Pediatric End-stage Liver Disease Score Research Articles

Prognostic markers in hepatitis A-related pediatric acute liver failure and validation of the Peds-hepatitis A virus prognostic model.

Hepatitis A virus (HAV) is the commonest cause for pediatric acute liver failure (PALF) in India. The objective of the study was to identify the predictors of mortality and to evaluate the utility of Peds-HAV model in a cohort of non-LT HAV-PALF. The study included HAV-related PALF from two non-transplant centers. The predictors of outcome were identified by univariate analysis followed by Cox regression analysis. The prognostic accuracy of Peds-HAV model, King's College Hospital (KCH) criteria and pediatric end-stage liver disease score (PELD) were evaluated. As many as 140 children with PALF were included, of whom 96 (68.6%) children had HAV-PALF. On Cox regression analysis, international normalized ratio (INR) (p < 0.001), jaundice to encephalopathy (JE) interval (p < 0.001) and hepatic encephalopathy (HE) grade 3/4 (p = 0.01) were independent predictors of mortality. The mortality rates were 0% (0/42), 14.3% (3/21), 60% (9/15) and 94.4% (17/18) when none, 1, 2 or 3 criteria of the Peds-HAV were met, respectively. Peds-HAV model at a listing cut-off of ≥ 2 criteria predicted death with 89.7% sensitivity and 89.6% specificity. In contrast, KCH criteria had a lower sensitivity of 62.1%. PELD score had a sensitivity of 89.7% and specificity of 85.1% at a cut-off of 30. The overall prognostic accuracy of Peds-HAV model (89.6%) was higher than those of KCH (83.3%) and PELD (86.5%). INR, HE grade and JE interval were independent predictors of mortality. The study provides an external validation of Peds-HAV model as a prognostic score in HAV-PALF. Not applicable as this is a retrospective study.

CHALF Score: A Novel Tool to Rapidly Risk Stratify Children in Need of Liver Transplant Evaluation During Acute Liver Failure.

Pediatric acute liver failure (PALF) can require emergent liver transplantation (LT, >25%) or lead to death (~15%). Existing models cannot predict clinical trajectory or survival with native liver (SNL). We aimed to create a predictive model for PALF clinical outcomes based on admission variables. A retrospective, single-center PALF cohort (April 2003 to January 2022) was identified using International Classification of Disease codes, selected using National Institutes of Health PALF Study Group (PALFSG) criteria, and grouped by clinical outcome (SNL, LT, or death). Significant admission variables were advanced for feature selection using least absolute shrinkage and selection operator regression with bootstrapping (5000×). A predictive model of SNL versus LT or death was created using logistic regression and validated using PALFSG data. Our single-center cohort included 147 patients (58% SNL, 32% LT, 10% expired), while the PALFSG validation cohort included 492 patients (50% SNL, 35% LT, 15% expired). Admission variables associated with SNL included albumin (odds ratio [OR], 16; P < 0.01), ammonia (OR, 2.37; P < 0.01), and total bilirubin (OR, 2.25; P < 0.001). A model using these variables predicted SNL versus LT or death with high accuracy (accuracy [0.75 training, 0.70 validation], area under the curve [0.83 training, 0.78 validation]). A scaled score (CHLA-acute liver failure score) was created that predicted SNL versus LT or death with greater accuracy (C statistic 0.83) than Pediatric End-Stage Liver Disease (C statistic 0.76) and admission liver injury unit (C statistic 0.76) scores. The CHLA-acute liver failure score predicts SNL versus LT or mortality in PALF using admission laboratories with high accuracy. This novel, externally validated model offers an objective guide for urgent referral to a pediatric LT center.

Primary liver transplantation vs transplant after Kasai portoenterostomy in children with biliary atresia: A retrospective Brazilian single-center cohort.

