Browsing by Issue Date, starting with "2012"
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- Buschke-Lowenstein TumorPublication . Pinto, A.; Guedes-Martins, L.; Marques, C.; Cabral, J.Introduction: Giant condyloma acuminatum belongs to a spectrum of diseases with malignant degeneration. Clinically, it presents as exophytic, fungating masses, sometimes with a cauliflower-like morphology. Case presentation: We present a case of a 32-year-old female patient with a 180x95x80mm exophytic mass of the vulvar region suggestive of Buschke-Lowenstein Tumour. Treatment included wide local excision with electrosurgery and CO2 vaporization of recurrent focal lesions. Histopathological analysis confirmed the expected diagnosis. Surgery went without complications and the patient is lesion-free at the 12th month of follow-up. Conclusion: There is a lack of consistent trials regarding optimal treatment of BLT. Surgery, when feasible, remains the mainstay of treatment. It allows quick lesion size reduction, with fewer side effects and more rapid return to daily living activities, when compared to other treatment options.
- Anafilaxia induzida por fármacos: Registo Nacional 2007-2010Publication . Faria, E.; Rodrigues-Cernadas, J.; Gaspar, A.; Botelho, C.; Castro, E.; Lopes, A.; Gomes, E.; Malheiro, D.; Cadinha, S.; Campina-Costa, S.; Neto, M.; Sousa, N.; Rodrigues-Alves, R.; Romeira, A.; Caiado, J.; Morais-Almeida, M.Introdução: A anafilaxia a fármacos constitui uma situação potencialmente fatal e imprevisível, desconhecendo-se a real prevalência em diferentes grupos populacionais e os factores de risco relacionados.Objectivo: Contribuir para o melhor conhecimento epidemiológico da anafilaxia induzida por fármacos no nosso país. Métodos: Durante um período de 4 anos (Janeiro de 2007 a Dezembro de 2010) foi implementado um sistema de notificação nacional de anafilaxia, focalizado na notificação voluntária por clínicos com diferenciação em patologia imunoalérgica. Foram recebidas e analisadas notificações de anafilaxia a fármacos de 313 doentes. No estudo estatístico foram aplicados testes de distribuição e análise de regressão logística múltipla para obter significância e coeficientes de regressão e efeitos marginais. Resultados: A média de idade foi de 43,8 ±17,4 anos, sendo 8% de idade inferior a 18 anos. A relação género feminino/masculino foi de 2/1. A média de idade do primeiro episódio foi de 39 ±18,2 anos. Nove doentes apresentaram mais que uma causa de anafilaxia, correspondendo a um total de 322 notificações de grupos de fármacos envolvidos. As principais causas da anafilaxia a fármacos foram os anti-inflamatórios não esteróides (AINEs), os antibióticos e os agentes anestésicos, com respectivamente 48%, 36% e 6% dos casos. Outros fármacos implicados foram citostáticos, corticosteróides, inibidores da bomba de protões e meios de contraste iodados, entre outros. Houve predomínio de manifestações mucocutâneas (92%), seguido de respiratórias (81%) e de cardiovasculares (49%). Os doentes com anafilaxia a AINEs apresentaram aumento significativo da associação de manifestações mucocutâneas e respiratórias. Não foram observadas diferenças significativas em idade, género ou antecedentes de atopia entre os diferentes grupos de fármacos envolvidos. As reacções ocorreram em ambiente hospitalar em 45% dos casos. Em 53% nos 15 minutos após a administração do fármaco e 35% motivaram internamento. A recorrência da anafilaxia foi observada em 26% e o risco foi significativamente mais elevado nos casos de anafilaxia a AINEs. Apenas 48% dos doentes receberam tratamento com adrenalina e somente em 9% dos casos foi prescrito dispositivo para auto-administração de adrenalina. Conclusões: Neste estudo os AINEs foram os fármacos mais frequentes e os mais associados a recorrência de anafilaxia. Destaca -se o sub-tratamento com adrenalina e a necessidade de serem tomadas medidas no sentido do tratamento eficaz e da prevenção da recorrência de anafilaxia a fármacos.
