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Eur J Pediatr ; 158 Suppl 3: S180-2, 1999 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-10650865

RESUMEN

UNLABELLED: We report on a 14-year-old boy with severe haemophilia A who developed a portal vein thrombosis during continuous infusion of F VIII. For treatment of a posttraumatic intramural jejunal haematoma with extension into the mesenterium the patient received continuous infusion (CI) of a high purity F VIII concentrate, starting with an initial bolus injection of 100 IU F VIII/kg bw and followed by 4-5 IU F VIII/kg bw/h i.v. F VIII plasma activity ranged between 47 and 88%. Resorption of the haematoma was proven by abdominal ultrasonic follow-ups. After 3 weeks of CI a thrombus formation in the portal vein was detected by ultrasound and confirmed by duplex ultrasound. Subsequent to diagnosis the patient was heparinised with unfractionated heparin (UFH 300-450 IU/kg/d i.v.). In order to induce further resorption of the haematoma. F VIII concentrate was given concomitantly (50 IU/kg bw twice daily) during the initial phase of treatment. After 14 days of anticoagulant therapy with UFH, the regimen was changed to low molecular weight heparin (LMWH; Fraxiparin 0.3; 2850 IU anti-X activity/d s.c.; bw 60 kg). F VIII dosage was gradually reduced with advanced resorption of the haematoma and thereafter switched to prophylaxis (40 IU/kg bw 3 times weekly). Complete lysis of the thrombus was observed after 6 months of treatment with UFH and LMWH respectively without any further complications. Thereafter LMWH was discontinued. Thrombophilic screening revealed no abnormalities except heterozygous F V G1691A. CONCLUSION: The coexistence of a common prothrombotic risk factor and haemophilia may cause severe complications, in particular if the bleeding disorder has to be corrected temporarily by administration of the concerning deficient agent.


Asunto(s)
Factor V/genética , Hemofilia A/complicaciones , Vena Porta , Trombofilia/complicaciones , Trombosis de la Vena/complicaciones , Adolescente , Anticoagulantes/uso terapéutico , Coagulantes/efectos adversos , Factor VIII/efectos adversos , Hemorragia Gastrointestinal/tratamiento farmacológico , Heparina/uso terapéutico , Humanos , Infusiones Intravenosas/efectos adversos , Enfermedades del Yeyuno/tratamiento farmacológico , Masculino , Trombosis de la Vena/etiología
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