Exosome-Transmitted miR-224-5p Promotes Colorectal Cancer Cell Proliferation via Targeting ULK2 in p53-Dependent Manner / 生物医学与环境科学（英文）

OBJECTIVE@#To investigate the role and molecular mechanism of exosomal miR-224-5p in colorectal cancer (CRC).@*METHODS@#The miR-224-5p expression in CRC patient tissues and cell-derived exosomes was measured by laser capture microdissection and qRT-PCR, respectively. Dual-luciferase reporter gene assay was used to determine the target gene of miR-224-5p. The protein expressions of p53 and unc-51 like kinase 2 (ULK2) in CRC cells were detected by western blot. Flow cytometry was used to detect cell cycle and apoptosis. Cell proliferation was measured by CCK8 and EdU assay.@*RESULTS@#The miR-224-5p expression was upregulated in CRC tissues and increased progressively with the rise of CRC stage. CRC cells secreted extracellular miR-224-5p mainly in an exosome-dependent manner, and then miR-224-5p could be transferred to surrounding tumor cells to regulate cell proliferation in the form of autocrine or paracrine. Moreover, ULK2 was characterized as a direct target of miR-224-5p and was downregulated in CRC tissues. Interestingly, ULK2 inhibited CRC cell proliferation in a p53-dependent manner. Furthermore, exosome-derived miR-224-5p partially reversed the proliferation regulation of ULK2 on CRC cells.@*CONCLUSION@#Our findings demonstrate that exosome-transmitted miR-224-5p promotes p53-dependent cell proliferation by targeting ULK2 in CRC, which may offer promising targets for CRC prevention and therapy.

Clinical Research Progress of L-carnitine in the Treatment of Primary Carnitine Deficiency / 医药导报

L-carnitine is a carrier that assist in transport of long-chain fatty acids into mitochondria and an effective drug for treating primary carnitine deficiency(PCD).Starting supplementation as early as possible before irreversible organ damage oc-cured can alleviate organ damage,reduce the risk of sudden death,and improve the quality of life.However,in recent years,some studies have suggested that long-term oral L-carnitine has potential risks,the metabolism of L-carnitine through intestinal flora re-sults in the production of trimethylamine oxide(TMAO),which can increase the risk of cardiovascular disease(CVD).There-fore,this paper summarizes the clinical research progress of L-carnitine in treating PCD to provide a theoretical basis for the clini-cal application of L-carnitine in PCD.

Research progress on the role of glutamine metabolism-related proteins in tumor metastasis / 中国癌症杂志

Tumor metastasis is closely related to high mortality rate of cancer.It is well known that glutamine plays an important role in the malignant progression of cancer.Notably,as an important carbon and nitrogen donor,glutamine has been found to be closely related to tumor metastasis in recent years.Glutamine is not only involved in regulating the proliferation of tumor cells,but is also closely related to the migration and invasion of tumor cells.Furthermore,various enzymes along with transporters in the metabolism of glutamine are involved in the process of tumor metastasis through different signaling pathways.This review provided a summary of the role of glutamine in tumor metastasis in recent years and proposed therapeutic targets to provide new strategies for the clinical treatment of tumor metastases.

Mechanism of Yantiao Fang regulating the Rho/ROCK signaling pathway in apoptosis of intestinal epithelial cells in mice with acute gastrointestinal injury caused by sepsis / 中国比较医学杂志

Objective To investigate the effect of Yantiao Fang on apoptosis of intestinal epithelial cells in mice with acute gastrointestinal injury caused by sepsis by regulating the Rho/ROCK signaling pathway.Methods Seventy BALB/c mice were randomly divided into normal,sham,and model groups.Except for normal and sham groups,mice were subjected to cecal ligation and perforation to establish a mouse model of acute gastrointestinal injury caused by sepsis.The mouse models were randomly divided into model,Low,Medium,and High dose of Yantiao Fang,and ROCK inhibitor groups.Histopathological changes of the ileum were observed by HE staining.Serum levels of IL-1β,IL-6,TNF-α,and IL-10 were measured by ELISA.PCNA and Ki-67 expression was detected by immunohistochemistry.Cleaved caspase3 and Bax expression was detected by Western blot.ROCK and MLC mRNA expression in the ileum was measured by RT-qPCR.Results Compared with normal and sham groups,Chiu's pathological score,proinflammatory factor(IL-1β,IL-6,and TNF-α)levels,cleaved caspase3 and Bax protein expression,and ROCK and MLC mRNA levels were increased in the model group(P＜0.05).Moreover,anti-inflammatory mediator IL-10 and expression of PCNA and Ki-67 in the ileum were decreased in the model group(P＜0.05).Compared with the model group,histopathological changes of the ileum in all Yantiao Fang groups were improved by various degrees with the increase in dose.Chiu's pathological score,IL-1β,IL-6,and TNF-α serum levels,cleaved caspase3 and Bax protein expression,and ROCK and MLC mRNA levels were decreased in Yantian Fang groups(P＜0.05).The IL-10 level and expression of PCNA and Ki-67 in the ileum were increased in Yantian Fang groups(P＜0.05).Conclusions Yantiao Fang may inhibit apoptosis of intestinal epithelial cells in mice with acute gastrointestinal injury due to sepsis by regulating the Rho/ROCK signaling pathway,thereby alleviating intestinal tissue inflammation and ultimately preventing intestinal mucosal tissue injury.

