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71.
Further delineation of a generalized bone dysplasia which we call spondylo-meta-epiphyseal dysplasia, short limb-abnormal calcification type is presented. This dwarfing condition has several serious complications, with the most common cause of death being spinal cord damage secondary to atlantoaxial instability. It is a heritable condition with an autosomal recessive mode of transmission. Radiologic diagnostic criteria are developed on the basis of studies in 8 patients with the oldest being between 4 and 5 years old. The condition is clinically and radiographically apparent neonatally or in early infancy, and it is probable that all or almost all affected individuals will come to medical attention in the age range screened by this study.  相似文献   
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In a previous paper we described the experiments and the framework of a model for the exchange of monooleoylphosphatidylcholine with a single egg phosphatidylcholine membrane. In the present paper a model is presented that relates the experimentally measured apparent characteristics of the overall kinetics of lysolipid exchange to the true rates of lysolipid exchange and interbilayer transfer. It is shown that the adsorption of the lysolipid follows two pathways: one through the adsorption of lipid monomers and other through the fusion of micelles. The desorption of lysolipid follows a single pathway, namely, the desorption of monomers. The overall rate of fast desorption under convective flow conditions gives the true rate of monomer desorption from the outer membrane monolayer. The overall rate of both slow lysolipid uptake and slow desorption gives the rate of interbilayer transfer. Because of the uneven distribution of lysolipid between the two monolayers during its uptake, one of the membrane monolayers is apparently extended relative to the other. This relative extension of one of the monolayers induces a monolayer tension. The induced monolayer tension can increase up to 7 mN.m-1, when most of the intercalated lysolipid only partitions into the monolayer facing the lysolipid solution. This value is similar to the measured value for the critical monolayer tension of membrane failure, which is on the order of 5 mN.m-1. The similarity of the magnitudes of the induced monolayer tension during monooleoylphosphatidylcholine exchange and the monolayer tension of membrane failure suggests that the interbilayer lipid transfer may be affected by the formation of short living membrane defects. Furthermore, the pH-induced interbilayer exchange of phosphatidylglycerol is considered. In this case, it is shown that the rate of interbilayer transfer is a function of the phosphatidylglycerol concentration in the membrane.  相似文献   
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The Tuberculosis in Animals Subsection of the International Union Against Tuberculosis and Lung Disease (IUATLD) recently identified a need to standardize the deoxyribonucleic acid (DNA) strain typing of Mycobacterium bovis. The standard method for strain typing of M. tuberculosis isolates cannot be directly extrapolated to M. bovis due to the low copy number of IS6110 identified in the majority of M. bovis strains, particularly from cattle. To improve the resolution of M. bovis strains, alternative methods and additional DNA probes have been investigated. In combination with studies of published literature, laboratories performing M. bovis DNA fingerprinting were surveyed. Results of these surveys allowed us to reach consensus and to make recommendations for DNA typing of M. bovis isolates, which hopefully will lead towards a standardized approach to the DNA fingerprinting of this organism. This approach, in conjunction with conventional epidemiological traceback approaches, should facilitate more accurate and effective investigations into the epidemiology, maintenance and transmission of M. bovis within and between man and domesticated, feral and wild animals, both at a local and a global level.  相似文献   
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Testicular germ cell tumour is said to be a model for curable neoplasm. However, the prognosis of primary extragonadal germ cell tumour does not appear to be as promising. Though similar in histology, the biology of primary extragonadal germ cell tumour is different as exemplified by the patients in this review. Eight patients with primary mediastinal germ cell tumours were treated with intensive cisplatin-based chemotherapy. All, except one, had non-seminomatous components. The poor prognosis of mediastinal germ cell tumour is due to a combination of poor treatment results with the cisplatin-based regimen and the development of non-germ cell and haematological malignancies.  相似文献   
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INTRODUCTION: The objective of this study was to examine the occurrence of asymptomatic myocardial ischemia prior to and after myocardial revascularization in patients with multivessel occlusive coronary disease. Asymptomatic ischemia can be described as real ischemia without anginal pain or other ischemic symptoms in patients with coronary disease or coronary artery spasm. Our study examined silent ischemia after myocardial revascularization. Early detection of silent ischemia is important for prevention of cardiac incidents. MATERIAL AND METHODS: We have examined patients with multivessel coronary disease with occurrence of continued preoperative silent ischemia. All patients have undergone ECG examination, exercise stress test and Holter-monitoring prior to and after myocardial revascularization. RESULTS: The investigation comprised 27 patients and their average age was 54.5 years. All patients with silent ischemia had a multivessel occlusive coronary disease and have undergone myocardial revascularization managed with triple or quadruple aortocoronary bypass surgery. Exercise stress test was performed postoperatively in elder patients, as well as ECG and Holter-monitoring. Silent ischemia was established in 21.6% of patients, while in 87.5% untreated diabetes mellitus was diagnosed. Silent ischemia most often occurred in the early morning hours and it was frequently associated with heart rhythm disturbances (VES) whereas these rhythm disturbances depended on the length of the ischemic episode. Intermittent 2nd degree atrioventricular block was found in one patient. CONCLUSION: Silent myocardial ischemia occurred in 21% of patients after myocardial revascularization. It is most often detected in the early morning hours and is associated with ventricular rhythm disorders. Silent ischemia is easily detected by simple examination procedures providing adequate therapy and prevention of cardiac incidents.  相似文献   
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The clinical and microbiological findings in a chronic case of lacrimal canaliculitis due to Arachnia propionica are described. Bacterial culture and identification should be performed in the investigation of the disease in order to establish the role of A. propionica and other specific actinomycetes at the acute as well as at the chronic stage.  相似文献   
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