GSD Type V with Severe Rhabdomyolysis: What to Do When Fluid Therapy Has Not Cleared Myoglobinuria or Restored Renal Function
Clinical scenario
In glycogen storage disease type V, severe rhabdomyolysis complicated by myoglobinuria is a serious acute emergency. Myoglobinuria from rhabdomyolysis can precipitate acute renal failure, and when first-line fluid management proves insufficient, a structured next-line approach is required.
Previous line — failure condition
This protocol applies when adequate fluid administration — given to prevent renal impairment while accounting for fluid balance to avoid hypervolaemia and acute pulmonary oedema — has failed to achieve clearance of myoglobinuria and a return of creatinine and glomerular filtration rate to baseline.
Next-line approach
When fluid therapy is insufficient, the structured protocol addresses the initiation of renal replacement therapy. The full criteria, sequencing, and management algorithm are available in the complete protocol.
References
DOI: 10.1016/j.nmd.2021.10.006
- Patients with severe rhabdomyolysis should be treated with adequate fluid administration to prevent renal impairment or be put on dialysis if warranted.
- Myoglobinuria due to rhabdomyolysis can produce ARF, but data from large cohorts of patients suggest that this emergency complication is a rare event in both GSD V and GSD VII.
- Standard criteria for dialysis initiation are: fluid overload unresponsive to loop diuretics, and electrolyte disturbances such as hyperkalaemia, metabolic acidosis and uraemic encephalopathy.
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