IgA deficiency allergic transfusion reaction and its prevention and treatment strategies / 中国输血杂志

IgA deficiency allergic transfusion reaction often occurs in patients with anti-IgA. When blood products containing IgA are transfused into these patients, it may lead to severe allergic transfusion reactions, which can be fatal in severe cases. This review summarizes the clinical manifestations and influencing factors of immunoglobulin A deficiency (IgAD), as well as the pathogenesis, laboratory test and prevention strategies of allergic transfusion reactions caused by IgA deficiency, so as to enhance prevention awareness of IgA deficiency allergic transfusion reaction in medical staff.

Blood security and management of transfusion-dependent thalassaemia (TDT) patients in Nanning: analysis of " collection-based-supply" blood management mode / 中国输血杂志

【Objective】 To retrospectively analyze the blood use of transfusion-dependent thalassemia (TDT) patients in 9 designated transfusion medical institutions from 2018 to 2023 in Nanning, and to evaluate the effect of " three designated " blood transfusion mode (hereby means TDT patients undergoing blood transfusion in designated transfusion medical institutions regularly) and " collection-based-supply" blood management mode on blood security of TDT patients. 【Methods】 The " three designated" blood transfusion mode was implemented to ensure that TDT patients registered in the local household registration (referred to as the " register" ) obtain the rights and interests of outpatient transfusion and blood security of designated medical institutions. The " collection-based-supply" blood management mode was implemented to assess the blood needs of "register" TDT patients and meet their needs to the maximum extent according to the blood inventory (collection). 【Results】 From 2018 to 2023, the total blood supply of "register" TDT patients was 10.37% of the total red blood supply of all medical institutions (138 509.5 U /1 335 788.0 U), with the highest proportion of type O blood as 46.34% (64 181.0 U/138 509.5 U) and the lowest proportion of type AB blood as 3.85% (5 331.0 U/138 509.5 U). In 2018, 9 transfusion medical institutions were designated for TDT patients.There were a total of 766 TDT patients in the register, with the per capita annual blood transfusion volume increased from 20.28 U (15 531.0 U/766 patients) in 2018 to 36.01 U (27 586.0 U/766 patients) in 2023, maintaining a positive growth every year(30.26%, 4.94%, 11.71%, 8.61%, 4.94% and 7.10%). 【Conclusion】 The " three designated" blood transfusion mode and the " collection-based-supply " blood management mode can effectively guarantee the blood supply of TDT patients.

Realationship between personality impulsivity and aggressivity in adolescents： the effecting path of sibling relationships / 四川精神卫生

BackgroundIn China， the structure shift from just one-child family to both one-child and more-than-one-child families is happening. Exploring how the sibling relationships effect between adolescent personality impulsivity and aggressivity is of great significance for promoting adolescent mental health as well as maintaining social harmony and stability. ObjectiveTo investigate the effecting path of sibling relationships between personality impulsivity and aggressivity in adolescents， so as to provide references for the prevention of violent and aggressive behavior in adolescents. MethodsFrom February to April， 2023， a total of 1 200 students with sibling relationships from 12 primary and secondary schools in a county of Sichuan province were included by random sampling. Barratt Impulsivity Scale （BIS-11）， Chinese Version of Buss & Perry Aggression Questionnaire （AQ-CV）， and Sibling Relationship Questionnaire （SRQ） were used for cross-sectional investigation. Pearson Correlation Analysis was used to analyze the correlation between the scores of these scales. Bootstrap method was used to test the effecting path of sibling relationships between personality impulsivity and aggressivity. ResultsThe total score of BIS-11 was positively correlated with that of AQ-CV as well as the scores of conflict and competition dimensions in SRQ （r=0.485、0.276、0.280，P<0.01）， while negatively correlated with the score of warmth/intimacy dimension in SRQ （r=-0.383， P<0.01）. The total score of AQ-CV was positively correlated with the scores of conflict and competition dimensions in SRQ （r=0.387， 0.340， P<0.01）， while negatively correlated with the score of warmth/intimacy dimension in SRQ （r=-0.304， P<0.01）. Within SRQ， negative correlations could be observed between the score of warmth/intimacy and scores of conflict and competition （r=-0.307， -0.375， P<0.01）， whereas positive correlation could be observed between the score of conflict and that of competition （r=0.267， P<0.01）. The total effect of personality impulsivity level on aggressivity level in adolescent was 0.480 （P<0.01）. Sibling relationships played a mediating role between personality impulsivity and aggressivity in adolescents. Meanwhile， the indirect effect values of warmth/intimacy， conflict and competition were 0.054， 0.075 and 0.062， with the effect values accounting for 11.21%， 15.70% and 12.93%， respectively. ConclusionThe personality impulsivity of adolescents can directly affect their aggressivity， and sibling relationships may act as an important channel connecting personality impulsivity and aggressivity. ［Funded by 2023 Project of the Psychology and Behavioral Science Research Center of the Deyang Federation of Social Sciences （number， XLYXW2023305）］

