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Journal Cover   Acta Médica Portuguesa
  [SJR: 0.155]   [H-I: 11]   Follow
  This is an Open Access Journal Open Access journal
   ISSN (Print) 0870-399X - ISSN (Online) 1646-0758
   Published by Ordem dos Médicos Homepage  [1 journal]
  • Lingua Villosa Nigra Associated with the Use of Electronic Cigarette

    • Authors: Helder Farinha, Vera Martins
      Abstract: Keywords: Smoking; Tobacco Products; Tongue, Hairy.
      PubDate: 2015-06-30
      Issue No: Vol. 28 (2015)
  • Global Mental Health: What is Your Role in This Movement'

    • Authors: Mariana Pinto da Costa
      Abstract: Keywords: Global Health; Mental Disorders.
      PubDate: 2015-06-09
      Issue No: Vol. 28 (2015)
  • Cancer-Related Pain Management and the Optimal Use of Opioids

    • Authors: Paulo Reis-Pina, Peter G. Lawlor, António Barbosa
      Abstract: Pain relief is vital to the treatment of cancer. Despite the widespread use and recognition of clinical recommendations for the management of cancer-related pain, avoidable suffering is still prevalent in patients with malignant disease. A gap exists between what is known about pain medical management and actual practices of patients, caregivers, healthcare professionals and institutions. Opioids are the pillar of the medical management of moderate to severe pain. The prescription of opioid analgesics – by a registered medical practitioner for absolute pain control – is a legitimate practice. In this article we look at patients’ fears and physicians’ general
      hesitations towards morphine and alike. We examine misconceptions that yield fallacies on the therapeutically use of opioids and, therefore, sustain inadequate pain management.
      PubDate: 2015-06-08
      Issue No: Vol. 28 (2015)
  • Hansen Neuropathy: Still a Possible Diagnosis in the Investigation of a
           Peripheral Neuropathy

    • Authors: Andreia Veiga, Alexandre Costa, Ricardo Taipa, António Guimarães, Manuel Melo Pires
      Abstract: Introduction: Leprosy is still one of the most frequent causes of peripheral neuropathy. Although regarded as eradicated in Portugal, is still documented in neuropathological study of patients with clinical peripheral neuropathy without proper diagnosis.
      Material and Methods: Review of the cases of Hansen disease neuropathy diagnosed in Neuropathology Unit of Centro Hospitalar do Porto between 1978 and 2013, atending to gender, age, clinical manifestations and neuropathological findings.
      Results: Twenty one patients were identified with neuropathological diagnosis of Hansen’s disease neuropathy, predominantly male. The mean age at diagnosis was 52 years, and sensory symptoms predominate as neurological manifestation of disease. Interval between symptoms and diagnosis was 1-38 years. In most nerve samples tuberculoid type of disease was identified. Bacilli were detected in skin and nerve in 44% of cases.
      Discussion: Mononeuritis is the most common presentation of leprosy but other clinical manifestations are possible, including skin lesions. Infection with M. leprae injures myelinated and unmyelinated fibres, with replacement of nerve tissue by collagen fibrosis. The diagnosis of leprosy is only achieved by neuropathological study of skin lesions and / or peripheral nerve, supported by the identification of the bacillus.
      Conclusion: Hansen disease remains a public health problem in tropical areas and, although rare, still described in Western countries reason why should still be considered as a diagnostic possibility in the investigation of peripheral neuropathy.
      PubDate: 2015-06-08
      Issue No: Vol. 28 (2015)
  • Validation of the Portuguese Version of EHP-30 (The Endometriosis Health

