摘要目的 探讨不同类型儿童吉兰-巴雷综合征(GBS)的临床特点及丙种球蛋白(IVIG)的治疗效果.方法 回顾性分析了我科近5年住院诊治的108例GBS患儿,其中本组75例患儿均在急性期应用大剂量IVIG 400 mg/(kg·d)静点治疗5 d,收集患儿的临床、电生理资料和治疗效果,并对患儿病情恢复进行随访.结果 75例GBS患儿中急性运动性轴索型GBS(AMAN)34例(45.3%),急性炎症性脱髓鞘多发性神经病(AIDP)32例(42.7%),急性运动感觉性轴索型GBS(AMSAN)3例(4.0%),神经失电位型4例(5.3%),难以分类2例(2.7%).AIDP型起病达病情高峰时间明显比AMAN型长,差异有统计学意义(t=3.4042,P＜0.01);病情高峰时Hughes功能障碍评分,AIDP和AMAN型差异无统计学意义(x2=1.5997,P＞0.05).二者在呼吸肌麻痹、颅神经麻痹及植物神经症状方面差异无统计学意义;AIDP型患儿感觉障碍症状明显多于AMAN型,二者差异有统计学意义(x2=6.0475,P＜0.05).经IVIG治疗后AIDP和AMAN型肌力开始改善平均时间分别为(5.59±3.63)、(7.21±4.68)d,二者经治疗肌力开始改善时间AIDP型较AMAN型短,但差异没有统计学意义(t=-1.5702,P＞0.05);肌力提高1级所需时间AIDP和AMAN型分别为(8.88±4.39)、(12.67±8.35)d,二者经治疗肌力提高一级的时间AIDP型比AMAN短4 d左右,差异有统计学意义(t=-2.3689,P＜0.05).本组无1例死亡,随访调查的病例中AIDP型和AMAN型治疗后完全恢复时间差异无统计学意义(t=0.2041,P＞0.05).结论 AMAN型患儿临床进展速度较AIDP型快,除感觉神经受累方面AIDP型多于AMAN型患儿外,二者在肌无力严重程度、呼吸肌麻痹、颅神经麻痹及植物神经受累方面无明显差异.经IVIG治疗AIDP型临床恢复比AMAN型快,但AIDP和AMAN型长期预后无明显差异.

abstractsObjective To study the clinical characteristics and effects of immunoglobulin treatment hospitalized for GBS were retrospectively analyzed; 75 cases in this group were given acute high dose of gamma globulin(IVIG)400mg/(kg·d)intravenously for 5d.Clinical and electrophysiological data and information on treatment and recovery of the children were collected during the follow-up and were analyzed.Result According to the clinical and electrophysiologic findings, 32 patients manifested acute inflammatory demyelinating polyradiculoneuropathy( AIDP), 34 had acute motor axonal neuropathy( AMAN), 3 had acute motor and sensory axonal neuropathy (AMSAN), 4 were inexcitable, 2 were unclassified. The clinical progress of the AMAN was faster than the AIDP group. Except for sensory nerve involvement, there was no significant difference in the clinical feature and severity. The mean time of the muscle strength began to recover was (5.59 +3.63) days in the AIDP group and (7. 21 ±4.68) days in the AMAN group after IVIG treatment. The time of the AIDP group was shorter than the AMAN group, but the difference was not statistically significant ( t = - 1. 5702, P ＞ 0. 05 ). The mean time of the muscle strength increased one grade was (8.88 ±4. 39) days in the AIDP group and ( 12. 67 ±8. 35) days in the AMAN group. The difference was statistically significant ( t = - 2. 3689, P ＜ 0. 05 ). No patients in this group died. Follow-up data showed that the complete recovery time was not significantly different ( t = 0. 2041, P ＞ 0. 05 ). Conclusion The clinical progress of the AMAN was faster than the AIDP group. Besides sensory nerve involvement,there was no significant difference in the clinical feature and severity. The AIDP group's clinical recovery was faster than AMAN's after the immunoglobulin treatment. The two groups were not significantly different in long-term prognosis.