Effect of Shugan Tiaoshen acupuncture combined with western medication on depression-insomnia comorbidity due to COVID-19 quarantine: a multi-central randomized controlled trial / 中国针灸

OBJECTIVE@#To observe the effect of Shugan Tiaoshen acupuncture (acupuncture for soothing the liver and regulating the mentality) combined with western medication on depression and sleep quality in the patients with depression-insomnia comorbidity due to COVID-19 quarantine, and investigate the potential mechanism from the perspective of cortical excitability.@*METHODS@#Sixty patients with depression-insomnia comorbidity due to COVID-19 quarantine were randomly divided into an acupuncture group and a sham-acupuncture group, 30 cases in each one. The patients of both groups were treated with oral administration of sertraline hydrochloride tablets. In the acupuncture group, Shugan Tiaoshen acupuncture was supplemented. Body acupuncture was applied to Yintang (GV 24+), Baihui (GV 20), Hegu (LI 4), Zhaohai (KI 6), Qihai (CV 6), etc. The intradermal needling was used at Xin (CO15), Gan (CO12) and Shen (CO10). In the sham-acupuncture group, the sham-acupuncture was given at the same points as the acupuncture group. The compensatory treatment was provided at the end of follow-up for the patients in the sham-acupuncture group. In both groups, the treatment was given once every two days, 3 times a week, for consecutive 8 weeks. The self-rating depression scale (SDS) and insomnia severity index (ISI) scores were compared between the two groups before and after treatment and 1 month after the end of treatment (follow-up) separately. The cortical excitability indexes (resting motor threshold [rMT], motor evoked potential amplitude [MEP-A], cortical resting period [CSP]) and the level of serum 5-hydroxytryptamine (5-HT) were measured before and after treatment in the two groups.@*RESULTS@#After treatment and in follow-up, SDS and ISI scores were decreased in both groups compared with those before treatment (P<0.05), and the scores in the acupuncture group were lower than those in the sham-acupuncture group (P<0.05), and the decrease range in the acupuncture group after treatment was larger than that in the sham-acupuncture group (P<0.05). After treatment, rMT was reduced (P<0.05), while MEP-A and CSP were increased (P<0.05) in the acupuncture group compared with that before treatment. The levels of serum 5-HT in both groups were increased compared with those before treatment (P<0.05). The rMT in the acupuncture group was lower than that in the sham-acupuncture group, while MEP-A and CSP, as well as the level of serum 5-HT were higher in the acupuncture group in comparison with the sham-acupuncture group (P<0.05).@*CONCLUSION@#Shugan Tiaoshen acupuncture combined with western medication can relieve depression and improve sleep quality in the patients with depression-insomnia comorbidity due to COVID-19 quarantine, which is probably related to rectifying the imbalanced excitatory and inhibitory neuronal functions.

Research progress of the regulatory role of autophagy in metabolic liver diseases / 中华肝脏病杂志

Autophagy is one of several hepatic metabolic processes in which starved cells are supplied with glucose, free fatty acids, and amino acids to produce energy and synthesize new macromolecules. Moreover, it regulates the quantity and quality of mitochondria and other organelles. As the liver is a vital metabolic organ, specific forms of autophagy are necessary for maintaining liver homeostasis. Protein, fat, and sugar are the three primary nutrients that can be altered by different metabolic liver diseases. Drugs that have an effect on autophagy can either promote or inhibit autophagy, and as a result, it can either increase or inhibit the three major nutritional metabolisms that are affected by liver disease. Thus, this opens up a novel therapeutic option for liver disease.

Analysis of clinical characteristics and ACADM gene variants in four children with Medium chain acyl-CoA dehydrogenase deficiency / 中华医学遗传学杂志

OBJECTIVE@#To explore the clinical and genetic characteristics of four patients with medium-chain acyl-CoA dehydrogenase deficiency (MCADD).@*METHODS@#Four children who had presented at the Children's Hospital Affiliated to Zhengzhou University between August 2019 and August 2021 were selected as the study subjects. Clinical data of the children were collected. The children were subjected to whole exome sequencing (WES).@*RESULTS@#All of the four children were diagnosed with MCADD. Blood amino acid and ester acyl carnitine spectrum test showed that the concentration of octanoyl carnitine (C8) was significantly increased. The main clinical manifestations included poor mental response (3 cases), intermittent diarrhea with abdominal pain (1 case), vomiting (1 case), increased transaminase (3 cases), and metabolic acidosis (2 cases). Five variants were identified by genetic testing, among which c.341A>G (p.Y114C) was unreported previously. Three were missense variants, one was frameshift variant and one was splicing variant.@*CONCLUSION@#The clinical heterogeneity of MCADD is obvious, and the severity of the disease may vary. WES can assist with the diagnosis. Delineation of the clinical symptoms and genetic characteristics of the disease can facilitate early diagnosis and treatment of the disease.

Clinical characteristics and genetic analysis of a child with Galactosemia due to compound heterozygous variants of GALT gene / 中华医学遗传学杂志

OBJECTIVE@#To explore the clinical features and genetic basis of a child with Galactosemia.@*METHODS@#A child who had presented at the Children's Hospital Affiliated to Zhengzhou University on November 20, 2019 was selected as the study subject. Clinical data of the child was collected. Whole exome sequencing was carried out for the child. Candidate variants were validated by Sanger sequencing.@*RESULTS@#Clinical manifestations of the child have included anemia, feeding difficulty, jaundice, hypomyotonia, abnormal liver function and coagulation abnormality. Tandem mass spectrometry showed increased citrulline, methionine, ornithine and tyrosine. Urine organic acid analysis showed increased phenyllactic acid, 4-hydroxyphenylacetic acid, 4-hydroxyphenyllactic acid, 4-hydroxyphenylpyruvate and N-acetyltyrosine. Genetic testing revealed that the child has harbored compound heterozygous variants of the GALT gene, namely c.627T>A (p.Y209*) and c.370G>C (p.G124R), which were respectively inherited from her healthy parents. Among these, c.627T>A (p.Y209*) was known as a likely pathogenic variant, while c.370G>C (p. G124R) was unreported previously and also predicted as a likely pathogenic variant(PM1+PM2_Supporting+PP3_Moderate+PPR).@*CONCLUSION@#Above discovery has expanded the spectrum of the GALT gene variants underlying Galactosemia. Patients with thrombocytopenia, feeding difficulties, jaundice, abnormal liver function and coagulation abnormality without obvious causes should be analyzed by screening of metabolic diseases in combination with genetic testing.

Exploring occupational hazards and protective strategies in power generation enterprises / 中国职业医学

Currently, power generation in China is dominated by thermal power, wind power, nuclear power, and hydropower enterprises. The power source mainly comes from thermal power generation. The occupational hazards in thermal power station are noise, high temperature, power frequency electric fields, dust, and chemical toxins and so on, with noise and dust (silica and coal dust) being the primary factors. The occupational hazards in wind power station are noise, power frequency electric fields, high temperature, low temperature, and chemical toxins (sulfur hexafluoride, toluene, styrene, etc.), with noise and power frequency electric fields being the major concerns. The occupational radiation hazards in nuclear power station are gamma rays, beta rays, X-rays, neutrons, alpha rays, and radioactive aerosols. There is special attention in radiation protection but not enough protection in non-radioactive hazards such as noise, high temperature, and ammonia. The occupational hazards in hydropower station are noise, power frequency electric fields, vibration, radon and its de-composites, and chemical toxins, with noise and power frequency electric fields being the primary hazards. Different categories of power generation enterprises should identify key hazards and work site for occupational disease prevention and control based on the features of occupational hazards. Improving occupational health management and protection levels are essential measures.

Application of endoscopic suturing instrument in laparoscopic gastrojejunostomy / 中华胃肠外科杂志

Objective: To investigate the safety and feasibility of using an endoscopic suturing instrument in laparoscopic gastrojejunostomy. Methods: A descriptive case series study was conducted to retrospectively analyze the clinical data of 5 patients with gastric cancer who underwent laparoscopic distal gastrectomy (Billroth II + Braun anastomosis) at Tangdu Hospital, Air Force Medical University from October 2022 to January 2023. The common opening was closed using an endoscopic suturing instrument. The indications were as follows: (1) patients aged between 18 and 80 years; (2) patients with gastric adenocarcinoma; (3) cTNM between I-III; (4) lower-third gastric cancer and radical gastrectomy is recommended; (5) no history of upper abdominal surgery (except for laparoscopic cholecystectomy). The surgery was performed as follows: A side-to-side gastrojejunostomy was performed with endoscopic linear cutter stapler. Then the common opening was closed with endoscopic suturing instrument. During suturing and closing the common opening, a vertical mattress suture was used to completely invert and close the mucosa-to-mucosa and serosa-to-serosa of the gastric and jejunum walls. After the first layer of suture was completed, the seromuscular layer was sutured from top to bottom to embed the common opening of stomach and jejunum. Results: Laparoscopic closure of the common gastrojejunal opening with endoscopic suturing instrument was successfully completed in all 5 patients. The operative time was (308.6±22.6) minutes, while the time of gastrojejunostomy was (15.4±3.1) minutes. The operative blood loss was (34.0±10.8) ml. No intraoperative or postoperative complications occurred in any of the patients. The first passage of gas occurred at (2.6±0.9) days, and the postoperative hospital stay was (7.0±1.9) days. Conclusion: The application of endoscopic suturing instrument in laparoscopic gastrojejunostomy is safe and feasible.

Effect of functional magnetic stimulation at different frequencies on urination rehabilitation of patients with urinary retention after stroke / 实用医学杂志

Objective To analyze the effect of functional magnetic stimulation(FMS)at different frequen-cies on the recovery of urination function in patients with urinary retention after stroke.Methods A total of 200 patients with urinary retention after stroke admitted to our hospital from January 2020 to January 2022 were recruited as the research objects.They were divided into 5 Hz,10 Hz,20 Hz,and control groups by simple random method,with 50 patients in each group.All four groups were treated with conventional medication,rehabilitation therapy,and intermittent catheterization.The 5 Hz group,10 Hz group,and 20 Hz group were treated with FMS at 5 Hz,10 Hz,and 20 Hz,respectively,and the control group was treated with sham stimulation at the same site and parameters as the 5 Hz group.Bladder pressure volume index,urination status,recovery time of spontaneous urina-tion,Quality of Life Questionnaire(SQLP)score,and urinary retention efficacy were compared among the four groups.Results There were no significant differences in bladder pressure volume,urination,recovery time of spontaneous urination,and SQLP score among the four groups before treatment(P>0.05).After treatment,the bladder volume and pressure of the first four groups were both increased,with those of the 5 Hz group,10 Hz group,and 20 Hz group significantly higher than those of the control group,while the residual urine volume of the first four groups was significantly lowered,with that of the 5 Hz group,10 Hz group,and 20 Hz group significantly lower than that of the control group(P<0.05),even showing an effect of dose-response;The average daily urination frequency and average single urination volume of the first four groups were all increased,with those of the 5 Hz group,10 Hz group and 20 Hz group all significantly higher than those of the control group,while the average daily urination frequency was all reduced,with that of the 5 Hz group,10 Hz group and 20 Hz group significantly less than the control group(P<0.05),even showing an effect of dose-response;The SQLP scores of the first four groups were all lowered,with those of the 5 Hz,10 Hz and 20 Hz groups significantly lower than those of the control group(P<0.05),and the recovery time of spontaneous urination in the 5 Hz,10 Hz,and 20 Hz groups was significantly shorter than that of the control group,showing an effect of dose-response;The total effective rate of urinary retention in the 5 Hz,10 Hz and 20 Hz groups was significantly higher than that in the control group(P<0.05),showing an effect of dose-response.Conclusion FMS at 5 Hz,10 Hz and 20 Hz can improve the urination function and quality of life of patients with urinary retention after stroke,showing a dose-responsive efficacy.The clinical efficacy by FMS at 20 Hz is better than that at 5 Hz and 10 Hz.

Neurotoxicity mechanism of aconitine in HT22 cells studied by microfluidic chip-mass spectrometry / 药物分析学报

Aconitine,a common and main toxic component of Aconitum,is toxic to the central nervous system.However,the mechanism of aconitine neurotoxicity is not yet clear.In this work,we had the hypothesis that excitatory amino acids can trigger excitotoxicity as a pointcut to explore the mechanism of neurotoxicity induced by aconitine.HT22 cells were simulated by aconitine and the changes of target cell metabolites were real-time online investigated based on a microfluidic chip-mass spectrometry system.Meanwhile,to confirm the metabolic mechanism of aconitine toxicity on HT22 cells,the levels of lactate dehydrogenase,intracellular Ca2+,reactive oxygen species,glutathione and superoxide dismutase,and ratio of Bax/Bcl-2 protein were detected by molecular biotechnology.Integration of the detected results revealed that neurotoxicity induced by aconitine was associated with the process of excitotoxicity caused by glutamic acid and aspartic acid,which was followed by the accumulation of lactic acid and reduction of glucose.The surge of extracellular glutamic acid could further lead to a series of cascade reactions including intracellular Ca2+overload and oxidative stress,and eventually result in cell apoptosis.In general,we illustrated a new mechanism of aconitine neurotoxicity and presented a novel analysis strategy that real-time online monitoring of cell metabolites can provide a new approach to mechanism analysis.

An MRI study of lateral vascular safety zones in oblique lumbar interbody fusion surgery / 中国修复重建外科杂志

OBJECTIVE@#To study the anatomical characteristics of blood vessels in the lateral segment of the vertebral body through the surgical approach of oblique lumbar interbody fusion (OLIF) using MRI imaging, and evaluate its potential vascular safety zone.@*METHODS@#The lumbar MRI data of 107 patients with low back and leg pain who met the selection criteria between October 2019 and November 2022 were retrospectively analyzed. The vascular emanation angles, vascular travel angles, and the length of vessels in the lateral segments of the left vertebral body of L 1-L 5, as well as the distance between the segmental vessels in different Moro junctions of the vertebral body and their distances from the edges of the vertebrae in the same sequence (bottom marked as I, top as S) were measured. The gap between the large abdominal vessels and the lateral vessels of the vertebral body was set as the lateral vascular safe zones of the lumbar spine, and the extent of the safe zones (namely the area between the vessels) was measured. The anterior 1/3 of the lumbar intervertebral disc was taken as the simulated puncture center, and the area with a diameter of 22 mm around it as the simulated channel area. The proportion of vessels in the channel was further counted. In addition, the proportions of segmental vessels at L 5 without a clear travel and with an emanation angel less than 90° were calculated.@*RESULTS@#Except for the differences in the vascular emanation angles between L 4 and L 5, the vascular travel angles between L 1, L 2 and L 4, L 5, and the length of vessels in the lateral segments of the vertebral body among L 1-L 4 were not significant ( P>0.05), the differences in the vascular emanation angles, vascular travel angles, and the length of vessels between the rest segments were all significant ( P<0.05). There was no significant difference in the distance between vessels of L 1, L 2 and L 2, L 3 at Moro Ⅰ-Ⅳ junctions ( P>0.05), in L 3, L 4 and L 4, L 5 at Ⅱ and Ⅲ junction ( P>0.05). There was no significant difference in the vascular distance of L 2, L 3 between Ⅱ, Ⅲ junction and Ⅲ, Ⅳ junction, and the vascular distance of L 3, L 4 between Ⅰ, Ⅱ junction and Ⅲ, Ⅳ junction ( P>0.05). The vascular distance of the other adjacent vertebral bodies was significant different between different Moro junctions ( P<0.05). Except that there was no significant difference in the distance between L 2I and L 3S at Ⅰ, Ⅱ junction, L 3I and L 4S at Ⅱ, Ⅲ junction, and L 2I and L 3S at Ⅲ, Ⅳ junction ( P>0.05), there was significant difference of the vascular distance between the bottom of one segment and the top of the next in the other segments ( P<0.05). Comparison between junctions: Except for the L 3S between Ⅰ, Ⅱ junction and Ⅱ, Ⅲ junction, and L 5S between Ⅰ, Ⅱ junction and Ⅱ, Ⅲ and Ⅲ, Ⅳ junctions had no significant difference ( P>0.05), there were significant differences in the distance between the other segmental vessels and the vertebral edge of the same sequence in different Moro junctions ( P<0.05). The overall proportion of vessels in the simulated channels was 40.19% (43/107), and the proportion of vessels in L 1 (41.12%, 44/107) and L 5 (18.69%, 20/107) was higher than that in the other segments. The proportion of vessels in the channel of Moro zone Ⅰ (46.73%, 50/107) and zone Ⅱ (32.71%, 35/107) was higher than that in the zone Ⅲ, while no segmental vessels in L 1 and L 2 were found in the channel of zone Ⅲ ( χ 2=74.950, P<0.001). Moreover, 26.17% (28/107) of the segmental vessels of lateral L 5 showed no movement, and 27.10% (29/107) vascular emanation angles of lateral L 5 were less than 90°.@*CONCLUSION@#L 1 and L 5 segmental vessels are most likely to be injured in Moro zones Ⅰ and Ⅱ, and the placement of OLIF channels in L 4, 5 at Ⅲ, Ⅳ junction should be avoided. It is usually safe to place fixation pins at the vertebral body edge on the cephalic side of the intervertebral space, but it is safer to place them on the caudal side in L 1, 2 (Ⅰ, Ⅱ junction), L 3, 4 (Ⅲ, Ⅳ junction), and L 4, 5 (Ⅱ, Ⅲ, Ⅳ junctions).

Clinical characteristics and literature review of a case of Christianson syndrome caused by SLC9A6 gene variation / 中华神经科杂志

Objective:To retrospectively analyze the clinical data of Christianson syndrome caused by SLC9A6 gene mutation and related literatures.Methods:The clinical data of one Christianson syndrome patient caused by SLC9A6 gene variation in Children′s Hospital of Zhengzhou University were collected, meanwhile the relevant literature was reviewed. The examination of video electroencephalogram, auditory brainstem response, and cranial magnetic resonance imaging (MRI) was performed. Whole exon sequencing and mitochondrial gene detection were performed for 3 persons in the family, and the suspected mutation sites were verified by Sanger sequencing.Results:A boy, 7 years old, presented with epilepsy, language retardation and mental retardation. Now he can only say overlapping words, execute simple instructions, denying family history of genetic disease and genetic metabolic disease. The patient′s uncle had the history of febrile convulsions in childhood. At present, speech and intelligence are impaired, and the left limb movement is slightly limited. The patient′s mother was mildly retarded, without epilepsy. The video- electroencephalogram of the patient was shown below (April 2021): abnormal electroencephalogram; background activity was slightly slow; the bilateral frontotemporal region was dominated by multi-focal spiky wave, spiky slow wave and slow wave in each waking and sleeping stage, which can be generalized and extensive; in the sleeping stage, the discharge index in non-rapid eye movement stage was about 75%. The auditory brainstem response was shown below (October 2021): the left 70 dB Ⅰwave latency was prolonged; the Ⅰwave Ⅴ wave shape was poor; the threshold was 20 dB (the high frequency threshold was normal); the right 70 dB Ⅰwave latency was prolonged; the wave form was poor; the amplitude was lower than that of the contralateral side; Ⅲ wave Ⅴ wave shape was poor; the threshold was 30 dB (the high frequency threshold slightly increased). Brain MRI thin-section scan was shown below (January 2021): subarachnoid space of bilateral temporal poles widened, and no obvious abnormal signal was found in brain parenchyma; sinusitis. Whole exome sequencing of 3 persons in the family indicated that the proband had a hemizygous variant c.616C>T (p.R206 *) in the SLC9A6 gene. Using the SLC9A6 gene and Christianson syndrome as the key words, 94 foreign literatures from January 1989 to January 2022 were researched. Totally, 81 Christianson syndrome patients caused by SLC9A6 gene mutation were reported. The age of onset ranged from neonatal period to adulthood, and the clinical manifestations were heterogeneous. The symptoms of male patients mainly included epilepsy, severe cognitive impairment, ataxia, cerebellar atrophy, and psychomotor retardation. Conclusions:The hemizygous variant of SLC9A6 gene (c.616C>T) is the etiology of this patient. The possibility of Christianson syndrome shall be considered for recurrent epilepsy with poor efficacy of antiepileptic drugs, status epilepticus during slow-wave sleep, and delayed development of motor intelligence. Genetic testing is helpful for definite diagnosis and treatment.

Childhood onset spinocerebellar ataxia type 2: a family report and literature review / 中华神经科杂志

Objective:To investigate the clinical characteristics, genetic characteristics and diagnosis of spinocerebellar ataxia type 2 (SCA2) patients with childhood onset.Methods:The clinical data of a SCA2 pedigree who diagnosed at Neurogenetic Metabolic Disease Clinic of Children′s Hospital Affiliated to Zhengzhou University in July 2019 were collected, and the reported cases of childhood-onset SCA2 were reviewed. The CAG repeat of ATXN2 gene was detected by polymerase chain reaction, capillary gel electrophoresis and Sanger sequencing techniques.Results:A total of 9 people in 4 generations of the family were affected, showing an autosomal dominant inheritance. The proband was a 3 years and 4 months old boy, who showed abnormal symptoms at 9 months which manifested as developmental retardation. At 1 year old, he developed progressive regression which represented neither to be amused, recognize others, stand and walk alone, nor had language development. Meanwhile, he had difficulty swallowing, long-term constipation, and a history of convulsions. His sister and mother were not yet sick. His grandmother could not walk, had slurred speech accompanied by nystagmus, and magnetic resonance imaging showed cerebellar atrophy. His granduncles and grandaunts had unstable walking and dysarthria. His great-grandfather required wheelchair to walk. This pedigree showed an autosomal dominant inheritance. One of the ATXN2 gene alleles of the proband, his sister, mother and grandmother all showed abnormal amplification with 99, 55, 44, and 43 times respectively and no inserting CAA sequence. A total of 14 literatures reported 20 cases of childhood-onset SCA2 patients who were genetically diagnosed. The majorities had onset in infancy, and few can develop into school age. The main clinical manifestations were developmental delay, dystonia or insufficiency, myoclonus or infantile spasms, motor retardation, abnormal eye movement, retinitis pigmentosa and dysphagia, while the classic cerebellar syndrome was only partially present. Abnormal rhythm was found on electroencephalogram, cerebellar atrophy on magnetic resonance imaging or CT of the head.Conclusions:This case is the youngest genetically-confirmed SCA2 patient reported in China. Reported patients usually have onset in infancy with excessive repeat sequence expansion. Their clinical characteristics are different from the classic patients and could only be diagnosed by dynamic mutation detection.

