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Background: Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) remain major public health issues. Although the primary and secondary prevention of RHD through appropriate management of bacterial pharyngitis and ARF are well-described in the literature, few studies address the knowledge, attitude, and practice (KAP) of developing countries. We aimed to evaluate the KAP of the frontline physicians in Egyptian university hospitals regarding pharyngitis and ARF. Methods: We employed a cross-sectional design between September 1st, 2022, and January 31st, 2023 using a self-administered questionnaire in 21 Egyptian universities. The questionnaire was developed based on previous studies and recent guidelines and included four domains: sociodemographic data, knowledge, attitude, and practice regarding pharyngitis and ARF. We utilized both online (Google Forms) and paper surveys. Frontline physicians, including interns, residents, and assistant lecturers, were conveniently invited to participate. Furthermore, with the help of participating phycisians in recruiting their colleagues, we utilized the snowball method. Data were analyzed using IBM SPSS version 27 software. Results: The final analysis included 629 participants, of whom 372 (59.1%) were males and 257 (40.9%) had direct contact with ARF patients. Most participants (61.5%) had a fair knowledge level while 69.5% had a fair level of practice regarding ARF and pharyngitis. Higher satisfactory knowledge levels were noted regarding pharyngitis (17.1% vs. 11.3%; p-value: 0.036) and ARF (26.8% vs. 18%; p-value: 0.008) among physicians dealing directly with ARF cases compared to physicians in departments not dealing directly with ARF cases. Physicians in Cairo region universities had significantly higher levels of satisfactory knowledge about ARF compared to Delta and Upper Egypt region universities (p = 0.014). Delta region universities showed significantly lower levels of practice compared to Cairo and Upper Egypt region universities (p = 0.027). The most frequently recognized barriers against health promotion were low socioeconomic status (90.3%) and lack of adequate public education (85.8%). Conclusions: Despite the fair knowledge and practice levels towards bacterial pharyngitis and ARF among participants, many gaps were still identified that might contribute to RHD prevalence. Educational interventions should be implemented by updating the local guidelines in Egypt for diagnosis and management based on the most recent guidelines. [ABSTRACT FROM AUTHOR]

The spectrum of diseases caused by Streptococcus pyogenes (Strep A) ranges from superficial to serious life-threatening invasive infections. We conducted a scoping review of published articles between 1980 and 2021 to synthesize evidence of state transitions across the Strep A disease spectrum. We identified 175 articles reporting 262 distinct observations of Strep A disease state transitions. Among the included articles, the transition from an invasive or toxin-mediated disease state to another disease state (i.e., to recurrent ARF, RHD or death) was described 115 times (43.9% of all included transition pairs) while the transition to and from locally invasive category was the lowest (n = 7; 0.02%). Transitions from well to any other state was most frequently reported (49%) whereas a relatively higher number of studies (n = 71) reported transition from invasive disease to death. Transitions from any disease state to locally invasive, Strep A pharyngitis to invasive disease, and chronic kidney disease to death were lacking. Transitions related to severe invasive diseases were more frequently reported than superficial ones. Most evidence originated from high-income countries and there is a critical need for new studies in low- and middle-income countries to infer the state transitions across the Strep A disease spectrum in these high-burden settings. [ABSTRACT FROM AUTHOR]

Background: Rheumatic heart disease (RHD) is the most commonly acquired heart disease in children and young people in low and middle-income settings. Fragile health systems and scarcity of data persist to limit the understanding of the relative burden of this disease. The aims of this study were to estimate the prevalence of RHD and to assess the RHD-related health care systems in Namibia. Methods: Data was retrieved from outpatient and inpatient registers for all patients diagnosed and treated for RHD between January 2010 to December 2020. We used descriptive statistics to estimate the prevalence of RHD. Key observations and engagement with local cardiac clinicians and patients helped to identify key areas of improvement in the systems. Results: The outpatient register covered 0.032% of the adult Namibian population and combined with the cumulative incidence from the inpatient register we predict the prevalence of clinically diagnosed RHD to be between 0.05% and 0.10% in Namibia. Young people (< 18 years old) are most affected (72%), and most cases are from the north-eastern regions. Mitral heart valve impairment (58%) was the most common among patients. We identified weaknesses in care systems i.e., lack of patient unique identifiers, missing data, and clinic-based prevention activities. Conclusion: The prevalence of RHD is expected to be lower than previously reported. It will be valuable to investigate latent RHD and patient follow-ups for better estimates of the true burden of disease. Surveillance systems needs improvements to enhance data quality. Plans for expansions of the clinic-based interventions must adopt the "Awareness Surveillance Advocacy Prevention" framework supported by relevant resolutions by the WHO. [ABSTRACT FROM AUTHOR]

