Skip to content
2000
Volume 23, Issue 13
  • ISSN: 1570-159X
  • E-ISSN: 1875-6190

Abstract

Background

This study aims to guide the diagnosis and treatment of hepatolenticular degeneration (also named Wilson's disease, WD) and aid multidisciplinary clinicians in making reasonable and personalized treatment regimens.

Objectives

The authors aim to establish a systemic structure for Chinese Multidisciplinary Expert Consensus on Diagnosis and Treatment of Hepatolenticular Degeneration.

Methods

We collaborated with experts from relevant branches of the Chinese Medical Association and multiple disciplines, along with statistical experts, to formulate this consensus. It is based on advancements in basic and clinical research on Wilson's disease, both domestically and internationally.

Results

It mainly consists of clinical manifestations, diagnosis, differential diagnosis, management, and prognosis in the context of Multi-Department treatment (MDT) in China.

Conclusion

This Chinese consensus incorporates four decades of institutional experience with thousands of Chinese Wilson’s disease (WD) inpatients, as well as decades of international inpatient cases from East to West. It is hoped that this consensus will garner broader attention from clinicians worldwide.

© 2025 Bentham Science Publishers
Loading

Article metrics loading...

/content/journals/cn/10.2174/011570159X349587250311072553
2025-04-07
2025-10-28

  • From This Site

    /content/journals/cn/10.2174/011570159X349587250311072553
    dcterms_title,dcterms_subject,pub_keyword
    -contentType:Contributor -contentType:Concept -contentType:Institution
    10
    5
Loading full text...

Full text loading...

