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OBJECTIVE: Enlargement of the ascending aorta is often combined with valvular, coronary, or other cardiac diseases. Reduction aortoplasty can be an optional therapy; however, indications regarding the diameter of aorta, the history of dilatation (poststenosis, bicuspid aortic valve), or the intraoperative management (wall excision, reduction suture, external reinforcement) are not established. METHODS: In a retrospective study between 1997 and 2005, we investigated 531 patients operated for aneurysm or ectasia of the ascending aorta (diameter: 45-76mm). Of these, in 50 patients, size-reducing ascending aortoplasty was performed. External reinforcement with a non-coated dacron prosthesis was added in order to stabilize the aortic wall. RESULTS: Aortoplasty was associated with aortic valve replacement in 47 cases (35 mechanical vs 12 biological), subvalvular myectomy in 29 cases, and CABG in 13 cases. The procedure was performed with low hospital mortality (2%) and a low postoperative morbidity. Computertomographic and echocardiographic diameters were significantly smaller after reduction (55.8+/-9mm down to 40.51+/-6.2mm (CT), p<0.002; 54.1+/-6.7mm preoperatively down to 38.7+/-7.1mm (echocardiography), p<0.002), with stable performance in long-term follow-up (mean follow-up time: 70 months). CONCLUSIONS: As demonstrated in this study, size reduction of the ascending aorta using aortoplasty with external reinforcement is a safe procedure with excellent long-term results. It is a therapeutic option in modern aortic surgery in patients with poststenotic dilatation of the aorta without impairment of the sinotubular junction of the aortic valve and root.  相似文献   
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Primary antibody deficiency syndromes are a rare group of disorders present at any age, with complex polygenic disorders. We report the forth case of polyglandular autoimmune syndrome (PAS) type IIIc worldwide with complex clinical features and no family history of endocrine disorders or primary immunodeficiencies. Our patient, a 44-year-old Caucasian female was diagnosed with PAS type IIIc due to the presence of autoimmune thyroiditis, autoimmune alopecia diffusa and primary ovarian insufficiency, associated with lymphoproliferative disease and primary antibody failure. Treatment included lifelong intravenous immunoglobulin, supplements and antibiotics. The clinical complexity and rare occurrence made it challenging to determine diagnosis and provide better treatment for the patient. The current case provides an insight of the challenges to determine primary antibody failure signs in the presence of PAS which will further help to determine diagnosis and therapeutic treatment for PAS patients.  相似文献   
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The endosomal Toll-like receptors (TLRs) TLR3, TLR7, TLR8 and TLR9 are important in sensing foreign nucleic acids encountered by phagocytes. Because TLR8 was initially thought to be non-functional in mice, less is known about TLR8 than the genetically and functionally related TLR7. Originally associated with the recognition of single-stranded RNA of viral origin, there is now evidence that human TLR8 is also able to sense bacterial RNA released within phagosomal vacuoles, inducing the production of both nuclear factor (NF)-κB-dependent cytokines and type I interferons (IFNs), such as IFN-β. The functions of TLR8 extend beyond the recognition of foreign pathogens and include cross-talk with other endosomal TLRs, a process that may also have a role in the generation of autoimmunity.  相似文献   
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We report our experience with duplex ultrasound in young patients with renal artery stenosis (RAS) or middle aortic syndrome (MAS) before and after surgery (1995 and 2009). Of 36 patients (mean age: 13 ± 7 y), 21 had RAS and 15 had MAS. For patients with RAS, the Vmax in the affected artery was 350 ± 111 cm/s before surgery and 145 ± 55 cm/s after surgery. The resistance index was 0.46 ± 0.1 in the post-stenotic kidney and increased to 0.60 ± 0.08 after revascularization. Determination of the flow profile in the iliac artery revealed triphasic flow. In individuals with MAS, Vmax in the aorta was 323 ± 98 and the resistance index in both kidneys was low, even in the absence of RAS. The flow profile in the iliac arteries was monophasic before surgery and became triphasic after surgery. Duplex ultrasound is useful for the evaluation of children and young adults both pre- and post-surgery. Duplex ultrasound criteria for RAS in adults appear to be applicable in children and young adults also. The diagnostic evaluation of suspected renal vascular disease should include assessment of the aorta and the flow profile in the iliac arteries, as this could help differentiate between aortic and isolated renal artery stenosis.  相似文献   
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Pain assessment and management in animals is still a new and growing field of research. Although it is still early days for pain research in birds, this article aims to provide information on the pain behaviours known so far, and describes current analgesic drugs and doses.  相似文献   
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