Back to Search
Start Over
Portal hypertension and its prognostic implications in patients with Wilson's disease.
- Source :
-
Alimentary pharmacology & therapeutics [Aliment Pharmacol Ther] 2024 Jul; Vol. 60 (2), pp. 257-266. Date of Electronic Publication: 2024 May 26. - Publication Year :
- 2024
-
Abstract
- Background and Aims: Wilson's disease may progress to cirrhosis and clinically significant portal hypertension (CSPH). We aimed to assess the prevalence and prognostic impact of CSPH-related features on hepatic decompensation and transplant-free survival in patients with Wilson's disease.<br />Methods and Results: About 137 patients with Wilson's disease (Leipzig score ≥4), followed for a median observation period of 9.0 (3.9-17.7) years at the Vienna General Hospital, were included in this retrospective study. Overall, 49 (35.8%) developed features of CSPH: 14 (10.2%) varices, 40 (29.2%) splenomegaly, 20 (14.6%) ascites, 18 (13.1%) hepatic encephalopathy and 3 (2.2%) experienced acute variceal bleeding. Overall, 8 (5.8%) patients died, including three deaths caused by CSPH-related complications. Within 10 years, compensated patients with features of CSPH developed more decompensation events (8.3% vs. 1.5% in patients without CSPH, p = 0.3) and had worse transplant-free-survival (91.7% vs. 98.6%), which further declined in patients with hepatic decompensation (26.7%, log-rank: p < 0.0001). Patients with liver stiffness <15 kPa and normal platelets (≥150 G/L) were less likely to decompensate within 10 years (2.6% vs. 8.4%, p = 0.002) and had a better 10-year transplant-free-survival (97.7% vs. 83.9%, p = 0.006).<br />Conclusions: Patients with Wilson's disease developing features of CSPH are at an increased risk for hepatic decompensation and liver-related mortality, warranting for regular screening and timely initiation of effective CSPH-directed treatments.<br /> (© 2024 The Author(s). Alimentary Pharmacology & Therapeutics published by John Wiley & Sons Ltd.)
- Subjects :
- Humans
Male
Female
Retrospective Studies
Prognosis
Adult
Adolescent
Young Adult
Liver Cirrhosis complications
Liver Cirrhosis mortality
Esophageal and Gastric Varices etiology
Child
Middle Aged
Gastrointestinal Hemorrhage etiology
Hepatic Encephalopathy etiology
Austria epidemiology
Disease Progression
Liver Transplantation
Hypertension, Portal etiology
Hypertension, Portal complications
Hypertension, Portal mortality
Hepatolenticular Degeneration mortality
Hepatolenticular Degeneration complications
Hepatolenticular Degeneration physiopathology
Subjects
Details
- Language :
- English
- ISSN :
- 1365-2036
- Volume :
- 60
- Issue :
- 2
- Database :
- MEDLINE
- Journal :
- Alimentary pharmacology & therapeutics
- Publication Type :
- Academic Journal
- Accession number :
- 38798050
- Full Text :
- https://doi.org/10.1111/apt.18060