Diagnosis and treatment of acute liver failure in children with Wilson's disease

Weiyuan FANG; Yi LU

doi:10.3969/j.issn.1001-5256.2022.02.004

Volume 38 Issue 2

Feb. 2022

Turn off MathJax

Article Contents

Article Navigation > Journal of Clinical Hepatology > 2022 > 38(2): 268-272

PDF( 1942 KB)

Diagnosis and treatment of acute liver failure in children with Wilson's disease

DOI: 10.3969/j.issn.1001-5256.2022.02.004

Weiyuan FANG,
Yi LU^,

Center for Pediatric Liver Diseases, Children's Hospital of Fudan University, Shanghai 201102, China

More Information

Corresponding author: LU Yi, luyi@fudan.edu.cn
Received Date: 2021-10-25
Accepted Date: 2021-12-18
Published Date: 2022-02-20

Abstract

Abstract

Acute liver failure (ALF) is a rare and extremely severe clinical form of Wilson's disease (WD), characterized by progressive aggravation of jaundice and significant coagulation disorder with acute intravascular hemolysis. There is a high risk of severe complications such as hepatic encephalopathy and acute renal failure, and the disease progresses rapidly after onset and has a high mortality rate. At present, it is difficult to diagnose WD presenting as ALF in the early stage due to a lack of unified indicators for rapid diagnosis. Liver transplantation was considered the only effective treatment method for this disease in the past; however, recent studies have shown that medical treatment without liver transplantation can achieve autologous liver relief and recovery in some patients with WD-ALF.
- Hepatolenticular Degeneration,
- Liver Failure, Acute,
- Plasma Exchange,
- Liver Transplantation,
- Diagnosis,
- Therapeutics

FullText(HTML)

References(43)

