Mild Carditis Research Articles

Serum Endocan Levels in Children with Acute Rheumatic Fever.

Acute rheumatic fever is an immunologically delayed autoimmune sequel of throat infection caused by group A streptococcus. The aim of this study was to evaluate endocan levels in patients with acute rheumatic fever and compare with the control group. The aim of this study was to evaluate endocan levels in patients with acute rheumatic fever and compare with the control group. Twenty-three children with acute rheumatic fever (11 men, 12 females; mean age 13 ± 2.7 years; range 5 to 15 years) and a healthy control group of 31 children (16 men, 15 females; mean age 13.8 ± 2.4 years; range 5 to 15 years) were recruited. The sedimentation rate, C-reactive protein, antistreptolysin-O titres, and endocan levels were examined in each group. Before anti-inflammatory therapy, endocan levels in the acute rheumatic fever group were not statistically significant to those in the control group, respectively (200.64 ng/L, 120.71 ng/L, P = 0.208). After anti-inflammatory therapy, endocan levels were significantly higher in the acute rheumatic fever group than in the control group, respectively (260.87 ng/L vs. 120.71 ng/L, P < 0.01). A significant difference was found in endocan levels before and after anti-inflammatory therapy in the group of acute rheumatic fever, respectively (200.64 ng/L vs. 260.87 ng/L, P = 0.033). Endocan levels after anti-inflammatory therapy were statistically higher in the severe carditis group compared to those of the mild carditis group, respectively (344.56 ng/L vs. 191.01 ng/L, P < 0.01). Our study showed that serum endocan levels increased during the subacute phase of acute rheumatic fever. We suggest that serum endocan level can be used as a new biomarker to identify the degree of cardiac involvement in acute rheumatic fever.

Bilateral vocal cords palsy in adolescent Systemic Lupus Erythematosus patient

Background: Executive functions such as speech are uncommonly involved in systemic lupus erythematosus (SLE) presentation. We report a case of SLE who presented with a seizure followed by an inability to speak. Case description: A 17-year-old girl was presented with a sudden loss of voice for 2 days after a seizure following fever for 4 months. The patient was aware and could understand words but could not execute verbal sounds; the sound she produced was muffled. The generalized seizure occurred without any trigger, lasted 2 minutes, and stopped spontaneously. Fever occurred primarily at night which subsided without treatment. She also complained of severe hair loss. Patient with leucopenia, lymphopenia, anemia hemolytic, thrombocytopenia, proteinuria, hematuria, brain atrophy from magnetic resonance imaging (MRI) examination, and echocardiography revealed mild carditis and mild circumferential pericardial effusion. Immunological assay showed antinuclear antibodies (ANA) &gt;1:1000 with nuclear homogenous, low C3 complement, and positive Anti-dsDNA. Systemic Lupus International Collaborating Clinics (SLICC) 2015 total score was 14, and the European League Against Rheumatism (EULAR/ACR) 2019 total score was 31, confirmed patient for SLE diagnosis. Further exploration from fiberoptic laryngoscopy confirmed bilateral vocal cord palsy due to SLE. Management included high dose methylprednisolone, cyclophosphamide, and speech therapy which results in gradual improvement from producing mumble sounds, then words followed by sentences with nasal sounds. Laryngeal involvement is uncommonly reported as an SLE manifestation. Management includes steroids and immunosuppressive for pharmacological and speech therapy as an exercise for vocal cord palsy. Conclusion: The patient must be continuously monitored for complete resolution of the palsy.

Les complications cardiaques du rhumatisme articulaire aigu et ses facteurs de risque : étude de 50 observations

IntroductionAcute 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 methodsIt 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). ResultsWe 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. ConclusionCR 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.

The effectiveness and safety of ibuprofen and acetylsalicylic acid in acute rheumatic fever.

