OBJECTIVE To explore the safety and efficacy of tocilizumab in the treatment of refractory anti-N-methyl-D-aspartate receptor encephalitis in children, and to provide reference for the treatment of this disease. METHODS The clinical manifestations, diagnosis and treatment, safety and efficacy of tocilizumab in a case of refractory anti-NMDAR encephalitis in Children’s Hospital, Zhejiang University School of Medicine were analyzed retrospectively, and the relevant literature was reviewed. RESULTS The clinical symptoms of the patient were not improved after 2 doses of methylprednisolone, immunoglobulin and 6 doses of rituximab. After 6 doses of tocilizumab, the modified Rankin scale(mRS) score was improved without adverse reactions. CONCLUSION Tocilizumab, as a escalation second-line immunotherapy, is effective and well tolerated in the treatment of refractory anti-NMDAR encephalitis. It is one of the potential therapeutic means.

A retrospective analysis was made on the clinical data and gene mutation characteristics of 2 children admitted to the Children′s Hospital of Zhejiang University School of Medicine for epilepsy without periventricular nodules caused by the ARF1 gene mutation from August 2023 to February 2024, and relevant literature was reviewed.Both patients presented with seizures and psychomotor retardation, and 1 of them was diagnosed with West syndrome.Whole exome sequencing confirmed that the 2 patients carried a missense mutation in the ARF1 gene (c.55C>A, p.R19S).Brain magnetic resonance imaging (MRI) of 2 patients revealed no obvious abnormalities.A summary analysis of 5 cases of ARF1 gene mutations reported in three foreign literatures showed that patients with ARF1 gene mutations usually presented with seizures, developmental delay, hypotonia, mental retardation, and motor stereotypies.MRI showed periventricular nodular heterotopia, corpus callosum dysplasia, subcortical white matter abnormalities, and delayed myelination.This study found for the first time that ARF1-related disorders can occur without significant brain structural malformations, indicating that there are inconsistencies in neuroimaging findings, adding valuable phenotypic information to this gene.The differences in imaging findings may be the result of genetic background or variation in ARF1-interacting proteins, or may be caused by altered regulatory mechanisms of protein activity.

Objective:To investigate the effects of periacetabular osteotomy (PAO) combined with hip arthroscopy in treating adult developmental dysplasia of the hip (DDH).Methods:A total of sixty-one patients with DDH who received PAO surgery were enrolled in the present retrospective study. Of all patients, forty of them were treated by PAO combined with open arthrotomy from December 2015 to June 2018 as arthrotomy group. There were three males and 37 females in this group. Twenty-one DDH patients, including 2 males and 19 females, were treated by PAO combined with hip arthroscopy from July 2018 to June 2019 as arthroscopy group. The average ages were 31.08±11.24 and 33.25±11.70 years, respectively. Radiological parameters before and after surgery were compared between two groups. Modified Harris hip score (mHHS), nonarthritic hip score (NAHS), hip outcome score activity of daily living scale (HOS-ADL) at 6 months and 12 months postoperatively were compared between two groups.Results:The preoperative LCE (lateral center edge) angle, ACE (anterior center edge) angle, T?nnis angle, extrusion index in each group were 8.53°±9.09° vs. 9.15°±10.86°, 13.69°±12.43° vs. 7.18°±15.74°, 20.12°±6.24° vs. 22.13°±8.86° and 38.97%±7.55% vs. 37.64%±10.01% respectively. There was no statistical difference between two groups. Cam deformity (α angle >50°) existed in 17 patients (81%, 17/21) in arthroscopy group and in 31 patients (78%, 31/40) in arthrotomy group without significant difference between two groups (χ 2=0.096, P=0.756). At 12 months follow-up, all radiological parameters were improved from those before surgery. The LCE angle, ACE angle, T?nnis angle, extrusion index were 34.29°±5.07° vs. 32.76°±9.48°, 32.87°±4.23° vs. 30.26°±5.39°, -1.82°±5.88° vs. 2.16°±7.89° and 16.81%±4.53% vs. 18.20%±9.16% between groups without significant difference. The preoperative mHHS, NAHS, HOS-ADL in two groups were 60.38±12.19, 50.90±8.54, 72.23±11.86 and 60.00±13.53, 52.55±7.92, 72.70±12.18 respectively. At 12 months, there was no statistical difference in mHHS between the two groups (87.90±4.34 vs. 86.50±5.11, t=1.135, P=0.261). The NAHS and HOS-ADL in arthroscopy group were higher than those in arthrotomy group at 12 months follow-up (88.71±4.49 vs. 82.17±5.23, t=6.066, P<0.001; 90.33±2.71 vs. 87.12±3.33, t=3.800, P<0.001). Conclusion:During PAO in treating DDH, better short-term clinical results could be achieved when it combined with hip arthroscopy.

Objective: To investigate the effects of capsular repair versus that of unrepaired capsulotomy during hip arthroscopy in treating femoracetabular impingement (FAI). Methods: A total of 102 cases with FAI who underwent hip arthroscopy from July 2014 to August 2017 were collected retrospectively. Patients were divided into unrepaired group (64 cases) and repair group (38 cases) according to capsular management strategies. In the repair group, in addition to the management of osteochondroplasty of pincer and cam deformity and labral repair, the capsulotomies were routinely repaired. Modified Harris hip score (mHHS), nonarthritic hip score (NAHS), and hip outcome score activity of daily living scale (HOS-ADL) were used to evaluate the clinical outcomes. The preoperative hip scores and those at 1 year postoperative follow up were collected. Operation duration and occurrence of complication were also collected. Results: There were 28 males and 36 females in the unrepaired group, while 17 males and 21 females were in the repair group. The average age were 38.59±11.27 years and 37.64±11.10 years respectively. There were 18 pincer deformities, 12 cam deformities and 34 mixed deformities in the unrepaired group, while 7 pincer deformities, 7 cam deformities and 24 mixed deformities in the repair group. There was no difference in gender, average age and impingement type between two groups (P>0.05). The preoperative mHHS score, NAHS score and HOS-ADL score were 59.56±12.44, 58.79±12.90 and 64.11±14.43 in the unrepaired group. These scores elevated to 85.52±4.69, 80.76±5.27 and 86.08±5.85 at 1 year follow up. The preoperative mHHS score, NAHS score and HOS-ADL score were 61.68±12.41, 60.62±10.48 and 65.27±17.47 in the repair group. These scores elevated to 88.84±3.46, 89.24±3.94 and 91.03±3.97 at 1 year follow up. The scores in the repair group were higher than those in unrepaired group at 1 year follow up (P<0.05). The average operation duration was 137.62±31.21 minutes and 151.35±31.55 minutes (P<0.05), respectively. The complication occurrence rate in unrepaired group was 31% (20/64) and 24% (9/38) in repaired group with no statistical difference between two groups (P>0.05). Conclusion During hip arthroscopy in treating FAI, short-term clinical results could be achieved after routine repair of capsulotomy without increased risk of complication.

When facing a sudden danger or aversive condition while engaged in on-going forward motion, animals transiently slow down and make a turn to escape. The neural mechanisms underlying stimulation-induced deceleration in avoidance behavior are largely unknown. Here, we report that in Drosophila larvae, light-induced deceleration was commanded by a continuous neural pathway that included prothoracicotropic hormone neurons, eclosion hormone neurons, and tyrosine decarboxylase 2 motor neurons (the PET pathway). Inhibiting neurons in the PET pathway led to defects in light-avoidance due to insufficient deceleration and head casting. On the other hand, activation of PET pathway neurons specifically caused immediate deceleration in larval locomotion. Our findings reveal a neural substrate for the emergent deceleration response and provide a new understanding of the relationship between behavioral modules in animal avoidance responses.

Objective To study the effect of the preset temperature,heating time and the spacing of radio frequency electrodes.Methods Fresh egg white was used to study the effects of heater spacing,temperature and heating time.Two electrodes were fixed to keep the needles perpendicular to the center of egg white confined on a square plate.Temperatures from 50 to 90℃ were tested with heating times of 60 and 120 seconds and electrode spac ings of 2.0,1.0,0.5 and 0.25 centimetres.The real-time temperature and the time to the appearance of a spindleshaped bridge between the electrodes was observed.The egg white's impedance and resistance,the lowest temperature of denaturation,and the mass (or volume) denatured at different time points were also observed.Results With an electrode spacing of 2 cm and the thermostat set at 63℃,the true electrode temperatures were 63.7 and 52.6℃,with the secondary electrode the cooler.Significant differences between the secondary electrode temperature and the preset temperature as well as the temperature between the primary and the secondary electrodes were observed.It was also found that different electrode spacing caused significant differences between the preset temperature and the primary as well as the secondary electrode temperature.Denaturation began at 56℃ with a spacing of 0.25 cm.At spacings of 0.25 and 0.50 cm,a spindle-shaped connection between the two electrodes was observed at temperatures above 70℃,but higher temperature was required at 1.00 and 2.00 cm.When the spacing was 0.25 cm and the preset temperature was 75℃,the denatured volume after 60 s of heating was 90.21+0.64 mm3.Heating for 120 s denatured 95.08+ 0.53 mm3 and two 60.s heatings 92.88+0.74 mm3,all significant differences.There were no significant differences in the resistance of the egg white before and after a single heating for 60 s,but after a single heating lasting 120 s it had increased significantly from 128.41+8.04 to 121.29±8.04 Ω.Conclusion Bipolar radio frequency heating can denature egg white.Higher temperature,longer heating and smaller electrode spacing heat more effectively.

