Acute rheumatic fever in western Pennsylvania and the tristate area

Acute rheumatic fever is reported to have declined and perhaps be vanishing. Prompted by the occurrence of 17 cases of acute rheumatic fever in an 18-month period in 1985 and 1986, we reviewed the records of 243 children with acute rheumatic fever who were cared for at Children's Hospital of Pittsburgh or Mercy Hospital between 1965 and 1986. Acute rheumatic fever was diagnosed using the modified Jones criteria and cases were classified by major criteria as arthritis, arthritis and carditis, carditis alone, carditis and chorea, chorea alone, and arthritis and chorea. Among the 17 recent patients, 59% had carditis, 30% had chorea, and 24% had arthritis alone. The proportion of children who had particular major manifestations was similar in the last two decades and in 1985 to 1986. The recent children with acute rheumatic fever ranged in age from 6 to 13 years with a mean and median age of 10 years. There were 16 white children and one Asian child. Only four children lived in an urban setting. When demographic features of the children were contrasted with those in the previous two decades, a decrease in the proportion of children who lived in urban areas and who were black was noted. Four children had a history of preceding sore throat but only three sought medical care; nine children had no memorable illness and four had either a nonrespiratory illness or a respiratory infection without sore throat. This resurgence of rheumatic fever serves as a reminder that a diligent approach to the diagnosis and therapy of streptococcal infections remains essential.     

Acute rheumatic fever in New York City (1969 to 1988): a comparative study of two decades

One hundred fifteen attacks of acute rheumatic fever were observed during the past two decades (1969 to 1988) at the Columbia-Presbyterian Medical Center; 104 were initial attacks and 11 were recurrences. The maximum number of cases (18) occurred during the first year of this period, 1969; thereafter the annual incidence decreased until a resurgence was apparent in 1985 to 1986, with 25 cases. There were no differences in the frequency of major manifestations nor the severity of carditis in the 1970s compared with the 1980s. Polyarthritis alone was present in 51 cases, carditis alone in 31, and combined carditis and polyarthritis in 28; chorea was diagnosed in 5. Congestive heart failure occurred in 17 attacks of carditis, including one death from fulminant disease in 1982. In contrast to recent reports, the majority of the affected population was urban, low income, and from crowded communities: more than half were Hispanic, predominantly from Dominican Republic families. The prevalence of acute rheumatic fever underscores the need for early detection and treatment of streptococcal pharyngitis. The observation that 9.5% of the attacks were recurrent reflects failure to comply with antimicrobial prophylaxis and reaffirms the need for more effective secondary prevention programs.     

Acute rheumatic fever without early carditis: an atypical clinical presentation

The original Jones criteria, first introduced in 1944, have been modified four times and updated-revised criteria were published in 1992. A variety of clinical manifestations, which may be the presenting signs and symptoms of acute rheumatic fever, are not included in the updated-revised Jones criteria. A retrospective study was conducted on all children previously diagnosed to have acute rheumatic fever between September 1998 and September 2002. Review was focused on clinical presentation; out of 60 medical records reviewed, 4 patients with unusual clinical presentation were recognised and are reported here to highlight the potential diagnostic problems of acute rheumatic fever. They presented with atypical articular involvement, silent carditis and low-grade fever in the presence some time of a positive family history for rheumatic fever. Conclusion:a high index of suspicion and an awareness of the absence of early carditis are necessary to make the diagnosis of acute rheumatic fever.Abbreviations ARF acute rheumatic fever - PSReA post-streptococcal reactive arthritis - RF rheumatic fever     

Rheumatic fever in Nigerian children: a prospective study

The initial attack of acute rheumatic fever is hardly ever diagnosed in our environment. Most cases of acute rheumatic fever are seen during recurrent illness when cardiac damage is already severe and death from cardiac failure common. In the absence of effective primary prophylaxis against rheumatic fever in the foreseeable future it is important to find every case of acute rheumatic fever at the first attack, as on this would depend effective secondary prophylaxis and, hopefully, reduction of the morbidity and mortality rates. A prospective study was set up to achieve this aim. All cases suspected of having rheumatic fever among children seen at the Lagos University Teaching Hospital over a period of five years were subjected to the Jones' diagnostic criteria for diagnosis, and grouped into "Initial illness" and "Recurrent illness" groups. Twenty-one cases of acute rheumatic fever were diagnosed during the period, out of which ten (47.6%) were in the initial stage of the illness. There was a direct relationship between the severity of cardiac involvement and delay in recognition of the condition. It was concluded that efforts aimed at prompt recognition of the initial illness would be rewarding in minimising cardiac morbidity and mortality.     

