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Author(s): Michal Maternik [sup.1] , Roman Andrunevych [sup.2] , Dorota Drozdz [sup.3] , Piotr Czauderna [sup.4] , Ryszard Grenda [sup.5] , Marcin Tkaczyk [sup.6] Author Affiliations: (1) grid.11451.30, 0000 0001 [...]

Author(s): Ron Wald [sup.1] [sup.2] , William Beaubien-Souligny [sup.3] , Rahul Chanchlani [sup.4] , Edward G. Clark [sup.5] , Javier A. Neyra [sup.6] , Marlies Ostermann [sup.7] , Samuel A. [...], Critical illness is often complicated by acute kidney injury (AKI). In patients with severe AKI, renal replacement therapy (RRT) is deployed to address metabolic dysfunction and volume excess until kidney function recovers. This review is intended to provide a comprehensive update on key aspects of RRT prescription and delivery to critically ill patients. Recently completed trials have enhanced the evidence base regarding several RRT practices, most notably the timing of RRT initiation and anticoagulation for continuous therapies. Better evidence is still needed to clarify several aspects of care including optimal targets for ultrafiltration and effective strategies for RRT weaning and discontinuation.

Author(s): Ryoko Harada [sup.1] , Yuko Hamasaki [sup.2] , Yusuke Okuda [sup.3] , Riku Hamada [sup.1] , Kenji Ishikura [sup.3] Author Affiliations: (1) grid.417084.e, 0000 0004 1764 9914, Department of [...], Although the concept of chronic kidney disease (CKD) in children is similar to that in adults, pediatric CKD has some peculiarities, and there is less evidence and many factors that are not clearly understood. The past decade has witnessed several additional registry and cohort studies of pediatric CKD and kidney failure. The most common underlying disease in pediatric CKD and kidney failure is congenital anomalies of the kidney and urinary tract (CAKUT), which is one of the major characteristics of CKD in children. The incidence/prevalence of CKD in children varies worldwide. Hypertension and proteinuria are independent risk factors for CKD progression; other factors that may affect CKD progression are primary disease, age, sex, racial/genetic factors, urological problems, low birth weight, and social background. Many studies based on registry data revealed that the risk factors for mortality among children with kidney failure who are receiving kidney replacement therapy are younger age, female sex, non-White race, non-CAKUT etiologies, anemia, hypoalbuminemia, and high estimated glomerular filtration rate at dialysis initiation. The evidence has contributed to clinical practice. The results of these registry-based studies are expected to lead to new improvements in pediatric CKD care.

Author(s): Fatma Tuba Eminoglu [sup.1] , Ümmühan Öncül [sup.1] , Fevzi Kahveci [sup.2] , Emel Okulu [sup.3] , Elvis Kraja [sup.3] , Engin Köse [sup.1] , Tanil Kendirli [sup.2] Author [...], 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 ([greater than or equal to] 500 [mu]mol/L), blood ammonia levels > 250 [mu]mol/L in the presence of moderate encephalopathy, high blood leucine levels ([greater than or equal to] 1500 [mu]mol/L), and blood leucine levels < 1500 [mu]mol/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 [micro]mol/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 [micro]mol/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.

Author(s): Wael M. Abukwaik [sup.1] , Rossana Baracco [sup.1] , Amrish Jain [sup.1] , Melissa Gregory [sup.1] , Rudolph P. Valentini [sup.1] , Gaurav Kapur [sup.1] Author Affiliations: (1) grid.253856.f, [...], Introduction No data exist on the epidemiology of children incidentally diagnosed with advanced kidney failure (KF) during evaluation for non-specific symptoms. This is likely related to unrecognized symptoms and signs of CKD. The objective of our study was to evaluate incidentally diagnosed patients with advanced KF requiring long-term kidney replacement therapy (KRT). Methods An IRB-approved retrospective chart review of children who started KRT with dialysis (hemo- or peritoneal) was conducted. Included were children with no prior knowledge or diagnosis of underlying kidney disease with chronic kidney disease (CKD) disease stage 4 (GFR 15-29 mL/min/1.73 m.sup.2) or 5 (GFR < 15 mL/min/1.73 m.sup.2) at initial presentation and started on chronic KRT within 2 months of presentation. Results Of 177 patients initiating KRT during the study period, 26 (15%) were categorized as incidental advanced KF. This cohort with mean age 12.25 years consisted of 42% males, 54% African Americans included 46% with glomerular, and 54% with non-glomerular etiology for kidney failure. Vomiting (42%) and fatigue (39%) were most common, while growth failure (19%) and hyperkalemia (7%) were less frequent on initial presentation. Anemia (100%), hypertension (96%), hyperparathyroidism (96%), and hyperphosphatemia (92%) were the most frequently seen CKD comorbidities. Chronic KRT was started within 24 h in 62% and within 2 weeks in 88% of the cohort. Conclusion Under-diagnosis of patients with advanced KF is most likely related to milder non-specific clinical symptoms and normal growth in the majority of patients. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information.

Author(s): Bogdana Sabina Zoica [sup.1] , Akash Deep [sup.1] Author Affiliations: (1) grid.46699.34, 0000 0004 0391 9020, Pediatric Intensive Care Unit, King's College Hospital, , 3rd Floor Cheyne Wing, SE5 [...], The liver is the only organ which can regenerate and, thus, potentially negate the need for transplantation in acute liver failure (ALF). Cerebral edema and sepsis are leading causes of mortality in ALF. Both water-soluble and protein-bound toxins have been implicated in pathogenesis of various ALF complications. Ammonia is a surrogate marker of water-soluble toxin accumulation in ALF and high levels are associated with higher grades of hepatic encephalopathy, raised intracranial pressure, and mortality. Therefore, extracorporeal therapies aim to lower ammonia and maintain fluid balance and cytokine homeostasis. The most common and easily available modality is continuous kidney replacement therapy (CKRT). Early initiation of high-volume CKRT utilizing an anticoagulation regimen minimizing treatment downtime and delivering the prescribed dose is highly desirable. Ideally, extracorporeal liver-assist devices (ECLAD) should perform both synthetic and detoxification functions of the liver. ECLAD may temporarily replace lost liver function and serve as a bridge, either to spontaneous recovery or liver transplantation. Various bioartificial and biologic liver-assist devices are described in specialty literature, including molecular adsorbent recirculating system (MARS), single pass albumin dialysis (SPAD), and total plasma exchange (TPE); however, clinicians commonly use modalities easily available in intensive care units. There is a lack of standardization of indications for ECLAD, availability of different extracorporeal devices with varied technical approaches, and, of note, the differences in doses of ECLAD provided in clinical practice. We review the practicalities and evidence regarding these four artificial liver support devices in pediatric ALF.