Six-Digit CPK and Mildly Affected Renal Function in McArdle Disease

Abstract

A previously healthy, white 12-year-old girl presented with diffuse body aches and poor perfusion. She developed severe respiratory failure and marked rhabdomyolysis and was mechanically ventilated. Although her CPK peaked at 500,000 IU/L, her renal function was mildly affected and her creatinine did not exceed the 0.8 mg/dL. The rhabdomyolysis was gradually resolved following aggressive fluid hydration. The patient did not require dialysis and made a complete recovery. Genetic studies revealed the diagnosis of McArdle disease.

Seven-Digit Creatine Kinase in Acute Rhabdomyolysis in a Child

“…2 Although there is no established cutoff levels, a concentration 5 to 10 times higher than the upper limit of normal reference range (ie, 500-1000 U/L) is commonly used. 2,6 Other laboratory indicators include serum and urine myoglobin concentrations that may be useful but not essential for the diagnosis. 2 The most common complication of rhabdomyolysis is kidney damage.…”

“…2,6 Other laboratory indicators include serum and urine myoglobin concentrations that may be useful but not essential for the diagnosis. 2 The most common complication of rhabdomyolysis is kidney damage. 1,2 In recent large pediatric studies reported by Mannix et al 7 and Wu et al, 8 it was shown that the rate of acute renal failure in the pediatric patients with rhabdomyolysis ranged from 5% to 8.7%.…”

“…2 The most common complication of rhabdomyolysis is kidney damage. 1,2 In recent large pediatric studies reported by Mannix et al 7 and Wu et al, 8 it was shown that the rate of acute renal failure in the pediatric patients with rhabdomyolysis ranged from 5% to 8.7%. The risk factors that predispose a patient with rhabdomyolysis for developing acute renal injury are creatine kinase level concentration more than 5000 U/L, 2 creatine kinase value upon admission, and slower decline in the serum creatine kinase level.…”

“…15 The mainstay for the prevention and treatment of acute kidney injury is early and aggressive volume resuscitation. 2,5 Other management options include alkalization of the urine, forced diuresis with mannitol, and loop diuretics. 2 In severe cases or when the treatment fails, patients can undergo renal replacement therapy.…”

“…2,5 Other management options include alkalization of the urine, forced diuresis with mannitol, and loop diuretics. 2 In severe cases or when the treatment fails, patients can undergo renal replacement therapy. 11,12 In our case, the child developed extremely high creatine kinase levels that were not reported in the literature.…”