[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"$f65-g5LTUgHIk8dGZIHhGe2pKpxnfW0g-O4HGxk3yxcs":3,"$fIve2x4PbxwNfXRNRy8lrWeM7bKcdF5zsbcKOrvlM4m4":8},{"code":4,"msg":5,"message":6,"data":6,"success":7},401,"认证失败，无法访问系统资源",null,false,{"code":9,"msg":10,"message":6,"data":11,"success":37},200,"操作成功",{"id":12,"name":13,"describes":6,"symptoms":6,"checkup":6,"treatment":6,"seoTitle":6,"seoKeywords":6,"seoDescription":6,"coverVertical":6,"coverAcross":6,"introduceClassifications":14,"departments":35},8350,"小儿丙酸血症",[15,20,25,30],{"id":16,"classification":17,"description":18,"sort":19,"diseaseId":12},141373,"概述","\u003Cp>　　丙酸血症(propionic acidemia)是丙酸分解代谢过程中的一种遗传性缺陷，系丙酰辅酶A羧化酶(propionyl CoA carboxylase)缺乏所致，为常染色体隐性遗传。以反复发作的代谢性酮症酸中毒，蛋白质不耐受和血浆甘氨酸水平显著增高为特征。本病多在摄入蛋白尤其是富含支链氨基酸、甲硫氨酸和苏氨酸饮食后发作。病人血中有大量丙酸积聚，白细胞中丙酸氧化障碍，成纤维细胞中羧化酶缺陷等。\u003C/p>",0,{"id":21,"classification":22,"description":23,"sort":24,"diseaseId":12},141374,"症状","\u003Cp>　　常见症状：生长缓慢、骨质疏松、肌张力降低、拒食、嗜睡、昏迷、脱水、呕吐、惊厥\u003C/p>\u003Cp>　　新生儿期出现酮症或酸中毒均应考虑到丙酸羧化缺陷。诊断需测定血或尿中丙酸及其代谢产物浓度，以及白细胞或成纤维细胞中丙酰辅酶A羧化酶活性，酶活性测定才能最终确诊。对高危新生儿测定脐血中酶活性可诊断。\u003C/p>",1,{"id":26,"classification":27,"description":28,"sort":29,"diseaseId":12},141375,"检查","\u003Cp>　　检查项目：尿常规、血常规、羊水检查、颅脑MRI检查、颅脑CT检查\u003C/p>\u003Cp>　　脑电图、脑CT检查，可见异常脑波、脑萎缩，腹部B超可见肝脏肿大。X线检查可见骨质疏松。\u003C/p>",2,{"id":31,"classification":32,"description":33,"sort":34,"diseaseId":12},141376,"治疗","\u003Cp>　　(一)治疗\u003C/p>\u003Cp>　　低蛋白0.5～1.5g/(kg·d)或低丙酸前体饮食为目前最佳治疗，可减少酮症酸中毒发作次数。由于空腹会增加丙酸代谢物排泄，故应增加喂养次数。酮症酸中毒发作时应立即停止所有含蛋白饮食，并给予葡萄糖以避免分解代谢。急性发作尤伴有高氨血症者可考虑腹膜透析。生物素为丙酰辅酶A羧化酶辅酶，治疗多种羧化酶缺乏症有效，对生物素10mg/d，反应敏感，作用迅速而持久。L-肉碱口服(100m/kg)有一定临床效果。肠道细菌产生一定量的丙酸，口服抗生素有可能降低血清和组织中丙酸浓度。\u003C/p>\u003Cp>　　(二)预后\u003C/p>\u003Cp>　　已发生脑萎缩、神经系统损害者预后差。\u003C/p>",3,[36],"营养科",true]