Liver Failure In Children Research Articles

Study of Acute Liver Failure in Children Using Next Generation Sequencing Technology

To use next generation sequencing (NGS) technology to identify undiagnosed, monogenic diseases in a cohort of children who suffered from acute liver failure (ALF) without an identifiable etiology. We identified 148 under 10years of age admitted to King's College Hospital, London, with ALF of indeterminate etiology between 2000 and 2018. A custom NGS panel of 64 candidate genes known to cause ALF and/or metabolic liver disease was constructed. Targeted sequencing was carried out on 41 children in whom DNA samples were available. Trio exome sequencing was performed on 4 children admitted during 2019. A comparison of the clinical characteristics of those identified with biallelic variants against those without biallelic variants was then made. Homozygous and compound heterozygous variants were identified in 8 out of 41 children (20%) and 4 out of 4 children (100%) in whom targeted and exome sequencing were carried out, respectively. The genes involved were NBAS (3 children); DLD (2 children); and CPT1A, FAH, LARS1, MPV17, NPC1, POLG, SUCLG1, and TWINK (1 each). The 12 children who were identified with biallelic variants were younger at presentation and more likely to die in comparison with those who did not: median age at presentation of 3months and 30months and survival rate 75% and 97%, respectively. NGS was successful in identifying several specific etiologies of ALF. Variants in NBAS and mitochondrial DNA maintenance genes were the most common findings. In the future, a rapid sequencing NGS workflow could help in reaching a timely diagnosis and facilitate clinical decision making in children with ALF.

A STUDY ON ETIOLOGY, CLINICAL FEATURES, MANAGEMENT AND OUTCOME OF ACUTE LIVER FAILURE IN PAEDIATRIC INTENSIVE CARE UNIT OF BURDWAN MEDICAL COLLEGE

Introduction: In the present study, etiology, important clinical features, management and outcome of acute liver failure in children admitted in Pediatric Intensive Care Unit (PICU) of BURDWAN MEDICALCOLLEGE, WB are studied. Aims and objectives:To study different clinical features among those children having acute liver failure. I. To study the management and outcome of those children with acute liver failure in the Pediatric Intensive Care Unit. Material and methods: Pediatric Intensive Care Unit of BURDWAN MEDICALCOLLEGE, WB. 1 Year [December 2019 to November 2020]. Patients from 3months of age to 12years of age are eligible for enrolment if they meet the following criteria. Conclusion: Present study showed that cerebral edema was signicantly associated with mortality whereas AKI and spontaneous bacterial peritonitis were not signicantly related with mortality. Mean TSB and Prothrobin Time were higher but mean serum albumin was lower in those cases that had died.

Acute-on-Chronic Liver Failure in Children: A Single-Center Experience.

Acute-on-chronic liver failure and its outcomes have not yet been evaluated in detail in children. We aimed to evaluate the etiology, acute events, and prognostic factors of acute-on-chronic liver failure in children. Pediatric patients (age 2-18 years) diagnosed with acute-on-chronic liver failure between April 2014 and April 2020 were evaluated retrospectively. Acute-on-chronic liver failure was defined as the presence of acute hepatic insult in previously diagnosed or undiagnosed chronic liver disease causing jaundice (total serum bilirubin ≥5 mg/dL) and coagulopathy (international normalized ratio of ≥2.0) and clinical and/or radiological ascites and/or hepatic encephalopathy within 4 weeks. Acute-on-Chronic Liver Failure Research Consortium and Chronic Liver Failure-Sequential Organ Failure Assessment scores were calculated for patients at first admission and at end of day 5 or before liver transplant. Our study included 29 patients. Underlying chronic liver diseases were mostly autoimmune hepatitis (51.72%) and Wilson disease (27.58%), with flare-ups of these diseases also the most common acute events (48.28% and 27.58%, respectively). Seven patients (24.14%) received liver transplants. At first admission, Acute-on-Chronic Liver Failure Research Consortium and Chronic Liver Failure-Sequential Organ Failure Assessment cut-off scores to predict liver transplant were 7.5 and 6.5; at end of day 5 or before transplant, cut-off scores were 8.5 and 7.5, respectively. The 8.5 cut-off score on day 5 was the most specific and sensitive to predict liver transplant. International normalized ratio cut-off of 3.04 predicted transplant requirement with maximum sensitivity and specificity. Wilson disease and autoimmune hepatitis were the most common underlying chronic and acute events of acute-on-chronic liver failure in children. Although an Acute-on-Chronic Liver Failure Research Consortium score ≥ 8.5 best predicted liver transplant, for patients with scores ≥ 7.5 and being followed in a nontransplant center, patient referral to a transplant center is appropriate.

