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排序方式: 共有241条查询结果,搜索用时 15 毫秒
111.
112.
K U Aziz M H Paul F S Idriss A D Wilson A J Muster 《The American journal of cardiology》1979,44(2):290-297
Four infants with d-transposition of the great arteries and intact ventricular septum who manifested early clinical symptoms and deterioration due to dynamic left ventricular outflow stenosis are presented. All four had an anatomically adequate atrial septal defect, made at the initial balloon atrial septostomy, that was later confirmed intraoperatively. Two infants continued to have a low arterial oxygen saturation level because of inadequate interatrial mixing, and one of these had severe persistent cyanosis and was treated with the Mustard operation at age 4 days. The other two infants subsequently presented with hypercyanotic spells at age 3 months. All four infants had features of dynamic left ventricular outflow stenosis on hemodynamic, angiocardiographic and echocardiographic studies. The left ventricular outflow pressure gradient was shown to increase after administration of isoproterenol in one infant, and relief of a cyanotic spell with reduction of left ventricular systolic pressure was achieved in another after intravenous administration of propranolol. The Mustard operation relieved symptoms in all infants. The effect of left ventricular outflow tract stenosis on the mechanisms responsible for interatrial mixing in d-transposition of the great arteries with intact ventricular septum is discussed. 相似文献
113.
114.
DNase I-hypersensitive sites I and II of the human growth hormone locus control region are a major developmental activator of somatotrope gene expression 总被引:4,自引:0,他引:4 下载免费PDF全文
115.
Koole Michel Lohith Talakad G. Valentine John L. Bennacef Idriss Declercq Ruben Reynders Tom Riffel Kerry Celen Sofie Serdons Kim Bormans Guy Ferry-Martin Sandrine Laroque Philippe Walji Abbas Hostetler Eric D. Briscoe Richard J. de Hoon Jan Sur Cyrille Van Laere Koen Struyk Arie 《Molecular imaging and biology》2020,22(1):173-180
Molecular Imaging and Biology - [18F]MK-6240 is a selective, high-affinity positron emission tomography tracer for imaging neurofibrillary tangles, a key pathological signature that correlates with... 相似文献
116.
Rhonda S. Lappen Alexander J. Muster Farouk S. Idriss Thomas W. Riggs Michel Ilbawi Milton H. Paul Saroja Bharati Maurice LEV 《The American journal of cardiology》1983,52(3):336-340
Five patients with ostium primum atrial septal defect (ASD) and a cleft mitral valve had no hemodynamic evidence of left ventricular (LV) outflow tract obstruction on preoperative cardiac catheterization. After surgical closure of the ASD and repair of the mitral cleft, all 5 patients manifested subaortic stenosis with pressure gradients ranging from 10 to 120 mm Hg. Postoperative LV angiograms revealed systolic narrowing of the outflow tract, and the same outflow tract dynamics were recognized on reviewing the preoperative angiograms and echocardiograms. Persistence or exaggeration of the characteristic diastolic “goose-neck” deformity during LV systole in atrioventricular canal defects is diagnostic of a potential or actual subaortic obstruction. This diagnostic sign is also readily recognizable by 2-dimensional echocardiography, and when present, the surgeon should be alerted to explore the LV outflow tract because the outflow tract anatomy is not readily apparent at operation aimed solely at closing the ASD and repairing the cleft mitral valve. 相似文献
117.
Annette K Ansong Jennifer S Li Eva Nozik-Grayck Richard Ing Richard M Kravitz Salim F Idriss Ronald J Kanter Henry Rice Y T Chen Priya S Kishnani 《Genetics in medicine》2006,8(5):297-301
PURPOSE: Electrocardiogram (ECG) abnormalities are universal in infantile Pompe disease or glycogen storage disease type II, a fatal genetic muscle disorder caused by deficiency of acid alpha-glucosidase (GAA). Hallmarks of this disease include a shortened PR interval, an increased QT dispersion (QTd), and large left ventricular (LV) voltages. We evaluated the effect of recombinant human GAA (rhGAA) enzyme replacement therapy (ERT) on these ECG parameters in patients with infantile-onset Pompe disease. METHODS: A total of 134 ECGs were evaluated from 19 patients (5 females and 14 males) with a median age of 5.5 months at the time of enrollment in open-label clinical trials exploring the safety and efficacy of ERT at a single center from 1999 to 2004. rhGAA was purified from genetically engineered Chinese hamster ovary cells overproducing GAA and infused intravenously at doses ranging from 10 mg/kg per week to 20 to 40 mg/kg every 2 weeks in patients with infantile-onset Pompe disease. The PR interval, QTd (longest to shortest QT), and LV voltage (SV1 + RV6) were blindly determined by two independent observers. RESULTS: The median follow-up period was 6 months (range 2-30 months). The PR interval lengthened from 83 (42-110) ms to 107 (95-130) ms (P < .001), and the QTd decreased from 83 (40-125) ms to 53 (20-80) ms (P = .003). There were significant decreases in LV voltage (67 [17-83] mV vs. 48 [18-77] mV, P = .03), which correlated with decrease in LV mass on two-dimensional echocardiogram. There was no evident change in the QTc interval (429 [390-480] ms vs. 413 [370-450] ms, P = not significant). CONCLUSION: rhGAA ERT for infantile Pompe disease results in an increase in PR interval and a decrease in both the QTd and the LV voltage. These results suggest that these ECG parameters may be useful markers of the severity of cardiac disease and the response to ERT treatment in patients with infantile Pompe disease. 相似文献
118.
A modified technique of infant orthotopic cardiac transplantation with arch reconstruction using bicaval cannulation is described, and the results in 4 infants with hypoplastic left heart syndrome are presented. This technique minimizes donor myocardial ischemic time and recipient circulatory arrest time. 相似文献
119.
120.
Xuejin Cao Zan Wang Xiaohui Chen Yanli Liu Wei Wang Idriss Ali Abdoulaye Shenghong Ju Xi Yang Yuancheng Wang Yijing Guo 《Human brain mapping》2021,42(14):4750
Diffusion tensor imaging (DTI) studies have revealed distinct white matter (WM) characteristics of the brain following diseases. Beyond the lesion‐symptom maps, stroke is characterized by extensive structural and functional alterations of brain areas remote to local lesions. Here, we further investigated the structural changes over a global level by using DTI data of 10 ischemic stroke patients showing motor impairment due to basal ganglia lesions and 11 healthy controls. DTI data were processed to obtain fractional anisotropy (FA) maps, and multivariate pattern analysis was used to explore brain regions that play an important role in classification based on FA maps. The WM structural network was constructed by the deterministic fiber‐tracking approach. In comparison with the controls, the stroke patients showed FA reductions in the perilesional basal ganglia, brainstem, and bilateral frontal lobes. Using network‐based statistics, we found a significant reduction in the WM subnetwork in stroke patients. We identified the patterns of WM degeneration affecting brain areas remote to the lesions, revealing the abnormal organization of the structural network in stroke patients, which may be helpful in understanding of the neural mechanisms underlying hemiplegia. 相似文献