Biliary atresia (BA) is the most common indication for pediatric liver trans plantation, although portoenterostomy is usually performed first. However, due to the high failure rate of portoenterostomy, liver transplantation has been advocated as the primary procedure for patients with BA. It is still unclear if a previous portoenterostomy has a negative impact on liver transplantation outcomes. To investigate the effect of prior portoenterostomy in infants un dergoing liver transplantation for BA. This was a retrospective cohort study of 42 pediatric patients with BA who underwent primary liver transplantation from 2013 to 2023 at a single tertiary center in Brazil. Patients with BA were divided into two groups: Those under going primary liver transplantation without portoenterostomy and those undergoing liver transplantation with prior portoenterostomy. Continuous variables were compared using the Student's t-test or the Kruskal-Wallis test, and categorical variables were compared using the χ2 or Fisher's exact test, as appropriate. Multivariable Cox regression analysis was performed to determine risk factors for portal vein thrombosis. Patient and graft survival analyses were conducted with the Kaplan-Meier product-limit estimator, and patient subgroups were compared using the two-sided log-rank test. Forty-two patients were included in the study (25 [60%] girls), 23 undergoing liver transplantation without prior portoenterostomy, and 19 undergoing liver transplantation with prior portoenterostomy. Patients with prior portoenterostomy were older (12 vs 8 months; P = 0.02) at the time of liver transplantation and had lower Pediatric End-Stage Liver Disease scores (13.2 vs 21.4; P = 0.01). The majority of the patients (35/42, 83%) underwent living-donor liver transplantation. The group of patients without prior portoenterostomy appeared to have a higher incidence of portal vein thrombosis (39 vs 11%), but this result did not reach statistical significance. Prior portoenterostomy was not a protective factor against portal vein thrombosis in the multivariable analysis after adjusting for age at liver transplantation, graft-to-recipient weight ratio, and use of vascular grafts. Finally, the groups did not significantly differ in terms of post-transplant survival. In our study, prior portoenterostomy did not significantly affect the outcomes of liver transplantation.

Pre-operative Serum Albumin Predicts Native Liver Survival in Biliary Atresia.

To predict native liver survival (NLS) after Kasai portoenterostomy (KP) for biliary atresia (BA) using pre-operative clinical data. Pre-operative data were collected from 29 patients with BA who underwent KP at our department between 1989 and 2017 and were analysed including serum albumin, bilirubin, prothrombin time-international normalised ratio, body height, body weight, age at KP, paediatric end-stage liver disease score calculated using the pre-operative data and the period of NLS. The 10-year NLS rate of all patients was 51%. A multivariate analysis revealed that among all factors, the pre-KP serum albumin level was the only independent predictor of NLS (P = 0.04, hazard ratio = 0.269, 95% confidence interval = 0.077-0.934). The area under the receiver operating characteristic curve for NLS, determined using pre-KP serum albumin was 0.760 and 3.75 mg/dl was selected as the cut-off value. There was a significant difference in NLS between patients with high (≥3.8 mg/dl) and low (≤3.7 mg/dl) pre-KP serum albumin (90.0% vs. 31.5%, P < 0.01). Decreased pre-KP serum albumin may reflect not only functional impairment of the liver, but also the inflammatory process, which is hypothesized to occur during its advancement. The pre-KP serum albumin level may be a good prognostic factor for NLS in post-KP BA patients.

Therapeutic Plasma Exchange in Children With Acute and Acuteon-Chronic Liver Failure: A Single-Center Experience.

Acute liver failure is a life-threatening condition that may result in death if liver transplant is not performed. The aim of our study was to evaluate patients with acute liver failure or acute-on-chronic liver failure who were followed and treated with therapeutic plasma exchange in a pediatric intensive care unit until they achieved clinical recovery or underwent liver transplant. In this retrospective, singlecenter study, we included patients with acute liver failure or acute-on-chronic liver failure who received therapeutic plasma exchange between April 2020 and December 2021. Clinical findings, laboratory findings, extracorporeal therapies, Pediatric Risk of Mortality III and liver injury unit scores and pretherapy and posttherapy hepatic encephalopathy scores, Model for End-Stage Liver Disease score, and Pediatric End-Stage Liver Disease score were retrospectively analyzed. Nineteen patients were included in the study. One patient was excluded because of positivity for COVID-19. The mean age of children was 62.06 months, ranging from 5 months to 16 years (12 boys, 6 girls). Thirteen patients (72.2%) had acute liver failure, and 5 patients (27.8%) had acute-on-chronic liver failure. No significant difference was shown for mean liver injury unit score (P = .673) and Pediatric Logistic Organ Dysfunction score (P = .168) between patients who died and patients who received treatment at the inpatient clinic and transplant center. However, Pediatric Risk of Mortality score and the mean Model for End-Stage Liver Disease/Pediatric End-Stage Liver Disease scores before therapeutic plasma exchange and after therapeutic plasma exchange (after 3 consecutive days of treatment) were statistically significant (P = .001 and P = .004). Therapeutic plasma exchange may assist bridge to liver transplant or assist with spontaneous recovery of liver failure in pediatric patients with acute liver failure or acute-on-chronic liver failure.

