Manifestations and clinical impact of pediatric inherited thrombophilia

Klaassen, Irene L. M.; Ommen, C. Heleen van

doi:10.1182/blood-2014-05-536060

Cited by 36 publications

(31 citation statements)

References 38 publications

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“…At least one risk factor is identified in the majority of patients (19–21). This stands in contrast to adults where 30–50% of VTE events are idiopathic or spontaneous.…”

Section: Incidencementioning

confidence: 99%

Epidemiology and Risk Assessment of Pediatric Venous Thromboembolism

Mahajerin

Croteau

2017

Front. Pediatr.

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The incidence of diagnosed venous thromboembolism (VTE) has been increasing concurrent with advances in technology and medical care that enhance our ability to treat pediatric patients with critical illness or complex multiorgan system dysfunction. Although the overall incidence of VTE is estimated at 0.07–0.49 per 10,000 children, higher rates are observed in specific populations including hospitalized children, those with central venous catheters (CVCs) or patients convalescing from a major surgery. While the absolute number of pediatric VTE events may seem trivial compared to adults, the increasing incidence, associated with increased mortality and morbidity, the availability of novel therapies, and the impact on the cost of care have made investigation of VTE risk factors and prevention strategies a high priority. Many putative risk factors for pediatric VTE have been reported, primarily from single-institution, retrospective studies which lack appropriate methods for verifying independent risk factors. In addition, some risk factors have inconsistent definitions, which vex meta-analyses. CVCs are the most prevalent risk factors but have not consistently been assigned the highest level of risk as defined by odds ratios from retrospective, case–control studies. Few risk-assessment models for hospital-acquired pediatric VTE have been published. Some models focus exclusively on hospitalized pediatric patients, while others target specific populations such as patients with cancer or severe trauma. Multicenter, prospective studies are needed to identify and confirm risk factors in order to create a pediatric risk-assessment tool and optimize preventive measures and reduce unintended harm.

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“…At least one risk factor is identified in the majority of patients (19–21). This stands in contrast to adults where 30–50% of VTE events are idiopathic or spontaneous.…”

Section: Incidencementioning

confidence: 99%

Epidemiology and Risk Assessment of Pediatric Venous Thromboembolism

Mahajerin

Croteau

2017

Front. Pediatr.

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“…The neonatal hemostatic system can be tipped toward thrombosis by a variety of acquired risk factors, and greater than 95% of neonatal VTE is associated with at least one clinical risk factor (5, 9) (Table 1). The most common clinical risk factor is the presence of a CVC (5, 9–13).…”

Section: Risk Factorsmentioning

confidence: 99%

“…The most common clinical risk factor is the presence of a CVC (5, 9–13). A prospective study of neonates who underwent CVC placement indicated a 13% incidence of CVC-associated VTE (14), while another evaluating only umbilical catheter revealed a higher incidence of 22% (15).…”

Section: Risk Factorsmentioning

confidence: 99%

“…A recent systematic review analyzed 13 publications from 2008 to 2014 evaluating the role of inherited thrombophilia in neonatal VTE development and treatment. The authors concluded that neonatal VTE is multifactorial and clinical risk factors weigh more heavily on the prothrombotic scale than inherited thrombophilia, particularly in CVC-associated VTE (9). In an earlier study, the overall prevalence of inherited thrombophilia in neonates with VTE was not different than that of the healthy population, concluding that screening neonates with VTE for inherited thrombophilia was not necessary (12).…”

Section: Risk Factorsmentioning

confidence: 99%

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Neonatal Venous Thromboembolism

Haley

2017

Front. Pediatr.

