Tratamento Cirúrgico dos Defeitos do Septo Auriculo-Ventricular. Experiência Uni-Institucional

OBJECTIVES: Atrio-ventricular septal (AVSD) defects include a variable spectrum of congenital malformations with different forms of clinical presentation. We report the surgical results, from a single institution, with this type of congenital cardiac malformation. Patients with hypoplasia of one of the ventricles were excluded from this analysis. POPULATION: Between November of 1998 and June of 2005, 49 patients with AVSD were operated on by the same team and in the same department. The average age was 37.3 months (medium 6 months) and 31 patients were female. In 38 patients (78%) an inter-ventricular communication was present (AVSD-complete) and of these, 26 were of the type A of Rastelli, being 13 of type B or C. The age for defect correction of the complete form was of 5.5 months, palliative surgery was not carried out on any of the patients. Associated lesions included: Down's syndrome in 22 patients (45%), patent arterial duct in 17 patients (35%), severe AV regurgitation in 4 patients (8%), tetralogy of Fallot in two (4%) and sub-aortic stenosis in one patient (2%). Pre-operatively 10 patients presented severe congestive heart failure and two were mechanically ventilated. RESULTS: Complete biventricular correction was carried out in all patients. The average time on bypass (ECC) was 74.1+/-17.5 min. and time of aortic clamping was 52.0+/-12.9 min. The complete defects were corrected by the double patch technique, and in all patients the mitral cleft was closed, except in two with single papillary muscle. There was no intra-operative mortality, but hospital mortality was 8%(4 patients), due to pulmonary hypertension crises, in the first 15 post-operative days. The mean ventilation time was of 36.5+/-93 hours (medium 7 h) and the average ICU stay was of 4.3+/-4.8 days (medium 3 days). The minimum follow-up period is 1 month and the maximum is 84 months (medium 29.5 months), during which time 4 re-operations (8%) took place: two for residual VSD's and two for mitral regurgitation. There was no mortality at re-do surgery. At follow up there was residual mitral regurgitation, mild in 17 patients and moderate in two. Four other patients presented with minor residual defects. CONCLUSIONS: The complete correction of AVSD can be carried out with acceptable results, in a varied spectrum of anatomic forms and of clinical severity. Despite the age of correction, for the complete forms, predominantly below 12 months, pulmonary hypertension was the constant cause for post operative mortality. Earlier timing of surgery and stricter peri-operative control might still improve results.

Impacto da Idade no Tratamento e Resultados Após Enfarte Agudo do Miocárdio em Particular nos Muito Idosos

INTRODUCTION: The elderly population admitted for acute myocardial infarction is increasing. This group is not well studied in international trials and is probably treated with a more conservative approach. OBJECTIVES: To evaluate the presentation and treatment of myocardial infarction according to age, particularly in very elderly patients. METHODS: We studied 1242 consecutive patients admitted with acute myocardial infarction, assessing in-hospital, 30-day and one-year mortality during follow-up for each age-group. Patients were divided into four groups according to age: <45 years (7.6%); 45-64 years (43.3%); 65-74 years (23.4%); and ≥75 years (25.7%). RESULTS: Elderly patients had a worse risk profile (except for smoking), more previous history of coronary disease and a worse profile on admission, with the exception of lipid profile, which was more favorable. With regard to treatment of the elderly, although less optimized than in other age-groups, it was significantly better compared to other registries, including for percutaneous coronary angioplasty. Both complications and mortality were worse in the older groups. In elderly patients (≥75 years), adjusted risk of mortality was 4.9-6.3 times higher (p<0.001) than patients in the reference age-group (45-64 years). In these patients, the independent predictors of death were left ventricular function and renal function, use of beta-blockers being a predictor of survival. CONCLUSIONS: Elderly patients represent a substantial proportion of the population admitted with myocardial infarction, and receive less evidenced-based therapy. Age is an independent predictor of short- and medium-term mortality.

