Centro Hospitalar do Porto
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International multicentre observational study to evaluate the association between perioperative red blood cell transfusions and 1-year mortality after major cancer surgery (ARCA-1): study design, statistical analysis plan and study protocol
Get PDFIntroduction: Blood transfusion is still common in patients undergoing major cancer surgery. Blood transfusion can be associated with poor prognosis in patients with cancer. Perioperative Care in the Cancer Patient -1 (ARCA-1) aims to assess in a large cohort of patients the current incidence, pattern of practice and associations between perioperative blood transfusions and 1-year survival in patients undergoing major cancer surgery.
Methods and analysis: ARCA-1 is a prospective international multicentre observational study that will include adult patients scheduled to have major cancer surgical procedures with the intention to cure, and an overnight planned hospital admission. The study will be opened for 1 year for enrolment (7 January 2020-7 February 2021). Each centre will enrol patients for 30 days. The primary endpoint of this study is all-cause mortality 1 year after major cancer surgery. Secondary endpoints are rate of perioperative blood product use, cancer-specific mortality at 1 year and PFSs and 30-day morbidity and mortality.
Ethics and dissemination: This study was approved by the Institutional Review Board at The University of Texas-MD Anderson Cancer Center. The study results will be published in peer-reviewed journals and disseminated at international conferences.
Trial registration number: NCT04491409.info:eu-repo/semantics/publishedVersio
Digital Rehabilitation for Acute Ankle Sprains: Prospective Longitudinal Cohort Study
Get PDFClinicalTrials.gov NCT04819022; https://clinicaltrials.gov/ct2/show/NCT04819022Background: Ankle sprains are one of the most prevalent soft-tissue injuries worldwide. Physical therapy, especially progressive exercise, has proven effective in improving function, while preventing recurrence.
Objective: We aim to present the results of a fully remote and digitally guided rehabilitation program for acute ankle sprains.
Methods: We performed a prospective longitudinal cohort study of individuals eligible for workers' compensation, who were referred for digital rehabilitation therapy for a sprained ankle. Therapeutic exercise sessions were to be performed independently by the patient at home using the biofeedback device provided by SWORD Health. Primary endpoints were the change in self-reported Numerical Pain Rating Scale (NPRS) and Foot and Ankle Ability Measure-activities of daily living (FAAM-ADL) and FAAM-Sports scores. Participants were assessed at baseline, end of the program, and 6 months after program completion. Secondary outcomes included digital therapy dosage, pain and fatigue during sessions, and satisfaction.
Results: In total, 93 (89.4%) patients completed the program and 79 (76.0%) were available for follow-up. Changes in the primary outcomes between baseline and the 6-month follow-up were both significant (P<.001) and clinically meaningful: mean difference of -2.72 points (95% CI -3.31 to -2.13) on the NPRS (49.8% reduction), 21.7 points (95% CI 17.13-26.27) on the FAAM-ADL (41.1% increase), and 37.8 points (95% CI 30.45-45.15) on the FAAM-Sports (151.8% increase). Longer waiting periods between the accident date and treatment initiation were found to negatively impact functional status at baseline and at the end of the program, triggering an extension in the program duration. The total training volume (12.5 hours, SD 10.5 hours) was similar to that of other interventions for ankle sprains, but the dosage per week was much higher (2.4 hours per week, SD 0.87 hours per week). The mean patient satisfaction score was 8.8 (SD 1.57) out of 10. Among program completers, 83.9% attained full recovery and were discharged with no residual disability.
Conclusions: Being far less demanding in terms of human resources, the digital program presented constituted a viable, clinically effective, and convenient solution for ankle sprain rehabilitation, particularly during the pandemic. This is the first study presenting a fully remote home-based rehabilitation program for acute ankle sprains, with patients achieving sustained long-term results. This was a prospective cohort study and, as such, did not include a control group, but the results appear comparable to those published for face-to-face interventions.info:eu-repo/semantics/publishedVersio
Aggressive Neuroblastoma in a Pediatric Patient with Severe Hemophilia A
Get PDFDespite the extensive information regarding hemophilia's hemorrhagic complications, the literature on cancer in hemophilia is scarce, especially in pediatric patients. Many uncertainties remain concerning diagnosis and workup. We report a rare case of two severe diseases (neuroblastoma and hemophilia A (HA)) concomitantly present in the same pediatric patient. We highlight that the diagnosis of severe HA did not have a negative impact on the patient's oncologic course. This case also illustrates the significance of the cooperation among different specialties and hospitals when caring for the same patient.info:eu-repo/semantics/publishedVersio
A Importância dos Testes Epicutâneos na Dermatite Anogenital: Dez Anos de Experiência de um Centro de Referência Terciário no Norte de Portugal
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International Consensus on Antinuclear Antibody em Portugal
Get PDFIntroduction: Screening for autoantibodies in HEp-2 cells by indirect immunofluorescence is currently accepted as the gold-standard test for the diagnosis of systemic autoimmune diseases. The main objective of the International Consensus on ANA Patterns is to achieve a consensus on the nomenclature and description of antinuclear antibody morphological patterns. This work aims to build on the International Consensus on ANA Patterns project to establish a nomenclature consensus in Portugal, thus contributing to harmonization in autoimmune diagnosis and promoting diagnostic quality in autoimmune systemic rheumatic diseases.