Background: Acute Rheumatic Fever (ARF) is a critically important condition for which there is no diagnostic test. Diagnosis requires the use of a set of criteria comprising clinical, laboratory, electrocardiographic and echocardiographic findings. The complexity of the algorithm and the fact that clinicians lack familiarity with ARF, make ARF diagnosis ideally suited to an electronic decision support tool. The ARF Diagnosis Calculator was developed to assist clinicians in diagnosing ARF and correctly assign categories of 'possible, 'probable' or 'definite' ARF. This research aimed to evaluate the acceptability, accuracy, and test performance of the ARF Diagnosis Calculator.Methods: Three strategies were used to provide triangulation of data. Users of the calculator employed at Top End Health Service, Northern Territory, Australia were invited to participate in an online survey, and clinicians with ARF expertise were invited to participate in semi-structured interviews. Qualitative data were analysed using inductive analysis. Performance of the calculator in correctly diagnosing ARF was assessed using clinical data from 35 patients presenting with suspected ARF. Diagnoses obtained from the calculator were compared using the Kappa statistic with those obtained from a panel of expert clinicians.Results: Survey responses were available from 23 Top End Health Service medical practitioners, and interview data were available from five expert clinicians. Using a 6-point Likert scale, participants highly recommended the ARF Diagnosis Calculator (median 6, IQR 1), found it easy to use (median 5, IQR 1) and believed the calculator helped them diagnose ARF (median 5, IQR 1). Clinicians with ARF expertise noted that electronic decision making is not a substitute for clinical experience. There was high agreement between the ARF Diagnosis Calculator and the 'gold standard' ARF diagnostic process (κ = 0.767, 95% CI: 0.568-0.967). Incorrect assignment of diagnosis occurred in 4/35 (11%) patients highlighting the greater accuracy of expert clinical input for ambiguous presentations. Sixteen changes were incorporated into a revised version of the calculator.Conclusions: The ARF Diagnosis Calculator is an easy-to-use, accessible tool, but it does not replace clinical expertise. The calculator performed well amongst clinicians and is an acceptable tool for use within the clinical setting with a high level of accuracy in comparison to the gold standard diagnostic process. Effective resources to support clinicians are critically important for improving the quality of care of ARF. [ABSTRACT FROM AUTHOR]

Background: Rheumatic heart disease is preventable, yet associated with significant health burden, mostly in low-resourced settings. It is prevalent among children and young adults living in impoverished areas. Primordial, primary, and secondary preventive measures have been recommended through health interventions and comprehensive programmes, although most implemented interventions are the high-resourced settings. The proposed review aims to synthesise the evidence of prevention effectiveness of implemented health interventions for the prevention of rheumatic heart disease. Methods and design: This article describes a protocol for a systematic review. A predefined search strategy will be used to search for relevant literature published from the year 2000 to present. Electronic databases Medline, Web of Science, Scopus, and Cochrane Central Register of Controlled Trials will be searched for the studies, as well as reference lists of relevant studies included. Risk of bias and quality appraisal will be done for the included studies using ROBINS-I tool and Cochrane tool for assessing risk of bias in randomised control trials. Findings will be analysed in subgroups based on the level of intervention and prevention strategy implemented. We will present the findings in descriptive formats with tables and flow diagrams. Discussion: This review will provide evidence on the prevention effectiveness of interventions or strategies implemented for the prevention of RHD. The findings of this will be significant for policy, practice, and research in countries planning to implement interventions. Registration: PROSPERO ID: CRD42020170503. [ABSTRACT FROM AUTHOR]

