OBJECTIVE:To investigate the effect of shen-fu injection on toxic reaction relief in the chemotherapy for moderate to advanced non-small cell lung cancer(NSCLC).METHODS:130 patients of NSCLC were randomly divided into treatment group and control group,2 groups both received the second-generation regimen for chemotherapy,the major chemotherapeutic agents included vinorelbine,gemcitabine and paclitaxel,the treatment group was given intravenous shen-fu injection 60ml/d for continuous2weeks plus chemotherapy.RESULTS:The toxic reactions in treatment group significantly decreased compared to that in the control group(P

OBJECTIVETo summarize clinical features and diagnosis of Chinese infantile patients with glycogen storage disease type II (GSD II).

METHODSix infant patients with GSD II diagnosed from January 2012 to June 2014 in the Department of Pediatrics, Peking University First Hospital were enrolled into this study. Clinical information of the 6 patients, including clinical manifestation, blood biochemistry, chest X-ray, echocardiogram, electrocardiogram, acid alpha-glucosidase (GAA) activity and GAA gene mutation analysis by direct sequencing of polymerase chain reaction (PCR) product were reviewed.

RESULTOf the 6 patients, five were female and one was male, five of whom were classic infantile type while the other one was atypical. The age of onset ranged from birth to 3-month-old. All patients had varying degrees of generalized muscle weakness, hypotonia and development retardation or retrogression. Other common findings were feeding difficulties in two patients, tongue weakness in two patients, respiratory distress in four patients, macroglossia in one patient, and hepatomegaly in two patients. Left ventricular hypertrophy and cardiomegaly were obvious in all the six patients. All six patients were found to have a enlarged heart in physical examination, and three patients who underwent a chest X-ray examination had an enlarged heart shadow. Four patients who had an echocardiography were found to have myocardial hypertrophy. The electrocardiogram in three patients showed short PR intervals and high voltage. The creatine kinase (CK) levels were three to seven times elevated. The mildest elevated CK was 441 IU/L, and the highest CK level was 1 238 U/L. Assay of GAA enzyme activity in whole blood showed significantly reduced activity (1.3 nmol/ (spot·d) to 2 nmol/(spot·d)) in the patients tested. Gene sequencing in 4 patients showed 8 pathogenic mutations, including 6 missense mutations, one nonsense mutation and one frameshift mutation. The missense mutations were c.998C > A (p.Thr333Lys), c.1280T > C (p.Met427Thr), c.1760T > C (p.Leu587Pro), c.1924G > T (p.Val642Phe), c.2012T > A (p.Met671Lys) and c.2105G > A (p.Arg702His). The nonsense mutation was c2662G > T (p.Glu888X), and the frameshift mutation was c2812_2813delTG (p.Cys938fs). The 5 classic infantile patients died at the age of 7 to 22 months. The atypical infantile patient was 2 years and five months old according to our latest follow up.

CONCLUSIONInfantile GSD II had similar motor manifestations and cardiac involvements, blood biochemical test, imaging findings, enzyme assays, though there were slight differences. The probability of GSD II should be taken into consideration if an infant has both muscular disease and cardiac involvement.

Asian Continental Ancestry Group ; Female ; Glycogen Storage Disease Type II ; diagnosis ; pathology ; Humans ; Infant ; Infant, Newborn ; Macroglossia ; congenital ; Male ; Muscle Weakness ; Mutation ; Mutation, Missense ; Polymerase Chain Reaction ; alpha-Glucosidases ; genetics ; metabolism

Objective To explore the relationships between the screen time with parent-child relations as well as social ability and behavioral problem in preschool children.Methods A total of 866 children aged 3-6 years old in Dazhou City conducted the sampling survey by adopting the children screen time questionnaire,parent-child relationship self-rating scale and young children's social ability and behavior assessment scale(SCBE-30).The single factor analysis and multinomial Logistic regression method were used to conduct the statistical analysis.Results The surveyed preschool children watching TV every day accounted for 99.31 ％,those playing mobile phones every day accounted for 81.87％ and those using computer accounted for 68.36％s.The average daily screen time at ordinary time was 1.75 h/d,those ≥2 h/d accounted for 16.05％;the average screen time at the weekend was 2.32 h/d,those ≥2 h/d accounted for 46.57％.The univariate analysis showed that the screen time in boy,left behind children,only child,mothers engaging in agriculture of peasant-worker or housewife was longer(P＜0.05);the screen time affected the social ability and behavior problems of children(P＜0.05).The longer the time children spent with their parents every day,the higher the parent-child relationship score(P＜0.05);the screen content affected the parent-child relationship and anxiety withdrawal behavior (P＜0.05).The polynormial regression analysis between the children's screen time with social ability,behavior problems,parent-child relation and contacting screen mode showed that the screen time had the negative effect relation with the social ability and parent-child relation(parents and children questionnaire) score(β=-1.115,-1.728,-1.909,P＜0.05),and had the positive effect relation with the scores of anger attack behavior and anxiety retreat behavior,and individual contacting screen (β=0.982,1.474,0.877,P＜0.05).Conclusion The screen time in preschool children is related with parent-child relation,social ability and behavioral problems.The parental accompany is beneficial to the development of parentchild relations in preschool children.

Objective:To evaluate the effect of intrathecal insulin-like growth factor-1 (IGF-1) on chemotherapy-induced neuropathic pain (NP) in mice.Methods:Forty clean-grade healthy male C57 mice, aged 7-9 weeks, weighing 22-24 g, were divided into 4 groups ( n=10 each) using a random number table method: control group (group C), chemotherapy-induced NP group (group CIPN), low-dose IGF-1 group (group I1) and high-dose IGF-1 group (group I2). In CIPN, I1 and I2 groups, oxaliplatin 5 mg/kg was intraperitoneally injected for 5 consecutive days to establish chemotherapy-induced NP model.Normal saline 0.2 ml was given in group C. After measurement of the pain threshold at 10 days after establishment of the model, IGF-1 0.5 and 1.0 μg were intrathecally injected in group I1 and group I2, respectively.Normal saline 5 μl was intrathecally injected in C and CINP groups.Mechanical withdrawal threshold (MWT) was measured at 3, 5, 8, 10, 11, 13 and 15 days after establishment of the model.After measurement of the pain threshold at 15 days after establishment of the model, the expression of spinal IGF-1, IGF-1receptor (IGF-1R), interleukin (IL)-17A, IL-1β and tumor necrosis factor (TNF)-α was detected, and IGF-1 positive cells were counted using immunofluorescence. Results:Compared with group C, MWT was significantly decreased, the expression of spinal IGF-1 was down-regulated, the count of IGF-1 positive cells was decreased, and expression of IL-17A, IL-1β and TNF-α was up-regulated at 3-25 days after establishment of the model in CINP, I1 and I2 groups ( P<0.05). Compared with group CIPN, MWT was significantly increased at 15 days after establishment of the model in group I1, and MWT was increased, the expression of spinal IGF-1 was up-regulated, the count of IGF-1 positive cells was increased, and expression of IL-17A, IL-1β and TNF-α was down-regulated at 13 and 15 days after establishment of the model in group I2 ( P<0.05). Compared with group I1, the count of IGF-1 positive cells in spinal dorsal horn was increased in group I2 ( P<0.05). There was no significant difference in the expression of spinal IGF-1R among the 4 groups ( P>0.05). Conclusion:Intrathecal IGF-1 can alleviate chemotherapy-induced NP, and the mechanism may be related to inhibiting the inflammatory responses in spinal cord of mice.

