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Woon H. Chong Biplab K. Saha Scott Beegle 《The American journal of the medical sciences》2021,361(4):526-533
Antisynthetase syndrome (AS) is a rare disease that affects patients with inflammatory myopathies such as polymyositis (PM) and dermatomyositis (DM). In patients with AS, up to 95% of patients develop antisynthetase syndrome-associated interstitial lung disease (AS-ILD). Although AS-ILD commonly occurs in patients with a well-established diagnosis of AS, it can be the first or only manifestation of an occult AS. The frequency of interstitial lung disease (ILD), myopathy, and skin involvement are often dependent on the type of myositis-specific antibodies present. AS-ILD patients who are positive for both anti-Jo-1 and anti-SSA/RO-52 autoantibodies often present with a severe degree of lung restriction on pulmonary function tests and radiologic imaging with an inadequate response toward immunosuppressive therapies. We describe a 65-year-old woman who presents with chronic dyspnea. She was initially diagnosed with corticosteroid-resistant cryptogenic organizing pneumonia based on the radiological findings on her CT chest. Her symptoms did not improve, and she suffered from intolerable corticosteroid-related side effects. Reviews of systems were positive for arthritis and Raynaud's phenomenon. She was found to have elevated inflammatory markers and autoantibodies such as anti-Jo-1, anti-RO-52, and anti-SSA. A diagnosis of AS-ILD resistant to corticosteroid therapy was made. Her lung function improved with combination therapy of mycophenolate and rituximab. Our case highlights that a detailed history and physical exam, compatible radiologic imaging, and autoantibodies are essential for the diagnosis of AS-ILD. 相似文献
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The effects of manganese chloride (MnCl2) and verapamil on automaticity of digitlazied Purkinje fibers were studied using conventional microelectrode techniques. The stduied wer made in isolated, spontaneously beating Purkinje prearations. Quabain alone consistently increased the automatic rate, whereas no such increase was observed when the preparations were superfused with a mixture of ouabain adn MnCl2. MnCl2 was also shown to be effective is suppressing the enhanced automaticity induced by ouabain. Mncl2 alone did not have a significant effect on the spontaneous rate of Purkinje fibers. The effect of verapamil was similar to that of MnCl2 in preventing and suppressing the ouabain-induced increase in automaticity. MnCl2 and verapamil have been shown to inhibt tha slow inward calcium current of cardiac fibers. The results therefore suggest that an inward calcium ion current may play a role in the development of digitalis-induced increase in the stope of phase 4 depolarization in Purkinje fibers. 相似文献
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There is suggestive evidence that bundle branch re-entry occurs in man in response to premature right ventricular stimulation. Demonstration of the activation sequence during re-entrant excitation in the in vivo dog was accomplished by placing recording electrodes on the major portions of the specialized conduction system. A temporary right heart bypass was utilized to place two or more electrodes on both right and left bundle branches and place electrodes on the His bundle and on the left and right ventricular endocardium. Premature excitation of the right ventricle was found not to retrogradely activate the right bundle but was able to cause slow right to left myocardial activation that resulted in retrograde activation within the left bundle branch. Retrograde conduction in the left bundle caused activation of the His bundle and the proximal right bundle. Activation of the right bundle resulted in antegrade conduction of the impulse across the site of previous conduction block and re-excitation of the right ventricle, to complete the re-entrant circuit. This type of re-entry, utilizing the bundle branches, was demonstrated in 19 dogs. This re-entry circuit was found to be facilitated by shortening of the right ventricular refractory period by local epicardial warming and was abolished by interruption of conduction in the right bundle by anodal blocking current applied to the right bundle. The sites of slow conduction, site of unidirectional block, and pathways of conduction were demonstrated. The validity of the concept of re-entry occurring within the specialized conduction system is substantiated. 相似文献
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Polytetrafluoroethylene grafts in the rapid reconstruction of acute contaminated peripheral vascular injuries 总被引:2,自引:0,他引:2
Conventional wisdom dictates that autogenous tissue interposition be used in contaminated wounds when direct vascular repair is not feasible. Although there are few reports of successful use of PTFE grafts in grossly contaminated wounds, doubt still exists regarding the use of any prosthetic material in such wounds for reconstruction of vascular injury. Twenty-five vascular reconstructions were performed in 20 patients during a 3.5 year period. These patients had life-threatening multiple trauma and severe local tissue damage along with their arterial and venous injuries in open contaminated wounds after blunt (16 patients) and penetrating (4 patients) trauma. In all patients, 6 mm PTFE was used for interposition bypass for arterial injuries, and in five of these patients, 8 mm PTFE was used for concomitant venous interposition bypass. One patient died and there was one arterial and one venous graft thrombosis in the same patient 3 months after a shotgun blast injury to the groin, but there was no limb loss. All other grafts remained patent without wound infection, sepsis, or anastomatic disruption. Under the circumstances of these peripheral vascular injuries, PTFE was an acceptable choice for primary reconstruction in our patients. Its ready availability in many calibers, sparing of autogenous vein for future use, and its expedience in vascular reconstruction comprise the advantages of using PTFE in multiply traumatized patients without producing the feared evidence of infected prosthetic grafts. 相似文献
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David A. Crawford Joanne B. Adams Michael J. Morris Keith R. Berend Adolph V. Lombardi 《The Journal of arthroplasty》2019,34(12):3048-3053
BackgroundManagement of an infected total hip arthroplasty (THA) is challenging. The eradication of infection as well as complications of component removal must all be considered. This study is an update on previous reports of treating periprosthetic infection of the hip with a partial 2-stage exchange with retention of the femoral component.MethodsA retrospective review of our practice’s arthroplasty registry from 2000 to 2018 revealed 41 hips with 2-year minimum follow-up that were treated with a 2-stage partial exchange for an infected THA. All first-stage procedures allowed an articulating construct with 1 of 3 variations: cemented constrained liner (13 hips), StageOne Hip Cement Spacer Mold (14 hips), or an antibiotic polymethylmethacrylate head molded from a bulb syringe (14 hips). Of 41 cases, 34 were culture positive, with 3 cases having methicillin-resistant Staphylococcus.ResultsMean follow-up was 5.5 years (range, 1.5-18.5 years). The second-stage reimplantation was accomplished in 39 of the 41 hips (95%) at a mean interval of 9.2 weeks (range, 5-9 weeks). Two patients underwent repeat radical debridement with removal of all components before reimplantation for persistent clinical evidence of infection. Thirty-three of the 41 hips (81%) were infection free at most recent follow-up. The mean postoperative Harris hip score at most recent evaluation was 63.6 (range, 24-100).ConclusionEradication of periprosthetic joint infections, while minimizing patient morbidity, continues to be a challenge. Partial 2-stage exchange may be considered in cases where removal of a well-fixed femoral component may result in significant bony destruction. 相似文献
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