急性全自主神经功能不全13例临床分析  

Clinical analysis of 13 cases with acute pandysautonomia

作　　者：杨欣英[1] 韩彤立[1] 丁昌红[1] 吕俊兰[1] 李久伟[1] 张珅[1] 巩帅[1] 张炜华[1] 周锦[2] 刘婷婷[3] Yang Xinying;Han Tongli;Ding Changhong;Lyu Junlan;Li Jiuwei;Zhang Shen;Gong Shuai;Zhang Weihua;Zhou Jin;Liu Tingting(Department of Neurology,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China;Department of Gastroenterology,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China;Department of Emergency,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China)

机构地区：[1]国家儿童医学中心,首都医科大学附属北京儿童医院神经科,北京100045 [2]国家儿童医学中心,首都医科大学附属北京儿童医院消化科,北京100045 [3]国家儿童医学中心,首都医科大学附属北京儿童医院急诊科,北京100045

出　　处：《中华实用儿科临床杂志》2020年第15期1166-1168,共3页Chinese Journal of Applied Clinical Pediatrics

摘　　要：目的总结急性全自主神经功能不全(APD)患儿临床资料,探讨其治疗方案及预后。方法回顾性分析2010年1月至2019年12月首都医科大学附属北京儿童医院神经科收治的13例APD患儿资料。采集患儿一般资料、临床症状、自主神经查体及功能测试、实验室检查、治疗方案及预后随访进行分析。结果 13例患儿中,男4例,女9例;平均年龄8岁5个月(3岁8个月~12岁5个月);平均确诊时病程94.5 d(14~410 d)。常见首发症状为胃肠道动力障碍(11例)、排尿困难(3例)、直立晕厥/眩晕(3例)。病程中患儿均存在胃肠道动力障碍及皮肤泌汗障碍,腺体受累及体位性低血压各12例,瞳孔异常9例,尿潴留7例。其他症状包括乏力9例,情绪障碍4例,肌力下降、感觉障碍各2例。患儿均接受静脉用免疫球蛋白(IVIG)治疗,3例联合糖皮质激素。胃肠道症状严重无法进食者6例,均接受静脉营养治疗;4例接受空肠喂养,其中3例1~12个月恢复正常饮食,1例随访5年2个月仍未恢复。低钠血症7例,对症治疗2~30 d恢复。9例患儿随访1个月~9年,7例正常学习及工作,营养状态满意,情绪稳定,无病情反复及复发。结论 APD临床表现多样,首发症状多为胃肠道动力障碍、体位性低血压、尿潴留,常见低钠血症。针对症状进行个体化多学科综合管理,尤其是胃肠道动力障碍相关综合治疗、体位性低血压及泌尿系统管理及低钠血症的判断、个体化治疗可有效改善预后。Objective To summarize the clinical data of patients with acute pandysautonomia(APD)and discuss the treatment and prognosis of them.Methods A total of 13 patients with APD in the Department of Neurology,Beijing Children′s Hospital,Capital Medical University,from January 2010 to December 2019,were investigated retrospectively.The general data,clinical symptoms,autonomic nerve examination and function test,laboratory examination,treatment and follow-up were collected and analyzed.Results There were 4 males and 9 females in 13 patients with APD,with an average age was 8 years and 5 months(3 years and 8 months to 12 years and 5 months).The average course of disease was 94.5 d(14-410 d).The common initial symptoms were gastrointestinal motility disorder(11 cases),dysuria(3 cases),and upright syncope/vertigo(3 cases).During the course of the disease,all the patients manifested with gastrointestinal motility disfunction and dyshidrosis,glands involvement and orthostatic hypotension in 12 cases,abnormal pupil in 9 case and urinary retention in 7 case.Other symptoms included fatigue in 9 cases,emotional disorder in 4 cases,limb weakness in 2 cases,and sensory disturbance in 2 cases.All the patients were treated with intravenous immunoglobulin(IVIG),and 3 cases combined with glucocorticoid.Six patients with severe gastrointestinal symptoms were treated with intravenous nutrition;4 patients were fed with jejunum,3 cases of whom returned to normal diet within 1-12 months,and 1 patient was followed up for 5 years and 2 months.Hyponatremia was found in 7 cases,which recovered in 2-30 d.Nine cases were followed up for 1 month to 9 years.Seven cases were normal in daily work and study,with satisfactory nutritional status,stable mood and no relapse.Conclusions The clinical manifestations of APD are varied.The initial symptoms are gastrointestinal motility disorders,orthostatic hypotension,urinary retention and hyponatremia.Individualized multi-disciplinary comprehensive management for symptoms,especially the comprehensive treat

关 键 词：急性全自主神经功能不全 自主神经 低钠血症 空肠喂养 

分 类 号：R748[医药卫生—神经病学与精神病学]