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Query: UMLS:C0684275 (haemophilia)
 10,958 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Three patients with hemophilic pseudotumors of the mandible were described and the treatment of the lesions was discussed. Surgical intervention, accompanied by adequate clotting factor replacement, was used in all three cases with satisfactory results. Pseudotumors of the jaws are a rare but serious complication of hemophilia. The cause of the mandibular lesion can be intraosseous hemorrhage, soft tissue hemorrhage with periosteal stripping and subperiosteal hematoma formation, or a combination of these factors. Surgical management with adequate replacement of clotting factor and the use of EACA is an effective method of treatment.
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PMID:Hemophilic pseudotumors of the mandible. 26 71

A survey of 11 hemophilia centers produced data concerning 28 females with extremely low levels of factor VIII or IX coagulant activity. Ten of the 28 have hemophilia A, six have hemophilia B, and 12 have severe von Willebrand's disease. The 16 females who have severe factor VIII or factor IX deficiency as an isolated defect exemplify several of the possible genetic explanations for the occurrence of hemophilia in females. All 16 bruise excessively, and several have had recurrent hemarthroses. Three of these girls, ages five, 10 and 23 years, have evidence of chronic hemophilic arthropathy. The 12 females with severe von Willebrand's disease are either homozygous for von Willebrand's disease or severely affected heterozygotes. All 12 have mucous membrane bleeding. In addition, five of the 12 have recurrent hemarthroses and three have evidence of chronic joint disease. However, the major problem in the adult females with von Willebrand's disease has been extreme menorrhagia. One of the seven adults underwent irradiation sterilization and another had a hysterectomy because of menorrhagia. The others have been managed with anovulatory drugs or plasma infusions and EACA. Despite menorrhagia, five pregnancies and deliveries have been uneventful in three of these women.
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PMID:Severe factor VIII and factor IX deficiency in females. 30 82

The outpatient treatment program for dental extraction in persons with the various forms of hemophilia has been reported. This program was initiated at the Hemophilia Rehabilitation Center at Orthopaedic Hospital in Los Angeles in 1966. During the nine years of this program, 260 patients have had 642 teeth extracted. eighty percent of these patients were outpatients and 64% of the extractions were performed on an outpatient basis. This outpatient treatment has been accompanied by an absence of a significant number of complications as evidenced by the postextraction hospitalization of only five patients during the nine-year period. The surgical technique uses a local anesthetic containing a vasoconstrictor or an ultralight intravenous general anesthetic in addition to the local anesthetic for the apprehensive or acutely infected patient. EACA is used as an antifibrinolytic agent postsurgically. Patient education and cooperation, diet control, and daily contact are important factors for the success of an outpatient oral surgery program for hemophilic patients.
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PMID:Outpatient treatment of hemophiliacs for dental extractions. 106 25

The results of a double-blind controlled trial, previously reported, showed that EACA is a useful adjunct to preoperative therapeutic concentrates of factor VIII or IX for dental extractions in hemophilia and Christmas disease. To estimate the amount of factor VIII and IX conserved in hemophiliacs receiving EACA for dental surgery compared with those not receiving EACA we have surveyed the usage of therapeutic materials in ten hemophilia centers in the U.S. and at Oxford. The amount of postoperative factor-VIII containing materials given to 20 U.S. hemophiliacs not receiving EACA averaged 11 062 units of factor-VIII activity per patient; 4,146 units for each of 22 U.S. patients receiving EACA; and 717 units for each of 56 patients at Oxford receiving EACA. Conservation of factor-IX-containing material was not as great. At Oxford 62.5% of patients receiving preoperative factor-VIII or -IX concentrates sufficient to raise the deficient factor to 50% of normal together with EACA, 24 g per day for ten days, required no postoperative therapeutic materials to control bleeding. The remainder required an average of two postoperative doses to control bleeding.
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PMID:The therapeutic role of epsilon-aminocaproic acid (EACA) for dental extractions in hemophiliacs. 107 19

