Objective:To establish and assess the quality control and improvement system for metabolic and bariatric surgery in Beijing.Methods:Based on relevant documents from the National Health Commission and the Beijing Municipal Health Commission,and referencing the Metabolic and Bariatric Surgery Accreditation and Quality Improvement Program (MBSAQIP) by the American Society for Metabolic and Bariatric Surgery,a quality control system was developed under the Beijing Quality Control and Improvement Center of Metabolic and Bariatric Surgery. The system incorporated on-site evaluations,data registration,and specialized training. From May to December 2023,on-site assessments were conducted at 21 hospitals in Beijing performing bariatric surgery,evaluating personnel qualifications,infrastructure,clinical workflows,and postoperative follow-up. A quality control database was created to collect real-time surgical data,and training was provided for data entry and professional skills. Assessment results were classified as excellent,qualified,or needing improvement,with rectification suggestions offered and follow-up visits conducted to track progress.Results:All 21 hospitals achieved a 100% compliance rate for surgical indications, 16 (76.2%) met standardized surgical operation criteria,and 14 (66.7%) had standardized postoperative management. However,only 5 (23.8%) achieved a 12-month postoperative follow-up rate of ≥60%,and 4 (19.1%) had established specialized databases. Key challenges included insufficient specialized staffing (19.1%), lack of multidisciplinary collaboration (47.6%), inadequate equipment (57.1%), and low follow-up rates (57.1%). The database collected data from over 2 000 patients across 111 fields. After rectification, specialized database coverage rose to 61.9% (13 hospitals). Multi-level training programs developed backbone physicians and specialized nurses,significantly addressing the shortage of specialized personnel.Conclusion:The quality control system established in this study,through the integration of on-site evaluation,data registration,and specialized training,effectively enhances the standardization of surgical practices and data management capabilities.

Objective:To analyze the serological and molecular characteristics of rare A el and B el subtypes in the ABO blood group system, and to explore their genotype-phenotype correlation and the potential clinical significance. Methods:From January 1st, 2021, to January 1st, 2025, 289, 815 samples subjected to ABO blood grouping in Henan Provincial People′s Hospital were selected. Samples demonstrating discrepancies between forward and reverse typing, or consistent typing but with abnormal agglutination degree were included. Those affected by underlying diseases, transplantation, age-related and other interferences were excluded. A total of 169 suspected ABO subgroup samples were identified. Sanger sequencing of exons 1-7 and relevant regulatory regions of the ABO gene was performed. Protein structure modeling and mutation effect analysis for two'el′ subtype glycosyltransferases (GTs) were conducted using SWISS-MODEL and PyMOL.Results:A total of 12 Ael, 6 B el, and 2 AB el subtypes were identified. Serological analysis revealed that all 18 A el/B el samples exhibited O phenotype in forward typing. Among them, A el subtypes showed weaker agglutination in reverse typing with A 1c than with Bc (>2+), while the opposite pattern was observed in B el subtypes. The two AB el samples were typed as A in forward typing, with agglutination ranging from 0-1+with Bc in reverse typing. Genetic analysis indicated that AEL.02 (c.646T>A, p.Phe216Ile) was the predominant allele in A el samples accounting for 7 cases. Also, we found an AEL.02-like variant (lacking c.681G>A), AEL.10 (c.963insC), and carrying a compound variant of c.322C>T (p.Gln108Ter) and c.296C>T (p.Thr99Ile). Among B el samples, BEL.03 (c.502C>T, p.Arg168Trp) accounted for 4 cases, one of which lacked the c.297A>G mutation, and novel mutations such as c.145_146dupCG were detected. Structural simulation demonstrated that AEL.02 and BEL.03 disrupted the hydrogen-bonding network within the active centers of GTA and GTB, respectively, and these mutations probably significantly impaired the structural stability of the corresponding GTs. Additionally, the c.296C>T mutation also markedly affected GTA structural stability. Conclusion:A el/B el subtypes are prone to mis-identify routine blood types. Their molecular mechanisms involved a variety of functional variantions, and integrating molecular detection is crucial for achieving accurate sub-typing and transfusion safety.