Infecções Urinárias e Gravidez - Diagnóstico, Terapêutica e Prevenção

Objectives: Literature review of classification, epidemiology, pathophysiology, microbiology, clinical presentation, diagnosis, complications, treatment and prevention of urinary tract infections (UTI) in pregnancy. Data Sources and Review Methods: Bibliographic research in Medline, through PubMed and Medscape, of systematic reviews, observational studies, clinical guidelines, meta-analyses and randomized controlled trials published between January 1992 and December 2010. Results: Asymptomatic bacteriuria occurs in 2 to 10% of pregnant women, 30% of which will develop acute pyelonephritis if left untreated. Treatment of asymptomatic bacteriuria has been shown to reduce the risk of pyelonephritis in pregnancy. Therefore, screening and treatment of this form of UTI has become a standard of obstetrical care, although the ideal duration of the treatment is still controverse. Acute pyelonephritis is one of the most common medical complications of pregnancy and may be associated with maternal, obstetric and perinatal complications. There is no clear consensus in the literature regarding antibiotic choice or duration of therapy for symptomatic UTI. Increasing antibiotic resistance complicates the choice of empirical regimens and local resistance rates need to be taken into account when deciding the therapy. Considering the high rate of recurrence of UTI during pregnancy, prophylactic measures need to be taken in pregnant women who have already experienced UTI during their current pregnancy. Conclusions: Although UTI is a generally benign condition in non-pregnant women, it may be a potentially serious complication during pregnancy. Early diagnosis and treatment of UTI during pregnancy are mandatory and can prevent severe maternal and perinatal complications.

Lymph scrotum: an unusual urological presentation of lymphatic filariasis. A case series study

Lymphatic filariasis (LF) causes a wide range of clinical signs and symptoms, including urogenital manifestations. Transmission control and disability/morbidity management/control are the two pillars of the overall elimination strategy for LF. Lymph scrotum is an unusual urological clinical presentation of LF with important medical, psychological, social and economic repercussions. A retrospective case series study was conducted on outpatients attended at the National Reference Service for Filariasis, in an endemic area for filariasis (Recife, Brazil), between 2000 and 2007. Over this period, 6,361 patients were attended and seven cases with lymph scrotum were identified. Mean patient age was 45 years (range, 26 to 64 years). Mean disease duration was 8.5 years (range, two to 15 years). All patients had evidence of filarial infection from at least one laboratory test (parasitological, antigen investigation or "filarial dance sign" on ultrasound). Six patients presented histories of urological surgery. The authors highlight the importance of the association between filarial infection and the inadequate surgical and clinical management of hydrocele in an endemic area, as risk factors for lymph scrotum. Thus, filarial infection should be routinely investigated in all individuals presenting urological morbidity within endemic areas, in order to identify likely links in the transmission chain.

The development and implementation of math projects in a HEI: expectations, objectives, experiences and analysis

An overwhelming problem in Math Curriculums in Higher Education Institutions (HEI), we are daily facing in the last decade, is the substantial differences in Math background of our students. When you try to transmit, engage and teach subjects/contents that your “audience” is unable to respond to and/or even understand what we are trying to convey, it is somehow frustrating. In this sense, the Math projects and other didactic strategies, developed through Learning Management System Moodle, which include an array of activities that combine higher order thinking skills with math subjects and technology, for students of HE, appear as remedial but important, proactive and innovative measures in order to face and try to overcome these considerable problems. In this paper we will present some of these strategies, developed in some organic units of the Polytechnic Institute of Porto (IPP). But, how “fruitful” are the endless number of hours teachers spent in developing and implementing these platforms? Do students react to them as we would expect? Do they embrace this opportunity to overcome their difficulties? How do they use/interact individually with LMS platforms? Can this environment that provides the teacher with many interesting tools to improve the teaching – learning process, encourages students to reinforce their abilities and knowledge? In what way do they use each available material – videos, interactive tasks, texts, among others? What is the best way to assess student’s performance in these online learning environments? Learning Analytics tools provides us a huge amount of data, but how can we extract “good” and helpful information from them? These and many other questions still remain unanswered but we look forward to get some help in, at least, “get some drafts” for them because we feel that this “learning analysis”, that tackles the path from the objectives to the actual results, is perhaps the only way we have to move forward in the “best” learning and teaching direction.

