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Objective: In Australia, accurate case ascertainment of acute rheumatic fever (ARF) and rheumatic heart disease (RHD) diagnoses for disease surveillance and control purposes requires the use of multiple data sources, including RHD registers and hospitalisation records. Despite drawing on multiple data sources, the true burden of ARF/RHD is likely to be underestimated. Methods: This study used capture–recapture methods to quantify the missing number of ARF/RHD cases in data from hospitals and jurisdictional RHD registers. Linked datasets comprised reported cases of ARF/RHD in register records and administrative hospital data. Results: Capture–recapture analyses indicated the total number of new ARF/RHD cases in three Australian jurisdictions (Queensland, South Australia and Western Australia), among people aged 3–54 years, was 3480 (95% CI = 3366–3600) during 2011–2016. This included 894 (25.7%) individuals who were not listed in either the hospital or register datasets. Non-Indigenous, urban and older people with ARF/RHD were least likely to be identified in either the hospital or register data sources. Conclusions: The 894 likely ARF/RHD cases our analyses detected that are not included in the routine surveillance datasets are concerning and quantify the magnitude and characteristics of under-notification to RHD registers in Australia, especially for groups that are not typically at high risk of ARF. What is known about the topic? In Australia, the true burden of acute rheumatic fever (ARF) and rheumatic heart disease (RHD) is likely to be underestimated. What does this paper add? Capture–recapture method provided a measure of the undetected cases of ARF/RHD based on two data sources and characteristics of groups where under-notification occurred. What are the implications for practitioners? This study identifies a need to support clinicians to maintain an index of suspicion of ARF/RHD when working with groups that are not typically at high risk of ARF. [ABSTRACT FROM AUTHOR]

Objective: This study aims to describe the pattern and trends in acute rheumatic fever (ARF)/rheumatic heart disease (RHD)-related hospitalisations and costs for Australians aged <65 years. Methods: This retrospective linked data study measured trends in hospitalisations and costs for ARF, RHD and complications of ARF/RHD in Northern Territory, South Australia, Western Australia, Queensland and New South Wales between 1 July 2012 and 30 June 2017. Persons with ARF/RHD were identified from RHD registers and/or hospital records. Results: Over the 5-year study period, 791 children, aged <16 years (86.3% Indigenous), and 2761 adults, aged 16–64 years (44.8% Indigenous), were hospitalised for ARF, RHD or associated complications. On average there were 296 paediatric admissions per year, increasing 6.1% annually (95% CI: 2.4–9.6%, P = 0.001) and 1442 adult admissions per year, increasing 1.7% annually (95% CI: 0.1–3.4%, P = 0.03). Total 5-year costs were AU$130.6 m (AU$17.6 m paediatric, AU$113.0 m adult). Paediatric costs were mostly for ARF-related admissions whereas adult costs mostly involved valvular surgery. Emergency admissions and air ambulance transfers were common, particularly for non-metropolitan residents. Conclusions: Successful ARF/RHD prevention would deliver significant hospital cost savings. Investment in primary and specialist health care in regional areas may reduce emergency admissions and regional transfers, further reducing hospital burden. What is known about the topic? Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) cause significant morbidity and mortality. Australian treatment costs for 2016–2031 are predicted to be AU$317 million, with 60% attributed to hospitalisations. What does this paper add? We report the first 'real-world' hospitalisation patterns and costs associated with ARF, RHD and complications. Admissions have increased annually over 2012–2017 with costs of AU$130.6 million. What are the implications for practitioners? Successful ARF/RHD prevention would deliver significant hospital cost savings. Investment in primary and specialist health care in regional areas may reduce frequent emergency admissions and regional transfers. [ABSTRACT FROM AUTHOR]

Group A Streptococcus (GAS) is a widely distributed bacterium that is Gram-positive and serves as the primary cause of acute rheumatic fever (ARF) episodes. Rheumatic heart disease (RHD) is a sequela resulting from repeated ARF attacks which are also caused by repeated GAS infections. ARF/RHD morbidity and mortality rates are incredibly high in low- and middle-income countries. This is closely related to poor levels of sanitation which causes the enhanced incidence of GAS infections. Management of carditis in RHD cases is quite challenging, particularly in developing countries, considering that medical treatment is only palliative, while definitive treatment often requires more invasive procedures with high costs. Preventive action through vaccination against GAS infection is one of the most effective steps as a solution in reducing RHD morbidity and mortality due to curative treatments are expensive. Various developments of M-protein-based GAS vaccines have been carried out over the last few decades and have recently begun to enter the clinical stage. Nevertheless, this vaccination generates cross-reactive antibodies that might trigger ARF assaults as a result of the resemblance between the M-protein structure and proteins found in many human tissues. Consequently, the development of a vaccine utilizing L-Rhamnose derived from the poly-rhamnose backbone of Group A Carbohydrate (GAC) commenced. The L-Rhamnose-based vaccine was chosen due to the absence of the Rhamnose biosynthesis pathway in mammalian cells including humans thus this molecule is not found in any body tissue. Recent pre-clinical studies reveal that L-Rhamnose-based vaccines provide a protective effect by increasing IgG antibody titers without causing cross-reactive antibodies in test animal tissue. These findings demonstrate that the L-Rhamnose-based vaccine possesses strong immunogenicity, which effectively protects against GAS infection while maintaining a significantly higher degree of safety.

Group A Streptococcus (GAS) is a widely distributed bacterium that is Gram-positive and serves as the primary cause of acute rheumatic fever (ARF) episodes. Rheumatic heart disease (RHD) is a sequela resulting from repeated ARF attacks which are also caused by repeated GAS infections. ARF/RHD morbidity and mortality rates are incredibly high in low- and middle-income countries. This is closely related to poor levels of sanitation which causes the enhanced incidence of GAS infections. Management of carditis in RHD cases is quite challenging, particularly in developing countries, considering that medical treatment is only palliative, while definitive treatment often requires more invasive procedures with high costs. Preventive action through vaccination against GAS infection is one of the most effective steps as a solution in reducing RHD morbidity and mortality due to curative treatments are expensive. Various developments of M-protein-based GAS vaccines have been carried out over the last few decades and have recently begun to enter the clinical stage. Nevertheless, this vaccination generates cross-reactive antibodies that might trigger ARF assaults as a result of the resemblance between the M-protein structure and proteins found in many human tissues. Consequently, the development of a vaccine utilizing L-Rhamnose derived from the poly-rhamnose backbone of Group A Carbohydrate (GAC) commenced. The L-Rhamnose-based vaccine was chosen due to the absence of the Rhamnose biosynthesis pathway in mammalian cells including humans thus this molecule is not found in any body tissue. Recent pre-clinical studies reveal that L-Rhamnose-based vaccines provide a protective effect by increasing IgG antibody titers without causing cross-reactive antibodies in test animal tissue. These findings demonstrate that the L-Rhamnose-based vaccine possesses strong immunogenicity, which effectively protects against GAS infection while maintaining a significantly higher degree of safety.

Abstract 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.

