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Biomedical subjects

G Dolan

Publications and source records attributed to G Dolan.

At least 19 recordsLinked to original sources
PubMed2006

Clinical implications of emerging pathogens in haemophilia: the variant Creutzfeldt-Jakob disease experience.

The impact of variant Creutzfeldt-Jakob disease (vCJD) on the clinical practice of haemophilia in the UK is coloured by the haemophilia community's experience of hepatitis C virus and human immunodeficiency virus (HIV) transmission via plasma-derived therapies in the 1980s, when the delay in recognizing and acting on the potential risks cost many patients their lives and left others to manage another chronic disease. This crisis prompted organisations such as the United Kingdom Haemophilia Centre Doctors' Organisation to advocate for the introduction of haemophilia therapies that would not be susceptible to contamination with blood-borne pathogens. After the identification of vCJD in 1996, a number of public health measures were taken in response to a government-sponsored vCJD risk assessment, and following reports of transfusion-transmission of vCJD, additional guidelines have been developed to prevent person-to-person transmission, some of which may impact the quality and availability of medical and surgical care. Variant CJD has had a significant negative effect on the UK haemophilia community, shaking patient confidence in the therapies they have received over the last 21 years, affecting the quality of care and creating the risk of stigmatizing the community as it was in the 1980s. As with HIV and vCJD, emerging blood-borne infectious agents will likely affect blood and blood-derived therapies well before we become aware of its presence. As a result, only therapies with the lowest level of risk should be used for care of patients with haemophilia.

Animals↗
PubMed2006

Management of acute bleeding in a patient with congenital afibrinogenaemia.

Congenital afibrinogenaemia is a rare bleeding disorder characterized by absence of fibrinogen and varying bleeding tendency. Treatment with fibrinogen concentrates is considered to be the best choice for afibrinogenaemic patients who experience bleeding. We report the case of a 22-year-old Greek patient who presented with large muscular haematomas and was treated with fibrinogen concentrates. The efficacy of this treatment and the problems that arose during his hospitalization are being discussed.

Adult↗
PubMed2006

Spontaneous superficial venous thrombophlebitis: does it increase risk for thromboembolism? A historic follow-up study in primary care.

OBJECTIVE: To determine the risk of arterial and venous complications after a spontaneous superficial venous thrombophlebitis (SVTP) in the leg in a general practice population. STUDY DESIGN: Retrospective cohort study (LOE: 2b [CEBM]). Exposure consisted of the diagnosis of SVTP of the lower limbs on an index date. The exposed cohort was compared with an (unexposed) cohort of practice-, age-, and sex-matched controls without SVTP. POPULATION: Patients with spontaneous SVTP in the leg were identified through diagnostic coding in the medical registers of 40,013 patients, enlisted with 5 health centers in Amsterdam, The Netherlands. OUTCOMES: Primary outcomes were deep venous thrombosis (DVT), pulmonary embolism (PE), acute coronary events, or ischemic stroke over a 6-month follow-up period. Odds ratios (OR) were used to quantify the associations between SVTP and outcome events. RESULTS: No statistically significant odds ratios were found for PE, coronary events or stroke. DVT was the only primary outcome to show a significant relationship. DVT occurred in 2.7% of all SVTP patients as compared with 0.2% in the controls (OR=10.2; 95% confidence interval [CI], 2.0-51.6). When controlling for prior history of DVT, the OR decreased to 7.1 and the confidence interval crossed 1.0 (95% CI, 0.9-65.6). DISCUSSION: Spontaneous SVTP in the leg is a risk factor for DVT, but is less predictive in patients with prior DVT. Although effective treatments for the prevention of DVT are available, the absolute risk is too low to advocate prophylaxis in a general practice population. More research on prophylaxis is needed to stratify these patients at risk.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed2005

Mutation analysis in 51 patients with haemophilia A: report of 10 novel mutations and correlations between genotype and clinical phenotype.

