Issue |
J Extra Corpor Technol
Volume 52, Number 1, March 2020
|
|
---|---|---|
Page(s) | 58 - 62 | |
DOI | https://doi.org/10.1051/ject/202052058 | |
Published online | 15 March 2020 |
Case Report
ECMO as a Platform for Rapid Ammonia Removal in a Neonate with Multienzyme Urea Cycle Disorder
* Department of Pediatrics, St. Christopher’s Hospital for Children, Philadelphia, Pennsylvania
† Drexel University College of Medicine, Philadelphia, Pennsylvania
‡ Pharmacy Department, St. Christopher’s Hospital for Children, Philadelphia, Pennsylvania
Address correspondence to: Vilmaris Quinones Cardona, MD, Division of Neonatology, Department of Pediatrics, St. Christopher’s Hospital for Children, 160 East Erie Avenue, Philadelphia, PA 19123. E-mail: vq23@drexel.edu
Received:
6
October
2019
Accepted:
8
January
2020
Since the initial deployment of neonatal extracorporeal membrane oxygenation (ECMO) for respiratory failure, the use of ECMO in this population has diversified. We present a term female infant with carbamoyl phosphate synthetase 1 and partial N-acetylglutamate synthase deficiencies who developed severe hyperammonemia refractory to medical management requiring venoarterial ECMO-driven continuous veno-venous hemodiafiltration for ammonia detoxification. This case report illustrates a subpopulation where neonatal ECMO may improve survival and neurodevelopmental outcomes. To our knowledge, this is the first reported case of a urea cycle defect arising from two proximal enzyme deficiencies. Also, this is one of the few reported patients with UCD associated with peak ammonia levels >2,000 μmol/L who survived to hospital discharge after the successful use of ECMO for ammonia reduction. This case will add to the existing scant literature supporting the use of ECMO as a platform for rapid removal of serum ammonia.
Key words: hyperammonemia / urea cycle disorder / ECMO (extracorporeal membrane oxygenation) / CVVH (continuous veno-venous hemofiltration)
Presented at the Extracorporeal Life Support Organization 30th Annual Meeting in Austin, Texas, September 13, 2019.
The senior author has stated that the authors have reported no material, financial, or other relationship with any healthcare-related business or other entity whose products or services are discussed in this paper.
Contributors’ Statement: Dr. Ikeri obtained IRB approval, acquired and interpreted case report data, and drafted, reviewed, and revised the manuscript. Dr. Quinones Cardona obtained IRB approval, interpreted case report data, and co-drafted, critically reviewed, and revised the manuscript for important intellectual content. Dr. Hagan-Brown co-drafted, acquired case report data, and reviewed and revised the manuscript. Megan Young interpreted case report data, and co-drafted, reviewed, and revised the manuscript. Dr. Schneider interpreted case report data, and critically reviewed and revised the manuscript for important intellectual content. Dr. Menkiti interpreted case report data, and co-drafted, critically reviewed, and revised the manuscript for important intellectual content.
© 2020 AMSECT
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