    • Authors: Cristina Nogueira-Silva, Patrício Costa, Carla Martins, Sónia Barata, Conceição Alho, Carlos Calhaz-Jorge, Filipa Osório
      Abstract: Introduction: Endometriosis Health Profile Questionnaire-30 is currently the most used questionnaire for quality of life measurement in women with endometriosis. The aim of this study is to evaluate the psychometric properties and to validate the Portuguese Endometriosis Health Profile Questionnaire-30 version.
      Material and Methods: A sequential sample of 152 patients with endometriosis, followed in a Portugal reference center, were asked to complete a questionnaire on social and demographic features, the Portuguese version of the Endometriosis Health Profile Questionnaire-30 and of the Short Form Health Survey 36 Item – version 2. Appropriate statistical analysis was performed using descriptive statistics, factor analysis, internal consistency, item-total correlation and convergent validity.
      Results: Factorial analysis confirmed the validity of the five-dimension structure of the Endometriosis Health Profile Questionnaire-30 core questionnaire, which explained 83.2% of the total variance. All item-total correlations presented acceptable results and high internal consistency, with Cronbach’s alpha ranging between 0.876 and 0.981 for the core questionnaire and between 0.863 and 0.951 for the modular questionnaire. Significant negative associations between similar scales of Endometriosis Health Profile Questionnaire-30 and Short Form Health Survey 36 Item – version 2 were demonstrated. Data completeness achieved was high for all dimensions. The emotional well-being scale in the core questionnaire and the infertility scale in the modular section had the highest median scores, and therefore the most negative impact on the quality of life of participating women.
      Discussion: The test-retest reliability and responsiveness of the questionnaire should be evaluated in future studies.
      Conclusion: The present study demonstrates that the Portuguese version of the Endometriosis Health Profile Questionnaire-30 is a valid, reliable and acceptable tool for evaluating the health-related quality of life of Portuguese women with endometriosis.
      PubDate: 2015-06-05
      Issue No: Vol. 28 (2015)
  • Superwarfarine Poisoning

    • Authors: Ana Freixo, Luís Lopes, Manuela Carvalho, Fernando Araújo
      Abstract: The superwarfarin-type anticoagulant rodenticides are used throughout the world and distinguish themselves from warfarin for its high potency and long acting anticoagulant activity. Easy access to these products enables the accidental or deliberate human poisoning. A case of voluntary rodenticide poisoning (RATIBRON®) by a woman who ingested an estimated 27.5 mg of bromadiolone total quantity for two weeks, with minor bleeding episodes, whose reversal of the anticoagulant effect with the correction of the abnormal values of the clotting tests took about one month to reverse is reported here. The correction of the haemostasis defects takes usually a long time
      and there are no treatment guidelines, but a gradually vitamin K dosage reduction, as out patients, along with the monitoring of the International Normalized Ratio levels, allows a safe evaluation of the therapeutic response.
      PubDate: 2015-06-05
      Issue No: Vol. 28 (2015)
  • Urinary Tuberculosis: Serious Complications May Occur when Diagnosis is

    • Authors: Inês Sanches, Cláudia Pinto, Mário Sousa, Aurora Carvalho, Raquel Duarte, Manuel Pereira
      Abstract: Genitourinary tuberculosis is the third most common form of extrapulmonary tuberculosis, comprising 4 - 17% of extrapulmonary forms. The authors describe the case of a patient with recurrent urinary tract infections, without isolation of an infectious agent and without symptomatic resolution, despite antibiotic treatment. Imaging exams showed left ureteral stenosis with moderate hydronephrosis. The attempt of retrograde catheterization was impossible so we opted for percutaneous nephrostomy to renal relief. Microbiological urine
      analysis colleted by that way was positive for Mycobacterium tuberculosis complex. The patient started therapy with classical quadruple therapy and underwent nephrostomy for catheter placement. Despite therapeutic measures the patient required nephrectomy due to nonfunctioning kidney. Genitourinary tuberculosis is a diagnosis that should be considered in the presence of a persistent sterile pyuria.
      PubDate: 2015-06-04
      Issue No: Vol. 28 (2015)
  • Invasive Meningococcal Disease: Application of Base Excess and Platelets
           Score in a Portuguese Paediatric Intensive Care Unit

