The right ventricle in Fabry disease

Aim: Left ventricular (LV) hypertrophy is a common feature in Fabry disease-related progressive infiltrative hypertrophic cardiomyopathy and affects both men and women, but at different ages. To date, however, little is known about the role of right ventricular (RV) function in Fabry disease. Therefore, this study aimed to investigate the extent of RV involvement in patients with Fabry disease. Methods: Echocardiographic examination of the right and left ventricle was carried out in 129 patients (80 women and 49 men) with Fabry disease. Results: RV hypertrophy was present in 46 patients (35.7%). Of these patients, 13 showed signs of severely depressed right systolic function (tricuspid annulus movement <10 mm and a prolonged RV pre-ejection period/pulmonary ejection time ratio) and six patients showed additional severe depression of parameters of diastolic function (pseudo-normal or restrictive RV filling patterns). Those patients with RV hypertrophy and severely compromised systolic and diastolic function had the highest LV masses (92±11.7 g/m^2.7).
Conclusion: RV involvement is common in Fabry disease and ultimately progresses to severe systolic and diastolic RV dysfunction. These findings might explain why patients with preserved LV function can develop clinical features such as reduced exercise capacity, organomegaly and lymphoedema.     

Secondary changes in diabetes mellitus with particular reference to retinopathy and neuropathy

It has previously been suggested (1) that respiratory modulation of the action of insulin on glucose metabolism may contribute to macrosomia in neonates from diabetic mothers. It was proposed that this was due to a relative hypoxaemia which resulted in an increase in glucose metabolised through the hexose monophosphate shunt pathway in fetal adipocytes. This could be understood on the basis of the Crabtree-Pasteur Effects. It is now suggested that a similar mechanism may lead, in certain tissues, to an increase in the metabolism of glucose through polyol pathways and that this may play a role in the development of diabetic sequelae.     

The task of nursing and risk of smoking

A survey of hospital nurses was conducted to determine whether certain aspects of nurses' working and living conditions could explain their smoking practices. Nurses (246) of all grades answered a questionnaire about their smoking practices, job characteristics, job satisfaction, life satisfaction and anxieties. The prevalence of regular smoking was low among first and second year student nurses but reached 22% among final year students and 27% among staff nurses. Those nurses who reported stress at work, high and low overall job satisfaction, low lifestyle satisfaction and high anxiety about patients were more likely to smoke. The importance of these factors was especially marked among final year students. It seems that the changes in the life and work routine of nurses during their final year of training appear to increase their risk of regular smoking.