References

  1. AlkhouriN. Gonzalez-PeraltaR.P. MediciV. Wilson disease: A summary of the updated AASLD Practice Guidance.Hepatol. Commun.202376e015010.1097/HC9.0000000000000150 37184530
     [Google Scholar]
  2. ShribmanS. MarjotT. SharifA. VimalesvaranS. AlaA. AlexanderG. DhawanA. DooleyJ. GillettG.T. KellyD. McNeillA. WarnerT.T. WheaterV. GriffithsW. BandmannO. Investigation and management of Wilson’s disease: A practical guide from the British Association for the Study of the Liver.Lancet Gastroenterol. Hepatol.20227656057510.1016/S2468‑1253(22)00004‑8 35429442
     [Google Scholar]
  3. WallaceD.F. DooleyJ.S. ATP7B variant penetrance explains differences between genetic and clinical prevalence estimates for Wilson disease.Hum. Genet.202013981065107510.1007/s00439‑020‑02161‑3 32248359
     [Google Scholar]
  4. GaoJ. BrackleyS. MannJ.P. The global prevalence of Wilson disease from next-generation sequencing data.Genet. Med.20192151155116310.1038/s41436‑018‑0309‑9
     [Google Scholar]
  5. LinderM.C. Apoceruloplasmin: Abundance, detection, formation, and metabolism.Biomedicines20219323310.3390/biomedicines9030233 33669134
     [Google Scholar]
  6. MordauntC.E. KiefferD.A. ShibataN.M. CzłonkowskaA. LitwinT. WeissK.H. ZhuY. BowlusC.L. SarkarS. CooperS. WanY.J.Y. AliM.R. LaSalleJ.M. MediciV. Epigenomic signatures in liver and blood of Wilson disease patients include hypermethylation of liver-specific enhancers. Epigenet.Chro.20191211010.1186/s13072‑019‑0255‑z 30709419
     [Google Scholar]
  7. MediciV. ShibataN.M. KharbandaK.K. LaSalleJ.M. WoodsR. LiuS. EngelbergJ.A. DevarajS. TörökN.J. JiangJ.X. HavelP.J. LönnerdalB. KimK. HalstedC.H. Wilson’s disease: Changes in methionine metabolism and inflammation affect global DNA methylation in early liver disease.Hepatology201357255556510.1002/hep.26047 22945834
     [Google Scholar]
  8. PatilM. ShethK.A. KrishnamurthyA.C. DevarbhaviH. A review and current perspective on Wilson disease.J. Clin. Exp. Hepatol.20133432133610.1016/j.jceh.2013.06.002 25755520
     [Google Scholar]
  9. JacobsD.A. MarkowitzC.E. LiebeskindD.S. GalettaS.L. The “double panda sign” in Wilson’s disease.Neurology200361796910.1212/01.WNL.0000085871.98174.4E 14557570
     [Google Scholar]
  10. CarpenterT.O. CarnesD.L.Jr AnastC.S. Hypoparathyroidism in Wilson’s disease.N. Engl. J. Med.19833091587387710.1056/NEJM198310133091501 6888480
     [Google Scholar]
  11. WalsheJ.M. Pregnancy in Wilson’s disease.Q. J. Med.1977461817383 866569
     [Google Scholar]
  12. SochaP. JanczykW. DhawanA. BaumannU. D’AntigaL. TannerS. IorioR. VajroP. HouwenR. FischlerB. DezsofiA. HadzicN. HierroL. JahnelJ. McLinV. NobiliV. SmetsF. VerkadeH.J. DebrayD. Wilson’s disease in children.J. Pediatr. Gastroenterol. Nutr.201866233434410.1097/MPG.0000000000001787 29341979
     [Google Scholar]
  13. MerleU. SchaeferM. FerenciP. StremmelW. Clinical presentation, diagnosis and long-term outcome of Wilson’s disease: A cohort study.Gut200756111512010.1136/gut.2005.087262 16709660
     [Google Scholar]
  14. HlubockáZ. MarečekZ. LinhartA. KejřováE. PospíšilováL. MartásekP. AschermannM. Cardiac involvement in Wilson disease.J. Inherit. Metab. Dis.200225426927710.1023/A:1016546223327 12227457
     [Google Scholar]
  15. GrandisD.J. NahG. WhitmanI.R. VittinghoffE. DewlandT.A. OlginJ.E. MarcusG.M. Wilson’s disease and cardiac myopathy.Am. J. Cardiol.2017120112056206010.1016/j.amjcard.2017.08.025 28947309
     [Google Scholar]
  16. FerenciP. CzłonkowskaA. MerleU. FerencS. GromadzkaG. YurdaydinC. VogelW. BruhaR. SchmidtH.T. StremmelW. Late-onset Wilson’s disease.Gastroenterology200713241294129810.1053/j.gastro.2007.02.057 17433323
     [Google Scholar]
  17. AlaA. BorjiginJ. RochwargerA. SchilskyM. Wilson disease in septuagenarian siblings.Hepatology200541366867010.1002/hep.20601 15723329
