scholarly journals The use of fresh frozen plasma or a concentrate of factor IX as replacement therapy before liver biopsy.

Gut ◽  
1975 ◽  
Vol 16 (8) ◽  
pp. 621-625 ◽  
Author(s):  
B G Gazzard ◽  
J M Henderson ◽  
R Williams
Author(s):  
Hortensia De la Corte-Rodriguez ◽  
E. Carlos Rodriguez-Merchan ◽  
M. Teresa Alvarez-Roman ◽  
Monica Martin-Salces ◽  
Victor Jimenez-Yuste

Background: It is important to discard those practices that do not add value. As a result, several initiatives have emerged. All of them try to improve patient safety and the use of health resources. Purpose: To present a compendium of "do not do recommendations" in the context of hemophilia. Methods: A review of the literature and current clinical guidelines has been made, based on the best evidence available to date. Results: The following 13 recommendations stand out: 1) Do not delay the administration of factor after trauma; 2) do not use fresh frozen plasma or cryoprecipitate; 3) do not use desmopressin in case of hematuria; 4) do not change the product in the first 50 prophylaxis exposures; 5) do not interrupt immunotolerance; 6) do not administer aspirin or NSAIDs; 7) do not administer intramuscular injections; 8) do not do routine radiographs of the joint in case of acute hemarthrosis; 9) Do not apply closed casts for fractures; 10) do not discourage the performance of physical activities; 11) do not deny surgery to a patient with an inhibitor; 12) do not perform instrumental deliveries in fetuses with hemophilia; 13) do not use factor IX (FIX) in patients with hemophilia B with inhibitor and a history of anaphylaxis after administration of FIX. Conclusions: The information mentioned previously can be useful in the management of hemophilia, from different levels of care. As far as we know, this is the first initiative of this type regarding hemophilia.


Blood ◽  
1987 ◽  
Vol 70 (4) ◽  
pp. 1208-1210 ◽  

A 1986 survey of seven hemophilia treatment centers in Pennsylvania (PA) has revealed that 22 hemophiliacs residing in PA have developed the acquired immunodeficiency syndrome (AIDS), representing 9.2% of the total 238 United States hemophiliac AIDS cases. These 22 included ten (45.5%) from western PA (W-PA), eleven (50.0%) from central PA (C-PA), and one (0.5%) from eastern PA (E-PA). The HIV antibody prevalence for these three geographic groups is comparable, with 84 of 178 (47.2%) of hemophiliacs in W-PA seropositive, 102 of 182 (56.0%) in C-PA seropositive, and 105 of 177 (59.3%) in E-PA seropositive. Blood product usage for these three areas is comparable: 47.8 X 10(3) (W-PA) v 43.9 (C-PA) v 53.3 (E-PA) units factor VIII concentrate per patient per year; 36.5 v 24.5 v 33.7 for factor IX concentrate; 8.4 v 4.7 v 7.7 for cryoprecipitate; and 1.3 v 2.7 v 1.0 for fresh frozen plasma, respectively. These data demonstrate a geographic variation in hemophilia AIDS incidence in PA, with a tenfold higher incidence in W- PA and C-PA than E-PA, which is unrelated to differences in HIV antibody prevalence, patient blood product usage, or inaccuracies in AIDS case reporting. Because of the greater than or equal to 5 year median latency between HIV infection and development of AIDS, the AIDS incidence will continue to change, but other factors appear to be operative in the development of AIDS in hemophiliacs.


Blood ◽  
1987 ◽  
Vol 70 (4) ◽  
pp. 1208-1210 ◽  

Abstract A 1986 survey of seven hemophilia treatment centers in Pennsylvania (PA) has revealed that 22 hemophiliacs residing in PA have developed the acquired immunodeficiency syndrome (AIDS), representing 9.2% of the total 238 United States hemophiliac AIDS cases. These 22 included ten (45.5%) from western PA (W-PA), eleven (50.0%) from central PA (C-PA), and one (0.5%) from eastern PA (E-PA). The HIV antibody prevalence for these three geographic groups is comparable, with 84 of 178 (47.2%) of hemophiliacs in W-PA seropositive, 102 of 182 (56.0%) in C-PA seropositive, and 105 of 177 (59.3%) in E-PA seropositive. Blood product usage for these three areas is comparable: 47.8 X 10(3) (W-PA) v 43.9 (C-PA) v 53.3 (E-PA) units factor VIII concentrate per patient per year; 36.5 v 24.5 v 33.7 for factor IX concentrate; 8.4 v 4.7 v 7.7 for cryoprecipitate; and 1.3 v 2.7 v 1.0 for fresh frozen plasma, respectively. These data demonstrate a geographic variation in hemophilia AIDS incidence in PA, with a tenfold higher incidence in W- PA and C-PA than E-PA, which is unrelated to differences in HIV antibody prevalence, patient blood product usage, or inaccuracies in AIDS case reporting. Because of the greater than or equal to 5 year median latency between HIV infection and development of AIDS, the AIDS incidence will continue to change, but other factors appear to be operative in the development of AIDS in hemophiliacs.


