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Chorea is a common movement disorder in children, requiring thorough clinical assessment and appropriate tests for etiological diagnosis. Early identification of treatable conditions can lead to effective treatment, reducing morbidity and improving quality of life. To describe the clinical, demographic, and epidemiological characteristics of children and adolescents diagnosed with chorea. A retrospective cross-sectional study of pediatric patients treated in a fourth-level hospital in Bogotá, Colombia, from January 2008 to January 2022. 81 patients with chorea were found. The most frequent etiologies were rheumatic fever (50.6 %), vascular chorea (29.3 %), and chorea secondary to lupus (11.1 %). Patients with chorea secondary to rheumatic fever (Sydenham's chorea) were older compared to other etiologies, 10.36 ± 3.41 years vs 8.29 ± 5.16, p = 0.037. The presence of another movement disorder or abnormalities during the physical examination suggests a different etiology from rheumatic fever. Moreover, the presence of psychiatric symptoms was similar in all etiologies. However, a correlation between age and psychiatric symptoms was observed with an odds ratio of 1.14 95 % CI 1.02–1.29 per year. Red flags in Sydenham's chorea suggesting an alternate etiology are younger age, the presence of other abnormal movements or other findings in the neurological exam or in the magnetic resonance imaging. No significant statistical differences were found between the etiologies with the presence of neuro-psychiatric manifestations. Nevertheless, these manifestations are very frequent in abnormal movements. This study evidenced the positive correlation between age and the presence of psychiatric symptoms. [ABSTRACT FROM AUTHOR]

We analyzed 3,081 invasive and noninvasive Streptococcus pyogenes cases (January 2005-December 2023) at a tertiary care hospital in southwest Germany. Absolute numbers of case-patients increased each year from 2005 until the COVID-19 pandemic. Odds ratios for invasive streptococcal disease were significantly influenced by year, male sex, and older age. [ABSTRACT FROM AUTHOR]

The article discusses a case of acute rheumatic fever presenting as fulminant myocarditis in a 35-year-old woman. The patient had a history of recurrent pharyngitis and Streptococcus pyogenes bacteremia, leading to severe cardiac complications. The diagnosis was confirmed through various tests, and the patient was treated with antibiotics, anti-inflammatory drugs, and intravenous immunoglobulin. The article highlights the importance of considering acute rheumatic fever in patients with specific symptoms and the need for secondary prophylaxis to prevent recurrence and progression of rheumatic heart disease. [Extracted from the article]

Background: Although there has been a large increase in the number of extensive macular atrophy with pseudodrusen (EMAP) cases, the basic aspects of this disease remain unknown. Brazilian patients have a common past history of rheumatic fever (RF) and/or benzathine penicillin (BP) treatment possibly related to the disease. We analyzed how RF and BP might be correlated with EMAP in Brazilian patients. Design: Observational, retrospective, case-control study. Methods: The databases of three private eye clinics in Brazil were searched for patients with an EMAP-like appearance. Each patient was asked about a previous history of RF and/or long-term use of BP. Patients underwent best-corrected visual acuity (BCVA) measurement, color fundus imaging, fundus autofluorescence (FAF) imaging, optical coherence tomography (OCT) imaging, and electroretinography (ERG). The following characteristics were analyzed: subretinal drusenoid deposits (SDD), pigment mottling, retinal pigment epithelial/basement membrane (RPE/BM) separation, outer retinal or RPE atrophy, and identification of a paving stone-like appearance. The choroidal thickness was measured using enhanced depth imaging OCT. The central atrophic area was measured manually on ultra-wide-field FAF. Results: A total of 154 eyes of 77 patients (women, 66.2%; mean age, 58.6 years) with EMAP were included; 90.9% of patients were diagnosed with RF; 94.8% had been treated with BP and treatment was started at an average age of 7.3 years (mean duration, 11.8 years). The treatment duration was significant for the area of atrophy (P = 0.027) in which each 1-year increase in treatment duration led to an average reduction of 6.91 mm2 in area. The age at diagnosis of RF was significant (P = 0.026) for SDD. The increase of 1 year in the diagnosis of RF (late disease) led to a reduction of 24% in the chance of central SDD being present. On OCT, 65.5% eyes had SDD and more than 70% had a split RPE/BM and outer retinal or RPE atrophy. The choroidal thickness in patients with EMAP was significantly (P < 0.001) thinner than the control group. The ERG was abnormal in all eyes. Conclusion: These findings may suggest a relation between RF and EMAP in Brazilian patients. Patients with EMAP should be questioned about a history of RF. [ABSTRACT FROM AUTHOR]

Paediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal Infection (PANDAS) occurs in the child and adolescent age group. It is characterised by the acute onset of Obsessive-compulsive Disorders (OCD) and/or tic disorders following an infection with Group A Beta-haemolytic Streptococci (GABHS), but it can also present with a variety of unique clinical manifestations. Despite the high prevalence of streptococcal infections in the Indian subcontinent, very few cases have been reported due to a lack of awareness. Hereby, the authors present a case series of four cases (one female and three males) of PANDAS with rare presenting symptoms, including dissociative (conversion) disorder and hoarding disorder, each with evidence of a preceding streptococcal infection. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Concurrent recurrence of acute disseminated encephalomyelitis (ADEM) and poststreptococcal glomerulonephritis (PSGN) in a thalassemia intermedia patient is rare and underscores the complexity of autoimmune disorders. This case emphasizes the importance of considering ADEM in the differential diagnosis of children presenting with PSGN accompanied by neurological symptoms. Post‐streptococcal glomerulonephritis (PSGN) is a common group A streptococcal (GAS) infection sequela. The pathophysiology of PSGN involves immune complex deposition, with type 3 hypersensitivity reaction triggered by GAS. Certain neurological conditions may also arise following a GAS infection, possibly due to molecular mimicry in the brain, a pathophysiology similar to rheumatic fever, another common sequel of GAS infection. We present the case of a child with β‐thalassemia intermedia who exhibited the classic triad (edema, hypertension, hematuria) of PSGN along with neurological manifestations, including a low glasgow coma scale (GCS) score and seizures. Magnetic resonance imaging (MRI) of the brain indicated changes consistent with acute disseminated encephalomyelitis (ADEM). Initially treated with methylprednisolone, the patient eventually received intravenous immunoglobulin (IVIG) due to lack of response. The patient had a good outcome, with complete resolution of all symptoms and no residual neurological deficits. This case underscores the importance of considering ADEM in the differential diagnosis for patients presenting with neurological signs and symptoms following a recent throat infection with GAS. Furthermore, given the increased risk of infection in thalassemia, patients with thalassemia who have a throat infection and neurological symptoms should be evaluated for the possible presence of ADEM. [ABSTRACT FROM AUTHOR]

Adapting indigenous qualitative research methods to an online platform was essential for indigenous qualitative research to continue during the COVID-19 pandemic. Online qualitative data collection is a new method supporting health and other research. During the pandemic, shifting to online qualitative data collection was largely driven by impacts of COVID-19 restrictions where in-person data collection could not be undertaken. Recent research has evaluated online focus groups compared to the in-person focus group approach. However, less is understood about how Māori, Pacific and other Indigenous cultural protocols translate to an online approach. This article is based on a study that undertook an Indigenous approach to online data collection during a period of COVID-19 restrictions in 2022. The study was based in Auckland, Aotearoa New Zealand, focused on gaining Māori, Samoan, Tongan and Cook Island Māori perspectives of streptococcus isolate use for rheumatic fever surveillance and research. In Aotearoa New Zealand, rheumatic fever inequitably affects Māori and Pacific children aged 5–15 years. The project used Kaupapa Māori and Pacific centred research methodologies that included online wānanga and fono methods to collect qualitative data from Māori, Tongan, Samoan and Cook Island Māori participants. This article critically reflects on the strengths and challenges of using these methodologies and Māori and Pacific cultural practices in an online health research. The article demonstrates how Indigenous methodologies can effectively be undertaken in online contexts and still maintain cultural integrity and provide rich qualitative data compared to in-person and western informed online qualitative data collection methods. [ABSTRACT FROM AUTHOR]

Background: Pulmonary hypertension (PH) often complicates mitral stenosis (MS). The prognostic impact of pulmonary vascular resistance (PVR) in MS patients remains unclear. Previous study has demonstrated the prognostic impact of right atrial pressure (RAP) in patients with primary PH. We aim to determine the prognostic impact of PVR and RAP in patients with rheumatic MS undergoing percutaneous mitral balloon valvuloplasty (PMBV). Methods: A total of 58 patients with symptomatic severe rheumatic MS who underwent PMBV between 2016 and 2020 were included. Patients were divided into two groups: PVR ≤ 2WU (N = 26) and PVR > 2WU (N = 32). The composite endpoint included death, reintervention or persistent NYHA functional class III-IV during follow-up. Results: The median age was 50 (42–60) years, with 82.8% being female. Median pulmonary artery systolic pressure (PASP) was 42 (35–50.5) mmHg. Patients with PVR ≤ 2WU had lower PASP on both echocardiogram and catheterization. The PMBV success rate was 75.9%. Multivariate analysis, adjusted for PVR, showed RAP as the only independent predictor of the composite endpoint (HR:1.507, 95% CI:1.015–2.237, p = 0.042). The optimal RAP cutoff was 9.5 mmHg (HR:3.481, 95% CI:1.041–11.641; p = 0.043). Conclusions: RAP was an independent predictor of adverse outcomes in patients with rheumatic MS undergoing PMBV, while PVR did not show prognostic significance. These findings suggest that the prognostic value of PVR may be lower than expected. [ABSTRACT FROM AUTHOR]

