GB82
常染色体显性遗传肾小管间质肾病
Autosomal dominant tubulointerstitial disease
- 上级条目
- 囊性或发育不良性肾病
- 层级
- 4 级
定义
非肾小球性、常染色体显性遗传性肾病，其特点是进行性肾小管间质纤维化和进展至终末期肾病。目前有 4 种已知的遗传缺陷--在oromudulin、mucin-1、肾素和肝细胞核因子1-ß。最后一种与青少年发病的成人型糖尿病（MODY）有关，因此被归类为MODY-5。
Nonglomerular, autosomal dominant kidney diseases characterised by progressive tubulointerstitial fibrosis and progression to end-stage renal disease. Currently there are 4 known genetic defects - in uromodulin, mucin-1, renin and hepatocyte nuclear factor 1-beta. The last is associated with Maturity-Onset Diabetes of the Young (MODY) and thus is classified as MODY-5
跨章引用 同一概念在其他章节另有编码
索引词 可用于检索该条目的同义词与别名
- 常染色体显性遗传肾小管间质肾病
- ADTKD[常染色体显性遗传肾小管间质病]NOS
- 常染色体显性遗传肾小管间质肾病NOS
- 髓质囊性肾病
- MCKD [髓质囊性肾病]
- 髓质囊性疾病 [possible translation]
- 髓质囊性肾 [possible translation]
- 髓质囊性肾
- 髓质囊性疾病
- 常染色体显性遗传小管间质性肾病，1型
- 常染色体隐性遗传髓质囊性病
- 肾消耗病
- MCKD[髓质囊性肾病1型]
- 髓质囊性肾病，1型
- Mucin-1肾病
- ADTKD-MUC1[常染色体显性遗传肾小管间质病]
- 常染色体显性肾小管间质病，1型
- 常染色体显性遗传肾小管间质病2型
- 髓质囊性肾病，2型- [MCKD]
- 髓质囊性肾病，2型
- ATDKD-UMOD[常染色体显性遗传肾小管间质病-尿调蛋白]
- 尿调蛋白相关性肾病 [possible translation]
- 尿调蛋白肾病 [possible translation]
- 尿调蛋白相关性肾病
- 尿调蛋白肾病
英文词条（20）
- Autosomal dominant tubulointerstitial disease
- ADTKD - [autosomal dominant tubulointerstitial disease] NOS
- MCKD [medullary cystic kidney disease]
- autosomal dominant tubulointerstitial kidney disease NOS
- medullary cystic disease
- medullary cystic kidney
- medullary cystic kidney disease
- Autosomal dominant tubulointerstitial disease, Type 1
- ADTKD-MUC1 [autosomal dominant tubulointerstitial kidney disease MUC1]
- MCKD - [medullary cystic kidney disease] type 1
- Mucin-1 Kidney disease
- autosomal recessive medullary cystic disease
- medullary cystic kidney disease, Type 1
- nephronopthisis
- Autosomal dominant tubulointerstitial disease Type 2
- ATDKD-UMOD - [Autosomal dominant tubulointerstitial disease-uromodulin]
- MCKD - [medullary cystic kidney disease] type 2
- Uromodulin associated kidney disease
- Uromodulin kidney disease
- medullary cystic kidney disease, Type 2
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。