Group A Streptococcus (GAS) is a widely distributed bacterium that is Gram-positive and serves as the primary cause of acute rheumatic fever (ARF) episodes. Rheumatic heart disease (RHD) is a sequela resulting from repeated ARF attacks which are also caused by repeated GAS infections. ARF/RHD morbidity and mortality rates are incredibly high in low- and middle-income countries. This is closely related to poor levels of sanitation which causes the enhanced incidence of GAS infections. Management of carditis in RHD cases is quite challenging, particularly in developing countries, considering that medical treatment is only palliative, while definitive treatment often requires more invasive procedures with high costs. Preventive action through vaccination against GAS infection is one of the most effective steps as a solution in reducing RHD morbidity and mortality due to curative treatments are expensive. Various developments of M-protein-based GAS vaccines have been carried out over the last few decades and have recently begun to enter the clinical stage. Nevertheless, this vaccination generates cross-reactive antibodies that might trigger ARF assaults as a result of the resemblance between the M-protein structure and proteins found in many human tissues. Consequently, the development of a vaccine utilizing L-Rhamnose derived from the poly-rhamnose backbone of Group A Carbohydrate (GAC) commenced. The L-Rhamnose-based vaccine was chosen due to the absence of the Rhamnose biosynthesis pathway in mammalian cells including humans thus this molecule is not found in any body tissue. Recent pre-clinical studies reveal that L-Rhamnose-based vaccines provide a protective effect by increasing IgG antibody titers without causing cross-reactive antibodies in test animal tissue. These findings demonstrate that the L-Rhamnose-based vaccine possesses strong immunogenicity, which effectively protects against GAS infection while maintaining a significantly higher degree of safety.

Sydenham's chorea (SC), an autoimmune disorder affecting the central nervous system, is a pivotal diagnostic criterion for acute rheumatic fever. Primarily prevalent in childhood, especially in developing countries, SC manifests with involuntary movements and neuropsychiatric symptoms. Predominantly occurring between ages 5 and 15, with a female bias, SC may recur, particularly during pregnancy or estrogen use. The autoimmune response affecting the basal ganglia, notably against dopamine, underlies the pathophysiology. Clinical management necessitates an integrated approach, potentially involving immunomodulatory therapies. To address discrepancies in SC management, a survey was conducted across Italy, targeting specialists in neurology, pediatrics, child neuropsychiatry, and rheumatology. Of the 51 responding physicians, consensus favored hospitalization for suspected SC, with broad support for laboratory tests and brain MRI. Treatment preferences showed agreement on oral prednisone and IVIG, while opinions varied on duration and plasmapheresis. Haloperidol emerged as the preferred symptomatic therapy. Post-SC penicillin prophylaxis and steroid therapy gained strong support, although opinions differed on duration. Follow-up recommendations included neuropsychological and cardiological assessments. Despite offering valuable insights, broader and more studies are needed in order to guide treatment decisions in this well-known yet challenging complication of acute rheumatic fever, which continues to warrant scientific attention and concerted clinical efforts. • Sydenham's Chorea is a major concern, especially in children from developing countries, with long-term neuropsychiatric challenges. • We surveyed 51 clinicians via an online questionnaire, revealing current diagnostic and therapeutic practices for SC. • Physicians agreed on hospitalization and prefer MRI as the primary neuroimaging technique for diagnosing SC in children. • Treatment preferences varied, influenced by side effects and disease severity, showing differing clinical judgments. • Findings highlight the need for standardized treatment guidelines to enhance SC management, especially in Italy. [ABSTRACT FROM AUTHOR]

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]

Acute rheumatic fever and Takayasu arteritis are examples of autoimmune diseases that commonly affect the cardiovascular system. We report an infrequent co-occurrence of both these diseases in an adolescent male. It may appear that in some individuals, the rheumatic fever may act as a trigger for the development of large vessel vasculitis. This possibility should be considered in patients on follow-up if they develop fresh features of cardiovascular compromise despite appropriate medical, interventional, or surgical therapy for rheumatic heart disease. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Rheumatic heart disease is a preventable disease. Patients may not present with a typical history of sore throat and polyarthritis but may present with Sydenham's chorea. We should not rely completely on clinical findings to rule out carditis. Echocardiography should be done to rule out subclinical carditis. Sydenham's chorea is a major manifestation of rheumatic fever. It occurs primarily in children and is seen rarely after the age of 20 years. We describe a 16‐year‐old girl who presented with purposeless involuntary movements of her upper and lower limbs. Laboratory blood reports showed raised erythrocyte sedimentation rate and anti‐streptolysin O. 2D Doppler Echocardiography confirmed subclinical carditis, thickened mitral and aortic valve with mild mitral regurgitation. She was managed as Acute Rheumatic Fever with oral Phenoxymethyl penicillin and Carbamazepine. At the latest follow‐up interviewing the caregiver, the patient had no sequelae. Early diagnosis is key to preventing late consequences of acute rheumatic fever and rheumatic heart disease. Sydenham's chorea is a rare presentation of acute rheumatic fever. The absence of clinical carditis does not rule out carditis. [ABSTRACT FROM AUTHOR]

Background: Acute rheumatic fever (ARF) is an autoimmune response to GAS (Group A ß-Hemolytic Streptococci) infection. (ARF) is the cause of Rheumatic heart disease. Aim: The objective of this study was the assessment of serum vitamin D levels of children diagnosed with rheumatic vulvular lesion. Patients & methods: This is a case control study that was performed on 50 patients with valvular lesion who were diagnosed previously and 50 healthy children as (control), age & sex matched to the patients at the Cardiology Unit of the pediatric department at El-Hussein Hospital, Al-Azhar University in the period between January 2023 and January 2024.All the studied children were subjected to full history taking, general, cardiac examination. lastly laboratory evaluation of serum vitamin D (25 OH), calcium, phosphorus, Mg, ALP, PTH, WBCs, ESR, CRP and ASO. level were done. Results: There was highly statistically significant difference between patients, control groups according to Serum 25 (OH)D (ng/ml), WBCs (cell/µL), ESR (mm/h), CRP (mg/L), ASO (IU/L), Ca (mg/dL), P (mg/dL), Mg (mg/dl), ALP (U/L), and PTH (pg/ml). There was significant decrease in Serum 25 (OH)D (ng/ml), Ca (mg/dL), P (mg/dL), while there was significant increase in WBCs (cell/µL), ESR (mm/h), CRP (mg/L), ASO (IU/L), Mg (mg/dl), ALP (U/L), PTH (pg./ml) among rheumatic group compared with control group. Conclusion: We found that WBCs, ESR, CRP, ASO, was elevated in children with rheumatic valvular lesion while Serum 25(OH)D, Ca and P was decreased. [ABSTRACT FROM AUTHOR]

ABSTRACT Controlled human infection (CHI) models can provide insights into transmission of pathogens such as Streptococcus pyogenes (Strep A). As part of the Controlled Human Infection with Penicillin for Streptococcus pyogenes (CHIPS) trial, we explored the potential for transmission among participants deliberately infected with the Strep A emm75 strain. Three approaches to understanding transmission were employed: the use of agar settle plates to capture possible droplet or airborne spread of Strep A; measurement of distance droplets could spread during conversation; and environmental swabbing of high-touch items to detect Strep A on surfaces. Of the 60 (27%) CHIPS trial participants across five cohorts, 16 were enrolled in this sub-study; availability of study staff was the primary reason for selection. In total, 189 plates and 260 swabs were collected. Strep A was grown on one settle plate from a participant on the second day, using plates placed 30 cm away. This participant received the placebo dose of penicillin and had met the primary endpoint of pharyngitis. Whole-genome sequencing identified this to be the challenge strain. Strep A was not detected on any swabs. In this small sample of CHI participants, we did not find evidence of Strep A transmission by the airborne route or fomites, and just one instance of droplet spread while acutely symptomatic with streptococcal pharyngitis. Although these experiments provide evidence of minimal transmission within controlled clinical settings, greater efforts are required to explore Strep A transmission in naturalistic settings.IMPORTANCEStreptococcus pyogenes remains a significant driver of morbidity and mortality, particularly in under-resourced settings. Understanding the transmission modalities of this pathogen is essential to ensuring the success of prevention methods. This proposed paper presents a nascent attempt to determine the transmission potential of Streptococcus pyogenes nested within a larger controlled human infection model.

