Objective To explore the clinical characteristics of neonatal-onset carbamoyl phosphate synthetase I deficiency (CPS1D). Methods Clinical data and result of genetic detection of one neonate with CPS1D were retrospectively analyzed. The pertinent literature was reviewed. Results A 3-day old girl, with onset symptoms of nonspecific performance, such as poor feeding, less activity, tachypnea, and seizures. After fasting, anti-infection, and respiratory support etc. the condition was improved. However, the condition deteriorated and developed rapidly after feeding restarted. MRI showed extensive cerebral white matter lesions. Blood ammonia?>?500 μmol/L. Gene detection found two heterozygous mutations in pathogenic gene CPS1 in twentieth exon of c.2407C?>?G (p.803, R, G) and fourth exon C.323G?>?A (p.108, G, E), according to which CPS1D was diagnosed finally. Conclusions For neonate with normal birth, had feeding difficulty, seizures, and consciousness disorder after establishment of normal feeding, if blood ammonia level significantly increased, the blood and urine amino acids analysis and gene detection should be performed to confirm the diagnosis.

Objective Self-management was adopted to intervene patients with breast cancer during chemotherapy in order to know about its effects on breast cancer patients′medical compliance behavior. Methods Samples were 154 breast cancer patients undergoing chemotherapy after surgery in breast surgery of the first affiliated hospital of Zhengzhou university. They accepted the docetaxel and cyclophosphamide (TC) program of chemotherapy. Patients were divided into two groups by random number table, one group as control group(80 patients) and the other group as experimental group (74 patients). Different nursing care were conducted during the two groups, the control group patients given routine nursing care, the experimental group patients given routine nursing care and self-management. The medical behavior compliance and upper extremities edema incidence for the two groups of patient were surveyed after the intervention and then evaluate the effect of the intervention. SPSS (version 17.0) was used to carry out statistical analysis. Results The total score of medical compliance behavior, the experimental group was (18.36±1.30) points, the control group was (16.70±1.57) points, the experimental group was higher than the control group, and there was statistically significant difference between two groups(t=7.093, P<0.05).The experimental group scores were higher than the control group in the functional exercise compliance (4.33±0.77 vs. 3.55±0.90), prevent or reduce the edema compliance (4.41± 0.61 vs. 3.84±0.88), PICC or venous access port of regular maintenance compliance (4.62±0.52 vs. 4.33± 0.57)(t=5.704,4.594,3.268,P<0.05);There were statistically significant differences between two groups (t=5.704,4.594,3.268,P < 0.05). There was no statistically significant difference between two groups (P >0.05) in scores of chemotherapy compliance on time.The influence of self-management on the incidence of upper extremities edema was as follow: The upper extremities edema incidence in control patients was 23.68% (18/76) which was higher than 11.11% (8/72) in the experimental group (χ2=4.036, P < 0.05). Conclusions Given self- management intervention for breast cancer patients with postoperative chemotherapy, the medical behavior compliance was increased, and the incidence of upper extremities edema was decreased.

Objective To investigate the clinical and immunological features,therapeutic response as well as prognosis of adult onset Still's disease (AOSD).Methods AOSD was diagnosed in 137 patients referred to our department.Clinical and immunological data were retrospectively analyzed.Therapeutic response and prognosis were systemically reviewed during the follow-up period.Intergroup incidence divergence was analyzed by chi-square test.Cox regression analysis was adopted to determine factors related with relapse.Results Fever,rash and arthritis were the cardinal clinical features of AOSD patients.Elevated inflammatory indices including ferritin (128 suhjects,97.1％ ) along with neutrophilia and liver dysfunction were the main laboratory findings.Ninety-eight patients were followed up and 75％ (73 subjects) had achieved complete remission after 4 weeks treatment.Forty-one patients (42％) who had achieved remission relapsed during follow-up period.Combination of glucocorticoid steroid and disease modifying antirheumatic drugs (DMARDs) were more effective than glucocorticoid steroid only in inducing remission and preventing relapse.More patients received glucocorticoid combined with methotrcxate and hydroxychloroquine achieved remission (8 of 8 patients) than patients who were treated with glucocorticoid and methotrexate (25 of 28 patients,89％ ) and those treated with glucocorticoid and hydroxychloroquine (14 of 16 patients,88％ ).Patients with glucocorticoid were more likely to suffer disease recurrence than those who took glucocorticoid combined with DMARDs (61％ vs 29％,P=0.004).Cox regression analysis suggested that methotrexate had protec-tive effect against recurrence [RR=0.418,95％CI (0.192-0.909),P=0.028].5％ of patients were diagnosed to other diseases during the follow up period.Conclusion Initial treatment with combined glucocorticoid and DMARDs is beneficial to induce remission and prevent reoccurrence.Methotrcxate has a protective effect against recurrence.

