Objective To investigate the effects of human leukocyte-associated antigen-G (HLA-G) expression in silencing trophoblast cell line JEG-3 under normal and hypoxic conditions on invasion and proliferation of JEG-3 cells. Methods Inhibition of HLA-G expression in JEG-3 cells by transfection of small interfering RNA (siRNA),the transfected JEG-3 cells were divided into 4 groups: normoxia control group, hypoxia control group, normoxia inhibition group and hypoxia inhibition group. The levels of HLA-G mRNA and protein in 4 groups of cells were detected by real-time quantitive PCR and western blot. The proliferation activity and invasion ability of 4 groups of cells were determined by methylthiazolyl tetrazolium (MTT) assay and invasion assay.Results (1) Real-time quantitive PCR technology showed: the level of HLA-G mRNA in the hypoxic inhibition group (0.220±0.050) was significantly different (P<0.05), when compared with that in the hypoxic control group (0.630±0.030) and normoxic inhibition group (0.400± 0.020). (2) Western blot analysis showed: the expression level of HLA-G protein in the hypoxic inhibition group was 0.260±0.010, statistically different from that in the hypoxic control group (0.850±0.100) and the normoxic inhibition group (0.560±0.020; P<0.05).(3) MTT showed: proliferative activity of JEG-3 cells in the normoxic inhibition group was 0.490 ± 0.070, the ability of cell proliferation was reduced. When compared with that in the normoxic control group (0.850±0.050), the differences was statistically significant (P<0.05). The proliferative activity of JEG-3 cells in the hypoxic inhibition group (0.330±0.070) was lower than that in the normoxic inhibition group (0.490±0.070), and there was a significant difference (P<0.05). (4) Invasion assay showed: compared with the normoxic control group (98±7), the invasive ability of JEG-3 cells in the normoxic inhibition group (73 ± 7) was weakened, and the difference was statistically significant (P<0.05). The number of transmembrane cells (52±11) of JEG-3 cells in the hypoxic inhibition group was lower than that in the hypoxic control group (72±7), and the difference was statistically significant (P<0.05). Compared with the normoxic inhibition group, the invasion ability of JEG-3 cells in the hypoxic inhibition group decreased, and the difference was statistically significant (P<0.05). Conclusion Under hypoxia, using siRNA technology to down-regulate the expression of HLA-G may affect the proliferation and invasion ability of trophoblast cells, which may be involved in the occurrence of hypertensive disorder of pregnancy.

Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) myopathy is one of the subtypes of immune-mediated necrotizing myopathy. Anti-HMGCR antibodies induce complement activation,subsequently resulting in myofiber necrosis,regeneration with autophagy abnormalities and mitochondrial changes. The age of onset is from children to adulthood. Some patients have a history of exposure to statins. Most patients are subacute onset. The patients with chronic progressive process, are more like muscular dystrophy. The main symptoms are proximal symmetrical weakness of limbs and usually accompanied with extra-muscle symptoms. The MRI showed muscle edema in all patients and fatty infiltrates in some patients. Myositis-specific auto-antibodies and muscle biopsies play key roles in diagnosis of HMGCR myopathy. Corticosteroids and immunosuppressants were first line therapy. Pediatric patients or patients with chronic course are usually refractory, and the efficacy of different combinations of immunosuppressants needs to be further investigated.

