메뉴 건너뛰기




Volumn 4, Issue 12, 2003, Pages 2239-2251

Current and future therapy in haemochromatosis and Wilson's disease

Author keywords

Chelation; Haemochromatosis; Thalassaemia; Wilson's disease

Indexed keywords

ASCORBIC ACID; CHELATING AGENT; COPPER; DEFERASIROX; DEFERIPRONE; DEFEROXAMINE; FERRITIN; HLA ANTIGEN CLASS 2; HYDROXYBENZYLETHYLENEDIAMINEDIACETIC ACID; IRON; IRON BINDING PROTEIN; IRON CHELATING AGENT; MELARSOPROL; PENICILLAMINE; PYRIDOXINE; SUCCIMER; TETRATHIOMOLYBDIC ACID; TRIENTINE; UNCLASSIFIED DRUG; VITAMIN K GROUP; ZINC ACETATE;

EID: 0347365793     PISSN: 14656566     EISSN: None     Source Type: Journal    
DOI: 10.1517/14656566.4.12.2239     Document Type: Review
Times cited : (6)

References (114)
  • 2
    • 0032451073 scopus 로고    scopus 로고
    • Celtic origin of the C282Y mutation of hematochromatosis
    • LUCOTTE G: Celtic origin of the C282Y mutation of hematochromatosis. Blood Cells Mol. Dis. (1998) 24:433-438.
    • (1998) Blood Cells Mol. Dis. , vol.24 , pp. 433-438
    • Lucotte, G.1
  • 3
    • 9344224529 scopus 로고    scopus 로고
    • A novel MHC class 1-like gene is mutated in patients with hereditary hematochromatosis
    • FEDER JN, GNIRKE A, THOMAS W et al.: A novel MHC class 1-like gene is mutated in patients with hereditary hematochromatosis. Nat. Genet. (1996) 13:399-408.
    • (1996) Nat. Genet. , vol.13 , pp. 399-408
    • Feder, J.N.1    Gnirke, A.2    Thomas, W.3
  • 4
    • 0037132786 scopus 로고    scopus 로고
    • Penetrance of 845G- > A (C282Y)HFE hereditary hemochromatosis mutation in the USA
    • BEUTLER E, FELITTI VJ, KOZIOL JA, HO NJ, GELBART T: Penetrance of 845G- > A (C282Y)HFE hereditary hemochromatosis mutation in the USA. Lancet (2002) 359:211-218.
    • (2002) Lancet , vol.359 , pp. 211-218
    • Beutler, E.1    Felitti, V.J.2    Koziol, J.A.3    Ho, N.J.4    Gelbart, T.5
  • 5
    • 0036177909 scopus 로고    scopus 로고
    • A population-based study of the biochemical and clinical expression of the H63D hemochromatosis mutation
    • Erratum in: Gastroenterology (2002) 122(4):1191
    • GOCHEE PA, POWELL LW, CULLEN DJ, DU SART D, ROSSI E, OLYNYK JK: A population-based study of the biochemical and clinical expression of the H63D hemochromatosis mutation. Gastroenterology (2002) 122(3):646-651. Erratum in: Gastroenterology (2002) 122(4):1191.
    • (2002) Gastroenterology , vol.122 , Issue.3 , pp. 646-651
    • Gochee, P.A.1    Powell, L.W.2    Cullen, D.J.3    Du Sart, D.4    Rossi, E.5    Olynyk, J.K.6
  • 6
    • 0033358675 scopus 로고    scopus 로고
    • Juvenile hematochromatosis locus maps to chromosome 1q
    • ROETTO A, TOTARO A, CAZZOLLA M et al.: Juvenile hematochromatosis locus maps to chromosome 1q. Am. J. Hum. Genet. (1999) 64:1398-1393.
    • (1999) Am. J. Hum. Genet. , vol.64 , pp. 1393-1398
    • Roetto, A.1    Totaro, A.2    Cazzolla, M.3
  • 8
    • 0031030733 scopus 로고    scopus 로고
    • The relationship between iron overload, clinical symptoms, and age in 410 patients with genetic hemochromatosis
    • ADAMS PC, DEUGNIER Y, MOIRAND R, BRISSOT P: The relationship between iron overload, clinical symptoms, and age in 410 patients with genetic hemochromatosis. Hepatology (1997) 25:162-166.
    • (1997) Hepatology , vol.25 , pp. 162-166
    • Adams, P.C.1    Deugnier, Y.2    Moirand, R.3    Brissot, P.4
  • 9
    • 0033599057 scopus 로고    scopus 로고
    • Disorders of iron metabolism
    • ANDREWS NC: Disorders of iron metabolism. N. Engl. J. Med (1999) 341:1986-1995.
    • (1999) N. Engl. J. Med. , vol.341 , pp. 1986-1995
    • Andrews, N.C.1
  • 10
    • 0035793856 scopus 로고    scopus 로고
    • An iron-regulated ferric reductase associated with the absorption of dietary iron
    • MCKIE AT, BARROW D, LATUNDE-DADA GO et al.: An iron-regulated ferric reductase associated with the absorption of dietary iron. Science (2001) 291:1755-1759.
    • (2001) Science , vol.291 , pp. 1755-1759
    • Mckie, A.T.1    Barrow, D.2    Latunde-Dada, G.O.3
  • 11
    • 0030755366 scopus 로고    scopus 로고
    • Cloning and characterization of a mammalian proton-coupled metal-ion transported
    • GUNSHIN H, MACKENZIE B, BERGER UV et al.: Cloning and characterization of a mammalian proton-coupled metal-ion transported. Nature (1997) 388:482-488.
    • (1997) Nature , vol.388 , pp. 482-488
    • Gunshin, H.1    Mackenzie, B.2    Berger, U.V.3
  • 12
    • 0033861745 scopus 로고    scopus 로고
    • A novel duodenal iron-regulated transporter, IREG 1, implicated in die basolateral transfer of iron to the circulation
    • MCKIE AT, MARCIANI P, ROLFS A et al.: A novel duodenal iron-regulated transporter, IREG 1, implicated in die basolateral transfer of iron to the circulation. Molec. Cell (2000) 5:299-309.
