Objective:To analyze the incidence and risk factors of acute kidney injury in children with biliary atresia after liver transplantation.Methods:The retrospective case-control study was conducted.The clinical data of 115 children with biliary atresia who received liver transplantation for the first time in Beijing Friendship Hospital Affiliated to Capital Medical University from December 2018 to November 2020 were collected.The patients were divided into AKI group ( n=39) and non-AKI group ( n=76) according to the diagnostic criteria of the Kidney Disease Improving Global Outcomes(KDIGO). The differences of clinical indicators between the two groups were compared, and multivariate logistic regression analysis was performed for statistically significant variables ( P<0.05) to further determine the independent risk factors for AKI after liver transplantation. The measurement data of normal distribution were expressed as mean±standard deviation ( ± s), and t-test was used for comparison between groups.Measurement data with non-normal distribution were represented by M( Q1, Q3), and Mann-Whitney U test was used for comparison between groups.Count data were expressed as cases and percentage, and comparisons between groups were made using Chi-square test or Fisher′s exact test. Results:The incidence of AKI in biliary atresia patients after liver transplantation was 33.9%. Univariate analysis showed that there were statistically significant differences in age ( OR=0.721, 95% CI: 0.553-0.938, P=0.014), preoperative infection ( OR=3.307, 95% CI: 1.294-8.468, P=0.013), PELD score ( OR=1.065, 95% CI: 1.031-1.101, P<0.001), serum creatinine numerical value ( OR=0.745, 95% CI: 0.657-0.858, P<0.001), intraoperative red blood cell transfusion ( OR=1.034, 95% CI: 1.028-1.051, P<0.001) and intraoperative plasma transfusion ( OR=1.055, 95% CI: 1.025-1.086, P=0.002) between the AKI group and the non-AKI group ( P< 0.05). Multivariate logistic regression analysis was performed on the selected indicators by univariate analysis, and the results showed that preoperative infection ( OR=3.763, 95% CI: 1.185-11.945, P=0.025) and low serum creatinine ( OR=0.685, 95% CI: 0.570-0.823, P<0.001), intraoperative red blood cell transfusion ( OR=1.033, 95% CI: 1.015-1.056, P=0.028) was independently associated with postoperative AKI ( P<0.05). The inpatient treatment time in ICU and in hospital between the two groups were statistically significant ( P<0.05). Conclusions:Preoperative infection, low creatinine numerical value and intraoperative red blood cell transfusion are independent risk factors for postoperative AKI in children with biliary atresia. AKI may prolong the time in ICU and in hospital.

Objective:To understand the vancomycin dose, therapeutic drug monitoring(TDM) situation and therapeutic effect of children after liver transplantation.Methods:A retrospective analysis of the data of 98 children who received intravenous vancomycin treatment after liver transplantation were conducted in the Department of Critical Care Medicine of Beijing Friendship Hospital from January 2017 to June 2019, including demographic data, vancomycin dose, serum trough concentration, drug-related adverse reactions and clinical outcome data.Results:A total of 98 children received intravenous vancomycin treatment and at least one steady-state TDM blood sample was collected.Among them, 53 cases (54.1%) were male, and the median age was 9 months(5 months to 14 years old). The median first daily dose of vancomycin treatment was 50(30-60)mg/(kg·d), and the median duration of treatment was 14(3-54)days.Only 27.5%(27/98)of the children′s initial trough concentration reached the target concentration (10-20 mg/L), while 26 cases(26.5%) did not reach the target after adjusting the treatment.Six children(6.1%)had renal toxicity caused by vancomycin, and two children had skin rash.The effective treatment rate accounted for 51.7%(15/29). The initial trough concentrations of vancomycin in the effective and ineffective groups were(5.92±3.82)mg/L and(10.43±5.37)mg/L, respectively.The difference was statistically significant ( P=0.041). Conclusion:The rate of intravenous vancomycin in children after liver transplantation is low, and the dose needs to be adjusted individually.

On January 22, 2020, China National Center for Bioinformation (CNCB) released the 2019 Novel Coronavirus Resource (2019nCoVR), an open-access information resource for the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). 2019nCoVR features a comprehensive integra-tion of sequence and clinical information for all publicly available SARS-CoV-2 isolates, which are manually curated with value-added annotations and quality evaluated by an automated in-house pipeline. Of particular note, 2019nCoVR offers systematic analyses to generate a dynamic landscape of SARS-CoV-2 genomic variations at a global scale. It provides all identified variants and their detailed statistics for each virus isolate, and congregates the quality score, functional annotation,and population frequency for each variant. Spatiotemporal change for each variant can be visualized and historical viral haplotype network maps for the course of the outbreak are also generated based on all complete and high-quality genomes available. Moreover, 2019nCoVR provides a full collection of SARS-CoV-2 relevant literature on the coronavirus disease 2019 (COVID-19), including published papers from PubMed as well as preprints from services such as bioRxiv and medRxiv through Europe PMC. Furthermore, by linking with relevant databases in CNCB, 2019nCoVR offers data submission services for raw sequence reads and assembled genomes, and data sharing with NCBI. Collectively, SARS-CoV-2 is updated daily to collect the latest information on genome sequences, variants, hap-lotypes, and literature for a timely reflection, making 2019nCoVR a valuable resource for the global research community. 2019nCoVR is accessible at https://bigd.big.ac.cn/ncov/.

