OBJECTIVE To explore the transformation of the dispensing and drug pickup process in traditional Chinese medicine pharmacy （TCM Pharmacy） in our hospital based on data-intelligence-driven， aiming to improve pharmacists’ work efficiency and patients’ drug pickup experience. METHODS Value stream mapping and journey mapping were used to systematically identify non-value-added links in pharmacists’ dispensing process and key pain points in patients’ drug pickup under the traditional process. An intelligent dispensing and drug pickup system for the TCM Pharmacy was developed based on the C# and Android television platforms， and a machine-learning model was adopted to predict patients’ drug pickup waiting time. A comprehensive evaluation was performed from three perspectives： system performance， prediction accuracy， and satisfaction of pharmacists and patients. RESULTS The system successfully streamlined non-value-added links such as “waiting for writing on the board” and “searching for drugs”， and realized multimodal dynamic prompts of dispensing status through auditory （number calling） and visual （television terminal） channels. The constructed model for predicting drug pickup waiting time exhibited good fitting degree and generalization ability （mean absolute error＝4.28 min， R 2 ＝0.882）. The comprehensive satisfaction scores of pharmacists and patients in the traditional mode were significantly increased from （70.99±1.74） and （73.58±1.98） to （90.02±1.30） and （88.61±2.08） in the new system， respectively （ P ＜0.01）. CONCLUSIONS The transformation of the intelligent drug dispensing and pickup system for TCM pharmacy based on data-intelligence-driven effectively improves the efficiency of pharmacists’ dispensing work， realizes process transparency and waiting time predictability， and significantly enhances patients’ drug pickup experience.

ObjectiveTo develop a full-process data platform of renal rehabilitation, diagnosis and quality control information. MethodsA hierarchical architectural design was proposed, adhering to clinical pathway models and standardized data protocols. The platform comprehensively covered assessment, intervention, follow-up and quality control for maintenance hemodialysis (MHD) patients. By integrating multidisciplinary resources and standardizing rehabilitation workflows, it delivered standardized and intelligent rehabilitation services. ResultsThe platform achieved standardized and intelligent management of rehabilitation services, effectively improved the physiological function, psychological state and quality of life convenience for MHD patients, while significantly reduced the economic and care burden on patients' families and society. ConclusionThe rehabilitation service model based on a full-process data platform may provide scientific and systematic support for MHD patients.

OBJECTIVE To establish a scientific， systematic， multi-dimensional performance management system for pharmacy practice， so as to improve the efficiency and quality of pharmacy practice performance management in public hospitals. METHODS Based on the four dimensions of the balanced scorecard theory， finance， customer， internal process， learning and growth， reference indicators for pharmacy practice performance management were summarized. The Delphi method was used to screen indicators， and the analytic hierarchy process was applied to determine the weights of indicators. A pharmacy practice performance management system was then constructed. Based on this system， action plans were formulated and implemented. The effectiveness was evaluated from two aspects： customer reviews and changes in pharmacy practice outcomes. RESULTS A total of 28 reference indicators were summarized， and a performance management system for pharmacy practice was constructed， consisting of 4 primary indicators， 9 secondary indicators， and 20 tertiary indicators. Compared with action plans implementation before， the satisfaction of clinical departments was significantly improved， and 11 pharmacy practice performance management indicators were optimized after implementation. CONCLUSIONS A scientific and systematic performance management system for pharmacy practice has been successfully established， which can provide a reference for the innovation of hospital pharmacy practice management and the high quality development of pharmacy practice.

Psoriasis is an incurable chronic inflammatory disease that requires new interventions. Here, we found that fibroblasts exacerbate psoriasis progression by promoting macrophage recruitment via CCL2 secretion by single-cell multi-omics analysis. The natural small molecule celastrol was screened to interfere with the secretion of CCL2 by fibroblasts and improve the psoriasis-like symptoms in both murine and cynomolgus monkey models. Mechanistically, celastrol directly bound to the low-density lipoprotein receptor-related protein 1 (LRP1) β-chain and abolished its binding to the transcription factor c-Jun in the nucleus, which in turn inhibited CCL2 production by skin fibroblasts, blocked fibroblast-macrophage crosstalk, and ameliorated psoriasis progression. Notably, fibroblast-specific LRP1 knockout mice exhibited a significant reduction in psoriasis like inflammation. Taken together, from clinical samples and combined with various mouse models, we revealed the pathogenesis of psoriasis from the perspective of fibroblast-macrophage crosstalk, and provided a foundation for LRP1 as a novel potential target for psoriasis treatment.