    • Authors: Luis Martins, Patrícia Mação, Carla Pinto, Teresa Dionísio, Andrea Dias, Alexandra Dinis, Leonor Carvalho, José Farela Neves
      Abstract: Introduction: Meningococcal infection has a high mortality and morbidity. Recently a new prognostic scoring system was developed for paediatric invasive meningococcal disease, based on platelet count and base excess – base excess and platelets score. The main objective of this study was to evaluate the accuracy of base excess and platelets score to predict mortality in children admitted to intensive care due to invasive meningococcal disease.
      Material and Methods: Observational study, with retrospective data collection, during a 13.5 years period (01/2000 to 06/2013). Mortality by invasive meningococcal disease and related factors (organ dysfunction and multi-organ failure) were analysed. The base excess and platelets score was calculated retrospectively, to evaluate its accuracy in predicting mortality and compared with Paediatric Risk of Mortality and Paediatric Index of Mortality2.
      Results: Were admitted 76 children with invasive meningococcal disease. The most frequent type of dysfunction was cardiovascular (92%), followed by hematologic (55%). Of the total, 47 patients (62%) had criteria for multi-organ failure. The global mortality was 16%. Neurologic and renal dysfunction showed the strongest association with mortality, adjusted odds ratio 315 (26 - 3 804) and 155 (20 - 1 299).
      After application of receiver operating characteristic curves, Base Excess and Platelets score had an area under curve of 0.81, Paediatric Index of Mortality2 of 0.91 and Paediatric Risk of Mortality of 0.96.
      Discussion: The Base Excess and Platelets score showed good accuracy, although not as high as Paediatric Risk of Mortality or
      Paediatric Index of Mortality2.
      Conclusions: The Base Excess and Platelets score may be useful tool in invasive meningococcal disease because is highly sensitive and specific and is objectively measurable and readily available at presentation.
      PubDate: 2015-06-04
      Issue No: Vol. 28 (2015)
  • Cephalometric Evaluation of Children with Allergic Rhinitis and Mouth

    • Authors: Helena Afonso Agostinho, Ivo Álvares Furtado, Francisco Salvado e Silva, Josep Ustrell Torrent
      Abstract: Introduction: Orthodontists frequently treat children with mouth breathing. The purpose of the present study was to examine dental positions, skeletal effects and the pharyngeal airway space of children with chronic allergic rhinitis, when compared with a control group exhibiting a normal breathing pattern.
      Material and Methods: Seventy Caucasian children from Santa Maria University Hospital - North Lisbon Hospital Center were evaluated, between September 2009 and February 2013. The study group comprised of 35 children with chronic allergic rhinitis, both genders, aged 5 - 14, with positive reaction to allergens, mouth breathing and malocclusion. The control group was composed of 35 children, both genders, displaying normal nasal breathing and malocclusion, who resorted to the orthodontic department. Measures of Ricketts, Steiner and McNamara’s analysis were used and the t- Student test was applied to the data obtained.
      Results: Statistically significant differences were observed between the oral and nasal breathers, respectively: lower facial height (49.1/45.9 mm), Frankfurt – mandibular plane angle (30.1/26.9º) and Sela-Nasion - oclusal plane angle (17.3/15º), maxillary length (78.4/82.4 mm), mandibular length (102.4/107 mm), overbite (0.8/3.1mm) and overjet (4/4.7 mm).
      Discussion: Comparison between the allergic rhinitis and control group showed that there is an increased lower facial height, larger Frankfurt – mandibular plane angle and Sela-Nasion oclusal plane angle in children with chronic allergic rhinitis. This group also had a shorter maxillary and mandibular length, less overbite and decreased upper airway space.
      Conclusions: Children with allergic rhinitis and mouth breathing have longer faces, shorter maxillas and mandibles and a narrowed pharyngeal airway space. No statistical differences between the groups in sagital relationships or in dental inclinations were found.
      PubDate: 2015-06-04
      Issue No: Vol. 28 (2015)
  • Cystatin C: A Promising Marker of Renal Function in Patients with Systemic
           Lupus Erythematosus'

