Centro Hospitalar do Porto

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    Pulmonary vascular dysfunction among people aged over 65 years in the community in the Atherosclerosis Risk In Communities (ARIC) Study: A cross-sectional analysis

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    Background: Heart failure (HF) risk is highest in late life, and impaired pulmonary vascular function is a risk factor for HF development. However, data regarding the contributors to and prognostic importance of pulmonary vascular dysfunction among HF-free elders in the community are limited and largely restricted to pulmonary hypertension. Our objective was to define the prevalence and correlates of abnormal pulmonary pressure, resistance, and compliance and their association with incident HF and HF phenotype (left ventricular [LV] ejection fraction [LVEF] ≥ or < 50%) independent of LV structure and function. Methods and findings: We performed cross-sectional and time-to-event analyses in a prospective epidemiologic cohort study, the Atherosclerosis Risk in Communities study. This is an ongoing, observational study that recruited 15,792 persons aged 45-64 years between 1987 and 1989 (visit 1) from four representative communities in the United States: Minneapolis, Minnesota; Jackson, Mississippi; Hagerstown, Maryland; and Forsyth County, North Carolina. The current analysis included 2,810 individuals aged 66-90 years, free of HF, who underwent echocardiography at the fifth study visit (June 8, 2011, to August 28, 2013) and had measurable tricuspid regurgitation by spectral Doppler. Echocardiography-derived pulmonary artery systolic pressure (PASP), pulmonary vascular resistance (PVR), and pulmonary arterial compliance (PAC) were measured. The main outcome was incident HF after visit 5, and key secondary end points were incident HF with preserved LVEF (HFpEF) and incident HF with reduced LVEF (HFrEF). The mean ± SD age was 76 ± 5 years, 66% were female, and 21% were black. Mean values of PASP, PVR, and PAC were 28 ± 5 mm Hg, 1.7 ± 0.4 Wood unit, and 3.4 ± 1.0 mL/mm Hg, respectively, and were abnormal in 18%, 12%, and 14%, respectively, using limits defined from the 10th and 90th percentile limits in 253 low-risk participants free of cardiovascular disease or risk factors. Left heart dysfunction was associated with abnormal PASP and PAC, whereas a restrictive ventilatory deficit was associated with abnormalities of PASP, PVR, and PAC. PASP, PVR, and PAC were each predictive of incident HF or death (hazard ratio per SD 1.3 [95% CI 1.1-1.4], p < 0.001; 1.1 [1.0-1.2], p = 0.04; 1.2 [1.1-1.4], p = 0.001, respectively) independent of LV measures. Elevated pulmonary pressure was predictive of incident HFpEF (HFpEF: 2.4 [1.4-4.0, p = 0.001]) but not HFrEF (1.4 [0.8-2.5, p = 0.31]). Abnormal PAC predicted HFrEF (HFpEF: 2.0 [1.0-4.0, p = 0.05], HFrEF: 2.8 [1.4-5.5, p = 0.003]), whereas abnormal PVR was not predictive of either (HFpEF: 0.9 [0.4-2.0, p = 0.85], HFrEF: 0.7 [0.3-1.4, p = 0.30],). A greater number of abnormal pulmonary vascular measures was associated with greater risk of incident HF. Major limitations include the use of echo Doppler to estimate pulmonary hemodynamic measures, which may lead to misclassification; inclusions bias related to detectable tricuspid regurgitation, which may limit generalizability of our findings; and survivor bias related to the cohort age, which may result in underestimation of the described associations. Conclusions: In this study, we observed abnormalities of PASP, PVR, and PAC in 12%-18% of elders in the community. Higher PASP and lower PAC were independently predictive of incident HF. Abnormally high PASP predicted incident HFpEF but not HFrEF. These findings suggest that impairments in pulmonary vascular function may precede clinical HF and that a comprehensive pulmonary hemodynamic evaluation may identify pulmonary vascular phenotypes that differentially predict HF phenotypes.info:eu-repo/semantics/publishedVersio

    The role of point-of-care ultrasound in the diagnosis and management of necrotizing soft tissue infections