Material and methods: Participating laboratories identified all the nuclear and cytoplasmic pattern designations in the International Consensus on ANA Patterns (including the anti-cell pattern code), and matched them with the corresponding Portuguese nomenclature in use. The results were aggregated and used as a foundation for nomenclature harmonization work. Consensus meetings followed an iterative process, until a final consensual proposal was drafted.
Results: Prior agreement between laboratories was over 75% for 23 of the total 29 anti-cell patterns. The degree to which each laboratory is aligned with the International Consensus on ANA Patterns international reference ranges from 22.1% to 100%. It was possible to write a consensual version of the International Consensus on ANA Patterns nomenclature for Portugal.
Discussion: There was a good consensus basis for the nomenclature in the International Consensus on ANA Patterns, despite relevant differences with some translations. The study highlights the need for collaboration among laboratories towards an unambiguous description of laboratory results.
Conclusion: This study shows that there is good potential for collaboration between laboratories in order to produce the consensus needed to improve diagnosis and patient follow-up.Introdução: A pesquisa de autoanticorpos em células HEp-2 através de imunofluorescência indireta é o teste padrão atualmente aceite como a ferramenta central para o diagnóstico das doenças autoimunes sistémicas. O International Consensus on Antinuclear Antibody (ANA) Patterns tem como objetivo principal alcançar um consenso na nomenclatura e na descrição dos diferentes padrões morfológicos de anticorpos antinucleares. Este trabalho tem como objetivo ampliar o projeto do International Consensus on ANA Patterns de forma a estabelecer um consenso em Portugal para a sua nomenclatura, procurando contribuir para a harmonização no diagnóstico autoimune e promover a qualidade diagnóstica nas doenças reumáticas sistémicas autoimunes.Material e Métodos: Os laboratórios participantes identificaram cada designação de padrão citoplasmático e nuclear do International Consensus on ANA Patterns (incluindo o código padrão anti-célula), e fizeram corresponder a cada uma a respetiva nomenclatura portuguesa em uso. Os resultados foram agregados e serviram de base ao trabalho de harmonização da nomenclatura. Seguiram-se reuniões de consenso, num processo iterativo até à redação de uma proposta final consensualizada.Resultados: A concordância prévia entre laboratórios era superior a 75% para 23 do total de 29 padrões anti-célula. O grau em que cada laboratório está alinhado com a referência internacional do International Consensus on ANA Patterns varia entre 22,1% e 100%. Foi possível elaborar uma versão consensualizada da nomenclatura do International Consensus on ANA Patterns para Portugal.Discussão: Existia uma boa base de consenso para a nomenclatura do International Consensus on ANA Patterns, mas com diferenças importantes em algumas das traduções da terminologia. O estudo realça a necessidade de colaboração entre laboratórios para uma descrição inequívoca dos resultados laboratoriais.Conclusão: Este trabalho mostra o potencial positivo da colaboração entre laboratórios para gerar consensos que contribuam para a melhoria do diagnóstico e acompanhamento dos doentes.info:eu-repo/semantics/publishedVersio
Complicações Cardiovasculares Associadas à Infeção por COVID-19
Get PDFIntroduction: Reports of cardiovascular complications related to the COVID-19 infection have been frequent.
Methods: Narrative review for relevant articles on the topic. The classic cardiovascular risk factors, like age, obesity, diabetes, and hypertension are associated with adverse outcomes in COVID-19 patients. Cardiovascular complications can have a diverse clinical presentation including silent myocardial injury, acute coronary syndromes, thromboembolism, cardiac arrhythmias, and heart failure. There are multiple mechanisms of cardiac injury that are not mutually exclusive. The approach to diagnosis and management should be carried out according to usual practice, while considering the particularities of COVID-19 infection.
Conclusion: The interaction between SARS-CoV-2 and the heart is complex and is manifested in multiple ways. Regardless of the clinical presentation, cardiac complications convey a worse prognosis. Patients should be actively monitored and treated accordingly.Introdução: As complicações cardiovasculares associadas a infeção por COVID-19 têm sido frequentemente reportadas. Métodos: Revisão da literatura sobre os artigos relevantes neste tópico. Os fatores de risco cardiovasculares clássicos como idade, obesidade, diabetes e hipertensão foram associados a um risco maior de evolução desfavorável. As complicações cardiovasculares podem ter uma apresentação clínica variável incluindo lesão miocárdica, síndrome coronário agudo, trombo-embolismo, arritmias e insuficiência cardíaca. Estão descritos múltiplos mecanismos de lesão cardíaca os quais não são mutualmente exclusivos. A abordagem diagnóstica e terapêutica deve seguir a prática comum tendo, no entanto, em consideração as particularidades da infeção por COVID-19. Conclusão: A interação entre a infeção por COVID-19 e o coração é complexa e manifesta-se de várias formas. Independentemente da apresentação clínica, as complicações cardíacas conferem um prognostico desfavorável pelo que devem ser monitorizadas ativamente e tratadas de forma apropriada.info:eu-repo/semantics/publishedVersio
Detailed Clinical and Psychological Phenotype of the X-linked HNRNPH2-Related Neurodevelopmental Disorder
Get PDFObjective: To expand the clinical phenotype of the X-linked HNRNPH2-related neurodevelopmental disorder in 33 individuals.
Methods: Participants were diagnosed with pathogenic or likely pathogenic variants in HNRNPH2 using American College of Medical Genetics and Genomics/Association of Molecular Pathology criteria, largely identified via clinical exome sequencing. Genetic reports were reviewed. Clinical data were collected by retrospective chart review and caregiver report including standardized parent report measures.
Results: We expand our clinical characterization of HNRNPH2-related disorders to include 33 individuals, aged 2-38 years, both females and males, with 11 different de novo missense variants, most within the nuclear localization signal. The major features of the phenotype include developmental delay/intellectual disability, severe language impairment, motor problems, growth, and musculoskeletal disturbances. Minor features include dysmorphic features, epilepsy, neuropsychiatric diagnoses such as autism spectrum disorder, and cortical visual impairment. Although rare, we report early stroke and premature death with this condition.
Conclusions: The spectrum of X-linked HNRNPH2-related disorders continues to expand as the allelic spectrum and identification of affected males increases.Grant support for L. Boyle provided by TL1TR001875.info:eu-repo/semantics/publishedVersio
Microperimetry as Part of Multimodal Assessment to Evaluate and Monitor Myopic Traction Maculopathy
Get PDFPurpose: To characterize a population of high myopes with myopic traction maculopathy (MTM), to assess their retinal function, and to correlate it with anatomic status.
Patients and methods: This was an observational cross-sectional study including 50 eyes from 27 patients. Demographic and clinical data were analyzed. Macular structure was assessed with spectral domain optical coherence tomography (SD-OCT, Heidelberg®) and macular function was studied with Microperimeter MP-3, NIDEK®.
Results: The average for central foveal thickness (CFT) and choroid thickness (CT) was 213±151 μm and 36±23 μm, respectively, in a total of 50 eyes from 27 patients. In the microperimetry analysis, the average sensitivity on the foveal-centered 12º polygon (CPS) was 14.37±9.1 dB. CT was negatively associated with the bivariate contour ellipse areas (BCEA) 1 (r=-0.314; p=0.034), 2 (r=-0.314; p=0.034), and 3 (r=-0.316; p=0.033). CPS had a strong positive correlation with best-corrected visual acuity (BCVA) (r=0.661; p=0.000). We found a trend to worse microperimetric results in eyes with schisis (n=19) (p>0.05) but eyes with atrophic areas (n=33) presented significant inferior CPS (p<0.001). The presence of staphyloma showed significant impact on macular sensitivities in eyes with areas of macular atrophy/fibrosis (p<0.05).
Conclusion: Macular microperimetry analysis can have a role as part of a multimodal anatomo-functional assessment for a more precise characterization of the high myopic patients with MTM, optimizing medical and surgical decisions.info:eu-repo/semantics/publishedVersio
The Role of Corneal Biomechanics in the Assessment of Ectasia Susceptibility Before Laser Vision Correction
Get PDFPurpose: To describe the tomographic and corneal biomechanical status of a sample of eyes excluded from LVC and to present the differences in biomechanical behavior in relation to cutoffs of clinical- and tomography-based screening methods used in clinical practice.
Patients and methods: Observational cross-sectional study including 61 eyes from 32 consecutive patients who were excluded from LVC in our department. Clinical and demographic data were collected from the patients' clinical records. Tomographic data was assessed with a Scheimpflug camera (Pentacam, OCULUS®). Ablation depth (µm) and residual stromal bed (µm) were calculated by the WaveLight® EX500 laser system software (Alcon, EUA). The corneal biomechanical assessment was made through ultra-high speed Scheimpflug imaging during noncontact tonometry (Corvis ST, OCULUS®). Several ectasia risk scores were analyzed.
