Cases reported "Hemophilia A"

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1/336. 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.
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2/336. Management of a premature infant with moderate haemophilia A using recombinant factor viii.

    The birth of a very premature infant with haemophilia A is a rare event. In this case report the problems posed in the management of a child with a factor viii level of 0.03 IU mL-1 born at 28 weeks of gestation and weighing 1590 g are considered. The value of recombinant factor viii, the pharmacokinetics of factor VIII in this situation and the importance of close cooperation between paediatricians and haematologists are discussed.
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3/336. Central venous catheter-associated thrombosis in severe haemophilia.

    Significant subclavian vein thromboses associated with indwelling fully implanted (port-a-cath) devices are described in two boys with severe haemophilia A and factor viii inhibitors. Investigations were prompted by prominent chest wall veins in one case, whereas the thrombosis was a chance finding in the other case during investigation of mechanical dislocation of the catheter tubing. Extensive collateral venous circulations were demonstrated by venography in both instances indicating that the thrombus had been present for some time. Possible contributing factors to the thromboses included desensitization therapy (both patients), high-dose FEIBA (in one patient) and use of lower doses of heparin for line flush than that recommended by some authors. Neither patient had a familial or non-familial predisposition to thrombosis.
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keywords = haemophilia
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4/336. Antifactor VIII antibody inhibiting allogeneic but not autologous factor viii in patients with mild hemophilia a.

    Two unrelated patients with the same Arg2150His mutation in the factor viii (FVIII) C1 domain, a residual FVIII activity of 0.09 IU/mL, and inhibitor titres of 300 and 6 Bethesda Units, respectively, were studied. Further analysis of patient LE, with the highest inhibitor titer, showed that (1) plasma or polyclonal IgG antibodies prepared from LE plasma inhibited the activity of allogeneic (wild-type) but not of self FVIII; (2) the presence of von Willebrand factor (vWF) increased by over 10-fold the inhibitory activity on wild-type FVIII; (3) the kinetics of FVIII inhibition followed a type II pattern, but in contrast to previously described type II inhibitors, LE IgG was potentiated by the presence of vWF instead of being in competition with it; (4) polyclonal LE IgG recognized the FVIII light chain in enzyme-linked immunosorbent assay and the recombinant A3-C1 domains in an immunoprecipitation assay, indicating that at least part of LE antibodies reacted with the FVIII domain encompassing the mutation site; and (5) LE IgG inhibited FVIII activity by decreasing the rate of FVIIIa release from vWF, but LE IgG recognized an epitope distinct from ESH8, a murine monoclonal antibody exhibiting the same property. We conclude that the present inhibitors are unique in that they clearly distinguish wild-type from self, mutated FVIII. The inhibition of wild-type FVIII by LE antibody is enhanced by vWF and is associated with an antibody-dependent reduced rate of FVIIIa release from vWF.
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5/336. The locked patella. An unusual complication of haemophilia.

    Mechanical derangements of the knee are an uncommon complication of chronic haemophiliac arthropathy. Two patients with locking of the patella were treated by manipulation. The mechanism of the injury was forced flexion of the knee joint beyond the limit of its restricted range. The injury is a serious one and may take six months to recover.
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6/336. CD4 depletion in hiv-infected haemophilia patients is associated with rapid clearance of immune complex-coated CD4 lymphocytes.

