Background: Maturity-onset diabetes of the young (MODY) due to variants of hepatocyte nuclear factor 1-beta (HNF1β) (MODY5) has not been well studied in the Chinese population. This study aimed to estimate its prevalence and evaluate the application of a clinical screening method (Faguer score) in Chinese early-onset diabetes (EOD) patients. Methods: Among 679 EOD patients clinically diagnosed with type 2 diabetes mellitus (age at diagnosis ≤40 years), the exons of HNF1β were sequenced. Functional impact of rare variants was evaluated using a dual-luciferase reporter system. Faguer scores ≥8 prompted multiplex ligation-dependent probe amplification (MLPA) for large deletions. Pathogenicity of HNF1β variants was assessed following the American College of Medical Genetics and Genomics (ACMG) guidelines. Results: Two rare HNF1β missense mutations (E105K and G454R) were identified by sequencing in five patients, showing functional impact in vitro. Another patient was found to have a whole-gene deletion by MLPA in 22 patients with the Faguer score above 8. Following ACMG guidelines, six patients carrying pathogenic or likely pathogenic variant were diagnosed with MODY5. The estimated prevalence of MODY5 in Chinese EOD patients was approximately 0.9% or higher. Conclusion MODY5 is not uncommon in China. The Faguer score is helpful in deciding whether to perform MLPA analysis on patients with negative sequencing results.

Background: Maturity-onset diabetes of the young (MODY) due to variants of hepatocyte nuclear factor 1-beta (HNF1β) (MODY5) has not been well studied in the Chinese population. This study aimed to estimate its prevalence and evaluate the application of a clinical screening method (Faguer score) in Chinese early-onset diabetes (EOD) patients. Methods: Among 679 EOD patients clinically diagnosed with type 2 diabetes mellitus (age at diagnosis ≤40 years), the exons of HNF1β were sequenced. Functional impact of rare variants was evaluated using a dual-luciferase reporter system. Faguer scores ≥8 prompted multiplex ligation-dependent probe amplification (MLPA) for large deletions. Pathogenicity of HNF1β variants was assessed following the American College of Medical Genetics and Genomics (ACMG) guidelines. Results: Two rare HNF1β missense mutations (E105K and G454R) were identified by sequencing in five patients, showing functional impact in vitro. Another patient was found to have a whole-gene deletion by MLPA in 22 patients with the Faguer score above 8. Following ACMG guidelines, six patients carrying pathogenic or likely pathogenic variant were diagnosed with MODY5. The estimated prevalence of MODY5 in Chinese EOD patients was approximately 0.9% or higher. Conclusion MODY5 is not uncommon in China. The Faguer score is helpful in deciding whether to perform MLPA analysis on patients with negative sequencing results.

Background: Maturity-onset diabetes of the young (MODY) due to variants of hepatocyte nuclear factor 1-beta (HNF1β) (MODY5) has not been well studied in the Chinese population. This study aimed to estimate its prevalence and evaluate the application of a clinical screening method (Faguer score) in Chinese early-onset diabetes (EOD) patients. Methods: Among 679 EOD patients clinically diagnosed with type 2 diabetes mellitus (age at diagnosis ≤40 years), the exons of HNF1β were sequenced. Functional impact of rare variants was evaluated using a dual-luciferase reporter system. Faguer scores ≥8 prompted multiplex ligation-dependent probe amplification (MLPA) for large deletions. Pathogenicity of HNF1β variants was assessed following the American College of Medical Genetics and Genomics (ACMG) guidelines. Results: Two rare HNF1β missense mutations (E105K and G454R) were identified by sequencing in five patients, showing functional impact in vitro. Another patient was found to have a whole-gene deletion by MLPA in 22 patients with the Faguer score above 8. Following ACMG guidelines, six patients carrying pathogenic or likely pathogenic variant were diagnosed with MODY5. The estimated prevalence of MODY5 in Chinese EOD patients was approximately 0.9% or higher. Conclusion MODY5 is not uncommon in China. The Faguer score is helpful in deciding whether to perform MLPA analysis on patients with negative sequencing results.

Background: Maturity-onset diabetes of the young (MODY) due to variants of hepatocyte nuclear factor 1-beta (HNF1β) (MODY5) has not been well studied in the Chinese population. This study aimed to estimate its prevalence and evaluate the application of a clinical screening method (Faguer score) in Chinese early-onset diabetes (EOD) patients. Methods: Among 679 EOD patients clinically diagnosed with type 2 diabetes mellitus (age at diagnosis ≤40 years), the exons of HNF1β were sequenced. Functional impact of rare variants was evaluated using a dual-luciferase reporter system. Faguer scores ≥8 prompted multiplex ligation-dependent probe amplification (MLPA) for large deletions. Pathogenicity of HNF1β variants was assessed following the American College of Medical Genetics and Genomics (ACMG) guidelines. Results: Two rare HNF1β missense mutations (E105K and G454R) were identified by sequencing in five patients, showing functional impact in vitro. Another patient was found to have a whole-gene deletion by MLPA in 22 patients with the Faguer score above 8. Following ACMG guidelines, six patients carrying pathogenic or likely pathogenic variant were diagnosed with MODY5. The estimated prevalence of MODY5 in Chinese EOD patients was approximately 0.9% or higher. Conclusion MODY5 is not uncommon in China. The Faguer score is helpful in deciding whether to perform MLPA analysis on patients with negative sequencing results.

