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PubMed1992

An epidemiological survey of rheumatic fever and rheumatic heart disease in Sahafa Town, Sudan.

STUDY OBJECTIVE: The aim was to determine the prevalence of rheumatic fever and rheumatic heart disease and to initiate a programme of secondary prophylaxis in Sahafa Town, Sudan. DESIGN: The study was a prospective case finding survey, carried out by a specially trained team headed by a cardiologist. SETTING: The study involved high risk school children (5-15 years of age) from Sahafa Town in the period 1986-1989. SUBJECTS: A total of 13,332 children on the school registers (7892 boys and 5430 girls) were examined generally and specifically for evidence of rheumatic fever or rheumatic heart disease. MAIN RESULTS: Out of the 13,322 children screened 351 were suspected cases and 146 were confirmed cases of rheumatic fever or rheumatic heart disease. The prevalence rates for all ages were 10/1000 for boys and 14/1000 for girls. The overall prevalence rate of the whole programme area was 11/1000, prevalence of rheumatic fever was 8/1000, and prevalence of rheumatic heart disease was 3/1000. The prevalence rate was significantly increased among the inner town inhabitants (15/1000) compared to the outer town inhabitants 4/1000 (p < 0.001). Monthly prophylactic benzathine penicillin in a dose of 1,200,000 IU was given to both suspected and confirmed cases. Penicillin coverage rate was 72%. CONCLUSIONS: Rheumatic fever continues to be a serious health problem. With economic pressures causing impending change in socioeconomic conditions in most Third World countries in the immediate future, rheumatic fever will continue to have a high prevalence rate and rheumatic fever and rheumatic heart disease prevention programmes will remain a central goal.

Adolescent↗
PubMed1997

HLA-A, B, DR, and DQ antigens in Indian patients with severe chronic rheumatic heart disease.

To determine whether genetic factors could be involved in the pathogenesis of rheumatic heart disease, we performed HLA-A and HLA-B typing in 59 Indian patients with severe chronic rheumatic heart disease requiring cardiac surgery, and HLA-DR and HLA-DQ typing in 58 of these patients. The HLA typing was done by a standard microlymphocytotoxicity method. Patients were 12 to 59 years old (mean 32.9 years). No significant differences in HLA-A, HLA-B, HLA-DR and HLA-DQ frequencies between patients and controls were noted. The role of genetically determined immune-response factors in the pathogenesis of chronic rheumatic heart disease was not evident in this study.

Adult↗
PubMed1998

[The expression of proliferating cell nuclear antigen and alpha smooth muscle actin in papillary muscles of the patient with rheumatic heart disease and its significance].

OBJECTIVES: To reveal the change of chronic rheumatic cardiomyocyt phenotype and its relationship with myocardium fibrosis. METHODS: The papillary muscles samples were obtained from patients with rheumatic heart disease during mitral valve replacement. Normal papillary muscles samples obtained from adult and fetal heart during autopsy in control group. The specimens were examined using immunohistochemistry and immunoelectron microscopy observation with monoclonal antibody of antiproliferating cell nuclear antigen (PCNA) and anti-alpha smooth muscles actin (alpha-SMA), and imaging analysis was performed. RESULTS: The interstitial fibrosis (24.7% +/- 3.05%) and the ratio between inner and outer diamater of arteriolas (0.405 +/- 0.035) in rheumatic myocardium were higher significantly than those in normal myocardium. Immunohistochemical PCNA expression was not only in interstitial but also in hypertrophic cardiomyocytes of rheumatic heart disease. The positive expression in rheumatic myocardium increased. There were a lot of rough endoplasmic reticula, ribosomes and Golgi bodies in cytoplasm of PCNA and alpha-SMA positive cells. The degree of PCNA and alpha-SMA positive expression was increased with enlargement of fibrosis areas. CONCLUSIONS: There is not only a high expression of PCNA and alpha-SMA in the myocardium of patients with rheumatic heart disease but also in fetal myocardium. We suggested that pathological myocardial cells are constructive phenotype and synthetive phenotype. PCNA and alpha-SMA positive expression cells are in the proliferating stage, they take part in fibrotic progress and vascular reconstruction in the myocardium.

