Craniopharyngioma is a kind of benign tumor of the relict squamous epithelial cells originated from the pituitary gland of cranial cheek pouch in the process of embryonic development. With an overall incidence of 0.13 cases per 100 000 person-years, they may represent up to 5%-10% of intracranial tumors in children. There is always debate regarding the appropriate treatment for craniopharyngiomas which often present symptomatically given their proximity to critical brain structures, and pose significant surgical challenges. And the complications caused by the treatment such as diabetes insipidus, hypothalamic obesity, and hypopituitarism, that throw out a challenge to the postoperative management and the quality of the patients. Craniopharyngioma should be recognized as a chronic disease requiring constant monitoring along with the clinical and multidisciplinary treatment in order to provide optimal care of surviving patients.

Objective To explore the effect of training course based on the model of practical performance which was designed to improve the humanistic caring ability of nursing postgraduates. Methods After conceived, designed and trained the nursing postgraduates, we combined quantitative research method and qualitative research method to understand the effect and the true feelings of the students after taking the training. Results “Humanistic Caring Ability of Nursing Undergraduates Assessment scale (HCANU)“was used before and after 22 nursing postgraduates accepted the training. After training, their total score (86.06±5.16) was higher than before (67.70±5.00) (t=2.665, P<0.01) and 8 dimensions scores were respectively higher than before(P<0.01 or 0.05). The course has improved the humanistic caring ability of nursing postgraduates, enhanced their empathy of nursing objects and promote self-reflection. Conclusions This training course based on the model of practical performance helps to improve the humanistic caring ability of nursing postgraduates and cultivate their humantistic quality.

Objective To evaluate the effect of community management of diabetic patients with hypertension in Beijing Cuigezhuang community in last three years.Methods A community diabetic management program was started from 2007 in Beijing Cuigezhuang community.Three hundred and seventy six patients who participated in the program for more than 3 years were enrolled in the study, including 196 with type 2 diabetes mellitus (T2DM) only (DM group) and 180 with T2DM and hypertension (DMH). The control rate of blood glucose, blood pressure, lipids and the comprehensive control rate were compared between two groups after 3-year intervention.Results There were no significant differences in age, gender ratio, course of diabetes, education background, monthly income and the history of stoke between two groups;while prevalence of dyslipidemia in DMH group was significantly higher than that in DM group [41.7%(75/180) vs.24.5%(48 196),χ2 =11.938,P=0.001].Compared with the baseline data, the types of antidiabetic drugs used were not significantly changed in two groups after 3-year intervention ( DM group:1.32 ±0.81 vs.1.31 ±0.93, t=-0.155, P=0.877, DMH group:1.43 ±0.72 vs.1.48 ±0.82, t=0,831, P =0.407) .The types of antihypertensive drug in DMH group were significantly decrease. (1.12 ±0.77 vs.1.25 ±0.45, t=2.484, P=0.014), while the rate of statins usage in DM group was significantly increased [13.3%(26/196) vs.5.1%(10/196),χ2 =7.830, P=0.005].The hemoglobin A1c (HbA1c) levels in DM group was decreased [(7.4 ±1.5)% vs.(7.8 ±2.1)%, t=2.586, P=0.011].The systolic pressure [(129 ±12) mmHg (1 mmHg=0.133 kPa) vs.(133 ±16) mmHg, t=3.503, P=0.001] and the diastolic pressure [(80 ±8) mmHg ratio (82 ±10) mmHg, t=2.436, P=0.016] in DMH group were significantly declined. The average LDL-C level [ DM group: ( 3.0 ± 0.9) mmol/L vs.(3.2 ±1.0) mmol/L, t =2.165, P=0.032; DMH group (2.9 ±1.0) mmol/L vs. (3.2 ±1.1) mmol/L, t=3.210, P=0.002] were also significantly decrease.Compared with the baseline, the comprehensive control rates of blood glucose, blood pressure and lipid level were increased in both groups [DM group:9.7% (19/196) vs.6.1%(12/196),χ2 =1.716, P=0.190, DMH group 13.9%(25/180) vs.5.0%(9/180),χ2 =8.315, P =0.004] .Conclusions The community management program is effective for improvement of comprehensive control rates of blood glucose, blood pressure and blood lipids in diabetic patients with hypertension in Beijing Cuigezhuang community.

