A rare case of hepatolenticular degeneration with double panda sign on imaging
نویسندگان
چکیده
Wilson's disease is an inborn error of copper metabolism that characterized by deficiency ceruloplasmin, the serum transport protein for copper. Copper collected in liver, and after hepatic binding sites are saturated, it released. Systemic then develops there abnormal accumulation brain, particularly putamen globus pallidus. Presenting this case a 32-year-old male patient who presented with peculiar features Wilson’s disease.
منابع مشابه
Hepatolenticular degeneration combined with primary antiphospholipid syndrome: a case report.
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متن کاملHepatolenticular Degeneration (Wilson's Disease)
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متن کاملWilson's disease; hepatolenticular degeneration.
The inclusion of a rare neurological disease in an issue of this journal devoted to disorders of the liver would seem to warrant some explanation for although cirrhosis of the liver is an integral part of the syndrome of Wilson's disease, it accounts for an insignificant proportion of all patients suffering from hepatic cirrhosis. However, although quantitatively unimportant, its significance r...
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15 صفحه اولClinical and pathological findings in a case of hepatolenticular degeneration treated with penicillamine.
Hepatolenticular degeneration, a condition inherited in an autosomal recessive fashion, is generally thought to be due to a biochemical defect associated with the retention of copper in the tissues, principally the brain, liver, and kidneys, an increased excretion of the metal in the urine, and a low level of caeruloplasmin, the copper-binding alpha-globulin in the plasma. A number of products ...
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ژورنال
عنوان ژورنال: International Journal of Advances in Medicine
سال: 2022
ISSN: ['2349-3925', '2349-3933']
DOI: https://doi.org/10.18203/2349-3933.ijam20222675