246 research outputs found
Hereditary angio-oedema
No full textSummaryHereditary angio-oedema is caused by a heterozygous deficiency of C1 inhibitor. This inhibitor regulates several inflammatory pathways, and patients with hereditary angio-oedema have intermittent cutaneous or mucosal swellings because of a failure to control local production of bradykinin. Swellings typically evolve in several hours and persist for a few days. In addition to orofacial angio-oedema, painless swellings affect peripheries, which causes disfigurement or interference with work and other activities of daily living. Angio-oedema affecting the gastrointestinal tract or abdominal viscera causes severe pain often with vomiting due to oedematous bowel obstruction. About 2% of swellings involve the larynx and can be fatal if untreated. About 50% of patients have laryngeal swellings that are potentially fatal despite prophylaxis. In this Seminar we review the clinical features, diagnosis, and management of hereditary angio-oedema, with specific emphasis on the new treatments available for acute swellings
Management of acute attacks of hereditary angioedema: potential role of icatibant
No full textHilary J LonghurstDepartment of Immunology, Barts and The London NHS Trust, London, UKAbstract: Icatibant (Firazyr®) is a novel subcutaneous treatment recently licensed in the European Union for acute hereditary angioedema. Hereditary angioedema, resulting from inherited partial C1 inhibitor deficiency, is a disabling condition characterized by intermittent episodes of bradykinin-mediated angioedema. Icatibant blocks bradykinin B2 receptors, attenutating the episode. Randomized double-blind, placebo-controlled trials of icatibant, showed significant superiority over oral tranexamic acid in 74 European patients and a trend to improvement in a similar US trial comparing icatibant with placebo in 55 patients. Outcomes for several endpoints did not reach significance in the US trial, perhaps because of low participant numbers and confounding factors: a further trial is planned. Open label studies have shown benefit in multiple treatments for attacks at all sites. Approximately 10% of patients require a second dose for re-emergent symptoms, usually 10 to 27 hours after the initial treatment. Its subcutaneous route of administration, good tolerability and novel mode of action make icatibant a promising addition to the limited repertoire of treatments for hereditary angioedema.Keywords: hereditary angioedema, bradykinin, icatibant, C1 inhibitor deficienc
Health ‘in queer street’: constituting sickness, sexualities and bodies in the spaces of lesbian health
No full textThis thesis explores lesbian health. The appeal of this project is its attention to the constitutions, resistances, and reproductions involved in the construction of ‘lesbian health’. ‘Lesbian health’ provides the context for exploring the meanings of health, sickness, sexuality, and bodies. The analysis takes place in three stages. It begins with a review of gay-positive studies of lesbian health. Second, is a discourse analysis of constructions of ‘health’, ‘illness’, and ‘sexuality’. It concludes with a critical analysis of the fluid and leaky constitutions of gendered sexualities and bodies.
I conduct a study with 17 self-identified lesbians, using open-ended interviews that cover a variety of topics in the areas of health practices and concerns. Feminist Foucauldian approaches inform the critical discourse analysis of the interviewee’s accounts. An examination of the co-constitution of discourses about ‘health’ and ‘sickness’ in relation to ‘lesbian’ sexualities reveals a binary relationship. That is, health for lesbians is constituted as overcoming the societal pressures and exclusions related to ‘being gay’, avoiding the implication that lesbian ‘health’ or ‘sickness’ may be inherent. I draw on Elizabeth Grosz's critical analysis of the corporeal as I examine lesbian bodies in the contexts of cervical screening and dental dams. In these health promotion contexts, ‘lesbian’ bodies are brought into being, but their presence is potentially threatening. Lesbian bodies are constituted, materially and discursively, as liminal, fluid, and abject. The consequence is that disclosure as ‘lesbian’ is self-regulated. Other consequences for cervical screening and safe sex promotion are explained by the leaky fluidity of bodies and places, which reinsert ‘lesbian’ into the hegemonic space and simultaneously render ‘lesbian’ external to such space.
I suggest that health practices, concerns, and promotion are ‘in queer street’, literally troubled by the unsettling of hegemonic assumptions. Focusing on the lived, sexed body in health research, using discursive and critical theoretical tools, may offer one more way of challenging assumptions about sexuality and gender, and raise questions in the practice and analysis of health that address diversity and difference
Long-term effects upon rituximab treatment of acquired angioedema due to C1-inhibitor deficiency.
No full textMisdiagnosis trends in patients with hereditary angioedema from the real-world clinical setting
No full textHereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) causes swelling in the skin and upper airways and pain in the abdomen because of mucosal swelling. C1-INH-HAE is frequently misdiagnosed, leading to delays in diagnosis, inadequate treatment, and unnecessary procedures
A randomised, placebo-controlled, double blind phase III study of the efficacy and safety of recombinant human C1 inhibitor for the treatment of acute attacks in patients with hereditary angioedema
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Optimum Use of Acute Treatments for Hereditary Angioedema: Evidence-Based Expert Consensus
No full textAcute treatment of hereditary angioedema due to C1 inhibitor deficiency has become available in the last 10 years and has greatly improved patients’ quality of life. Two plasma-derived C1 inhibitors (Berinert and Cinryze), a recombinant C1 inhibitor (Ruconest/Conestat alpha), a kallikrein inhibitor (Ecallantide), and a bradykinin B2 receptor inhibitor (Icatibant) are all effective. Durably good response is maintained over repeated treatments and several years. All currently available prophylactic agents are associated with breakthrough attacks, therefore an acute treatment plan is essential for every patient. Experience has shown that higher doses of C1 inhibitor than previously recommended may be desirable, although only recombinant C1 inhibitor has been subject to full dose–response evaluation. Treatment of early symptoms of an attack, with any licensed therapy, results in milder symptoms, more rapid resolution and shorter duration of attack, compared with later treatment. All therapies have been shown to be well-tolerated, with low risk of serious adverse events. Plasma-derived C1 inhibitors have a reassuring safety record regarding lack of transmission of virus or other infection. Thrombosis has been reported in association with plasma-derived C1 inhibitor in some case series. Ruconest was associated with anaphylaxis in a single rabbit-allergic volunteer, but no further anaphylaxis has been reported in those not allergic to rabbits despite, in a few cases, prior IgE sensitization to rabbit or milk protein. Icatibant is associated with high incidence of local reactions but not with systemic effects. Ecallantide may cause anaphylactoid reactions and is given under supervision. For children and pregnant women, plasma-derived C1 inhibitor has the best evidence of safety and currently remains first-line treatment
The faith and patience of departed saints, [electronic resource] : recommended to the imitation of surviving Christians. A sermon occasioned by the death of Mr Joseph Longhurst, ... who departed this life June 12th, 1769, in the sixty-ninth year of his age. ... By Samuel Wilton.
No full textHalf-title: 'A funeral sermon for Mr Joseph Longhurst'.'A funeral oration .. ' has a separate half-title.Electronic reproduction.English Short Title Catalog,Reproduction of original from British Library
HAE international home therapy consensus document
No full textHereditary angioedema (C1 inhibitor deficiency, HAE) is associated with intermittent swellings which are disabling and may be fatal. Effective treatments are available and these are most useful when given early in the course of the swelling. The requirement to attend a medical facility for parenteral treatment results in delays. Home therapy offers the possibility of earlier treatment and better symptom control, enabling patients to live more healthy, productive lives. This paper examines the evidence for patient-controlled home treatment of acute attacks ('self or assisted administration') and suggests a framework for patients and physicians interested in participating in home or self-administration programmes. It represents the opinion of the authors who have a wide range of expert experience in the management of HAE
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