Objective To improve fresh nurses′ implementation rate of standard clinical administration checking by applying situational simulation method, and enhancetheir ability of identifying hidden perils in nursing. Methods Totally 75 fresh nurses who graduated in 2016 were enrolled and divided into two groups (36 in control group and 39 in experimental group) according to randomized digital table method. Situational simulation method was applied in the experimental group, while the control group applied traditional nursing safety check training method. Then both groups′ satisfaction rate of lessons and self-confidence after training were recorded. Besides, their clinical administration checking behaviors after 3 months, the errors, hidden perilsand examining situations related to administration checking after 6 months,were also evaluated. Results Significant statistical differences were shown in satisfaction score (23.62 ± 2.02 in experimental group vs. 21.39 ± 1.98 in control group, t=4.818, P<0.01) and self-confidential evaluating score (35.67±2.02 in experimental group vs. 21.39±1.98 in control group, t=2.768, P<0.01). Observation of clinical administration checking behaviors 3 months after training had indicated significant differences in the number of nurses who check drug concentration (33 vs. 8, χ2=29.406, P<0.05) and executing time (30vs. 12, χ2=14.436, P<0.05).Statistical differences (χ2= 5.080-29.545, P< 0.01 or < 0.05) were also observed in the number of nurses checking bed number, patient name, drug name, dose, drug concentration and methods in and after operation. The potential nursing perils identified, and the number of nurses who identify hidden nursing perils 3 months and 6 months after training were significantly larger (χ2=50.591, 23.658, 3.914, P<0.05) in experimental group (33, 2, 10) than in control group (18, 1, 3),and there were no significant differences in the rate of potential perils and nursing error between both groups. Conclusion Situational method of administration, which is applied for fresh nurses in their initial working phase, transforms the basic and core checking system into clinical real-situation cases. It also shows an advantage of boosting their self-confidence of learning, improving the implementation rate of standard clinical administration checking and the ability of avoiding potential nursing perils.

Objective:To evaluate the efficacy of neonatal resuscitation simulation exercise for perinatal medical personnel.Methods:From August 2020 to July 2021, perinatal medical personnel receiving simulated training of neonatal resuscitation in our hospital were prospectively enrolled. The professional backgrounds of the trainees were collected and their performances on both knowledge skills and behavioral skills were scored. The knowledge skills included pre-resuscitation preparation, initial resuscitation, positive pressure ventilation, tracheal intubation, chest compression and umbilical vein catheterization. The behavioral skills included situational awareness, problem solving, resource utilization, communication and leadership. SPSS 26.0 was used for data analysis.Results:Among the 200 participants, 127(63.5%) were neonatal/pediatric doctors and nurses, 65(32.5%) were obstetricians and midwives, 8(4.0%) were anesthesiologists and the ratio of doctors to nurses was 1.74∶1. The score of knowledge skills was (19.52±2.92) at the beginning of the simulation exercise and (27.02±2.72) at the end. The scores on preparation before resuscitation, initial resuscitation and positive pressure ventilation were significantly improved ( P<0.05). The score of behavioral skills was (16.60±2.34) at the beginning and (20.58±1.77) at the end. The scores of resource utilization, communication and leadership were significantly improved ( P<0.05). Conclusions:The simulation exercise provides multidisciplinary teamwork training for perinatal medical personnel, may significantly improve neonatal resuscitation skills and is worth promoting.

OBJECTIVETo understand the dietary consumption of residents in Xiamen and the content of phthalic acid esters (PAEs) in food, and to assess the plasticizer exposure risk of diet in Xiamen.

METHODSThe survey was conducted by stratified cluster random sampling method in Xiamen from September to October in 2010. According to the Xiamen administrative division, six neighborhood communities were selected as sampling units, then 25 families were randomly chosen from each sampling units.From the above 150 families, the permanent residents over the age of six were permitted to our study. The survey included 495 residents totally. These participants' information, such as basic personal information, physical activity levels, meal frequency and the average consumption of 33 kinds of food in 13 categories were collected using questionnaires. Thirteen categories included cereal and tubers, beans, vegetables, fungi and algae, fruits, dairy products, meat, seafood, eggs, snacks, beverages, cooking oil and spices. The height and weight of residents were measured and the average daily dietary intake was calculated. Thirty-three kinds of food in 13 categories were collected in supermarkets in Xiamen. According to the annual sales ranking, the top three-five brands of each kinds of food were selected and numbered, then two or three brands were chosen by random number table method from them; three completely individual packed samples in the same batch of each brand were detected; 243 samples were included in our study.100-500 g solid samples or 100-500 ml liquid samples were collected. The content of diethyl phthalate (DEP), dibutyl phthalate (DBP), di (2-ethylhexyl) phthalate (DEHP) in food were detected by liquid chromatography mass spectrometry, which expressed by median (minimum-maximum). The exposure dose, contribution rate and risk index of PAEs were calculated by point estimation method.

