Background Continuous kidney replacement therapies (CKRT) have been reported to be an effective approach to removing toxic metabolites in inborn errors of metabolism (IEM). The present study evaluates efficiency and complications of CKRT in children with IEM. Methods Patients diagnosed with IEM who underwent CKRT in pediatric and neonatal intensive care units were analyzed. CKRT were initiated in patients with persistently high blood ammonia levels (≥ 500 μmol/L), blood ammonia levels > 250 μmol/L in the presence of moderate encephalopathy, high blood leucine levels (≥ 1500 μmol/L), and blood leucine levels < 1500 μmol/L in the presence of deteriorating neurological status or persistent metabolic acidosis. Results Of 22 patients enrolled, nine (40.9%) Maple syrup urine disease (MSUD), eight (36.4%) urea cycle disorders (UCD), and five (22.7%) organic acidemias (OA). Median age was 72.3 [9.9–1040.8] days. In total, 28 dialysis sessions were analyzed [16 (57.1%) continuous venovenous hemodialysis, and 12 (42.9%) continuous venovenous hemodiafiltration]. A significant decrease was noted in leucine levels (from 1608.4 ± 885.3 to 314.6 ± 109.9 µmol/L) of patients with MSUD, while ammonia levels were significantly decreased in patients with UCD and OA (from 1279.9 ± 612.1 to 85.1 ± 21.6 µmol/L). The most frequent complications of CKRT were thrombocytopenia (60.7%), hypotension (53.6%), and hypocalcemia (42.9%). Median age of patients with hypotension treated with vasoactive medications was significantly lower than median age of those with normal blood pressure. Conclusion CKRT is a reliable approach for effective and rapid removal of toxic metabolites in children with IEM, and CKRT modalities can be safely used and are well-tolerated in infants.

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Background: The aim of this study is to determine the indication, timing, and administration of extracorporeal therapies such as total plasma exchange and continuous renal replacement therapy in children with acute liver failure or acute-on-chronic liver failure. Methods: This study is conducted as a retrospective, single-center study. Between January 2016 and December 2021, pediatric acute liver failure or acute-on-chronic liver failure patients for whom total plasma exchange and/or continuous renal replacement therapy was performed were included in this study. Results: Thirty-four children with acute liver failure or acute-on-chronic liver failure were included during the study period. The children comprised 14 (41.1%) males, and the median age of the patients was 54 months (5-21). Twenty-four patients (70.6%) had pediatric acute liver failure, and 10 patients (29.4%) had acute-on-chronic liver failure. Patients’ median model for end-stage liver disease and pediatric end-stage liver disease scores were 24.7/23.5, respectively. Total plasma exchange therapy was performed on all patients whereas continuous renal replacement therapy was performed on 13 patients (38.2%). The median duration of continuous renal replacement therapy was 2.5 days (2-24). The median number of the total plasma exchange sessions was 3 (1-20). The median length of stay in pediatric intensive care unit was 4.5 (2-74) days. Eleven (32.5%) patients had 1 or more improvements in hepatic encephalopathy scores after extracorporeal therapy. Eleven (32.5%) patients died. There was a significant difference between the survivors and non-survivors with respect to levels of albumin, ammonia, pediatric risk of mortality scores, and pre-hepatic encephalopathy scores. Liver transplantation was performed in 4 of 24 pediatric acute liver failure patients, and all of them survived. Conclusion: Total plasma exchange and continuous renal replacement therapy are life-saving, and both methods may reduce morbidity and mortality, also bridging to liver transplantation.

IntroductionThere have been some significant changes regarding healthcare utilization during the COVID-19 pandemic. Majority of the reports about the impact of the COVID-19 pandemic on diabetes care are from the first wave of the pandemic. We aim to evaluate the potential effects of the COVID-19 pandemic on the severity of diabetic ketoacidosis (DKA) and new onset Type 1 diabetes presenting with DKA, and also evaluate children with DKA and acute COVID-19 infection.MethodsThis is a retrospective multi-center study among 997 children and adolescents with type 1 diabetes who were admitted with DKA to 27 pediatric intensive care units in Turkey between the first year of pandemic and pre-pandemic year.ResultsThe percentage of children with new-onset Type 1 diabetes presenting with DKA was higher during the COVID-19 pandemic (p &lt; 0.0001). The incidence of severe DKA was also higher during the COVID-19 pandemic (p &lt; 0.0001) and also higher among children with new onset Type 1 diabetes (p &lt; 0.0001). HbA1c levels, duration of insulin infusion, and length of PICU stay were significantly higher/longer during the pandemic period. Eleven patients tested positive for SARS-CoV-2, eight were positive for new onset Type 1 diabetes, and nine tested positive for severe DKA at admission.DiscussionThe frequency of new onset of Type 1 diabetes and severe cases among children with DKA during the first year of the COVID-19 pandemic. Furthermore, the cause of the increased severe presentation might be related to restrictions related to the pandemic; however, need to evaluate the potential effects of SARS-CoV-2 on the increased percentage of new onset Type 1 diabetes.

Background This study’s objective was to investigate the incidence of acute kidney injury (AKI) in children with severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) and multisystem inflammatory syndrome (MIS-C) and to report our clinical experience. Methods Acute COVID-19 and MIS-C-diagnosed patients observed in two pediatric intensive care units (PICUs) between 2019 and 2021 were examined for AKI and retrospectively compared to children with AKI. Results The study comprised 163 children, of whom 98 (60.1%) were diagnosed with acute COVID-19 and 65 (39.9%) with MIS-C. AKI was observed in 40 (40.8%) of the acute COVID-19 patients and 18 (27.7%) of the MIS-C patients. Low calcium level and hypotension were linked with AKI at initial presentation (OR: 0.56, 95% CI: 0.369–0.560, p = 0.006 and OR: 3.64, 95% CI: 1.885–7.152, p = 0.001, respectively). A history of nephrotoxic medication usage played an essential role in the development of AKI in patients who acquired AKI after hospitalization ( p = 0.001, odds ratio: 9.32, confidence interval: 3.106–27.973). In clinical practice, individuals with respiratory distress and cough had a high chance of having AKI (OR: 4.47, 95% confidence interval: 2.25–8,892 and OR: 3.48, 95% confidence interval: 1.76–6.88). AKI patients had a greater demand for respiratory assistance and a longer period of stay in the PICU. Conclusions AKI in the COVID-19 and MIS-C patient groups is related with increased mortality and extended hospitalization, according to the findings. These statistics imply that identifying and preventing risk factors is necessary. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information Supplementary Information The online version contains supplementary material available at 10.1007/s00467-023-05987-x.