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.     

Reversibility of mitral regurgitation following rheumatic fever: Clinical profile and echocardiographic evaluation

The clinical disappearance of the murmur of rheumatic mitral regurgitation after period of time has been documented by many researchers. However no studies have related the disappearance of the murmur with the functional or anatomical state of the mitral valve. This study was done to elucidate the mitral valve status using doppler and color coded echocardiography among those children who have lost their apical pansystolic murmur on auscultation following a documented attack of rheumatic fever. The study sample consisted of 51 patients including 31 patients in whom the murmur has disappeared (group I), and 20 patients with persistent isolated mitral regurgitation (group II). Patients of group I had significantly lower grades of murmur intensity, lower incidence of cardiomegaly, and had no heart failure in the initial attack. They were more compliant with prophylaxis and had less recurrences than patients of group II. The murmur disappeared in patients of group I from 1/2 to 14 years after the initial attack. Echocardiography revealed that such patients had a normal mitral valve apparatus, and a normal heart size and function. Only 5 patients of this group had a significant regurgitant jet demonstrated by colour doppler. We concluded that recovery of the mitral valve and return of cardiac functions to normal is possible in patients who had mitral regurgitation following rheumatic fever. Some of them may still have an inaudible mild regurgitation. Patients who have lost their murmur may be allowed to exorcise freely, yet penicillin prophylaxis should not be discontinued.     

小儿急性风湿热的实验室与心脏器械检查及分析

通过有关实验室与心脏器械检查,以发现急性风湿热(ARF)诊断的新指标。方法对114例 ARF患儿,检查抗链球菌溶血素“O”(ASO),其中60例做了咽部A组β溶血性链球菌快速鉴定(GABHSRA), 且对二者阳性率进行对比;将114例的肌酸激酶同功酶(CK-MB)、多普勒超声心动图,与健康儿童对比。 结果ARF患儿GABHSRA阳性率为90％(54/60),显著高于ASO阳性率65.8％(75/114)。114例ARF患儿 CK-MB升高者46.5％,α-羟丁酸脱氢酶升高者32.7％;超声心动图左房增大者23.7％,左室扩大者16.7％,右 室扩大者7.1％;二尖瓣增厚者13.2％,主动脉瓣增厚者8.8％;多普勒超声心动图检查二尖瓣返流者29.8％, 主动脉瓣返流者9.6％,与健康儿童相比,差异均有显著性意义。34例有二尖瓣返流患儿中29例二尖瓣返流流 速时间积分增大。结论GABHSRA、心肌酶和多普勒超声心动图检查有助于ARF的诊断与病情了解。     

Antistreptolysin O titer profile in acute rheumatic fever diagnosis

OBJECTIVE: To determine ASO titer profile by establishing ARF differential diagnoses of other diseases with high levels of ASO antibodies. METHODS: We investigated 78 patients with ARF at onset and follow-up, 22 with isolated chorea at onset, 45 with recurrent oropharyngeal tonsillitis, and 23 with recent flare of juvenile idiopathic arthritis. We tested ASO with automated particle-enhanced immunonephelometric assay (Behring(R)-Germany). The ASO (IU/ml) titers were assessed at the following time intervals: 0-7 days, 1-2 weeks, 2-4 weeks, 1-2 months, 2-4 months, 4-6 months, 6-12 months, 1-2 years, 2-3 years, 3-4 years, and 4-5 years after onset of ARF. RESULTS: ASO titers in patients diagnosed with ARF had a significant increase up to the 2-4-month time interval (P<0.0001). Baseline levels were observed afterwards in patients under regular penicillin prophylaxis. The levels of ASO in ARF were also significantly higher than in patients with isolated chorea, recurrent oropharyngeal infections or juvenile idiopathic arthritis (P=0.0025), when age-matched samples of these groups were compared. The testacute;s sensitivity was 73.3% and the specificity was 57.6%, and it was calculated taking into account the upper limit of normality at 320 IU/ml, as well as the established diagnosis of ARF. The testacute;s specificity and positive predictive value increased with rising or higher titers, being higher with titers above 960 UI/ml. CONCLUSION: This reappraisal of ASO profile in ARF patients indicates a remarkable response during the acute phase, and that points to the extent to which ASO levels may differentiate ARF from other diseases with high levels of ASO antibodies, as coincidental but unrelated streptococcal infection or chronic arthritis flareup.     

Atrioventricular conduction in children with acute rheumatic fever.

Atrioventricular conduction was quantitatively evaluated in 118 children with acute rheumatic fever. The mean PR index in children with acute rheumatic fever, 1.06 +/- 0.38, was significantly higher than normal children or children who had febrile illness of nonrheumatic or nonstreptococcal origin (P is less than .001). Among 35 children with rheumatic fever and an abnormal PR index, the disease presented as carditis in 21, arthritis in ten, and chorea in four. The mean PR index and the frequency distribution of abnormal PR indices were significantly higher in children with carditis (P is less than .001). Five children who initially had an abnormal PR index and arthritis or chorea subsequently developed carditis. These observations suggest that children with acute rheumatic fever and abnormal PR index warrant close observation for possible clinical evidence of myocardial involvement during subsequent course of the illness.     

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.     

Erroneous diagnoses in children referred with acute rheumatic fever

We reviewed 53 patients referred to a pediatric rheumatology clinic in Asuncion, Paraguay. In 6 patients, a diagnosis of rheumatic fever was confirmed and in 47 patients other clinically significant diagnoses were made. Eighteen children had nonspecific findings and did not develop a rheumatologic condition on follow-up. Overdiagnosis of rheumatic fever can falsely inflate incidence and prevalence statistics and clinically significant diagnoses may be overlooked.     

