Mechanisms and treatment strategies of demyelinating and dysmyelinating Charcot-Marie-Tooth disease

Schwann cells,the myelinating glia of the peripheral nervous system,wrap axons multiple times to build their myelin sheath.Myelin is of paramount importance for axonal integrity and fast axon potential propagation.However,myelin is lacking or dysfunctional in several neuropathies including demyelinating and dysmyelinating Charcot-M arie-To oth disease.Charcot-Marie-To oth disease represents the most prevalent inherited neuropathy in humans and is classified either as axonal,demyelinating or dysmyelinating,or as intermediate.The demyelinating or dysmyelinating forms of Charcot-Marie-Tooth disease constitute the majority of the disease cases and are most frequently due to mutations in the three following myelin genes:peripheral myelin protein 22,myelin protein ze ro and gap junction beta 1(coding for Connexin 32) causing Charcot-M arie-Tooth disease type 1A,Charcot-Marie-Tooth disease type 1B,and X-linked Charcot-M arie-Tooth disease type 1,respectively.The resulting perturbation of myelin structure and function leads to axonal demyelination or dysmyelination and causes severe disabilities in affected patients.No treatment to cure or slow down the disease progression is currently available on the market,howeve r,scientific discoveries led to a better understanding of the pathomechanisms of the disease and to potential treatment strategies.In this review,we describe the features and molecular mechanisms of the three main demyelinating or dysmyelinating forms of Charcot-Marie-Tooth disease,the rodent models used in research,and the emerging therapeutic approaches to cure or counteract the progression of the disease.

Emergent treatment of patients with traumatic aorta ruptures

Objective ： To discuss our experience on the diagnosis and treatment of thoracic aorta rupture （TAR） that is one of the main common causes of death in the victims under blunt chest trauma. Methods： Between July 2001 and March 2006, 9 patients （6 men and 3 women, aged from 20 to 54 years ） suffering from acute traumatic aorta rupture after motor vehicle accidents received emergent surgical treatments in our hospital. Based on our experience in the rescue of the first TAR patient we introduced a practical procedure on the diagnosis and treatment of TAR in our department. All the other patients generally followed this procedure. Eight patients received contrast material enhanced helical computerized tomography scan before the operation. The leakage of coustrast medium from the aorta isthmus was found, and diagnosis of TAR was confirmed. Seven patients underwent immediate operation within 14 hours after accidents. One patient was treated on the 5th day of the accident because of delayed diagnosis of aortic rupture. All patients received general anesthesia with double lumen endotracheal tube and normothermic femoro-femoral partial cardiopulmonary bypass, with beating heart and aortic clamping. One patient received simple repair, and others received partial replacement of thoracic aorta with artificial vascular graft. Results： Seven TAR patients were successfully salvaged. Three patients combined brain injury as well as extremitiy hemiplegia before operation. After treatments one was fully and two partially recovered without paraplegia. Conclusions： Proper practical protocol is emphasized for the surgical repair of TAR because it will reduce the mortality of severe blunt chest injury.

Evaluating the Effectiveness of Emergency Ventilator Treatment for Severe Acute Left Ventricular Heart Failure

Objective:To evaluate the efficacy of emergency ventilator therapy in severe acute left heart failure.Methods:A total of 75 patients with severe acute left ventricular heart failure who were admitted to the hospital from July 2020 to July 2023 were randomly divided into two groups.Group A received additional emergency ventilator treatment,and group B received conventional treatment.The efficacy was compared.Results:The curative effect of patients with severe acute left heart failure in group A was higher than that in group B(P<0.05);all blood gas indicators in group A were better than those in group B(P<0.05);all vital signs indicators in group A were better than those in group B(P<0.05);group A was more satisfied with the treatment of severe acute left ventricular heart failure than group B(P<0.05).Conclusion:Patients with severe acute left heart failure who receive emergency ventilator treatment can stabilize vital signs,improve blood oxygen supply,and enhance curative effect.

