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  • 1
    Electronic Resource
    Electronic Resource
    Towards achieving global haemophilia care — World Federation of Hemophilia programmes (1998)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 4 (1998), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary. The overall purpose of the “Decade Plan” published in 1992 was to do everything possible to make effective treatment available to people with haemophilia throughout the world. In 1990 it was estimated that there were 350,000 individuals with haemophilia worldwide, with 80% or 280,000 without treatment. By the year 2020 this may have reached the figure 440,000, or the population of Jerusalem. Earlier this century Carroll Birch reported a series of 113 patients where 82 died before their 15th year, and only six survived beyond 40. Thus as Jones has pointed out for the developing world this is a paediatric problem for the WFH to face now. The examples described in this paper illustrating the WFH programmes and the committment of many people from the 88 member organisations worldwide as well as the doctors and scientists shows what can be done through cooperation. There is no doubt that these achievements are capitalising from the unique quality of WFH from the beginning, that is a membership which includes both those with haemophilia and their families, and those with an interest in treating haemophilia.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.1998.440463.x
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  • 2
    Electronic Resource
    Electronic Resource
    The obstetric experience of carriers of haemophilia (1997)
    Oxford, UK : Blackwell Publishing Ltd
    BJOG 104 (1997), S. 0 
    Details
    ISSN: 1471-0528
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Objective To review the obstetric problems, pregnancy outcome and management of carriers of haemophilia.Design Retrospective review of haemophilia and maternity unit records.Setting Haemophilia Comprehensive Care Centre.Participants Thirty-two carriers of haemophilia (24 haemophilia A, eight haemophilia B) who had their obstetric care at the Royal Free Hospital over a 10-year period (1985–1995).Main outcome measures Uptake and results of prenatal diagnosis, changes in factor levels during pregnancy, effect of knowledge of fetal gender on obstetric management and neonatal outcome, and maternal haemorrhagic complications.Results There were 82 pregnancies and 32 resulted in miscarriage or social abortion. The option of prenatal diagnosis was taken up in only 35% (17/48) of pregnancies. There were five affected male fetuses diagnosed prenatally but only three women opted for termination of the pregnancy. Knowledge of fetal gender was unavailable to the attending obstetrician in 46% (21/46) of pregnancies. A fetal scalp electrode was applied in eight, fetal blood sampling was performed in four, and ventouse delivery was conducted in one of these pregnancies. No adverse effects were reported from the first two procedures, but the ventouse delivery was associated with a huge cephalhaematoma requiring blood transfusion. On the other hand, in five cases fetal blood sampling was withheld because fetal gender was unknown. Four of the eight caesarean sections performed might have been avoided if the gender had been known. The incidence of primary and secondary postpartum haemorrhage was high, 22% (including two cases with massive haemorrhage) and 11%, respectively.Conclusion Carriers of haemophilia A and B require special obstetric care with close liaison with the haemophilia centre, and management guidelines should be available and observed. Knowledge of fetal gender is very valuable for management in labour and should be determined antenatally even if the mother declines prenatal diagnosis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1471-0528.1997.tb12024.x
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  • 3
    Electronic Resource
    Electronic Resource
    Acquired haemophilia, an unusual cause of severe postpartum haemorrhage (1997)
    Oxford, UK : Blackwell Publishing Ltd
    BJOG 104 (1997), S. 0 
    Details
    ISSN: 1471-0528
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1471-0528.1997.tb12036.x
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  • 4
    Electronic Resource
    Electronic Resource
    Schottky barrier diode on a submicron-thick silicon membrane using a dual surface fabrication technique (1986)
    [S.l.] : American Institute of Physics (AIP)
    Journal of Applied Physics 60 (1986), S. 4038-4040 
    Details
    ISSN: 1089-7550
    Source: AIP Digital Archive
    Topics: Physics
    Notes: The successful fabrication of a Schottky barrier diode on a 150-nm-thick single-crystal silicon membrane using a new dual surface technique is described. The cut-off frequency of the diode was calculated from the zero-bias capacitance and series resistance to be approximately 2 THz, which compares favorably with currently achieved values in GaAs. The advantages of the dual surface device fabrication technique pioneered in this paper are noted.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1063/1.337533
