Cuprous Sebacate Treatment of Menkes’ Disease

Abstract

In 1962, Menkes et al, first described an inherited disorder that was characterized by severe neurologic, integumentary, skeletal, and developmental abnormalities and that was also associated with early death in male infants.¹ In 1972, Danks and his group demonstrated that this disorder was associated with abnormal copper metabolism.^2–11 Copper absorption was impaired in affected infants, with resultant hypocupremia and hypocerulo-plasminemia. Since then, a number of attempts have been made to correct the abnormalities of Menkes’ disease with copper administration.^3–11 In general, these attempts have been successful in raising levels of serum copper and ceruloplasmin to normal levels, but convincing changes in the clinical course of treated patients has not been demonstrated. (Table 1)