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Background Rheumatic heart disease (RHD) is a chronic cardiovascular condition stemming from an infectious origin, posing a substantial health burden, particularly in economically disadvantaged regions. It starts with acute rheumatic fever (ARF), a complication following group A Streptococcus infection, leading to heart valve damage and, over time, structural heart abnormalities. RHD contributes to premature deaths, especially in low-middle-income countries. Although the incidence and prevalence have generally reduced globally due to antibiotics and improved healthcare, it remains a significant public health concern in Brazil, echoing its prevalence in many developing nations around the world. RHD stands as a poignant testament to the intersection of socio-economic disparities and healthcare challenges within Brazil's diverse population. In Brazil, despite advancements in healthcare, RHD continues to impact communities, highlighting the urgent need for enhanced prevention strategies, access to quality healthcare services, and heightened awareness to combat this preventable, yet persistent, cardiac condition. Understanding the epidemiological landscape and socio-cultural factors influencing RHD in Brazil is crucial for developing targeted interventions aimed at mitigating its burden on individuals, families, and the healthcare system at large. Thus, our study focuses on analyzing age-related mortality rates linked to ARF and chronic RHD (ARHD) in Brazil from 2000 to 2021, particularly examining gender disparities. Materials and methods This retrospective cohort study employed a descriptive time-series approach, utilizing comprehensive nationwide data from Brazil spanning from 2000 to 2021 to assess trends in diverse age groups, among both sexes, enabling a detailed analysis of temporal patterns. Mortality data, extracted and categorized meticulously, were subjected to Joinpoint statistical analyses enabling comparative assessments, with average annual percent change (AAPC) and annual percent change (APC) serving as key metrics to quantify and interpret trends over the analyzed period. Results The acute RHD (ARHD)-related mortality declined over the analyzed years supported by AAPC, with higher mortality reduction in females. The age-adjusted mortality rate for "males and females" decreased from 78 to 67 deaths/100,000 from 2000 to 2021. Female mortality dropped from 85 to 69/100,000, and male mortality decreased from 73 to 63/100,000 over the same period. For ARHD, male age groups (20-29, 60-69, 70-79, 80+) showed declining mortality, while the 30-59 age group exhibited an upward. Females AAMR for chronic RHD (CRHD) decreased across all age groups, with significant reductions in the 80 years and above age group from 2000-2002 (APC: -11.94*) and steadily from 2002 onwards (APC: -1.33). Conclusions Our study revealed an overall decline in mortality rates for both acute and CRHD across both sexes. Females consistently exhibited higher mortality rates and a more pronounced reduction compared to males in both acute and CRHD. In ARHD, males experience the highest mortality in the 50-59 age group, while females have a peak in the 40-49 age group. The 60-69 age group had the highest mortality in CRHD for both sexes. Conversely, the 20-29 age group displayed the lowest mortality in CRHD, and the 80-89 age group had the lowest mortality in ARHD.

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OBJECTIVE: To understand the burden of acute rheumatic fever (ARF) among children living in low-income countries who present to the hospital with febrile illness and to determine the role of handheld echocardiography (HHE) in uncovering subclinical carditis as a major manifestation of ARF. STUDY DESIGN: This was a cross-sectional study carried at the Pediatric Hospital in Al Obeid, North Kordofan, Sudan, from September 2022 to January 2023 and including febrile children 3 through 18 years of age with or without clinical features of ARF and without another cause for their fever (not excluding malaria). History, examination, blood investigations, and HHE were done. ARF was diagnosed according to the Jones criteria. Clinical ARF was diagnosed if there was a major clinical Jones criterion and silent ARF if the only major Jones criteria was subclinical carditis. RESULTS: The study cohort included 400 children with a mean age of 9 years. Clinical ARF was diagnosed in 95 patients (95/400, 24%), most of whom presented with a joint major manifestation (88/95, 93%). Among the 281 children who did not present with a clinical manifestation of ARF, HHE revealed rheumatic heart disease (RHD) in 44 patients (44/281, 16%); 31 of them fulfilled criteria for silent ARF (31/281, 11%). HHE increased the detection of ARF by 24%. HHE revealed mild RHD in 41 of 66 (62%) and moderate or severe RHD in 25 of 66 (38%) patients. Both sensitivity and specificity of HHE compared with standard echocardiography were 88%. CONCLUSIONS: There is a significant burden of ARF among febrile children in Sudan. HHE increased the sensitivity of diagnosis, with 11% of children having subclinical carditis as their only major manifestation (ie, silent ARF). RHD-prevention policies need to prioritize decentralization of echocardiography to improve ARF detection.

