Utility of cystatin C to monitor renal function in Duchenne muscular dystrophy.

Abstract

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.

• Authors: Laurence Viollet, Susan Gailey, David J Thornton, Neil R Friedman, Kevin M Flanigan, John D Mahan, Jerry R Mendell
• Journal: Muscle & nerve (Muscle Nerve) Vol. 40 Issue 3 Pg. 438-42 (Sep 2009) ISSN: 0148-639X [Print] United States
• PMID: 19623638 (Publication Type: Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)

Chemical References

• Adrenal Cortex Hormones
• Cystatin C
• Creatinine

Topics

• Adolescent
• Adrenal Cortex Hormones (blood)
• Child
• Creatinine (blood)
• Cystatin C (blood)
• Dependent Ambulation
• Female
• Humans
• Kidney Diseases, Cystic (blood, etiology)
• Male
• Monitoring, Physiologic (methods)
• Muscular Dystrophy, Duchenne (blood, complications)