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  • 1
    ISSN: 1365-2044
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: In this study cerebral oxygenation was measured using the NIRO 300 and the INVOS 5100 spectrophotometers in 10 healthy adult volunteers, exposed to varying degrees of hyperoxia and hypoxia. The results showed similar baseline values for tissue oxygenation index and regional cerebral oxygen saturation with mean (SD) values being 64.9% (5.1) and 62.3% (6.0), respectively. The overall bias was –2.1%, with the INVOS 5100 under-reading cerebral oxygenation compared to the NIRO 300, with limits of agreement of ±14.7%. Both monitors demonstrated similar changes in response to hyperoxia and hypocapnia (coefficient of variance for FIo2 0.45 = 10.0%, FIo2 1.0 = 10.1%, hypocapnia = 14.5%). The reasons for the bias and variability may relate to differences in the methodological approaches of the two monitors. The correlation between the monitors in response to changes in cerebral oxygenation implies that they may be useful as trend monitors in clinical practice.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  We present the clinical, biochemical and genomic findings of a family with congenital factor XIII (FXIII) deficiency. Congenital FXIII deficiency is a very rare autosomal recessive bleeding disorder, characterized by umbilical cord bleeding at birth and spontaneous intracranial haemorrhage. Routine clotting tests are normal, which may delay the diagnosis, leading to an increased chance of severe sequelae. The propositus and her brother, known with haemorrhagic diathesis, were found to be compound heterozygous with a known missense mutation (1050 G → T transversion in exon 7, Val316Phe substitution) and a novel mutation 889 G → A in exon 6, which predicts a Gly262Glu substitution. As these mutations were known in the family, DNA obtained from cord blood of the youngest sister was analysed for mutations in exons 6 and 7 only. We postulate that the diagnosis was facilitated by determining the two different mutations in the genotype of this family. The analysis showed that she was heterozygous for the exon 7 mutation. Hence, she was not at risk of experiencing haemorrhagic diathesis. This diagnosis avoided the administration of FXIII concentrate to the newborn.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 10 (2004), S. 0 
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  This study was conducted to evaluate the effect of an unaware carriership on the delay in diagnosis of haemophilia and the resulting effect of this delay on morbidity. Information on 73 haemophilia patients (〈18 years) and their mothers was gathered from data of patients’ medical records and completed by interviews with the parent(s). Although a positive family history was present in 52 gravidae, 16 of them (31%) were not aware of their carrier status at moment of delivery. Fifteen of these 16 unaware carriers, were carriers of a non-severe form of haemophilia. In mothers who were unaware of carriership for haemophilia instrumental delivery occurred more frequently than in mothers who knew they were carriers. This is disquieting since instrumental delivery poses a significant risk (relative risk: 17.8, 95% CI: 4.0–78.4) for intra- or extracranial bleedings in newborn haemophiliacs in comparison to spontaneous deliveries or caesarean sections. In 83% of the patients with a positive family history, diagnosis was established before the first bleeding episode. Patients diagnosed by bleedings presented more often with iatrogenic bleedings (38%) then patients who were diagnosed because of a positive family history (9%) (P 〈 0.05). In comparison to previous studies, more patients had a positive family history and in more haemophiliacs with a positive family history diagnosis was established before the first bleeding episode. Although this reflects the yield of improved diagnostic methods, further reduction of iatrogenic bleeding is possible by intensifying counselling of mild haemophilia carriers.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  AAFACT®, a monoclonal purified, solvent/detergent treated human plasma-derived coagulation factor VIII concentrate obtained from plasma of voluntary, non-remunerated blood donors, is manufactured and marketed in the Netherlands by Sanquin Plasma Products since 1995. In a postmarketing surveillance study, 70 previously treated haemophilia A patients were included (73% severe, 14% moderate and 13% mild haemophilia A). Most of these patients were followed during 4 years for the appearance of adverse events, possible transmissions of blood-borne viruses and the occurrence of antibodies against FVIII. The efficacy of treatment was determined in each patient by the in vivo recovery of FVIII. During this study, only six adverse events, possibly related to the use of AAFACT®, were reported. None of these were indicated as serious. Transmissions of HIV, HAV, HBV and HCV in the seronegative patients have not been observed. In none of the patients, inhibitors to FVIII were detected. The in vivo recovery of FVIII during this study was not different from the in vivo recovery observed in eight patients during the preregistration study. There was a correlation of in vivo recovery with age and body weight. From these results, we conclude that the clinical usage of this human plasma-derived FVIII product is efficient and safe.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: A Dutch nation-wide study on young haemophilia patients, whose former treatment placed them at risk for HIV infection, was done to examine the effect of HIV testing, HIV status, disclosure of HIV status and the child's experiences with disease-related information on emotional adjustment. In the Netherlands HIV tests are not routinely performed for paediatric haemophilia patients, but the number of HIV-infected children is estimated to be low (8%). Seventy-two boys with haemophilia, five HIV positive, 51 HIV negative and 16 not tested, and their mothers were interviewed and administered standardized questionnaires. Multiple regression analyses showed no differences in anxiety and depression between HIV-positive, HIV-negative or untested boys, nor between children who were informed or not informed about their HIV status. Conversely, children who experienced more difficulties to obtain information about the their disease were significantly more anxious and tended to be more depressed. No significant differences in emotional distress were found between the patients and a standardization sample of healthy peers. The data provide evidence that children and adolescents with haemophilia cope effectively with their disease and the emotional impact of the HIV threat. There seems to be no reason to protect children from testing or knowing their HIV status. In the absence of compelling medical reasons, however, health care workers should not forcefully advocate testing or early disclosure. Distinct disease and treatment-related factors as well as social factors modify disclosure practices.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  We evaluated inhibitor formation in a group of patients with mild haemophilia A caused by an Arg593 to Cys mutation. A remarkably high cumulative inhibitor incidence of 14% over 22 years was observed. Three of 49 patients developed transient, low-titre inhibitors, which remained below 2.0 BU mL−1. Four patients with an Arg593 to Cys mutation developed high-titre inhibitors (〉5.0 BU mL−1). Three of these patients have been described previously. In this study, we characterized inhibitory antibodies in a fourth patient with high-titre inhibitors. Epitope mapping studies revealed that antibodies were predominantly directed to the A2 domain of factor VIII. We addressed the role of human leucocyte antigen (HLA) class II alleles in inhibitor development in patients with an Arg593 to Cys mutation by HLA genotyping. In the group of inhibitor patients raised frequencies of HLA-DRB1*01 and HLA-DQB1*05 were observed that did not reached statistical significance. Our data suggest that inhibitor development in mild haemophilia A patients with an Arg593 to Cys mutation is not linked to HLA class II profile.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science Ltd
    Journal of oral rehabilitation 32 (2005), S. 0 
    ISSN: 1365-2842
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: summary  The purpose of this in vitro study was to evaluate the surface and resin–dentine interface characteristics of permanent tooth dentine cut with diamond or carbide burs and treated with phosphoric acid (PA) or an acidic conditioner. Labial surfaces of permanent incisors were prepared into dentine with high-speed carbide or diamond burs and divided into two halves. Phosphoric acid 36% was applied on one half and non-rinse conditioner (NRC) was applied on the other half. Ten randomly selected scanning electron microscopy (SEM) fields from each specimen (n = 15) were evaluated. Occlusal surfaces of third molars were divided in two halves for evaluation of the resin–dentine interface. The halves were randomly assigned to one of each conditioner and restored with Prime & Bond NT/Spectrum. Ten specimens were analysed by SEM to evaluate hybrid layer formation and interfacial seal. We observed that surfaces prepared with carbide bur presented less residual smear plugs (P 〈 0·05) than surfaces prepared with diamond burs. Surfaces conditioned with NRC, which is a smear layer modifier, presented more residual smear plugs than surfaces conditioned with PA (P 〈 0·05). Treatment with PA resulted in more sealed interfaces than specimens treated with NRC. Within the limitations of this study the results showed that carbide burs leave a surface that is more conducive to bonding than diamond burs.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science Ltd
