Résumé :
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[BDSP. Notice produite par INIST-CNRS GR0x4CVH. Diffusion soumise à autorisation]. The objective of this study was to estimate probability of survival of Huntington's disease (HD) patients in Serbia as a function of CAG repeat length and selected demographic variables. This follow-up study was carried out at the Institute of Neurology, Clinical Centre of Serbia, Belgrade, 1982-2004. The study group consisted of 112 HD patients. The significant inverse correlation was found between CAG repeat length and age at onset of HD (r=-0.732, P=0.001) and age at death (r=-0.760, P=0.001). The cumulative probabilities of survival in a five, ten, fifteen, and twenty-years'period were 90.9,63.2,10.3 and 4.5%, respectively. Higher survival probabilities were registered in female patients, as well as in those with older age at onset and lower number of CAG repeat length (<=46). The Cox regression analysis showed that significantly poorer outcome of HD in our population was related to younger age at onset (HR-hazard ratio=1.9 ; P=0.047), and larger CAG numbers (HR=2.4 ; P=0.071). The female sex was statistically significantly associated with longer survival (HR=0.4 ; P=0.007). These data might be of some importance for further exploration of natural history and prognosis of HD.
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