Objective:To analyze the serological and molecular characteristics of rare A el and B el subtypes in the ABO blood group system, and to explore their genotype-phenotype correlation and the potential clinical significance. Methods:From January 1st, 2021, to January 1st, 2025, 289, 815 samples subjected to ABO blood grouping in Henan Provincial People′s Hospital were selected. Samples demonstrating discrepancies between forward and reverse typing, or consistent typing but with abnormal agglutination degree were included. Those affected by underlying diseases, transplantation, age-related and other interferences were excluded. A total of 169 suspected ABO subgroup samples were identified. Sanger sequencing of exons 1-7 and relevant regulatory regions of the ABO gene was performed. Protein structure modeling and mutation effect analysis for two'el′ subtype glycosyltransferases (GTs) were conducted using SWISS-MODEL and PyMOL.Results:A total of 12 Ael, 6 B el, and 2 AB el subtypes were identified. Serological analysis revealed that all 18 A el/B el samples exhibited O phenotype in forward typing. Among them, A el subtypes showed weaker agglutination in reverse typing with A 1c than with Bc (>2+), while the opposite pattern was observed in B el subtypes. The two AB el samples were typed as A in forward typing, with agglutination ranging from 0-1+with Bc in reverse typing. Genetic analysis indicated that AEL.02 (c.646T>A, p.Phe216Ile) was the predominant allele in A el samples accounting for 7 cases. Also, we found an AEL.02-like variant (lacking c.681G>A), AEL.10 (c.963insC), and carrying a compound variant of c.322C>T (p.Gln108Ter) and c.296C>T (p.Thr99Ile). Among B el samples, BEL.03 (c.502C>T, p.Arg168Trp) accounted for 4 cases, one of which lacked the c.297A>G mutation, and novel mutations such as c.145_146dupCG were detected. Structural simulation demonstrated that AEL.02 and BEL.03 disrupted the hydrogen-bonding network within the active centers of GTA and GTB, respectively, and these mutations probably significantly impaired the structural stability of the corresponding GTs. Additionally, the c.296C>T mutation also markedly affected GTA structural stability. Conclusion:A el/B el subtypes are prone to mis-identify routine blood types. Their molecular mechanisms involved a variety of functional variantions, and integrating molecular detection is crucial for achieving accurate sub-typing and transfusion safety.

Objective:To explore the effectiveness and safety of different anticoagulation strategies during continuous blood purification (CBP) treatment,providing a reference for anticoagulation strategies in critically ill children undergoing CBP.Methods:A retrospective study was conducted,including children admitted to the PICU of Children's Hospital of Fudan University from January 2019 to December 2024.According to the anticoagulation methods used during CBP treatment,patients were divided into the sodium citrate group and the heparin group.CBP was performed using continuous venovenous hemofiltration or continuous venovenous hemodialysis filtration mode,with a blood flow rate of 3-5 mL/(kg·min),replacement fluid rate of 30-50 mL/(kg·h),and dialysis fluid rate of 20-30 mL/(kg·h).The filter lifespan,28-day all-cause mortality,total length of hospital stay,PICU stay duration,adverse events,and associated costs were compared between the two groups.Results:A total of 221 children were included (105 in the sodium citrate group and 116 in the heparin group),with a cumulative use of 666 filters (284 in the sodium citrate group and 382 in the heparin group).(1) There were no statistically significant differences in general data,including age,sex ratio,underlying diseases,the ratio and duration of invasive mechanical ventilation,vasopressor scores at baseline,and indications for CBP between the two groups (all P>0.05).(2) The filter lifespan was 20(14,32) hours for the sodium citrate group and 21(13,35) hours for the heparin group,with no statistically significant difference between the two groups ( P>0.05); the proportion of accidental downstroke was 2.8% and 6.5%,respectively,with a statistically significant difference ( P=0.029); among the 221 children,86 died,with 38 deaths (35.2%) in the sodium citrate group and 49 deaths (38.9%) in the heparin group,showing no statistically significant difference.(3) The sodium citrate group had a higher incidence of metabolic alkalosis,hypocalcemia,and sodium citrate accumulation (44.4% vs. 1.6%,32.7% vs 9.4%,7.7% vs 0,all P<0.01); the heparin group had a greater proportion of bleeding (6.0% vs. 2.9%) and was more likely to develop heparin-induced thrombocytopenia (10.2% vs. 0, P<0.01).(4) The total hospitalization costs for the sodium citrate group were significantly higher than for the heparin group (200 327 yuan vs. 152 077 yuan, P=0.05); costs related to the use of anticoagulants and monitoring indicators during CBP treatment were also higher in the sodium citrate group (2 479 yuan vs. 682 yuan, P<0.01). Conclusions:Sodium citrate is a safe and effective anticoagulation method for critically ill children undergoing CBP,which can reduce the risk of filter clotting compared to systemic heparin anticoagulation.

