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Venous thromboembolism in cystic fibrosis.
Pediatr Pulmonol 2012; 47(2):105-12PP

Abstract

The incidence of venous thromboembolism (VTE) is increasing in the pediatric population. Individuals with cystic fibrosis (CF) have an increased risk of thrombosis due to central venous catheters (CVCs), as well as acquired thrombophilia secondary to inflammation, or deficiencies of anticoagulant proteins due to vitamin K deficiency and/or liver dysfunction. CVC-associated thrombosis commonly results in line occlusion, but may develop into serious life-threatening conditions such as deep venous thrombosis (DVT), superior vena cava syndrome or pulmonary embolism (PE). Post-thrombotic syndrome (PTS) may be a long complication. Local occlusion of the catheter tip may be managed with instillation of thrombolytics (such as tPA) within the lumen of the catheter; however, CVC-associated thrombosis involving the proximal veins is most often is treated with systemic anticoagulation. Initial treatment with heparin is a standard approach, but thrombolytic therapy, which may carry higher bleeding risks, should be considered for life and limb threatening episodes of VTE. Recommended duration of anticoagulation with low molecular weight heparin (LMWH) or warfarin ranges from 3 to 6 months for major removable thrombotic risks; longer anticoagulation is considered for recurrent thrombosis, major persistent thrombophilia, or the continued presence of a major risk factor such as a CVC. While CVCs are the most common risk for development of VTE in children, studies have not demonstrated a clear benefit with routine use of systemic thromboprophylaxis. The incidence and risk factors of VTE in CF patients will be reviewed and principles of diagnosis and management will be summarized.

Authors+Show Affiliations

Division of Pediatric Hematology, The Johns Hopkins University, Baltimore, Maryland, USA. ctakemot@jhmi.edu

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

22006666

Citation

Takemoto, Clifford M.. "Venous Thromboembolism in Cystic Fibrosis." Pediatric Pulmonology, vol. 47, no. 2, 2012, pp. 105-12.
Takemoto CM. Venous thromboembolism in cystic fibrosis. Pediatr Pulmonol. 2012;47(2):105-12.
Takemoto, C. M. (2012). Venous thromboembolism in cystic fibrosis. Pediatric Pulmonology, 47(2), pp. 105-12. doi:10.1002/ppul.21566.
Takemoto CM. Venous Thromboembolism in Cystic Fibrosis. Pediatr Pulmonol. 2012;47(2):105-12. PubMed PMID: 22006666.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Venous thromboembolism in cystic fibrosis. A1 - Takemoto,Clifford M, Y1 - 2011/10/17/ PY - 2011/06/20/received PY - 2011/09/13/accepted PY - 2011/10/19/entrez PY - 2011/10/19/pubmed PY - 2012/5/23/medline SP - 105 EP - 12 JF - Pediatric pulmonology JO - Pediatr. Pulmonol. VL - 47 IS - 2 N2 - The incidence of venous thromboembolism (VTE) is increasing in the pediatric population. Individuals with cystic fibrosis (CF) have an increased risk of thrombosis due to central venous catheters (CVCs), as well as acquired thrombophilia secondary to inflammation, or deficiencies of anticoagulant proteins due to vitamin K deficiency and/or liver dysfunction. CVC-associated thrombosis commonly results in line occlusion, but may develop into serious life-threatening conditions such as deep venous thrombosis (DVT), superior vena cava syndrome or pulmonary embolism (PE). Post-thrombotic syndrome (PTS) may be a long complication. Local occlusion of the catheter tip may be managed with instillation of thrombolytics (such as tPA) within the lumen of the catheter; however, CVC-associated thrombosis involving the proximal veins is most often is treated with systemic anticoagulation. Initial treatment with heparin is a standard approach, but thrombolytic therapy, which may carry higher bleeding risks, should be considered for life and limb threatening episodes of VTE. Recommended duration of anticoagulation with low molecular weight heparin (LMWH) or warfarin ranges from 3 to 6 months for major removable thrombotic risks; longer anticoagulation is considered for recurrent thrombosis, major persistent thrombophilia, or the continued presence of a major risk factor such as a CVC. While CVCs are the most common risk for development of VTE in children, studies have not demonstrated a clear benefit with routine use of systemic thromboprophylaxis. The incidence and risk factors of VTE in CF patients will be reviewed and principles of diagnosis and management will be summarized. SN - 1099-0496 UR - http://www.unboundmedicine.com/medline/citation/22006666/full_citation L2 - https://doi.org/10.1002/ppul.21566 DB - PRIME DP - Unbound Medicine ER -