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Abnormalities in renal tubular phosphate handling in children with sickle cell disease

Vimal Master Sankar Raj1, Michael Freundlich, Dima Hamideh

  • 1Division of Pediatric Nephrology, University of Miami, Miami, Florida.

Pediatric Blood & Cancer
|August 19, 2014
PubMed

Insights

Children with sickle cell disease (SCD) show elevated phosphorus levels due to increased tubular reabsorption and resistance to FGF23, even with normal kidney function. This suggests early kidney changes in SCD patients.

Area of Science:

  • Nephrology
  • Hematology
  • Endocrinology

Background:

  • Hyperphosphatemia mechanisms in sickle cell disease (SCD) with preserved glomerular filtration rate (GFR) are unclear.
  • The role of fibroblast growth factor 23 (FGF23) in SCD-related hyperphosphatemia has not been studied.
  • This study investigates renal tubular phosphorus handling and FGF23 levels in pediatric SCD patients.

Purpose of the Study:

  • To evaluate renal tubular phosphorus handling parameters in children with SCD.
  • To assess the relationship between these parameters and fibroblast growth factor 23 (FGF23) levels.
  • To understand the mechanisms of hyperphosphatemia in SCD patients with normal GFR.

Main Methods:

  • Cross-sectional study of 24 children with SCD and normal GFR.
  • Measurement of renal tubular phosphate handling (TRP, TMP/GFR) and serum levels of FGF23 and parathyroid hormone (PTH).
  • Correlation and regression analyses to determine relationships between serum phosphorus and various analytes.

Main Results:

  • Elevated serum phosphorus and increased tubular reabsorption of phosphorus (TMP/GFR) were observed in most children.
  • Plasma intact FGF23 concentrations were elevated, while PTH levels were mostly normal.
  • Serum phosphorus correlated with TMP/GFR, alkaline phosphatase, age, LDH, and FGF23; TMP/GFR showed a significant correlation with FGF23.

Conclusions:

  • Patients with SCD exhibit elevated serum phosphorus and increased TMP/GFR, alongside elevated FGF23 levels.
  • These findings suggest proximal tubular resistance to FGF23 action in SCD patients prior to any GFR decline.
  • This indicates early renal tubular dysfunction in sickle cell disease.
Abstract

Keywords:
estimated glomerular filtration ratefibroblast growth factor 23intact parathyroid hormonesickle cell diseasetubular maximum re-absorption of phosphorus per unit of GFRtubular re-absorption of phosphorus

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