Polycystic Kidney Disease 1 with or Without Polycystic Liver Disease (PKD1)

多囊肾1型伴有或不伴有多囊肝(来自ICD-11)

别称:

Polycystic Kidney Disease 1

Polycystic Kidney Disease, Adult Type

Adpkd

Pkd1

Potter Type Iii Polycystic Kidney Disease

Polycystic Kidney Disease, Adult, Type I

Apkd1

Polycystic Kidney, Type 1 Autosomal Dominant Disease

Potter Type Iii Polycystic Kidney Disease, Formerly

Polycystic Kidney Type 1 Autosomal Dominant Disease

Autosomal Dominant Polycystic Kidney Disease 1

Polycystic Kidney Disease, Adult Type I

Adult Polycystic Kidney Disease Type 1

Polycystic Kidney, Autosomal Dominant

Kidney Disease, Polycystic, Type 1

Polycystic Kidney Disease, Type 1

Polycystic Kidney Disease, Adult

Polycystic Kidney Disease Type I

Polycystic Kidney Disease Adult

Polycystic Kidney Diseases

Polycystic Kidneys

Adpkd1

Pkd-1

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基础信息

疾病表征

基因 & 突变

靶点药物

疾病模型

文献报道

多囊肾病1型，伴有或不伴有多囊肝，也称为多囊肾病1型，与多囊肾病2型，伴有或不伴有多囊肝和婴儿严重多囊肾病，伴有结节性硬化症有关。与多囊肾病1型，伴有或不伴有多囊肝相关的基因是PKD1（多囊蛋白1，瞬时受体电位通道相互作用），其相关通路/超级通路包括p70S6K信号通路和Angiopoietin-like protein 8调节通路。在该疾病的背景下，已提到的药物包括血管紧张素II和坎地沙坦酯。相关组织包括肾脏和肝脏，相关表型包括二尖瓣反流和二尖瓣脱垂。

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