Objective:To report the clinical, electrophysiological, and genetic mutation characteristics of a family with paramyotonia congenita, to enhance clinical understanding of ion channelopathies, provide better guidance for clinical diagnosis, and optimize treatment plans.Methods:The clinical data, nerve conduction studies, needle electromyography, long exercise test, short exercise test, and whole-exome sequencing results of the proband with paramyotonia congenita and his family members admitted to Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, in August 2023 were collected and analyzed.Results:The proband was a 15-year-old male who presented with episodic muscle weakness for 1 year, accompanied by elevated creatine kinase during episodes. Between the ages of 3 and 4, he exhibited facial and bilateral hand myotonia triggered by cold exposure. His mother, sister, maternal grandmother, maternal aunt and uncle also displayed similar cold-induced facial and hand myotonia during childhood. Additionally, his cousin exhibited walking difficulties, frequent falls, mild difficulty in opening eyes after closure, and mild chewing and swallowing difficulties at the age of 1. Electromyography of the proband showed widespread myotonic discharges and myopathic impairment, while the electromyography of his mother and sister revealed only widespread myotonic discharges. The proband′s long exercise test results were consistent with periodic paralysis, while the short exercise test results aligned with paramyotonia congenita. His sister′s long exercise test results were within normal range, while the short exercise test results of his mother and sister were consistent with paramyotonia congenita. Genetic testing of the family revealed that all tested patients carried a heterozygous SCN4A mutation, c.4343G>A (R1448H). Conclusions:The R1448H mutation in the SCN4A gene is the genetic cause of this family. Patients carrying the SCN4A R1448H mutation may exhibit clinical features of cold-induced myotonia with or without episodic muscle weakness. In addition to widespread myotonic discharges on electromyography, certain patients may also show myopathic impairment. The long and short exercise tests can further assist in differentiating various types of myotonia and periodic paralysis for clinical diagnosis.

Objective To explore the clinical application value of gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid(Gd-EOB-DTPA)enhanced MRI T1 mapping in the evaluation of liver function.Methods Sixty-four patients who underwent enhanced MRI T1 mapping with Gd-EOB-DTPA and completed the laboratory examination of liver function within one week were prospectively enrolled.All patients were divided into normal control group(NCG),cirrhosis Child-Pugh A(CCA)group,cirrhosis Child-Pugh B(CCB)group,and cirrhosis Child-Pugh C(CCC)group.CCB+CCC groups were defined as a moderate and severe abnormal liver function group.The T1 mapping images of pre-enhanced,post-enhanced 10 min and 20 min were collected,and the T1 mapping val-ues of liver and spleen were measured.The ΔT1 and hepatocyte enhancement fraction(HEF)were calculated.The differences of parameters in different liver function groups were analyzed and compared,and the diagnostic efficacy of each index in distinguishing different liver function groups was evaluated.Results There were significant differences in T1plain scan,T110 min,T120 min,ΔT110 min,ΔT120 min,HEF10 min and HEF20 min among the three groups(P<0.05).The difference of T1plain scan between NCG and CCA groups,and between NCG and CCB+CCC groups was statistically significant(P<0.05).The area under the curve(AUC)of differentiating normal liver function group from abnormal liver function group was 0.761.There were significant differences in T110 min,T120 min,ΔT110 min,ΔT120 min,HEF10 min and HEF20 min between CCA and CCB+CCC groups.The AUC of differentiating the two groups was 0.757,0.820,0.735,0.820,0.790 and 0.853,respectively,and HEF20 min had the highest diagnostic efficacy.Conclusion Gd-EOB-DTPA enhanced MRI T1 mapping can be used as an effective method to evaluate liver function.

The Occupational Classification Dictionary of the People's Republic of China (2015 Edition) has added a new occupation type, Medical Nursing Assistants, aiming to meet the strong demand for medical care in the context of the aging population in China.In order to standardize the services of medical nursing assistants for the elderly in home and community settings and contribute to healthy aging, the National Health Commission issued the " Service Standards for Medical Nursing Assistants of Older Adults in Home and Community" ( WS/ T 803—2022) on September 28, 2022.The standards regulate the service processes, service items and requirements, as well as service evaluation and improvement for elderly medical nursing assistants.The interpretation of the standard's formulation background, the compilation process, and the standard's content are as follows.

