Abstract
Wilson disease is a rare autosomal recessive disorder of the copper metabolism caused by homozygous or compound heterozygous mutations in the ATP-ase Cu(2+) transporting polypeptide (ATP7B) gene. The copper accumulation in different organs leads to the suspicion of Wilson disease. We describe a child with clinical zinc deficiency as presenting symptom of Wilson disease, which was confirmed by 2 mutations within the ATP7B gene and an increased copper excretion.
MeSH terms
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Adenosine Triphosphatases / genetics*
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Biological Transport
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Cation Transport Proteins / genetics*
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Child, Preschool
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Copper / metabolism*
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Copper-Transporting ATPases
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Deficiency Diseases / etiology
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Deficiency Diseases / genetics*
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Deficiency Diseases / metabolism
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Hepatolenticular Degeneration / complications
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Hepatolenticular Degeneration / pathology*
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Homozygote
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Humans
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Liver / metabolism
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Male
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Mutation*
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Zinc / deficiency*
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Zinc / metabolism
Substances
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Cation Transport Proteins
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Copper
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Adenosine Triphosphatases
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ATP7B protein, human
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Copper-Transporting ATPases
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Zinc