多囊肾病
肾
医学
疾病
常染色体显性多囊肾病
病理
计算机科学
生物信息学
内科学
生物
出处
期刊:
日期:2016-02-11
卷期号:352: i679-i679
被引量:38
摘要
#### What you need to know
Polycystic kidney disease (PKD) is an inherited condition defined by the pathological development of fluid-filled cysts throughout the kidneys leading to organ enlargement (fig 1⇓) and chronic kidney disease. Autosomal dominant polycystic kidney disease (ADPKD) is the commonest inherited kidney disease1 and is the fourth commonest cause of kidney failure worldwide.2 Autosomal recessive PKD is a rare disease usually identified antenatally or during the neonatal period by enlarged echogenic kidneys on ultrasound.3 This review will focus on ADPKD.
Fig 1 Anatomical illustration of a polycystic kidney (right) compared with a normal kidney (left)
#### Sources and selection criteria
I searched PubMed, Clinical Evidence, and the Cochrane Library for primary and review articles available in English until October 2015. Articles included systematic reviews, randomised clinical trials, and observational studies. The following search terms were used in isolation and combination: “ADPKD,” “polycystic kidney disease,” “progression,” “treatment,” “humans,” “complications,” “mutations.” I identified additional references from my personal archive. Where evidence based medicine was not available, published expert opinion was referenced.
ADPKD presents to a variety of doctors and is more prevalent than the combination …
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