The Journal of Pediatrics
Volume 153, Issue 3 , Pages 402-407.e1 , September 2008

Decreased Total Serum Coenzyme-Q10 Concentrations: A Longitudinal Study in Children with Cystic Fibrosis

  • Theresa A. Laguna, MD

      Affiliations

    • From the Department of Pediatrics, University of Colorado, Denver, School of Medicine, Aurora, CO
    • Corresponding Author InformationReprint requests: Theresa A. Laguna, MD, The Children's Hospital, 13123 East 16th Avenue, B-395, Aurora, CO 80045
    • ,
  • Marci K. Sontag, PhD

      Affiliations

    • Department of Preventive Medicine and Biometrics, University of Colorado, Denver, School of Medicine, Aurora, CO
    • Mike McMorris Cystic Fibrosis Research and Treatment Center, The Children's Hospital, Denver, CO
    • ,
  • Iris Osberg, MS

      Affiliations

    • From the Department of Pediatrics, University of Colorado, Denver, School of Medicine, Aurora, CO
    • ,
  • Jeffrey S. Wagener, MD

      Affiliations

    • From the Department of Pediatrics, University of Colorado, Denver, School of Medicine, Aurora, CO
    • ,
  • Frank J. Accurso, MD

      Affiliations

    • From the Department of Pediatrics, University of Colorado, Denver, School of Medicine, Aurora, CO
    • ,
  • Ronald J. Sokol, MD

      Affiliations

    • From the Department of Pediatrics, University of Colorado, Denver, School of Medicine, Aurora, CO
    • Section of Pediatric Gastroenterology, Hepatology and Nutrition, The Children's Hospital, Denver, CO

Received 12 June 2007 ,Revised 12 December 2007 ,Accepted 18 March 2008.

References 

  1. Welsh MJ, Ramsey BW, Accurso FJ, Cutting GR. Cystic fibrosis. In:  Scribers CR,  Beaudet AL,  Valle D,  Sly WS editor. Metabolic and Molecular Basis of Inherited Disease. 8th edition. New York: McGraw-Hill; 2001;p. 521–588
  2. Starosta V, Rietschel E, Paul K, Baumann U, Griese M. Oxidative changes of bronchoalveolar proteins in cystic fibrosis. Chest. 2006;129:431–437
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  7. Winklhofer-Roob BM, Ziouzenkova O, Puhl H, Ellemunter H, Greiner P, Muller G, et al. Impaired resistance to oxidation of low-density lipoprotein in cystic fibrosis: improvement with vitamin E supplementation. Free Radic Biol Med. 1995;19:725–733
  8. Artuch R, Vilaseca MA, Moreno J, Lambruschini N, Cambra FJ, Campistol J. Decreased serum ubiquinone-10 concentrations in phenylketonuria. Am J Clin Nutr. 1999;70:892–895
  9. Crane FL. Biochemical functions of coenzyme Q10. J Am Coll Nutr. 2001;20:591–598
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  11. Oudshoorn JH, Lecluse ALY, van den Berg R, Vaes WHJ, van der Laag J, Houwen RHJ. Decreased coenzyme Q10 concentration in plasma of children with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2006;43:646–650
  12. Axcan Pharma Inc.. ADEK vitamins. http://www.axcan.comAccessed June 1, 2007
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  14. Source CF. Source CF Multivitamin Softgels. http://www.sourcecf.comAccessed June 1, 2007
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  17. Sontag MK, Corey M, Hokanson JE, Marshall JA, Sommer SS, Zerbe GO, et al. Genetic and physiologic correlates of longitudinal immunoreactive trypsinogen decline in infants with cystic fibrosis identified through newborn screening. J Pediatr. 2006;149:650–657
  18. Port CF. Consensus conferences. Concepts in CF care: pediatric nutrition for patients with cystic fibrosis. http://www.portcf.org/educationalmaterials.aspAccessed May 15, 2007
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  23. Grossi G, Bargossi AM, Fiorella PL, Piazzi S, Battino M, Bianchi GP. Improved high-performance liquid chromatography method for determination of coenzyme Q10 in plasma. J Chromatogr. 1992;593:217–226
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  25. Sokol RJ, Heubi JE, Iannaccone ST, Bove KE, Balistreri WF. Vitamin E deficiency with normal serum vitamin E concentrations in children with chronic cholestasis. N Engl J Med. 1984;310:1209–1212
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  28. Jacob RA, Sanstead HH, Solomons NW, Rieger C, Rothberg R. Zinc status and vitamin A transport in cystic fibrosis. Am J Clin Nutr. 1978;31:638–644
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 Supported by grants from the Cystic Fibrosis Foundation (CFF ACCURS00A1 and CFF LAGUNA06A0) and the National Institutes of Health (1U01HL081335-01 and M01RR00069).

 The authors have no potential, perceived, or real conflicts of interest to declare.

PII: S0022-3476(08)00221-7

doi: 10.1016/j.jpeds.2008.03.028

The Journal of Pediatrics
Volume 153, Issue 3 , Pages 402-407.e1 , September 2008

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