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Objective <p>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).</p> Study design <p>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.</p> Results <p>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.</p> Conclusion <p>Neonatal-onset UCDs are associated with significant morbidity and mortality, but heterogeneity exists among the specific named UCDs.</p>

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A multicenter descriptive study of neonatal-onset urea cycle disorder patients hospitalized in level IV NICUs

  • 期刊论文

  • 发表日期：

    2026年1月12日

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  • Whitney S. Thompson,
  • Ellen M. Bendel-Stenzel,
  • Isabella Zaniletti,
  • Theresa R. Grover,
  • Karna Murthy,
  • Michael A. Padula,
  • Queenie K. G. Tan,
  • Kristen R. Suhrie

摘要

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.