Objective:To explore the effectiveness and safety of different anticoagulation strategies during continuous blood purification (CBP) treatment,providing a reference for anticoagulation strategies in critically ill children undergoing CBP.Methods:A retrospective study was conducted,including children admitted to the PICU of Children's Hospital of Fudan University from January 2019 to December 2024.According to the anticoagulation methods used during CBP treatment,patients were divided into the sodium citrate group and the heparin group.CBP was performed using continuous venovenous hemofiltration or continuous venovenous hemodialysis filtration mode,with a blood flow rate of 3-5 mL/(kg·min),replacement fluid rate of 30-50 mL/(kg·h),and dialysis fluid rate of 20-30 mL/(kg·h).The filter lifespan,28-day all-cause mortality,total length of hospital stay,PICU stay duration,adverse events,and associated costs were compared between the two groups.Results:A total of 221 children were included (105 in the sodium citrate group and 116 in the heparin group),with a cumulative use of 666 filters (284 in the sodium citrate group and 382 in the heparin group).(1) There were no statistically significant differences in general data,including age,sex ratio,underlying diseases,the ratio and duration of invasive mechanical ventilation,vasopressor scores at baseline,and indications for CBP between the two groups (all P>0.05).(2) The filter lifespan was 20(14,32) hours for the sodium citrate group and 21(13,35) hours for the heparin group,with no statistically significant difference between the two groups ( P>0.05); the proportion of accidental downstroke was 2.8% and 6.5%,respectively,with a statistically significant difference ( P=0.029); among the 221 children,86 died,with 38 deaths (35.2%) in the sodium citrate group and 49 deaths (38.9%) in the heparin group,showing no statistically significant difference.(3) The sodium citrate group had a higher incidence of metabolic alkalosis,hypocalcemia,and sodium citrate accumulation (44.4% vs. 1.6%,32.7% vs 9.4%,7.7% vs 0,all P<0.01); the heparin group had a greater proportion of bleeding (6.0% vs. 2.9%) and was more likely to develop heparin-induced thrombocytopenia (10.2% vs. 0, P<0.01).(4) The total hospitalization costs for the sodium citrate group were significantly higher than for the heparin group (200 327 yuan vs. 152 077 yuan, P=0.05); costs related to the use of anticoagulants and monitoring indicators during CBP treatment were also higher in the sodium citrate group (2 479 yuan vs. 682 yuan, P<0.01). Conclusions:Sodium citrate is a safe and effective anticoagulation method for critically ill children undergoing CBP,which can reduce the risk of filter clotting compared to systemic heparin anticoagulation.