Sistema de recrutamento e seleção num grande grupo empresarial

Este Projeto de Intervenção visou a reestruturação do sistema de Recrutamento e Seleção para o Grande Grupo Empresarial tendo em conta a realidade das várias Empresas. Este projeto surgiu da necessidade de reconfiguração da visão, filosofia e estrutura da Direção de Pessoas e Comunicação (DPC) e pretendeu desenvolver uma proposta de valor aliciante para os talentos atuais e potenciais do mercado e da Organização, numa lógica de prestação de serviços às seis empresas do Grande Grupo Empresarial, designadas por grupo incubador, que foram o público-alvo deste projeto. Numa primeira fase, foi realizada uma breve apresentação do Grande Grupo Empresarial, seguindo-se de uma auditoria às práticas de Recursos Humanos e uma revisão da literatura ao tema identificado para intervenção deste projeto. Baseado na conjugação das análises realizadas anteriormente, definiu-se a finalidade do projeto que consistiu em garantir que, a partir de Janeiro de 2016, o Grande Grupo Empresarial tenha definido um Sistema de Recrutamento e Seleção adequado à estratégia de comunicação da DPC. Esta finalidade foi desdobrada em dois objetivos estratégicos: Concluir a preparação dos novos procedimento de Recrutamento e Seleção para implementação e Concluir a preparação das ferramentas de Recrutamento e Seleção para implementação e foi desenvolvido todo o planeamento operacional, onde se delinearam atividades, tarefas, recursos, calendarização, orçamento e o momento da avaliação do projeto. A fase de implementação teve a duração de 5 meses (Junho a Outubro de 2015) e a sua avaliação realizou-se durante e no final da implementação. Os resultados confirmam o cumprimento dos objetivos operacionais, estratégicos e da finalidade a que este projeto se propôs. A avaliação inclui o feedback obtido da equipa de Recrutamento e Seleção ao longo do desenvolvimento do projeto. A apreciação final por parte de todos os envolvidos é encorajadora para a adoção de uma nova metodologia de trabalho que permitirá à organização a captação e potenciação de talentos internos.

Sulfite Oxidase Deficiency – An Unusual Late and Mild Presentation

Introduction: Sulfite oxidase deficiency (SOD) is an autosomal recessive inherited disease usually presenting in the neonatal period with severe neurological symptoms including seizures, often refractory to anticonvulsant therapy, and a rapidly progressive encephalopathy resembling neonatal hypoxic ischemia, with premature death. Most patients develop dislocated ocular lenses. Later or milder presentations of SOD are being reported with increasing frequency. These presentations include neurological regression with loss of previously acquired milestones or movement disorders. Case report: We report a four years old girl presenting with intermittent ataxia and uncoordinated limb movements. A similar episode of ataxia had occurred previously, one year before, with complete neurologic recovery and normal developmental milestones. Bilateral lens dislocation had been recently diagnosed. Cranial MRI demonstrated bilateral globus pallidus enhancement. Low homocysteine was found in plasma and SulfitestR was positive. Further investigations led to confirmation of isolated sulfite oxidase deficiency with no enzyme activity detected on skin fibroblasts culture. Discussion: This case illustrates the clinical variability of SOD and it is not only atypical but also seems to be the mildest form described so far. The association of ectopia lentis with a movement disorder, even without psychomotor regression, should prompt us to look for this diagnosis.

AMERICAN CUTANEOUS LEISHMANIASIS WITH UNUSUAL CLINICAL PRESENTATION AND RESPONSE TO TREATMENT

The clinical manifestations and prognosis of cutaneous leishmaniasis (CL) can be influenced by the immune response of the patient and the species of the parasite. A case of atypical clinical presentation of CL, with development of non-characteristic lesions, poor response to therapy, and a long time to resolution is reported. Confirmatory laboratory tests included parasite detection, indirect immunofluorescence, Montenegro skin test, polymerase chain reaction, and parasite identification by multilocus enzyme electrophoresis. The parasite was identified as Leishmaniabraziliensis. The lesion was unresponsive to three complete courses of N-methylglucamine antimoniate intramuscular, and to treatment with pentamidine. The patient did not tolerate amphotericin B. The lesion finally receded after treatment with intravenous N-methylglucamine antimoniate. It is essential to ensure the accuracy of diagnosis and the appropriate treatment, which can include the use a second choice drug or a different route of administration.