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    Background: Necrotizing soft tissue infections are associated with high morbidity and mortality, even when the correct treatment is initiated. The diagnosis of these conditions is hard and the most sensitive methods are time-consuming, expensive and not readily available. Point-of-care ultrasound can complement clinical evaluation to increase the diagnostic accuracy. Case presentation: We bring a case of a woman, without comorbidities, who presented to the emergency department with signs of soft tissue infection. Bedside ultrasound showed subcutaneous tissue thickening, with fluid accumulation, and subcutaneous gas in the affected area. Based on the clinical suspicion and the ultrasound findings, the patient underwent prompt medical treatment and surgical debridement. Conclusions: This case shows the utility of bedside ultrasound for the decision-making process in a disease where an early diagnosis is important. This information should always be used together with the clinical judgement, as it has a relative low sensitivity.info:eu-repo/semantics/publishedVersio

    Azathioprine-Induced Acute Submandibular Sialadenitis in a Crohn's Disease Patient

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    Introduction: Azathioprine (AZA) is a widely used immunosuppressive drug in inflammatory bowel disease (IBD). The occurrence of adverse effects (AEs) is a major downside in the use of this drug, leading to treatment withdrawal in a variable proportion of patients. Case presentation: We report the case of a Crohn's disease patient who developed sialadenitis as an AE to AZA. Discussion and conclusion: To our knowledge this AE has been reported only once in the literature. Sialadenitis is a common disorder and refers to inflammation of a salivary gland. It has many causes, such as bacterial and viral infections, ductal obstruction, and drugs. There are several AEs related to this drug, categorized as dose-dependent and dose-independent. Their knowledge is essential for therapeutic management in IBD, which is already challenging, requiring an individualized approach.info:eu-repo/semantics/publishedVersio

    Artrodese versus artroplastia da primeira articulação metatarsofalângica no tratamento do hallux rigidus – Estudo comparativo de pacientes selecionados apropriadamente

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    Objective Historical results of arthroplasty of the first metatarsophalangeal joint (1MTP) are relatively poor; however, improvements in the understanding of the normal foot biomechanics, implant materials and design currently make arthroplasty a reasonable option in appropriately selected patients. The present study aimed to compare the clinical and radiographic results of 1MTP arthrodesis and arthroplasty in the treatment of hallux rigidus and to present a rationale for patient selection for arthroplasty. Methods A total of 36 patients (38 feet) with hallux rigidus submitted to surgery (12 arthrodesis and 26 arthroplasties) were prospectively included in the study. Pain was assessed using the visual analogue scale (VAS) and the functional status was assessed using the American Orthopedic Foot and Ankle Society Hallux Metatarsophalangeal-Interphalangeal (AOFAS-HMI) scale. Complications and radiographic results were also analyzed, and survival rates were calculated for both procedures. Results All of the patients reported significant improvement in pain and functional status after surgery. Patients submitted to arthroplasty had better functional results on the AOFAS-HMI scale (89.7 versus 65.7 points; p < 0.001) and better pain relief (VAS 1.6 versus 3.9 points; p = 0.002) when compared with the group submitted to arthrodesis. There was one case of infection in the arthroplasty group and 2 cases of pseudarthrosis in the arthrodesis group. Conclusion Arthrodesis provides pain relief and satisfactory results but alters the biomechanics of gait. Like arthrodesis, arthroplasty improves pain significantly, being a more physiological alternative to preserve the biomechanics of the foot. While the two surgical methods yielded good clinical results, selected patients submitted to arthroplasty had better clinical scores and lower revision rates.info:eu-repo/semantics/publishedVersio

    Echocardiographic Assessment of Right Ventriculo-arterial Coupling: Clinical Correlates and Prognostic Impact in Heart Failure Patients Undergoing Cardiac Resynchronization Therapy

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    Background: Right ventriculo-arterial coupling (RV-PA) can be estimated by echocardiography using the ratio between tricuspid annular plane systolic excursion (TAPSE) and pulmonary artery systolic pressure (PASP) and it has prognostic value in the general heart failure (HF) population. We aimed to study the clinical correlates and prognostic value of RV-PA in HF patients undergoing cardiac resynchronization therapy (CRT). Methods: We retrospectively studied 70 HF patients undergoing CRT implantation. Results: RV-PA coupling was estimated by TAPSE/PASP ratio using baseline echocardiography. Non-response to CRT was defined as improvement of left ventricular ejection fraction < 5% in a follow-up echo 6-12 months after CRT. Those with lower TAPSE/PASP ratios (worse RV-PA coupling) had higher NT-proBNP concentrations and increased E/e' ratio. TAPSE/PASP ratio and PASP, but not TAPSE, predicted nonresponse to CRT with TAPSE/PASP ratio showing the best discriminative ability with a sensitivity of 76% and specificity of 71%. Among these parameters, PASP independently predicted all-cause mortality. Conclusions: RV-PA coupling estimated by TAPSE/PASP ratio was associated with established prognostic markers in HF. It numerically outperformed PASP and TAPSE in predicting the response to CRT. Our data suggest that this simple and widely available echocardiographic parameter conveys significant pathophysiological and prognostic meaning in HF patients undergoing CRT.info:eu-repo/semantics/publishedVersio