    • (2000) Molec. Cell , vol.5 , pp. 299-309
    • Mckie, A.T.1    Marciani, P.2    Rolfs, A.3
  • 13
    • 0034733635 scopus 로고    scopus 로고
    • A novel mammalian iron-regulated protein involved in intracellular iron metabolism
    • ABBOUD S, HAILE DJ: A novel mammalian iron-regulated protein involved in intracellular iron metabolism. J. Biol. Chem. (2000) 275:19906-19912.
    • (2000) J. Biol. Chem. , vol.275 , pp. 19906-19912
    • Abboud, S.1    Haile, D.J.2
  • 14
    • 0032909207 scopus 로고    scopus 로고
    • Hephaestin, a ceruloplasmin homologue implicated in intestinal iron transport, is defective in the sla mouse
    • VULPE CD, KUO YM, MURPHY TL et al.: Hephaestin, a ceruloplasmin homologue implicated in intestinal iron transport, is defective in the sla mouse. Nat. Genet. (1999) 21:195-199.
    • (1999) Nat. Genet. , vol.21 , pp. 195-199
    • Vulpe, C.D.1    Kuo, Y.M.2    Murphy, T.L.3
  • 15
  • 16
    • 0028049495 scopus 로고
    • Regulators of iron balance in humans
    • FINCH C: Regulators of iron balance in humans. Blood (1994) 84:1697-1702.
    • (1994) Blood , vol.84 , pp. 1697-1702
    • Finch, C.1
  • 17
    • 0034672236 scopus 로고    scopus 로고
    • Iron homeostasis: New tales from the crypt
    • ROY CN, ENNS CA: Iron homeostasis: new tales from the crypt. Blood (2000) 96:4020-4027.
    • (2000) Blood , vol.96 , pp. 4020-4027
    • Roy, C.N.1    Enns, C.A.2
  • 18
    • 0031002910 scopus 로고    scopus 로고
    • Immunohistochemistry of HLA-H, the protein defective in patients with hereditary hemochromatosis, reveals unique pattern of expression in gastrointestinal tract
    • PARKKILA S, WAHEED A, BRITTON RS et al.: Immunohistochemistry of HLA-H, the protein defective in patients with hereditary hemochromatosis, reveals unique pattern of expression in gastrointestinal tract. Proc. Natl. Acad. Sci. USA (1997) 94:2534-2539.
    • (1997) Proc. Natl. Acad. Sci. USA , vol.94 , pp. 2534-2539
    • Parkkila, S.1    Waheed, A.2    Britton, R.S.3
  • 19
    • 0033574075 scopus 로고    scopus 로고
    • Association of HFE protein with transferrin receptor in crypt enterocytes of human duodenum
    • WAHEED A, PARKKILA S, SAARNIO J et al.: Association of HFE protein with transferrin receptor in crypt enterocytes of human duodenum. Proc. Natl. Acad. Sci. USA (1999) 96:1579-1584.
    • (1999) Proc. Natl. Acad. Sci. USA , vol.96 , pp. 1579-1584
    • Waheed, A.1    Parkkila, S.2    Saarnio, J.3
  • 20
    • 0033605595 scopus 로고    scopus 로고
    • The hereditary hemochromatosis protein, HFE, specifically regulates transferrin-mediated iron uptake in HeLa cells
    • ROY CN, PENNY DM, FEDER JN, ENNS CA: The hereditary hemochromatosis protein, HFE, specifically regulates transferrin-mediated iron uptake in HeLa cells. J. Biol. Chem. (1999) 274:9022-9028.
    • (1999) J. Biol. Chem. , vol.274 , pp. 9022-9028
    • Roy, C.N.1    Penny, D.M.2    Feder, J.N.3    Enns, C.A.4
  • 21
    • 0034610781 scopus 로고    scopus 로고
    • Crystal structure of the hereditary hemochromatosis protein HFE complexed with transferrin receptor
    • BENNETT MJ, LEBRON JA, BJORKMAN PJ: Crystal structure of the hereditary hemochromatosis protein HFE complexed with transferrin receptor. Nature (2000) 403:45-53.
    • (2000) Nature , vol.403 , pp. 45-53
    • Bennett, M.J.1    Lebron, J.A.2    Bjorkman, P.J.3
  • 22
    • 13044317291 scopus 로고    scopus 로고
    • Mechanism of increased iron absorption in murine model of hereditary hemochromatosis: Increased duodenal expression of the iron transported DMT1
    • FLEMING RE, MIGAS MC, ZHOU X et al.: Mechanism of increased iron absorption in murine model of hereditary hemochromatosis: increased duodenal expression of the iron transported DMT1. Proc. Natl. Acad. Sci. USA (1999) 96:3143-3148.
    • (1999) Proc. Natl. Acad. Sci. USA , vol.96 , pp. 3143-3148
    • Fleming, R.E.1    Migas, M.C.2    Zhou, X.3
  • 23
    • 0344085880 scopus 로고    scopus 로고
    • Mechanism of increased iron absorption in murine model of hereditary hemochromatosis: Increased duodenal expression of the iron transported DMT1
    • ZOLLER H, PIETRANGELO A, VOGEL W, WEISS G: Mechanism of increased iron absorption in murine model of hereditary hemochromatosis: increased duodenal expression of the iron transported DMT1. Lancet (1999) 353:2120-2123.
    • (1999) Lancet , vol.353 , pp. 2120-2123
    • Zoller, H.1    Pietrangelo, A.2    Vogel, W.3    Weiss, G.4
  • 24
    • 0029856326 scopus 로고    scopus 로고
    • The control of iron absorption
    • ANDERSON GJ: The control of iron absorption. J. Gastroenterol. Hepatol. (1996) 11:1030-1032.
    • (1996) J. Gastroenterol. Hepatol. , vol.11 , pp. 1030-1032
    • Anderson, G.J.1
  • 26
    • 0025877002 scopus 로고
    • Clinical presentation of hemochromatosis: A changing scene
    • ADAMS PC, KERTESZ AE, VALBERG LS: Clinical presentation of hemochromatosis: A changing scene. Am. J. Med. (1991) 90:445-449.
