Centro Hospitalar do Porto
Repositório Científico da Unidade Local de Saúde de Santo AntónioNot a member yet
2440 research outputs found
Sort by
Evaluation of Patterns of Presentation, Practice, and Outcomes of Upper Tract Urothelial Cancer: Protocol for an Observational, International, Multicenter, Cohort Study by the Clinical Research Office of the Endourology Society
Get PDFBackground: Available guidelines on the management of upper tract urothelial carcinoma (UTUC) are restricted due to the lack of strong evidence-based recommendations. Adequate, well-powered randomized trials are missing due to the rarity of the disease. To overcome this problem, we need alternative study designs to provide generalizable data.
Objective: The primary aim of this registry is to provide a real-world overview on patterns of presentation and management of UTUC. Secondary objectives include comparison of outcomes of different treatments and tumor stages and evaluation of compliance with the current European Association of Urology recommendations for UTUC.
Methods: For this observational, international, multicenter, cohort study, clinical data of consecutive patients suspected of having UTUC, irrespective of type of management, will be prospectively collected up to 5 years after inclusion. Data on the patterns of presentation, diagnostics, and treatment as well as short-, mid-, and long-term oncological and functional outcomes will be analyzed. Possible associations between variables, basal characteristics, and outcomes will be tested by multivariable analyses. The methodology will address potential sources of bias and confounders.
Results: The registry was initiated in November 2014 after obtaining institutional review board approval. Data collection started in December 2014. At the time of submission of this manuscript, 2451 patients from 125 centers from 37 countries were included. Inclusion of patients will be closed 5 years after initiation of the registry. Quality checks will be performed centrally with continuous communication and feedback with the centers to ensure accuracy. The first results are expected in the first trimester of 2020.
Conclusions: This large observational prospective cohort will generate landmark "real-world" data and hypotheses for further studies. We expect these data to optimize the management of UTUC, provide insights on harms and benefits of treatment, and serve as quality control.info:eu-repo/semantics/publishedVersio
The long-term safety and efficacy of vestronidase alfa, rhGUS enzyme replacement therapy, in subjects with mucopolysaccharidosis VII
Get PDFVestronidase alfa (recombinant human beta-glucuronidase) is an enzyme replacement therapy (ERT) for Mucopolysaccharidosis (MPS) VII, a highly heterogeneous, ultra-rare disease. Twelve subjects, ages 8-25 years, completed a Phase 3, randomized, placebo-controlled, blind-start, single crossover study (UX003-CL301; NCT02377921), receiving 24-48 weeks of vestronidase alfa 4 mg/kg IV. All 12 subjects completed the blind-start study, which showed significantly reduced urinary glycosaminoglycans (GAG) and clinical improvement in a multi-domain responder index, and enrolled in a long-term, open-label, extension study (UX003-CL202; NCT02432144). Here, we report the final results of the extension study, up to an additional 144 weeks after completion of the blind-start study. Three subjects (25%) completed all 144 weeks of study, eight subjects (67%) ended study participation before Week 144 to switch to commercially available vestronidase alfa, and one subject discontinued due to non-compliance after receiving one infusion of vestronidase alfa in the extension study. The safety profile of vestronidase alfa in the extension study was consistent with observations in the preceding blind-start study, with most adverse events mild to moderate in severity. There were no treatment or study discontinuations due to AEs and no noteworthy changes in a standard safety chemistry panel. Out of the eleven subjects who tested positive for anti-drug antibodies at any time during the blind-start or extension study, including the baseline assessment in the blind-start study, seven subjects tested positive for neutralizing antibodies and all seven continued to demonstrate a reduction in urinary GAG levels. There was no association between antibody formation and infusion associated reactions. Subjects receiving continuous vestronidase alfa treatment showed a sustained urinary GAG reduction and clinical response evaluated using a multi-domain responder index that includes assessments in pulmonary function, motor function, range of motion, mobility, and visual acuity. Reduction in fatigue was also maintained in the overall population. As ERT is not expected to cross the blood brain barrier, limiting the impact on neurological signs of disease, and not all subjects presented with neurological symptoms, outcomes related to central nervous system pathology are not focused on in this report. Results from this study show the long-term safety and durability of clinical efficacy in subjects with MPS VII with long-term vestronidase alfa treatment.This work was supported by Ultragenyx Pharmaceutical Inc. LZ, TC, DM, and AJ, authors of this manuscript, are employees of Ultragenyx Pharmaceutical and contributed to the conduct of the research; the study design; data collection, analysis and interpretation; development of this manuscript; and the decision to submit this article.info:eu-repo/semantics/publishedVersio
