【Objective】 To study and compare the effects of different storage temperature and time on coagulation factor after cryoprecipitated antihemophilic factor(CAF) melting, and to provide reference for the establishment of industry standards. 【Methods】 From June 2021 to May 2023, a total of 96 bags of CAF were sampled in 4 bags per month, and timely detected in the same month. After the CAF was melted in a 37℃ water bath, the mild to moderate lipemic blood was labeled. Each bag of CAF and two 50 mL transfer bags were divided into two bags and two groups of 20 mL each using a sterile adapter. One group was placed in a 4℃ refrigerator and the other in a 22℃ water bath for 0 h, 4 h, 8 h, 12 h, 24 h and 48 h. Then 2 mL of aseptic sample was taken separately and put into the test tube, and 1mL of sample and 3 mL of buffer were added into the other test tube with the sampling gun and mixed on the machine for testing. The experimental data of 60 bags without mild to moderate lipemic blood cryoprecipitation and coagulation factor were randomly selected and statistically analyzed by SPSS21.0. 【Results】 After melting, CAF was stored for 0 h, 4 h, 8 h, 12 h, 24 h and 48 h to detect the average content and growth rate of coagulation factor in the two groups: 1) Storage at 4℃, factor Ⅷ content was 118.62, 111.57(-5.95%), 105.51(-11.05%), 103.30(-12.92%), 94.35(-20.46%) and 83.25(-29.82%) IU/ bag, respectively; Storage at 22℃, the factor Ⅷ content was 118.62, 112.69(-5.00%), 111.41(-6.08%), 109.01(-8.10%), 101.55(-14.39%) and 92.75(-21.81%) IU/ bag, and the storage results of the two groups were compared. At 24 h at 4℃ and 48 h at 22℃, the content of factor Ⅷ had significant statistical significance(P<0.01), and when stored at 22℃, the decay rate of factor Ⅷ was slower; 2) When stored at 4℃, the content of factor V was 41.19, 41.31(0.29%), 40.52(-1.64%), 40.27(-2.23%), 39.05(-5.19%) and 36.99(-10.21%) IU/ bag, respectively; Stored at 22℃, the factor V content was 41.19, 41.71(1.25%), 42.54(3.28%), 41.94(1.80%), 39.21(-4.80%) and 35.64(-13.48%) IU/ bag, respectively. Comparison of storage results between the two groups showed that the content of factor V was statistically significant(P<0.05) and significantly significant(P<0.01) at 4℃48 h and 22℃48 h, respectively, and the decay rate of factor V was faster when stored at 22℃; 3) When stored at 4℃, the Fbg content was 268.86, 268.17(-0.26%), 262.46(-2.38%), 270.50(0.61%), 267.52(-0.50%) and 261.92(-2.58%) mg/ bag, respectively; Stored at 22℃, the Fbg content was 268.86, 265.86(-1.12%), 264.12(-1.77%), 265.89(-1.11%), 266.04(-1.05%) and 261.04(-2.91%) mg/ bag, respectively. There was no statistical significance between the 2 groups and the original 0 h content in each time period(P>0.05). 【Conclusion】 After CAF melting, coagulation factor decreased with the extension of storage time, especially the decrease of factor Ⅷ, followed by factor V, while Fbg basically unchanged. Comparison between the two groups showed that, factor Ⅷ decay rate is slower, factor V decay rate is faster of storage at 22℃. CAF should be transfused as soon as possible after melting. If the delay is unavoidable, for the delay time less than 12 h, storage at 4℃ is recommended, fot the delay time more than 12 h and less than 24 h, storage at 22℃ is recommended.

In view of the problem of slow development of intensive care medicine in Xizang, the research team made full use of the national partner assistance to Xizang, gathered resources across all cities in Xizang, and formed a national academic platform for critical care medicine in plateau areas. Adhering to the academic orientation with hemodynamics as the main topic, critical care ultrasound as the bedside dynamic monitoring and evaluation method, and blood flow-oxygen flow resuscitation as the core connotation, we have achieved the goals of improving the critical care talent echelon throughout Xizang, driving the overall progress of intensive care medicine in Xizang, making a figure in China, and focusing on training of top-notch talents.

