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婴儿痉挛症:神经学及发育随访——以色列两个族群的比较:以色列犹太人与以色列南部的贝都因人

Infantile spasms: neurological and developmental follow-up--a comparison between two ethnic groups: Israeli Jews and Bedouin in the South of Israel.

作者信息

Vardi Gideon, Snapir Shir Melamed, Merick Joav, Shorer Zamir, Levy Jacov, Friger Michael, Galil Aharon

机构信息

Zusman Child Development Center, Soroka University Medical Center, Ben Gurion University of the Negev, Beer-Sheva, Israel.

出版信息

Med Sci Monit. 2005 Mar;11(3):CR117-22.

Abstract

BACKGROUND

The aim of this study was to examine all cases of infantile spasms (IS) diagnosed at the Soroka University Medical Center, Ben Gurion University, Israel during 1981--1997.

MATERIAL/METHODS: 31 children, 17 (55%) males. 17 (55%) were Jews and 14 (45%) Bedouins. Four (13%) died. Data was gathered from hospital files, neuropediatric unit and Zusman Child Development Center. Demographic and ethnic data, characteristics of the disease type of seizures, EEG pattern imaging studies, type of treatment, psychomotor development, rehabilitation and educational services were analysed.

RESULTS

Mean age at diagnosis 7.22 months. Etiology for one third was pre- or perinatal insult, one third postnatal and one third unknown. 26 (84%) were symptomatic and five (16%) cryptogenic. Significant statistical difference was found with more Bedouin children symptomatic with moderate or severe mental retardation, cerebral palsy with severe motor difficulties and recurrence of the disease. Statistically significant correlation existed between poor response to initial treatment and placement in special education, recurrence of disease and cerebral palsy with moderate or severe motor difficulties, the appearence of a different type of epilepsy during follow-up and placement in special education. 18 children (58%) received first treatment with ACTH, 10 (32%) children with IVIG (immunoglobolin iv), 3 (10%) with neither ACTH nor IVIG. Regarding therapy response we found no significant difference between Jews and Bedouins.

CONCLUSIONS

No significant statistical difference was found between the two treatments concerning clinical course or developmental follow-up. Due to small numbers multi-center research is needed.

摘要

背景

本研究旨在调查1981年至1997年期间在以色列本古里安大学索罗卡大学医学中心诊断的所有婴儿痉挛症(IS)病例。

材料/方法:31名儿童,17名(55%)为男性。17名(55%)为犹太人,14名(45%)为贝都因人。4名(13%)死亡。数据收集自医院档案、神经儿科病房和祖斯曼儿童发展中心。分析了人口统计学和种族数据、疾病类型、癫痫发作特征、脑电图模式成像研究、治疗类型、精神运动发育、康复和教育服务。

结果

诊断时的平均年龄为7.22个月。三分之一的病因是产前或围产期损伤,三分之一是产后损伤,三分之一病因不明。26名(84%)为症状性病例,5名(16%)为隐源性病例。发现贝都因儿童在中度或重度智力低下、伴有严重运动困难的脑瘫和疾病复发方面有更多症状性病例,存在显著统计学差异。初始治疗反应不佳与接受特殊教育、疾病复发和伴有中度或重度运动困难的脑瘫、随访期间出现不同类型癫痫以及接受特殊教育之间存在统计学显著相关性。18名儿童(58%)首次接受促肾上腺皮质激素(ACTH)治疗,10名(32%)儿童接受静脉注射免疫球蛋白(IVIG)治疗,3名(10%)既未接受ACTH也未接受IVIG治疗。关于治疗反应,我们发现犹太人和贝都因人之间没有显著差异。

结论

两种治疗方法在临床病程或发育随访方面未发现显著统计学差异。由于样本量小,需要进行多中心研究。

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