Systematic review: Clinical efficacy of chelator agents and zinc in the initial treatment of Wilson disease

Alimentary Pharmacology and Therapeutics

Volumn 29, Issue 9, 2009, Pages 947-958

  Wiggelinkhuizen, M ^a   Tilanus, M E C ^a   Bollen, C W ^a   Houwen, R H J ^a  

^a UNIVERSITY MEDICAL CENTER UTRECHT   (Netherlands)

Author keywords

[No Author keywords available]

Indexed keywords

PENICILLAMINE; TETRATHIOMOLYBDIC ACID; TRIENTINE; ZINC;

ARTICLE; CLINICAL FEATURE; CLINICAL TRIAL; DRUG EFFICACY; DRUG TOLERANCE; DRUG WITHDRAWAL; GASTROINTESTINAL SYMPTOM; HUMAN; MONOTHERAPY; PRIORITY JOURNAL; SYSTEMATIC REVIEW; UNSPECIFIED SIDE EFFECT; WILSON DISEASE;

ADOLESCENT; ADULT; CHELATING AGENTS; CHILD; CHILD, PRESCHOOL; FEMALE; HEPATOLENTICULAR DEGENERATION; HUMANS; INFANT; MALE; MIDDLE AGED; RANDOMIZED CONTROLLED TRIALS AS TOPIC; TRACE ELEMENTS; TREATMENT OUTCOME; YOUNG ADULT; ZINC;

EID: 63849343881     PISSN: 02692813     EISSN: 13652036     Source Type: Journal    
DOI: 10.1111/j.1365-2036.2009.03959.x     Document Type: Article

References (75)