Development and validation of a nomogram to predict allograft survival after pediatric liver transplantation.

Liver transplantation is the main treatment for cholestatic liver disease and some metabolic liver diseases in children. However, no accurate prediction model to determine the survival probability of grafts prior to surgery exists. This study aimed to develop an effective prognostic model for allograft survival after pediatric liver transplantation. This retrospective cohort study included 2032 patients who underwent pediatric liver transplantation between January 1, 2006, and January 1, 2020. A nomogram was developed using Cox regression and validated based on bootstrap sampling. Predictive and discriminatory accuracies were determined using the concordance index and visualized using calibration curves; net benefits were calculated for model comparison. An online Shiny application was developed for easy access to the model. Multivariable analysis demonstrated that preoperative diagnosis, recipient age, body weight, graft type, preoperative total bilirubin, interleukin-1β, portal venous blood flow direction, spleen thickness, and the presence of heart disease and cholangitis were independent factors for survival, all of which were selected in the nomogram. Calibration of the nomogram indicated that the 1-, 3-, and 5-year predicted survival rates agreed with the actual survival rate. The concordance indices for graft survival at 1, 3, and 5 years were 0.776, 0.757, and 0.753, respectively, which were significantly higher than those of the Pediatric End-Stage Liver Disease and Child-Pugh scoring systems. The allograft dysfunction risk of a recipient could be easily predicted using the following URL: https://aspelt.shinyapps.io/ASPELT/ / CONCLUSION: The allograft survival after pediatric liver transplantation (ASPELT) score model can effectively predict the graft survival rate after liver transplantation in children, providing a simple and convenient evaluation method for clinicians and patients.

Disseminated Intravascular Coagulation as a Risk Factor for Clinical Outcome After Liver Transplantation in Pediatric Patients With Kasai Portoenterostomy Failure

Disseminated intravascular coagulation (DIC) is a serious complication in critically ill pediatric patients. This study aimed to evaluate the association between pretransplant DIC and perioperative clinical outcomes of liver transplantation (LT) in pediatric patients with Kasai portoenterostomy (KPE) failure. We enrolled pediatric patients who received LT after KPE failure between January 2005 and April 2021. We retrospectively reviewed the electronic medical records of included patients and evaluated the presence of DIC using the International Society on Thrombosis and Hemostasis (ISTH) criteria and association with perioperative clinical outcome. The study included 106 patients. Their median age and body weight at the time of pediatric intensive care unit (PICU) admission were 28.7 months and 9.25 kg, respectively. Of these patients, 23 had undergone pretransplant DIC (22%). Patients with pretransplant DIC required significantly more blood transfusions during operation. They had significantly higher serum lactate levels, pediatric end-stage liver disease scores, pediatric risk for mortality III (PRISM III) scores, longer durations of mechanical ventilator support, and longer PICU stays (all P < .05). The presence of pretransplant DIC in pediatric patients requiring LT after KPE failure was associated with poor clinical outcomes, which required more intensive and meticulous supportive management in the perioperative period of LT. DIC would be a promising prognostic factor in these patients.

Risk factors for myocardial injury during living donor liver transplantation in pediatric patients with biliary atresia.