Objective To analyze clinical characteristics and gene mutation of two patients diagnosed with P450 oxidoreductase deficiency(PORD). Methods Clinical data of 2 patients with PORD was collected from Ruijin hospital. POR gene mutation was analyzed by PCR-Sanger sequencing. A retrospective analysis of literatures concerning PORD was performed. Results Patient 1, female, 16 years old, with 46,XX karyotype, presented with anorectal anomalies, clitoral hypertrophy at birth and irregular menstruation; Patient 2, female, 32 years old, with 46,XX karyotype, showed irregular menstruation and infertility, both without obvious skeletal deformity. Genetic test of POR gene mutation revealed that patient 1 carried a homozygous missense mutation (R457H) and patient 2 carried a heterozygous mutation (R223X/ Y607C). The two mutations (R223X and Y607C) are reported for the first time in China. Conclusion P450 oxidoreductase deficiency which caused by mutations in POR gene has a variety of clinical manifestations, including abnormal steroid hormone synthesis with or without Antley-Bixler syndrome. The affirmative diagnosis should rely on steroid hormone measurement and POR gene analysis.

Objective:To investigate the effect of growth hormone replacement therapy(GHRT) on glucose and lipid metabolism in patients with hypopituitarism.Methods:Clinical data of patients with hypopituitarism who received GHRT in Department of Endocrine and Metabolic Diseases, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine from December 2016 to February 2020 were retrospectively analyzed. The patients were divided into normal glucose regulation(NGR) group and impaired glucose regulation(IGR) group according to their glucose metabolism status before GHRT. The changes of the characteristics of glucose metabolism before and after GHRT were analyzed.Results:A total of 30 patients aged(23.0±5.2) years were included, 23 patients in NGR group and 7 patients in IGR group. After 12 months of GHRT, there were no significant changes in fasting plasma glucose(FPG), 2-hour postprandial plasma glucose(2hPG), and insulin sensitivity index(ISI) in both groups(all P>0.05), while homeostasis model assessment insulin resistance(HOMA-IR) in IGR group was significantly decreased compared with that before GHRT( P<0.05). None of the patients in NGR group progressed to IGR or diabetes mellitus, and none of the 7 patients in the IGR group progressed to diabetes mellitus, while 4 of them recovered from impaired glucose tolerance(IGT) to NGR. Triglyceride, total cholesterol, and low density lipoprotein-cholesterol levels were all significantly decreased in two groups(all P<0.05). Multivariate linear regression analysis showed that the increase of body mass index was an independent risk factor for the increase of FPG and 2hPG( P<0.05). Conclusion:12-month GHRT significantly improved their blood lipid profiles in patients with hypopituitarism without adversely affecting glucose homeostasis.

Background and Objectives: p21, an important member of the Cip/Kip family, is involved in inhibitory effects of RUNX1b overexpression during the early stage of human hematopoiesis. Methods: and Results: We established a human embryonic stem cell (hESC) line with inducible expression of p21 (p21/hESCs). Overexpression of p21 did not influence either mesoderm induction or emergence of CD34＋ cells, but it significantly decreased the production of CD43＋ cells and changed the expression profile of hematopoiesis-related factors, leading to the negative effects of p21 on hematopoiesis. Conclusions In RUNX1b/hESC co-cultures when RUNX1b was induced from D0, perturbation of the cell cycle caused by upregulation of p21 probably prevented the appearance of CD43＋ cells, but not CD34＋ cells. The mechanisms via which CD34＋ cells are blocked by RUNX1b overexpression remain to be elucidated.

The clinical features and laboratory data of two patients with congenital lipoid adrenal hyperplasia (CLAH)were collected. The genomic DNA was extracted from the peripheral blood white cells in the two patients and their family members and the STAR gene was screened for mutations by PCR and Sanger sequencing. Patient 1 was a girl aged 2 years and 8 months,and she visited us because of continual cyanosis for more than two years. Physical examination showed no obvious pigmentation or clitoral hypertrophy,and Tanner stage was B1P1. Clinical examination revealed serum ACTH 1 284.1 pg/ml and 17α-hydoxyprogesterone(17-OHP)0.54 ng/ml, with Karyotype 46, XX. Genetic analysis showed compound heterozygous mutations of c.201_202delCT and c.229C>T in the STAR gene. Her father carried heterozygous c.201_202delCT mutation, and her mother showed heterozygous c.229C>T mutation. Patient 2 was a girl aged 22 years and referred to us because of dark skin for more than 21 years. Physical examination revealed generalized hyperpigmentation,with Tanner stage B5P2. Hormone examination showed ACTH>2 000 pg/ml and serum cortisol 0.77μg/dl. Karyotype analysis revealed 46,XX. Genetic analysis found compound heterozygous mutations of c.64+1G>C and c.707_708delinsCTT in the STAR gene,which descended from her father and mother respectively. Of note,c.64+1G>C is a novel splicing mutation of STAR gene.

