The importance of total kidney volume in evaluating progression of polycystic kidney disease.
The rate at which autosomal dominant polycystic kidney disease (ADPKD) progresses to end-stage renal disease varies widely and is determined by genetic and non-genetic factors. The ability to determine the prognosis of children and young adults with ADPKD is important for the effective life-long management of the disease and to enable the efficacy of emerging therapies to be determined. Total kidney volume (TKV) reflects the sum volume of hundreds of individual cysts with potentially devastating effects on renal function. The sequential measurement of TKV has been advanced as a dynamic biomarker of disease progression, yet doubt remains among nephrologists and regulatory agencies as to its usefulness. Here, we review the mechanisms that lead to an increase in TKV in ADPKD, and examine the evidence supporting the conclusion that TKV provides a metric of disease progression that can be used to assess the efficacy of potential therapeutic regimens in children and adults with ADPKD. Moreover, we propose that TKV can be used to monitor treatment efficacy in patients with normal levels of renal function, before the pathologic processes of ADPKD cause extensive fibrosis and irreversible loss of functioning renal tissue.
The Kidney Institute, Division of Nephrology and Hypertension, University of Kansas Medical Center, 3901 Rainbow Boulevard, Kansas City, Kansas 66160, USA.
Jared J
JJ
Grantham
Division of Nephrology and Hypertension, Mayo Clinic, 200 First Street SW, Minnesota 55905, USA.
Vicente E
VE
Torres
D018450:Disease Progression; D006801:Humans; D007668:Kidney / Q000473:pathology*; D009929:Organ Size; D007690:Polycystic Kidney Diseases / Q000188:drug therapy / Q000209:etiology / Q000473:pathology*; D016891:Polycystic Kidney, Autosomal Dominant / Q000473:pathology; D011379:Prognosis; D016896:Treatment Outcome
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