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Growth in Children with Autosomal Recessive Polycystic Kidney Disease in the CKiD Cohort Study

Erum A Hartung1, Katherine M Dell2, Matthew Matheson3

  • 1Division of Nephrology, Department of Pediatrics, Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania , Philadelphia, PA , USA.

Frontiers in Pediatrics
|August 26, 2016
PubMed

Insights

Children with autosomal recessive polycystic kidney disease (ARPKD) experience growth issues, but these are comparable to other congenital kidney diseases. ARPKD does not appear to uniquely impact growth in children with mild-to-moderate chronic kidney disease (CKD).

Area of Science:

  • Pediatric Nephrology
  • Growth and Development
  • Genetics

Background:

  • Autosomal recessive polycystic kidney disease (ARPKD) may cause growth impairment disproportionate to chronic kidney disease (CKD).
  • Previous studies suggest potential growth issues in children with ARPKD.
  • A systematic comparison is needed to understand ARPKD's specific impact on growth.

Purpose of the Study:

  • To systematically compare growth parameters in children with ARPKD against those with other congenital CKD causes.
  • To determine if ARPKD has a disease-specific effect on growth in children.
  • To analyze height z-scores, severe short stature prevalence, and growth hormone use.

Main Methods:

  • Analysis of height SD scores (z-scores) and severe short stature (< -1.88).
  • Comparison of ARPKD patients (n=22) with matched controls (aplastic/hypoplastic/dysplastic kidneys, n=44; obstructive uropathy, n=44).
  • Statistical analysis using Wilcoxon rank-sum, Fisher's exact, and Wilcoxon signed-rank tests.

Main Results:

  • Median height z-score in ARPKD was -1.1; 14% had severe short stature, and 18% used growth hormone.
  • No significant differences were found in height z-score, severe short stature, or growth hormone use between ARPKD and control groups.
  • Annual change in height z-score also showed no significant differences between groups.

Conclusions:

  • Children with ARPKD and mild-to-moderate CKD exhibit a high prevalence of growth abnormalities.
  • These growth abnormalities are similar to those seen in children with other congenital CKD causes.
  • The study does not support a disease-specific growth impact of ARPKD in this pediatric cohort.
Abstract

Keywords:
autosomal recessive polycystic kidney diseasechildrengrowthgrowth hormone

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