Bill
Bill is 35 years old and has been diagnosed with ADPKD by a kidney ultrasound. Bill is at risk for rapid disease progression, as indicated by the following risk factors: being male, hypertension before age 35, urologic events before age 35, truncating PKD1 mutation, and TKV greater than expected for age. Let's take a closer look with some prognostic tools to confirm the risk of rapid disease progression and assess how rapidly his disease may progress.1,2
- Age35
- Height5'11"
- Weight171 lbs
- SexM
- Race (AA/O)AA
Baseline Assessment3-7
- Serum creatinine (mg/dL)1.15
- eGFR (mL/min/1.73m2)82
- Ultrasound kidney length (cm)Not obtained
- Hypertension before 35?Yes
- Urological event before 35?Yes
- Family members with ESKD?No
- MutationsTruncating PKD1
- PROPKD Score9 - HIGH risk of progression to ESKD
- htTKV (mL/m)1,343
- Other risk factorsNone
- ADPKD Imaging Classification
Class 1E: High Risk for eGFR decline
>6% estimated yearly percentage increase in kidney growth
ESTIMATED AGE AT ESKD47
MRI/CT SCANS
Disease Progression2-5,7-10
Click on the flags below to walk through an assessment that confirms rapid disease progression risk and assesses how rapidly Bill’s disease may progress
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Gansevoort RT, et al. Recommendations for the use of tolvaptan in autosomal dominant polycystic kidney disease: a position statement on behalf of the ERA-EDTA Working Groups on Inherited Kidney Disorders and European Renal Best Practice. Nephrol Dial Transplant. 2016;31(3):337-48.
Wetzels JFM, et al. Age- and gender-specific reference values of estimated GFR in caucasians: the Nijmegen biomedical study. Kidney Int. 2007;72:632-637.
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Imaging classification of ADPKD: a simple model for selecting patients for clinical trials. http://www.mayo.edu/research/documents/pkd-center-adpkd-classification/doc-20094754. Accessed January 09, 2019.
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Cheong B, et al. Normal values for renal length and volume as measured by magnetic resonance imaging. Clin J Am Soc Nephrol. 2007;2:38-45.
Levey AS, et al. Definition and classification of chronic kidney disease: a position statement from kidney disease: improving global outcomes (KDIGO). Kidney Int. 2005;67:2089-2100.
PKD Charity. Fast Facts about ADPKD. The Polycystic Kidney Disease Charity. 2017. https://pkdcharity.org.uk/about-adpkd/just-diagnosed/fast-facts-about-adpkd Accessed January 09, 2019.
Rangan GK, et al. Autosomal dominant polycystic kidney disease: a path forward. Semin Nephrol. 2015;35(6):524-537.