Objective:To construct a social loss indicator system based on the perspective of epidemic prevention and control against background of emerging major infectious diseases at the national, hospital, and individual levels, and to provide decision-making basis for public general hospitals to formulate prevention and control strategies for emerging major infectious diseases.Methods:Literatures published before December 23, 2020 were retrieved from CNKI, Wanfang, VIP, Web of Science, Google Scholar with such keywords as " infectious diseases" " public health emergencies" " social losses" " COVID-19" " evaluation". Then a preliminary social loss indicator system of public general hospitals for emerging major infectious diseases was constructed from such dimensions as country, hospital, and individual, by implementing literature analysis and expert consultation. Based on such a preliminary system, three questionnaires were designed for the country, hospitals, and individuals and the corresponding data were collected from December 25, 2021 to May 20, 2022. In the end, a factor analysis was made on the questionnaire data to optimize the social loss indicator system and determine the weights of each indicator.Results:The social loss indicator system consists of 14 level-1 indicators and 60 level-2 indicators. Level-1 indicators at country aspect consisted of government governance capacity, direct economic losses, social security, online public opinion, indirect economic losses, and international cooperation; indicators at hospital aspect consisted of hospital manpower, material resources, and information resources, hospital services, and hospital operations; indicators at the individual aspect consisted of physical health, psychological and social health, and external environment. Level-1 indicators of the highest weight at all aspects were social security, manpower, material and information resources, as well as physiological health. And the level-2 indicators of the highest weight were high medical expenses, overwork for staff other than doctors and nurses and medical insurance.Conclusions:The social loss indicator system for emerging major infectious diseases constructed in this study proves scientific and reasonable, helpful for the formulation of prevention and control strategies of public general hospitals.

Objective: To study the current situation of sleep quality of senior high school students and analyze the influencing factors, so as to provide a reference for targeted sleep quality improvement of high school students. Methods: A total of 4 793 senior high school students of different grades were recruited from four different regions (Shanghai, Qinghai, Macao, Henan) in 2020. The information of demographic, sleep status, mental factors, living habits and other information were collected by questionnaire. Descriptive statistics and Logistic regression were used to analyze sleep quality and its influencing factors of the senior high school students. Results: About 24.2% of senior high school students reported sleep problems and 71.4% slept less than 7 hours at night. Interpersonal sensitivity, depression, anxiety symptoms, learning, family and interpersonal stress had significant effects on sleep problems in senior high school students( χ 2=1 147.98, 1 228.35, 1 032.54, 371.05, 497.61, 475.39, P <0.01). Drinking coffee and tea, drinking alcohol, smoking (including secondhand smoke), long term use of electronic products, late sleep, bedtime diet, poor sleep environment, parents with sleep problems were the risk factors for sleep disorders of senior high school students, regular exercise was the protective factors for sleep disorders of senior high school students, the influence was significant ( χ 2=160.28, 127.73 , 108.02, 113.27, 470.91, 340.95, 72.02, 155.53, 116.30, 76.96, 28.75, 12.89, P <0.05). Conclusion The sleep quality of senior high school students needs to be improved. Schools and parents should pay attention to the mental health and living habits of senior high school students to help them improve their sleep quality and grow up smoothly.

Objective:To investigate the role of peptidyl arginine deiminase 4 (PAD4) in anti-β 2GP1/β 2GP1 complex-induced formation of neutrophil extracellular trapping networks (NETs). Methods:Peripheral blood neutrophils were isolated from healthy humans by density gradient centrifugation. PAD4 expression was detected by Western blot after the neutrophils were incubated with anti-β 2GP1/β 2GP1 complex (100 μg/ml) for a certain period of time. PAD4 inhibitor Cl-amidine (10 μmol/L) was used to pretreat neutrophils. Changes in the expression of citrullinated histone 3 (CitH3) at protein level and the relative content of myeloperoxidase (MPO)-DNA were detected by Western blot and ELISA, respectively. A mouse thrombus model of antiphospholipid syndrome (APS) was established by inferior vena cava stenosis. Intervention experiments were performed by intraperitoneal injection of Cl-amidine (50 mg/kg). The expression of CitH3 at protein level in plasma was detected by Western blot. The concentration of circulating free DNA (cf-DNA) in plasma was measured with fluorescent staining. Thrombus in inferior vena cava was collected and weighted to evaluate whether inhibiting the activity of PAD4 would suppress the APS-IgG-induced formation of NETs and thrombosis. Differences among groups were analyzed by t test or one-way analysis of variance (ANOVA). Results:The expression of PAD4 induced by anti-β 2GP1/β 2GP1 complex was significantly down-regulated in the cytoplasm, but increased in the nucleus [(3.67±0.32) vs (1.47±0.19), t=10.22, P<0.05; (0.57±0.19) vs (2.97±0.31), t=11.49, P<0.05]. Cl-amidine significantly inhibited the anti-β 2GP1/β 2GP1 complex-induced expression of CitH3 protein by neutrophils [(2.46±0.47) vs (0.46±0.13), t=12.24, P<0.01], and reduced the MPO-DNA content in the culture supernatants [(4.09±0.94) vs (2.80±0.57), t=4.23, P<0.05]. In vivo, Cl-amidine significantly inhibited the expression of CitH3 protein [(3.97±0.56) vs (1.09±0.45), t=11.83, P<0.01] and decreased the content of cf-DNA [(2 685.0±735.8) vs (1 784.0±577.0), t=3.93, P<0.05] in plasma of APS mice. Compared with the experimental APS mice in the control group, the weight of thrombus in the APS mice pretreated with Cl-amidine was significantly reduced [(8.22±3.06) vs (4.89±1.90), t=2.27, P<0.05]. Conclusions:PAD4 was involved in the formation of NETs induced by anti-β 2GP1/β 2GP1 complex, which might play an important role in APS thrombosis.