    Ablação Guiada por Ecoendoscopia de Lesões Focais do Pâncreas: Perspetiva do GRUPUGE

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    Focal pancreatic lesions include a heterogeneous group of solid and cystic lesions, with different natures and variable clinical, imagiological, and pathological characteristics. Several endoscopic ultrasound (EUS)-guided ablative techniques have been tested during the last decade for the treatment of these pancreatic lesions, mostly consisting of the injection of ablative agents and, more recently, radiofrequency ablation. The most encouraging EUS-guided ablation outcomes are being reached in the treatment of some pancreatic cystic neoplasms and small (≤2 cm) pancreatic neuroendocrine tumours (pNETs). Data supporting a potential role of ablative therapies in the treatment of pancreatic ductal adenocarcinoma is still lacking. In this article, GRUPUGE presents an updated perspective of the potential role of EUS-guided ablation for the treatment of pancreatic cystic neoplasms and pNETs, addressing the selection criteria and technical issues of different techniques and analysing recent data on their safety and efficacy.As lesões focais do pâncreas integram grupos heterogéneos de lesões sólidas e quísticas, de diferentes naturezas e com características clínicas, imagiológicas e patológicas variáveis. Na última década foram avaliadas diversas técnicas ablativas guiadas por ecoendoscopia para o tratamento destas lesões pancreáticas focais, consistindo maioritariamente na injeção de agentes ablativos e, mais recentemente, na ablação por radiofrequência. Os resultados mais promissores das técnicas ablativas guiadas por ecoendoscopia têm surgido no tratamento de algumas lesões quísticas do pâncreas e pequenos tumores neuro-endócrinos pancreáticos (≤2 cm). Ainda existem poucos dados a suportar um potencial papel das terapêuticas ablativas no tratamento do adenocarcinoma ductal do pancreas. No presente artigo o GRUPUGE apresentada uma perspectiva atual do potencial papel da ablação guiada por ecoendoscopia no tratamento de neoplasias quísticas do pâncreas e de tumores neuro-endócrinos pancreáticos, focando aspectos relativos à seleção dos doentes, questões técnicas dos vários procedimentos disponíveis e analisando dados recentes relativos à sua segurança e eficácia.info:eu-repo/semantics/publishedVersio

    Anuário Científico do CHUP 2018

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    O Anuário Científico de 2018 é um documento integrador da produção científica do Centro Hospitalar Universitário do Porto (CHUP). O Serviço de Investigação Clínica do Departamento de Ensino, Formação e Investigação (DEFI), com a edição deste anuário, pretende partilhar a atividade científica hospitalar com a comunidade e os seus profissionaisinfo:eu-repo/semantics/publishedVersio

    Escondido pelo cabelo - um caso de puberdade precoce

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    McCune-Albright syndrome (MAS) is one of the conditions causing precocious gonadotropin-independent puberty. It is a rare disease, characterized by two of the three following features: precocious puberty, polyostotic fibrous dysplasia (PFD), and café au lait (CAL) skin spots. Herein is presented the case of a girl with pubarche, acne, and transient thelarche since the age of three and menarche since the age of six years old. Besides transitory follicular cysts and advanced bone age, no other changes were found. Diagnosis was only established after brain magnetic resonance imaging showed fibrous dysplasia involving left craniofacial bones. The girl’s parents later mentioned that she had multiple café au lait skin spots on the scalp since birth, disclosing how the key diagnostic sign had been covered by the child’s hair. MAS is a rare disorder and diagnosis depends on a high index of suspicion. CAL skin spots are generally the first manifestation, but can easily go unnoticed. Additionally, PFD may only affect some bones, like craniofacial.info:eu-repo/semantics/publishedVersio

    Sintomas psicóticos durante o tratamento com psicoestimulantes para a perturbação de hiperatividade com défice de atenção

