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- A Prediction Rule to Stratify Mortality Risk of Patients with Pulmonary TuberculosisPublication . Bastos, H.; Osório, N.; Castro, A.; Ramos, A.; Carvalho, T.; Meira, L.; Araújo, D.; Almeida, L.; Boaventura, R.; Fragata, P.; Chaves, C.; Costa, P.; Portela, M.; Ferreira, I.; Magalhães, S.; Rodrigues, F.; Sarmento-Castro, R.; Duarte, R.; Guimarães, J.; Saraiva, M.Tuberculosis imposes high human and economic tolls, including in Europe. This study was conducted to develop a severity assessment tool for stratifying mortality risk in pulmonary tuberculosis (PTB) patients. A derivation cohort of 681 PTB cases was retrospectively reviewed to generate a model based on multiple logistic regression analysis of prognostic variables with 6-month mortality as the outcome measure. A clinical scoring system was developed and tested against a validation cohort of 103 patients. Five risk features were selected for the prediction model: hypoxemic respiratory failure (OR 4.7, 95% CI 2.8-7.9), age ≥50 years (OR 2.9, 95% CI 1.7-4.8), bilateral lung involvement (OR 2.5, 95% CI 1.4-4.4), ≥1 significant comorbidity-HIV infection, diabetes mellitus, liver failure or cirrhosis, congestive heart failure and chronic respiratory disease-(OR 2.3, 95% CI 1.3-3.8), and hemoglobin <12 g/dL (OR 1.8, 95% CI 1.1-3.1). A tuberculosis risk assessment tool (TReAT) was developed, stratifying patients with low (score ≤2), moderate (score 3-5) and high (score ≥6) mortality risk. The mortality associated with each group was 2.9%, 22.9% and 53.9%, respectively. The model performed equally well in the validation cohort. We provide a new, easy-to-use clinical scoring system to identify PTB patients with high-mortality risk in settings with good healthcare access, helping clinicians to decide which patients are in need of closer medical care during treatment.
- Implications for patients waiting for a kidney transplant of using the calculated panel reactive antibody (cPRA)Publication . Magriço, R.; Malheiro, J.; Tafulo, S.; Pedroso, S.; Almeida, M.; Martins, L.; Dias, L.; Castro-Henriques, A.; Cabrita, A.Introduction: Kidney transplant improves survival even in highly‑sensitized (HS) patients. To overcome their disadvantage in accessing transplantation, those with high Complement Dependent Cytotoxic PRA (CDC‑PRA) receive additional points during allocation. Whether this strategy reaches all HS patients and how long they wait for a transplant is largely undetermined. Methods: Patients on our unit’s active wait‑list for kidney transplantation in the year 2014 were analyzed. CDC‑PRA and calculated PRA (cPRA) were recorded. To obtain cPRA, antibodies in the last serum available specific for HLA‑A, ‑B or –DR with an intensity > 1000 MFI were considered. Results: The cPRA values in the population (N=551) were 0% (N=312), 1‑79% (N=118) and ≥ 80% (22%; N=121). Among these groups, the proportion of women (29.5, 55.9 and 61.2%, P<0.001), prior sensitizing events (43.3, 80.5 and 96.7%, P<0.001) and time on dialysis (median of 3.9, 4.1 and 6.0 years, P<0.001) increased with cPRA, respectively. In most of those with a cPRA ≥ 80%, the CDC‑PRA raised no suspicion of HS status (median 0%, P25‑75 0‑8%) and only 35 (28.9%) or 12 patients (9.9%) had a CDC‑PRA in the peak serum higher than 50 or 80%, respectively (cut‑offs needed to obtain additional points during allocation). HS patients by cPRA corresponded to 71% vs 15% of patients waiting for ≥ or <8 years, respectively (P<0.001). Even after exclusion of patients with a CDC‑PRA above 50%, this disproportionate representation remained (58% versus 13%, P<0.001). Conclusion: HS patients as measured by cPRA remained longer on the wait‑list, both in the primary analysis and when excluding those with a CDC‑PRA> 50%. Moreover, only 30% of HS by cPRA patients received the extra points designed to improve their transplantability. We consider that both CDC‑PRA and cPRA should be taken into account when defining HS status.