Thoracic giant cell tumor after two total en bloc spondylectomies including one emergency surgery:A case report

BACKGROUND For patients with acute paraplegia caused by spinal giant cell tumor(GCT)who require emergency decompressive surgery,there is still a lack of relevant reports on surgical options.This study is the first to present the case of an acute paraplegic patient with a thoracic spinal GCT who underwent an emergency total en bloc spondylectomy(TES).Despite tumor recurrence,three-level TES was repeated after denosumab therapy.CASE SUMMARY A 27-year-old female patient who underwent single-level TES in an emergency presented with sudden severe back pain and acute paraplegia due to a thoracic spinal tumor.After emergency TES,the patient's spinal cord function recovered,and permanent paralysis was avoided.The postoperative histopathological examination revealed that the excised neoplasm was a rare GCT.Unfortunately,the tumor recurred 9 months after the first surgery.After 12 months of denosumab therapy,the tumor size was reduced,and tumor calcification.To prevent recurrent tumor progression and provide a possible cure,a three-level TES was performed again.The patient returned to an active lifestyle 1 month after the second surgery,and no recurrence of GCT was found at the last follow-up.CONCLUSION This patient with acute paraplegia underwent TES twice,including once in an emergency,and achieved good therapeutic results.TES in emergency surgery is feasible and safe when conditions permit;however,it may increase the risk of tumor recurrence.

Key technologies of earthquake early warning system for China’s high-speed railway

Purpose–The purpose of this study is to introduce the top-level design ideas and the overall architecture of earthquake early-warning system for high speed railways in China,which is based on P-wave earthquake early-warning and multiple ways of rapid treatment.Design/methodology/approach–The paper describes the key technologies that are involved in the development of the system,such as P-wave identification and earthquake early-warning,multi-source seismic information fusion and earthquake emergency treatment technologies.The paper also presents the test results of the system,which show that it has complete functions and its major performance indicators meet the design requirements.Findings–The study demonstrates that the high speed railways earthquake early-warning system serves as an important technical tool for high speed railways to cope with the threat of earthquake to the operation safety.The key technical indicators of the system have excellent performance:The first report time of the P-wave is less than three seconds.From the first arrival of P-wave to the beginning of train braking,the total delay of onboard emergency treatment is 3.63 seconds under 95%probability.The average total delay for power failures triggered by substations is 3.3 seconds.Originality/value–The paper provides a valuable reference for the research and development of earthquake early-warning system for high speed railways in other countries and regions.It also contributes to the earthquake prevention and disaster reduction efforts.

Regression of hepatic fibrosis after pharmacological therapy for nonalcoholic steatohepatitis

The global incidence of nonalcoholic fatty liver disease(NAFLD)is escalating considerably.NAFLD covers a range of liver conditions from simple steatosis to the more severe form known as nonalcoholic steatohepatitis,which involves chronic liver inflammation and the transformation of hepatic stellate cells into myofibroblasts that generate excess extracellular matrix,leading to fibrosis.Hepatocyte ballooning is a key catalyst for fibrosis progression,potentially advancing to cirrhosis and its decompensated state.Fibrosis is a critical prognostic factor for outcomes in patients with NAFLD;therefore,those with substantial fibrosis require timely intervention.Although liver biopsy is the most reliable method for fibrosis detection,it is associated with certain risks and limitations,particularly in routine screening.Consequently,various noninvasive diagnostic techniques have been introduced.This review examines the increasing prevalence of NAFLD,evaluates the noninvasive diagnostic techniques for fibrosis,and assesses their efficacy in staging the disease.In addition,it critically appraises current and emerging antifibrotic therapies,focusing on their mechanisms,efficacy,and potential in reversing fibrosis.This review underscores the urgent need for effective therapeutic strategies,given the dire consequences of advanced fibrosis.