Background: Despite a notable decrease in acute rheumatic fever (ARF) incidence in the past few decades, there are still cases in our setting. Sydenham chorea (SC) may be the initial manifestation for this condition in childhood in a significant proportion of children. We report two cases of choreoathetosis in children as the first manifestation of ARF.Case Presentation: A previously healthy 8-year-old boy presented with right hemichorea with a predominance in the brachial region, orofacial dyskinesias and speech difficulties for the past 2 weeks. The only medical history of interest was a common catarrhal illness 3 weeks before and nonspecific bilateral tenosynovitis in both feet since a year prior. A brain computerized tomography was normal and the echocardiogram showed mild mitral and aortic regurgitation, meeting ARF criteria. He demonstrated clinical improvement with treatment based on prednisone and carbamazepine. The second patient was a 10-year-old girl with choreic movements of the right half of the body and repetitive right eye closure of 1 week duration. She had symptoms of fever and rash the previous week and pharyngitis that resolved without antibiotic 2 months before. Blood tests revealed elevated C reactive protein (12 mg/dl) and erythrocyte sedimentation rate (96 mm/h). Brain magnetic resonance was normal and echocardiogram showed left ventricle dilation and mild mitral regurgitation, leading to the diagnosis of ARF. Due to neurological involvement, she received corticosteroids and intravenous immunoglobulin treatment, with worsening of neurological symptoms that required valproic acid with remission of the hemichorea. In addition skin lessions compatible with erythema marginatum appeared on the upper limbs.Conclusions: SC should be the main diagnostic consideration in cases of hemichorea with normal neuroimaging in children. The cases reported highlight the need to maintain a high index of suspicion even in settings where incidende of ARF is low and the need to perform cardiological investigations in all patients with suspected SC, due to the possibility of subclinical valve lesions. Good adherence to secondary prophylaxis is crucial to avoid chorea relapses and worsening valve disease. [ABSTRACT FROM AUTHOR]

Background: Rheumatic heart disease (RHD) is a chronic cardiac condition with an infectious aetiology, causing high disease burden in low-income settings. Affected individuals are young and associated morbidity is high. However, RHD is relatively neglected due to the populations involved and its lower incidence relative to other heart diseases.Methods and Results: In this narrative review, we describe how RHD care can be informed by and integrated with models of care developed for priority non-communicable diseases (coronary heart disease), and high-burden communicable diseases (tuberculosis). Examining the four-level prevention model (primordial through tertiary prevention) suggests primordial and primary prevention of RHD can leverage off existing tuberculosis control efforts, given shared risk factors. Successes in coronary heart disease control provide inspiration for similarly bold initiatives for RHD. Further, we illustrate how the Chronic Care Model (CCM), developed for use in non-communicable diseases, offers a relevant framework to approach RHD care. Systems strengthening through greater integration of services can improve RHD programs.Conclusion: Strengthening of systems through integration/linkages with other well-performing and resourced services in conjunction with policies to adopt the CCM framework for the secondary and tertiary prevention of RHD in settings with limited resources, has the potential to significantly reduce the burden of RHD globally. More research is required to provide evidence-based recommendations for policy and service design. [ABSTRACT FROM AUTHOR]

Background: Acute rheumatic fever (ARF) rates have declined to near zero in nearly all developed countries. However, in New Zealand rates have not declined since the 1980s. Further, ARF diagnoses in New Zealand are inequitably distributed-occurring almost exclusively in Māori (the indigenous population) and Pacific children-with very low rates in the majority New Zealand European population. With ARF diagnosis, secondary prophylaxis is key to prevent recurrence. The purpose of this study was to identify the perceived enablers and barriers to secondary recurrence prophylaxis following ARF for Māori patients aged 14-21. Methods: This study took a systems approach, was informed by patient voice and used a framework method to explore potential barriers and enablers to ongoing adherence with monthly antibiotic injections for secondary prophylaxis. Qualitative interviews were conducted with 19 Māori ARF patients receiving recurrence prophylaxis in the Waikato District Health Board region. Participants included those fully adherent to treatment, those with intermittent adherence or those who had been "lost to follow-up." Results: Barriers and enablers were presented around three factors: system (including access/resources), relational and individual. Access and resources included district nurses coming to patients as an enabler and lack of income and time off work as barriers. Relational characteristics included support from family and friends as enablers and district nurse communication as predominantly a positive although not enabling factor. Individual characteristics included understanding, personal responsibility and fear/pain of injections. Conclusion: This detailed exploration of barriers and enablers for ongoing secondary prophylaxis provides important new information for the prevention of recurrent ARF. Among other considerations, a national register, innovative engagement with youth and their families and a comprehensive pain management programme are likely to improve adherence to ongoing secondary prophylaxis and reduce the burden of RHD for New Zealand individuals, families and health system. [ABSTRACT FROM AUTHOR]

