title: Various copper and iron overload patterns in the livers of patients with Wilson disease and idiopathic copper toxicosis.
abstract: Wilson disease (WD) is a major type of primary copper toxicosis associated with hypoceruloplasminemia, while idiopathic copper toxicosis (ICT) is a minor type characterized by normoceruloplasminemia. Because ceruloplasmin is the major circulating ferroxidase, iron metabolism may be affected in patients with WD. Biopsied liver specimens obtained from patients with primary copper toxicosis were fixed with glutaraldehyde solution and embedded in epoxy resin. Ultrathin sections that had or had not been stained with uranyl acetate solution were examined under an electron microscope equipped with an energy dispersive X-ray analyzer. A 7-year-old boy with WD was free from any metal overloading at the pre-treatment stage. Pre-treatment liver specimens of another 16 patients showed a variety of copper and iron overload patterns, from isolated copper to evenly distributed combined overloading. A 19-year-old female patient was free from any metal overloading after 7 years of treatment. Post-treatment overloading in another 6 patients ranged between evenly distributed combined patterns and isolated iron patterns. All patients had hypoceruloplasminemia throughout treatment periods. A patient with normoceruloplasminemic ICT continued to display isolated copper overloading after 2.5 years of treatment. In conclusion, these observations support the hypothesis that iron accumulates in patients with hypoceruloplasminemia.
authors: Department of Medicine, Aichi Gakuin University School of Pharmacy, Kusumoto-cho, Chikusa-ku, Nagoya, 464-8650, Japan, hhayashi@dpc.agu.ac.jp.
authors: Hisao
authors: H
authors: Hayashi
authors: Ai
authors: A
authors: Hattori
authors: Yasuaki
authors: Y
authors: Tatsumi
authors: Kazuhiko
authors: K
authors: Hayashi
authors: Yoshiaki
authors: Y
authors: Katano
authors: Jun
authors: J
authors: Ueyama
authors: Shinya
authors: S
authors: Wakusawa
authors: Motoyoshi
authors: M
authors: Yano
authors: Hidemi
authors: H
authors: Goto
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D003300:Copper / Q000378:metabolism* / Q000633:toxicity*; D005260:Female; D006527:Hepatolenticular Degeneration / Q000378:metabolism* / Q000628:therapy; D006801:Humans; D019190:Iron Overload / Q000378:metabolism* / Q000628:therapy; D008099:Liver / Q000378:metabolism*; D008103:Liver Cirrhosis / Q000378:metabolism* / Q000628:therapy; D008297:Male; D008664:Metal Metabolism, Inborn Errors / Q000378:metabolism* / Q000628:therapy; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D003300:Copper