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Importance: In Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD), interventions reducing the progression of myocardial disease could affect survival., Objective: To assess the effect of early angiotensin-converting enzyme (ACE) inhibitor therapy in patients with normal left ventricular function on the progression of myocardial fibrosis (MF) identified on cardiovascular magnetic resonance (CMR)., Design, Setting, and Participants: A randomized clinical trial conducted in 2 centers included 76 male patients with DMD or BMD undergoing 2 CMR studies with a 2-year interval for ventricular function and MF assessment. In a non-intent-to-treat trial, 42 patients with MF and normal left ventricular ejection fraction (LVEF) were randomized (1:1) to receive or not receive ACE inhibitor therapy. The study was conducted from June 26, 2009, to June 30, 2012. Data analysis was performed from June 30, 2013, to October 3, 2016., Interventions: Randomization (1:1) to receive or not receive ACE inhibitor therapy., Main Outcomes and Measures: Primary outcome was MF progression from baseline to the 2-year CMR study., Results: Of the 76 male patients included in the study, 70 had DMD (92%) and 6 had BMD (8%); mean (SD) age at baseline was 13.1 (4.4) years. Myocardial fibrosis was present in 55 patients (72%) and LV systolic dysfunction was identified in 13 patients (24%). Myocardial fibrosis at baseline was an independent indicator of lower LVEF at follow-up (coefficient [SE], -0.16 [0.07]; P = .03). Among patients with MF and preserved LVEF (42 [55%]), those randomized (21 patients in each arm) to receive ACE inhibitors demonstrated slower MF progression compared with the untreated group (mean [SD] increase of 3.1% [7.4%] vs 10.0% [6.2%] as a percentage of LV mass; P = .001). In multivariate analysis, ACE inhibitor therapy was an independent indicator of decreased MF progression (coefficient [SE], -4.51 [2.11]; P = .04). Patients with MF noted on CMR had a higher probability of cardiovascular events (event rate, 10 of 55 [18.2%] vs 0 of 21 [0%]; log-rank P = .04)., Conclusions and Relevance: In this 2-year, follow-up, randomized clinical trial of patients with Duchenne or Becker muscular dystrophy whose LVEF was preserved and MF was present as determined on CMR, ACE inhibitor therapy was associated with significantly slower progression of MF. The presence of MF was associated with worse patient prognosis., Trial Registration: clinicaltrials.org Identifier: NCT02432885.

Background: Neuropsychiatric comorbidities are frequent in Sydenham chorea. However, cognitive impairment in Sydenham chorea has not been sufficiently described. The objective of this study was to evaluate expressive and receptive language deficits in adolescents with Sydenham chorea., Methods: Twenty patients with Sydenham chorea were compared with 20 patients with rheumatic fever without chorea and 20 healthy controls. Participants were matched for age and gender. Participants were assessed with verbal fluency tasks (phonemic and semantic) and with verbal comprehension tasks (Token Test). Patients with Sydenham chorea were also assessed with the Universidade Federal de Minas Gerais Sydenham Chorea Rating Scale., Results: Performance in verbal fluency and in verbal comprehension tasks differed significantly (P < 0.01) among the three groups. Patients with Sydenham chorea performed significantly worse than healthy control group in phonemic and semantic verbal fluency tasks as well as in the Token Test. The group with rheumatic fever also performed worse than healthy controls in phonemic verbal fluency. Severity of motor signs in Sydenham chorea inversely correlated with performance in phonemic verbal fluency (words beginning with letter S, and total sum of words beginning with letters F, A, and S)., Conclusions: Adolescents with Sydenham chorea show difficulties in verbal fluency and in verbal comprehension. Patients with rheumatic fever also have some degree of language impairment. Future studies must investigate language impairment in difference stages of Sydenham chorea (acute, persistent, and remission) and putative biological markers., (Copyright © 2015 Elsevier Inc. All rights reserved.)