Rapid and effective treatment of traumatic spinal cord injury using stem cell derived exosomes

Traumatic spinal cord injury is a fatal acute event without effective clinical therapies.Following the trauma,immediate neural protection and microenvironment mitigation are vitally important for nerve tissue repair,where stem cell-based therapies could be eclipsed by the deficiency of cells due to the hostile microenvironment as well as the transport and preservation processes.Effective emergency strategies are required to be convenient,biocompatible,and stable.Herein,we assess an emergency cell-free treatment using mesenchymal stem cell-derived exosomes,which have proven capable of comprehensive mitigation of the inhibitory lesion microenvironment.The clinically validated fibrin glue is utilized to encapsulate the exosomes and in-situ gelates in transected rat spinal cords to provide a substrate for exosome delivery as well as nerve tissue growth.The emergency treatment alleviates the inflammatory and oxidative microenvironment,inducing effective nerve tissue repair and functional recovery.The therapy presents a promising strategy for effective emergency treatment of central nervous system trauma。

Current status of emergency treatment of chemical eye burns in workplaces

Chemical eye burns present an avoidable,but frequent,occupational injury with potentially detrimental consequences for the quality of life and occupational rehabilitation of the injured.A periodical review of guidelines is required to assure the optimal emergency management.We reviewed the literature with emphasis on current German guidelines,primarily MEDLINE.If the crucial first-line measure,the injury prevention has failed and an eye burn has been sustained,the immediate and copious rinsing of the eye is the pivotal emergency treatment modality.Whereas the immediacy and sufficiency of the emergency rinsing are largely unanimous,there is an ongoing debate about the benefits and risks of specific rinsing solutions,and regular updates on guidelines and recommendations for the emergency treatment are warranted.The easiest and readily available rinsing solution is tap water,which fulfils the crucial criteria conveniently in most industrialized countries:purity,sterility,and neutral p H.Other rinsing solutions are proposing higher osmolality to stabilize the physiological p H,because of their superior buffering capacity.However,there is no compelling evidence for a substantial benefit,and some reports suggest that there could be unwanted side effects.In combination with the substantially increased expenditure and a more complex handling procedure,currently a general recommendation of any other solution than tap water is not warranted.

Effect of typhoon with extreme precipitation on mountain reservoir drinking water treatment: a case study in Ningbo, China

Due to frequent drinking water pollution accidents in the past decade, it is common that mountain reservoirs were used as the source of drinking water in China. However, some coastal areas frequently suffer from typhoon with extreme precipitation, which results in the water quality deterioration of the reservoirs. The influence of typhoons with extreme precipitation on Jiaokou reservoir and the emergency treatment process of Maojiaping water treatment plant in the past three typical typhoons with extreme precipitation from the year of 2012-2015 were studied. It was found that the degradation of water quality, such as the increase of turbidity and bacteria index, may not merely appear during the events, but last for several days. Changing the dosage of water purification agent, such as coagulant and disinfectant at right time and place may be an efficient emergency water treatment process. Based on the analysis of water quality variation rule during and after the events, it was also found that emergency treatment can be fully prepared before the arrival of a typhoon with extreme precipitation. And in order to better respond to the typhoon with extreme precipitation, several suggestions are also proposed in this paper as follows: establishing vegetated buffers at right place, such as macrophanerophytes,shrub or herbage, increasing investments in infrastructure management, merging or cancelling the small-scale water treatment plants, preparing adequate water purification agent before the typhoon comes, etc.

Emergency Treatment of Testicular Torsion and Postoperative Follow-up: A 71 Case Report

This study presented our experience in the treatment of testicular torsion,which may help achieve early diagnosis and improve therapeutic effects.A retrospective analysis was conducted in 71 patients with testicular torsion who were treated in our hospital from October 2007 to April 2011.The age of the patients ranged from 16 days to 34 years.All the patients had unilateral testicular torsion,which took place on the left side in 43 cases and on the right side in 28 cases.The course of the disease varied between three hours to 30 days.Post-operative follow-up was conducted until October 2011.Items examined included signs and symptoms at their first clinical visit,ultrasound findings,treatment in emergency surgery,and post-operative follow-up.In this study,the 71 patients were diagnosed with testicular torsion by color Doppler sonography,7 had testicular fixation,63 patients received orchiectomy,while 1 patient did not undergo surgery due to pressure from family members.Post-operative follow-up showed that the one patient’s testicle,which had been reserved,atrophied,while all the other survived.No recurrence was found during the follow-up visits.It is concluded that an early diagnosis and surgery is important in improving the survival rate of testicular torsion,and the diagnosis and treatment by the first attending clinician is of critical importance.

