Management Principles for Acute Illness in Patients With Medium-Chain Acyl-Coenzyme A Dehydrogenase Deficiency

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...

Full description

Saved in:
Bibliographic Details
Published in:Pediatrics (Evanston) 2021-01, Vol.147 (1), p.1
Main Authors: McGregor, Tracy L, Berry, Susan A, Dipple, Katrina M, Hamid, Rizwan
Format: Article
Language:English
Subjects:
Acute disease
Acyl-CoA Dehydrogenase - deficiency
Care and treatment
Carnitine - therapeutic use
Child
Children
Coenzyme A
Dehydrogenases
Diseases
Emergencies
Fluid Therapy
Glucose - administration & dosage
Humans
Hypoglycemia - etiology
Hypoglycemia - therapy
Intraoperative Complications - prevention & control
Lipid Metabolism, Inborn Errors - complications
Lipid Metabolism, Inborn Errors - diagnosis
Lipid Metabolism, Inborn Errors - therapy
Medium chain acyl-CoA dehydrogenase deficiency
Metabolism
Morbidity
Oxidation
Patients
Pediatric diseases
Pediatrics
Postoperative Complications - prevention & control
Premedication
Primary care
Sweetening Agents - administration & dosage
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:0031-4005
1098-4275
DOI:10.1542/PEDS.2020-040303