Technical details of gastrointestinal reconstruction using linear stapler in totally laparoscopic total gastrectomy / 中华胃肠外科杂志

With the development of instrument, equipment and surgical skills, especially the emergence of a series of high-level medical evidence, the laparoscopic techniques in the field of gastric surgery has been further expanded. Totally laparoscopic total gastrectomy (TLTG) has certain technical difficulties, and more challenges are reflected in the digestive tract reconstruction. The use of linear staplers has reduced the difficulty of digestive tract reconstruction to a certain extent and has strongly promoted the transition from laparoscopic-assisted total gastrectomy to TLTG. However, for TLTG, there are still many details that should be carefully concerned, so as to effectively avoid the surgical pitfalls and ensure the fluency and safety of the procedure. In this article, we discuss the surgical details based on our own experiences, including how to obtain surgical field exposure well, how to manage specific accidents when using linear stapler for esophagojejunostomy, how to prevent intra-abdominal hernias and Roux stasis syndrome, and how to prevent the stapled lines of the esophageal or jejunal stumps from direct contact with aorta.

Overlap esophagojejunostomy with multi-mode modifications in totally laparoscopic total gastrectomy: safety and feasibility of 152 cases from a single center / 中华胃肠外科杂志

Objective: Currently, the Overlap anastomosis is one of the most favored reconstruction methods of intracorporeal esophagojejunostomy (EJS). Despite many advantages of the method, it remains some shortcomings to be improved when it comes to the retraction of the esophagus stump, the insertion of the anvil fork of the linear stapler into a "pseudo" lumen, and the closure of the common entry hole. This study aims to investigate the safety and feasibility of a multi-mode modified Overlap anastomosis. Methods: A descriptive case series study was conducted. Medical records of 152 consecutive patients who underwent totally laparoscopic total gastrectomy (TLTG) with our multi-mode modified Overlap EJS method by the same surgical team at our department from February 2017 to June 2020 were retrospectively analyzed. The multi-mode modified Overlap method mainly included (1) After ensuring the safety of tumor resection margin (proximal margin was at least 3 cm from the tumor), the esophagus was partially transected from left to right (with 5-8 mm width esophagus continuation). The specimen was then placed in a plastic bag which was tied up at the mouth using strings with a part of the esophageal wall poking through. Then the plastic bag containing the specimen was transferred to the right lumbar region, while the patient's body position was adjusted so that the abdominal esophagus could be pulled by the gravity of the specimen. (2) Using the "three-direction traction" method. The esophageal lumen was properly exposed, then guided by the gastric tube, the anvil fork was accurately placed into the esophageal lumen for completing the side-to-side EJS. (3) The 3-0 barbed suture was used in the closure of the common entry hole of the stapler from dorsally to ventrally with simple one-layer continuous suture (the stitch going from inside to inside) followed by continuous Lembert's suture (the stitch going from outside to outside). Combined with clinicopathological characteristics, the perioperative outcomes and postoperative complications of the whole group were analyzed and evaluated. Results: The study cohort included 129 men and 23 women, with a mean age of (60.2±9.1) years and a mean body mass index (BMI) of (23.2±3.1) kg/m(2). Of the 152 patients, 23 patients (15.1%) had a history of previous abdominal surgery; dentate line was invaded by tumor in 21 patients (13.8%). The mean length of the proximal resection margin was (3.3±0.3) cm and the postoperative pathological examination indicated negative resection margin tumor. The mean operative time and anastomotic time were (302.1±39.9) minutes and (29.8±5.4) minutes, respectively. The mean estimated blood loss was (87.9±46.4) ml. The mean length of postoperative hospital stay was (12.3±7.3) days. The overall severe postoperative complications (Clavien-Dindo ≥ II) occurred in 22 patients (14.5%). Six cases of pancreatic leakage were successfully recovered by adequate drainage, inhibition of pancreatic exocrine secretion and nutritional support. Ten cases of pneumonia and three cases of abdominal infection were cured with anti-infection and physical therapy. Two patients developed anastomotic leakage postoperatively. One case was caused by excessive tension of the Roux loop of the jejunum and excessive opening on the side of the jejunum after side-to-side anastomosis, and the other case was caused by an accidental intraoperative occurrence of "nasogastric tube stapled to the side-to-side anastomosis". Both of them recovered after conservative treatment including adequate drainage, anti-infection, and adequate nutritional support. One patient underwent immediate open surgery because of Peterson's hernia 7 days after TLTG, and the patient died due to extensive small bowel necrosis. Conclusions: Multi-mode modified overlap method simplifies the operation and reduces the difficulty of EJS. It is a safe and feasible method for EJS.