Acetylsalicylic acid (ASA) is a non-steroidal anti-inflammatory drug used in the treatment of acute rheumatic fever (ARF) and it can cause serious adverse effects. This study aimed to evaluate the clinical efficacy and side effects of ibuprofen in the treatment of ARF compared to the classic treatment, ASA. Children who were hospitalized for the treatment of ARF with isolated arthritis and mild carditis between October 2015 and October 2018 and who received non-steroidal anti-inflammatory therapy were evaluated in the study. We compared the demographic data, clinical findings, efficacy, and side effects of the treatments of the children, who were divided into ASA and ibuprofen groups. Of the 38 patients included in the study, 21 were treated with ASA (ASA group), and 17 were given ibuprofen (IBU group). There was no difference between the groups regarding the length of hospital stay, total treatment time, time to resolution of clinical symptoms, and normalization of acute-phase reactants. Liver enzymes increased during treatment in 13 patients (62%) in the ASA group and three patients (18%) in the IBU group (P = 0.009). In the ASA group, 69% of patients with increased liver enzymes were under 11 years of age during treatment. Clinical side effects were observed in three patients in the ASA group while none were observed in the IBU group. The results of this study suggest that ibuprofen can be a safe alternative in the treatment of ARF, especially in young children. Although ibuprofen can be a safe and effective alternative to ASA, studies including larger series are needed on this subject.

Pentraxin 3 levels and correlation with disease severity in patients with acute rheumatic fever

Objectives This study aims to investigate serum pentraxin 3 (PTX3) levels during acute episode of acute rheumatic fever (ARF) and their relationship with disease severity.Patients and methods The prospective study was conducted between January 2015 and December 2018 and included 52 ARF patients (22 girls, 30 boys, mean age 10.7±2.1 years; range, 5 to 16 years) experiencing an acute episode and 22 healthy children (13 girls, 9 boys, mean age 10.3±3.8 years; range, 5 to 16 years). ARF patients were classified into three groups based on the clinical course: isolated arthritis (n=17), mild carditis (n=19), and moderate/severe carditis (n=16). Blood samples were collected from all patients before treatment and from the healthy children in the control group to measure PTX3 levels. PTX3 was measured using sandwich enzyme-linked immunosorbent assay method.Results Plasma PTX3 levels were significantly higher in ARF group compared to the control group (4.7±5.2 and 1.2±1.7 ng/mL, p<0.001). Subgroup analysis of serum PTX3 levels in ARF patients with isolated arthritis, mild carditis, and moderate/severe carditis (3.2±3.1 ng/mL, 4.3±5 ng/mL, and 6.7±6.6 ng/mL, respectively) showed that serum PTX3 was significantly higher in the moderate/severe carditis group compared to the other groups (p<0.05). Analysis of echocardiographic data showed that serum PTX3 was positively correlated with left ventricular end-diastolic diameter, left atrial diameters, and mitral A velocity and negatively correlated with E/A ratio (p<0.05; r=0.231, 0.402, 0.562, -0.586, respectively).ConclusionHigh PTX3 level during an acute episode of ARF may help predict the clinical course and the severity of accompanying carditis. However, prospective studies with larger sample sizes are needed.

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.

The Case Files

The Case Files

A retrospective study: Acute rheumatic fever and post-streptococcal reactive arthritis in Japan