Cold ischemia-reperfusion of the liver is an inevitable occurrence in liver transplantation that may also cause damage to the heart. Perioperative myocardial injury during liver transplantation can increase the incidence of postoperative mortality, but there is little research on the incidence of myocardial injury in children who undergo living donor liver transplantation (LDLT). Therefore, this study mainly explores the independent risk factors for myocardial injury in children who undergo LDLT. To analyze the data of children who underwent LDLT to determine the risk factors for intraoperative myocardial injury. We retrospectively analyzed the inpatient records of pediatric patients who underwent LDLT in Tianjin First Central Hospital from January 1, 2020, to January 31, 2022. Recipient-related data and donor-related data were collected. The patients were divided into a myocardial injury group and a nonmyocardial injury group according to the value of the serum cardiac troponin I at the end of surgery for analysis. Univariate analysis and multivariate logistic regression were used to evaluate the risk factors for myocardial injury during LDLT in pediatric patients. A total of 302 patients met the inclusion criteria. The myocardial injury group had 142 individuals (47%), and the nonmyocardial injury group included 160 patients (53%). Age, height, and weight were significantly lower in the myocardial injury group (P < 0.001). The pediatric end-stage liver disease (PELD) score, total bilirubin, and international standardized ratio were significantly higher in the myocardial injury group (P < 0.001). The mean arterial pressure, lactate, hemoglobin before reperfusion, duration of the anhepatic phase, cold ischemic time, incidence of postreperfusion syndrome (PRS), and fresh frozen plasma transfusion were significantly different between the two groups (P < 0.05). The postoperative intensive care unit stay and peak total bilirubin values in the first 5 d after LDLT were significantly higher in the myocardial injury group (P < 0.05). The pediatric patients with biliary atresia in the nonmyocardial injury group who underwent LDLT had a considerably higher one-year survival rate than those in the myocardial injury group (P = 0.015). Multivariate logistic regression revealed the following independent risk factors for myocardial injury: a high PELD score [odds ratio (OR) = 1.065, 95% confidence interval (CI): 1.013-1.121; P = 0.014], a long duration of the anhepatic phase (OR = 1.021, 95%CI: 1.003-1.040; P = 0.025), and the occurrence of intraoperative PRS (OR = 1.966, 95%CI: 1.111-3.480; P = 0.020). A high PELD score, a long anhepatic phase duration, and the occurrence of intraoperative PRS were independent risk factors for myocardial injury during LDLT in pediatric patients with biliary atresia.

Impact of patent foramen ovale on short-term outcomes in children with biliary atresia undergoing living donor liver transplantation: a retrospective cohort study

ObjectiveTo investigate the impact of patent foramen ovale (PFO) on the short-term outcomes of living donor liver transplantation (LDLT) in children with biliary atresia.MethodsWith the approval of the hospital ethics committee, 304 children with biliary atresia who underwent LDLT in our center from January 2020 to December 2021 were enrolled. According to the results of echocardiography before the operation, the subjects were divided into the PFO group (n = 73) and the NoPFO group (n = 231). The baseline characteristics; intraoperative recipient-related data and donor-related data; incidence of postreperfusion syndrome (PRS); postoperative mechanical ventilation time; ICU stay duration; postoperative hospital stay duration; liver function index; incidences of postoperative complications including acute renal injury (AKI), graft dysfunction, hepatic artery thrombosis (HAT) and portal vein thrombosis (PVT); and one-year survival rate were compared between the two groups.ResultsThe median age in the PFO group was 6 months and that in the NoPFO group was 9 months (P < 0.001), and the median height (65 cm) and weight (6.5 kg) in the PFO group were significantly lower than those in the NoPFO group (68 cm, 8.0 kg) (P < 0.001). The preoperative total bilirubin level (247 vs. 202 umol/L, P = 0.007) and pediatric end-stage liver disease (PELD) score (21 vs. 16, P = 0.001) in the PFO group were higher than those in the NoPFO group. There were no significant differences in the intraoperative PRS incidence (46.6% vs. 42.4%, P = 0.533 ), postoperative mechanical ventilation time (184 vs. 220 min, P = 0.533), ICU stay duration (3.0 vs. 2.5 d, P = 0.267), postoperative hospital stay duration (22 vs. 21 d, P = 0.138), AKI incidence (19.2% vs. 24.7%, P = 0.333), graft dysfunction incidence (11.0% vs. 12.6%, P = 0.716), HAT incidence (5.5% vs. 4.8%, P = 0.762), PVT incidence (2.7% vs. 2.2%, P = 0.675) or one-year survival rate (94.5% vs. 95.7%, P = 0.929) between the two groups.ConclusionThe presence of PFO has no negative impact on short-term outcomes in children with biliary atresia after LDLT.

