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Renal tubular dysfunction in children with sickle cell haemoglobinopathy

Mohamed Badr1, Mohamed A El Koumi, Yasser F Ali

  • 1Pediatric Department, Zagazig University Children Hospital, Zagazig, Egypt. mohamed_197228@hotmail.com

Nephrology (Carlton, Vic.)
|February 26, 2013
PubMed

Insights

Children with sickle cell disease (SCD) show impaired kidney function, specifically in proximal tubular function. Higher urinary excretion of retinol binding protein (RBP) and beta-2 microglobulin (β2 MG) indicates this dysfunction in SCD patients.

Area of Science:

  • Nephrology
  • Pediatrics
  • Hematology

Background:

  • Children with sickle cell disease (SCD) are at increased risk for renal dysfunction.
  • Kidney damage in SCD can stem from chronic anemia and vaso-occlusive events.
  • Proximal tubular function is a critical aspect of overall renal health.

Purpose of the Study:

  • To evaluate proximal tubular function in Saudi children diagnosed with sickle cell disease (SCD).
  • To compare renal markers between children with SCD and those with sickle cell trait (SCT).

Main Methods:

  • A study involving 34 children with SCD (HBSS) and 27 children with SCT (HBAS) was conducted in Saudi Arabia.
  • Urinary excretion of retinol binding protein (RBP) and beta-2 microglobulin (β2 MG) was measured in both groups.
  • Urinary concentrating ability was also assessed.

Main Results:

  • Children with SCD exhibited significantly impaired urinary concentrating ability compared to the SCT group.
  • Urinary excretion of RBP and β2-microglobulin was significantly higher in the SCD group.
  • Elevated levels of RBP and β2-microglobulin suggest compromised proximal tubular function in SCD.

Conclusions:

  • Significant proximal tubular dysfunction is evident in children with SCD, marked by increased urinary RBP and β2-microglobulin.
  • Monitoring urinary excretion of these low molecular weight proteins can provide valuable clinical information for managing renal health in SCD patients.
  • Early detection and follow-up of renal tubular function are crucial for children with sickle cell disease.
Abstract

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