Acute Liver Failure due to Wilson Disease: Eight Years of the National Liver Transplant Program in Uruguay

Mainardi, Victoria; Rando, Karina; Valverde, Marcelo; Olivari, Daniela; Castelli, Jorge; Rey, Gabriela; Gerona, Solange

doi:10.5604/01.3001.0012.7911

Cited by 20 publications

(22 citation statements)

References 23 publications

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“…Finally, 45 patients were included in this study. Due to the lack of an established disease‐specific staging system for chronic hepatitis in WD, the pathologic result was evaluated by METAVIR scores for patients who underwent liver biopsy for diagnosis on the same day as the LS measurement, similar to previous studies . The scores were as follows: F0, no fibrosis; F1, portal fibrosis with no septa; F2, portal fibrosis with rare fibrous septa; F3, bridging fibrosis with many fibrous septa; and F4, cirrhosis …”

Section: Methodsmentioning

confidence: 99%

Diagnostic Performance of Ultrasound Elastography and Serologic Fibrosis Indices for Evaluation of Hepatic Involvement in Wilson Disease

Hwang

Yoon

Jung

et al. 2020

J of Ultrasound Medicine

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Objectives-To investigate the diagnostic value of transient elastography (TE), 2-dimensional (2D) shear wave elastography (SWE), and the serologic fibrosis indices aspartate transaminase-to-platelet ratio index (APRI) and Fibrosis-4 (FIB-4) score for Wilson disease (WD). Methods-We retrospectively identified patients with a diagnosis of WD who underwent TE and 2D SWE on the same day. Their APRI and FIB-4 scores were calculated. Hepatic involvement was classified into 5 clinical categories (I-V) based on the laboratory findings, hepatic morphologic characteristics on ultrasound (US) imaging, and clinical symptoms of cirrhosis: I, normal (n = 17); II, only biochemical abnormality (n = 15); III, altered hepatic morphologic characteristics (n = 10); IV, compensated liver cirrhosis (n = 3); and V, decompensated liver cirrhosis (n = 0). We compared the area under the receiver operating characteristic curve (AUROC) data for TE, 2D SWE, the APRI, and the FIB-4 score. A combined assessment of the serologic markers and US elastography was performed, and the AUROCs of the combinations were compared. Results-Forty-five patients were included in the study (median age, 16.0 years; range, 3-35 years). Transient elastography, 2D SWE, and APRI were comparable in distinguishing the clinical categories (AUROC, 0.799-0.928). The FIB-4 score showed lower diagnostic value in distinguishing clinical category I from the other categories (AUROC, 0.647). Combining the serologic markers and US elastography significantly increased the AUROC value of the FIB-4 score (with TE and 2D SWE, P = .01 and .02). Conclusions-Transient elastography and 2D SWE showed excellent diagnostic accuracy for differentiating the clinical categories of hepatic involvement. The APRI showed better diagnostic performance than the FIB-4 score. The assessment of hepatic manifestations in WD can be improved by combining US elastography with serologic indices. Key Words-elasticity imaging techniques; liver fibrosis; ultrasound; Wilson disease W ilson disease (WD) is a congenital copper metabolism disorder that results in liver cirrhosis and neuropsychological deterioration due to copper accumulation in the liver and central nervous system. 1 Wilson disease is the manifestation of an abnormal ATP7B (ATPase copper-transporting beta) gene, which

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Section: Methodsmentioning

confidence: 99%

Diagnostic Performance of Ultrasound Elastography and Serologic Fibrosis Indices for Evaluation of Hepatic Involvement in Wilson Disease

Hwang

Yoon

Jung

et al. 2020

J of Ultrasound Medicine

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“…The authors concluded that early referral was the determinant of prognosis. 186 Consensus statement: Special situations -Wilson disease 71. The NWI $ 11 has been used as a predictor of death in those without a liver transplant.…”

Section: Transplantation In Wilson Diseasementioning

confidence: 99%

Indian National Association for the Study of Liver Consensus Statement on Acute Liver Failure (Part-2): Management of Acute Liver Failure

Anand

Nandi²,

Acharya

et al. 2020

Journal of Clinical and Experimental Hepatology

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Acute liver failure (ALF) is not an uncommon complication of a common disease such as acute hepatitis. Viral hepatitis followed by antituberculosis drug-induced hepatotoxicity are the commonest causes of ALF in India. Clinically, such patients present with appearance of jaundice, encephalopathy, and coagulopathy. Hepatic encephalopathy (HE) and cerebral edema are central and most important clinical event in the course of ALF, followed by superadded infections, and determine the outcome in these patients. The pathogenesis of

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“…In 1991, Berman et al reported uniformly fatal outcomes in patients with WD-ALF, and not until 2007 were the first cases of survival without liver transplantation published [ 19 , 20 ]. Patients surviving ALF due to WD are mostly from the pediatric population [ 18 , 20 – 23 ]. Scarce data exist of adults with WD-ALF who survived without liver transplantation; 1 case was published by Damsgaard et al in 2019.…”

Section: Discussionmentioning

confidence: 99%

Diagnostic Dilemma and Treatment Outcome in Acute Liver Failure Due to Wilson’s Disease

2021

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Acute Liver Failure due to Wilson Disease: Eight Years of the National Liver Transplant Program in Uruguay

Cited by 20 publications

References 23 publications

Diagnostic Performance of Ultrasound Elastography and Serologic Fibrosis Indices for Evaluation of Hepatic Involvement in Wilson Disease

Diagnostic Performance of Ultrasound Elastography and Serologic Fibrosis Indices for Evaluation of Hepatic Involvement in Wilson Disease

Indian National Association for the Study of Liver Consensus Statement on Acute Liver Failure (Part-2): Management of Acute Liver Failure

Diagnostic Dilemma and Treatment Outcome in Acute Liver Failure Due to Wilson’s Disease

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