Endometrial cancer: predictors and oncological safety of tumor tissue manipulation.

PURPOSE: The main goal of this study is to assess the impact of tumor manipulation on the presence of lympho-vascular space invasion and its influence on oncological results. METHODS: We performed a retrospective multi-centric study amongst patients who had received primary surgical treatment for apparently early-stage endometrial cancer. A multivariate statistical analysis model was designed to assess the impact that tumor manipulation (with the use of uterine manipulator or preoperative hysteroscopy) has on lympho-vascular development (LVSI) in the final surgical specimen. RESULTS: A total of 2852 women from 15 centers were included and divided into two groups based on the lympho-vascular status in the final surgical specimen: 2265 (79.4%) had no LVSI and 587 (20.6%) presented LVSI. The use of uterine manipulator was associated with higher chances of lympho-vascular involvement regardless of the type used: Balloon manipulator (HR: 95% CI 4.64 (2.99-7.33); p < 0.001) and No-Balloon manipulator ([HR]: 95% CI 2.54 (1.66-3.96); p < 0.001). There is no evidence of an association between the use of preoperative hysteroscopy and higher chances of lympho-vascular involvement (HR: 95% CI 0.90 (0.68-1.19); p = 0.479). CONCLUSION: Whilst performing common gynecological procedures, iatrogenic distention and manipulation of the uterine cavity are produced. Our study suggests that the use of uterine manipulator increases the rate of LVSI and, therefore, leads to poorer oncological results. Conversely, preoperative hysteroscopy does not show higher rates of LVSI involvement in the final surgical specimen and can be safely used.

Accuracy and Survival Outcomes after National Implementation of Sentinel Lymph Node Biopsy in Early Stage Endometrial Cancer.

BACKGROUND: Sentinel lymph node (SLN) biopsy has recently been accepted to evaluate nodal status in endometrial cancer at early stage, which is key to tailoring adjuvant treatments. Our aim was to evaluate the national implementation of SLN biopsy in terms of accuracy to detect nodal disease in a clinical setting and oncologic outcomes according to the volume of nodal disease. PATIENTS AND METHODS: A total of 29 Spanish centers participated in this retrospective, multicenter registry including patients with endometrial adenocarcinoma at preoperative early stage who had undergone SLN biopsy between 2015 and 2021. Each center collected data regarding demographic, clinical, histologic, therapeutic, and survival characteristics. RESULTS: A total of 892 patients were enrolled. After the surgery, 12.9% were suprastaged to FIGO 2009 stages III-IV and 108 patients (12.1%) had nodal involvement: 54.6% macrometastasis, 22.2% micrometastases, and 23.1% isolated tumor cells (ITC). Sensitivity of SLN biopsy was 93.7% and false negative rate was 6.2%. After a median follow up of 1.81 years, overall surivial and disease-free survival were significantly lower in patients who had macrometastases when compared with patients with negative nodes, micrometastases or ITC. CONCLUSIONS: In our nationwide cohort we obtained high sensitivity of SLN biopsy to detect nodal disease. The oncologic outcomes of patients with negative nodes and low-volume disease were similar after tailoring adjuvant treatments. In total, 22% of patients with macrometastasis and 50% of patients with micrometastasis were at low risk of nodal metastasis according to their preoperative risk factors, revealing the importance of SLN biopsy in the surgical management of patients with early stage EC.

Lymphovascular Space Invasion in Early-Stage Endometrial Cancer (LySEC): Patterns of Recurrence and Predictors. A Multicentre Retrospective Cohort Study of the Spain Gynecologic Oncology Group.

