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O Que Pensam e Sentem os Cardiologistas Portugueses Sobre o Seu Trabalho?
No full textIntroduction and objectives: This study reports the results of an online survey carried out by the Portuguese Society of Cardiology about its medical members' work characteristics before and during the COVID-19 pandemic, their job satisfaction, work motivation, and burnout.
Methods: A sample of 157 participants answered a questionnaire with demographic, professional, and health-related information, followed by questionnaires on job satisfaction and motivation designed and validated for this study and a Portuguese version of the Maslach Burnout Inventory. Data were analyzed through descriptive statistics, ANOVA, and MANOVA, considering gender, professional level, and sector of activity, respectively. Multiple regression was used to assess the impact of job satisfaction and motivation on burnout.
Results: The only variable that distinguished participants was sector of activity. Cardiologists working in the private sector worked fewer weekly hours during COVID-19, while those in the public sector worked more. The latter expressed more desire to reduce their working hours than those who worked in private medicine and in both sectors. There were no differences between sectors in work motivation, while job satisfaction was higher in the private sector. Moreover, job satisfaction negatively predicted burnout.
Conclusions: Our findings point to a deterioration in working conditions during the COVID-19 pandemic, with its consequences being felt especially in the public sector, which may have contributed to the lower levels of satisfaction among cardiologists who worked exclusively in this sector, but also for those working in both public and private sectors.info:eu-repo/semantics/publishedVersio
Strategy Nursing in Children with Compromised Ventilation: Umbrella Review
No full textProblem: Changes in the ventilation demand nursing interventions duly adapted to the management of said impairment and to the adaptability of the child/parents. This revision aimed to investigate the evidence behind the interventions performed on children with impaired ventilation.'
Eligibility criteria: Systematic reviews of literature in English, Spanish, French, and Portuguese from studies on nursing interventions related to children with impaired ventilation in all contexts of the clinical practice. The Joanna Briggs Institute recommendations were followed.
Sample: We conducted a comprehensive search as of January 2022 and updated as of June 2023. The following electronic databases were searched: SCOPUS, Web of Science, Joanna Briggs Institute Database of Systematic Reviews and Implementation Reports, MEDLINE (via PubMed), CINAHL (via EBSCO), MedicLatina (via EBSCO), The Cochrane Database of Systematic Reviews (via EBSCO), and Database of Abstracts of Reviews of Effects (DARE). Nineteen articles published between 2012 and 2022 were included in this review.
Results: Nineteen studies investigated the efficacy of respiratory exercises (Breathing Control - relaxed breathing, pursed lip breathing, Diaphragmatic breathing exercises, respiratory expansion exercise - deep breathing exercise, thoracic expansion exercises (with device), exercises for respiratory muscle strengthening and position to optimize ventilation. In the majority of the studies, it was not possible to evaluate the interventions separately. Thirteen studies evidenced the efficacy of respiratory exercises, BIPAP, and oxygen therapy. Seven articles demonstrated the effectiveness of respiratory muscle-strengthening exercises, and only three mentioned the efficacy of positioning regarding impaired ventilation. Interventions based on respiratory exercises and respiratory muscle training were the most common ones.
Conclusions: The results suggest that nursing interventions to optimize ventilation are efficient. Nevertheless, the same present a low to moderate evidence degree, justified by the population characteristics (small and heterogeneous).
Implications: There is proof of evidence for the studied interventions. However, the lack of methodological robustness points to future research to duly describe interventions, data, and comparable results, using reliable samples in which the focus of the study is clear.info:eu-repo/semantics/publishedVersio
Intestinal Hemangiomatosis: Case Report of an Uncommon Cause of Rectal Bleeding
No full textA four-month-old girl presented with recurrent low gastrointestinal hemorrhage. Abdominal ultrasound showed diffuse parietal thickening and hyperemia of the colon. Computed tomography (CT) demonstrated diffuse thickening of the colon but also intense arterial globular mural enhancement with diffuse filling in the portal phase. Colonoscopy revealed multiple pseudopolipoid lesions along the colon which were histologically diagnosed as hemangiomas. The infant was diagnosed with gastrointestinal hemangiomatosis and was treated with propranolol resulting in complete resolution of symptoms.info:eu-repo/semantics/publishedVersio
The GAIN Registry — a New Prospective Study for Patients with Multi-organ Autoimmunity and Autoinflammation
No full textPatient registries are a very important and essential tool for investigating rare diseases, as most physicians only see a limited number of cases during their career. Diseases of multi-organ autoimmunity and autoinflammation are especially challenging, as they are characterized by diverse clinical phenotypes and highly variable expressivity. The GAIN consortium (German multi-organ Auto Immunity Network) developed a dataset addressing these challenges. ICD-11, HPO, and ATC codes were incorporated to document various clinical manifestations and medications with a defined terminology. The GAIN dataset comprises detailed information on genetics, phenotypes, medication, and laboratory values. Between November 2019 and July 2022, twelve centers from Europe have registered 419 patients with multi-organ autoimmunity or autoinflammation. The median age at onset of symptoms was 13 years (IQR 3-28) and the median delay from onset to diagnosis was 5 years (IQR 1-14). Of 354 (84.5%) patients who were genetically tested, 248 (59.2%) had a defined monogenetic cause. For 87 (20.8%) patients, no mutation was found and for 19 (4.5%), the result was pending. The most common gene affected was NFkB1 (48, 11.5%), and the second common was CTLA4 (40, 9.5%), both genetic patient groups being fostered by specific research projects within GAIN. The GAIN registry may serve as a valuable resource for research in the inborn error of immunity community by providing a platform for etiological and diagnostic research projects, as well as observational trials on treatment options.info:eu-repo/semantics/publishedVersio
The Impact of Donor Risk Index, Recipients’ and Operative Characteristics on Post Liver Transplant One-Year Graft Failure: A Cohort Analysis
No full textBackground and Aims: The donor risk index (DRI) quantifies donor-related characteristics potentially associated with increased risk of early graft failure. We aimed to assess the impact of the DRI, recipient and perioperative factors on post liver transplant (LT) outcomes. Methods: This was a singlecenter retrospective cohort study including all adult (≥18 years) patients who underwent LT from 01/2019 to 12/2019 at Curry Cabral Hospital, Lisbon, Portugal. Primary endpoint was 1-year graft failure post LT. Associations were studied with logistic regression. Results: A total of 131 cadaveric donor LT procedures were performed in 116 recipients. Recipients’ median (IQR) age was 57 (47–64) years and 101/131 (77.1%) were males. Cirrhosis was the underlying etiology in 95/131 (81.2%) transplants. Based on 8 predefined donors’ characteristics, median (IQR) DRI was 1.96 (1.67–2.16). Following adjustment for MELDNa score pre LT and SOFA score (adjusted odds ratio [aOR], 95% confidence interval [CI] = 0.91 [0.56–1.47]) or lactate (aOR [95% CI] = 2.76 [0.71–10.7]) upon intensive care unit (ICU) admission post LT, DRI was not associated with 1-year graft failure. However, higher SOFA
score (aOR [95% CI] = 1.20 [1.05–1.37]) or lactate (aOR [95% CI] = 1.27 [1.10–1.46]) upon ICU admission post LT were independently associated with higher odds of 1-year graft failure. Conclusions: In a recent cohort of patients who underwent LT, DRI, despite being high, was not associated with 1-year graft failure, but SOFA score or lactate upon ICU admission post LT were.info:eu-repo/semantics/publishedVersio
Improving Care of Mycotic Aneurysms: Can We Identify the Ideal Endovascular Candidate?
No full textinfo:eu-repo/semantics/publishedVersio
A Novel Genetic Variant in MBD5 Associated with Severe Epilepsy and Intellectual Disability: Potential Implications on Neural Primary Cilia
No full textDisruptions in the MBD5 gene have been linked with an array of clinical features such as global developmental delay, intellectual disability, autistic-like symptoms, and seizures, through unclear mechanisms. MBD5 haploinsufficiency has been associated with the disruption of primary cilium-related processes during early cortical development, and this has been reported in many neurodevelopmental disorders. In this study, we describe the clinical history of a 12-year-old child harboring a novel MBD5 rare variant and presenting psychomotor delay and seizures. To investigate the impact of MBD5 haploinsufficiency on neural primary cilia, we established a novel patient-derived cell line and used CRISPR-Cas9 technology to create an isogenic control. The patient-derived neural progenitor cells revealed a decrease in the length of primary cilia and in the total number of ciliated cells. This study paves the way to understanding the impact of MBD5 haploinsufficiency in brain development through its potential impact on neural primary cilia.info:eu-repo/semantics/publishedVersio
Adrenal Vein Sampling in the Management of Primary Aldosteronism: The Added Value of Intraprocedural Cortisol Assessment
No full textIntroduction: Primary aldosteronism is the most common cause of secondary hypertension. Adrenal vein sampling is the gold standard for subtyping primary aldosteronism. However, this procedure is technically challenging and often has a low success rate. Our center is one of the very few performing this technique in our country with an increasing experience.
