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Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
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Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis

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Increased thrombophilic tendency in pediatric cystic fibrosis patients

Vaughan Williams1, Adrian B M Griffiths, Zen L Yap

  • 1Department of Haematology, Women's and Children's Hospital, North Adelaide, South Australia. williams@health.sa.gov.au

Clinical and Applied Thrombosis/Hemostasis : Official Journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis
|July 17, 2009
PubMed

Insights

Cystic fibrosis (CF) patients show increased thrombophilia, indicated by higher rates of lupus anticoagulants and reduced protein C and S levels. This heightened risk for blood clots in CF requires further investigation despite no observed thrombotic events.

Area of Science:

  • Hematology
  • Pediatrics
  • Pulmonology

Background:

  • Thrombophilia, a tendency towards blood clots, has been increasingly reported in cystic fibrosis (CF) patients.
  • Understanding the prevalence and characteristics of thrombophilia in CF is crucial for patient management.

Purpose of the Study:

  • To investigate the incidence of thrombophilic tendencies in a pediatric cohort of cystic fibrosis patients.
  • To compare these findings with previously reported data on thrombophilia in CF.

Main Methods:

  • Seventy-one pediatric CF patients underwent assessment for thrombophilia.
  • Tests included lupus anticoagulant screen, protein C, protein S, antithrombin assays, and activated protein C resistance (APCR) screening.

Main Results:

  • Elevated rates of lupus anticoagulants (18%) and deficiencies in protein C (14%) and protein S (19.7%) were observed, significantly exceeding general population levels.
  • Incidence of activated protein C resistance and antithrombin deficiency were within expected ranges.
  • No correlation was found between thrombophilia and specific CF phenotypes; liver function abnormalities were unlikely to explain all findings.

Conclusions:

  • Pediatric CF patients exhibit a significant increase in specific thrombophilic markers, particularly lupus anticoagulants and reduced protein C/S levels.
  • While no thrombotic events occurred during the study, the identified thrombophilic tendency raises concerns, especially with indwelling catheter use in CF patients.
  • The exact cause of thrombophilia in cystic fibrosis remains undetermined and warrants further research.

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