The main aim is to compare oncological outcomes and patterns of recurrence of patients with early-stage endometrioid endometrial cancer according to lymphovascular space invasion (LVSI) status. The secondary objective is to determine preoperative predictors of LVSI. We performed a multicenter retrospective cohort study. A total of 3546 women diagnosed with postoperative early-stage (FIGO I-II, 2009) endometrioid endometrial cancer were included. Co-primary endpoints were disease-free survival (DFS), overall survival (OS), and pattern of recurrence. Cox proportional hazard models were used for time-to-event analysis. Univariate and multivariate logistical regression models were employed. Positive LVSI was identified in 528 patients (14.6%) and was an independent prognostic factor for DFS (HR 1.8), OS (HR 2.1) and distant recurrences (HR 2.37). Distant recurrences were more frequent in patients with positive LVSI (78.2% vs. 61.3%, p < 0.01). Deep myometrial invasion (OR 3.04), high-grade tumors (OR 2.54), cervical stroma invasion (OR 2.01), and tumor diameter ≥ 2 cm (OR 2.03) were independent predictors of LVSI. In conclusion, in these patients, LVSI is an independent risk factor for shorter DFS and OS, and distant recurrence, but not for local recurrence. Deep myometrial invasion, cervical stroma invasion, high-grade tumors, and a tumor diameter ≥ 2 cm are independent predictors of LVSI.

Extracorporeal Membrane Oxygenation in an Adolescent with Multisystem Inflammatory Syndrome in Children.

Multisystem inflammatory syndrome in children is a rare and potentially life-threatening disease that is associated with SARS-CoV-2 infection, characterized by hyperinflammation and multiorgan involvement. Cardiovascular involvement is common, including myocardial dysfunction often leading to cardiogenic shock. We present the case of a 17-year-old boy with fever, odynophagia, maculopapular rash and abdominal pain who developed a cardiogenic shock. Due to progressive deterioration of cardiac function despite optimized vasoactive support, veno-arterial extracorporeal membrane oxygenation support was initiated 12 hours after admission, with successful decannulation after seven days and discharge after 23 days, with normal cardiac function. The patient received corticosteroids and intravenous immunoglobulin. Early recognition and intensive care support are crucial for ensuring a successful outcome in severe cases of multisystem inflammatory syndrome. In cases of severe cardiogenic shock, extracorporeal membrane oxygenation support can be critical for survival and rapid recovery.

Precocious puberty and anal stenosis in an African patient with Rothmund-Thomson syndrome.

Rothmund-Thomson syndrome (RTS) is a rare autosomal recessive disorder characterized by a rash that progresses to poikiloderma. Other common features include sparse hair, eyelashes and eyebrows, short stature, variable skeletal abnormalities, dental defects, cataracts, hypogonadism, and an increased risk for cancer, especially osteosarcoma and skin cancer. RTS is caused by biallelic pathogenic variants in ANAPC1 (Type 1 RTS) or RECQL4 (Type 2 RTS). We present an African girl with Type 2 RTS caused by a nonsense variant and an intronic variant in RECQL4. The patient presented precocious puberty, which has not been previously reported in RTS and that was treated with a GnRH analog, and anal stenosis, which has only been reported once. This case highlights the need to consider deep intronic variants in patients with RTS when pathogenic variants in the coding regions and exon/intron boundaries are not identified and expands the phenotypic spectrum of this disorder.

Preoperative sampling in endometrial cancer: evaluation of the histopathological agreement with definitive surgical specimen

Purpose The main goal of this study is to assess the diagnostic agreement between preoperative biopsy and definitive histology of the surgical specimen to determine which sampling method is most suitable for diagnosis of early-stage endometrial cancer. Methods We performed a retrospective multicentric study to assess the correlation between three endometrial sampling methods (hysteroscopy, pipelle and D&C) in patients who had undergone preoperative endometrial biopsy and received primary surgical treatment for endometrial cancer. The primary objective was the agreement rate between hysteroscopy (HSC), endometrial biopsy (pipelle) and dilatation and curettage (D&C). Results A total of 1833 women who underwent preoperative sampling at 15 centers were included: 1042 biopsies were performed by HSC, 703 by pipelle and 88 by D&C. All three methods presented a moderate diagnostic concordance (κ = 0.40–0.61) with the definitive specimen’s histology: HSC (κ = 0.47), pipelle sampling (κ = 0.48) and D&C (κ = 0.48). Likewise, a subgroup analysis was performed by histological subtype comparing HSC and endometrial biopsy, showing that neither is superior as a diagnostic method. Conclusions According to this study, the use of pipelle sampling could become an adequate diagnostic method in endometrial cancer due to its similar agreement to HSC, ease of use and affordability (AU)

MDA-LDL vaccination induces athero-protective germinal-center-derived antibody responses.

Atherosclerosis is a chronic inflammatory disease of the arteries that can lead to thrombosis, infarction, and stroke and is the leading cause of mortality worldwide. Immunization of pro-atherogenic mice with malondialdehyde-modified low-density lipoprotein (MDA-LDL) neo-antigen is athero-protective. However, the immune response to MDA-LDL and the mechanisms responsible for this athero-protection are not completely understood. Here, we find that immunization of mice with MDA-LDL elicits memory B cells, plasma cells, and switched anti-MDA-LDL antibodies as well as clonal expansion and affinity maturation, indicating that MDA-LDL triggers a bona fide germinal center antibody response. Further, Prdm1fl/flAicda-Cre+/kiLdlr-/- pro-atherogenic chimeras, which lack germinal center-derived plasma cells, show accelerated atherosclerosis. Finally, we show that MDA-LDL immunization is not athero-protective in mice lacking germinal-center-derived plasma cells. Our findings give further support to the development of MDA-LDL-based vaccines for the prevention or treatment of atherosclerosis.

Preoperative sampling in endometrial cancer: evaluation of the histopathological agreement with definitive surgical specimen.

PURPOSE: The main goal of this study is to assess the diagnostic agreement between preoperative biopsy and definitive histology of the surgical specimen to determine which sampling method is most suitable for diagnosis of early-stage endometrial cancer. METHODS: We performed a retrospective multicentric study to assess the correlation between three endometrial sampling methods (hysteroscopy, pipelle and D&C) in patients who had undergone preoperative endometrial biopsy and received primary surgical treatment for endometrial cancer. The primary objective was the agreement rate between hysteroscopy (HSC), endometrial biopsy (pipelle) and dilatation and curettage (D&C). RESULTS: A total of 1833 women who underwent preoperative sampling at 15 centers were included: 1042 biopsies were performed by HSC, 703 by pipelle and 88 by D&C. All three methods presented a moderate diagnostic concordance (κ = 0.40-0.61) with the definitive specimen's histology: HSC (κ = 0.47), pipelle sampling (κ = 0.48) and D&C (κ = 0.48). Likewise, a subgroup analysis was performed by histological subtype comparing HSC and endometrial biopsy, showing that neither is superior as a diagnostic method. CONCLUSIONS: According to this study, the use of pipelle sampling could become an adequate diagnostic method in endometrial cancer due to its similar agreement to HSC, ease of use and affordability.

Advanced Echocardiographic Techniques in Cardio-Oncology: the Role for Early Detection of Cardiotoxicity.

PURPOSE OF REVIEW: Implementation of advanced echocardiographic techniques in cardio-oncology is a growing need as they are the cornerstone of early detection of cancer therapy-related cardiovascular toxicity (CTR-CVT). RECENT FINDINGS: Three-dimensional echocardiography and myocardial deformation techniques have shown more accuracy and reproducibility than classic 2D measurements in detecting cardiovascular adverse effects in patients undergoing anticancer therapies. Application of advanced echo techniques to daily monitoring of patients with cancer helps to identify those at risk of developing CTR-CVT during and after cancer treatment. Furthermore, advanced echo parameters improve early initiation of cardioprotective treatments in order to minimize cardiovascular events and cancer treatment interruption.

Oncological safety of hysteroscopy in endometrial cancer.

OBJECTIVE: It has been suggested that the manipulation of neoplastic tissue during hysteroscopy may lead to dissemination of tumor cells into the peritoneal cavity and worsen prognosis and overall survival. The goal of this study was to assess the oncological safety comparing hysteroscopy to Pipelle blind biopsy in the presurgical diagnosis of patients with endometrial cancer. METHODS: We performed a retrospective multicentric study among patients who had received primary surgical treatment for endometrial cancer. A multivariate statistical analysis model was used to compare relapse and survival rates in patients who had been evaluated preoperatively either by hysteroscopy or Pipelle biopsy. The relapse rate, disease-free survival, and overall survival were assessed as the main outcomes. The histological type, tumor size, myometrial invasion, International Federation of Gynecology and Obstetrics (FIGO) stage, surgical approach, use of a uterine manipulator, and adjuvant treatment were also included in the analysis. RESULTS: A total of 1731 women from 15 centers were included: 1044 in the hysteroscopy group and 687 in the Pipelle sampling group. 225 patients relapsed during the 10 year follow-up period: 139 (13.3%) in the hysteroscopy group and 86 (12.4%) in the Pipelle sampling group. There is no evidence of an association between the use of hysteroscopy as a diagnostic method and relapse rate (HR 1.24, 95% CI 0.92 to 1.66; p=0.16), lower disease-free survival (HR 1.23, 95% CI 0.92 to 1.66; p=0.15), or overall survival (HR 0.95, 95% CI 0.70 to 1.29; p=0.76). CONCLUSION: Hysteroscopy is a safe diagnostic method for patients with endometrial cancer with no impact on oncological outcomes when compared with sampling by Pipelle.

Battery Ingestion in Children, an Ongoing Challenge: Recent Experience of a Tertiary Center.

Introduction: Morbidity related to childhood battery ingestions (BI) has increased recently due to the expanding use of larger lithium cells. A prompt endoscopic removal is vital to prevent severe complications in cases of esophageal batteries (EB). Materials and Methods: A retrospective, descriptive study of admissions for BI requiring endoscopic removal in a tertiary hospital's pediatric emergency department (Jan. 2011/Dec. 2020). Results: We had 35 cases, with an increasing incidence in the last 6 years; median age, 26 m (8 m-10 years), witnessed ingestion in 86%. On the X-ray: 14 (40%) had an EB, 21 (60%), a gastric battery (GB). Symptoms were present in 57% (100% EB/24% GB), and vomiting was the most frequent (50%). Endoscopy revealed: EB, 13 (37%); GB, 17 (49%); duodenal battery, 1 (3%); no battery, 4 (11%). Median time to removal: EB, 7 h (2 h-21days); GB, 12 h (2 h-3 days). All the patients with EB on the X-ray (14) had severe mucosal injury (Zargar classification): Grade IIIa, 7 (50%); IIIb, 5 (36%); IV, 2 (14%). CT-scan showed perforation in 2 patients (total, 4; 29% of EB). In patients with GB (21), 14 (67%) had mucosal damage; 13 (93%), mild (< Grade III, two esophageal erosions); 1 (7%) IIIa (esophageal ulceration). A statistically significant association between exposure time, younger age or battery size and severity of endoscopic lesions was found in EB location. There were no mortality cases. Acute complications occurred in 57% of EB: infection, 50%; perforation, 29%; pneumomediastinum/stridor, 14%; pneumothorax/subglottic stenosis/hemodynamic instability, 7 vs. 0% GB. Stenosis subsequently developed in 6 (43%) of EB: mild, 4 cases (29%); severe, 2 cases (14%, one resolved after endoscopic dilation; one needed a gastrostomy and esophagocoloplasty). Conclusion: We verified recent increase in admissions due to battery ingestions and associated complications, despite the availability of an emergency pediatric endoscopy team. The patients with EB had more severe mucosal injury and poorer short/long-term outcomes. Children with GB had milder lesions, although the presence of a GB did not exclude esophageal injury. The availability of actual data from national referral centers will support advocacy efforts among stakeholders, including industry representatives and policy makers, in preventing worldwide button battery injury.

Subcutaneous fat necrosis of the newborn - An atypical case with typical complications.

