Next Article in Journal
Testis-Sparing Surgery for Non-Palpable Leydig Cell Tumors in Prepubertal Children
Previous Article in Journal
Adrenocorticotropic Hormone Therapy Improved Spasms and Sleep Disturbance in Smith–Magenis Syndrome: A Case Report
Open AccessCase Report

Irritability, Poor Feeding and Respiratory Alkalosis in Newborns: Think about Metabolic Emergencies. A Brief Summary of Hyperammonemia Management

1
Neonatal Intensive Care Unit, Hôpital Universitaire des Enfants Reine Fabiola, Université Libre de Bruxelles, 1020 Brussels, Belgium
2
Department of Nutrition and Metabolism, Hôpital Universitaire des Enfants Reine Fabiola, Université Libre de Bruxelles, 1020 Brussels, Belgium
3
Pediatric Intensive Care Unit, Hôpital Universitaire des Enfants Reine Fabiola, Université Libre de Bruxelles, 1020 Brussels, Belgium
4
Neonatal Non-Intensive Care Unit, Centre Hospitalier de Wallonie Picarde (Site Union), 7500 Tournai, Belgium
*
Author to whom correspondence should be addressed.
Pediatr. Rep. 2020, 12(3), 77-85; https://doi.org/10.3390/pediatric12030019
Received: 25 June 2020 / Accepted: 27 July 2020 / Published: 25 October 2020
The urea cycle is a series of metabolic reactions that convert ammonia into urea in order to eliminate it from the body. Urea cycle disorders are characterized by hyperammonemia, which can cause irreversible damages in central nervous system. We report a series of three newborns presenting irritability, poor feeding and tachypnea. Their first gas analysis revealed respiratory alkalosis. Hyperammonemia was confirmed, and three different enzymatic blocks in the urea cycle were diagnosed. Immediate treatment consisted in the removal of ammonia by reduction of the catabolic state, dietary adjustments, use of nitrogen scavenging agents and ultimately hemodiafiltration. Hyperammonemia is a medical emergency whose treatment should not be delayed. This report aims to highlight the importance of suspecting urea cycle disorders in newborns with aspecific signs of hyperammonemia and respiratory alkalosis, and to sum up the broad lines of hyperammonemia management. View Full-Text
Keywords: hyperammonemia; respiratory alkalosis; urea cycle disorder; newborn; continuous venovenous hemodiafiltration hyperammonemia; respiratory alkalosis; urea cycle disorder; newborn; continuous venovenous hemodiafiltration
Show Figures

Figure 1

MDPI and ACS Style

Del Re, S.; Empain, A.; Vicinanza, A.; Balasel, O.; Johansson, A.-B.; Stalens, J.-P.; De Laet, C. Irritability, Poor Feeding and Respiratory Alkalosis in Newborns: Think about Metabolic Emergencies. A Brief Summary of Hyperammonemia Management. Pediatr. Rep. 2020, 12, 77-85.

Show more citation formats Show less citations formats

Article Access Map by Country/Region

1
Back to TopTop