1/111. The management of a person with haemophilia who has a fixed flexed hip and intractable pain.The clinical picture of a fixed flexed hip associated with pain in a person with haemophilia is suggestive of a haemorrhage in that area. Sonography facilitates differentiation between a haemarthrosis, intraperitoneal haemorrhage, subperiosteal bleed, a bleed into the soft tissue around the hip joint or a psoas haematoma. All these aforementioned causes may result in the same clinical presentation. Two cases are described in which coxhaemarthrosis resulted in a flexion contracture of the joint associated with severe intractable pain. Narcotic drugs failed to alleviate the severe pain. Joint aspiration produced dramatic pain relief and early joint rehabilitation.- - - - - - - - - - ranking = 1keywords = haemorrhage, haematoma (Clic here for more details about this article) |
2/111. Postoperative use of rFVIIa by continuous infusion in a haemophilic boy.Continuous infusion of coagulation factor concentrates has proved to be safe and effective. Because rFVIIa (NovoSeven is a very expensive product and very frequent doses are needed, continuous infusion is expected to be highly cost-effective. The postoperative use of continuous infusion of rFVIIa in a haemophilic boy with a high titre FVIII inhibitor is reported. He presented with a large right knee haemarthrosis and was treated with intermittent doses of rFVIIa. After a transient improvement the haemarthrosis became worse and an open evacuation of the joint had to be made under treatment with bolus injections of rFVIIa for 3 days (120 microg kg(-1) every 2 h). A previous pharmacokinetic evaluation in this patient had showed that FVIIa recovery and half-life were less than expected. Continuous infusion of rFVIIa (20 microg kg(-1) h(-1)), with added low molecular heparin to prevent local thrombophlebitis, was started on the fourth postoperative day and maintained unchanged for 7 days. Four additional single bolus injections were given for early joint mobilization. The intervals between replacements of the pump syringes were progressively increased from 6 to 12 h and then up to 24 h. FVIIa plasma levels during continuous infusion ranged between 6.3 and 10.4 IU mL(-1). Although FVIIa assays seemed to show good stability, we observed the formation of precipitates inside the syringes. The precipitates seemed to contain FVIIa. We concluded that FVIIa plasma levels of 6-10 IU mL(-1) were safe and effective to prevent postoperative haemorrhage in this patient. The addition of heparin to the rFVIIa concentrates, however, may cause precipitation and should be avoided. Individual pharmacokinetic evaluation may be useful to select the appropriate initial doses, especially in young patients.- - - - - - - - - - ranking = 0.43301791389975keywords = haemorrhage (Clic here for more details about this article) |
3/111. Spontaneous spinal subdural hematoma in a young adult with hemophilia.Spontaneous spinal subdural hemorrhage is a rare clinical problem that usually manifests with a sudden onset of pain and paralysis. This article reports on an 18-year-old male with hemophilia a and cerebral palsy, who experienced a several month history of transient back, hip, and leg pain accompanied by gait difficulties that ultimately culminated in a more striking episode of lower extremity weakness, irritability, and diffuse pain involving the neck, back, and legs. In the absence of any clinical or radiographic evidence of hemarthrosis, osteomyelitis, or intracranial hemorrhage, imaging of the spine disclosed a large, apparently multicompartmentalized intraspinal lesion, consistent with old hemorrhage. This extended from the thoracic to the sacral region, with the largest extent at the lumbosacral junction. Following correction of factor viii levels, surgical exploration was undertaken and demonstrated liquefied blood within the subdural space without violation of the underlying arachnoid. Because the chronic subdural blood flowed quite easily through the dural opening by simply angling the operating table, a limited exposure was required to achieve a substantial evacuation of the clot. This case calls attention to the often protean manifestations of this process, the potential for a chronic course to the clinical symptoms, and the possibility of achieving substantial clot evacuation and clinical recovery with a limited operative approach.- - - - - - - - - - ranking = 0.0016092581290607keywords = cerebral (Clic here for more details about this article) |
4/111. Dealing with a hemophilia-A patient undergoing cerebral aneurysm surgery.In this article anesthesiologic and hematologic aspects of a patient with Hemophilia-A, who underwent craniotomy for a right middle cerebral artery aneurysm, are discussed.- - - - - - - - - - ranking = 0.0080462906453036keywords = cerebral (Clic here for more details about this article) |