    • (1991) Am. J. Med. , vol.90 , pp. 445-449
    • Adams, P.C.1    Kertesz, A.E.2    Valberg, L.S.3
  • 27
    • 0033039405 scopus 로고    scopus 로고
    • A survey of 2851 patients with hemochromatosis: Symptoms and response to treatment
    • MCDONNELL SM, PRESTON BL, JEWELL SA et al.: A survey of 2851 patients with hemochromatosis: Symptoms and response to treatment. Am. J. Med. (1999) 106:619-624.
    • (1999) Am. J. Med. , vol.106 , pp. 619-624
    • Mcdonnell, S.M.1    Preston, B.L.2    Jewell, S.A.3
  • 28
    • 0030957346 scopus 로고    scopus 로고
    • Hereditary hemochromatosis: Presentation and diagnosis in the 1990s
    • BACON BR, SADIQ SA: Hereditary hemochromatosis: presentation and diagnosis in the 1990s. Am. J. Gast. (1997) 92:784-789.
    • (1997) Am. J. Gast. , vol.92 , pp. 784-789
    • Bacon, B.R.1    Sadiq, S.A.2
  • 29
    • 0035038147 scopus 로고    scopus 로고
    • Diagnosis and management of hemochromatosis
    • TAVILL AS: Diagnosis and management of hemochromatosis. Hepatology (2001) 33:1321-1328.
    • (2001) Hepatology , vol.33 , pp. 1321-1328
    • Tavill, A.S.1
  • 30
    • 0021215227 scopus 로고
    • Diagnosis of hemochromatosis in young subjects: Predictive accuracy of biochemical screening tests
    • BASSETT ML, HALLIDAY JW, FERRIS RA, POWELL LW: Diagnosis of hemochromatosis in young subjects: Predictive accuracy of biochemical screening tests. Gastroenterology (1984) 87:628-633.
    • (1984) Gastroenterology , vol.87 , pp. 628-633
    • Bassett, M.L.1    Halliday, J.W.2    Ferris, R.A.3    Powell, L.W.4
  • 31
    • 0037326566 scopus 로고    scopus 로고
    • Hemochromatosis and iron overload study research investigators. Hemochromatosis and iron overload screening (HEIRS) study design for an evaluation of 100,000 primary care-based adults
    • MCCLAREN CE, BARTON JC, ADAMS PC et al.: Hemochromatosis and iron overload study research investigators. Hemochromatosis and iron overload screening (HEIRS) study design for an evaluation of 100,000 primary care-based adults. Am. J. Med. Sci. (2003) 325:53-62.
    • (2003) Am. J. Med. Sci. , vol.325 , pp. 53-62
    • Mcclaren, C.E.1    Barton, J.C.2    Adams, P.C.3
  • 32
    • 0027987205 scopus 로고
    • Prognostic factors for hepatocellular carcinoma in genetic hemochromatosis
    • FARGION S, FRACANZANI AL, PIPERNO A et al.: Prognostic factors for hepatocellular carcinoma in genetic hemochromatosis. Hepatology (1994) 20:1426-1431.
    • (1994) Hepatology , vol.20 , pp. 1426-1431
    • Fargion, S.1    Fracanzani, A.L.2    Piperno, A.3
  • 33
    • 0029620882 scopus 로고
    • Diabetes and hemochromatosis: Current concepts, management and prevention
    • YAOUANQ JM: Diabetes and hemochromatosis: current concepts, management and prevention. Diabetes Metab. (1995) 21:319-329.
    • (1995) Diabetes Metab. , vol.21 , pp. 319-329
    • Yaouanq, J.M.1
  • 35
  • 36
    • 0035096370 scopus 로고    scopus 로고
    • Arthropathy of genetic hemochromatosis: A major and distinctive manifestation of the disease
    • INES IS, DA SILAVA JA, MALCATA AB, PORTO AL: Arthropathy of genetic hemochromatosis: a major and distinctive manifestation of the disease. Clin. Exp. Rheum. (2001) 19:98-102.
    • (2001) Clin. Exp. Rheum. , vol.19 , pp. 98-102
    • Ines, I.S.1    Da Silava, J.A.2    Malcata, A.B.3    Porto, A.L.4
  • 38
    • 0023082840 scopus 로고
    • Expression of iron-regulated proteins in Yersinia species and their relation to virulence
    • CARNIEL E, MAZIGH D, MOLLARET HH: Expression of iron-regulated proteins in Yersinia species and their relation to virulence. Infect. Immun. (1987) 55:277-280.
    • (1987) Infect. Immun. , vol.55 , pp. 277-280
    • Carniel, E.1    Mazigh, D.2    Mollaret, H.H.3
  • 40
    • 85047699496 scopus 로고
    • Mucormycosis
    • SUGAR AM: Mucormycosis. Clin. Infect. Dis. (1992) 14(Suppl. 1):S126-S129.
    • (1992) Clin. Infect. Dis. , vol.14 , Issue.SUPPL. 1
    • Sugar, A.M.1
  • 41
    • 7444249085 scopus 로고
    • The biochemistry of desferrioxamine and its relation to iron metabolism
    • KEBERLE H: The biochemistry of desferrioxamine and its relation to iron metabolism. Ann. NY Acad. Sci. (1964) 119:758-765.
    • (1964) Ann. NY Acad. Sci. , vol.119 , pp. 758-765
    • Keberle, H.1
  • 42
    • 0027080934 scopus 로고
    • Cellular pool of transient ferric iron, chelatable by deferoxamine and distinct from ferritin, that is involved in oxidase cell injury
    • ROTHMAN RJ, SERRONI A, FARBER JL: Cellular pool of transient ferric iron, chelatable by deferoxamine and distinct from ferritin, that is involved in oxidase cell injury. Mol. Pharmacol. (1992) 42:703-710.
    • (1992) Mol. Pharmacol. , vol.42 , pp. 703-710
    • Rothman, R.J.1    Serroni, A.2    Farber, J.L.3
  • 43
    • 0029978555 scopus 로고    scopus 로고
    • Kinetics of removal and reappearance pf non-transferrin-bound plasma iron with deferoxamine therapy
    • PORTER JB, ABEYSINGHE RD, MARSHALL L, HIDER RC, SINGH S: Kinetics of removal and reappearance pf non-transferrin-bound plasma iron with deferoxamine therapy. Blood (1996) 88:705-713.
