Survival of Huntington's disease patients in Serbia: longer survival in female patients.
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.
Pekmezovic
Tatjana
T
Institute of Neurology, Clinical Centre of Serbia, School of Medicine, University of Belgrade, Dr Subotica 6, 11000, Belgrade, Serbia. pekmezovic@sezampro.yu
Svetel
Marina
M
Maric
Jelena
J
Dujmovic-Basuroski
Irena
I
Dragasevic
Natasa
N
Keckarevic
Milica
M
Romac
Stanka
S
Kostic
Vladimir S
VS
D000328:Adult; D017677:Age Distribution; D017668:Age of Onset; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006816:Huntington Disease / Q000453:epidemiology / Q000235:genetics* / Q000401:mortality*; D008297:Male; D011379:Prognosis; D016016:Proportional Hazards Models; D017678:Sex Distribution; D016019:Survival Analysis; D018911:Trinucleotide Repeats / Q000235:genetics*; D015022:Yugoslavia / Q000453:epidemiology