Journal article
ECMO as a Platform for Rapid Ammonia Removal in a Neonate with Multienzyme Urea Cycle Disorder
The Journal of extra-corporeal technology, v 52(1)
Mar 2020
PMID: 32280145
Abstract
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.
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Details
- Title
- ECMO as a Platform for Rapid Ammonia Removal in a Neonate with Multienzyme Urea Cycle Disorder
- Creators
- Kelechi Ikeri - St. Christopher's Hospital for ChildrenVilmaris Quinones Cardona - Drexel UniversityAbena Hagan-Brown - St. Christopher's Hospital for ChildrenMegan Young - St. Christopher's Hospital for ChildrenMichael Schneider - Drexel UniversityOgechukwu Menkiti - Drexel UniversityMegan Schneider - Physician Assistant
- Publication Details
- The Journal of extra-corporeal technology, v 52(1)
- Publisher
- American Society of Hematology (ASH)
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Pediatrics; Physician Assistant
- Scopus ID
- 2-s2.0-85083231707
- Other Identifier
- 991019173710704721