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Autosomal dominant polycystic kidney disease
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==Prognosis== In ADPKD patients, gradual cyst development and expansion result in kidney enlargement, and during the course of the disease, [[glomerular filtration rate]] remains normal for decades before [[kidney function]] starts to progressively deteriorate, making early prediction of renal outcome difficult.<ref name="TP-150519-E38">{{cite journal | vauthors = Cornec-Le Gall E, Le Meur Y | title = Polycystic kidney disease: Kidney volume--a crystal ball for ADPKD prognosis? | journal = Nature Reviews. Nephrology | volume = 10 | issue = 9 | pages = 485β486 | date = September 2014 | pmid = 25092148 | doi = 10.1038/nrneph.2014.132 | s2cid = 22042874 }}</ref> The CRISP study,<ref name="TP-150515-017" /><ref name="TP-150519-E39"/> mentioned in the '''treatment''' section above, contributed to build a strong rationale supporting the prognostic value of total kidney volume (TKV) in ADPKD; TKV (evaluated by [[MRI]]) increases steadily and a higher rate of kidney enlargement correlated with accelerated decline of GFR, while patient height-adjusted TKV (HtTKV) β₯600 ml/m predicts the development of stage 3 chronic kidney disease within 8 years.<ref name="TP-150519-E38" /> Besides TKV and HtTKV, the [[glomerular filtration rate|estimated glomerular filtration rate]] (eGFR) has also been tentatively used to predict the progression of ADPKD.<ref name="TP-150519-E38" /> After the analysis of CT or MRI scans of 590 patients with ADPKD treated at the [[Mayo Clinic|Mayo Translational Polycystic Kidney Disease Center]], Irazabal and colleagues developed an imaging-based classification system to predict the rate of eGFR decline in patients with ADPKD.<ref name="TP-150519-E38" /><ref name="TP-150519-E39"/> In this prognostic method, patients are divided into five subclasses of estimated kidney growth rates according to age-specific HtTKV ranges (1A, <1.5%; 1B, 1.5β3.0%; 1C, 3.0β4.5%; 1D, 4.5β6.0%; and 1E, >6.0%) as delineated in the CRISP study.<ref name="TP-150519-E38" /><ref name="TP-150519-E39" /> The decline in eGFR over the years following initial TKV measurement is significantly different between all five patient subclasses, with those in subclass 1E having the most rapid decline.<ref name="TP-150519-E38" /> Some of the most common causes of death in patients with ADPKD are various infections (25%), a ruptured berry aneurysm (15%), or coronary/hypertensive heart disease (40%).<ref>{{cite book | vauthors = Kumar V, Abbas AK, Aster JC |title=Robbins and Cotran Pathologic Basis of Disease |date=2015 |location=Philadelphia, PA |isbn=978-1-4557-2613-4 |edition=Ninth |pages=947|oclc=879416939 }}</ref>
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