TABLE 1.
Management Options | Commonly Used Measures |
---|---|
Outpatient management | Oral nitrogen scavenger (e.g., sodium phenylbutyrate, glycerol phenylbutyrate) and l-citrulline |
Emergent cases | CVVH; i.v. sodium benzoate and sodium phenylacetate, arginine hydrochloride, and D10-based fluids |
Refractory to medical therapy | Consideration of debulking surgery to reduce FLC tumor volume, in conjunction with medical management, when feasible, and consistent with the overall goals of care |