit of the nation has never called into question; always ready to denounce the Lisbon centralism, even though forgetting that this centralism was the one that once gave them the genuineness feature of, whatever it is, being Portuguese.

Introduction : Myeloproliferative neoplasms (MPN), namely polycythaemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) have been associated with an increased risk of thrombotic and haemorrhagic events, mainly when januskinase-2 (JAK 2) V617F mutation is detected1. This association is higher for thrombotic events (TE) contributing for overall mortality in MPN patients, although the pathophysiological mechanisms remain unclear. Although age and history of thrombosis have been considered the most important factors for thrombosis risk assessment, cardiovascular risk factors have recently been included in prognostic scores for thrombosis in individuals with MPN. Aim : The primary end-point of this study was to evaluate the prevalence of thrombotic and haemorrhagic events in a population with MPN (PV, ET, PMF) harbouring the JAK-2 V617F mutation. A secondary end-point was to identify possible risk factors for these events. Methods: We retrospectively analysed the records of MPN patients with JAK-2 V617F mutation, identified between January 2010 and September 2013. Variables analysed included blood counts, gender, age at diagnosis, therapy, vascular events and cardiovascular risk (body mass index obesity-defined (WHO), diabetes, dyslipidemia, hypertension and smoking status). Multiple linear regression analysis was performed to identify the variables that best predict thrombosis after controlling for potential confounders. Variables significant in the univariate analysis were included in the regression. Analysis was performed using the SPSS version 22.0 (SPSS, Chicago, IL, USA). p-value lower than .05 was considered significant. Results: Excluding blood counts, MPN subgroups did not differ considering the studied variables, namely, thrombotic and bleeding events. In this MPN JAK-2 V617F mutated population (N=99; PV:38, ET:56, PMF:5), the prevalence of thrombotic and bleeding events was 35.4% (PV: 42.1%; ET: 30.4%; PMF: 40%) and 19.2% (PV: 15.8%; ET: 17.9%; PMF: 60%), respectively. With patients having more than one TE (1-5), there were 49 episodes, presented in either arterial (53.1%) or venous (46.9%) territories. The commonest sites of arterial thrombosis (N=26) were cerebral (50%), coronary (15.4%), peripheral arteries (15.4%) and ophthalmic (11.5%); venous thrombosis locations (N=23) were deep venous thrombosis (31.1%), superficial venous thrombosis (21.7%), pulmonary embolism (17.4%) and splanchnic (17.4%) (3 portal and 1 splenic). Prediction of TE was explained in 75% by a model including the number of thrombosis and obesity [R2 =.747; F (2, 36) = 53.255; p Bleeding episodes (N=28) occurred mostly under antiplatelet and/or anticoagulant therapy (N=22) and local/traumatic causes were identified in 42.9% of the events. In 2 haemorrhagic cases platelet blood count was >1x106/mL. The commonest affected sites were gastrointestinal tract (21.4%), nasal (21.4%), subdural (14.3%). In our study population, mortality was associated to older age (p= .002), male gender (p=.026), smoking (p=.007) and number of TE (p=.029). Mortality was found to be correlated with venous but not with arterial thrombosis (r=0.232, p=.021; r=0.105, p=.299, respectively). Conclusions and Discussion: In this MPN JAK-2 V617F mutated population we found higher prevalence of thrombotic events then bleeding episodes. The prediction of TE was explained in 75% by a model that includes the number of thrombosis episodes and obesity. Since JAK-2 V617F is a driver mutation in MPN acting through JAK/STAT inflammatory pathway and the finding of a TE prediction model that includes obesity, a chronic inflammatory condition, the present study underlies: 1) the importance of inflammation in the pathophysiology of thrombosis-MPN associated; 2) the relevance of the recent inclusion of cardiovascular risk factors as variables in prognostic scores for thrombosis in individuals with MPN. References Falchi L, Kantarjian HM, Verstovsek S (2017). Assessing the thrombotic risk of patients with essential thrombocythemia in the genomic era. Leukemia 31, 1845-1854. Disclosures Silva: Gilead Sciences: Research Funding; Abbvie, Gilead Sciences, Janssen, BMS: Consultancy, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Roche, Janssen, Celgene: Other: Travel Support; Roche, Janssen: Other: Institution's payment for consultancy.

We describe a case of a patient with CD34+, TdT+, CD13-, CD33-, MPO- undifferentiated acute leukemia who refused chemotherapy and who achieved complete hematological remission 14 months after the diagnosis, during a short course of granulocyte-colony stimulating factor (G-CSF) for neutropenia and life threatening infection. Relapse occurred approximately one year later and G-CSF was reintroduced, being maintained for 4 months, at a dose and frequency adapted to maintain normal blood counts, a complete hematological remission being achieved again. Five months after withdrawing the G-CSF therapy a second relapse was observed; G-CSF was tried again with success, resulting in a very good hematological response that was sustained by G-CSF maintenance therapy. One year latter there was the need of increasing the doses of G-CSF in order to obtain the same hematological effect, at same time blast cells acquired a more mature CD34+, TdT-, CD13+, CD33-, MPO+ myeloid phenotype. Finally, the patient developed progressive neutropenia, anemia, thrombocytopenia and acute leukemia in spite of G-CSF therapy, dying 64 months after initial diagnosis (50 months after starting G-CSF therapy) with overt G-CSF resistant acute myeloblastic leukemia (AML), after failure of conventional induction chemotherapy.