Moses Ochora,1 Lydia Kyasimire,1 Dan Lutasingwa,1 Tamara Namata,2 Muna Ahmed,1 Alain Favina,3 Elias Kumbakumba,1 Dorah Nampijja1 1Department of Paediatrics and Child Health, Mbarara University of Science and Technology, Mbarara City, Uganda; 2Department of Community Health, Mbarara University of Science and Technology, Mbarara City, Uganda; 3Department of Psychiatry, Mbarara University of Science and Technology, Mbarara City, UgandaCorrespondence: Moses Ochora, Tel +256-751455470, Email mtochora@gmail.comIntroduction: Timely identification and treatment of a streptococcal throat infection prevents acute rheumatic fever (ARF) and its progression to Rheumatic Heart Disease (RHD). However, children in developing countries still present with established RHD, due to either missed, untreated or sub-optimally treated sore throats and ARF. We aimed to determine the level of knowledge, skills, and practices of primary health workers in South Western Uganda in providing care such children.Methods: We conducted a comparative quantitative cross-sectional study to assess knowledge, practices, and skills regarding the care of a child with a sore throat, ARF, and RHD. The responses were scored against a structured guide. The Fisher’s exact test and the chi-squared test with level of significance set at 0.05 were utilized to compare differences in knowledge, skills, and practices among health workers in private and public health facilities about ARF and RHD.Results: Eighty health workers from health facilities were interviewed in Mbarara district with a median age of 29.5 years (IQR 27.34) and median duration in practice of 5 years (IQR: 2, 10). On average, there were at least 3 children with sore throats weekly. At least 95% (CI: 87.25%– 98.80%) of the health worker had awareness about ARF and RHD. Only 43.75% (95% CI: 33.18%– 54.91%) had good knowledge about ARF and RHD. Majority, 61.25% (95% CI: 50.03%− 71.39%) did not know the proper prophylaxis and investigations for a child with ARF. There were no statistically significant differences but a clinically meaningful differentials in the level of knowledge among health workers in public and private facilities.Conclusion: The knowledge and skill level of health workers in primary healthcare facilities about ARF and RHD in South Western Uganda remains low, with no difference between practitioners in public and private facilities.Keywords: acute rheumatic fever, rheumatic heart disease, awareness, knowledge, healthcare provider

There is still no evidence-based guideline and consensus on the treatment Sydenham's Chorea (SC). The first-line medication preference of specialists depends on personal experience and is variable. In this study, we evaluate the treatment results of pediatric patients who were treated with valproic acid (VPA). The medical records of 17 patients diagnosed with SC were reviewed retrospectively. The mean time to clinical improvement was found as approximately 5 days, the mean duration of remission as 13.60 ± 3.94 weeks and the mean duration of medication use was found as 17.96 ± 3.81 weeks. No side effects were observed in any of the patients and relapse occurred in 2 patients. A positive correlation was found between the initial C-reactive protein (CRP) level and the duration of medication use. Until evidence-based guidelines are established, VPA can be used as an effective, safe, and inexpensive first-line treatment option, especially in pediatric patients. [ABSTRACT FROM AUTHOR]

Rheumatic carditis is the leading cause of permanent disability caused by damage of the cardiac valve. This study aimed to determine the outcome and predictors of valve surgery in patients with acute rheumatic fever (ARF) and recurrent rheumatic fever (RRF). This was a retrospective study of patients diagnosed with ARF and RRF between 2006 and 2021. The predictors of valve surgery were analysed using multivariable Cox proportional regression. The median age of patients with ARF and RRF (n=92) was 11 years (range 5–18). Seventeen patients (18%) were diagnosed with RRF. The most common presenting symptoms included clinical carditis (87%), heart failure (HF) (63%), fever (49%) and polyarthralgia (24%). Patients with moderate-to-severe rheumatic carditis (88%) were given prednisolone. After treatment, the severity of valvular regurgitation was reduced in 52 patients (59%). Twenty-three patients (25%) underwent valve surgery. The incidence of HF, RRF, severe mitral regurgitation on presentation, left ventricular enlargement and pulmonary hypertension was greater in the surgical group than in the non-surgical group. Recurrent rheumatic fever (hazard ratio 7.9, 95% CI 1.9–33.1), tricuspid regurgitation (TR) gradient ≥ 42 mmHg (HR 6.3, 95%CI 1.1–38.7) and left ventricular end-diastolic dimension (LVEDD) ≥6 cm (HR 8.7, 95% CI 2.1–35.9) were predictors of valve surgery (multivariable Cox proportional regression analysis). Clinical carditis was the most common presenting symptom in patients with ARF and RRF. The majority of patients responded positively to prednisolone. These findings highlight the predictors of valve surgery following ARF, including RRF, TR gradient ≥ 42 mmHg and LVEDD ≥ 6 cm. Abbreviations: ARF: acute rheumatic fever; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; GAS: group A beta-haemolytic Streptococcus; HF: heart failure; HR: hazard ratio; LVEDD: left ventricular end-diastolic dimension; MR: mitral regurgitation; RHD: rheumatic heart disease; RRF: recurrent rheumatic fever; TR: tricuspid regurgitation. [ABSTRACT FROM AUTHOR]

Objectives: This observational case-control study aimed to assess the safety and efficacy of oral Naproxen as an alternative to oral Aspirin/acetylsalicylic acid (ASA) for treating acute rheumatic fever (ARF). Methodology: Patients meeting the revised Jones criteria 2015 for ARF were enrolled from November 2018 to May 2019 at a single tertiary care Children's Hospita l. They were divided into two groups: Group-A receiving ASA (control) and Group-B receiving Naproxen (case). Primary outcome measures included the number of days until complete resolution of arthralgia or arthritis, while secondary outcome measures included resolution of fever and normalization of erythrocyte sedimentation rate (ESR). Results: Sixty-four consecutive patients with ARF were enrolled, with 32 in each group, matched for age and gender. The majority (80%) had recurrent ARF. Median age at presentation was similar in both groups. ESR levels did not differ between the groups at admission or at the end of treatment. Median time for resolution of fever was 9(6-11) days in Group-A and 7.5(5-10) days in Group-B. Resolution time for arthritis was similar in both groups, with a median of 3(2-4) days. Gastric pain and vomiting were significantly lower in Group-B than in Group-A. Overall response rates were comparable between the groups. Conclusion: Naproxen demonstrates equal effectiveness, safety, and better tolerance compared to Aspirin in treating ARF patients. [ABSTRACT FROM AUTHOR]