Incidence of acute rheumatic fever in New Zealand children and youth

Aim: To estimate acute rheumatic fever (ARF) incidence rates for New Zealand children and youth by ethnicity, socioeconomic deprivation and region. Methods: National hospital admissions with a principal diagnosis of ARF (ICD9_AM 390‐392; ICD10‐AM I00‐I02) were obtained from routine statistics and stratified by age, ethnicity, socioeconomic deprivation index (NZDep2006) and District Health Board (DHB). Results: The mean incidence rate for ARF in 2000–2009 peaked at 9 to 12 years of age. Incidence rates for children 5 to 14 years of age for Māori were 40.2 (95% confidence interval 36.8, 43.8), Pacific 81.2 (73.4, 89.6), non‐Māori/Pacific 2.1 (1.6, 2.6) and all children 17.2 (16.1, 18.3) per 100 000. Māori and Pacific incidence rates increased by 79% and 73% in 1993–2009, while non‐Māori/Pacific rates declined by 71%. Overall rates increased by 59%. In 2000–2009, Māori and Pacific children comprised 30% of children 5–14 years of age but accounted for 95% of new cases. Almost 90% of index cases of ARF were in the highest five deciles of socioeconomic deprivation and 70% were in the most deprived quintile. A child living in the most deprived decile has about one in 150 risk of being admitted to the hospital for ARF by 15 years of age. Ten DHBs containing 76% of the population 5 to 14 years of age accounted for 94% of index cases of ARF. Conclusions: ARF with its attendant rheumatic heart disease is an increasing public health issue for disadvantaged North Island communities with high concentrations of Māori and/or Pacific families.     

Clinico-laboratory profile and outcome of Japanese encephalitis in Nepali children

BACKGROUND: Japanese encephalitis (JE) is associated with high mortality and neurological sequelae. The unpredictable course and lack of specific treatment pose major challenges in management. The tropical climate and paddy ecosystem in Nepal provide a suitable setting. AIMS: To determine the aetiology of febrile encephalopathy and describe the clinico-laboratory profile and outcome of JE in Nepali children. METHODS: A hospital-based prospective and observational study was conducted over a 1-year period (2000-2001). Children aged from >1 month to 14 years with fever >38 degrees C for <2 weeks and altered sensorium were recruited. JE was confirmed by anti-JE IgM in cerebrospinal fluid and/or serum. RESULTS: Of 117 consecutively enrolled patients, 58 had JE. Ten patients had concomitant infections, four with malaria and six with bacterial meningitis, and were excluded from analysis. Clinical findings were as follows: boys, 69%; age 4-14 years, 71%; presentation during summer and autumn, 83%; fever >3 days, 69%; altered sensorium <2 days, 50%; Glasgow coma score 8-12, 63%; seizures, 58%. Four (8.3%) died. At discharge, neurological sequelae were detected in 24 (50%) and hemiparesis was the most common form. Longer duration of vomiting, altered sensorium and focal neurological deficit on admission were independently associated with sequelae at discharge. Sequelae persisted in nine (18.8%) at 6 weeks follow-up. Long duration of altered sensorium (beta co-efficient 0.35, odds ratio 1.4, p=0.042) and presence of focal neurological deficit on admission (beta co-efficient 1.6, odds ratio 5.2, p=0.049) were independent predictors of sequelae at 6 weeks. CONCLUSION: JE was the commonest cause of febrile encephalopathy. Neurological sequelae were common but resolved in two-thirds of cases.     

Cytokines in acute rheumatic fever

Plasma concentrations of inflammatory cytokines (IL-1α, IL-1β, IL-6, IL-8 and TNFα) were determined by ELISA in 27 patients with acute rheumatic fever (RF), 12 with only arthritis (RFA) and 15 with rheumatic heart disease (RHD), before, during and after treatment. Altogether, significant increases in TNFα, IL-8 and IL-6 levels were observed in the acute phase as compared to the data found during and after treatment. No significant differences were observed for the other cytokines. Elevations of one or more of the inflammatory cytokines were observed in 9 of 12 patients with RFA, and 12 of 15 with RHD. Increase of TNFα (6/9) and IL-8 (5/9) levels were higher in RHD patients with cardiac failure. These cytokines were below the detection limits on day 7 of treatment in all 22 patients, except in two, and in all 10 days after treatment. Conclusions?These findings suggest that inflammatory cytokines, as TNFα, IL-8 and IL-6, may play a patho‐genic role in rheumatic fever.     

Cytokines in acute rheumatic fever

 Plasma concentrations of inflammatory cytokines (IL-1α, IL-1β, IL-6, IL-8 and TNFα) were determined by ELISA in 27 patients with acute rheumatic fever (RF), 12 with only arthritis (RFA) and 15 with rheumatic heart disease (RHD), before, during and after treatment. Altogether, significant increases in TNFα, IL-8 and IL-6 levels were observed in the acute phase as compared to the data found during and after treatment. No significant differences were observed for the other cytokines. Elevations of one or more of the inflammatory cytokines were observed in 9 of 12 patients with RFA, and 12 of 15 with RHD. Increase of TNFα (6/9) and IL-8 (5/9) levels were higher in RHD patients with cardiac failure. These cytokines were below the detection limits on day 7 of treatment in all 22 patients, except in two, and in all 10 days after treatment. Conclusions These findings suggest that inflammatory cytokines, as TNFα, IL-8 and IL-6, may play a patho‐genic role in rheumatic fever. Received: 10 October 1995 / Accepted: 2 July 1996