Objective:To explore the genetic basis and clinical phenotype of a Chinese pedigree affected with Focal segmental glomerulosclerosis (FSGS).Methods:A male patient who was admitted to the First Affiliated Hospital of Zhengzhou University on July 26, 2018 was selected as the study subject. Clinical data of the patient was collected. Next generation sequencing and Sanger sequencing were carried out to detect the variant sites. Bioinformatic software was used to simulate the effect of candidate variants on the protein functions.Results:Ultrasound exam of the patient showed enhanced echo for the renal parenchyma. Kidney biopsy had confirmed the pathological diagnosis of FSGS (non-specific). Electronic microscopy displayed segmental sclerosis of the glomeruli, mild hyperplasia of mesangial cells and matrix. The proband was found to harbor two novel variants of the PLCE1 gene, namely c. 3199delA (p.N1067Mfs*15) and c.4441_4443delATC (p.1481_1481del), which were respectively inherited from his mother and father. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), both variants were rated as pathogenic (PVS1+ PM2_Supporting+ PP3; PM2_Supporting+ PM3+ PP3). Bioinformatic simulation suggested that both variants could significantly affect the tertiary structure of the PLCE1 protein. Conclusion:The c. 4441_4443delATC and c. 3199delA variants of the PLCE1 gene probably underlay the pathogenesis of the FSGS in this pedigree.

Temporomandibular joint (TMJ) diseases are common clinical conditions. The number of patients with TMJ diseases is large, and the etiology, epidemiology, disease spectrum, and treatment of the disease remain controversial and unknown. To understand and master the current situation of the occurrence, development and prevention of TMJ diseases, as well as to identify the patterns in etiology, incidence, drug sensitivity, and prognosis is crucial for alleviating patients′suffering.This will facilitate in-depth medical research, effective disease prevention measures, and the formulation of corresponding health policies. Cohort construction and research has an irreplaceable role in precise disease prevention and significant improvement in diagnosis and treatment levels. Large-scale cohort studies are needed to explore the relationship between potential risk factors and outcomes of TMJ diseases, and to observe disease prognoses through long-term follw-ups. The consensus aims to establish a standard conceptual frame work for a cohort study on patients with TMJ disease while providing ideas for cohort data standards to this condition. TMJ disease cohort data consists of both common data standards applicable to all specific disease cohorts as well as disease-specific data standards. Common data were available for each specific disease cohort. By integrating different cohort research resources, standard problems or study variables can be unified. Long-term follow-up can be performed using consistent definitions and criteria across different projects for better core data collection. It is hoped that this consensus will be facilitate the development cohort studies of TMJ diseases.

Objective:To analyze the clinical features and follow-up data in children with neuropsychiatric lupus erythematosus (NPSLE), and provide reference for the diagnosis and treatment of NPSLE.Methods:The clinical data of 22 children with NPSLE who were admitted to the Department of Rheumatology Immunology and Allergy, Children′s Hospital of Zhejiang University School of Medicine from January 2019 to March 2022 were included and were followed-up for 24~60 months. The data were analyzed retrospectively. Statistical descriptive analysis was performed using the SPSS 23.0.Results:Twenty-two (26.8%, 22/82) children with NPSLE occurred in the hospitalized children with SLE during the study period. The ratio of male to female was 1∶4.5, and the onset age was (10.7±2.0) years. Among these cases, 86.4% (19/22) patients were newly diagnosed and had severe disease activity and 16 cases (72.7%) occurred within 1 month after disease onset. Nineteen cases (86.4%) had mucocutaneous involvement, 16 cases (72.7%) had lupus nephritis, 14 cases (63.2%) had hematological system involvement, 13 cases (59.1%) had skeletal muscle involvement, 10 cases (45.5%) had serositis, 10 cases (45.5%) were complicated with hypertension, and 8 cases (36.5%) with macrophage activation syndrome (MAS). The main clinical symptoms of the nervous system included headache (11/19, 57.9%), dizziness (10/19, 52.6%), listlessness (7/19, 36.8%), blurred vision (4/19, 21.1%), convulsions (3/19, 15.8%), lethargy (2/19, 10.5%). Twenty patients (20/22, 90.9%) demonstrated abnormal signals on brain MRI, 7 cases (7/12, 45.5%) showed abnormal signals on brain CT, 10 cases (10/22, 45.5%) showed abnormal waves on EEG, and 6 cases (6/20, 30.0%) demonstrated abnormal results of cerebrospinal fluids analysis. The follow-up duration was 34 (28, 48) months. Clinical remission or low disease activity was found in 19 patients (86.4%), and no death were observed. Three cases had residual cerebral infarction lesions, no neurological sequelae were found in all patients.Conclusion:The most common symptoms of NPSLE in children are headache and dizziness, which are more likely to occur in patients with initial onset and severe disease activity, and approximately 36.5% children with NPSLE may complicated by MAS.The results of 24-60 months follow-up showed that the prognosis of the disease is good.

