This protocol covers Glycogen storage disease type V presenting with severe rhabdomyolysis and myoglobinuria — an acute complication requiring prompt assessment and targeted management to protect renal function.
Myoglobinuria resulting from rhabdomyolysis can lead to acute renal failure. Evidence from large patient cohorts indicates that while this emergency complication is a rare event in GSD V, it demands timely clinical action when it occurs.
Management centres on a fluid-based strategy directed at preventing renal impairment. The full protocol details the specific approach and the important fluid balance considerations that determine safe management — access it below.
Patients with severe rhabdomyolysis should be treated with adequate fluid administration to prevent renal impairment or be put on dialysis if warranted.
However, it is important to take into account the fluid balance to avoid further complications such as hypervolaemia and acute pulmonary oedema.
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.
Discharge criteria are contingent upon a lack of myoglobinuria and a return to baseline renal function (creatinine and glomerular filtration rate).
DOI: 10.1016/j.nmd.2021.10.006 View source ↗