Pediatric chronic liver failure-sequential organ failure assessment score and outcome of acute liver failure in children (original) (raw)
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Prognostic factors and scoring systems associated with outcome in pediatric acute liver failure
BMC Pediatrics, 2022
Background: Pediatric acute liver failure (PALF) is an uncommon, devastating illness with significant mortality. Liver transplantation remains the mainstay of treatment for irreversible PALF. The purpose of this study was to determine the etiology and prognostic factors associated with outcome of PALF in South Africa and to evaluate prognostic scoring systems used. Methods: Records of 45 pediatric patients younger than 16 years of age who presented with PALF from 1 January 2015 till 31 October 2020 were analysed. Patients were divided into two groups with one group consisting of patients with spontaneous recovery of the liver with supportive treatment (6/45:13.3%) and the second group consisting of patients with poor outcomes who demised (19/45: 42%) or underwent liver transplantation (20/45: 44%). Results: The median age of presentation was 3.3 years (IQR 1.8-6.9) with the 1-5 years age group constituting majority of patients (55.6%). Median time to follow up was 6.1 months (IQR 0.2-28.8). Higher liver injury unit scores were observed in patients who had poorer outcomes (P = 0.008) with a threshold of greater than 246 having a sensitivity of 84% and specificity of 83% (P < 0.001). Higher peak PELD/MELD (P = 0.006) and admission UKELD (P = 0.002) scores, were found in patients with poorer outcomes. Kings College Hospital criteria (KCHC) was useful in predicting which patients would die without liver transplantation (P = 0.002). Liver transplantation was performed in 20/45 (44%) patients with a post transplantation 1 year patient and graft survival of 80%. Conclusion: Although, survival of PALF patients was lower than high and other low-middle income countries, outcomes post transplantation were good. Our study demonstrates the utility of dynamic scoring systems in PALF patients, it underscores the need for early referral and clinical monitoring in a tertiary center once the criteria for PALF have been met.
Pediatric End-stage Liver Disease Score in Acute Liver Failure to Assess Poor Prognosis
Journal of Pediatric Gastroenterology & Nutrition, 2012
Background and Aim: Although establishing accurate prognosis in acute liver failure (ALF) is of paramount importance, prognostic scoring systems still fail to achieve success. The pediatric end-stage liver disease (PELD) score has been used as a predictor of mortality in children with chronic liver disease listed for liver transplantation (LT); however, experience with the PELD score in ALF is limited. The goal of the present study was to investigate the prognostic accuracy of the PELD score in children with ALF. Patients and Methods: PELD score was calculated based on results of blood tests obtained at hospital admission from June 1999 to January 2009, in 40 consecutive patients younger than 18 years who presented with ALF. Poor outcome was defined as LT or death. Results: Mean (AESD) age of patients was 5.3 AE 4.4 years (range 6 months-17 years); 52.5% were girls (n ¼ 21). Etiologies of ALF were hepatitis A in 42.5% (17), indeterminate in 35% (14), autoimmune hepatitis in 17.5% (type 1 12.5% [n5], type 2 5% [n2]), and toxic in 5% (2). Mean PELD score was 34.92 AE 10.48 (range 6-55). PELD scores obtained on admission were significantly higher among nonsurvivors (39.8 AE 9.5) and recipients of an LT (39 AE 7.1) compared with those who survived without LT (31.3 AE 3) (P < 0.001). A cutoff of 33 in PELD score using receiver operating characteristic curves showed 81% specificity and 86% sensitivity for poor outcome (positive predictive value 92% and negative predictive value 69%; area under curve 0.88 95% confidence interval 0.77-1.0; P < 0.0001). Conclusions: PELD score obtained upon admission may be of help to establish the optimal timing for LT evaluation and listing. Further validation in larger and more diverse populations is needed.
Annals of Intensive Care, 2020
Background and aims Data on outcome of critically ill children with cirrhosis are scarce. We aimed to evaluate the prognostic accuracy of sequential organs scoring systems in children with cirrhosis admitted to Paediatric Intensive Care Units (PICU). Methods We performed a multicentre retrospective analysis of children with cirrhosis admitted into four European PICUs between 2011 and 2016. Investigators were members of the ESPNIC liver failure and support working group. Paediatric End-Stage Liver Disease (PELD) and paediatric chronic liver failure sequential organ failure assessment score (pCLIF-SOFA) diagnostic accuracy for 28- and 60-day liver transplantation, 28-day mortality and 60-day composite outcome (ie. death or liver transplantation) were tested. Results One-hundred-and-thirty children were included. The main causes for PICU admission were acute-on-chronic liver failure (ACLF), gastrointestinal bleeding and sepsis. Twenty-nine percent died and 22.3% were transplanted by da...
Journal of Hepatology, 2006
Background/Aims: Acute liver failure (ALF) in children has been associated with an overall mortality of approximately 70% in the pretransplant era and 50-80% survival in those undergoing orthotopic liver transplantation. There is currently no system for staging severity of ALF in children. The aim of this study was to characterize pediatric ALF in a tertiary hospital and to derive a scoring system to stratify severity of ALF based on predicted mortality.
