R Cheung1, D O Sillence, M C Tchan. 1. Department of Genetic Medicine, Westmead Hospital, 533, Wentworthville, NSW, 2145, Australia.
Abstract
BACKGROUND: Cerebrovascular disease (CVD) is a common clinical problem in Fabry disease; however, expression of this disease manifestation is not uniform and risk factors for its development are not well studied. A number of common CVD risk factors are known in the general population, and these may also play a role in the development of CVD in Fabry disease. AIM: To evaluate the potential associations between various risk factors and CVD in patients with Fabry disease. METHODS AND RESULTS: Thirty-two Fabry disease patients were studied, with 15 having evidence of CVD. T-tests were used to compare the positive and negative CVD groups and logistic regression was used to look for correlations with CVD history. CVD-positive patients were older (49.73 vs. 37.59 years, p<0.001) and had worse renal function (GFR 61.53 vs. 96.61 mL/min/1.73 m(2), p < 0.005), higher homocysteine (17.79 vs. 10.53 μmol/L, p < 0.05) and erythrocyte sedimentation rate (ESR) levels (23.8 vs. 7.64 mm/h, p < 0.001), and elevated Mainz Severity Score Index (MSSI) scores (23.8 vs. 11.8, p < 0.001). Correlations were found between age (odds ratio (OR) 1.11), DTPA glomerular filtration rate (OR 0.95), homocysteine concentration (OR 1.22), ESR (OR 1.16) and the MSSI (OR 1.19) scores with a positive CVD history (all p < 0.05). CONCLUSION: Elevated homocysteine and ESR are independent risk factors for CVD in Fabry disease. This finding adds to our ability to predict those patients with Fabry disease who are at a higher risk of developing CVD, and may be an aid in deciding which patients should have primary CVD prevention therapies.
BACKGROUND:Cerebrovascular disease (CVD) is a common clinical problem in Fabry disease; however, expression of this disease manifestation is not uniform and risk factors for its development are not well studied. A number of common CVD risk factors are known in the general population, and these may also play a role in the development of CVD in Fabry disease. AIM: To evaluate the potential associations between various risk factors and CVD in patients with Fabry disease. METHODS AND RESULTS: Thirty-two Fabry diseasepatients were studied, with 15 having evidence of CVD. T-tests were used to compare the positive and negative CVD groups and logistic regression was used to look for correlations with CVD history. CVD-positive patients were older (49.73 vs. 37.59 years, p<0.001) and had worse renal function (GFR 61.53 vs. 96.61 mL/min/1.73 m(2), p < 0.005), higher homocysteine (17.79 vs. 10.53 μmol/L, p < 0.05) and erythrocyte sedimentation rate (ESR) levels (23.8 vs. 7.64 mm/h, p < 0.001), and elevated Mainz Severity Score Index (MSSI) scores (23.8 vs. 11.8, p < 0.001). Correlations were found between age (odds ratio (OR) 1.11), DTPA glomerular filtration rate (OR 0.95), homocysteine concentration (OR 1.22), ESR (OR 1.16) and the MSSI (OR 1.19) scores with a positive CVD history (all p < 0.05). CONCLUSION: Elevated homocysteine and ESR are independent risk factors for CVD in Fabry disease. This finding adds to our ability to predict those patients with Fabry disease who are at a higher risk of developing CVD, and may be an aid in deciding which patients should have primary CVD prevention therapies.
Authors: Allon N Friedman; Andrew G Bostom; Jacob Selhub; Andrew S Levey; Irwin H Rosenberg Journal: J Am Soc Nephrol Date: 2001-10 Impact factor: 10.121
Authors: David F Moore; Gheona Altarescu; W Craig Barker; Nicholas J Patronas; Peter Herscovitch; Raphael Schiffmann Journal: Brain Res Bull Date: 2003-12-30 Impact factor: 4.077