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External Validation of the SINCALC Score for Outcomes Following Cerebral Venous Thrombosis.
No full textObjectives: Prognostication for cerebral venous thrombosis (CVT) remains difficult. We sought to validate the SI2NCAL2C score in an international cohort.
Materials and methods: The SI2NCAL2C score was originally developed to predict poor outcome (modified Rankin Scale (mRS) 3-6) at 6 months, and mortality at 30 days and 1 year using data from the International CVT Consortium. The SI2NCAL2C score uses 9 variables: the absence of any female-sex-specific risk factors, intracerebral hemorrhage, central nervous system infection, focal neurological deficits, coma, age, lower level of hemoglobin, higher level of glucose, and cancer. The ACTION-CVT study was an international retrospective study that enrolled consecutive patients across 27 centers. The poor outcome score was validated using 90-day mRS due to lack of follow-up at the 6-month time-point in the ACTION-CVT cohort. Model performance was evaluated using the area under the receiver operating characteristic curve (AUC) and calibration plots. Missing data were imputed using the additive regression and predictive mean matching methods. Bootstrapping was performed with 1000 iterations.
Results: Mortality data were available for 950 patients and poor outcome data were available for 587 of 1,025 patients enrolled in ACTION-CVT. Compared to the International CVT Consortium, the ACTION-CVT cohort was older, less often female, and with milder clinical presentation. Mortality was 2.5% by 30 days and 6.0% by one year. At 90-days, 16.7% had a poor outcome. The SI2NCAL2C score had an AUC of 0.74 [95% CI 0.69-0.79] for 90-day poor outcome, 0.72 [0.60-0.82] for mortality by 30 days, and 0.82 [0.76-0.88] for mortality by one year.
Conclusions: The SI2NCAL2C score had acceptable to good performance in an international external validation cohort. The SI2NCAL2C score warrants additional validation studies in diverse populations and clinical implementation studies
Smooth Muscle Tumours of the Uterus: MR Imaging Malignant Predictive Features-a 12-Year Analysis in a Referral Hospital in Portugal.
No full textPurpose: To evaluate the magnetic resonance imaging (MRI) features that may help distinguish leiomyosarcomas from atypical leiomyomas (those presenting hyperintensity on T2-W images equal or superior to 50% compared to the myometrium).
Materials and methods: The authors conducted a retrospective single-centre study that included a total of 57 women diagnosed with smooth muscle tumour of the uterus, who were evaluated with pelvic MRI, between January 2009 and March 2020. All cases had a histologically proven diagnosis (31 Atypical Leiomyomas-ALM; 26 Leiomyosarcomas-LMS). The MRI features evaluated in this study included: age at presentation, dimension, contours, intra-tumoral haemorrhagic areas, T2-WI heterogeneity, T2-WI dark areas, flow voids, cyst areas, necrosis, restriction on diffusion-weighted imaging (DWI), apparent diffusion coefficient (ADC) values, signal intensity and heterogeneity after contrast administration in T1-WI, presence and location of unenhanced areas. The association between the MRI characteristics and the histological subtype was evaluated using Chi-Square and ANOVA tests.
Results: The MRI parameters that showed a statistically significance correlation with malignant histology and thus most strongly associated with LMS were found to be: irregular contours (p < 0.001), intra-tumoral haemorrhagic areas (p = 0.028), T2-WI dark areas (p = 0.016), high signal intensity after contrast administration (p = 0.005), necrosis (p = 0.001), central location for unenhanced areas (p = 0.026), and ADC value lower than 0.88 × 10-3 mm2/s (p = 0.002).
Conclusion: With our work, we demonstrate the presence of seven MRI features that are statistically significant in differentiating between LMS and ALM
Membranous Nephropathy as a Manifestation of IgG4‑Related Disease (IgG4‑RD): A Case Report
No full textIgG4‑related disease (IgG4‑RD) is an immune‑mediated, inflammatory condition that can affect multiple organs, marked by lymphoplasmocytic infiltration and IgG4‑positive plasma cells. Elevated serum IgG4 is common but not essential. Sole renal manifestation is rare, usually appearing as tubulointerstitial nephritis (TIN) or, less commonly, membranous nephropathy (MN).
We present a case of an 87‑year‑old man with a medical history of hypertension, dyslipidemia, ischemic cardiomy‑
opathy, and COPD. The patient presented with nephrotic syndrome (proteinuria/creatininuria ratio (P/C ratio) of 11
g/g) and acute kidney injury (creatinine 4 mg/dL). Urinalysis indicated proteinuria and haematuria without dysmor‑
phic erythrocytes. Investigation revealed normochromic normocytic anaemia, hypoalbuminemia, and no evidence of
monoclonal gammopathy or hypocomplementemia. Serologies for HIV, hepatitis B, and hepatitis C were negative. An‑
tinuclear, anti‑neutrophil, and anti‑phospholipase A2 antibodies (anti‑PLA2r) were also negative. Treatment including
anti‑proteinuric and anticoagulation therapies was initiated. Kidney biopsy displayed membranous nephropathy and
interstitial nephritis; immunofluorescence showed parietal granular deposits of IgG‑κ and IgG‑λ; immunohistochemistry revealed lymphocytes, plasma cells (with more than 10 IgG4‑positive cells per high‑power field), and IgG4 deposits on the glomerular basement membrane. Serum IgG subclass levels detected a slight elevation of IgG4. Other organ involvements hinting at IgG4‑RD were ruled out. Assuming IgG4‑RD with renal involvement therapy with prednisolone (40 mg/day) was started and after one month the patient demonstrated a significant improvement in kidney function, anaemia, hypoalbuminemia, and proteinuria.
