Proceedings of the European Society for Pediatric Gastroenterology, Hepatology and Nutrition Monothematic Conference, 2020: "Acute Liver Failure in Children": Treatment and Directions for Future Research.

OBJECTIVES: The Hepatology Committee of the European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) aims to educate pediatric gastroenterologists, members of ESPGHAN and professionals from other specialties promoting an exchange of clinical expertise in the field of pediatric hepatology. METHODS: The 2020 single topic ESPGHAN monothematic 3-day conference on pediatric liver disease, was organized in Athens, Greece and was entitled " Acute Liver Failure" (ALF). ALF is a devastating disease with high mortality and in a considerable fraction of patients, the cause remains unresolved. As knowledge in diagnosis and treatment of ALF in infants and children has increased in the past decades, the objective was to update physicians in the field with developments in medical therapy and indications for liver transplantation (LT) and to identify areas for future research in clinical and neurocognitive outcomes in ALF. RESULTS: We recently reported the epidemiology, diagnosis, and initial intensive care management issues in separate manuscript. Herewith we report on the medical treatment, clinical lessons arising from pediatric studies, nutritional and renal replacement therapy (RRT), indications and contraindications for LT, neurocognitive outcomes, new techniques used as bridging to LT, and areas for future research. Oral presentations by experts in various fields are summarized highlighting key learning points. CONCLUSIONS: The current report summarizes the current insights in medical treatment of pediatric ALF and the directions for future research.

Combined Use of Rotational Thromboelastometry (Rotem) and Platelet Impedance Aggregometry (Multiplate Analyzer) in Cyanotic and Acyanotic Infants and Children Undergoing Cardiac Surgery With Cardiopulmonary Bypass: Subgroup Analysis of a Randomized Clinical Trial.

OBJECTIVES: Few studies have investigated the Multiplate platelet function analyzer in pediatrics. The authors evaluated Multiplate combined with Rotem in terms of guiding platelet transfusion after pediatric cardiac surgery with cardiopulmonary bypass (CPB). The authors further compared coagulation parameters between cyanotic and acyanotic patients. DESIGN: Subgroup analysis of a randomized clinical trial. SETTING: Tertiary hospital. PARTICIPANTS: Patients weighing between seven and 15 kg. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Rotem and Multiplate tests were performed (1) after anesthesia induction, (2) upon CPB separation, and (3) upon intensive care unit arrival. Among a total of 59 subjects, 9 patients required platelet transfusion. In multivariate linear regression, analysis EXTEM maximum clot firmness upon CPB separation was associated with the volume of transfused platelets (regression coefficient = -0.348 [95% confidence interval -1.006 to -0.028]; p = 0.039). No such association was found for the Multiplate test. Acyanotic and cyanotic heart disease were present in 32 and 27 children, respectively. There were no significant differences between these two groups in terms of platelet count and function. Postoperative blood loss was significantly higher in the cyanotic group compared with the acyanotic arm (p = 0.015; difference [95% confidence interval -2.40 {-4.20 to -0.60}]). There were no differences between groups regarding transfusion of allogeneic blood products. CONCLUSIONS: This study showed that Rotem, but not Multiplate results, were associated with platelet transfusion in pediatric cardiac surgery with no intake of platelet inhibitors. The usefulness of combining these tests in platelet transfusion decision-making needs to be evaluated in larger populations.

Does Postoperative Cardiac Troponin-I Have Any Prognostic Value in Predicting Midterm Mortality After Congenital Cardiac Surgery?

OBJECTIVES: This study evaluated the prognostic value of postoperative cardiac troponin-I (cTnI) in predicting all-cause mortality up to 3 months after normothermic congenital cardiac surgery. DESIGN: Prospective observational study. SETTING: University hospital. PARTICIPANTS: All children ages 0 to 10 years. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: cTnI was measured after the induction of anesthesia but before the surgery, at the pediatric intensive care unit arrival, and at 4, 12, and 24 hours postoperatively. Follow-up was extended up to 6 months. Overall, 169 children were analyzed, of whom 165 were survivors and 4 were nonsurvivors. cTnI levels were significantly higher in nonsurvivors only at 24 hours (p = 0.047). Children undergoing surgery with cardiopulmonary bypass (CPB) had significantly higher cTnI concentrations compared with those without CPB (p<0.001). Logistic regression analysis was performed on the 146 children in the CPB group with the following predictive variables: CPB time, postoperative cTnI concentrations, the presence of a cyanotic malformation, and intramyocardial incision. None of the variables predicted mortality. Postoperative cTnI concentrations did not predict 6 months׳ mortality. Only cTnI at 24 hours predicted the length of stay in the pediatric intensive care unit. CONCLUSIONS: This study did not find that postoperative cTnI concentration predicted midterm mortality after normothermic congenital heart surgery. (ClinicalTrials.gov identifier: NCT01616394).