Risk of burnout in radiology technicians in care units of Porto
Get PDFIntrodução – O burnout é descrito como uma síndroma que envolve exaustão emocional, física e mental e que resulta da exposição continuada ao stress laboral. É caracterizada por elevada exaustão emocional, despersonalização e baixa realização pessoal. Objetivo – Este estudo procurou avaliar o risco de burnout nos técnicos de radiologia que exercem funções em instituições de saúde da região do Porto, Portugal. Materiais e Métodos – Os níveis de burnout foram estimados através do Maslach Burnout Inventory – Human Services Survey composto por 22 questões. A amostra foi constituída por 122 indivíduos, provenientes de sete instituições de saúde, públicas e privadas, em que 61,5% eram do sexo feminino, com média de idades e desvio-padrão de 38,3 (±9,3) anos. Na análise relacional entre as variáveis sociodemográficas e os resultados do MBI-HSS foram utilizados os testes Qui-quadrado de Pearson ou Exato de Fisher, conforme a percentagem de células com contagem menor que 5 era menor ou maior que 20% para as variáveis qualitativas. Para as variáveis quantitativas realizaram-se os testes ANOVA ou Kruskal-Wallis, conforme a homogeneidade da variância entre grupos. A interpretação dos testes estatísticos correlacionais foi efetuada com base no nível de significância de α=0,05 com intervalo de confiança de 95%. Resultados – A análise dos níveis das dimensões do burnout revelou valores médios de 21,73 (±11,37) para a exaustão emocional, de 7,42 (±5,40) para a despersonalização e de 32,79 (±8,46) para a realização pessoal, o que se coaduna com níveis moderados para as duas primeiras dimensões e baixos para a última. Deste grupo de técnicos, 39,3% e 29,5% exibem elevados níveis de exaustão emocional e despersonalização, respetivamente. A baixa realização pessoal é experienciada por 48,4% dos inquiridos. Discussão – As mulheres são mais afetadas pela exaustão emocional (51%), bem como os profissionais com tempo na função entre os 11 e os 30 anos. Em serviços compostos por 20 a 40 técnicos observa-se um nível mais elevado de exaustão emocional (63%). A maioria dos inquiridos afirma que o trabalho afeta a sua autoestima, vida familiar e social, nomeadamente de forma negativa, o que posteriormente se relaciona com alguns valores elevados de exaustão emocional e despersonalização e baixos de realização pessoal. Conclusão – A ocorrência de burnout nos técnicos de radiologia é relevante e estes resultados alertam para a necessidade de intervir no sentido de melhorar as condições de trabalho, formação contínua e implementação de medidas de suporte emocional dos profissionais de saúde, de forma a garantir a qualidade do serviço prestado aos utentes e o bem-estar pessoal destes profissionais.info:eu-repo/semantics/publishedVersio
Caso clínico dermatológico
Get PDFA previously healthy 15-year-old girl was sent to the Pediatric Emergency Department by the general practitioner due to suspicion of a hematological condition. The girl presented with ecchymotic lesions on the anterior region of the thorax and antero-proximal region of the upper limbs, which had apparently worsened in the last 24 hours. She denied trauma or impact sports, fever or recent infections, and blood loss or other symptoms. Laboratory evaluation showed no alterations and urine toxicology screening was negative. When confronted with normal laboratory evaluation, the girl confessed intentional self-inflicted injuries following a discussion with her mother. Parents were made aware of the situation, and a pediatric psychiatry consultation was recommended. After discharge, follow-up in the girl’s home country was assured.
With this report, the author intend to raise awareness of the importance of maintaining a high index of suspicion to non-suicidal self-injury, identifying alarm signs and establishing early preventive strategies.info:eu-repo/semantics/publishedVersio
Asymmetric Thickness Intracorneal Ring Segments for Keratoconus
Get PDFPurpose: To describe the anatomical and functional results of the implantation of asymmetric thickness intracorneal ring segments (AS-ICRS) in eyes with keratoconus and asymmetric/irregular astigmatism (type 2 - Duck - and type 3 - Snowman - phenotypes, Fernandez-Vega/Alfonso morphologic Keratoconus classification).
Materials and methods: Retrospective observational study including 19 consecutive patients (21 eyes) with keratoconus who underwent manual implantation of the Keraring® Asymmetric ICRS (AS). Analysis included demographic and clinical data and Pentacam (Oculus®) measurements: topographic astigmatism; topographic flat meridian (K1), steepest meridian (K2) and the maximum keratometric point (Kmax); total root mean square (RMS) and high order RMS (HOA) aberrations and comatic Zernike coefficients (Z31;Z3-1) at 0º and 90º meridians.
Results: Overall mean age was 35.3±11.7 years and median follow-up was 7.1 months (range 6-12). At the end of follow-up, a significant improvement from baseline was achieved in both UDVA (0.24±0.22; p=0.017) and CDVA (0.21±0.17; p0.05 in all).