     [Google Scholar]
  18. SchilskyM.L. RobertsE.A. BronsteinJ.M. DhawanA. HamiltonJ.P. RivardA.M. WashingtonM.K. WeissK.H. ZimbreanP.C. A multidisciplinary approach to the diagnosis and management of Wilson disease: 2022 Practice Guidance on Wilson disease from the American Association for the Study of Liver Diseases.Hepatology20221610.1002/hep.32801 36151586
     [Google Scholar]
  19. SvetelM. PotrebićA. PekmezovićT. TomićA. KresojevićN. JešićR. DragaševićN. KostićV.S. Neuropsychiatric aspects of treated Wilson’s disease.Parkinsonism Relat. Disord.2009151077277510.1016/j.parkreldis.2009.01.010 19559640
     [Google Scholar]
  20. AkilM. BrewerG.J. Psychiatric and behavioral abnormalities in Wilson’s disease.Adv. Neurol.199565171178 7872138
     [Google Scholar]
  21. DeningT.R. BerriosG.E. Wilson’s disease.Arch. Gen. Psychiatry198946121126113410.1001/archpsyc.1989.01810120068011 2589927
     [Google Scholar]
  22. YuanX.Z. YangR.M. WangX.P. Management perspective of wilsons disease: Early diagnosis and individualized therapy.Curr. Neuropharmacol.202119446548510.2174/18756190MTA2tMjQDy 32351182
     [Google Scholar]
  23. SeniόwJ. BakT. GajdaJ. PoniatowskaR. CzlonkowskaA. Cognitive functioning in neurologically symptomatic and asymptomatic forms of Wilson’s disease.Mov. Disord.20021751077108310.1002/mds.10195 12360563
     [Google Scholar]
  24. HegdeS. SinhaS. RaoS. TalyA. VasudevM.K. Cognitive profile and structural findings in Wilson′s disease: A neuropsychological and MRI-based study.Neurol. India201058570871310.4103/0028‑3886.72172 21045492
     [Google Scholar]
  25. FrotaN.A.F. BarbosaE.R. PortoC.S. LucatoL.T. OnoC.R. BuchpiguelC.A. CaramelliP. Cognitive impairment and magnetic resonance imaging correlations in Wilson’s disease.Acta Neurol. Scand.2013127639139810.1111/ane.12037 23215772
     [Google Scholar]
  26. SendaM. TeradaS. TakenoshitaS. Diagnostic utility of the Addenbrooke’s Cognitive Examination - III (ACE-III), Mini-ACE, mini-mental state examination, montreal cognitive assessment, and hasegawa dementia scale-revised for detecting mild cognitive impairment and dementia.Psychogeriatrics202020215616210.1111/psyg.12480
     [Google Scholar]
  27. European Association for Study of Liver Wilson’s diseaseJ. Hepatol.201256367168510.1016/j.jhep.2011.11.007 22340672
     [Google Scholar]
  28. ReubenA. TillmanH. FontanaR.J. DavernT. McGuireB. StravitzR.T. DurkalskiV. LarsonA.M. LiouI. FixO. SchilskyM. McCashlandT. HayJ.E. MurrayN. ShaikhO.S. GangerD. ZamanA. HanS.B. ChungR.T. SmithA. BrownR. CrippinJ. HarrisonM.E. KochD. MunozS. ReddyK.R. RossaroL. SatyanarayanaR. HassaneinT. HanjeA.J. OlsonJ. SubramanianR. KarvellasC. HameedB. SherkerA.H. RobuckP. LeeW.M. Outcomes in adults with acute liver failure between 1998 and 2013.Ann. Intern. Med.20161641172473210.7326/M15‑2211 27043883
     [Google Scholar]
  29. SchilskyM.L. Wilson Disease.Clin. Liver Dis.201721475576710.1016/j.cld.2017.06.011 28987261
     [Google Scholar]
  30. SchilskyM.L. ScheinbergH.I. SternliebI. Liver transplantation for Wilson’s disease: Indications and outcome.Hepatology199419358358710.1002/hep.1840190307 8119682
     [Google Scholar]
  31. FerenciP. StremmelW. CzłonkowskaA. SzalayF. ViveirosA. StättermayerA.F. BruhaR. HouwenR. PopT.L. StauberR. GschwantlerM. PfeiffenbergerJ. YurdaydinC. AignerE. Steindl-MundaP. DienesH.P. ZollerH. WeissK.H. Age and sex but not ATP7B genotype effectively influence the clinical phenotype of wilson disease.Hepatology20196941464147610.1002/hep.30280 30232804
     [Google Scholar]
  32. PandeyN. JohnS. Kayser-Fleischer Ring.Disclosure: Savio John declares no relevant financial relationships with ineligible companiesStatPearls: Treasure Island (FL)2023