Etiology of acute liver failure in children

Etiology of acute liver failure in children

Analysis of future liver remnant parameters in children after extended liver resection

To analyze the initial data on future liver remnant volume and its function evaluated by 99mTc-Bromesida hepatobiliary scintigraphy in children with liver tumors. Extended liver resections were performed in 58 patients aged 2 months - 208 months (median 26 months) for various neoplasms. Before hepatectomy, all children underwent contrast-enhanced CT with volumetry and hepatobiliary scintigraphy with 99mTc-Bromezida and subsequent quantitative assessment of its accumulation in the future liver remnant. All consecutive patients eligible for extended liver resection were retrospectively analyzed. The analysis included patients who underwent extended liver resection between June 2017 and March 2021. Among 91 liver resections, 58 (64%) procedures were extended hepatectomies including 2 ALPPS procedures. Median volume of future liver remnant was 44.5% (16.5-91.4), median future liver remnant function - 10.14%/min/m2 (1.8-30). Four patients with adequate liver function had insufficient volume of future liver remnant. Insufficient future liver remnant volume and its appropriate function were observed in 2 patients. Not life-threatening post-resection liver failure developed in 2 patients. Evaluation of future liver remnant function is the most sensitive method to predict post-hepatectomy liver failure in children. The cut off value of future liver remnant volume in children is below 25% and probably below 16.5%. Further data collection and research are warranted to determine significant values. These data will contribute to define the new indications for two-staged hepatectomies in children.

Etiology and Outcome of Acute Liver Failure in Children—The Experience of a Single Tertiary Care Hospital from Romania

Background: Acute liver failure (ALF) is a rare disease, associated with high mortality, despite optimal medical therapy without emergency liver transplantation. Knowing the possible cause of ALF plays a vital role in the management, as the child could benefit from effective specific therapies in emergencies. Methods: We have analyzed the etiology and outcome of ALF in children followed-up in a tertiary care hospital between January 2012–December 2018. The patients were grouped into different age categories: neonates (0–1 month), infants (1–12 months), children (1–14 years), and teenagers (14–18 years). Results: 97 children (46 males, 47.42%, the mean age of 7.66 ± 8.18 years) were admitted with ALF. The most important causes of ALF were in neonates and infants, infections (72.72%), and metabolic disorders (43.47%), in children and adolescents were the toxic causes (60% and 79.41%). The mortality rate was 31.95% (31 patients), mainly in ALF due to infections or metabolic disorders. Conclusions: In neonates and infants, the main causes of ALF were infections and metabolic diseases, while in older children and teenagers, were toxin-induced liver injuries. The mortality among neonates and infants was significantly higher than in other ages. Early recognition and immediate therapeutic intervention could improve the outcome of these patients.

Etiological analysis and short-term efficacy evaluation of acute liver failure in children