Tropical acute rheumatic fever and associated streptococcal infections compared with concurrent acute glomerulonephritis.

Ninety-three patients with acute rheumatic fever and 195 patients with acute glomerulonephritis were observed in Trinidad during an outbreak of scabies with a high incidence of secondary streptococcal infections. Clinical and laboratory manifestations of ARF were the same as those seen in temperate zones, except that antistreptolysin O titers were less markedly increased. The patients with ARF were similar to those with AGN in respect to sex, race, location of residence, and living conditions, but were older and had markedly fewer skin infections. Currently prevalent nephritogenic streptococcal strains never were isolated from patients with ARF even when M55 streptococci appeared and led to an epidemic of AGN.     

Dermatoglyphic alterations associated with acute rheumatic fever in children

The dermatoglyphic configurations of 78 children with acute rheumatic fever were compared with those of 46 first-degree relatives and 1,310 normal subjects. Of the children with acute rheumatic fever, 75% had an ulnar deviation of the axial triradius. In about 40% of this group, the ulnar deviation was associated with a concomitant distal displacement, which resulted in a significantly higher mean maximal angle atd (P less than .001) and significantly lower mean ab and td ridge counts (P less than .001) relative to normal control values. The palmar dermatoglyphics of patients with acute rheumatic fever were more closely related to the configurations of first-degree relatives than to normal controls. The dermatoglyphic profiles of six patients were nearly identical to those of their first-degree relatives, all of whom had a history of acute rheumatic fever. Presence of abnormal dermatoglyphic profiles in a large proportion of children with acute rheumatic fever supports the hypothesis that certain individuals have a genetic predisposition to this disease.     

儿童风湿热及风湿性心瓣膜病85例临床分析

为观察儿童风湿热及风湿性心瓣膜病临床动态变化趋势,分析本院90年代诊治的85例风湿热及心瓣膜病发生状况、临床特点和治疗预防复发情况。结果示本组风湿热造成急性心瓣膜炎占77.65%,首诊慢性心瓣膜病占22.35%,其中三分之二患儿以往无急性风湿热病史。首次发病复发率为14.12%,风湿活动复发常以不同程度心力衰竭（心衰）症状就诊,激素抗风湿治疗能够迅速缓解急性心脏炎和改善心功能。1例反复风湿活动顽固性心力衰竭接受二尖瓣置换术效果甚佳。结果表明,减少儿童风湿性心瓣膜病形成不仅要控制急性风湿热,而且需要及时发现潜隐的风湿活动。     

Rheumatic chorea in northern Australia: a clinical and epidemiological study.

To describe the epidemiology and clinical features of Sydenham's chorea in the Aboriginal population of northern Australia a review was conducted of 158 episodes in 108 people: 106 were Aborigines, 79 were female, and the mean age was 10.9 years at first episode. Chorea occurred in 28% of cases of acute rheumatic fever, carditis occurred in 25% of episodes of chorea, and arthritis in 8%. Patients with carditis or arthritis tended to have raised acute phase reactants and streptococcal serology. Two episodes lasted at least 30 months. Mean time to first recurrence of chorea was 2.1 years compared with 1.2 years to second recurrence. Established rheumatic heart disease developed in 58% of cases and was more likely in those presenting with acute carditis, although most people who developed rheumatic heart disease did not have evidence of acute carditis with chorea. Differences in the patterns of chorea and other manifestations of acute rheumatic fever in different populations may hold clues to its pathogenesis. Long term adherence to secondary prophylaxis is crucial following all episodes of acute rheumatic fever, including chorea, to prevent recurrence.     

Rheumatic chorea in northern Australia: a clinical and epidemiological study

To describe the epidemiology and clinical features of Sydenham's chorea in the Aboriginal population of northern Australia a review was conducted of 158 episodes in 108 people: 106 were Aborigines, 79 were female, and the mean age was 10.9 years at first episode. Chorea occurred in 28% of cases of acute rheumatic fever, carditis occurred in 25% of episodes of chorea, and arthritis in 8%. Patients with carditis or arthritis tended to have raised acute phase reactants and streptococcal serology. Two episodes lasted at least 30 months. Mean time to first recurrence of chorea was 2.1 years compared with 1.2 years to second recurrence. Established rheumatic heart disease developed in 58% of cases and was more likely in those presenting with acute carditis, although most people who developed rheumatic heart disease did not have evidence of acute carditis with chorea. Differences in the patterns of chorea and other manifestations of acute rheumatic fever in different populations may hold clues to its pathogenesis. Long term adherence to secondary prophylaxis is crucial following all episodes of acute rheumatic fever, including chorea, to prevent recurrence.     