    • (1996) Blood , vol.88 , pp. 705-713
    • Porter, J.B.1    Abeysinghe, R.D.2    Marshall, L.3    Hider, R.C.4    Singh, S.5
  • 44
    • 0018777993 scopus 로고
    • Studies in desferrioxamine and ferrioxamine metabolism in normal and iron-loaded subjects
    • SUMMERS MF, JACOBS A, TUDWAY D, PERERA P, RICKETTS C: Studies in desferrioxamine and ferrioxamine metabolism in normal and iron-loaded subjects. Br. J. Haematol. (1979) 42:547-555.
    • (1979) Br. J. Haematol. , vol.42 , pp. 547-555
    • Summers, M.F.1    Jacobs, A.2    Tudway, D.3    Perera, P.4    Ricketts, C.5
  • 45
    • 0034651775 scopus 로고    scopus 로고
    • Long-term outcome of continuous 24-hour deferoxamine infusion via inswelling intravenous catheters in high-risk beta-thalassemia
    • DAVIS BA, PORTER JB: Long-term outcome of continuous 24-hour deferoxamine infusion via inswelling intravenous catheters in high-risk beta-thalassemia. Blood (2000) 95:1229-1236.
    • (2000) Blood , vol.95 , pp. 1229-1236
    • Davis, B.A.1    Porter, J.B.2
  • 46
    • 0031001278 scopus 로고    scopus 로고
    • Iron-chelating therapy and the treatment of thalassemia
    • OLIVIERI NF, BRITTENHAM GM: Iron-chelating therapy and the treatment of thalassemia. Blood (1997) 89:739-761.
    • (1997) Blood , vol.89 , pp. 739-761
    • Olivieri, N.F.1    Brittenham, G.M.2
  • 47
    • 0028059813 scopus 로고
    • Efficacy of Deferoxamine in preventing complications of iron overload in patients with thalassemia major
    • BRITTENHAM GM, GRIFFITH PM, NIENHUIS AW et al.: Efficacy of Deferoxamine in preventing complications of iron overload in patients with thalassemia major. N. Engl. J. Med. (1994) 331:567-573.
    • (1994) N. Engl. J. Med. , vol.331 , pp. 567-573
    • Brittenham, G.M.1    Griffith, P.M.2    Nienhuis, A.W.3
  • 48
    • 0022628511 scopus 로고
    • Visual and auditory neurotoxicity in patients receiving subcutaneious Deferoxamine infusions
    • OLIVIERI NF, BUNCIC RJ, CHEW E et al.: Visual and auditory neurotoxicity in patients receiving subcutaneious Deferoxamine infusions. N. Engl. J. Med. (1986) 314:869-873.
    • (1986) N. Engl. J. Med. , vol.314 , pp. 869-873
    • Olivieri, N.F.1    Buncic, R.J.2    Chew, E.3
  • 50
    • 0032514421 scopus 로고    scopus 로고
    • Iron chelation therapy with oral deferiprone-toxicity or lack of efficacy?
    • KOWDLEY KV, KAPLAN MM: Iron chelation therapy with oral deferiprone-toxicity or lack of efficacy? N. Engl. J. Med. (1998) 339:468-469.
    • (1998) N. Engl. J. Med. , vol.339 , pp. 468-469
    • Kowdley, K.V.1    Kaplan, M.M.2
  • 51
    • 0025344815 scopus 로고
    • Pulmonary syndrome in patients with thalassemia major receiving intravenous Deferoxamine infusions
    • FREEDMAN MH, GRISARU D, OLIVIERI NF, MACLUSKY I, THORNER PS: Pulmonary syndrome in patients with thalassemia major receiving intravenous Deferoxamine infusions. Am. J. Dis. Child. (1990) 144:565-569.
    • (1990) Am. J. Dis. Child. , vol.144 , pp. 565-569
    • Freedman, M.H.1    Grisaru, D.2    Olivieri, N.F.3    Maclusky, I.4    Thorner, P.S.5
  • 52
    • 0024310392 scopus 로고
    • Desferrioxamine ototoxicity: Evaluation of risk factos in thalassaemic patients and guidelines for safe dosage
    • PORTER JB, JASWON MS, HUEHNS ER, EAST CA, HAZELL JW: Desferrioxamine ototoxicity: Evaluation of risk factos in thalassaemic patients and guidelines for safe dosage. Br. J. Haematol. (1989) 73:403-409.
    • (1989) Br. J. Haematol. , vol.73 , pp. 403-409
    • Porter, J.B.1    Jaswon, M.S.2    Huehns, E.R.3    East, C.A.4    Hazell, J.W.5
  • 54
    • 0030830275 scopus 로고    scopus 로고
    • Evaluation of a new method of administration of the iron chelating agent deferoxamine
    • BORGNA-PIGNATTI C, COHEN A: Evaluation of a new method of administration of the iron chelating agent deferoxamine. J. Pediatr. (1997) 130:86-88.
    • (1997) J. Pediatr. , vol.130 , pp. 86-88
    • Borgna-Pignatti, C.1    Cohen, A.2
  • 55
    • 0029894858 scopus 로고    scopus 로고
    • A novel delivery system for continuous desferrioxamine infusion in transfusional iron overload
    • ARAUJO A, KOSARYAN M, MACDOWELL A et al.: A novel delivery system for continuous desferrioxamine infusion in transfusional iron overload Br. J. Haematol. (1996) 93:835-837.
    • (1996) Br. J. Haematol. , vol.93 , pp. 835-837
    • Araujo, A.1    Kosaryan, M.2    Macdowell, A.3
  • 56
    • 0032887235 scopus 로고    scopus 로고
    • Deferiprone: A review of its clinical potential in iron overload in beta-thalassaemia major and other transfusion-dependent diseases
    • BARMAN BALFOUR JA, FOSTER RH: Deferiprone: a review of its clinical potential in iron overload in beta-thalassaemia major and other transfusion-dependent diseases. Drugs (1999) 58:553-578.