Cost-effectiveness analysis of liver transplantation in biliary atresia according to the severity of end-stage liver disease

BackgroundTiming for liver transplantation (LT) in biliary atresia (BA) children with end-stage liver disease (ESLD) is associated with all-cause mortality. The cut-off value of pediatric end-stage liver disease (PELD) score for LT consideration varies across institutions. We aimed to determine the cost-effectiveness of LT to prevent death among BA children registered on the waiting list with different severities of ESLD.MethodsSubjects were BA children aged < 12 years at a transplant center between 2010 and 2021. A decision tree was developed for cost-effectiveness analysis from a hospital perspective to compare all-cause death between patients initially registered with a low PELD score (< 15) and a high PELD score (≥ 15). Each patient’s direct medical cost was retrieved from the beginning of registration until 5 years after LT, adjusted with an inflation rate to 2022 Thai Baht (THB).ResultsAmong 176 children, 138 (78.4%) were initially registered with the high PELD score. The cost and mortality rate of the low PELD score group (THB1,413,424 or USD41,904 per patient and 31.6% mortality) were less than the high PELD score group (THB1,781,180 or USD52,807 per patient and 47.9% mortality), demonstrating the incremental cost-effectiveness ratio (ICER) of THB2,259,717 or USD66,994 per death prevented. The cost of early post-operative admission had the highest effect on the ICER. Considering the break-even analysis, cost among children initially registered at the low PELD score was also less expensive over time.ConclusionsRegistration for LT at PELD score < 15 was more cost-effective to prevent death among BA children with ESLD.

Preoperative low muscle mass and early postoperative outcomes in children undergoing living donor liver transplantation: A retrospective study.

Low skeletal muscle mass may develop in children with end-stage liver disease, affecting postoperative outcomes. We retrospectively investigated whether preoperative low muscle mass was associated with early postoperative outcomes in pediatric patients undergoing living donor liver transplantation (LDLT). Electronic medical records of children (age below 12y) who underwent LDLT between February 1, 2007, and January 31, 2018, were reviewed. The cross-sectional areas of psoas, quadratus lumborum, and erector spinae muscles at the level of fourth-fifth lumbar intervertebral disks were measured using abdominal CT images, divided by the square of the height and were added to obtain the total skeletal muscle index (TSMI). The patients were divided into two groups according to the median TSMI in the second quintile (1859.1mm 2 /m 2 ). Complications in the early postoperative period (within 30d after surgery) classified as Clavien-Dindo grade 3 or higher were considered major complications. Logistic regression analyses were performed to determine the association between preoperative low muscle mass and early postoperative outcomes. In the study population of 123 patients (median age, 14mo; range, 8-38mo) who underwent LDLT, 29% and 71% were classified in the low (mean TSMI, 1642.5 ± 187.0mm 2 /m 2 ) and high (mean TSMI 2188.1 ± 273.5mm 2 /m 2 ) muscle mass groups, respectively. The rates of major complications, mechanical ventilation >96 hours, intensive care unit stay >14 days, hospital stay >30 days, and in-hospital mortality were not significantly different between the 2 groups. Additionally, adverse outcomes according to pediatric end-stage liver disease scores and sex were not significantly different between the 2 groups. In conclusion, preoperative low muscle mass defined by TSMI was not associated with early postoperative outcomes in pediatric patients undergoing LDLT.

Evaluation of Vitamin D Levels in Children With Liver Transplant.

Vitamin D deficiency is common in pediatric chronic liver disease despite oral replacement. We evaluated vitamin D deficiency before and after liver transplant and the relationship between posttransplant and pretransplant vitamin D deficiency and graft rejection. Pediatric recipients with chronic liver disease (N =138) were divided into 4 groups: cholestatic liver diseases, cirrhosis, metabolic disorders, and acute liver failure. Pretransplant and posttransplant vitamin D levels, liver function tests, Pediatric End-Stage Liver Disease scores, rejection activity index scores by graft liver biopsy, and posttransplant patient survival were recorded. There were 62 (45%) female and 76 (55%) male participants (mean transplant age, 6.1 ± 5.6 years). Pretransplant mean available vitamin D of 90 patients was 25.2 ± 20.9 ng/mL, with 36 (40%) within reference range. Posttransplant level for 109 patients was 27.3 ± 18 ng/mL, with 64 (58.7%) within reference range. Pretransplant and posttransplant levels were available for 61 patients, and mean pretransplant levels were lower than posttransplant levels (23.7 ± 19.3 vs 28.3 ± 16.9 ng/mL; P = .01). Patients with cholestatic liver disease had lower pretransplant vitamin D levels (P = .04), which disappeared after transplant. Pretransplant vitamin D levels were positively correlated with serum albumin levels (r = 0.20) in all patients and negatively correlated with total/direct bilirubin (r = 0.29 and r = -0.30) in those with liver diseases and cirrhosis. No correlations were found between pretransplant vitamin D levels and Pediatric End-Stage Liver Disease scores, rejection activity index scores, and posttransplant mortality. Vitamin D deficiency is prevalent in pediatric chronic liver disease before and after transplant, especially for cholestatic liver diseases. However, no association between vitamin D levels and liver graft rejection or patient survival was noted. We recommend close monitoring and individualized vitamin D supplementation before and after liver transplant.