Although renal manifestation as the first and/or only manifestation of IgG4‑RD is rare, should be suspected in renal cases showing MN with or without TIN, especially when anti‑PLA2R is negative
Predicting Post-Discharge Complications in Cardiothoracic Surgery: a Clinical Decision Support System to Optimize Remote Patient Monitoring Resources
No full textCardiac surgery patients are highly prone to severe complications post-discharge. Close follow-up through remote patient monitoring can help detect adverse outcomes earlier or prevent them, closing the gap between hospital and home care. However, equipment is limited due to economic and human resource constraints. This issue raises the need for efficient risk estimation to provide clinicians with insights into the potential benefit of remote monitoring for each patient. Standard models, such as the EuroSCORE, predict the mortality risk before the surgery. While these are used and validated in real settings, the models lack information collected during or following the surgery, determinant to predict adverse outcomes occurring further in the future. This paper proposes a Clinical Decision Support System based on Machine Learning to estimate the risk of severe complications within 90 days following cardiothoracic surgery discharge, an innovative objective underexplored in the literature. Health records from a cardiothoracic surgery department regarding 5 045 patients (60.8% male) collected throughout ten years were used to train predictive models. Clinicians' insights contributed to improving data preparation and extending traditional pipeline optimization techniques, addressing medical Artificial Intelligence requirements. Two separate test sets were used to evaluate the generalizability, one derived from a patient-grouped 70/30 split and another including all surgeries from the last available year. The achieved Area Under the Receiver Operating Characteristic curve on these test sets was 69.5% and 65.3%, respectively. Also, additional testing was implemented to simulate a real-world use case considering the weekly distribution of remote patient monitoring resources post-discharge. Compared to the random resource allocation, the selection of patients with respect to the outputs of the proposed model was proven beneficial, as it led to a higher number of high-risk patients receiving remote monitoring equipment.info:eu-repo/semantics/publishedVersio
Uso de Eltrombopag em Crianças com Trombocitopenia Imune Primária Persistente e Crónica num Hospital Pediátrico Português
No full textinfo:eu-repo/semantics/publishedVersio
Perceção da Cardiologia, Preferências Profissionais e Impacto na Escolha de Carreira Entre Cardiologistas Portugueses: Resultados do Questionário da Task Force Mulheres na Cardiologia da Sociedade Portuguesa de Cardiologia
No full textIntroduction and objectives: Cardiology has not been seen as an attractive specialty, and women have avoided it for many years. Some surveys have been performed in other countries, but in Portugal, the situation is largely unknown.
Methods: An online survey on perceptions of cardiology and professional preferences was sent to 1371 members of the Portuguese Society of Cardiology, of whom 18.2% completed the survey.
Results: We included 219 cardiologists or cardiology trainees, of whom 50.2% were female, with decreasing proportions from younger to older age groups, in which males still predominate. Women are less often married and more frequently childless, particularly those working in an invasive subspecialty, where they represent only 16% of all respondents working in these areas. Men's perception is that women do not choose these areas due to family reasons, radiation concerns and difficult working conditions, but from the female perspective, male dominance, lack of female role models and restricted access are the main barriers. Women consider it is difficult for them to obtain a leadership role, but men do not think the same (75.5% vs. 27.5%).
Conclusion: In Portugal, females predominate in younger age groups, suggesting a paradigm change. Women are less frequently married and more frequently childless, particularly women working in invasive subspecialties. Women consider that it is more difficult for them to obtain a leadership role. Moreover, the barriers reported by women are substantially different from men regarding the reasons for not choosing an invasive subspecialty.info:eu-repo/semantics/publishedVersio
Lobar Lung Transplantation: A Single-Center 10-Year Experience
No full textBackground: The shortage of donors for lung transplants is the main limitation of the preceding. Lobar transplantation is an alternative especially useful in patients with short stature and small thoracic cavities. The aim of this study was to perform a descriptive analysis of Portuguese patients who underwent lobar lung transplantation.
Methods: A retrospective study was conducted, and patients submitted to lobar lung transplantation from January 2012 to December 2023 were evaluated. A descriptive analysis was made, including demographic data, lung diseases, waiting list dynamics, pre-transplant evaluations, and post-transplant outcomes.