    • (1999) Drugs , vol.58 , pp. 553-578
    • Barman Balfour, J.A.1    Foster, R.H.2
  • 57
    • 0026680591 scopus 로고
    • Reduction of tissue iron stores and normalization of serum ferritin during treatment with the oral iron chelator deferiprone in thalassemia intermedia
    • OLIVIERI NF, KOREN G, MATSUI D et al.: Reduction of tissue iron stores and normalization of serum ferritin during treatment with the oral iron chelator deferiprone in thalassemia intermedia. Blood (1992) 79:2741-2748.
    • (1992) Blood , vol.79 , pp. 2741-2748
    • Olivieri, N.F.1    Koren, G.2    Matsui, D.3
  • 58
    • 0028898064 scopus 로고
    • Iron-chelation therapy with oral deferiprone in patients with thalassemia major
    • OLIVIERI NF, BRITTENHAM GM, MATSUI D et al.: Iron-chelation therapy with oral deferiprone in patients with thalassemia major. N. Engl. J. Med. (1995) 332:918-922.
    • (1995) N. Engl. J. Med. , vol.332 , pp. 918-922
    • Olivieri, N.F.1    Brittenham, G.M.2    Matsui, D.3
  • 59
    • 0032514558 scopus 로고    scopus 로고
    • Long-term safety and effectiveness of iron-chelation therapy with deferiprone for thalassemia major
    • OLIVIERI NF, BRITTENHAM GM, MCLAREN CE et al.: Long-term safety and effectiveness of iron-chelation therapy with deferiprone for thalassemia major. N. Engl. J. Med. (1998) 339:417-423.
    • (1998) N. Engl. J. Med. , vol.339 , pp. 417-423
    • Olivieri, N.F.1    Brittenham, G.M.2    Mclaren, C.E.3
  • 60
    • 0031985039 scopus 로고    scopus 로고
    • Long-term trial of deferiprone in 51 transfusion-dependent iron overloaded patients
    • HOFFBRAND AV, AL-REFAIE F, DAVIS B et al.: Long-term trial of deferiprone in 51 transfusion-dependent iron overloaded patients. Blood (1998) 91:295-300.
    • (1998) Blood , vol.91 , pp. 295-300
    • Hoffbrand, A.V.1    Al-Refaie, F.2    Davis, B.3
  • 61
    • 0034783438 scopus 로고    scopus 로고
    • Chelation therapy in beta-thalassemia: An optimistic update
    • GIARDINA PJ, GRADY RW: Chelation therapy in beta-thalassemia: an optimistic update Semin Hema (2001) 38:360-366.
    • (2001) Semin. Hema. , vol.38 , pp. 360-366
    • Giardina, P.J.1    Grady, R.W.2
  • 63
    • 0348101415 scopus 로고    scopus 로고
    • Deferiprone: A review of Indian study over 10 years
    • Proceedings of the 10th International Conference on Oral Chelators in the Treatment of Thalassemia and Other Diseases and Biomed Meeting, Limassol, Cyprus, March 22-26
    • GOGTEY J, AGARWAL MB, RAJADHYAKSHA G, MUNOT S: Deferiprone: A review of Indian study over 10 years, Proceedings of the 10th International Conference on Oral Chelators in the Treatment of Thalassemia and Other Diseases and Biomed Meeting, Limassol, Cyprus, March 22-26 (2000).
    • (2000)
    • Gogtey, J.1    Agarwal, M.B.2    Rajadhyaksha, G.3    Munot, S.4
  • 64
    • 0346840731 scopus 로고    scopus 로고
    • Liver iron and fibrosis during long-term treatment with deferiprone in Swiss thalassemia patients, 1989-2000
    • Proceedings of the 10th International Conference on Oral Chelators in the Treatment of Thalassemia and Other Diseases and Biomed Meeting, Limassol, Cyprus, March 22-26
    • TONDURY P, NIELSEN P, FISCHER R, HIRT A: Liver iron and fibrosis during long-term treatment with deferiprone in Swiss thalassemia patients, 1989-2000, Proceedings of the 10th International Conference on Oral Chelators in the Treatment of Thalassemia and Other Diseases and Biomed Meeting, Limassol, Cyprus, March 22-26 (2000).
    • (2000)
    • Tondury, P.1    Nielsen, P.2    Fischer, R.3    Hirt, A.4
  • 65
    • 0034928342 scopus 로고    scopus 로고
    • Exploring the 'iron shuttle' hypothesis in chelation therapy: Effects of combined deferoxamine and deferiprone treatment in hypertransfused rats with labeled iron stores and in iron-loaded rat heart cells in culture
    • LINK G, KONJIN AM, BREUER W, CABANTCHIK ZI, HERSHKO C: Exploring the 'iron shuttle' hypothesis in chelation therapy: effects of combined deferoxamine and deferiprone treatment in hypertransfused rats with labeled iron stores and in iron-loaded rat heart cells in culture. J. Lab. Clin. Med. (2001) 138:130-138.
    • (2001) J. Lab. Clin. Med. , vol.138 , pp. 130-138
    • Link, G.1    Konjin, A.M.2    Breuer, W.3    Cabantchik, Z.I.4    Hershko, C.5
  • 66
    • 0031784438 scopus 로고    scopus 로고
    • Combined therapy with deferiprone and desferrioxamine
    • WONKE B, WRIGHT C, HOFFBRAND AV: Combined therapy with deferiprone and desferrioxamine. Br. J. Haematol. (1998) 103:361-364.
    • (1998) Br. J. Haematol. , vol.103 , pp. 361-364
    • Wonke, B.1    Wright, C.2    Hoffbrand, A.V.3
  • 67
    • 0032820582 scopus 로고    scopus 로고
    • Sequential use of deferiprone and desferrioxamine in primary school children with thalassemia major in Turkey
    • AYDINOK Y, NISLI G, KAVAKLI K, COKER C, KANTAR M, CETINGUL N: Sequential use of deferiprone and desferrioxamine in primary school children with thalassemia major in Turkey. Acta Hema (1999) 102:17-21.
