Related Experiment Video
Updated: Jul 4, 2026

Quantification of Coenzyme A in Cells and Tissues
Published on: September 27, 2019
Decreased total serum coenzyme-Q10 concentrations: a longitudinal study in children with cystic fibrosis
Theresa A Laguna1, Marci K Sontag, Iris Osberg
1Department of Pediatrics, University of Colorado, Denver, School of Medicine, Aurora, CO, USA.
Insights
Low coenzyme Q(10) (Co-Q(10)) levels are common in children with cystic fibrosis (CF) and pancreatic insufficiency (PI). Further research is needed to determine if Co-Q(10) supplementation benefits these patients.
Area of Science:
- Biochemistry
- Pediatric Medicine
- Nutritional Science
Background:
- Coenzyme Q(10) (Co-Q(10)) is a vital antioxidant.
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs.
- Assessing Co-Q(10) levels in pediatric CF patients is crucial for understanding their health status.
Purpose of the Study:
- To measure total serum Co-Q(10) levels in children with CF.
- To explore the relationship between Co-Q(10) levels and clinical outcomes in pediatric CF patients.
Main Methods:
- A prospective cohort study involved 381 children with CF.
- Serum Co-Q(10) levels were analyzed annually using high-performance liquid chromatography.
- Associations with demographic data and clinical outcomes were investigated.
Main Results:
- 188 out of 381 initial measurements showed deficient Co-Q(10) levels.
- Low Co-Q(10) was significantly more common in patients with pancreatic insufficiency (PI) (55%) versus pancreatic sufficiency (PS) (3%).
- Low Co-Q(10) correlated with Pseudomonas aeruginosa colonization in infants with PI and CF, but not with lung function or hospitalizations.
Conclusions:
- Persistently low serum Co-Q(10) is prevalent in children with CF and PI.
- Further prospective studies are warranted to evaluate the efficacy of Co-Q(10) supplementation in CF patients.
Objective:
To assess total serum levels of coenzyme Q(10) (Co-Q(10)), an important antioxidant, in children with cystic fibrosis (CF) and to investigate an association between Co-Q(10) level and clinical outcome.
Study Design:
Co-Q(10) levels were measured annually in a prospective cohort study of 381 children with CF. A total of 1092 serum levels of total Co-Q(10) were obtained by high-performance liquid chromatography and ultraviolet light detection. Associations of Co-Q(10) with demographic variables and clinical outcomes were investigated.
Results:
Of the 381 initial total serum Co-Q(10) measurements, 188 were in the deficient range. Low Co-Q(10) was significantly more prevalent in patients with pancreatic insufficiency (PI) (55%) compared with patients with pancreatic sufficiency (PS) (3%); 22% of the patients with PI exhibited persistently low Co-Q(10) levels. Low Co-Q(10) levels were significantly associated with Pseudomonas aeruginosa colonization in patients with PI and CF under age 24 months, but not with subsequent lung function or hospitalization rates. Low Co-Q(10) levels were related to other markers of nutritional status, including total lipids, beta-carotene, and alpha-tocopherol.
Conclusions:
Persistently low total serum Co-Q(10) levels are common in children with CF and PI. A prospective study is indicated to determine whether Co-Q(10) supplementation in CF is beneficial.
Related Concept Videos
Cystic Fibrosis: Management
Sinus disease and chronic sinusitis...
Cystic Fibrosis: Pathogenesis
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...
Pharmacokinetics in Pediatric Patients: Drug Metabolism
Chronic Obstructive Pulmonary Disease-IV: Assessement and Diagnostic Studies
Medical History
Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test
Effect of Hepatic Disease on Pharmacokinetics: Dose Adjustments Due to Hepatic Impairment

