Haff disease medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: M. Hassan, M.B.B.S
Overview
The medical therapy of Haff disease consists of managing rhabdomyolysis by aggressive fluid hydration, managing electrolyte abnormalities (hyperkalemia and hypocalcemia), and other supportive care.
Medical therapy
Management of Rhabdomyolysis
The goal of rhabdomyolysis is adequate fluid hydration with normal isotonic saline to prevent acute kidney injury. Urine output of 200 to 300 mL/h should be maintained with daily serial monitoring of CPK level to document a downward trend. In case of fluid overload from aggressive fluid resuscitation, loop diuretics may be considered.[1] In severe cases with CPK level > 30,000 IU/L, alkalization of the urine with bicarbonate can be considered.[1][2]
Management of Electrolytes Abnormalities
Rhabdomyolysis is associated with hyperkalemia and hypocalcemia.[1]
Hyperkalemia[1]
- Potassium levels less than 6 mEq/L without EKG changes: managed with potassium binders and use of bicarbonate in fluids.
- Potassium levels 6 mEq/L or above, with or without EKG changes: ampule of D50 followed by zero units of regular insulin and IV sodium bicarbonate.
Hypocalcemia[1]
- Symptomatic hypocalcemia: should be treated with IV calcium gluconate.
Other Supportive Care[1]
- Disseminated intravascular coagulation: should be managed with fresh frozen plasma, cryoprecipitate, and platelet transfusion.
- Compartment syndrome: emergent orthopedic consultation is required.