Diagnosis of rheumatic fever: Current status of Jones criteria and role of echocardiography

The original Jones Criteria as proposed by Dr. T. Duckett Jones have been modified four times and the updated revised criteria were published in 1992. According to this latest publication major manifestations are carditis, polyarthritis, chorea, erythema marginatum and subcutaneous nodules. Minor manifestations include fever, arthralgia and laboratory findings of elevated erythrocyte sedimentation rate, C-reactive protein and prolonged PR interval on ECG. For making a diagnosis of acute rheumatic fever, two major, or one major and two minor manifestations must be accompanied by supporting evidence of antecedent group A streptococcal infection in the form of positive throat culture or elevated or rising anti-streptolysin titre. The updated guidelines also highlighted a subgroup of “exceptions to Jones Criteria” for patients with chorea, indolent carditis and previous history of rheumatic fever or “rheumatic heart disease”. Role of echocardiography has not been defined in these modifications but may be important, as clinical detection of soft murmurs may be difficult due to tachycardia. Doppler and color flow mapping is more sensitive in picking up minor digress of valvular regurgitation. Several studies have confirmed that the yield of carditis with valvular regurgitation increased with use of echocardiography in patients with acute rheumatic fever. Also echocardiography is of great help in mixed valve lesions to determine the severity of each lesion. Other abnormalities detected on echocardiography in acute carditis include prolapse of the valve, focal nodular thickening of leaflets and pericardial effusion. Jones Criteria are guidelines to assist the physician and should not be substituted for clinical judgement as strictly following them may result in underdiagnosis of this disease in our country.     

The first attack of acute rheumatic fever in childhood--clinical and laboratory profile

One hundred consecutive cases of 'first attack' of acute rheumatic fever were studied. There were 52 males and 48 females, constituting 1.12% of total hospital admissions. Nearly 10% of children were below the age of 5 years, stressing the early onset of rheumatic fever in tropics. Only 47% gave a definite history of overcrowding at home. Sore throat was present in 67%, overt arthritis in 66%, carditis in 57%, arthralgia alone in 22% of which 45.45% had carditis. Small joint involvement was noticed in 23% of cases of which 73.91% had carditis. Only 33.33% had congestive cardiac failure. Ten per cent of children had chorea, while subcutaneous nodules were seen in 4% of cases, all of whom had associated carditis. Erythrocyte sedimentation rate (ESR) showed good correlation with clinical profile. Throat cultures were positive for beta hemolytic streptococci only in 12% of cases. Anti-streptolysin 'O' (ASO) titre showed significant titres on 68% of cases, anti-deoxyribonuclease "B" (ADN-B) in 69.32%, antibody to group A carbohydrate (ACHO) in 70.65%. ASO, ADN-B, and ACHO titres together gave 87.5% positivity while estimations in paired sera showed ASO 79.54%, ADN-B 82.27% and ASO, ADN-B together 99.92% significant titres. Study of blood groups showed A group children to be more vulnerable to rheumatic fever (37.5%) and rheumatic carditis (47.37%). Mortality in the present study was nil.     

Acute rheumatic fever during childhood in Kuwait: the mild nature of the initial attack

A prospective clinical study is reported of the initial attack of acute rheumatic fever in 210 children seen in Kuwait over a period of four years. The main presenting feature was pain in the joints, caused by arthritis in 79% of our patients and by arthralgia in 15%. Five per cent presented with chorea alone and 1% with congestive heart failure alone. Carditis was detected in 46.2%, cardiomegaly in 10%, pericarditis in 1.4% and congestive heart failure in 4.8%. The incidence of chorea was 7.6%, of erythema marginatum 1% and of subcutaneous nodules 0.5%. The mortality rate was 0.5%. Data from this study reflect the mild nature of acute rheumatic fever in Kuwait, in contrast to the aggressive nature of the disease described from neighbouring and developing countries.     

Intravenous immunoglobulin in acute Sydenham's chorea: A systematic review

Sydenham's chorea (SC) is a major manifestation seen in 25% of patients with acute rheumatic fever. SC is the prototypic autoimmune neurological disorder, which has a less appreciated associated risk of psychiatric morbidity. We undertook a systematic review to examine whether the use of intravenous immunoglobulin affects clinical recovery and morbidity.     

