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ABSTRACT: Mucocutaneous bleeding is common in childhood and may be the result of primary hemostatic disorders such as vascular abnormalities,
von Willebrand disease, thrombocytopenia, and platelet dysfunction. A detailed bleeding history and physical examination are
essential to distinguish between normal and abnormal bleeding and to decide whether it is necessary to perform further laboratory
evaluation. Initial laboratory tests include complete blood count, peripheral blood smear, mean platelet volume, von Willebrand
factor (VWF) antigen assay, VWF ristocetin cofactor activity, and factor VIII activity. Once thrombocytopenia and von Willebrand
disease have been excluded, platelet function should be tested by platelet aggregation. Additional specific diagnostic tests,
such as platelet secretion tests and flow cytometry for the detection of platelet surface glycoprotein expression, are needed
to confirm the raised hypothesis.
KeywordsPrimary hemostasis–Thrombocytopenia–Von Willebrand disease–Platelet function disorders–The bleeding child–Diagnostics
European Journal of Pediatrics 04/2012; 171(1):1-10. · 1.88 Impact Factor
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ABSTRACT: Inherited thrombophilia is defined as a genetically determined tendency to develop venous thromboembolism. In children, inherited thrombophilia contributes to the development of pediatric thromboembolic disease. As a consequence, pediatric hematologists are increasingly requested to test thrombophilia in pediatric patients with thrombosis or asymptomatic children from thrombophilic families. This article reviews the benefits and limitations of testing for thrombophilic disorders, for example, factor V Leiden, prothrombin mutation, and deficiencies of antithrombin, protein C, or protein S in childhood.
Seminars in Thrombosis and Hemostasis 10/2011; 37(7):794-801. · 4.52 Impact Factor
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ABSTRACT: Bleeding complications in children may be caused by disorders of secondary hemostasis or fibrinolysis. Characteristic features in medical history and physical examination, especially of hemophilia, are palpable deep hematomas, bleeding in joints and muscles, and recurrent bleedings. A detailed medical and family history combined with a thorough physical examination is essential to distinguish abnormal from normal bleeding and to decide whether it is necessary to perform diagnostic laboratory evaluation. Initial laboratory tests include prothrombin time and activated partial thromboplastin time. Knowledge of the classical coagulation cascade with its intrinsic, extrinsic, and common pathways, is useful to identify potential defects in the coagulation in order to decide which additional coagulation tests should be performed.
European Journal of Pediatrics 09/2011; 171(2):207-14. · 1.88 Impact Factor
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ABSTRACT: Mucocutaneous bleeding is common in childhood and may be the result of primary hemostatic disorders such as vascular abnormalities, von Willebrand disease, thrombocytopenia, and platelet dysfunction. A detailed bleeding history and physical examination are essential to distinguish between normal and abnormal bleeding and to decide whether it is necessary to perform further laboratory evaluation. Initial laboratory tests include complete blood count, peripheral blood smear, mean platelet volume, von Willebrand factor (VWF) antigen assay, VWF ristocetin cofactor activity, and factor VIII activity. Once thrombocytopenia and von Willebrand disease have been excluded, platelet function should be tested by platelet aggregation. Additional specific diagnostic tests, such as platelet secretion tests and flow cytometry for the detection of platelet surface glycoprotein expression, are needed to confirm the raised hypothesis.
European Journal of Pediatrics 07/2011; 171(1):1-10. · 1.88 Impact Factor
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ABSTRACT: Survival of children with chronic intestinal failure has increased as result of administration of home parenteral nutrition. Crucial for the successful management of home parenteral nutrition is the availability of an adequate central venous access. Venous access can be fraught by episodes of catheter-infection, recurrent replacements of catheters and finally catheter-related thrombosis. Management and prevention of catheter-related thrombosis are of vital importance. For patients with compromised venous access, alternative measures are reported, most of them used as a bridge to bowel transplantation.
Thrombosis Research 12/2010; 126(6):465-70. · 2.44 Impact Factor
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ABSTRACT: Low-molecular-weight heparins are increasingly used for treatment of pediatric venous thromboembolic disease (VTE). Pediatric data about therapeutic doses of nadroparin are not available. To evaluate pharmacodynamics and safety of therapeutic doses of nadroparin, consecutive patients (age 0 to 18 y) with objectively diagnosed VTE and treated with nadroparin were included in this single center study over a 12-year period. All patients started with 85.5 IU/kg of nadroparin twice daily. The target therapeutic range (TTR) was set at 0.5 to 1.0 anti-Xa IU/mL 4 hours postdose. Safety end points were major bleeding and therapy-related death. A total of 84 patients were enrolled, of whom 8 patients did not undergo measurement of anti-Xa levels. Fifty-four (71%) of 76 patients achieved TTR. The maintenance dose (mean+/-SE) was 448+/-42 IU/kg/d in neonates (<2 mo, n=6), 253+/-22 IU/kg/d in infants (2 mo to 1 y, n=10), 214+/-8 IU/kg/d in children (2 to 11 y, n=13), and 183+/-5 IU/kg/d in adolescents (12 to 18 y, n=25). Neonates required significantly more dose adjustments and time to achieve TTR than adolescents. No major bleeding or therapy-related death occurred. In summary, an age-dependent response to nadroparin exists in pediatric patients. Nadroparin therapy seems to be safe for treatment of pediatric VTE.