    • Authors: Lígia Peixoto, Patrício Aguiar, Raquel de Bragança, Joana Rosa Martins, Alba Janeiro Acabado, José Luís Ducla-Sores
      Abstract: Purpose: Cystatin C has a higher correlation with glomerular filtration rate and a more significant clinical prognosis than creatinine. We sought to determine whether it is a marker of renal function different from creatinine (cystatin C potentially superior to creatinine), in patients with systemic lupus erythematosus.
      Material and Methods: 37 patients with systemic lupus erythematosus were evaluated. Serum cystatin C was determined by nephelometry and creatinine by modified Jaffe method. We compared five formulas: Chronic Kidney Disease – Epidemiology Collaboration cystiatin; Chronic Kidney Disease – Epidemiology Collaboration creatinine-cystatin; Cockcroft-Gault; Modification of Diet in Renal Disease and Chronic Kidney Disease – Epidemiology Collaboration creatinine, using the latter as a reference. We analyzed the influence of clinical and laboratory factors in cystatin C variation, using multivariate linear regression.
      Results: Cystatin C was singly elevated in ten participants, versus none isolated creatinine elevation, and this difference was significant (p = 0.002). There was a difference between the estimated glomerular filtration rate by Chronic Kidney Disease – Epidemiology Collaboration cystatin and by Chronic Kidney Disease – Epidemiology Collaboration creatinine (-6.0541 mL/min/1.73 m2, p = 0.07), more pronounced for lower glomerular filtration rate. Consequently, Chronic Kidney Disease – Epidemiology Collaboration cystatin reclassified 4 patients as having chronic kidney disease de novo and 1 patient as not having chronic kidney disease (p = 0.375).
      Cystatin C was only significantly influenced by age (p < 0.001).
      Discussion: Several reports showed cystatin C as a better marker to define chronic kidney disease, allowing more accurate classification and risk stratification, compared with creatinine. In this study, Cystatin C revealed as a promisor marker of renal function in patient with lupus, mainly in patients with lower glomerular filtration rates. The correlation between age and cystatin C seems to be a confounding
      factor, as glomerular filtration rate physiologically declines with ageing.
      Conclusion: Cystatin C was potentially superior to creatinine and in this study and cystatin C seems to detect changes in glomerular filtration rate earlier than creatinine and may be a better screening method for chronic kidney disease in systemic lupus erythematosus.
      PubDate: 2015-06-03
      Issue No: Vol. 28 (2015)
  • Hormona Paratiróideia Como Factor Predictivo de Hipocalcemia
           Após Tiroidectomia: Estudo Prospectivo em 100 Doentes

    • Authors: Fernando Melo, António Bernardes, Ana Velez, Catarina Campos de Melo, Fernando José de Oliveira
      Abstract: Introdução: A hipocalcemia é uma complicação frequente após tiroidectomia total e o principal motivo pelo internamento prolongado destes doentes.
      Material e Métodos: Estudaram-se prospectivamente 112 doentes submetidos a tiroidectomia total ou restante entre Junho de 2012 e Novembro de 2013. Foram excluídos 12 casos por apresentarem alterações pré-operatórias da função da paratiróide. Doseou-se a paratormona e cálcio pré-operatórios, imediatamente após a cirurgia, no primeiro dia e no 14º dia após a cirurgia.
      Resultados: Dos 100 doentes incluídos 60 desenvolveram hipocalcemia (60%), revelando-se sintomática em 14. Surgiu, maioritariamente, 24 horas após a cirurgia (76,7%). Foi definitiva em três doentes e transitória nos restantes. Nos 60 doentes com hipocalcemia, verificou-se hipoparatiroidismo em 19 doentes no doseamento imediatamente após a cirurgia, 14 no doseamento no primeiro dia mas apenas três mantiveram hipoparatiroidismo após 14 dias (doentes com hipocalcemia definitiva). Comparando o grupo de doentes com e sem hipocalcemia constatou-se uma diminuição da paratormona em ambos (imediatamente após a cirurgia e no primeiro dia) mas
      foi mais importante no grupo com hipocalcemia (p = 0,004 e p < 0,001). O decréscimo da paratormona foi mais acentuado no grupo com hipocalcemia, com maior significado no primeiro dia (50,29% vs 22,47% - p < 0,001). O melhor factor preditivo de hipocalcemia identificado foi o decréscimo da paratormona no primeiro dia após a cirurgia > 19,4% (sensibilidade = 82%; especificidade = 63%).
      Discussão: No nosso estudo verificou-se uma incidência elevada de hipocalcemia (60%), que se manifestou maioritariamente 24 horas após a cirurgia e que condicionou, nesses doentes, um tempo de internamento mais prolongado. No entanto, em apenas três doentes (3%) a hipocalcemia foi definitiva. Constatou-se ainda uma correspondência na oscilação dos valores séricos do cálcio e da paratormona que permitiu identificar o decréscimo da paratormona no primeiro dia após a cirurgia como um fiável factor predictivo de
      Conclusão: O decréscimo da paratormona no primeiro dia após a cirurgia > 19,4% é um bom factor preditivo de hipocalcemia após tiroidectomia total/restante, permitindo identificar os doentes com maior risco, medicá-los profilaticamente e obter altas precoces e seguras.
      PubDate: 2015-06-02
      Issue No: Vol. 28 (2015)
  • Analysis of the Cochrane Review: Angiotensin Converging Enzyme Inhibitors
           Versus Angiotensin Receptor Blockers for Primary Hypertension. Cochrane
           Database Syst Rev. 2014,8: CD009096.