Comparison of Liver Transplant Outcomes in Biliary Atresia Patients, Considering Whether They Underwent the Kasai Procedure Beforehand: A Single Center Analysis of 72 Patients

This study examines the results of liver transplantation (LT) in patients with biliary atresia, considering whether they underwent the Kasai procedure beforehand. LT and determine postoperative and long-term graft outcomes. This single-center, retrospective study included 72 pediatric patients diagnosed with postpartum biliary atresia who underwent LT between 2010 and 2022. We included patients who underwent LT either after or without the Kasai procedure and compared the demographic data of the patients with various factors, such as the Pediatric End-Stage Liver Disease scores and laboratory values. The study included 72 patients, with 39 of them being female (54.2%) and 33 of them being male (45.8%). Of the 72 patients in the study, 47 (65.3%) had undergone the Kasai procedure, and 25 (34.7%) had not. The preoperative and postoperative month 1 bilirubin values were lower in patients who underwent the Kasai procedure and were higher in postoperative months 3 and 6. Preoperative bilirubin values, postoperative month 3 bilirubin values, and preoperative albumin values were higher in patients who developed mortality (P < .05). Cold ischemia time was longer in patients who developed mortality (P < .05). Our study showed a higher mortality rate in patients who underwent the Kasai procedure. The results also showed that LT was more effective in children, as patients with Kasai had higher mean bilirubin values and higher preoperative albumin values than patients without Kasai.

The Factors Affecting Mortality After Pediatric Liver Transplantation and Long-Term Survival Outcomes: A Single Center Experience

BackgroundLiver transplantation (LT) is a treatment modality in the pediatric population for several diseases like biliary atresia, metabolic liver disease, hepatoblastoma, and so on. According to the Organ Procurement and Transplantation Network, 5-year survival was reported as 85.4% to 93.5% by age after pediatric liver transplantation (PLT). This study aimed to evaluate our single-center experience of PLT by analyzing long-term results, comparing the outcomes with the literature, and identifying predictors of patient survival. MethodsThe data of 40 patients who underwent LT at <18 years of age between June 2015 and June 2021 were studied retrospectively. Recipient characteristics such as age, sex, etiology of liver disease follow-up time, postoperative vascular and biliary complications, and donor characteristics were evaluated. ResultsThere were 20 (50%) girls and 20 (50%) boys, and the median age was 42 (IQR = 9-117) months. The most common indications of LT were biliary disorders (45%). A whole liver graft was used in 7 (17%), a right lobe graft in 9 (23%), a left lobe graft in 4 (10%), and a left lateral lobe graft in 20 (50%) of the recipients. The 1-, 3-, 5-, and 7-year survival rates were 85%, 82.1%, 82.1%, and 82.1%, respectively. The multivariate survival analysis revealed that the pediatric end-stage liver disease score, hepatic artery thrombosis, and portal vein thrombosis are associated with overall mortality. ConclusionIn conclusion, our long-term survival is similar to the literature, with satisfactory results. However, reducing the vascular complication rates can provide superior results on PLT.