Journal of Clinical Medicine, 2022
Objectives: In children, acute liver failure (ALF) is a severe condition with high mortality. As some patients need liver transplantation (LT), it is essential to predict the fatal evolution and to refer them early for LT if needed. Our study aimed to evaluate the prognostic criteria and scores for assessing the outcome in children with ALF. Methods: Data of 161 children with ALF (54.66% female, mean age 7.66 ± 6.18 years) were analyzed based on final evolution (32.91% with fatal evolution or LT) and etiology. We calculated on the first day of hospitalization the PELD score (109 children), MELD, and MELD-Na score (52 children), and King’s College Criteria (KCC) for all patients. The Nazer prognostic index and Wilson index for predicting mortality were calculated for nine patients with ALF in Wilson’s disease (WD). Results: PELD, MELD, and MELD-Na scores were significantly higher in patients with fatal evolution (21.04 ± 13.28 vs. 13.99 ± 10.07, p = 0.0023; 36.20 ± 19.51 vs. 20.08 ± ...
Journal of Hepatology
Abstract BACKGROUND & AIMS: Acute-on Chronic Liver Failure (ACLF) is a frequent syndrome (30% prevalence) characterized by acute decompensation of cirrhosis, organ failure(s) and high short-term mortality. This study develops and validates a specific prognostic score for ACLF patients. METHODS: Data from 1,349 patients included in the CANONIC study were used. First, a simplified organ function scoring system (CLIF-Consortium Organ Failure score, CLIF-C OFs) to diagnose ACLF was developed using data from all patients. Subsequently, in 275 patients with ACLF, CLIF-C OFs and two other independent predictors of mortality (age and white-cell count) were combined to develop a specific prognostic score for ACLF (CLIF CONSORTIUM score for ACLF, CLIF-C ACLFs). Concordance index (C-index) was used to compare the discrimination abilities of CLIF-C ACLFs, MELD (MELDs), MELD-Sodium (MELD-Nas) and Child-Pugh (CPs) scores. CLIF-C ACLFs was validated in an external cohort and assessed for sequentia...
Egyptian Liver Journal, 2022
Background Acute-on-chronic liver failure (ACLF) is a distinct syndrome associated with high short-term mortality. Early identification of patients at high risk is essential to determine emergency for transplantation and decide and prioritize the need for intensive care unit (ICU). We aimed to evaluate the performance of the different prognostic scores in the prediction of in-hospital mortality in patients with ACLF. A total of 249 patients with ACLF were included and followed till discharge from the hospital. Univariate and Cox regression analyses were used to assess the performance of liver-specific (Child-Pugh and MELD) and ACLF prognostic scores (CLIF-C OF, CLIF-SOFA, CLIF-C AD, CLIF-C ACLF) in the prediction of in-hospital mortality. Results Patients were mostly males (71.1%) with a mean age of 53.9 ± 12.8 years. The etiology of pre-existing liver disease was HCV in 57.8%. Sepsis was the most common precipitating factor (49.8%) and the mortality rate was 74.3%. In univariate an...
Clinics and Research in Hepatology and Gastroenterology, 2014
Background and aims: To evaluate risk of mortality in children with intestinal failure associated liver disease (IFALD) compared with other liver disease using two validated scores. Methods: Sixty-seven children listed for transplant were studied: cholestatic liver disease (CLDn23); liver disease secondary to other processes (LDsec n11); (IFALDn22), acute liver failure (ALFn11). Paediatric Hepatology Score (PHD) score and Pediatric end-stage liver disease score (PELD) were evaluated by Receiver Operating Curves (ROC), proportional hazards regression. Results: The highest PHD and PELD scores were found in ALF; the lowest in LDsec. Both scores correlated well in identifying waiting list (WL) mortality in patients with CLD and ALF, but not in those with IFALD where PELD scores were lower. Cox proportional hazard regression of time spent on the waiting list prior to death or transplant/delisting showed significant associations with PHD (P = 0.006) and PELD (P = 0.008). WL mortality was strongly predicted by disease group (6/8 deaths in IFALD). ROC analysis of all data showed that a PHD score greater than 15.5 had sensitivity of 87.5% and specificity of 81% for waiting list mortality (P < 0.001); PELD greater than 8 had a sensitivity of 87.5% and specificity of 40%. Neither PHD nor PELD scores correlated with post-transplant mortality.
Pakistan Journal of Medical Sciences, 2012
Objective: To compare the Model for End-Stage Liver Disease (MELD) and The King's College Hospital Criteria (KCH) as early clinical prognostic indicators in patients with Acute Liver Failure. Methodology: This descriptive Case series study was conducted in emergency medical wards of Liaquat University Hospital Jamshoro and Hyderabad from February 2008 to July 2010. This study included 76 consecutive patients with ALF defined as onset of hepatic encephalopathy occurring within 12 weeks of onset of jaundice. The patients using sedatives, anticoagulants or if any evidence of chronic liver disease were excluded. Laboratory workup was done from laboratory of University. MELD score of ≥33 and presence of positive criteria for KCH category were taken as a bad prognostic indicator. The primary end point was death during hospital stay. Continuous variables were computed as mean ± standard deviation (SD) and categorical variables as frequency and percentage. Results: Out of 76 patients 49 were male (64.47%) and 27 (35.53%) female. The mean age of patients was 24.62 ± 10.3. Out of 76 patients a total of 59 patients (77.63%) died during the study period. The KCH criteria cut off point was reached in a total of 63 patients (out of 76) of which 50 patients died. The MELD criteria cut off point (MELD Score >33) was reached in 49 patients (out of 76) of which 46 eventually died.KCH predicted outcome with the sensitivity of 80% and Positive predictive value (PPV) of 89% (p=0.001). MELD predicted outcome with sensitivity of 82.4% and Positive predictive value (PPV) of 94%(p=0.001).Viral hepatitis B was the most common cause of Acute Liver Failure. Conclusion: Both criteria are good predictors of the outcome in acute liver failure.