Objective: To explore etiology, optimize the search direction of etiology, and short-term efficacy evaluation of acute liver failure in children. Methods: Children with acute liver failure who were diagnosed in the First Affiliated Hospital of Zhengzhou University from November 2011 to November 2019 were collected. Gender difference, age, living environment, etiological factors on the seasonal onset and different etiological profile of short-term prognosis were statistically analyzed. Chi-square test or Fisher's exact probability method was used to compare the rate and the constituent ratio between groups. Results: Among the 236 cases, 114 (48.31%) had unknown etiology and 122 (51.69%) had definite etiology. In the definite etiology group, 38 (16.10%), 27 (11.44%), 22 (9.32%), 15 (6.36%), 17 (7.20%), and 3 (1.27%) cases were related to drugs/toxicants, infection, congenital/vascular/biliary diseases, genetic metabolic diseases, tumor-related, and autoimmune diseases. In addition, the overall etiological distribution did not change statistically with factors such as gender, age, and seasonal onset (P > 0.05), while the difference in different living environments was statistically significant (P < 0.05). Different initial-diagnosed departments had different etiological rates, with liver transplantation department being the highest (78.26%), and pediatrics department the lowest (26.09%). In 122 children with definite etiology, there was no significant difference in gender ratio between the different etiology groups (P>0.05), but there were significant differences in age, living environment and seasonal onset (P < 0.05). Among them, congenital development/vascular/biliary tract and infectivity were the main factors in children < 3 years old, and drug/toxin-related factors were the main factors in children > 3 years old. Most of the rural children had drug/toxin and infection-related etiology, while most of the urban children had tumor and drug/toxin-related etiology. In summer and autumn, drug/toxicant correlation was dominant, while in winter and spring, infection correlation was dominant. In the unknown etiology group, the clinical cure + improvement rate between the medical treatment group and the surgical treatment group was not statistically significant (37.50% vs. 20.00%, P > 0.05), while in the definite etiology group, the above differences were statistically significant (43.96% vs. 83.87%, P < 0.05). Among the different etiology groups, the congenital/vascular/biliary group had the best short-term efficacy (72.72%, 16/22), followed by the drug/toxicity-related group (60.53%, 23/38), and the tumor-related group had the worst (23.53%, 4/17). Conclusion: The definite etiology of acute liver failure in about half of children is still vague. The population distribution of children with definite etiology is related to age, living environment and season, but not to gender. However, the different ages, different living environments, and different seasonal onsets have definite etiology, and the short-term clinical efficacy of the treatment is significantly improved after the definite etiology is identified.

Mutations in NBAS and SCYL1, genetic causes of recurrent liver failure in children: Three case reports and a literature review

Acute liver failure (ALF) in childhood is a life-threatening emergency. ALF is often caused by drug toxicity, autoimmune hepatitis, inherited metabolic diseases, and infections. However, despite thorough investigations, a cause cannot be determined in approximately 50% of cases. Here, we report three cases with recurrent ALF caused by NBAS and SCYL1 pathogenic variants. These patients did not present with any other phenotypic sign usually associated with NBAS and SCYL1 pathogenic variants. Two of them underwent liver transplantation and are healthy without recurrence of ALF. We propose NBAS and SCYL1 genetic analysis in children with unexplained fever-triggered recurrent ALF even without a typical phenotype.

Pediatric chronic liver failure-sequential organ failure assessment score and outcome of acute liver failure in children.

Aim of the studyLiver transplantation remains the only definitive treatment for children with acute liver failure proven to have irreversible liver injury. Many prognostic models have been used for outcome prediction in pediatric acute liver failure to select patients in a real need of liver transplantation, but unfortunately all have shown inconsistent reproducibility and prognostic accuracy. The aim of this study was to evaluate the pediatric chronic liver failure sequential organ failure assessment (pCLIF-SOFA) score as a predictor of pediatric acute liver failure outcome.Material and methodsClinical and laboratory data of 41 children with acute liver failure admitted to the National Liver Institute – Menoufia University were collected retrospectively and used for calculation of both pCLIF-SOFA and Pediatric End-Stage Liver Disease (PELD)/Model for End-Stage Liver Disease (MELD) scores on the day of admission, then statistical analysis was performed to identify the ability of these scores to predict the outcome.ResultsAccording to the outcome, children enrolled in this study were allocated to survived (n = 16) and died (n = 25) groups, which were age and sex matched. The non-survival group had significantly higher values of both pCLIF-SOFA score (11 [7-13]) and PELD/MELD score (36 [18-42]) than those of the survival group (8 [7-11], 27.5 [15-45]; p < 0.001, p = 0.004) respectively. Both pCLIF-SOFA and PELD/MELD scores at cut-off values > 8 and > 30 respectively on admission could predict death in children with acute liver failure (ALF) with high sensitivity, but with higher specificity, positive and negative predictive values for pCLIF-SOFA.ConclusionspCLIF-SOFA is a good predictor of death in pediatric acute liver failure.

