1/14. Prophylactic treatment of severe factor x deficiency with prothrombin complex concentrate.Factor X (FX) deficiency is an autosomal recessive trait that occurs in fewer than 1 in 500 000 people. Not surprisingly, reports of prophylactic treatment for FX deficiency are exceedingly rare. We now report our experience of the use of prophylactic therapy in a FX-deficient patient. This 18-year-old African-American male presented at the age of 4(1/2) years with an FX level < 1%. Treatment was on demand with prothrombin complex concentrates (PCCs) given at two times the dose per kilogram of body weight for factor ix. He experienced frequent epistaxis, soft tissue bleeding and joint bleeding. The development of a target joint (right ankle) prompted the initiation of prophylactic treatment in the beginning of 1998 to the present with 30 units kg(-1) Profilnine twice per week via a home infusion programme. If breakthrough bleeding occurred, he was instructed to infuse another dose. He was instructed that Profilnine should not be infused in more than two doses in 24 h or on more than three consecutive days. A trough level drawn 48 h post-infusion showed an FX level of 30%. In the initial 12 months with prophylactic treatment, there was no breakthrough bleeding. Subsequently, with an additional 11 months of follow-up, he has reported one bleed. He rates his quality of life improved since starting prophylactic treatment. There have been no thrombotic events. Prophylaxis with PCC for FX deficiency with adequate education and follow-up can be performed capably in the home setting with a resultant decrease in the frequency of bleeding and attendant complications.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/14. Recombinant human factor viia in the management of amyloid-associated factor x deficiency.factor x deficiency is an important complication of amyloidosis. It is associated with severe bleeding that is difficult to control with plasma or prothrombin complex concentrates. splenectomy ameliorates the factor x deficiency, but achieving satisfactory haemostasis for this operation is problematic. We report that a new clotting concentrate, recombinant factor viia, readily controls bleeding and makes splenectomy feasible.- - - - - - - - - - ranking = 0.2keywords = complex (Clic here for more details about this article) |
3/14. danazol therapy in factor x deficiency.Factor X (FX) deficiency is a rare coagulation disorder that usually presents with bleeding manifestations and is treated with fresh frozen plasma or prothrombin complex concentrates. We report a case of FX deficiency in which the patient presented with bleeding as well as thrombosis. The patient responded to danazol and relapsed when the drug was stopped. The occurrence of thrombosis in FX deficiency and the role of danazol in coagulopathies are reviewed.- - - - - - - - - - ranking = 0.2keywords = complex (Clic here for more details about this article) |
4/14. A novel type I factor X variant (factor X Cys350Phe) due to loss of a disulfide bond in the catalytic domain.We report a novel mutation within the coagulation factor X (FX) that we have designated FX Padua 4. The phenotype and genotype of the proband and family members were studied. The proband was a child affected by a complex neurological syndrome who, after birth, experienced severe bleeding. The proband showed a laboratory pattern characterized by a severe reduction of FX activity and FX antigen, suggesting a true deficiency. Molecular analysis disclosed a new FX mutation localized in the catalytic domain responsible for a Cys350Phe substitution. The proband was homozygous for this mutation. The proband's mother and father showed a heterozygous pattern and had approximately one-half the normal FX activity and FX antigen. Residual purified FX Cys350Phe had an identical behavior to normal FX as showed by sodium dodecyl sulfate-polyacrylamide gel electrophoresis. Molecular modeling confirms that the mutation leads to the disruption of a disulfide bridge in the catalytic region of FX. Comparison with other topologically equivalent mutations in other vitamin k-dependent proteins suggests that this disruption could adversely affect protein folding/stability, accounting for the cross-reactive material negative phenotype.- - - - - - - - - - ranking = 0.2keywords = complex (Clic here for more details about this article) |
5/14. Successful perioperative management of factor x deficiency associated with primary amyloidosis.Acquired bleeding abnormalities are common in patients with primary amyloid light-chain amyloidosis. factor x deficiency is the most common coagulopathy associated with life-threatening hemorrhagic complications when surgery is indicated. Fresh-frozen plasma (FFP) or prothrombin complex concentrates (PCCs) are the most frequently used blood products in this disease; however, FFP is often ineffective in controlling bleeding and PCCs have a significant risk of thrombosis when used intraoperatively. This report describes a patient with primary amyloidosis and factor x deficiency who underwent hemicolectomy with preoperative and intraoperative administration of recombinant human factor viia and postoperative administration of Bebulin (a PCC that contains the highest concentration of factor X). The management was successful with no signs of bleeding postoperatively. To our knowledge, few reports of successful perioperative management of factor x deficiency have been published to date. This is the first case report using recombinant human factor viia and Bebulin in the perioperative management of factor x deficiency associated with primary amyloidosis. Recombinant human factor viia and Bebulin may allow for successful perioperative management of bleeding disorders in patients with primary amyloidosis.- - - - - - - - - - ranking = 0.2keywords = complex (Clic here for more details about this article) |