Adult↗
PubMed1987

HLA-A, B, DR, and DQ antigens in black patients with severe chronic rheumatic heart disease.

To determine whether genetic factors could be involved in the pathogenesis of rheumatic heart disease, we performed HLA-A and HLA-B typing in 120 black patients with severe chronic rheumatic heart disease requiring cardiac surgery, and HLA-DR and HLA-DQ typing in 103 and 97 of these patients, respectively. The HLA typing was done by a standard microlymphocytotoxicity method. Patients were 12 to 60 years old (mean 27.6 +/- 14.5). No differences in HLA-A, HLA-B, and HLA-DQ frequencies between patients and controls were noted. HLA-DR 1 antigen was present in 12.6% of patients compared with 2.7% of normal control subjects (corrected p less than .045; relative risk = 5.2) and the HLA-DRw6 antigen was present in 31.1% of patients compared with 15% of control subjects (corrected p less than .045; relative risk = 2.6). These findings suggest that genetically determined immune-response factors may play a role in the pathogenesis of severe chronic rheumatic heart disease.

Adolescent↗
PubMed1983

The changing prevalence and pattern of acute rheumatic fever and rheumatic heart disease in Hong Kong--(1968-1978).

The occurrence and pattern of rheumatic fever and rheumatic heart disease in Hong Kong in the years 1968 to 1978 were surveyed. The incidence of rheumatic fever in hospitals had decreased by four-fifths in the past 10 years. Rheumatic fever nowadays still predominantly affects children and younger adults, but middle-aged patients may also be affected. There are a slight female dominance (1.3 to 1) and a relative predominance of patients from urban areas. A less severe form of manifestation in adult patients is encountered nowadays. Carditis was the dominant feature in 1968, but joint involvement was more common in recent years. Rheumatic nodules, chorea or erythema marginatum remained uncommon throughout the years. Only 49% of cases satisfied the revised Jones criteria (1965). In comparison, chronic rheumatic heart disease still remained prevalent, with a slower trend of decreasing prevalence. It accounted for (36 to 50%) of total cardiac admissions. The mitral valve was involved in (82.7 to 91.7%), aortic valve in (15.5 to 36.3%), while involvement of tricuspid or pulmonary valve was relatively uncommon. The socio-economic implication of this changing trend was analysed, and the problem encountered in diagnosing rheumatic fever discussed.

Acute Disease↗
PubMed1995

Results of mitral valve reconstruction in children with rheumatic heart disease.

BACKGROUND: Between January 1988 and November 1994, we developed techniques of reconstructing diseased mitral valves in patients with rheumatic heart disease. Four hundred thirteen patients underwent mitral valve repair using these techniques. Of these, 125 children and adolescents less than 15 years of age form the study group. METHODS: The mean age was 8.9 +/- 4.3 years (range, 5 to 15 years). One hundred seventeen patients (93.6%) had rheumatic heart disease. There were 72 boys (57.6%) and 53 girls (42.4%). All of these patients were symptomatic: New York Heart Association class III or IV. Mitral regurgitation alone was present in 49 patients (39.2%), and combined mitral stenosis and regurgitation were present in 76 patients (60.8%). Surgical techniques included commissurotomy (n = 70; 56%), annuloplasty (n = 122; 97.6%), chordal shortening (n = 46; 36.8%), cusp thinning (n = 27; 5.6%), and associated procedures for tricuspid valve disease (6 patients) and aortic valve disease (2 patients). RESULTS: The operative mortality rate was 4.8% (6 patients), and late deaths occurred in 1.6% (2 patients). Follow-up was 378.25 patient-years. In 15 patients, severe mitral regurgitation developed after a mean follow-up of 37.14 +/- 20.47 months (seven reoperations). At 6 years, actuarial and event-free survival rates were 92.1% +/- 3.19% and 75% +/- 8.18%, respectively. One patient (0.15%/patient-year) had transient right hemiparesis. None had anticoagulation-related bleeding. CONCLUSION: Mitral valve reconstruction in children and adolescents with rheumatic mitral regurgitation provides satisfactory early results. Progression of disease is the most important risk factor for reoperation. The technique described provided stable repair in the majority of patients.