5/111. Spinal epidural haematoma in haemophilia A with inhibitors--efficacy of recombinant factor viia concentrate.We report the case of a 21-year-old man with severe haemophilia A and factor viii inhibitors who presented with an extensive spinal epidural haematoma (C2-T12), probably induced by sit-up exercises. The bleed was defined by magnetic resonance imaging of the cervical and thoracic spine and prompt treatment with recombinant factor viia concentrate led to complete resolution at 4 weeks. Neurological sequelae were averted and surgical decompression was not necessary. We discuss the difficulties in diagnosis and management of such a case.- - - - - - - - - - ranking = 0.66982086100254keywords = haematoma (Clic here for more details about this article) |
6/111. Expanding portal haematomata as a complication of knee arthroscopies in persons with haemophilia.Recurrent haemarthroses stimulate the hypertrophy of synovial tissues that if left in situ will eventually cause joint destruction. Synovectomies have been the cornerstone of joint preservation and a number of different methods exist. We report two patients who suffered complications after an arthroscopic procedure. No previous complications of this nature have been reported in the literature.- - - - - - - - - - ranking = 0.53585668880203keywords = haematoma (Clic here for more details about this article) |
7/111. Use of recombinant factor viia (NovoSeven) in a haemophilia A patient with inhibitor in kuwait.Development of inhibitors is a known complication in some haemophiliacs receiving coagulation factor replacement therapy. We report on the successful management of a young boy with haemophilia A with inhibitor using recombinant factor viia. We had failed to control bleeding in this patient following his circumcision, despite infusion with high doses of factor viii concentrate for 2 weeks. Recombinant factor viia is a useful 'factor viii bypassing agent' for the control of bleeding in patients with haemophilia A and B who develop inhibitors. We suggest that severely affected haemophiliacs should be absolved of ritual circumcision as a protective measure against what might become a life-threatening haemorrhage - especially in those with inhibitors.- - - - - - - - - - ranking = 0.43301791389975keywords = haemorrhage (Clic here for more details about this article) |
8/111. Major surgery for a gastric cancer in a haemophilic with high inhibitor titre successfully performed by the use of recombinant FVIIa.A total gastrectomy with omentectomy and resection of the distal oesophagus in a 69-year-old haemophilia A patient with high inhibitor of 128 Bethesda units is described. Surgery was successfully performed after infusion of 112 microg kg-1 bw of recombinant FVIIa. Ninety-two microg kg-1 were given thereafter at time intervals of 2 h until 12 h, then every 3 h until 24 h, and every 4 h until 48 h after surgery. Doses were gradually reduced in the following days and finally discontinued on day 28 after surgery. The complete treatment schedule required the administration of a total of 708 mg of recombinant FVIIa. Using this approach, we observed normal haemostasis, and there were no signs of excessive postoperative bleeding or wound haematoma. No clinical side-effects or evidence of systemic activation of coagulation occurred during the treatment. As judged from the clinical course of this major surgery, recombinant FVIIa appears to be highly efficacious and safe and should be used as first line treatment in high titre inhibitor patients with cross-reactivity to porcine factor viii, undergoing surgery.- - - - - - - - - - ranking = 0.13396417220051keywords = haematoma (Clic here for more details about this article) |
9/111. Thrombosis in a duplicated superficial femoral vein in a patient with haemophilia A.venous thrombosis is a very rare occurrence in patients with haemophilia A. We report the case of a haemophiliac in whom initially a calf haematoma was suspected, but neither this nor deep venous thrombosis (DVT) could be confirmed on ultrasound scanning. Subsequently, a high segment venous thrombosis was diagnosed by venography in a portion of a duplicated superficial femoral vein. Treatment with factor viii (FVIII) and low molecular weight heparin led to a successful resolution. The only other case we have been able to find in the literature occurred during FVIII replacement therapy, which was not the situation with our patient.- - - - - - - - - - ranking = 0.13396417220051keywords = haematoma (Clic here for more details about this article) |
10/111. portal vein thrombosis in a patient with severe haemophilia A and F V G1691A mutation during continuous infusion of F VIII after intramural jejunal bleeding--successful thrombolysis under heparin therapy.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.- - - - - - - - - - ranking = 0.53585668880203keywords = haematoma (Clic here for more details about this article) |
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