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Glomerular hyperfiltration and renal progression in children with autosomal dominant polycystic kidney disease

Imed Helal1, Berenice Reed, Kim McFann

  • 1University of Colorado Denver, Division of Renal Diseases and Hypertension, Aurora, CO 80045, USA.

Clinical Journal of the American Society of Nephrology : CJASN
|September 10, 2011
PubMed

Insights

Glomerular hyperfiltration (GH) in children with autosomal dominant polycystic kidney disease (ADPKD) indicates faster disease progression. This early sign predicts accelerated kidney enlargement and declining function in pediatric ADPKD patients.

Area of Science:

  • Pediatric Nephrology
  • Genetics
  • Renal Physiology

Background:

  • Autosomal dominant polycystic kidney disease (ADPKD) is a common genetic disorder.
  • Early indicators of disease progression in pediatric ADPKD are crucial for management.
  • Glomerular hyperfiltration (GH) is a potential early marker in ADPKD.

Purpose of the Study:

  • To investigate if early glomerular hyperfiltration (GH) in children with ADPKD predicts more rapid disease progression.
  • To establish GH as a potential early diagnostic marker for severe ADPKD in pediatric populations.

Main Methods:

  • 180 children (ages 4-18) with ADPKD and normal renal function were studied.
  • Renal ultrasound assessed total kidney volume; creatinine clearance measured renal function.
  • GH defined as creatinine clearance ≥140 ml/min per 1.73 m(2).

Main Results:

  • Children with GH showed significantly increased total renal volume growth over 5 years (19.3 cm(3)/year) compared to those without GH (-4.3 cm(3)/year).
  • Patients with GH experienced a faster decline in creatinine clearance (-5.0 ml/min/year) versus those without GH (1.0 ml/min/year).
  • These differences were statistically significant (P=0.008 for volume, P<0.0001 for clearance).

Conclusions:

  • Early glomerular hyperfiltration in pediatric ADPKD is linked to accelerated kidney enlargement.
  • GH in ADPKD children is associated with a more rapid decline in renal function over time.
  • GH and increased renal volume may serve as early indicators of severe ADPKD progression in children.
Abstract

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