Anomalous aortic origin of a coronary artery（AAOCA）is a rare congenital malformation of the coronary artery，characterized by the coronary orifice origin from the inappropriate sinus of valsalva. Clinical presentations of this condition are occult，and different types present with various clinical manifestations，including exercise-induced angina，syncope，and even sudden death，particularly when the coronary artery follows an interarterial（intramural）course. Coronary computed comography angiography is an effective diagnostic method for precisely delineating the origin and shape of the coronary artery. Surgical correction is recommended upon evidence of myocardial ischemia and potential risk，and offers favorable long-term prognosis post-surgery. Despite low morbidity，limited experience in diagnosis and treatment，early diagnosis and timely treatment are of great importance because of the high potential risk related to the high-risk types. This paper aims to review the advances in the pathophysiology，clinical manifestations，diagnosis，and treatment of AAOCA to enhance recognition among pediatricians and reduce the incidence rate of sudden cardiac death in children with AAOCA.

OBJECTIVE To build a standardized simulation teaching system for resident pharmacists and evaluate its effects， and to provide reference for improving the competency of resident pharmacists. METHODS The established simulation teaching system for pharmacy residents’ standardized training in the study included revising the simulation teaching syllabus， setting up simulation teaching courses， implementing the teaching method through “six types of simulations”， applying objective structured clinical examination （OSCE） for assessment， building a simulation teaching team and strengthening the simulation teaching management. The effect evaluation was perfermed with mixed research method， and qualitative and quantitative research methods were used to collect and analyze data and information. RESULTS ＆＆CONCLUSIONS Compared with the traditional teaching system， the passing rate of graduation examination （71.4% vs. 100%） and the score of after-department examination （[ 76.2±7.8） vs. （90.4±4.9）] under the simulation teaching mode were higher； through questionnaire surveys and qualitative interviews， we found that resident pharmacists who went through simulation teaching gave positive feedback on the role and impact of this system. The simulation teaching system can be used with good generalizability for the standardized training of resident pharmacists， and can provide strong basis and support for the high-quality development of hospital pharmacy.

Objective:To explore the associations of urinary retinol binding protein (RBP) and β 2-microglobulin (β 2-MG) with urinary albumin to creatinine ratio (UACR) and renal function in hospitalized patients with type 2 diabetes mellitus (T2DM). Methods:A total of 1 030 Chinese patients with T2DM were included in this study. The subjects were divided into the UACR normal group (<30 mg/g), microalbuminuria group (30-300 mg/g) and macroalbuminuria group (>300 mg/g). Patients with normal UACR were further divided into two groups according to the estimated glomerular filtration rate (eGFR): the eGFR low group (<90 ml·min -1·1.73m -2) and the normal eGFR group (≥90 ml·min -1·1.73m -2). Urine RBP and β 2-MG levels among the groups were compared. Multiple linear regression analyses were applied to evaluate risk factors of urine RBP and β 2-MG. Results:In all patients ( n=1 030), urine RBP and β 2-MG increased gradually with the increase of UACR across the three groups, the proportions of abnormal urine RBP (>0.7 mg/L) and β 2-MG (>370 μg/L) in these groups were 3.8%, 8.5%, 39.0% ( P<0.001), and 12.9%, 26.7%, 46.8% ( P<0.001), respectively. In the UACR normal group ( n=788), 12.2% of the patients were with eGFR<90 ml·min -1·1.73m -2. The proportion of abnormal β 2-MG (>370 μg/L) was higher in the eGFR low group than that in the eGFR normal group (29.2% vs. 10.7%, P<0.001). Multivariate linear stepwise regression analyses were performed using natural logarithm of urine RBP or β 2-MG as dependent variable, and showed that urine RBP was independently associated with UACR ( β=0.0005, P<0.001), serum creatinine ( β=0.006, P<0.001) and glycosylated hemoglobin A1c ( β=0.050, P=0.001), and β 2-MG was independently correlated with UACR ( β=0.000 4, P<0.001), serum creatinine ( β=0.011, P<0.001), systolic blood pressure ( β=0.005, P=0.031) and fasting blood-glucose ( β=0.027, P=0.046). Conclusions:Urine RBP and β 2-MG are positively associated with high UACR and impaired renal function in T2DM patients, and these changes could occur before UACR and eGFR turned out to be abnormal. It is recommended that urine RBP and β 2-MG be detected as early as possible to identify diabetic kidney disease in patients with normal UACR and eGFR.

