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Updated: Jan 14, 2026

Assessment and Evaluation of the High Risk Neonate: The NICU Network Neurobehavioral Scale
Published on: August 25, 2014
Un estudio descriptivo multicéntrico de pacientes con trastorno del ciclo de la urea de inicio neonatal
Whitney S Thompson1, Ellen M Bendel-Stenzel2, Isabella Zaniletti3
1Mayo Clinic, Rochester, MN, USA. Thompson.Whitney@mayo.edu.
Objective:
The objective of this study was to characterize the neonatal intensive care unit (NICU) course and outcomes for infants with neonatal-onset urea cycle disorders (UCDs).
Study Design:
Using the multicenter Children's Hospitals Neonatal Database, a descriptive, retrospective cohort study was performed to characterize the presentation, interventions, comorbidities, and hospital outcomes for 176 patients with neonatal-onset UCDs that required level IV NICU care.
Results:
The median age of presentation was 5 [3,7] days, with 167 (95%) infants requiring transfer to a level IV NICU. Renal replacement therapy was undertaken for at least 34 (20%) patients. Neurologic complications occurred most frequently, with 71 (40%) patients affected. Twenty-one (12%) infants experienced in-hospital mortality. Interventions, complications, and mortality were observed most frequently in the ornithine transcarbamylase deficiency group.
Conclusion:
Neonatal-onset UCDs are associated with significant morbidity and mortality, but heterogeneity exists among the specific named UCDs.
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