This investigation aims to analyze the profile of long-term evolution of rheumatic fever in children and adolescents and outcomes after the control of recurrences. The cohort involved 702 patients followed from 1.3 to 16.9 years covering the two periods, before and after the implementation of a prevention program. Besides the establishment of the Reference Center in the State of Minas Gerais and the implementation of strategies to promote the compliance to prophylaxis, a project for education of health professionals was carried out in 23 cities. In addition to the clinical and epidemiological profile, the severity of the disease was analyzed. Mixed lesions were found in 27.1%, valvar regurgitation in 72.9%, and complete regression of the valvar lesions was seen in 34.4% of the patients, mostly presenting mild dysfunctions. The recurrence rate per patient-year was 0.058 and out of a total of 85 recurrences, 21.4% occurred in the first and 7.5% in the second period. More severe degrees of carditis and significant valvar sequels presented a higher prevalence in patients with recurrences. The comparative analysis between the two periods showed no changes regarding the age at the primary attack, gender, type, and site of valvar lesions and affected joints; however, important modifications in the indices of severity were observed after the control of recurrences. A significant decrease in the prevalence of severe carditis, obstructive valvar sequels, hospital admissions, surgical approach, and deaths was seen. This investigation showed that although the clinical profile of presentation remains unchanged, the control of repeated attacks can improve the morbimortality rates. In this context, the secondary prophylaxis should be the first priority in the control of the disease in developing countries, taking into account the difficulties found for effective primordial and primary prevention.

Background: Acute gastroenteritis norovirus (NoV) in a country of continental dimensions like Brazil has resulted in under-reporting of the number of outbreaks, as well as the genotypes associated., Objectives: To demonstrate the role of NoV in outbreaks occurring in the State of Rio Grande do Sul, Southern Brazil, we determined its prevalence, as well as the genotypes associated, and evaluated clinical and epidemiological aspects., Study Design: NoV investigation was carried out in rotavirus group A negative stool samples from 2265 patients from 741 outbreaks that occurred in the State of Rio Grande do Sul, Brazil, during a period of eight years (2004-2011). NoV detection and nucleotide sequencing for genotype characterization was carried by using sets of primers targeting a conservative Rd-Rp polymerase genome region and the viral capsid gene, respectively., Results: NoVs were detected in 817 stool samples (36.1%) and associated with 327 outbreaks (44.1%). NoV GII.2, GII.3, GII.4, GII.6, GII.12, GII.13, GII.14, GII.15, GII.17, GII.21; and GI.1 and GI.3 were characterized. GII.4 was the most frequently detected (72.3%), with five variants identified (Asia&#95;2003, Hunter&#95;2004, Yerseke&#95;2006a, Den&#95;Haag&#95;2006b, New Orleans&#95;2009). This study describes the first detection of GI.1 and GII.13 and GII.15 in Brazil and demonstrates NoV winter-spring seasonality in this region of the country., Conclusions: NoVs were responsible for almost 50% of outbreaks, with about 70% of them resulting from genotype GII.4 and its variants. The seasonality observed could help health authorities to establish a system of active surveillance in order to reduce NoV impact especially in congregate settings., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Background: Previous studies indicate that compared with physical examination, Doppler echocardiography identifies a larger number of cases of rheumatic heart disease in apparently healthy individuals., Objectives: To determine the prevalence of rheumatic heart disease among students in a public school of Belo Horizonte by clinical evaluation and Doppler echocardiography., Methods: This was a cross-sectional study conducted with 267 randomly selected school students aged between 6 and 16 years. students underwent anamnesis and physical examination with the purpose of establishing criteria for the diagnosis of rheumatic fever. They were all subjected to Doppler echocardiography using a portable machine. Those who exhibited nonphysiological mitral regurgitation (MR) and/or aortic regurgitation (AR) were referred to the Doppler echocardiography laboratory of the Hospital das Clínicas of the Universidade Federal of Minas Gerais (HC-UFMG) to undergo a second Doppler echocardiography examination. According to the findings, the cases of rheumatic heart disease were classified as definitive, probable, or possible., Results: Of the 267 students, 1 (0.37%) had a clinical history compatible with the diagnosis of acute rheumatic fever (ARF) and portable Doppler echocardiography indicated nonphysiological MR and/or AR in 25 (9.4%). Of these, 16 (6%) underwent Doppler echocardiography at HC-UFMG. The results showed definitive rheumatic heart disease in 1 student, probable rheumatic heart disease in 3 students, and possible rheumatic heart disease in 1 student., Conclusion: In the population under study, the prevalence of cases compatible with rheumatic involvement was 5 times higher on Doppler echocardiography (18.7/1000; 95% CI 6.9/1000-41.0/1000) than on clinical evaluation (3.7/1000-95% CI).