     [Google Scholar]
  33. Langwińska-WośkoE. LitwinT. DzieżycK. KarlinskiM. CzłonkowskaA. Optical coherence tomography as a marker of neurodegeneration in patients with Wilson’s disease.Acta Neurol. Belg.2017117486787110.1007/s13760‑017‑0788‑5 28488258
     [Google Scholar]
  34. FactorS.M. ChoS. SternliebI. ScheinbergI.H. GoldfischerS. The cardiomyopathy of Wilson’s disease.Virchows Arch. A Pathol. Anat. Histol.1982397330131110.1007/BF00496571 7157667
     [Google Scholar]
  35. TarnackaB. RodoM. CichyS. CzłonkowskaA. Procreation ability in Wilson’s disease.Acta Neurol. Scand.2000101639539810.1034/j.1600‑0404.2000.90140a.x 10877157
     [Google Scholar]
  36. KaushanskyA. FrydmanM. KaufmanH. HomburgR. Endocrine studies of the ovulatory disturbances in Wilson’s disease (hepatolenticular degeneration).Fertil. Steril.198747227027310.1016/S0015‑0282(16)50004‑1 3817173
     [Google Scholar]
  37. KellyJ. RaizmanJ.E. BevilacquaV. Complex reference value distributions and partitioned reference intervals across the pediatric age range for 14 specialized biochemical markers in the CALIPER cohort of healthy community children and adolescents.Clin. Chim. Acta201545019620210.1016/j.cca.2015.08.020
     [Google Scholar]
  38. MerleU. EisenbachC. WeissK.H. TumaS. StremmelW. Serum ceruloplasmin oxidase activity is a sensitive and highly specific diagnostic marker for Wilson’s disease.J. Hepatol.200951592593010.1016/j.jhep.2009.06.022 19720421
     [Google Scholar]
  39. RyanA. NevittS.J. TuohyO. CookP. Biomarkers for diagnosis of Wilson’s disease.Cochrane Libr.2019201911CD01226710.1002/14651858.CD012267.pub2 31743430
     [Google Scholar]
  40. BurrowsS. PekalaB. Serum copper and ceruloplasmin in pregnancy.Am. J. Obstet. Gynecol.1971109690790910.1016/0002‑9378(71)90805‑2 4994775
     [Google Scholar]
  41. MakC.M. LamC.W. TamS. Diagnostic accuracy of serum ceruloplasmin in Wilson disease: Determination of sensitivity and specificity by ROC curve analysis among ATP7B-genotyped subjects.Clin. Chem.20085481356136210.1373/clinchem.2008.103432 18556333
     [Google Scholar]
  42. MarchiG. BustiF. ZidanesL.A. CastagnaA. GirelliD. Aceruloplasminemia: A severe neurodegenerative disorder deserving an early diagnosis.Front. Neurosci.20191332510.3389/fnins.2019.00325 31024241
     [Google Scholar]
  43. CauzaE. Maier-DobersbergerT. PolliC. KasererK. KramerL. FerenciP. Screening for Wilson’s disease in patients with liver diseases by serum ceruloplasmin.J. Hepatol.199727235836210.1016/S0168‑8278(97)80182‑1 9288611
     [Google Scholar]
  44. NicastroE. RanucciG. VajroP. VegnenteA. IorioR. Re-evaluation of the diagnostic criteria for Wilson disease in children with mild liver disease.Hepatology20105261948195610.1002/hep.23910 20967755
     [Google Scholar]
  45. SezerO.B. PerkP. HosnutF.O. Is it necessary to re-evaluate diagnostic criteria for Wilson disease in children?Turk. J. Gastroenterol.201425669069510.5152/tjg.2014.7787
     [Google Scholar]
  46. XuZ.X. ZhouY.Y. WuR. ZhaoY.J. WangX.P. Brain iron deposition and whole-exome sequencing of non-Wilson’s disease hypoceruloplasminemia in a family.J. Neurorestoratology202311110002710.1016/j.jnrt.2022.100027
     [Google Scholar]
  47. DuncanA. YacoubianC. BeethamR. CatchpoleA. BullockD. The role of calculated non-caeruloplasmin-bound copper in Wilson’s disease.Ann. Clin. Biochem.201754664965410.1177/0004563216676843 27742851
     [Google Scholar]
  48. PoujoisA. TrocelloJ.M. Djebrani-OussedikN. PouponJ. ColletC. Girardot-TinantN. SobeskyR. HabèsD. DebrayD. VanlemmensC. FluchèreF. Ory-MagneF. LabreucheJ. PredaC. WoimantF. Exchangeable copper: A reflection of the neurological severity in Wilson’s disease.Eur. J. Neurol.201724115416010.1111/ene.13171 27739240
     [Google Scholar]
