Copper Metabolism in the Steely-Hair Syndrome

Life‐span and Menkes kinky hair syndrome: report of a 13‐year course of this disease

“…Many different compounds were used (Cu-sulphate, Cuchloride, Cu-EDTA, Cu-glycinate, Cu-histidinate or Cu-albumin-complex). Substitution by oral administration was achieved only with extremely high doses (Danks et al 1972, Danks 1983, Horn 1984, Lott et al 1975; better absorption was obtained with Cu-nitriloacetate (Danks 1983). Normal serum copper levels and liver values were attained, but only slight clinical improvement was registered (Danks 1983, Danks & Camakaris 1983, Horn 1984, Lott et al 1975, Wehinger et al 1975.…”

“…No improvement of the severe symptoms of the central nervous system was achieved (Danks et ai. 1972, Danks 1983, Danks'& Camakaris 1983, Horn 1984, Lott et al 1975, Wehinger et al 1975. A delay of the psychomotoric regression due to early treatment has been claimed (Baerlocher et al 1983, Danks 1983, Danks & Camakaris 1983, Grover & Scrutton 1975, Grover et al 1979.…”

Menkes Kinky Hair Syndrome: Is it a treatable disorder?

“…There remain, however, many unanswered questions concerning the nature of Menkes Syndrome, among which is the explanation for the failure of parenteral copper therapy to bring about either clinical improvement or an increase in certain copper metalloenzymes. Several patients have been reported (Walker-Smith 1973, Wehringer et al 1975, Lott 1975a, Grover & Scrutton 1975 in serum copper concentrations were et al e t al. whom maintained at more than 100 mg/100 ml for periods longer than 1 month, with no improvement in the clinical status.…”

Copper Metabolism in the Steely-Hair Syndrome

“…This patient was given supplemental copper as cupric sulfate in two divided doses totalling 0.52 mg per kilogram per day. Within seven days of such supplemental oral copper, the serum copper rose three-fold (from 15 [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] per 100 ml) and remained at approximately 75 percent of the normal concentration for the remaining 22 days of therapy. After the end of supplementation, the serum copper returned to baseline in about ten days.…”