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.     

Alport's syndrome in 78 patients: epidemiological and clinical study

In a nationwide study in Finland, 78 patients, 38 male and 40 female in 25 families, were found to have Alport's syndrome, corresponding to 1 in 53 000 live births. This frequency of clinically manifest Alport's syndrome was much lower than expected from earlier reports. The first sign of the disease was most often haematuria, but was sometimes proteinuria or hearing loss. These signs were detected at a similar median age in both boys and girls, namely 6.2 and 6.0 years, respectively. The patients were followed up over a median period of 12.1 years (range 0.1-34.0 years). The clinical course of the disease was more severe in the male subjects than in the female subjects: 53% of the males and 13% of the females developed terminal renal failure at median ages of 24.9 and 31.1 years, respectively. At the last observation, 34% males and 78% females were free of renal insufficiency at median ages of 10.3 and 26.8 years. Hearing loss was detected in 74% of the males and 5% of the females. Regarding the rate of deterioration of renal function, no statistically significant difference was noticed between males and females. The routine use of dialysis and transplantations has dramatically changed the life expectancy of the patients.     

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.     

Childhood brucellosis--a microbiological, epidemiological and clinical study

A total of 5726 blood specimens (from children aged 14 years and younger) were studied for the serological evidence of brucellosis. Ninety-three (1.6 per cent) showed diagnostic agglutinin titres with a geometric mean titre of 403 (SD +/- 547). Forty-three (59.7 per cent) blood specimens yielded the growth of Brucella melitensis. Thirty-nine patients (41.93 per cent) were shepherds, who constituted the major occupational group affected in the present series. More than 60 per cent of the patients had a history of both consumption of fresh goat's milk and close animal contact. The habit of consuming fresh goat's milk to obtain relief from chronic ailments was noted in nine patients. Seventy-three (78.49 per cent) were males and 20 (21.51 per cent) were females, with a male to female ratio of 3:1. The disease occurred mainly in the school age group (mean age 10.3 years). All the patients had an acute history of less than 2 months. Forty-nine (52.68 per cent) patients presented with persistent fever, 19 (20.43 per cent) with joint pain, and the rest with a combination of fever and joint pain with and without low backache, fever being the commonest complaint. One case presented with involuntary movements of limbs alone and the other with burning feet only. Pityriasis alba was the consistent physical finding, with fever in the majority of the patients. The major joint found to be involved was the knee (52.77 per cent). The synovial fluid obtained from the knee joint of five patients demonstrated Brucella agglutinins and also three grew B. melitensis. Eight patients presented with complications that included skin lesions (3), carditis (2), neurobrucellosis such as chorea (1), peripheral neuritis (1), and meningitis (1). Brucella melitensis biotype 1 was successfully isolated from the papular eruption of one out of three cases who presented with skin lesions. To our knowledge this is the fourth confirmed isolation of B. melitensis from skin lesions with brucellosis, reported in the literature. The cerebrospinal fluid obtained from the meningitis patient was positive for B. agglutinins. To our knowledge chorea of brucellar origin appears to be the first case reported in the literature. In 15 cases (16.13 per cent) brucellosis was suspected clinically whereas 78 (83.87 per cent) cases, only serological evidence of brucellosis confirmed the diagnosis. None of the cases relapsed. In our experience an initial combination therapy with a three-drug regimen followed by a two-drug regimen for a minimum of 6 weeks has been found to be effective in the prevention of a relapse.     

Cardiac involvement in Sydenham's chorea: clinical and Doppler echocardiographic findings

Sydenham's chorea is often encountered in pure form, but it may also occur in association with carditis. Recently, it has been reported that silent, mild valvular regurgitation has been encountered in patients with rheumatic fever manifested by isolated polyarthritis and pure chorea. We studied 22 children with Sydenham's chorea to determine clinical and subclinical heart involvement at the time of the initial diagnosis. Ages of the patients ranged from 6-19 y (mean 13.14 ± 3.0), and the female/male ratio was 3.4:1. All patients were evaluated by clinical and echocardiographic examination with respect to heart involvement. Carditis was detected in only 5 of 22 patients (23%) by auscultation. Clinical valvular involvement was determined: mitral regurgitation in three and combined mitral and aortic regurgitation in two. The frequency of valvular involvement increased significantly to 63% (14/22) after adding silent mitral regurgitation detected by colour Doppler echocardiography (χ² = 7.50, p = 0.0062). We found that clinically inaudible, but echocardiographically significant, mild valvular regurgitation was frequent in patients with pure chorea. It is concluded that colour Doppler echocardiography may be useful in detecting silent valvular regurgitation and in deciding the duration of prophylaxis.     

Cerebral blood flow abnormalities in children with Sydenham's chorea: a SPECT study

Functional imaging studies in patients with Sydenhams chorea have suggested brain perfusional abnormalities. In this study, we aimed to investigate the cerebral perfusion patterns of the cortical/subcortical structures by using Tc-99m hexamethylpropylenamine oxime single photon emission computed tomography in children with Sydenhams chorea, accompanied with magnetic resonance imaging and cranial Doppler studies. Brain MRI and Doppler studies of the all cases were normal. In the brain SPECT study, six patients were determined to have cerebral perfusion abnormalities. Although six patients responded well to the therapy, two girls who had hypoperfusion in the right frontal region as well as hypo- / hyperperfusion in the basal ganglia did not respond to therapy. While the chorea form of the patients who have cerebral perfusional abnormality was generalized, the clinical picture of the patients with normal cerebral perfusion was in the form of hemichorea.     

Rheumatic heart disease in Nigerian children: clinical and preventive aspects

Ninety-two children with rheumatic heart disease were studied clinically, and through a post-diagnosis surveillance period of varying duration over a 10-year period. The logistics and effectiveness of secondary penicillin prophylaxis, administered monthly, were assessed during the surveillance period. Eighty-one (88%) of the 92 children were seen for the first time with already established rheumatic heart disease. Mitral insufficiency was the major functional defect in these children whose mean age was 8.6 years. There was a high default rate of just under 50% among children referred to the surveillance clinic. Socio-economic inadequacies were found to be major factors in the high default rate. Regular monthly parenteral penicillin was found to be effective in reducing recurrence of rheumatic fever and maintaining good health in these children.     

Acute chorea and type 1 diabetes mellitus: clinical and neuroimaging findings

The association of type 1 diabetes mellitus (T1DM) and acute chorea is rare. We report an 8-yr-old boy with T1DM who developed acute hemichorea-hemiballism of the right arm in whom magnetic resonance imaging (MRI) of the brain revealed hyperintense signal in putamen and considerable atrophy and focal area of gliosis in the right putamen. There was a deposition of deoxyhemoglobin indicating recent bleeding in the left lentiform nucleus. A control MRI 2 months later showed complete reabsorption of the blood component in the left lentiform nucleus. Multislice computerized tomography revealed hyperdensities in the corpus striatum and subcortical calcifications. This report describes, for the first time, findings of calcifications in the corticomedullary junction in the brain hemispheres of a child with T1DM and chorea and reviews the possible causal mechanisms of this unusual association.     

开展儿科多中心研究和临床流行病学调查的倡议

随着20世纪80年代循证医学的开创和发展，为临床医学的进一步科学化提供了方法上的可行性。循证医学的核心是证据，证据的创造、提供和应用是临床实践循证医学的关键。多中心临床研究和试验结果作为循证医学中的强力证据，为临床诊断、治疗以及预后判断等提供了很好的规范与指导。