Novel method of primary endoscopic realignment for high-grade posterior urethral injuries:A case report

BACKGROUND A male urethral disruption injury is a urological emergency.Primary endoscopic realignment(PER)refers to reestablishment of urethral alignment via indwelling urethral catheter by cystoscope,which is recommended as the optimal emergent treatment approach for reducing the likelihood of complications following injury.However,the prior literature suggests the success rate of PER to be relatively low due to complicated urethral disruption.We report a modified PER approach that serves to improve both the success rate and safety of the treatment.CASE SUMMARY A 19-year-old male patient presented with multiple pelvic fractures and complete urethral disruption following a high-velocity traffic accident.The patient’s abdominal computed tomography and retrograde urethrography results revealed complete urethral disruption at the bulbar urethra,with hematoma and contrast medium extravasation that extended into the extraperitoneal space.The conventional retrograde PER by cystoscope failed due to severe disruption and considerable hematoma.Modified simultaneous antegrade and retrograde PER was performed by means of semi-rigid ureteroscopy via a suprapubic Foley catheter and cystoscopy via the external urethra.An antegrade guidewire was passed through the bladder neck and then pulled out through the external urethral meatus with a cystoscope.Urethral continuity was achieved after a 16-Fr silicone Foley catheter was indwelled into the bladder along the guidewire.The patient recovered well,achieving voiding continence and avoiding further operation for urethral stricture.CONCLUSION Modified PER via suprapubic Foley catheter represents a promising and safe treatment approach in patients with posterior urethral injuries.

Application effect for a care bundle in optimizing nursing of patients with severe craniocerebral injury

BACKGROUND Severe craniocerebral injury(STBI)is a critical physical trauma caused by a sudden external force acting on the head.The condition is complex and changeable,and disability and mortality rates are high.Although the life of STBI patients can be saved through treatment,the sequelae of consciousness,speech,cognitive impairment,stiffness,spasm,pain and abnormal behavior in the early rehabilitation stage can be a heavy burden to a family.In the past,routine nursing was often used to treat/manage STBI;however,problems,such as improper cooperation and untimely communication,reduced therapeutic effectiveness.AIM To investigate the effect of a proposed care bundle to optimize the first aid process and assess its effectiveness on the early rehabilitation nursing of patients with STBI.METHODS From January 2019 to December 2020,126 STBI patients were admitted to the emergency department of Chongqing Emergency Medical Center.These patients were retrospectively selected as the research participants in the current study.The study participants were then divided into a control group(61 cases)and a study group(65 cases).The control group was treated with routine nursing.The study group adopted the proposed care bundle.The National Institutes of Health Stroke Scale/Score and Glasgow Coma Scale(GCS)were used to evaluate neurological function before and after emergency treatment.After 3 mo of rehabilitation,experimental outcomes were assessed.These included the GCS,Barthel Index,complication rate,muscle strength grade and satisfaction.RESULTS There was no significant difference in gender,age,cause of injury and GCS between the two groups.After emergency,the National Institutes of Health Stroke Scale/Score of the study group(10.23±3.26)was lower than that of the control group(14.79±3.14).The GCS score of the study group(12.48±2.38)was higher than that of the control group(9.32±2.01).The arrival time of consultation in the study group was 20.56±19.12,and the retention time in the emergency room was 45.12±10.21,which were significantly shorter than those in the control group.After 3 mo of rehabilitation management,the GCS and Barthel Index of the study group were 14.56±3.75 and 58.14±12.14,respectively,which were significantly higher than those of the control group.The incidence of complications in the study group(15.38%)was significantly lower than that in the control group(32.79%).The proportion of muscle strength≥grade III in the study group(89.23%)was significantly higher than that in the control group(50.82%).The satisfaction of patients in the study group was significantly higher than that in the control group.CONCLUSION Care bundles are used to optimize the nursing process.During first-aid,care bundles can effectively improve the rescue effect and improve neurological function of STBI patients as well as shorten the treatment time.In early rehabilitation,they can effectively improve the consciousness of STBI patients,improve the activities of daily living,reduce the risk of complications,accelerate the recovery of muscle strength and improve their satisfaction.

