Doppler echocardiography and the early diagnosis of carditis in acute rheumatic fever

BACKGROUND: The incidence of acute rheumatic fever in New Zealand remains relatively high. Reliable early diagnosis of carditis is difficult and important in management. AIM: To determine if Doppler echocardiography contributed to the early diagnosis of carditis in acute rheumatic fever. METHODS: Forty-seven patients admitted to hospital with suspected acute rheumatic fever and 19 control patients, with a febrile illness due to a documented non-cardiac bacterial infection, were assessed two days and two weeks following admission. Presence or absence of clinical carditis was determined by a cardiologist unaware of the suspected diagnosis, from clinical examination, chest radiograph, electrocardiogram (ECG) and two dimensional echocardiogram. Doppler echocardiography was then performed and interpreted by a second cardiologist unaware of the diagnosis. After completion of the study the Jones criteria were applied, to categorise the patients with suspected acute rheumatic fever into four groups for the final diagnosis: no acute rheumatic fever, possible acute rheumatic fever, definite acute rheumatic fever without carditis, and definite acute rheumatic fever with carditis. RESULTS: In 19 patients with a final diagnosis of acute rheumatic fever and carditis at the baseline assessment carditis was detected by clinical assessment in 15 patients, compared with 19 patients with evidence of significant valve regurgitation by Doppler echocardiography. Following the two week assessment, all 19 patients had both clinical and Doppler evidence of carditis. Five patients with a final clinical diagnosis of possible acute rheumatic fever or definite acute rheumatic fever without carditis, had a Doppler abnormality detected. There was no clinical or Doppler abnormality in the febrile controls. CONCLUSIONS: Doppler echocardiography is more sensitive than clinical assessment in the detection of carditis in acute rheumatic fever, and can contribute to earlier diagnosis.     

Pigmented villonodular synovitis of the temporomandibular joint with invasion of middle cranial fossa

Forty six attacks of acute rheumatic fever (ARF) in forty patients were diagnosed between November 1987 and August 1995. Thirty four were initial attacks and 12 were recurrences. Arthritis was the commonest feature, 84.8%. Carditis occurred in 65.2% of the group, 67.6% of the initial attacks and 58.3% of the recurrences; however, the frequency of moderate/severe carditis was higher in recurrences, 25% versus 11.8%. Of those with carditis, mitral regurgitation occurred in 93.3%, aortic regurgitation in 16.7% and significant tricuspid regurgitation in 6.7%. Mitral stenosis was not encountered. No mortality occurred during ARF. Chorea, erythema marginatum and subcutaneous nodules were infrequent. These data are similar with those from a previous study which demonstrated the mild nature of ARF in Saudi Arabia, but showed higher frequency of carditis and suggested the frequency of carditis was not significantly higher during recurrences as compared to frequency of moderate/severe carditis.     

Anaemia and thrombocytopenia due to haemophagocytosis in a 7-month-old boy with galactosialidosis

There is a need for revising the current practice for treatment of acute tonsillitis in France, i.e., systematic antibiotic treatment. There are three main reasons for this revision: 1) group A betahemolytic streptococcus is involved in only 20% of acute tonsillitis (80% being viral); 2) rheumatic fever has become very rare; 3) efficient rapid diagnostic tests are now available, allowing a selection of patients with streptococcal tonsillitis who must be treated.     

Salmonella typhi infections. A 10-year retrospective study

Enteric fever is still a common health problem in many countries, especially in children. Thus a ten-year retrospective study was carried out to evaluate the clinical and laboratory properties of enteric fever and the incidence of antimicrobial resistance in children. Throughout the past 10 years, Salmonella was isolated in 105 patients by blood culturing, 27 of which were Salmonella typhi. Most of the patients were above the age of two. Besides the typical symptoms and signs of enteric fever, 29.2% of the patients had some neurologic findings. Besides, 68.5% had elevated liver enzymes while only 44.4% had hepatomegaly with or without splenomegaly. Anemia was present in 44%, leukopenia in 16% and leukocytosis in 11.1% of the cases. The emergence of antimicrobial resistance during the last five years against ampicillin, chloramphenicol and trimetoprim-sulfamethoxazole has created a challenge in treating these infections.     

Acute and chronic diarrhoea and abdominal colic associated with enteroaggregative Escherichia coli in young children living in western Europe

BACKGROUND: Enteroaggregative Escherichia coli (EAggEC or EAEC) can spread and cause disease in developing countries, but it is not presently known whether it spreads disease in industrialised countries. Therefore, we did a prospective study to assess the incidence and the clinical manifestations of infections due to EAEC in children in Germany. METHODS: 798 children with diarrhoea, admitted to hospital within a defined geographical area during a 24-month period, were included in the trial. EAEC were cultured from stool specimens, screened by PCR, and identified by colony hybridisation from DNA sequences found on the virulence plasmid. The findings were confirmed by aggregative adherence to HEp-2 cells. Stool samples from 580 children admitted to hospital without diarrhoea were also studied as controls. FINDINGS: EAEC were found in the stools of 16 (2%) of 798 children with diarrhoea, but in none of 580 children without diarrhoea. Only four of the EAEC-infected children had travelled to developing countries. Most EAEC infections were acquired in the summer months. Infection with EAEC was associated with acute, watery diarrhoea in 12 children, and with chronic diarrhoea of up to 5 months' duration in four. Five children had abdominal colic that lasted for 2-4 weeks as their main symptom. The incidence of EAEC infection was 7.7 patients admitted to hospital per 100,000 children in the general population aged younger than 16 years. INTERPRETATION: EAEC infection is associated with acute, watery diarrhoea and may be acquired in industrialised countries. Chronic diarrhoea or abdominal colic of unknown aetiology in young children may also be caused by EAEC infection.     

Incidence and prevalence of juvenile chronic arthritis in East Berlin 1980-88

OBJECTIVE: To estimate the incidence and prevalence rates of juvenile chronic arthritis (JCA). METHODS: The study population was children under 16 years of age living in the East Berlin area (part of the former German Democratic Republic). By admission order that was effective up to 1990, all children with symptoms of a rheumatic disease living in the East Berlin area had to be referred to the 2nd Children's Hospital at Berlin-Buch. This specific condition allowed us to ascertain cases from the clinical records and to calculate population rates. Based upon this data, the results of surveys with different methods of case ascertainment are compared. RESULTS: An incidence rate of 3.5 per 100,000 and a prevalence rate of 2.0 per 10,000 children were calculated. The frequency of JCA is higher for girls, with an incidence of 4.3 per 100,000 and a prevalence of 2.3 per 10,000. The figures for boys are 2.7 per 100,000 and 1.7 per 10,000, respectively. CONCLUSION: Because of the specific prerequisites, the population rates of prevalence and incidence that were based on clinical records can be regarded as valid in this study. Deviant results of other surveys can be explained by differences in the study design or in the diagnostic procedures used.     

The effect of prevention and other factors in the decrease in rheumatic fever

933 children with rheumatic fever hospitalised in Clinic in the period of 40 years (from 1995 to the end of 1994) were analysed in order to establish the fluctuation of patients number and changes in clinical appearance. Patients were divided in 4 groups: I. the patients hospitalised from 1955-1964 (472 patients), II. from 1965-1974 (307), III. from 1975-1984 (135), IV. from 1985-1994 (19). The data for Republic Croatia show the steady fall of the number of hospitalised children with rheumatic fever. Carditis with polyarthritis were present in the largest part of our patients (446 namely 47.8%). The number of recurrences fell equally with the number of patients with rheumatic fever. However, ratio between the number of patients with rheumatic fever and the number of recurrences did not change essentially, that was 11-15.85% in the periods considered. The percent of hospitalised children with fixed rheumatic heart disease was 4.23% in the I. period to as much as 15.5% in the III. period from totally hospitalised children with rheumatic fever. In the last time the disease became mild in its development. All children with suspicion on rheumatic fever should be hospitalised. The prophylaxis, primary or secondary, should be carried out intramusculary with benzithine penicillin G, as proved as the best, in order to ensure that the child virtually received the prophylaxis.     