Background and objective Recent studies have indicated that Nm23-H1 is found in the nucleus,but previous studies have been based on the overexpression or suppression of Nm23-H1 in the cytoplasm.Due to the lacking nuclear localization signal of Nm23-H1,these results cannot reflect or repeat cells in which Nm23-H1 mainly positioned in nuclei and whether they cause clinical biological effects.Therefore,to explore the effects of transposing Nm23-H1 from the cytoplasm to the nucleus during lung cancer cell proliferation,a vector with a nuclear localization signal of Nm23-H1 was constructed and A549 cells were transfected.Methods Gene recombination technology was used to construct pLentis-CMV-NME1-IRES2-PURO lentiviral vectors using a nuclear localization signal sequence,and the recombinant plasmid was verified using restriction enzyme analysis and sequencing.Nm23-H1 positioning and expression were performed after the stably transfected A549 cells were assessed by Western blot and confocal laser scanning microscope.The A549 cell proliferation was assessed using a cell counting kit-8.Flow cytometry was performed to assess the cell cycle distribution ofA549 cells.Results The directional Nm23-H1 lentiviral vector was successfully constructed within the nucleus.Compared with that of the empty vector group,the proliferation rates of the transfection groups at 72 h,96 h,and 120 h were remarkably increased (P＜0.000,1).Moreover,the empty vector group ofA549 cells in the G0/G1 phase proportion was 35.69％,which was higher than the 28.28％ of the transfection group (t=1.461,P=0.217);furthermore,the transfection group ofA549 cells in the G2/M phase proportion was 58.7％ and that of the empty vector group was 31.30％ (t=4.560,P=0.010).Conclusion Human lung adenocarcinoma cell line A549 cells of Nm23-H1 nuclear localized mainly in the G2/M phase and the nuclear Nm23-H1 promoted A549 cell proliferation in vitro.

Objective:To develop the Nurse Managers Negative Leadership Behavior Scale and test its reliability and validity.Methods:This study is a cross-sectional study. The Nurse Managers Negative Leadership Behavior Scale item pool was formed through literature review and semi-structured interviews. A total of 28 nursing management experts from Hubei Province, Beijing, Guangdong Province and other places were selected for expert consultation to form the first draft of the scale. The scale was further revised through pre-experiment and item analysis. In May 2022, 300 nurses from the Union Hospital, Tongji Medical College, Huazhong University of Science and Technology were selected by convenient sampling for a questionnaire survey to test the reliability and validity of the scale. A total of 300 questionnaires were distributed, and 265 valid questionnaires were recovered, with an effective recovery rate of 88.33%. One week after the survey, 20 nurses were randomly selected to re-issue the questionnaire for the retest reliability test of the scale.Results:Totally, 19 experts completed two rounds of expert consultation, with expert authority coefficient of 0.880, Kendall harmony coefficient of 0.160 and 0.130 ( P<0.05) . Exploratory factor analysis extracted six factors, including neglect of needs, personal attack, right satisfaction, unpredictable behavior, slack work and improper supervision, and the cumulative variance interpretation rate was 75.125%. The confirmatory factor analysis showed that the model fitted well and the factor structure was stable. The Nurse Managers Negative Leadership Behavior Scale included six dimensions and 36 items. The total Cronbach's α coefficient of the scale was 0.880, the split-half reliability coefficient was 0.895, and the retest reliability coefficient was 0.876. The content validity index of the scale was 0.930, and the content validity index of each item was 0.800 to 1.000. Conclusions:The Nurse Managers Negative Leadership Behavior Scale has good reliability and validity, and can be used to evaluate the negative leadership behavior of head nurses in China.

