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- Agostoni, Angelo, et al.
(författare)
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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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Ingår i: Journal of Allergy and Clinical Immunology. - : Elsevier BV. - 1097-6825 .- 0091-6749. ; 114:3 Suppl, s. 51-131
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Tidskriftsartikel (refereegranskat)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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| 2. |
- Amundsen, T, et al.
(författare)
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A closed-chest pulmonary artery occlusion/reperfusion model in the pig: detection of experimental pulmonary embolism with MR angiography and perfusion MR imaging
- 2000
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Ingår i: Investigative Radiology. - 0020-9996. ; 35:5, s. 295-303
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Tidskriftsartikel (refereegranskat)abstract
- RATIONALE AND OBJECTIVES: To establish a pig model suitable for imitating pulmonary emboli to facilitate research in the diagnosis of pulmonary embolism. METHODS: Thirteen animals were anesthetized, mechanically ventilated, and subjected to pulmonary artery catheterization initiated from the right external jugular vein. With the use of a Swan-Ganz catheter, repetitive occlusion/reperfusion maneuvers were done at different locations of the pulmonary arterial tree. Conventional pulmonary angiography, MR angiography, and perfusion MR imaging were performed. RESULTS: The model remained hemodynamically stable throughout the 13 experiments, without any significant difference between the blood pressure measurements at the start and at the end of the right-heart and pulmonary artery catheterizations. In each of the nine animal experiments that investigated MR imaging, four of four using perfusion MR imaging (proximal and distal occlusions) and five of five using MR angiography (larger pulmonary artery occlusions), all repeated pulmonary artery occlusions were successfully performed (reproducibility of 100%). CONCLUSIONS: The closed-chest pulmonary artery occlusion/reperfusion model in the pig allowed repetitive, controlled imitations of pulmonary emboli at different levels of the pulmonary artery in the same experiment. MR angiography and perfusion MR imaging were adequate to detect the pulmonary artery occlusions and the nonperfused lung regions, respectively. The model may be a helpful tool for future research in this field.
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