    The predominant immunological finding in hiv haemophilia patients is a decrease of CD4 lymphocytes during progression of the disease. Depletion of CD4 lymphocytes is paralleled by an increase in the proportion of immune complex-coated CD4 cells. We examined the hypothesis that the formation of immune complexes on CD4 lymphocytes is followed by rapid clearance of immune complex-coated CD4 lymphocytes from the circulation. In this study, the relationship of relative to absolute numbers of immune complex-loaded CD4 blood lymphocytes and their association with viral load were studied. Two measurements of relative and absolute numbers of gp120-, IgG- and/or IgM-loaded CD4 lymphocytes were analysed in hiv and hiv- haemophilia patients, with a median interval of approx. 3 years. Immune complexes on CD4 lymphocytes were determined using double-fluorescence flow cytometry and whole blood samples. viral load was assessed using NASBA and Nuclisens kits. Whereas the proportion of immune complex-coated CD4 lymphocytes increased with progression of the disease, absolute numbers of immune complex-coated CD4 lymphocytes in the blood were consistently low. Relative increases of immune complex-coated CD4 blood lymphocytes were significantly associated with decreases of absolute numbers of circulating CD4 lymphocytes. The gp120 load on CD4 blood lymphocytes increased in parallel with the viral load in the blood. These results indicate that immune complex-coated CD4 lymphocytes are rapidly cleared from the circulation, suggesting that CD4 reactive autoantibodies and immune complexes are relevant factors in the pathogenesis of AIDS. Relative increases of immune complex-positive cells seem to be a consequence of both an increasing retroviral activity as well as a stronger loading with immune complexes of the reduced number of CD4 cells remaining during the process of CD4 depletion. The two mechanisms seem to enhance each other and contribute to the progressive CD4 decrease during the course of the disease.
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ranking = 1.2
keywords = haemophilia
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7/336. Joint replacement for a spontaneously ankylosed hip in a haemophilic patient.

    A cemented Charnley total hip prosthesis was implanted in a 48-year-old man with mild haemophilia (factor viii 4 IU dL-1) in his right spontaneously ankylosed hip. At the time of surgery he was anti-HCV positive, anti-hiv negative, and no circulating inhibitors were encountered. The indication for surgery was long-lasting intractable low back and ipsilateral knee pain. At 4-month follow-up, relief of pain was achieved as well as correction of limb-length discrepancy, with a good result according to the Mayo Clinic hip score. Doses of 50 IU kg-1 body weight of recombinant factor viii (Recombinate; Baxter, Glendale, california, USA) was used during the 2 weeks of admittance to the hospital. The dosage was adjusted according to the recoveries of factor viii, with an overall factor consumption of 68 000 IU. As far as we know this is the first case reported in the literature of a person with haemophilia in whom a spontaneous hip ankylosis has been satisfactorily converted in a total hip arthroplasty with a short-term follow-up. However, a much longer follow-up is still needed to ascertain the efficacy of this surgical procedure in haemophilia.
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keywords = haemophilia
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8/336. Acute renal failure complicating high-dose intravenous immunoglobulin therapy for acquired haemophilia.

    Acquired haemophilia is a rare disorder requiring therapy to control bleeding and to suppress the inhibitory antibody. High-dose intravenous immunoglobulin is commonly used as part of immunosuppressive regimens for this condition. We describe the case of an elderly patient who developed acute oliguric renal failure as a result of intravenous immunoglobulin therapy. All patients receiving such treatment should have renal function carefully monitored both during and after the infusion.
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keywords = haemophilia
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9/336. Does avascular necrosis cause collapse of the dome of the talus in severe haemophilia?

    Arthropathy of the ankle joint is commonly observed in people with severe haemophilia. A review of the radiological appearance of ankle arthropathy provides evidence that the changes in the talar bone of the ankle joint are probably due to avascular necrosis. This may arise due to impairment to the arterial supply, as it enters the talar neck, secondary to the increased pressure at the time of haemarthroses.
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keywords = haemophilia
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10/336. Characteristics of septic arthritis in human immunodeficiency virus-infected haemophiliacs versus other risk groups.

    The cases are presented of four haemophiliacs infected with human immunodeficiency virus (hiv) and with septic arthritis among the 340 patients followed at our centre. The data of these cases and 39 additional hiv-infected haemophiliacs with septic arthritis, identified in a literature search, are reviewed. The spectrum of bacterial pathogens is limited and somewhat different from that in other risk groups. The localization is exclusively to joints affected by haemophilic arthropathy. The laboratory picture is characterized by the absence of peripheral leucocytosis, varying CD4-helper cell counts, a high erythrocyte sedimentation rate and fever. The clinical picture mimics that of haemarthrosis, often causing a delay in diagnosis. Treatment with systemic antibiotics is often sufficient, obviating the need for arthrotomy and open drainage. prognosis related to the joint function is relatively good, but poor when related to the medium- to long-term survival of the patient.
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ranking = 1.2
keywords = haemophilia
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