Background: In Iran, there is a lack of information and studies on acute rheumatic fever (ARF), a global health issue. The limited understanding of ARF's prevalence and primary clinical symptoms has led to confusion. This research investigates the characteristics of children aged 3-17 years who experience ARF with monoarthritis as their initial symptom. Methods: A retrospective evaluation of medical records of children diagnosed with ARF was conducted. The study aimed to determine the prevalence of monoarthritis as the first manifestation of ARF and its association with age, gender, family history, and cardiac involvement. Categorical variables were analyzed using the chi-square test with a significance level of < 0.05 and a confidence interval of 95%, using SPSS software (Version 23). Results: The study included 62 patients with ARF, comprising 41 (66.1%) boys with an average age of 8.48±3.27 years. Among these patients, 12 exhibited cardiac involvement according to the revised Jones criteria, with 5 clinical carditis and 7 cases of subclinical carditis. Monoarthritis was the initial symptom in seven patients (11.29%); five (71.4%) also had carditis. There was a significant association (p<0.001) between monoarthritis and carditis. Conclusion: The study concludes that monoarthritis may be an early sign of ARF in children and correlates significantly with cardiac involvement. However, more extensive research with more significant participant numbers is necessary to understand ARF in Iran comprehensively. A thorough cardiac examination is also crucial for patients with ARF and monoarthritis. [ABSTRACT FROM AUTHOR]

Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) are autoimmune conditions triggered by Group A Streptococcus skin or throat infections. If ARF/RHD is undetected, misdiagnosed or antibiotic treatment is not provided early, patients may develop cardiac failure, leading to premature death. Although it is an easily preventable disease, ARF/RHD remains the most significant cause of heart disease-associated deaths in people under 25 years old, both in low- and middle-income countries and among First Nations in high-income countries. Up to 30% of the patients with ARF/RHD present with a neurobehavioral condition – Sydenham's chorea (SC). The clinical course of SC is mostly self-limiting and is characterized by the onset of involuntary choreiform movements and neuropsychiatric features such as obsessive-compulsive disorder, tics, depression and anxiety, psychosis, and attention-deficit hyperactivity disorder. While the precise mechanism as to why only a proportion of patients with ARF/RHD develop SC remains unknown, an impaired blood–brain barrier is considered to play a central role in its development. The most well-characterized neurobehavioral outcome is stroke which may occur in isolation or as part of systemic thromboembolism. Both infective endocarditis and mitral valve disease with or without aortic valve disease increase the embolic and ischemic stroke risk. ARF/RHD is known to significantly impact the quality of life with neuropsychiatric consequences. Another neurobehavioral syndrome which occurs in the absence of ARF/RHD is "pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections" (PANDAS). PANDAS has been categorized as a subset of pediatric acute-onset neuropsychiatric syndromes. However, establishing a diagnosis of PANDAS has been challenging. In this review, we discuss the current status of our understanding regarding the different manifestations of poststreptococcal neurobehavioral changes. Particular attention is given to ARF/RHD-associated SC, and we highlight the areas for further research to understand the association between poststreptococcal sequelae and neurobehavioral abnormalities. [ABSTRACT FROM AUTHOR]

Acute rheumatic fever (ARF) is an autoimmune, multiorgan inflammatory disease that occurs in genetically susceptible individuals after group A-hemolytic streptococcal infection. Carditis and polyarthritis are the most common symptoms of ARF. Repeated streptococcal infections can cause recurrent valvulitis and valvular heart disease. Prolonged atrioventricular conduction (first-degree heart block) is a well-known symptom of ARF, occurring in one-fifth to three-fifths of patients. This condition is a minor criterion for diagnosing ARF based on Jones's criteria. Advanced conduction delay, such as a second-degree or a complete atrioventricular (AV) block, is a rare condition of ARF that can occur before symptoms like carditis, polyarthritis, and Sydenham chorea appear. We present a case of ARF in an adolescent with complete AV block.

Background: In Iran, there is a lack of information and studies on acute rheumatic fever (ARF), a global health issue. The limited understanding of ARF's prevalence and primary clinical symptoms has led to confusion. This research investigates the characteristics of children aged 3-17 years who experience ARF with monoarthritis as their initial symptom. Methods: A retrospective evaluation of medical records of children diagnosed with ARF was conducted. The study aimed to determine the prevalence of monoarthritis as the first manifestation of ARF and its association with age, gender, family history, and cardiac involvement. Categorical variables were analyzed using the chi-square test with a significance level of < 0.05 and a confidence interval of 95%, using SPSS software (Version 23). Results: The study included 62 patients with ARF, comprising 41 (66.1%) boys with an average age of 8.48±3.27 years. Among these patients, 12 exhibited cardiac involvement according to the revised Jones criteria, with 5 clinical carditis and 7 cases of subclinical carditis. Monoarthritis was the initial symptom in seven patients (11.29%); five (71.4%) also had carditis. There was a significant association (p

Abstract 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.

Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) are autoimmune conditions triggered by Group A Streptococcus skin or throat infections. If ARF/RHD is undetected, misdiagnosed or antibiotic treatment is not provided early, patients may develop cardiac failure, leading to premature death. Although it is an easily preventable disease, ARF/RHD remains the most significant cause of heart disease-associated deaths in people under 25 years old, both in low- and middle-income countries and among First Nations in high-income countries. Up to 30% of the patients with ARF/RHD present with a neurobehavioral condition – Sydenham's chorea (SC). The clinical course of SC is mostly self-limiting and is characterized by the onset of involuntary choreiform movements and neuropsychiatric features such as obsessive-compulsive disorder, tics, depression and anxiety, psychosis, and attention-deficit hyperactivity disorder. While the precise mechanism as to why only a proportion of patients with ARF/RHD develop SC remains unknown, an impaired blood–brain barrier is considered to play a central role in its development. The most well-characterized neurobehavioral outcome is stroke which may occur in isolation or as part of systemic thromboembolism. Both infective endocarditis and mitral valve disease with or without aortic valve disease increase the embolic and ischemic stroke risk. ARF/RHD is known to significantly impact the quality of life with neuropsychiatric consequences. Another neurobehavioral syndrome which occurs in the absence of ARF/RHD is "pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections" (PANDAS). PANDAS has been categorized as a subset of pediatric acute-onset neuropsychiatric syndromes. However, establishing a diagnosis of PANDAS has been challenging. In this review, we discuss the current status of our understanding regarding the different manifestations of poststreptococcal neurobehavioral changes. Particular attention is given to ARF/RHD-associated SC, and we highlight the areas for further research to understand the association between poststreptococcal sequelae and neurobehavioral abnormalities.

