摘要:

SummaryThere are numerous new coagulation factor concentrates that are being manufactured using recombinant technology. Some are available for use currently for the treatment of bleeding disorders and others are in clinical trials. Recombinant factor VIII concentrates are licenced in most countries and one FVIII concentrate with the B domain deleted should be available in the near future. Recombinant VIIa concentrate is in advanced phase III testing for the treatment of patients with inhibitor antibodies. Recombinant factor IX has been used successfully in animals and will be tested in humans shortly. This paper reviews these products and discusses their uses and possible side‐effect

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00059.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00060.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

SummaryFour questionnaire surveys were conducted over a period of 20 years to evaluate long‐term effects of haemophilia treatment in the Netherlands. The response to the prestructured questionnaires in 1972, 1978, 1985 and 1992 varied between 70% and 84%. Data concerned treatment modalities, bleeding episodes, hospitalization, absenteeism, joint impairment and employment. Results over the period 1972–92 for patients with severe and moderately severe haemophilia showed that the use of prophylaxis had sharply increased (from 21% to 45%), as was the case for home treatment (from 4% to 62%). Consequently, the annual mean number of bleeds diminished from 19 to 13. Absence from school was markedly reduced (from 32 to 5 days), and sick leave in employed patients had also diminished (from 26 to 22 days). Furthermore, the use of inpatient hospital facilities, as well as employment in haemophilia patients, had nearly equalled that of the general Dutch male population. The self‐reported degree of joint impairment showed no overall improvement, but in patients aged under 35 years there seemed to be a slight reduction in severe impairment. Patients aged under 15 years finally had no severe impairment at all. Social participation can only be further improved if arthropathy is prevented from an early age. Therefore adequate prophylactic regimens and close monitoring of joint impairment in young adults are n

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00061.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

SummaryThe evolution of comprehensive care centres for haemophilia has altered the work of those involved in the care of haemophilia patients. The role of the haemophilia sister has expanded and the concept of the haemophilia nurse specialist has emerged.We describe the implementation of a local policy which has enabled haemophilia nurse specialists to requisition and administer clotting factor concentrates, DDAVP, tranexamic acid and hepatitis vaccines independently, in hospital and community settings. Since the introduction of this policy on 2 August 1994, prescribing practice has not changed in the haemophilia centre, nor has the involvement of medical staff in the care of haemophilia patients. This approach is widely applicable in other centres and should be a logical progression of the haemophilia nurse specialist's role.

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00062.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

SummaryWe tested for antibodies against factor VIII by using monoclonal antibody‐purified factor VIII preparation as a source of antigen. The factor VIII was adsorbed on nitrocellulose membranes and stored in a refrigerator until later use. Plasma or serum was incubated with the factor VIII containing strip and the antibody was detected by another incubation with peroxidase‐labelled antihuman immunoglobulin antibodies. The test was efficient in detecting antibodies in haemophilic and normal subjects with acquired inhibitors to factor VIII. It also detected antibodies to the factor VIII protein in a haemophilic subject with no evidence of inhibitor. The technique is simple, readily applicable, and serves as a useful screening tool for detecting factor VIII antibodies. The stability of the antigen‐containing strips in a refrigerator is a practical advantage with potential commercial applic

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00063.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

SummaryAlthough asymptomatic haemophiliacs have been shown to have abnormalities of their immune response, independent of HIV, clinical evidence of significant immunosuppression is limited. The only clinical report has been an outbreak ofM. tuberculosisin which a group of haemophilic boys appeared unduly susceptible to infection. These boys are now all HIV seropositive. Along with a group of HIV seronegative children with coagulation disorders and non‐haemophilic HIV seropositive men, these boys have been restudied to examine immune response to PPD. The HIV seropositive haemophilic boys that had hadM. tuberculosisinfection had reduced cytolytic response to PPD pulsed macrophages comparable to the non‐haemophilic HIV seropositive men. The HIV seronegative children with coagulation disorders showed a reduction in cytolytic activity at low effector:target ratios compared to normal controls.In vitrostudies showed that exogenous factor VIII concentrate could inhibit cytolytic activity to PPD pulsed macrophages. The possible role of chronic blood‐borne virus infection and factor VIII concentrates in the original outbreak are disc