    • (1999) Acta Hema. , vol.102 , pp. 17-21
    • Aydinok, Y.1    Nisli, G.2    Kavakli, K.3    Coker, C.4    Kantar, M.5    Cetingul, N.6
  • 68
    • 0035865702 scopus 로고    scopus 로고
    • ICL670A: A new synthetic oral chelator; evaluation in hypertransfused rats with selective radioiron probes of hepatocellular and reticuloendothelial iron stores and in iron-loaded rat heart cells in culture
    • HERSHKO C, KONJIN AM, NICK HP, BREUER W, CABANTCHIK ZI, LINK G: ICL670A: a new synthetic oral chelator; evaluation in hypertransfused rats with selective radioiron probes of hepatocellular and reticuloendothelial iron stores and in iron-loaded rat heart cells in culture. Blood (2001) 97:1115-1122.
    • (2001) Blood , vol.97 , pp. 1115-1122
    • Hershko, C.1    Konjin, A.M.2    Nick, H.P.3    Breuer, W.4    Cabantchik, Z.I.5    Link, G.6
  • 69
    • 0037089226 scopus 로고    scopus 로고
    • HBED ligand: Preclinical studies of a potential alternative to deferoxamine for treatment of chronic imn overload and acute iron poisoning
    • BERGERON RJ, WIEGAND J, BRITTENHAM GM: HBED ligand: preclinical studies of a potential alternative to deferoxamine for treatment of chronic imn overload and acute iron poisoning. Blood (2002) 99:3019-3026.
    • (2002) Blood , vol.99 , pp. 3019-3026
    • Bergeron, R.J.1    Wiegand, J.2    Brittenham, G.M.3
  • 70
    • 84963072124 scopus 로고
    • Progressive lenticular degeneration: A familial nervous disease associated with cirrhosis of the liver
    • WILSON SAK: Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver. Brain (1912) 34:295-509.
    • (1912) Brain , vol.34 , pp. 295-509
    • Wilson, S.A.K.1
  • 71
    • 0344375104 scopus 로고
    • Familial cirrhosis of the liver: Four cases of acute fatal cirrhosis of the liver in the same family, the patients being respectively nine, ten, fourteen and fourteen years of age; suggested relationship to Wilson's progressive degeneration of the lenticular nucleus
    • BRAMWELL B: Familial cirrhosis of the liver: four cases of acute fatal cirrhosis of the liver in the same family, the patients being respectively nine, ten, fourteen and fourteen years of age; suggested relationship to Wilson's progressive degeneration of the lenticular nucleus. Edin. Med. J. (1916) 17:90-99.
    • (1916) Edin. Med. J. , vol.17 , pp. 90-99
    • Bramwell, B.1
  • 72
    • 0001435653 scopus 로고
    • The copper and iron content of brain and liver in the normal and in hepato-lenticular degeneration
    • CUMINGS JN: The copper and iron content of brain and liver in the normal and in hepato-lenticular degeneration. Brain (1948) 71:410-415.
    • (1948) Brain , vol.71 , pp. 410-415
    • Cumings, J.N.1
  • 74
    • 0027364961 scopus 로고
    • The Wilson disease gene is a copper transporting ATPase with homology to the Menkes disease gene
    • TANZI RE, PETRUKHIN KE, CHERNOV I et al.: The Wilson disease gene is a copper transporting ATPase with homology to the Menkes disease gene. Nat. Genet. (1993):344.
    • (1993) Nat. Genet. , pp. 344
    • Tanzi, R.E.1    Petrukhin, K.E.2    Chernov, I.3
  • 75
    • 0027452091 scopus 로고
    • The Wilson disease gene is a putative copper transporting P-type ATPase similar to the Menkes gene
    • BULL PC, THOMAS GR, ROMMENS JM, FORBES JR, COX DW: The Wilson disease gene is a putative copper transporting P-type ATPase similar to the Menkes gene. Nat. Genet. (1993):327.
    • (1993) Nat. Genet. , pp. 327
    • Bull, P.C.1    Thomas, G.R.2    Rommens, J.M.3    Forbes, J.R.4    Cox, D.W.5
  • 76
    • 0030803730 scopus 로고    scopus 로고
    • Biochemical characterization of the Wilson disease protein and functional expression in he yeast Saccharomyces cerevisiae
    • HUNG IH, SUZUKI M, YAMAGUCHI Y, YUAN DS, KLAUSNER RD, GITLIN JD: Biochemical characterization of the Wilson disease protein and functional expression in he yeast Saccharomyces cerevisiae. J. Biol. Chem. (1997):21461.
    • (1997) J. Biol. Chem. , pp. 21461
    • Hung, I.H.1    Suzuki, M.2    Yamaguchi, Y.3    Yuan, D.S.4    Klausner, R.D.5    Gitlin, J.D.6
  • 78
    • 0035128658 scopus 로고    scopus 로고
    • Biological functions of ceruloplasmin and their deficiency caused by mutation in genes regulating copper and iron metabolism
    • MZHEL'SKAYA TI: Biological functions of ceruloplasmin and their deficiency caused by mutation in genes regulating copper and iron metabolism. Bull. Exp. Biol. Med. (2000) 130:719-727.
    • (2000) Bull. Exp. Biol. Med. , vol.130 , pp. 719-727
    • Mzhel'skaya, T.I.1
  • 80
    • 0348101414 scopus 로고    scopus 로고
    • Wilson disease
    • Feldman M, Friedman LS, Sleisenger MH, (Eds), Saunders, Philadelphia, USA
    • COX DW, ROBERTS EA: Wilson disease. In: Gastrointestinal and liver disease. Vol. 2. Feldman M, Friedman LS, Sleisenger MH, (Eds), Saunders, Philadelphia, USA (2002):1269-1277.
    • (2002) Gastrointestinal and Liver Disease , vol.2 , pp. 1269-1277
    • Cox, D.W.1    Roberts, E.A.2
  • 83
    • 0027483379 scopus 로고
    • Chelation treatment of neurological Wilson's disease
    • WALSHE JM, YEALLAND M: Chelation treatment of neurological Wilson's disease. QJ Med. (1993) 86:197-204.