Subcutaneous fat necrosis of the newborn should be considered in newborns with suggestive skin lesions, even in the absence of perinatal distress. SCFN may cause long-standing complications, like hypertriglyceridemia or hypercalcemia. Hypercalcemia can be refractory to therapy and lead to poor weight gain and nephrocalcinosis, which should be closely monitored.

Validation of cardiovascular outcomes and risk factors in the Clinical Practice Research Datalink in the United Kingdom.

PURPOSE: Strategies to identify and validate acute myocardial infarction (AMI) and stroke in primary-care electronic records may impact effect measures, but to an unknown extent. Additionally, the validity of cardiovascular risk factors that could act as confounders in studies on those endpoints has not been thoroughly assessed in the United Kingdom Clinical Practice Research Datalink's (CPRD's) GOLD database. We explored the validity of algorithms to identify cardiovascular outcomes and risk factors and evaluated different outcome-identification strategies using these algorithms for estimation of adjusted incidence rate ratios (IRRs). METHODS: First, we identified AMI, stroke, smoking, obesity, and menopausal status in a cohort treated for overactive bladder by applying computerized algorithms to primary care medical records (2004-2012). We validated these cardiovascular outcomes and risk factors with physician questionnaires (gold standard for this analysis). Second, we estimated IRRs for AMI and stroke using algorithm-identified and questionnaire-confirmed cases, comparing these with IRRs from cases identified through linkage with hospitalization/mortality data (best estimate). RESULTS: For AMI, the algorithm's positive predictive value (PPV) was >90%. Initial algorithms for stroke performed less well because of inclusion of codes for prevalent stroke; algorithm refinement increased PPV to 80% but decreased sensitivity by 20%. Algorithms for smoking and obesity were considered valid. IRRs based on questionnaire-confirmed cases only were closer to IRRs estimated from hospitalization/mortality data than IRRs from algorithm-identified cases. CONCLUSIONS: AMI, stroke, smoking, obesity, and postmenopausal status can be accurately identified in CPRD. Physician questionnaire-validated AMI and stroke cases yield IRRs closest to the best estimate.

Impact of uterine manipulator on oncological outcome in endometrial cancer surgery.

BACKGROUND: There are limited data available to indicate whether oncological outcomes might be influenced by the uterine manipulator, which is used at the time of hysterectomy for minimally invasive surgery in patients with endometrial cancer. The current evidence derives from retrospective studies with limited sample sizes. Without substantial evidence to support its use, surgeons are required to make decisions about its use based only on their personal choice and surgical experience. OBJECTIVE: To evaluate the use of the uterine manipulator on oncological outcomes after minimally invasive surgery, for apparent early-stage endometrial cancer. STUDY DESIGN: We performed a retrospective multicentric study to assess the oncological safety of uterine manipulator use in patients with apparent early-stage endometrial cancer, treated with minimally invasive surgery. The type of manipulator, surgical staging, histology, lymphovascular space invasion, International Federation of Gynecology and Obstetrics stage, adjuvant treatment, recurrence, and pattern of recurrence were evaluated. The primary objective was to determine the relapse rate. The secondary objective was to determine recurrence-free survival, overall survival, and the pattern of recurrence. RESULTS: A total of 2661 women from 15 centers were included; 1756 patients underwent hysterectomy with a uterine manipulator and 905 without it. Both groups were balanced with respect to histology, tumor grade, myometrial invasion, International Federation of Gynecology and Obstetrics stage, and adjuvant therapy. The rate of recurrence was 11.69% in the uterine manipulator group and 7.4% in the no-manipulator group (P<.001). The use of the uterine manipulator was associated with a higher risk of recurrence (hazard ratio, 2.31; 95% confidence interval, 1.27-4.20; P=.006). The use of uterine manipulator in uterus-confined endometrial cancer (International Federation of Gynecology and Obstetrics [FIGO] I-II) was associated with lower disease-free survival (hazard ratio, 1.74; 95% confidence interval, 0.57-0.97; P=.027) and higher risk of death (hazard ratio, 1.74; 95% confidence interval, 1.07-2.83; P=.026). No differences were found regarding the pattern of recurrence between both groups (chi-square statistic, 1.74; P=.63). CONCLUSION: In this study, the use of a uterine manipulator was associated with a worse oncological outcome in patients with uterus-confined endometrial cancer (International Federation of Gynecology and Obstetrics I-II) who underwent minimally invasive surgery. Prospective trials are essential to confirm these results.

