Cases reported "Rheumatic Fever"

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1/100. Acute rheumatic fever with three major criteria: polyarthritis, carditis and chorea. A case report.

    An eight-year-old girl is presented with three major criteria of acute rheumatic fever: polyarthritis, carditis and chorea. The diagnosis was confirmed with a history of pharyngitis 15 days prior to admission and with the findings of positive acute phase reactants such as elevated erythrocyte sedimentation rate (ESR) and c-reactive protein (CRP), elevated anti-streptolysin-O (ASO) titration, and clinical findings of polyarthritis, carditis and chorea. Patient responded well to salicylate and phenobarbital treatment. The rare association of these three major criteria in acute rheumatic fever is emphasized.
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2/100. Generalized eruptive histiocytoma of childhood associated with rheumatic fever.

    We describe a widespread papular eruption in a 5-year-old girl with rheumatic fever. Histological examination revealed a dense histiocytic infiltration in the dermis. On immunohistochemical studies, the cells were positive for vimentin, CD68, MAC387, alpha1-antichymotrypsin and lysozyme, but negative for CD1a and S-100 protein. Electron microscopic studies showed no Birbeck granules in their cytoplasm. A diagnosis of generalized eruptive histiocytoma of childhood was established. The skin lesions completely disappeared within 8 months.
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3/100. The prophylaxis of acute rheumatic fever in a pair of monozygotic twins. The public health implications.

    Recurrent acute rheumatic fever and rheumatic heart disease can be prevented by antibiotic intervention. We report the case of genetically identical twins, one of whom had overt rheumatic fever, received penicillin prophylaxis and did not have rheumatic heart disease. The other must have had inapparent rheumatic fever, received no chemotherapy prophylaxis and proceeded to develop rheumatic heart disease. A greater clinical and laboratory vigilance is required for the diagnosis of acute rheumatic fever in the asymptomatic identical twin of a patient with rheumatic fever. This case provides further evidence of a genetic predisposition to rheumatic fever and demonstrates the continued value of penicillin in the prophylaxis of acute rheumatic fever. It emphasises the need to maintain the integrity of preventive programmes against rheumatic fever worldwide. Studies which explore the HLA and other genetic linkages with rheumatic fever should be encouraged.
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4/100. One year's rheumatic fever from one general practice in Wairoa, Hawke's Bay.

    Sixteen cases of rheumatic fever from one general practice are described and the indication for prophylactic penicillin is discussed.
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5/100. Perforated gastric ulcer complicating corticosteroid therapy in acute rheumatic fever.

    We report an 11-year-old boy with acute rheumatic fever who presented with gastric perforation while treated with corticosteroids (CS). He had been treated initially with acetylsalicylic acid for 11 days, CS replaced the treatment with acetylsalicylic acid due to deterioration of carditis. The possible pathogenesis is discussed.
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6/100. role of vitamin e in rheumatic chorea.

    Rheumatic chorea is the sole neurologic manifestation of rheumatic fever. It is a debilitating illness lasting for weeks to months. Drugs like diazepam, haloperidol, chlorpromazine take four to six weeks for functional improvement and can cause serious side effects. The authors investigated the role of vitamin e in reducing rheumatic chorea. A case series of patients of rheumatic chorea were administered vitamin e in the dose 50 IU daily for fifteen days. The various clinical signs of rheumatic chorea were scored with MAIMS score (Modified Abnormal Involuntary movement Scale score) which is used for tardive dyskinesia. No other drug for abnormal movements was used. In all the 4 patients who received vitamin e, there was remarkable change by 7th day and almost complete functional improvement by 14th day. vitamin e is safer than the conventional drugs used for chorea in children. It was found effective in this case series. Its role needs further evaluation by a double-blind randomized controlled trial.
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7/100. Acquired anti-FVIII inhibitors in children.