  49. LyonT.D.B. FellG.S. GaffneyD. McGawB.A. RussellR.I. ParkR.H.R. BeattieA.D. CurryG. CroftonR.J. GunnI. SturnioloG.S. D’incaR. PatriarcaM. Use of a stable copper isotope (65Cu) in the differential diagnosis of Wilson’s disease.Clin. Sci.199588672773210.1042/cs0880727 7634759
     [Google Scholar]
  50. CzlonkowskaA. RodoM. Wierzchowska-CiokA. Accuracy of the radioactive copper incorporation test in the diagnosis of Wilson disease.Liver Int.201838101860186610.1111/liv.13715
     [Google Scholar]
  51. CoffeyA.J. DurkieM. HagueS. McLayK. EmmersonJ. LoC. KlaffkeS. JoyceC.J. DhawanA. HadzicN. Mieli-VerganiG. KirkR. AllenE.K. NichollD. WongS. GriffithsW. SmithsonS. GiffinN. TahaA. ConnollyS. GillettG.T. TannerS. BonhamJ. SharrackB. PalotieA. RattrayM. DaltonA. BandmannO. A genetic study of Wilson’s disease in the United Kingdom.Brain201313651476148710.1093/brain/awt035 23518715
     [Google Scholar]
  52. KluskaA. KuleckaM. LitwinT. Whole-exome sequencing identifies novel pathogenic variants across the ATP7B gene and some modifiers of Wilson’s disease phenotype.Liver Int.201939117718610.1111/liv.13967
     [Google Scholar]
  53. PrzybyłkowskiA. GromadzkaG. ChabikG. WierzchowskaA. LitwinT. CzłonkowskaA. Liver cirrhosis in patients newly diagnosed with neurological phenotype of Wilson’s disease.Funct. Neurol.20142912329 25014046
     [Google Scholar]
  54. MooresA. FoxS. LangA. Wilson disease: Canadian perspectives on presentation and outcomes from an adult ambulatory setting.Can. J. Gastroenterol.201226633333910.1155/2012/123431
     [Google Scholar]
  55. StremmelW. MeyerroseK.W. NiederauC. HefterH. KreuzpaintnerG. StrohmeyerG. Wilson disease: Clinical presentation, treatment, and survival.Ann. Intern. Med.1991115972072610.7326/0003‑4819‑115‑9‑720 1929042
     [Google Scholar]
  56. WalsheJ.M. Hepatic Wilson’s disease: Initial treatment and long-term management.Curr. Treat. Options Gastroenterol.20058646747210.1007/s11938‑005‑0033‑9 16313864
     [Google Scholar]
  57. FerenciP. Steindl-MundaP. VogelW. Diagnostic value of quantitative hepatic copper determination in patients with Wilson’s disease.Clin. Gastroenterol. Hepatol.20053881181810.1016/S1542‑3565(05)00181‑3
     [Google Scholar]
  58. WalsheJ.M. The acute haemolytic syndrome in Wilson’s disease—a review of 22 patients.Inter. J. Med.2013106111003100810.1093/qjmed/hct137
     [Google Scholar]
  59. SteindlP. FerenciP. DienesH.P. GrimmG. PabingerI. MadlC. DobersbergerM.T. HernethA. DragosicsB. MerynS. KnoflachP. GranditschG. GanglA. Wilson’s disease in patients presenting with liver disease: A diagnostic challenge.Gastroenterology1997113121221810.1016/S0016‑5085(97)70097‑0 9207280
     [Google Scholar]
  60. BruhaR. MarecekZ. PospisilovaL. Long-term follow-up of Wilson disease: Natural history, treatment, mutations analysis and phenotypic correlation.Liver Int.2011311839110.1111/j.1478‑3231.2010.02354.x
     [Google Scholar]
  61. TauberJ. SteinertR.F. Pseudo-kayser-fleischer ring of the cornea associated with non-wilsonian liver disease. a case report and literature review.Cornea1993121747710.1097/00003226‑199301000‑00013 8458236
     [Google Scholar]
  62. WilliamsE.J. GleesonD. BurtonJ.L. StephensonT.J. Kayser–Fleischer like rings in alcoholic liver disease.Eur. J. Gastroenterol. Hepatol.2003151919310.1097/00042737‑200301000‑00016 12544701
     [Google Scholar]
  63. Broniek-KowalikK. DzieżycK. LitwinT. CzłonkowskaA. SzaflikJ.P. Anterior segment optical coherence tomography (AS ‐ OCT) as a new method of detecting copper deposits forming the Kayser–Fleischer ring in patients with Wilson disease.Acta Ophthalmol.2019975e757e76010.1111/aos.14009 30635971
     [Google Scholar]
