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

Kandamany N, Elnazir B, Greally P - Front Pediatr (2014)

View Article: PubMed Central - PubMed

Affiliation: Paediatric Cystic Fibrosis Department, Adelaide and Meath National Children's Hospital, Tallaght , Dublin , Ireland.

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Cystic fibrosis (CT) is a genetically inherited, multisystem condition, resulting from abnormal salt and water transport across epithelial surfaces... Advances in medical treatments have resulted in vastly improved care of CF patients... The use of TIVADs in children with CF was first described in 1986... These devices require minimal care, do not limit physical activity and are relatively tamper-proof... These range from microthrombi occluding the tip of venous catheters to potentially life-threatening venous thrombosis (VTs), occasionally, resulting in pulmonary and paradoxical emboli... Deficiencies in both these proteins are possibly due to vitamin K deficiency or hepatic dysfunction... 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... While TIVADs are not the sole risk factor for VTs in CF patients, the risk of venous thromboembolism (VTE) does increase with the duration of the CVCs... Munck et al. concluded that laboratory thrombophilia screening was poorly predictive and thus not recommended routinely in patients prior to TIVAD insertion, but that a focused medical history (assessing for thrombotic risk factors) and prospective Doppler US scans might be useful in identifying asymptomatic catheter VT... Given the evidence we have reviewed, we suggest that an extended coagulation panel (incorporating at a minimum, protein C, protein S, anti-thrombin, and factor VIII and factor V Leiden levels) and Doppler ultrasound screening be conducted at regular intervals in patients with TIVADs who have had a history of a VT, as well as prior to TIVAD insertion... There has also been a move toward using peripherally inserted central catheters (PICCs) in CF patients, which are not without their own complications... In conclusion, the marked improvement in the early detection and survival of CF patients has seen a concomitant rise in challenges faced by late childhood and early adolescence in this group of patients... Presently, there is no clear consensus as to whether to routinely screen CF patients to identify those at risk for significant thrombosis prior to the development of VT... Thromboprophylaxis cannot be recommended routinely at this stage... We strongly feel this is an area that would benefit from larger and more robust studies to achieve early identification of CF patients who are at risk for serious thrombotic and thromboembolic complications arising from their indwelling central lines.

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Related in: MedlinePlus

(A) Right atrial thrombus on echocardiography. (B) CTPA showing bilateral arterial filing defects. (C) Venogram showing complete obstruction of right subclavian vein.
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Figure 1: (A) Right atrial thrombus on echocardiography. (B) CTPA showing bilateral arterial filing defects. (C) Venogram showing complete obstruction of right subclavian vein.

Mentions: A month later, her Port-A-Cath developed complete resistance on flushing during routine home intravenous antibiotics. A venogram showed a small thrombus at the tip of her Port-A-Cath, causing no obstruction. The patient was asymptomatic. An echocardiogram was performed 4 days later and revealed a very large, well-organized right atrial thrombus at the level of the tricuspid valve, attached to the Port-A-Cath tip (see Figure 1A). The right ventricle appeared normal.


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

Kandamany N, Elnazir B, Greally P - Front Pediatr (2014)

(A) Right atrial thrombus on echocardiography. (B) CTPA showing bilateral arterial filing defects. (C) Venogram showing complete obstruction of right subclavian vein.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4237125&req=5

Figure 1: (A) Right atrial thrombus on echocardiography. (B) CTPA showing bilateral arterial filing defects. (C) Venogram showing complete obstruction of right subclavian vein.
Mentions: A month later, her Port-A-Cath developed complete resistance on flushing during routine home intravenous antibiotics. A venogram showed a small thrombus at the tip of her Port-A-Cath, causing no obstruction. The patient was asymptomatic. An echocardiogram was performed 4 days later and revealed a very large, well-organized right atrial thrombus at the level of the tricuspid valve, attached to the Port-A-Cath tip (see Figure 1A). The right ventricle appeared normal.

View Article: PubMed Central - PubMed

Affiliation: Paediatric Cystic Fibrosis Department, Adelaide and Meath National Children's Hospital, Tallaght , Dublin , Ireland.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Cystic fibrosis (CT) is a genetically inherited, multisystem condition, resulting from abnormal salt and water transport across epithelial surfaces... Advances in medical treatments have resulted in vastly improved care of CF patients... The use of TIVADs in children with CF was first described in 1986... These devices require minimal care, do not limit physical activity and are relatively tamper-proof... These range from microthrombi occluding the tip of venous catheters to potentially life-threatening venous thrombosis (VTs), occasionally, resulting in pulmonary and paradoxical emboli... Deficiencies in both these proteins are possibly due to vitamin K deficiency or hepatic dysfunction... 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... While TIVADs are not the sole risk factor for VTs in CF patients, the risk of venous thromboembolism (VTE) does increase with the duration of the CVCs... Munck et al. concluded that laboratory thrombophilia screening was poorly predictive and thus not recommended routinely in patients prior to TIVAD insertion, but that a focused medical history (assessing for thrombotic risk factors) and prospective Doppler US scans might be useful in identifying asymptomatic catheter VT... Given the evidence we have reviewed, we suggest that an extended coagulation panel (incorporating at a minimum, protein C, protein S, anti-thrombin, and factor VIII and factor V Leiden levels) and Doppler ultrasound screening be conducted at regular intervals in patients with TIVADs who have had a history of a VT, as well as prior to TIVAD insertion... There has also been a move toward using peripherally inserted central catheters (PICCs) in CF patients, which are not without their own complications... In conclusion, the marked improvement in the early detection and survival of CF patients has seen a concomitant rise in challenges faced by late childhood and early adolescence in this group of patients... Presently, there is no clear consensus as to whether to routinely screen CF patients to identify those at risk for significant thrombosis prior to the development of VT... Thromboprophylaxis cannot be recommended routinely at this stage... We strongly feel this is an area that would benefit from larger and more robust studies to achieve early identification of CF patients who are at risk for serious thrombotic and thromboembolic complications arising from their indwelling central lines.

No MeSH data available.


Related in: MedlinePlus