【Objective】 Utilizing a specially engineered miR-144-GFP-H1 human embryonic stem cell (hESC) reporter line, this study leverages GFP fluorescence as an indicator of miR-144 expression to gauge the progression of erythropoiesis. The investigation is aimed at elucidating the potential roles of lncRNAs within the erythropoietic framework and conducting an initial assessment of their functional impact. 【Methods】 The miR-144/451-GFP-H1 cell line (hereafter referred to as 144-H1) was utilized for in vitro erythrocyte induction culture. The subpopulations of cells entering the erythropoiesis stage were characterized by the surface molecules CD71 and GPA. The GFP reporter gene of miR-144 served as a critical determinant to distinguish between GFP-positive cells (with a high propensity for erythropoiesis) and GFP-negative cells (with a low propensity for erythropoiesis). Transcriptome sequencing was performed on both groups to identify differentially expressed long non-coding RNAs (lncRNAs). LncRNA entries with potential for validation were selected for preliminary functional verification. The CRISPR/Cas9 gene editing technique was employed to design functional interference strategies for the targeted lncRNAs, obtaining 144-H1 cell lines with knocked-out function of the specific lncRNAs. These knockout cell lines, along with non-knockout 144-H1 cell lines, were used for parallel erythrocyte induction culture to identify differential nodes. This approach preliminarily verified their impact on erythropoiesis in an in vitro development model. 【Results】 1)The constructed 144-H1 cell line was capable of expressing GFP fluorescence upon entering the stage of in vitro erythrocyte induction, indicating the activation of miR-144/451. 2)Within the CD71, GPA double-positive group, significant differences in lncRNA expression were observed between the GFP-positive and GFP-negative subpopulations. 3) Gene editing strategies involving the deletion of sequence segments capable of effectively interfering with the function of multiple lncRNA entries were designed and verified for successful editing. In the knockout cell lines, parts of the lncRNA sequences were directly deleted. 4)In parallel validation experiments of erythrocyte induction culture, cell lines with LINC01569 knocked out exhibited significant differences in flow cytometric subpopulations and cell proliferation capabilities compared to the non-knockout cell lines: ①The knockout cell lines showed sustained high expression of GFP fluorescence. ②The proportion of the CD71-GPA double-positive group in the knockout cell lines continuously decreased during erythrocyte maturation. ③No significant expression of hemoglobin was observed in the knockout cell lines, lacking the characteristic red color. ④The cell proliferation capability of the knockout cell lines was significantly lower than that of the non-knockout cell lines (P<0.05). 【Conclusion】 The successful employment of the 144-H1 cell line facilitated an exploration into the potential functions of lncRNAs in erythropoiesis. This enables the design of more refined in vitro developmental experiments to enhance the precision in capturing lncRNA functions. Among the differentially expressed lncRNA entries, LINC01569 was preliminarily validated to play a regulatory role in erythropoiesis. The functional absence of LINC01569 severely impacts the normal differentiation and proliferation of erythrocytes. The specific regulatory mechanism of LINC01569 in erythropoiesis warrants further investigation and research.

【Objective】 To explore the establishment methods of transgenic human umbilical cord mesenchymal stem cells (hUC-MSCs) overexpressing tumor necrosis factor(TNF)-related apoptosis-inducing ligand (TRAIL) based on the transposons, and attempt to apply it on the nude mice mode with glioma. 【Methods】 PiggyBac transposon system specially designed by us was used to prepare non-targeting and Her2-targeting hUC-MSCs that can stably express TRAIL through puromycin screening. The glioma cells expressing firefly luciferase (U87MG-LUC) were injected into the skull of the immunodeficient mice (BALB/c-nu/nu) with 1×10⁶ cells per mouse. After 7 days of injection, the mice transplanted with U87MG were detected with a small animal living imager to determine the size and location of the tumors in skull. Then we injected the glioma-transplantation nude mouse with two kinds of transgenic hUC-MSCs expressing TRAIL (named as untarget-TRAIL and target-TRAIL, respectively), or the non-transgenic hUC-MSCs (all 1×10⁶ cells per mouse) or PBS (named as WT-MSCs and PBS for negative control) respectively, and then monitored the changes of tumor signals by a small animal living imager every week for 3~4 weeks. 【Results】 After six passages to expand the cells, the both transgenic cell lines can stably express TRAIL gene. Their ratio of green fluorescent protein (GFP) positive cells can reach 93%-97%, and the positive ratio of their MSC-specific surface markers still maintained normal (CD34⁺, CD45⁺, and HLA-DR⁺ all <0.1%, CD90>99%, CD73>88%, and CD105 >60%). The median survival time (d) of U87MG-transplanted nude mice in the groups of untarget-TRAIL, target-TRAIL, WT-MSCs, and PBS was 41 vs 39 vs 24 vs 23(P<0.05). 【Conclusion】 The transgenic hUC-MSCs overexpressing TRAIL gene can significantly prolong the survival time of nude mice with brain glioma.