Research progress in autosomal dominant polycystic kidney disease.
10.11817/j.issn.1672-7347.2019.180110
- Author:
Sheng HU
1
;
Dongjie LI
2
,
3
;
Xinji TAN
1
;
Jie GU
1
;
Mingquan CHEN
1
;
Xiaobo ZHANG
2
,
3
Author Information
1. International Medical Center,
Xiangya Hospital, Central South University, Changsha 410008, China.
2. International Medical Center
3. National Clinical Research Center for Geriatric Disorders,
Xiangya Hospital, Central South University, Changsha 410008, China.
- Publication Type:Journal Article
- MeSH:
Disease Progression;
Glomerular Filtration Rate;
Humans;
Kidney;
Polycystic Kidney, Autosomal Dominant;
Tolvaptan
- From:
Journal of Central South University(Medical Sciences)
2019;44(10):1179-1187
- CountryChina
- Language:Chinese
-
Abstract:
Autosomal dominant polycystic kidney disease (ADPKD) is a common hereditary disease, mainly caused by polycystic kidney disease 1/2 (PKD1/2) gene mutation. The main manifestation is the formation of multiple progressive enlarged cysts in both kidneys, which can be accompanied by decreased glomerular filtration rate, hypertension, liver cyst and cerebral aneurysm. About 45% of patients will progress to end-stage renal failure before the age of 60. ADPKD gene sequencing can be chosen for suspicious patients with atypical clinical features, no positive family history, and inconspicuous imaging findings. In the ADPKD positive families, imaging examination is the main means of diagnosing ADPKD. Height-adjusted total kidney volume (htTKV) and kidney growth rate are commonly used to monitor ADPKD disease progression and prognosis. There is no effective treatment for ADPKD to stop its progress. Drugs such as tolvaptan and bosutinib can delay the renal disfunction and they have been applied to clinical therapy in Europe and America.