Objective:To establish a method for the detection of bacterial endotoxin in terbutaline sulfate active pharmaceutical ingredients(API).Methods:The method of terbutaline sulfate was verified by gel method,and interference test and bacterial endotoxin test were performed on three batches of samples.Results:The limit for terbutaline sulfate API that can be used for bacterial endotoxin test was set as"the amount of endotoxin contained in terbutaline sulfate should be less than 0.50 EU per 1 mg".Conclusion:Limulus gel method can be used to detect bacterial endotoxin of terbutaline sulfate API.

Purpose: The risk stratification of pediatric anaplastic large cell lymphoma (ALCL) has not been standardized. In this study, new risk factors were included to establish a new risk stratification system for ALCL, and its feasibility in clinical practice was explored. Materials and Methods: On the basis of the non-Hodgkin’s lymphoma Berlin–Frankfurt–Munster 95 (NHL-BFM-95) protocol, patients with minimal disseminated disease (MDD), high-risk tumor site (multiple bone, skin, liver, and lung involvement), and small cell/lymphohistiocytic (SC/LH) pathological subtype were enrolled in risk stratification. Patients were treated with a modified NHL-BFM-95 protocol combined with an anaplastic lymphoma kinase inhibitor or vinblastine (VBL). Results: A total of 136 patients were enrolled in this study. The median age was 8.8 years. The 3-year event-free survival (EFS) and overall survival of the entire cohort were 77.7% (95% confidence interval [CI], 69.0% to 83.9%) and 92.3% (95% CI, 86.1% to 95.8%), respectively. The 3-year EFS rates of low-risk group (R1), intermediate-risk group (R2), and high-risk group (R3) patients were 100%, 89.5% (95% CI, 76.5% to 95.5%), and 67.9% (95% CI, 55.4% to 77.6%), respectively. The prognosis of patients with MDD (+), stage IV cancer, SC/LH lymphoma, and high-risk sites was poor, and the 3-year EFS rates were 45.3% (95% CI, 68.6% to 19.0%), 65.7% (95% CI, 47.6% to 78.9%), 55.7% (95% CI, 26.2% to 77.5%), and 70.7% (95% CI, 48.6% to 84.6%), respectively. At the end of follow-up, one of the five patients who received maintenance therapy with VBL relapsed, and seven patients receiving anaplastic lymphoma kinase inhibitor maintenance therapy did not experience relapse. Conclusion This study has confirmed the poor prognostic of MDD (+), high-risk site and SC/LH, but patients with SC/LH lymphoma and MDD (+) at diagnosis still need to receive better treatment (ClinicalTrials.gov number, NCT03971305).

ObjectiveTo reveal the effects of different microbial agents on quality of Lycii Fructus by comparing the differences in the contents of multiple types of chemical components in Lycii Fructus after the application of different microbial agents. MethodTaking Ningqi No. 7 as experimental material， four microbial agents， namely Peiyuan combined with Xinterui（TP group）， Trichoderma harzianum combined with Bacillus subtilis（BW group）， Genwuyou（MT group） and Junyiduo（JYD group）， were applied， and no microbial agents was used as the blank group（CK group）. Then the contents of total phenolics， total flavonoids， saccharides， amino acids， nucleosides and bases， betaine and other components in Lycii Fructus were determined by ultraviolet spectrophotometry（UV）， high performance liquid chromatography（HPLC） and ultra-high performance liquid chromatography-mass spectrometry（UHPLC-MS/MS）， and the methods such as multiple comparisons， principal component analysis（PCA） and partial least squares-discriminant analysis（PLS-DA） were used to analyze the effect of different microbial agents on the quality of Lycii Fructus. ResultMicrobial agents had different effects on chemical components of Lycii Fructus. The content of total phenolics was the highest in the TP group， and it varied significantly from the CK group（P<0.05）. The total flavonoid content was the highest in the BW group， followed by the TP group. Both polysaccharide and alduronic acid contents were the highest in the JYD group. Betaine content in the TP and BW groups were significantly higher than that in the CK group（P<0.05）. For the determined 23 kinds of amino acids， most of them were the lowest in the JYD group， and the highest in the MT group， while the nucleoside bases were higher in the MT and BW groups. It indicated that Lycii Fructus from different treatment groups could be distinguished clearly based on the determined 45 chemical components. The result of PLS-DA showed that the major differential components in each group were polysaccharides， glucose， fructose， betaine， alduronic acid， asparagine， sucrose， threonine， total flavonoids， alanine and total phenolics. The results of PCA composite scores based on the main differential components showed that composite scores of chemical components in each group were BW group>TP group>MT group>CK group>JYD group. ConclusionThe application of microbial agents of BW， TP and MT can promote the quality improvement of Lycii Fructus， and the application of JYD can promote the accumulation of polysaccharides and alduronic acid to a certain extent， but the overall effect on the quality of Lycii Fructus is not clear. This study lays the foundation for the green and healthy development of Lycii Fructus industry.

OBJECTIVE To interpret Teacher Training Syllabus for Clinical Pharmacist Training Program （2023 edition） （hereinafter referred as to the “new syllabus”）， and to provide reference and guidance for promoting the implementation of the new syllabus and realizing the quality-improving goal of the reform of the clinical pharmacist teacher training program initiated by China Hospital Association. METHODS From the perspective of the management and based on the position of the designer， the new syllabus was interpreted from four aspects： the background of its compilation and release， the process of its compilation and its characteristics， the key improvements of the program and the points for attention about its subsequent implementation. RESULTS & CONCLUSIONS The development and release of the new syllabus provide a “construction blueprint” for the reform of the clinical pharmacist teacher training program of the China Hospital Association. The whole process of compiling the new syllabus is characterized by four basic features： theory-led， goal-oriented， research-based， and synergistic. Compared with the previous syllabus， in addition to the adjustment of the text structure，the new syllabus presents more complete and clearer competence requirements for clinical teaching competence in terms of training objectives； in terms of training content， it further structures the group of task items， pays attention to the 育。E-mail：zhenjiancun@163.com sequential planning and time arrangement of items， and puts forward both quantitative and qualitative refinement requirements for each specific training task；in terms of training methods， it emphasizes the interaction of lecturing， demonstrating and guiding， and the progression of observation， operation and reflection， with the intention of guiding teacher trainees to “learn how to teach by teaching”. In the subsequent implementation of the new syllabus， it is necessary for the teacher training bases to attach great importance to the guarantee of training conditions and process quality management， and to organize the teacher training team to do a good job in the two training programs of “clinical pharmacist training” and “clinical pharmacist teacher training”. Based on further improving the connection between the two training programs， the teacher training team should continue to explore the scientific model of clinical pharmacist teacher training oriented by clinical teaching competence.

Acute respiratory infection caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) had caused a global pandemic since 2019, and posed a serious threat to global health security. Traditional Chinese medicine (TCM) has played an indispensable role in the battle against the epidemic. Many components originated from TCMs were found to inhibit the production of SARS-CoV-2 3C-like protease (3CLpro) and papain-like protease (PLpro), which are two promising therapeutic targets to inhibit SARS-CoV-2. This study describes a systematic investigation of the roots and rhizomes of Sophora tonkinensis, which results in the characterization of 12 new flavonoids, including seven prenylated flavanones (1-7), one prenylated flavonol (8), two prenylated chalcones (9-10), one isoflavanone (11), and one isoflavan dimer (12), together with 43 known compounds (13-55). Their structures including the absolute configurations were elucidated by comprehensive analysis of MS, 1D and 2D NMR data, and time-dependent density functional theory electronic circular dichroism (TDDFT ECD) calculations. Compounds 12 and 51 exhibited inhibitory effects against SARS-CoV-2 3CLpro with IC₅₀ values of 34.89 and 19.88 μmol·L^-1, repectively while compounds 9, 43 and 47 exhibited inhibitory effects against PLpro with IC₅₀ values of 32.67, 79.38, and 16.74 μmol·L^-1, respectively.
Flavonoids/chemistry* ; SARS-CoV-2 ; Rhizome ; COVID-19 ; Peptide Hydrolases ; Antiviral Agents/chemistry*

[This corrects the article DOI: 10.1016/j.apsb.2021.07.006.].

Safety is the core of the quality of Chinese materia medica products， and microbial pollution caused by medicinal materials， decoction pieces， intermediate products and others can bring certain impact on the quality and safety of Chinese materia medica products. The reasons for this are not only the problems of medicinal materials themselves， but also the exogenous pollution introduced in the production process. How to effectively use microbial detection technology and establish an appropriate microbial quality control strategy in the whole process of Chinese materia medica production is of great significance to improve the quality of Chinese materia medica products. Therefore， the authors put forward a microbial quality control strategy in the whole process of Chinese materia medica production based on the guidance of quality by design （QbD） concept， emphasizing the scientific linkage between the internal and external microbial quality control systems to jointly ensure the quality of products in all aspects. Among them， the internal microbial quality control system includes the control of the whole chain of Chinese materia medica-decoction pieces-intermediate products-excipitents-packaging materials-final products， which should be carried out by stages and characteristics， while the external microbial quality control system includes the control of personnel-equipment and facilities-pharmaceutical water-environment， emphasizing the principle of quality risk management and the development of monitoring programs， aiming to closely integrate microbial quality risk management with the production process of Chinese materia medica products， and to classify and develop microbial control strategies in order to minimize the impact of contaminating microorganisms and effectively guarantee the quality of Chinese materia medica products.