Objective: The aim of this study was to evaluate the role of the cortisol intraprocedural assay in improving the performance of adrenal vein sampling.
Design: We enrolled all of the patients with primary aldosteronism that underwent adrenal vein sampling from February 2016 to April 2023. The cortisol intraprocedural assay was introduced in October 2021.
Methods: We enrolled a total of 50 adrenal vein samplings performed on 43 patients with the diagnosis of primary aldosteronism. In this sample, 19 patients and 24 patients underwent adrenal vein sampling before and after intraprocedural cortisol measurement, respectively. The procedure was repeated in seven patients (one before and six after intraprocedural cortisol measurement), given the unsuccess of the first exam. Selectivity of the adrenal vein sampling was assumed if the serum cortisol concentration from the adrenal vein was at least five times higher than that of the inferior vena cava. Lateralization was assumed if the aldosterone to cortisol ratio of one adrenal vein was at least four times the aldosterone to cortisol ratio of the contralateral side.
Results: The mean age of the patients that underwent adrenal vein sampling (N = 43) was 55.2 ± 8.9 years, and 53.5% (n = 23) were female. The mean interval between the diagnosis of hypertension and the diagnosis of primary aldosteronism was 9.8 years (±9.9). At diagnosis, 62.8% of the patients (n = 27) had hypokalemia (mean value of 3 mmol/L (±0.34)), 88.4% (n = 38) had adrenal abnormalities on preprocedural CT scan, and 67.4% (n = 29) described as unilateral nodules. There were no statistically significant differences in the patients' baseline characteristics between the two groups (before and after intraprocedural cortisol measurement). Before intraprocedural cortisol measurement, adrenal vein sampling selectivity was achieved in 35% (n = 7) patients. Selectivity increased to 100% (30/30) after intraprocedural cortisol measurement (p < 0.001). With the exception of one patient who refused it, all patients with lateralized disease underwent unilateral adrenalectomy with normalization of the aldosterone to renin ratio postoperatively.
Conclusions: The lack of effective alternatives in subtyping primary aldosteronism highlights the need to improve the success rate of adrenal vein sampling. In this study, intraprocedural cortisol measurement allowed a selectivity of 100%. Its addition to this procedure protocol should be considered, especially in centers with a low success rate.info:eu-repo/semantics/publishedVersio
Os Desafios da Implementação de um Estudo Clínico com o Novo Regulamento de Dispositivos Médicos da União Europeia
No full textinfo:eu-repo/semantics/publishedVersio
Validation of a Heart Failure Risk Score in a Cohort of Cardiac Resynchronization Therapy Patients Under Remote Monitoring: Results from the TriageHF™ Algorithm
No full textThe heart failure risk status (HFRS) is a validated dynamic tool for risk score prediction, based on the TriageHF™ algorithm (Medtronic, Minneapolis, MN, USA), for the occurrence of a heart failure (HF) event in the 30 days following a remote monitoring (RM) transmission. The aim of this study was to evaluate the accuracy of the HFRS in predicting an unplanned hospital admission due to HF decompensation in a real-world cohort of patients submitted to cardiac resynchronization therapy (CRT). We conducted a single-center review of a cohort of 40 consecutive HF patients, under RM, with CRT devices using the HFRS of the TriageHF™ algorithm. The correlation of the HFRS with hospital admissions was analyzed. During a mean follow-up of 36 months, a stepwise increase in the HFRS was significantly associated with a higher risk of HF admission (odds ratio, 12.7; 95% confidence interval, 3.2-51.5; P < .001), and the HFRS was demonstrated to have good discrimination for HF hospitalization, with an area under the receiver-operating characteristic curve of 0.812. The TriageHF™ algorithm effectively predicted HF-related hospitalization in a cohort of CRT patients during long-term RM follow-up, providing a novel clinical pathway to optimize the clinical management of this complex population.info:eu-repo/semantics/publishedVersio