Denise

Denise is 35 years old and has been diagnosed with ADPKD by a kidney ultrasound. Denise is at risk for rapid disease progression, as indicated by the following risk factors: US-KL >16.5 cm in patients less than 45 years old*, hypertension before age 35, urologic events before age 35, overweight/obesity and family history of ESKD by age 58. Let's take a closer look with some prognostic tools to confirm the risk of rapid disease progression and assess how rapidly her disease may progress.1,2,5

  • Age35
  • Height5'4"
  • Weight210 lbs
  • SexF
  • Race (AA/O)O

Baseline Assessment3-8

  • Serum creatinine (mg/dL)0.85
  • eGFR (mL/min/1.73m2)88.8
    1
  • Ultrasound kidney length (cm)17.2
    3
  • Hypertension before 35?Yes
  • Urological event before 35?Yes
  • Family members with ESKD?Yes (58 yrs)
  • MutationsNot available
  • PROPKD ScoreNot available
  • htTKV (mL/m)Perform MRI/CT
  • Other risk factorsObesity
  • ADPKD Imaging Classification

    Denise does not need an MRI or CT scan to confirm her risk for rapid progression, but it would provide more information.

    4

Disease Progression2,3,6-11

Kidney Size (height adjusted Total Kidney Volume)
Normal htTKV range: 150-250Years202530354045505560mL/m02004006008001000
2
Kidney function (eGFR): 88.8 mL/min/1.73m2
CKD 1CKD 2CKD 3CKD 4CKD 5/ESRDYears202530354045505560mL/min/1.73m²0102030405060708090100110
20
25
30
35
40
45
50
55
60

Years

Click on the flags below to walk through an assessment that confirms risk of rapid disease progression and assesses how rapidly Denise’s disease may progress

* When rapid progression is defined as CKD stage 3 development within 8 years.5

ADPKD=autosomal dominant polycystic kidney disease; US-KL=ultrasound kidney length; ESKD=end stage kidney disease; AA=African American; O=other; eGFR=estimated glomerular filtration rate; PKD=polycystic kidney disease; htTKV=height-adjusted total kidney volume; 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. 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.

  5. Bhutani H, et al. A comparison of ultrasound and magnetic resonance imaging shows that kidney length predicts chronic kidney disease in autosomal dominant polycystic kidney disease. Kidney Int. 2015;88:146-151.

  6. 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.

  7. 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.

  8. 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.

  9. 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.

  10. 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.

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

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