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The Jones criteria proposed in 1944 for the diagnosis of rheumatic fever (RF) underwent a modification in 1955, and then a revision in 1965. The importance of establishing antecedent streptococcal infection was stressed, and the criteria became more difficult to meet with. Thirty-two children, whose clinical and laboratory manifestations met the modified but not the revised Jones criteria were encountered at the National Taiwan University Hospital during 1967-1971. They were labeled as probable RF, and were treated and follwed for 1 month to 6 years (average 3.7 years). The diagnosis in each case was evaluated in the light of their outcomes. The diagnosis of RF was justified in 27 cases based upon the following observation: Favorable responses to anti-rheumatic treatment (27 cases); normalization of the enlarged heart with disappearance of the murmurs (3 cases); normalization of the enlarged heart with persistence of the murmurs (5 cases); significant reduction of the enlarged heart (12 cases); typical RF recurrence (8 cases); development of pure mitral stenosis (1 case); valvular pathology verified at surgery or autopsy (4 cases). The diagnosis in the rest of 5 cases remained not confirmed or negated because that: the enlarged heart stayed unchanged (1 case); the child remained uneventful and free of cardiac involvement (2 cases); and the patient died and no postmortem study obtained (2 cases). The risk factors leading to underdiagnosis in these patients are: 1) late coming under observation; 2) no response in ASO titers; 3) limited studies for the evidence of streptococcal infection; and 4) drug induced modification of the clinical manifestations. This study implicates that patients who are very suspicious of RF on the clinical grounds, yet fail to meet the revised Jones criteria, especially those with established valvular heart disease, should be labeled as cases of probable RF, and be treated, followed, and placed on prolonged chemoprophylaxis until proved otherwise. Recurrence of RF may thus by prevented, and the regression or even natural healing of the rheumatic heart disease will become a possibility.

Outcomes of Children with Rheumatic Fever Not Diagnosed by Revised (1965) Jones Criteria