Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) in a two-year-old.

Streptococcal pharyngitis testing and treatment is not routinely recommended in children under the age of 3 because of the unlikely occurrence of infection and negligible risk of serious complications. However, streptococcal pharyngitis and its resulting complications are not uncommon in this age group and can have serious consequences. We report a case of Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections in a 2-year-old with streptococcal pharyngitis. Testing and treatment for streptococcal pharyngitis should be strongly considered when there is evidence of infection and/or an immune-mediated streptococcal complication to prevent and/or decrease the severity of short- and long-term complications.

Clinical profile of paediatric acute rheumatic fever and rheumatic heart disease in Western Australia: 1987 to 2020.

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.

Sydenham's chorea in a 16-year-old female from Bhutan: A case report.

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. Abstract: 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.

Primary Healthcare Workers' Awareness of Acute Rheumatic Fever & Rheumatic Heart Disease: A Study in Public Health Facilities in South Western Uganda.

Introduction: 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.

Tonsils at Telethon: developing a standardised collection of tonsil photographs for group A streptococcal (GAS) research.

Introduction: Group 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. Methods: A 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. Results: Four 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, <1% were positive. Discussion: We report the first standardised use of paediatric tonsil photographs to assess tonsil size in urban-living Australian children. This provides a proof of concept from an urban-living cohort that could be compared with children in other settings with high risk of GAS pharyngitis or rheumatic fever such as remote-living Australian Indigenous populations.

Monoarthritis as the initial presentation of acute rheumatic fever in Iran: A single-center retrospective cross-sectional study.

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.

Acute Rheumatic Fever in Children: The Known and the Unknown Among Saudi Mothers in the Jazan Region.

Background Acute rheumatic fever (ARF) is a significant public health problem that causes morbidity and mortality in low and middle-income countries. Therefore, this study aims to assess mothers' knowledge of acute rheumatic fever and their attitudes in the Jazan region. Methods A cross-sectional study was conducted between the mothers using an online survey. The knowledge level was ranked as poor, fair, and good. In contrast, the attitudes were ranked as positive or negative. Association with sociodemographic variables was assessed, and statistical significance was set at p < 0.05. Results Four hundred thirty-six (436) mothers were included; 39.9% of them were aged 21-30 years, 68.8% were married, 57.3% were non-workers, and 72.2% were university-educated. Most of the mothers had a poor level of knowledge (53%); however, positive attitudes toward the disease were reported in 79.1% of mothers. The poor knowledge levels were related to age, marital status, occupation, and monthly income. Conclusion Despite having positive attitudes toward diagnosing and managing ARF, most of the mothers showed poor knowledge of acute rheumatic fever. This study highlights the situation in the Jazan region, which could be an essential basis for constructing an educational program to raise awareness and knowledge of acute rheumatic fever in the community.

Distinguishing Acute Rheumatic Fever From Post-streptococcal Reactive Arthritis.

We report an initial episode of post-streptococcal reactive arthritis (PRSA) in a 61-year-old male with group A streptococcal (GAS) bacteremia. The disease is commonly reported in young children and young adults. Additionally, this patient exemplifies the nonlinear boundaries of acute rheumatic fever (ARF) and PRSA, bringing into question whether they are truly distinct disease entities. These two conditions oftentimes present in similar fashions, making it difficult for clinicians to determine a specific diagnosis. We highlight the importance of recognizing ARF versus PRSA as an incorrect diagnosis can lead to the development of harmful complications including rheumatic heart disease (RHD).

Adherence to penicillin treatment is essential for effective secondary prevention of rheumatic heart disease: a systematic review and meta-analysis.

Background: Penicillin is essential for secondary prevention of acute rheumatic fever (ARF) and rheumatic heart disease (RHD). However, the incidences of ARF recurrence and RHD progression remain high, particularly in endemic countries. This meta-analysis evaluated the effectiveness of penicillin adherence in secondary prevention of ARF recurrence and RHD progression. Methods: The authors included original articles employing an observational study design in which the study population included patients with ARF or RHD and documented adherence to secondary prophylaxis with penicillin for secondary prevention. Systematic searches of the PubMed, Scopus, and Cochrane databases were performed. Moreover, the authors also conducted a snowballing literature search from Europe PMC to expand the included studies. The quality of each study was assessed using the National Institute of Health Quality Assessment Tool. The statistical analyses were conducted using Review Manager 5.4.1 software developed by Cochrane. In addition, the authors utilized pooled odds ratios (ORs) to compare the adherence techniques. Results: A total of 310 studies were identified, of which 57 full-text articles were assessed for eligibility. The authors included six studies with 1364 patients for the qualitative synthesis and meta-analysis. Good adherence to penicillin for the secondary prophylaxis of ARF and RHD, significantly reduced the odds of ARF recurrence or RHD progression by up to 71% compared to that associated with poor adherence [pooled OR 0.29 (0.21-0.40); I²=0% (p=0.56); Z=7.64 (p <0.00001)]. Conclusion: Good adherence to penicillin for secondary prophylaxis in patients with ARF or RHD is essential for reducing the risk of ARF recurrence or RHD progression.

Chorea as the only presenting clinical feature of rheumatic fever: a case report.

Introduction and importance: Sydenham's chorea (SC), a major neurological manifestation of acute rheumatic fever (ARF), is commonly seen in young children and adolescents. It is characterized by rapid, unpredictable, involuntary, and nonpatterned contractions affecting mostly distal limbs. It can also be associated with clinical or subclinical carditis. SC has been reported as a major manifestation in only 3.87% cases of acute rheumatic fever in Nepal. Case presentation: The authors report a case of a 12-year-old boy with abnormal movement of his right hand and unsteady gait for 12 days. On examination, he had an abnormal hand grip with difficulty maintaining a tetanic contraction (Milkmaid's grip). Laboratory investigations revealed increased anti-Streptolysin O titre and erythrocyte sedimentation rate. Echocardiography revealed subclinical carditis. After thorough clinical examination and pertinent investigations, the final diagnosis of ARF with SC was made. Clinical discussion: SC is a major clinical feature of rheumatic fever according to the revised Jones criteria. It is related to a previous Group A ß-haemolytic Streptococcus pyogenes (GABHS) infection. Approximately 50-65% of the patients with rheumatic fever later develop clinically detectable carditis. Although a self-limiting condition, it might need treatment with antiepileptics, neuroleptics, and phenothiazines. Conclusion: Any child presenting with a movement disorder should also be considered for SC, necessitating additional testing, including a cardiovascular assessment. It needs to be distinguished from other causes of movement disorders as well as psychiatric conditions. Treatment is necessary for moderate to severe chorea that interfere with daily activities. Compliance with subsequent antibiotic prophylaxis is essential for avoiding future cardiac complications.

Silent Acute Rheumatic Fever Unmasked by Using Handheld Echocardiography for Febrile Children Presenting in a Rheumatic Heart Disease-Endemic Area.

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

Preventive Interventions to Reduce the Burden of Rheumatic Heart Disease in Populations at Risk: A Systematic Review.

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.

Outcome following acute and recurrent rheumatic fever.

BACKGROUND: 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). METHODS: 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. RESULTS: 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). CONCLUSION: 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.

State transitions across the Strep A disease spectrum: scoping review and evidence gaps.

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.

Spondyloarthropathies and arthritis post-infection: a historical perspective.

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.

[Rheumatic cardiopathies and its risk factors: about 50 cases]. / Les complications cardiaques du rhumatisme articulaire aigu et ses facteurs de risque : étude de 50 observations.

INTRODUCTION: Acute rheumatic fever (ARF) is a multi-systemic disease, in which cardiac involvement is the most serious major manifestation of disease. The aim of this study was to analyse cardiac involvement in children with ARF and his risk factors. MATERIALS AND METHODS: It were a retrospective study including all children under the age of 14 years who were hospitalized for ARF in the pediatric department of the CHU Hédi Chaker of Sfax, during a period of twelve years (2010-2022). RESULTS: We collected 50 cases (31 boys and 19 girls). Twenty-two patients (44%) developed cardiac lesions. The mean age at diagnosis was 9.6 years [5-14 years]. A pathological heart murmur was detected in 14 cases (n = 14/22) was classified as mild carditis in 15 cases, moderate carditis in 5 cases and severe in 2 cases. The median follow-up time was 3,3 years. Nineteen patients developed valvular sequelae Risk factors of cardiac lesions was: age more than 8 years, heart murmur, allonged PR, CRP > 100 mg/l and VS > 100 mm. CONCLUSION: CR is still a public health problem in Tunisia. It is a serious pathology that can cause serious increases in morbidity rates. Thus, we must strengthen preventive strategies.

Revising the value of Antistreptolysin O titre in childhood and its interpretation in the diagnostic approach of rheumatic diseases.

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.

Current Clinical Profile of Acute Rheumatic Fever and Recurrent Acute Rheumatic Fever in Pakistan.

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.

Acquired brown syndrome in a child with acute rheumatic fever.

PURPOSE: To describe a rare case of acquired Brown syndrome (ABS) associated with acute rheumatic fever (ARF). CASE REPORT: a healthy 9-year-old girl developed polyarthritis, carditis, and elevated inflammatory markers 10 days after suffering from a sore throat. She was diagnosed with ARF and was treated with systemic antibiotics and high-dose non-steroidal anti-inflammatory drug (NSAID). After 2 weeks, despite improvement of ARF symptoms, the patient presented with binocular vertical diplopia, periorbital pain, and limited elevation in adduction of the left eye. Imaging revealed a thickened trochlea-superior oblique tendon complex, and ABS due to trochleitis was diagnosed. The patient underwent a successful regional corticosteroid injection as a second-line treatment, and within a month, all signs and symptoms of ABS resolved. CONCLUSIONS: ABS is an infrequent yet possible manifestation of ARF.

Pediatric Rheumatic Fever With Acute Fulminant Carditis: A Case Report.

Acute rheumatic fever (ARF) is a multi-system inflammatory autoimmune disease. It is a significant cause of heart disease and early death worldwide, especially in children in developing countries. We present a case of acute fulminant rheumatic carditis in a child with no obvious predisposing factors, who resided in a developed country where this disease is not endemic. After pathological examination, a diagnosis of ARF with pancarditis was confirmed. This disease was not suspected before the pathological examination because of its low prevalence in Belgium.