Objectives: The objectives of this study were to determine the colonization of the throat and skin among patients of rheumatic heart disease (RHD) or acute rheumatic fever (ARF) and to elucidate the different Lancefield serotypes of streptococci (Group A, Group C, and Group G) among them. Methods: The prospective observational surveillance study was conducted from November 2016 to March 2018 among 161 enrolled family members belonging to 30 families with one of their patients being diagnosed with RHD/ARF. The outcome measures were period prevalence of serotypes of streptococci, streptococcal colonization of throat and skin lesions, and association of age and antibody titers with RHD/ARF. Results: Out of 30 families enrolled in our study, positive throat (n = 18)/skin (n = 1) cultures were found in 7 families (n = 19). The cultures were positive only for Group A streptococci (GAS), while none of the samples showed positivity for Group C or G serotypes. Overall period prevalence for our study period was 3.2%. Among the 18 members positive for GAS throat culture, 13 (72.2%) belonged to the age group 0.05). Conclusion: In conclusion, we found only GAS colonization in significantly higher proportion among the family members of cases of ARF in comparison to RHD. Rather there was no case of Group C and G streptococci colonization in cases or family members of RHD/ARF.

Abstract Background Rheumatic fever is a long-term inflammatory disease that can happen if group A beta-hemolytic streptococci bacteria are not treated well enough. Rheumatic fever is recognized globally as the leading cause of heart disease in the pediatric population. This disease destroys the heart muscle, progressively deteriorating its structure and impairing the function of its valves over time. Aim The aim of this study is to determine the role of serum tenascin-C in the diagnosis of acute rheumatic fever and chronic rheumatic heart disease. Methods This case–control study involved a group of 60 Egyptian children. Among them, 20 were diagnosed with acute rheumatic fever, identified using the updated Jones criteria from 2015. Another 20 children, who were suffering from chronic rheumatic heart disease, were also act as a part of the study. The remaining 20 participants, healthy children carefully matched in age and sex, served as the control group. Results Serum tenascin-C level was significantly increased in acute rheumatic fever (ARF) and highly significantly increased in chronic rheumatic heart disease (CRHD) groups when compared with control group (P = 0.04, 0.01), respectively. There were highly significant difference between and within the studied groups regarding the mean of serum tenascin-C. Serum tenascin-C mean of ARF, CRHD, and control was 4.82 ± 18.7, 5.46 ± 1.6, and 3.78 ± 2.4, respectively, P = 0.02. Level of serum tenascin-C was lower in cases with severe mitral valve insufficiency. No significant link was found between the level of serum tenascin-C and C-reactive protein (CRP), ESR, and ASO titer, with a P-value greater than 0.5. ROC curve for serum tenascin-C in ARF patients was area under the curve = 0.682 (P = 0.05) with optimal serum tenascin-C cut-off point (> 3.76 ng/ml); ROC curve for serum tenascin-C in CRHD patients was AUC = 0.73 (P = 0.01) with cut-off point level (73.76 ng/ml). Conclusion Patients with ARF and CRHD have increased level of serum tenascin-C. Serum tenancin-C is superior in the diagnosis of ARF in comparison to CRP, ESR, and ASOT. Tenascin-C level can be used as a diagnostic marker for ARF and CRHD.

Background Rheumatic heart disease (RHD) is an autoimmune heart disease following unresolved or untreated Acute Rheumatic Fever (ARF), which results in irreversible valve damage and heart failure. Strategies for managing RHD could be planned to understand the disease determinants in local settings. Objective To explore the determinants of RHD among the family with an RHD case. Methods The study was a qualitative design using a grounded theory approach after in-depth interviews with respondents from families with RHD patients. Analysis was conducted after the scripts were finalized. Initial, intermediate, and advanced codings were performed. Ten respondents agreed to participate and completed the qualitative data collection process. Results The analysis yielded two theme categories of what could have led to RHD among respondents due to internal and external causes. Internal causes were considered factors that could be managed or manipulated to improve an individual and family’s capacity. In contrast, external causes were considered factors that could not be managed or manipulated to improve an individual’s capacity or family. Therefore, these factors were considered beyond their control. Conclusion This study explored RHD determinants according to the patients’ and their families’ perspectives. A holistic approach can be applied to managing RHD by considering these factors.

IntroductionGroup A streptococcus (GAS) infections, such as pharyngitis and impetigo, can lead to rheumatic fever and rheumatic heart disease (RHD). Australian Aboriginal and Torres Strait Islander populations experience high rates of RHD and GAS skin infection, yet rates of GAS pharyngitis are unclear. Anecdotally, clinical presentations of pharyngitis, including tonsillar hypertrophy and sore throat, are uncommon. This study aimed to develop a standardised set of tonsil photographs and determine tonsil size distribution from an urban paediatric population.MethodsA prospective cohort of children aged 3–15 years were recruited at the public events “Discover Day” and “Telethon Weekend” (October 2017) in Perth, Western Australia, Australia. Tonsil photographs, symptomatology, and GAS rapid antigen detection tests (RADT) were collected. Tonsil size was graded from the photographs using the Brodsky Grading Scale of tonsillar hypertrophy (Brodsky) by two independent clinicians, and inter-rater reliability calculated. Pharyngitis symptoms and GAS RADT were correlated, and immediate results provided.ResultsFour hundred and twenty-six healthy children participated in the study over three days. The median age was seven years [interquartile range (IQR) 5.9–9.7 years]. Tonsil photographs were collected for 92% of participants, of which 62% were rated as good-quality photographs and 79% were deemed of adequate quality for assessment by both clinicians. When scored by two independent clinicians, 57% received the same grade. Average Brodsky grades (between clinicians) were 11%, 35%, 28%, 22% and 5% of grades 0,1,2,3 and 4, respectively. There was moderate agreement in grading using photographs, and minimal to weak agreement for signs of infection. Of 394 participants, 8% reported a sore throat. Of 334 GAS RADT performed,

The burden of group A streptococcus (GAS) infection and its rheumatic sequelae remains dramatically high, especially in low-income countries. Recently, an increased number of Acute Rheumatic Fever (ARF) cases was documented in many regions of Italy. The diagnosis of rheumatic sequelae relies on clinical signs and on the evaluation of the Antistreptolysin O titre (ASO), whose variations are globally reported. To re-examine the standard reference value of ASO titre, by measuring either its upper limit of normal (ULN) in a population of healthy children (HC) or comparing these values with streptococcal antibodies registered in a cohort of patients affected by the rheumatic sequelae of GAS infection. We performed a multicenter retrospective study. We enrolled 125 HC, aged 2–17 years, and a total of 181 patients affected by ARF, acute streptococcal pharyngitis, post-streptococcal arthritis, Henoch-Schönlein purpura and erythema nodosum, divided into four groups. The levels of ASO and anti-deoxyribonuclease B (anti-DNase B) titres were analyzed and compared among the various groups. Moreover, the 80th percentile value was calculated and established as the ULN for ASO titre in HC group. The ULN for ASO titre in overall HC group was 515 IU/mL, resulting in higher than used in the routine investigation. The ASO titre was significantly higher in patients with rheumatic sequelae compared with HC group, with a peak in the age between 5 and 15 years. Conclusion: Our study established a new ULN normal value of streptococcal serology in a childhood and adolescent population of Italy, suggesting the need to extend this revaluation to the critical areas, in order to avoid underestimating ARF diagnosis. The correct interpretation of ASO and anti-DNase B values in the context of rheumatic diseases has been discussed. What is Known: • The global burden of disease caused by group A streptococcus is not known and remains an important cause of morbidity and mortality. Acute rheumatic fever continues to be a serious worldwide public health problem and a recent recurrence of group A streptococcus infection cases is observed. • The streptococcal sequelae requires evidence of preceding streptococcal infection, commonly elevated streptococcal antibody titre, but the upper limit for these titres varies considerably based on age group, region, and origin. What is New: • This study provides population-specific values for streptococcal antibody titres in Italy. • Interpret the results of group A streptococcal antibody tests within the clinical context. [ABSTRACT FROM AUTHOR]