- Pericardial and pleural effusions associated with sirolimus and discussion of possible mechanismsPublication . Rocha, S.; Pedroso, S.; Almeida, M.; Dias, L.; Martins, L.; Castro-Henriques, A.; Cabrita, A.Sirolimus, a mammalian target of rapamycin inhibitor, is an increasingly used immunosuppressant in solid-organ transplantation. There are an increasing number of reports of unusual oedematous adverse effects associated with this drug, including lymphoedema, ascites and pleural effusions, and a few reports of pericardial effusions. No pathophysiological explanation for these phenomena has been disclosed. We report a 33-year-old sirolimus-treated kidney transplant recipient with chronic pericardial and pleural effusions identified nine years after transplantation. He was initially treated for a presumed tuberculous pericarditis, even though cultures for Mycobacterium tuberculosis were negative. After 12 months of antitubercular therapy, visceral effusions persisted. Pericardial effusion was drained and stabilised. After exclusion of other causes, sirolimus toxicity was considered the most likely cause. Two months after discontinuation of sirolimus, visceraleffusions disappeared. Interaction of mammalian target of rapamycin inhibitors with mediators of lymphangiogenesis may be a common link in oedematous states associated with sirolimus.
- Nutritional requirements of the critically ill patientPublication . Costa, N.; Marinho, A.; Cançado, L.Objective: Given the inaccessibility of indirect calorimetry, intensive care units generally use predictive equations or recommendations that are established by international societies to determine energy expenditure. The aim of the present study was to compare the energy expenditure of critically ill patients, as determined using indirect calorimetry, to the values obtained using the Harris-Benedict equation. Methods: A retrospective observational study was conducted at the Intensive Care Unit 1 of the Centro Hospitalar do Porto. The energy requirements of hospitalized critically ill patients as determined using indirect calorimetry were assessed between January 2003 and April 2012. The accuracy (± 10% difference between the measured and estimated values), the mean differences and the limits of agreement were determined for the studied equations. Results: Eighty-five patients were assessed using 288 indirect calorimetry measurements. The following energy requirement values were obtained for the different methods: 1,753.98±391.13 kcal/ day (24.48 ± 5.95 kcal/kg/day) for indirect calorimetry and 1,504.11 ± 266.99 kcal/day (20.72±2.43 kcal/kg/day) for the HarrisBenedict equation. The equation had a precision of 31.76% with a mean difference of -259.86 kcal/day and limits of agreement between -858.84 and 339.12 kcal/day. Sex (p=0.023), temperature (p=0.009) and body mass index (p< 0.001) were found to significantly affect energy expenditure Conclusion: The Harris-Benedict equation is inaccurate and tends to underestimate energy expenditure. In addition, the Harris-Benedict equation is associated with significant differences between the predicted and true energy expenditure at an individual level
- Langerhans cell histiocytosis: Two clinical presentations in the same patientPublication . Oliveira, A.; Pinto-Almeida, T.; Lobo, I.; Machado, S.; Selores, M.Langerhans cell histiocytosis (LCH) is a heterogeneous group of diseases characterized by a pathological proliferation of cells phenotypically similar to Langerhans cells. The disease course is variable, alternating between resolving and potentially fatal forms. The diagnosis is based on clinical appearance and confirmed by CD1a positivity and / or immunohistochemistry. We report the case of a male child of 3 months with two different presentations of Langerhans cell-histiocytosis (LCH) at different times. The first presentation was classified as a self-healing LCH (formerly known as Hashimoto-Pritzker). The last presentation, although clinically suggestive of Letterer-Siwe (former designation), was not associated with systemic disease. This emphasizes that LCH cannot be compartmentalized into four groups, but considered a single disease with a wide spectrum of clinical presentations. This case underscores the importance of frequent and long-term follow-up of these patients.
- Atypical haemolytic-uraemic syndrome caused by factor H mutation: case report and new management strategies in childrenPublication . Araújo, L.; Faria, M.; Rocha, L.; Costa, T.; Barbot, J.; Mota, C.Atypical haemolytic uraemic syndrome is causedby alternative complement pathway dysregulation. It has recently been recognised that most cases are due to genetic factors and a growing list of mutations has been described. Atypical haemolytic uraemic syndrome is associated with a dismal prognosis, a relapsing course, high acute mortality and frequent progression to end-stage renal disease. We describe a five-year-old boy admitted with a first recurrence of atypical haemolytic uraemic syndrome. The primary onset of the disease was at 15 months of age, following which there was complete recovery of haematological and renal parameters. His family history was significant in that his mother had died at the age of only 23 years of a stroke with associated thrombotic microangiopathy, suggesting a familial form of the disease. Sequencing of the gene encoding complement factor H revealed a heterozygous SCR20 mutation (3644G>T, Arg1215Leu), confirming the diagnosis. The patient was successfully treated with fresh frozen plasma infusions that induced disease remission. We also review currently evolving concepts about atypical haemolytic uraemic syndrome caused by factor H mutation, its diagnosis, the role of genetic testing and management strategies in children.