Objective: To analyze clonal evolution and clinical significance of trisomy 8 in patients with acquired bone marrow failure. Methods: The clinical data of 63 patients with acquired bone marrow failure accompanied with isolated trisomy 8 (+8) from June 2011 to September 2018 were analyzed retrospectively, the clonal evolution patterns and relationship with immmunosuppressive therapy were summarized. Results: Totally 24 male and 39 female patients were enrolled, including 39 patients with aplastic anemia (AA) and 24 patients with relatively low-risk myelodysplastic syndrome (MDS) . Mean size of+8 clone in MDS patients[65% (15%-100%) ]was higher than that of AA patients[25% (4.8%-100%) , z=3.48, P=0.001]. The patients were was divided into three groups (<30%, 30%-<50%,and ≥50%) according to the proportion of+8 clone. There was significant difference among the three groups between AA[<30%:55.6% (20/36) ; 30-50%: 22.2% (8/36) ; ≥50%22.2% (8/36) ]and MDS patients[<30%:19.0% (4/21) ; 30%-<50%:19.0% (4/21) ; ≥50%61.9% (13/21) ] (P=0.007) . The proportion of AA patients with+8 clone <30% was significantly higher than that of MDS patients (P=0.002) ; and the proportion of AA patients with+8 clone ≥50%was significantly lower than that of MDS patients (P=0.002) . The median age of AA and MDS patients was respectively 28 (7-61) years old and 48.5 (16-72) years old. Moreover, there was no correlation between age and+8 clone size in AA or MDS (r_s=0.109, P=0.125; r_s=-0.022, P=0.924, respectively) . There was statistical difference in total iron binding capacity, transferrin and erythropoietin between high and low clone group of AA patients (P=0.016, P=0.046, P=0.012, respectively) , but no significant difference in MDS patients. The immunosuppressive therapy (IST) efficacy of AA and MDS patients was respectively 66.7% and 43.8% (P=0.125) . Comparing with initial clone size (27.3%) , the +8 clone size (45%) of AA patients was increased 1-2 year after IST, but no statistical difference (z=0.83, P=0.272) . Consistently, there was no significant change between initial clone size (72.5%) and 1-2 year clone size (70.5%) after IST in MDS patients. There was no significant difference in IST efficient rate between +8 clone size expansion and decline group of in AA patients at 0.5-<1, 1-2 and>2 years after IST. We found four dynamic evolution patterns of +8 clone, which were clone persistence (45%) , clone disappearance (30%) , clone emergence (10%) and clone recurrence (15%) . Conclusions AA patients had a low clone burden, while MDS patients had a high burden of +8 clone. The +8 clone of AA patients didn’t significantly expanded after IST, and the changes of +8 clone also had no effect on IST response.

OBJECTIVE: To analyze the clinical characteristics and genetic basis of a child affected with Glass syndrome. METHODS: Clinical manifestations and auxiliary examination results of the child were analyzed. Potential mutation was detected with next generation sequencing and validated by Sanger sequencing. RESULTS: The child has featured growth and mental retardation, delayed speech, cleft palate, crowding of teeth, and downslanting palpebral fissures. DNA sequencing revealed a de novo heterozygous missense mutation c.1166G>A (p.R389H) in exon 8 of the SATB2 gene in the child. CONCLUSION The heterozygous mutation c.1166G>A (p.R389H) of the SATB2 gene probably account for the Glass syndrome in the patient.
Abnormalities, Multiple ; genetics ; Child ; Chromosome Deletion ; Chromosomes, Human, Pair 2 ; Humans ; Intellectual Disability ; genetics ; Matrix Attachment Region Binding Proteins ; genetics ; Mutation ; Transcription Factors ; genetics

