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Volume progression in polycystic kidney disease

Jared J Grantham1, Vicente E Torres, Arlene B Chapman

  • 1Kidney Institute and the Department of Internal Medicine, Kansas University Medical Center, Kansas City, KS 66160, USA. jgrantha@kumc.edu

The New England Journal of Medicine
|May 19, 2006
PubMed

Insights

Autosomal dominant polycystic kidney disease (ADPKD) causes progressive kidney enlargement. This study shows kidney and cyst volume increase is continuous and linked to declining kidney function, especially with larger initial kidney size.

Area of Science:

  • Nephrology
  • Medical Imaging
  • Genetics

Background:

  • Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder characterized by the progressive development of kidney cysts.
  • These cysts lead to significant kidney enlargement, often resulting in impaired renal function.

Purpose of the Study:

  • To quantify the rates of change in total kidney volume (TKV) and total cyst volume (TCV) in ADPKD patients over three years.
  • To investigate the correlation between kidney volume changes and renal function decline (iothalamate clearance).
  • To identify factors influencing the rate of kidney enlargement in ADPKD.

Main Methods:

  • A three-year longitudinal study involving 241 ADPKD patients (232 without azotemia, aged 15-46 at baseline).
  • Magnetic-resonance imaging (MRI) was used to measure TKV and TCV.
  • Iothalamate clearance was measured to assess renal function. Statistical analyses included ANOVA, Pearson correlation, and multivariate regression.

Main Results:

  • TKV and TCV increased exponentially, indicating continuous growth. Mean TKV increased by 5.27% per year (P<0.001).
  • A baseline TKV >1500 ml was associated with a significant decline in glomerular filtration rate (4.33 ml/min/year, P<0.001).
  • Patients with PKD1 mutations showed greater TKV increase compared to those with PKD2 mutations (P=0.03).

Conclusions:

  • Kidney enlargement in ADPKD is a continuous, quantifiable process driven by cyst expansion.
  • The rate of kidney enlargement is directly associated with the rate of renal function decline.
  • Baseline TKV is a predictor of future kidney volume increase and functional decline.
Abstract

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