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  • 5
    Electronic Resource
    Electronic Resource
    High-resolution resonant refractive index lithography and applications (1989)
    [S.l.] : American Institute of Physics (AIP)
    Journal of Applied Physics 66 (1989), S. 4481-4487 
    Details
    ISSN: 1089-7550
    Source: AIP Digital Archive
    Topics: Physics
    Notes: We describe resonant refractive index lithography (RRIL), a new technique to enhance the resolution of optical lithography. RRIL utilizes a highly refractive layer between the mask and the photosensitive layer to increase optical resolution. The refractive index of this layer is enhanced by a resonant absorption near the wavelength of the exposing radiation. In this work GaAs, which has a large resonant refractive index increase conveniently centered around the mercury spectral g line, is used as the refractive layer. It is shown that the large reduction in wavelength, which is more than a factor of 5 at 431 nm, enables this technique to increase resolution by as much as 226%. In addition, the absorption associated with the increased refractive index significantly attenuates the diffracted light and virtually eliminates standing waves that would be produced by reflections between the mask and the substrate. Furthermore, the refractive medium enhances the collimation of the exposing radiation. We demonstrate the RRIL technique by fabricating an opposed gate-source transistor with a sub-quarter-micrometer source electrode. The dimensions and alignment precision achieved were less than one-half the wavelength of the incident radiation. In this paper RRIL is introduced, experimental results are presented and compared with a model of the diffraction effects, and finally the RRIL technique is applied to planar lithography and microscopy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1063/1.343946
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  • 6
    Electronic Resource
    Electronic Resource
    Fluconazole for oropharyngeal candidiasis in anti-HIV positive haemophiliacs (1991)
    Oxford, UK : Blackwell Publishing Ltd
    Alimentary pharmacology & therapeutics 5 (1991), S. 0 
    Details
    ISSN: 1365-2036
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Candidiasis of the oropharynx and oesophagus is one of the most common problems encountered in patients with HIV disease. Fluconazole is a bis-triazole antifungal agent with a long serum half-life. Sixteen anti-HIV positive patients (15 haemophiliacs and one blood transfusion recipient) with a clinical diagnosis of oropharyngeal candidiasis were treated with 50 mg fluconazole daily for 14–28 days and then either 150 mg fluconazole or placebo weekly for 6 months in a prophylactic phase. Clinical cure occurred in all patients, and mycological cure occurred in 13/16 (81%) patients. In the prophylactic phase, there were 2/5 (40%) relapses in the placebo arm compared with 1/8 (12.5%) in the fluconazole arm, but this was not statistically significant by Fisher's onesided exact test (P= 0.31). It is concluded that fluconazole is an effective treatment of oropharyngeal candidiasis and has potential for prophylactic use.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2036.1991.tb00021.x
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  • 7
    Electronic Resource
    Electronic Resource
    The use of intermediate-purity clotting factor concentrates and HIV disease progression in men with haemophilia (1996)
    Oxford, UK : Blackwell Publishing Ltd
    Haemophilia 2 (1996), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary. On the basis of evidence from the immune systems of patients with haemophilia infected with HIV, patients in the UK have been switched to high-purity clotting factor concentrates. However, there is very little information currently available on the effect of intermediate-purity clotting factor concentrates on progression of HIV disease. Among 99 HIV-positive men with severe factor VIII deficiency registered at The Royal Free Hospital Haemophilia Centre, a 100 IU kg-1 increase in the yearly amount of concentrate received did not appear to be associated with more rapid progression to AIDS, death or to a low CD4 count. However, the use of total concentrate usage may mask a more subtle effect of specific concentrate contaminants on the progression of HIV disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2516.1996.tb00019.x
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  • 8
    Electronic Resource
    Electronic Resource
    A post-marketing safety and efficacy assessment of a monoclonal antibody purified high-purity factor VIII concentrate (1996)
    Oxford, UK : Blackwell Publishing Ltd