Background. Streptococcus pyogenes is the most frequent cause of pharyngitis and skin infections in children and causes immune complications like rheumatic fever and rheumatoid heart disease (RHD), particularly in developing countries like Ethiopia. The aim of this study was to determine the prevalence, antibiotic resistance pattern, and associated factors of Streptococcus pyogenes among pediatric patients suspected of acute pharyngitis in Sidama Region, Southern Ethiopia. Methods. A cross‐sectional study was conducted on 213 acute pharyngitis suspected pediatric patients from April to September 2022 at Hawassa University Compressive Specialized Hospital and Yirgalem Hospital. Sociodemographic and clinical data were collected using a structured questionnaire. A throat swab was cultured to isolate S. pyogenes, and antimicrobial susceptibility testing was done using standard bacteriological techniques. Data were analyzed using SPSS version 25, and P value of <0.05 was considered as statistically significant. Result. Out of 213 throat swabs cultured, 22 (10.3%) with 95% CI (6.6–14.6%) were S. pyogenes positive. All isolates of S. pyogenes were sensitive to penicillin and amoxicillin. In contrast, 8 (36.4%) isolates exhibited resistance to tetracycline, 7 (31.8%) to ceftriaxone, 6 (27.3%) to erythromycin, and 5 (22.7%) isolates showed multidrug resistance. The presence of palatal petechiae (P = 0.037) and tonsillar swelling or exudate (P = 0.007) were significantly associated with S. pyogenes carriage in children suspected of having acute pharyngitis. Conclusion. In this study, the prevalence of S. pyogenes among children suspected with acute pharyngitis was low compared to other studies. The isolates showed a high level of resistance to commonly used antibiotics. Therefore, the treatment of pediatric acute S. pyogenes pharyngitis should depend on an antimicrobial susceptibility test. Furthermore, evaluation of S. pyogenes pediatric acute pharyngitis risk factors and tracking of antibiotic resistance are crucial in the controlling of pediatric acute S. pyogenes pharyngitis. [ABSTRACT FROM AUTHOR]

Rheumatic heart disease (RHD) remains prevalent within First Nations Australian communities. RHD is more common in females and peak prevalence corresponds with childbearing age. Significant valvular disease can complicate pregnancy. Current practice in Northern Australia is to refer pregnant women for echocardiography if there are signs or symptoms of possible cardiac pathology or a history of acute rheumatic fever (ARF) or RHD. It is not currently routine practice to offer echocardiographic screening for all pregnant women at high risk of RHD. This study aimed to assess the current referral practices for echocardiography and disease patterns in pregnant women in the Northern Territory, Australia—a region with a known high prevalence of RHD in the First Nations population. A retrospective analysis of all echocardiography referrals of pregnant women over a 4-year period was performed. Data included indication for echocardiography, clinical history, echocardiographic findings, and location of delivery. Comparisons were made using Fisher's exact and Mann–Whitney U tests. A total of 322 women underwent echocardiography during pregnancy: 195 First Nations and 127 non-Indigenous women (median age, 25 vs 30 years, respectively; p<0.01). Indications for echocardiography differed by ethnicity, with history of ARF or RHD being the most common indication in First Nations women, and incidental murmur the most common in non-Indigenous women. First Nations women were more likely to have abnormal echocardiograms (35.9% vs 11.0% in non-Indigenous women; p<0.01) or a history of ARF or RHD (39.5% vs 0.8%; p<0.01), but less likely to have documented cardiac symptoms as an indication for echocardiography (8.2% vs 20.5%; p<0.01). New cardiac diagnoses were made during pregnancy in 11 (5.6%) First Nations and two (1.6%) non-Indigenous women (p=0.02). Moderate or severe valve lesions were detected in 26 (13.3%) First Nations women (all previously diagnosed), and 11 (5.6%) had previous cardiac surgery. No severe valve lesions were identified in the non-Indigenous group. Interstate transfer to a tertiary centre with valve intervention services was required during pregnancy or the puerperium for 12 (6.2%) First Nations women and no non-Indigenous women. Amongst pregnant women in the Northern Territory who had an indication for echocardiography, First Nations women were more likely to have abnormal echocardiograms. This was mainly due to valvular disease secondary to RHD. Cardiac symptoms were infrequently recorded as an indication for echocardiography in First Nations women, suggesting possible underappreciation of symptoms. Having a low threshold for echocardiographic investigation, including consideration of universal screening during pregnancy, is important in a high RHD-burden setting such as ours. A better understanding of the true prevalence and spectrum of disease severity in this population would enable health services to invest in appropriate resources. [ABSTRACT FROM AUTHOR]

Streptococcal infections may contribute to psoriasis development, and antistreptococcal treatments are considered potential therapies, but their effectiveness remains uncertain due to limited systematic evidence. Our objective was to analyze antistreptococcal therapies' effectiveness in improving psoriasis. We conducted a systematic review following PRISMA guidelines, evaluating antistreptococcal treatment efficacy in psoriasis patients from PubMed, Scopus, and Embase databases until August 14, 2022. Eligible studies included psoriasis patients undergoing antistreptococcal therapy, regardless of demographics or psoriasis type. 50 studies (1778 patients) were analyzed, with penicillins/aminopenicillins as the most studied antibiotics (21 studies), showing mixed outcomes, some reporting significant improvement in guttate psoriasis, while others showed no significant difference. Rifampin demonstrated positive results in most of ten studies, and macrolides showed varying effectiveness in two studies. Tonsillectomy in 14 studies (409 patients) mainly focusing on guttate and chronic plaque psoriasis showed positive outcomes, indicating improved symptoms and quality of life. Limitations include heterogeneous studies, sampling bias, and quality of evidence. This systematic review reveals limited and varied evidence for systemic antibiotic therapy efficacy in psoriasis treatment, while tonsillectomy emerges as a potentially beneficial antistreptococcal option, urging further well-designed, controlled studies with larger sample sizes and standardized protocols for better comparisons. [ABSTRACT FROM AUTHOR]

