Consensus guidelines for management of hyperammonaemia in paediatric patients receiving continuous kidney replacement therapy.

Rupesh Raina, Jirair K Bedoyan, Uta Lichter-Konecki, Philippe Jouvet, Stefano Picca, Nicholas Ah Mew, Marcel C Machado, Ronith Chakraborty, Meghana Vemuganti, Manpreet K Grewal, Timothy Bunchman, Sidharth Kumar Sethi, Vinod Krishnappa, Mignon McCulloch, Khalid Alhasan, Arvind Bagga, Rajit K Basu, Franz Schaefer, Guido Filler, Bradley A Warady

Journal: Nature reviews. Nephrology 2020;16(8):471-482

PMID: 32269302

Abstract

Hyperammonaemia in children can lead to grave consequences in the form of cerebral oedema, severe neurological impairment and even death. In infants and children, common causes of hyperammonaemia include urea cycle disorders or organic acidaemias. Few studies have assessed the role of extracorporeal therapies in the management of hyperammonaemia in neonates and children. Moreover, consensus guidelines are lacking for the use of non-kidney replacement therapy (NKRT) and kidney replacement therapies (KRTs, including peritoneal dialysis, continuous KRT, haemodialysis and hybrid therapy) to manage hyperammonaemia in neonates and children. Prompt treatment with KRT and/or NKRT, the choice of which depends on the ammonia concentrations and presenting symptoms of the patient, is crucial. This expert Consensus Statement presents recommendations for the management of hyperammonaemia requiring KRT in paediatric populations. Additional studies are required to strengthen these recommendations.

Address: Department of Nephrology, Akron Children's Hospital, Akron, OH, USA. [email protected].; Akron Nephrology Associates/Cleveland Clinic Akron General, Akron, OH, USA. [email protected].; Center for Human Genetics, University Hospitals Cleveland Medical Center and Department of Genetics and Genome Sciences, Case Western Reserve University, Cleveland, OH, USA.; Division of Medical Genetics, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.; Department of Paediatrics, Sainte-Justine Hospital, University of Montreal, Montreal, Quebec, Canada.; Division of Nephrology and Dialysis, Department of Paediatrics, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.; Children's National Rare Disease Institute, The George Washington University, Washington, DC, USA.; Department of Emergency Medicine, University of São Paulo School of Medicine, São Paulo, Brazil.; Akron Nephrology Associates/Cleveland Clinic Akron General, Akron, OH, USA.; Department of Pediatrics, Rainbow Babies and Children's Hospital, Cleveland, OH, USA.; Department of Pediatric Nephrology, Children's Hospital of Michigan, Detroit, MI, USA.; Pediatric Nephrology & Transplantation, Children's Hospital of Richmond, Virginia Commonwealth University, Richmond, VA, USA.; Paediatric Nephrology & Paediatric Kidney Transplantation, Kidney and Urology Institute, Medanta, The Medicity Hospital, Gurgaon, India.; Akron Nephrology Associates/Cleveland Clinic Akron General, Akron, OH, USA.; Northeast Ohio Medical University, Rootstown, OH, USA.; Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa.; Department of Paediatrics, King Saud University, College of Medicine, Riyadh, Saudi Arabia.; Division of Paediatric Nephrology, All India Institute of Medical Sciences, New Delhi, India.; Department of Pediatric Critical Care Medicine, Children's Healthcare of Atlanta, Atlanta, GA, USA.; Division of Paediatric Nephrology, University Children's Hospital Heidelberg, Heidelberg, Germany.; Division of Paediatric Nephrology, Department of Paediatrics, Western University, London, Ontario, Canada.; Division of Nephrology, University of Missouri-Kansas City School of Medicine, Children's Mercy, Kansas City, MO, USA.
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