Journal of Pediatric Hematology/Oncology 04/2008; 30(3):230-4. · 1.16 Impact Factor
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ABSTRACT: Pulmonary embolism is an uncommon, but potentially fatal disease in children. Most children with pulmonary embolism have underlying clinical conditions, of which the presence of a central venous catheter is the most frequent. The clinical presentation is often subtle, or masked by the underlying clinical condition. Diagnostic as well as therapeutic strategies for pulmonary embolism in children are mostly extrapolated from studies in adults. Pulmonary angiography is still the gold standard in diagnosing pulmonary embolism, but several other radiographic tests can be used to diagnose pulmonary embolism, including ventilation-perfusion lung scanning, helical computed tomography, magnetic resonance imaging and echocardiography. The choice of treatment depends on the clinical presentation of the patient. Anticoagulation is the mainstay of therapy for children with pulmonary embolism. However, thrombolytic therapy can be considered for patients with hemodynamic instability. The outcome of pediatric pulmonary embolism is uncertain and needs to be studied.
Thrombosis Research 02/2006; 118(1):13-25. · 2.44 Impact Factor
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ABSTRACT: Venous thrombosis is an uncommon disorder in childhood with an incidence of about 1 in 100000 children (ages 0 to 18 years). Multiple clinical underlying conditions as well as congenital prothrombotic disorders contribute to the development of pediatric venous thromboembolic disease. The majority of thrombi are catheter-related and therefore situated in the upper venous system. Diagnosis of thrombosis in either the upper or lower venous system is most frequently made by noninvasive radiographic techniques, especially ultrasonography. Children are treated according to recommendations based on small pediatric studies and guidelines adapted from adult patient protocols. The long-term complications, including mortality, recurrence, and the post-thrombotic syndrome, seem to be considerable. Because the incidence of pediatric venous thrombosis will probably rise due to the increased use of central venous catheters and the increased survival of children with chronic diseases associated with a prothrombotic state, prevention of this disease will become an important issue.
Seminars in Thrombosis and Hemostasis 09/2003; 29(4):391-404. · 4.52 Impact Factor
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ABSTRACT: The lower extremities of 28 unselected children with congenital heart disease were investigated and classified according to the criteria for postthrombotic syndrome five to ten years after their first cardiac catheterization. For the clinical criteria, all patients completed a questionnaire and underwent a standardized physical examination of both legs. For the pathophysiologic criteria, the presence of venous outflow obstruction and reflux was evaluated by color duplex sonography in 24 of the 28 patients. Mild postthrombotic syndrome was present in half the patients. Partial or complete occlusion of the investigated vein was found in four patients (17%). In all patients studied, the venous valves of the deep system were competent. Postthrombotic syndrome frequently occurs in children with congenital heart disease. Prospective studies seem to be justified to investigate the precise incidence and potential risk factors.
Journal of Pediatrics 11/2002; 141(4):582-6. · 4.11 Impact Factor
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ABSTRACT: In childhood, the incidence of venous thrombosis is increasing, resulting in a growing number of children with complications of this disease, such as the post-thrombotic syndrome. Diagnosis of the post-thrombotic syndrome is based on the presence of typical symptoms and signs. Using standardized clinical criteria, pediatric studies show that the frequency of the post-thrombotic syndrome after symptomatic and asymptomatic venous thrombosis is 50 to 65%. Most post-thrombotic signs and symptoms are mild, including swelling, collateral veins and pain and/or heaviness in the affected extremity. Moderate post-thrombotic syndrome occurs in 10% of the pediatric patients after symptomatic venous thrombosis and includes swelling, collateral veins, telangiectases, malleolar flare, and pigmentation of the skin and complains of heaviness or pain in the affected leg while standing or walking. Potential risk factors for development of the post-thrombotic syndrome are recurrent, ipsilateral venous thrombosis, and the extent of the initial thrombus and clot lysis. The best method to prevent the syndrome is to prevent the initial thrombosis. The efficacy of compression stockings after venous thrombosis in preventing the post-thrombotic syndrome is unknown and needs to be evaluated in children.
Progress in Pediatric Cardiology.
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ABSTRACT: Postvaricella purpura fulminans is a rare disease in children that is probably caused by an acquired protein S deficiency resulting from antiprotein S antibodies. The epitope of these antibodies is unknown. A 5-year-old girl is described with postvaricella purpura fulminans and an acquired protein S deficiency. In this patient and in her 3-year-old sister with uncomplicated varicella, the concentrations of antiprotein S antibodies were measured and followed with enzyme-linked immunosorbent assay techniques. The epitope of the antiprotein S antibodies was studied using miniprotein S, a recombinant variant of protein S that consists of the first 242 amino acids of protein S, lacking the sex hormone binding globulin-like domain. In the patient's plasma, concentrations of free protein S antigen and total protein S antigen reached normal levels in 4 months and 5 weeks, respectively. The concentrations of the antiprotein S antibodies decreased to 25% of the initial level in the course of 5 months. In the sister, antiprotein S antibodies were present as well, but the concentrations were lower than those in the patient. Most of the antiprotein S antibodies were directed against the first 242 amino acids of protein S. After varicella, a heterozygous autoantibody response may develop that may result in severe acquired protein S deficiency leading to purpura fulminans. Epitopes of these antiprotein S antibodies are situated on both the first 242 amino acids of protein S and the sex hormone binding globulin-like domain.
Journal of Pediatric Hematology/Oncology 24(5):413-6. · 1.16 Impact Factor
