伴有LADD综合征的角膜缘干细胞缺乏

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。     

Percutaneous drainage of 335 consecutive abscesses: results of primary drainage with 1-year follow-up.

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.     

Management of neurotrophic keratopathy

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.     

Involvement of the Anterior Segment of the Eye in Patients with Mucopolysaccharidoses: A Review of Reported Cases and Updates on the Latest Diagnostic Instrumentation

Mucopolysaccharidoses (MPS) are a heterogeneous group of rare inherited disorders, characterized by the lack or malfunction of lysosomal enzymes necessary for glycosaminoglycan (GAGs) catabolism, and their subsequent accumulation in many tissues and organs throughout the body. An overview of the current knowledge of corneal and anterior segment manifestations in patients with MPS was provided and clinical guidelines for their diagnosis and management were furnished. The anterior segment of the eye is usually involved in every subtype of MPS, with major complications including varying degrees of corneal opacification and raised intraocular pressure (IOP) with development of glaucoma. Their recognition and management can be very useful in the diagnosis of MPS. Novel techniques are available to objectively measure the grade and extent of corneal clouding and give information about the anatomy of the anterior chamber and the structures of the angle beyond the clouded cornea. It is advisable to take advantage of this new instrumentation in order to obtain thorough information on the ocular involvement and its related anterior chamber complications for a better management of patients with MPS, both in terms of visual prognosis and therapeutic outcome.     

Modified technique to extract malpositioned or migrated self-expanding stents from the esophagus and stomach

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.     

Initial experience of the High-Density Grid catheter in patients undergoing catheter ablation for atrial fibrillation

Purpose

A significant proportion of patients undergoing catheter ablation for atrial fibrillation (AF) experience arrhythmia recurrence. This is mostly due to pulmonary vein reconnection (PVR). Whether mapping using High-Density Wave (HDW) technology is superior to standard bipolar (SB) configuration at detecting PVR is unknown. We aimed to evaluate the efficacy of HDW technology compared to SB mapping in identifying PVR.

Methods

High-Density (HD) multipolar Grid catheters were used to create left atrial geometries and voltage maps in 36 patients undergoing catheter ablation for AF (either due to recurrence of an atrial arrhythmia from previous AF ablation or de novo AF ablation). Nineteen SB maps were also created and compared. Ablation was performed until pulmonary vein isolation was achieved.

Results

Median time of mapping with HDW was 22.3 [IQR: 8.2] min. The number of points collected with HDW (13299.6±1362.8 vs 6952.8±841.9, p<0.001) and used (2337.3±158.0 vs 1727.5±163.8, p<0.001) was significantly higher compared to SB. Moreover, HDW was able to identify more sleeves (16 for right and 8 for left veins), where these were confirmed electrically silent by SB, with significantly increased PVR sleeve size as identified by HDW (p<0.001 for both right and left veins). Importantly, with the use of HDW, the ablation strategy changed in 23 patients (64% of targeted veins) with a significantly increased number of lesions required as compared to SB for right (p=0.005) and left veins (p=0.003).

Conclusion

HDW technology is superior to SB in detecting pulmonary vein reconnections. This could potentially result into a significant change in ablation strategy and possibly to increased success rate following pulmonary vein isolation.

     

A State-of-the-Art Review on Advanced Joining Processes for Metal-Composite and Metal-Polymer Hybrid Structures

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.