Neonatal Metabolic Crises: A Practical Approach

Katie B Williams; Patrice K Held; Jessica Scott Schwoerer

doi:10.1016/j.clp.2019.10.003

Neonatal Metabolic Crises: A Practical Approach

Clin Perinatol. 2020 Mar;47(1):155-170. doi: 10.1016/j.clp.2019.10.003. Epub 2019 Oct 17.

Authors

Katie B Williams¹, Patrice K Held², Jessica Scott Schwoerer³

Affiliations

¹ Department of Pediatrics, University of Wisconsin Hospital and Clinics, 1500 Highland Avenue, Madison, WI 53705, USA.
² Department of Pediatrics, Wisconsin State Laboratory of Hygiene, University of Wisconsin School of Medicine and Public Health, 465 Henry Mall, Madison, WI 53706, USA.
³ Department of Pediatrics, Division of Genetics and Metabolism, University of Wisconsin Hospital and Clinics, 1500 Highland Avenue, Madison, WI 53705, USA. Electronic address: jscottschwoerer@pediatrics.wisc.edu.

PMID: 32000923
DOI: 10.1016/j.clp.2019.10.003

Abstract

Metabolic disorders are disruptions in nutrient metabolism or basic cellular processes that can result in severe neonatal crisis. Basic laboratory findings may reveal hypoglycemia, acidosis, or hyperammonemia, but may also be normal even in infants with severe metabolic decompensation. Breast milk or milk-based formulas often contain the nutrient that precipitates the metabolic crisis and may need to be held during acute illness. Instead, infants with suspected metabolic disorders should be administered a high glucose infusion rate with isotonic fluids to reverse catabolism. Combined advanced biochemical and molecular testing is often needed to identify specific metabolic disorders and guide ongoing treatment.

Keywords: Crisis; Metabolic disorder; Neonate; Newborn.

Publication types

Review

MeSH terms

Diagnosis, Differential
Humans
Infant, Newborn
Metabolism, Inborn Errors / diagnosis*
Metabolism, Inborn Errors / therapy*
Neonatal Screening