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1.
泪管-耳-牙-指(趾)(LADD)综合征是一种具有多种表现的常染色体显性遗传疾病。最初在1967年由Levy报道。1例双侧泪液系统缺失、杯状耳、干嘴,以及牙、手臂和指(趾)异常的单发病例。之后一些新发现的临床表现,如肾脏异常、唾液腺缺乏、先天性髋脱位、先天性裂孔疝和横膈疝、感觉性耳聋和传导性耳聋、牙发育不全、四肢异常、口腔干燥和眼干都被报道与此综合征有关。  相似文献   
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Retrospective review of percutaneous abscess drainage (PAD) of 335 abscesses in 323 consecutive patients was undertaken. Particular attention was directed to body location, associated organ system, communications and fistulae, and to the underlying immunologic status of the patient. One-year follow-up was available in all patients. Overall, the cure rate was 62.4% (209 of 335 abscesses), with a failure rate of 8.95% (30 of 335 abscesses). There were 14.2% (46 of 323 patients) deaths in the follow-up period, of which 4.6% (15 of 323 patients) were believed attributable to sepsis or septic complications. The overall complication rate was 9.8% (33 of 335 abscesses), most of which were minor in nature. For the patient exhibiting immunocompromise, representing 53.1% (172 of 323 patients) of the patient population, the cure rate was 53.4% (95 of 178 abscesses), which was significantly lower than the cure rate of 72.6% (114 of 157 abscesses) for the immunocompetent patient population (n = 151) (P less than .001). The recurrence rate was 2.1% (seven of 335 abscesses), with all recurrences within 3 months of initial drainage. PAD is effective and permanent treatment for both immunocompromised and immunocompetent patients.  相似文献   
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Neurotrophic keratopathy is a degenerative corneal disease caused by an impairment of corneal sensitivity. Lack of the sensory nerve's trophic effect is responsible for the impairment in corneal healing and for the changes on the ocular surface that lead to corneal epithelial deficit, ulcer, and perforation. The etiology and recent advances in understanding of the pathogenetic mechanisms of neurotrophic keratopathy are reviewed here. An accurate history and a clinical examination that covers the function of cranial nerves often identify the cause of the disease. Clinical features and guidelines for the differential diagnosis and treatment are presented. Specific medical and surgical treatments, selected on the basis of clinical staging of the disease, can often halt disease progression. Future developments in medical treatment, including the use of neuropeptide and growth factors, are discussed. The identification of corneal anesthesia associated with an epithelial defect allows appropriate treatment and prevention of progression to stromal lysis and perforation.  相似文献   
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Background and Aim:  Various methods have been used to remove self expandable stents (SES) because of either malposition or migration. The main difficulties encountered in such situations are the anatomic obstacle of the lower and upper esophageal sphincters as well as risk of mucosal injury during removal.
Methods:  We describe a modified approach using an esophagogastroduodenoscope (EGD) in combination with a foreign body hood protector, rat tooth forceps and snare allowing for successful SES removal from the upper gastrointestinal tract in four cases.
Results:  In all cases, the SES were successfully removed from upper gastrointestinal tract using this technique. No complications were noted after extraction.
Conclusion:  The foreign body hood protector combined with rat tooth forceps/snare technique is a safe and effective alternative to previously described methods for extraction of SES from the upper gastrointestinal tract. This method may be applicable for the removal of other such objects within the endoscope's reach.  相似文献   
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Multi-materials of metal-polymer and metal-composite hybrid structures (MMHSs) are highly demanded in several fields including land, air and sea transportation, infrastructure construction, and healthcare. The adoption of MMHSs in transportation industries represents a pivotal opportunity to reduce the product’s weight without compromising structural performance. This enables a dramatic reduction in fuel consumption for vehicles driven by internal combustion engines as well as an increase in fuel efficiency for electric vehicles. The main challenge for manufacturing MMHSs lies in the lack of robust joining solutions. Conventional joining processes, e.g., mechanical fastening and adhesive bonding involve several issues. Several emerging technologies have been developed for MMHSs’ manufacturing. Different from recently published review articles where the focus is only on specific categories of joining processes, this review is aimed at providing a broader and systematic view of the emerging opportunities for hybrid thin-walled structure manufacturing. The present review paper discusses the main limitations of conventional joining processes and describes the joining mechanisms, the main differences, advantages, and limitations of new joining processes. Three reference clusters were identified: fast mechanical joining processes, thermomechanical interlocking processes, and thermomechanical joining processes. This new classification is aimed at providing a compass to better orient within the broad horizon of new joining processes for MMHSs with an outlook for future trends.  相似文献   
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