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: This communication summarizes our single-center experience with the use of anti-IL-1 biologic response modifiers for treating autoimmune and autoinflammatory conditions in children. Methods: We outline our rationale for the off-label use of anakinra and discuss emerging treatment paradigms that necessitate further research and validation. Results: Anakinra has enabled personalized treatment, whether used as a single agent on an as-needed basis, as part of a background treatment regimen, or in combination with colchicine. Our data also highlight the significance of anakinra in treating post-infectious inflammatory diseases, demonstrating its high efficacy in novel applications such as rheumatic fever and post-viral arthritis. Canakinumab, on the other hand, has provided long-term remission. Both medications were well-tolerated, with no serious adverse effects reported. Conclusions: Based on our observations and successful outcomes, we advocate for future collaborative efforts to improve access to anti-IL-1 medications to better manage excessive and harmful inflammation in children. [ABSTRACT FROM AUTHOR]

The article provides a historical overview of rheumatic fever (RF) and rheumatic heart disease (RHD) and their prevalence in low- and middle-income countries (LMICs), with a focus on India. Early detection and treatment of Group A β-hemolytic streptococcus (GAS) infections are crucial in preventing RF. Echocardiography plays a significant role in diagnosing and managing RHD, with updated guidelines published in 2023. RF and RHD continue to be significant causes of morbidity and mortality in LMICs, particularly among children, adolescents, and young adults. The global burden of RHD varies depending on diagnostic criteria, income level, and region. Echocardiography shows promise as a screening tool in resource-limited settings. A vaccine for RHD is being developed, and research is ongoing to improve diagnostic tests and prophylaxis methods. The Streptococcus A Vaccine Consortium is collaborating with the World Health Organization to enhance the development process. [Extracted from the article]

Abstract Background Although there has been a large increase in the number of extensive macular atrophy with pseudodrusen (EMAP) cases, the basic aspects of this disease remain unknown. Brazilian patients have a common past history of rheumatic fever (RF) and/or benzathine penicillin (BP) treatment possibly related to the disease. We analyzed how RF and BP might be correlated with EMAP in Brazilian patients. Design Observational, retrospective, case-control study. Methods The databases of three private eye clinics in Brazil were searched for patients with an EMAP-like appearance. Each patient was asked about a previous history of RF and/or long-term use of BP. Patients underwent best-corrected visual acuity (BCVA) measurement, color fundus imaging, fundus autofluorescence (FAF) imaging, optical coherence tomography (OCT) imaging, and electroretinography (ERG). The following characteristics were analyzed: subretinal drusenoid deposits (SDD), pigment mottling, retinal pigment epithelial/basement membrane (RPE/BM) separation, outer retinal or RPE atrophy, and identification of a paving stone-like appearance. The choroidal thickness was measured using enhanced depth imaging OCT. The central atrophic area was measured manually on ultra-wide-field FAF. Results A total of 154 eyes of 77 patients (women, 66.2%; mean age, 58.6 years) with EMAP were included; 90.9% of patients were diagnosed with RF; 94.8% had been treated with BP and treatment was started at an average age of 7.3 years (mean duration, 11.8 years). The treatment duration was significant for the area of atrophy (P = 0.027) in which each 1-year increase in treatment duration led to an average reduction of 6.91 mm2 in area. The age at diagnosis of RF was significant (P = 0.026) for SDD. The increase of 1 year in the diagnosis of RF (late disease) led to a reduction of 24% in the chance of central SDD being present. On OCT, 65.5% eyes had SDD and more than 70% had a split RPE/BM and outer retinal or RPE atrophy. The choroidal thickness in patients with EMAP was significantly (P

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Background: Although infrequent, Sydenham’s chorea (SC) may occur as a result of injury to the basal ganglia in children with acute rheumatic fever (ARF) secondary to group A Streptococcal infection. Certain hallmarks of SC, such as movement disorders, could be utilized as a predictive marker for carditis. The present study aimed to investigate neurologic and cardiologic symptoms in children with suspected SC after ARF.Methods: All children aged 5-16 who were admitted at Shahid Madani Pediatric Hospital (Tabriz, Iran), with an initial diagnosis of ARF and SC between 2009 and 2022 were included for echocardiographic assessment and prospective follow-up within 6 and 12 months after the start point. The pattern and severity of valvulopathy, as well as the prevalence of Jones criteria for rheumatic fever, were used to assess the effect. The collected data were analyzed using SPSS Statistics software (version 22.0) using Chi square and Fisher’s exact tests. P