In this presentation we have contrasted the normal blood-clotting mechanism with the failure to form blood clots in hemophiliacs due to the absence of protein factors necessary for conversion of prothrombin to thrombin. The statistics, hereditary basis, and long-term disabling consequences of hemophilia to the severely ffected patient are described. The systemic means of minimizing severe joint disabilities and serious internal bleeding hazards by employing concentrates of antihemophilic factors to reverse the bleeding defects are discussed. Availability and advantages of the types of concentrates are explained. The fatalistic attitude of hemophiliacs toward hepatitis is discussed, along with admonitions to avoid the use of aspirin, alcohol, and buttock injections. Alternative medications for pain are recommended; and injection sites for pediatric patients are suggested. The details of simplified oral surgical management of hemophilic patients without hospitalization are described, including local anesthetic injection technique, method of performing extractions, general anesthesia techniques when indicated, materials for packing of extraction sockets, regimen and precautions in use of Amicar administration for clot maintenance, postoperative diet, and postsurgical activity guidelines. Also noted is the self-administration of intravenous concentrate infusions at home in the event of hemorrhagin, so that bleeding is on the way to bein controlled even before the patient reaches the hospital. We avoided orthodontic treatment of hemophilic patients in the past; however, recently developed bracket-fixation techniques and auxiliary aids; along with an enlightened understanding that gingival bleeding is ot to be feared, have changed our attitude, and we now treat hemophilic patients in much the same manner as otherwise normal orthodontic patients...
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PMID:Orthodontics and dentistry for the hemophilic patient. 110 95

Four years prior to transplantation, a 14-year-old boy with severe haemophilia A and a high-responding factor VIII (FVIII) inhibitor developed an anteroseptal myocardial infarct while receiving high doses of an activated prothrombin complex concentrate (PCC). Cardiac transplantation was required for survival because of the ensuing cardiomyopathy. At surgery, the patient's inhibitor titre was 1.8 Bethesda units (BU). High-dose bolus therapy, followed by a continuous infusion of FVIII provided excellent operative and initial postoperative haemostasis without additional blood-product support. Once anamnaesis developed on day 6 postoperatively, recombinant factor VIIa (rFVIIa) therapy was initiated. Haemostasis remained excellent, except for the transient increase in chest-tube bleeding that was noted on day 7. epsilon-Aminocaproic acid was added and haemostasis was re-established. On day 15, rFVIIa was replaced with alternate day infusions of prothrombin complex concentrates (PCCs). On day 21 following the transplant, the patient was discharged, remaining on daily FVIII immune tolerance and thrice-weekly PCC prophylaxis. He remains well 24 months after transplant with an inhibitor titre of 39 BU. This paper describes the second case of cardiac transplantation complicated by haemophilia and an inhibitor, and discusses preoperative planning and operative and postsurgical haemostasis management.
Haemophilia 2001 Mar
PMID:Heart transplant in a factor VIII-deficient patient with a high-titre inhibitor: perioperative management using high-dose continuous infusion factor VIII and recombinant factor VIIa. 1126 Feb 85

Bernard-Soulier syndrome (BSS) is a rare congenital platelet disorder characterized by defective platelet adhesion and manifested by spontaneous and often profuse bleeding. Recombinant factor VIIa (rFVIIa) is a haemostatic agent licensed for the treatment of bleeding episodes in patients with haemophilia and inhibitors, which may represent a low-risk alternative to existing therapies in the management of patients with BSS. Here, we describe the use of rFVIIa for the treatment of three severe bleeding episodes in two patients with BSS. Data were extracted by automated searching of the international, Internet-based registry http://www.haemostasis.com . Patient 1, a 24-year-old woman, was admitted with severe epistaxis and hypotension. The diagnosis of BSS was confirmed by macrothrombocytopenia, absence of ristocetin-induced platelet agglutination (RIPA) and absence of glycoprotein (GP) Ibalpha and IX on the platelet surface. Epsilon aminocaproic acid (EACA; two 50-mg/kg doses), packed red blood cells (PRBCs, 2 U) and platelets (30 U) failed to control the bleeding and, after 13 h, three bolus doses of rFVIIa (90 microg/kg body weight) and a third dose of EACA were administered; bleeding stopped after the third dose of rFVIIa. Patient 2, a 15-year-old girl, initially presented with severe menorrhagia. A lack of RIPA and severe deficiency of GPIbalpha on the platelet surface confirmed the diagnosis of BSS. EACA and fresh-frozen plasma did not control the haemorrhage, but two bolus doses of rFVIIa (98 microg/kg body weight) resulted in a marked decrease in bleeding. On second admission, patient 2 had severe epistaxis and mild menorrhagia. Two rFVIIa doses (98 and 122.5 microg/kg body weight) were given, and the bleeding stopped. No adverse events were reported in these cases. These three admissions highlight the potential of rFVIIa for the treatment of severe bleeds in patients with BSS.
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PMID:Use of recombinant factor VIIa in the management of severe bleeding episodes in patients with Bernard-Soulier syndrome. 1604 15

