Positive impact of training rural health workers in identification and prevention of acute rheumatic fever in eastern Uganda.

BACKGROUND: Diagnosis of acute rheumatic fever (ARF) is mainly clinical. Delayed or missed diagnosis and failure to administer appropriate and timely treatment of ARF leads to rheumatic heart disease (RHD), which could necessitate expensive treatments such as open-heart surgery. Implementation of preventative guidelines depends on availability of trained healthcare workers. As part of the routine support supervision, the Uganda Heart Institute sent out a team to rural eastern Uganda to evaluate health workers' knowledge level regarding management of ARF. METHODS: Health workers from selected health facilities in Tororo district, eastern Uganda, were assessed for their knowledge on the clinical features and role of benzathine penicillin G (BPG) in the treatment and prevention of ARF recurrence. Using the RHD Action Needs assessment tool, we generated and administered a pre-test, then conducted training and re-administered a post-test. Eight months later, health workers were again assessed for knowledge retention and change in practices. Statistical analysis was done using Stata version 15. RESULTS: During the initial phase, 34 of the 109 (31%) health workers passed the pre-test, indicating familiarity with clinical features of ARF. The level of knowledge of BPG use in ARF was very poor in all the health units [25/109 (22.6%)] but improved after training to 80%, as shown by the chi-squared test ( χ2 = 0.000). However, retention of this knowledge waned after eight months and was not significantly different compared to pre-training (χ2 ≥ 0.2). CONCLUSIONS: A critical knowledge gap is evident among health workers, both in awareness and treatment of ARF, and calls for repetitive training as a priority strategy in prevention.

Diagnosing rheumatic heart disease: where are we now and what are the challenges?

INTRODUCTION: Rheumatic heart disease (RHD), a sequela of acute rheumatic fever (ARF), affects 40.5 million people worldwide. The burden of disease disproportionately falls on low- and middle-income countries (LMIC) and sub-populations within high-income countries (HIC). Advances have been made in earlier detection of RHD, though several barriers to ideal management persist. AREAS COVERED: This article reviews the current burden of RHD, highlighting the disparate impact of disease. It also reviews the clinical and echocardiographic presentation of RHD, as some may present in late stages of disease with associated complications. Finally, we review the advances which have been made in echocardiographic screening to detect latent RHD, highlighting the challenges which remain regarding secondary prophylaxis management and uncertainty of best practices for treatment of latent RHD. EXPERT OPINION: Advances in technology and validation of portable echocardiography have made screening and identifying latent RHD feasible in the most burdened regions. However, uncertainty remains around best management of those with latent RHD and best methods to ensure ideal secondary prophylaxis for RHD. Research regarding latent RHD management, as well as continued work on innovative solutions (such as group A streptococcal vaccine), are promising as efforts to improve outcomes of this preventable disease persist.

Incidence and clinical characteristics of acute rheumatic fever in Turkey: Results of a nationwide multicentre study.

AIM: To evaluate the incidence and clinical features of acute rheumatic fever (ARF) in Turkey, following the revised Jones criteria in 2015. METHODS: This multicentre study was designed by the Acquired Heart Diseases Working Group of the Turkish Pediatric Cardiology and Pediatric Cardiac Surgery Association in 2016. The data during the first attack of 1103 ARF patients were collected from the paediatric cardiologists between 1 January 2016 and 31 December 2016. RESULTS: Turkey National Institute of Statistics records of 2016 were used for the determination of ARF incidence with regard to various cities and regions separately. The estimated incidence rate of ARF was 8.84/100 000 in Turkey. The ARF incidence varied considerably among different regions. The highest incidence was found in the Eastern Anatolia Region as 14.4/100 000, and the lowest incidence was found in the Black Sea Region as 3.3/100 000 (P < 0.05). Clinical carditis was the most common finding. The incidence of clinical carditis, subclinical carditis, polyarthritis, aseptic monoarthritis, polyarthralgia and Sydenham's Chorea was 53.5%, 29.1%, 52.8%, 10.3%, 18.6% and 7.9%, respectively. The incidences of clinical carditis, subclinical carditis, polyarthritis and polyarthralgia were found to be significantly different among different regions (P < 0.05). CONCLUSION: The findings of this nationwide screening of ARF suggest that Turkey should be included in the moderate-risk group.

[NEW ONSET OF VALVULAR HEART DISEASE ALONG WITH ANCIENT PATHOLOGIC FINDING].

