Around 50% of people with APKD will be in end-stage kidney disease and require dialysis or transplantation by the age of 60 in PKD1 and the age of 75 in PKD2. There is great variability in the age of onset of renal failure (1) .
predictors of more rapid progression to renal failure include the following: (1)
earlier age at diagnosis
male sex
sickle cell trait
PKD1 genotype
larger or rapidly increasing kidney size
gross haematuria
hypertension
being black
increasing proteinuria
the most lethal extrarenal manifestations of ADPKD are intracranial aneurysms, which have been found to be present in up to 40% of ADPKD patients (2). Patients with mutations in the 5' region of PKD1 are more likely to have aneurysm rupture. (3)
Reference:
Ong AC, Devuyst O, Knebelmann B, et al. Autosomal dominant polycystic kidney disease: the changing face of clinical management. Lancet. 2015 May 16;385(9981):1993-2002.
Bergmann C, Guay-Woodford LM, Harris PC, et al. Polycystic kidney disease. Nat Rev Dis Primers. 2018 Dec 6;4(1):50.
Rossetti S, Harris PC. The genetics of vascular complications in autosomal dominant polycystic kidney disease (ADPKD). Curr Hypertens Rev. 2013 Feb;9(1):37-43.
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