1/6. Combined deficiency of factor V and factor viii. A report of another case.A patient with combined factor V and factor viii deficiency is presented. The bleeding manifestations were: easy bruising, post-traumatic bleeding, bleeding after tooth extractions. The main laboratory feature was a prolonged partial thromboplastin time which was corrected by the addition of adsorbed normal plasma but not by the addition of normal serum, hemophilia a plasma of another patient with combined factor V and factor viii deficiency. The thromboplastin generation test was clearly abnormal and was corrected by the addition of adsorbed normal plasma but not by addition of normal serum. Prothrombin consumption was also defective. prothrombin time was slightly prolonged too, Thrombin time, platelet and vascular tests were within normal limits and there was no hyperfibrinolysis. factor viii was 8% of normal, whereas factor V was 14% of normal. factor viii associated antigen was normal. All other clotting factors were within normal limits. The parents of the propositus were consanguineous (first cousins) but had normal factor V and factor viii activity and normal factor viii antigen. The same was true for other family members. The hereditary transmission of the condition appears autosomal recessive.- - - - - - - - - - ranking = 1keywords = extraction (Clic here for more details about this article) |
2/6. Combined factor V and factor viii deficiency in a Thai patient: a case report of genotype and phenotype characteristics.A Thai woman, with no family history of bleeding disorders, presented with excessive bleeding after minor trauma and tooth extraction. The screening coagulogram revealed prolonged activated partial thromboplastin time and prothrombin time. The specific-factor assay confirmed the diagnosis of combined factor V and factor viii deficiency (F5F8D). Her plasma levels of factor V and factor viii were 10% and 12.5% respectively. The medications and blood product treatment to prevent bleeding from invasive procedure included 1-deamino-8-d-arginine vasopressin, cryoprecipitate, factor viii concentrate, fresh frozen plasma and antifibrinolytic agent. Gene analysis of the proband identified two LMAN1 gene mutations; one of which is 823-1 G --> C, a novel splice acceptor site mutation that is inherited from her father, the other is 1366 C --> T, a nonsense mutation that is inherited from her mother. Thus, the compound heterozygote of these two mutations in LMAN1 cause combined F5F8D.- - - - - - - - - - ranking = 1keywords = extraction (Clic here for more details about this article) |
3/6. The treatment of patients with factor-V deficiency.Two patients with congenital factor-V deficiency and a third with a combined deficiency of factor V and factor viii are described. Under cover of fresh frozen plasma, tooth extractions were performed on two of these patients and spontaneous bleeding arrested. It is concluded that the achievement of a blood level of factor V of 20 per cent once daily is sufficient to assure adequate haemostasis. A low recovery of factor V activity in the plasma following infusion was found. However, in spite of this, adequate blood levels were easily attained due to the excellent preservation of factor V activity in the stored fresh frozen plasma.- - - - - - - - - - ranking = 1keywords = extraction (Clic here for more details about this article) |
4/6. A case of congenital factor v deficiency combined with multiple congenital anomalies: successful management of palatoplasty.A patient with congenital factor v deficiency combined with mental retardation and several congenital anomalies including cleft palate, dwarfism, microcephaly and right hydrocele testis is described. The levels of factor V activity and factor V antigen of plasma were significantly decreased. The platelet lysate obtained from him also showed a significantly low level of factor V activity. Palatoplasty and tooth extraction were successfully performed under transfusion therapy with fresh-frozen plasma.- - - - - - - - - - ranking = 1keywords = extraction (Clic here for more details about this article) |
5/6. Dental extraction in a patient with congenital deficiencies of factors V and VIII.A case of combined congenital deficiencies of factors V and VIII is reported. The patient's right mandibular first molar was extracted and a combination of local hemostatic treatment and the transfusion of fresh plasma resulted in healing of the socket without further postoperative bleeding.- - - - - - - - - - ranking = 4keywords = extraction (Clic here for more details about this article) |
6/6. Hemostatic treatment after tooth extraction in a patient with factor v deficiency.BACKGROUND: factor v deficiency is a very rare hereditary coagulation disorder and tooth extraction in the patient with factor v deficiency has not been reported except in one case. patients AND TREATMENT: A 38-year-old woman with factor v deficiency was referred for extraction of the impacted lower third molar. After intravenous administration of frozen fresh plasma (FFP) and recognition of an increase of factor V level from 1-31%, upper and lower third molars were extracted. Eighteen and 48 h after the extraction, factor V was intermittently supplemented by injection of 4 and 2 units of FFP, respectively, and factor V was maintained above 12%. To form fast coagula and to protect the wound, the lower extraction socket was filled with a fibrin glue composed of factor xiii and fibrinogen (Beriplast P) and a plastic splint was applied. The wound was healed and epithelized within 2 weeks after the extraction without any bleeding or infectious consequences. CONCLUSION: Extraction in the patient with factor V hereditary deficiency is safely performed by both supplementation of factor V and application of local hemostasis.- - - - - - - - - - ranking = 9keywords = extraction (Clic here for more details about this article) |