Adolescent↗
PubMed1997

[HLA-DQA1 genes involved in genetic susceptibility to rheumatic fever and rheumatic heart disease in southern Hans].

The incidence of rheumatic fever (RF) or rheumatic heart disease (RHD) is high in southern China. We studied the genetic susceptibility of HLA-DQA1 alleles to RF or RHD with emphasis on the mechanisms 106 unrelated healthy individuals and 54 patients with RF or RHD of Chinese Han nationality in Guangdong were included. DNA extraction from various biological material using phenol/chloroform method and HLA-DQA1 genotyping by PCR-PAGE and then with silver dyeing was used to show the electrophoretic patterns. A total 6 alleles of HLA-DQA1 were found. Increased allele frequencies of DQA1*0101 (31.48%, RR = 2.89, P < 0.005, EF = 0.206) and decreased allele frequencies of DQA1*0102 (1.85%, RR = 0.106, P < 0.005, PF = 0.134) were observed. Two increased genotyping of HLA-DQA1 (DQA1*0101/0301, chi 2 = 8.84, P < 0.005 and DQA1*0101/0401, chi 2 = 6.23, P < 0.0025) and decreased genotyping of DQA1*0102/0301 (chi 2 = 11.98, P < 0.005) were also observed. These findings suggested that DQA1*0101 contribute to genetic susceptibility for RF or RHD in Guangdong hans while DQA1*0102 to its genetic resistance. Digesting the genotyping of HLA-DQA1 may provide scientific basis for finding susceptible individuals to RF or RHD. Using PCR-PAGE and silver dyeing technique, a new genotyping method for HLA-DQA1, which is simple sensitive and precise, was established and applied.

Adolescent↗
PubMed1999

Rheumatic heart disease among school children in Addis Ababa City: awareness and adequacy of its prophylaxis.

One of the objectives of this large scale cross-sectional study of school children of the Addis Ababa city was to assess the status of rheumatic heart disease (RHD) prophylaxis among rheumatic heart disease patients identified during the survey. Awareness about the presence of the illness in those affected and reasons for poor coverage, when detected, were also assessed. Sixty of the 9388 school children surveyed were found to have rheumatic heart disease. On interviewing parents of the children with rheumatic heart disease, ten acknowledged being informed of their children's cardiac illness. Of these parents, 15% (or 9/60) had some idea that their children had heart disease related to some form of infection. However, only two of the nine (22%) children whose parents had some idea about their disease were on regular monthly benzathine penicillin prophylaxis in the previous 12 months preceding the interview. Three (33%) of the nine children had six or fewer injections in the 12 months preceding the interview. The remaining 4 parents (44%) reported that their children took treatment that included injections only at the time of initial diagnosis several years earlier and had not had any follow up since then. Their reasons for not seeking medical care for their children included lack of information on prophylaxis, inability to pay for the treatment and distance of the health facilities. The lack of awareness and the extremely low rate of regular prophylaxis, therefore, highlight the need for an urgent control programme that takes active case detection, treatment access and health education into consideration.

Child↗
PubMed1985

Application of scanning electron microscopy in the characterization of Aschoff nodules in rheumatic heart disease.