    Anaesthesia 55 (2000), S. 0 
    ISSN: 1365-2044
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 9
    Publication Date: 2016-05-26
    Description: Background The Stroke Impact Scale (SIS) is a stroke-specific, quality of life measure recommended for research and clinical practice. Completion rates are suboptimal and could relate to test burden. We derived and validated a short form SIS (SF-SIS). Methods and Results We examined data from the Virtual International Stroke Trial Archive, generating derivation and validation populations. We derived an SF-SIS by selecting 1 item per domain of SIS, choosing items most highly correlated with total domain score. Our validation described agreement of SF-SIS with original SIS and the SIS-16 and correlation with Barthel Index, modified Rankin Scale, National Institutes of Health Stroke Scale, and Euro-QoL 5 dimensions visual analog scales. We assessed discriminative validity (associations between SF-SIS and factors known to influence outcome [age, physiological parameters, and comorbidity]). We assessed face validity and acceptability by sharing the SF-SIS with a focus group of stroke survivors and multidisciplinary stroke healthcare staff. From 5549 acute study patients (mean age 68.5 [SD 13] years, mean SIS 64 [SD 32]) and 332 rehabilitation patients (mean age 65.7 [SD 11] years, mean SIS 61 [SD 11]), we derived an 8-item SF-SIS that demonstrated good agreement with original SIS and good correlation with our chosen functional and quality of life measures (all 〉0.70, P 〈0.0001). Significant associations were seen with our chosen predictors of stroke outcome in the acute group ( P 〈0.0001). The focus group agreed with the choice of items for SF-SIS across 7 of 8 domains . Conclusions Using multiple, complementary methods, we have derived an SF-SIS and demonstrated content, convergent, and discriminant validity. This shortened SIS should allow collection of robust quality of life data with less associated test burden.
    Electronic ISSN: 2047-9980
    Topics: Medicine
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  • 10
    Publication Date: 2013-08-28
    Description: Background and Purpose— The Stroke Impact Scale (SIS) covers 8 dimensions and a composite disability score. This study evaluates the SIS in the UK context, and develops a single index and an 8-item short form. Methods— Patients with a diagnosis of stroke were recruited through general practices in London and the North-West of England. Patients completed the SIS and the EQ-5D. Results— Internal consistency of the SIS dimensions and the disability score ranged from α 0.86 to 0.95. Complete data were available on 73 questionnaires (48.34%). Factor analysis suggested the 8 domains could be aggregated into a single index. A short-form SIS (SF-SIS) index was created by summing 1 item per dimension. Selected items were those that most highly correlated with their respective domain score ( ranged from 0.77–0.94, P 〈0.001). The SF-SIS index scores were highly correlated with those gained from the parent form (=0.98; P 〈0.001). The correlation of the SIS index and SF-SIS index with the EQ-5D was identical (=0.83; P 〈0.001). The disability score, whether scored from the dimensions of the SIS, or relevant items on the SF-SIS, were highly correlated (=0.97; P 〈0.001). Conclusions— The SIS covers aspects of health, which are of importance to stroke patients, and the dimensions were found to have high levels of internal consistency in the UK context. The amount of incomplete data suggests that the length of the questionnaire may present a substantial patient burden. In comparison to the parent form the SF-SIS can accurately provide the disability score and overall index score with considerable brevity.
    Keywords: Health policy and outcome research, Behavioral/psychosocial - stroke
    Print ISSN: 0039-2499
    Electronic ISSN: 1524-4628
    Topics: Medicine
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