Total Kidney Volume as a Biomarker of Disease Progression in Autosomal Dominant Polycystic Kidney Disease

Abstract

Purpose of review:Autosomal dominant polycystic kidney disease (ADPKD) is an inherited disorder characterized by the formation of kidney cysts and kidney enlargement, which progresses to kidney failure by the fifth to seventh decade of life in a majority of patients. Disease progression is evaluated primarily through serum creatinine and estimated glomerular filtration rate (eGFR) measurements; however, it is known that serum creatinine and eGFR values typically do not change until the fourth or fifth decade of life. Until recently, therapy only existed to target complications of ADPKD. As therapeutic agents continue to be investigated for use in ADPKD, a suitable biomarker of disease progression in place of serum creatinine is needed.Sources of information:This review summarizes recent research regarding the use of total kidney volume as a biomarker in ADPKD, as presented at the Canadian Society of Nephrology symposium held in April 2015.Findings:Measurement of patients’ total kidney volume made using ultrasound (US) or magnetic resonance imaging (MRI) has been shown by the Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP) study to be directly correlated with both increases in cyst volume and change in glomerular filtration rate (GFR). Additional studies have shown total kidney volume to have an association with complications of ADPKD as well.Limitations:Areas for further study continue to exist in comparison of methods of measuring total kidney volume.Implications:We believe that the evidence suggests that total kidney volume may be an appropriate surrogate marker for ADPKD disease progression.

A model to predict disease progression in patients with autosomal dominant polycystic kidney disease (ADPKD): the ADPKD Outcomes Model

“…Despite this, validation exercises found that in the absence of TKV data, disease progression predicted by the ADPKD-OM remained plausible over a range of baseline values. As the prognostic value of TKV growth in ADPKD patients is increasingly recognised and advocated [ 8 , 38 ], the adoption of this measure in routine clinical practice will only enhance predictions of disease progression generated by the ADPKD-OM.…”

Thiol/Disulphide Homeostasis in Autosomal Dominant Polycystic Kidney Disease: A Single Center, Cross-Sectional Study

“…Autosomal dominant polycystic kidney (ADPK) disease is one of the most common hereditary disorders and is characterized by progressive cyst formation and the enlargement of the kidney and other organs (e.g., liver, pancreas, and spleen) (1). Ultimately, gradually growing cysts lead to kidney failure by the fifth of sixth decade of life (2).…”

ADPKD—what the radiologist should know

“…32 Of interest, these methods show more accuracy on CT in comparison to MRI due to slice thickness and utilisation of the Cavalieri method; however, this would not be feasible due to the chronic nature of ADPKD and serial imaging requirements over decades and the radiation hazards carried by CT. MRI is therefore the most accurate method practically applicable to imaging requests, with the time requirement being the biggest factor to impact on MRI reporting adding on average at least 30 additional minutes to produce accurate reproducibility. 33 We must evaluate the additional value this carries in enhancing the radiological report, considering volume estimation is useful in such patients particularly around decision making on Tolvaptan commencement. 34 A request from the renal physician specifying volume measurement after patient selection would lend huge help in guiding radiologists to prioritise the imaging requiring additional reporting time, with a pathway similar to vasculature evaluation for potential kidney donorsthese can then be assigned subspecialists within Radiology to take on responsibility for TKV estimation reporting in addition to producing a standard MRI report.…”