    Acquired inhibitors to FVIII (anti-FVIII) are uncommon in children. An acquired anti-FVIII developed in a previously healthy 4-year-old boy treated with penicillin for streptococcal pharyngitis. aspirin prophylaxis begun for suspected rheumatic fever led to compartment syndromes of all four extremities, which resolved with high-dose FVIII and surgical decompression. Anti-FVIII in this patient, and the five additional cases identified in a survey of 160 haemophilia treatment centres, occurred at a median age of 8 years, with median initial and peak titres of 4.6 and 6.9 Bethesda Units (BU), respectively. All six presented with bleeding, including haematomas (three intramuscular, one intracranial), and ecchymoses in three. The median baseline FVIII was 0.05 U mL(-1), and the median baseline activated partial thromboplastin time (APTT) was 79.8 s. The inhibitor resolved completely in five patients (83%) within a median 5 months, after treatment with FVIII concentrate, steroids, cytoxan, methotrexate, and no treatment. The inhibitor persisted in the patient with Goodpasture's disease, despite steroids, cytoxan, cyclosporin, and intravenous gamma globulin. aspirin therapy, in two, worsened ongoing bleeding. The association of penicillin-like drugs in this and three other cases in the literature suggest that to avoid potential catastrophic bleeding, it is prudent to obtain an APTT prior to initiating aspirin for suspected rheumatic fever. In conclusion, acquired anti-FVIII inhibitors in children may cause severe bleeding, and remit in the majority after FVIII and/or immunosuppressive therapy.
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8/100. Acute rheumatic fever in a patient with glycogen storage disease type Ib: causal or coincidental simultaneous occurrence?

    We report a Caucasian female who was diagnosed with glycogen storage disease type Ib (GSD-Ib) at the age of 4 months and whose clinical course was complicated by neutropenia and very frequent episodes of infection, including tonsillopharyngitis. Recurrent group A streptococcal infections resulted in multiple episodes of extremely high serum levels of antibodies to streptolysin O (5,000 IU/ml) and DNAse B (6,000 IU/ ml). At the age of 14 years she presented with carditis, migratory arthritis, fever, elevated erythrocyte sedimentation rate as well as serological evidence for recent streptococcal infection providing a diagnosis of acute rheumatic fever. CONCLUSION: the occurrence of these two very rare disorders in our patient may indicate that this association is not coincidental because neutrophil dysfunction in glycogen storage disease type Ib may have predisposed this patient to acute rheumatic fever due to increased susceptibility to group A streptococcal infections. aberrant glycogenolysis and gluconeogenesis, neutropenia and neutrophil dysfunction are regular findings in GSD-Ib. neutropenia and neutrophil dysfunction in patients with GSD-Ib are due to defects in myeloid maturation, impaired neutrophil motility, defective chemotaxis and phagocytosis and diminished bactericidal activity resulting in recurrent bacterial infections.
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9/100. Transient complete heart block complicating acute rheumatic fever.

    First-degree heart block is a common electrocardiographic manifestation of acute rheumatic fever and is included in Jones' diagnostic criteria. Other electrocardiographic changes such as sinus tachycardia, bundle branch blocks. nonspecific ST-T wave changes, atrial and ventricular premature complexes have been reported with variable frequency. However, complete heart block is an exceptionally rare manifestation of acute rheumatic fever. We report the clinical course of a 16-year-old boy with acute rheumatic fever who had prolonged P-R interval in the electrocardiogram on admission which subsequently progressed to complete heart block. The patient regained normal sinus rhythm within a few minutes without any pharmacologic or electrical intervention.
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10/100. Acute rheumatic fever associated with Henoch-Schonlein purpura: report of three cases and review of the literature.

    AIM: To describe a possible relationship between Henoch-Schonlein purpura and rheumatic fever. methods: patients with features of both diseases were identified by reviewing the hospital records. medline and reference lists from published articles were used to search for previous reports of the two conditions occuring simultaneously. RESULTS: Three newly described cases, and three previous reports of Henoch-Schonlein purpura associated with rheumatic carditis or chorea were identified. CONCLUSIONS: The coexistence of these two disorders in some patients supports the view that Group A streptococcus may have a pathogenic role in Henoch-Schonlein purpura.
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