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00064.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

SummaryIn order to determine the gene frequencies of nine polymorphic sites associated with FVIII and FIX genes in the Turkish population a sample of 50‐235 unrelated X chromosomes from healthy individuals were analysed by using PCR‐based assays. The Turkish population was found to be as polymorphic as Europeans in the FVII and FIX genes. Analysis of FIX haplotypes revealed that the most frequent haplotype observed in European populations and Anglo‐Americans was also very common among Turks. The present population‐based study indicates that two marker loci, namely HindIII and St14 in the factor VIII gene andDdeI andHhaI in the factor IX gene, are highly informative and useful markers that can be used in DNA linkage analysis for the assessment of haemophilia carriers and affected fetuses in the Turkish pop

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00065.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

SummaryA demographic survey was made of all children (n= 137) born with severe or moderate haemophilia in Sweden during the period 1970‐92. Bn addition, all prenatal diagnoses (n= 86) performing during the period were evaluated. The annual incidence of severe and moderate haemophilia, having remained constant for decades, increased from 0.78/10,000 males in the 1970s to 1.34 in the 1980s, levelling off at 1.31 in the 1990s. Although prenatal diagnosis did not affect the incidence of haemophilia in the 1970s and 1980s, it did so in the 1990s, because the incidence would have been 40% higher (1.83) had not prental diagnosis been available and 16 affected fetuses been aborted. The average proportion of sporadic cases, 62%, remained almost unchanged during the study period, suggesting mutation rates to be constant. There were fewere children in families with known haemophilia than in sporadic families, but no evidence was found to suggest that the frequency of female offspring (i.e. potential carriers) born in haemophilia families had increased since the option of prenatal diagnosis was introduce

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00066.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

SummarySerum samples taken from 73 patients with haemophilia A were tested for the presence of total antibody against hepatitis A (antiHAV) by radiommunoassay. 29 (39.7%) were antiHAV positive and 44 (60.3%) were antiHAV negative. These figures are comparable with local prevalence figures for immunity (40.3%) and susceptibility (59.7%) to HAV in adults. 30 patients were subsequently exposed to Scottish National Blood Transfusion Service High‐Purity Factor VIII (SNBTS HPVIII). They were re‐tested for the presence of antiHAV at a median of 5 months after exposure. Of these, 25 (83.3%) were antiHAV negative and five (16.7%) were antiHAV positive. All five of these antiHAV‐positive patients were positive on samples taken prior to exposure. No cases of seroconversion to hepatitis A occ

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00067.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

SummaryForty‐seven HIV‐infected haemophilic patients were entered into a hepatitis A vaccination programme. 10 patients (21%) were lgG seropositive for hepatitis A consistent with past exposure. Of the 37 patients offered vaccination, one refused and 31 completed the vaccination course, 17/13 (55%) seroconverted, nine after the second and eight after the third injection, and 14 patients failed to seroconvert. The CD4 lymphocyte counts immediately prior to vaccination were significantly higher in the patients who developed immunity compared to the nonresponders (median CD4 count in the immune group 380 × 106/1 (range 170–1290), median CD4 count in nonimmune group 110 × 106/1 (range 10–590),P== 0.003). No patient with a CD4 count<170 × 106/1 seroconverted and five patients with well‐preserved CD4 counts also failed to seroconvert. We conclude that HIV‐infected haemophilic patients, especially those with more advanced disease, have an impaired response to hepatitis A vaccination. Due to the likely failure of response in patients with CD4 counts<150 × 106/1, it is reasonable not to include these patients in a hepatitis A vaccin

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00068.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY