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Assessment of Mitochondrial Functions and Cell Viability in Renal Cells Overexpressing Protein Kinase C Isozymes
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Assessment of Mitochondrial Functions and Cell Viability in Renal Cells Overexpressing Protein Kinase C Isozymes

Published on: January 7, 2013

Evaluation of renal function in children with mitochondrial respiratory chain complex defect: usefulness of cystatin

Soon Min Lee1, Ji Hong Kim, Young Mock Lee

  • 1Department of Pediatrics, Severance Children's Hospital, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.

Acta Paediatrica (Oslo, Norway : 1992)
|March 17, 2009
PubMed

Insights

Serum creatinine is unreliable for assessing kidney function in children with mitochondrial respiratory chain complex (MRC) defects. Cystatin C offers a more accurate and accessible method for evaluating glomerular filtration rate (GFR) in these patients.

Area of Science:

  • Pediatric Nephrology
  • Mitochondrial Diseases
  • Biochemical Markers

Background:

  • Mitochondrial disorders often present with diverse clinical symptoms.
  • The impact of mitochondrial respiratory chain complex (MRC) defects on renal function is understudied.
  • Accurate assessment of glomerular filtration rate (GFR) is crucial in pediatric patients.

Purpose of the Study:

  • To evaluate renal function in children with MRC defects.
  • To compare different markers of GFR, including serum creatinine, cystatin C, and DTPA renal scintigraphy.
  • To determine the most reliable GFR marker in this specific pediatric population.

Main Methods:

  • Studied 22 children with confirmed MRC I defects.
  • Assessed renal function using DTPA renal scintigraphy, urine creatinine clearance, serum creatinine (Scr), and cystatin C (Cys C).
  • Biochemical enzyme assays on muscle tissue confirmed MRC defects.

Main Results:

  • Serum creatinine levels were within the normal range but did not correlate significantly with DTPA-GFR.
  • Cystatin C demonstrated a significant correlation with DTPA-GFR (p < 0.01).
  • Cystatin C provided higher diagnostic accuracy for GFR compared to creatinine in patients with MRC defects.

Conclusions:

  • Serum creatinine may not accurately reflect renal function in children with MRC defects due to small body mass.
  • Cystatin C is a more reliable indicator of GFR in these patients.
  • Given the limited availability of DTPA scintigraphy, cystatin C is recommended as a primary tool for GFR evaluation in mitochondrial disorders.
Abstract

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