Utility of cystatin C to monitor renal function in duchenne muscular dystrophy

Viollet, Laurence; Gailey, Susan; Thornton, David J.; Friedman, Neil; Flanigan, Kevin M.; Mahan, John D.; Mendell, Jerry R.

doi:10.1002/mus.21420

Cited by 90 publications

(66 citation statements)

References 31 publications

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“…Serum BUN and CysC levels lay outside the reference range at the renal dysfunction time, but the change in Cr was so subtle that even the higher value did not exceed the normal range. These findings are in agreement with a proposal that CysC and its related equations are more reliable renal markers in DMD patients [7,9]. Although the effect of corticosteroids on CysC levels remain unclear [10,11], none of the four patients here were taking corticosteroids at the time of renal failure.…”

Section: Discussionsupporting

confidence: 93%

See 1 more Smart Citation

Treatable renal failure found in non-ambulatory Duchenne muscular dystrophy patients

Motoki

Shimizu‐Motohashi

Komaki

et al. 2015

Neuromuscular Disorders

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Section: Discussionsupporting

confidence: 93%

“…Therefore, CysC may be a better marker of renal function in reduced muscle mass cases, including DMD patients [7]. eGFRCys measurement is based on serum CysC level [8], and in DMD patients, eGFRCys correlates better with GFR, as measured by 51 Cr-EDTA than the serum Cr-based equation [9].…”

Section: Discussionmentioning

confidence: 99%

Treatable renal failure found in non-ambulatory Duchenne muscular dystrophy patients

Motoki

Shimizu‐Motohashi

Komaki

et al. 2015

Neuromuscular Disorders

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“…In addition, it is important to monitor renal function as well, keeping in mind that the renal filtration rate cannot be accurately calculated based on creatinine in DMD due to reduced muscle mass. Cystatin can be used to follow renal function because serum creatinine is so dependent on muscle mass [30].…”

Section: Pharmacotherapymentioning

confidence: 99%

Cardiac Assessment in Duchenne and Becker Muscular Dystrophies

Romfh

McNally

2010

Curr Heart Fail Rep

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Mutations in the dystrophin gene cause Duchenne and Becker muscular dystrophies. In addition to muscle disease, there nearly always is an associated cardiomyopathy in Duchenne or Becker muscular dystrophy. In these muscular dystrophies, the severity of cardiomyopathy and congestive heart failure may not parallel the severity of skeletal muscle disease. Loss of normal dystrophin function in the heart produces four-chamber dilation and reduction in left ventricular function that develop after the onset of muscle weakness. Arrhythmias affecting both atrial and ventricular rhythms occur and may be life threatening. The degree to which hypoventilation and pulmonary dysfunction are present also directly affect cardiac function in muscular dystrophy. Care guidelines recently were issued to outline surveillance and treatment strategies for the younger patient with Duchenne muscular dystrophy. Herein, we review those guidelines, and additionally, provide recommendations for monitoring and treating cardiac disease in the populations of advanced Duchenne and Becker muscular dystrophies.

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“…Therefore, we evaluated other accurate and validated creatinine-independent methods for monitoring GFR and urinary excretions. In this regard, CysC, which is independent of muscle mass and hydration, was suggested as a valuable alternative for calculating estimated GFR (eGFR) in these patients [11,12].…”

Section: Introductionmentioning

confidence: 99%

Renal function in children and adolescents with Duchenne muscular dystrophy

Braat

Hoste

Waele

et al. 2015

Neuromuscular Disorders

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Improved life expectancy and the need for robust tools to monitor renal safety of emerging new therapies have fueled the interest in renal function in Duchenne muscular dystrophy (DMD) patients. We aimed to establish a methodology to accurately assess their renal function. Twenty DMD patients (5-22 years) were included in this prospective study. After obtaining medical history, all patients underwent a clinical examination, 24-hour ambulatory blood pressure monitoring, ultrasound of the kidneys, direct GFR measurement ( 51 Cr-EDTA, mGFR), complete blood and urine analysis. Seventeen of 20 patients were treated with corticosteroids and 5/20 with angiotensin converting enzyme inhibitor (lisinopril). No patient suffered from urinary tract infections or other renal diseases. Hypertension (systolic or diastolic blood pressure >P95) was found in 9/20 patients (8/9 patients were on steroid treatment) and a non-dipping blood pressure profile in 13/20 subjects (10/13 patients were on steroid treatment). Urinary protein to creatinine ratio was elevated in 17/18 patients, whereas 24-hour urine protein excretion was normal in all subjects. Median interquartile range (IQR) mGFR was 130.4 (29.1) mL/min/1.73 m 2 . Hyperfiltration (mGFR >150 mL/min/1.73 m 2 ) was found in 5/20 patients. Inverse correlation between mGFR and age was observed (R 2 = 0.45, p = 0.001). Serum creatinine based estimated GFR (eGFR) equations overestimated mGFR up to 300%. eGFR based on cystatin C Filler equation was closest to the mGFR (median eGFR (IQR) of 129.5 (39.7) mL/min/1.73 m 2 ). Our study demonstrates a high prevalence of hyperfiltration and hypertension in children and adolescents with DMD. Because the majority of hypertensive patients were under corticosteroid treatment, the iatrogenic cause of hypertension cannot be excluded. Serum or urine creatinine measurements are of no value to evaluate renal function in DMD patients due to the reduced skeletal muscle mass.

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Utility of cystatin C to monitor renal function in duchenne muscular dystrophy

Cited by 90 publications

References 31 publications

Treatable renal failure found in non-ambulatory Duchenne muscular dystrophy patients

Treatable renal failure found in non-ambulatory Duchenne muscular dystrophy patients

Cardiac Assessment in Duchenne and Becker Muscular Dystrophies

Renal function in children and adolescents with Duchenne muscular dystrophy

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