Unilateral pulmonary edema and acute rheumatic fever

Although the diagnostic criteria for acute rheumatic fever (ARF) are well known, a high index of suspicion is necessary in order to assure timely diagnosis and appropriate treatment. We present a case of an 8-year-old child who presented with unilateral pulmonary edema secondary to acute mitral insufficiency due to ARF. ARF should be considered in the differential diagnosis of unilateral pulmonary edema in children.     

Acute rheumatic fever in Konya, Turkey

Abstract Background : Patients with acute rheumatic fever (ARF), who were admitted to Pediatric Cardiology Unit of Selçuk University Faculty of Medicine from July 1993 to 1998, were studied retrospectively to verify the clinical profile of the disease and to compare the results with those from other countries. Methods : All patients were examined by one of the two pediatric cardiologists in our study group. Every patient had a chest X‐ray, electrocardiogram and an echocardiographic investigation. Throat culture, antistreptolysin O test, C‐reactive protein and sedimentation rates were investigated for each patient. Results : During the study period, 274 cases with ARF were identified among patients admitted to the present institution. There were 8032 visits during the study period, giving an occurrence rate of 3.4%. Arthritis was the most common major manifestation (81.4%). It was followed by carditis (60.9%) and chorea (17.9%). Subcutaneous nodules (0.7%) and erythema marginatum (0.4%) were both seen in patients with carditis. The mitral valve was the most commonly affected valve (95.8%), followed by the aortic valve (40.1%). Two patients died and regurgitation disappeared in 21% of patients with mitral regurgitation. Fifteen patients (14%) with isolated arthritis and pure chorea had mitral regurgitation demonstrated by echocardiographic investigation but without any significant murmur. Conclusion : The present study indicates that ARF is still a significant problem in Konya and that recurrences can be prevented by administering a 3‐week benzathine penicillin G regimen.     

Acute rheumatic fever in Saudi Arabia: Mild pattern of initial attack

Summary Fifty-one children with the initial attack of acute rheumatic fever (ARF) were studied prospectively to verify the sociodemographic and clinical profile and to compare results with those from other countries. Most children belonged to large families who lived in an urban setting with ready access to medical care. Unlike reports from many developing countries, the clinical manifestations in this study paralleled data from the West and included arthritis in 76% of the cases, carditis in 43%, and chorea in 8%. Among the 22 cases with carditis, 18 had mitral regurgitation, three developed combined mitral and aortic regurgitation, and one had aortic regurgitation. This study demonstrates the mild nature of ARF in Saudi Arabia and supports the concept that climate and geography appear to bear little relationship to the incidence and severity of ARF.     

Lung function in children and adolescents with antecedents of acute rheumatic fever

Static lung volumes, CO-lung transfer, airway resistance, maximal expiratory flows and lung elastic properties were studied in 29 children and adolescents 1–10 years after recovery from acute rheumatic fever. There were essentially no changes in lung function even in the subjects with a residual valvular disease. The only abnormality was a tendency for the elastic lung recoil at TLC to be low, which is interpreted as probably reflecting a decrease in inspiratory muscle force.Abbreviations ARF acute rheumatic fever - VC vital capacity - TGV thoracic gas volume - Raw airway resistance - P-V pressure-volume - TLC total lung capacity - MEFV maximal expiratory flow-volume - T_LCO CO-lung transfer - FRC functional residual capacity     

Acute rheumatic fever in children in the Ankara area in 1990–1992 and comparison with a previous study in 1980–1989

Two hundred and twenty-eight patients with acute rheumatic fever (ARF), who were admitted to Dr Sami Ulus Children's Hospital between January 1990 and November 1992, were evaluated. Compared with the 1980s, an increase in the frequency of the disease was observed. The majority of patients (56.5%) were between 9 and 12 years old and 36.8% were admitted in winter. One hundred patients had arthritis only, 59 carditis and 40 chorea; 5 had carditis and chorea and 24 had arthritis and carditis. Nineteen percent of patients had a history of a previous attack. Seven of 84 patients with carditis had congestive heart failure and 2 had pericarditis. Cardiomegaly was present in 36 patients. The mitral valve was affected in 77 patients, tricuspid valve in I patient and both miral and aortic valves in 6 patients. One patient died as a result of severe congestive cardiac failure. Twenty-one patients had a recurrent attack. We observed that ARF is still a significant cause of morbidity in our country. As the disease is preventable by the eradication of streptococcus, we conclude that more effort should be made in the early detection and treatment of streptococcal infections.     

Hemolysis in a glucose-6-phosphate dehydrogenase-deficient boy with rheumatic fever

Summary A 12-year-old black boy with the A type of glucose-6-phosphate dehydrogenase (G6PD) deficiency had a hemolytic episode concurrent with acute rheumatic fever. Aspirin nevertheless remains the drug of choice for acute rheumatic fever even in the A type of G6PD deficiency.     