    • (1993) QJ Med. , vol.86 , pp. 197-204
    • Walshe, J.M.1    Yealland, M.2
  • 84
    • 0036204701 scopus 로고    scopus 로고
    • Diagnosis and treatment of Wilson's disease
    • SCHILSKY M: Diagnosis and treatment of Wilson's disease. Pediatr. Transplant. (2002) 6:15-19.
    • (2002) Pediatr. Transplant. , vol.6 , pp. 15-19
    • Schilsky, M.1
  • 85
    • 0034845675 scopus 로고    scopus 로고
    • Zinc acetate for the treatment of Wilson's disease
    • BREWER GJ: Zinc acetate for the treatment of Wilson's disease. Expert Opin. Pharmacother. (2001) 2:1473-1477.
    • (2001) Expert Opin. Pharmacother. , vol.2 , pp. 1473-1477
    • Brewer, G.J.1
  • 86
    • 77049283245 scopus 로고
    • Penicillamine, a new oral therapy for Wilson's disease
    • WALSHE JM: Penicillamine, a new oral therapy for Wilson's disease. Am. J. Med. (1956) 21:487-495.
    • (1956) Am. J. Med. , vol.21 , pp. 487-495
    • Walshe, J.M.1
  • 87
    • 0346840730 scopus 로고    scopus 로고
    • PHYSICIANS' DESK REFERENCE: Montvale: Medical Economics Company, Inc
    • PHYSICIANS' DESK REFERENCE: Vol. 1. Montvale: Medical Economics Company, Inc., (2002):3635.
    • (2002) , vol.1 , pp. 3635
  • 88
    • 84920245415 scopus 로고
    • Congential connective tissue defect probably due to D-penicillamine treatment in pregnancy
    • MJOLNEROD OK, DOMMERUD SA, RASMUSSEN K, GJERULDSEN ST: Congential connective tissue defect probably due to D-penicillamine treatment in pregnancy. Lancet (1971) 1:673-675.
    • (1971) Lancet , vol.1 , pp. 673-675
    • Mjolnerod, O.K.1    Dommerud, S.A.2    Rasmussen, K.3    Gjeruldsen, S.T.4
  • 89
    • 0017617513 scopus 로고
    • Neonatal abnormalities associated with d-penicillamine treatment during pregnancy
    • SOLOMON L, ABRAMS G, DINNER M, BERMAN L: Neonatal abnormalities associated with d-penicillamine treatment during pregnancy. N. Engl. J. Med. (1977) 296:54-55.
    • (1977) N. Engl. J. Med. , vol.296 , pp. 54-55
    • Solomon, L.1    Abrams, G.2    Dinner, M.3    Berman, L.4
  • 90
    • 0033491315 scopus 로고    scopus 로고
    • Steroids used to desensitize penicillamine allergy in Wilson disease
    • HSU H, HUANG F, NI Y, CHANG M: Steroids used to desensitize penicillamine allergy in Wilson disease. Acta Paediatr. (1999) 40:448-450.
    • (1999) Acta Paediatr. , vol.40 , pp. 448-450
    • Hsu, H.1    Huang, F.2    Ni, Y.3    Chang, M.4
  • 91
    • 0035353761 scopus 로고    scopus 로고
    • Penicillamine-induced lethal status dystonicus in a patient with Wilson's disease
    • SVETEL M, STERNI N, PEJOVI S, KOSTI VS: Penicillamine-induced lethal status dystonicus in a patient with Wilson's disease. Mov. Disord. (2001) 16:568-569.
    • (2001) Mov. Disord. , vol.16 , pp. 568-569
    • Svetel, M.1    Sterni, N.2    Pejovi, S.3    Kosti, V.S.4
  • 92
    • 0029877302 scopus 로고    scopus 로고
    • Wilson's disease: Resolution of MRI lesions following long-term oral zinc therapy
    • HUANG CC, CHU NS: Wilson's disease: resolution of MRI lesions following long-term oral zinc therapy. Acta Neurol. Scand. (1996) 93:215-218.
    • (1996) Acta Neurol. Scand. , vol.93 , pp. 215-218
    • Huang, C.C.1    Chu, N.S.2
  • 93
    • 0033455569 scopus 로고    scopus 로고
    • Pencillamine should not be used as initial therapy in Wilson's disease
    • BREWER GJ: Pencillamine should not be used as initial therapy in Wilson's disease. Mov. Disord. (1999) 14:551-554.
    • (1999) Mov. Disord. , vol.14 , pp. 551-554
    • Brewer, G.J.1
  • 94
    • 0342529016 scopus 로고
    • Scheinberg IH, Walshe JM (Eds), Manchester University Press, London, UK
    • GIBBS KR, WALSHE LM: In: Orphan diseases and orphan drugs. Scheinberg IH, Walshe JM (Eds), Manchester University Press, London, UK (1986):33-42.
    • (1986) Orphan Diseases and Orphan Drugs , pp. 33-42
    • Gibbs, K.R.1    Walshe, L.M.2
  • 95
    • 0030791354 scopus 로고    scopus 로고
    • Metabolism of administered triethylene tetramine dihydrochloride in humans
    • KODAMA H, MURATA Y, IITSUKA T, ABE T: Metabolism of administered triethylene tetramine dihydrochloride in humans. Life Sci. (1997) 61:899-907.
    • (1997) Life Sci. , vol.61 , pp. 899-907
    • Kodama, H.1    Murata, Y.2    Iitsuka, T.3    Abe, T.4
  • 96
    • 0032508265 scopus 로고    scopus 로고
    • Effects of treatment of Wison's disease on natural history of haemochromatosis
    • WALSHE JM, COX DW: Effects of treatment of Wison's disease on natural history of haemochromatosis. Lancet (1998) 352:112-113.
    • (1998) Lancet , vol.352 , pp. 112-113
    • Walshe, J.M.1    Cox, D.W.2
  • 98
    • 0003926908 scopus 로고
    • Teratogenic effect of copper deficiency and excess
    • Sorenson JRJ (Ed.), Humana Press, Clifton NJ, USA
    • KEEN CL, LONNERDAL B, HURLEY LS: Teratogenic effect of copper deficiency and excess. In: Inflammatory diseases and Copper. Sorenson JRJ (Ed.), Humana Press, Clifton NJ, USA (1982):109-121.