Objective:To explore experiential teaching to physical restraint training for intensive care nurses and its effects in reducing restraint actions.Methods:903 inpatients in ICU from January 2017 to October 2018 in a ClassⅢ Grade A hospital in Shijiazhuang were selected as the subjects by convenient sampling. 491 patients from January to October 2017 were set as the control group, and 412 patients who implemented the experiential teaching method from January to October 2018 were set as the test group. The control group was given routine restraint nursing, and the test group was given reduced restraint nursing. The physical restraint rates, qualified rates of physical restraint, writing quality of physical restraint nursing documents and patient satisfaction scores were compared between the two groups.Results:The restraint rate of patients in the test group was 62.14% (256/412) , which was lower than that 71.69% (352/491) in the control group, the qualified rate of restraint in the test group was 89.06% (228/256) , which was higher than that 80.68% (284/352) in the control group, the differences between the two groups were statistically significant ( P<0.05) . Quality score of writting quality of physical restraint nursing documents in the test group was (98.32±2.22) , and the patients satisfaction score was (98.11±2.53) , which were higher than those in the control group, there were statistically significant differences between the two groups ( P<0.05) . Conclusions:Reduced restraint nursing based on experiential teaching reduces the physical restraint rate of ICU patients, improves the quality of physical restraint and patient's satisfaction of nursing, and it is worthy of clinical promotion.

Objective To detect the levels of neutrophil extracellular traps (NETs) in patients with antiphospholipid syndrome (APS) and to preliminarily explore its formation mechanism.Methods Plasma samples from 27 APS patients and 30 healthy controls were collected.The circulating free DNA (cf-DNA) in plasma was detected by the PicoGreen nucleic acid quantitative assay kit,and the concentration of citrulline histone 3 (CitH3) was analyzed by enzyme-linked immuno sorbent assay (ELISA).The association of cf-DNA/NETs with thrombotic events in APS patients was further analyzed.The neutrophils in healthy controls were separated by density gradient centrifugation and stimulated with anti-β2GPl/β2GPI complex (100 μg/mL) for 4 h,and the cf-DNA/NETs in the culture supernatant was determined.TLR-4 inhibitor-TAK242 (5 μmol/L) was further used to observe whether the stimulation of the anti-β2GPI/β2GPI complex on cells could be intervened.The differences between groups were analyzed by analysis of variance (ANOVA) or rank sum test,Sidak or Dunnett's test were used to compare the mean of multiple samples and the correlation between variables was analyzed by Spearman's correlation test.Results The concentration of cf-DNA/NETs and CitH3 were significantly increased in plasma of APS patients compared with that in healthy controls [175.7(70.6,205.7) ng/ml vs 29.8(7.6,115.7) ng/ml,Z=-3.654,P＜0.05;19.5(7.8,26.4) ng/ml vs 3.3(0.84,10.3) ng/ml,Z=-3.932,P＜0.05],and there was a significant positive correlation between the cf-DNA/NETs and CitH3 (r=0.447,P=0.019).In the APS group,there was no significant difference in cf-DNA/NETs between patients with arterial thrombosis and those with venous thrombosis [177.1(67.8,297.2) ng/ml vs 184.7(82.4,233.9) ng/ml,Z=-0.301,P=0.786],whereas cf-DNA/NETs in the patients who experienced a new thrombotic event in 1 month was significantly higher than those with a history of thrombosis [192.1(83.6,328.8) ng/ml vs 90.0(42.8,184.7) ng/ml,Z=-2.006,P=0.046].In vitro,anti-β2GPI/β2GPI complex (100 μg/ml) stimulated the release of cf-DNA/NETs from neutrophils,which was significantly increased compared with the control group (t=10.39,P＜0.05),while TAK242 significantly inhibited the stimulating effects of anti-β2GPI/β2GPI complex on cells (t=4.22,P＜0.05).Conclusion The level of cf-DNA/NETs in peripheral blood of APS patients is significantly increased,which may play an important role in APS thrombosis.Anti-β2GPI/β2GPI complex induces the formation of cf-DNA/NETs through TLR4 and participates in the pathological process of APS.