Socio-economic issues in the diagnostic approach and decision to treat of a child with familial cholestasis

Objectives. The diagnostic approach in familial cholestasis cases involves using expensive investigations. The need for complex therapeutic resources is predictable. Material and methods. We present the case of a 3 years 5 months old boy diagnosed with cholestasis and liver cirrhosis, admitted in the Pediatrics Department of “Grigore Alexandrescu” Children’s Hospital for evaluation in order to check the criteria for liver transplantation. Outcomes. The patient is known to our clinic since he was 1 year 3 months; he was admitted at that time for green jaundice. He comes from a family with nine children, low socio-economic status and low education. The first four children have a different father, two of them were stillborn. The next five come from a consanguineous union (the father is maternal uncle of the mother): three (the patient included) presented with the same symptoms, two of them died before 3 years of age with liver cirrhosis. In time, infectious causes, autoimmune, neoplastic and metabolic disease were excluded. The liver biopsy describes cholestasis and fibrosis. Alagille syndrome and progressive familial intrahepatic cholestasis are still debated, but the genetic testing necessary for a positive diagnosis are not financially available to the family. The patient presents with failure to thrive, developmental delay, intense jaundice and pruritus, hippocratic fingers, dyspnea, enlarged abdomen with visible collateral circulation, significant hepatosplenomegaly. The lab tests show anemia, thrombocytopenia, liver cytolysis, normal gamma-glutamyl transferase, low prothrombin activity, normal renal function, no inflammatory syndrome, increased ammonia level, very high bile acids level. The upper endoscopy describes first degree esophageal varices and portal hypertensive gastropathy. A PELD (Pediatric End-Stage Liver Disease) score is calculated – 13.6 (76.3% one year survival rate on the transplant list, 90.9% one year survival rate after liver transplantation). The patient is enrolled on the transplant waiting list provided we would expect suboptimal results considering the developmental delay and difficult post-transplant monitoring. Conclusions. The low education level of the family correlated with a postponed positive diagnosis due to financial reasons and the lack of genetic advice already lead to the demise of four brothers. In this setting, the prognosis of the patient is very likely to be poor.

Fluid balance in pediatric postoperative liver transplant recipients.

Positive fluid balance (FB) is associated with poor outcomes in critically ill children but has not been studied in pediatric liver transplant (LT) recipients. Our goal is to investigate the relationship between postoperative FB and outcomes in pediatric LT recipients. We performed a retrospective cohort study of first-time pediatric LT recipients at a quaternary care children's hospital. Patients were stratified into three groups based on their FB in the first 72h postoperatively: <10%, 10-20%, and > 20%. Outcomes were pediatric intensive care unit (PICU) and hospital length of stay, ventilator-free days (VFD) at 28 days, day 3 severe acute kidney injury, and postoperative complications. Multivariate analyses were adjusted for age, preoperative admission status, and Pediatric Risk of Mortality (PRISM)-III score. We included 129 patients with median PRISM-III score of 9 (interquartile range, IQR 7-15) and calculated Pediatric End-stage Liver Disease score of 15 (IQR 2-23). A total of 37 patients (28.7%) had 10-20% FB, and 26 (20.2%) had >20% FB. Greater than 20% FB was associated with an increased likelihood of an additional PICU day (adjusted incident rate ratio [aIRR] 1.62, 95% CI: 1.18-2.24), an additional hospital day (aIRR 1.39, 95% CI: 1.10-1.77), and lower likelihood of a VFD at 28 days (aIRR 0.85, 95% CI: 0.74-0.97). There were no differences between groups in the likelihood of postoperative complications. In pediatric LT recipients, >20% FB at 72hpostoperatively is associated with increased morbidities, independent of age and severity of illness. Additional studies are needed to explore the impact of fluid management strategies on outcomes.

MELD 3.0 for adolescent liver transplant candidates.

