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.

Increased vigilance needed for the detection of thrombotic complications of central venous access in adolescent cystic fibrosis patients

“…Balfour-Lynn et al also reported greater than expected rates of protein C deficiency (4 vs. 0.2–0.4%) and protein S deficiency (5 vs. 0.3%) in addition to anti-thrombin deficiency (1 vs. 0.2%) and lupus anticoagulant deficiency (9 vs. 1–5%) in CF patients compared to the general population ( 13 ). This study of 204 CF patients was conducted in asymptomatic patients in an out-patient setting, and thus, did not investigate levels in patients with confirmed VT. Another study of 71 patients concurred with Balfour-Lynn et al reporting elevated rates of lupus anti-coagulants (18%), protein C deficiency (14%), and protein S deficiency (19.7%) ( 14 ). Anti-phospholipid antibody levels have also been noted to be raised in children with CF (4–9.5 vs. 1–3%) ( 15 , 16 ).…”

Complications of long and intermediate term venous catheters in cystic fibrosis patients: A multicenter study

“…Thrombophilia is reported in diabetic patients in the general population [21][22][23], presumably on the basis of a proinflammatory state. A thrombophilic state may also pertain in CF, in part due to vitamin K deficiency, which can associate with protein C or S deficiency [24][25][26][27]. Increased frequency of venous thrombosis has been reported in children [28] and adults [29] with B. cepacia spp.…”

Combined lung-liver-pancreas transplantation in a recipient with cystic fibrosis

“…The incidental discovery of a non-occlusive aortic thrombus in the aortic segment of the liver-pancreas graft highlights a potential drawback of this technique. Although some reports link CF with hypercoagulability [19,20], we believe that stasis, particularly at the blind-stapled end of the supra-celiac aorta, was the main contributing factor. Reconstruction of the celiac and superior mesenteric arteries with a donor iliac artery Y-graft, as described by Young et al [12], might reduce this risk.…”