    • Authors: Luís Nogueira-Silva, João A. Fonseca
      Abstract: Angiotensin converting enzyme inhibitors and angiotensin receptor blockers are first line drugs in the treatment of hypertension. The aim of this review was to assess if there are differences between these drug classes regarding the prevention of total mortality, occurrence of cardiovascular events and of adverse effects. A systematic review and metanalysis was performed, searching for studies that compare angiotensin converting enzyme inhibitors and angiotensin receptor blockers face-to-face, in several databases until July 2014. The study selection and data extraction were performed by 2 independent researchers. Nine studies were included, with a total of 10 963 participants, 9 398 of which participated in the same study and had high cardiovascular risk. No differences were observed regarding total mortality, cardiovascular mortality or total cardiovascular events. A slightly smaller risk was observed with angiotensin receptor blockers regarding withdrawal due to adverse effects (55 people were needed to be treated with angiotensin receptor blockers for 4.1 years to avoid one withdrawal due to adverse effect), mainly due to the occurrence of dry cough with angiotensin converting enzyme inhibitors. Thus, no differences were observed between angiotensin converting enzyme inhibitors and angiotensin receptor blockers in the prevention of total mortality and cardiovascular events, and angiotensin receptor blockers were better tolerated. Given the large proportion of participants with a high cardiovascular risk, the generalization of these results to other populations is limited.
      PubDate: 2015-06-01
      Issue No: Vol. 28 (2015)
  • The Role of Haemoglobin A1c in Screening Obese Children and Adolescents
           for Glucose Intolerance and Type 2 Diabetes

    • Authors: Júlia Galhardo, Julian Shield
      Abstract: Introduction: In 2012, an international expert committee in diabetes wrote in favor of screening adult and paediatric patients for glucose intolerance and type 2 diabetes using glycated haemoglobin. The aim of this study was to evaluate glycated haemoglobin utility as a screening tool in a young obese mainly Caucasian population.
      Material and Methods: Children [(n = 266), body mass index z-score 3.35 ± 0.59, 90% Caucasian 90%, 55% female, median age 12.3 (range: 8.9 - 17.6) years old] recently referred to a tertiary hospital-based obesity clinic underwent a routine oral glicose tolerance test and glycated haemoglobin measurement. Exclusion criteria: abnormal forms of haemoglobin and conditions linked to increased erythrocyte turnover.
      Results: The oral glicose tolerance test diagnosed 13 (4.9%) subjects as prediabetic but none as diabetic. According to glycated haemoglobin, 32 would be prediabetic (29 false positives) and one would be diabetic (when he was only glucose intolerant). On the other hand, 10 prediabetic patients would not have been identified (false negatives). Glycated haemoglobin receiver operator characteristic analysis area under the curve was 0.59 (CI 95% 0.40 - 0.78), confirming its reduced capacity to identify prediabetes. Better results were achieved when calculating receiver operator characteristic analysis area under the curve for fasting glucose (0.76;
      CI 95% 0.66 - 0.87), homeostasis model assessment for insulin resistance (0.77; CI 95% 0.64 - 0.90) and triglycerides:HDL cholesterol ratio (0.81; CI 95% 0.66 - 0.96).
      Discussion: In Paediatric populations, especially when mainly Caucasian, glycated haemoglobin does not seem to be a useful
      screening tool for prediabetes.
      Conclusion: For this reason, it would appear premature to advise it as a diagnostic tool until significantly more data is available. Homeostasis model assessment for insulin resistance and triglycerides: HDL cholesterol have higher precision and can be calculated using a fasting blood sample.
      PubDate: 2015-05-29
      Issue No: Vol. 28 (2015)
  • On Tentações de Santo Antão: The Way Things Mean

    • Authors: Nuno Félix da Costa
      Abstract: Keywords: Ergotism; History, Medieval; Paint; Portugal; Religion and Medicine; Saints/history.
      PubDate: 2015-05-20
      Issue No: Vol. 28 (2015)
  • Clinical Guidelines 2.0: Translation of Tables 2 and 3 and Apendix 1 of
           Schünemann HJ et al. CMAJ. 2014; 186:E123-42.

