Improved Long-Term Stability of In-House Compounded Histidine-Copper Injection for Menkes Disease by Frozen Storage

Menkes disease is an X-linked recessive disorder caused by mutations in the ATP7A gene. The mutations lead to impaired copper transport and severe systemic copper deficiency. Although therapeutic development has been established, histidine-copper (His-Cu) injection, prepared as a hospital based special formulation, remains essential for treatment. However, copper (II) hydroxide precipitation occurs when the formulation is stored under refrigerated conditions for more than four weeks, limiting its shelf life and imposing substantial burdens on pharmacists as well as patients and their families.