The incidence of pediatric rheumatic diseases: results from the Canadian Pediatric Rheumatology Association Disease Registry

OBJECTIVE: To determine the incidence of rheumatic diseases in children, and the frequency of musculoskeletal disorders seen by pediatric rheumatology specialists in Canada. METHODS: Applying standardized disease definitions and disease codes modified from ICD-9, members of the Canadian Pediatric Rheumatology Association from 13 centers in all 10 provinces of Canada registered all new patients seen between May 1, 1991 and April 30, 1993. Patient data included age, sex, ethnicity, date of birth, date of disease onset, date of diagnosis, and diagnostic codes (more than one diagnosis could be entered). To minimize the bias of right censoring, only data from patients with disease onset between May 1, 1991 and October 31, 1992 were used to estimate disease incidence. RESULTS: 3362 records totalling 3683 diagnoses (92 separate diagnoses) were registered. Median referral rate per year to a pediatric rheumatology center was 26 per 100,000 children at risk. The frequency of diseases seen was 23.3% for all forms of chronic arthritis, 6.5% for connective tissue diseases, and 6.1% for all forms of vasculitis. The minimum incidence rates per 100,000 children at risk per year calculated from the whole registry were: all forms of chronic arthritis 4.08 (95% CI: 3.62, 4.60), systemic lupus erythematosus 0.28 (0.18, 0.45), and dermatomyositis 0.15 (0.09, 0.29). Substantially higher figures were obtained if the figures were calculated excluding the 2 provinces (Alberta and Quebec) that had disproportionately low referral rates. CONCLUSION: Pediatric rheumatologists see children with a wide variety of diseases. It is important that pediatric rheumatology training reflects this and does not focus exclusively on the classical inflammatory arthropathies. The minimum incidence data show there are substantial numbers of children developing potentially lifelong chronic rheumatic diseases each year in Canada. These data should be helpful in planning the delivery of pediatric rheumatology services not only in Canada, but also in other developed countries.     

Selective attachment of beta-haemolytic streptococci group A to oropharyngeal epithelium in health and disease

Localization and semiquantification of beta-haemolytic streptococci, Group A (GABHS), GABHS attachment and general bacterial attachment to epithelial cells (bacterial number and morphology) were studied during GABHS-positive acute tonsillitis and pharyngitis infections and among healthy GABHS carriers. Samples were collected from various areas of the oropharynx (palatine tonsils, posterior oropharyngeal wall, palatoglossal arch and buccal mucosa). During acute tonsillitis and pharyngitis, GABHS grew in samples obtained from the palatine tonsils and posterior oropharyngeal wall. The ratio of GABHS colonies to other aerobic colonies increased, and GABHS became attached to epithelial cells of both palatine tonsils and posterior oropharyngeal wall. In GABHS carriers, GABHS were found mainly on the palatine tonsils, but these microorganisms were not attached to the epithelium. Overall bacterial attachment to tonsillar and oropharyngeal epithelial cells increased during active tonsillitis and pharyngitis.     

Concurrent dengue hemorrhagic fever and typhoid fever infection in adult: case report

In Indonesia as well as in many developing countries both typhoid fever and dengue hemorrhagic fever (DHF) are still endemic and prevalent. In Indonesia the incidence of DHF in 1994 was 9.72/100,000 population with CFR of 2.5% and each year about 640,000-1,500,000 cases of typhoid fever were reported with mortality of 1.6-3%. The concurrent infection of both diseases may occur in one patient.     

