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

 Soon Min Lee  ;  Ji Hong Kim  ;  Young Mock Lee  ;  Joon Soo Lee  ;  Heung Dong Kim 
 ACTA PAEDIATRICA, Vol.98(6) : 1014-1018, 2009 
Journal Title
Issue Date
Biomarkers/blood* ; Biomarkers/urine ; Child ; Child, Preschool ; Creatinine/blood ; Creatinine/urine ; Cystatin C/blood* ; Female ; Glomerular Filtration Rate* ; Humans ; Male ; Mitochondrial Diseases/diagnosis* ; Mitochondrial Diseases/diagnostic imaging ; Radionuclide Imaging ; Radiopharmaceuticals ; Reproducibility of Results ; Technetium Tc 99m Pentetate
Cystatin C ; Glomerular filtration rate ; Mitochondria ; Respiratory chain
AIM: Mitochondrial disorders with respiratory chain complex defect are known to show a broad spectrum of clinical phenotypes, though their contribution to renal disease has not received much attention. The aim of this study was to evaluate renal function using different markers of the glomerular filtration rate (GFR) in children with mitochondrial respiratory chain complex (MRC) defects. METHODS: Twenty-two patients with MRC I defect confirmed by biochemical enzyme assays using muscle tissue, were evaluated for renal function with diethylenetriaminepentaacetic acid (DTPA) renal scintigraphy, urine creatinine clearance, serum creatinine (Scr) and cystatin C (Cys C) levels. RESULTS: All patients showed lower levels within normal range of Scr (0.4-0.7 mg/dL, mean 0.47 mg/dL), and with no significant correlation with DTPA-GFR. Cys C showed significant correlation with DTPA-GFR (p < 0.01) providing higher diagnostic accuracy than creatinine in patients with MRC defect. CONCLUSION: In mitochondrial disorder with MRC defect, Scr could not fully reflect renal function due to the relatively small body mass of patients. Considering the limited clinical availability of DTPA renal scintigraphy, Cys C should be taken as the first step to evaluate GFR in mitochondrial disorders.
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1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Ji Hong(김지홍) ORCID logo https://orcid.org/0000-0001-5352-5423
Kim, Heung Dong(김흥동) ORCID logo https://orcid.org/0000-0002-8031-7336
Lee, Soon Min(이순민) ORCID logo https://orcid.org/0000-0003-0174-1065
Lee, Young Mock(이영목) ORCID logo https://orcid.org/0000-0002-5838-249X
Lee, Joon Soo(이준수) ORCID logo https://orcid.org/0000-0001-9036-9343
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