pCLIF-SOFA is a reliable outcome prognostication score of critically ill children with cirrhosis: an ESPNIC multicentre study

Abstract

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 day-60 after PICU admission. On multivariable analysis, pCLIF-SOFA was the only predictor of mortality at day-28 and of composite outcome. Both pCLIF-SOFA and ACLF were independently associated with emergent liver transplantation. The pCLIF-SOFA score higher than 9 well predicted a 28-day mortality with a sensitivity of 87.8% and a specificity of 77.3%. A pCLIF-SOFA score higher than 7 was independently associated with liver transplantation on day-60. Stage 3 AKI assessed with KDIGO classification was significantly associated with 28-day mortality. Conclusions Half of critically ill cirrhotic children admitted to PICU either died or were transplanted within the initial 28-day period. On admission pCLIF-SOFA score accurately identify patients transplanted at day-28 and day-60 to those alive without LT and is associated with 28-day mortality and composite outcome at day-60.

Acute-on-chronic liver failure before liver transplantation does not impact post-transplant survival in children with biliary atresia

“…Several recent studies have identified that other scoring systems, which include detailed characterization of organ failures in children with cirrhosis, are more successful in predicting mortality in children when compared with MELD/PELD. [5,19] Specifically, the pediatric chronic liver failure sequential organ failure assessment score, which has specific criteria that define degrees of organ failure based on laboratory values, has an improved sensitivity (94.9%) and specificity (91.5%) in predicting 28-day mortality as compared with PELD. [5,19] Mataya et al [11] suggest a policy change for pediatric patients to "stratify children within the Status 1B priority group and prioritize those with ACLF over non-ACLF".…”

Accuracy of SIRS, age-adapted pSOFA, and quick SOFA scoring systems for predicting outcomes in paediatric patients with sepsis: a meta-analysis

“…Eleven studies reported information on the predictive capability of age-adapted pSOFA scores, 8e18 six studies reported on SIRS, 1,8,13,17e19 and four studies reported on qSOFA. 13,14,19,20,21 Concerning geographic distribution, five studies were conducted in China, 8,11,12,14,18 two in the UK 19,20 and South Korea, 22,23 one multicentric (Canada, USA and Uganda), 1 and one each was conducted in India, 9 Egypt, 10 Australia, 13 Europe, 15 Iran, 16 and Indonesia 17 (Table 1). The description of scoring system is shown in Supplementary Table 1.…”

Paediatric Acute-on-Chronic Liver Failure: A Review of Current Evidence in Children

“…19 Other studies have also found that ACLF accounted for nearly 18% of patients with liver cirrhosis admitted to the paediatric intensive care unit. 20 In the adult population, the incidence of ACLF similarly differs based on the definition used. A study of 72,316 patients with decompensated cirrhosis has shown that the prevalence of ACLF was 26.4% and 9.8% based on the European and North American definitions, respectively.…”

“…30 Claude et al also showed that a pCLIF-SOFA score of >9 was predictive of mortality within 28 days with a sensitivity of 87.8% and a specificity of 77.3%, while a pCLIF-SOFA score of >7 was associated with increased odds of liver transplantation on day-60. 20 Lal et al 24 evaluated the APASL ACLF Research Consortium (AARC) acute-on-chronic liver failure (AARC-ACLF) score and its paediatricadapted version (Table 2) in prognosticating ACLF in children. The authors found that AARC-ACLF and CLIF-SOFA scores were superior to other prognostic scores in paediatric ACLF, and paediatric modifications of AARC-ACLF and CLIF-SOFA did not perform better than their original scores, all having AUROC of greater than 0.9 for predicting poor outcome in paediatric ACLF.…”