Rheumatic heart disease in the developing world: prevalence, prevention, and control

Rheumatic heart disease (RHD) continues to be a common health problem in the developing world, causing morbidity and mortality among both children and adults. Although little longitudinal data are available, evidence suggests that there has been little if any decline in the occurrence of RHD over the past few decades. Recent reports from the developing world have documented rheumatic fever (RF) incidence rates as high as 206/100,000 and RHD prevalence rates as high as 18.6/1000. The high frequency of RHD in the developing world necessitates aggressive prevention and control measures. The major interventions for prevention and control include: (1) reduction of exposure to group A streptococci, (2) primary prophylaxis to prevent initial episodes of RF, and (3) secondary prophylaxis to prevent recurrent episodes of RF. Because recurrent episodes of RF cause increasingly severe cardiac complications, secondary prophylaxis is the most crucial feature of an effective RHD programme. For some impoverished countries, secondary prophylaxis may be the only intervention that can realistically be implemented. In addition to this intervention, however, financial and human resources must be committed, and all of these elements must be integrated into existing primary health care systems. Because RHD continues to be a common health problem in the developing world, greater emphasis needs to be placed on the simple and cost-effective prevention and control measures that are currently available to combat this disabling disease.     

Azithromycin. A review of its use in paediatric infectious diseases

Azithromycin is an azalide antimicrobial agent active in vitro against major pathogens responsible for infections of the respiratory tract, skin and soft tissues in children. Pathogens that are generally susceptible to azithromycin include Haemophilus influenzae (including ampicillin-resistant strains), Moraxella catarrhalis, Chlamydia pneumoniae, Chlamydia trachomatis, Mycoplasma pneumoniae, Legionella spp., Streptococcus pyogenes and Streptococcus agalactiae. Azithromycin is also generally active against erythromycin- and penicillin-susceptible Streptococcus pneumoniae and methicillin-susceptible Staphylococcus aureus. Azithromycin is administered once daily, achieves clinically relevant concentrations at sites of infection, is slowly eliminated from the body and has few drug interactions. In children, azithromycin is usually given as either a 3-day course of 10 mg/kg/day or a 5-day course with 10 mg/kg on the first day, followed by 5 mg/kg/day for a further 4 days. These standard regimens were as effective as amoxicillin/clavulanic acid, clarithromycin, cefaclor and amoxicillin in the treatment of children with otitis media. Azithromycin was also as effective as either phenoxymethylpenicillin (penicillin V), erythromycin, clarithromycin or cefaclor against streptococcal pharyngitis or tonsillitis in children, but appears to result in more recurrence of infection than phenoxymethylpenicillin in this indication, necessitating a dosage of 12 mg/kg/day for 5 days. Community-acquired pneumonia, bronchitis and other respiratory tract infections in children responded as well to azithromycin as to amoxicillin/clavulanic acid, cefaclor, erythromycin or josamycin. Azithromycin was similar or superior to ceftibuten in mixed general practice populations of patients. However, symptoms of lower respiratory tract infections resolved more rapidly with azithromycin than with erythromycin, josamycin or cefaclor. Skin and soft tissue infections responded as well to azithromycin as to cefaclor, dicloxacillin or flucloxacillin, and oral azithromycin was as effective as ocular tetracycline in treating trachoma. Although not as well tolerated as phenoxymethylpenicillin in the treatment of streptococcal pharyngitis, azithromycin is at least as well tolerated as most other agents used to treat respiratory tract and other infections in children and was better tolerated than amoxicillin/clavulanic acid. Adverse events that do occur are mostly gastrointestinal and tend to be mild to moderate in severity. CONCLUSIONS: Azithromycin is an effective and well tolerated alternative to first-line agents in the treatment of respiratory tract, skin and soft tissue infections in children, offerring the convenience of a short, once-daily regimen.     

