Elizabeth G Ames1, Corey Powell2, Rachel M Engen3, Donald J Weaver4, Asif Mansuri5, Michelle N Rheault6, Keia Sanderson7, Uta Lichter-Konecki8, Ankana Daga9, Lindsay C Burrage10, Ayesha Ahmad11, Scott E Wenderfer10, Kera E Luckritz12. 1. Division of Pediatric Genetics, Metabolism, and Genomic Medicine, Department of Pediatrics, University of Michigan Health System, Ann Arbor, MI. Electronic address: amese@med.umich.edu. 2. Consulting for Statistics, Computing and Analytics Research, University of Michigan, Ann Arbor, MI. 3. Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL. 4. Division of Nephrology and Hypertension, Department of Pediatrics, Atrium Health Levine Children's, Charlotte, NC. 5. Children's Hospital of Georgia, Augusta University, Augusta, GA. 6. University of Minnesota Masonic Children's Hospital, Minneapolis, MN. 7. University of North Carolina Department of Medicine-Nephrology, Chapel Hill, NC. 8. Division of Genetic and Genomic Medicine, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh, Pittsburgh, PA. 9. Division of Nephrology, Boston Children's Hospital, Boston, MA. 10. Department of Pediatrics, Baylor College of Medicine, Houston, TX; Texas Children's Hospital, Houston, TX. 11. Division of Pediatric Genetics, Metabolism, and Genomic Medicine, Department of Pediatrics, University of Michigan Health System, Ann Arbor, MI. 12. Division of Pediatric Nephrology, Department of Pediatrics, University of Michigan Health System, Ann Arbor, MI.
Abstract
OBJECTIVE: To assess the outcomes of neonates in a contemporary multi-institutional cohort who receive renal replacement therapy (RRT) for hyperammonemia. STUDY DESIGN: We performed a retrospective analysis of 51 neonatal patients with confirmed inborn errors of metabolism that were treated at 9 different children's hospitals in the US between 2000 and 2015. RESULTS: Twenty-nine patients received hemodialysis (57%), 21 patients received continuous renal replacement therapy (41%), and 1 patient received peritoneal dialysis (2%). The median age at admission of both survivors (n = 33 [65%]) and nonsurvivors (n = 18) was 3 days. Peak ammonia and ammonia at admission were not significantly different between survivors and nonsurvivors. Hemodialysis, having more than 1 indication for RRT in addition to hyperammonemia, and complications during RRT were all risk factors for mortality. After accounting for multiple patient factors by multivariable analyses, hemodialysis was associated with a higher risk of death compared with continuous renal replacement therapy. When clinical factors including evidence of renal dysfunction, number of complications, concurrent extracorporeal membrane oxygenation, vasopressor requirement, and degree of hyperammonemia were held constant in a single Cox regression model, the hazard ratio for death with hemodialysis was 4.07 (95% CI 0.908-18.2, P value = .067). To help providers caring for neonates with hyperammonemia understand their patient's likelihood of survival, we created a predictive model with input variables known at the start of RRT. CONCLUSIONS: Our large, multicenter retrospective review supports the use of continuous renal replacement therapy for neonatal hyperammonemia.
OBJECTIVE: To assess the outcomes of neonates in a contemporary multi-institutional cohort who receive renal replacement therapy (RRT) for hyperammonemia. STUDY DESIGN: We performed a retrospective analysis of 51 neonatal patients with confirmed inborn errors of metabolism that were treated at 9 different children's hospitals in the US between 2000 and 2015. RESULTS: Twenty-nine patients received hemodialysis (57%), 21 patients received continuous renal replacement therapy (41%), and 1 patient received peritoneal dialysis (2%). The median age at admission of both survivors (n = 33 [65%]) and nonsurvivors (n = 18) was 3 days. Peak ammonia and ammonia at admission were not significantly different between survivors and nonsurvivors. Hemodialysis, having more than 1 indication for RRT in addition to hyperammonemia, and complications during RRT were all risk factors for mortality. After accounting for multiple patient factors by multivariable analyses, hemodialysis was associated with a higher risk of death compared with continuous renal replacement therapy. When clinical factors including evidence of renal dysfunction, number of complications, concurrent extracorporeal membrane oxygenation, vasopressor requirement, and degree of hyperammonemia were held constant in a single Cox regression model, the hazard ratio for death with hemodialysis was 4.07 (95% CI 0.908-18.2, P value = .067). To help providers caring for neonates with hyperammonemia understand their patient's likelihood of survival, we created a predictive model with input variables known at the start of RRT. CONCLUSIONS: Our large, multicenter retrospective review supports the use of continuous renal replacement therapy for neonatal hyperammonemia.
Authors: S Picca; C Dionisi-Vici; D Abeni; A Pastore; C Rizzo; M Orzalesi; G Sabetta; G Rizzoni; A Bartuli Journal: Pediatr Nephrol Date: 2001-11 Impact factor: 3.714
Authors: Gregory M Enns; Susan A Berry; Gerard T Berry; William J Rhead; Saul W Brusilow; Ada Hamosh Journal: N Engl J Med Date: 2007-05-31 Impact factor: 91.245