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Age on Onset, Sex, & Cardiomyopathy as Predictors of Disability and Survival in Friedreich's Disease
Neurol 47:1260-1264, DeMichele,G.,et al, 1996
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Article Abstract
We performed a retrospective study on a series of 119 of our patients who have Friedreich's disease to assess the predictive value of age at onset, gender,and left ventricular hypertrophy in regard to disease progression. Outcome variables were survival,time to loss of independent gait,and time to confinement in a wheelchair.Diabetes was considered to be an outcome variable when defining time to diabetes and an explanatory variable when testing its effect on survival.Eleven patients died.The median estimated survival from onset was 36 years,and the median time to loss of independent gait was 8 years and to confinement in a wheelchair was 15 years from onset.Nineteen patients developed diabetes after a median time of 16 years.The presence of left ventricular hypertrophy or diabetes significantly reduced survival based on univariate analysis.Onset at the age of
 
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cardiomyopathy
diabetes mellitus
disability,neurological
electrocardiogram,LVH
Friedreich's ataxia
life expectancy
prognosis
wheelchair

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