Effect of an antioxidant-rich multivitamin supplement in cystic fibrosis

Sagel, Scott D.; Sontag, Marci K.; Anthony, Meg; Emmett, Peggy; Papas, Konstantinos

doi:10.1016/j.jcf.2010.09.005

Cited by 51 publications

(29 citation statements)

References 36 publications

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“…AquADEKs ® by Yasoo Health, Inc. is a vitamin supplement specifically formulated to meet the antioxidant needs of those with CF, and is commercially available. It has been shown to improve lung function, normalize vitamin levels in plasma and reduce neutrophilic inflammation (Sagel et al, 2011).…”

Section: Current Cf Therapiesmentioning

confidence: 99%

Channel Replacement Therapy for Cystic Fibrosis

Tomich¹,

Bukovnik²,

Layman³

et al. 2012

Cystic Fibrosis - Renewed Hopes Through Research

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Section: Current Cf Therapiesmentioning

confidence: 99%

Channel Replacement Therapy for Cystic Fibrosis

Tomich¹,

Bukovnik²,

Layman³

et al. 2012

Cystic Fibrosis - Renewed Hopes Through Research

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“…In fact, based in large measure on the results of the experimental animal studies mentioned above, a clinical trial of combined cholesterol-antioxidant therapy for SLOS is currently underway at Children’s Hospital Colorado.The initial results appear promising [23] based upon electroretinographic (ERG) testing outcomes, significant improvement in retinal function has been achieved by treating children affected with SLOS with cholesterol supplementation augmented with AquADEKs ® , a multivitamin and mineral formulation that includes several antioxidants and micronutrients, including vitamins A, D, E, and K. AquADEKs ® has been used previously for treatment of cystic fibrosis patients [24,25], but has been “repurposed” for off-label use in this SLOS treatment trial.…”

mentioning

confidence: 99%

Antioxidants: The Missing Key to Improved Therapeutic Intervention in Smith-Lemli-Opitz Syndrome

Fliesler

2013

Hereditary Genetics

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Smith-Lemli-Opitz Syndrome (SLOS) is a recessive hereditary disease caused by an enzymatic defect in the biosynthesis of cholesterol. To date, the therapeutic standard of care for this disease has been cholesterol supplementation therapy. However, the efficacy of this treatment is extremely variable and, in many if not most cases, is poor. Results of studies using animal models of SLOS have suggested that cholesterol deficiencyand/or the aberrant accumulation of the immediate precursor of cholesterol (7-dehydrocholesterol (7DHC)), per se, may not be the sole culprits in the pathobiology of this disease. Rather, cytotoxic oxysterol by-products derived specifically from 7DHC are thought to be additional, significant, causative players in the disease mechanism. Based in large measure upon such studies, a recent clinical trial, comparing the therapeutic efficacyof cholesterol supplementation alone vs. combined cholesterol-antioxidant supplementation in SLOS patients, has provided extremely encouraging results that tend to both validate the proposed role of oxysterols in the pathobiology of SLOS as well as indicate an improved treatment for this and related diseases.

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“…The increase in antioxidant vitamin serum levels occurred with respect to the other liposoluble vitamins (D and K). Respiratory function barely improved, and the negligible reduction in urinary 8-isoprostanes meant any impact of this supplementation on OxS was unlikely (134,161). Some authors also reported that a resistance to oxidation correlated with a-tocopherol levels in low-density lipoprotein and a reduction in malondialdehyde (156) ( Table 2).…”

Section: Antioxidantsmentioning

confidence: 99%

Cystic Fibrosis-Related Oxidative Stress and Intestinal Lipid Disorders

Kleme

Lévy²

2015

Antioxidants & Redox Signaling

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Significance: Cystic fibrosis (CF) is the most common lethal genetic disorder in the Caucasian people. It is due to the mutation of cystic fibrosis transmembrane conductance regulator (CFTR) gene located on the long arm of the chromosome 7, which encodes for CFTR protein. The latter, an adenosine triphosphate binding cassette, is a transmembrane chloride channel that is also involved in glutathione transport. As glutathione/glutathione disulfide constitutes the most important pool of cellular redox systems, CFTR defects could thus disrupt the intracellular redox balance. Resulting multisystemic diseases are essentially characterized by a chronic respiratory failure, a pancreatic insufficiency, an essential fatty acid deficiency (EFAD), and inadequate levels of antioxidant vitamins. Recent Advances: The pathophysiology of CF is complex; however, several mechanisms are proposed, including oxidative stress (OxS) whose implication is recognized and has been clearly demonstrated in CF airways. Critical Issues: Little is known about OxS intrinsic triggers and its own involvement in intestinal lipid disorders. Despite the regular administration of pancreatic supplements, high-fat high-calorie diets, and antioxidant fat-soluble vitamins, there is a persistence of steatorrhea, EFAD, and harmful OxS. Intriguingly, several trials with elevated doses of antioxidant vitamins have not yielded significant improvements. Future Directions: The main sources and self-maintenance of OxS in CF should be clarified to improve treatment of patients. Therefore, this review will discuss the potential sources and study the mechanisms of OxS in the intestine, known to develop various complications, and its involvement in intestinal lipid disorders in CF patients. Antioxid. Redox Signal. 22, 614-631.

show abstract

Effect of an antioxidant-rich multivitamin supplement in cystic fibrosis

Cited by 51 publications

References 36 publications

Channel Replacement Therapy for Cystic Fibrosis

Channel Replacement Therapy for Cystic Fibrosis

Antioxidants: The Missing Key to Improved Therapeutic Intervention in Smith-Lemli-Opitz Syndrome

Cystic Fibrosis-Related Oxidative Stress and Intestinal Lipid Disorders

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