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Vascular complications in autosomal dominant polycystic kidney disease
#MMPMID26260542
Perrone RD
; Malek AM
; Watnick T
Nat Rev Nephrol
2015[Oct]; 11
(10
): 589-98
PMID26260542
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Autosomal dominant polycystic kidney disease (ADPKD) is the most common
hereditary kidney disease. Relentless cyst growth substantially enlarges both
kidneys and culminates in renal failure. Patients with ADPKD also have vascular
abnormalities; intracranial aneurysms (IAs) are found in ?10% of asymptomatic
patients during screening and in up to 25% of those with a family history of IA
or subarachnoid haemorrhage. As the genes responsible for ADPKD?PKD1 and
PKD2?have complex integrative roles in mechanotransduction and intracellular
calcium signalling, the molecular basis of IA formation might involve focal
haemodynamic conditions exacerbated by hypertension and altered flow sensing. IA
rupture results in substantial mortality, morbidity and poor long-term outcomes.
In this Review, we focus mainly on strategies for screening, diagnosis and
treatment of IAs in patients with ADPKD. Other vascular aneurysms and
anomalies?including aneurysms of the aorta and coronary arteries, cervicocephalic
and thoracic aortic dissections, aortic root dilatation and cerebral
dolichoectasia?are less common in this population, and the available data are
insufficient to recommend screening strategies. Treatment decisions should be
made with expert consultation and be based on a risk-benefit analysis that takes
into account aneurysm location and morphology as well as patient age and
comorbidities.
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