Antihyaluronidase level in children with rheumatic fever and other streptococcal infection

1) The serum ASO and AH assays were performed and compared in a series of 90 healthy children and 7 pediatric patients with rheumatic fever and other disorders related to hemolytic streptococcus infection. 2) The upper physiological limit of serum AH titer was estimated to be 256X for children. 3) In rheumatic fever, sometimes the serum AH level rises earlier than elevation of serum ASO and remains to be high over a long period even after the serum ASO has returned to normal level. 4) The results suggest importance of the serum AH determination in the serologic diagnosis of rheumatic fever and other conditions of hemolytic streptococcus infection.     

Prevalence of heart disease in school children in rural Kenya using colour-flow echocardiography

In a survey using colour-flow echocardiography and a portable generator, of one thousand one hundred fifteen children, three had clinical and echocardiographic evidence of rheumatic heart disease giving a prevalence rate of 27/1000. Sixty nine (6.2%) of the children examined had trivial mitral regurgitation (TMR). Of these, three had associated trivial aortic regurgitation. Isolated trivial aortic regurgitation was not seen. Four children had isolated mild-moderate regurgitation of the pulmonary valve. Congenital heart disease (CHD) was found in two children-one with secundum atrial septal defect and one with a ventricular septal defect and pulmonary stenosis, giving a prevalence of 1.8/1000. One child had a bicuspid aortic valve and two persistent left superior vena cava. It is feasible to carry out an echocardiographic survey using a portable generator in schools where electricity is not available. The prevalence of rheumatic heart disease (RHD) is higher than previously found in Kenya. The prevalence of TMR is surprisingly high. This was associated with familial clustering reminiscent of RHD. The importance of these findings must await the results of further investigation.     

Cellular composition of palatine tonsil imprints in the differential diagnosis of uncomplicated chronic tonsillitis with a tonsillo-cardiac syndrome and chronic tonsillitis with rheumatism

The authors studied imprints of tonsillar sections stained both by the method of Pappeneheim and that of Stockinger and Kellner taken from 205 patients with chronic non-complicated tonsillitis, chronic tonsillitis with tonsillocardiac syndrome and chronic tonsillitis with rheumatism, the rheumatic process being of various stages of activity. A comparison of the preparations received shows that the organism's immunologic reformation resulting from the rheumatic process is characterised by growing reticulolymphoblastic, plasmocellular and macrophage reactions and vivification of sinuslymphocytopoiesis which proved to be in strict correlation with the extent of rheumatic activity.     

Epidemiology of encephalitis in children. A prospective multicentre study

We found 175 cases with acute encephalitis in a population of 791,712 children aged 1 month-15 years during a 2-year surveillance period in 1993-1994. The overall incidence was 10.5/100,000 child-years with the highest figure in children < 1 year of age, 18.4/100,000 child-years. The microbial diagnosis was considered proven or suggested in 110 cases (63%); varicella zoster, respiratory and enteroviruses comprised 61% of these, and adeno, Epstein Barr-, herpes simplex and rota viruses comprised 5% each. A clearcut change seems to have occurred in the aetiology of encephalitis. Mumps, measles, and rubella virus associated encephalitides have been almost eliminated. Varicella zoster, respiratory, and enteroviruses have increased in frequency and occur in younger age groups. New causes were identified, especially Chlamydia pneumoniae and HHV-6. Our data should assist in making a specific diagnosis and defining appropriate antimicrobial therapy. CONCLUSIONS: The spectrum of encephalitis in children has changed due to vaccination programs. The incidence, however, appears to be about the same due to increasing frequency of other associated old and new microbes.     