1990 ◽  
Vol 63 (01) ◽  
pp. 027-030 ◽  
Author(s):  
Maureen Andrew ◽  
Barbara Schmidt ◽  
Lesley Mitchell ◽  
Bosco Paes ◽  
Frederick Ofosu

SummaryThe ability to generate thrombin is decreased and delayed in plasma from the healthy newborn infant compared to the adult. Only 30 to 50% of peak adult thrombin activity can be produced in neonatal plasma. To test whether this observation can be explained by the low neonatal levels of the contact or vitamin K dependent factors, we measured neonatal thrombin generation after raising the concentration of these factors to adult values. We also determined whether the addition of a variety of blood products to neonatal plasma improved thrombin generation. An amidolytic method was used to quantitate intrinsic (APTT) and extrinsic (PT) pathway thrombin generation in defibrinated pooled cord plasma from healthy term infants. Added individually, factors VII, IX, X or the contact factors (CF) failed to alter the rate or the total amount of thrombin generated in neonatal plasma. In contrast, the addition of prothrombin increased the total amount of thrombin generated to above adult values in both the APTT and the PT systems but did not alter the rate of thrombin generation. The rate of thrombin generation in cord plasma shortened after a combination of II, IX, X and CF was added to the APTT system or II, VII and X to the PT system. In both systems, the total amount of thrombin generated was linearly related to the initial prothrombin concentration. Each of fresh frozen plasma, cryoprecipitate, plasma from platelet concentrates, or factor IX concentrate (in amounts used therapeutically) caused an increase in the total amount of thrombin generated which was related to the increase in prothrombin concentration. Thus, the total amount of thrombin generated in newborn plasma is critically dependent on the prothrombin concentration whereas the rate at which thrombin is generated is dependent on the levels of many other coagulation proteins in combination.


1976 ◽  
Vol 35 (02) ◽  
pp. 377-381 ◽  
Author(s):  
Joel A. Spero ◽  
Jessica H. Lewis ◽  
Ute Hasiba ◽  
Lawrence D. Ellis

SummaryThis is the tenth patient in thirteen years to be reported with the findings of an isolated factor X deficiency associated with primary amyloidosis. A favorable response to factor IX concentrate was manifested by temporary clinical and laboratory correction of her diathesis. This mode of treatment, therefore, provides an approach to therapy for bleeding complications in this group of patients who have previously failed to respond to fresh frozen plasma.


Blood ◽  
1986 ◽  
Vol 67 (3) ◽  
pp. 592-595 ◽  
Author(s):  
MV Ragni ◽  
GE Tegtmeier ◽  
JA Levy ◽  
LS Kaminsky ◽  
JH Lewis ◽  
...  

Abstract Antibodies to the AIDS retrovirus, specifically to human T cell lymphotropic virus, type III, and AIDS-associated retrovirus, were detected with increasing prevalence in a population of 190 hemophiliacs from western Pennsylvania between 1981 and 1984: 7.7% in 1981, 20.0% in 1982, 45.5% in 1983, and 62.5% in 1984. The seropositive included approximately three fourths of those receiving factor VIII concentrate, nearly one third of those receiving factor IX concentrate, nearly one fifth of those receiving cryoprecipitate, and none of those receiving fresh frozen plasma. The seroconversion rate, determined on 43 seropositive hemophiliacs from this group who were serially sampled, was 0% in 1977, 4.7% in 1978, 4.9% in 1979, 2.6% in 1980, 10.5% in 1981, 52.9% in 1982, 87.5% in 1983, and 100% in 1984. Of 27 seropositive for three or more years (since 1982 or before), four (15%) have developed AIDS and seven (26%), diffuse lymphadenopathy (ARC); of 16 seropositive for less than three years, none has developed AIDS and three (19%) have developed ARC. The mean time from seroconversion to onset of ARC, 0.8 +/- 0.2 years (SEM), was shorter (P less than .001) than the time to onset of AIDS, 4.1 +/- 0.6 years. These findings confirm the widespread presence of AIDS retrovirus and support the association of these retroviruses with the acquired immunodeficiency syndrome and related conditions.


1997 ◽  
Vol 77 (03) ◽  
pp. 477-480 ◽  
Author(s):  
Mike Makris ◽  
Mike Greaves ◽  
Wendy S Phillips ◽  
Steve Kitchen ◽  
Frits R Rosendaal ◽  
...  

SummaryHaemorrhage, including intracranial bleeding, is a common, potentially lethal complication of warfarin therapy and rapid and complete reversal of anticoagulation may be life-saving. Fresh frozen plasma (FFP) and vitamin K are most frequently administered. Because of the variable content of vitamin K-dependent clotting factors in FFP, and the effects of dilution, the efficacy of this approach is open to doubt. We have therefore compared the effects of FFP and clotting factor concentrates on the INRs and clotting factor levels of orally anticoagulated subjects requiring rapid correction of their haemostatic defect. In many, the pre-treatment INR was considered to be dangerously above the target therapeutic range. In the 12 patients given FFP, the INR did not completely correct (range 1.6-3.8, mean 2.3) indicating an ongoing anticoagulated state in all. In contrast, the INR in 29 subjects given clotting factor concentrates was completely corrected in 28 (range 0.9-3.8, mean 1.3). Following treatment, marked differences were observed in clotting factor IX levels between the two groups. The median factor IX level was 19 u/dl (range 10-63) following FFP infusion and 68.5 u/dl (range 31-111) following concentrate. In FFP treated patients, poorer responses were also observed for each of the other vitamin K-depen- dent clotting factors but these were less marked than for factor IX, which was present in low concentrations in some batches of FFP. Thus, haemostatically effective levels of factor IX cannot be achieved, in most instances, by the conventional use of FFP in patients requiring reversal of their anticoagulant therapy. Clotting factor concentrates are the only effective option where complete and immediate correction of the coagulation defect is indicated in orally anticoagulated patients with life or limb-threatening haemorrhage.


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