1. Frydman M. Genetic aspects of Wilson's disease. J Gastroenterol Hepatol 1990 5 : 483 90.
2. Scheinberg IH, Sternlieb I. Wilson's disease. In : Smith LH Jr., ed. Major Problems in Internal Medicine. XXIII. Philadelphia, PA : WB Saunders Co., 1984. 126 55.
3. Brewer GJ, Yuzbasiyan-Gurkan V. Wilson disease. Medicine 1992 71 : 139 64.
4. Bearn AG. A genetical analysis of thirty families with Wilson's disease (hepatolenticular degeneration). Ann Hum Genet 1960 24 : 33 43.
5. Giagheddu A, Demelia L, Puggioni G, et al. Epidemiologic study of hepatolenticular degeneration (Wilson's disease) in Sardinia (1902-1983). Acta Neurol Scand 1985 72 : 43 55.
6. 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 5 : 327 37.
7. Sternlieb I, Steinberg IH. Wilson's disease. In : Wright R, ed. Liver and Biliary Disease. London : Bailliere Tinddall, 1985. 949 61.
8. Schoen RE, Sternlieb I. Clinical aspects of Wilson's disease. Am J Gastroenterol 1990 85 : 1453 7.
9. Merle U, Schaefer M, Ferenci P, Stremmel W. Clinical presentation, diagnosis and long-term outcome of Wilson's disease: a cohort study. Gut 2007 56 : 115 20.
10. Medici V, Rossaro L, Sturniolo GC. Wilson disease - a practical approach to diagnosis, treatment and follow-up. Dig Liver Dis 2007 39 : 601 9.
11. Walshe JM. Penicillamine, a new oral therapy for Wilson's disease. Am J Med 1956 21 : 487 95.
12. Walshe JM. Treatment of Wilson's disease with trientine (triethylene tetramine) dihydrochloride. Lancet 1982 1 : 643 7.
13. Mills CF, El-Gallad TT, Bremner I. Effects of molybdate, sulfide, and tetrathiomolybdate on copper metabolism in rats. J Inorg Biochem 1981 14 : 189 207.
14. Brewer GJ, Dick RD, Yuzbasiyan-Gurkin V, Tankanow R, Young AB, Kluin KJ. Initial therapy of patients with Wilson's disease with tetrathiomolybdate. Arch Neurol 1991 48 : 42 7.
15. Jones HB, Gooneratne SR, Howel JM. X-ray microanalysis of liver and kidney in copper loaded sheep with and without thiomolybdate administration. Res Vet Sci 1984 37 : 273.
16. Yuzbasiyan-Gurkan V, Grider A, Nostrant T, Cousins RJ, Brewer GJ. Treatment of Wilson's disease with zinc: X. Intestinal metallothionein induction. J Lab Clin Med 1992 120 : 380 6.
17. Jablońska-Kaszewska I, Drobińska-Jurowiecka A, Dabrowska E, Trocha H. Results of treatment of Wilson's disease - own observations. Med Sci Monit 2003 9 (Suppl. 3 9 14.
18. Lee DY, Brewer GJ, Wang YX. Treatment of Wilson's disease with zinc. VII. Protection of the liver from copper toxicity by zinc-induced metallothionein in a rat model. J Lab Clin Med 1989 114 : 639 45.
19. Goering PL, Tandon SK, Klaassen CD. Induction of hepatic metallothionein in mouse liver following administration of chelating agents. Toxicol Appl Pharmacol 1985 80 : 467 72.
20. Ala A, Walker AP, Ashkan K, Dooley JS, Schilsky ML. Wilson's disease. Lancet 2007 369 : 397 408.
21. Checklist for appraisal of scientific publications [Internet]. Available from: http://www.ohg.cochrane.org/forms/ (accessed 8 January 2008).
22. Chang CF, Shen MT, Wu JC, et al. Clinical study of Wilson's disease in Taiwan. Chin J Gastroenterol 1992 9 : 149 56.
23. On A, Choi HJ, Heyman MB, Vargas J, Ament ME. Pediatric Wilson's disease: presentation and management. Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi 1997 38 : 98 103.
24. Askari FK, Greenson J, Dick RD, Johnson VD, Brewer GJ. Treatment of Wilson's disease with zinc. XVIII. Initial treatment of the hepatic decompensation presentation with trientine and zinc. J Lab Clin Med 2003 142 : 385 90.
25. Bona I, Broussolle E, Neuschwander P, et al. Treatment of Wilson's disease with zinc. 5 cases. Rev Neurol 1993 149 : 393 7.
26. Brewer GJ, Yuzbasiyan-Gurkan V, Johnson V, Dick RD, Wang Y. Treatment of Wilson's disease with zinc: XI. Interaction with other anticopper agents. J Am Coll Nutr 1993 12 : 26 30.
27. Brewer GJ, Askari F, Lorincz MT, et al. Treatment of Wilson disease with ammonium tetrathiomolybdate: IV. Comparison of tetrathiomolybdate and trientine in a double blind study of treatment of the neurologic presentation of Wilson disease. Arch Neurol 2006 63 : 521 7.