Background: In Victoria, Australia, children with Pacific Islander ('Pacific') ethnicities are overrepresented in acute rheumatic fever (ARF) and rheumatic heart disease (RHD). In June 2023, ARF and RHD became notifiable in Victoria. To inform public health and clinical practice, we described young Pacific patients' and their caregivers' understandings and experiences of ARF/RHD, and identified possible ways to improve the delivery of clinical care. Methods: We established a project reference group including local Pacific people to guide this research. Pacific patients who attended an ARF/RHD clinic at The Royal Children's Hospital, Melbourne, were invited to participate, as were their caregivers. A Samoan researcher conducted qualitative 'talanoa' (conversational) interviews with patients and caregivers. A second researcher conducted semi-structured interviews with treating clinicians and other stakeholders. Interview transcripts underwent thematic analysis guided by the Tuilaepa Youth Mentoring Services Pacific Youth Wellbeing Framework. Results: We interviewed 27 participants. This included nine patients and nine caregivers, all of whom were Samoan. These 18 participants expressed a desire to learn more about ARF/RHD and connect with other affected people. While some shared their experiences of having well-liked and trusted healthcare providers, patients often struggled to have two-way clinical conversations. The need to support clinicians working with high-risk populations to improve their awareness of ARF was identified. Receiving treatment on time was a top priority for affected families, despite injection pain, inconvenience and financial costs. The need to support continuity of care for young adult patients was raised by participants. Conclusions: Pacific people living with ARF/RHD and their families require additional support to receive high quality management in Victoria. Introducing a patient register and a specialist RHD nurse would enhance access to treatment, as would removing cost barriers, improving clinical awareness of ARF/RHD and creating Victoria-specific patient resources. Author summary: Acute rheumatic fever (ARF) and its serious complication rheumatic heart disease (RHD) are key markers of social injustice. In Victoria, Australia, ARF and RHD became notifiable in mid-2022. This change was instigated in response to recent work showing an ongoing burden of disease in young Victorians, particularly people of Pacific Islander ('Pacific') ethnicities. Until recently, nothing has been documented of the experiences that patients and their families have had when seeking clinical care for ARF/RHD in Victoria. We undertook this qualitative study to address this knowledge gap. Our study was co-designed by a project reference group which included local Pacific people. We used traditional Pacific 'talanoa' conversational style interviews to report on patients' and their caregivers' experiences of receiving clinical care. Our findings provide critical guidance for best practice public health management in areas such as Victoria with a low but severely ethnically inequitable disease burden. We make recommendations to support clinicians in areas with at-risk populations to enhance their awareness of ARF and RHD, and improve the delivery of clinical care. Our recommendations provide direction for future initiatives to prevent and control these serious health conditions, and better support affected people. [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]

Aim: To describe the clinical profile of acute rheumatic fever (ARF) presentations to paediatric cardiology tertiary services in Western Australia (WA). Methods: A retrospective clinical audit of individuals with confirmed ARF referred to the only paediatric tertiary cardiac service in WA (1 January 1987 to 31 December 2020). Comparisons between inpatient, outpatient, remote and non‐remote groups were assessed. Results: Four hundred seventy‐one episodes of ARF in 457 individuals (235 male; median age = 8 years) met clinical criteria. The majority were Aboriginal and Torres Strait Islander children (91.2%), with 62.1% living in remote areas. The number of ARF and rheumatic heart disease (RHD) diagnoses per year increased from 1987 to 2017 with notable peaks in 2013 and 2017. The average annual incidence of tertiary‐referred ARF in WA of 4–15‐year‐olds from 1987 to 2020 was 4.96 per 100 000. ARF features included carditis (59.9%), chorea (31%), polyarthritis (30%) and polyarthralgia (24.2%). RHD was evident in 61.8% of cases and predominantly manifested as mitral regurgitation (55.7%). Thirty‐four children (7.4%) with severe RHD underwent valvular surgery. 12% had at least one recurrent ARF episode. Remote individuals had more than double the rate of recurrence compared to non‐remote individuals (P = 0.0058). Compared to non‐remote episodes, remote presentations had less polyarthritis (P = 0.0022) but greater proportions of raised ESR (P = 0.01), ASOT titres (P = 0.0073), erythema marginatum (P = 0.0218) and severe RHD (P = 0.0133). Conclusion: The high proportion of Aboriginal and Torres Strait Islander Australians affected by ARF/RHD in WA reflects the significant burden of disease within this population. Children from remote communities were more likely to present with concurrent severe RHD. Our study reinforces the persisting need to improve primary and secondary ARF initiatives in rural and remote communities. [ABSTRACT FROM AUTHOR]

Background: New Zealand is one of the last high‐income countries in the world experiencing significant rates of rheumatic fever. Nurses play a crucial role in rheumatic fever prevention; however, little is understood as to how nurses can best achieve this. Aim: To explore nursing practices that optimise rheumatic fever prevention. Design: An integrative review. Methods: Four electronic databases (CINAHL, SCOPUS, Medline via, and Ovid) were searched for peer‐reviewed empirical articles published from 2013 to 2023. Grey literature (guidelines/reports) was also sourced. Critical appraisal was applied using the Mixed‐Methods Appraisal Tools and the Joanna Briggs Critical Appraisal checklist. Qualitative Research in Psychology, 3(2), 77–101, thematic analysis method was used to generate themes. Results: Seven research articles and three national reports were included. Four themes—in‐depth nursing knowledge and improving prophylaxis adherence, cultural competency, and therapeutic nurse–patient relationships—were found. Conclusion: While nursing knowledge and ways to improve injection adherence are essential, being culturally receptive and developing therapeutic relationships are equally important. Without strong and trusting relationships, it is difficult to deliver care required for prevention success. Implications to care: When working with vulnerable populations it is important to be culturally receptive in all interactions with patients and their families. Impact: New Zealand has high rates of rheumatic fever, especially among vulnerable populations such as Pacific Islanders and Māori. Nurses are often frontline primary care providers who, when skilled with the right tools, can help reduce the prevalence of this disease. Reporting method: The Preferred Reporting Items for Systematic Reviews and Meta‐Analysis flow chart. Patient or public contribution: No Patient or Public Contribution was required for this research. [ABSTRACT FROM AUTHOR]

