1/7. Successful use of recombinant factor viia in a patient with inhibitor secondary to severe factor xi deficiency.Factor XI (FXI) inhibitors are a rare complication of inherited FXI deficiency. We report the successful use of recombinant factor viia (FVIIa) in a patient with a high-responding inhibitor undergoing cataract extraction. At the time of surgery there were limited available data on the optimal management of patients with FXI deficiency. A 62-year-old Ashkenazi Jewish woman had a lifelong history of excessive bleeding secondary to severe FXI deficiency (2 U dL-1), and received FXI concentrate (FXI:C) when she underwent a colposuspension procedure. She was subsequently diagnosed with a FXI inhibitor of 16 Bethesda units (BU) when she developed a poor response to FXI:C at the time of total hip replacement. Two months later she was admitted for cataract extraction. The FXI level was < 1 U dL-1 with an inhibitor titre of 48 BU. She received 90 microg kg-1 of FVIIa immediately preoperatively followed by continuous infusion at a rate of 20 microg kg-1 h-1 for 24 h. The cataract extraction was successful and there was no excess bleeding during surgery or in the postoperative period. mutation analysis of the FXI gene showed that the patient was homozygous for the type II genotype [exon 5, Glu117-->Ter]. The reason for the low prevalence of inhibitor formation in patients with FXI deficiency is unclear but may reflect a number of factors including reporting bias, the rarity of absent circulating FXI:C activity, and the infrequent use of FXI replacement therapy.- - - - - - - - - - ranking = 1keywords = extraction (Clic here for more details about this article) |
2/7. Factor XI: a review of its biochemistry and deficiency.The biochemistry and physiology of Factor XI have been reviewed. The clinical history of our 25 patients was reviewed and compared with others' experiences. factor xi deficiency remains enigmatic in that there is no correlation between Factor XI levels and clinical manifestations. Approximately 40 to 50% of all persons lacking Factor XI are of Ashkenazi Jewish extraction, and the remainder are represented by nearly all populations. persons may be found to lack Factor XI because of evaluation for hemorrhage, evaluation of a prolonged PTT, or through family or other genetic studies. hemorrhage is not as severe as in patients with hemophilia a or B. Data are presented suggesting that hemorrhage may, in part, be associated with aspirin use.- - - - - - - - - - ranking = 0.33333333333333keywords = extraction (Clic here for more details about this article) |
3/7. factor xi deficiency disclosed following haemorrhage related to a dental extraction. Brief review and case report.factor xi deficiency is a relatively common hereditary coagulation disorder manifested generally as diffuse oozing from a surgical site. dentists may be the first to discover this deficiency and other coagulopathies after simple tooth extraction. A case is reported which illustrates a typical presentation of this disorder. The need for haematological examination and special dental care is discussed.- - - - - - - - - - ranking = 1.6666666666667keywords = extraction (Clic here for more details about this article) |
4/7. Hereditary plasma thromboplastin antecedent (PTA, FXI) deficiency in a Saudi family.A rare case of factor XI (PTA) deficiency was discovered in a Saudi family in the Riyadh area. Nine members of the family were studied. Two were found to have a severe PTA deficiency; levels of factor XI clotting activity were 0.01 i.u./ml and 0.02 i.u./ml respectively. Both plasmas were markedly deficient in factor XI antigen and appeared to be negative for cross-reactive material (CRM-). The parents were first cousins and both were found to have a minor PTA deficiency. Factor XI levels were: mother 0.048 i.u./ml and father 0.33 i.u./ml. Another sibling was found to have a FXI level of 0.47 i.u./ml. menorrhagia and bleeding for 1 day after tooth extraction were the main bleeding manifestations found in one member with severe PTA deficiency. Clinically this member presented with iron deficiency anaemia. Other family members had no significant history of bleeding tendency. This is the first report of a Saudi Arabian family with PTA deficiency.- - - - - - - - - - ranking = 0.33333333333333keywords = extraction (Clic here for more details about this article) |
5/7. Ophthalmic surgery and plasma thromboplastin antecedent (factor XI) deficiency.A patient had bilateral cataracts and severe plasma thromboplastin antecedent deficiency (factor xi deficiency). This patient underwent successful cataract extraction in both eyes without complication. The problems inherent in performing eye surgery with factor xi deficiency are discussed.- - - - - - - - - - ranking = 0.33333333333333keywords = extraction (Clic here for more details about this article) |
6/7. Combined hereditary factor XI (plasma thromboplastin antecedent) deficiency, von Willebrand's disease, and xeroderma pigmentosum in a Japanese family.We report a 28-year-old-Japanese male who had a skin tumor derived from variant type xeroderma pigmentosum (XP), combined with factor XI (FXI) deficiency and type IIB von Willebrand's disease (vWd). The patient had abnormal bleeding history on tooth extraction. FXI clotting activity (FXI:C) and antigen (FXI:Ag) were remarkably decreased (< 0.01 U/ml, < 0.02 U/ml, respectively). factor viii (FVIII) clotting activity, von willebrand factor antigen (vWf:Ag), and ristocetin cofactor (RCoF) were 0.43 U/ml, 45%, and 57%, respectively. ristocetin-induced platelet agglutination (RIPA) revealed hyper-aggregation compared with a normal control. Multimeric composition of vWf in plasma showed a reduction in high molecular weight forms. The family study revealed two other subjects with homozygous hereditary FXI deficiency and vWd, and five subjects with heterozygous FXI deficiency. The relationship between FXI deficiency and vWd is discussed and previously reported cases are reviewed.- - - - - - - - - - ranking = 0.33333333333333keywords = extraction (Clic here for more details about this article) |
7/7. Clinical usefulness of desmopressin for prevention of surgical bleeding in patients with symptomatic heterozygous factor xi deficiency.Heterozygous factor XI (FXI) deficiency is sometimes associated with a significant bleeding tendency. Fresh frozen plasma of FXI concentrates are the mainstay of treatment in patients with a clear bleeding history, especially prior to surgery. However, these treatments are not completely free of risk. Furthermore, thrombosis has been reported in patients with FXI deficiency infused with FXI concentrate. No data are available on the possible efficacy of desmopressin in these patients. Two patients with a clear bleeding history associated with FXI deficiency and no additional haemostatic defects agreed to be treated with desmopressin before carpal tunnel surgery and dental extraction. The reduced basal FXI activity and antigen levels slightly increased after infusion, reaching borderline values. No bleeding was observed after surgical procedures. Desmopressin treatment seems a reasonable and useful choice in symptomatic, heterozygous FXI-deficient patients, thus reducing the cost of treatment, the risk of transmission of blood-borne viruses, and of thrombosis.- - - - - - - - - - ranking = 0.33333333333333keywords = extraction (Clic here for more details about this article) |