ALDH4A1 is an atherosclerosis auto-antigen targeted by protective antibodies.

Cardiovascular disease (CVD) is the leading cause of mortality in the world, with most CVD-related deaths resulting from myocardial infarction or stroke. The main underlying cause of thrombosis and cardiovascular events is atherosclerosis, an inflammatory disease that can remain asymptomatic for long periods. There is an urgent need for therapeutic and diagnostic options in this area. Atherosclerotic plaques contain autoantibodies1,2, and there is a connection between atherosclerosis and autoimmunity3. However, the immunogenic trigger and the effects of the autoantibody response during atherosclerosis are not well understood3-5. Here we performed high-throughput single-cell analysis of the atherosclerosis-associated antibody repertoire. Antibody gene sequencing of more than 1,700 B cells from atherogenic Ldlr-/- and control mice identified 56 antibodies expressed by in-vivo-expanded clones of B lymphocytes in the context of atherosclerosis. One-third of the expanded antibodies were reactive against atherosclerotic plaques, indicating that various antigens in the lesion can trigger antibody responses. Deep proteomics analysis identified ALDH4A1, a mitochondrial dehydrogenase involved in proline metabolism, as a target antigen of one of these autoantibodies, A12. ALDH4A1 distribution is altered during atherosclerosis, and circulating ALDH4A1 is increased in mice and humans with atherosclerosis, supporting the potential use of ALDH4A1 as a disease biomarker. Infusion of A12 antibodies into Ldlr-/- mice delayed plaque formation and reduced circulating free cholesterol and LDL, suggesting that anti-ALDH4A1 antibodies can protect against atherosclerosis progression and might have therapeutic potential in CVD.

[Case Series of 103 Children with SARS-CoV-2 Infection in Portugal]. / Série de Casos de 103 Crianças com Infeção por SARS-CoV-2 em Portugal.

INTRODUCTION: The North Lisbon University Hospital Center was activated for referral of SARS-CoV-2 infected patients on the 11th March 2020. The aim of this study is to describe the experience at the Department of Pediatrics in the approach and the clinical outcomes of infected children. MATERIAL AND METHODS: A descriptive observational study was performed. Children and adolescents (0 to 18 years) with SARS-CoV-2 infection, diagnosed in the emergency room or admitted to the Department of Pediatrics between March 11th and June 18th, were included. Hospital records and Trace COVID-19 platform were reviewed and patient caregivers were interviewed to assess follow up. RESULTS: Among 103 diagnosed children, 83% had a known previous contact with an infected patient, 43% presented fever and 42% presented respiratory symptoms. Ten percent had risk factors and 21% were aged under one year old. Ten percent were hospitalised, one needing intensive care, with paediatric inflammatory multisystem syndrome. Blood tests were performed in 9% and chest radiograph in 7%. No children required ventilation, antiviral therapy or underwent thoracic computed tomography scan. Eight percent of children returned to the emergency room and one child was hospitalised. The clinical outcome is known in 101 patients and is favourable in all. DISCUSSION: Most children had an epidemiological link and little clinical repercussion, even during the first year of life. The expected mild severity in children justified the use of established clinical criteria and recommendations for similar conditions, regarding tests and hospitalizations. No antiviral treatments were given due to lack of evidence of its benefits. CONCLUSION: This strategy contributed to a low consumption of hospital resources and proved safe in this series.