Background: Paediatric patients with autoimmune rheumatic diseases (pARD) have a dysregulated immune system, so infections present a major threat to them. To prevent severe COVID-19 infections we aimed to vaccinate them as soon as possible. Studies have shown that the BNT162b2 vaccine is safe, effective, and immunogenic, however, in a short observation period, only. Methods: The main objective was to compare the serological response between three groups of pARD: after SARS-CoV-2 infection, after vaccination against COVID-19 with two doses of the BNT162b2 vaccine, and after experiencing both events. Data on demographics, diagnosis, therapy, and serology (anti-SARS-CoV-2 IgG/IgA) were collected from March 2020 to April 2022. For statistical analysis ANOVA, Mann-Whitney U test, Chi-square test and Fisher's exact test were applied. To compare adverse events (AE) after vaccination we included a control group of healthy adolescents. Results: We collected data from 115 pARD; from 92 after infection and 47 after vaccination. Twenty-four were included in both groups. Serological data were available for 47 pARD after infection, 25 after vaccination, and 21 after both events. Serological response was better after vaccination and after both events compared to after infection only. No effect of medication on the antibody levels was noted. The safety profile of the vaccine was good. Systemic AE after the first dose of the vaccine were more common in healthy adolescents compared to pARD. In the observation period of 41.3 weeks, 60% of vaccinated pARD did not experience a symptomatic COVID-19 infection. Conclusions: IgG and IgA anti-SARS-CoV-2 levels were higher after vaccination and after both events compared to after infection only. Six months after vaccination we observed an increase in antibody levels, suggesting that pARD had been exposed to SARS-CoV-2 but remained asymptomatic. Trial registration: The study was approved by the Medical Ethics Committee of the Republic of Slovenia (document number: 0120–485/2021/6). [ABSTRACT FROM AUTHOR]

Key Points: Questions: What is the risk of major clinical outcomes in patients with rheumatic heart disease (RHD) and what are the risk predictors in endemic countries? Findings: In this prospective observational study including 13 696 patients enrolled from 24 low- and middle-income countries, nearly 15% of patients died at 3 years, mostly due to heart failure or sudden death. Corrective valve surgery or valvuloplasty was independently associated with a reduced risk of death. However, although most patients were symptomatic, only about 5% underwent valve surgery in 3 years. Meaning: Improved availability and access to surgical and interventional care for patients with RHD in endemic countries are needed. Importance: Rheumatic heart disease (RHD) remains a public health issue in low- and middle-income countries (LMICs). However, there are few large studies enrolling individuals from multiple endemic countries. Objective: To assess the risk and predictors of major patient-important clinical outcomes in patients with clinical RHD. Design, Setting, and Participants: Multicenter, hospital-based, prospective observational study including 138 sites in 24 RHD-endemic LMICs. Main Outcomes and Measures: The primary outcome was all-cause mortality. Secondary outcomes were cause-specific mortality, heart failure (HF) hospitalization, stroke, recurrent rheumatic fever, and infective endocarditis. This study analyzed event rates by World Bank country income groups and determined the predictors of mortality using multivariable Cox models. Results: Between August 2016 and May 2022, a total of 13 696 patients were enrolled. The mean age was 43.2 years and 72% were women. Data on vital status were available for 12 967 participants (94.7%) at the end of follow-up. Over a median duration of 3.2 years (41 478 patient-years), 1943 patients died (15% overall; 4.7% per patient-year). Most deaths were due to vascular causes (1312 [67.5%]), mainly HF or sudden cardiac death. The number of patients undergoing valve surgery (604 [4.4%]) and HF hospitalization (2% per year) was low. Strokes were infrequent (0.6% per year) and recurrent rheumatic fever was rare. Markers of severe valve disease, such as congestive HF (HR, 1.58 [95% CI, 1.50-1.87]; P <.001), pulmonary hypertension (HR, 1.52 [95% CI, 1.37-1.69]; P <.001), and atrial fibrillation (HR, 1.30 [95% CI, 1.15-1.46]; P <.001) were associated with increased mortality. Treatment with surgery (HR, 0.23 [95% CI, 0.12-0.44]; P <.001) or valvuloplasty (HR, 0.24 [95% CI, 0.06-0.95]; P =.042) were associated with lower mortality. Higher country income level was associated with lower mortality after adjustment for patient-level factors. Conclusions and Relevance: Mortality in RHD is high and is correlated with the severity of valve disease. Valve surgery and valvuloplasty were associated with substantially lower mortality. Study findings suggest a greater need to improve access to surgical and interventional care, in addition to the current approaches focused on antibiotic prophylaxis and anticoagulation. This prospective observational study investigates mortality rates and predictive risk factors for rheumatic heart disease in 24 endemic low- and middle-income countries and evaluates interventions like corrective valve surgery and valvuloplasty. [ABSTRACT FROM AUTHOR]

