This study presents a case of a girl of three year and 4 month old with ataxia and severe sen-sorineural hearing loss for 2 years.In order to improve hearing,she was hospitalized in the PUMC Hospital.Ge-netic testing performed found compound heterozygous variants of c.1186C＞T(p.P396S)and c.1357C＞T(p.R453W)in TWNK gene.After a multidisciplinary discussion of the case,the team suspected mitochondrial DNA depletion syndrome type 7(hepatocerebral type).The patient has shown nervous system impairment in-volvement but no evidence of liver dysfunction.The efficacy of cochlear implantation is uncertain and general anesthesia if applied will accelerate the progress of encephalopathy and might lead to multiple organ failure.Unsure of the perioperative safety,the parents of the girl did not chose the option of hearing intervention tempo-rarily,but chose oral symptomatic supportive treatment with coenzyme Q10,folate,levocarnitine,and complex vitamins as recommended.

Objective:To determine the median effective dose (ED 50) of remimazolam for preoperative sedation in pediatric patients of different ages. Methods:This was a prospective study. American Society of Anesthesiologists Physical Status classification I or Ⅱ pediatric patients, aged 1-6 yr, scheduled for elective surgery in our hospital from July to December 2023, in whom the preoperative anxiety was still not relieved after non-drug intervention (preoperative separation anxiety score [PSAS]≥3), were selected. According to the age, the children were divided into 1-<2 yr group, 2-<3 yr group, 3-<4 yr group, 4-<5 yr group and 5-6 yr group. A child's PSAS score = 1 at the time of separation from parents was classified as satisfactory sedation, and a PSAS score ≥ 2 was classified as unsatisfactory sedation. The initial dose of remimazolam in each group was 0.3 mg/kg, dose ratio 1.15. If the child was satisfactorily sedated, the next patient received a lower dose of remimazolam, or conversely if the child was not satisfactorily sedated, a higher dose was given in the next patient. The test was ended when 7 alternating waveforms appeared. The Dixon-Massey method was used to calculate the ED 50 and 95% confidence interval. Results:In 1-<2 yr group, 2-<3 yr group, 3-<4 yr group, 4-<5 yr group and 5-6 yr group, a total of 120 children were included in this study, with 26, 23, 21, 27 and 23 cases, respectively, and the ED 50 (95% confidence interval) of remimazolam for preoperative sedation was 0.152 (0.126-0.178), 0.159 (0.135-0.183), 0.171 (0.147-0.196), 0.150 (0.126-0.174), and 0.146 (0.121-0.170) mg/kg, respectively. There was no significant difference between the groups ( P>0.05). Conclusions:The ED 50 of remimazolam for preoperative sedation is 0.152, 0.159, 0.171, 0.150 and 0.146 mg/kg for every 1 yr in children aged 1-6 yr, and the age factor does not affect the preoperative sedative effect of remimazolam in children of this age group.

Pyroptosis， an atypical new cell death mode other than apoptosis and necrosis， has been discovered in recent years. Pyroptosis depends on the cleavage of gasdermins （GSDMs） by Caspases. The activated GSDMs act on the plasma membrane to form a perforation， which results in cell lysis and triggers inflammation and immune response. Pyroptosis can be induced by four distinct signaling pathways， including canonical and non-canonical inflammasome pathways， apoptosis-associated Caspases-mediated pathway， and granzyme pathway. In these signaling pathways， GSDMs are the executors of pyroptosis. Pyroptosis is associated with the death of tumor cells and the inflammatory damage of normal tissues. Recent studies have demonstrated that moderate pyroptosis can lead to tumor cell death to exert an anti-tumor effect， and meanwhile stimulate the tumor immune microenvironment， while it can promote tumor development. Despite the good performance， drug-based anti-tumor therapies such as tumor immunotherapy， chemotherapy， and targeted therapy have some shortcomings such as drug resistance， recurrence， and damage to normal tissues. The latest research shows that a variety of natural compounds have anti-tumor effects in the auxiliary treatment of tumors by mediating the pyroptosis pathways in a multi-target and multi-pathway manner， which provide new ideas for the study of anti-tumor therapy. We reviewed the molecular mechanism of pyroptosis and the regulatory role of pyroptosis in tumors and tumor immune microenvironment， and summarized the recent research progress in the natural medicinal components regulating pyroptosis in anti-tumor therapy， with a view to providing ideas for the research on the anti-tumor therapy based on pyroptosis.