BackgroundAcute rheumatic fever (ARF) and post-streptococcal reactive arthritis (PSRA) are immune-mediated consequences of group A streptococcal pharyngitis. ARF has declined in developed nations. No prevalence survey of PSRA has been conducted. This study evaluated the incidence and characteristics of ARF and PSRA in Japanese children. MethodsFrom 2010 to 2015, ARF and PSRA were evaluated using clinical data retrospectively collected by chart review from 528 hospitals. ResultsFrom 323 hospitals (61% response rate), 44 cases of ARF and 21 cases of PSRA were reported. Patients with ARF and/or PSRA were mainly from large cities in Japan. The mean age of ARF occurrence was 8.5 years, and the ratio of female/male patients was 16:28. Major manifestations in the acute phase included carditis, 27 cases (61.4%); polyarthritis, 22 cases (50%); erythema marginatum, 7 cases (15.9%); Sydenham chorea, 3 cases (6.8%); and subcutaneous nodules, 1 case (2.3%). Twenty-one (58.3%) patients had migratory arthritis. During the follow-up period, 6 patients (13.6%) showed mild carditis. For PRSA, the mean age was 8.2 years, and the ratio of female/male patients was 12:9. Six (28.6%) patients had monoarthritis, and 4 (19%) patients had migratory arthritis. No patient had carditis. ConclusionsAlthough ARF and PSRA are rare in the Japanese pediatric population, substantial numbers of patients with both conditions were identified in this study. We observed a high incidence of arthritis and carditis in ARF patients. No PSRA case was complicated with carditis. General pediatricians need to have updated information about ARF and PSRA, even in industrialized countries.

Jaccoud's Arthropathy with Radial Shaft Deformity in a Patient with a History of Mild Carditis and Sydenham's Chorea

Background: Jaccoud’s arthropathy was first described in 1869 in a patient with recurrent attacks of acute rheumatic fever and recurrent migratory polyarthritis. The typical features include characteristic joint deviation with metacarpophalangeal subluxation, ulnar deviation, swan neck deformity of the fingers, and Z deformity of both thumbs and hook formation and alignment disorders without noticeable erosions on radiographs. In this report, Jaccoud’s arthropathy presenting with deformity of shaft of the radius is described. Case presentation: A 12-year-old-girl was diagnosed to have chorea at the age of 8 years after she presented with irregular, jerky, rapid and involuntary movements that involved the face, the trunk and the distal extremities. She was treated with haloperidol. The chorea resolved after 3 months of treatment. Echocardiogram showed thickening of the mitral valve leaflets and mild mitral regurgitation. Three years later, the child presented with progressive symmetric deformity of the wrist joints. She reported no pain or limitation of movement. On physical examination, there was no cardiac murmur. There was varus deformity of both wrist joints. There was no tenderness or limitation of movement. Anti-streptolysin O was negative at the first presentation and on this visit. Rheumatoid factor was negative but antinuclear antibody test was positive. Echocardiogram showed complete resolution of the mitral regurgitation. X-ray of the wrists showed marked symmetric curving of the distal radius and varus deformity of the wrists. There was joint deviation with metacarpophalangeal subluxation and ulnar deviation of the hand. There was no Z deformity of the thumbs. Conclusion: The common presentation of Jaccould’s arthropathy is involvement of the wrist, metacarpophalangeal joints and joints of the feet. The metacarpal and metatarsal bones are the ones, which are commonly affected. Our patient has an unusual presentation of deformity of the distal radial bone. This has not been described, at least to our knowledge.

The resurgence of rheumatic fever in a developed country area: the role of echocardiography

The annual incidence of ARF ranges from 5 to 51/100, 000 population worldwide in the 5- to 15-year age group. In the past, there was a decline in the incidence of ARF; however, focal outbreaks have been reported. This study evaluated the incidence of ARF in 2007-08 in a region of a developed country compared with the previous decade. A retrospective review of all admission records for ARF in Trieste between January 2007 and December 2008 was undertaken. The diagnosis of ARF was established by the Jones criteria according to the 1992 revision. Between January 2007 and December 2008: 13 cases of ARF were recorded, 11 females and 2 males. The estimated incidence was 23 and 27/100, 000 population new cases each year, respectively, in the 5- to 15-year age group. Migratory polyarthritis occurred in 6/13, chorea in 7/13 and clinical carditis in 5/13 cases. Five out of 13 patients had only echocardiographic abnormalities, with no clinical cardiac manifestations. Another two patients did not fulfil diagnostic criteria for ARF, presenting with only three minor criteria, but they revealed silent carditis at echocardiography evaluation. During the follow-up, in one case the carditis receded and in the other it significantly improved. Our experience underlines that ARF has not yet disappeared in industrialized countries. We observed a high incidence of chorea, always associated with mild carditis. Echocardiographic assessment should be routinely performed in all patients with suspected ARF in order to identify those subclinical cases of valvulitis that would otherwise pass undiagnosed without receiving proper prophylaxis.

