ICD-11 编码查询_国际疾病分类第 11 版中文版_在线疾病编码检索工具
5C50.E0

经典有机酸尿症

Classical organic aciduria

类目第 05 章 内分泌、营养或代谢疾病
上级条目
5C50.E 有机酸尿症
层级
6 级

定义

这是一个用于分类一组代谢紊乱的术语，它破坏了正常的氨基酸代谢，特别是支链氨基酸代谢，导致通常不存在的氨基酸的堆积。

This a term used to classify a group of metabolic disorders which disrupt normal amino acid metabolism, particularly branched-chain amino acids, causing a buildup of acids which are usually not present.

跨章引用 同一概念在其他章节另有编码

英文词条（1）

索引词 可用于检索该条目的同义词与别名

  • 经典有机酸尿症
  • 甲基丙二酸尿症-同型半胱氨酸尿症
  • 甲基丙二酸血症-同型半胱氨酸尿症
  • 甲基丙二酸尿症-同型半胱氨酸尿症cbl C型
  • 甲基丙二酸血症-同型半胱氨酸尿症cbl C型
  • 甲基丙二酸尿症-同型半胱氨酸尿症cbl D型
  • 甲基丙二酸血症-同型半胱氨酸尿症cbl D型
  • 甲基丙二酸尿症-同型半胱氨酸尿症cbl F型
  • 甲基丙二酸血症-同型半胱氨酸尿症cbl F型
  • 甲基丙二酸尿症
  • 甲基丙二酸血症
  • 甲基丙二酸尿症，维生素B12非反应型
  • 甲基丙二酸单酰-CoA变位酶完全缺失症
  • 突变酶0甲基丙二酸尿症
  • 突变酶0甲基丙二酸血症
  • 甲基丙二酸单酰-CoA变位酶部分缺失症
  • 突变酶甲基丙二酸尿症
  • 突变酶甲基丙二酸血症
  • 甲基丙二酰-CoA差向异构酶缺乏症
  • 甲基丙二酰-CoA消旋酶缺乏症
  • 甲基丙二酰-CoA差向异构酶缺乏症伴墨蝶呤还原酶缺乏症
  • 甲基丙二酰-CoA消旋酶缺乏症伴墨蝶呤还原酶缺乏症
  • 甲基丙二酸尿症，维生素B12反应型
  • 腺苷酰钴胺素缺乏症
  • 维生素B12反应型甲基丙二酸尿症cbl A型
  • 维生素B12反应型甲基丙二酸尿症cbl B型
  • 维生素B12反应型甲基丙二酸尿症cbl mut-型
  • 丙酸尿症
  • 酮症酸血症
  • 高甘氨酸血症伴酮症和白细胞减少症
  • 丙酸血症
  • 丙酰-CoA羧化酶缺乏症
  • 酮症甘氨酸血症
  • 异戊酸尿症
  • 异戊酸血症
  • 异戊酸CoA脱氢酶缺乏症
  • 多羧化酶缺乏症
  • 生物素酶缺乏引起的多羧化酶缺乏症
  • 羧化酶合成酶全酶缺乏引起的多羧化酶缺乏症
  • 羧化全酶合成酶缺乏引起的多羧化酶缺乏症
  • 3-羟异丁酰基-CoA水解酶缺乏引起的神经变性
  • β-羟异丁酰基-CoA脱酰酶缺乏症
  • 甲基丙烯酸尿症
  • 3-羟基异丁酰辅酶A水解酶缺陷症
  • 3-羟异丁酰基-CoA水解酶缺乏引起的神经退行性变
  • 3-羟基异丁酸尿症
  • 3-羟基异丁酸脱氢酶缺乏症
  • 3-羟基3-甲基戊二酰酸尿症
  • 3-甲基巴豆酰甘氨酸尿症
  • 3-甲基巴豆酰辅酶A羧化酶缺乏症
  • MCC - [甲基巴豆酰辅酶A羧化酶] 缺乏
  • MCCD - [甲基巴豆酰辅酶A羧化酶缺乏症]
  • 甲基巴豆酰辅酶A羧化酶缺乏症
  • 3-甲基戊烯二酸尿症
  • MGA[3-甲基戊烯二酸尿症]
  • 3-甲基戊烯二酸尿症1型
  • MGA1[3-甲基戊烯二酸尿症1型]
  • 3-甲基戊烯二酰-CoA水合酶缺乏症
  • 3MG--CoA水合酶缺乏症
  • Barth综合征
  • MGA2[3-甲基戊烯二酸尿症2型]
  • 心脏骨骼肌-中性粒细胞减少
  • 3-甲基戊烯二酸尿症2型
  • Tafazzin基因缺陷
  • 3-甲基戊烯二酸尿症3型
  • MGA3[3-甲基戊烯二酸尿症3型]
  • 婴儿视神经萎缩伴舞蹈症和痉挛性截瘫
  • 常染色体隐性遗传视神经萎缩3型
  • Costeff视神经萎缩综合征
  • 视神经萎缩附加综合征
  • Costeff综合征
  • 3-甲基戊烯二酸尿症4型
  • MGA4[3-甲基戊烯二酸尿症4型]
  • 3-甲基戊烯二酸尿症5型
  • 3-甲基戊烯二酸尿症伴正常3-甲基谷氨酰-Co A水合酶
  • DCMA[扩张型心肌病伴共济失调]综合征
  • 扩张型心肌病伴共济失调
  • 2-甲基丁酸尿症
  • SBCAD[短/支链酰基-CoA脱氢酶]缺乏症
  • 短/支链酰基-CoA脱氢酶缺乏症
  • 2-甲基丁酰甘氨酸尿症
  • 2-甲基丁酰-CoA脱氢酶缺乏引起的发育迟缓
  • 异丁酸尿症
  • 异丁酸血症
  • 异丁酰-CoA脱氢酶缺乏症
  • 甲基丙二酸半醛脱氢酶缺乏症
  • 戊二酸尿症3型
  • 戊二酸血症3型
  • 戊二酰-CoA氧化酶缺乏症
英文词条（86）
  • Classical organic aciduria
  • Methylmalonic aciduria - homocystinuria
  • Methylmalonic acidaemia - homocystinuria
  • Methylmalonic aciduria - homocystinuria type cbl C
  • Methylmalonic acidaemia - homocystinuria type cbl C
  • Methylmalonic aciduria - homocystinuria type cbl D
  • Methylmalonic acidaemia - homocystinuria type cbl D
  • Methylmalonic aciduria - homocystinuria type cbl F
  • Methylmalonic acidaemia - homocystinuria type cbl F
  • Methylmalonic aciduria
  • Methylmalonic acidaemia
  • Methylmalonic aciduria, vitamin B12 unresponsive
  • Complete deficiency of methylmalonyl-CoA mutase
  • Mutase0 methylmalonic acidaemia
  • Mutase0 methylmalonic aciduria
  • Partial deficiency of methylmalonyl-CoA mutase
  • Mutase methylmalonic acidaemia
  • Mutase methylmalonic aciduria
  • Methylmalonyl-CoA epimerase deficiency