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O diagnóstico etiológico de quadros coréicos é amplo e algumas características da apresentação auxiliam no raciocínio diagnóstico, como o tempo de instalação do quadro (agudo/crônico), a distribuição corporal (focal/generalizada) e sintomas associados. Na infância, a principal causa da forma aguda é a coreia de Sydenham. Descrevemos o caso de uma paciente do sexo feminino de 13 anos que apresentou hemicoreia de instalação aguda relacionada a febre reumática, sendo a manifestação dimidiada atípica nesta condição.

There are numerous causes of chorea, and some characteristics of the presentation of this symptom help in the diagnosis reasoning, such as the onset time of the condition (acute/chronic), body distribution (local/generalized), and associated symptoms. In childhood, the main cause of acute chorea is Sydenham chorea. In childhood, the main cause of the acute form is Sydenham chorea. We report a case of a 13-year-old female patient who presented with acute onset hemichorea, being diagnosed with Sydenham's chorea.

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In patients with Chronic Rheumatic Carditis, active carditis is an often underdiagnosed condition. Rheumatic attacks promote the aggravation of existing lesions, leading to a deterioration of the patient's clinical condition. Thus, reducing the morbidity and mortality of the disease depends, in part, on controlling relapses through secondary prophylaxis. Underdiagnosis is due in part to the occurrence of subclinical rheumatic attacks. This study was carried out with data from patients who were diagnosed with Chronic Rheumatic Carditis and who underwent cardiac surgery for valve replacement or repair, without clinical or laboratory evidence of rheumatic outbreak. A fragment of myocardium was sent for histopathological analysis. Data on the frequency of histopathological alterations compatible with a rheumatic outbreak were analyzed. After analysis, 80% of patients showed changes compatible with inflammatory activity. Of these, 87.5% had lymphocytic infiltrate; 25% had Aschoff's nodules. The most frequent histopathological findings of chronic disease were myocardial hypertrophy in 56.7% of patients and fibrosis in 53.3% (AU).

Nos pacientes com Cardite Reumática Crônica, a cardite em atividade é uma condição frequentemente subdiagnos-ticada. Os surtos reumáticos promovem o agravamento das lesões já existentes, levando a uma deterioração da condição clínica do paciente. Dessa forma a redução da morbimortalidade da doença depende, em parte, do contro-le de recidivas a partir da profilaxia secundária. O subdiagnóstico deve-se em parte a ocorrência de surtos reumáti-cos subclínicos. Este estudo foi realizado com dados dos pacientes que tiveram o diagnóstico de Cardite Reumática Crônica e foram submetidos à cirurgia cardíaca para troca ou plastia valvar, sem evidência clínica ou laboratorial de surto reumático. Um fragmento de miocárdio foi enviado para análise histopatológica. Foram analisados os dados de frequência de alterações histopatológicas compatíveis com surto reumático. Após análises, 80% dos pacientes apresentaram alterações compatíveis com atividade inflamatória. Desses, 87,5% apresentavam infiltrado linfocitá-rio; 25% apresentavam nódulos de Aschoff. Os achados histopatológicos de doença crônica mais frequentes foram hipertrofia miocárdica em 56,7% pacientes e fibrose em 53,3% (AU).

