Adams R, McKie V, Nichols F, Carl E, Zhang D L, McKie K, Figueroa R, Litaker M, Thompson W, Hess D
Department of Neurology, Medical College of Georgia, Augusta 30912.
N Engl J Med. 1992 Feb 27;326(9):605-10. doi: 10.1056/NEJM199202273260905.
Stroke, especially cerebral infarction, is a major cause of morbidity and mortality in children with sickle cell disease. Primary prevention of stroke by transfusion therapy may be feasible if there is a way to identify the patients at greatest risk. Transcranial Doppler ultrasonography can measure flow velocity in the large intracranial arteries. The narrowing of these arteries, which leads to cerebral infarction, is characterized by an increased velocity of flow.
Using transcranial Doppler ultrasonography, we prospectively measured the velocity of cerebral blood flow in children and young adults being followed because of sickle cell disease. The results were classified as either normal or abnormal on the basis of the highest velocity of flow in the middle cerebral artery. Abnormal velocity was defined as a flow greater than or equal to 170 cm per second, a definition determined by post hoc analysis to maximize the predictive success of the test. The end point was a clinically apparent first cerebral infarction.
Two hundred eighty-three transcranial ultrasound examinations were performed in 190 patients with sickle cell disease (age at entry, 3 to 18 years). After an average follow-up of 29 months, cerebral infarction was diagnosed in seven patients. In 23 patients the results of the ultrasound examinations were abnormal, and in 167 patients they were normal. The clinical and hematologic characteristics of the two groups were similar, but six of the seven strokes occurred among the 23 patients with abnormal ultrasound results (P less than 0.00001 by Fisher's exact test). In this group, the relative risk of stroke was 44 (95 percent confidence interval, 5.5 to 346).
Transcranial ultrasonography can identify the children with sickle cell disease who are at highest risk for cerebral infarction. Periodic ultrasound examinations and the selective use of transfusion therapy could make the primary prevention of stroke an achievable goal.
中风,尤其是脑梗死,是镰状细胞病患儿发病和死亡的主要原因。如果有一种方法能够识别出风险最高的患者,那么通过输血疗法对中风进行一级预防可能是可行的。经颅多普勒超声检查可测量颅内大动脉的血流速度。这些动脉的狭窄会导致脑梗死,其特征是血流速度增加。
我们使用经颅多普勒超声检查,对因镰状细胞病而接受随访的儿童和青年进行了前瞻性的脑血流速度测量。根据大脑中动脉的最高血流速度,将结果分为正常或异常。异常速度定义为血流速度大于或等于每秒170厘米,这一定义是通过事后分析确定的,以使该检查的预测成功率最大化。终点是临床上明显的首次脑梗死。
对190例镰状细胞病患者(入组年龄3至18岁)进行了283次经颅超声检查。平均随访29个月后,7例患者被诊断为脑梗死。23例患者的超声检查结果异常,167例患者的结果正常。两组的临床和血液学特征相似,但7例中风患者中有6例发生在23例超声检查结果异常的患者中(费舍尔精确检验,P<0.00001)。在这组患者中,中风的相对风险为44(95%置信区间,5.5至346)。
经颅超声检查能够识别出镰状细胞病患儿中脑梗死风险最高的患者。定期进行超声检查并选择性地使用输血疗法可使中风的一级预防成为一个可实现的目标。