  • Methylmalonyl-CoA racemase deficiency
  • Methylmalonyl-CoA epimerase deficiency with sepiapterin reductase deficiency
  • Methylmalonyl-CoA racemase deficiency with sepiapterin reductase deficiency
  • Methylmalonic aciduria, vitamin B12 responsive
  • Adenosylcobalamin deficiency
  • Vitamin B12 responsive methylmalonic aciduria type cbl A
  • Vitamin B12 responsive methylmalonic aciduria type cbl B
  • Vitamin B12 responsive methylmalonic aciduria type cbl mut-
  • Propionic aciduria
  • Hyperglycinaemia with ketosis and leucopaenia
  • Ketotic glycinaemia
  • Ketotic hyperglycinaemia
  • Propionic acidaemia
  • Propionyl-CoA carboxylase deficiency
  • Isovaleric aciduria
  • Isovaleric acid CoA dehydrogenase deficiency
  • Isovaleric acidaemia
  • Multiple carboxylase deficiency
  • Multiple carboxylase deficiency due to biotinidase deficiency
  • Multiple carboxylase deficiency due to holocarboxylase synthetase deficiency
  • Neurodegeneration due to 3-hydroxyisobutyryl-CoA hydrolase deficiency
  • Beta-hydroxyisobutyryl-CoA deacylase deficiency
  • Methacrylic aciduria
  • 3-hydroxyisobutyric aciduria
  • 3-hydroxyisobutyrate dehydrogenase deficiency
  • 3-hydroxy-3-methylglutaric aciduria
  • 3-methylcrotonylglycinuria
  • 3-methylcrotonyl-CoA carboxylase deficiency
  • MCC - [methylcrotonyl-CoA carboxylase] deficiency
  • MCCD - [methylcrotonyl-CoA carboxylase deficiency]
  • methylcrotonyl-CoA carboxylase deficiency
  • 3-methylglutaconic aciduria
  • MGA - [3-methylglutaconic aciduria]
  • 3-methylglutaconic aciduria type 1
  • 3-methylglutaconyl-CoA hydratase deficiency
  • 3MG-CoA hydratase deficiency
  • MGA1- [3-methylglutaconic aciduria type 1]
  • Barth syndrome
  • 3-methylglutaconic aciduria type 2
  • Cardioskeletal myopathy-neutropaenia
  • MGA2 - [3-methylglutaconic aciduria type 2]
  • Tafazzin gene defect
  • 3-methylglutaconic aciduria type 3
  • Autosomal recessive optic atrophy type 3
  • Costeff optic atrophy syndrome
  • Costeff syndrome
  • Infantile optic atrophy with chorea and spastic paraplegia
  • MGA3 - [3-methylglutaconic aciduria type 3]
  • Optic atrophy plus syndrome
  • 3-methylglutaconic aciduria type 4
  • MGA4 - [3-methylglutaconic aciduria type 4]
  • 3-methylglutaconic aciduria type 5
  • 3-methylglutaconic aciduria with normal3 -methylglutaconyl-Co A hydratase
  • DCMA - [Dilated cardiomyopathy with ataxia] syndrome
  • Dilated cardiomyopathy with ataxia
  • 2-methylbutyric aciduria
  • 2-methylbutyryl glycinuria
  • Developmental delay due to 2-methylbutyryl-CoA dehydrogenase deficiency
  • SBCAD - [Short/branched-chain acyl-coA dehydrogenase] deficiency
  • Short/branched-chain acyl-coA dehydrogenase deficiency
  • Isobutyric aciduria
  • Isobutyric acidaemia
  • Isobutyryl-CoA dehydrogenase deficiency
  • Methylmalonate semialdehyde dehydrogenase deficiency
  • Glutaric aciduria type 3
  • Glutaric acidaemia type 3
  • Glutaryl-CoA oxidase deficiency

后组配轴 Postcoordination

该条目未定义后组配轴，直接使用上面的编码即可。后组配是在主干编码之后追加侧别、具体解剖部位、严重程度、病因等细节的可选组合方式，不是必填项。