References

[1]	SANDAHL TD, LAURSEN TL, MUNK DE, et al. The prevalence of Wilson's disease: An update[J]. Hepatology, 2020, 71(2): 722-732. DOI: 10.1002/hep.30911.
[2]	XIE JJ, WU ZY. Wilson's disease in China[J]. Neurosci Bull, 2017, 33(3): 323-330. DOI: 10.1007/s12264-017-0107-4.
[3]	HU WB, HAN YZ, YANG RM, et al. Epidemiological investigation on hepatolenticular degeneration of Han population in Jinzhai county and Lixin county, Anhui province: Report of a family pedigree[J]. Anhui Med J, 2012, 33(6): 734-737. DOI: 10.3969/j.issn.1000-0399.2012.06.036. 胡文彬, 韩咏竹, 杨任民, 等. 安徽省金寨县和利辛县汉族人群肝豆状核变性的患病率调查(附一家系报道)[J]. 安徽医学, 2012, 33(6): 734-737. DOI: 10.3969/j.issn.1000-0399.2012.06.036.
[4]	HU WB, HAN YZ, XUE BC, et al. Epidemiological study of hepatolenticular degeneration at Hanshan County, Anhui Province[J]. Natl Med J China, 2011, 91(13): 894-897. DOI: 10.3760/cma.j.issn.0376-2491.2011.13.008. 胡文彬, 韩咏竹, 薛本春, 等. 安徽省含山县肝豆状核变性的流行病学研究[J]. 中华医学杂志, 2011, 91(13): 894-897. DOI: 10.3760/cma.j.issn.0376-2491.2011.13.008.
[5]	DHAWAN A, TAYLOR RM, CHEESEMAN P, et al. Wilson's disease in children: 37-year experience and revised King's score for liver transplantation[J]. Liver Transpl, 2005, 11(4): 441-448. DOI: 10.1002/lt.20352.
[6]	SINTUSEK P, CHONGSRISAWAT V, POOVORAWAN Y. Wilson's disease in Thai children between 2000 and 2012 at King Chulalongkorn Memorial Hospital[J]. J Med Assoc Thai, 2016, 99(2): 182-187. http://www.researchgate.net/profile/Palittiya_Sintusek/publication/299454802_Wilson's_disease_in_Thai_children_between_2000_and_2012_at_king_chulalongkorn_memorial_hospital/links/5704108108ae74a08e245f16.pdf
[7]	RUKUNUZZAMAN M. Wilson's disease in Bangladeshi children: Analysis of 100 cases[J]. Pediatr Gastroenterol Hepatol Nutr, 2015, 18(2): 121-127. DOI: 10.5223/pghn.2015.18.2.121.
[8]	SQUIRES RH Jr, SHNEIDER BL, BUCUVALAS J, et al. Acute liver failure in children: The first 348 patients in the pediatric acute liver failure study group[J]. J Pediatr, 2006, 148(5): 652-658. DOI: 10.1016/j.jpeds.2005.12.051.
[9]	OSTAPOWICZ G, FONTANA RJ, SCHIØDT FV, et al. Results of a prospective study of acute liver failure at 17 tertiary care centers in the United States[J]. Ann Intern Med, 2002, 137(12): 947-954. DOI: 10.7326/0003-4819-137-12-200212170-00007.
[10]	VANDRIEL SM, AYOUB MD, RICCIUTO A, et al. Pediatric Wilson disease presenting as acute liver failure: An individual patient data Meta-analysis[J]. J Pediatr Gastroenterol Nutr, 2020, 71(3): e90-e96. DOI: 10.1097/MPG.0000000000002777.
[11]	MARKIEWICZ-KIJEWSKA M, SZYMCZAK M, ISMAIL H, et al. Liver transplantation for fulminant Wilson's disease in children[J]. Ann Transplant, 2008, 13(2): 28-31. http://www.onacademic.com/detail/journal_1000039411237310_c65e.html
[12]	SHAH D. Wilson's disease: Hepatic manifestations[J]. Dis Mon, 2014, 60(9): 465-474. DOI: 10.1016/j.disamonth.2014.07.004.
[13]	FANG WY, ABUDUXIKUER K, SHI P, et al. Pediatric Wilson disease presenting as acute liver failure: Prognostic indices[J]. World J Clin Cases, 2021, 9(14): 3273-3286. DOI: 10.12998/wjcc.v9.i14.3273.
[14]	DEVARBHAVI H, SINGH R, ADARSH CK, et al. Factors that predict mortality in children with Wilson disease associated acute liver failure and comparison of Wilson disease specific prognostic indices[J]. J Gastroenterol Hepatol, 2014, 29(2): 380-386. DOI: 10.1111/jgh.12356.
[15]	KIDO J, MATSUMOTO S, SAKAMOTO R, et al. Recovery of severe acute liver failure without transplantation in patients with Wilson disease[J]. Pediatr Transplant, 2018, 22(8): e13292. DOI: 10.1111/petr.13292.
[16]	SOCHA P, JANCZYK W, DHAWAN A, et al. Wilson's disease in children: A position paper by the Hepatology Committee of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition[J]. J Pediatr Gastroenterol Nutr, 2018, 66(2): 334-344. DOI: 10.1097/MPG.0000000000001787.
[17]	PROOST R, CASSIMAN D, LEVTCHENKO E, et al. Fulminant Wilson disease in children: Recovery after plasma exchange without transplantation[J]. J Pediatr Gastroenterol Nutr, 2020, 71(6): 720-725. DOI: 10.1097/MPG.0000000000002894.
[18]	GUILLAUD O, DUMORTIER J, SOBESKY R, et al. Long term results of liver transplantation for Wilson's disease: Experience in France[J]. J Hepatol, 2014, 60(3): 579-589. DOI: 10.1016/j.jhep.2013.10.025.
[19]	STREMMEL W, MERLE U, WEISKIRCHEN R. Clinical features of Wilson disease[J]. Ann Transl Med, 2019, 7(Suppl 2): s61. DOI: 10.21037/atm.2019.01.20.
[20]	ATTRI S, SHARMA N, JAHAGIRDAR S, et al. Erythrocyte metabolism and antioxidant status of patients with Wilson disease with hemolytic anemia[J]. Pediatr Res, 2006, 59(4 Pt 1): 593-597. DOI: 10.1203/01.pdr.0000203098.77573.39.
[21]	FERNANDO M, VAN MOURIK I, WASSMER E, et al. Wilson disease in children and adolescents[J]. Arch Dis Child, 2020, 105(5): 499-505. DOI: 10.1136/archdischild-2018-315705.
[22]	GÜNGÖR Ş, SELIMOǦLU MA, BAǦ H, et al. Is it possible to diagnose fulminant Wilson's disease with simple laboratory tests?[J]. Liver Int, 2020, 40(1): 155-162. DOI: 10.1111/liv.14263.
[23]	WALSHE JM. The acute haemolytic syndrome in Wilson's disease—a review of 22 patients[J]. QJM, 2013, 106(11): 1003-1008. DOI: 10.1093/qjmed/hct137.