Objective To detect the serum level of 3-carboxy-4-methyl-5-propyl-2-furanpropanoic acid (CMPF),a significant metabolite offish oil,in subjects with normal glucose tolerance (NGT) in local communities,and to investigate the association of CMPF with fatty acid metabolism.Methods A total of 272 NGT participants from screening for diabetes in Shanghai in 2013 were enrolled.Anthropometric measurements,biochemical evaluation,and questionnaire interview were performed for all the participants.The participants were divided into normal weight group [body mass index (BMI) ≤23.9 kg/m2,n =143] and overweight/obesity group (BMI ≥ 24 kg/m2,n =129).The serum CMPF concentrations were determined using an enzyme-linked immunosorbent assay.Results Serum CMPF level in overweight/obesity group was lower than that in normal weight group [96.50 (46.11,169.56) μmol/L vs 153.20 (83.16,282.97) μmol/L,P＜0.05].The serum CMPF level was negatively correlated with BMI (r =-0.256,P＜0.01),triglycerides (r =-0.175,P =0.004),and free fatty acid (r =-0.126,P =0.041) according to bivariate correlation analyses.A multivariate stepwise linear regression analysis showed that the serum CMPF level was independently associated with BMI,triglycerides,free fatty acid,and HbA1C.A logistic regression analysis showed that the CMPF was a protective factor against obesity (OR =0.324,95％ CI 0.158,0.664).Conclusion Serum CMPF level is reduced in overweight/obese subjects.CMPF is beneficial to lipid metabolism.

Objective: To determine the valuable hemolytic characteristics in differential diagnosis of paroxysmal nocturnal hemoglobinuria (PNH), autoimmune hemolytic anemia (AIHA) and hereditary spherocytosis (HS). Method: The clinical and hemolytic characteristics of 108 PNH patients, 127 AIHA patients and 172 HS patients diagnosed from January 1998 to April 2017 were compared. Results: ①Reticulocyte percentage (Ret%) of PNH patients [6.70% (0.14%-22.82%)] was significantly lower than that of AIHA [14.00%(0.10%-55.95%), P<0.001] and HS patients [11.83%(0.60%-57.39%), P<0.001]. The Ret% in PNH patients were significantly lower than those in AIHA and HS patients at the same levels of anemia, except for in mild anemia between PNH and AIHA patients. However, when comparing the Ret% between AIHA and HS patients, there was significant difference only in mild anemia [7.63%(1.87%-29.20%)% vs 11.20%(3.31%-22.44%), z=-2.165, P=0.030]. ②The level of TBIL in HS patients was significantly higher than that in AIHA and PNH patients [79.3 (11.2-244.0) μmol/L vs 57.6 (7.6-265.0) μmol/L, z=5.469, P<0.001; 79.3(11.2-244.0) μmol/L vs 26.2(4.6-217.7) μmol/L, z=-2.165, P<0.001], and the proportion of HS patients with TBIL more than 4 times the upper limit of normal (ULN) (64.1%) was significantly higher than that of AIHA (37.7%, χ²=19.896, P<0.001) and PNH patients (4.6%, P<0.001). ③The LDH level of PNH patients was significantly higher than that of AIHA and HS [1 500 (216-5 144) U/L vs 487 (29-3 516) U/L, z=-9.556, P<0.001; 1 500 (216-5 144) U/L vs 252 (132-663) U/L, z=-11.518, P<0.001], and the proportion of PNH patients with LDH more than 1 000 U/L (79.1%) was significantly higher than that of AIHA patients (13.0%, χ²=93.748, P<0.001) and HS patients (0, P<0.001). ④Splenomegaly occurred in 43.5% of PNH patients, including 16.0% with severe splenomegaly. In contrast, the occurrence of splenomegaly was 98.6% in AIHA patients and 100.0% in HS patients (P<0.001), and 63.0% of AIHA patients (P<0.001) and 90.4% of HS patients (P<0.001) were with severe splenomegaly. ⑤The prevalence of cholelithiasis in HS patients was up to 43.1%, significantly higher than that in AIHA patients (10.5%, P<0.001) and PNH patients (2.9%, P<0.001). Conclusion The comprehensive assessment of the five hemolytic characteristics is simplified, practical and efficient, with great clinical significance, providing specific indicators for differential diagnosis and efficient approach for making further work-up.

Objective To explore the correlation between the self-management and self-efficacy of senile patients with chronic heart failure (CHF). Methods In this study, the conveninent sampling method was adopted. From June to December 2016, totally 416 senile CHF patients hospitalized in the Department of Cardiology of 5 general class Ⅲ grade A hospitals in Xi'an were investigated with the general demographic data questionnaire, self-management scale for CHF patients and chronic disease self-efficacy scale. Results The score of self-management in the senile CHF patients in Xi'an totaled (50.76±9.98), with a scoring rate of 63.5%; the scores of medication management, food and beverage management, psychological and social management and symptom management were (13.38±3.38), (7.89±2.14), (12.81±2.91) and (16.69±4.26), with the scoring rates of 66.9%, 65.8%, 64.1% and 59.6%, respectively, showing a medium level; and there was statistically significant difference in the total self-management score between patients with different marital status, per capita income and heart function grading (t=-2.443, 10.906, 16.229;P<0.05). The score of self-efficacy totaled (34.30±11.90), with a scoring rate of 57.17%, showing a medium level. The correlation analysis showed that there was a positive correlation between self-efficacy and self-management (r=0.161, P< 0.05). Conclusions The self-management and self-efficacy of the senile CHF patients in Xi'an remain at a medium level, which could be possibly improved, and the two are closely correlated. The results suggest that we should pay more attention to the patient's management confidence in the disease itself, attach importance to their psychological and social support and enhance their self-efficacy, thus improving their self-management. This is of great significance in controlling CHF and improving the patient's quality of life.

