Cases reported "Hemophilia A"

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1/23. Diagnosis of hemophilia made after intraoperative bleeding during attempted penetrating keratoplasty in an elderly patient.

    PURPOSE: To report an unusual case where the diagnosis of hemophilia was made after attempted penetrating keratoplasty in an elderly patient. methods: A 75 year old white male with a full-thickness corneal scar in the visual axis and a visually significant cataract OD was to undergo penetrating keratoplasty and cataract extraction with lens implantation for visual rehabilitation. There was no history of bleeding diathesis given. RESULTS: During placement of the Flieringa ring, a progressively enlarging 360 degrees subconjunctival hemorrhage was observed. Given the unusual bleeding,the procedure was aborted and the patientwas referred to the hematology service for further evaluation. Laboratory studies revealed a diagnosis of atypical hemophilia of mild severity. CONCLUSIONS: The initial diagnosis of hemophilia in any elderly patient is unusual. This case is even more unusual asthe diagnosis of a bleeding disorder was first considered after excessive subconjunctival hemorrhage developed during attempted penetrating keratoplasty.
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2/23. Successful use of recombinant factor viia in a haemophiliac with inhibitor undergoing cataract surgery.

    A 40-year-old patient with severe haemophilia A and an inhibitor against factor viii underwent a cataract extraction under local anaesthesia. Recombinant activated factor VII was use to achieve haemostasis. The procedure was successful. Neither bleeding complications nor side effects occurred.
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3/23. Acquired hemophilia a in a patient with systemic lupus erythematosus.

    A patient with systemic lupus erythematosus (SLE) developed acquired hemophilia a. The patient, a 24-year-old Japanese woman, was referred to our hospital because of uncontrollable bleeding following a tooth extraction. Laboratory examination revealed prolonged APTT (116 seconds), reduced factor viii activity (2.8 %) and the presence of factor viii inhibitor at a titer of 46.5 Bethesda units/ml. Transfusion of prothrombin complex concentrate and activated prothrombin complex concentrate followed by administration of prednisolone and cyclophosphamide successfully arrested bleeding and reduced the factor viii inhibitor level. Acquired hemophilia a is a rare but lethal condition. Rapid diagnosis and introduction of adequate therapies are critical.
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4/23. 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.
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5/23. Cementable implant-supported prosthesis, serial extraction, and serial implant installation: case report.

    Cement-retained implant-supported prostheses are particularly indicated where access for screw placement is limited or impossible like in posterior locations or where there is limited jaw opening. The patient in this case report suffered from limited jaw opening as a result of a long history of temporomandibular joint ankylosis related to hemophilia. Cement-retained implant-supported prostheses coupled with serial extraction, serial implant installations, and chairside provisional restorations made uneventful treatment possible.
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6/23. Management of oral bleedings with recombinant factor viia in children with haemophilia A and inhibitor.

    Dental extraction in patients with haemophilia A and high-titre inhibitor is always a high-risk procedure, which often presents a lot of problems associated with bleeding. Prothrombin complex concentrates or recombinant activated factor VII (rFVIIa) has been used to control bleeding. rFVIIa was administered to five boys with severe haemophilia A complicated with inhibitor, who underwent seven dental extractions. The age of the patients ranged between 8 and 13 years (median 10 years). The concentrate was administered in doses of 90-100 microg kg(-1) body weight. Duration in the therapy and intervals between rFVIIa doses depended on the severity of bleeding. rFVIIa was proven to be highly effective and no side-effects of the product were observed.
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7/23. 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.
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8/23. rehabilitation of a hemophiliac with implants: a medical perspective and case report.

    A patient suffering from classical hemophilia had previous surgery for ankylosis of the right temporomandibular joint. This was replaced by a costochondral graft and an overlay of temporalis muscle. A bilateral sagittal split was performed for a micrognathic mandible and a sleep apnea problem. That procedure solved the sleep apnea; however, it resulted in a prognathic mandible and an anterior open bite. The lower anterior teeth were periodontally involved with impaired alveolar support. The restricted opening of the oral cavity of 18 mm between maxillary and mandibular centrals and the potential danger of bleeding complicated the surgical and restorative procedures. The patient was prepared medically on each of 4 occasions with factor viii replacement concentrate, and oral antifibrinolytic therapy (tranexamic acid). The treatment of choice was the extraction of the remaining lower incisors and their replacement with an implant-supported temporarily cemented retrievable fixed prosthesis. Serial extractions and chairside temporization provided the surgeon with precise guides for implant placement, and enabled the patient to enjoy unimpaired function through periods of healing and osseointegration.
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9/23. Postdelivery head bleeding in hemophilic neonates. Causes and management.

    During a 12-month period, four of the five infants with hemophilia known to have been born in arkansas were examined for head bleeding. Three of the infants had had traumatic delivery, with use of low forceps in two and vacuum extraction in one. In the fourth patient, hemophilia was prenatally diagnosed, and vaginal delivery resulted in cephalohematoma. Diagnosis was delayed in three patients, including one with a family history of hemophilia. central nervous system bleeding may be more common in hemophilic neonates than has been presumed. pregnancy management should include consideration of family history of bleeding disorders and carrier testing in appropriate cases. In confirmed carriers, prenatal diagnosis is justified to allow choice of the least traumatic delivery method. Any term neonate with intracranial hemorrhage should be treated as being possibly hemophilic until proved otherwise.
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10/23. Use of microcrystalline collagen for hemostasis after oral surgery in a hemophiliac.

    A microcystalline collagen hemostat effected rapid hemostasis in dental extractions for a hemophilic patient with a significant level of inhibitor where preoperative transfusion of deficient factor was contraindicated. It appears that MCH can be used safely and effectively for a variety of oral procedures. In addition to its hemostatic properties, the ease with which it can be handled and its adherence make it adaptable for use in locations of limited access. In comparison with currently used materials, such as gelatin foam, it may have wider application and may be of greater benefit in achieving hemostasis in procedures in the oral cavity.
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