- Pancreas-kidney transplantation: clinical, metabolic and immunological outcomesPublication . Martins, L.; Castro-Henriques, A.
- Anuário Científico 2015Publication . DEFIO Anuário Científico de 2015 é um documento integrador da produção científica do Centro Hospitalar do Porto (CHP). O Gabinete Coordenador da Investigação do Departamento de Ensino, Formação e Investigação (DEFI), com a edição do anuário, pretende partilhar com o CHP e com a comunidade a atividade científica hospitalar
- Malignant tumors of the temporal bone - our experiencePublication . Silva, A.; Breda, E.; Monteiro, E.INTRODUCTION: Malignant tumors of the temporal bone are rare, with an estimated incidence of about 0.8-1.0 per 1,000,000 inhabitants per year. The vast majority of these tumors are squamous cell carcinomas and their treatment is eminently surgical. OBJECTIVE: This study is an attempt at systematizing the forms of clinical presentation, the therapeutic possibilities, and oncological outcomes of patients with malignant tumors of the temporal bone in a tertiary hospital in Portugal. METHODS: The authors present a retrospective study of temporal bone tumors treated and followed during otorhinolaryngology consultations between 2004 and 2014. A review of the literature is also included. RESULTS: Of the 18 patients included in the study, 16 had a primary tumor of the temporal bone, in most cases with squamous cell carcinoma histology. Of these, 13 patients were treated with curative intent that always included the surgical approach. Disease persistence was observed in one patient and local recurrence in five patients, on average 36.8 months after the initial treatment. CONCLUSIONS: The anatomical complexity of the temporal bone and the close associations with vital structures make it difficult to perform tumor resection with margins of safety and thus, tumor relapses are almost always local. A high level of suspicion is crucial for early diagnosis, and stringent and prolonged follow-up after treatment is essential for diagnosis and timely treatment of recurrances.
- Late onset pityriasis rubra pilaris type IV treated with low-dose acitretinPublication . Mota, F.; Carvalho, S.; Sanches, M.; Selores, M.Pityriasis rubra pilaris is a chronic inflammatory dermatosis of unknown etiology and great clinical variability. It has been divided into six categories. Types III, IV, and V occur in childhood and are distinguished by their clinical presentation, age of onset, and course. We report a 19-year-old male patient with a 2-week history of pruritic, scaling dermatosis of the hands, feet, elbows, and knees. He had no family history of skin disease. On physical examination, we observed circumscribed, reddish-orange, scaling plaques affecting the elbows and knees and a waxy palmoplantar keratoderma. The skin biopsy showed acanthosis, alternating orthokeratosis, parakeratosis, and follicular plugging suggestive of pityriasis rubra pilaris. The patient started treatment with oral acitretin, 25 mg every other day. The treatment was tolerated well, and after 6 months the lesions had resolved completely. Pityriasis rubra pilaris is a chronic papulosquamous disorder of unknown pathogenesis, characterized by reddish-orange scaly plaques, palmoplantar keratoderma, and keratotic follicular papules. There is still no consensus regarding the treatment, but therapeutic options include systemic retinoids, particularly acitretin in the recommended dose of 0.5 to 0.75 mg/kg/day. In our case, the patient was treated with a low-dose regimen of acitretin, which was effective and well tolerated.
- Compared to Palliative Care, Working in Intensive Care More than Doubles the Chances of Burnout: Results from a Nationwide Comparative StudyPublication . Martins-Pereira, Sandra; Teixeira, Carla; Carvalho, A; Hernández-Marrero, P.Professionals working in intensive and palliative care units, hence caring for patients at the end-of-life, are at risk of developing burnout. Workplace conditions are determinant factors to develop this syndrome among professionals providing end-of-life care.
