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We examined six patients with Gardner's syndrome, eight first-degree relatives, and 31 age- and sex-matched controls to document the presence, distribution, and morphologic features of congenital hypertrophy of the retinal pigment epithelium. Patients with Gardner's syndrome had multiple, bilateral lesions, with 288 of 346 foci (83%) located posterior to the equator. Linear-shaped congenital hypertrophy of the retinal pigment epithelium, a distinctive finding in these patients, accounted for 44 of 140 large lesions (31%). Despite multifocal fundus involvement, results of electro-oculography were normal in all eyes tested.  相似文献   
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Ruggieri  PM; Laub  GA; Masaryk  TJ; Modic  MT 《Radiology》1989,171(3):785-791
The technique and feasibility of magnetic resonance (MR) angiography of intracranial vessels were studied in 35 healthy volunteers. Variations in image orientation, repetition time (TR), and flip angle were evaluated to determine their effects on flow-related enhancement. Gradient modifications--including echo time (TE), motion compensation, bandwidth, and field of view--were also studied in an effort to reduce motion-induced phase shifts. Results indicated that a FISP (fast imaging with steady precession) sequence with a TR of 50 msec, TE of 15 msec, velocity compensation in the read and section-select directions, acceleration compensation in the read direction, anisotropic volume, and a 1.25-mm partition thickness produced three-dimensional angiographic MR images that were accurate and reproducible in the depiction of the major intracranial vessels. Difficulties with field of view, persistent signal void secondary to higher-order motion, and spatial resolution remain major problems requiring additional study.  相似文献   
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A patient with end-stage renal failure, two previously failed kidney transplants and high serum lymphocytotoxic antibody levels was transplanted electively with an ABO-mismatched, HLA-identical kidney from his sibling. Immunosuppression consisted of pretransplant splenectomy and plasma exchange, followed by rabbit antithymocyte serum, azathioprine, prednisone and plasma exchange in the early post-transplant period. He is now 3 years post-transplant with normal renal function on conventional immunosuppressive therapy. This case, and a review of the literature, suggest that ABO blood group incompatibility need not be an absolute barrier to successful kidney transplantation. ABO incompatible kidney transplantation may be a potentially fruitful area for further research as the demand for donor organs continues to outstrip the supply.  相似文献   
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Pharmacokinetics of intravenous bepridil in patients with coronary disease   总被引:1,自引:0,他引:1  
The pharmacokinetics of intravenous bepridil (1-[2-(N-benzylanilino)-1-(isobutoxymethyl)ethyl]pyrrolidine ) were studied in 16 patients undergoing cardiac catheterization for evaluation of coronary disease, all with normal base-line hemodynamic and renal functions. Ten patients received 3 mg/kg and six patients received 4 mg/kg of bepridil infused over a period of 30 min. Plasma bepridil concentrations were measured by HPLC and analyzed by model-dependent and model-independent methods. The mean (+/- SD) maximum plasma bepridil concentrations at the end of the infusion were 2047 +/- 820 ng/mL (3 mg/kg) and 2478 +/- 1426 ng/mL (4 mg/kg). Postinfusion bepridil concentrations were best described by a two-compartment open model. The model-dependent harmonic mean distribution and elimination half-lives were 1.7 h (range: 1.1-2.2 h) and 19.7 h (range: 8.0-61.9 h), respectively. The harmonic mean elimination half-life from model-independent analysis was 14.9 h (range: 7.4-64.0 h). The arithmetic means of other model-independent kinetic parameters were systemic clearance, 0.524 +/- 0.215 L X kg-1 X h-1; Vd, 15.3 +/- 10.9 L/kg; and Vdss, 10.1 +/- 6.0 L/kg. Model-dependent and model-independent estimates of half-life and clearance agreed reasonably well. Bepridil was well tolerated, effecting little or no change in central hemodynamics or EKG intervals. The extensive distribution and relatively slow clearance of bepridil account for its long elimination half-life. Intravenous bepridil appears to be a safe calcium (II) antagonist that is suitable for once-a-day dosing.  相似文献   
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OBJECTIVES: To determine the type of electrocardiogram (ECG) interpretation instruction in emergency medicine (EM) residency programs, the use and perceived value of teaching modalities and resources, and the methods used to assess competency of ECG interpretation. METHODS: An interactive survey instrument was posted on the Internet using SurveySuite, Inc., software and e-mailed to program directors (PDs) of all 125 Accreditation Council for Graduate Medical Education-approved U.S. EM residency programs. Responses are reported in total numbers and percentages. RESULTS: Ninety-nine of 125 PDs completed the online survey (response rate, 79.2%). Emergency department instruction (99%), case-based lectures (98%), and didactic lectures (98%) were most commonly used to teach interpretation of ECGs, followed by computer-based instruction (34%) and ECG laboratory (12%). The majority of programs (53%) spent more than eight hours on formal ECG lectures per year, while 11% spent less than three hours. Observation during clinical time (99%), lecture time (76%), and hypothetical cases (57%) were the most common ways to determine competency in reading ECGs, while clinical observation and hypothetical cases were perceived as the most valuable. The most commonly used resource was personal or departmental ECG files (91%), and this had the highest perceived value. The majority of PDs were comfortable with residents' abilities to read ECGs by the third year (96%) and fourth year (91%) of residency. CONCLUSIONS: These data suggest that EM PDs believe that EM residency is adequately preparing graduates to interpret ECGs. This goal is achieved through a variety of methods.  相似文献   
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The development of a unifying framework for conceptualizing the commonalities in various forms of substance abuse must encompass the data base focused upon the stimulus functions of drugs. In the first instance, for example, the research on drug self-administration has provided convincing evidence of a remarkable concordance between laboratory animals and human substance abusers in the reinforcing stimulus functions of a range of chemical agents. The recognition of these cross-species and cross-drug generalities has radically changed conceptualizations of substance abuse from a reactive to a more active process and has encouraged the kind of functional analysis of drug-seeking and drug-taking that has proven productive and useful in the study of other behavioral interactions. In this regard as well, recent refinements in the analysis of the discriminative stimulus functions of drugs have provided a more comprehensive basis for characterizing a chemical agent's spectrum of action and evaluating its abuse liability. While the correlation between the discriminative stimulus functions and the reinforcing stimulus functions is remarkably high for some drug classes, there are notable exceptions. Finally, the assessment of abuse liability requires an analysis of the eliciting stimulus functions of drugs as reflected by the physiological and behavioral changes, both acute and chronic, that follow drug administration. The methods used to evaluate both physiological dependence and behavioral toxicity in relationship to sensory and motor effects for a range of abused drugs have depended heavily upon an assessment of the eliciting stimulus functions of such compounds.  相似文献   
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