Inclusion of echocardiography as diagnostic tool and polyarthralgia and monoarthritis as major criteria for high-risk populations in the Revised Jones Criteria 2015 is likely to surface substantial variability in clinical manifestations among various populations. This study aimed to compare clinical profile of patients presenting with first and recurrent episodes of acute rheumatic fever (ARF) using most recent criteria. 130 consecutive patients with ARF were included in the study from August 2019 to March 2022. World Heart Federation standardized echocardiographic criteria were used for cardiac evaluation. The socio-demographic variables, clinical details and relevant investigations were recorded. Median age was 13(6–26) years. Male to female ratio was 1.6:1. Majority was of low socioeconomic status (90%) and with > 5 family members in a house (83.8%). 27 patients (20.8%) were with ARF while 103 (79.2%) with recurrent ARF. Carditis was the most common presenting feature (n = 122, 93.8%), followed by polyarthralgia (n = 46, 35.4%), polyarthritis (n = 32, 24.6%), subcutaneous nodules (n = 10, 7.7%), monoarthritis (n = 10, 7.7%), and chorea (n = 5, 3.8%). Monoarthralgia was more common in ARF than recurrence (29.4% vs. 3.2%, p = 0.004). Carditis (97.1% vs. 81.5%, p = 0.01) and congestive cardiac failure (18.5% vs. 5.9%, p = 0.001) were more common in recurrent ARF than ARF. Diagnostic categorization of Jones criteria for different populations has highlighted important variability in clinical presentation of ARF. Monoarthralgia is common in first episode of ARF. Carditis is the most common feature in recurrent ARF. Polyarthralgia is seen with higher frequency that polyarthritis. Subcutaneous nodules seem to be more common in our population. [ABSTRACT FROM AUTHOR]

Background In patients without ethnicity risk factors for acute rheumatic fever (ARF), our local guidelines recommend limiting antibiotic use following a positive throat swab culture (TSC). If symptoms are severe, a 5–7 day course is recommended. Despite this, most local patients with a positive TSC for group A Streptococcus (GAS) or Streptococcus dysgalactiae subsp. equisimilis (SDSE) were being prescribed 10 days of antibiotics. In response, we added comments to positive TSC reports recommending shorter treatment durations in those without ARF risk factors. No other antimicrobial stewardship initiatives were implemented. Objectives To assess the effect of these comments on antibiotic course duration after positive TSC. Methods All community TSC results from 1 October 2021 to 31 March 2023 (1 year pre- to 6 months post-change) were matched to antibiotic dispensing data. Patients who had been empirically dispensed an antibiotic prior to the culture report were excluded. The outcome of interest was the antibiotic duration dispensed in the 5 day period after the TSC report. Results Following introduction of the comments, median course duration reduced from 10 (IQR 5–10) to 7 days (IQR 0–10; P < 0.01) and from 7 (IQR 0–10) to 0 days (IQR 0–5; P < 0.01) following GAS- and SDSE-positive TSC, respectively, in those without ARF risk factors. The percentage of people receiving 10 days of antibiotics decreased from 63.0% to 37.0% (P < 0.01) and 41.2% to 14.6% (P < 0.01) for GAS and SDSE, respectively. Conclusions The introduction of comments providing direct prescribing advice to requestors appears to have been highly effective at improving guideline-compliant prescribing following positive TSC report. [ABSTRACT FROM AUTHOR]

The spondyloarthropathies are a group of conditions characterised by spinal joint pain and have related clinical, epidemiological and genetic-related features. Ankylosing spondylitis, reactive arthritis, the spinal form of psoriatic arthritis and Crohn's and colitis enteropathic arthritis are the major clinical entities of the spondyloarthropathies, and principally occur in HLA-B27 positive individuals. Ankylosing spondylitis is much more common in males than females. Patients are usually seronegative for rheumatoid factor, and extra-articular features including iridocyclitis, mucous membrane and skin lesions: aortitis, may occur in some patients. The reactive arthritis form classically occurs following an infection of the gastrointestinal or genitourinary tract. The Crohn's and colitis enteropathic arthritis forms often have an associated large joint asymmetrical arthritis. Also discussed are acute rheumatic fever and Lyme disease which are conditions where the individual develops arthritis after an infection. [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]

The incidence of acute rheumatic fever (ARF), which most commonly affects children aged 5-15 years after group A Streptococcus (GAS) infection, ranges from 8 to 51 per 100 000 people worldwide. Rheumatic heart disease (RHD), which occurs when patients with ARF are inappropriately treated or not given regular prophylaxis, is the most common cause of non-congenital heart disease in children and young adults in low-income countries. Timely treatment of GAS infection can prevent ARF, and penicillin prophylaxis can prevent recurrence of ARF. Secondary prophylaxis with benzathine penicillin G has been shown to decrease the incidence of RHD and is a key aspect of RHD control. The most important factor determining the prognosis of RHD is the severity of cardiac involvement. Although approximately 70% of patients with carditis in the acute phase of the disease recover without sequelae, carditis is important because it is the only complication of ARF that causes sequelae. One-third of patients with ARF are asymptomatic. Patients with mild symptoms of recurrent ARF and silent RHD will develop severe morbidities within 5-10 years if they do not receive secondary preventive treatments. A new screening program should be established to prevent cardiac morbidities of ARF in moderate- and highrisk populations. In the present study, we examined the applicability of echocardiographic screening programs for RHD. [ABSTRACT FROM AUTHOR]

Rheumatic heart disease (RHD) is a preventable heart disease that remains endemic in developing countries. More than 30 million people in the world suffer from RHD, of which approximately 300,000 die every year, despite the fact that this disease is preventable and treatable. After a period of relative neglect of rheumatic heart diseases due to a decrease in the incidence in developed countries, interest in this problem has increased again over the past decade, due, apparent­ly, to an underestimation of its true prevalence due to the subclinical course of carditis. Research over the past two decades has demonstrated the advantage of diagnosing RHD with echocardiographic screening based on World Heart Federation echocardiographic criteria, which is 10 times greater than the clinical auscultatory picture only and it allowsearly detection of it in patients, while prevention is to be more likely to be effective. Although understanding of the pathogenesis of the disease has advanced in recent years, key issues remain unresolved. Preventing or providing early treatment for streptococcal infections is the most important step in reducing the burden of this disease. The manage­ment of women with rheumatic heart disease before, during and after pregnancy remains a serious task requiring the efforts of a multidisciplinary team. In 2015, a civil society movement was launched aimed at raising awareness and supporting countries seeking to solve the RHD problem. In May 2018, the World Health Organization adopted a resolu­tion aimed at intensifying global and national efforts to prevent and combat acute rheumatic fever/RHD. Ultimately, a combination of treatment options, research and advocacy based on existing knowledge and science provides the best opportunity to cope with the burden of rheumatic heart disease. The article summarizes the latest achievements in the science of RHD and presents priorities for current actions and future research.