- Efficacy And Safety Of Implantable Loop Recorder: Experience Of A CenterPublication . Silveira, I.; Sousa, M.; Antunes, N.; Silva, V.; Roque, C.; Pinheiro-Vieira, A.; Lagarto, V.; Hipólito-Reis, A.; Luz, A.; Torres, S.Introduction: Symptoms like syncope or palpitations frequently present a diagnostic challenge. An implantable loop recorder (ILR) is an important aid in the management of these patients. Methods: A retrospective study of patients that underwent ILR implantation from November 2007 to 2014. For each patient the indication for implantation, baseline characteristics, previous study, complications, recorded tracing and interventions were evaluated. Results: A total of 62 patients were included, 50% men, with a mean age of 62.5±18.8 years old. Previously to ILR implantation 88.7% of patients had performed Holter, 17.7% external events recorder, 33.9% Tilt test and 29% an electrophysiological study. The implantation indications were recurrent syncope in 90.3%, palpitations 8.1% and ischemic stroke in one patient. Mean follow-up time was 17.1±16.3 months. Symptoms were reported in 66.1% of the patients, 46.8% of those yielding a diagnostic finding. In all cases of palpitation complaints with diagnosis we found atrial fibrillation (AF). In patients with syncope atrioventricular conduction disturbance was demonstrated in 19.6%, sinus node dysfunction in 16.1%, paroxysmal supra-ventricular tachycardia 7.1% and AF in 1.8%. These finding resulted in 19 pacemaker and one CRT-D implantation, introduction of anticoagulation in five patients and one ablation of accessory pathway. There were no major complications. Conclusion: ILR proved to be safe and efficient. It has enabled the identification or exclusion of serious rhythm disturbances in more than half of patients and provided a targeted therapeutic intervention.
- Peripheral Blood Cell Gene Expression Diagnostic for Identifying Symptomatic Transthyretin Amyloidosis Patients: Male and Female Specific SignaturesPublication . Kurian, S.; Novais, M.; Whisenant, T.; Gelbart, T.; Buxbaum, J.; Kelly, J.; Coelho, T.; Salomon, D.BACKGROUND: Early diagnosis of familial transthyretin (TTR) amyloid diseases remains challenging because of variable disease penetrance. Currently, patients must have an amyloid positive tissue biopsy to be eligible for disease-modifying therapies. Endomyocardial biopsies are typically amyloid positive when cardiomyopathy is suspected, but this disease manifestation is generally diagnosed late. Early diagnosis is often difficult because patients exhibit apparent symptoms of polyneuropathy, but have a negative amyloid biopsy. Thus, there is a pressing need for an additional early diagnostic strategy for TTR-aggregation-associated polyneuropathy and cardiomyopathy. METHODS AND FINDINGS: Global peripheral blood cell mRNA expression profiles from 263 tafamidis-treated and untreated V30M Familiar Amyloid Neuropathy patients, asymptomatic V30M carriers, and healthy, age- and sex-matched controls without TTR mutations were used to differentiate symptomatic from asymptomatic patients. We demonstrate that blood cell gene expression patterns reveal sex-independent, as well as male- and female-specific inflammatory signatures in symptomatic FAP patients, but not in asymptomatic carriers. These signatures differentiated symptomatic patients from asymptomatic V30M carriers with >80% accuracy. There was a global downregulation of the eIF2 pathway and its associated genes in all symptomatic FAP patients. We also demonstrated that the molecular scores based on these signatures significantly trended toward normalized values in an independent cohort of 46 FAP patients after only 3 months of tafamidis treatment. CONCLUSIONS: This study identifies novel molecular signatures that differentiate symptomatic FAP patients from asymptomatic V30M carriers as well as affected males and females. We envision using this approach, initially in parallel with amyloid biopsies, to identify individuals who are asymptomatic gene carriers that may convert to FAP patients. Upon further validation, peripheral blood cell mRNA expression profiling could become an independent early diagnostic. This quantitative gene expression signature for symptomatic FAP could also become a biomarker to demonstrate significant disease-modifying effects of drugs and drug candidates. For example, when new disease modifiers are being evaluated in a FAP clinical trial, such surrogate biomarkers have the potential to provide an objective, quantitative and mechanistic molecular diagnostic of disease response to therapy.