Unilateral Oral Mucous Membrane Pemphigoid: Refractory Atypical Presentation Successfully Treated with Intravenous Immunoglobulins

A 57-year-old male presented with a 6-month history of blisters and painful erosions on the right buccal mucosa. No skin or other mucosal involvement was seen. The findings of histopathological and direct immunofluorescence examinations were sufficient for the diagnosis of oral mucous membrane pemphigoid in the context of adequate clinical correlation. No response was seen after topical therapies and oral corticosteroids or dapsone. Intravenous immunoglobulin was started and repeated every three weeks. Complete remission was achieved after three cycles and no recurrence was seen after two years of follow-up. The authors report a rare unilateral presentation of oral mucous membrane pemphigoid on the right buccal and hard palate mucosa, without additional involvement during a period of five years. Local trauma or autoimmune factors are possible etiologic factors for this rare disorder, here with unique presentation.

Porokeratosis Ptychotropica of the Scrotum: Dermoscopic Evaluation of an Atypical Presentation

Porokeratosis ptychotropica is a rare variant of porokeratosis that is classically located on the gluteal and perianal regions, seldom extending to the genitalia. The authors report an atypical presentation of porokeratosis ptychotropica and discuss the use of dermoscopy in evaluating this dermatosis. Dermoscopic findings, although not specific to this variant of porokeratosis, are helpful in the differential diagnosis of other genital disorders. Histopathology, through the visualization of multiple cornoid lamellae, prevails as the gold standard for the definite diagnosis of porokeratosis ptychotropica.

Does direct cash flow presentation help in predicting future operating cash flow?

A Work Project, presented as part of the requirements for the Award of a Masters Degree in Finance from the NOVA – School of Business and Economics

Acute Upper Limb Ischemia, a Rare Presentation of Giant Cell Arteritis

Giant cell arteritis (GCA) is a systemic large vessel vasculitis, with extracranial arterial involvement described in 10-15% of cases, usually affecting the aorta and its branches. Patients with GCA are more likely to develop aortic aneurysms, but these are rarely present at the time of the diagnosis. We report the case of an 80-year-old Caucasian woman, who reported proximal muscle pain in the arms with morning stiffness of the shoulders for eight months. In the previous two months, she had developed worsening bilateral arm claudication, severe pain, cold extremities and digital necrosis. She had no palpable radial pulses and no measurable blood pressure. The patient had normochromic anemia, erythrocyte sedimentation rate of 120 mm/h, and a negative infectious and autoimmune workup. Computed tomography angiography revealed concentric wall thickening of the aorta extending to the aortic arch branches, particularly the subclavian and axillary arteries, which were severely stenotic, with areas of bilateral occlusion and an aneurysm of the ascending aorta (47 mm). Despite corticosteroid therapy there was progression to acute critical ischemia. She accordingly underwent surgical revascularization using a bilateral carotid-humeral bypass. After surgery, corticosteroid therapy was maintained and at six-month follow-up she was clinically stable with reduced inflammatory markers. GCA, usually a chronic benign vasculitis, presented exceptionally in this case as acute critical upper limb ischemia, resulting from a massive inflammatory process of the subclavian and axillary arteries, treated with salvage surgical revascularization.

Human mucocutaneous leishmaniasis in Três Braços, Bahia - Brazil: an area of Leishmania braziliensis braziliensis transmission. II. Cutaneous disease. Presentation and evolution

The clinical records of 182 patients with cutaneous leishmaniasis probably due to Leishmania braziliensis braziliensis are analysed. 68% had a single lesion which was usually an ulceron the lower anterior tibial third. Many had short histories of one to two months and all age groups were represented 13% had closed lesions of a verrucose or plaque like nature. Evolution of these skin lesions after treatment was related to the regularity of antimony therapy. Although healing usually occurred in three months, the time to scarring after commencing treatment was variable and related to the size ofthe lesion (p < 0.01). Usually if sufficient antimony treatment was given the lesion closed. Seven of the ten patients with initially negative leishmanin skin tests converted to positive after treatment. A significant decline of indirect fluorescent antibody titres occurred in patients followed, during and after therapy.