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The authors draw attention to possible extraocular changes in children with congenital aniridia. Of 31 investigated patients they were found in 5 children. Among these changes, because of its serious character, Wilms tumour holds the first place; it was found by the authors in two children. To ensure its early detection, the authors emphasize the necessity to dispensarize all children with congenital aniridia. Collaboration with an experienced X-ray specialist and child oncologist is essential. In addition to Wilms' tumour congenital aniridia can be associated with serious somatic developmental changes. The authors observed in one child and AGR triad and in two patients deformities of the skeleton of the head and lower extremities.  相似文献   
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In a twelve-year investigation the authors compare the results of three types of weakening operations of the inferior oblique muscle in 298 eyes. The evaluation lead to their views regarding indications of different surgical techniques. In their opinion the best operation is triple partial myotomy of the inferior oblique muscle as it does not influence the horizontal position and preserves the original insertion of the muscle. They modify the techniques of Romer-Apis and Martinez-Oropeza. Fixation of the inferior and external rectus on hooks reveals the surgical field, and the concurrent stretching of the inferior oblique muscle beneath the conjunctiva facilitates its dissection. The authors include triple partial myotomy except for hypertropias in the comprehensive surgical procedure together with plication of the ipsilateral superior oblique muscle in paresis of the trochlear nerve. They indicate retroposition of the inferior oblique muscle only in divergence associated with hyperfunction of the inferior oblique muscle. The operation has a favourable effect on convergent synkinesis and enhances convergence by +2 to +5 degrees. The authors consider free myectomy of the inferior oblique muscle the least physiological operation and use it no longer.  相似文献   
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BACKGROUND: Many cardiac transplant programs have liberalized donor eligibility criteria in an attempt to maximize donor supply and to accommodate increasing demand. Although many studies have evaluated the potential adverse effects of prolonged donor ischemic time (DIT) in adults undergoing cardiac transplantation, relatively few have focused specifically on pediatric recipients that include a substantial number of patients and long-term follow-up. The focus of this study was to examine the effect of extended DIT on mortality after pediatric heart transplantation. METHODS: We conducted a retrospective review of our pediatric cardiac transplant experience in the past 11 years, comparing patients who received allografts and had ischemic times >240 minutes with those who had ischemic times <240 minutes. RESULTS: A total of 129 pediatric patients (<19 years) underwent orthotopic heart transplantation, of whom 78 (60.5%) had DIT <240 minutes and 51 (39.5%) had DIT >240 minutes. We found no statistically significant difference in age, sex, race, height, weight, or donor age between the groups (p = not significant). Post-transplant survival at 1, 5, and 10 years was similar for both groups: 91.2%, 88.0%, and 85.2%, respectively, for patients with DIT <240 minutes vs 89.6%, 87.2%, and 79.8%, respectively, for patients with DIT >240 minutes (p = 0.433). Additionally, using Cox proportional hazard models, extended DIT >240 minutes was not a statistically significant independent predictor of post-transplant mortality (odds ratio, 0.655; 95% confidence interval, 0.518-0.972; p = 0.684; standard error = 0.468). CONCLUSION: Procurement of hearts from distant locations with associated extended DIT is justified in the setting of increased demand and a fixed donor population.  相似文献   
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A promising treatment method for type 1 diabetes mellitus is transplantation of pancreatic islets containing beta-cells. The aim of this study was to develop an MR technique to monitor the distribution and fate of transplanted pancreatic islets in an animal model. Twenty-five hundred purified and magnetically labeled islets were transplanted through the portal vein into the liver of experimental rats. The animals were scanned using a MR 4.7-T scanner. The labeled pancreatic islets were clearly visualized in the liver in both diabetic and healthy rats as hypointense areas on T2*-weighted MR images during the entire measurement period. Transmission electron microscopy confirmed the presence of iron-oxide nanoparticles inside the cells of the pancreatic islets. A significant decrease in blood glucose levels in diabetic rats was observed; normal glycemia was reached 1 week after transplantation. This study, therefore, represents a promising step toward possible clinical application in human medicine.  相似文献   
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Ohne Zusammenfassung The online version of the original article can be found at  相似文献   
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Intestinal neuronal dysplasia (IND) has been reported as an innervation disorder that can present as isolated disease or may be associated with Hirschsprung's disease (HD). The interest in this disorder is growing as it mimics HD at clinical level but can be managed with a more conservative approach if an accurate diagnosis can be made. Many workers have tried to set up diagnostic criteria of this condition. But the importance of one criterion varied from one study to another. In our study we analysed seven cases of suspected innervation disorder that had undergone resection. A detailed histological study on these cases was performed and four of them were found to fulfill the diagnostic criteria of IND laid down by Kobayashi and his co-workers. These patients had hyperganglionosis, giant ganglia and ectopic ganglion cells in the lamina propria. In the other three cases some features were highly suggestive of the diagnosis of the IND and can be considered to be so if we follow other workers who have not given much importance to the simultaneous presence of all three criteria in a single case.  相似文献   
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