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Rheumatic fever (RF) and rheumatic heart disease (RHD) are still highly prevalent, particularly in low- and middle-income countries. RHD is a neglected and underdiagnosed disease for which no specific laboratory diagnostic test is completely reliable. This is a retrospective observational study, which included 118 patients with RHD who underwent cardiac surgery from 1985 to 2018. The aim of this investigation was to evaluate the clinical, epidemiological, echocardiographic and pathological characteristics in two cohorts of RHD patients: one cohort with Aschoff bodies present in their pathological results and the other without such histopathological characteristics. No conventional clinical and laboratory tests for RHD myocarditis were able to identify active carditis during the preoperative phase of valve repair or replacement. Patients who had Aschoff bodies in their pathological results were younger (median age of 13 years (11-24 years) vs. 27 years (17-37 years), p = 0.001) and had higher rate of late mortality (22.9% vs. 5.4%, p = 0.043). In conclusion, the presence of Aschoff bodies in pathological findings may predict increased long-term mortality, emphasizing the importance of comprehensive pathology analysis for suspected myocarditis during heart surgery.

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PURPOSE: To present electroretinogram findings in extensive macular atrophy with pseudodrusen (EMAP) and describe associated systemic factors. DESIGN: Retrospective case series. METHODS: Data on medical history, visual symptoms, multimodal imaging findings, and visual field were collected from the medical records of patients with extensive macular atrophy with pseudodrusen who attended a visual electrophysiology laboratory. Electrophysiological tests, including full-field electroretinogram, multifocal electroretinogram and photopic negative response, were performed. RESULTS: Eighteen patients (10 [56%] females, age 49-66 years) were included. Of these, 17 (94%) had a history of rheumatic fever in childhood and/or adolescence, 7 (39%) had cardiovascular disease, 4 (22%) had autoimmune disease, and 10 (56%) had inflammatory conditions. The primary visual complaints were nyctalopia (95%), followed by visual field loss (67%) and dyschromatopsia (67%). The key retinal findings included retinal pigmented epithelium atrophy in the macular region and subretinal drusenoid deposits. Regarding electrophysiological results, 100% of patients had abnormalities on multifocal electroretinogram, 94% displayed alterations in photopic negative response, and 78% showed changes in the full-field electroretinogram. CONCLUSIONS: In this cohort, electrophysiologic evaluation demonstrated diffuse retinal dysfunction affecting all layers of the retina in patients with EMAP. The disease is associated with immune-mediated systemic conditions, chiefly rheumatic fever.

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Background: Rheumatic heart disease (RHD) disproportionately affects low-income and middle-income countries. Latin America and the Caribbean (LAC) have been less represented in scientific literature. We aimed to describe the epidemiology, burden and implemented screening and prevention strategies of RHD in LAC. Methods: We systematically searched PubMed, Embase, LILACS, and SciELO from 1990 to April 2021. Observational and experimental studies that described data on the epidemiology, burden, or prevention/screening strategies of RHD, regardless of age or language, were included. The risk of bias was assessed by previously published tools depending on their study design. Pre-specified data were independently extracted and presented by each topic (epidemiology, burden, prevention/screening). PROSPERO registration number: CRD42021250043. Results: Forty-eight studies out of 1692 non-duplicate records met the eligibility criteria. They were mainly from Brazil, observational in design, and hospital-based. Data on the epidemiology of acute rheumatic fever (ARF) was not recent (most before 2000) with studies describing decreasing incidence through the years. The prevalence of RHD was described in six studies, ranging from 0.24 to 48 per 1,000 among studies evaluating schoolchildren. Nine studies described data based on admissions, ranging from 0.04% to 7.1% in single-center studies. Twenty-four studies assessed the burden of RHD with most of them reporting mortality rates/proportions and complications such as the need for intervention, atrial fibrillation, or embolism. Six preventive strategies were identified that included educational, register-based, and/or secondary prophylaxis strategies. Three well-established echocardiographic screening studies in Brazil and Peru were identified. Conclusions: Most ARF/RHD research in LAC comes from a single country, Brazil where preventive/screening efforts have been conducted. There was a paucity of data from several countries in the region, reflecting the need for epidemiological studies from more countries in LAC which will provide a better picture of the current situation of ARF/RHD and guide the implementation of preventive strategies.

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