OBJECTIVE：To provid e reference for pharmaceutical workers to better understand Novel Coronavirus Infection ： Expert Consensus on Guidance and Prevention Strategies for Hospital Pharmacists and the Pharmacy Workforce （hereinafter referred to as “expert consensus ”），and to apply and practice in specific work ，so as to give full play to the role of pharmacists to help fight the epidemic.METHODS ：The background of the formulation and revision of the expert consensus were introduced ，and its main contents and viewpoints were interpreted. RESULTS & CONCLUSIONS ：The text of expert consensus is divided into 8 parts，mainly including disease diagnosis and treatment [SARS-CoV- 2 infection related background ，clinical manifestations and diagnosis， treatment]，hospital pharmacy （prevention and control strategy ，work guidance ），drug and facility support management（key drug/facility/equipment support ，management and use of the drug in special circumstances ），information sources and related resources ，etc.，which comprehensively and detailedly provide information ，guidance and strategies for coronavirus SARS-CoV-2 infection prevention and control to play the role of pharmacists in hospital pharmacy well ，do well in the protection of staff in different pharmaceutical posts ，drug security work in response to epidemic situation ，and develop pharmaceutical care. So far，the understanding of SARS-CoV- 2 in the pharmaceutical industry is relatively limited. Based on the accumulated experience and progress in epidemic prevention and control ，the expert consensus will be updated and improved continuously ，so as to provide guidance and help for hospital pharmaceutical personnel.

Objective To investigate the clinical efficiency,safety and prognostic factors of secondline chemotherapy regimen with gemcitabine combined with rituximab in the treatment of relapsed or refractory B-cell non-Hodgkin lymphoma.Methods A total of 157 patients with relapsed or refractory B-cell nonHodgkin lymphoma were selected from July 2008 to February 2015 in Xi'an Central Hospital.Among them,87 patients were given GEMOX regimen (gemcitabine + oxaliplatin) combined with rituximab,and 70 patients were given GDP program (gemcitabine + cisplatin + dexamethasone) combined with rituximab.The chemotherapy efficacies of the two groups were evaluated.At the same time,the patients were grouped according to whether rituximab was applied or not,and the total objective response rate (ORR) difference was compared.The relevant prognostic factors affecting overall survival (OS) were found.The adverse reactions of patients after treatment were observed.Results The ORR of the GEMOX regimen combined with rituximab group was 65.5％,and the ORR of the GDP regimen combined with rituximab group was 55.7％,but the difference between the two groups was not statistically significant (x2 =1.58,P =0.210).The ORR was 75.2％ in 105 patients who had not used rituximab,and the ORR was 32.7％ in 52 patients who had previously received rituximab.The difference between the two groups was statistically significant (x2 =29.50,P ＜ 0.001).Univariate analysis showed that middle-high risk or high risk of the lymphoma international prognostic index (IPI) score (x2 =69.21,P ＜0.001),lactate dehydrogenase (LDH) increased (x2 =16.90,P ＜0.001),refractory patients (x2 =14.43,P =0.001),large mass (x2 =4.57,P =0.030),and failure to achieve CR or PR after salvage chemotherapy (x2 =50.85,P ＜ 0.001) were risk factors for OS.Cox multivariate analysis showed that middle-high risk or high risk of IPI (HR =2.138,95％ CI:1.301-3.512,P =0.001),refractory patients (HR =3.157,95％CI:1.001-10.644,P =0.014),failure to achieve CR or PR after salvage chemotherapy (HR=3.017,95％CI:2.218-7.366,P＜0.001),LDH increased (HR =2.236,95％ CI:1.797-2.781,P =0.001),large mass (HR =1.792,95％ CI:1.255-2.558,P ＜ 0.001) were independent risk factors affecting OS.Adverse reactions to chemotherapy were neutropenia,thrombocytopenia,nausea and vomiting,liver damage and cardiotoxicity,with no treatment-related death.Conclusion The second-line chemotherapy regimen containing gemcitabine combined with rituximab has a better curative effect on relapsed or refractory B-cell non-Hodgkin lymphoma,and the safety is good.Middle-high risk or high risk of IPI,refractory patients,failure to achieve CR or PR after salvage chemotherapy,elevated LDH and large mass were independent risk factors for OS.In patients with relapsed or refractory disease after rituximab treatment,re-application of rituximab was not effective.

The suitable experimental animal model is important in research of pathogenesis and therapeutic strategies of diabetic foot ulcer, and the murine model is the most commonly used one at present. It can be divided into two types: the animal model simulating pathological conditions and the model simulating clinical symptoms. This article reviews the current research progress on the mechanisms of diabetic ulcer pathogenesis, and relevant treatment strategies, including the inhibition of matrix metalloproteinases (MMPs) expression, promotion of angiogenesis and anti-inflammatory therapy.
Animals ; Anti-Inflammatory Agents ; therapeutic use ; Diabetic Foot ; etiology ; genetics ; therapy ; Disease Models, Animal ; Humans ; Matrix Metalloproteinase Inhibitors ; therapeutic use ; Matrix Metalloproteinases ; genetics ; metabolism ; Mice ; Neovascularization, Physiologic ; physiology