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Anesthetic Management for Fracture Head of Radius in a Child with Glutaric Aciduria type-1

Glutaric aciduria Type 1 (GA-1) is an autosomal recessive metabolic disorder that results from deficiency of enzyme glutaryl-CoA dehydrogenase. This gives rise to elevated neurotoxic glutaric acid and 3-hydroxyglutaric acid as well as nontoxic glutarylcarnitine in body fluids. The enzyme defect lead...

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Autores principales: Mehta, Nandita, Kaur, Gurleen
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6020594/
https://www.ncbi.nlm.nih.gov/pubmed/29962642
http://dx.doi.org/10.4103/aer.AER_34_18
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author Mehta, Nandita
Kaur, Gurleen
author_facet Mehta, Nandita
Kaur, Gurleen
author_sort Mehta, Nandita
collection PubMed
description Glutaric aciduria Type 1 (GA-1) is an autosomal recessive metabolic disorder that results from deficiency of enzyme glutaryl-CoA dehydrogenase. This gives rise to elevated neurotoxic glutaric acid and 3-hydroxyglutaric acid as well as nontoxic glutarylcarnitine in body fluids. The enzyme defect leads to secondary damage to central nervous system due to the accumulation of glutaric acid. Approximately 90% people will develop the neurological disease during a finite period of brain development (3–36 months) following an acute encephalopathic crisis often precipitated by gastroenteritis, immunization, surgical intervention, and intercurrent febrile illness. GA-1 can also develop insidiously without clinically apparent crisis in 10%–20% of the patients. We present a 10-year-old male child with GA-1 who required anesthetic care for fracture (left) neck of radius. Strategies for anesthetic management should include prevention of hypoglycemia, dehydration, electrolyte imbalance, and sufficient analgesia to prevent surgical stress.
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spelling pubmed-60205942018-06-29 Anesthetic Management for Fracture Head of Radius in a Child with Glutaric Aciduria type-1 Mehta, Nandita Kaur, Gurleen Anesth Essays Res Case Report Glutaric aciduria Type 1 (GA-1) is an autosomal recessive metabolic disorder that results from deficiency of enzyme glutaryl-CoA dehydrogenase. This gives rise to elevated neurotoxic glutaric acid and 3-hydroxyglutaric acid as well as nontoxic glutarylcarnitine in body fluids. The enzyme defect leads to secondary damage to central nervous system due to the accumulation of glutaric acid. Approximately 90% people will develop the neurological disease during a finite period of brain development (3–36 months) following an acute encephalopathic crisis often precipitated by gastroenteritis, immunization, surgical intervention, and intercurrent febrile illness. GA-1 can also develop insidiously without clinically apparent crisis in 10%–20% of the patients. We present a 10-year-old male child with GA-1 who required anesthetic care for fracture (left) neck of radius. Strategies for anesthetic management should include prevention of hypoglycemia, dehydration, electrolyte imbalance, and sufficient analgesia to prevent surgical stress. Medknow Publications & Media Pvt Ltd 2018 /pmc/articles/PMC6020594/ /pubmed/29962642 http://dx.doi.org/10.4103/aer.AER_34_18 Text en Copyright: © 2018 Anesthesia: Essays and Researches http://creativecommons.org/licenses/by-nc-sa/4.0 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Case Report
Mehta, Nandita
Kaur, Gurleen
Anesthetic Management for Fracture Head of Radius in a Child with Glutaric Aciduria type-1
title Anesthetic Management for Fracture Head of Radius in a Child with Glutaric Aciduria type-1
title_full Anesthetic Management for Fracture Head of Radius in a Child with Glutaric Aciduria type-1
title_fullStr Anesthetic Management for Fracture Head of Radius in a Child with Glutaric Aciduria type-1
title_full_unstemmed Anesthetic Management for Fracture Head of Radius in a Child with Glutaric Aciduria type-1
title_short Anesthetic Management for Fracture Head of Radius in a Child with Glutaric Aciduria type-1
title_sort anesthetic management for fracture head of radius in a child with glutaric aciduria type-1
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6020594/
https://www.ncbi.nlm.nih.gov/pubmed/29962642
http://dx.doi.org/10.4103/aer.AER_34_18
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