    Haemophilia 2 (1996), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary. The identification of infrequent side-effects of clotting factor concentrates, undetected by clinical trials, is facilitated by post-marketing surveillance. We present a post-marketing surveillance study in which 97 patients with haemophilia A, attending three haemophilia centres, were treated over a median follow-up period of 284 days (range 1–1074), and a total follow-up period of 30,080 days, with a pasteurized immunoaffinity purified factor VIII concentrate (Monoclate-P, Armour, Collegeville, USA). 5216 infusions, using 10,527,000 units of Monoclate-P, were carried out, mostly for routine haemarthroses or prophylaxis.No new inhibitors were observed during the study. At the start of the study 60/97 were HIV seropositive, 67/97 HBs antibody positive, 12 HbsAb negative and the remainder HBsAb positive before the study period. 13/14 tested were HAV seropositive at the beginning of the study. One patient became HAV seropositive during the study period, an infection thought to be community acquired. No other seroconversions were observed. Only one mild transfusion reaction was observed. This study confirms the safety and efficacy of Monoclate-P. Post-marketing surveillance or nationally organized pharmaco-vigilance should be practiced more widely to enable identification of low-frequency side-effects of treatment.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2516.1996.tb00007.x
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  • 9
    Electronic Resource
    Electronic Resource
    Clinical experience of factor XI deficiency: the role of fresh frozen plasma and factor XI concentrate (1995)
    Oxford, UK : Blackwell Publishing Ltd
    Haemophilia 1 (1995), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary. Factor XI deficiency is a rare autosomally transmitted coagulopathy that is associated with a variable bleeding tendency. Recently there have been reports of thrombotic events following the administration of a virally inactivated factor XI concentrate (BPL) to factor XI deficient patients. We have therefore reviewed a single centre's experience of the use of factor XI concentrate over a 6-year period and compared this to our previous experience of either no treatment or treatment with fresh frozen plasma (FFP) in 103 patients.There were 156 procedures performed without haemo- static cover. The incidence of bleeding was greatest following tonsillectomy (71%) and dental extraction (Sl'h). There was a trend for bleeding complications to be associated with lower levels of factor XI but patients with all levels of factor XI suffered bleeding complica- tions. There were 38 procedures carried out under FFP cover, with only one patient suffering excessive bleeding and no serious complications.Factor XI concentrate was given to 25 patients to cover 45 episodes. There were no bleeding complications. Three patients suffered serious complications. One patient, with a previous history of cardiovascular disease, died of a myocardial infarction and a second had an ischaemic episode resulting in a %day hospital admission. These episodes both occurred on the same day as the factor XI infusion. A third patient suffered bilateral pulmonary emboli 7 weeks after a prolonged course of factor XI concentrate.These finding suggest that factor XI concentrate should be contraindicated in patients with a history of cardiovascular disease, when FFP should be used. Guide- lines for the use of factor XI concentrates should be revised, and work performed to establish the mechanism of these thrombotic events.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2516.1995.tb00080.x
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  • 10
    Electronic Resource
    Electronic Resource
    Successful use of recombinant factor VIIa in a patient with inhibitor secondary to severe factor XI deficiency (2002)
    Oxford UK : Blackwell Science Ltd
    Haemophilia 8 (2002), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Factor XI (FXI) inhibitors are a rare complication of inherited FXI deficiency. We report the successful use of recombinant factor VIIa (FVIIa) in a patient with a high-responding inhibitor undergoing cataract extraction. At the time of surgery there were limited available data on the optimal management of patients with FXI deficiency. A 62-year-old Ashkenazi Jewish woman had a lifelong history of excessive bleeding secondary to severe FXI deficiency (2 U dL−1), and received FXI concentrate (FXI:C) when she underwent a colposuspension procedure. She was subsequently diagnosed with a FXI inhibitor of 16 Bethesda units (BU) when she developed a poor response to FXI:C at the time of total hip replacement. Two months later she was admitted for cataract extraction. The FXI level was 〈 1 U dL−1 with an inhibitor titre of 48 BU. She received 90 μg kg−1 of FVIIa immediately preoperatively followed by continuous infusion at a rate of 20 μg kg−1 h−1 for 24 h. The cataract extraction was successful and there was no excess bleeding during surgery or in the postoperative period. Mutation analysis of the FXI gene showed that the patient was homozygous for the type II genotype [exon 5, Glu117→Ter]. The reason for the low prevalence of inhibitor formation in patients with FXI deficiency is unclear but may reflect a number of factors including reporting bias, the rarity of absent circulating FXI:C activity, and the infrequent use of FXI replacement therapy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.2002.00590.x
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