We report the results of genetic analysis on a series of 51 patients attending this Haemophilia Comprehensive Care Centre. The most common cause of severe haemophilia A--the factor VIII intron 22 inversion was detected in eight families and the factor VIII intron 1 inversion in three families. Mutation analysis was carried out on the remaining patients by nucleotide sequencing of genomic DNA after screening with conformation-sensitive gel electrophoresis (CSGE) or denaturing high-performance liquid chromatography (dHPLC). A total of 27 different FVIII non-inversion mutations were detected. Severe haemophilia was associated with 12 null mutations (six nonsense, six frameshift) and four missense mutations. A further 11 different missense mutations were associated with moderate or mild disease. To our knowledge, six null mutations [1950del 4(tttg), 3270-75insA, 4416del 10, 6735-38delA, W1029X, Y1792X] and four missense mutations (E1682K, M1947V, P2048L, P2143L) have not been previously published. Each novel missense mutation occurred at a highly conserved residue, no other candidate mutation was detected on screening the entire coding region of the FVIII gene and they were not detected in a screen of individuals without haemophilia A. The genotype-phenotype correlations of the FVIII mutations detected will be discussed.

Base Sequence↗
PubMed2005

A framework for genetic service provision for haemophilia and other inherited bleeding disorders.

This framework document offers guidance to patients, doctors, nurses, laboratory scientists, funders and hospitals on the provision of clinical and laboratory genetic services for haemophilia. With recent advances in molecular laboratory techniques it is now possible to give the vast majority of individual patients and family members very reliable genetic information. To enable these genetic data to be used for both the optimal treatment of patients with inherited bleeding disorders and for appropriate reproductive decisions in carriers, there needs to be a clear and robust framework for systematically acquiring the necessary clinical, personal, family and laboratory information upon which decisions can be made. This document provides guidance on the range and standards of clinical and laboratory genetic services which should be offered to patients and their families. Included are arrangements for genetic counselling and testing (including consent and confidentially issues), management of early pregnancy, standards for laboratory genetic services, as well as advice on data storage, security and retrieval.

Adult↗
PubMed2005

ESOS: a European study on the orthopaedic status of patients with haemophilia and inhibitors.

Published studies highlight the paucity of data relating to orthopaedic health and quality of life in haemophilia patients with inhibitors. A European Study on the Orthopaedic Status of Patients with Haemophilia and Inhibitors (ESOS) has been set up to address this lack of data. This cross-sectional retrospective study aims to enrol 400 patients from 50 sites in nine European countries. The primary outcome measure of the study is the burden of orthopaedic complications in patients with severe haemophilia and inhibitors. The data that will be generated should demonstrate the high level of arthropathy in haemophilia patients with inhibitors and show the need to focus on improving orthopaedic health in this rare, but particularly vulnerable, patient population.

Blood Coagulation Factor Inhibitors↗
PubMed2004

The rare coagulation disorders--review with guidelines for management from the United Kingdom Haemophilia Centre Doctors' Organisation.

The rare coagulation disorders are heritable abnormalities of haemostasis that may present significant difficulties in diagnosis and management. This review summarizes the current literature for disorders of fibrinogen, and deficiencies of prothrombin, factor V, FV + VIII, FVII, FX, the combined vitamin K-dependent factors, FXI and FXIII. Based on both collective clinical experience and the literature, guidelines for management of bleeding complications are suggested with specific advice for surgery, spontaneous bleeding, management of pregnancy and the neonate. We have chosen to include a section on Ehlers-Danlos Syndrome because haematologists may be consulted about bleeding manifestations in such patients.

Afibrinogenemia↗
PubMed2002

Use of ultramolecular potencies of allergen to treat asthmatic people allergic to house dust mite: double blind randomised controlled clinical trial.