INTRODUCTION: Rheumatic fever (RF) is an autoinflammatory disease that is caused by the host response to an infection with group A ß-hemolytic streptococcus. In this case report we describe a 15 years old boy with Down syndrome who had unusual presentation of acute rheumatic fever with a fulminant multisystemic which included heart failure secondary to pancarditis and adult respiratory distress syndrome. The final diagnosis was confirmed after cardiac biopsy that was performed during valve replacement surgery and demonstrated Aschoff bodies - a pathognomonic finding in acute rheumatic fever.

Enfermos com histórico de Febre Reumática e suas implicações em odontologia: uma revisão de literatura / Sick with history of rheumatic fever and its implications in dentistry: a literature review

Objetivo: analisar informações presentes na literatura sobre a citada patologia com enfoque odontológico, baseando-se em evidências atuais, a fim de estruturar uma conduta clínica, métodos preventivos e terapêuticos. Revisão de literatura: executou-se busca na literatura em dezembro de 2020, por meio dos descritores pré-estabelecidos, nos bancos de dados LILACS e PubMed/MEDLINE, além de complementações utilizando o Google Scholar. Foram encontrados 563 artigos, refinados em 10, juntamente ao uso de 3 livros. Considerações finais: a partir dos achados, constatou-se que a realização de uma anamnese criteriosa durante a primeira consulta faz-se imprescindível, por auxiliar na compreensão dos aspectos fisiopatológicos do paciente, os quais irão determinar o emprego ou não de fármacos, principalmente antes de procedimentos invasivos. Em contrapartida, verificou-se a negligência desse assunto no âmbito odontológico, pelo fato de existirem poucos estudos relacionados ao manejo clínico de pacientes com Febre Reumática (FR), sendo necessárias maiores pesquisas, com o fito de construir-se uma conduta clínica adequada, reduzindo-se os riscos e a incidência de tal enfermidade.(AU)

Objective: to analyze information in the literature about mentioned pathology with a dental focus, based on current evidence in order to structure a clinical conduct, preventive and therapeutic methods. Literature Review: literature searches were performed in December 2020, using pre-established descriptors, in the databases LILACS and PubMed/MEDLINE, in addition to complementations using Google Scholar. About 563 articles were found, refined in 10, along with the use of 3 books. Final Considerations: From the findings, it was found that the performance of a careful anamnesis during the first consultation is essential, as it helps to understand the patient's pathophysiological aspects, which will determine the use of drugs, mainly, before invasive procedures. On the other hand, this matter was neglected in the Dental field, due to the fact that there are few studies related to the clinical management of patients with Rheumatic Fever (RF), and further research is necessary, with the aim of building an adequate clinical conduct, reducing the risks and the incidence of such a disease.(AU)

Coexistence of acute poststreptococcal glomerulonephritis and acute rheumatic fever in a Japanese girl with primary Sjögren's syndrome.

Although acute poststreptococcal glomerulonephritis (APSGN) and acute rheumatic fever (ARF) are well-known complications of group A streptococcus infection, concomitant occurrence of both diseases is rare. We report an 11-year-old Japanese girl with primary Sjögren's syndrome complicated by acute renal failure about 2 weeks after the onset of pharyngitis. Although histopathological findings of the kidney were not confirmative, APSGN was suggested by the spontaneous recovery of her renal function, typical latent period with high levels of antistreptolysin O and low serum levels of C3 but not of C4. In addition, cardiac hypomotility and regurgitation of the 4 valves progressed in the convalescent phase of APSGN, which was accompanied by elevation of serum C-reactive protein and plasma brain natriuretic peptide (BNP) levels. Myocarditis was suggested by delayed gadolinium-enhancement of cardiac walls on cardiac magnetic resonance imaging. She was diagnosed with APSGN and ARF and was treated with a combination of short course prednisolone and prophylactic penicillin G. There is no relapse of renal or cardiac symptoms during 6 years follow-up. Unexpected elevation of plasma BNP in a convalescent stage of APSGN suggests the development of ARF. Underlying Sjögren's syndrome (SS) may modify the histopathological findings and make it difficult to differentiate APSGN from CTD-associated nephritis such as lupus nephritis (LN) even by renal biopsy.

Acute rheumatic fever in south-east of Turkey: clinical features and epidemiological evaluation of the patients over the last 25 years.