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    Introduction: Attention-deficit/hyperactivity disorder (ADHD) is a common neurodevelopmental disorder. Pharmacological treatment, either alone or in combination with psychosocial interventions, is important in the therapeutic strategy. Psychostimulants remain the most effective medication and are generally well tolerated. However, one of its rare but significant side effects is development of psychotic symptoms. Objectives: To perform a literature review about the risk of psychotic symptoms during ADHD treatment with stimulants and discuss possible pharmacological treatment implications.Methods: A literature search was conducted on PubMed database using the terms “ADHD”, “central nervous system stimulants”, and “psychosis”. The search was supplemented with data retrieved from published guidelines.Results and discussion: ADHD diagnosis has been increasing, as well the use of stimulants for its treatment. However, using these drugs is not without controversy. ADHD pharmacological treatment is rarely associated with psychotic symptoms. Reported symptoms include hallucinations and paranoid delusions, and occasionally also euphoria, grandiosity, and other mania and hypomania features. These symptoms are generally short-lived and self-limited within days after stimulant discontinuation. One study comparing amphetamines and methylphenidate suggested that the former was more likely to cause psychosis compared with the latter. Additionally, study authors agreed that stimulant-induced psychosis seems to be associated to susceptibility factors.Conclusion: Psychostimulants are highly beneficial for ADHD patients. However, small studies and case reports suggest an association with new-onset psychotic symptoms. Physicians should be aware that psychotic symptoms may arise during stimulant ADHD treatment. Further investigation is required to identify patients who are most at risk of experiencing such adverse events.info:eu-repo/semantics/publishedVersio

    Cutaneous Manifestations in Mastocytosis: Update

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    Introdução: As mastocitoses caraterizam-se pela expansão clonal de mastócitos, com acumulação de mastócitos morfológica e imunofenotipicamente anormais em diferentes órgãos. A pele é o órgão mais frequentemente envolvido. Virtualmente, todas as crianças e mais de 80% dos adultos com mastocitose apresentam lesões cutâneas. Material e Métodos: O presente artigo descreve os sinais e sintomas associados à mastocitose na pele, tendo por base a revisão das normas de orientação de consenso internacionais, recentemente publicadas. Discussão: De acordo com a classificação proposta pela Organização Mundial de Saúde em 2016, a mastocitose divide-se em mastocitose cutânea, mastocitose sistémica e sarcoma de mastócitos. A mastocitose cutânea pode subdividir-se em três subtipos: a mastocitose cutânea maculopapular (também denominada urticária pigmentosa), mastocitose cutânea difusa e mastocitoma cutâneo. A telangiectasia macular eruptiva perstans já não é considerada uma entidade independente. Conclusão: As manifestações cutâneas da mastocitose são variáveis, dependendo da idade de início da doença. Recentemente a classificação da mastocitose cutânea foi atualizada. Nas crianças, a mastocitose ocorre como mastocitose cutânea que tende à regressão espontânea durante a adolescência. Quando tem início na idade adulta, a mastocitose é geralmente sistémica, sendo a forma mais frequente a mastocitose sistémica indolente, que normalmente também cursa com manifestações cutâneas e tem um curso crónico.Introduction: Mastocytosis is characterized by the clonal expansion of morphological and immunophenotypically abnormal mast cells in different organs. The skin is the most frequently affected tissue. Virtually all children and more than 80% of adult patients with mastocytosis show cutaneous lesions. Material and methods: The present article describes the symptoms and signs in cutaneous mastocytosis, based on the review of recently published international consensus guidelines. Discussion: According to the 2016 World Health Organization classification, mastocytosis can be divided in cutaneous mastocytosis, systemic mastocytosis and mast cell sarcoma. Cutaneous mastocytosis is subclassified in three subtypes: maculopapular cutaneous mastocytosis, diffuse cutaneous mastocytosis and cutaneous astocytoma. Telangiectasia macularis eruptiva perstans is no longer considered a distinct entity. Conclusion: Based on the age of onset, cutaneous manifestations of mastocytosis can be variable. The classification of cutaneous mastocytosis has recently been updated. Typically, in patients with childhood-onset mastocytosis, the disease occurs as cutaneous mastocytosis and shows spontaneous resolution around puberty. In contrast, adult patients, despite having also cutaneous lesions, often show systemic involvement and the course of the disease is usually chronic.info:eu-repo/semantics/publishedVersio

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