    • Authors: Guilherme Ferreira dos Santos, Pedro Correia Azevedo, António Vaz-Carneiro
      Abstract: Clinical Practice Guidelines are instruments to support decision to improve the quality of clinical care. An expert group from McMaster University (Canada) has developed - from high-quality literature sources – a guidance on the practical steps for their development, dissemination, implementation and evaluation. This is the 1st time anyone seeks to bring together in one document all information regarding the Clinical Practice Guidelines. Due to the interest of this paper, the Centre for Evidence Based Medicine at the University of Lisbon School of Medicine contacted the authors of the article and the journal where it was published (the Canadian Medical Association Journal) in order to translate the most relevant parts of the article (including the practice tables), which was agreed. This guide should be useful to those who, being interested in the development, dissemination and implementation of Clinical Practice Guidelines, want to ensure their intrinsic quality based on relevant and updated evidence.
      PubDate: 2015-05-12
      Issue No: Vol. 28 (2015)
  • Metallosis: A Rare Cause of Autoimmune Hemolytic Anemia

    • Authors: Joana Duarte, Lurdes Correia, Adélia Simão, António Figueiredo, Armando Carvalho
      Abstract: Introduction: Hemolytic anemia may be associated with multiple etiologies, including toxic substances, such as metals, which is a rare cause.
      Case Study: 55-year-old male, who underwent a total arthroplasty of the right hip (uncemented prostheses with ceramic-ceramic articulation with an acetabular component consisting of a dome composed of an alloy of titanium, aluminum and vanadium into which fitted a ceramic ‘insert’). Approximately 4 years after surgery the patient complained of noise originating from the prosthesis which occurred on movement. A surgical revision was performed and showed the presence of dark thick intracapsular fluid, fracture of the ceramic acetabular ‘insert’ and signs of wear of the acetabular metal dome. Extensive washing was carried out and the fractured ceramic ‘insert’ was replaced for a polyethylene ‘insert’. Two months later he was referred to the Emergency Room due to worsening of his general health, floating in the right hip and mucocutaneous jaundice. Laboratory tests suggested autoimmune hemolytic anemia. Arthrocentesis was performed and a large volume of metal fluid was drained off. The CT scan showed a large heterogeneous pelvic cystic collection seeded with prosthesis fragments, suggestive of metallosis. Hemolytic anemia was explained as toxicity of the particles and metal ions caused by the wear of the prosthesis. The patient was started on a high-dose steroid treatment. Afterwards, when he was stable, prosthesis components replacement and drainage of pelvic debris fluid were carried out.
      Discussion: After the fracture of the ceramic ‘insert’ the ceramic head began to articulate directly with the metallic acetabular component, causing noise and wear with release of particles and ions. This caused a cystic pelvic abscess, which went unnoticed on the first surgical revision. Surgical debridement lead to the cystic collection extending into the adjacent tissues and the systemic circulation, triggering serious systemic effects, such as autoimmune hemolytic anemia. The potential toxicity of each of the metal elements of this prosthesis is unknown, and there are still no available laboratory tests for its detection.
      Conclusion: Metallosis is a rare cause of autoimmune hemolytic anemia.
      PubDate: 2015-04-30
      Issue No: Vol. 28 (2015)
  • Clinical and Radiological Characterization of Progressive Multifocal
           Leukoencephalopathy in HIV-Infected Patients: A Retrospective Analysis and
           Review of the Literature