  64. AkhanO. AkpinarE. KarcaaltincabaM. HalilogluM. AkataD. KaraosmanogluA.D. OzmenM. Imaging findings of liver involvement of Wilson’s disease.Eur. J. Radiol.200969114715510.1016/j.ejrad.2007.09.029 17981419
     [Google Scholar]
  65. CançadoE.L. RochaM.S. BarbosaE.R. ScaffM. CerriG.G. MagalhãesA. CanelasH.M. Abdominal ultrasonography in hepatolenticular degeneration. A study of 33 patients.Arq. Neuropsiquiatr.198745213113610.1590/S0004‑282X1987000200005 3322239
     [Google Scholar]
  66. LiW. ZhaoX. ZhanQ. ChaiW. MahyoubR. YangY. ShenB. ChenK. Unique CT imaging findings of liver in Wilson’s disease.Abdom. Imaging2011361697310.1007/s00261‑010‑9606‑8 20195595
     [Google Scholar]
  67. PaternostroR. PfeiffenbergerJ. FerenciP. Non-invasive diagnosis of cirrhosis and long-term disease monitoring by transient elastography in patients with Wilson disease.Liver Int.202040489490410.1111/liv.14368
     [Google Scholar]
  68. LitwinT. GromadzkaG. CzłonkowskaA. GołębiowskiM. PoniatowskaR. The effect of gender on brain MRI pathology in Wilson’s disease.Metab. Brain Dis.2013281697510.1007/s11011‑013‑9378‑2 23315358
     [Google Scholar]
  69. LiX. FengZ. TangW. YuX. QianY. LiuB. LiX. YangR. YuY. Sex differences in clinical characteristics and brain MRI change in patients with Wilson’s disease in a chinese population.Front. Physiol.20189142910.3389/fphys.2018.01429 30356741
     [Google Scholar]
  70. SinhaS. TalyA.B. RavishankarS. PrashanthL.K. VenugopalK.S. ArunodayaG.R. VasudevM.K. SwamyH.S. Wilson’s disease: Cranial MRI observations and clinical correlation.Neuroradiology200648961362110.1007/s00234‑006‑0101‑4 16752136
     [Google Scholar]
  71. PrashanthL.K. SinhaS. TalyA.B. VasudevM.K. Do MRI features distinguish Wilson’s disease from other early onset extrapyramidal disorders? An analysis of 100 cases.Mov. Disord.201025667267810.1002/mds.22689 20437536
     [Google Scholar]
  72. PrashanthL.K. TalyA.B. SinhaS. Prognostic factors in patients presenting with severe neurological forms of Wilson’s disease.QJM200598855756310.1093/qjmed/hci095
     [Google Scholar]
  73. LitwinT. DzieżycK. KarlińskiM. ChabikG. CzepielW. CzłonkowskaA. Early neurological worsening in patients with Wilson’s disease.J. Neurol. Sci.20153551-216216710.1016/j.jns.2015.06.010 26071888
     [Google Scholar]
  74. YangX. TangX. ZhangY. LuoK. JiangY. LuoH. LeiJ. WangW. LiM. ChenH. DengS. LaiL. LiangJ. ZhangM. TianY. XuY. Prospective evaluation of the diagnostic accuracy of hepatic copper content, as determined using the entire core of a liver biopsy sample.Hepatology20156261731174110.1002/hep.27932 26095812
     [Google Scholar]
  75. LiG. WuR. TongR. BoB. ZhaoY. GillenK.M. SpincemailleP. KuY. DuY. WangY. WangX. LiJ. Quantitative measurement of metal accumulation in brain of patients with Wilson’s disease.Mov. Disord.202035101787179510.1002/mds.28141 32681698
     [Google Scholar]
  76. FanniD. GuidoM. GerosaC. VallascasV. MoiM. ConiP. VallebonaE. EykenV.P. BarcellonaD. ScanoA. OrrùG. PampaloniP. CastagnolaM. FaaG. Liver changes in Wilson’s disease: The full spectrum. A report of 127 biopsies from 43 patients.Eur. Rev. Med. Pharmacol. Sci.202125124336434410.26355/eurrev_202106_26142 34227068
     [Google Scholar]
  77. Cope-YokoyamaS. FinegoldM.J. SturnioloG.C. KimK. MescoliC. RuggeM. MediciV. Wilson disease: Histopathological correlations with treatment on follow-up liver biopsies.World J. Gastroenterol.201016121487149410.3748/wjg.v16.i12.1487 20333789
     [Google Scholar]
  78. AignerE. StrasserM. HaufeH. SonnweberT. HohlaF. StadlmayrA. SoliozM. TilgH. PatschW. WeissG. StickelF. DatzC. A role for low hepatic copper concentrations in nonalcoholic Fatty liver disease.Am. J. Gastroenterol.201010591978198510.1038/ajg.2010.170 20407430
     [Google Scholar]
  79. FerenciP. CacaK. LoudianosG. Diagnosis and phenotypic classification of Wilson disease.Liver Int.200323313914210.1034/j.1600‑0676.2003.00824.x
     [Google Scholar]
  80. NazerH. EdeR.J. MowatA.P. WilliamsR. Wilson’s disease: Clinical presentation and use of prognostic index.Gut198627111377138110.1136/gut.27.11.1377 3792921
     [Google Scholar]
  81. WeissK.H. GotthardtD.N. KlemmD. MerleU. Ferenci-FoersterD. SchaeferM. FerenciP. StremmelW. Zinc monotherapy is not as effective as chelating agents in treatment of Wilson disease.Gastroenterology2011140411891198.e110.1053/j.gastro.2010.12.034 21185835
     [Google Scholar]
  82. MarcelliniM. CiommoD.V. CalleaF. DevitoR. ComparcolaD. SartorelliM.R. CarelliF. NobiliV. 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.2005145313914310.1016/j.lab.2005.01.007 15871305
     [Google Scholar]
  83. SantiagoR. GottrandF. DebrayD. BridouxL. LachauxA. MoraliA. LapeyreD. LamireauT. Zinc therapy for wilson disease in children in french pediatric centers.J. Pediatr. Gastroenterol. Nutr.201561661361810.1097/MPG.0000000000000926 26230903
     [Google Scholar]
  84. XueB.C. YangR.M. HuJ.Y. Effect of Gandou Decoction IV combined with short-term decoppering therapy with sodium dimercapto-sulphonate on serum indexes of hepatic fibrosis in patients with Wilson’ s disease.J. Inte. Trad. West. Med.2007279785788
     [Google Scholar]
  85. LiL.Y. YangW.M. ChenH.Z. WuY.H. FangX. ZhangJ. WangZ. HanY.S. WangY. Successful splenectomy for hypersplenism in Wilson’s disease: A single center experience from china.PLoS One2015104e012456910.1371/journal.pone.0124569 25910248
     [Google Scholar]
  86. ChenH. WangX. ZhangJ. XieD. Effect of splenectomy based on inverse probability weighting of the propensity score on Wilson’s disease with hypersplenism: A retrospective cohort study.Exp. Ther. Med.202325522010.3892/etm.2023.11919 37123215
     [Google Scholar]
  87. YaoY. ZhangJ. JiangH. LiR. XieD. JiangF. ZhangW. MaM. Nomogram for prediction of portal vein system thrombosis after splenectomy for hypersplenism in patients with Wilson disease.Ann. Palliat. Med.202211123626363510.21037/apm‑22‑826 36571168
     [Google Scholar]
  88. LiL.Y. ChenH.Z. BaoY.C. Successful treatment of hypersplenism in Wilson’s disease by partial splenic embolization.J. Invest. Surg.2018312758110.1080/08941939.2016.1278058
     [Google Scholar]
  89. DhawanA. TaylorR.M. CheesemanP. SilvaD.P. KatsiyiannakisL. Mieli-VerganiG. Wilson’s disease in children: 37-Year experience and revised King’s score for liver transplantation.Liver Transpl.200511444144810.1002/lt.20352 15776453
     [Google Scholar]
  90. PetrasekJ. JirsaM. SperlJ. KozakL. TaimrP. SpicakJ. FilipK. TruneckaP. Revised King’s College score for liver transplantation in adult patients with Wilson’s disease.Liver Transpl.2007131556110.1002/lt.20920 17154398
     [Google Scholar]
  91. RobertsE.A. SchilskyM.L. Current and emerging issues in Wilson’s disease.N. Engl. J. Med.20233891092293810.1056/NEJMra1903585 37672695
     [Google Scholar]
  92. AhmadA. Torrazza-PerezE. SchilskyM.L. Liver transplantation for Wilson disease.Handb. Clin. Neurol.201714219320410.1016/B978‑0‑444‑63625‑6.00016‑1 28433103
     [Google Scholar]
  93. PalP.K. SinhaS. PillaiS. TalyA.B. AbrahamR.G. Successful treatment of tremor in Wilson’s disease by thalamotomy: A case report.Mov. Disord.200722152287229010.1002/mds.21750 17914724
     [Google Scholar]
  94. TeiveH.A.G. MunhozR.P. SouzaM.M. AntoniukS.A. SantosM.L.S.F. TeixeiraM.J. BarbosaE.R. CarvalhoR.C. ScaffM. WerneckL.C. Status dystonicus: Study of five cases.Arq. Neuropsiquiatr.2005631262910.1590/S0004‑282X2005000100005 15830060
     [Google Scholar]
  95. LitwinT. DusekP. AntosA. CzłonkowskaA. BembenekJ. Tackling the neurological manifestations in Wilson’s disease – currently available treatment options.Expert Rev. Neurother.202323121249125910.1080/14737175.2023.2268841 37842984
