Thrombosis Research
Volume 121, Issue 2 , Pages 159-162 , 2007

Cystic fibrosis heterozygotes do not have increased platelet activation

  • Inge Tarnow

      Affiliations

    • Department of Physiology and Biochemistry, University of Copenhagen, The Faculty of Life Sciences, Frederiksberg C, Denmark
    • Department of Pediatrics, UMass Memorial Health Care and University of Massachusetts Medical School, Worcester, MA, United States
    • Center for Platelet Function Studies, University of Massachusetts Medical School, Worcester, MA, United States
  • ,
  • Alan D. Michelson

      Affiliations

    • Department of Pediatrics, UMass Memorial Health Care and University of Massachusetts Medical School, Worcester, MA, United States
    • Center for Platelet Function Studies, University of Massachusetts Medical School, Worcester, MA, United States
  • ,
  • Andrew L. Frelinger III

      Affiliations

    • Department of Pediatrics, UMass Memorial Health Care and University of Massachusetts Medical School, Worcester, MA, United States
    • Center for Platelet Function Studies, University of Massachusetts Medical School, Worcester, MA, United States
  • ,
  • Matthew D. Linden

      Affiliations

    • Department of Pediatrics, UMass Memorial Health Care and University of Massachusetts Medical School, Worcester, MA, United States
    • Center for Platelet Function Studies, University of Massachusetts Medical School, Worcester, MA, United States
  • ,
  • Youfu Li

      Affiliations

    • Department of Pediatrics, UMass Memorial Health Care and University of Massachusetts Medical School, Worcester, MA, United States
    • Center for Platelet Function Studies, University of Massachusetts Medical School, Worcester, MA, United States
  • ,
  • Marsha L. Fox

      Affiliations

    • Department of Pediatrics, UMass Memorial Health Care and University of Massachusetts Medical School, Worcester, MA, United States
    • Center for Platelet Function Studies, University of Massachusetts Medical School, Worcester, MA, United States
  • ,
  • Marc R. Barnard

      Affiliations

    • Department of Pediatrics, UMass Memorial Health Care and University of Massachusetts Medical School, Worcester, MA, United States
    • Center for Platelet Function Studies, University of Massachusetts Medical School, Worcester, MA, United States
  • ,
  • Brian P. O'Sullivan

      Affiliations

    • Department of Pediatrics, UMass Memorial Health Care and University of Massachusetts Medical School, Worcester, MA, United States
    • Corresponding Author InformationCorresponding author. Department of Pediatrics, UMass Memorial Health Care, 55 Lake Avenue, North, Worcester, MA 01655, United States. Tel.: +1 508 856 4155; fax: +1 508 856 2609.

Received 22 January 2007 ,Revised 10 April 2007 ,Accepted 16 April 2007.

References 

  1. Gibson RL, Burns JL, Ramsey BW. Pathophysiology and management of pulmonary infections in cystic fibrosis. Am J Respir Crit Care Med. 2003;168:918–951
  2. Sharer N, Schwarz M, Malone G, Howarth A, Painter J, Super M, et al. Mutations of the cystic fibrosis gene in patients with chronic pancreatitis. N Engl J Med. 1998;339:645–652
  3. Wang X, Kim J, McWilliams R, Cutting GR. Increased prevalence of chronic rhinosinusitis in carriers of a cystic fibrosis mutation. Arch Otolaryngol Head Neck Surg. 2005;131:237–240
  4. Chillon M, Casals T, Mercier B, Bassas L, Lissens W, Silber S, et al. Mutations in the cystic fibrosis gene in patients with congenital absence of the vas deferens. N Engl J Med. 1995;332:1475–1480
  5. Freedman SD, Blanco PG, Zaman MM, Shea JC, Ollero M, Hopper IK, et al. Association of cystic fibrosis with abnormalities in fatty acid metabolism. N Engl J Med. 2004;350:560–569
  6. Zaman MM, Gelrud A, Junaidi O, Regan MM, Warny M, Shea JC, et al. Interleukin 8 secretion from monocytes of subjects heterozygous for the deltaF508 cystic fibrosis transmembrane conductance regulator gene mutation is altered. Clin Diagn Lab Immunol. 2004;11:819–824
  7. O'Sullivan BP, Linden MD, Frelinger AL, Barnard MR, Spencer-Manzon M, Morris JE, et al. Platelet activation in cystic fibrosis. Blood. 2005;105:4635–4641
  8. Samuels CE, Robinson PG, Elliott RB. Decreased inhibition of platelet aggregation by PGE1 in children with cystic fibrosis and their parents. Prostaglandins. 1975;10:617–621
  9. Ulane MM, Butler JD, Peri A, Miele L, Ulane RE, Hubbard VS. Cystic fibrosis and phosphatidylcholine biosynthesis. Clin Chim Acta. 1994;230:109–116
  10. Mikhailidis DP, Stead RJ, Barradas MA, Hodson ME, Batten JC, Dandona P. Platelet abnormalities in patients with cystic fibrosis and obligate heterozygotes. Haematologica. 1990;75:137–140
  11. Linden MD, Frelinger AL, Barnard MR, Przyklenk K, Furman MI, Michelson AD. Application of flow cytometry to platelet disorders. Semin Thromb Hemost. 2004;30:501–511
  12. Michelson AD, Barnard MR, Krueger LA, Valeri CR, Furman MI. Circulating monocyte-platelet aggregates are a more sensitive marker of in vivo platelet activation than platelet surface P-selectin: studies in baboons, human coronary intervention, and human acute myocardial infarction. Circulation. 2001;104:1533–1537
  13. Yoshimura K, Nakamura H, Trapnell BC, Chu CS, Dalemans W, Pavirani A, et al. Expression of the cystic-fibrosis transmembrane conductance regulator gene in cells of nonepithelial origin. Nucleic Acids Res. 1991;19:5417–5423

PII: S0049-3848(07)00159-4

doi: 10.1016/j.thromres.2007.04.004

Thrombosis Research
Volume 121, Issue 2 , Pages 159-162 , 2007