Background: Rheumatic heart disease (RHD), caused by acute rheumatic fever (ARF), is a major health problem in Australian Aboriginal communities. Progress in controlling RHD requires improvements in the delivery of secondary prophylaxis, which comprises regular, long-term injections of penicillin for people with ARF/RHD.Methods/design: This trial aims to improve uptake of secondary prophylaxis among Aboriginal people with ARF/RHD to reduce progression or worsening of RHD. This is a stepped-wedge, randomised trial in consenting communities in Australia's Northern Territory. Pairs of randomly-chosen clinics from among those consenting enter the study at 3-monthly steps. The intervention to which clinics are randomised comprises a multi-faceted systems-based package, in which clinics are supported to develop and implement strategies to improve penicillin delivery, aligned with elements of the Chronic Care Model. Continuous quality improvement processes will be used, including 3-monthly feedback to clinic staff of adherence rates of their ARF/RHD clients. The primary outcome is the proportion of people with ARF/RHD receiving ≥ 80% of scheduled penicillin injections over a minimum 12-month period. The sample size of 300 ARF/RHD clients across five community clusters will power the study to detect a 20% increase in the proportion of individuals achieving this target, from a worrying low baseline of 20%, to 40 %. Secondary outcomes pertaining to other measures of adherence will be assessed. Within the randomised trial design, a mixed-methods evaluation will be embedded to evaluate the efficiency, effectiveness, impact and relevance, sustainability, process and fidelity, and performance of the intervention. The evaluation will establish any causal link between outcomes and the intervention. The planned study duration is from 2013 to 2016.Discussion: Continuous quality improvement has a strong track record in Australia's Northern Territory, and its use has resulted in modest benefits in a pilot, non-randomised ARF/RHD study. If successful, this new intervention using the Chronic Care Model as a scaffold and evaluated using a well-developed theory-based framework, will provide a practical and transferable approach to ARF/RHD control.Trial Registration: Australian New Zealand Clinical Trials Registry: ACTRN12613000223730. Date registered: 25 February 2013. [ABSTRACT FROM AUTHOR]

Background The New Zealand (NZ) Government has made a strong commitment to reduce the incidence of rheumatic fever (RF) by two thirds, to 1.4 cases per 100,000, by mid-2017. We reviewed the NZ RF surveillance sector, aiming to identify potential improvements which would support optimal RF control and prevention activities. Methods This review used a recently developed surveillance sector review method. Interviews with 36 key informants were used to describe the sector, assess it and identify its gaps. Priorities for improvement and implementation strategies were determined following discussion with these key informants, with policy advisors and within the research team. Results Key improvements identified included the need for a comprehensive RF surveillance strategy, integrated reporting and an online national RF register. At a managerial level this review provided evidence for system change and built support for this across the surveillance sector. Conclusions The surveillance sector review approach can be added to the small set of tools currently available for developing and evaluating surveillance systems. This new approach is likely to prove useful as we confront the challenges of combating new emerging infectious diseases, responding to global environmental changes, and reducing health inequalities. [ABSTRACT FROM AUTHOR]

Background: Incidence of acute rheumatic fever (ARF) and prevalence of rheumatic heart disease (RHD) in the Pacific region, including New Caledonia, are amongst the highest in the world. The main priority of long-term management of ARF or RHD is to ensure secondary prophylaxis is adhered to. The objectives of this study were to evaluate rates of adherence in people receiving antibiotic prophylaxis by intramuscular injections of penicillin in Lifou and to determine the factors associated with a poor adherence in this population. Methods: We conducted a retrospective cohort study and we included 70 patients receiving injections of antibiotic prophylaxis to prevent ARF recurrence on the island of Lifou. Patients were classified as "good-adherent" when the rate of adherence was ⩾ 80% of the expected injections and as "poor-adherent" when it was <80%. Statistical analysis to identify factors associated with adherence was performed using a multivariate logistic regression model. Results: Our study showed that 46% of patients from Lifou receiving antibiotic prophylaxis for ARF or RHD had a rate of adherence <80% and were therefore at high risk of recurrence of ARF. Three independent factors were protective against poor adherence: a household with more than five people (odds ratio, 0.25; 95% confidence interval [CI], 0.08 to 0.75), a previous medical history of symptomatic ARF (odds ratio, 0.20; 95% CI, 0.04 to 0.98) and an adequate healthcare coverage (odds ratio, 0.21; 95% CI 0.06 to 0.72). Conclusions: To improve adherence to secondary prophylaxis in Lifou, we therefore propose the following recommendations arising from the results of this study: i) identifying patients receiving antibiotic prophylaxis without medical history of ARF to strengthen their therapeutic education and ii) improving the medical coverage in patients with ARF or RHD. We also recommend that the nurse designated for the ARF prevention program in Lifou coordinate an active recall system based on an updated local register. But the key point to improve adherence among Melanesian patients is probably to give appropriate information regarding the disease and the treatment, taking into account the Melanesian perceptions of the disease. [ABSTRACT FROM AUTHOR]

