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Frequency of glomerular dysfunction in children with Beta thalassaemia major

Basma A Ali1, Ahmed M Mahmoud1

  • 1Department of Pediatrics, University Children Hospital, Minia University, Minia, Egypt.

Sultan Qaboos University Medical Journal
|February 12, 2014
PubMed

Insights

Children with beta thalassaemia major (β-TM) often experience glomerular dysfunction. Cystatin C shows promise as a sensitive marker for monitoring kidney health in these patients.

Area of Science:

  • Pediatric Nephrology
  • Hematology
  • Biochemistry

Background:

  • Beta thalassaemia major (β-TM) is a severe inherited blood disorder.
  • Chronic anemia and iron overload are common in β-TM patients.
  • Potential for kidney complications necessitates investigation.

Purpose of the Study:

  • To determine the frequency of glomerular dysfunction in children with β-TM.
  • To evaluate different markers of kidney function.
  • To correlate these markers with serum ferritin and iron chelation therapy.

Main Methods:

  • Study included 100 β-TM patients (two groups: chelation therapy vs. follow-up) and 50 healthy controls.
  • Assessed cystatin C, serum creatinine, serum ferritin, urine albumin/creatinine ratio, estimated glomerular filtration rate (eGFR), and creatinine clearance.
  • Compared markers between patient groups and controls.

Main Results:

  • β-TM patients showed significantly higher cystatin C, serum creatinine, serum ferritin, and urine albumin/creatinine ratio compared to controls.
  • Patients had significantly lower eGFR and creatinine clearance.
  • Cystatin C demonstrated higher sensitivity and specificity for detecting small GFR changes than serum creatinine and creatinine clearance.

Conclusions:

  • Glomerular dysfunction is frequent in β-TM, potentially due to anemia, iron overload, or chelation therapy.
  • Regular renal assessment is crucial for early detection of complications.
  • Cystatin C is a valuable marker for monitoring glomerular dysfunction in β-TM.
Abstract

Keywords:
Abnormalities, glomerularChelation TherapyCystatin CEgyptbeta-Thalassemia

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