Etedal Ahmed A Ibrahim,1,2 Rogia Hussein Mohamed,3 Khabab Abbasher Hussien Mohamed Ahmed,4 Malaz Tarig AbdAlla Mohamed,4 Mohammed Mahmmoud Fadelallah Eljack5 1Department of Medicine, Al-Neelain University, Khartoum, Sudan; 2Department of Neurology, The National Centre for Neurological Sciences, Khartoum, Sudan; 3Department of Medicine, Latifa Hospital for Women and Children, Dubai, United Arab Emirates; 4Department of Medicine, University of Khartoum, Khartoum, Sudan; 5Department of Community Medicine, University of Bakht Alruda, Ad Duwaym, White Nile State, SudanCorrespondence: Mohammed Mahmmoud Fadelallah Eljack, Department of Community Medicine, University of Bakht Alruda, Ad Duwaym, White Nile State, Sudan, Tel +249964656914, Email m.mahmmoud96@gmail.comIntroduction: Sydenham’s chorea (SC) is the most common form of acquired chorea in childhood, it is considered a neurological complication of streptococcal pharyngitis. In this study, we aimed to determine the clinical pattern, association of Sydenham’s chorea with other manifestations of acute rheumatic fever, and the laboratory findings of Sydenham’s chorea among Sudanese patients.Methods: A prospective cross-sectional study involving fifty patients of various ages diagnosed with Sydenham’s chorea and followed up at The National Center for Neurological Sciences from January 2017 to November 2019. Data were obtained after patients’ consent through personal interviews or personal review of patients’ records via a structured questionnaire composed of demographic data, symptoms, co-morbid illness, risk factors, physical examination, and related investigations.Results: About 50 patient was enrolled in this study with a median age of 13.7 years. Females were (n=35) (70%) and (30%) (n=15) s were males. Generalized chorea was seen in 33 (66%) and hemichorea in 17 (34%) patients. Weakness (38%) and hypotonia (46%) were common, such as behavior change (44%), dysarthria (70%), gait change (18%), and deterioration of handwriting (12%). Arthritis occurred in (36%), carditis in 30 (60%), both arthritis and carditis in 18 (36%), and pure chorea in 14 (28%). Erythema marginatum and subcutaneous nodules were not observed in our patients. Only 13 patients (26%) gave a history of pharyngitis.Conclusion: Sydenham chorea is more common in young female Sudanese, with a familial predominance and a tendency towards mitral valve disease. All pediatric Patients with chorea should be screened for Sydenham’s chorea.Keywords: Sydenham, chorea, acute rheumatic fever, presentations, psychiatric

Introduction: Rheumatic fever and RHD constitutes an important public health problem in India. The relatively low attack rate of RF, the high concordance rate for RF in monozygotic twins (19%) compared to dizygotic twins (2.5%), and the high familial incidence of RF suggest the involvement of host genetic factors in susceptibility to RF with consequential progression to RHD. Objective: To study the association of HLA CLASS II DR/DQ alleles in children and adolescents with RHD from a tertiary care center in North India. Methods: 30 RHD patients and 30 age and sex-matched controls were included in our study and blood samples for HLA typing were processed through LAB Type™ reverse SSO DNA typing method. The assignment of the HLA typing was based on a comparison with already published HLA gene sequences. Results: The mean age of RHD patients and matched control groups were 12.97 ± 2.95 and 11.93 ± 3.23, respectively. In the cases and control group, males accounted for 63.3% and 50% of the patients respectively. A significant difference was found between the cases and controls for HLA DR∗ 15 (p-value 0.002), HLA DR∗ B4 (p-value 0.045), HLA DR∗ B5 (p-value 0.017), and HLA DQB1∗ 02 (p-value 0.005). Conclusion: Our study suggests that HLA class II haplotypes may provide insight into the molecular mechanism of RHD and be a useful tool in predicting the clinical outcome in RF patients, thereby affording new means of intervention or vaccine design. Larger studies are needed to address this in our population.

Background: Ataxia-telangiectasia is a multi-organ disease. It is due to a mutation of the Exon No. 5 ataxia telangiectasia mutated gene (c.381delA: p.v128fls). Complications including recurrent infections, progressive cerebellar ataxia, and varying degrees of humoral and cellular immunodeficiency arise. Case Presentation: We report a 7-year-old girl patient with A-T who developed acute rheumatic fever. Conclusion: Rheumatoid disorders and or infectious diseases such as acute rheumatic fever could be observed in A-T patient.

Background: Rheumatic heart disease occurs as a sequel to acute rheumatic fever. Many cases of acute rheumatic fever, however, go undiagnosed as many patients present with otherwise common symptoms and in the absence of overt carditis may go undetected. Most cases present late following the development of valvular damage. A high index of suspicion is key in managing this disease. Aim: To determine the Presentation and outcome of patients managed for Rheumatic fever and Rheumatic heart disease at Ahmadu Bello University Teaching Hospital Zaria. Methodology: A retrospective study was done using information collected from patient records from the Paediatric cardiology ward and clinic of Ahmadu Bello University Teaching Hospital Zaria over 4 years from March 2015 to March 2019. Results: Eight per cent of the total patients seen in the cardiopulmonary clinic had rheumatic heart disease. Fifty-one patients were diagnosed with rheumatic heart disease, forty-five patients presented with established chronic rheumatic valvular involvement and six had acute rheumatic fever. The mean age (±SD) at presentation was 10.7 ± 3.2 years. There was a slight female preponderance M: F 1:1.3 and most patients (88 percent) presented late (already having valvular disease). Most patients had mixed mitral valve disease. The clinic dropout rate was 39 percent while the mortality rate was 25 percent. All patients received medical management, the mortality rate was 25 % (13 patients) while only 3.9 per cent received successful surgical management. Conclusion: Rheumatic heart disease is the commonest preventable and curable type of acquired heart disease, yet the outcome of care provided in our settings remains poor. There is a need to increase awareness, diagnosis and available treatment of rheumatic heart disease.