Background: The World Health Organisation Essential Medicines List (WHO EML) guides National Essential Medicines Lists and Standard Treatment Guidelines for clearly identified disease priorities especially in low- and middle-income countries. This study compares the degree to which the basket of medicines recommended for rheumatic diseases in children and young people in National Essential Medicines Lists of countries in the WHO Africa region, corresponds to the 2021 WHO EML and WHO EML for children, as a proxy of availability. Methods: An online search of the WHO medicines and health technology portal, the Health Ministry websites of the 54 African countries, PUBMED and Google Scholar, with search terms for 'National Essential Medicines List', AND/OR 'standard treatment guidelines' AND/OR 'Lista Nacional de Medicamentos Essenciais' AND/ OR 'Liste Nationale de Medicaments Essentiels' AND Africa AND/OR < Name of African country > was conducted. The number of medicines on the national lists were compared according to a predefined template of medicines; and the percentage similarity calculated. Descriptive statistics were derived using STATA. Results: Forty-seven countries in the WHO Africa region have developed a National Essential Medicines List. Eleven countries do not have any medicines listed for rheumatic diseases. The majority of countries had less than or equal to 50% similarity with the WHO EML for rheumatic disease in children and young people, median 3 medicines (IQR 1— 4). The most common medicines on the national lists from Africa were methotrexate, sulfasalazine and azathioprine, with etanercept available in 6 countries. Seven countries had only one medicine, acetylsalicylic acid listed in the section 'Juvenile Joint diseases'. A multiple linear regression model for the predictors of the number of medicines on the national lists established that 20% of the variability was predicted by health expenditure per capita, socio-demographic index and the availability of rheumatology services (adult and/or paediatric) p = 0.006, with socio-demographic index (p = 0.035, 95% CI 0.64—16.16) and the availability of rheumatology services (p = 0.033, 95% CI 0.13 – 2.90) significant. Conclusion: Four countries (8.5%) in Africa have updated their National Essential Medicines Lists to reflect adequate care for children and young people with rheumatic diseases. Moving forward, efforts should focus on aligning available medicines with the WHO EML, and strengthening healthcare policy for rheumatology and pharmaceutical services, for affordable access to care and medicines. [ABSTRACT FROM AUTHOR]

Background: Nigella sativa L., Anongessius latifolia (Roxb. ex DC.) Wall. ex Euill. and Perr. and shilajit have been used traditionally to treat numerous infectious diseases, including many caused by bacterial pathogens. However, extracts of these traditionally medicines have been poorly studied and are yet to be tested for the ability to inhibit the growth of bacterial triggers of multiple sclerosis and rheumatic fever. Materials and Methods: Antimicrobial activity of selected plant extracts was assessed using disc diffusion and liquid dilution minimum inhibitory concentration (MIC) assays against some bacterial triggers of multiple sclerosis and rheumatic fever. Interactions between the extracts and conventional antibiotics were studied and classified using the sum of the fractional inhibitory concentration (SFIC). The toxicity of the individual samples and the combinations was assessed using the artemia lethality assay (ALA) assay. Results: The methanolic A. latifolia extract displayed notable antibacterial activity against the bacterial triggers of multiple sclerosis (A. baylyi and P. aeruginosa), and rheumatic fever (S. pyogenes). Furthermore, combining the methanolic A. latifolia extract with tetracycline and chloramphenicol resulted in potentiation of the inhibitory activity against P. aeruginosa and S. pyogenes. None of the individual components (nor the combinations) were toxic in the ALA assay. Conclusion: The A. latifolia methanolic displayed clinically relevant antibacterial activity against A. baylyi, P. aeruginosa and S. pyogenes when tested alone. Furthermore, that extract also potentiated the activity of tetracycline and chloramphenicol against some bacteria. The lack of toxicity of the extracts and combinations indicates that these combinations may provide leads in the development of new therapies to prevent and treat the autoimmune diseases multiple sclerosis and rheumatic fever. [ABSTRACT FROM AUTHOR]

Background: Although infrequent, Sydenham's chorea (SC) may occur as a result of injury to the basal ganglia in children with acute rheumatic fever (ARF) secondary to group A Streptococcal infection. Certain hallmarks of SC, such as movement disorders, could be utilized as a predictive marker for carditis. The present study aimed to investigate neurologic and cardiologic symptoms in children with suspected SC after ARF. Methods: All children aged 5-16 who were admitted at Shahid Madani Pediatric Hospital (Tabriz, Iran), with an initial diagnosis of ARF and SC between 2009 and 2022 were included for echocardiographic assessment and prospective follow-up within 6 and 12 months after the start point. The pattern and severity of valvulopathy, as well as the prevalence of Jones criteria for rheumatic fever, were used to assess the effect. The collected data were analyzed using SPSS Statistics software (version 22.0) using Chi square and Fisher's exact tests. P<0.05 was considered statistically significant. Results: The study enrolled 85 children, 36 girls and 49 boys, with a mean age of 9.7±2.7. On the first echocardiography, 42.4% of patients had mitral valve regurgitation (MR), with a predominance of female patients (P=0.04). Of those diagnosed with SC (12 girls and 6 boys), 66.7% showed cardiac involvement, with a higher prevalence of MR in both sexes (P=0.04). The pattern of cardiac involvement after 6 months was significantly different between the groups (P=0.04). However, no such difference was observed during the one-year follow-up (P=0.07). Female sex was found to have a significant relationship with SC localization (P=0.01). Conclusion: In addition to its neurological manifestations, SC can be associated with clinical or subclinical cardiac valve dysfunction that might last for more than a year. In addition to attempting early detection and appropriate management, a precise cardiac and neurologic assessment during admission and follow-up is recommended. A preprint version of this manuscript is available at DOI: 10.21203/rs.3.rs-772662/v1 (https://www.researchsquare.com/ article/rs-772662/v1). [ABSTRACT FROM AUTHOR]