Historical aspects of rheumatic fever

Few diseases have experienced such a remarkable change in their epidemiology over the past century, without the influence of a vaccine, than rheumatic fever. Rheumatic fever has all but disappeared from industrialised countries after being a frequent problem in the 1940s and 1950s. That the disease still occurs at high incidence in resource limited settings and in Indigenous populations in industrialised countries, particularly in Australia and New Zealand, is an indication of the profound effect of socio‐economic factors on the disease. Although there have been major changes in the epidemiology of rheumatic fever, diagnosis remains reliant on careful clinical judgement and management is remarkably similar to that 50 years ago. Over the past decade, increasing attention has been given to rheumatic fever and rheumatic heart disease as public health issues, including in Australia and particularly in New Zealand, as well as in selected low and middle income countries. Perhaps the greatest hope for public health control of rheumatic fever is the development of a vaccine against Streptococcus pyogenes, and there are encouraging initiatives in this area. However, an effective vaccine is some time away and in the meantime public health efforts need to focus on effective translation of the known evidence around primary and secondary prophylaxis into policy and practice.     

Childhood acute rheumatic fever in Greece: experience of the past 18 years

This study reports on the authors' experience with acute rheumatic fever (ARF) during the years 1980-1997. The objectives were to estimate the incidence of the disease an area of Greece to characterize its epidemiology, to determine the frequency of the antecedent symptoms and to describe its clinical presentation. The medical records of 66 confirmed cases admitted to the First Department of Pediatrics, "Aghia Sophia" Children's Hospital, were reviewed. Two outbreaks occurred during this period. In contrast to the 3-4 cases seen every year, 14 cases were diagnosed during the 6 mo period from October 1989 to March 1990. An additional 10 cases were diagnosed in 1993. Most of the children (76%) were between 8 and 14 y old. The children were predominantly from middle-class families with ready access to medical care. Carditis, evident by auscultation, and arthritis were the dominant major manifestations in 70% and 68% of the cases, respectively. Mild carditis was present in 54% of children with valvular disease. CONCLUSION: ARF exists in the paediatric Greek population with exacerbations and remissions, but the cardiac manifestations appear mild or moderate.     

Mortality and hospitalisation costs of rheumatic fever and rheumatic heart disease in New Zealand

Aims: To estimate the annual mortality and the cost of hospital admissions for acute rheumatic fever (ARF) and rheumatic heart disease (RHD) for New Zealand residents. Methods: Hospital admissions in 2000–2009 with a principal diagnosis of ARF or RHD (ICD9_AM 390‐398; ICD10‐AM I00‐I099) and deaths in 2000–2007 with RHD as the underlying cause were obtained from routine statistics. The cost of each admission was estimated by multiplying its diagnosis‐related group (DRG) cost weight by the national price for financial year 2009/2010. Results: There were on average 159 RHD deaths each year with a mean annual mortality rate of 4.4 per 100 000 (95% confidence limit 4.2, 4.7). Age‐adjusted mortality was five‐ to 10‐fold higher for Māori and Pacific peoples than for non‐Māori/Pacific. The mean age at RHD death (male/female) was 56.4/58.4 for Māori, 50.9/59.8 for Pacific and 78.2/80.6 for non‐Māori, non‐Pacific men and women. The average annual DRG‐based cost of hospital admissions in 2000–2009 for ARF and RHD across all age groups was $12.0 million (95% confidence limit $11.1 million, $12.8 million). Heart valve surgery accounted for 28% of admissions and 71% of the cost. For children 5–14 years of age, valve surgery accounted for 7% of admissions and 27% of the cost. Two‐thirds of the cost occurs after the age of 30. Conclusions: ARF and RHD comprise a burden of mortality and hospital cost concentrated largely in middle age. Māori and Pacific RHD mortality rates are substantially higher than those of non‐Māori/Pacific.     

Tolmetin and salicylate therapy in acute rheumatic fever: Comparison of clinical efficacy and side-effects

BACKGROUND: The arthritis of rheumatic fever is very responsive to treatment with salicylates, but there are many adverse reactions, especially hepatotoxicity, due to aspirin (acetylsalicylic acid) therapy. These side-effects change the course and duration of rheumatic fever. Other non-steroidal anti-inflammatory drugs may be equally effective, although no reports are available. METHODS: We studied 72 patients with rheumatic fever who were admitted to Dr Sami Ulus Children's Hospital between 1995 and 1999. Twenty patients with arthritis were treated with tolmetin (25 mg/kg per day; group I) and 52 patients with arthritis and/or mild carditis were put on aspirin therapy (75-100 mg/kg per day) for 4-6 weeks (group II). Arthritis had disappeared at the same time in both the aspirin and tolmetin groups (P = 0.675). RESULTS: The erythrocyte sedimentation rates of patients upon admission, at the first week and at the end of therapy were not different in the two groups (P > 0.05). No adverse effect of tolmetin therapy was observed, whereas side-effects of salicylate were observed in 19 patients (36.5%) in the aspirin group. Hepatotoxicity, gastric irritation and salicylism were found in 16, four and three patients, respectively. Renal toxicity and Reye syndrome were not demonstrated. Because of these side-effects of aspirin, therapy had to be stopped for 10-20 days and the duration of hospitalization in this group was lengthened unnecessarily. CONCLUSION: Tolmetin was safe and effective treatment for arthritic rheumatic fever patients without carditis. Tolmetin can be used particularly in patients who cannot tolerate aspirin.     

