Crawford T O, Skolasky R L, Fernandez R, Rosquist K J, Lederman H M
Department of Neurology, The Johns Hopkins Hospital, Baltimore, Maryland 21287, USA.
Arch Dis Child. 2006 Jul;91(7):610-1. doi: 10.1136/adc.2006.094268.
Ataxia telangiectasia is a rare, multiorgan neurodegenerative disorder with enhanced vulnerability to cancer and infection. Median survival in two large cohorts of patients with this disease, one prospective and one retrospective, is 25 and 19 years, with a wide range. Life expectancy does not correlate well with severity of neurological impairment.
共济失调毛细血管扩张症是一种罕见的多器官神经退行性疾病,对癌症和感染的易感性增强。在两个大型患者队列中,一个是前瞻性队列,另一个是回顾性队列,该疾病患者的中位生存期分别为25年和19年,范围较广。预期寿命与神经功能障碍的严重程度相关性不佳。