6/14. Prevention of recurrent intracranial hemorrhage in a factor X-deficient infant.We describe a case of a child with severe factor x deficiency who had three episodes of intracranial hemorrhage during the first 6 months of life. Since that time he has been successfully managed with prophylactic therapy using prothrombin complex infusions via an indwelling central venous line. This prophylactic therapy may prevent the poor outcome usually described in these patients.- - - - - - - - - - ranking = 0.2keywords = complex (Clic here for more details about this article) |
7/14. Neonatal congenital factor x deficiency.Four neonates with congenital factor x deficiency presented soon after birth with bleeding episodes. Two of the newborns had intracranial hemorrhages; one of them also had antenatal ventricular dilatation and postnatal hydrocephalus and died of massive intracerebral hemorrhage at four months. One patient was lost for follow up. The two surviving infants were followed up for four years and two years respectively, while on replacement therapy with three injections of 40 units/kg prothrombin complex a month. In spite of markedly elevated prothrombin time and partial thromboplastin time, these two infants remain free of major bleeding manifestations except for troublesome petechiae and ecchymoses. A schedule for substitution therapy with Factor X is proposed for infants and children to prevent bleeding in severe factor x deficiency.- - - - - - - - - - ranking = 0.2keywords = complex (Clic here for more details about this article) |
8/14. A case of acquired factor x deficiency with in vivo and in vitro evidence of inhibitor activity directed against factor X.A 67-year-old woman had symptoms of an upper respiratory tract infection for which she received a five-day course of erythromycin. epistaxis and gross hematuria subsequently developed, and the patient was found to have a selective factor x deficiency. She received supportive therapy and prothrombin complex concentrates (Factors II, VII, IX, and X), with subsequent resolution of her transient factor x deficiency. Her hospital course, however, was complicated by the development of multiple cerebral infarctions. This is the tenth reported case of transient factor x deficiency not associated with amyloidosis. In seven of the previous cases, as in this patient, the deficiency was associated with a preceding upper respiratory infection. This is the only case, however, with evidence of inhibitory activity in the plasma that was directed toward Factor X.- - - - - - - - - - ranking = 0.2keywords = complex (Clic here for more details about this article) |
9/14. Transient acquired factor x deficiency: report of the use of activated clotting concentrate to control a life-threatening hemorrhage.Acquired deficiency of factor X is an uncommon occurrence. It has usually developed in association with amyloidosis [8] and, in that setting, it has been irreversible. Transient deficiency appears to be associated with an acute respiratory infection in the majority of cases. Bleeding in these patients can be life-threatening and has been difficult to control. Konyne produces a brief correction of the prothrombin time and an elevation in the factor X level, but has not been effective in stopping bleeding. We report the first successful correction of prothrombin time and clinical resolution of bleeding attending the use of Autoplex T. If bleeding persists after appropriate specific factor replacement and the clinical condition warrants, the use of Autoplex T (activated prothrombin complex) deserves prompt consideration.- - - - - - - - - - ranking = 0.2keywords = complex (Clic here for more details about this article) |
10/14. Acquired, transient factor X (Stuart factor) deficiency in patient with mycoplasma pneumonial infection.A case of severe haemorrhagic diathesis due to acquired deficiency of factor X (both immunologically and in procoagulant activity) is presented. The clinical and serological features of this case indicated mycoplasma pneumonial infection. Factor X in the peripheral blood did not appear to be influenced by administration of vitamin k, prothrombin-complex concentrate, fresh plasma or fresh whole blood. Circulating inhibitors of blood coagulation were absent and systemic amyloidosis could not be demonstrated. After 20 d, factor X spontaneously returned to normal. In view of the absence of other known causes of factor x deficiency, a possible relationship with mycoplasma pneumonial infection is suggested.- - - - - - - - - - ranking = 0.2keywords = complex (Clic here for more details about this article) |
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