Details der Publikation - Late-onset acute liver failure due to Wilson’s disease managed by plasmapheresis and hemodiafiltration successfully serving as a bridge for deceased donor liver transplantation: a case report and literature review

Late-onset acute liver failure due to Wilson’s disease managed by plasmapheresis and hemodiafiltration successfully serving as a bridge for deceased donor liver transplantation: a case report and literature review

Abstract Late-onset acute liver failure due to Wilson’s disease (WD-ALF) is rare. A 44-year-old female patient presenting acute hepatic decompensation with extreme coagulopathy was transferred to our hospital for evaluation for liver transplantation (LT). Alveolar hemorrhage and Coombs-negative acute hemolysis occurred during workup. Mechanical ventilation, plasmapheresis, and hemodiafiltration with zinc and chelation were started immediately before placing the patient on the waitlist for deceased donor LT (DDLT), with a tentative diagnosis of WD-ALF using the Leipzig score and quick diagnostic criteria suggested by the Acute Liver Failure Study Group Registry. The peak MELD score was 40, and the revised version of King’s score for WD was 13. Serum free copper levels and the patient’s overall general condition were stabilized with artificial support systems, although triphasic wave on electroencephalogram and liver atrophy were noted. She successfully underwent emergent DDLT approximately 2 weeks after suffering from acute hemolysis and survived. The genetic tests confirmed mutations at 2 loci in the ATP7B gene and, therefore, the diagnosis of WD. This is the first and oldest patient reported in Japan to present late-onset WD-ALF that was successfully treated with emergent DDLT..

Medienart:	E-Artikel

Erscheinungsjahr:	2020
Erschienen:	2020

Enthalten in:	Zur Gesamtaufnahme - volume:13
Enthalten in:	Clinical journal of gastroenterology - 13(2020), 6 vom: 08. Juli, Seite 1239-1246

Sprache:	Englisch

Beteiligte Personen:	Sukezaki, Akira [VerfasserIn] Chu, Po-sung [VerfasserIn] Shinoda, Masahiro [VerfasserIn] Hibi, Taizo [VerfasserIn] Taniki, Nobuhito [VerfasserIn] Yoshida, Aya [VerfasserIn] Kawaida, Miho [VerfasserIn] Hori, Shutarou [VerfasserIn] Morikawa, Rei [VerfasserIn] Kurokouchi, Arata [VerfasserIn] Wakino, Shu [VerfasserIn] Kameyama, Kaori [VerfasserIn] Obara, Hideaki [VerfasserIn] Kitagawa, Yuko [VerfasserIn] Saito, Hidetsugu [VerfasserIn] Kanai, Takanori [VerfasserIn] Nakamoto, Nobuhiro [VerfasserIn]

Links:	Volltext [lizenzpflichtig]

BKL:	44.87 / Gastroenterologie / Gastroenterologie
Themen:	Acute liver failure Acute-on-chronic liver failure Adult Deceased donor liver transportation Wilson’s disease

Anmerkungen:	© Japanese Society of Gastroenterology 2020

doi:	10.1007/s12328-020-01175-8

funding:
Förderinstitution / Projekttitel:

PPN (Katalog-ID):	OLC2121291032

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520			\|a Abstract Late-onset acute liver failure due to Wilson’s disease (WD-ALF) is rare. A 44-year-old female patient presenting acute hepatic decompensation with extreme coagulopathy was transferred to our hospital for evaluation for liver transplantation (LT). Alveolar hemorrhage and Coombs-negative acute hemolysis occurred during workup. Mechanical ventilation, plasmapheresis, and hemodiafiltration with zinc and chelation were started immediately before placing the patient on the waitlist for deceased donor LT (DDLT), with a tentative diagnosis of WD-ALF using the Leipzig score and quick diagnostic criteria suggested by the Acute Liver Failure Study Group Registry. The peak MELD score was 40, and the revised version of King’s score for WD was 13. Serum free copper levels and the patient’s overall general condition were stabilized with artificial support systems, although triphasic wave on electroencephalogram and liver atrophy were noted. She successfully underwent emergent DDLT approximately 2 weeks after suffering from acute hemolysis and survived. The genetic tests confirmed mutations at 2 loci in the ATP7B gene and, therefore, the diagnosis of WD. This is the first and oldest patient reported in Japan to present late-onset WD-ALF that was successfully treated with emergent DDLT.
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Late-onset acute liver failure due to Wilson’s disease managed by plasmapheresis and hemodiafiltration successfully serving as a bridge for deceased donor liver transplantation: a case report and literature review

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