Atenolol versus losartan in children and young adults with Marfan's syndrome

BACKGROUND : Aortic-root dissection is the leading cause of death in Marfan's syndrome. Studies suggest that with regard to slowing aortic-root enlargement, losartan may be more effective than beta-blockers, the current standard therapy in most centers. METHODS : We conducted a randomized trial comparing losartan with atenolol in children and young adults with Marfan's syndrome. The primary outcome was the rate of aortic-root enlargement, expressed as the change in the maximum aortic-root-diameter z score indexed to body-surface area (hereafter, aortic-root z score) over a 3-year period. Secondary outcomes included the rate of change in the absolute diameter of the aortic root; the rate of change in aortic regurgitation; the time to aortic dissection, aortic-root surgery, or death; somatic growth; and the incidence of adverse events. RESULTS : From January 2007 through February 2011, a total of 21 clinical centers enrolled 608 participants, 6 months to 25 years of age (mean [+/- SD] age, 11.5 +/- 6.5 years in the atenolol group and 11.0 +/- 6.2 years in the losartan group), who had an aorticroot z score greater than 3.0. The baseline-adjusted rate of change (+/- SE) in the aortic-root z score did not differ significantly between the atenolol group and the losartan group (-0.139 +/- 0.013 and -0.107 +/- 0.013 standard-deviation units per year, respectively; P = 0.08). Both slopes were significantly less than zero, indicating a decrease in the degree of aortic-root dilatation relative to body-surface area with either treatment. The 3-year rates of aortic-root surgery, aortic dissection, death, and a composite of these events did not differ significantly between the two treatment groups. CONCLUSIONS : Among children and young adults with Marfan's syndrome who were randomly assigned to losartan or atenolol, we found no significant difference in the rate of aorticroot dilatation between the two treatment groups over a 3-year period

Nature and frequency of genetic disease

No abstract available

Psychosocial adaptation in adolescents and young adults with Marfan syndrome: an exploratory study.

We conducted a pilot study to evaluate the psychological effects and consequences of Marfan syndrome in 17 patients between 16 and 35 years of age. Through a semi-structured interview, we investigated how the patients coped daily with Marfan syndrome and evaluated the impact of the disease on specific items such as schooling, occupational choices, self-image, and social behaviour. A second part of the study consisted of a battery of standardised psychological tests to evaluate the patients' anxiety and depression levels and coping styles. The following psychological tests were used: State and Trait Anxiety Inventory (STAI), Beck Depression Inventory (BDI), and the Utrecht Coping List (UCL). The interviews showed that the disease represented a significant burden on the daily physical activities of the patients, as well as on their schooling and job opportunities. During childhood, most of them were insensitively teased by peers because of their typical phenotypic features. This had its consequences on their social behaviour in that they are all more introvert. In the female patients, the risk associated with child bearing represented a major concern. Most patients emphasised the need for accurate information about the illness immediately after knowing the diagnosis and all found psychological support helpful. Depression and anxiety levels were higher in the female than in the male group, without being significantly different from the normal population. Nevertheless, most patients have come to terms with their disease and consider themselves happy most of the time