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目的探讨家族性蛛网膜下腔出血(subarachnoid hemorrhage,SAH)的发病情况及临床特点,以降低此病死亡率、致残率。方法回顾分析5个家系第一、二或三代18例SAH患者的临床资料。结果本组家族性SAH18例与颅内动脉瘤的破裂密切相关,男性发病率高于女性,Hunt和Hess分级法,Ⅴ级患者达55.56%;第一次出血率为83.33%,24h内死亡率55.56%,总死亡率77.78%。结论家族性SAH多系动脉瘤破裂所致,有家族聚集性,发病急,病情凶险,短时间内死亡率高,并发症严重,预后较差。
Objective To investigate the incidence and clinical characteristics of subarachnoid hemorrhage (SAH) in order to reduce the mortality and morbidity of this disease. Methods The clinical data of 18 SAH patients in the first, second or third generation of five pedigrees were retrospectively analyzed. Results The 18 cases of familial SAH were closely related to the rupture of intracranial aneurysm. The incidence of males was higher than that of females. The Hunt and Hess grading methods accounted for 55.56% of grade Ⅴ patients. The first hemorrhage rate was 83.33% 55.56%, total mortality 77.78%. Conclusions The familial SAH multi-line aneurysm ruptures, with familial aggregation, acute onset, dangerous condition, high mortality rate in a short time, serious complications and poor prognosis.