Objective:To evaluate the effect of age factors on the pharmacodynamics of intranasal dexmedetomidine for sedation in the pediatric patients undergoing transthoracic echocardiography(TTE).Methods:American Society of Anesthesiologists Physical Status classification Ⅰ-Ⅲ pediatric patients, aged 1-24 months, undergoing TTE from August 2019 to May 2022, were selected. This trial was performed in two parts. Part Ⅰ Pediatric patients were divided into 4 age groups: 1-6 month group, 7-12 month group, 13-18 month group and 19-24 month group. The initial dose of dexmedetomidine was 2.0 μg/kg in 0.1 μg/kg increment/decrement. The dose of dexmedetomidine was determined by using modified Dixon′s up-and-down method. The ED 50 and 95% confidence interval of intranasally administered dexmedetomidine for sedation were calculated by the Dexon-Massey method. Part Ⅱ One hundred patients were divided into 4 age groups ( n= 25 each): 1-6 month group, 7-12 month group, 13-18 month group and 19-24 month group. The 4 groups were further divided into 5 subgroups ( n=5 each) according to the dose of dexmedetomidine: 2.1 μg/kg subgroup, 2.2 μg/kg subgroup, 2.3 μg/kg subgroup, 2.4 μg/kg subgroup, and 2.5 μg/kg subgroup. Part Ⅰ and part Ⅱ trials were combined, and the ED 95 and 95% confidence interval of intranasally administered dexmedetomidine for sedation were calculated using the probit method. Results:A total of 220 pediatric patients were enrolled. There was no significant difference in ED 50 and ED 95 of dexmedetomidine intranasally administered for sedation among groups ( P>0.05). Conclusions:The pharmacodynamics of intranasal dexmedetomidine for sedation shows no significant difference in age in the pediatric patients aged 1-24 months undergoing TTE.

Hepatitis B virus (HBV) infection is closely associated with the adverse events such as liver cirrhosis, liver cancer, and liver failure and remains a serious threat to human health. Pegylated interferon is an indispensable drug for the treatment of chronic hepatitis B (CHB), and interferon-stimulated genes are associated with a variety of viruses, but few studies have mentioned their association with hepatitis B and their predictive effect after the treatment of hepatitis B with interferon. This article introduces the predictive factors for interferon treatment of CHB and summarizes the association of interferon-stimulated genes with hepatitis B and their predictive effect, so as to provide a reference for clinical work and basic research.

Abstract： Arsenic and arsenic compounds have been listed as one of the toxic and harmful environment pollutants, and drinking, seafood intake, use of skincare products and inhalation of tobacco smoke are main routes of exposure to human arsenic exposure. The adverse effects of arsenic on pregnant outcomes have been paid much attention. Prenatal exposure to high-level arsenic has been found to increase the risk of spontaneous abortion among pregnant women. Based on national and international epidemiological studies on the correlation between arsenic exposure and spontaneous abortion during the period between 1992 and 2020, we review the association between arsenic exposure and spontaneous abortion and describe the mechanisms underlying spontaneous abortion caused by arsenic exposure, so as to provide insights into early prevention of spontaneous abortion.

Background::Pre-operative assessment with high-resolution magnetic resonance imaging (MRI) is useful for assessing the risk of local recurrence (LR) and survival in rectal cancer. However, few studies have explored the clinical importance of the morphology of the anterior mesorectum, especially in patients with anterior cancer. Hence, the study aimed to investigate the impact of the morphology of the anterior mesorectum on LR in patients with primary rectal cancer.Methods::A retrospective study was performed on 176 patients who underwent neoadjuvant treatment and curative-intent surgery. Patients were divided into two groups according to the morphology of the anterior mesorectum on sagittal MRI: (1) linear type: the anterior mesorectum was thin and linear; and (2) triangular type: the anterior mesorectum was thick and had a unique triangular shape. Clinicopathological and LR data were compared between patients with linear type anterior mesorectal morphology and patients with triangular type anterior mesorectal morphology.Results::Morphometric analysis showed that 90 (51.1%) patients had linear type anterior mesorectal morphology, while 86 (48.9%) had triangular type anterior mesorectal morphology. Compared to triangular type anterior mesorectal morphology, linear type anterior mesorectal morphology was more common in females and was associated with a higher risk of circumferential resection margin involvement measured by MRI (35.6% [32/90] vs. 16.3% [14/86], P = 0.004) and a higher 5-year LR rate (12.2% vs. 3.5%, P = 0.030). In addition, the combination of linear type anterior mesorectal morphology and anterior tumors was confirmed as an independent risk factor for LR (odds ratio = 4.283, P = 0.014). Conclusions::The classification established in this study was a simple way to describe morphological characteristics of the anterior mesorectum. The combination of linear type anterior mesorectal morphology and anterior tumors was an independent risk factor for LR and may act as a tool to assist with LR risk stratification and treatment selection.

