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- Agostoni, Angelo, et al.
(author)
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Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond
- 2004
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In: Journal of Allergy and Clinical Immunology. - : Elsevier BV. - 1097-6825 .- 0091-6749. ; 114:3 Suppl, s. 51-131
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Journal article (peer-reviewed)abstract
- Hereditary angioedema (HAE), a rare but life-threatening condition, manifests as acute attacks of facial, laryngeal, genital, or peripheral swelling or abdominal pain secondary to intra-abdominal edema. Resulting from mutations affecting C1 esterase inhibitor (C1-INH), inhibitor of the first complement system component, attacks are not histamine-mediated and do not respond to antihistamines or corticosteroids. Low awareness and resemblance to other disorders often delay diagnosis; despite availability of C1-INH replacement in some countries, no approved, safe acute attack therapy exists in the United States. The biennial C1 Esterase Inhibitor Deficiency Workshops resulted from a European initiative for better knowledge and treatment of HAE and related diseases. This supplement contains work presented at the third workshop and expanded content toward a definitive picture of angioedema in the absence of allergy. Most notably, it includes cumulative genetic investigations; multinational laboratory diagnosis recommendations; current pathogenesis hypotheses; suggested prophylaxis and acute attack treatment, including home treatment; future treatment options; and analysis of patient subpopulations, including pediatric patients and patients whose angioedema worsened during pregnancy or hormone administration. Causes and management of acquired angioedema and a new type of angioedema with normal C1-INH are also discussed. Collaborative patient and physician efforts, crucial in rare diseases, are emphasized. This supplement seeks to raise awareness and aid diagnosis of HAE, optimize treatment for all patients, and provide a platform for further research in this rare, partially understood disorder.
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- Gennery, A. R., et al.
(author)
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Treatment of CD40 ligand deficiency by hematopoietic stem cell transplantation: a survey of the European experience, 1993-2002
- 2004
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In: Blood. - : American Society of Hematology. - 0006-4971 .- 1528-0020. ; 103:3, s. 1152-7
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Journal article (peer-reviewed)abstract
- CD40 ligand (CD40L) deficiency causes recurrent sinopulmonary infection, Pneumocystis carinii pneumonia, and Cryptosporidium parvum infection. Approximately 40% to 50% of patients survive to the third decade: long-term survival is unclear. Hematopoietic stem cell transplantation (HSCT) is curative. We present a retrospective analysis of 38 European patients undergoing HSCT for CD40L deficiency in 8 European countries between 1993 and 2002. Donor stem cell source included 14 HLA-identical siblings, 22 unrelated donors, and 2 phenotypically matched parental stem cells (12 T-cell depleted). Of the patients, 34 engrafted and 26 (68%) survived; 3 had autologous reconstitution, 22 (58%) were cured, and 1 engrafted but has poor T-cell immune reconstitution. There were 18 evaluated patients who responded to vaccination. Of the patients, 12 (32%) died from infection-related complications, with severe cryptosporidiosis in 6. Grades 2 to 4 graft-versus-host disease (GvHD) associated with infection occurred in 6 of 12 fatal cases. HSCT cured 58% of patients, 72% of those without hepatic disease. Early T-cell function following whole marrow HSCT may limit cryptosporidial disease, but survival was similar after T-cell-depleted HSCT. Preexisting lung damage was the most important adverse risk factor. Further studies will determine optimal timing and type of HSCT.
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- Prinzleve, M., et al.
