ISSN:0042-9007    

DOI:10.1111/j.1423-0410.1996.tb03670.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

AbstractOne of the most serious complications of the treatment of haemophilia A is the development of inhibitors. Former studies mostly considered the prevalence of inhibitor development, thus underestimating its true risk. Prevalences ranged widely (7–18%) probably due to the populations studied and the study design. Recent prospective previously untreated patients (PUP) studies were more comparable because of similar study designs. Eight PUP studies regarding the incidence of factor VIII inhibitors were analyzed: The inhibitor incidences (Independent of severity of haemophilia) ranged from 18.4 to 28%. Evaluating only severe haemophiliacs (factor VIII<2%) significantly higher incidences were found. After 9–36 exposure days (as medians inhibitor development occurred at 0.8‐3.3 years of age (as med

ISSN:0042-9007    

DOI:10.1111/j.1423-0410.1996.tb01341.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

AbstractThe purpose of the present study was to compare the 24‐hour recovery of red blood cells stored for 9 weeks in a hypoosmolar additive solution containing 150 mMglycerol to cells stored in Adsol®. Seven units of packed red cells were split into 2 aliquots. To one sample, 100 ml of the experimental additive solution (EAS 25) was added, and to the other, 50 ml of Adsol. At the end of the storage period the cells were labeled with51Cr. A double chromium technique was used to make it possible to perform comparative autologous studies in the same donor. The 24‐hour51Cr recovery value for EAS 25 was 73.03 ± (SD) 4.2% and for Adsol 60.9 ± 7.1. At 9 weeks the adenosine triphosphate levels were not significantly better compared to Adsol but the other in vitro measurements were better. New approaches to the study of red cell preservation are sug

ISSN:0042-9007    

DOI:10.1111/j.1423-0410.1996.tb00988.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

AbstractRecombinant polypeptides derived from coagulation factor VIII (fVIII) have been used to determine the epitopes and characteristics of human pathologic anti‐fVIII antibodies. The results of immunoprecipitation assays indicate that 70% of patient plasmas contain antibodies to the A2 as well as the C2 domains of the fVIII protein. The same polypeptides were used for inhibitor neutralization assays to demonstrate that about 60% of plasmas contain 2 or 3 different antibodies which collectively make up the inhibitor titer. The results of neutralization assays indicate that there is a third important inhibitor epitope within the light chain outside C2. Anti‐A2 antibodies prevent normal function of the factor Xase complex of the intrinsic pathway of blood coagulation. Anti‐C2 antibodies prevent the binding of fVIII to phospholipid and to von Willebrand factor, both of which are important for normal fVIII fun

ISSN:0042-9007    

DOI:10.1111/j.1423-0410.1996.tb01342.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

AbstractFive days storage of pooled platelet concentrates (PCs) with high yields often results in a pH fall and poor platelet morphology despite the use of specific containers. In this study we evaluated two techniques for prolonged storage of PCs with high platelet counts, by measuring pH and platelet swirl. In routine procedures, 90 PCs, prepared from five buffy coats, were stored in a single 1‐litre PL 732 container. After 5 days storage, 51 PCs with platelet counts below 3.1 times 1011/U showed a pH above 6.8 and optimal swirling scores. 29 of 39 PCs (74%) with yields above 3.1 times 1011/U showed pH values below 6.8 and poor swirling scores. These poor results can be prevented by shortening the duration of storage. PCs with high yields can be stored for 5 days either by dividing the concentrate into two containers or by adjusting the platelet count to 3.1 times 1011/U. In the latter procedure, we reduced the volume of the concentrate and found that of 102 PCs, only 9 showed poor values for pH and platelet swirl. The data clearly indicate the importance of measuring the prestorage platelet count to select the optimal procedure for concentrate storag

ISSN:0042-9007    

DOI:10.1111/j.1423-0410.1996.tb00989.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

ISSN:0042-9007    

DOI:10.1111/j.1423-0410.1996.tb01343.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

AbstractClerical errors occurring during specimen collection, issue and transfusion of blood are the most common cause of ABO incompatible transfusions. 40–50% of the transfusion fatalities result from errors in properly identifying the patient or the blood components. The frequency and type of errors observed, despite the implementation of measures to prevent them, suggests that errors are inevitable unless major changes in procedures are adopted. A fail‐safe system, which physically prevents the possibility of error, was adopted in January 1993 and concurrently a quality improvement program was implemented to monitor any transfusion errors. Up to December 1994, 10,995 blood units (5,057 autologous and 5,938 allogeneic) were transfused to 3,231 patients. Seventy‐one methodological errors (1/155 units) were observed, half of which were concentrated during the first 4 months of introducing the system. However the system detected and avoided four potentially fatal errors (1/2,748 units). Two cases involved the interchanging of recipient sample tubes, 1 case was due to patient misidentification and the other involved misidentification of blood units. In conclusion the system is effective in detecting otherwise undiscovered errors in transfusion practice and can prevent potential transfusion‐associated fatalities caused by misidentification of blood units or rec

ISSN:0042-9007    

DOI:10.1111/j.1423-0410.1996.tb00990.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

AbstractThe management of patients with classic hemophilia who develop inhibitory antibodies against transfused factor VIII has been the frequent subject of international and national conferences. While treatment of such patients cannot be considered as standardized, several therapeutic approaches are currently available. These include the induction of immune tolerance, removal or eradication of inhibitory antibodies by immune adsorption and/or immune suppression, neutralization of the inhibitor by porcine factor VIII or high doses of human factor VIII, and bypassing of the inhibitor using recombinant factor VIIa or other bypassing agents. In addition to currently available therapeutic strategies, other approaches are under investigation, including the use of selected factor VIII peptides that block the inhibitor; the use of immunologically mute factor VIII products, for example a human‐porcine factor VIII chimera not recognized by factor VIII inhibitors, and the use of anti‐idiotypic antibodies. The ability to recognize those patients destined to develop inhibitors is also under active investigat

ISSN:0042-9007    

DOI:10.1111/j.1423-0410.1996.tb01344.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

AbstractWhile screening Le(a+b+) Polynesian DN A samples for a candidateSewallele, a point mutation (C571→T) resulting in a new stop codon (Arg191→stop) in the α(1,2)fucosyltransferase gene (FUT2) was identified. This point mutation resulted in the gaining of a new restriction enzyme cleavage site (DdeI), which allowed restriction enzyme cleavage screening of 40 selected Polynesians and 42 random Caucasians. The nonsecretor phenotype in two of the three nonsecretor Polynesians analyzed was due to homozygosity for the ‘new’ mutation, whereas the third Polynesian nonsecretor (with Caucasian ancestors) was due to homozygosity of the ‘old’ (Trp143→stop) mutation. The nonsecretor phenotype in all Caucasians analyzed was a consequence of homozygosity for the ‘old’ mutation. Both the new and the old nonsecretor mutations were identified in the heterozygous state in some secretor‐positive Polynesians, while only the old mutation was found in the heterozygous state in Caucasians o

ISSN:0042-9007    

DOI:10.1111/j.1423-0410.1996.tb00991.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

ISSN:0042-9007    

DOI:10.1111/j.1423-0410.1996.tb01345.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY