metricas
covid
Buscar en
Revista Española de Anestesiología y Reanimación (English Edition)
Toda la web
Inicio Revista Española de Anestesiología y Reanimación (English Edition) Perioperative anesthetic management of very long-chain acyl-coenzyme a dehydroge...
Journal Information
Vol. 70. Issue 4.
Pages 231-234 (April 2023)
Share
Share
Download PDF
More article options
Vol. 70. Issue 4.
Pages 231-234 (April 2023)
Case report
Perioperative anesthetic management of very long-chain acyl-coenzyme a dehydrogenase deficiency
Manejo anestésico perioperatorio de la deficiencia de acil-coenzima A deshidrogenasa de cadena muy larga
C. Peixotoa,
Corresponding author
claudiapeixoto_950@hotmail.com

Corresponding author.
, C. Douradoa, H. Santosb, P. Robertoa
a Medical Degree, Department of Anesthesiology, Centro Hospitalar de Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal
b Medical Degree, Department of Pediatrics, Centro Hospitalar de Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal
Article information
Abstract
Full Text
Bibliography
Download PDF
Statistics
Abstract

Very long-chain acyl-coenzyme A dehydrogenase deficiency is a rare disorder of β-oxidation fatty acid metabolism that results in susceptibility to hypoglycemia, liver failure, cardiomyopathy and rhabdomyolysis during catabolic situations. We report the case of a 10-year-old male undergoing a totally implanted central venous catheter placement during hospitalisation for rhabdomyolysis, who was successfully managed with general anesthesia with nitrous oxide, sevoflurane and remifentanil. No hypoglycemia occurred and creatine kinase levels did not increase in the perioperative period. We describe the challenges encountered and the strategies used to avoid further decompensation of the disease due to surgical stress.

Keywords:
Anesthesia
Very long-chain acyl-coenzyme A dehydrogenase deficiency
Lipid metabolism
Fatty acids
Rhabdomyolysis
Metabolic disease
Resumen

La deficiencia de acil-coenzima A deshidrogenasa de cadena muy larga es un trastorno infrecuente del metabolismo de β-oxidación de los ácidos grasos que origina susceptibilidad a hipoglucemia, fallo hepático, cardiomiopatía y rabdomiólisis durante las situaciones catabólicas. Reportamos el caso de un varón de diez años de edad programado para colocación de catéter venoso central totalmente implantado durante su hospitalización por rabdomiólisis, que fue exitosamente gestionada con anestesia general con óxido nitroso, sevoflurano y remifentanilo. No se produjo hipoglucemia y los niveles de creatina quinasa no se incrementaron durante el periodo perioperatorio. Describimos las dificultades a que nos enfrentamos y las estrategias utilizadas para evitar mayor descompensación de la enfermedad debida al estrés quirúrgico.

Palabras clave:
Anestesia
Deficiencia de acil-coenzima A deshidrogenasa de cadena muy larga
Metabolismo lipídico
Ácidos grasos
Rabdomiolisis
Enfermedad metabólica

Article

These are the options to access the full texts of the publication Revista Española de Anestesiología y Reanimación (English Edition)
Subscriber
Subscriber

If you already have your login data, please click here .

If you have forgotten your password you can you can recover it by clicking here and selecting the option “I have forgotten my password”
Subscribe
Subscribe to

Revista Española de Anestesiología y Reanimación (English Edition)

Purchase
Purchase article

Purchasing article the PDF version will be downloaded

Price 19.34 €

Purchase now
Contact
Phone for subscriptions and reporting of errors
From Monday to Friday from 9 a.m. to 6 p.m. (GMT + 1) except for the months of July and August which will be from 9 a.m. to 3 p.m.
Calls from Spain
932 415 960
Calls from outside Spain
+34 932 415 960
E-mail
Article options
es en pt

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?

Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos

Quizás le interese:
10.1016/j.redare.2023.03.004
No mostrar más