炎症小体病35例临床特征分析

目的:总结炎症小体病临床特点,提高儿科临床医生对该类疾病的认识,帮助早期诊断。方法:回顾性总结2008年1月1日至2020年12月31日由北京协和医院儿科诊断的35例炎症小体病患儿的发热、皮疹、受累系统情况以及实验室检查结果等临床特征。结果:35例炎症小体病患儿中男20例、女15例,起病年龄为1（0,7）岁,诊断年龄为...     

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目的探讨儿童感染性心内膜炎(IE)的临床特点、治疗方法及转归。方法回顾分析中南大学湘雅二医院儿科1980—2004年确诊的174例IE患儿的临床特点。174例患儿中男94例,女80例,年龄3个月至14岁,病程3d至4个月。结果(1)99例IE发生在先天性心脏病的基础上,35例发生在风湿性心脏病的基础上,35例发生在无器质性心脏病基础上,4例于先天性心脏病手术后发生,1例发生在肥厚性心肌病的小儿。(2)临床主要表现:发热(150例,86·2%)、肝大(74例,42·5%)、脾大(55例,31·6%)、贫血(65例,37·4%)、血沉增快(68例,39·1%)、多发性脏器栓塞(34例,17·8%)。细菌培养76例(76/174)阳性,阳性率43·7%,其中55例为葡萄球菌。(3)受累瓣膜以二尖瓣赘生物最多见,占48·3%。(4)并发症以顽固性心衰为主(25·2%),其次为神经系统并发症(13·2%),瓣膜腱索断裂最少见(0·57%)。(5)由于IE临床表现很不典型,病例早期被误诊为流感、肺结核、急性风湿热、肾小球肾炎等10余种疾病。(6)治愈率为60·9%,其中单用抗生素治疗治愈76例(71·6%),抗感染治疗联合外科手术治愈30例(28·3%)。死亡28例(16·1%),脑栓塞及顽固性心衰是IE最常见的死亡原因。结论发生IE的基础心脏病中,先天性心脏病跃居首位,风湿性心脏病逐渐减少。血培养、超声心动图检查有助于IE的诊断。对于IE最重要的治疗措施是应用抗生素,若经内科治疗效果不满意应尽早考虑外科治疗。     

What is the True Frequency of Carditis in Acute Rheumatic Fever? A Prospective Clinical and Doppler Blind Study of 56 Children with up to 60 Months of Follow-Up Evaluation

BACKGROUND: This study aimed to evaluate prospectively clinical and echocardiographic findings of patients who had rheumatic fever with and without clinical features of cardiac involvement. METHODS: For this study, 56 consecutive patients (mean age, 11.4 years) with acute rheumatic fever diagnosed according to the 1992 modified Jones criteria were evaluated at diagnosis, after 3 and 6 months, then at 2 and 5 years. All assessments were performed blindly and included physical and cardiac examination, electrocardiogram, chest X-ray, and two-dimensional color-flow Doppler echocardiography. RESULTS: Initial clinical carditis was observed for 27 patients (48.2%), all of whom had positive echocardiographic abnormalities. Echocardiographic abnormalities were observed in 11 patients who had arthritis or chorea presentation without initial clinical carditis. Persistence of the abnormalities was observed at a late follow-up evaluation in 72.7% of the cases. Sydenham's chorea was present in 8 patients with clinical carditis and in 10 without this disorder, 8 of whom had echocardiographic abnormalities. CONCLUSION: Patients who had acute rheumatic fever without clinical signs of carditis showed acute and late follow-up echocardiographic abnormalities suggestive of cardiac involvement. Clinicians should be attentive for the presence of cardiac involvement among patients with chorea.     

P-Wave Dispersion in Children With Acute Rheumatic Fever

As a new and simple electrocardiographic marker, P-wave dispersion is reported to be associated with inhomogeneous and discontinuous propagation of sinus impulses. The current study aimed to investigate P-wave dispersion in children with acute rheumatic fever. The study population consisted of 47 children with acute rheumatic fever (29 patients with carditis and 18 patients without carditis) and 31 healthy control subjects. Maximum and minimum P-wave durations were measured from the 12-lead surface electrocardiogram. The P-wave dispersion was calculated as the difference between maximum and minimum P-wave durations. The maximum P-wave duration and the P-wave dispersion of the patients with and without carditis were significantly greater than those of the control subjects. The P-wave dispersion of the patients with carditis was significantly greater than that of the patients without carditis. In conclusion, the P-wave dispersion was higher in the children with acute rheumatic fever than in the healthy control subjects.