The pattern of acute rheumatic fever in children: Experience at the children’s hospital, Riyadh, Saudi Arabia

Background The study was carried out in Riyadh City Hospital to determine the hospital prevalence of acute rheumatic fever (ARF), its characteristics and to determine the proportion of the ARF population that have recurrent attacks. Methods The study was an analysis of 83 children with ARF, admitted to the Children’s Hospital, Riyadh, over a 10-year period (1994–2003). The diagnosis of ARF was based on clinical features as defined in the modified Jones criteria with evidence of recent streptococcal infection. The diagnosis of recurrence of rheumatic fever in children with rheumatic heart disease was based on the presence of one major criterion apart from carditis or two minor criteria, in addition to evidence of preceding streptococcal infection. Results The mean age at presentation was 9 years. In 31 (37%) cases, arthritis was the only major Jones criterion. In 30 (36%) others, arthritis was associated with carditis and in 3 (4%), with chorea. Cardiac involvement was documented in 44 (53%) cases; it occurred alone in 5 (6%), with arthritis in 30 (36%), and with chorea in 9 (11%) others. Among the 44 with carditis, the pattern of cardiac involvement was valvular only (mild carditis) in 30 (68%), while it was severe in the remaining 14 (32%) cases who also had heart failure. The involvement of the mitral valve alone occurred in 26 (59%) cases in the form mitral regurgitation, while both aortic and mitral valve regurgitation were present in 11 (25%) cases, and aortic valve regurgitation alone in four (9%) others. Chorea was the only major criterion of ARF in 5 children (6%), while it occurred in association with other major criteria in 12 (15%) others. Nineteen (23%) children had recurrent attacks of ARF. Conclusion ARF continues to occur in Saudi Arabia in the period (1994–2003), despite the progress made in the socio-economic development of the country , and this is often associated with severe cardiac involvement.

Changes of manifestations of 122 patients with rheumatic fever in South China during last decade

The main objective of this study is to investigate changes of features of rheumatic fever (RF) in recent 10 years. A total of 315 patients with RF during 1985–1995 (group 1) and 1997–2007(group 2) were selected. Their manifestations were compared. Results show that the female/male ratio was 2.0. Group 2 had higher rate of low-grade fever and carditis, and lower rate of heart failure, lower positive rate of C-reactive protein and antistreptolycin o than group 1. In group 2, 61.4% patients fulfilled the updated Jones criteria, however, 76.2% fulfilled 2002–2003 WHO criteria. Diagnosing rheumatic carditis, sensibility and specificity of lymphocyte procoagulant activity (PCA) were 79.1 and 71.4%, respectively, and those of antibody to streptococcal polysaccharide (ASP) were 70.3 and 70%, respectively. Follow-up data of 35 cases were available. Recurrent rate of RF was 62.8%. Only 1/3 cases received regular secondary prevention. In conclusion, mild carditis was increasing. PCA and ASP were valuable tests for diagnosing rheumatic carditis. Atypical cases and secondary prevention need more attention.

Association of polymorphisms within the promoter region of the tumor necrosis factor-α with clinical outcomes of rheumatic fever

Rheumatic fever (RF)/rheumatic heart disease (RHD) is an inflammatory disease with a complex etiology in which Group A streptococci within a genetically susceptible host untreated for strep-throat may deviate the innate and adaptive arms of the immune system towards recognition of autoantigens. The TNFA gene has been associated with a number of autoimmune diseases, including RF. We investigated whether the G−308A and G−238A polymorphisms of the TNFA gene are associated with clinical outcomes of RF in a cohort of 318 patients and 281 healthy controls (HC). Both polymorphisms showed borderline associations with RF ( TNFA −308G/A, OR = 1.4 [1–2.2], P = 0.026; TNFA −238G/A, OR = 1.9 [1–3.3], P = 0.015). The presence of either one of the minor alleles (−308A and −238A) was more common among patients with RF/RHD than controls ( P = 0.0006). Stratification of patients according to clinical phenotype also showed significant associations between presence of either one of the minor alleles and RHD ( Pc = 0.0006) when compared with controls. This association was stronger with the development of aortic valve lesions. In contrast, there was no association between genotype and Sydenham's chorea or RF patients with mild carditis. In conclusion, we show that the TNFA is a susceptibility locus for RF. The ability to predict which RF patients will develop valve lesion may have therapeutic, economic and social implications.

Carditis in patients with gastro-oesophageal reflux disease: results of a controlled study based on both endoscopy and 24-h oesophageal pH monitoring.

There are conflicting reports on the role of gastro-oesophageal reflux disease (GERD) and Helicobacter pylori infection in the aetiology of carditis. The role of reflux and H. pylori infection in causing carditis was assessed in 113 consecutive patients with GERD and in 25 controls. All subjects underwent endoscopy and pH test and carditis was diagnosed on biopsies taken across the squamocolumnar junction. Helicobacter pylori was assessed by histology and rapid urease test. GERD was diagnosed by endoscopic oesophagitis or abnormal pH test. Carditis was detected in 53 of 71 GERD patients and in 15 of 20 controls. Among patients, 18 showed absent, 39 mild and 14 marked cardia inflammation and their H. pylori infection rates were 17, 23 and 57%, respectively (P < 0.025). Most patients with carditis (68%) lacked H. pylori infection. pH-metry was abnormal in 15 of 18 patients with normal cardia, 33 of 39 with mild carditis and 12 of 14 with marked inflammation. Carditis is a frequent finding in GERD and controls. Mild, non-active carditis is frequent in GERD patients. Marked inflammation is associated with both H. pylori and abnormal pH testing. Thus, both GERD and H. pylori infection may play a role in inducing carditis.

Subclinical rheumatic valvitis: a long-term follow-up

In order prospectively to investigate the frequency and evolution of subclinical valvitis, we selected 40 consecutive patients suffering their initial attack of rheumatic fever, seen in our clinic from 1992 to 1994, and followed-up until 2001, with a mean period of follow-up of 8.1 years, and a standard deviation of 0.6 year. We also assembled a matched control group of 37 healthy children and adolescents. We discovered a murmur of mitral regurgitation in 28 (70.0%) of the patients. In 3 (7.5%) of these patients, there was also a murmur of aortic regurgitation. In the group of 28 symptomatic patients, Doppler echocardiography showed mitral regurgitation in all, and aortic regurgitation in 17. In the group of 12 patients without clinical evidence of cardiac involvement, Doppler echocardiography identified mitral regurgitation in 2, isolated in one and associated with aortic regurgitation in the other. Thus, the frequency of subclinical valvitis was 16.7%. In patients with subclinical valvitis only the aortic regurgitation regressed during the period of follow-up. In the group of 28 symptomatic patients, mitral regurgitation disappeared in 6 (21.4%), aortic regurgitation in 7 of the 17 having this feature (41.2%), while 2 patients (7.1%) developed mitral stenosis. The sensitivity and specificity of cardiac auscultation were, respectively, 93.3%, with 95% confidence intervals between 72.3% and 97.4%, and 100%, with 95% confidence intervals between 65.5% and 100%, for the diagnosis of mitral regurgitation, and 16.7%, with 95% confidence intervals between 4.4% and 42.3%, and 100%, with 95% confidence intervals between 81.5% and 100%, for that of aortic regurgitation. We conclude that the Doppler echocardiogram is an important means of diagnosing and assessing the evolution of subclinical rheumatic valvar lesions, which are not always transient. We suggest that Doppler echocardiography should be performed in all patients with acute rheumatic fever. Subclinical valvitis should be considered as mild carditis, provided that strict criterions are observed in the differential diagnosis from physiological regurgitation, and Doppler echocardiographic findings are analyzed in the context of the other manifestations of the disease.

