The availability of plasma products and the care of hemophilia patients.
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Biomedical subjects
Publications and source records attributed to L M Aledort.
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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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Hemophilic patients must be thought of as special patients. Since routine dental procedures, however, do not usually involve bleeding, there is no contraindication to general dental treatment for hemophiliacs. Aspirin-containing compounds should never be prescribed. Caution must be used with local anesthetic block techniques. Preventive dentistry is vital to the younger hemophiliac; older hemophiliacs may require extensive treatment to restore mouths that have been neglected for years. Surgical dental procedures are now considered possible for hemophiliacs and may be performed on an outpatient basis, but they must be judiciously coordinated by the dental and medical team.
To determine the specificity and sensitivity of current technics for detecting carriers of classic hemophilia, three investigators simultaneously tested obligate carriers and noncarriers for immunologic and procoagulant factor VIII activity. Overall correct classification ranged from 72 per cent (36 of 50) to 94 per cent (47 of 50). The maximum accuracy obtained with the same linear-discriminant-function method on all data was 90 per cent (26 of 29) in detecting carriers without misclassifying normal persons as carriers (none of 21). Lowest accuracy by the same technic was 66 per cent (19 of 29) carrier detection while misclassifying 19 per cent (four of 21) normal persons. Precision of testing for both factor VII activity and antigen was high (standard deviations from 0.004 to 0.026 on a log scale). Differences between participants seemed related to laboratory technics rather than to statistical methods. The factor VIII activity/antigen measurement is a valid technic for detecting in the carrier state of hemophilia A.
Fractionation technology has made Factor IX concentrates available. In addition to a very high incidence of hepatitis, thrombosis is being recognized as a consequence of their use. Contradictory reports exist in the literature as to the incidence of thrombotic events. A recent study of thrombohemorrhagic phenomena after infusion with Factor IX concentrates in patients with congenital Factor IX deficiency (hemophilia B) or liver disease, revealed an incidence of twenty episodes in 188 cases (11%). It is imperative to delineate the thrombogenic factors and find methods for their removal to insure the safety of Factor IX products.
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Factors determining thrombus formation on a foreign surface were studied with the use of plastic flow chambers introduced into extracorporeal shunts. Silicone rubber shunts, joining the carotid artery and jugular vein, were implanted in dogs and remained patent for several weeks. The flow chamber geometry consisted of a 4.8 mm diameter straight tube having a 3.2 X 3.2 mm circumferential cavity in the wall. Chambers were introduced sequentially into the shunts for exposure times of 10 to 30 minutes and regulated blood flow rates of 100 to 400 ml/min. The dry weight of thrombus accumulated in the chamber (5 to 50 mg) was found to increase with exposure time up to 20 minutes and to decrease with increasing flow rate. Various components of the process of thrombus formation were altered by the administration of acetylsalicylic acid, heparin and lysozyme, used alone and in pairs. Heparin was found to be the most effective antithrombotic agent, dry weights of accumulated thrombus being on the order of 50 percent lower when compared to control values. The efficacy of heparin was found to be unaffected by the presence of aspirin and lysozyme, which themselves were not effective antithrombotic agents under the conditions of these experiments. The technique described here may provide a useful animal model for studying the influence of blood flow and different biomaterials on thrombus formation.
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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