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Neonates are the pediatric population at highest risk for development of venous thromboembolism (VTE), and the incidence of VTE in the neonatal population is increasing. This is especially true in the critically ill population. Several large studies indicate that the incidence of neonatal VTE is up almost threefold in the last two decades. Central lines, fluid fluctuations, sepsis, liver dysfunction, and inflammation contribute to the risk profile for VTE development in ill neonates. In addition, the neonatal hemostatic system is different from that of older children and adults. Platelet function, pro- and anticoagulant proteins concentrations, and fibrinolytic pathway protein concentrations are developmentally regulated and generate a hemostatic homeostasis that is unique to the neonatal time period. The clinical picture of a critically ill neonate combined with the physiologically distinct neonatal hemostatic system easily fulfills the criteria for Virchow’s triad with venous stasis, hypercoagulability, and endothelial injury and puts the neonatal patient at risk for VTE development. The presentation of a VTE in a neonate is similar to that of older children or adults and is dependent upon location of the VTE. Ultrasound is the most common diagnostic tool employed in identifying neonatal VTE, but relatively small vessels of the neonate as well as frequent low pulse pressure can make ultrasound less reliable. The diagnosis of a thrombophilic disorder in the neonatal population is unlikely to change management or outcome, and the role of thrombophilia testing in this population requires further study. Treatment of neonatal VTE is aimed at reducing VTE-associated morbidity and mortality. Recommendations for treating, though, cannot be extrapolated from guidelines for older children or adults. Neonates are at risk for bleeding complications, particularly younger neonates with more fragile intracranial vessels. Developmental alterations in the coagulation proteins as well as unique pharmacokinetics must also be taken into consideration when recommending VTE treatment. In this review, epidemiology of neonatal VTE, pathophysiology of neonatal VTE with particular attention to the developmental hemostatic system, diagnostic evaluations of neonatal VTE, and treatment guidelines for neonatal VTE will be reviewed.

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“…oral contraceptives, asparaginase). [5][6][7][8][9] In addition, several hereditary risk factors for thromboembolism have been discussed. 7 May-Thurner syndrome (MTS), a compression of the common iliac vein by the crossing common iliac artery, is observed in up to 50% of adult patients with descending iliofemoral DVT and is associated with a recurrence rate of up to 73% for thrombosis.…”

mentioning

confidence: 99%

Ultrasound Assisted Endovascular Thrombolysis in Adolescents: 2 Case Reports

Olivieri¹,

Kurnik²,

Hoffmann

et al. 2016

Pediatrics

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Descending iliofemoral thrombosis in children is a rare event. Anticoagulation therapy with low-molecular-weight-heparin is standard of care. However, patency cannot be achieved in all cases, increasing the risk for rethrombosis and postthrombotic syndrome. To reduce the risk of venous valve failure in adults, local catheter-directed thrombolysis is used to reopen vessels. Two adolescent girls (17 and 15 years old) presented with acute descending iliofemoral thrombosis of the left common iliac, external, and common femoral veins. Anticoagulation with enoxaparin was started until insertion of an EkoSonic Mach 4e catheter for ultrasound-assisted local thrombolysis with recombinant tissue plasminogen activator and administration of unfractionated heparin. Success was monitored by increases in D-dimer levels and ultrasound findings. After 24 hours respectively 48 hours, complete recanalization was obtained. No complication occurred except minimal local bleeding. Screening for hereditary thrombophilia revealed a heterozygous antithrombin mutation in 1 girl (ie, the 15-year-old). May-Thurner syndrome was identified in both girls, necessitating stenting of the left common iliac veins and continuation of anticoagulation therapy with enoxaparin and acetylsalicylic acid. No rethrombosis or complications occurred during the follow-up period. Ultrasound-assisted catheter-directed local thrombolysis with the EkoSonic Mach 4e system was effective in achieving immediate recanalization of the occluded veins and should be considered in children experiencing descending iliofemoral thrombosis. The fast recanalization might reduce the incidence of postthrombotic syndrome. May-Thurner syndrome is regularly found in these patients, and if present, requires stenting of the common iliac vein to avoid early reocclusion. However, long-term patency of iliac vein stenting in children remains to be examined.

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Manifestations and clinical impact of pediatric inherited thrombophilia

Cited by 36 publications

References 38 publications

Epidemiology and Risk Assessment of Pediatric Venous Thromboembolism

Epidemiology and Risk Assessment of Pediatric Venous Thromboembolism

Neonatal Venous Thromboembolism

Ultrasound Assisted Endovascular Thrombolysis in Adolescents: 2 Case Reports

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