     [Google Scholar]
  96. RobertsE.A. SchilskyM.L. Diagnosis and treatment of Wilson disease: An update.Hepatology20084762089211110.1002/hep.22261 18506894
     [Google Scholar]
  97. BrewerG.J. JohnsonV.D. DickR.D. HederaP. FinkJ.K. KluinK.J. Treatment of Wilson’s disease with zinc. XVII: Treatment during pregnancy.Hepatology200031236437010.1002/hep.510310216 10655259
     [Google Scholar]
  98. ShimonoN. IshibashiH. IkematsuH. KudoJ. ShirahamaM. InabaS. MaedaK. YamasakiK. NihoY. Fulminant hepatic failure during perinatal period in a pregnant woman with Wilson’s disease.Gastroenterol. Jpn.1991261697310.1007/BF02779512 2007459
     [Google Scholar]
  99. KodamaH. AnanY. IzumiY. SatoY. OgraY. Copper and zinc concentrations in the breast milk of mothers undergoing treatment for Wilson’s disease: A prospective study.BMJ Paediatr. Open202151e00094810.1136/bmjpo‑2020‑000948 34222678
     [Google Scholar]
  100. CzłonkowskaA. LitwinT. DusekP. FerenciP. LutsenkoS. MediciV. RybakowskiJ.K. WeissK.H. SchilskyM.L. Wilson disease.Nat. Rev. Dis. Primers2018412110.1038/s41572‑018‑0018‑3 30190489
     [Google Scholar]
  101. HefterH. TezayakO. RosenthalD. Long-term outcome of neurological Wilson’s disease.Parkinsonism Relat. Disord.201849485310.1016/j.parkreldis.2018.01.007 29331561
     [Google Scholar]
  102. LowH.L. AlexanderS.K. MisbahuddinA. GillettG.T. Posterior subthalamic area deep brain stimulation for treatment of tremor and dystonia in Wilson’s disease.Brain Stimul.20191251304130610.1016/j.brs.2019.05.014 31138489
     [Google Scholar]
  103. Beaulieu-BoireI. AquinoC.C. FasanoA. PoonY.Y. FallisM. LangA.E. HodaieM. KaliaS.K. LozanoA. MoroE. Deep brain stimulation in rare inherited dystonias.Brain Stimul.20169690591010.1016/j.brs.2016.07.009 27743838
     [Google Scholar]
  104. SidiropoulosC. HutchisonW. MestreT. MoroE. PrescottI.A. MizrachiA.V. FallisM. RughaniA.I. KaliaS.K. LozanoA. FoxS. Bilateral pallidal stimulation for Wilson’s disease.Mov. Disord.20132891292129510.1002/mds.25446 23554137
     [Google Scholar]
  105. NagralA. SarmaM.S. MatthaiJ. KukkleP.L. DevarbhaviH. SinhaS. AlamS. BavdekarA. DhimanR.K. EapenC.E. GoyalV. MohanN. KandadaiR.M. SathiyasekaranM. PoddarU. SibalA. SankaranarayananS. SrivastavaA. ThapaB.R. WadiaP.M. YachhaS.K. DhawanA. Wilson’s disease: Clinical practice guidelines of the indian national association for study of the liver, the Indian society of pediatric gastroenterology, hepatology and nutrition, and the movement disorders society of india.J. Clin. Exp. Hepatol.201991749810.1016/j.jceh.2018.08.009 30765941
     [Google Scholar]
  106. CaiH. ChengX. WangX.P. ATP7B gene therapy of autologous reprogrammed hepatocytes alleviates copper accumulation in a mouse model of Wilson’s disease.Hepatology20227641046105710.1002/hep.32484 35340061
     [Google Scholar]
/content/journals/cn/10.2174/011570159X349587250311072553
Loading
Chinese Multidisciplinary Expert Consensus on Orphan/Anticopper Drugs and Other Non-drug Management of Hepatolenticular Degeneration
Curr. Neuropharmacol. 23, 1683 (2025); https://doi.org/10.2174/011570159X349587250311072553
/content/journals/cn/10.2174/011570159X349587250311072553
/content/journals/cn/10.2174/011570159X349587250311072553
Loading

Data & Media loading...


  • Article Type:     Review Article
Keyword(s): anticopper drugs; consensus; guideline; hepatolenticular degeneration; Multidisciplinary expert; Wilson's disease

Most Cited Most Cited RSS feed

This is a required field
Please enter a valid email address
Approval was a Success
Invalid data
An Error Occurred
Approval was partially successful, following selected items could not be processed due to error
Please enter a valid_number test