This study evaluates clinical and epidemiological features of acute rheumatic fever using the data of last 25 years in our hospital in south-east of Turkey. The medical records of 377 patients with acute rheumatic fever admitted to Pediatric Cardiology Department of Çukurova University during 1993-2017 were retrospectively analysed. Two hundred and six patients were admitted between 1993 and 2000, 91 between 2001 and 2008, and 80 between 2009 and 2017. The largest age group (52%) were between 9 to 12 years of age and approximately two-thirds of the patients presented in the spring and winter seasons (62.8%). Among the major findings, the most common included carditis 83.6% (n = 315), arthritis at 74% (n = 279), Sydenham's chorea at 13.5% (n = 51), and only two patients (0.5%) had erythema marginatum and two patients (0.5%) had subcutaneous nodule. Carditis was the most common manifestation observed in 315 patients (83.6%). The most commonly affected valve was the mitral valve alone (54.9%), followed by a combined mitral and aortic valves (34%) and aortic valve alone (5.7%). Of the patients with carditis, 48.6% (n = 153) had mild carditis, of which 45 had a subclinical. Sixty-two patients (19.7%) had moderate and 100 patients (31.7%) had severe carditis. At the follow-up, 2 patients died and 16 patients underwent valve surgery. Twenty-eight (7.4%) patients' valve lesions were completely resolved. Conclusion: Although the incidence of acute rheumatic fever decreased, it still is an important disease that can cause serious increases in morbidity and mortality rates in our country.

Dry eye in rheumatoid arthritis: relation to disease activity.

The aim of this study was to find the correlation between severity of dry eye and rheumatoid arthritis (RA) disease activity. Forty- two RA patients with dry eye were recruited from Rheumatology Outpatient Clinic in Minia University Hospital. Assessment of RA disease activity was performed using disease activity score (DAS-28). Ocular tests include Schirmer test I, tear film break up time (TBUT) and ocular staining score (OSS) was performed by ophthalmologist to find evidence of ocular dryness. Erythrocyte sedimentation rate (ESR), rheumatoid factor (RF), anti SSA/Ro and anti SSB/La was also tested. Patients with severe dry (OSS ≥ 3) underwent minor salivary gland biopsy (MSGB) as suspected to have secondary Sjögren's syndrome (SS). Of 42 RA patients, 30 had definite dry eye. DAS-28 did not show significant correlation with any of ocular tests for dryness while the duration of RA was significantly positively correlated with Schirmer test and OSS. The biopsy results of RA patients with severe dry eye show no evidence of SS. The severity of dry eye is not correlated with activity of RA but with its duration.

Atypical presentation of acute rheumatic fever (ARF) in a 25-year-old woman in the Caribbean: a challenging diagnosis.

A 25-year-old woman presented a challenging diagnosis of acute rheumatic fever (ARF). Initial symptoms included dry cough and three minor Jones criteria (unabating fever (38.4°C, 0d), elevated acute phase reactants (C-reactive protein, 13d) and joint pain (monoarthralgia) in her neck (0d)). ARF was diagnosed only after presentation of two major Jones criteria (polyarthritis/polyarthralgia (16d) and erythema marginatum (41d)) and positive antistreptolysin O titre (44d). Parotid swelling, peripheral oedema, elevated liver enzymes and diffuse lymphadenopathy complicated the diagnosis. Throat swab, chorea and carditis were negative or absent. Atypical ARF is challenging to recognise. There is no diagnostic test and its presentation is similar to that of other diseases. While the 2015 Jones criteria modification increased specificity of ARF diagnosis, atypical cases may still be missed, especially by physicians in developed countries. Suspicion of atypical ARF, especially after travel to high incidence regions, would allow for earlier treatment and prevention of rheumatic heart disease.

Higher rate of rheumatic manifestations and delay in diagnosis in Brazilian Fabry disease patients.

