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Irritability, Poor Feeding and Respiratory Alkalosis in Newborns: Think about Metabolic Emergencies. A Brief Summary of Hyperammonemia Management
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 irritabil...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7717652/ https://www.ncbi.nlm.nih.gov/pubmed/33113778 http://dx.doi.org/10.3390/pediatric12030019 |
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author | Del Re, Stefano Empain, Aurélie Vicinanza, Alfredo Balasel, Ovidiu Johansson, Anne-Britt Stalens, Jean-Philippe De Laet, Corinne |
author_facet | Del Re, Stefano Empain, Aurélie Vicinanza, Alfredo Balasel, Ovidiu Johansson, Anne-Britt Stalens, Jean-Philippe De Laet, Corinne |
author_sort | Del Re, Stefano |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-7717652 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-77176522020-12-05 Irritability, Poor Feeding and Respiratory Alkalosis in Newborns: Think about Metabolic Emergencies. A Brief Summary of Hyperammonemia Management Del Re, Stefano Empain, Aurélie Vicinanza, Alfredo Balasel, Ovidiu Johansson, Anne-Britt Stalens, Jean-Philippe De Laet, Corinne Pediatr Rep Case Report 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. MDPI 2020-10-25 /pmc/articles/PMC7717652/ /pubmed/33113778 http://dx.doi.org/10.3390/pediatric12030019 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Case Report Del Re, Stefano Empain, Aurélie Vicinanza, Alfredo Balasel, Ovidiu Johansson, Anne-Britt Stalens, Jean-Philippe De Laet, Corinne Irritability, Poor Feeding and Respiratory Alkalosis in Newborns: Think about Metabolic Emergencies. A Brief Summary of Hyperammonemia Management |
title | Irritability, Poor Feeding and Respiratory Alkalosis in Newborns: Think about Metabolic Emergencies. A Brief Summary of Hyperammonemia Management |
title_full | Irritability, Poor Feeding and Respiratory Alkalosis in Newborns: Think about Metabolic Emergencies. A Brief Summary of Hyperammonemia Management |
title_fullStr | Irritability, Poor Feeding and Respiratory Alkalosis in Newborns: Think about Metabolic Emergencies. A Brief Summary of Hyperammonemia Management |
title_full_unstemmed | Irritability, Poor Feeding and Respiratory Alkalosis in Newborns: Think about Metabolic Emergencies. A Brief Summary of Hyperammonemia Management |
title_short | Irritability, Poor Feeding and Respiratory Alkalosis in Newborns: Think about Metabolic Emergencies. A Brief Summary of Hyperammonemia Management |
title_sort | irritability, poor feeding and respiratory alkalosis in newborns: think about metabolic emergencies. a brief summary of hyperammonemia management |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7717652/ https://www.ncbi.nlm.nih.gov/pubmed/33113778 http://dx.doi.org/10.3390/pediatric12030019 |
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