Objective To investigate the diagnostic value of Enzymes linked immunosorbent assay(ELISA)detection of anti phospholipase A2 receptor antibody(anti-PLA2R)in idiopathic membranous nephropathy patients.Methods All articles published from January 2012 to April 2017 about ELISA test on anti phospholipase A2 recepeor antibody as a maker for IMN were collected, and the quality of the studies was evaluated by using the guidelines of the updated Quality Assessment of Diagnostic Accuracy Stud-ies(QUADAS-2)tool.According to the inclusion criteria and exclusion criteria,the research objects were selected and the data were extracted,and the analysis was carried out by Meta-Disc1.4 software.The results are summarized on the basis of sensitivity,speci-ficity,and summary receiver operating characteristic curve(ROC curve).Results Ten articles were selected according to inclusion criteria and 1517 cases were included in the study.The combined sensitivity was 0.68[95% CI(0.64,0.70)];the merge specificity was 0.92[95% CI(0.90,0.95)],and the subjects'working curve showed AUC 0.807 4.Conclusion The detection of anti-PLA2R by ELISA is effective in the diagnosis of idiopathic membranous nephropathy,which can be used as an effective supplement for the diagnosis of idiopathic membranous nephropathy.

Objective To analyze the characteristics of change in plasma cholesterol level in patients with sepsis, and to explore its relationship with prognosis and its clinical significance. Methods A retrospective analysis was conducted. 568 patients with sepsis admitted to Department of Critical Care Medicine of Beijing Friendship Hospital Affiliated to Capital Medical University from August 2013 to August 2015 were enrolled, and 475 patients without sepsis hospitalized in the same period served as the control. The basic clinical data of the two groups were collected, and the results of blood fat and biochemical parameters were compared. The patients with sepsis were divided into death group and survival group, and risk factors influencing the prognosis of patients with sepsis were analyzed with multivariate logistic model regression analysis. Results Compared with non-sepsis patients, the levels of plasma total cholesterol (TC) and high density lipoprotein cholesterol (HDL-C) in the patients with sepsis were significantly lower [TC (mmol/L): 2.5±1.2 vs. 3.4±1.4, t = 4.274, P = 0.021; HDL-C (mmol/L): 1.6±0.9 vs. 2.5±0.8, t = 3.413, P = 0.018], and that of low density lipoprotein cholesterol (LDL-C) showed no statistically significant difference (mmol/L: 1.9±0.9 vs. 2.1±0.9, t = 0.749, P = 0.614). In the patients with sepsis, the patients in death group (n = 227) were older than those of the survival group (n = 341, years: 74.3±15.5 vs. 65.5±17.5, t = 4.037, P = 0.012), serum creatinine (SCr) was higher than that of survival group (μmol/L: 251.0±115.6 vs. 167.4±108.7, t = 3.254, P = 0.023), the levels of plasma TC, HDL-C and LDL-C were significantly lower than those of survival group [TC (mmol/L): 2.2±1.6 vs. 2.9±1.1, t = 3.057, P = 0.023; HDL-C (mmol/L): 1.4±0.8 vs. 1.9±0.8, t = 4.692, P = 0.016; LDL-C (mmol/L): 1.7±0.7 vs. 2.0±0.8, t = 2.541, P = 0.038]; there was no significant difference in the proportion of male, body mass index (BMI), based disease, intensive care unit (ICU) hospitalization time, the severity of the disease and other biochemical indexes between two groups. With single factor analysis with indicators of statistical significance as a covariate into binary logistic regression equation, the results show that age was the risk factor of death in patients with sepsis [odds ratio (OR) = 1.024, 95% confidence interval (95%CI) = 1.010-1.048, P = 0.009], and TC was the protective factor on the prognosis of patients with sepsis (OR = 0.747, 95%CI = 0.682-0.811, P = 0.013). Conclusions Plasma cholesterol levels in patients with sepsis were significantly lowered, and the levels in death group was significantly lower than that in the survival group. TC may be used as a clinical indicator to assess the outcome of patients with sepsis.