Differential Diagnosis of Acute Liver Failure in Children: A Systematic Review.

PurposeTo develop a probability-based differential diagnosis for pediatric acute liver failure (PALF) based on age and socioeconomic status of the country of origin.MethodsComprehensive literature search using PubMed, EMBASE, and SCOPUS databases was performed. Children 0–22 years of age who met PALF registry criteria were included. Articles included >10 children, and could not be a case report, review article, or editorial. No language filter was utilized, but an English abstract was required. Etiology of PALF, age of child, and country of origin was extracted from included articles.Results32 full text articles were reviewed in detail; 2,982 children were included. The top diagnosis of PALF in developed countries was acetaminophen toxicity (9.24%; 95% CredI 7.99–10.6), whereas in developing countries it was Hepatitis A (28.9%; 95% CredI 26.3–31.7). In developed countries, the leading diagnosis of PALF in children aged <1 year was metabolic disorder (17.2%; 95% CredI 10.3–25.5), whereas in developing countries it was unspecified infection (39.3%; CredI 27.6–51.8). In developed countries, the leading diagnosis in children aged >1 year was Non-A-B-C Hepatitis (8.18%; CredI 5.28–11.7), whereas in developing countries it was Hepatitis A (32.4%; CredI 28.6–36.3).ConclusionThe leading causes of PALF in children aged 0-22 years differ depending on the age and developmental status of their country of origin, suggesting that these factors must be considered in the evaluation of children with PALF.

Etiology, clinical presentation, and outcome of children with fulminant hepatic failure: Experience from a tertiary center in Pakistan

Objectives:To determine etiologies, clinical presentations and outcomes of children with fulminant hepatic failure in the first liver transplant center of Pakistan.Methods:It was a retrospective, observational study, conducted in Paediatric Gastroenterology Department of Shifa International Hospital. Patients between one month to 16 years were included who fulfilled the Pediatric Acute Liver Failure study group (PALFSG) definition of acute liver failure as biochemical evidence of liver injury with no known co-existing chronic liver disease, coagulopathy not corrected by vitamin K, an International Normalized Ratio (INR) greater than 1.5 if the patient has encephalopathy, or greater than 2.0 if the patient does not have encephalopathy. The data collected was recorded on a self-constructed proforma after IRB approval.Results:There were 28 patients in the study which ncluded 17 males and 11 females with a mean age of 72.86±52.50 months. The most common etiologies were Hepatitis A (29%) in isolation or co-infection with Wilson Disease, typhoid fever. It was followed by seronegative hepatitis (29%). Majority (64%) had acute presentation (7 to 28 days), jaundice (82%) being the most common symptom. Severity of encephalopathy was significantly associated with outcome (p=0.02). There were 6 (21%) patients who succumbed to death.Conclusions:The study highlights infective diseases as the predominant etiology causing fulminant liver failure in children. Our study highlights lower mortality in children

Alginate microencapsulated human hepatocytes for the treatment of acute liver failure in children