Objective:To explore the clinical feature and genetic variation of NPHS1 variant-associated nephropathy ( NPHS1-VAN) in Chinese patients. Methods:This study was a case-series analysis. Patients with NPHS1-VAN, who were treated and/or followed in the Department of Pediatrics, Nanfang Hospital, Southern Medical University between 2018 and 2023 were recruited into this study. Genotype, phenotype and their relationship were analyzed. Results:Nine NPHS1-VAN patients from 8 non-consanguineous Chinese families were collected, including 5 males and 4 females. There were 7 cases with an onset age within 3 months and 2 cases with an onset age of 6 months and 13 years, respectively. Seven patients harbored compound heterozygous variants, two had homozygous variants, including 8 missense variations,3 frameshift variants, and 1 splicing site variant. Four patients in 3 families harbored missense variant c.928G>A, two of them experienced spontaneous remission of proteinuria at the age of 1 year and 2 years, respectively, another one had persistent proteinuria and entered end stage renal disease (ESRD) at 11 years old. The other one had an onset age of 6 months with no response to steroids initially. She got complete remission by tacrolimus administered, but relapse frequently and partially responded to steroids later. Two patients of this group died, one of them died of respiratory failure 3 days after birth. Excessive amniotic fluid and fetal edema were acknowledged at 28 weeks of gestational age. He harbored compound heterozygous variants of NPHS1, c.1135C>G (R379G) and c.1339G>A (E447K). His mother previously experienced fetal death at 28 weeks gestational age for her first pregnant and stillborn at 36 weeks of gestational age for her second pregnant, respectively. One patient in this study who harbored homozygous variant of c.1339G>A (E447K) presented with a mild phenotype, onset age was 13 years old and didn't progress to ESRD yet at 21 years. Thus, variant E447K was hypothesized to be weakly pathogenic, while R379G may be strongly pathogenic with a risk of death. Five novel variants were identified in this group of patients, 3 missense variants (c.1135C>G, c.1157A>T, c.3197T>A) and 2 frameshift variants (c.709_710delCT, c.3193delG). Renal biopsy was performed in 4 cases, of whom two were focal segmental glomerular sclerosis and another two were minimal change disease. Conclusions:NPHS1-VAN possesses remarkable clinical and genetic heterogeneity. Five novel variants were identified. Missense variant is the most common variant type and c.928G>A is the most common one in this group of patients, in consistent with previous report in China. Children harbor c.928G>A may have a mild phenotype with possible spontaneous remission and may be response to steroids and calcineurin inhibitor. Variant c.1135C>G (R379G) may have a strong pathogenicity, and patient who harbors this variant may have a severe phenotype.

Objective:To investigate the clinical characteristics of urea cycle disorder (UCD), the efficacy and safety of glyceryl phenylbutyrate (GPB) therapy in pediatric patients with UCD.Methods:This study was a retrospective, single-arm, multicenter clinical study. The clinical data of 20 pediatric patients with UCD who received GPB treatment at 9 hospitals nationwide between December 2021 and August 2024 were collected. The clinical manifestations, laboratory results, and molecular genetic characteristics were analyzed, ammonia levels and other laboratory results were evaluated pre-post GPB therapy by paired t-tests or Wilcoxon tests. Results:Among the 20 pediatric patients with UCD, there were 8 males and 12 females, and the onset age was 2.8 (1.4, 5.7) years. The ammonia levels were 174 (125, 342) μmol/L at first onset. The symptoms included vomiting in 6 cases, drowsiness in 5 cases, epilepsy in 5 cases, developmental delay in 5 cases, psychiatric and behavioral abnormalities in 3 cases, and lethargy in 1 case, and 18 cases exhibited abnormal liver function. Twenty cases included 6 UCD subtypes, with 11 cases being ornithine transcarbamylase deficiency. A total of 27 variants were identified, 11 (41%) of which were novel. The age of patients who began GPB therapy was 4.0 (1.5, 6.6) years. Ten cases stopped GPB after 4.2 (3.4, 5.3) months, with 4 patients undergoing liver transplantation and 6 discontinuing for financial reasons. The remaining ten patients continued GPB therapy for 11.6 (8.6, 14.0) months. The duration of GPB treatment was 6.0 (4.2, 12.3) months, at the final visit, the levels of ammonia, platelets and aspartate aminotransferase were lower compared to those of pre-treatment (all P<0.05). The serum albumin level was higher than that of pre-treatment ( P=0.016). Two patients suffered only one episode of acute hyperammonaemia, with ammonia levels of 232 and 141 μmol/L, respectively. Nine cases experienced adverse effects potentially related to GPB, decreased appetite in 6 cases, vomiting in 3 cases, abnormal skin oil odor in 2 cases, somnolence, fatigue and diarrhea each in 1 case, with symptoms improved within 6 (3, 10) days. Conclusions:UCD primarily manifests with neurological and gastrointestinal symptoms, and early diagnosis of UCD could be achieved through the analysis of ammonia. GPB may effectively reduce ammonia levels in UCD pediatric patients, with favorable safety and tolerability.

Artificial intelligence(AI)has emerged as a cutting-edge technology leading the future and is a key engine for China's development.In the innovation and research of medical devices,AI has provided critical support in the areas of intelligent diagnostic assistance,intelligent therapeutic assis-tance,intelligent monitoring,life support,et al.Ma-chine learning-enabled device software functions(ML-DSFs)have become an essential component of many medical devices.Recently,the United States Food and Drug Administration(FDA)released a draft guidance titled"Marketing Submission Rec-ommendations for a Predetermined Change Con-trol Plan for Artificial Intelligence/Machine Learn-ing(AI/ML)-Enabled Device Software Functions(Draft)."that aimed to provide a forward-looking approach to foster the development of ML medical devices.By supporting iterative updates through modifications,this approach ensures the continu-ous safety and effectiveness of the devices.This guidance represents the latest in regulatory direc-tion and is especially beneficial for enhancing the quality and efficiency of clinical trials for AI prod-ucts.Therefore,we plan to provide a detailed intro-duction and interpretation of the guidance,with the aim of learning from international advanced regulatory concepts and experiences to promote the development of ML-DSFs with more profound international influence.