Copper is a building block of many body proteins almost AND ALL copper in the body is bound to proteins. Unbound (free) copper ions are toxic. Genetic mechanisms control the installation of copper in apoproteins and processes that prevent toxic accumulation of copper in the body. If more copper absorbed than is necessary for the metabolic function, it is excreted in the bile. Acquired copper deficiency functioning genetically controlled mechanisms in copper metabolism normal, rarely resulted in insufficient supply a clinically significant deficiency. Causes include: severe protein deficiency in childhood persisting infantile diarrhea (usually associated with a limited milk diet) severe malabsorption (as with celiac disease or cystic fibrosis) stomach operation (which could also be a vitamin B12 deficiency), excessive zinc intake. Copper deficiency can lead to neutropenia, impaired Knochenkalzifizierung, myelopathy, neuropathy and hypochromic anemia, which does not decay when taking iron supplements. The diagnosis is based on low serum levels of copper and ceruloplasmin, although these tests are not always reliable. Treatment depends on the cause; it will be given orally as copper sulphate daily 1.5-3 mg of copper. Hereditary copper deficiency (Menkes syndrome) Hereditary copper deficiency affects boys in infancy, on the X-chromosome a gene is mutated. This syndrome occurs in about one in 100,000-250,000 live births. Copper is absent in the liver, serum and essential copper proteins such as cytochrome c oxidase, ceruloplasmin, and lysyl oxidase. Symptoms and complaints as deficiency symptoms in hereditary copper deficiency severe mental retardation, vomiting, diarrhea, protein-losing Enteropathy, hypopigmentation, bone changes and of artery apply. The hair is thin, stringy or brittle. Most affected children die at the age of 10 Jahren.Diagnose serum levels of copper and ceruloplasmin the diagnosis of hereditary copper deficiency is based on low copper and Ceruloplasminspiegel in serum. Since early diagnosis and treatment is associated with a better prognosis, the fault is ideally in the first two weeks of life. However, the diagnostic accuracy of these tests is limited. Therefore, other tests entwickelt.Therapie Kupferhistidin As a rule parenteral copper s.c. as Kupferhistidin at a dose of 250 micrograms given up to the age of 1 year, after 250 ug s.c. once a day until the age of 3 years. During treatment, monitoring of renal function is important. Despite the early treatment, many children show an unusual development of the nervous system. Acquired Kupferintoxikation Acquired Kupferintoxikation can consist of excessive intake or absorption of copper, for example. B. after the consumption of an acidic food or beverage, which was kept in a copper container over time, result. It can be a self-limiting gastroenteritis occur in typical form with nausea, vomiting and diarrhea. The intoxication takes a severe course if it through the feed (usually in suicidal intent) of copper salt such. was triggered as copper sulfate, in grams dose or by absorption of large amounts of copper through the skin (z. B. when applying impregnated with copper salt solution compresses on large-area burns). As a result, hemolytic anemia, and anuria occur, possibly with fatal outcome. The so-called. Indian Childhood cirrhosis, non-Indian Childhood cirrhosis and idiopathic Kupferintoxikation are identical diseases caused by excessive copper intake and lead to cirrhosis. All forms are caused by the consumption of milk, which was cooked in damaged copper or brass vessels or stored. Studies led to the discovery that an idiopathic Kupferintoxikation occurs only in infants with unknown genetic defect. Diagnostic liver biopsy is performed, could be detected with the hyaline Mallory bodies. Treatment of acquired Kupferintoxikation chelation Supporting tests In a Kupferintoxikation by uptake of copper in gram quantities is carried out an immediate gastric lavage. A Kupferintoxikation which causes complications such as anemia, anuria or hepatotoxicity is also treated with 250 mg penicillamine p.o. every 6 hours up to 750 mg every 12 hours (1000-1500 mg / day in 2-4 doses) or 3-5 mg / kg i.m. dimercaprol every 4 hours for 2 days, then every 4-6 h (Chelattherapy). If a hemodialysis time performed, it can be used therapeutically. Occasionally, the Kupferintoxikation fatal despite case of treatment measures.