Unplanned intensive care unit admission after general anaesthesia in children: A single centre retrospective analysis.

OBJECTIVES: To determine the main causes for unplanned admission of children to the paediatric intensive care unit (PICU) following anaesthesia in our centre. To compare the results with previous publications and propose a data sheet for the prospective collection of such information. METHODS: Inclusion criteria were any patient under 16 years who had an unplanned post-anaesthetic admission to the PICU from 1999 to 2010 in our university hospital. Age, ASA score, type of procedure, origin and causes of the incident(s) that prompted admission and time of the admission decision were recorded. RESULTS: Out of a total of 44,559 paediatric interventions performed under anaesthesia during the study period, 85 were followed with an unplanned admission to the PICU: 67% of patients were younger than 5 years old. Their ASA status distribution from I to IV was 13, 47, 39 and 1%, respectively. The cause of admission was anaesthetic, surgical or mixed in 50, 37 and 13% of cases, respectively. The main causes of anaesthesia-related admission were respiratory or airway management problems (44%) and cardiac catheterisation complications (29%). In 62%, the admission decision was taken in the operating room. CONCLUSION: Unplanned admission to the PICU after general anaesthesia is a rare event. In our series, most cases were less than 5 years old and were associated with at least one comorbidity. The main cause of admission was respiratory distress and the main type of procedure associated with admission was cardiac catheterisation.

Please Do Whatever It Takes to End Our Daughter's Suffering!

What is the best way to care for a child with severe neurologic impairment who seems to be dying and is in intractable pain? Can we give sedation as we remove life support? Is it ethically permissible to hasten death? In the United States, 5 states have legalized assisted suicide (although only for competent adults). In Belgium and the Netherlands, euthanasia is legal for children under some circumstances. We present a case in which parents and doctors face difficult decisions about palliative care. Experts from Belgium, the Netherlands, and the United States then discuss how they would respond to such a case.

Feasibility Study on Neurally Adjusted Ventilatory Assist in Noninvasive Ventilation After Cardiac Surgery in Infants.

BACKGROUND: Our objective was to evaluate the feasibility, the quality of synchronization, and the influence on respiratory parameters of the noninvasive neurally adjusted ventilatory assist (NIV-NAVA) mode in infants after cardiac bypass surgery. We conducted a prospective, randomized cross-over study in infants undergoing noninvasive ventilation (NIV) after cardiac surgery. METHODS: Subjects were 10 infants < 5 kg. After extubation, subjects underwent 2 consecutive ventilatory modes after randomization into groups. In the CPAP first group, the subjects were ventilated first in nasal CPAP-1 and then in NIV-NAVA-2 for 30 min in each mode. In the NIV-NAVA first group, periods were reversed. All children were ventilated using the same interface. RESULTS: The analysis of curves showed a synchronization rate of 99.3% for all respiratory cycles. The rate of pneumatic inspiratory trigger was 3.4%. Asynchronies were infrequent. Some typical respiratory patterns (continuous effort and discontinuous inspiration) were found at rates of 10.9% and 31.1%, respectively. The respiratory trends showed a lower maximum diaphragmatic electrical activity (EAdi(max)) in NIV-NAVA periods compared with CPAP periods (P < .001 in the beginning of periods). The breathing frequency decreased significantly during the nasal CPAP-2 and NIV-NAVA-1 periods (P < .05). The inspiratory pressure increased significantly during the NIV-NAVA-1 and NIV-NAVA-2 periods (P < .05), but there was no significant difference for each parameter when comparing Δ values between the beginning and the end of each period. The EAdi signal was easy to obtain in all subjects, and no major side effects were associated with the use of NIV-NAVA. CONCLUSIONS: NIV-NAVA allows good synchronization in bi-level NIV in infant cardiac subjects weighing < 5 kg. The analysis of respiratory parameters shows that NIV NAVA decreases the work of breathing more effectively than nasal CPAP. The study shows some typical respiratory patterns in infants. (ClinicalTrials.gov registration NCT01570933.).

Prehospital paediatric emergencies in Belgium: an epidemiologic study.

OBJECTIVES: In Belgium, emergency medical services (EMS) are staffed with a medical team if mandatory according to the regulation authority procedures. Children are involved in interventions, but no extensive data are available in the country. We analysed the characteristics of the children involved in EMS to gain better knowledge of the pathologies and the needs of these patients. MATERIALS AND METHODS: A retrospective review of all patients under 16 years of age dealt with by our EMS team during a 2-year period. RESULTS: During the 2010-2011 period, our EMS performed 229 paediatric missions. Most of the patients (76.0%) presented medical conditions. Seizure was the most common diagnosis (34.1%), including febrile convulsions in 55.1% of the cases. Five patients (2.2%) suffered a cardiac arrest. All of them died despite advanced life support. Two more patients died before or just after admission to the emergency room. In the subgroup of patients admitted to our hospital, 26.6% needed drug administration and 43.2% were discharged home after emergency room management. CONCLUSION: Prehospital paediatric emergencies are rarely life-threatening conditions and seldom need advanced medical interventions. However, the outcome of real life-threatening conditions is poor, therefore emphasizing the need for better trained teams.

Levosimendan in congenital cardiac surgery: a randomized, double-blind clinical trial.

OBJECTIVE: In this study, the authors used a continuous infusion of either levosimendan or milrinone as inotropic support after corrective congenital cardiac surgery. The hemodynamic and biochemical parameters were compared. DESIGN: A prospective, randomized, double-blind clinical study. SETTING: A university hospital. PARTICIPANTS: Forty-one patients between 0 and 5 years old requiring inotropic support for corrective congenital heart surgery under cardiopulmonary bypass (CPB) were enrolled in this trial. Thirty-six patients completed the study. INTERVENTIONS: Patients were randomized in a double-blind fashion to a continuous infusion of either levosimendan at 0.05 µg/kg/min or milrinone at 0.4 µg/kg/min started at the onset of CPB. Epinephrine was started at 0.02 µg/kg/min after aortic cross-clamp release in both groups. MEASUREMENTS AND MAIN RESULTS: There was no significant difference between serum lactate levels of groups. The rate-pressure index (the product of heart rate and systolic blood pressure), which is an indicator of myocardial oxygen demand, was significantly lower at 24 hours and 48 hours postoperatively in the levosimendan group (p < 0.001) in comparison to the milrinone group. Although not significantly different, the troponin values in the levosimendan group were less at 1 hour (median [P(25)-P(75)]: 20.7 [15.3- 48.3] v 34.6 [23.8- 64.5] ng/mL and 4 hours postoperatively: 30.4 [17.3-59.9] v 33.3 [25.5-76.7] ng/mL). CONCLUSION: Levosimendan is at least as efficacious as milrinone after corrective congenital cardiac surgery in neonates and infants.

Fomepizole alone for severe infant ethylene glycol poisoning.

OBJECTIVE: To report a case of a massive ingestion of ethylene glycol in an infant successfully treated by fomepizole without hemodialysis. DESIGN: Descriptive case report. SETTING: Pediatric intensive care unit. PATIENT: A 5-mo-old boy who ingested 200 mL of an antifreeze solution. INTERVENTIONS: Antidotal therapy with a total of seven doses of fomepizole administered intravenously with an interval of 12 hrs (15 mg/kg as loading dose, then 10 mg/kg). Hemodialysis was not performed. MEASUREMENTS AND MAIN RESULTS: Iterative determination of ethylene glycol concentration was obtained in blood and urine. Kinetics were calculated for ethylene glycol and fomepizole elimination. The infant made a complete recovery with no change in renal function. CONCLUSIONS: Although not yet approved for this indication in the child, fomepizole seemed safe and effective in a case of severe ethylene glycol poisoning, without the need for hemodialysis.