(author)
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Cocaine use in Europe - a multi-centre study : Patterns of use in different groups
- 2004
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In: European Addiction Research. - 1022-6877 .- 1421-9891. ; 10:4, s. 147-155
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Journal article (peer-reviewed)abstract
- AIM: The study investigates patterns of cocaine powder and crack cocaine use of different groups in nine European cities. DESIGN, SETTING, PARTICIPANTS: Multi-centre cross-sectional study conducted in Barcelona, Budapest, Dublin, Hamburg, London, Paris, Rome, Vienna, and Zurich. Data were collected by structured face-to-face interviews. The sample comprises 1,855 cocaine users out of three subgroups: 632 cocaine users in addiction treatment, mainly maintenance treatment; 615 socially marginalized cocaine users not in treatment, and 608 socially integrated cocaine users not in treatment. MEASUREMENTS: Use of cocaine powder, crack cocaine and other substances in the last 30 days, routes of administration, and lifetime use of cocaine powder and crack cocaine. Findings: The marginalized group showed the highest intensity of cocaine use, the highest intensity of heroin use and of multiple substance use. 95% of the integrated group snorted cocaine powder, while in the two other groups, injecting was quite prevalent, but with huge differences between the cities. 96% of all participants had used at least one other substance in addition to cocaine in the last 30 days. CONCLUSIONS: The use of cocaine powder and crack cocaine varies widely between different groups and between cities. Nonetheless, multiple substance use is the predominating pattern of cocaine use, and the different routes of administration have to be taken into account.
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- Bechta, Sevostian, et al.
(author)
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New experimental results on the interaction of molten corium with reactor vessel steel
- 2004
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In: Proceedings of the 2004 International Congress on Advances in Nuclear Power Plants, ICAPP'04. - : American Nuclear Society. - 0894486802 ; , s. 1072-1081
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Conference paper (peer-reviewed)abstract
- In order to justify the concept of in-vessel core melt retention, it is necessary to understand the thermal and physico-chemical phenomena. Especially the interaction of the molten pool with the reactor vessel during outside cooling needs to be understood. These phenomena are very complex, in particular, where interactions with the oxidic melt are concerned. In the early stages of the retention process, the oxidic corium and the vessel steel interact under the conditions of low oxygen potential in the melt. These conditions can be simulated by a molten corium having the composition UO2/ZrO 2Zr, where the degree of Zr-oxidation is in the range between 30 % (C-30) and 100 % (C-100). Corresponding experiments with prototypic melts at low oxygen potentials are being performed in the ISTC METCOR project 2nd phase. These are: MC 5 of corium composition 71w%UO2-29w%ZrO 2 (C-100) in neutral atmosphere (argon), MC 6 of corium composition 76w%UO2-9w%ZrO2-15w%Zr (C∼30), also in argon. In test MC 5, the interaction of molten C-100 corium with a water-cooled steel specimen was studied for the following maximum temperatures at the specimen surface: 1075°C, 1180°C, 1315°C and 1435°C. The total duration of the experiment was ∼ 36 hours. The MC5 test serves as a reference test for determining the characteristics of the interaction between oxidic melt and steel specimen under the conditions of minimum chemical interaction potential. To investigate the effect of substoichiometry, test MC 6 was then performed with suboxidized molten corium C∼30. The maximum surface temperature of the cooled steel specimen was held at ∼ 1400°C. The test duration was ∼ 10 hours. The ablation phenomena were found to differ significantly from those observed both in the reference test, as well as in former tests with oxidized melts, as they involved the formation of a low-melting metallic phase at the interface which contains iron, zirconium and uranium. The paper summarizes the results of the experiments and of the performed posttest analysis for tests MC 5 and MC 6.
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- Berntorp, Erik, et al.
(author)
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Consensus perspectives on prophylactic therapy for haemophilia: summary statement.
- 2003
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In: Haemophilia. - : Wiley. - 1351-8216. ; 9:Suppl 1, s. 41278-41278
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Journal article (peer-reviewed)abstract
- Participants in an international conference on prophylactic therapy for severe haemophilia developed a consensus summary of the findings and conclusions of the conference. In the consensus, participants agreed upon revised definitions for primary and secondary prophylaxis and also made recommendations concerning the need for an international system of pharmacovigilance. Considerations on starting prophylaxis, monitoring outcomes, and individualizing treatment regimens were discussed. Several research questions were identified as needing further investigation, including when to start and when to stop prophylaxis, optimal dosing and dose interval, and methods for assessment of long-term treatment effects. Such studies should include carefully defined cohorts, validated orthopaedic and quality-of-life assessment instruments, and cost-benefit analyses.
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