    • (1982) Inflammatory Diseases and Copper , pp. 109-121
    • Keen, C.L.1    Lonnerdal, B.2    Hurley, L.S.3
  • 100
    • 0348101416 scopus 로고
    • De hepato-cerebrale degeneratic (met een onderzoek van de zinkstofwisseling). Arnhem, Van der Wiel
    • SCHOUWINK G: De hepato-cerebrale degeneratic (met een onderzoek van de zinkstofwisseling). Arnhem, Van der Wiel (1961).
    • (1961)
    • Schouwink, G.1
  • 101
    • 0020550863 scopus 로고
    • Three years of continuous zinc therapy in four patients with Wilson's disease
    • HOOGENRADD TU, VAN DEN HAMER CJA: Three years of continuous zinc therapy in four patients with Wilson's disease. Acta Neurol. Scand. (1983) 67:356-264.
    • (1983) Acta Neurol. Scand. , vol.67 , pp. 264-356
    • Hoogenradd, T.U.1    Van Den Hamer, C.J.A.2
  • 103
    • 0004948293 scopus 로고
    • Teratologic evaluation of FDA 71-49 (zinc sulfate)
    • FOOD AND DRUG ADMINISTRATION: Food and Drug Administration, United States Department of Commerce Publications PD-221 805
    • FOOD AND DRUG ADMINISTRATION: Teratologic evaluation of FDA 71-49 (zinc sulfate). Food and Drug Administration, United States Department of Commerce Publications PD-221 805 (1973).
    • (1973)
  • 105
    • 0029957173 scopus 로고    scopus 로고
    • Regression of Kayser-Fleischer rings during oral zinc therapy: Correlation with systemic manifestations of Wilson's disease
    • ESMAELI B, BURNSTINE MA, MARTONYI CL, SUGAR A, JOHNSON V, BREWER GJ: Regression of Kayser-Fleischer rings during oral zinc therapy: correlation with systemic manifestations of Wilson's disease. Cornea (1996) 15:582-588.
    • (1996) Cornea , vol.15 , pp. 582-588
    • Esmaeli, B.1    Burnstine, M.A.2    Martonyi, C.L.3    Sugar, A.4    Johnson, V.5    Brewer, G.J.6
  • 107
    • 0032771207 scopus 로고    scopus 로고
    • Comparative mechanism and toxicity of tetre- and dithiomolybdates in the removal of copper
    • OGRA Y, KOMADA Y, SUZUKI KT: Comparative mechanism and toxicity of tetre- and dithiomolybdates in the removal of copper. J. Inorg. Biochem. (1999):199-204.
    • (1999) J. Inorg. Biochem. , pp. 199-204
    • Ogra, Y.1    Komada, Y.2    Suzuki, K.T.3
  • 108
    • 0003029225 scopus 로고
    • 4) as an 'anti-copper' agent in man
    • Scheinberg IH, Walshe JM (Eds), Manchester University Press, Manchester, UK
    • 4) as an 'anti-copper' agent in man. In: Orphan diseases and orphan drugs (Vol. 1) Scheinberg IH, Walshe JM (Eds), Manchester University Press, Manchester, UK (1986):68-75.
    • (1986) Orphan Diseases and Orphan Drugs , vol.1 , pp. 68-75
    • Walshe, J.M.1
  • 109
    • 0029821201 scopus 로고    scopus 로고
    • Treatment of Wilson disease with ammonium tetrathiomolybdate: II. Initial therapy in 33 neurologically affected patients and follow-up with zinc therapy [original contribution]
    • BREWER GJ, JOHNSON V, DICK RD, KLUIN KJ, FINK JK, BRUNBERG JA: Treatment of Wilson disease with ammonium tetrathiomolybdate: II. initial therapy in 33 neurologically affected patients and follow-up with zinc therapy [original contribution]. Arch. Neurol. (1996) 53:1017-1025.
    • (1996) Arch. Neurol. , vol.53 , pp. 1017-1025
    • Brewer, G.J.1    Johnson, V.2    Dick, R.D.3    Kluin, K.J.4    Fink, J.K.5    Brunberg, J.A.6
  • 110
    • 0031960993 scopus 로고    scopus 로고
    • Cytopenias secondary to copper depletion complicating ammonium tetrathiomolybdate therapy for Wilson's disease
    • KARUNAJEEWA H, WALL A, METZ J, GRIGG A: Cytopenias secondary to copper depletion complicating ammonium tetrathiomolybdate therapy for Wilson's disease. Aust. NZ J. Med. (1998) 28:215-216.
    • (1998) Aust. NZ J. Med. , vol.28 , pp. 215-216
    • Karunajeewa, H.1    Wall, A.2    Metz, J.3    Grigg, A.4
  • 111
    • 77957187189 scopus 로고
    • The effect of BAL in hepato-lenticular degeneration
    • CUMINGS JN: The effect of BAL in hepato-lenticular degeneration. Brain (1951) 74:10-22.
    • (1951) Brain , vol.74 , pp. 10-22
    • Cumings, J.N.1
  • 112
    • 78651036626 scopus 로고
    • The effect of BAL (2,3-dimercaptopropanol) on hepatolenticular degeneration (Wilson's disease)
    • DENNY BROWN D, PORTER H: The effect of BAL (2,3-dimercaptopropanol) on hepatolenticular degeneration (Wilson's disease). N. Engl. J Med. (1951) 245:917-925.
    • (1951) N. Engl. J Med. , vol.245 , pp. 917-925
    • Denny Brown, D.1    Porter, H.2
  • 114
    • 9844268500 scopus 로고    scopus 로고
    • Clinical curative effects of dimercaptusuccinic acid on hepatolenticular degeneration and the impact of DMSA on biliary trace elements
    • MINGSHAN R, RENMIN Y: Clinical curative effects of dimercaptusuccinic acid on hepatolenticular degeneration and the impact of DMSA on biliary trace elements. Chin. Med. J. (Engl) (1997) 110:694-697.
    • (1997) Chin. Med. J. (Engl) , vol.110 , pp. 694-697
    • Mingshan, R.1    Renmin, Y.2


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.