BACKGROUND: Fabry disease (FD) is an X-linked lysosomal disorder due to mutations in the GLA gene resulting in defective enzyme alpha-galactosidase A. FD patients are frequently misdiagnosed, commonly for rheumatic diseases. Determining pathogenicity of a mutation depends of in silico predictions but mostly on available clinical information and interpretation may change in light of evolving knowledge. Similar signs and symptoms in carriers of GLA gene genetic variants of unknown significance or of benign variants may hamper diagnosis. This study reviews rheumatic and immune-mediated manifestations in a cohort of Brazilian FD patients with classic mutations and also in subjects with GLA gene A143T and R118C mutations. Misdiagnoses, time to correct diagnosis or determination of GLA gene status, time to treatment initiation and reasons for treatment prescription in A143T and R118C subjects are reviewed. METHODS: Genotype confirmed classic FD patients (n = 37) and subjects with GLA gene mutations A143T and R118C (n = 19) were referred for assessment. Subjects with R118C and A143T mutations had been previously identified during screening procedures at hemodialysis units. All patients were interviewed and examined by a rheumatologist with previous knowledge of disease and/or mutation status. A structured tool developed by the authors was used to cover all aspects of FD and of common rheumatic conditions. All available laboratory and imaging data were reviewed. RESULTS: Thirty-seven consecutive FD patients were interviewed - 16 male / 21 female (mean age: 43.1 years) and 19 consecutive subjects with GLA gene mutations R118C and A143T were evaluated - 8 male / 11 female (mean age: 39.6 years); 15 [R118C] / 4 [A143T]. Misdiagnosis in FD patients occurred in 11 males (68.8%) and 13 females (61.9%) of which 10 males and 9 females were previously diagnosed with one or more rheumatic conditions, most frequently rheumatic fever or "rheumatism" (unspecified rheumatic disorder). Median time for diagnosis after symptom onset was 16 years (range, 0-52 years). Twenty-two patients were treated with enzyme replacement therapy (ERT) - 13 male and 9 female. Median time to ERT initiation after FD diagnosis was 0.5 years (range, 0-15 years). Rheumatic manifestations occurred in 68.4% of R118C and A143T subjects. Two subjects had been prescribed ERT because of renal disease [R118C] and neuropsychiatric symptoms [A143T]. CONCLUSION: Misdiagnoses occurred in 64.8% of FD patients, most frequently for rheumatic conditions. Median time for correct diagnosis was 16 years. Rheumatic manifestations are also frequent in subjects with GLA gene R118C and A143T mutations. These results reinforce the need to raise awareness and increase knowledge about Fabry disease among physicians, notably rheumatologists, who definitely have a role in identifying patients and determining disease burden. Decision to start treatment should consider expert opinion and follow local guidelines.

Oxidative stress parameters in children with acute rheumatic fever.

BACKGROUND: The aim of this study was to investigate of the role of oxidative stress (OS) in acute rheumatic fever (ARF) and its relationship with the progress of the disease. METHODS: Thirty patients with ARF and 31 age-sex-matched healthy children were enrolled in this study. Serum malondialdehyde (MDA), protein carbonyl (PCO), high-sensitivity C-reactive protein (hs-CRP), cytokines (tumor necrosis factor-α and interleukin-6) and total antioxidant capacity (TAC) were measured. The diagnosis of ARF was based on the Jones criteria. RESULTS: Patients with ARF had significantly higher serum MDA, PCO, hs-CRP and cytokine levels and lower TAC than healthy controls. On Pearson's correlation analysis we found that oxidative stress markers were positively correlated with hs-CRP and cytokines, while TAC was negatively correlated with MDA, PCO, hs-CRP and cytokines. The number of valves involved as well as the level of mitral valve involvement was also significantly related to the oxidative stress parameters and TAC. All oxidative stress parameters decreased significantly with anti-inflammatory therapy while TAC increased. CONCLUSION: Malondialdehyde and PCO, as biomarkers, as well as hs-CRP together with the other available diagnostic tools, can be used in the evaluation of patients with ARF.

Childhood Blastic Plasmacytoid Dendritic Cell Neoplasm Mimicking Acute Rheumatic Fever: Report of an Unusual Clinical Presentation and Review of Literature.

BACKGROUND: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is very rarely diagnosed in children with less than 50 cases in the literature. OBSERVATION: We report a case of childhood BPDCN who mimicked acute rheumatic fever at presentation. Majority of the reported childhood BPDCN received acute lymphoblastic leukemia-like chemotherapy with/without stem cell therapy, whereas those who received acute myeloid leukemia-like therapy predominantly succumbed to disease or sepsis. Overall 68% of the patients were alive and achieved complete remission with an overall prognosis slightly better in children compared with adults. CONCLUSIONS: The case is reported due to its unique unusual clinical presentation and its rarity in pediatric population.

The 2017 Seventh World Congress of Pediatric Cardiology & Cardiac Surgery: week in review - ambulatory pediatric cardiology.

The Seventh World Congress of Pediatric Cardiology was held in Barcelona in July, 2017. The central philosophy of the congress was "bridging together" all major specialties in the field. This article summarises the highlights of the meeting as it relates to ambulatory paediatric cardiology. There is a now a more unified approach to children with CHD, including assessment of neuro-developmental outcomes. The new World Heart Foundation criteria for the diagnosis of rheumatic fever remain controversial.

