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Prevalence of thrombophilia and catheter-related thrombosis in cystic fibrosis.
Pediatr Pulmonol 2005; 39(2):156-61PP

Abstract

Venous thrombosis in children and young adults is frequently associated with predisposing conditions and with an indwelling catheter or totally implantable venous access device (TIVAD). These systems are commonly used for the delivery of antibiotic therapy in patients with cystic fibrosis (CF). We reviewed our CF center's history of catheter-related events over 13 years and prospectively investigated the presence of risk factors for thrombosis in 66 children and adults with CF (age, 3-38 years; 32 females). Five thrombotic events had occurred in 4 patients, 2 of whom carried the factor V Leiden mutation. Five asymptomatic patients were diagnosed with heterozygous mutations of the factor V or prothrombin gene. Functional activity of protein C was decreased in 13 subjects, with a correlation to impaired liver function. Protein S activity was abnormal in 20 patients and was related to CF genotype. Anti-phospholipid antibodies (APA) were present in 6 asymptomatic patients. A reinvestigation after 3 years confirmed protein S deficiency in 12 of 14 patients, while most abnormalities for protein C or APA were inconsistent. In conclusion, a thrombophilic state was detected in 53% of patients, and 2 out of 4 subjects with TIVAD-related thrombosis carried a genetic defect. It may thus be helpful to include a hemostatic evaluation in the clinical decision process for or against TIVAD insertion in eligible CF patients.

Authors+Show Affiliations

Department of Pediatrics, University Hospital, University of Technology-Aachen, Aachen, Germany. Barker@rwth-aachen.deNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Journal Article

Language

eng

PubMed ID

15633202

Citation

Barker, Michael, et al. "Prevalence of Thrombophilia and Catheter-related Thrombosis in Cystic Fibrosis." Pediatric Pulmonology, vol. 39, no. 2, 2005, pp. 156-61.
Barker M, Thoenes D, Döhmen H, et al. Prevalence of thrombophilia and catheter-related thrombosis in cystic fibrosis. Pediatr Pulmonol. 2005;39(2):156-61.
Barker, M., Thoenes, D., Döhmen, H., Friedrichs, F., Pfannenstiel, C., & Heimann, G. (2005). Prevalence of thrombophilia and catheter-related thrombosis in cystic fibrosis. Pediatric Pulmonology, 39(2), pp. 156-61.
Barker M, et al. Prevalence of Thrombophilia and Catheter-related Thrombosis in Cystic Fibrosis. Pediatr Pulmonol. 2005;39(2):156-61. PubMed PMID: 15633202.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Prevalence of thrombophilia and catheter-related thrombosis in cystic fibrosis. AU - Barker,Michael, AU - Thoenes,Daniel, AU - Döhmen,Hiltrud, AU - Friedrichs,Frank, AU - Pfannenstiel,Claus, AU - Heimann,Gerhard, PY - 2005/1/6/pubmed PY - 2005/5/25/medline PY - 2005/1/6/entrez SP - 156 EP - 61 JF - Pediatric pulmonology JO - Pediatr. Pulmonol. VL - 39 IS - 2 N2 - Venous thrombosis in children and young adults is frequently associated with predisposing conditions and with an indwelling catheter or totally implantable venous access device (TIVAD). These systems are commonly used for the delivery of antibiotic therapy in patients with cystic fibrosis (CF). We reviewed our CF center's history of catheter-related events over 13 years and prospectively investigated the presence of risk factors for thrombosis in 66 children and adults with CF (age, 3-38 years; 32 females). Five thrombotic events had occurred in 4 patients, 2 of whom carried the factor V Leiden mutation. Five asymptomatic patients were diagnosed with heterozygous mutations of the factor V or prothrombin gene. Functional activity of protein C was decreased in 13 subjects, with a correlation to impaired liver function. Protein S activity was abnormal in 20 patients and was related to CF genotype. Anti-phospholipid antibodies (APA) were present in 6 asymptomatic patients. A reinvestigation after 3 years confirmed protein S deficiency in 12 of 14 patients, while most abnormalities for protein C or APA were inconsistent. In conclusion, a thrombophilic state was detected in 53% of patients, and 2 out of 4 subjects with TIVAD-related thrombosis carried a genetic defect. It may thus be helpful to include a hemostatic evaluation in the clinical decision process for or against TIVAD insertion in eligible CF patients. SN - 8755-6863 UR - https://www.unboundmedicine.com/medline/citation/15633202/Prevalence_of_thrombophilia_and_catheter_related_thrombosis_in_cystic_fibrosis_ L2 - https://doi.org/10.1002/ppul.20158 DB - PRIME DP - Unbound Medicine ER -