- Vesico-bullous subacute cutaneous lupus erythematosus – An uncommon entity successfully treated with dapsone and hydroxychloroquinePublication . Pinto-Almeida, T.; Sanches, M.; Alves, R.; Selores, M.Vesico-bullous subacute cutaneous lupus erythematosus is an uncommon and severe presentation. The authors report an exuberant case of vesico-bullous subacute cutaneous lupus erythematosus successfully treated with dapsone and hydroxychloroquine
- Do you know this syndrome?Publication . Rosmaninho, A.; Pinto-Almeida, T.; Fernandes, I.; Machado, S.; Selores, M.Abstract Lipomas are among the most common tumors seen in the soft body parts and usually are solitary lesions. The authors report a case of a male patient that presented for evaluation of multiple subcutaneous nodules that caused important functional and cosmetic impairment. The diagnosis of familial multiple lipomatosis was made. Physicians should be able to recognize and characterize this rare disease.
- Arterial Spin Labeling: Experiência Inicial, Indicações Clínicas e DificuldadesPublication . Carneiro, A.; Pina, S.; Moreira, B.RESUMO O arterial spin labeling (ASL) é uma técnica de perfusão por ressonância magnética (RM) que usa os protões das moléculas de água do sangue arterial como marcador endógeno. As suas principais vantagens residem no facto de ser um método não invasivo, rápido e que dispensa a administração de contraste. Actualmente os seus resultados são reprodutíveis de modo robusto, o que o torna uma ferramenta cada vez mais utilizada na prática clínica. O objectivo deste trabalho é apresentar a nossa experiência inicial com o ASL, salientando os aspectos técnicos, as principais solicitações clínicas, os resultados obtidos e as dificuldades experimentadas. Métodos: Foi efectuada uma revisão dos exames realizados durante um período de oito meses, usando uma técnica de ASL pulsado, num aparelho de 3T. A avaliação dos mapas de perfusão foi realizada de modo qualitativo. Resultados: As principais indicações clínicas para a realização de ASL foram epilepsia, doenças neuro-degenerativas e tumores intra-parenquimatosos. Embora o ASL não tenha sido, em nenhum dos casos, a principal ferramenta diagnóstica, contribuiu, por vezes, com dados fisiológicos importantes para o diagnóstico e para a orientação terapêutica. Salientam-se os casos de doentes com múltiplas crises epilépticas nos quais foi possível identificar focos de hiperperfusão pós-ictal (cujos resultados foram concordantes com o SPECT). Destacam-se ainda casos de doenças neuro-degenerativas nos quais o ASL identificou áreas de hipoperfusão típicas das respectivas entidades nosológicas. As principais dificuldades estiveram relacionadas com o carácter qualitativo da avaliação e com a valorização clínica dos achados. Conclusão: O estudo da perfusão cerebral por ASL tem um potencial diagnóstico importante. Com este trabalho mostramos que, com uma aquisição rápida e pós-processamento simples, pode facilmente integrar os estudos de RM de rotina. Abstract Arterial spin labelling (ASL) is a MR perfusion technique that uses protons from water molecules of the arterial blood as an endogenous tracer. It is fast, non-invasive and does not require gadolinium administration. Due to the increasing robustness of the results, it is becoming an important clinical tool. In this article we present our initial experience with ASL, highlighting some technical aspects, the main clinical applications, some achieved results and most important difficulties. Methods: Review of the examinations performed during eight months, using a pulsating ASL technique in a 3T machine. Perfusion maps were evaluated qualitatively. Results: The most frequent clinical applications were epilepsy, neurodegenerative disorders and tumours. Although perfusion data from ASL had never been crucial for diagnosis, it still provided substantial information. We highlight two epileptic patients who had had recent seizures, in which ASL depicted distinct post-ictal hyperperfusion areas (with the results being confirmed by SPECT studies). The impact was also remarkable in patients with neurodegenerative disorders in which ASL depicted hypoperfusion areas, typical of each nosological entity. The main difficulties were related to the lack of quantitative evaluation and to the clinical interpretation of the results obtained. Conclusion: ASL perfusion studies have a great potential in several clinical conditions. In this article we show that, with a fast acquisition and easy post-processing, it can integrate routine MRI examinations.