Rheumatic fever with severe carditis: still prevalent in the South West Pacific.

Rheumatic heart disease (RHD) has a worldwide prevalence of 33 million cases and 270 000 deaths annually, making it the most common acquired heart disease in the world. There is a disparate global burden in developing countries. This case report aims to address the minimal RHD coverage by the international medical community. A Tahitian boy aged 10 years was diagnosed with advanced heart failure secondary to RHD at a local clinic. Previous, subtle symptoms of changes in handwriting and months of fever had gone unrecognised. Following a rapid referral to the nearest tertiary centre in New Zealand, urgent cardiac surgery took place. He returned home facing lifelong anticoagulation. This case highlights the RHD burden in Oceania, the limited access to paediatric cardiac services in countries where the RHD burden is greatest and the need for improved awareness of RHD by healthcare professionals, and the general public, in endemic areas.

Association between rheumatic fever and takayasu's arteritis - case report / Associação entre febre reumática e arterite de Takayasu - Relato de caso

ABSTRACT Takayasu’s arteritis (TA) and rheumatic fever are diseases that can start with cardiac fea-tures, making the diagnosis difficult. There are reports of association of RF with Takayasu’sarteritis beginning with cardiac involvement in pediatric patients. The aim of this study isto report the possible association of RF and TA in patients with cardiac abnormalities. Wedescribe the case of an adolescent initially diagnosed with RF who progressed with changesthat allowed making the diagnosis of TA. TA and RF are two important causes of valveinvolvement that may have systemic manifestations.

RESUMO A arterite de Takayasu (AT) e a febre reumática (FR) são doenças que podem ter início commanifestação cardíaca, o que dificulta o diagnóstico. Há relatos de associação de FR com AT que se inicia com comprometimento cardíaco na faixa etária pediátrica. O objetivo deste estudo é relatar a possibilidade da associação de FR e AT em paciente com alteração cardíaca. Descrevemos o caso de uma adolescente diagnosticada inicialmente como FR que apresentou na evolução alterações que permitiram o diagnóstico de AT. A AT e a FR são duas causas importantes de envolvimento valvular que podem apresentar manifestações sistêmicas.

Dificuldades no diagnóstico diferencial entre arterite de Takayasu e febre reumática: relato de caso / Difficulties in the differential diagnosis between Takayasu arteritis and rheumatic fever: case report

Resumo Apresentamos o caso de uma paciente portadora de insuficiência cardíaca com prótese valvar aórtica biológica e alterações vasculares compatíveis com arterite de Takayasu (AT) que chegou ao serviço em uso de corticoides e em profilaxia para febre reumática (FR). Não foi possível afastar a associação entre ambas as enfermidades.

Abstract In this article, we present the case of a patient with heart failure with biological aortic valve prosthesis and multiple vascular changes consistent with Takayasu arteritis (TA) who was seen in our department receiving corticosteroids and secondary prevention of rheumatic fever (RF); it was not possible to exclude the association between both diseases.

Seven key actions to eradicate rheumatic heart disease in Africa: the Addis Ababa communiqué.

Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) remain major causes of heart failure, stroke and death among African women and children, despite being preventable and imminently treatable. From 21 to 22 February 2015, the Social Cluster of the Africa Union Commission (AUC) hosted a consultation with RHD experts convened by the Pan-African Society of Cardiology (PASCAR) in Addis Ababa, Ethiopia, to develop a 'roadmap' of key actions that need to be taken by governments to eliminate ARF and eradicate RHD in Africa. Seven priority areas for action were adopted: (1) create prospective disease registers at sentinel sites in affected countries to measure disease burden and track progress towards the reduction of mortality by 25% by the year 2025, (2) ensure an adequate supply of high-quality benzathine penicillin for the primary and secondary prevention of ARF/RHD, (3) improve access to reproductive health services for women with RHD and other non-communicable diseases (NCD), (4) decentralise technical expertise and technology for diagnosing and managing ARF and RHD (including ultrasound of the heart), (5) establish national and regional centres of excellence for essential cardiac surgery for the treatment of affected patients and training of cardiovascular practitioners of the future, (6) initiate national multi-sectoral RHD programmes within NCD control programmes of affected countries, and (7) foster international partnerships with multinational organisations for resource mobilisation, monitoring and evaluation of the programme to end RHD in Africa. This Addis Ababa communiqué has since been endorsed by African Union heads of state, and plans are underway to implement the roadmap in order to end ARF and RHD in Africa in our lifetime.