Liver transplantation (LT) is the most effective treatment for patients with acute liver failure (ALF), but is limited by surgical risks and the need for life-long immunosuppression. Transplantation of microencapsulated human hepatocytes in alginate is an attractive option over whole liver replacement. The safety and efficacy of hepatocyte microbead transplantation have been shown in animal models. We report our experience of this therapy in children with ALF treated on a named-patient basis. Clinical grade human hepatocyte microbeads (HMBs) and empty microbeads were tested in immunocompetent healthy rats. Subsequently, 8 children with ALF, who were awaiting a suitable allograft for LT, received intraperitoneal transplantation of HMBs. We monitored complications of the procedure, assessing the host immune response and residual function of the retrieved HMBs, either after spontaneous native liver regeneration or at the time of LT. Intraperitoneal transplantation of HMBs in healthy rats was safe and preserved synthetic and detoxification functions, without the need for immunosuppression. Subsequently, 8 children with ALF received HMBs (4 neonatal haemochromatosis, 2 viral infections and 2 children with unknown cause at time of infusion) at a median age of 14.5 days, range 1 day to 6 years. The procedure was well tolerated without complications. Of the 8 children, 4 avoided LT while 3 were successfully bridged to LT following the intervention. HMBs retrieved after infusions (at the time of LT) were structurally intact, free of host cell adherence and contained viable hepatocytes with preserved functions. The results demonstrate the feasibility and safety of an HMB infusion in children with ALF. Acute liver failure in children is a rare but devastating condition. Liver transplantation is the most effective treatment, but it has several important limitations. Liver cell (hepatocyte) transplantation is an attractive option, as many patients only require short-term liver support while their own liver recovers. Human hepatocytes encapsulated in alginate beads can perform the functions of the liver while alginate coating protects the cells from immune attack. Herein, we demonstrated that transplantation of these beads was safe and feasible in children with acute liver failure.

Aetiology, Clinical Profiles, Laboratory Profile, Outcome and Prognostic Factors of Pediatric Acute Liver Failure: Experience at a Tertiary Hospital of Bangladesh

Introduction: Acute liver failure is one of the common causes of death in pediatric gastroenterology and hepatology department. Outcome is different according to aetiology.&#x0D; Objective: To observe the aetiology, outcome and prognostic factors of pediatric acute liver failure.&#x0D; Methods: Consecutive 62 children aged 2 to 16 years of age who were diagnosed as acute liver failure from November 2015 to April 2018 were included in this study. All the clinical profiles, laboratory data and outcome were recorded in a preformed data sheet. Data were analysed by SPSS for Windows version 20.&#x0D; Results: Mean age was 8.5 years. Thirty-nine (62.9%) patients were between 5-10 years of age. Male were 53%. We made a diagnosis of 39 (63%) patients as Wilson disease alone, Another 3 Wilson disease acute liver failure patients had concomitent with either HAV, HEV or HSV in each one. HAV only was responsible for 17 patients and HEV for 1. One patient was Haemophagocyic lymphohistiocytosis and aetiology could not be identified in 1 patient. The overall death in study population was 48% (30). Twenty-four (57%) of 42 acute liver failure patients due to Wilson disease had died. Five (29%) of 17 patients due to HAV infection and 1 patient with HLH died. Ascites, high total bilirubin, high INR and etiology like Wilson disease were the worse pronostic factors for outcome of acute liver failure in children.&#x0D; Conclusion: Wilson disease was the most common aetiology of acute liver failure in children in this study. Early diagnosis is essential as outcome was worse. Majority of viral etiology improved with supportive care.&#x0D; Anwer Khan Modern Medical College Journal Vol. 10, No. 1: Jan 2019, P 56-61

Clinical effect and prognosis analysis of different blood purification patterns in the treatment of acute liver failure in children

Objective To compare the clinical efficacy of different blood purification methods in children with acute liver failure, and to explore the clinical application mode, time and prognosis of blood purification in children with acute liver failure. Methods The clinical data of 85 children with acute liver failure admitted to PICU of Hunan Children′s Hospital from January 2010 to October 2016 were retrospectively analyzed.Sixteen patients were treated with general integrated medical treatment(conservative group). Twenty-seven patients were treated with continuous venovenous hemodiafiltration (CVVHDF) model non-biological artificial liver on the basis of general integrated medical treatment(CBP group). Sixteen cases were treated with plasma exchange(PE group). Twenty-six cases were treated with plasma exchange combined with CVVHDF mode (combination group). The main biochemical indexes, coagulation function, model for end-stage liver disease(MELD) score and delta MELD before and after treatment among groups were compared. Results Compared with those before treatment, the improvement of liver function and prognosis in the conservative was not significant after treatment.There were significant differences in the improvement of liver function and prognosis among the other three groups treated with non-biological artificial liver.Comparing the biochemical indexes and prognosis of three groups of children treated with different modes of non-biological artificial liver, there was no significant difference in the total effective rate between PE group and CBP group[56.3% (9/16) vs 55.6% (15/27), P>0.05]. The total effective rate of combined group[84.6% (22/26)] was significantly higher than those of PE group and CBP group.There was no significant difference in the improvement of liver function between PE group and CBP group (all P>0.05), but the indexes of liver function in combined group were significantly lower than those in PE group and CBP group (P 40. Conclusion PE and CBP have a good effect on children with acute liver failure, and if combined with the two methods can improve the therapeutic effect.At the same time, MELD score should be monitored in children with acute liver failure, and non-biological artificial liver therapy should not be recommended for children with MELD 40. Key words: Acute liver failure; Non-biological liver support; Model for end-stage liver disease; Blood purification