RESULTSAccording to the average daily dietary intake of residents in Xiamen, the top three ones in 13 categories of food were cereal and tubers (337.16 g/d, 18.21%), vegetables (309.12 g/d, 16.69%) and fruits (213.20 g/d, 11.51%). The content of DEP, DBP or DEHP among different categories of food was significantly different (χ² values were 58.05, 50.19 and 102.10, P < 0.01). Among 13 categories of food, seafood contained the most DEP (0.090 (0.000-0.324)mg/kg); cooking oil had the most DBP (0.700(0.000-2.980) mg/kg) and DEHP (5.115(0.000-24.160) mg/kg). DEP, DBP and DEHP exposure(0.19, 4.20, 18.10 µg × kg⁻¹ ×d ⁻¹)in dietary food in Xiamen were less than the reference dose(RfD) (800, 100, 20 µg × kg⁻¹ × d⁻¹) proposed by the United States Environmental Protection Agency (EPA), and the risk indexes were 0.02%, 4.20% and 90.50%, respectively. Among 13 categories of foods, seafood was the main source of DEP dietary exposure. The exposure dose and contribution rate of DEP in seafood were 0.18 µg × kg⁻¹ × d⁻¹ and 94.74%, respectively.Vegetables were the main source of DBP and DEHP dietary exposure. The exposure dose and contribution rate of DBP and DEHP were 1.48 µg × kg⁻¹ × d⁻¹, 35.24% and 6.07 µg × kg⁻¹ × d⁻¹, 33.54%, respectively.

CONCLUSIONThe food consumed by residents in Xiamen was overall in a safe state, but to some extent, there still exists DEHP exposure risk in foods.

China ; Dibutyl Phthalate ; Diet ; Diethylhexyl Phthalate ; Food Contamination ; Humans ; Phthalic Acids ; Plasticizers ; Risk Assessment ; Seafood ; United States ; Vegetables

Objective:To analyze the clinical characteristics, imaging, myopathology and outcomes of patients with COVID-19 related autoimmune myopathy.Methods:The clinical features, serum creatine kinase (CK), myositis antibodies, muscle magnetic resonance imaging, myopathology and therapy of 5 patients with COVID-19 related autoimmune myopathy diagnosed in Peking University First Hospital from December 2022 to April 2023 were collected. The effects of the therapy after a short term follow up were analyzed.Results:Among the 5 patients, there were 3 males and 2 females, with onset age of 42-86 years. All patients presented with proximal muscle weakness in the recovery term of COVID-19. Myalgia was noted in 3 cases, dysphagia in 1, skin damage in 2, interstitial lung disease in 1. The serum CK of the 5 patients was 1 663-16 000 IU/L, 1 patient had anti-3-hydroxy-3-methylglutaryl-CoA reductase autoantibodies and 1 patient had anti-signal recognition particle autoantibodies. The electromyography showed myogenic lesions in all patients. Muscle magnetic resonance imaging showed diffuse muscle edema in all patients, myofascial edema in 3 and subcutaneous-tissue edema in 3. The muscle biopsies in 4 patients revealed necrotic myopathy,with high P62 expression in muscle fibers. The electromicroscopy of 2 patients revealed vacuolated mitochondria and intranuclear tubulofilamentous inclusions in muscle fibers. Four patients were treated with glucocorticoids, of whom 2 patients combined with intravenous immunoglobulin, tacrolimus or cyclophosphamide. One case had close monitoring without drug therapy. They showed significant improvement, but the CK was still abnormal in 4 patients.Conclusions:COVID-19 leads to immune mediated myopathy. The manifestation of patients is characterized by proximal predominant weakness and high creatine kinase level. Muscle magnetic resonance imaging shows diffuse muscle edema. The muscle biopsies reveal necrotic myopathy. The effectiveness of immunosuppression needs to be further studied.