Fanconi anaemia (FA) is an inherited disorder characterized by chromosomal instability. The phenotype is variable, which raises the possibility that it may be affected by other factors, such as epigenetic modifications. These play an important role in oncogenesis and may be pharmacologically manipulated. Our aim was to explore whether the epigenetic profiles in FA differ from non-FA individuals and whether these could be manipulated to alter the disease phenotype. We compared expression of epigenetic genes and DNA methylation profile of tumour suppressor genes between FA and normal samples. FA samples exhibited decreased expression levels of genes involved in epigenetic regulation and hypomethylation in the promoter regions of tumour suppressor genes. Treatment of FA cells with histone deacetylase inhibitor Vorinostat increased the expression of DNM3T beta and reduced the levels of CIITA and HDAC9, PAK1, USP16, all involved in different aspects of epigenetic and immune regulation. Given the ability of Vorinostat to modulate epigenetic genes in FA patients, we investigated its functional effects on the FA phenotype. This was assessed by incubating FA cells with Vorinostat and quantifying chromosomal breaks induced by DNA cross-linking agents. Treatment of FA cells with Vorinostat resulted in a significant reduction of aberrant cells (81\% on average). Our results suggest that epigenetic mechanisms may play a role in oncogenesis in FA. Epigenetic agents may be helpful in improving the phenotype of FA patients, potentially reducing tumour incidence in this population. publishersversion published

Objective: Blood use in the perioperative period has been changing over time. The increasing costs of transfusions led to a transformation in the clinical practice. In our institution until April 2012 all patients who underwent cerebral aneurysm clipping surgery had at least 2 units of RBCs (red blood cells) cross-matched before surgery and from then on only type and screen is performed. This study assesses the policy changes and the costs associated with blood use in cerebral aneurysm clipping surgery. Methods: Records of 206 patients who underwent cerebral aneurysm clipping surgery at Centro Hospitalar do Porto in Portugal were retrospectively reviewed between January 2011 and July 2014. Results: A total of 206 patients underwent cerebral aneurysm clipping surgery during the reviewed period. The patients were separated in two groups: cross-match group and type and screen group.104 patients had its blood cross-matched and 102 had it typed and screened. There was a trend to transfuse less when the blood was typed and screened (6% vs 13%). The cross-match group presented average losses of 1,8 units of blood per surgery which represented 188 units of blood that were not used. In this group all the 104 patients had blood prepared, and only 13 (12,5%) used the blood. In the type and screen group the blood was prepared in 13 cases and used only in 6 cases (46%). The financial analyses revealed that typing and screening cost about 48% less than cross-matching. This means that if the type and screen method was used since the beginning of the period under analysis, the hospital would have had a saving of 1022€. Conclusion: Routine type and screen should be made for every patients proposed for aneurysm clipping surgery. Financial benefits with the reduction of transfusion costs would lead to a better management of our blood products.

Submitted by deniseoa@stj.jus.br (deniseoa@stj.jus.br) on 2018-01-11T16:54:17Z No. of bitstreams: 2 caso_reley_california_paschoal.pdf: 517616 bytes, checksum: 23ebbb9b237d2ef322a0553780a7167d (MD5) license.txt: 1239 bytes, checksum: c9b4c351324448672315a00808efb725 (MD5) Approved for entry into archive by allanr@stj.jus.br (allanr@stj.jus.br) on 2018-01-31T17:00:09Z (GMT) No. of bitstreams: 2 caso_reley_california_paschoal.pdf: 517616 bytes, checksum: 23ebbb9b237d2ef322a0553780a7167d (MD5) license.txt: 1239 bytes, checksum: c9b4c351324448672315a00808efb725 (MD5) Made available in DSpace on 2018-01-31T17:00:09Z (GMT). No. of bitstreams: 2 caso_reley_california_paschoal.pdf: 517616 bytes, checksum: 23ebbb9b237d2ef322a0553780a7167d (MD5) license.txt: 1239 bytes, checksum: c9b4c351324448672315a00808efb725 (MD5) Previous issue date: 2015

Transformation of chronic myeloid leukemia (CML) often results in acute myeloblastic or, less frequently, in precursor B-cell acute lymphoblastic leukemia (ALL). T-cell blast crisis is rare. Hypercalcemia has also been described as a rare complication of CML, but this usually occurs as a terminal event. Here we report a case of a 35-year-old woman who developed a CD4(+)/CD8(+) T-cell ALL 2 years after the diagnosis of a typical Ph(+) CML. Polymyositis and polyarthritis preceded by 4 months, and symptomatic hypercalcemia occurred just before blastic transformation, probably representing paraneoplastic manifestations of the disease.

This case report details a single patient with pure red cell aplasia (PRCA) associated with clonal CD3+, TCRαβ+, TCR-Vβ8+, CD8+, CD57+ large granular lymphocytosis whose anaemia did not respond to conventional immunosuppressive therapy but did respond to cyclosporin A (CsA). The patient has become dependent on CsA for 7 years in order to control anaemia due to associated PRCA.