GB81
常染色体显性遗传多囊肾
Autosomal dominant polycystic kidney disease
- 上级条目
- 囊性或发育不良性肾病
- 层级
- 4 级
定义
从青春期开始，双肾多发性囊肿的数量和大小都在增加，与高血压和慢性肾功能衰竭的发展有关。常染色体显性家族模式常见，是由16号和4号染色体突变所致。非肾性表现包括肝脏囊肿和较少见的胰腺囊肿。也可发生伴有蛛网膜下腔出血的脑动脉瘤和其他非肾血管异常。
Multiple cysts in both kidneys increasing in number and size from adolescence, associated with development of hypertension and chronic renal failure. Autosomal dominant familial pattern is usual and due to mutations on chromosomes 16 and 4. Non-renal manifestations can include cysts in the liver and less commonly pancreas. Cerebral arterial aneurysms with subarachnoid haemorrhage, and other non-renal vascular abnormalities can also occur.
索引词 可用于检索该条目的同义词与别名
- 常染色体显性遗传多囊肾
- 成人型多囊肾
- 成人型多囊肾
- APCKD[常染色体多囊肾]
- 常染色体显性遗传多囊肾病1型，不伴结节性硬化症
- ADPCKD1[常染色体显性遗传多囊肾病1型]
- 常染色体多囊肾病1型[APCKD1]
- 常染色体显性遗传多囊肾病2型
- ADPCKD2[常染色体显性遗传多囊肾病2型]
- APCKD2[常染色体多囊肾病]
- PKD2基因[多囊肾病2型基因]
英文词条（11）
- Autosomal dominant polycystic kidney disease
- APCKD - [autosomal polycystic kidney disease]
- Polycystic kidney, adult type
- adult polycystic kidney disease
- Autosomal dominant polycystic kidney disease type 1 without tuberous sclerosis
- ADPCKD1 - [autosomal dominant polycystic kidney disease type 1]
- APCKD1 - [autosomal polycystic kidney disease type 1]
- Autosomal dominant polycystic kidney disease, Type 2
- ADPCKD2 - [autosomal dominant polycystic kidney disease type 2]
- APCKD2 - [autosomal polycystic kidney disease type 2]
- PKD2 gene - [polycystic kidney disease type 2 gene]
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。