FAMILIAL DILATED CARDIOMYOPATHY IN THE UNITED-KINGDOM

OBJECTIVES--To determine the frequency and mode of inheritance of familial dilated cardiomyopathy in the United Kingdom. BACKGROUND--Two recent prospective studies have suggested that familial forms of dilated cardiomyopathy are common but have been limited by selective screening methods, inadequate diagnostic criteria, and low rates of ascertainment. METHODS--Prospective screening study of 236 relatives from 40 families of patients with dilated cardiomyopathy. Screening consisted of clinical examination, 12 lead electrocardiogram, and two-dimensional Doppler echocardiography. Relatives with systemic hypertension and other cardiac diseases were excluded from the study. All echocardiograms were performed by an experienced echocardiographer who was blinded to clinical information. Relatives were classified as having dilated cardiomyopathy, left ventricular enlargement (method of Henry), depressed fractional shortening, or as being normal. Relatives with abnormal investigations underwent further evaluation as appropriate. RESULTS--Twenty five cases of dilated cardiomyopathy were identified and came from 10 (25%) of the 40 families screened. Pedigree analysis was most consistent with autosomal dominant inheritance and variable penetrance (65-95%). Of the remaining apparently healthy relatives, 37 (18%) were found to have left ventricular enlargement and nine (4%) depressed fractional shortening; these values were significantly higher than those observed in 239 healthy controls (24 (10%), P = 0.02 and one (0.4%), P = 0.01, respectively). CONCLUSIONS--Patients with dilated cardiomyopathy commonly have an affected family member and a high proportion of apparently healthy relatives with minor echocardiographic abnormalities. Segregation analysis suggests that familial dilated cardiomyopathy is the result of the transmission of a rare autosomal dominant gene. Further studies are currently underway to characterise the molecular basis of familial dilated cardiomyopathy and identify early disease within these families

Enterprise Ownership, Market Competition and Manufacturing Priorities in a Sub-Saharan African Emerging Economy: Evidence from Ghana

The economic liberalization policies being implemented by many African economies have led to significant efficiency and performance improvements in the activities of privately owned enterprises. This study examines the effect of the economic liberalization policies on the entrepreneurial development of domestic-owned enterprises. This is done by examining how the type of enterprise ownership (wholly domestic-owned enterprises vs. foreign–domestic joint ventures enterprises), and the increase in competition affect the manufacturing priorities of privately owned enterprises in Ghana. The results show that the enhancement in manufacturing efficiency and quality improvement in privately owned enterprises could be traced to the activities of foreign–domestic joint venture enterprises. However, as market competition increases, wholly domestic-owned enterprises emphasize manufacturing efficiency and quality improvement more that foreign–domestic joint venture enterprises. Implications for policy are discussed. Copyright Springer 2005economic liberalization, foreign–domestic joint ventures, manufacturing priorities, market competition, Ghana, wholly domestic-owned enterprises,