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Titolo:
Iron accumulation in the liver of male patients with Wilson's disease
Autore:
Shiono, Y; Wakusawa, S; Hayashi, H; Takikawa, T; Yano, M; Okada, T; Mabuchi, H; Kono, S; Miyajima, H;
Indirizzi:
Hokuriku Univ, Fac Pharmaceut Sci, Dept Med, Kanazawa, Ishikawa 9201181, Japan Hokuriku Univ Kanazawa Ishikawa Japan 9201181 wa, Ishikawa 9201181, Japan Nagoya Univ, Sch Med, Dept Internal Med 3, Nagoya, Aichi 466, Japan NagoyaUniv Nagoya Aichi Japan 466 ternal Med 3, Nagoya, Aichi 466, Japan Kanazawa Univ, Sch Med, Dept Internal Med 2, Kanazawa, Ishikawa 920, JapanKanazawa Univ Kanazawa Ishikawa Japan 920 , Kanazawa, Ishikawa 920, Japan Hamamatsu Med Coll, Dept Internal Med 1, Hamamatsu, Shizuoka, Japan Hamamatsu Med Coll Hamamatsu Shizuoka Japan , Hamamatsu, Shizuoka, Japan
Titolo Testata:
AMERICAN JOURNAL OF GASTROENTEROLOGY
fascicolo: 11, volume: 96, anno: 2001,
pagine: 3147 - 3151
SICI:
0002-9270(200111)96:11<3147:IAITLO>2.0.ZU;2-J
Fonte:
ISI
Lingua:
ENG
Soggetto:
PORPHYRIA-CUTANEA-TARDA; HEPATIC IRON; FERROXIDASE ACTIVITY; JAPANESE PATIENTS; GENE; HEMOCHROMATOSIS; MUTATIONS; CERULOPLASMIN; PREVALENCE; DEFICIENCY;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
39
Recensione:
Indirizzi per estratti:
Indirizzo: Hayashi, H Hokuriku Univ, Fac Pharmaceut Sci, Dept Med, Kanazawa, Ishikawa9201181, Japan Hokuriku Univ Kanazawa Ishikawa Japan 9201181 a 9201181, Japan
Citazione:
Y. Shiono et al., "Iron accumulation in the liver of male patients with Wilson's disease", AM J GASTRO, 96(11), 2001, pp. 3147-3151

Abstract

OBJECTIVES: There is accumulating evidence that ceruloplasmin, a copper protein with ferroxidase activity, plays an important role in iron metabolism. The genetic disorder, aceruloplasminemia, can lead to tissue storage of iron as in hemochromatosis. Because most patients with Wilson's disease, a genetic copper toxicosis, have hypoceruloplasminemia, some could be affectedby iron overload. METHODS: Four male patients with Wilson's disease were enrolled in this study of pre- and post-treatment iron metabolism. RESULTS: Pretreatment copper contents of the liver were high in all four male patients studied as diagnostic of Wilson's disease. Genetic analysis supported their clinical diagnosis of Wilson's disease without a background of hemochromatosis. Pretreatment serum ceruloplasmin levels were < 20 mg/dl in all four patients. A standard penicillamine treatment for 3-8.5 yr further decreased their serum ceruloplasmin levels. Post-treatment serum ferroxidase activity was low as was the serum ceruloplasmin protein. Copper contents in the liver decreased after treatment in all subjects. In contrast, nonheme iron in the liver increased during treatment. Pretreatment liver specimens were positive for histochemical iron in two patients, and post-treatment Specimens were positive in all four patients. In two patients, serum aminotransferase levels rebounded with elevation of serum ferritin concentration during the treatment period. Subsequent iron reduction by phlebotomy ameliorated their biochemical liver damage. CONCLUSION: Iron overload related to hypoceruloplasminemia may be clinically important, particularly in male patients with Wilson's disease. (C) 2001by Am. Coll. of Gastroenterology.

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Documento generato il 29/03/20 alle ore 10:01:54