Introdução: O Centro Hospitalar Universitário Lisboa Norte foi ativado para referência de doentes com infeção SARS-CoV-2 em 11 de março de 2020. O objetivo deste estudo é descrever a experiência do Departamento de Pediatria na abordagem e evolução clínica de crianças infetadas.Material e Métodos: Realizámos um estudo observacional descritivo. Incluímos as crianças e adolescentes (0 aos 18 anos) com infeção por SARS-CoV-2 diagnosticados na urgência e internamento do nosso departamento entre 11 de março e 18 de junho de 2020. Consultámos registos internos e a plataforma Trace COVID-19 e contactámos os cuidadores para avaliação de seguimento.Resultados: De 103 crianças diagnosticadas, 83% tiveram contacto prévio identificado com doente infetado, 43% doentes apresentaram febre e 42% sintomas respiratórios. Em 10% havia fatores de risco; 21% tinham idade inferior a um ano. Foram internadas 10% das crianças, uma em cuidados intensivos com síndrome inflamatória multissistémica pediátrica. Foi efetuada avaliação laboratorial em 9%, radiografia torácica em 7%. Nenhum recebeu suporte ventilatório, terapêutica antiviral ou realizou tomografia computorizada torácica. Foram reobservadas em serviço de urgência 8% das crianças, sendo internada uma. A evolução foi conhecida em 101 casos sendo favorável em todos.Discussão: A maioria dos doentes tinha link epidemiológico familiar e pouca repercussão clínica, mesmo no primeiro ano de vida. A menor gravidade esperada na criança motivou a adoção de critérios habituais noutros quadros clínicos semelhantes para a realização de exames complementares de diagnóstico e internamento hospitalar. Não foi administrada terapêutica antiviral em nenhum doente por se considerar haver pouca evidência de benefício.Conclusão: Esta estratégia traduziu-se num baixo consumo de recursos hospitalares e revelou-se segura nesta série.

Evolución a mediano plazo de pacientes con diagnóstico de cardiopatía amiloidótica por transtiretina / Mid-term Outcome of Patients with Diagnosis of Transthyretin Cardiac Amyloidosis

RESUMEN Introducción: El compromiso cardíaco es la principal causa de morbimortalidad en la amiloidosis, independientemente de la patogenia productora del amiloide subyacente. La amiloidosis por transtiretina (TTR) es una de las variantes más frecuentes, por lo cual el objetivo de este trabajo fue evaluar las características de una cohorte de pacientes con diagnóstico de cardiopatía amiloidótica por TTR (CA-TTR) Material y métodos: Se recabaron datos de los estudios basales, de la metodología diagnóstica y de la evolución de 49 pacientes en seguimiento en la Clínica de Miocardiopatías de nuestra institución. Resultados: La mediana del tiempo de seguimiento fue de 1258 días (410-2004) y la edad promedio de 79 ± 9 años. Al inicio del seguimiento, el 57% de los pacientes estaban en clase funcional I, el 26%, en II y el 16%, en III-IV. El diagnóstico se basó en centellograma con difosfonatos en el 92%; en el 24% requirió biopsia. La mortalidad global fue del 19%, con 15% de muerte cardiovascular. La tasa de internación por insuficiencia cardíaca fue 29%; el 63% de los pacientes empeoró su clase funcional. Conclusiones: El seguimiento de nuestros pacientes con CA-TTR expresa los cambios que ha sufrido el proceso diagnóstico, con una reducción de estudios invasivos y tiempo para la caracterización. El diagnóstico de pacientes en etapas "tempranas" de la enfermedad parece impactar en los resultados a mediano plazo.