Applying chemical sedimentation process in drinking water treatment plant to address the emergent arsenic spills in water sources

Arsenic （As） spills and sometimes polluted water occurred more frequently sources in recent years in China. It is as urgent need to develop emergency treatment technologies to address the arsenic threat for large-scale water treatment plants. In response, we developed a chemical sedimentation technology to remove arsenic contaminants for water treatment plants. Bench-scale experiments were conducted to investigate the efficiency of arsenic removal and the influencing factors of the chemical sedimentation treatment process. The influencing factors included the choice and dosage of coagulants, the valence of arsenic and pH value of solution. The As（V） contaminants can be almost completely removed by ferric or alum coagulants. The As（III） contaminants are more recalcitrant to chemical sedimentation, 75% for ferric coagulant and 40% for alum coagulant. The quantitative results of arsenic removal load by different ferric or alum coagulants were presented to help determine the parameters for arsenic treatment technology. The dominant mechanism for arsenic removal is static combination, or adsorption of negative arsenic species onto positive ferric hydroxide or alum hydroxide flocs. The efficiency of this treatment technology has also been demonstrated by a real production test in one water treatment plant with arsenicrich source water and one emergency response. This technology was verified to be quick to set-up, easy to operate and highly efficient even for high concentration of arsenic.

The reliability of the Australasian Triage Scale:a meta-analysis

BACKGROUND: Although the Australasian Triage Scale(ATS) has been developed two decades ago, its reliability has not been def ined; therefore, we present a meta-analyis of the reliability of the ATS in order to reveal to what extent the ATS is reliable.DATA SOURCES: Electronic databases were searched to March 2014. The included studies were those that reported samples size, reliability coefficients, and adequate description of the ATS reliability assessment. The guidelines for reporting reliability and agreement studies(GRRAS) were used. Two reviewers independently examined abstracts and extracted data. The effect size was obtained by the z-transformation of reliability coefficients. Data were pooled with random-effects models, and meta-regression was done based on the method of moment's estimator.RESULTS: Six studies were included in this study at last. Pooled coefficient for the ATS was substantial 0.428(95%CI 0.340–0.509). The rate of mis-triage was less than fifty percent. The agreement upon the adult version is higher than the pediatric version.CONCLUSION: The ATS has shown an acceptable level of overall reliability in the emergency department, but it needs more development to reach an almost perfect agreement.

Oncologic safety of colonic stenting as a bridge to surgery in leftsided malignant colonic obstruction:Current evidence and prospects

Approximately 7%-29%of patients with colorectal cancer present with colonic obstruction.The concept of self-expandable metal stent(SEMS)insertion as a bridge to surgery(BTS)is appealing.However,concerns on colonic stenting possibly impairing oncologic outcomes have been raised.This study aimed to review current evidence on the short-and long-term oncologic outcomes of SEMS insertion as BTS for left-sided malignant colonic obstruction.For short-term outcomes,colonic stenting facilitates a laparoscopic approach,increases the likelihood of primary anastomosis without a stoma,and may decrease postoperative morbidity.However,SEMS-related perforation also increases local recurrence and impairs overall survival.Moreover,colonic stenting may cause negative oncologic outcomes even without perforation.SEMS can induce shear forces on the tumor,leading to increased circulating cancer cells and aggressive pathological characteristics,including perineural and lymphovascular invasion.The conflicting evidence has led to discordant guidelines.Well-designed collaborative studies that integrate both oncologic outcomes and data on basic research(e.g.,alteration of circulating tumors)are needed to clarify the actual benefit of colonic stenting as BTS.

