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Menkes' kinky hair syndrome is a rare condition caused by malabsorption of copper. Infants with this sex-linked recessive abnormality develop growth failure, mental retardation, bone lesions and brittle hair. Anaemia and neutropenia also occur.
In other words,
a hereditary abnormality in copper absorption; the resultant copper poisoning causes symptoms such as sparse brittle twisted scalp hair, severe cerebral degeneration, and arterial changes with death in infancy; it is transmitted as an X-linked recessive trait.
Menkes' disease can lead to growth retardation, white hair that has a kinky texture, and mental deterioration.
Menkes syndrome condition, which serves as a model for copper deficiency, supports the idea that some of the clinical features seen in PEM are due to copper deficiency. Breast and cows' milk are low in copper and supplementation is occasionally necessary when first treating PEM.
Treatment of Menkes syndrome
There is no effective common treatment known. Injection of copper has been used with mixed results. Present therapy consists of: Copper acetate administered into a vein (intravenously) and Subcutaneous (injected under the skin) copper histidinate.
Treatment can even begin before birth; while still pregnant, mothers of babies identified with Menkes' disease can receive injections of copper histidine under the skin. Healthcare professionals, including geneticists (specialists in hereditary diseases), should be consulted in the treatment of Menkes' disease.
Seizures, abnormally light pigmentation of the skin and hair. Death usually occurs in the first few years of life.
Menkes is a serious disorder with early death being common.
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