OBJECTIVE: To evaluate the efficacy of homoeopathic immunotherapy on lung function and respiratory symptoms in asthmatic people allergic to house dust mite. DESIGN: Double blind randomised controlled trial. SETTING: 38 general practices in Hampshire and Dorset. PARTICIPANTS: 242 people with asthma and positive results to skin prick test for house dust mite; 202 completed clinic based assessments, and 186 completed diary based assessments. INTERVENTION: After a four week baseline assessment, participants were randomised to receive oral homoeopathic immunotherapy or placebo and then assessed over 16 weeks with three clinic visits and diary assessments every other week. OUTCOME MEASURE: Clinic based assessments: forced expiratory volume in one second (FEV(1)), quality of life, and mood. DIARY BASED ASSESSMENTS: morning and evening peak expiratory flow, visual analogue scale of severity of asthma, quality of life, and daily mood. RESULTS: There was no difference in most outcomes between placebo and homoeopathic immunotherapy. There was a different pattern of change over the trial for three of the diary assessments: morning peak expiratory flow (P=0.025), visual analogue scale (P=0.017), and mood (P=0.035). At week three there was significant deterioration for visual analogue scale (P=0.047) and mood (P=0.013) in the homoeopathic immunotherapy group compared with the placebo group. Any improvement in participants' asthma was independent of belief in complementary medicine. CONCLUSION: Homoeopathic immunotherapy is not effective in the treatment of patients with asthma. The different patterns of change between homoeopathic immunotherapy and placebo over the course of the study are unexplained.

Adult↗
PubMed2001

Vigam-S, a solvent/detergent-treated intravenous immunoglobulin, in idiopathic thrombocytopenic purpura.

The efficacy of Vigam-S, a highly purified intravenous immunoglobulin, was investigated by an open, noncomparative study in 20 adults with chronic idiopathic thrombocytopenic purpura (ITP). Fifteen patients responded to the initial 3-day infusion of 0.4 g kg(-1) day(-1) by exhibiting an incremental increase in platelet count of >or= 30 x 10(9) L(-1), in eight of whom platelet count normalized (> 150 x 10(9) L(-1)). The peak platelet count for responders on day 4 was 163 x 10(9) L(-1) (baseline = 18 x 10(9) L(-1)). No benefit was derived from an extra 2 days infusion in nonresponders. Further treatment (either a single 0.8 g kg(-1) dose or another 3-day infusion) given to responders when platelet counts fell below 30 x 10(9) L(-1) was effective on eight of 14 occasions. Increases in total serum IgG concentration (to a mean peak of 25.3 g L(-1)) were not correlated with platelet response. There was no evidence of seroconversion to virus markers, or of alteration in renal function, following Vigam-S infusion. Most adverse events were mild and transient; however, three patients had severe headache and vomiting (possible aseptic meningitis syndrome) and one had marked superficial thrombophlebitis. Therefore Vigam-S provides effective and well tolerated therapy in the management of adults with ITP although individual patient response remains difficult to predict.

Adolescent↗
PubMed2001

Reflecting on the use of student portfolios.

The use of portfolios in nursing has increased during recent years. We reflect upon the design and implementation of a portfolio for pre-registration nursing students in the School of Care Sciences(1)at the University of Glamorgan. The portfolio was introduced as the result of the need to address the theory-practice divide and to provide nurse students with the skills to maintain a professional profile upon registration. We also discuss other potential benefits associated with using the portfolio. These include the use of a portfolio as a focus for discussion between the student and their preceptor or personal tutor, a vehicle for the development of reflective skills and a means of assessment. We acknowledge that along with benefits, there are a number of potential problems and limitations in their use, for example, an increase in the workload for the student, preceptor and personal tutor. We include preliminary findings of an evaluation of our portfolio, revealing that both students and staff value the introduction of the portfolio, although currently it is not a high priority in terms of the course as a whole.

Documentation↗
PubMed2001

F X Nottingham and F X Taunton. Two novel mutations in factor X resulting in loss of functional activity and an interpretation using molecular modelling.

We report two novel mutations in the Factor X gene which result in a bleeding tendency in two unrelated Caucasian families. Although the mutations occur at adjacent codons in exon 8 and result in reduced functional activity with normal antigen levels, the patterns of inheritance appear to be quite distinct. Factor X Nottingham (alanine 404 threonine) appears to be associated with an autosomal recessive pattern of inheritance. In contrast, Factor X Taunton (arginine 405 glycine) results in a mode of inheritance consistent with an autosomal dominant pattern, all five of the heterozygotes in this family being clinically affected. Molecular modelling studies suggest that, in the case of Factor X Nottingham, a drastic conformational change causes major unfolding of the protein. For Factor X Taunton, less extreme conformational changes occur causing loss of functional activity such that substrate binding sites might be maintained. It is proposed that competition with wild type for substrate binding could occur leading to a dominant negative effect.

Adolescent↗