BACKGROUND: Postoperative chylous ascites is an infrequent condition after colorectal surgery and is easily treatable. However, its effect on the long-term oncological prognosis is not well established. This study aimed to investigate the short-term and long-term impact of chylous ascites treated with neoadjuvant therapy followed by rectal cancer surgery and to evaluate the incidence of chylous ascites after different surgical approaches. METHODS: A total of 898 locally advanced rectal cancer patients treated with neoadjuvant chemoradiotherapy followed by surgery between January 2010 and December 2018 were included. The clinicopathological data and outcomes of the patients with chylous ascites were compared with those of the patients without chylous ascites. The primary endpoint was recurrence-free survival (RFS). To balance baseline confounders between groups, propensity score matching (PSM) was performed for each patient with a logistic regression model. RESULTS: Chylous ascites was detected in 3.8% (34/898) of the patients. The incidence of chylous ascites was highest after robotic surgery (6.9%, 6/86), followed by laparoscopic surgery (4.2%, 26/618) and open surgery (1.0%, 2/192, P = 0.021). The patients with chylous ascites had a significantly higher number of lymph nodes harvested (15.6 vs. 12.8, P = 0.009) and a 3-day longer postoperative hospital stay (P = 0.017). The 5-year RFS rate was 64.5% in the chylous ascites group, which was significantly lower than the rate in the no chylous ascites group (79.9%; P = 0.007). The results remained unchanged after PSM was performed. The chylous ascites group showed a nonsignificant trend towards a higher peritoneal metastasis risk (5.9% vs. 1.6%, P = 0.120). Univariate analysis and multivariate analysis confirmed chylous ascites (hazard ratio= 3.038, P < 0.001) as an independent negative prognostic factor for RFS. CONCLUSIONS Considering the higher incidence of chylous ascites after laparoscopic and robotic surgery and its adverse prognosis, we recommend sufficient coagulation of the lymphatic tissue near the vessel origins, especially during minimally invasive surgery.
Chylous Ascites/etiology* ; Humans ; Incidence ; Laparoscopy ; Rectal Neoplasms/surgery* ; Retrospective Studies ; Risk Factors ; Robotic Surgical Procedures/adverse effects*

Objective:To investigate the effects of subanesthetic concentration of sevoflurane on the plasticity of dendritic spines in the prefrontal cortex neurons of juvenile rats.Methods:Thirty-six clean-grade male Sprague-Dawley rats, aged 24 days, weighing 50-60 g, were divided into control group (group C) and sevoflurane anesthesia group (group S) using a random number table method, with 18 rats in each group.Group S inhaled 1.2% sevoflurane and 50% oxygen (flow rate 1 L/min) for 3 h, while group C inhaled 50% oxygen (flow rate 1 L/min) for 3 h. Open-field test and Morris water maze test were performed at 3 days after anesthesia.Animals were sacrificed, and brain samples were then taken for determination of the number of apoptotic neurons in layer Ⅱ-Ⅲ of the prefrontal cortex, density of dendritic spines, and expression of postsynaptic density protein 95 and gephyrin by TUNEL staining, Golgi staining or Western blot.Results:Compared with group C, no significant change was found in total distance or time of staying at the central region in the open-field test or the average swimming velocity, escape latency or the number of apoptotic neurons in the Morris water maze test ( P>0.05), and the density of dendritic spines was significantly increased, and the expression of postsynaptic density protein 95 and gephyrin was up-regulated in group S ( P<0.05). Conclusion:Subanesthetic concentration of sevoflurane can enhance the plasticity of dendritic spines in the prefrontal cortex neurons of juvenile rats.

Objective: To explore the effect of health education based on behavioral phased transformation theory in remote peritoneal dialysis patients in remote mountainous areas. Methods: Totally 84 cases of peritoneal dialysis catheterization in our hospital from April 2016 to May in -2018 were selected as the research subjects. After discharge, home peritoneal dialysis was performed. According to the random number table method, 42 cases in each group were divided into observation group and control group. The control group was given routine treatment and traditional health education. On the basis of conventional treatment, the observation group established a behavior based phased transformation of health education as a means of intervention. The two groups were all intervened for 6 months. Volume status and self-care behavior were evaluated and compared between the two groups within one week of admission and six months after intervention. Results: Before intervention, there was no significant difference in the capacity load between the observation group and the control group (P>0.05). The systolic and diastolic blood pressures of the observation group after intervention were (131.21±11.73) mmHg (1 mmHg=0.133kPa) and (80.24 ±10.62) mmHg respectively, which were significantly lower than those of the control group (140.31 ± 12.87) mmHg and (85.75 ± 11.73) mmHg, with significant differences (t=3.455, 2.302, P < 0.05); and the ultrafiltration volume of the observation group after intervention was (385.15 ± 105.1.8) ml was significantly higher than that of the control group (320.02±126.87) ml, with significant difference (t=2.561, P < 0.05); there was no significant difference in body mass and urine volume between the two groups after intervention (P > 0.05).The total score of the self-care ability scale in the observation group was (122.11 ± 8.52) points, which was significantly higher than that in the control group (94.24 ± 10.71) points. The difference was statistically significant (t=313.198, P<0.01) . Conclusion Health education based on the theory of behavioral phased transformation can help patients with peritoneal dialysis in remote mountainous areas to improve their capacity and improve their self-protection behavior, which has educational guiding significance.