28. Bono W, Moutie O, Benomar A, et al. Wilson's disease. Clinical presentation, treatment and evolution in 21 cases. Rev Med Interne 2002 23 : 419 31.
29. Brewer GJ, Yuzbasiyan-Gurkan V, Johnson V, Dick RD, Wang Y. Treatment of Wilson's disease with zinc XII: dose regimen requirements. Am J Med Sci 1993 305 : 199 202.
30. Bachmann H, Lössner J, Kühn HJ, et al. Long-term care and management of Wilson's disease in the GDR. Eur Neurol 1989 29 : 301 5.
31. Hoogenraad TU, Van den Hamer CJ. 3 years of continuous oral zinc therapy in 4 patients with Wilson's disease. Acta Neurol Scand 1983 67 : 356 64.
32. Brewer GJ, Hill GM, Dick RD, et al. Treatment of Wilson's disease with zinc: III. Prevention of reaccumulation of hepatic copper. J Lab Clin Med 1987 109 : 526 31.
33. Dahlman T, Hartvig P, Löfholm M, Nordlinder H, Lööf L, Westermark K. Long-term treatment of Wilson's disease with triethylene tetramine dihydrochloride (trientine). QJM 1995 88 : 609 16.
34. Dubois RS, Rodgerson DO, Hambidge KM. Treatment of Wilson's disease with triethylene tetramine hydrochloride (Trientine). J Pediatr Gastroenterol Nutr 1990 10 : 77 81.
35. Rossaro L, Sturniolo GC, Giacon G, et al. Zinc therapy in Wilson's disease: observations in five patients. Am J Gastroenterol 1990 85 : 665 8.
36. Saito H, Watanabe K, Sahara M, Mochizuki R, Edo K, Ohyama Y. Triethylene-tetramine (trien) therapy for Wilson's disease. Tohoku J Exp Med 1991 164 : 29 35.
37. Scheinberg IH, Jaffe ME, Sternlieb I. The use of trientine in preventing the effects of interrupting penicillamine therapy in Wilson's disease. N Engl J Med 1987 317 : 209 13.
38. Sinha S, Taly AB. Withdrawal of penicillamine from zinc sulphate-penicillamine maintenance therapy in Wilson's disease: promising, safe and cheap. J Neurol Sci 2008 264 : 129 32.
39. Yamaguchi Y. Triethylenetetramine therapy for D-penicillamine-intolerant patients with Wilson's disease: preclinical and clinical studies on the safety and efficacy of triethylenetetramine. J Med Soc Toho Univ 1992 38 : 756 72.
40. Taly AB, Meenakshi-Sundaram S, Sinha S, Swamy HS, Arunodaya GR. Wilson disease: description of 282 patients evaluated over 3 decades. Medicine (Baltimore) 2007 86 : 112 21.
41. Guan R, Yeo PP, Ng HS, et al. Wilson's disease revisited in the tropics. Ann Acad Med Singapore 1986 15 : 186 93.
42. Pellecchia MT, Criscuolo C, Longo K, Campanella G, Filla A, Barone P. Clinical presentation and treatment of Wilson's disease: a single-centre experience. Eur Neurol 2003 50 : 48 52.
43. Starosta-Rubinstein S, Young AB, Kluin K, et al. Clinical assessment of 31 patients with Wilson's disease. Correlations with structural changes on magnetic resonance imaging. Arch Neurol 1987 44 : 365 70.
44. Park RH, McCabe P, Fell GS, Russell RI. Wilson's disease in Scotland. Gut 1991 32 : 1541 5.
45. Schilsky ML, Scheinberg IH, Sternlieb I. Prognosis of Wilsonian chronic active hepatitis. Gastroenterology 1991 100 : 762 7.
46. 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. Arch Neurol 1996 53 : 1017 25.
47. Brewer GJ, Yuzbasiyan-Gurkan V, Lee DY, Appelman H. Treatment of Wilson's disease with zinc. VI. Initial treatment studies. J Lab Clin Med 1989 114 : 633 8.
48. Brewer GJ, Dick RD, Johnson V, et al. Treatment of Wilson's disease with ammonium tetrathiomolybdate. I. Initial therapy in 17 neurologically affected patients. Arch Neurol 1994 51 : 545 54.
49. 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 8.
50. Goldstein NP, Tauxe WN, McCall JT, Gross JB, Randall RV. Treatment of Wilson's disease (hepatolenticular degeneration) with penicillamine and low-copper diet. Trans Am Neurol Assoc 1969 94 : 34 7.
51. Brewer GJ, Dick RD, Yuzbasiyan-Gurkan V, Johnson V, Wang Y. Treatment of Wilson's disease with zinc. XIII: therapy with zinc in presymptomatic patients from the time of diagnosis. J Lab Clin Med 1994 123 : 849 58.