Pediatric Abdominal Organ Transplantation.

Pediatric liver and kidney transplantation have become the standard and accepted treatment for children with end-stage renal and liver disease. Since the first successful kidney transplant in 1954 by Dr Joseph Murray and the first liver transplant by Dr Thomas Starzl, the scope of indications for visceral organ transplantation as well as the range of recipient and donor ages has expanded. The first pediatric liver and kidney transplants, simultaneous multivisceral transplants, living-donor and donation-after-cardiac-death organs have evolved rapidly into the standard of care for end-stage renal and liver failure in children.

Treatment of acute liver failure in children

Acute liver failure (ALF) is a syndrome defined by the ra­pid decline of hepatic function, jaundice, coagulopathy (INR>1.5) and hepatic encephalopathy in patients with no evi­dence of previous liver disease. In children, the etiology of ALF is different, depending on age, and is associated with a high mortality, despite optimal medical therapy. The treat­ment for children with ALF will be directed to maintain vital functions, to prevent and treat the complications or against the etiologic agent when it is known. The the­ra­peu­tic measures consist in stabilizing the child by the administration or adjustment of liquids, vasoactive me­di­ca­tion or respiratory support if necessary, the treatment of encephalopathy through measures to reduce the am­monia in the blood, correction of hypoglycemia or hydroelectrolytic imbalances, the treatment of hemorrhage and coagulopathy. Specific treatment can be life-saving if the etiology is known, and includes administering anti­dots in toxic ALF, antivirals or antibacterial medication in infectious causes, immunosuppressive therapy in au­to­im­mune hepatitis, cooper chelators in Wilson disease, or specific diets in metabolic disorders. In severe ALF in chil­dren, extracorporeal methods (hemodiafiltration, plas­ma exchange or hepatic dialysis) may be used, but the only therapeutic option remains emergency liver trans­plan­ta­tion. The management of these cases requires a multi­dis­ci­pli­na­ry team, involving pediatric hepatologist, critical care spe­cia­list, nephrologist, or liver transplant surgeon.

Acute‐on‐chronic liver failure in children with biliary atresia awaiting liver transplantation

Acute-on-chronic liver failure (ACLF) is an acute decompensation of cirrhosis complicated by other organ failure and is associated with increased mortality and morbidity. ACLF has not been studied in children with biliary atresia (BA), which is the commonest indication for pediatric liver transplantation (LT) worldwide. This study aims to evaluate ACLF and outcomes in children with BA while awaiting deceased donor LT. This was a subanalysis of the dataset from a prospective cohort study of patients aged 0-18years who underwent portoenterostomy for BA and were listed for LT at King's College Hospital, London, between 1999 and 2003. Outcomes included the development of ACLF, mortality, and complications. Ninety-nine (41 male) children were included, and follow-up was 10 [6.0-15.0] years. A total of 20/99 children developed ACLF. ACLF was associated with increased mortality while awaiting LT (20% vs 4%; P=0.03). There were no associations between biochemical parameters at listing and death. Increased bilirubin levels 3months post-portoenterostomy was predictive of development of ACLF (AUROC=0.72, P<0.01). Age at LT and time on the waiting list in the ACLF subgroup were both lower compared to the non-ACLF group (P>0.05). Sepsis and gastrointestinal bleeding were the commonest precipitants of ACLF. Complications included ascites, hepatic encephalopathy, and hepatorenal syndrome; the ACLF subgroup required multisystem support and longer intensive care unit stay. ACLF in children with BA awaiting deceased donor LT carries increased mortality and morbidity. This warrants stratification of patients for earlier wait-listing and prioritization for LT.

