Management Principles for Acute Illness in Patients With Medium-Chain Acyl-Coenzyme A Dehydrogenase Deficiency.
Acyl-CoA Dehydrogenase
/ deficiency
Carnitine
/ therapeutic use
Child
Emergencies
Fluid Therapy
Glucose
/ administration & dosage
Humans
Hypoglycemia
/ etiology
Intraoperative Complications
/ prevention & control
Lipid Metabolism, Inborn Errors
/ complications
Postoperative Complications
/ prevention & control
Premedication
Sweetening Agents
/ administration & dosage
Journal
Pediatrics
ISSN: 1098-4275
Titre abrégé: Pediatrics
Pays: United States
ID NLM: 0376422
Informations de publication
Date de publication:
01 2021
01 2021
Historique:
pubmed:
30
12
2020
medline:
11
5
2021
entrez:
29
12
2020
Statut:
ppublish
Résumé
Medium-chain acyl-coenzyme A dehydrogenase deficiency (MCADD) is a fatty acid oxidation disorder in which the patient is unable to break down fats to produce energy. This disorder places children at risk for metabolic decompensation during periods of stress, such as routine childhood illnesses. The intent of this clinical report is to provide pediatricians with additional information regarding the acute clinical care of patients with MCADD. Although each patient with MCADD will still be expected to have a primary metabolic physician, the involvement of the primary care provider is crucial as well. Appropriate treatment of children with MCADD can lead to avoidance of morbidity and mortality.
Identifiants
pubmed: 33372121
pii: peds.2020-040303
doi: 10.1542/peds.2020-040303
pii:
doi:
Substances chimiques
Sweetening Agents
0
Acyl-CoA Dehydrogenase
EC 1.3.8.7
Glucose
IY9XDZ35W2
Carnitine
S7UI8SM58A
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Subventions
Organisme : NHGRI NIH HHS
ID : U01 HG007674
Pays : United States
Organisme : NINDS NIH HHS
ID : U01 NS134349
Pays : United States
Investigateurs
Emily Chen
(E)
Tracy L Trotter
(TL)
Leah W Burke
(LW)
Timothy A Geleske
(TA)
Robert J Hopkin
(RJ)
Wendy J Introne
(WJ)
Michael J Lyons
(MJ)
Angela E Scheuerle
(AE)
Joan M Stoler
(JM)
Debra L Freedenberg
(DL)
Marilyn C Jones
(MC)
Informations de copyright
Copyright © 2021 by the American Academy of Pediatrics.
Déclaration de conflit d'intérêts
POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.