Based on the theory of “manifestation-body and spirit-qi”， it is considered that the pathogenesis of tic disorders （TD） involves three manifestations of “wind， phlegm and fire”， with wind as the leading factor and phlegm and fire as the accompanying factors. It is advocated to identify the abnormality of body and spirit， summarize the evolution of manifestation， and prescribe flexibly based on qi movement of zang-fu organs. Among them， the common symptoms related to tics are mostly belong to wind symptoms， which originated from lung qi failing to purify and liver qi over-ascent. The method of soothing liver wood and purifying lung gold is adopted， and modified Xiaochaihu Decoction （小柴胡汤） and Cangerzi Powder （苍耳子散） are recommended； Patients with prolonged illness and strange symptoms are often mixed with phlegm， which stemmed from the fact that spleen failing to rise clear and stomach failing to downward turbid. The method of raising spleen-earth and lowering stomach-earth is adopted， and modified Yigong Powder （异功散） and Erchen Decoction （二陈汤） are recommended； Patients with severe symptoms are often mixed with fire symptoms， which originated from discordance of water and fire and hyperactivity of heart fire. The method of interacting heart and kidney and coordinating water and fire is adopted， and modified Xiegan Pill （泻肝丸） and Liuwei Dihuang Pill （六味地黄丸） are recommended， in order to regulate qi movement， harmonize body and spirit， and improve the symptoms.

Objective:To evaluate the quality of life (QoL) of Chinese chronic pancreatitis (CP) patients based on the Chinese version of the pancreatitis quality of life Instrument (PANQOLI) and explore its impact factors.Methods:404 patients with CP admitted to the Department of Gastroenterology of the First Affiliated Hospital of Naval Medical University between September 2021 and January 2022 were enrolled. The Chinese version of PANQOLI was used for questionnaire survey on QoL of CP patients. Univariate analysis and multiple linear regression analysis were used to explore the impact factors for QoL of CP patients.Results:The total score of QoL of 404 Chinese CP patients was 28-94(72.47±13.61), which declined by 29.64% compared to the highest total score (103) in the Chinese version of PANQOLI. Score of physical function, role function, emotional function, and self-worth domain was 25.63±4.84, 13.86±2.78, 16.98±6.21 and 16.00±4.65, respectively. Compared to the highest scores (30, 25, 24 and 24), the scores of aforementioned four domains declined by 14.57%, 44.56%, 29.25% and 33.33%, respectively. Univariate analysis showed that sex, age, employment status, smoking, alcohol consumption, and frequency of pancreatitis recurrence were significantly associated with QoL of CP patients. Multiple linear regression analysis indicated that older age (coefficient=-0.127), unemployment status (coefficient=-0.106), smoking (coefficient=-0.176), and high frequency of pancreatitis recurrence (coefficient=-0.123) were independent factors for QoL of CP patients (all P value <0.05). Conclusions:The Chinese version of PANQOLI could be effectively applied to Chinese CP patients. Older age, unemployment, smoking, and pancreatitis attacks were risk factors for QoL of CP patients, indicating that the formulation of personalized intervention measures may help to improve QoL of CP patients.

