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Coma With Hyperammonemia in a Patient With Kwashiorkor

We describe a case of coma-related hyperammonemia in a woman presenting with severe edematous malnutrition (Kwashiorkor-like), without underlying hepatic disease. Our main hypothesis is that the patient developed a functional urea cycle disorder, due to the inability to synthesize N-acetylglutamate...

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Autores principales: Vieille, Thibault, Feillet, Francois, Wiedemann, Arnaud, Winiszewski, Hadrien, Piton, Gael
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elmer Press 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10482601/
https://www.ncbi.nlm.nih.gov/pubmed/37691748
http://dx.doi.org/10.14740/gr1634
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author Vieille, Thibault
Feillet, Francois
Wiedemann, Arnaud
Winiszewski, Hadrien
Piton, Gael
author_facet Vieille, Thibault
Feillet, Francois
Wiedemann, Arnaud
Winiszewski, Hadrien
Piton, Gael
author_sort Vieille, Thibault
collection PubMed
description We describe a case of coma-related hyperammonemia in a woman presenting with severe edematous malnutrition (Kwashiorkor-like), without underlying hepatic disease. Our main hypothesis is that the patient developed a functional urea cycle disorder, due to the inability to synthesize N-acetylglutamate which is the activator of the first enzymes (carbamoyl phosphate synthetase) of urea cycle, in a context of severe deficiency of essential amino acids and of acetyl-CoA. Severe hyperammonemia is a medical emergency exposing to the risk of cerebral edema. Urgent treatment should interrupt protein intake, stimulate protein anabolism, and remove ammonia from the blood using renal replacement therapy and ammonia scavengers. Hyperammonemia should be searched in case of unexplained coma, even among patients without hepatic disorder, in particular among young patients. Hyperammonemia should also be searched among patients with severe protein-calorie malnutrition.
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spelling pubmed-104826012023-09-08 Coma With Hyperammonemia in a Patient With Kwashiorkor Vieille, Thibault Feillet, Francois Wiedemann, Arnaud Winiszewski, Hadrien Piton, Gael Gastroenterology Res Case Report We describe a case of coma-related hyperammonemia in a woman presenting with severe edematous malnutrition (Kwashiorkor-like), without underlying hepatic disease. Our main hypothesis is that the patient developed a functional urea cycle disorder, due to the inability to synthesize N-acetylglutamate which is the activator of the first enzymes (carbamoyl phosphate synthetase) of urea cycle, in a context of severe deficiency of essential amino acids and of acetyl-CoA. Severe hyperammonemia is a medical emergency exposing to the risk of cerebral edema. Urgent treatment should interrupt protein intake, stimulate protein anabolism, and remove ammonia from the blood using renal replacement therapy and ammonia scavengers. Hyperammonemia should be searched in case of unexplained coma, even among patients without hepatic disorder, in particular among young patients. Hyperammonemia should also be searched among patients with severe protein-calorie malnutrition. Elmer Press 2023-08 2023-08-26 /pmc/articles/PMC10482601/ /pubmed/37691748 http://dx.doi.org/10.14740/gr1634 Text en Copyright 2023, Vieille et al. https://creativecommons.org/licenses/by-nc/4.0/This article is distributed under the terms of the Creative Commons Attribution Non-Commercial 4.0 International License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Vieille, Thibault
Feillet, Francois
Wiedemann, Arnaud
Winiszewski, Hadrien
Piton, Gael
Coma With Hyperammonemia in a Patient With Kwashiorkor
title Coma With Hyperammonemia in a Patient With Kwashiorkor
title_full Coma With Hyperammonemia in a Patient With Kwashiorkor
title_fullStr Coma With Hyperammonemia in a Patient With Kwashiorkor
title_full_unstemmed Coma With Hyperammonemia in a Patient With Kwashiorkor
title_short Coma With Hyperammonemia in a Patient With Kwashiorkor
title_sort coma with hyperammonemia in a patient with kwashiorkor
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10482601/
https://www.ncbi.nlm.nih.gov/pubmed/37691748
http://dx.doi.org/10.14740/gr1634
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