History Of Acute Rheumatic Fever Research Articles

Aortic root replacement with pulmonary autograft in children

Between September 1988 and February 1993, 14 patients whose ages ranged from 3 months to 16 years (mean 11.1 +/- 4.3 years) underwent replacement of the aortic root with the autologous pulmonary root for aortic valve disease. The follow-up was 4 years (cumulative total of 25.2 patient-years). There was no early mortality. Late mortality (one patient) was 7.1% (95% confidence limits 0% to 21%). This patient had juvenile rheumatoid arthritis and died of consequent congestive heart failure with autograft failure 6 months after operation. Event-free survival after 4 years was 78.6% (95% confidence limits 50% to 95%). One patient was reoperated on because of autograft failure caused by a relapse of rheumatic fever. One patient operated on for critical neonatal aortic stenosis has subnormal exercise tolerance because of restrictive cardiomyopathy and pulmonary homograft regurgitation. The other 12 patients were in New York Heart Association functional class I at the end of follow-up. There was no prevalence of bacterial endocarditis. There were no signs of primary structural degeneration of the pulmonary autograft. During follow-up, in eight patients, increased anulus diameter of the pulmonary autograft could be demonstrated by precordial two-dimensional echocardiography, suggesting growth of the autograft. Our experience shows that aortic root replacement with the pulmonary autograft can be done with low mortality and morbidity in children with aortic valve disease. The operation seems to be contraindicated in children with juvenile rheumatoid arthritis because of the risk of recurrence of rheumatic disease in the autograft. The pulmonary autograft has also been shown to be susceptible to recurrence of rheumatic inflammation in children with a history of acute rheumatic fever. Despite pulmonary autograft replacement of the aortic valve in infants with critical valvular aortic stenosis and endocardial fibroelastosis, clinical results may be poor. Growth of the autograft is suggested by echocardiographic follow-up. We consider aortic root replacement with the pulmonary autograft the procedure of choice in children who require aortic valve replacement.

The natural history of acute rheumatic fever in Kuwait: A prospective six year follow-up report

One hundred and twenty-six children with the initial attack of acute rheumatic fever were followed up prospectively for 6 years. Sixty-six children maintained regular secondary prophylaxis (regular group) and 60 were irregular (irregular group). Two recurrences developed in the regular group with a recurrence rate of 0.005/patient/year follow-up, and 71 recurrences developed in the irregular group with a recurrence rate of 0.2/patient/year follow-up. These findings demonstrate the effect of secondary prophylaxis in reducing the frequency of recurrences. The prevalance rate of rheumatic heart disease in children who had carditis in the initial attack was 42% in the regular group vs 70% in the irregular group ( p < 0.05). These findings demonstrate the deleterious effect of recurrences in the evolution of rheumatic heart disease. The prevalence rate of rheumatic heart disease in children who maintained regular secondary prophylaxis, was 42% in those children who had carditis in the initial attack and 6% in those who had no carditis ( p < 0.05). These findings demonstrate the prognostic significance of the presence or absence of carditis during the initial attack, in the subsequent evolution of rheumatic heart disease. The prevalence rate of rheumatic heart disease in the 66 children who maintained regular prophylaxis was 23%. Comparison of these data with those of similarly designed studies shows that the evolution of rheumatic heart disease following the initial attack of acute rheumatic fever, seems to behave similarly in the tropics and subtropics as it did in temperate climates.

Mitral valve stenosis in the elderly.

Forty-seven elderly patients (aged 70-95 years) with mitral stenosis (MS) were studied. The initial presentation in 77% of patients was in the postretirement age and 55% were diagnosed after the age of 70 years. A history of acute rheumatic fever (ARF) was given by 43% of all patients. One patient had mitral valve stenosis secondary to mitral annular calcification. MS due to rheumatic heart disease may be mild or the progress may be slow enabling patients to live a near normal life; increased longevity of the population in general, use of potent diuretics, limitation of physical activity due to medical complications and decreased number of pregnancies may have been contributory factors. In 23% of patients the diagnosis was initially unrecognized, underlining the possible occult nature of the valve lesion in some elderly patients. The advent of echocardiography has helped in case detection.

HLA antigens and acute rheumatic fever: evidence for a recessive susceptibility gene linked to HLA.

From 60 probands with acute rheumatic fever (ARF), 19 multiplex families segregating for ARF were ascertained. The parents and rheumatic and normal sibs of the probands in these 19 families were also studied. HLA typing using the microlymphocytotoxic assay was then performed on the 60 unrelated probands, the multiplex families, and 234 unrelated controls using 23 antigens from the HLA-A and -B loci. The controls lacked a past history of ARF and were from the same geographic locality. Calculations of relative risk demonstrate an increase of HLA-B5 antigen in the 60 patients, but the result might not be significant from the point of view of multiple comparisons. Nevertheless, affected sib pairs from the multiplex families show 93% concordance for both or one HLA haplotype. A formal linkage analysis demonstrates that a recessive etiology is most likely (lod score of 3.3) with approximately 68% of cases being due to a gene closely linked to HLA and in linkage disequilibrium with HLA-B5. The remaining 32% of cases are due to other familial factors such as polygenic inheritance or common environmental factors. The results confirm a strong genetic predisposition to ARF and its heterogeneous nature in families.

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.

Human antibody response to group A streptococcal teichoic acid

The immunochemical characteristics and antibody levels in human sera to a specific group A streptococcal teichoic acid (TA) were studied. This phenol-extracted TA had DL-α-glycerophosphate as the major antigen determinant and not N-acetylglucosamine (N-AGA) as measured by an indirect hemagglutination assay (IHA). The group A streptococcal carbohydrate (A-CHO), N-AGA, and staphylococcal TA did not inhibit the streptococcal TA reaction. In studies of patients with or without history of acute rheumatic fever (ARF) or rheumatic heart disease (RHD), there was an inverse relationship between magnitude of TA hemagglutinating and A-CHO precipitating antibody (Ab) titers. Age-specific distribution of TA-Ab titers was not characteristic of titers observed against other streptococcal protein or carbohydrate antigens.

Decreasing incidence of a history of acute rheumatic fever in chronic rheumatic heart disease.

Decreasing incidence of a history of acute rheumatic fever in chronic rheumatic heart disease.

Attitudes of children with rheumatic fever

Summary 1. Fifty children with a history ofacute rheumatic fever or with existing evidence of a previous rheumatic infection were interviewed to discover their attitudes toward their disease and its required treatment. 2. The children had been treated athome, in hospitals, in convalescent institutions, and in a follow-up clinic. 3. Data were obtained concerning attitudes toward illness at home, hospitalization, preparation for convalescent care, reintegration into home and community, school attendance, parental handling, follow-up clinic care, and the meaning of the disease. 4. Suggestions are offered which are directed toward reducing the stresses met by these children and toward enabling them to cooperate fully in longrange treatment programs.

Observations on the Natural History of Acute Rheumatic Fever

Observations on the Natural History of Acute Rheumatic Fever