Aschoff nodules (ANs) in excised atrial appendages from cases of rheumatic heart disease were examined with a scanning electron microscope (SEM). ANs could be identified with ease as nodular, fairly well defined cell aggregates lying in the subendocardium closely apposed to the myocardium. Cells comprising ANs were loosely packed in stacks. They were rectangular, oblong and angulated. The surface of some cells had fine ribbing while others were smooth having occasional blunt projections. SEM observations coupled with the findings of conventional and electron microscopy suggest that cells composing ANs are of mesenchymal origin having characters of epithelioid cells, reticular cells and/or fibroblasts.

Endocardium↗
PubMed1993

Enhancement of IL-1, IL-2 production and IL-2 receptor generation in patients with acute rheumatic fever and active rheumatic heart disease; a prospective study.

In a prospective study, patients with quiescent rheumatic heart disease (CRHD), streptococcal pharyngitis (SP) and healthy normal subjects produced comparable amounts of IL-1 and IL-2, but acute rheumatic fever (ARF) patients produced significantly elevated amounts of IL-1 and IL-2 at all intervals up to 48 weeks. In active rheumatic heart disease (ARHD), IL-1 activity returned to within normal range at 48 weeks, but IL-2 activity remained persistently elevated compared with CRHD, SP and healthy age- and sex-matched volunteers. CD4+ T lymphocytes were significantly increased in the peripheral blood of ARF and ARHD patients. The amount of IL-2 produced by ARF and ARHD patients correlated with the percentage of helper T lymphocytes (CD4+ cells) but not with the percentage of suppressor/cytotoxic T lymphocytes (CD8+ cells). Moreover, pre- and post-phytohaemagglutinin (PHA)-stimulated peripheral blood mononuclear cell (PBMC) cultures from ARF and ARHD patients contained higher proportions of IL-2R+ (CD25+) cells than those from patients with SP, CRHD and normal individuals, which persisted up to 48 weeks. The percentage of CD25+ cells in both types of PBMC cultures directly correlated with the percentage of CD4+ cells and not with CD8+ cells in active rheumatic patients only. These findings indicate that the immune response in ARF and ARHD patients is skewed to produce activated helper T cells that release IL-2 which drives the accumulation of more T helper cells. The result is an undamped helper T cell response in the peripheral blood of these patients.

Adolescent↗
PubMed1987

Compartmentalization of cells bearing "rheumatic" cell surface antigens in peripheral blood and tonsils in rheumatic heart disease.

Monoclonal antibodies that recognize "rheumatic" antigens of peripheral blood non-T cells were used to study the compartmentalization of such cells in peripheral blood and tonsils of individuals with rheumatic heart disease (RHD) and suitable control subjects. The peripheral blood of most (71%) of the 42 individuals with RHD contained cells reacting with monoclonal antibody 83S19.23 or 256S.10, whereas these cells were present in only 17% of the 41 control subjects (P less than .02). However, none of 21 individuals with RHD had such cells in their tonsils, although they were present in the tonsils of 50% of the 40 control subjects (P less than .03). These results may reflect a failure in RHD or organ-specific homing of cells with the epitopes recognized by the antibodies. The presence of these cells in tonsils may be important in the immune response to streptococcal pharyngeal infection, and their absence in RHD may be involved in the unusual immune responses characteristic of this disease.

Adolescent↗
PubMedDate not supplied

[Rheumatic activity and clinico-pathologic dissociation. Clinical and pathologic parameters in rheumatic heart disease].

We studied atrial appendages and valvular apparatus from patients undergoing cardiac surgery for rheumatic heart disease, looking for active histologic lesions. After reviewing 673 specimens (1980-1985) we studied two groups: 31 cases with Aschoff nodules or ill-differentiated histopathological lesions, and 31 cases, without tissular inflammatory abnormalities. In the former we found 8 cases with suspected clinical activity in a 3 months period before surgery, in the latter only 2 cases had similar findings. The Jones criteria are not useful for recognizing rheumatic activity among patients with chronic rheumatic heart disease, there is no clinical-histopathological correlations and it is possible that chronic inflammation occurs at the heart as an organ-limited condition.

Adolescent↗