1/94. 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 = 1keywords = life (Clic here for more details about this article) |
2/94. 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 = 1keywords = life (Clic here for more details about this article) |
3/94. Orthotopic liver transplantation in a patient with severe haemophilia A and with advanced liver cirrhosis.A patient with severe haemophilia A underwent orthotopic liver transplantation because of changes correlated to end-stage liver cirrhosis due to hepatitis b, C and D infection. Replacement therapy was carried out for 4 days and the clinical course was uneventful. At the time of reporting the patient has a normal working life. FVIII plasma concentration is normal. The indirect hyperbilirubinaemia may be related to the Gilbert's anomaly of the donor.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
4/94. prostatectomy in hemophilia.The advent of factor viii concentrates has made it possible for true hemophiliac patients to survive to an age when prostatic obstruction is common. Formerly, operative procedures in these patients were limited to life-threatening situations but now prostatic operations should not be feared as long as adequate serum levels of factor viii can be achieved and maintained throughout the wound healing period. Allergic reactions, hepatitis and cost are significant factors to consider when the concentrates are administered but hemophiliac patients are able to undergo treatment without a significantly increased risk to their lives.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
5/94. Long-lasting remission and successful treatment of acquired factor viii inhibitors using cyclophosphamide in a patient with systemic lupus erythematosus.Acquired deficiency of clotting factor viii (FVIII) is a rare bleeding diathesis seldom encountered in systemic lupus erythematosus (SLE). Reduction of FVIII activity by autoantibodies can cause potentially life-threatening situations. Herein, an SLE patient with a positive lupus anticoagulant (LAC) test who abruptly developed metrorrhagia 4 yr after diagnosis is reported. Coagulation tests revealed FVIII activity reduced to 3% and a prolonged aPTT. FVIII inhibitor(s) were found to be as high as 3.0 Bethesda Units. plasmapheresis, immunoglobulins, prednisolone and FVIII plasma concentrates induced the cessation of metrorrhagia, but the clotting tests were barely improved. One month later, extensive ecchymosis appeared and worsened, despite re-administration of the previous therapy. pulse cyclophosphamide followed by oral administration was then started with normalization of coagulation parameters and long-lasting disease remission.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
6/94. gas gangrene in a patient with severe haemophilia A.The haemophilia patient tends to live a more protected life than his normal counterpart, this is particularly so in underdeveloped and developing countries where due to poor health infrastructure, financial constraints and nonavailability of factor concentrates, patients quickly learn that they need to live a protected life. Under such circumstances, gas gangrene seems to be a very unusual infection for this group of patients. We report here a 25-year-old male with severe haemophilia who developed gas gangrene due to inadequate medical management following a road traffic accident. Subsequently, his affected limb was salvaged by conservative therapy. A literature search failed to reveal any reports of similar patients in the English literature.- - - - - - - - - - ranking = 2keywords = life (Clic here for more details about this article) |
7/94. Acquired hemophilia masked by warfarin therapy.People without hemophilia but with autoantibodies specifically directed against the procoagulant activity of factor viii are known to have acquired hemophilia. The bleeding diathesis in these patients is often severe and life-threatening. The definite laboratory diagnosis of this disorder includes demonstration of low factor viii levels in plasma with a high titer of factor viii inhibitors, but the initial suspicion for its presence should rise in view of a prolonged partial thromboblastin time (PTT) and a normal prothrombin time associated with an acquired bleeding disorder. Oral anticoagulant treatment is known to prolong PTT as well, and the merger of these 2 situations may cause delayed diagnosis of acquired hemophilia with devastating consequences. We describe here the first reported case of acquired hemophilia diagnosed in a patient treated with warfarin. In such patients prolonged PTT may be ascribed to warfarin therapy rather than to acquired hemophilia, thus causing a dangerous delay in diagnosis.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
8/94. Bell's palsy during interferon therapy for chronic hepatitis c infection in patients with haemorrhagic disorders.Two adult patients with life-long severe haemorrhagic disorders commenced on interferon-alpha2b therapy for chronic hepatitis c infection. Both developed Bell's palsy several weeks after commencing therapy, They were started on steroids and, in addition, the first patient discontinued interferon-alpha2b therapy while the second patient elected to continue with therapy. In both cases facial paralysis improved over the ensuing weeks. Bell's palsy is often idiopathic but has been reported. in association with herpesviruses. It is not a recognised complication of chronic hepatitis b or C infection, or interferon-alpha2b therapy. However, the interferons are associated with numerous adverse reactions including various neuropsychiatric manifestations and neurological syndromes. There are several reports of nerve palsies, including optic tract neuropathy, occurring during interferon therapy, and immune-based mechanisms are thought to play a role in the aetiopathogenesis. No reports of Bell's palsy in association with interferon therapy were identified in our literature search, although one possible case has been reported to the Committee of safety in medicine. Although Bell's palsy in our patients may have occurred by chance, a neuropathic effect of interferon-alpha2b on the facial nerve cannot be excluded and we urge physicians using interferons to be aware of this potential side-effect.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
9/94. Acquired haemophilia complicating the remission of a patient with high grade non-Hodgkin's lymphoma treated by fludarabine.We report the development of a high titre antibody to factor viii in a patient with previous high grade B cell non-Hodgkin's lymphoma treated with fludarabine. Unlike previous reports of factor viii inhibitors and lymphoproliferative disease this patient's lymphoma was in remission. We speculate that the occurrence of the inhibitor is another manifestation of the increasingly recognized autoimmune side-effects of fludarabine.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
10/94. Progressive infantile neurodegeneration caused by 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency: a novel inborn error of branched-chain fatty acid and isoleucine metabolism.We report a novel inborn error of metabolism identified in a child with an unusual neurodegenerative disease. The male patient was born at term and recovered well from a postnatal episode of metabolic decompensation and lactic acidosis. Psychomotor development in the first year of life was only moderately delayed. After 14 mo of age, there was progressive loss of mental and motor skills; at 2 years of age, he was severely retarded with marked restlessness, choreoathetoid movements, absence of directed hand movements, marked hypotonia and little reaction to external stimuli. Notable laboratory findings included marked elevations of urinary 2-methyl-3-hydroxybutyrate and tiglylglycine without elevation of 2-methylacetoacetate, mild elevations of lactate in CSF and blood, and a slightly abnormal acylcarnitine profile. These abnormalities became more apparent after isoleucine challenge. Enzyme studies showed absent activity of 2-methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) in the mitochondrial oxidation of 2-methyl branched-chain fatty acids and isoleucine. Under dietary isoleucine restriction, neurologic symptoms stabilized over the next 7 months.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
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