Background: In Australia, rheumatic heart disease (RHD) is almost exclusively restricted to Aboriginal Australian and Torres Strait Islander people with children being at highest risk. International criteria for echocardiographic diagnosis of RHD have been developed but the significance of minor heart valve abnormalities which do not reach these criteria remains unclear. The Rheumatic Fever Follow-Up Study (RhFFUS) aims to clarify this question in children and adolescents at high risk of RHD. Methods/design: RhFFUS is a cohort study of Aboriginal and/or Torres Strait Islander children and adolescents aged 8–17 years residing in 32 remote Australian communities. Cases are people with non-specific heart valve abnormalities detected on prior screening echocardiography. Controls (two per case) are age, gender, community and ethnicity-matched to cases and had a prior normal screening echocardiogram. Participants will have echocardiography about 3 years after initial screening echocardiogram and enhanced surveillance for any history suggestive of acute rheumatic fever (ARF). It will then be determined if cases are at higher risk of (1) ARF or (2) developing progressive echocardiography-detected valve changes consistent with RHD. The occurrence and timing of episodes of ARF will be assessed retrospectively for 5 years from the time of the RhFFUS echocardiogram. Episodes of ARF will be identified through regional surveillance and notification databases, carer/subject interviews, primary healthcare history reviews, and hospital separation diagnoses.Progression of valvular abnormalities will be assessed prospectively using transthoracic echocardiography and standardized operating and reporting procedures. Progression of valve lesions will be determined by specialist cardiologist readers who will assess the initial screening and subsequent RhFFUS screening echocardiogram for each participant. The readers will be blinded to the initial assessment and temporal order of the two echocardiograms. Discussion: RhFFUS will determine if subtle changes on echocardiography represent the earliest changes of RHD or mere variations of normal heart anatomy. In turn it will inform criteria to be used in determining whether secondary antibiotic prophylaxis should be utilized in individuals with no clear history of ARF and minor abnormalities on echocardiography. RhFFUS will also inform the ongoing debate regarding the potential role of screening echocardiography for the detection of RHD in this setting. [ABSTRACT FROM AUTHOR]

Background: Group A streptococcus (GAS) is the most frequent cause of bacterial pharyngitis in school-aged children. The postinfection sequel as acute rheumatic fever (ARF) and rheumatic heart disease that cause morbidity and mortality among young people is public health concerns in several developing countries. Asymptomatic carriage state of GAS is not fully understood in terms of host and bacterial factors. Although the ability of transmitting GAS of the asymptomatic carriers is relatively low, they may present the reservoir of the epidemic. A fraction of GAS carriers is difficult to estimate in practice and may greatly vary between populations. Understanding the role of carriage on the transmission dynamic of GAS is important for assessing the public health impact of the ARF. Method: This study investigates the effect of GAS carriers on both the transmission and dynamic of ARF cases by using a mathematical model. Result: We derive the sufficient conditions for which the GAS can spread or extinct from the naive population under the variation of the fraction of symptomatic cases over the incidence of GAS. The threshold is possible to occur in general, but the last condition which is rather restrictive and involves parameter uncertainty. The increasing of carriers in the endemic state leads to the reduction in magnitude of the reproduction number and the number of ARF patients. We demonstrate that the adjustment of parameters can be carried out by the use of endemic state and some specific data. Conclusion: We show theoretically that the presence of asymptomatic carriers may induce the epidemic threshold and reduce the virulence of GAS and the prevalence of ARF. [ABSTRACT FROM AUTHOR]