Adolescents constitute a unique waitlist cohort that is distinct from younger children. Model for End-stage Liver Disease (MELD) 3.0, which was developed in an adult population of liver transplant candidates, is planned to replace MELD-Sodium in the current liver allocation system for both adults and adolescents aged 12-17. We evaluated the predictive performance of MELD-Sodium, MELD 3.0, and Pediatric End-stage Liver Disease for 90-day waitlist mortality risk among adolescent liver transplant registrants. New waitlist registrations for primary liver transplants among individuals aged 12-17 and 18-25 for comparison were identified using Organ Procurement and Transplantation Network (OPTN) data from November 17, 2004, to December 31, 2021. The predictive performance of the current and proposed MELD and Pediatric End-stage Liver Disease scores was assessed using Harrell's concordance ( c ) statistic. There were 1238 eligible listings for adolescents aged 12-17 and 1740 young adults aged 18-25. In the adolescent group, 90-day survival was 97.8%, compared with 95.9% in those aged 18-25 (log-rank p = 0.005), with no significant differences when stratified by sex or indication. Among adolescents, increasing MELD 3.0 was associated with an increased hazard of mortality (HR=1.27, 95% CI: 1.18-1.37), and the c -statistic for 90-day waitlist survival using MELD 3.0 was 0.893 compared with 0.871 using MELD-Sodium and 0.852 using Pediatric End-stage Liver Disease. The discriminative ability of MELD 3.0 to rank adolescents according to the risk of death within 90 days was robust. Although MELD 3.0 was initially developed and validated in adults, MELD 3.0 may also improve the prediction of waitlist mortality in adolescents and better represent their urgency for liver transplants.

Effect of Pre-Transplant Covid-19 Exposure on Post-Liver Transplant Clinical Outcomes

BackgroundCOVID-19 has led to an unprecedented global health crisis. This situation caused an immediate reduction in solid organ transplantation activity. This study aimed to present the follow-up results of patients with chronic liver disease who underwent liver transplantation (LT) after a history of COVID-19 infection. MethodsSociodemographic characteristics and clinicopathological data of 474 patients who underwent LT at Inonu University Liver Transplant Institute between March 11, 2020 and March 17, 2022 were prospectively recorded and analyzed retrospectively. Among these, the data of 35 patients with chronic liver disease who were found to be exposed to COVID-19 infection in the pre-LT period were analyzed for this study. ResultsThe median body mass index, Child score, and Model for end-stage liver disease/ Pediatric end-stage liver disease scores of the 35 patients were calculated as 25.1 kg/m2 (IQR: 7.4), 9 points (IQR: 4), and 16 points (IQR: 10), respectively. Graft rejection occurred in 4 patients at a median of 25 days post-transplant. Five patients underwent retransplantation at a median of 25 days post-transplant. The most common cause of retransplantation is early hepatic artery thrombosis. There were 5 deaths during postoperative follow-up. Mortality developed in 5 (14.3%) patients exposed to COVID-19 infection in the pretransplant period, whereas mortality occurred in 56 (12.8%) patients not exposed to COVID-19 infection. There was no statistically significant difference in mortality between the groups (P = .79). ConclusionsThe results of this study showed that exposure to COVID-19 before LT does not affect post-transplant patients and graft survival.

Early relaparotomy following pediatric living-donor liver transplantation: experience in an Indonesian national referral hospital

BackgroundLiving donor liver transplantation (LDLT) remains the only curative treatment for children with end-stage liver disease; however, complications of the procedure are associated with indications for early relaparotomy. Several risk...

Home Parenteral Nutrition for Children: What Are the Factors Indicating Dependence and Mortality?

Parenteral nutrition (PN) in children with short bowel syndrome is crucial and lifesaving. Taking care of such patients requires interprofessional practice and multiple team resource management. Home PN (HPN) usage allows patients and families to live regular lives outside hospitals. We share our experiences for the last two decades and identify the risk factors for complications and mortality. A retrospective study of HPN patients was conducted between January 2000 and February 2022. Medical records of age, body weight, diagnosis, length of residual intestines, HPN period, central line attempts, complications, weaning, and survival were collected and analyzed. The patients were classified as HPN free, HPN dependent, and mortality groups. A total of 25 patients received HPN at our outpatient clinic, and one was excluded for the adult age of disease onset. There were 13 patients (54.1%) who were successfully weaned from HPN until the record-enroled date. The overall mortality rate was 20.8% (five patients). All mortality cases had prolonged cholestasis, Child Class B or C, and a positive Pediatric End-Stage Liver Disease (PELD) score. For HPN dependence, extended resection and multiple central line placement were two significant independent factors. Cholestasis, Child Class B or C, and positive PELD score were the most important risk factors for mortality. The central line-related complication rate was not different in all patient groups. The overall central line infection rate was 1.58 per 1000 catheter days. Caution should be addressed to prevent cholestasis and intestinal failure-associated liver disease during the HPN period, to prevent mortality. By understanding the risks of HPN dependence and mortality, preventive procedures could be addressed earlier.