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Increased thrombophilic tendency in pediatric cystic fibrosis patients.

Abstract

Thrombophilia has recently been reported to be increased in patients with cystic fibrosis (CF). We wanted to determine whether this was applicable to our population with CF and how our patients compared to the previously reported groups. Seventy one pediatric CF patients were assessed for a thrombophilic tendency, using a lupus anticoagulant screen, protein C, protein S, antithrombin assay, and activated protein C resistance (APCR) screen. The incidence of activate protein C resistance (4.2%) was within expected limits for the general population as was the incidence of antithrombin deficiency. However there was a marked increase in the incidence of lupus anticoagulants (18%) and 14% and 19.7% of the patients showed a reduced protein C and protein S, respectively, far in excess of the general population. This increased incidence of thrombophilia was not related to any specific CF phenotype and while perturbed liver function cannot be entirely ruled out, it appeared unlikely to be responsible for all the abnormal coagulation findings. Despite the apparent thrombophilic tendency, no clinically evident thrombotic episodes were noted during the study period. Thrombophilia is of concern because of the increasingly frequent placement of indwelling catheters in CF patients. The precise cause for the thrombophilic tendency in CF patients is unknown at this stage.

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  • Authors+Show Affiliations

    ,

    Department of Haematology, Women's and Children's Hospital, North Adelaide, South Australia. williams@health.sa.gov.au

    , , , , ,

    Source

    MeSH

    Adolescent
    Antithrombin III
    Antithrombin III Deficiency
    Child
    Child, Preschool
    Cystic Fibrosis
    Female
    Humans
    Incidence
    Infant
    Lupus Coagulation Inhibitor
    Male
    Protein C
    Protein S
    Thrombophilia
    Young Adult

    Pub Type(s)

    Journal Article

    Language

    eng

    PubMed ID

    19605377

    Citation

    Williams, Vaughan, et al. "Increased Thrombophilic Tendency in Pediatric Cystic Fibrosis Patients." Clinical and Applied Thrombosis/hemostasis : Official Journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis, vol. 16, no. 1, 2010, pp. 71-6.
    Williams V, Griffiths AB, Yap ZL, et al. Increased thrombophilic tendency in pediatric cystic fibrosis patients. Clin Appl Thromb Hemost. 2010;16(1):71-6.
    Williams, V., Griffiths, A. B., Yap, Z. L., Martin, J., Smith, G., Couper, R., & Revesz, T. (2010). Increased thrombophilic tendency in pediatric cystic fibrosis patients. Clinical and Applied Thrombosis/hemostasis : Official Journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis, 16(1), pp. 71-6. doi:10.1177/1076029609334627.
    Williams V, et al. Increased Thrombophilic Tendency in Pediatric Cystic Fibrosis Patients. Clin Appl Thromb Hemost. 2010;16(1):71-6. PubMed PMID: 19605377.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Increased thrombophilic tendency in pediatric cystic fibrosis patients. AU - Williams,Vaughan, AU - Griffiths,Adrian B M, AU - Yap,Zen L, AU - Martin,James, AU - Smith,Gregory, AU - Couper,Richard, AU - Revesz,Tamas, Y1 - 2009/07/14/ PY - 2009/7/17/entrez PY - 2009/7/17/pubmed PY - 2010/3/31/medline SP - 71 EP - 6 JF - Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis JO - Clin. Appl. Thromb. Hemost. VL - 16 IS - 1 N2 - Thrombophilia has recently been reported to be increased in patients with cystic fibrosis (CF). We wanted to determine whether this was applicable to our population with CF and how our patients compared to the previously reported groups. Seventy one pediatric CF patients were assessed for a thrombophilic tendency, using a lupus anticoagulant screen, protein C, protein S, antithrombin assay, and activated protein C resistance (APCR) screen. The incidence of activate protein C resistance (4.2%) was within expected limits for the general population as was the incidence of antithrombin deficiency. However there was a marked increase in the incidence of lupus anticoagulants (18%) and 14% and 19.7% of the patients showed a reduced protein C and protein S, respectively, far in excess of the general population. This increased incidence of thrombophilia was not related to any specific CF phenotype and while perturbed liver function cannot be entirely ruled out, it appeared unlikely to be responsible for all the abnormal coagulation findings. Despite the apparent thrombophilic tendency, no clinically evident thrombotic episodes were noted during the study period. Thrombophilia is of concern because of the increasingly frequent placement of indwelling catheters in CF patients. The precise cause for the thrombophilic tendency in CF patients is unknown at this stage. SN - 1938-2723 UR - https://www.unboundmedicine.com/medline/citation/19605377/full_citation L2 - http://journals.sagepub.com/doi/full/10.1177/1076029609334627?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -