[copper Metabolism and Wilson's Disease].
نویسنده
چکیده
منابع مشابه
Copper metabolism in normal adults and in clinically normal relatives of patients with Wilson's disease.
Wilson's disease (hepato-lenticular degeneration) is now known to be associated with an inborn error of metabolism. The following biochemical abnormalities have been repeatedly confirmed in patients suffering from the disease (Scheinberg, 1956; Bickel, Neale, and Hall, 1957): Increased content of copper in the liver, brain, and other tissues, increased urinary excretion of copper, a decreased l...
متن کاملReview Article Wilson's Disease: a review
Introduction and Genetics Wilson's disease (WD; Hepatolenticular degeneration) is an autosomal-recessive disorder of copper metabolism due to absence or dysfunction of a copper-transporting, Ptype ATPase which is essential for the transport of copper into bile. Affected patients accumulate excessive copper within the liver as well as the brain and other tissues. This is mainly a disease of chil...
متن کاملSpontaneous hepatic copper accumulation in Long-Evans Cinnamon rats with hereditary hepatitis. A model of Wilson's disease.
Long-Evans Cinnamon (LEC) rats, an inbred strain of a mutant rat isolated from Long-Evans rats, develop hereditary hepatitis. To elucidate the role of copper metabolism in the development of the hepatitis in LEC rats, we examined the copper concentration in the tissues and serum levels of copper and ceruloplasmin. Copper concentration in the liver of LEC rats was over 40 times that of normal Lo...
متن کاملWilson's Disease : A Case Report
Wilson's disease is a rare metabolic disorder involving copper metabolism may present with hepatic, neurological and psychiatric manifestations. We present a case of Wilson's disease with behavioral symptoms, which responded to risperidone.
متن کاملInherited Copper Transport Disorders: Biochemical Mechanisms, Diagnosis, and Treatment
Copper is an essential trace element required by all living organisms. Excess amounts of copper, however, results in cellular damage. Disruptions to normal copper homeostasis are hallmarks of three genetic disorders: Menkes disease, occipital horn syndrome, and Wilson's disease. Menkes disease and occipital horn syndrome are characterized by copper deficiency. Typical features of Menkes disease...
متن کاملOrthotopic liver transplantation for Wilson's disease.
S An ll-year-old boy with terminal ummary hepatic failure due to Wilson's disease was treated 18 months ago with orthotopic liver transplantation. Postoperatively, there has been evidence of clearance of body copper stores but without accumulation of copper in biopsy specimens of the transplanted liver after 6 and 17 months. Further follow-up will be necessary before deciding whether the disord...
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عنوان ژورنال:
- Transactions of the Association of American Physicians
دوره 67 شماره
صفحات -
تاریخ انتشار 1954