Comments on plasma exchange for the treatment of liver failure in children

Comments on plasma exchange for the treatment of liver failure in children

A Learning Collaborative Approach Increases Specificity of Diagnosis of Acute Liver Failure in Pediatric Patients

Many pediatric patients with acute liver failure (PALF) do not receive a specific diagnosis (such as herpes simplex virus or Wilson disease or fatty acid oxidation defects)-they are left with an indeterminate diagnosis and are more likely to undergo liver transplantation, which is contraindicated for some disorders. Strategies to facilitate complete diagnostic testing should increase identification of specific liver diseases and might reduce liver transplantation. We investigated whether performing recommended age-specific diagnostic tests (AS-DTs) at the time of hospital admission reduces the percentage PALFs with an indeterminate diagnosis. We performed a multinational observational cohort study of 658 PALF participants in the United States and Canada, enrolled at 10 medical centers, during 3 study phases from December 1999 through December 2014. A learning collaborative approach was used to implement AS-DT using an electronic medical record admission order set at hospital admission in phase 3 of the study. Data from 10 study sites participating in all 3 phases were compared before (phases 1 and 2) and after (phase 3) diagnostic test recommendations were inserted into electronic medical record order sets. The percentage of subjects with an indeterminate diagnosis decreased significantly between phases 1-2 (48.0%) and phase 3 (to 30.8%) (P= .0003). The 21-day cumulative incidence rates for liver transplantation were significantly different among phase 1 (34.6%), phase 2 (31.9%), and phase 3 (20.2%) (P= .030). The 21-day cumulative incidence rates for death did not differ significantly among phase 1 (17.9%), phase 2 (11.9%), and phase 3 (11.3%) (P= .20). In a multinational study of children with acute liver failure, we found that incorporating diagnostic test recommendations into electronic medical record order sets accessed at time of admission reduced the percentage with an indeterminate diagnosis that may have reduced liver transplants without increasing mortality. Widespread use of this approach could significantly enhance care of acute liver failure in children.

Acute liver failure in children-Is living donor liver transplantation justified?

ObjectivesLiving donor liver transplantation (LDLT) in patients with acute liver failure (ALF) has become an acceptable alternative to transplantation from deceased donors (DDLT). The aim of this study was to analyze outcomes of LDLT in pediatric patients with ALF based on our center’s experience.Material and methodsWe enrolled 63 children (at our institution) with ALF who underwent liver transplantation between 1997 and 2016. Among them 24 (38%) underwent a LDLT and 39 (62%) received a DDLT. Retrospectively analyzed patient clinical data included: time lapse between qualification for transplantation and transplant surgery, graft characteristics, postoperative complications, long-term results post-transplantation, and living donor morbidity. Overall, we have made a comparison of clinical results between LDLT and DDLT groups.ResultsFollow-up periods ranged from 12 to 182 months (median 109 months) for LDLT patients and 12 to 183 months (median 72 months) for DDLT patients. The median waiting time for a transplant was shorter in LDLT group than in DDLT group. There was not a single case of primary non-function (PNF) in the LDLT group and 20 out of 24 patients (83.3%) had good early graft function; 3 patients (12.5%) in the LDLT group died within 2 months of transplantation but there was no late mortality. In comparison, 4 out of 39 patients (10.2%) had PNF in DDLT group while 20 patients (51.2%) had good early graft function; 8 patients (20.5%) died early within 2 months and 2 patients (5.1%) died late after transplantation. The LDLT group had a shorter cold ischemia time (CIT) of 4 hours in comparison to 9.2 hours in the DDLT group (p<0.0001).ConclusionsLDLT is a lifesaving procedure for pediatric patients with ALF. Our experience showed that it may be performed with very good results, and with very low morbidity and no mortality among living donors when performed by experienced teams following strict procedures.