OBJECTIVETo identify the genetic cause for a 11-year-old Chinese boy with Meier-Gorlin syndrome (MGS).

METHODSChromosomal microarray analysis (CMA) was used to detect potential variations, while whole exome sequencing (WES) was used to identify sequence variants. Sanger sequencing was used to confirm the suspected variants.

RESULTSThe boy has featured short stature, microtia, small patella, slender body build, craniofacial anomalies, and small testes with normal gonadotropin. A complete uniparental disomy of chromosome 16 was revealed by CMA. WES has identified a novel homozygous mutation c.67A>G (p.Lys23Glu) in ORC6 gene mapped to chromosome 16. As predicted by Alamut functional software, the mutation may affect the function of structural domain of the ORC6 protein.

CONCLUSIONThe patient is probably the first diagnosed MGS case in China, who carried a novel homozygous mutation of the ORC6 gene and uniparental disomy of chromosome 16. The effect of this novel mutation on the growth and development needs to be further investigated.

Base Sequence ; Child ; Chromosomes, Human, Pair 16 ; genetics ; Congenital Microtia ; genetics ; Family Health ; Fathers ; Growth Disorders ; genetics ; Heterozygote ; Humans ; Male ; Micrognathism ; genetics ; Mutation ; Origin Recognition Complex ; genetics ; Patella ; abnormalities ; Polymerase Chain Reaction ; methods ; Sequence Analysis, DNA ; methods ; Uniparental Disomy ; genetics

[Summary] Microcephalic or Majewski's osteodysplastic primordial dwarfism type Ⅱ ( MOPD Ⅱ) is an extremely rare genetic disease mainly caused by pericentrin ( PCNT) gene mutations. This paper reported one 13-year-old boy, who was admitted because of the slow growth for more than 13 years and deepened skin color over six months. He was diagnosed as MOPD Ⅱ associated with a combination of growth hormone deficiency, type 2 diabetes, hypertension, acanthosis nigricans, multiple café-au-lait spots. On magnetic resonance imaging of brain, no vascular malformations such as aneurysms were shown. There were novel compound heterozygous mutations of PCNT gene in the patient, with the nonsense mutations of c. 502C > T ( p. Gln168 * heterozygous variation) and c. 3103C > T (p. Arg1035* heterozygous variation). His father carried a nonsense mutation c. 3103C > T ( p. Arg1035 *heterozygous variation ) and his mother had a nonsense mutation c. 502C > T ( p. Gln168 * heterozygous variation). After treatment with metformin for three months, his blood glucose returned to normal, and acanthosis nigricans was improved. It seems critical to evaluate the abnormal condition of blood vessels regularly for MOPD Ⅱpatients with PCNT gene mutations.

Objective To analyze the clinical feature, diagnosis and treatment of Alstrom syndrome. Method The clinical data of a case of Alstrom syndrome and the result of her ALMS1 sequencing by the two generation sequencing were retrospectively reviewed. Results The 12 year and 10 month old female suffered from dilated cardiomyopathy, obesity, optic nerve diseases, sensorineural hearing loss, high blood glucose and irregular menstruation since one month of birth. Laboratory examination showed she had high testosterone level, hyperglycemia, hyperlipidemia and fatty liver. High-throughput sequencing confirmed there was ALMS1 gene mutation which includes hybrid frameshift mutations of c.5418delC and p.Y1807Tfs*23, and heterozygous nonsense mutation of c.10549C>T and p.Q3517*, and c.5418delC was a new variation reported for the first time. Conclusion Alstrom syndrome is an autosomal recessive genetic disease, which is characterized by multiple organ dysfunction and metabolic syndrome, and can be diagnosed by gene detection.