Sialidosis is a rare lysosomal storage disease caused by NEU1 gene mutation at 6p21.33. It is characterized by myoclonic, ataxia, epilepsy, and decreased vision. A pair of twins with sialidosis type 1 are reported to enrich clinicians ′ understanding of the disease, so as to improve the diagnosis and treatment. The proband was a 16-year-old male. The main symptom was intermittent limb involuntary trembling for 2 years, with paroxysmal loss of consciousness. Fundus examination showed cherry-red spots. His twin brother had similar symptoms, but the overall performance was mild. Whole exome sequencing results showed that both patients carried compound heterozygous mutations of c.239C>T (p.P80L) and c.803A>G (p.Y268C) in NEU1 gene, which were from their normal phenotype mother and father.

A novel structural dynamics test method and device were designed to test the biomechanical effects of dynamic axial loading on knee cartilage and meniscus. Firstly, the maximum acceleration signal-to-noise ratio of the experimental device was calculated by applying axial dynamic load to the experimental device under unloaded condition with different force hammers. Then the experimental samples were divided into non-specimen group (no specimen loaded), sham specimen group (loaded with polypropylene samples) and bovine knee joint specimen group (loaded with bovine knee joint samples) for testing. The test results show that the experimental device and method can provide stable axial dynamic load, and the experimental results have good repeatability. The final results confirm that the dynamic characteristics of experimental samples can be distinguished effectively by this device. The experimental method proposed in this study provides a new way to further study the biomechanical mechanism of knee joint structural response under axial dynamic load.
Animals ; Cattle ; Biomechanical Phenomena ; Knee Joint/physiology* ; Meniscus ; Mechanical Phenomena ; Weight-Bearing

Objective:To summarize the practice and experience of medical service support for Shenzhou manned spaceflight mission, and to explore the accurate medical service support strategy with new regions and new types.Methods:A systematic summary was made from the force command, action framework, technical support, and the practice and experience in the application of medical support force for Shenzhou manned flight mission were sorted out.. Furthermore, the key and difficult problems of the new domains with new types of military medical service support were analyzed, and the scheme and significance of establishing the accurate support mode of the new medical service support force were proposed.Results:The Shenzhou emergency rescue mission was an elite combat supported by a large platform. It was an important basis to realize accurate medical service support by the experience of utilizing the medical force of Shenzhou emergency rescue and the strengthening of war-building mode optimization.Conclusions:This paper systematically summarizes the experience and characteristics of medical force application in Shenzhou manned spaceflight missions, and provides new ideas and methods for future diversified and accurate s medical service support with new regions and new types.

Objective:To compare the clinical efficacy of lateral-rectus approach and ilioinguinal approach in the treatment of Torode-Zieg type IV pelvic fracture in toddlers.Methods:A retrospective cohort study was used to analyze the clinical data of 12 toddlers with Torode-Zieg type IV pelvic fracture admitted to Affiliated Hospital of Youjiang Medical College for Nationalities and Third Affiliated Hospital of Southern Medical University from June 2012 to June 2019. There were 6 males and 6 females, aged 13-36 months [(23.9±7.4)months]. Treatment via ilioinguinal approach was performed for 5 patients (ilioinguinal group), and via lateral-rectus approach for 7 patients (lateral-rectus group). Operation duration, intraoperative blood loss and postoperative complications were compared between the two groups. At the last follow-up, pelvic function was evaluated by Majeed function score, and fracture reduction was evaluated according to Mears-Velyvis radiological evaluation criterion.Results:All patients were followed for 24-72 months [(46.0±18.5)months]. In ilioinguinal group and lateral-rectus group, the operation time was (295.0±95.3)minutes and (165.1±52.2)minutes, respectively ( P<0.05), and the intraoperative blood loss was (190.0±65.2)ml and (225.7±92.0)ml, respectively ( P>0.05). In ilioinguinal group, the development of bilateral iliac wings was asymmetric in 3 patients. In lateral-rectus group, fracture-dislocation of epiphyseal plate together with abnormal development of iliac wing occurred in 1 patient, but had no effects on the function of lower limbs. According to Majeed function score at the last follow-up, the pelvic function in ilioinguinal approach group was rated as excellent in 1 patient, good in 2, fair in 1 and poor in 1, with the excellent and good rate of 60%, while the pelvic function in lateral-rectus group was excellent in 4 patients, good in 1, fair in 2 and poor in none, with the excellent and good rate of 71% ( P>0.05). According to Mears-Velyvis radiological evaluation criterion at the last follow-up, the fracture reduction in ilioinguinal group was rated as satisfied in 3 patients and dissatisfied in 2 patients, with the satisfaction rate of 60%, while the fracture reduction in lateral-rectus group was satisfied in 7 patients and dissatisfied in none, with the satisfaction rate of 100% ( P>0.05). Conclusions:For Torode-Zieg type IV pelvic fracture in toddles, both surgical approaches can complete the reduction and fixation. However, the lateral-rectus approach has shorter operation time and less damage to the epiphyseal structure around the pelvis than the ilioinguinal approach.

Objective:To review and summarize the main problems that have occurred in the history of manned spaceflight worldwide and the experience of the medical rescue support for astronauts at the main landing site since the first manned spaceflight mission of the Shenzhou-5 in China in 2003, focus on the technical status and return characteristics during the construction of the space station since the Shenzhou-12, and formulate a targeted injury treatment plan to ensure the safety of astronauts.Methods:This article summarized the lessons of accidental injuries of astronauts in foreign aerospace history, especially in the space station stage, combined with the experience of medical support of astronauts in manned spaceflight in China, and put forward a series of organizations, plans and treatments according to the mission characteristics of long-term orbits and adjustments of the main landing sites of space station mission spacecraft.Results:On the basis of the original pre-cabin emergency and helicopter ICU comprehensive rescue platform, the emergency procedures were further optimized, and the treatment plans under different complex terrains and the principles of rapid treatment and evacuation of astronauts were formulated.Conclusions:The effective treatment of astronauts returning from the space station in various situations could be ensured by the comprehensive rescue plan for the medical rescue of the space station mission and the construction and organization of the helicopter rescue platform.

Objective:This study aims to analyze the characteristics and basic principles of emergency surgery risks and anesthesia care of medical support at the landing site for China’s taikonauts of the Shenzhou-12, and to summarize China’s experience in medical support at the landing site for manned spaceflight, and ensure supports in special environments such as an emergency return of manned spaceflight.Methods:This study was carried out through literature research on relevant reports on the emergency surgery risks and aids of domestic and foreign astronauts at the landing sites, and summaries of the experience in medical support for taikonauts of spacecrafts from Shenzhou-5 to Shenzhou-11 at the landing sites. At the same time, according to the characteristics of Shenzhou-12 such as the long on-orbit time, the adjustment in the landing area, the optimization of the mission mode, and new search and rescue power, a series of organization, pre-arranged planning, equipment allocation, and effective anesthesia treatment plan were proposed and inspected in practice.Results:Based on the original anesthesia care plan of medical support, the first-aid carrier was adjusted and modified, the first-aid procedure was optimized, a new generation of supraglottic airway opening tool, video laryngoscope, portable ultrasound, and other devices were added, and the anesthesia care plan at the landing site for manned spaceflight was formulated to provide strong support for the medical care of taikonauts that had stayed in the outer space for a long time.Conclusions:Upon the targeted improvement and process optimization, the anesthesia care plan of medical support for taikonauts of Shenzhen-12 in the landing area fully meets the anesthesia requirement of medical support in special environments such as the emergency return of the taikonauts that have stayed in the outer space for a long time under the new orbital altitude.

Objective : To study the neuropathological changes and process brain astrocyte microglia and neuron injury in rats at different time after mild blast-related traumatic brain injury(bTBI), and to investigate the neuroprotective potential of dietary supplementation with fish oil on mild bTBI rats. Methods : 54 newly weaned SD rats were randomly divided into control group(n=18), model group(n=18) and treatment group(n=18). The model group and treatment group were fed with ordinary diet and oil-rich diet for 33 days to establish the mild bTBI model by shock wave, respectively. The control group rats were fed with ordinary diet without shock wave injury. Results : Compared with control group, the weight of rats in the model group and treatment group decreased to a certain extent within 2 days after injury, and then recovered to the level before injury. After bTBI injury for 6 h, 24 h and 3 d, the number of GFAP positive staining astrocytes and vertebral cells in hippocampal area decreased in both model group and treatment group, whereas the number of activated microglia and apoptotic neurons in the hippocampus increased in a time-dependent manner in model group and treatment group. In addition, compared with the model group, the treatment group with oil-rich diet increased the number of astrocytes and pyramidal cells in the hippocampus after injury, and decreased the number of activated microglia and apoptotic neurons in the lesions area. Conclusion Pre-injury dietary supplementation with fish oil shows neuroprotective benefits in alleviating neurons injury and inhibiting neuroinflammatory response in a rat model of mild bTBI.

Adipose-derived mesenchymal stem cells (ASCs) are pluripotent adult mesenchymal stem cells that are expected to be ideal seed cells for bone tissue engineering(BTE) due to their biosecurity, abundance, and easy access. However, in comparison with bone marrow mesenchymal stem cells, ASCs possess a relatively limited osteogenic capacity and often require further induction. Advances on strategies for promoting osteogenic differentiation of ASCs are reviewed from following aspects: optimization of scaffolds, addition of bioactive factors or drugs, non-coding RNA regulation, and physical stimulation, so as to provide references for the use of ASCs in BTE.

Adipose-derived mesenchymal stem cells (ASCs) are pluripotent adult mesenchymal stem cells that are expected to be ideal seed cells for bone tissue engineering(BTE) due to their biosecurity, abundance, and easy access. However, in comparison with bone marrow mesenchymal stem cells, ASCs possess a relatively limited osteogenic capacity and often require further induction. Advances on strategies for promoting osteogenic differentiation of ASCs are reviewed from following aspects: optimization of scaffolds, addition of bioactive factors or drugs, non-coding RNA regulation, and physical stimulation, so as to provide references for the use of ASCs in BTE.

Objective:Summarize the experience of the medical rescue team at the main landing site of China's manned space mission, analyze the new characteristics of the Shenzhou 12 space station mission, and take corresponding countermeasures and measures to ensure the strong medical rescue guarantee for astronauts.Methods:Search the publications about astronaut medical aid domestic and abroad, summarize the rescue and medical aid experiences from Shenzhou 5 to Shenzhou 11. In consideration of prolonged on-orbit time, Location adjustment of the landing site and the new characteristics of the complex terrain, new targeted strategies were presented.Results:The astronauts flew in orbit for 90 days, and the main landing site and launch site are in the same area. The medical security includes three parts: launch section, running section and return section. Desert rescue model were added. Ten injuries were simulated and each injury first-aid procedure was standardized.Conclusion:After targeted improvement and optimization, the Shenzhou 12 astronauts medical rescue support program ensures the safety of the whole process, all-weather and all-terrain emergency and rear delivery of the astronauts in the new mission environment and complex terrain.