Secondary prophylaxis remains the safest way to prevent or minimize heart valve damage in patients with rheumatic fever. However, criteria to determine the duration of prophylaxis have not been well established. This study aimed to evaluate the clinical and Doppler echocardiographic profile of patients with rheumatic fever and a normal clinical examination at least 5 years after the first episode and to discuss the contribution of Doppler echocardiography in supporting the decision to discontinue secondary prophylaxis. An observational longitudinal study analyzing 183 patients with rheumatic fever and a normal clinical examination 5 years or more after the initial attack was conducted. The patients underwent Doppler echocardiography to study the severity of mitral or aortic valvular disease. Of the 183 patients, 77 (42 %) had clinical carditis. Subclinical chronic heart disease occurred for 79 % of the patients with previous clinical carditis and for 25 % of the patients without clinical carditis. Of the 35 patients with previous clinical carditis who were in the period of discontinued prophylaxis, residual valvular heart disease was observed in all, whereas of the 62 patients without clinical carditis, only 27 % showed residual valvular heart disease. Considering Doppler echocardiographic criteria, prophylaxis would be continued for 13 (34 %) of the patients with previous clinical carditis and for only 2 (3 %) of those without clinical carditis. Return of cardiac auscultation to normal is not always accompanied by return of Doppler echocardiographic findings to normal. Criteria regarding Doppler echocardiographic findings and valve morphology should be evaluated by the time secondary prophylaxis is discontinued. However, further studies are needed to demonstrate whether prolonged prophylaxis provides any benefit to patients with persistent echocardiographic findings.

Introduction: The diagnosis of acute rheumatic fever (RF) is based on clinical findings. However, during the chronic phase of the disease, the clinical approach is not sufficient for the follow-up of the patients and the Doppler echocardiography is a tool for the diagnosis of cardiac involvement. Prognostic variables that influence long-term outcomes are not well known., Methods: 462 patients with RF according to Jones criteria were studied, and followed-up from the initial attack to 13.6 ± 4.6 years. All patients underwent clinical assessment and Doppler echocardiography for the detection of heart valve involvement in the acute and chronic phases. Multivariate logistic regression analysis was used to identify the factors influencing long-term heart valve disease., Results: Carditis occurred in 55.8% and subclinical valvulitis in 35.3% patients. In the chronic phase, 33% of the patients had significant valvular heart disease. No normal Doppler echocardiography exam was observed on patients who had severe valvulitis, although heart auscultation had become normal in 13% of these. In the multivariate analysis, only the severity of carditis and the mitral and/or aortic valvulitis were associated with significant valvular heart disease. Chorea or arthritis were protective factors for significant valvular heart disease, odds ratio 0.41 (95% C.I. 0.22 - 0.77) and 0.43 (95% C.I. 0.23 - 0.82), respectively., Conclusions: Our study suggests that the use of Doppler echocardiography during RF helps to identify prognostic factors regarding the development of significant valvular heart disease. Initial severe carditis is an important factor in the long-term prognosis of chronic RHD, whereas arthritis and chore during the initial episode of RF appears to be protective. Strict secondary prophylaxis should be mandatory in high risk patients.

Objective: To describe rubella outbreaks and control strategies in the Brazilian state of Rio Grande do Sul before rubella elimination., Methods: We analyzed rubella and congenital rubella syndrome surveillance data for the state of Rio Grande do Sul and calculated age- and gender-specific incidence of confirmed rubella cases in 2007. We obtained data on measles-rubella doses administered during the outbreak from the state immunization program and reviewed the timing of suspect case notification and implementation of control measures., Results: Of 2,842 confirmed rubella cases reported to the state health department in 2007, 2,145 (75.5%) were in males (39.5 cases per 100,000 population) and 697 (24.5%) were in females (12.3 per 100,000 population). Incidence among 15- to 39-year-olds was 1.8 to 5.5 times higher in males than in females. Rubella genotype 2B was detected in nasopharyngeal specimens from 13 patients from multiple chains of transmission. Eight children were born with congenital rubella syndrome (5.9 cases per 100 000 births in 2008). Delayed notification of initial cases hampered early control efforts, resulting in outbreak spread throughout the state. Rubella transmission was interrupted after mass vaccination of adult men and women as part of a national vaccination campaign., Conclusions: Routine vaccination strategies and mass vaccination of adolescents and adults for accelerated rubella control and elimination should target men and women.

The authors describe a case of Takayasu's arteritis in a child of only 3 years of age, emphasizing the rarity of this disease in this age group. The child was admitted to the emergency room in a post-convulsive state after a tonic-clonic seizure. After a detailed clinical examination, extensive diagnostic tests, and observation of the clinical evolution, the diagnosis was Takayasu's disease with severe cardiac and arterial involvement. The report warns pediatricians and cardiologists to awareness of the possibility of this disease in very small infants, in countries where it is underdiagnosed.

Sydenham's chorea (SC) is a complication of Streptococcus infection characterized by a combination of motor and non-motor features. We have investigated the presence of vocalizations in 89 consecutive patients with SC evaluated during a one-year period in the UFMG Movement Disorders Clinic. Seven (4/3 M/F) of the 89 patients (29/60 M/F) presented with simple vocalizations not preceded by premonitory sensations but in association with facial chorea in five patients. These findings suggest that vocalizations are not a common feature in SC and their phenomenology is quite distinct from the characteristics of vocal tics in tic disorders.

