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Utility of cystatin C to monitor renal function in duchenne muscular dystrophy
Author(s) -
Viollet Laurence,
Gailey Susan,
Thornton David J.,
Friedman Neil R.,
Flanigan Kevin M.,
Mahan John D.,
Mendell Jerry R.
Publication year - 2009
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.21420
Subject(s) - duchenne muscular dystrophy , cystatin c , medicine , renal function , creatinine , biomarker , endocrinology , cystatin , nephrotoxicity , population , neuromuscular disease , muscular dystrophy , urology , kidney , disease , chemistry , biochemistry , environmental health
Creatinine as a marker of renal function has limited value in Duchenne muscular dystrophy (DMD) because of reduced muscle mass. Alternative methods of assessing renal function are sorely needed. Cystatin C, a nonglycosylated protein unaffected by muscle mass, is potentially an ideal biomarker of nephrotoxicity for this population but requires validation. In all, 75 subjects were recruited: 35 DMD (mean age 10.8 ± 5.4 years, corticosteroids n = 19, ambulatory n = 26), 29 healthy controls, 10 with renal disease, and one DMD with renal failure. Cystatin C levels in DMD were normal irrespective of age, ambulation, or corticosteroid treatment. Serum cystatin C was 0.67 ± 0.11 mg/l compared to normal controls 0.69 ± 0.09. mg/l. In these same individuals serum creatinine was severely reduced (0.27 ± 0.12 mg/dl) versus normals (0.75 ± 0.15 mg/dl, P < 0.01). In one DMD subject in renal failure, cystatin C was elevated. This study demonstrates the potential value of cystatin C as a biomarker for monitoring renal function in DMD. Its applicability extends to other neuromuscular diseases. Muscle Nerve, 2009