ABSTRACT Background: Cardiac involvement is the main cause of morbidity and mortality in amyloidosis, regardless of the underlying pathogenesis of amyloid production, and transthyretin (TTR) amyloidosis is one of the most frequent variants. Objective: The aim of this study was thus to assess the characteristics of a cohort of patients with diagnosis of TTR cardiac amyloidosis (ATTR-CA). Methods: Baseline data and diagnostic and follow-up methodology were collected from 49 patients treated at the cardiomy-opathy clinic of our institution. Results: Median follow-up was 1,258 days (410-2004). Mean age was 79±9 years, and 57% of patients were in functional class (FC) I, 26% in FC II and 16% in FC III-IV at follow-up onset. Diagnosis was made with diphosphonate scintigraphy in 92% of patients and 24% required a biopsy. Overall mortality was 19%, with 15% of cardiovascular death. The rate of hospitalization for heart failure was 29% and 63% of patients worsened their FC. Conclusions: Follow-up of patients with ATTR-CA expresses the changes undergone by the diagnostic process, with a reduction of invasive studies and time to characterization. The diagnosis of patients at "early stages of the disease" seems to have an impact on mid-term outcomes.

[Correction to the article "Case Series of 103 Children with SARS-CoV-2 Infection in Portugal", published on Acta Med Port 2020 Dec;33(12):795-802]. / Errata ao artigo "Série de Casos de 103 Crianças com Infeção por SARS-CoV-2 em Portugal", publicado em Acta Med Port 2020 Dec;33(12):795-802.

On page 801, fifth, where it reads: "No início da pandemia, teorizou-se que a vacina BCG pudesse ter um efeito protetor relativamente à COVID-19,27,28 mas não se encontrou até à data evidência para tal, não estando atualmente recomendada a vacinação BCG na prevenção da COVID-19.28,29 No nosso estudo, a maioria dos doentes (76%) tinha sido vacinada. Analisámos separadamente o subgrupo de crianças nascidas após janeiro de 2016, altura em que passaram a ser vacinadas apenas as crianças pertencentes a grupos de risco.30 A taxa de vacinação neste grupo foi de 51%, sendo superior à taxa de 30,1% estimada para crianças nascidas em Portugal com um ano de idade referido a 2019.31" It should read: "No início da pandemia, teorizou-se que a vacina BCG pudesse ter um efeito protetor relativamente à COVID-19,27,28 mas não se encontrou até à data evidência para tal, não estando atualmente recomendada a vacinação BCG na prevenção da COVID-19.28 No nosso estudo, a maioria dos doentes (76%) tinha sido vacinada. Analisámos separadamente o subgrupo de crianças nascidas após janeiro de 2016, altura em que passaram a ser vacinadas apenas as crianças pertencentes a grupos de risco.29 A taxa de vacinação neste grupo foi de 51%, sendo superior à taxa de 30,1% estimada para crianças nascidas em Portugal com um ano de idade referido a 2019.30" Paper published with errors: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/14537.

Na página 801, quinto parágrafo, onde se lê: "No início da pandemia, teorizou-se que a vacina BCG pudesse ter um efeito protetor relativamente à COVID-19,27,28 mas não se encontrou até à data evidência para tal, não estando atualmente recomendada a vacinação BCG na prevenção da COVID-19.28,29 No nosso estudo, a maioria dos doentes (76%) tinha sido vacinada. Analisámos separadamente o subgrupo de crianças nascidas após janeiro de 2016, altura em que passaram a ser vacinadas apenas as crianças pertencentes a grupos de risco.30 A taxa de vacinação neste grupo foi de 51%, sendo superior à taxa de 30,1% estimada para crianças nascidas em Portugal com um ano de idade referido a 2019.31" Deverá ler-se: "No início da pandemia, teorizou-se que a vacina BCG pudesse ter um efeito protetor relativamente à COVID-19,27,28 mas não se encontrou até à data evidência para tal, não estando atualmente recomendada a vacinação BCG na prevenção da COVID-19.28 No nosso estudo, a maioria dos doentes (76%) tinha sido vacinada. Analisámos separadamente o subgrupo de crianças nascidas após janeiro de 2016, altura em que passaram a ser vacinadas apenas as crianças pertencentes a grupos de risco.29 A taxa de vacinação neste grupo foi de 51%, sendo superior à taxa de 30,1% estimada para crianças nascidas em Portugal com um ano de idade referido a 2019.30"Artigo publicado com erros: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/14537.