Thromboelastography (TEG) assesses the global pattern of blood coagulation in the whole blood. Present day management of haemophilia is based on replacement therapy with lost factor by parenteral administration of factor concentrates. It is very well known that interaction of cellular components in the blood also affect the thrombin generation which in turn might produce varied TEG patterns that might reflect the clinical severity in haemophilia patients. We evaluated 66 severe haemophilia A and B patients (as assessed by one-stage assay) by TEG and correlated the varied TEG patterns with the clinical severity in the patients. Four distinct TEG patterns were observed; Group A consisted of eight patients with hypercoagulable patterns while group B consisted of two patients showing hyperfibrinolytic patterns. Group C comprised of 17 patients whose TEG tracings did not show the initiation of clot formation at all while group D comprising of 39 patients showed varied clot initiation times ranging from almost normal pattern to a highly prolonged split times. Groups A and D patients were relatively milder clinically while groups B and C were clinically severe as assessed by the number of bleeding episodes, the frequency of transfusion and joint deformity. Subsequently we also evaluated the in vitro efficacy of the antifibrinolytic drug, EACA in normalizing the TEG patterns in 12 patients (group C) who did not show the initiation of clot formation in the TEG tracings to see the contribution of fibrinolysis in producing such patterns. The use of EACA in vitro in this group improved the TEG profile of these patients. In conclusion, the classification of severe haemophilia patients based on TEG patterns correlated well with the clinical severity and the ex vivo use of antifibrinolytics like EACA are effective in improving the TEG profile of all patients who had an abnormal TEG pattern without any clot initiation.
Haemophilia 2007 Nov
PMID:Correlation of thromboelastographic patterns with clinical presentation and rationale for use of antifibrinolytics in severe haemophilia patients. 1797 50

In patients with confirmed or suspected type 1 von Willebrand disease (VWD), adenotonsillectomy has been reported to be associated with a rate of peri-operative hemorrhage between 8 and 23%. Desmopressin acetate (DDAVP, 1-deamino 8-D arginine- vasopressin) is the treatment of choice for type 1 patients with baseline von Willebrand factor levels of 10 IU/dL or greater. DDAVP is generally well tolerated; however, severe hyponatremia and seizures have been reported in young children less than 2 years of age, limiting its use in this age group. Antifibrinolytic therapy plays an important adjunctive role in the effective treatment of mucocutaneous bleeding, particularly in the oropharynx where the salivary concentration of fibrinolytic enzymes is high. During the past 10 years, we treated 6 pediatric patients with mild/moderate type 1 VWD undergoing an adenotonsillar procedure at our institution with the same hemostatic regimen consisting of one single dose of DDAVP and an extended use of EACA. In this small case series, the above mentioned prophylactic treatment regimen was both well tolerated and efficacious in controlling hemorrhage. Furthermore, DDAVP-related complications were avoided in a pediatric population with a higher risk of developing them.
Haemophilia 2012 Mar
PMID:Haemostasis prophylaxis using single dose desmopressin acetate and extended use epsilon aminocaproic acid for adenotonsillectomy in patients with type 1 von Willebrand disease. 2177 Dec 8

The accurate tumor marker detection at an early stage can prevent people from getting cancer to a great extent. Herein, a novel tri-antibody dual-channel biosensing strategy is applied in multianalysis of carcino-embryonic antigen (CEA) and nuclear matrix protein 22 (NMP22). In this immunosensor fabrication process, graphene oxide/polyaniline nanostructures are used as matrix and mesoporous NKF-5-3 is used as labels. Two kinds of antigens can be obtained from the signals of neutral red and toluidine blue, respectively, which are modified on the labels. In this tri-antibody dual-channel biosensing platform, sulfur-doped graphene sheet is synthesized by click chemistry as the framework structure. Majority of the incubations are conducted in individual steps, which ensure the surface incubation more tightly. The detection limit of NMP22 and CEA are 25 and 30 fg/mL, respectively. The low detection limit and excellent stability can ascribe to the tri-antibody dual-channel strategy, which makes the sensor platform from surface to the space. The clinical urine sample analysis achieves a good performance. The urine-based test can avoid the secondary injury on hemophilia or ischemic patients, displaying a potential application in clinical diagnosis.
ACS Appl Mater Interfaces 2017 Nov 01
PMID:Sulfur-Doped Graphene-Based Immunological Biosensing Platform for Multianalysis of Cancer Biomarkers. 2899 81


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