Results: Mean age was 31.0±6 years old and mean manifest spherical equivalent was -2.01 ± 2.3D. Belin-Ambrósio deviation index was the tomographic parameter with higher proportion of eyes within the ectasia high risk interval. In the biomechanical assessment, more than 95% of eyes met the criteria for ectasia susceptibility in four of the first generation and in two of the second generation parameters. In a cutoff based comparative analysis, eyes with Kmax ≥45.5 D, eyes with VCOMA <0 and eyes with ARTmax ≤350 presented significantly softer corneal biomechanical behavior.
Conclusion: The majority of eyes excluded from LVC in the present study met the criteria for ectasia susceptibility in several biomechanical parameters, validating the clinical and tomographic based screening prior to LVC in our center. Differences found in the biomechanical assessment regarding cutoffs used in clinical practice highlight its differential role in characterizing risk profile of these patients. Tomography should not be overlooked and the integration of all data, including treatment-related parameters, can be the future of risk ectasia screening prior LVC.info:eu-repo/semantics/publishedVersio
A contribution to the establishment of diagnostic reference levels in intervention electrophysiology: study from a center in Portugal
Get PDFA maioria dos procedimentos eletrofisiologia de intervenção necessitam de fluoroscopia expondo à radiação ionizante todos os intervenientes. O recurso a níveis de referência de diagnóstico (NRD) pode ser uma ferramenta profícua para promover a otimização de dose. O principal objetivo deste estudo consistiu em investigar as doses de exposição, determinando os NRD. Realizou-se um estudo institucional, transversal e retrospetivo, através da revisão dos procedimentos de estudos eletrofisiológicos de diagnóstico e/ou tratamento e de implantação de dispositivos cardíacos, realizados entre 23 de maio de 2018 e 19 de fevereiro de 2020. Os procedimentos foram distribuídos em grupos de acordo com a sua complexidade e aferidos os seus NRD para os descritores de dose Cumulative air kerma (CK, mGy) e Air kerma area product (PKA, mGy.cm2).
Este estudo incluiu 516 procedimentos. Os NRD determinados foram: estudo eletrofisiológico de diagnóstico (12mGy e 3,5Gy.cm2); estudo eletrofisiológico com ablação em cavidades cardíacas direitas (62mGy e 20Gy.cm2); ou em cavidades cardíacas esquerdas (87,5mGy e 32Gy.cm2); ablação por fibrilhação auricular (103mGy e 34Gy.cm2); implantação de cardioversor-desfibrilhador ou pacemaker com um eletrocateter de fixação passiva (7mGy e 2,5Gy.cm2), ou ativa (10mGy e 3Gy.cm2), e com dois eletrocateteres de fixação passiva (9mGy e 3Gy.cm2), ou ativa (15mGy e 4,5Gy.cm2), ou um ativo e um passivo (12mGy e 4Gy.cm2); implantação de dispositivo de ressincronização cardíaca (158,5mGy e 35Gy.cm2) ou upgrade (98mGy e 36Gy.cm2). Os procedimentos mais complexos foram os que apresentaram os NRD mais elevados.
Verificou-se que a maioria dos NRD determinados se situa abaixo dos valores publicados na literatura.Most interventional electrophysiology procedures require fluoroscopy therefore exposing all intervening parties to ionizing radiation. The use of diagnostic reference levels (DRLs) can be a useful tool to promote dose optimization. The main objective of this study was to investigate the exposure doses, determining the DRLs.
An institutional study, cross-sectional and retrospective was carried out, through the review of electrophysiological diagnostic and/or treatment study procedures and the implantation of cardiac devices, performed between May 23, 2018 and February 19, 2020. The procedures were distributed in groups according to their complexity and determined their diagnostic reference levels for the dose descriptors Cumulative air kerma (CK, mGy) and Air kerma area product (PKA, mGy.cm2). This study included 516 procedures. The determined DRLs were: electrophysiological diagnostic study (12mGy and 3,5Gy.cm2); electrophysiological study with ablation in right heart cavities (62mGy and 20Gy.cm2); electrophysiological study with ablation in left heart cavities (87,5mGy and 32Gy.cm2); atrial fibrillation ablation (103mGy and 34Gy.cm2); implantation of a cardioverter-defibrillator or pacemaker with a passive fixation lead (7mGy and 2,5Gy.cm2) or active (10mGy and 3Gy.cm2) and with two passive fixation leads (9mGy and 3Gy.cm2), or active (15mGy and 4,5Gy.cm2), or an active and one passive (12mGy and 4Gy.cm2); implantation of a cardiac resynchronization device (158,5mGy and 38Gy.cm2) or upgrade (98mGy and 36Gy.cm2). The most complex procedures were those with the highest DRLs.
It was determined that most of the DRLs found are below the values published in the literature.info:eu-repo/semantics/publishedVersio