Some authors have suggested the administration subcutaneous of copper histidinate for the treatment of the Menkes disease. The purpose of this study was to control the complex formation of Cu (II)-L-Histidine in molar ratio 1/2 and to verify the stability in some conditions of determination and validation production.
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Huppke-Brendel syndrome (HBS) is an autosomal recessive disorder caused by mutations, a gene coding for the acetyl-CoA transporter-1 (AT-1). So far it has been described in nine pediatric and one adult patient. Therapeutic trials with copper histidinate failed to achieve any clinical improvement.
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Copper (II) histidinate injection solution, applied in Menkes disease treatment, is characterized by low stability due to sensitivity to oxidation. The aim of this article was to determine the critical points of the injection preparation procedure, taking into account selection of appropriate packaging, determining the solution pH or application of an excess of L-histidine. In order to assess the stability of the Cu(His) complex, the spectrophotometric method (VIS: 400-800 nm), and the colorimetric method using a reflectance colorimeter were applied.
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