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Multisite Retrospective Review of Outcomes in Renal Replacement Therapy for Neonates with Inborn Errors of Metabolism - 22/06/22

Doi : 10.1016/j.jpeds.2022.03.043 
Elizabeth G. Ames, MD, PhD 1, , Corey Powell, PhD 2, Rachel M. Engen, MD 3, Donald J. Weaver, MD, PhD 4, Asif Mansuri, MD 5, Michelle N. Rheault, MD 6, Keia Sanderson, MD, MSCR 7, Uta Lichter-Konecki, MD, PhD 8, Ankana Daga, MBBS 9, Lindsay C. Burrage, MD, PhD 10, 11, Ayesha Ahmad, MD 1, Scott E. Wenderfer, MD 10, 11, Kera E. Luckritz, DO 12
1 Division of Pediatric Genetics, Metabolism, and Genomic Medicine, Department of Pediatrics, University of Michigan Health System, Ann Arbor, MI 
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 
11 Texas Children's Hospital, Houston, TX 
12 Division of Pediatric Nephrology, Department of Pediatrics, University of Michigan Health System, Ann Arbor, MI 

Reprint requests: Elizabeth G. Ames, MD, PhD, Division of Genetics, Metabolism, and Genomic Medicine, Department of Pediatrics, University of Michigan Health System, D5240 Medical Professional Building, 1500 E Medical Center Dr, Ann Arbor, MI 48109Division of Genetics, Metabolism, and Genomic MedicineDepartment of PediatricsUniversity of Michigan Health SystemD5240 Medical Professional Building, 1500 E Medical Center DrAnn ArborMI48109

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.

Le texte complet de cet article est disponible en PDF.

Keywords : ammonia, dialysis, IEM, RRT, survival

Abbreviations : AKI, BSA, CRRT, CVVHD, CVVHDF, ECMO, HR, IEM, OA, RRT, UCD


Plan


 Supported by the Gerber Foundation, Fremont, MI (N026404-391375 to E.A). The authors declare no conflicts of interest.
 Portions of this study were presented as a oral presentation during the Neonatal Kidney Collaborative meeting, May 4, 2020, Virtual.


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Vol 246

P. 116 - juillet 2022 Retour au numéro
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