Objective To investigate the patellofemoral joint overstuffing in total knee replacement without patella resurfacing and try to define the incidence and the mechanism.Methods The imaging data of 117 patients (37 males and 80 females;55-73 years old with a mean age of 65.4±5.63) who underwent TKA operation without patella resurfacing for terminal osteoarthritis were analyzed retrospectively between 2005 to 2015.The DR X-ray system was used to get the full extension lateral films preand post-operatively (within one week) and to complete the digital measurement with the built-in PACS system.The involved parameters included patella thickness,anterior femur offset and patella femur distance pre-and post-operatively.The postoperative patellofemoral joint overstuffing was defined as the over 1 mm increase in the postoperative patella femur distance when compared with that preoperatively.The differences between the overstuffing group and non-overstuffing group and the characteristics between male group and female group were compared.Results The postoperative patella femur distance was 33.10± 1.56 mm,which was significantly thicker than that preoperatively (30.69± 1.45 mm).The anterior femur offset (6.93±0.36 mm) at postoperative was significantly larger than that at preoperative (4.50±0.92 mm).Postoperative patellofemoral joint overstuffing occurred in 79 patients with the incidence of 67.5％.There was no difference in the incidence of postoperative patellofemoral joint overstuffing among different manufacturers.In overstuffing group,the preoperative anterior femur offset was 3.89±0.21 mm,which was significantly less than that in none-overstuffing group (5.72±0.24 mm).However,there was no statistical difference between the two groups in preoperative patella thickness,postoperative patella thickness and postoperative anterior femur offset.The incidence of postoperative overstuffing in female group was 78.8％ (63/80),which was significantly higher than that in male group which was 43.2％ (16/37).The preoperative anterior femur offset (4.30±0.81 mm) in female was significantly less than that in man (4.94±1.00mm).The relative risk for postoperative overstuffing in female was 1.82.Conclusion Patellofemoral joint overstuffing occupies 67.5％ total knee replacement without patella resurfacing.Female patients have a higher incidence of patellofemoral joint overstuffing,which may be caused by the thinner anterior femoral offset in anatomy.

Objective: To investigate the efficacy and safety of using pegylated recombinant human granulocyte-colonystimulating factor (PEG-rhG-CSF) in preventing neutropenia in multiple chemotherapy cycles. Methods: A multicenter, prospective, open-label, singlearmstudy was designed. Patients with malignant tumors, such as lung, ovarian, and colorectal cancers, who received multiple cycles of chemotherapy with the prophylactic use of PEG-rhG-CSF for 2-4 consecutive cycles participated in the study. Results: After the prophylactic use of PEG-rhG-CSF, the incidence of grade IV neutropenia decreased from 4.76% (13/273) in the first cycle to 1.83% (5/273), 1.15% (2/174), and 2.08% (2/96) in subsequent cycles. Meanwhile, the incidence of grade III neutropenia decreased from 11.36% (31/ 273) in the first cycle to 6.23% (17/273), 2.87% (5/174), and 3.13% (3/96) in subsequent cycles. The incidence of febrile neutropenia (FN) during the first cycle was 0.73% (2/273). The duration of FN was 2 days in one case and 5 days in another case. FN was not observed during the second, third, or fourth cycle. After the secondary prophylactic use of PEG-rhG-CSF, the incidence of grade IV neutropenia decreased from 25% (7/28) to 3.57% (1/28), 0% (0/28), and 6.67% (1/15) in subsequent cycles. Meanwhile, the incidence of grade III neutropenia decreased from 71.43% (20/28) to 10.71% (3/28), 14.29% (4/28), and 0% (0/15) in subsequent cycles. The proportion of patients who received antibiotic therapy during the entire chemotherapy period was 10.48% (44/420). Conclusion: The application of PEG-rhG-CSF once per chemotherapy cycle can effectively reduce the occurrence of neutropenia in patients under multiple cycles of chemotherapy treatment with good safety.

Objective To explore the mediating effect of self-management behavior between nervous functional defects and health condition in patients with stroke.Methods A total of 100 patients with stroke chosen in Jianhu hospital affiliated to Nantong University from March to June 2015 were investigated using SSMBS,SSS and SRHMS through questionnaires after they left hospital for one year.The data was analyzed by stepwise regression analysis and Bootstrap procedure via SPSS13.0 and AMOS7.0.Results The total scores of SSS,SSMBS and SRHMS were (13.38±5.29),(162.59±21.43) and (278.13±51.48),respectively.Bivariate correlation analysis showed that there were correlations among self-management behavior,neurological impairment and self-rating health (r=-0.735,-0.568,0.693,P<0.01).The value of the mediating effect of self-management behavior was 0.137,accounting for 24.1% of the total effect.Bootstrap test showed 95% confidence interval of the direct mediating effect of self-management behavior was (0.006,0.208).Conclusions elf-management behavior has stronger mediating effect between nervous functional defects and health condition,it is a predictive factor of nervous functional defects and health condition.Medical workers should enhance the intervention of health self-management behavior for the patients with chronic diseases such as stroke.