Marfan's syndrome: early and severe form in siblings

Marfan's syndrome is an inherited disorder of the connective tissue. Cardiologic manifestations, especially aortic dilation, are important causes of morbidity and mortality in the clinical course of the disease in adults and teenagers. In children, the presence of aortic aneurysm and its dissection or rupture is rare, occurring in patients with genetic mutation of the fibrillin gene but not in those who have the familial form of the disease. We describe here 2 patients, from the same family (siblings), diagnosed with gigantic aortic aneurysm early in infancy, one of them successfully undergoing surgery.

Noninvasive cardiac assessment in children of women with systemic lupus erythematosus

OBJECTIVE: Noninvasive cardiac assessment of newborns and infants of women with systemic lupus erythematosus. The children had no congenital total atrioventricular block and were compared with the children of healthy women. METHODS: We prospectively assessed 13 newborns and infants aged 1 to 60 days, children of women with systemic lupus erythematosus and without congenital total atrioventricular block. These children were compared with 30 children of women who had no lupus or anti-Ro/SSA antibodies, and no risk factors for congenital heart disease either. Their age groups matched. The following examinations were performed: cardiological physical examination, electrocardiography, echocardiography, and signal-averaged electrocardiography. RESULTS: The statistical analysis showed no significant difference in ventricular function or in the cardiac conduction system between the groups. CONCLUSION: In regard to the conduction system and ventricular function in the absence of total atrioventricular block, no statistically significant difference was observed between the children of women with systemic lupus erythematosus and children of healthy women.

An introduction to structural botany.

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Avaliação do conhecimento de pacientes de reabilitação cardíaca: Brasil versus Canadá

FUNDAMENTO: Grande parte da relação entre o estado de saúde e o conhecimento sobre saúde e doença pode ser atribuída aos efeitos combinados de comportamento dísparrelacionado à saúde, condições ambientais e estruturas socioeconômicas, bem como o contato com a atenção à saúde e prestação da mesma. OBJETIVO: O objetivo do presente estudo foi descrever e comparar o conhecimento dos pacientes com doença arterial coronariana (DAC),incluídos em programas de reabilitação cardíaca (RC) no Brasil e no Canadá, sobre os fatores relacionados à DAC. MÉTODOS: Duas amostras de 300 pacientes brasileiros e 300 pacientes canadensesincluídos em RC foram comparadas transversalmente. Os pacientes brasileiros foram recrutados de dois centros de RC no Sul do Brasil, enquanto os pacientes canadenses foram recrutados de um centro de RC em Ontário. O conhecimento foi avaliado utilizando o Questionário de Educação de Doença Arterial Coronariana (CADE-Q), psicometricamente validado em Português e Inglês. Os dados foram processados por meio de estatísticas descritivas, post-hoc eteste t de Student. RESULTADOS: A pontuação média total de conhecimento para toda a amostra foi de 41,42 ± 9,3. Os entrevistados canadenses apresentaram pontuações totais de conhecimento significativamente maiores do que os entrevistados brasileiros. O domínio mais bem esclarecido em ambas as amostras foi o exercício físico.Em 13 de 19 questões, os entrevistados canadenses relataram pontuações de conhecimento significativamente maiores do que os entrevistados brasileiros. CONCLUSÃO: Pacientes ambulatoriais canadenses relataram conhecimento significativamente maior que suas contrapartes brasileiras. Os resultados também sugeremque um currículo educacional estruturado em programas de RC pode contribuir para um maior conhecimentodo paciente, o que pode em última análise facilitar as mudanças comportamentais.

A Comparison of Radiation Dose Between Standard and 3D Angiography in Congenital Heart Disease

Background: The use of three-dimensional rotational angiography (3D-RA) to assess patients with congenital heart diseases appears to be a promising technique despite the scarce literature available. Objectives: The objective of this study was to describe our initial experience with 3D-RA and to compare its radiation dose to that of standard two-dimensional angiography (2D-SA). Methods: Between September 2011 and April 2012, 18 patients underwent simultaneous 3D-RA and 2D-SA during diagnostic cardiac catheterization. Radiation dose was assessed using the dose-area-product (DAP). Results: The median patient age and weight were 12.5 years and 47.5 Kg, respectively. The median DAP of each 3D-RA acquisition was 1093µGy.m2 and 190µGy.m2 for each 2D-SA acquisition (p<0.01). In patients weighing more than 45Kg (n=7), this difference was attenuated but still significant (1525 µGy.m2 vs.413µGy.m2, p=0.01). No difference was found between one 3D-RA and three 2D-SA (1525µGy.m2 vs.1238 µGy.m2, p = 0.575) in this population. This difference was significantly higher in patients weighing less than 45Kg (n=9) (713µGy.m2 vs.81µGy.m2, P = 0.008), even when comparing one 3D-RA with three 2D-SA (242µGy.m2, respectively, p<0.008). 3D-RA was extremely useful for the assessment of conduits of univentricular hearts, tortuous branches of the pulmonary artery, and aorta relative to 2D-SA acquisitions. Conclusions: The radiation dose of 3D-RA used in our institution was higher than those previously reported in the literature and this difference was more evident in children. This type of assessment is of paramount importance when starting to perform 3D-RA.

A monograph of the British Spongiadæ. By J. S. Bowerbank.

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A monograph of the British Spongiadæ. By J. S. Bowerbank.

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A monograph of the British Spongiadæ. By J. S. Bowerbank.

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A monograph of the British Spongiadæ. By J. S. Bowerbank.

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