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Severe loss of appetite and refusal to eat as severe side effect of glycerol phenylbutyrate

Glycerol phenylbutyrate (GPB) is an ammonia scavenger drug commonly used in the therapy of patients with urea cycle defects. Reported side effects include body odor, abdominal pain, nausea, burning sensation in mouth, vomiting, and heartburn. We report on a 3‐year‐old late diagnosed female patient w...

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Autores principales: Grünert, Sarah Catharina, Schumann, Anke, Spiekerkoetter, Ute
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons, Inc. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9626662/
https://www.ncbi.nlm.nih.gov/pubmed/36341170
http://dx.doi.org/10.1002/jmd2.12286
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author Grünert, Sarah Catharina
Schumann, Anke
Spiekerkoetter, Ute
author_facet Grünert, Sarah Catharina
Schumann, Anke
Spiekerkoetter, Ute
author_sort Grünert, Sarah Catharina
collection PubMed
description Glycerol phenylbutyrate (GPB) is an ammonia scavenger drug commonly used in the therapy of patients with urea cycle defects. Reported side effects include body odor, abdominal pain, nausea, burning sensation in mouth, vomiting, and heartburn. We report on a 3‐year‐old late diagnosed female patient with ornithine transcarbamylase deficiency that experienced severe loss of appetite under treatment with GBP. Due to catabolism (calory intake about 400 kcal/day) and the associated risk of metabolic decompensation, GBP treatment was discontinued. Her appetite and eating behavior normalized within 1 day after discontinuation of GBP and switch to sodium benzoate. Our case demonstrates that GBP can cause severe loss of appetite that may put patients at risk of metabolic decompensation and require discontinuation of therapy.
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spelling pubmed-96266622022-11-03 Severe loss of appetite and refusal to eat as severe side effect of glycerol phenylbutyrate Grünert, Sarah Catharina Schumann, Anke Spiekerkoetter, Ute JIMD Rep Case Reports Glycerol phenylbutyrate (GPB) is an ammonia scavenger drug commonly used in the therapy of patients with urea cycle defects. Reported side effects include body odor, abdominal pain, nausea, burning sensation in mouth, vomiting, and heartburn. We report on a 3‐year‐old late diagnosed female patient with ornithine transcarbamylase deficiency that experienced severe loss of appetite under treatment with GBP. Due to catabolism (calory intake about 400 kcal/day) and the associated risk of metabolic decompensation, GBP treatment was discontinued. Her appetite and eating behavior normalized within 1 day after discontinuation of GBP and switch to sodium benzoate. Our case demonstrates that GBP can cause severe loss of appetite that may put patients at risk of metabolic decompensation and require discontinuation of therapy. John Wiley & Sons, Inc. 2022-09-30 /pmc/articles/PMC9626662/ /pubmed/36341170 http://dx.doi.org/10.1002/jmd2.12286 Text en © 2022 The Authors. JIMD Reports published by John Wiley & Sons Ltd on behalf of SSIEM. https://creativecommons.org/licenses/by/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Reports
Grünert, Sarah Catharina
Schumann, Anke
Spiekerkoetter, Ute
Severe loss of appetite and refusal to eat as severe side effect of glycerol phenylbutyrate
title Severe loss of appetite and refusal to eat as severe side effect of glycerol phenylbutyrate
title_full Severe loss of appetite and refusal to eat as severe side effect of glycerol phenylbutyrate
title_fullStr Severe loss of appetite and refusal to eat as severe side effect of glycerol phenylbutyrate
title_full_unstemmed Severe loss of appetite and refusal to eat as severe side effect of glycerol phenylbutyrate
title_short Severe loss of appetite and refusal to eat as severe side effect of glycerol phenylbutyrate
title_sort severe loss of appetite and refusal to eat as severe side effect of glycerol phenylbutyrate
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9626662/
https://www.ncbi.nlm.nih.gov/pubmed/36341170
http://dx.doi.org/10.1002/jmd2.12286
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