    • Authors: Luís Augusto, Nélia Neves, Carina Reis, Cândida Abreu, António Sarmento
      Abstract: Introduction: Progressive multifocal leukoencephalopathy is a demyelinating disease of the central nervous system caused by John Cunningham virus, mostly associated with immunodeficiency conditions, such as the human immunodeficiency virus infection. Progressive multifocal leukoencephalopathy can have multiple clinical features and usually presents a typical lesion pattern on brain magnetic resonance imaging. Its course may be rapidly progressive, although immunological responsiveness can be associated with
      an improved prognosis.
      Material and Methods: We performed a retrospective analysis of the clinical and radiological data from patients admitted in our institution between January 2005 and April 2014 with the diagnosis of definitive progressive multifocal leukoencephalopathy (ICD10:A81.2) in the setting of human immunodeficiency virus infection.
      Results: Twenty-one patients were included in our study, mostly men (n = 20, 95.2%). Mean age at diagnosis was 39 years. Motor deficits were the most common clinical finding. John Cunningham virus-DNA was detected in the cerebral spinal fluid in 20 patients (95.2%). Brain imaging studies most commonly disclosed bilateral supratentorial, asymmetric lesions. Four (19%) patients developed immune reconstitution inflammatory syndrome in the follow-up. Therapeutic approach included initiation and continuation/optimization of antiretroviral therapy, with adjunctive therapy with corticosteroids in four patients. Seventeen (81%) patients died during the study period; median survival time following progressive multifocal leukoencephalopathy diagnosis was 3 months (range 1 - 13).
      Discussion: The results of our study are in accordance with the data previously published on progressive multifocal leukoencephalopathy in human immunodeficiency virus patients. Progressive multifocal leukoencephalopathy is predominantly associated with severe immunosuppression, particularly in patients who are not under anti-retroviral therapy, and usually presents with motor and cognitive
      symptoms and signs. A typical bilateral asymmetric pattern in conventional magnetic resonance imaging is present in the majority of the patients. There is no specific therapy for progressive multifocal leukoencephalopathy and it is usually fatal, although outcomes can improve with highly active anti-retroviral therapy. Immune reconstitution inflammatory syndrome is also an important complication related with progressive multifocal leukoencephalopathy, usually associated with anti-retroviral therapy. Progressive multifocal leukoencephalopathy-immune reconstitution inflammatory syndrome presents with different imaging characteristics from progressive multifocal leukoencephalopathy and treatment with steroids can improve survival.
      Conclusion: The mortality rate and long-term neurological morbidity associated with progressive multifocal leukoencephalopathy are quite high. These data should increase clinician awareness to the occurrence of progressive multifocal leukoencephalopathy among human immunodeficiency virus patients and highlight the important role of magnetic resonance imaging, as early diagnosis may be
      associated with better outcome.
      PubDate: 2015-04-29
      Issue No: Vol. 28 (2015)
  • Prognostic Factors after Liver Resection for Colorectal Liver Metastasis

    • Authors: Margarida Matias, Mafalda Casa-Nova, Mariana Faria, Ricardo Pires, Joana Tato-Costa, Leonor Ribeiro, Luis Costa
      Abstract: Introduction: Surgery is the only potentially curative treatment for patients with colorectal liver metastases, resulting in 5-year survival rates of 36–58%. Although many studies have been performed to determine prognostic factors for tumor recurrence and survival after resection of colorectal liver metastases, there are few prognostic scoring systems stratifying patients undergoing surgery for colorectal liver metastases into risk group models.
      Objectives: To identify, evaluate and compare the existing prognostic scores for survival after surgery for resection of colorectal liver metastases.
      Material and Methods: Electronic search in PubMed, Cochrane and Embase from 1990 to 2013 using the terms ‘hepatic resection’, ‘colorectal cancer’, ‘liver metastasis’, ‘hepatectomy’, ‘prognostic’, and ‘score‘. Only studies proposing a prognostic model or risk stratification based on clinical and/or pathological variables were included.
      Results: From 1996 to June 2013, 19 scoring systems were identified, including one nomogram. Thirty prognostic factors were identified although none of the factors was common to all prognostic models. The 4 factors most often included were: number of liver metastases, regional lymph node metastization of primary tumor, preoperative CEA level and maximum size of metastases. The median study sample size was 305 patients (81-1 568 patients) and median follow-up was 33 months (16-54 months). All studies were retrospective and used the Cox proportional hazards model for multi-variable analysis.
      Conclusion: Several factors have been constantly reported as having prognostic value after liver resection of colorectal liver
      metastases, although there is no consensus on the ideal scoring system.
      PubDate: 2015-02-27
      Issue No: Vol. 28 (2015)
  • Off-Label and Unlicensed Drug Use in Neonatology: Reality in a Portuguese
           University Hospital