Results: Sixteen lobar transplants were performed with a predominance of female patients and a median age of 47 years. Most patients had interstitial lung disease or bronchiectasis either due to cystic fibrosis or non-cystic fibrosis. The median predicted total lung capacity (pTLC) ratio was 0.73. The median waiting list time was 6 months with 9 urgent transplants and 1 emergent lobar retransplant. Extracorporeal membrane oxygenation (ECMO) was used in pre-, intra-, and postoperative periods. Most transplanted lobes were the median lobe (ML) + right upper lobe (RUL) and left upper lobe (LUL). The median length of stay was 58 days, with complications such as PDG grade 3, bronchial tree ischemia, and concentrical stenosis of bronchial anastomosis. Six patients died in this period, 1 in the immediate postoperative period and 5 during the post-transplant hospitalization, with a median survival of 20.7 months and a 1-year and 5-year survival rate of 60%.
Conclusion: Our results show a population with an increased waiting list converging in many urgent cases, with an early mortality and high primary graft dysfunction rate. Nevertheless, mid- and long-term survival are promising.info:eu-repo/semantics/publishedVersio
Association Between COVID-19 and the Incidence of Type 1 Diabetes in Portugal - a Registry Study
No full textBackground: Viral respiratory infections may precipitate type 1 diabetes (T1D). A possible association between the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the virus responsible for COVID-19, and the incidence of T1D is being determined. This study was carried out using Portuguese registries, aiming at examining temporal trends between COVID-19 and T1D.
Methods: Hospital data, comparing the incidence before and during the COVID-19 pandemic, from children and young adults diagnosed with new-onset T1D, was acquired beginning in 2017 and until the end of 2022. Data was obtained from nine different Portuguese hospital units. The impact of the COVID-19 pandemic, beginning in March 2020, was assessed comparing the annual numbers of new-onset T1D cases. The annual median levels of glucose, glycated hemoglobin (HbA1c) and fasting C-peptide at T1D diagnosis were compared. The annual number of diabetic ketoacidosis (DKA) episodes among new T1D cases was also assessed at two centers.
Results: In total, data from 574 newly diagnosed T1D patients was analyzed, including 530 (92.3%) children. The mean ages for child and adult patients were 9.1 (SD 4.4) and 32.8 (SD 13.6) years, respectively. 57.8% (331/573) were male, one patient had unknown sex. The overall median (25-75 percentiles) levels of glucose, HbA1c and fasting C-peptide at diagnosis were 454 mg/dL (356-568), 11.8% (10.1-13.4) and 0.50 µg/L (0.30-0.79), respectively. DKA at T1D diagnosis was present in 48.4% (76/157). For eight centers with complete 2018 to 2021 data (all calendar months), no overall significant increase in T1D cases was observed during the COVID-19 pandemic, i.e. 90 cases in 2018, 90 cases in 2019, 112 in 2020 and 100 in 2021 (P for trend = 0.36). Two of the centers, Faro (CHUA) and Dona Estefânia (CHULC) hospitals, did however see an increase in T1D from 2019 to 2020. No significant changes in glucose (P = 0.32), HbA1c (P = 0.68), fasting C-peptide (P = 0.20) or DKA frequency (P = 0.68) at the time of T1D diagnosis were observed over the entire study period.
Conclusion: The T1D incidence did not increase significantly, when comparing the years before and during the COVID-19 pandemic, nor did key metabolic parameters or number of DKA episodes change
Health-Illness Transition Processes in Children with Complex Chronic Conditions and their Parents: a Scoping Review.
No full textBackground: The prevalence of complex chronic conditions (CCC), which cause serious limitations and require specialized care, is increasing. The diagnosis of a CCC is a health-illness transition for children and their parents, representing a long-term change leading to greater vulnerability. Knowing the characteristics of these transitional processes is important for promoting safe transitions in this population. This scoping review aimed to map the available evidence on health-illness transition processes in children with complex chronic conditions and their parents in the context of healthcare.
Methods: Six databases were searched for studies focusing on children aged 0-21 years with CCC and their parents experiencing health-illness transition processes, particularly concerning adaptation to illness and continuity of care, in the context of healthcare. Studies within this scope carried out between 2013 and 2023 and written in Portuguese or English were identified. The articles were selected using the PRISMA methodology. The data were extracted to an instrument and then presented with a synthesizing approach supporting the interpretation of the results.
Results: Ninety-eight methodologically broad but predominantly qualitative articles were included in this review. Children with CCC have specific needs associated with complex and dynamic health-illness transitions with a multiple influence in their daily lives. Several facilitating factors (p.e. positive communication and a supportive therapeutic relationship with parents and professionals, as well as involvement in a collaborative approach to care), inhibiting factors (p.e. the complexity of the disease and therapeutic regime, as well as the inefficient organization and coordination of teams) and both positive (p.e. well-being and better quality of life) and negative response patterns (p.e. negative feelings about the chronic illness) were identified. Some interventions to support the transitional process also emerged from the literature. Pediatric palliative care is seen as a good practice and an integrative approach for these children and families.
Conclusion: Health professionals play a fundamental role in supporting the transitional process and promoting positive response patterns. More significant investment is needed at the clinical and academic levels regarding production and dissemination of knowledge in this area to ensure the awareness of children with CCC and that their needs are fully enhanced