Clinical profile of acute rheumatic fever in Pakistan.

We designed a multi-hospital prospective study of children less than 12 years to determine the comparative clinical profile, severity of carditis, and outcome on follow up of patients suffering an initial and recurrent episodes of acute rheumatic fever. The study extended over a period of 3 years, with diagnosis based on the Jones criteria. We included 161 children in the study, 57 having only one episode and 104 with recurrent episodes. Those seen in the first episode were differentiated from those with recurrent episodes on the basis of the history. The severity of carditis was graded by clinical and echocardiographic means. In those suffering their first episode, carditis was significantly less frequent (61.4%) compared to those having recurrent episodes (96.2%). Arthritis was more marked in the first episode (61.4%) compared to recurrent episodes (36.5%). Chorea was also significantly higher in the first episode (15.8%) compared to recurrent episodes (3.8%). Sub-cutaneous nodules were more-or-less the same in those suffering the first (7%) as opposed to recurrent episodes (5.8%), but Erythema marginatum was more marked during the first episode (3.5%), being rare in recurrent episodes at 0.9%. Fever was recorded in approximately the same numbers in first (45.6%) and recurrent episodes (48.1%). Arthralgia, in contrast, was less frequent in first (21.1%) compared to recurrent episodes (32.7%). A history of sore throat was significantly increased amongst those suffering the first episode (54.4%) compared to recurrent episodes (21.2%). When we compared the severity of carditis in the first versus recurrent episodes, at the start of study mild carditis was found in 29.8% versus 10.6%, moderate carditis in 26.3% versus 53.8%, and severe carditis in 5.3% versus 31.8% of cases, respectively. At the end of study, 30.3% of patients suffering their first episode were completely cured of carditis, and all others showed significant improvement compared to those with recurrent episodes, where only 6.8% were cured, little improvement or deterioration being noted in the remainder of the patients. We conclude that the clinical profile of acute rheumatic fever, especially that of carditis, is milder in those suffering their first attack compared to those with recurrent episodes.

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.

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. ARF exists in the paediatric Greek population with exacerbations and remissions, but the cardiac manifestations appear mild or moderate.

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.

Relationships between coronary artery dilatation and severity of carditis detected by two-dimensional echocardiography and [99mTc]HMPAO-labeled white blood cell heart scan in children with Kawasaki disease.

Forty-one children (15 girls, 26 boys; ages 3.0 +/- 2.1 years) with Kawasaki disease (KD) were included in our study. The diameters of a total of 82 coronary arteries (CA), comprising 41 left (LCA) and 41 right (RCA), were computed by two-dimensional echocardiography (2D-Echo). If the diameter of the CA was > or = 20 mm, it was considered as dilated. The severity of the carditis was evaluated by [99mTc]HMPAO-labeled white blood cell (WBC) heart scans (Tc-WBC) carried out in all cases. On the basis of the results the children were divided into three groups. Group A children had the most severe carditis where the heart uptake was greater than the bone uptake; 11 of 32 (34.3%) of the CA in 16 cases were dilated. Group B had carditis of medium severity where the heart uptake was equal to the bone uptake; 11 of 28 (39.3%) of the CA in 14 cases were dilated. Group C had mild carditis where the heart uptake was less than the bone uptake; 9 of 22 (40.9%) of the CA in 11 cases were dilated. There were no significant differences among the three groups (p = 0.87 by a chi-squared test). We conclude that no definite relationship exists between the severity of carditis and the dilatation of the CA in children with KD.