Objective:To investigate the clinical, genetic, and pathological features of nemaline myopathy type 8 (NEM8) caused by KLHL40 gene variation. Methods:The clinical data, gene sequencing results, and musculoskeletal pathophysiology of two cases (a pair of twins) with NEM8 admitted to our hospital in July 2022 were collected. CNKI, VIP, Wanfang, Yiigle, PubMed, Embase, and Web of Science Database were searched with the English and Chinese terms "nemaline myopathy type 8", "nemaline body myopathy type 8", and " KLHL40" from January 2007 to February 2023. The clinical, genetic, and musculoskeletal characteristics of the NEM8 cases were summarized using the descriptive statistical analysis method. Results:(1) Case report: The mother (G2P2) of the twins (Ⅳ-2 and Ⅳ-3) was conceived by IVF-embryo transfer and delivered at 37 +1 gestational weeks. The two cases were dizygotic twins whose maternal grandmother had lip and palate cleft. The first baby (Ⅳ-1) of the woman exhibited absent left pinna, contracture at the end of both fingers, talipes equinovarus in both feet and died of respiratory failure two hours after birth. Fetal edema and fetal movement delay in the twins and varus right foot in one twin were found during pregnancy. Both cases developed intrapartum asphyxia with the clinical manifestations of generalized muscle weakness, respiratory failure, dysphagia, multiple joint contractures, and fractures. The families withdrew the treatment and the twins died after maintaining life with ventilators for 53 days after birth. Whole exome sequencing of the pedigree found c.1779G>T (p.W593C) homozygous variants of the KLHL40 gene in the twins and c.1779G>T (p.W593C) heterozygous variants of the KLHL40 gene in the parents, both were de novo. Musculoskeletal pathophysiology indicated that muscle fibers are thin and round with a fetal shape and absent rod. (2) Literature review: Among the 29 cases of NEM8, including two current cases and 27 cases retrieved from 15 papers, eight cases terminated the pregnancies, and 21 were live births; seven cases (24.1%) with positive family history; 19 cases (65.5%) were found with abnormalities during pregnancy, including abnormal fetal movements, polyhydramnios, joint contracture, and fetal edema. Of the 21 live births, 20 cases had intrapartum asphyxia, 21 had postnatal respiratory failure, 20 had generalized muscle weakness, and 19 had dysphagia. Among the 29 cases, 17 cases (58.6%) were homozygous variants of the KLHL40 gene, and 12 cases (41.4%) were compound heterozygous variants. The detection rate of c.1516A>C(p.Thr506Pro) was the highest [72.4% (21/29)], followed by c.602G>A(p.Trp201*) [17.2% (5/29)]. Out of the 15 cases who underwent musculoskeletal pathological examination, all had abnormal muscle fiber size and morphology; 10 cases had rods. Conclusions:NEM8 should be considered for those with abnormal fetal movements, polyhydramnios, joint malformation, fetal edema during the fetal period and failure to establish respiration at birth as well as postnatal generalized muscle weakness, respiratory failure, dysphagia, multiple joint contractures, and fracture. Moreover, genetic detection should be performed as soon as possible. NEM8 can be diagnosed in ones with KLHL40 gene homozygous or compound heterozygous variants and musculoskeletal pathological results of abnormal size and shape of muscle fibers, regardless of the presence of rods.

ObjectiveTo explore the characteristics of eye collaterals of patients with hepatic Wilson's disease （WD） and intervention effect of Gandouling （GDL） based on digital diagnosis information on eyes， in order to provide references for the clinical efficacy of GDL in the treatment of patients with hepatic WD. MethodEighty-two patients with hepatic WD （WD group） and 68 healthy volunteers （normal control group， NC） were enrolled. Eye images of all subjects were collected by the ophthalmoscope， followed by digital extraction of eye collateral characteristics. Differences in main eye collateral characteristics between the WD group and the NC group were analyzed. Pearson correlation test was used to analyze the eye collateral characteristics， serological indexes of liver damage ［alanine aminotransferase （ALT），aspartate transaminase （AST），and albumin （ALB）］， and Child-Pugh score in patients with hepatic WD. The 82 patients with hepatic WD were divided into a treatment group （42 cases） and a control group （40 cases）. All patients were treated with sodium 2，3-dimercapto-1-propane sulfonate （DMPS）， while those in the treatment group received additional GDL Tablets. One course of treatment contained eight days， and the treatment lasted six courses. The effects of GDL intervention on the levels of ALT，AST， and ALB，as well as the eye collateral characteristics score and Child-Pugh score were evaluated. Result① Compared with the NC group，the WD group had higher scores of "Qiu" （P<0.05） and "Xuemai" （P<0.05），which are the characteristics of eye collaterals. ② The levels of ALT and AST， and Child-Pugh score in patients with hepatic WD were positively correlated with the scores of "Qiu" and "vessel"，while the ALB level was negatively correlated with the "bulge" and "Xuemai" scores. ③ Compared with the conditions before treatment，the serum ALT and AST levels，Child-Pugh score，and the "Qiu" and " Xuemai " scores of the two groups were reduced （P<0.01），and the ALB levels were increased （P<0.01）. ④ Compared with the control group after treatment，the treatment group showed improved levels of ALT and ALB and the scores of "Qiu" and "Xuemai" （P<0.05）and better Child-Pugh score （P<0.01）. There was no significant difference in AST between the two groups. ConclusionCompared with healthy people，hepatic WD patients showed higher scores of "Qiu" and "Xuemai"， indicating that patients with hepatic WD were mainly characterized by dampness and heat，and the scores of "Qiu" and "Xuemai" were positively correlated with the severity of liver damage. Meanwhile，copper-expelling therapy combined with GDL can significantly improve the liver function of WD patients and improve the clinical efficacy.