BACKGROUND:High expression of stem cell transcription factors, Sox2 and Oct4, has been confirmed to be closely related to the occurrence of lung cancer.OBJECTIVE:To explore the expression of Sox2 and Oct4 in lung cancer tissues and its correlation with microvascular generation and clinical-pathologic features.METHODS:The expression levels of Sox2 and Oct4 and microvessel density (MVD) in lung cancer tissues (60 cases) and normal tissues (60 cases) were examined by immunohislochemistry. Then, the correlation of the expression of Sox2 and Oct4 and MVD value with clinical-pathologic features in lung cancer was analyzed.RESULTS AND CONCLUSION:The positive expression of Sox2 and Oct4 was 46.67% (28/60) and 71.67% (43/60) in lung cancer tissues, respectively, while Sox2 and Oct4 were negatively expressed in normal tissues, and there was a significant difference between lung cancer and normal tissues (P < 0.001). The MVD value was 16.22±2.18 in lung cancer tissues, which was significantly higher than that in normal tissues (4.36±2.07) (P < 0.001). Expression of Sox2,Oct4 and MVD value were correlated with TNM stage, differentiation degree, ymph node metastasis, vessel invasion,and liver metastasis (all P < 0.05), but not with the patient's age and gender (both P > 0.05). The MVD value with Sox2 and Oct4 positive expression was significantly higher than that with Sox2 and Oct4 negative expression (P < 0.001).Spearmen analysis results showed that there was no correlation between Sox2 and Oct4 in lung cancer tissues (r=2.752,P > 0.05). To conclude, the high expression of Sox2 and Oct4 has a high correlation with the initiation and progression of lung cancer, and has positive correlation with the MVD, both of which are significantly concerned with the microvascular generation, invasion and hematogenous metastasis of lung cancer.

Objective The objective of the present study was to evaluate the early auditory development in hearing impaired infants by LittlEARS? auditory questionnaire .Methods A total of 162 hearing impaired infants participated in the study .LEAQ consists of 35 items ,which were further divided into receptive auditory behavior , semantic auditory behavior ,and productive auditory behavior .The assessment was performed before hearing aid fit‐ting ,3 ,6 and 12 months after hearing aid fitting ,respectively .Results LEAQ scores improved dramatically in the first 12 months after hearing aid fitting ,total LEAQ scores prior to hearing aid fitting ,3 ,6 and 12 months after hearing aid fitting were 10 .47 ,17 .73 ,20 .64 and 26 .47 ,respectively .The receptive auditory behavior performance improved significantly after 3 months of hearing aid use .The duration of hearing aid use for dramatic improvement of semantic auditory behaviors ,and productive auditory behavior was 6 and 12 months ,respectively .The age of hearing aid fitting ,living environments ,degrees of parent education and degrees of hearing loss influenced LEAQscores significantly .Conclusion Hearing impaired infants exhibited significant auditory improvement in the first 12 months after hearing aids fitting .Early hearing aid fitting ,high level of parent education and well living environ‐ments promote preverbal auditory development .LEAQ is an effective tool for clinical auditory assessment and moni‐toring in infants and little toddlers .

Objective : To investigate the awareness of nutritional health knowledge regular unpaid blood donors in Zhoushan City, Zhejiang Province, so as to provide insights into nutrition and health education among unpaid blood donors. Methods: The regular unpaid blood donors were sampled using a convenient sampling method from Zhoushan Central Blood Station from January 1, 2021 to June 30, 2022. The nutritional health knowledge was investigated using the Chinese Nutrition Health Knowledge Questionnaire for Adults compiled by Chinese Center for Disease Control and Prevention, and the awareness and source of nutritional health knowledge was descriptively analyzed. Results: Totally 526 questionnaires were allocated, and 502 valid questionnaires were recovered, with an effective recovery rate of 95.44%. The respondents included 240 men (47.81%) and 262 women (52.19%), and included 343 individuals at ages of 18 to 44 years (68.33%), and 159 individuals at ages of 45 years and older (31.67%). The overall awareness of nutritional health knowledge was 14.54% among regular unpaid blood donors in Zhoushan City, and a higher awareness was seen among female regular unpaid blood donors (17.56%) than among males (11.25%) (P<0.05), while the awareness of nutritional health knowledge was significantly higher among respondents at ages of 18 to 44 years than among those at ages of 45 years and older (16.91% vs. 9.43%, P<0.05). The awareness of nutritional health knowledge was significantly higher among respondents with an educational level of junior college and above than among those with an educational level of high school/technical secondary school/technical school (17.24% vs. 12.22%, P<0.05), and the awareness was significantly higher among respondents with healthcare-related occupations than among those with other occupations (16.44% vs. 14.22%, P<0.05). Wechat, Weibo, Tik Tok and Kuaishou were main routes to obtain nutritional health knowledge (83.86%). Conclusions The awareness of nutritional health knowledge is low among regular unpaid blood donors in Zhoushan City. Men, middle-aged and elderly residents and residents with a low educational level are targets that should be given a high priority for nutritional health education, and new media may be fully utilized for nutritional health education.