Acute rheumatic fever and poststreptococcal acute glomerulonephritis caused by T serotype 12 Streptococcus

We present a rare case of a 10 year old Japanese boy with acute rheumatic fever accompanied with poststreptococcal acute glomerulonephritis. We isolated group A Streptococcus serotype T 12, a strain that was thought to be nephritogenic but not rheumatogenic, from throat culture. Although rare, physicians should be aware that acute renal disease may accompany rheumatic fever.     

Persistence of acute rheumatic fever in a tertiary children's hospital

Aim: To identify and describe all children admitted with acute rheumatic fever (ARF) to a tertiary paediatric hospital in Sydney over a 9‐year period and to describe their demographic and clinical characteristics, management and short‐term outcomes. Delays in diagnosis, recurrence of ARF and use of secondary prophylaxis were also documented. Methods: Retrospective review of medical records for children aged <15 years admitted to the Children's Hospital at Westmead, Sydney, with ARF (International Classification of Diseases (ICD)‐10 classification I0.0–I09.9) during 2000–2008. Only cases meeting the National Heart Foundation of Australia diagnostic criteria for ARF were included. Results: Twenty‐six children met the National Heart Foundation of Australia criteria for ARF. The median age was 11.5 years (range 5.8–14.6) and 15 (58%) were male. Ten (38%) identified as Pacific Islander, and 5 (19%) as Aboriginal and Torres Strait Islander (ATSI). Most (n= 20, 77%) lived in suburban Sydney, and 69% were classified in the two most disadvantaged quintiles on the Index of Relative Socioeconomic Disadvantage and Advantage. Four (15%) had Sydenham's chorea, and 81% had carditis (mitral and/or aortic regurgitation). Six (23%) children had previous ARF. Antibiotic prophylaxis to prevent recurrent ARF was prescribed in all cases, but 50% received oral penicillin, rather than by intramuscular injection. Barriers to timely diagnosis were identified in 81%, including delayed presentation and delayed referral. Conclusion: Most children presenting to the hospital with ARF lived in disadvantaged areas of Sydney. Pacific Islander and Aboriginal and Torres Strait Islander children were over‐represented. Mitigation of RHD requires early identification of ARF and appropriate delivery of secondary prophylaxis.     

小儿风湿热与白介素1受体拮抗剂基因多态性分析

用聚合酶链反应（ＰＣＲ）技术对２８例小儿风湿热患者和２５例正常对照组白介素１受体拮剂（ＩＬ－１ｒａ）基因数目可变的串联重复多态性进行了分析。发现小儿风湿热患者中ＩＬ１ＲＮ＊２等位基因频率明显高于正常对照组（Ｐ〈０．０５）。如将２８例患者分为两组,第一组为符合ｏｎｅｓ诊断标准中任何２个主要表现或１个主要表现加２个次要表现者,第二组为临床表现多于第一组一个或几个者,则发现前者ＩＬ１ＲＮ＊２频率与正常对照相比无显著差异（Ｐ〉０．０５）。而后者有显著差异（Ｐ〈０．０５）;提示ＩＬ１ＲＮ＊２可能不直接影响小儿风湿热的易感性,但３ＩＬ１ＲＮ＊２者患病后可能病情更严重。     

Lessons to be learned: Using National Immunisation strategies to improve adherence to acute rheumatic fever secondary prophylaxis

Rheumatic heart disease, as a result of a single or recurrent episode of acute rheumatic fever (ARF), remains a significant cause of morbidity and mortality in northern and remote Australia; ARF has a peak incidence among 5–14‐year‐old Aboriginal and Torres Strait Islander children. Long‐term regular benzathine penicillin G injections are the only currently successful secondary prevention strategy; however, rates of adherence remain critically low. In contrast, rates of adherence to immunisations on the National Immunisation Program (NIP) Schedule are high, even among this target population. This article compares strategies used to implement and improve ARF secondary prophylaxis with those used in the NIP. Some successful NIP strategies, such as Service Incentive Payment for health providers, home‐visiting delivery models and integration into the National Immunisation Register, if applied to ARF secondary prophylaxis have the potential to improve benzathine penicillin G adherence.