[24]	RING-LARSEN H, PALAZZO U. Renal failure in fulminant hepatic failure and terminal cirrhosis: A comparison between incidence, types, and prognosis[J]. Gut, 1981, 22(7): 585-591. DOI: 10.1136/gut.22.7.585.
[25]	TIAN Y, GONG GZ, YANG X, et al. Diagnosis and management of fulminant Wilson's disease: A single center's experience[J]. World J Pediatr, 2016, 12(2): 209-214. DOI: 10.1007/s12519-015-0026-2.
[26]	ROBERTS EA, SOCHA P. Wilson disease in children[J]. Handb Clin Neurol, 2017, 142: 141-156. DOI: 10.1016/B978-0-444-63625-6.00012-4.
[27]	XU Z, BERRY BR. Oxidative hemolysis due to Wilson disease[J]. Blood, 2019, 134(7): 657. DOI: 10.1182/blood.2019001988.
[28]	HASSOUN J, HAMMER N, MAGINI G, et al. Management of acute wilsonian hepatitis with severe hemolysis: A successful combination of chelation and MARS dialysis[J]. Case Reports Hepatol, 2021, 2021: 5583654. DOI: 10.1155/2021/5583654.
[29]	HSU EK, HORSLEN SP, REYES JD. Pediatric end-stage liver disease scores as a method of assessing mortality risk or prioritization to transplantability: Let us save the children[J]. JAMA Pediatr, 2018, 172(11): 1015-1017. DOI: 10.1001/jamapediatrics.2018.2889.
[30]	MAK CM, LAM CW, TAM S. Diagnostic accuracy of serum ceruloplasmin in Wilson disease: Determination of sensitivity and specificity by ROC curve analysis among ATP7B-genotyped subjects[J]. Clin Chem, 2008, 54(8): 1356-1362. DOI: 10.1373/clinchem.2008.103432.
[31]	EISENBACH C, SIEG O, STREMMEL W, et al. Diagnostic criteria for acute liver failure due to Wilson disease[J]. World J Gastroenterol, 2007, 13(11): 1711-1714. DOI: 10.3748/wjg.v13.i11.1711.
[32]	KORMAN JD, VOLENBERG I, BALKO J, et al. Screening for Wilson disease in acute liver failure: A comparison of currently available diagnostic tests[J]. Hepatology, 2008, 48(4): 1167-1174. DOI: 10.1002/hep.22446.
[33]	NAGRAL A, SARMA MS, MATTHAI J, et al. 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]. J Clin Exp Hepatol, 2019, 9(1): 74-98. DOI: 10.1016/j.jceh.2018.08.009.
[34]	SÁNCHEZ-MONTEAGUDO A, RIPOLLÉS E, BERENGUER M, et al. Wilson's disease: Facing the challenge of diagnosing a rare disease[J]. Biomedicines, 2021, 9(9): 1100. DOI: 10.3390/biomedicines9091100.
[35]	GÜNGÖR Ş, SELIMOǦLU MA, VAROL Fİ, et al. The effects of iron and zinc status on prognosis in pediatric Wilson's disease[J]. J Trace Elem Med Biol, 2019, 55: 33-38. DOI: 10.1016/j.jtemb.2019.05.010.
[36]	OKADA T, SHIONO Y, KANEKO Y, et al. High prevalence of fulminant hepatic failure among patients with mutant alleles for truncation of ATP7B in Wilson's disease[J]. Scand J Gastroenterol, 2010, 45(10): 1232-1237. DOI: 10.3109/00365521.2010.492527.
[37]	BERMAN DH, LEVENTHAL RI, GAVALER JS, et al. Clinical differentiation of fulminant Wilsonian hepatitis from other causes of hepatic failure[J]. Gastroenterology, 1991, 100(4): 1129-1134. DOI: 10.1016/0016-5085(91)90294-u.
[38]	PATIL M, SHETH KA, KRISHNAMURTHY AC, et al. A review and current perspective on Wilson disease[J]. J Clin Exp Hepatol, 2013, 3(4): 321-336. DOI: 10.1016/j.jceh.2013.06.002.
[39]	European Association for Study of Liver. EASL clinical practice guidelines: Wilson's disease[J]. J Hepatol, 2012, 56(3): 671-685. DOI: 10.1016/j.jhep.2011.11.007.
[40]	ALAM S, LAL BB, SOOD V, et al. AARC-ACLF score: Best predictor of outcome in children and adolescents with decompensated Wilson disease[J]. Hepatol Int, 2019, 13(3): 330-338. DOI: 10.1007/s12072-019-09938-3.
[41]	LEE EJ, KIM JW, MOON JS, et al. Development of a prognostic score to predict mortality in patients with pediatric acute liver failure[J]. J Pediatr Gastroenterol Nutr, 2020, 70(6): 777-782. DOI: 10.1097/MPG.0000000000002625.
[42]	RUSTOM N, BOST M, COUR-ANDLAUER F, et al. Effect of molecular adsorbents recirculating system treatment in children with acute liver failure caused by Wilson disease[J]. J Pediatr Gastroenterol Nutr, 2014, 58(2): 160-164. DOI: 10.1097/MPG.0b013e3182a853a3.
[43]	NAGATA Y, UTO H, HASUIKE S, et al. Bridging use of plasma exchange and continuous hemodiafiltration before living donor liver transplantation in fulminant Wilson's disease[J]. Intern Med, 2003, 42(10): 967-970. DOI: 10.2169/internalmedicine.42.967.

Relative Articles

Supplements(0)

Cited By

Proportional views

Proportional views

通讯作者: 陈斌, bchen63@163.com

1.
沈阳化工大学材料科学与工程学院沈阳 110142

Get Citation

PDF

XML

Article Metrics

Article views (545) PDF downloads(98)

Diagnosis and treatment of acute liver failure in children with Wilson's disease

DOI: 10.3969/j.issn.1001-5256.2022.02.004

Abstract

References

Proportional views

Catalog

通讯作者: 陈斌, bchen63@163.com

Article Metrics

Proportional views

Related

Diagnosis and treatment of acute liver failure in children with Wilson's disease

DOI: 10.3969/j.issn.1001-5256.2022.02.004

Abstract

References

Proportional views

Catalog

通讯作者: 陈斌, bchen63@163.com

Article Metrics

Proportional views

Related

About Journal

Submission Guideline

Journal Online

Hepatobiliary College

Guidelines

Export File

Citation

Format

Content