Objective: To investigate the genotype and genetic characteristics of the pathogens associated with the epidemic outbreak of acute gastroenteritis(AGE) in Guangyuan city, Sichuan province. Methods: Eighteen stool samples and 15 anal swab samples from 4 AGE outbreaks were collected from Feb 2017 to May 2017. Norovirus (NoV) nucleic acid was detected by Real-time PCR method , and the positive samples were amplified by conventional reverse transcription-polymerase chain reaction (RT-PCR) and nucleotide sequencing. Results: Four AGE outbreaks were all caused by NoV. And 20 (60.6%) samples were positive for NoV GⅡ. Gene sequence comparison and phylogenetic analysis showed that 4 AGE outbreaks were all caused by GⅡ.P16/GⅡ.2 NoV. All the strains of GⅡ.P16/GⅡ.2 NoV shared high homology in nucleotides. One of the outbreaks was caused by GⅡ.P16/GⅡ.2 and GⅡ.P7/GⅡ.14 NoV co-infection. Conclusions The 4 outbreaks of AGE in Guangyuan city, Sichuan province were major caused by GⅡ.P16/GⅡ.2 NoV, meanwhile GⅡ.P16/GⅡ.2 and GⅡ.P7/GⅡ.14 NoV co-infection existed.

Objectives:To summarize the clinical and genetic characteristics of the patients with isolated methylmalonic acidemia and investigate the strategies for the diagnosis, treatment and prevention.Methods:Three hundred and fourteen patients (180 males, 134 females) with isolated methylmalonic acidemia were ascertained from 26 provinces or cities across the mainland of China during January 1998 to March 2020. Genetic analysis was performed by Sanger sequencing, gene panel sequencing, whole exome sequencing, multiplex ligation-dependent probe amplification or quantitative PCR. According to the age of onset, the patients were divided to early-onset group (≤12 months of age) and the late-onset group (>12 months of age). They were treated by cobalamin, L-carnitine and (or) special diet and symptomatic treatment. Statistical analysis was done using Chi-square test.Results:Fifty-eight of 314 (18.5%) patients were detected by Newborn screening using liquid chromatography tandem mass spectrometry. Five cases (1.6%) had a postmortem diagnosis. Two hundred and fifty-one patients (79.9%) were clinically diagnosed with an age of onset ranged from 3 hours after birth to 18 years. One hundred and fifty-nine patients (71.0%) belonged to early-onset groups, 65 patients (29.0%) belonged to the late-onset group. The most common symptoms were metabolic crises, psychomotor retardation, epilepsy, anemia and multiple organ damage. Metabolic acidosis and anemia were more common in early-onset patients than that in late-onset patients (20.8%(33/159) vs. 9.2% (6/65), 34.6% (55/159) vs. 16.9% (11/165), χ 2=4.261, 6.930, P=0.039, 0.008). Genetic tests were performed for 236 patients (75.2%), 96.2%(227/236) had molecular confirmation. One hundred and twenty-seven variants were identified in seven genes (MMUT, MMAA, MMAB, MMADHC, SUCLG1, SUCLA2, and MCEE), of which 49 were novel. The mut type, caused by the deficiency of methylmalonyl-CoA mutase, was the most common ( n=211, 93%) cause of this condition. c.729_730insTT, c.1106G>A and c.914T>C were the three most frequent mutations in MMUT gene. The frequency of c.914T>C in early-onset patients was significantly higher than that in late-onset patients (8.3% (18/216) vs. 1.6% (1/64), χ 2=3.859, P=0.037). Metabolic crisis was more frequent in mut type than the other types (72.6% (114/157) vs. 3/13, χ 2=13.729, P=0.001),developmental delay and hypotonia were less frequent in mut type (38.2% (60/157) vs. 9/13, 25.5% (40/157) vs. 8/13, χ 2=4.789, 7.705, P=0.030, 0.006). Of the 58 patients identified by newborn screening, 44 patients (75.9%) who were treated from asymptomatic phase developed normally whereas 14 patients (24.1%) who received treatment after developing symptoms exhibited varying degrees of psychomotor retardation. Conclusions:The characteristics of phenotypes and genotypes among Chinese patients with isolated methylmalonic acidemia were analyzed. Expanded the mutation spectrum of the associated genes. Because of the complex clinical manifestations and severe early onset of isolated methylmalonic acidemia, Newborn screening is crucial for early diagnosis and improvement of prognosis. MMUT gene is recommended for carrier screening as an effort to move the test earlier as a part of the primary prevention of birth defects.