    • Authors: Joana Silva, Filipa Flor-de-Lima, Henrique Soares, Hercília Guimarães
      Abstract: Introduction: This study aims to characterize the drugs used in a Portuguese Neonatal Intensive Unit Care, assess the rate of off-label or unlicensed drugs use according to the information available in the Summary of Product Characteristics and compare results between preterm and full-term neonates.
      Material and Methods: A 6-month period retrospective cross-sectional study was conducted in our Neonatal Intensive Unit Care in 2013. Prescribed drugs data were recorded and compared with the paediatric information contained in drugs Summary of Product Characteristics.
      Results: We analyzed 1011 prescriptions of 84 active substances, made in 218 admissions. In 42.9% of the cases, medicines were used according to Summary of Product Characteristics information; 27.9% of drugs were approved for neonatal period but used in an off-label manner; off-label drugs for neonates were used in 10.1%, whereas those with undetermined approval state and contraindicated were used
      6.0% and 8.7% of the cases, respectively. Unlicensed prescriptions accounted for 4.4% of total. Preterm received a higher rate of drugs used according to Summary of Product Characteristics (p < 0.0001), whereas full-term received more off-label drugs for dose/frequency (p < 0.0001) and contra-indicated for neonates (p < 0.012).
      Discussion: Preterm neonates received a higher median number of drugs, since they stayed longer in the unit. The main reason for off-label prescribing was the use of doses/frequencies of administration different from those stated in the Summary of Product Characteristics, suggesting that updating these documents is necessary. Manipulation of medicines is one of the causes for unlicensed drugs use, emphasizing the lack of appropriate formulations for neonatal age.
      Conclusion: Progresses have been made to reduce the risks of off-label/unlicensed prescriptions, but competent authorities must continue their efforts to develop safer and more effective drugs for neonatal period.
      PubDate: 2015-01-16
      Issue No: Vol. 28 (2015)
  • “Apple Jelly” Sign: Diascopy in Cutaneous Sarcoidosis

    • Authors: Diogo Matos, Ricardo Coelho
      Abstract: Keywords: Diagnosis, Differential; Sarcoidosis; Skin Diseases.
      PubDate: 2015-01-16
      Issue No: Vol. 28 (2015)
  • Genetic Heterogeneity in Colorectal Cancer and its Clinical Implications

    • Authors: Rui Barranha, José Luís Costa, Fátima Carneiro, José Carlos Machado
      Abstract: Despite the recent advances in the development of complementary diagnostic exams and modern targeted therapies, colorectal cancer remains a major cause of morbidity and mortality worldwide. In this context, a lot of research has been conducted in the last years to find new markers of poor prognosis. The existence of a complex tumour architecture formed by multiple subclones genetically heterogeneous has been increasingly considered in recent studies as an element of particular importance. This feature seems to influence factors as relevant as the representativeness of tumour biopsies for genetic diagnosis and the efficacy of targeted therapies.
      There is growing evidence suggesting a relation between genetic heterogeneity and the patients’ prognosis. The widespread use of next-generation sequencing techniques will allow a better understanding of the true degree of genetic heterogeneity in colorectal tumours, its causes and impact on the course of the disease. In this review we intend to analyse the recent findings related to the genetic heterogeneity of colorectal cancer, as well as its major clinical implications.
      PubDate: 2015-01-16
      Issue No: Vol. 28 (2015)
  • Regulation of Parental Leave Among Parents of Very Preterm Infants

    • Authors: Mariana Amorim, Elisabete Alves, Henrique Barros, Susana Silva
      Abstract: Keywords: Infant, Premature; Parental Leave.
      PubDate: 2015-01-16
      Issue No: Vol. 28 (2015)
  • The Future of the Portuguese Health System

    • Authors: Nigel Crisp
      Abstract: The Portuguese health system has considerable strengths and an impressive track record of success, but is facing severe challenges due to increasing demand and restricted funding. Its continuing success will depend on how citizens and clinicians respond to these challenges as well as on a clear vision for the future and good collective leadership from health professionals, politicians and others in wider society.
      Issue No: Vol. 28
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