Conclusion: Implantation of the Keraring® Asymmetric ICRS in keratoconus with asymmetric/irregular astigmatism allowed an improvement of several clinical, topographic and aberrometric parameters, with clinical efficacy and safety, with a tendency to a greater effect in the type 2 cones.info:eu-repo/semantics/publishedVersio
Immediate Reactions to Fluorescein and Indocyanine Green in Retinal Angiography: Review of Literature and Proposal for Patient’s Evaluation
Get PDFIntroduction: Contrast rapid sequence angiography with fluorescein or indocyanine green (ICG) is a diagnostic procedure commonly used in ophthalmology. Adverse reactions to fluorescein and ICG are rare and may be classified as toxic, of hypersensitivity and non-specific. The evaluation and management of a patient with an adverse reaction is a challenge for the majority of ophthalmologists, as is the assessment of risk factors that may contraindicate the procedure.
Purpose: We aim to review the concepts underlying adverse reactions to fluorescein and ICG, especially those of hypersensitivity, and present a proposal or the evaluation of the patients in need to perform retinal angiography and for the treatment of immediate reactions to fluorescein and ICG.
Methods: The available literature was examined using PubMed-Medline, and using the MeSH terms "fluorescein", "Indocyanine green", "ophthalmic dyes", "retinal angiography", "adverse reactions", and "allergic reaction".
Conclusion: This review may help ophthalmologists to identify patients with higher risk of a hypersensitivity reaction and give them tools to recognize patients with suspected hypersensitivity that may benefit from an allergy study.info:eu-repo/semantics/publishedVersio
Anafilaxia em idade pediátrica: Uma visão global
Get PDFAnaphylaxis is defined as an acute severe, life-threatening hypersensitivity reaction. The condition’s real prevalence and incidence are difficult to estimate, but seem to be increasing, particularly in children. Anaphylaxis clinical presentation varies according to age and other individual factors. Although consensual clinical criteria exist, including in pediatric age, diagnosis can be challenging. Food allergy is the most common anaphylaxis cause in children, particularly in preschool age. Drug-induced reactions and hymenoptera venom sting are other major triggers, which importance increases after adolescence. Management involves diagnosis, appropriate identification of possible triggers, acute phase treatment, and long-term planning. Prompt referral to a Pediatric Allergy specialist is recommended, as complete allergy workup is usually required to implement future preventive measures. In this review, the authors discuss particular aspects regarding anaphylaxis in pediatric age to provide information that can help improve disease management.info:eu-repo/semantics/publishedVersio
Dermatomiosite juvenil: manifestações comuns de uma doença rara
Get PDFJuvenile dermatomyositis is an autoimmune systemic vasculopathy, mainly characterized by chronic inflammation of the striated muscle and skin. The authors report the case of a 15-year-old boy presenting with a four-month history of myalgia and proximal muscle weakness on the upper and lower limbs. These symptoms were associated with heliotrope palpebral exanthema, erythema in the dorsum of the hands, Gottron’s papules, erythematous and petechial rash on the extensor face of the thighs, and mild dysphagia for solids. Blood tests revealed an increase in muscle enzymes and electromyography showed changes suggestive of severe acute myopathy. Intravenous methylprednisolone was initiated, followed by a combination regimen of prednisolone and methotrexate. Progressive dysphagia, cutaneous abnormality, and muscular strength improvement were noted. With this case, the authors intend to raise awareness of a rare disease with an essentially clinical diagnosis, presenting in most cases with characteristic manifestations that should be recognised.info:eu-repo/semantics/publishedVersio
Surgical management of bone and soft tissue sarcomas and skeletal metastases during the COVID-19 pandemic
Get PDFinfo:eu-repo/semantics/publishedVersio
Freeze-Dried Softisan® 649-Based Lipid Nanoparticles for Enhanced Skin Delivery of Cyclosporine A
Get PDFInflammatory skin diseases, including psoriasis and atopic dermatitis, affect around one quarter to one third of the world population. Systemic cyclosporine A, an immunosuppressant agent, is included in the current therapeutic armamentarium of these diseases. Despite being highly effective, it is associated with several side effects, and its topical administration is limited by its high molecular weight and poor water solubility. To overcome these limitations, cyclosporine A was incorporated into solid lipid nanoparticles obtained from Softisan® 649, a commonly used cosmetic ingredient, aiming to develop a vehicle for application to the skin. The nanoparticles presented sizes of around 200 nm, low polydispersity, negative surface charge, and stability when stored for 8 weeks at room temperature or 4 °C. An effective incorporation of 88% of cyclosporine A within the nanoparticles was observed, without affecting its morphology. After the freeze-drying process, the Softisan® 649-based nanoparticles formed an oleogel. Skin permeation studies using pig ear as a model revealed low permeation of the applied cyclosporine A in the freeze-dried form of the nanoparticles in relation to free drug and the freshly prepared nanoparticles. About 1.0 mg of cyclosporine A was delivered to the skin with reduced transdermal permeation. These results confirm local delivery of cyclosporine A, indicating its promising topical administration.info:eu-repo/semantics/publishedVersio