Skin lesions are frequently observed in children with rheumatic diseases, particularly in conditions such as IgA vasculitis (IgAV) and Kawasaki disease (KD). In paediatric vasculitis, the presence of skin lesions serves as an early indicator, emphasising the importance of timely diagnosis to prevent complications, such as cardiac or renal involvement. Conversely, autoinflammatory disorders like juvenile systemic lupus erythematosus (SLE) and juvenile dermatomyositis (DM) may manifest with cutaneous manifestations either at the onset of disease or during its progression. Identifying these skin lesions prior to the appearance of systemic symptoms offers an opportunity for early diagnosis and treatment, which has a positive influence on the outcomes. Additionally, it is noteworthy that specific rheumatological conditions, such as acute rheumatic fever (ARF) or oligoarticular or polyarticular forms of juvenile idiopathic arthritis (JIA), may exhibit occasional, but significant skin involvement, which is strongly correlated with an unfavourable prognosis. The assessment of skin is important in the holist approach to assessing patients for potentially systemic/multisystem disorder and helps distinguish discrete conditions. [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]

"Eliminate rheumatic fever (RF) and minimize the burden of rheumatic heart disease by 2025" is the goal of World Heart Federation (WHF). The most important step to achieve the goal of WHF is the implementation of the prevention and control of RF in India. The program can be implemented with minimal fund allocation from government making use of the existing manpower in the government and private health sector and schools with the concurrence of National Health Mission, Ministry of Health and Family Welfare, Ministry of Public Education and under the guidance of Cardiological Society of India, National Rheumatic Heart Consortium, Rheumatic Heart Club India, Association of Physicians of India, Indian Academy of Pediatrics, and Association of Otolaryngologists of India. By the successful implementation of this program, the children of 5--15 years in India can be protected from RF. India eradicated smallpox in 1980 and Polio 2012. With this program, we can start our efforts to eliminate RF by 2025. [ABSTRACT FROM AUTHOR]

The article discusses the findings of a coroner's inquest into the deaths of three Aboriginal women from rheumatic heart disease (RHD) in the remote town of Doomadgee in Queensland, Australia. The inquest highlights the preventable nature of RHD and the inequitable burden experienced by Aboriginal and Torres Strait Islander peoples. The article presents recommendations for improving health service provision, including cultural safety, community engagement, data collection, and collaboration. It also addresses the issue of institutional racism within the healthcare system and calls for transformative action to achieve health equity for First Nations communities. [Extracted from the article]

Introduction: Childhood health problems can lead to bigger problems in the future if not diagnosed early. Low socioeconomic levels are closely related to the health of children, and more health problems are expected. This study aims to identify the health problems of Syrian refugee and Turkish school-age children and evaluate the common problems to plan health services accordingly. Methods: The study was planned to be conducted at schools in determined districts. Informed consent forms were prepared in Turkish and Arabic and delivered to 14,514 Turkish and Syrian refugee children. The field visits of the study were completed between April-June 2019. A total of 12,603 children--2,101 Turkish and 10,502 Syrian--were examined by trained health personnel and doctors in 31 planned schools in the districts using the designed examination form. Results: 12,603 students underwent health examinations; 7,025 students were found appropriate for further examination and tests, and 3,961 students were referred to hospitals for treatment. The most common health problems were dental issues and upper respiratory tract diseases. Acute rheumatic fever was detected in four children, and mitral valve insufficiency was detected in two children. Discussion and Conclusion: Consequently, our study revealed the need to refer 10,986 of the 12,603 children for further examination, tests, or treatment. The study aimed to determine the health problems of school-age Syrian immigrants and Turkish children and to shed light on the health services to be provided. [ABSTRACT FROM AUTHOR]

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This article provides a comprehensive overview of scarlet fever and invasive streptococcal disease, including their clinical features, diagnostic studies, and treatment options. It also discusses the epidemiology of streptococcal infections, particularly during the COVID-19 pandemic, and emphasizes the importance of accurate diagnosis and appropriate antibiotic prescribing to prevent complications and antibiotic resistance. The text covers various presentations and complications of invasive streptococcal infections, highlighting the changing pattern during the pandemic and the need for healthcare providers to be aware of these potentially deadly infections. Additionally, it provides information on the clinical features, management, and potential complications of different streptococcal infections, as well as the streptococcal carrier state and the development of rheumatic fever/rheumatic heart disease. The document underscores the importance of early recognition, prompt treatment, and antibiotic stewardship in managing these infections, and emphasizes the need for recognizing and treating complications to prevent long-term morbidity and mortality. Close follow-up and appropriate management are also highlighted for optimal outcomes. [Extracted from the article]