Objective To explore the expression and change of ski-interacting protein (SKIP) in rats after spinal cord injury. Methods A total of 60 adult female Sprague-Dawley rats were randomly divided into sham group (n=30) and spinal cord injury (SCI) group (n=30), each group was further divided into five time points including one day, three days, five days, seven days, and 14 days with six rats in each time points. The model was established at T10 with modified Allen's technique, and the sham group only bit the lamina of rats. The hindlimbs behavior was assessed with Basso-Beattie-Bresnahan (BBB) score at each time point. The pathological changes of spinal cord neurons were detected with Nissl staining. The expression of SKIP were observed with immunofluorescence staining. Results The BBB scores were signif-icantly lower in each time point in SCI group than in the sham group (t>48.267, P<0.001). Compared with the sham group, Nissl bodies in the cytoplasm of spinal cord neurons began to disintegrate, coalesce and irregularly distribute, the neurons began to degenerate and die on the fifth day, and the damage deteriorated on the 14th day. Immunofluorescence staining showed that SKIP expression was mainly expressed in the gray matter of the spinal cord and little expressed in the white matter. The expression of SKIP gradually increased after SCI, and reached a peak on the fifth day (t=-17.035, P<0.001) and decreased significantly on the 14th day (t=3.853, P<0.05). Conclusion SKIP may be a new signaling molecule, which play an important role in neuronal apoptosis after SCI.

Objective To investigate therapeutic effect of endoscope?guided bougie dilatation on children with benign esophageal stricture. Methods Data of 71 patients with benign esophageal stricture were retrospective analyzed.Patients were divided into group A (reflux stricture),B (congenital esophageal atresia stricture) and C ( caustic injuries stricture) ,based on different causes. The expansion effectiveness and factors of the three groups were analyzed. Results A total of 885 expansions were performed on 71 patients with the total efficacy rate 94?37%( 67/71) . No statistic differences were shown in expansion effectiveness among the 3 groups; group C ( 14?9 times/case, P < 0?05 ) showed more expansion frequencies than group A (9?1 times/case,P<0?05)and group B(10?7 times/case, P<0?05),more complications than group A(1?22%VS 0,P<0?05) and group B(1?22% VS 0?31%,P<0?05). Conclusion Endoscope?guided bougie dilatation is safe and effective for childrens′ benign esophageal stricture. Caustic injuries, refractory benign esophageal stricture,need more expansions and may be accompanied with more complications.

AIM:To explore the time-dependent change of Ski protein expression in normal and activated astrocytes in rats.METHODS:The astrocytes were obtained from rat cerebral cortex and cultured in vitro.The astrocytes were treated with LPS and scratch injury for activation.Western blot analysis was used to determine glial fibrillary acidic protein (GFAP) and Ski protein levels in activated astrocytes at a series of time points.The indirect immunofluorescence staining method was performed to detect the location of Ski protein in the astrocytes.RESULTS:The protein of GFAP was naturally expressed in the astrocytes, beginning to increase after treated with LPS and scratch injury.Little protein expression of Ski in the normal astrocytes was observed.The Ski protein expression began to increase after treated with 1 mg/L LPS, peaked at 4 d (P<0.05) and then deceased, but was stills higher than that in the normal cells.The protein expression level of Ski after scratch injury was highly consistent with above mentioned.Ski was mainly observed in the nucleus of the normal cells and the cells treated with LPS for 6 d, while it was observed in the cytoplasm 2 and 4 d after treated with LPS.CONCLUSION:The protein of Ski is expressed in the astrocytes, and the expression level is increased in activated astrocytes,mainly located in the nucelus.Ski may plays an essential roles in the processes of activation and proliferation of astrocytes.

Objective To explore the expression and the changes of ski with time in the injured spinal cord in rats. Methods Sixty adult female Sprague-Dawley rats were randomly divided into sham group (n=30) and injury group (n=30), each group were further divided into 1 week, 2 weeks, 4 weeks, 8 weeks and 12 weeks subgroups, with 6 rats in each subgroup. Spinal cord injury at T10 was established with modi-fied Allen's technique (10 g × 25 mm) in the injury group. The hindlimbs behavior of rats was rated with Basso-Beattie-Bresnahan (BBB) scores 1 day, 3 days, 1 week, 2 weeks, 4 weeks, 8 weeks and 12 weeks after spinal cord injury. Three rats in each subgroup were stained with HE staining to observe the pathological changes of the spinal cord and the formation of cavity. The other 3 rats were analyzed with im-munofluorescence staining of ski and semi quantitative analysis. Results The BBB scores of each time point were less in the injury group than in the sham group (P<0.05). Necrosis was the major pathological change in the injury groups 1 and 2 weeks after injury;cystic cavity completely formed 4 weeks after injury, with dense scar tissue around it;there was no significant change in the cavity and scar 8 and 12 weeks after injury, however, the adjacent spinal cord was obviously thinner. Ski expressed little in the normal spinal cord, and expressed more and more after injury, peaked at 8 weeks and decreased then. Ski was mainly observed in white matter in the sham group and 12 weeks injury subgroup, which was in gray matter 2, 4 and 8 weeks after injury. Ski was highly expressed around the cavity in injury center and formed high expression band. Conclusion Ski expresses after spinal cord injury in rats, that may be associated with the activation and prolif-eration of astrocytes and the formation of glial scar.