Sociological Features of the Casualties from the 1996 M_S7.0 Lijiang Earthquake, Yunnan Province

A statistics analysis was conducted on the basic information about fatalities from the Lijiang MS7.0 earthquake in 1996. The factors include age, gender, educational background, occupation, cause of death, death place, etc. We found that even though deaths caused by the Lijiang earthquake had some common features with those from earthquakes in other places in China, it also showed some specific features because of the multiracial and economically-underdeveloped situation of Lijiang area. The results reached in the paper can serve as a reference for studying the features of casualties caused by future strong earthquakes in Yunnan, and for the delicate and human-oriented emergency treatment.

A Woman with Psychogenic Non-epileptic Seizures and Pelvic Mass

MOST cases of encephalitis are caused by viruses but a few have an immunological basis, such as paraneoplastic encephalitis, with specific antibodies identified. One recently characterized encephalitis caused by antibodies is anti-N- methyl-D-aspartate （NMDA） receptor encephalitis. It is a form of paraneoplastic limbic encephalitis associated with ovarian teratoma and has recently been described.The NMDA receptor mediates excitatory neurotransmission. It is important for synaptic plasticity, and thus for higher function such as learning and memory. This disorder results in prominent psychiatric symptoms followed by a rapid decline of the level of consciousness, central hypoventilation, seizures, involuntary movements and dysautonomia.

Removal of VOCs from Sudden Polluted Raw Water by Air Stripping

Volatile organic compounds （VOCs） are widely used in various industrial processes and generate water pollutions. VOCs removal from raw water is an important task for waterworks to guarantee drinking water security. The removal of VOCs such as chlorobenzene （CB） and ethylbenzene （EB） from raw water by air stripping was investigated under various conditions, inciuding the variation of temperature （5-30~C）, pH （3.5-10.5）, and air/water ratio （10-60）. The air stripping removal efficiency of VOCs decreased with VOCs concentration declining in water. And Henry＇s law constant was demonstrated as an indicator of the estimation of VOCs removal efficiency for air stripping. The effects of temperature and the ratio of air and water were found to play a great role in VOCs removal, but the effect of pH seemed to be negligible. This study demonstrates that air stripping provides a promising opportunity in removing VOCs in drinking water treatment, especially for the relatively high concentration of VOCs.

Preliminary effect of proximal femoral nail antirotation on emergency treatment of senile patients with intertrochanteric fracture

Objective： To retrospectively analyse the clinical outcome of emergency treatment of senile intertrochanteric fractures with proximal femoral nail antirotation （PFNA）. Methods： From September 2008 to March 2009, 35 senile patients with intertrochanteric fracture, aged from 65 to 92 years with an average age of 76.5 years, were treated with PFNA within 24 hours after injury. There were 10 type Ⅰ fractures, 19 type Ⅱand 6 type Ⅲ according to upgraded Evans-Jensen classification system. All patients were complicated with osteoporosis, and 19 patients had preexisting internal medical diseases. According to the rating scale of disease severity by the American Society of Anesthesiologists （ASA）, there were 9 grade Ⅰ, 14 grade Ⅱ, 8 grade Ⅲ, and 4 grade Ⅳ. Results： The duration for operation ranged from 45 to 73 minutes with an average of 57.6 minutes. The volume of blood loss during operation ranged from 50 to 120 ml with an average of 77.5 ml. Patients could ambulate 2-4 days after operation （mean 3.5 days）. Hospital stay was 4-7 days （mean 5.3 days）. Full weight bearing time was 10-14 weeks （mean 12.8 weeks）. During hospitalization period, there was no regional or deep infection, hypostatic pneumonia, urinary tract infection and bedsore except for 2 cases of urine retention. All cases were followed up with an average period of 12.3 months, and bone healing achieved within 15-18 weeks （mean 16.6 weeks）. No complications such as delayed union, coxa vara or coxa valga, screw breakage or backout occurred and only 2 cases had troehanter bursitis because of thin body and overlong end of the antirotated nail. According to the Harris grading scale, the results were defined as excellent in 21 cases, good in 9 cases and fair in 5 cases, with the excellent and good rates of 85.7%. Conclusion： The emergency treatment of senile intertrochanteric fracture with proximal femoral nail antirotation has the advantages of minimal invasion, easy manipulation, less blood loss, shorter length of stay, less complications, and the preliminary clinical effect is satisfactory.