Mladenovic J, Pekmezovic T, Todorovic S, Rakocevic-Stojanovic V, Savic D, Romac S, Apostolski S
Clinic for Neurology and Psychiatry for Children and Adolescents, Belgrade, Serbia and Montenegro.
Eur J Neurol. 2006 May;13(5):451-4. doi: 10.1111/j.1468-1331.2006.01261.x.
The purpose of this investigation was to determine survival and mortality in patients with myotonic dystrophy type 1 (DM1) in the Belgrade population within the period from 1983 to 2002. Data of a number of diagnosed DM1 patients with their demographic, clinical and genetic characteristics were gathered from hospital records in all neurologic institutions in Belgrade for the period 1983-2002. Death certificates were reviewed to determine the cause of death. Survival analysis by life table method and Cox proportional hazard model was performed. Within the observed period, in the population of Belgrade, 15 fatal outcomes among 101 patients with DM1 were registered. Average DM1 mortality rate was 0.5/1,000,000 (95% CI 0.3-0.8), and standardized mortality ratio (SMR) was 5.3. A significant inverse correlation was found between age at onset of DM1 and CTG repeats (P=0.023). The cumulative probability of 15-year survival for DM1 patients in Belgrade was 49+/-5% (48+/-2% for males and 50+/-7% for females). Younger age at onset was a significant unfavorable prognostic factor (hazard ratio=4.2; P=0.012).
本研究的目的是确定1983年至2002年期间贝尔格莱德人群中1型强直性肌营养不良（DM1）患者的生存率和死亡率。收集了1983 - 2002年期间贝尔格莱德所有神经科机构医院记录中大量已诊断DM1患者的人口统计学、临床和遗传学特征数据。审查死亡证明以确定死因。采用寿命表法和Cox比例风险模型进行生存分析。在观察期内，贝尔格莱德人群中101例DM1患者中有15例出现致命结局。DM1平均死亡率为0.5/1,000,000（95%可信区间0.3 - 0.8），标准化死亡比（SMR）为5.3。发现DM1发病年龄与CTG重复序列之间存在显著负相关（P = 0.023）。贝尔格莱德DM1患者15年生存的累积概率为49±5%（男性为48±2%，女性为50±7%）。发病年龄较小是一个显著的不良预后因素（风险比 = 4.2；P = 0.012）。