Persistent elevation of immunoglobulin G titer against the C region of recombinant group A streptococcal M protein in patients with rheumatic fever

To analyze the immune response to the C region of group A streptococcal M protein in patients with rheumatic fever (RF), we cloned the structural gene for the C region of type 12 M protein and produced recombinant C region of M protein. IgG titers against the C region of M protein were measured by ELISA from sera of patients with RF (n = 10), uncomplicated streptococcal pharyngitis (n = 26), and age-matched controls (n = 49). IgG titers against the C region were significantly higher in patients with RF than in controls or patients with uncomplicated streptococcal pharyngitis (43 versus 1.5 or 1.8 micrograms/mL, p < 0.01). Studies using overlapping synthetic peptides of the C region demonstrated that increased IgG reactivity was observed against the amino-terminal halves of the C repeat blocks (C1, C2) in RF, indicating that these domains are the main immunodominant epitopes in the C region.     

Pediatric rheumatology in the United Kingdom: data from the British Pediatric Rheumatology Group National Diagnostic Register

OBJECTIVE: To establish a computerized national diagnostic register for pediatric rheumatology in the UK; to describe the demography and diagnostic classification of children referred to pediatric rheumatology clinics; to estimate the current incidence of juvenile arthritis (JA) in the UK. METHODS: A diagnostic register was established in 1989: 23 centers have contributed data on all new cases seen since they joined the register; 18 centers have also contributed data on all current attenders. For 2 centers with well defined catchment areas, the incidence of JA was estimated. RESULTS: A total of 4948 cases were registered, of whom 2962 (60%) were female. 1991 (40%) had a diagnosis of JA. The 2nd largest category was mechanical/orthopedic problems (24%). Pauciarticular juvenile chronic arthritis was the most common subtype of JA. Seropositive RA was rare. Tertiary referral centers saw proportionately more JA and district general hospitals saw proportionately more mechanical problems. The annual incidence rate for JA from 2 centers was 10/100,000, and for all rheumatic disorders was 32-42/100,000 children under age 16. CONCLUSION: The relative proportion of patient diagnoses varies between centers. Nevertheless, the incidence of hospital referred JA seems very uniform.     

Precursor lesions of Wilms' tumor in Indian children: a multiinstitutional study

BACKGROUND: Studies regarding different ethnic populations of children with Wilms' tumor (WT) in the U. S. have shown differences between age and gender incidence and the type of its precursor lesions (nephrogenic rests). To the authors' knowledge there are few reports in the literature regarding nephrogenic rests in a large number of cases from other parts of the world. METHODS: In this study, histopathology sections from 127 WTs were pooled from the archives of 4 institutions in northern India and studied for the incidence of nephrogenic rests. Their incidence also was cross-correlated with patient age gender, and tumor morphology. RESULTS: Significantly, the complete absence of perilobar nephrogenic rests was observed in all cases. Intralobar rests were associated with 45.3% of WTs and were observed predominantly with a triphasic histologic pattern and a relatively lower incidence of anaplasia compared with reports in the Western literature (1.5% vs. 4.5%). A lower median age of 2.5 years with a peak in the first 2 years of life was consistent with reported studies in Asian children. The male gender preponderance was in contrast to the equal male to female ratio reported in Western cases. CONCLUSIONS: The type of nephrogenic rests present in Indian children was noted to be different from that in children reported in the Western literature. Differences also existed with regard to the age and gender incidence of children with WT and its morphologic pattern, possibly due to the different genetic nature of the tumor. However, this theory requires confirmation by large epidemiologic and genetic studies.     

Mucocutaneous lymph node syndrome in Denver

Four children with acute febrile mucocutaneous lymph node syndrome (MLNS) were hospitalized in Denver over a two-year period. The children had most of the principle features of this recently described syndrome, including prolonged fever unresponsive to antibiotics, an erythematous rash, conjunctivitis, pharyngitis, and nonsuppurative cervical lymphadenitis. Three of the children exhibited characteristic desquamation from the fingertips or toes during convalescence. None developed clinical or electrocardiographic evidence of coronary thromboarteritis, the most serious complication of the disease. Additional cases undoubtedly will be reported in the United States as physicians become famililar with this new syndrome and learn to distinguish it from the other acute febrile exanthems.