Short-term continuous-flow ventricular assist devices (STCF-VADs) are increasingly being utilized in pediatrics. End-stage liver disease (ELD) models have been associated with outcomes in adult patients on mechanical circulatory support. We sought to determine the relationship between outcomes in children on STCF-VADs and three ELD models: model for end-stage liver disease-excluding international normalized ratio (MELD-XI; all) and MELD-XI (> 1 year), PELD, and a novel score, PedMELD-XI. All patients (< 19 years) supported with STCF-VADs, between June 2009 and December 2016 were included. The MELD-XI, PELD, and PedMELD-XI scores were calculated and their association with adverse events and a composite measure of death, major bleeding, and neurologic dysfunction was analyzed. Of 32 patients, median age was 0.57 years (interquartile range [IQR], 0.10-4.43), median weight was 7.15 kg (IQR, 3.68-16.53), 53.1% had congenital heart disease, and 53.1% were male. In total, 78.1% patients experienced an adverse event (78.1% a major bleed, 25.0% neurologic dysfunction, and 15.6% death). The median MELD-XI score was 11.17 (IQR, 9.44-30.01), MELD-XI (>1 year) 9.44 (IQR, 9.44-24.33), PELD 6.00 (IQR, 4.00-13.75), and PedMELD-XI -14.91 (IQR, -18.85 to -12.25). A higher MELD-XI for all ages (13.80 vs. 9.44, p = 0.037) and less negative PedMELD-XI (-14.16 vs. -19.34, p = 0.028) scores were significantly associated with bleeding and the composite outcome. PedMELD-XI was significantly associated with death (-12.87 vs. -16.84, p = 0.041) while a trend was seen for increased MELD-XI in all ages being associated with death (31.52 vs. 10.11, p = 0.051). Last, there was no association with the models and neurologic events. MELD-XI and PedMELD-XI were significantly associated with major bleeding and the composite endpoints with PedMELD-XI also being associated with death. These results suggest that ELD models can be used to predict outcomes in this specific patient population, however, further analysis in a larger population is required.
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http://dx.doi.org/10.1097/MAT.0000000000001078 | DOI Listing |
Liver Transpl
January 2025
Unit of Internal Medicine and Hepatology (UIMH), Department of Medicine - DIMED, University of Padova, Padova, Italy.
Mol Genet Metab
January 2025
Medical Genetics Service, HCPA, UFRGS, Porto Alegre, RS, Brazil; Graduate Program in Genetics and Molecular Biology, UFRGS, Porto Alegre, RS, Brazil; InRaras (National Institute of Science and Technology on Rare Diseases), Brazil.
Gaucher disease (GD) is a rare genetic disorder with multi-system involvement. Liver fibrosis is a long-term complication of GD, potentially leading to cirrhosis, end-stage liver disease, and hepatocellular carcinoma. There are currently no validated clinical tools for the monitoring of liver fibrosis in patients with GD.
View Article and Find Full Text PDFTransplant Proc
January 2025
Hepatobiliary Surgery and Liver Transplantation Unit, Cruces University Hospital, Bilbao, Spain; BioBizkaia Research Health Institute, Barakaldo, Bizkaia, Spain. Electronic address:
Polycystic liver disease (PLD) is a hereditary condition, and its symptoms are due to the growth of cysts. Liver transplantation (LT) is the only curative treatment. A retrospective single-center analysis was conducted on the 10 LTs performed for PLD between 2004 and 2023.
View Article and Find Full Text PDFZhonghua Gan Zang Bing Za Zhi
December 2024
Department of Infectious Disease Medicine, Fifth Medical Center, PLA General Hospital, National Clinical Research Center of Infectious Diseases, Beijing100039, China.
End-stage liver disease includes liver failure and decompensated cirrhosis resulting from various etiologies and often leads to patient mortality due to complications and clinical symptoms such as severe jaundice, ascites, hepatic encephalopathy, coagulopathy, and hepatorenal syndrome. Liver transplantation is currently regarded as the most effective treatment, but its clinical application is limited by the shortage of donors, elevated expenses, and post-transplant rejection. Stem cells are a group of cells with multidirectional differentiation potential and self-renewal ability, which can improve the clinical indicator outcomes through mechanisms such as immunoregulation and promotion of tissue repair in patients with end-stage liver disease.
View Article and Find Full Text PDFThromb Haemost
January 2025
Division of Gastroenterology, Department of Medicine, Faculty of Medicine, Chulalongkorn University and King Chulalongkorn Memorial Hospital, Bangkok, Thailand.
Background: Clot waveform analysis (CWA) provides a global assessment of hemostasis and may be useful for patients with cirrhosis with complex hemostatic abnormalities. This study aimed to assess the association between prothrombin time (PT-) and activated partial thromboplastin time (aPTT-) based CWA parameters and cirrhosis severity and prospectively evaluate the role of CWA in predicting mortality and acute decompensation (AD) over 1 year.
Methods: This prospective study included adult patients with cirrhosis between June 2021 and December 2023 at Chulalongkorn University Hospital.
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