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
- Weight210 lbs
- Race (AA/O)O
- Serum creatinine (mg/dL)0.85
- eGFR (mL/min/1.73m2)88.8
- Ultrasound kidney length (cm)17.2
- 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.
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.5ADPKD=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.
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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.
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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.