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
    1
  • Ultrasound kidney length (cm)Not obtained
  • Hypertension before 35?Yes
  • Urological event before 35?Yes
  • Family members with ESKD?No
  • MutationsTruncating PKD1
    3
  • PROPKD Score9 - HIGH risk of progression to ESKD
    4
  • htTKV (mL/m)1,343
    5
  • 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 SCANSCoronal ViewTransverse View
    6

Disease Progression2-5,7-10

Kidney Size (height adjusted Total Kidney Volume) : 1343.0 mL/m
Normal htTKV range: 150-250Years253545mL/m0500100015002000250030003500
2
Kidney function (eGFR): 82.0 mL/min/1.73m2
CKD 1CKD 2CKD 3CKD 4CKD 5/ESRDYears253545mL/min/1.73m²0102030405060708090100
25
35
45

Years

7

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

ADPKD=autosomal dominant polycystic kidney disease; PKD=polycystic kidney disease; TKV=total kidney volume; AA=African American; O=other; eGFR=estimated glomerular filtration rate; ESKD=end stage kidney disease; htTKV=height-adjusted TKV; MRI=magnetic resonance imaging; CT=computed tomography; CKD=chronic kidney disease.
References:
  1. Schrier RW, et al. Predictors of autosomal dominant polycystic kidney disease progression. J Am Soc Nephrol. 2014;25:2399-2418.

  2. 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.

  3. 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.

  4. Irazabal MV, et al. Imaging classification of autosomal dominant polycystic kidney disease: a simple model for selecting patients for clinical trials. J Am Soc Nephrol. 2015;26:160-172.

  5. 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.

  6. Cornec-Le Gall E, et al. The PROPKD score: a new algorithm to predict renal survival in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 2016;27(3):942-951.

  7. 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.

  8. 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.

  9. 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.

  10. Rangan GK, et al. Autosomal dominant polycystic kidney disease: a path forward. Semin Nephrol. 2015;35(6):524-537.

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