52. Arima M, Takeshita K, Yoshino K, Kitahara T, Suzuki Y. Prognosis of Wilson's disease in childhood. Eur J Pediatr 1977 126 : 147 54.
53. Berry WR, Aronson AE, Darley FL, Goldstein NP. Effects of penicillamine therapy and low-copper diet on dysarthria in Wilson's disease (hepatolenticular degeneration). Mayo Clin Proc 1974 49 : 405 8.
54. Durand F, Bernuau J, Giostra E, et al. Wilson's disease with severe hepatic insufficiency: beneficial effects of early administration of D-penicillamine. Gut 2001 48 : 849 52.
55. Giacchino R, Marazzi MG, Barabino A, et al. Syndromic variability of Wilson's disease in children. Clinical study of 44 cases. Ital J Gastroenterol Hepatol 1997 29 : 155 61.
56. Goldstein NP, Tauxe WN, McCall JT, Randall RV, Gross JB. Wilson's disease (hepatolenticular degeneration). Treatment with penicillamine and changes in hepatic trapping of radioactive copper. Arch Neurol 1971 24 : 391 400.
57. Lange J. Long-term treatment of Wilson's disease with D-penicillamine. Report on 20 cases. Dtsch Med Wochenschr 1967 92 : 1657 62.
58. Sternlieb I, Scheinberg IH. Prevention of Wilson's disease in asymptomatic patients. N Engl J Med 1968 278 : 352 9.
59. Brewer GJ, Dick RD, Johnson VD, Brunberg JA, Kluin KJ, Fink JK. Treatment of Wilson's disease with zinc: XV long-term follow-up studies. J Lab Clin Med 1998 132 : 264 78.
60. Hoogenraad TU, Van Hattum J, Van den Hamer CJ. Management of Wilson's disease with zinc sulphate. Experience in a series of 27 patients. J Neurol Sci 1987 77 : 137 46.
61. Marcellini M, Di Ciommo V, Callea F, et al. Treatment of Wilson's disease with zinc from the time of diagnosis in pediatric patients: a single-hospital, 10-year follow-up study. J Lab Clin Med 2005 145 : 139 43.
62. Wu ZY, Lin MT, Murong SX, Wang N. Molecular diagnosis and prophylactic therapy for presymptomatic Chinese patients with Wilson disease. Arch Neurol 2003 60 : 737 41.
63. Czlonkowska A, Gajda J, Rodo M. Effects of long-term treatment in Wilson's disease with D-penicillamine and zinc sulphate. J Neurol 1996 243 : 269 73.
64. Medici V, Trevisan CP, D'Incà R, et al. Diagnosis and management of Wilson's disease: results of a single center experience. J Clin Gastroenterol 2006 40 : 936 41.
65. Brewer GJ. Novel therapeutic approaches to the treatment of Wilson's disease. Expert Opin Pharmacother 2006 7 : 317 24.
66. Dhawan A, Taylor RM, Cheeseman P, DeSilva P, Katsiyiannakis L, Mieli-Vergani G. Wilson's disease in children: 37-year experience and revised King's score for liver transplantation. Liver Transpl 2005 11 : 441 8.
67. Santos Silva EE, Sarles J, Buts JP, Sokal EM. Successful medical treatment of severely decompensated Wilson disease. J Pediatr 1996 128 : 285 7.
68. Van Caillie-Bertrand M, Degenhart HJ, Visser HK, Sinaasappel M, Bouquet J. Oral zinc sulphate for Wilson's disease. Arch Dis Child 1985 60 : 656 9.
69. Walshe JM, Yealland M. Chelation treatment of neurological Wilson's disease. Q J Med 1993 86 : 197 204.
70. Brewer GJ, Terry CA, Aisen AM, Hill GM. Worsening of neurologic syndrome in patients with Wilson's disease with initial penicillamine therapy. Arch Neurol 1987 44 : 490 3.
71. Brewer GJ, Turkay A, Yuzbasiyan-Gurkan V. Development of neurological symptoms in a patient with asymptomatic Wilson's disease treated with penicillamine. Arch Neurol 1994 51 : 304 5.
72. Glass JD, Reich SG, DeLong MR. Wilson's disease. Development of neurological disease after beginning penicillamine therapy. Arch Neurol 1990 47 : 595 6.
73. Brewer GJ, Yuzbasiyan-Gurkan V, Dick R. Zinc therapy of Wilson's disease: VIII. Dose response studies. J Trace Elem Exp Med 1990 3 : 227 34.
74. Kumagi T, Horiike N, Michitaka K, et al. Recent clinical features of Wilson's disease with hepatic presentation. J Gastroenterol 2004 39 : 1165 9.
75. Brewer GJ, Hedera P, Kluin KJ, et al. Treatment of Wilson disease with ammonium tetrathiomolybdate: III. Initial therapy in a total of 55 neurologically affected patients and follow-up with zinc therapy. Arch Neurol 2003 60 : 379 85.