Background Rheumatic heart disease (RHD) is a devastating yet preventable condition that disproportionately affects low‐middle–income countries and indigenous populations in some high‐income countries. Various preventive interventions have been implemented across the globe, but evidence for the effectiveness of these measures in reducing the incidence or prevalence of acute rheumatic fever and RHD is scattered. This systematic review aims to assess the effectiveness of preventive interventions and identify the strategies used to reduce the burden of RHD. Methods and Results A comprehensive search was conducted to identify relevant studies on RHD prevention interventions including interventions for primordial, primary, and secondary prevention. Effectiveness measures for the interventions were gathered when available. The findings indicate that school‐based primary prevention services targeting the early detection and treatment of Group A Streptococcus pharyngitis infection with penicillin have the potential to reduce the incidence of Group A Streptococcus pharyngitis and acute rheumatic fever. Community‐based programs using various prevention strategies also reduced the burden of RHD. However, there is limited evidence from low‐middle–income countries and a lack of rigorous evaluations reporting the true impact of the interventions. Narrative synthesis was performed, and the methodological quality appraisal was done using the Joanna Briggs Institute critical appraisal tools. Conclusions This systematic review underscores the importance of various preventive interventions in reducing the incidence and burden of Group A Streptococcus pharyngitis, acute rheumatic fever, and RHD. Rigorous evaluations and comprehensive analyses of interventions are necessary for guiding effective strategies and informing public health policies to prevent and reduce the burden of these diseases in diverse populations. Registration URL: https://www.crd.york.ac.uk/prospero/; Unique identifier: CRD42020170503.

Background: Rheumatic fever is a long-term inflammatory disease that can happen if group A beta-hemolytic streptococci bacteria are not treated well enough. Rheumatic fever is recognized globally as the leading cause of heart disease in the pediatric population. This disease destroys the heart muscle, progressively deteriorating its structure and impairing the function of its valves over time. Aim: The aim of this study is to determine the role of serum tenascin-C in the diagnosis of acute rheumatic fever and chronic rheumatic heart disease. Methods: This case–control study involved a group of 60 Egyptian children. Among them, 20 were diagnosed with acute rheumatic fever, identified using the updated Jones criteria from 2015. Another 20 children, who were suffering from chronic rheumatic heart disease, were also act as a part of the study. The remaining 20 participants, healthy children carefully matched in age and sex, served as the control group. Results: Serum tenascin-C level was significantly increased in acute rheumatic fever (ARF) and highly significantly increased in chronic rheumatic heart disease (CRHD) groups when compared with control group (P = 0.04, 0.01), respectively. There were highly significant difference between and within the studied groups regarding the mean of serum tenascin-C. Serum tenascin-C mean of ARF, CRHD, and control was 4.82 ± 18.7, 5.46 ± 1.6, and 3.78 ± 2.4, respectively, P = 0.02. Level of serum tenascin-C was lower in cases with severe mitral valve insufficiency. No significant link was found between the level of serum tenascin-C and C-reactive protein (CRP), ESR, and ASO titer, with a P-value greater than 0.5. ROC curve for serum tenascin-C in ARF patients was area under the curve = 0.682 (P = 0.05) with optimal serum tenascin-C cut-off point (> 3.76 ng/ml); ROC curve for serum tenascin-C in CRHD patients was AUC = 0.73 (P = 0.01) with cut-off point level (73.76 ng/ml). Conclusion: Patients with ARF and CRHD have increased level of serum tenascin-C. Serum tenancin-C is superior in the diagnosis of ARF in comparison to CRP, ESR, and ASOT. Tenascin-C level can be used as a diagnostic marker for ARF and CRHD. [ABSTRACT FROM AUTHOR]

Introduction: The prevalence of acute rheumatic fever (ARF) and rheumatic heart disease (RHD) among Australia's First Nations populations are some of the highest in the world, accounting for 95% of the 2,244 ARF notifications between 2015 and 2019 in Australia. A key issue in treating ARF is long-term secondary prophylaxis, yet only one in five patients received treatment in 2019. This review identifies barriers to secondary prophylaxis of ARF in Australia's First Nations people. Methods: An integrative review was undertaken utilizing PubMed, CINAHL, ProQuest, and Wiley Online. Joanna Briggs Institute critical appraisal tools were used, followed by thematic analysis. Results: The key themes uncovered included: issues with database and recall systems, patient/family characteristics, service delivery location and site, pain of injection, education (including language barriers), and patient-clinician relationship. Conclusions: A national RHD register, change in operation model, improved pain management, improved education, and need for consistent personnel is suggested. [ABSTRACT FROM AUTHOR]

Background Rheumatic heart disease (RHD) is an autoimmune heart disease following unresolved or untreated acute rheumatic fever (ARF), which results in irreversible valve damage and heart failure. Strategies for managing RHD could be planned to understand the disease determinants in local settings. Objective To explore the determinants of RHD among the family with an RHD case. Methods The study was a qualitative design using a grounded theory approach after in-depth interviews with respondents from families with RHD patients. Analysis was conducted after the scripts were finalized. Initial, intermediate, and advanced codings were performed. Ten respondents agreed to participate and completed the qualitative data collection process. Results The analysis yielded two theme categories of what could have led to RHD among respondents due to internal and external causes. Internal causes were considered factors that could be managed or manipulated to improve an individual and family's capacity. In contrast, external causes were considered factors that could not be managed or manipulated to improve an individual's capacity or family. Therefore, these factors were considered beyond their control. Conclusion This study explored RHD determinants according to the patients' and their families' perspectives. A holistic approach can be applied to managing RHD by considering these factors. [ABSTRACT FROM AUTHOR]

In rheumatology, the problem of infectious pathology is quite acute. This is primarily due to the participation of various infectious agents in the development of immuno-inflammatory rheumatic diseases (IIRD), in which microorganisms play a trigger role, triggering the immunopathological mechanisms of inflammation. Vivid examples of such diseases are acute rheumatic fever and reactive arthritis. The infectious etiology of Lyme disease has been proven. An equally difficult task is the fight against comorbid infection (CI), which often complicates the course of many IIRD due to a violation of the immune status caused by both the background disease and the use of immunosuppressive drugs. The predominance of respiratory tract lesions in the structure of CI in patients with IIRD makes it necessary to use influenza and pneumococcal vaccines in them, since the risk of deaths from these infections among these patients is quite high. During the development of the COVID-19 pandemic, which has become a challenge to all mankind, a large number of new fundamental and medical problems have been revealed concerning the relationship between viral infection and many widespread chronic non-communicable diseases, among which IIRDs occupy an important position. As one of the methods of combating the current COVID-19 pandemic, great hopes are pinned on the widespread use of vaccination. The possibility of using mo noclonal antibodies for pre-exposure prophylaxis of COVID-19, including in patients with IIRD, is discussed.

Poststreptococcal reactive arthritis （PSRA） refers to reactive arthritis after Group A streptococcal （GAS） infection that does not meet the diagnostic criteria for acute rheumatic fever （ARF）. It is rare in clinical practice and prone to misdiagnosis due to the lack of characteristic symptoms and signs in the early stage of PSRA. In this article， an adult male patient with PSRA was reported， showing recurrent swelling and pain on the back of the right foot and the medial malleolus. The onset process of the patient was suspected of gout. He received the treatment of reducing uric acid and relieving pain in a local hospital， which was ineffective. The patient was finally diagnosed with PSRA because of the history of pharyngitis and elevated levels of C-reactive protein and antistreptolysin O （ASO） through detection. He received long-term use of benzathine benzylpenicillin combined with anti-inflammatory and analgesic therapy. Subsequently， the condition was improved and no recurrence occurred during follow-up. The diagnosis and treatment of this case prompt that for patients with recurrent arthritis， the possibility of PSRA should be considered. Extensive attention should be paid to differential diagnosis and treatment between gout and reactive arthritis.