Objectives: This study sought to analyze whether cardiovascular magnetic resonance (CMR) can detect and quantify myocardial damage in the early stages of cardiomyopathy in muscular dystrophies (MD)., Background: Muscular dystrophy is a genetic disease that involves skeletal and cardiac tissues of humans. Cardiomyopathy is common, and death secondary to cardiac or respiratory diseases occurs early in life. Cardiovascular magnetic resonance is a reliable method for assessing global and regional cardiac function, allowing also for the detection of myocardial fibrosis (MF)., Methods: Ten patients with Duchenne or Becker dystrophies were studied by CMR. Physical examination, Chagas disease serological tests, electrocardiogram, chest radiograph, total creatine kinase, and Doppler echocardiogram were also obtained in all patients., Results: Patients with MF had a lower ejection fraction than those without. Myocardial fibrosis (midwall and/or subepicardial) was observed in 7 of the 10 patients, and the lateral wall was the most commonly involved segment. There was moderate correlation between segmental MF and dysfunction., Conclusions: Cardiovascular magnetic resonance can identify MF and may be useful for detecting the early stages of cardiomyopathy in MD. Future work will be needed to evaluate whether CMR can influence cardiomyopathy and outcomes.

Objective: Compare clinical and Doppler echocardiographic evaluations in assessing valvular diseases in children and adolescents with rheumatic fever, as well as assess the progression of the disease in light of these assessments., Methods: This is a longitudinal study of 258 children and adolescents diagnosed with rheumatic fever according to Jones criteria. The follow-up period ranged from 2-15 years. The presence and quantification of valve diseases were determined by means of clinical and Doppler echocardiographic evaluations performed during the acute and chronic phases. The Kappa statistics method was used to estimate the degree of agreement between clinical and Doppler echocardiographic evaluations. Comparisons between clinical and Doppler echocardiographic findings on the progress of carditis and valvulitis, respectively, were made using chi-square test or Fishers exact test, p< 0.05., Results: Of the 109 patients who underwent Doppler echocardiographic evaluation during the acute phase, 31 did not present clinical evidence of carditis, but the Doppler echocardiograms of 17 (54.8%) of them showed valve lesions (subclinical valvulitis). During the chronic phase, 153 of the 258 patients had normal cardiovascular examination results; however, Doppler echocardiograms showed that 81 of them (52.9%) had valve lesions (subclinical chronic valvular diseases). Involution of the valvular lesions, as shown by Doppler echocardiographic evaluations, was less frequent and occurred in 10 (25.0%) patients with mild valvulitis, in only one (2.5%) patient with moderate valvulitis, and in none of the patients with severe valvulitis., Conclusion: The identification of rheumatic fever valve lesions can be enhanced when clinical evaluations are supplemented by Doppler echocardiographic examinations; also, clinical examinations are not as suitable to detect valvular lesion regression as the echocardiography. The diagnosis of subclinical valvulitis and valvulopathy influences the secondary prophylaxis of rheumatic fever and endocarditis.

Three phenotypes based on the polymorphism of band-3 protein from human red cells are described. Limited proteolysis of intact red cells from most individuals (homozygotes) yields a peptide of 60 kDa, but in some cases (heterozygotes), there is also a 63-kDa peptide, and rarely only the single peptide of 63 kDa is found. This is the first description of the 63-kDa homozygote. The interpretation that the three phenotypes are controlled by two alleles of a single autosomal locus, with no dominance, is supported by population and family studies. The frequencies of the allele, which we designate as p63, is 0.041 +/- 0.0068 in Caucasoids and 0.125 +/- 0.0121 in Negroids. The electrophoretic profiles and molecular weights of the peptides obtained with several commercial proteases from Streptomyces griseus are similar to those obtained with chymotrypsin. Whereas band-3 protein in two New-World monkeys (Saimiri and Cebus) resisted pronase attack, an Old-World monkey (Macaca mulatta) was monomorphic for a 63-kDa fragment, and in an ape (Pan troglodytes), a doublet of 62 kDa and 64 kDa was found. Band-3 protein polymorphism appears to be a good marker for genetic differentiation in human populations.

The changes that occurred on the childhood's view and on the way to handle it are described here, including that refers to odontology-odontopediatrics; the influence carried out by psychology is also discussed in this paper, which sets the proposal of an assimilation of psychological knowledge by odontopediatrics guided by the aim to promote child's development.