The clinical data of a child with neurodevelopmental disorders caused by a new mutation in the POLR2A gene were retrospectively analyzed.The patient was a 4-year and 2-month-old girl who presented at Xiamen Hospital, Children′s Hospital of Fudan University in June 2024 with clinical manifestations of early infantile hypotonia, muscle atrophy of both lower limbs, global developmental delay (including delays in motor and language development, mental retardation, etc.), sleep difficulties, feeding difficulties, autistic behaviors (namely, social withdrawal), epilepsy, and auditory deficits.The child had one seizure at the age of 2 years and 8 months and one at the age of 4 years, but seizures are currently controlled by drugs.Cranial magnetic resonance imaging and CT showed agenesis of the corpus callosum, bilateral ventricular widening and cerebellar hypoplasia.Whole exome sequencing showed a new mutation c. 3364C>T (p.P1122S) in the POLR2A gene (NM_000937) in the child, and no related gene variants were found in either parental lineage.According to the American College of Medical Genetics and Genomics rating guidelines, it was determined to be a suspected pathogenic variant (PS2+ PM2+ PP3+ PP4).The mutation site has not been reported at home and abroad.The c. 3364C > T ( p. P1122S ) mutation of the POLR2A gene can cause neurodevelopmental disorders, severe phenotypes and poor long-term prognosis.

Objective:To establish and assess the quality control and improvement system for metabolic and bariatric surgery in Beijing.Methods:Based on relevant documents from the National Health Commission and the Beijing Municipal Health Commission,and referencing the Metabolic and Bariatric Surgery Accreditation and Quality Improvement Program (MBSAQIP) by the American Society for Metabolic and Bariatric Surgery,a quality control system was developed under the Beijing Quality Control and Improvement Center of Metabolic and Bariatric Surgery. The system incorporated on-site evaluations,data registration,and specialized training. From May to December 2023,on-site assessments were conducted at 21 hospitals in Beijing performing bariatric surgery,evaluating personnel qualifications,infrastructure,clinical workflows,and postoperative follow-up. A quality control database was created to collect real-time surgical data,and training was provided for data entry and professional skills. Assessment results were classified as excellent,qualified,or needing improvement,with rectification suggestions offered and follow-up visits conducted to track progress.Results:All 21 hospitals achieved a 100% compliance rate for surgical indications, 16 (76.2%) met standardized surgical operation criteria,and 14 (66.7%) had standardized postoperative management. However,only 5 (23.8%) achieved a 12-month postoperative follow-up rate of ≥60%,and 4 (19.1%) had established specialized databases. Key challenges included insufficient specialized staffing (19.1%), lack of multidisciplinary collaboration (47.6%), inadequate equipment (57.1%), and low follow-up rates (57.1%). The database collected data from over 2 000 patients across 111 fields. After rectification, specialized database coverage rose to 61.9% (13 hospitals). Multi-level training programs developed backbone physicians and specialized nurses,significantly addressing the shortage of specialized personnel.Conclusion:The quality control system established in this study,through the integration of on-site evaluation,data registration,and specialized training,effectively enhances the standardization of surgical practices and data management capabilities.

The clinical data of a child with neurodevelopmental disorders caused by a new mutation in the POLR2A gene were retrospectively analyzed.The patient was a 4-year and 2-month-old girl who presented at Xiamen Hospital, Children′s Hospital of Fudan University in June 2024 with clinical manifestations of early infantile hypotonia, muscle atrophy of both lower limbs, global developmental delay (including delays in motor and language development, mental retardation, etc.), sleep difficulties, feeding difficulties, autistic behaviors (namely, social withdrawal), epilepsy, and auditory deficits.The child had one seizure at the age of 2 years and 8 months and one at the age of 4 years, but seizures are currently controlled by drugs.Cranial magnetic resonance imaging and CT showed agenesis of the corpus callosum, bilateral ventricular widening and cerebellar hypoplasia.Whole exome sequencing showed a new mutation c. 3364C>T (p.P1122S) in the POLR2A gene (NM_000937) in the child, and no related gene variants were found in either parental lineage.According to the American College of Medical Genetics and Genomics rating guidelines, it was determined to be a suspected pathogenic variant (PS2+ PM2+ PP3+ PP4).The mutation site has not been reported at home and abroad.The c. 3364C > T ( p. P1122S ) mutation of the POLR2A gene can cause neurodevelopmental disorders, severe phenotypes and poor long-term prognosis.

Objective:To characterize the histopathological subtypes and their clinicopathological parameters of gender and onset age by common, rare and sparse primary esophageal malignant tumors (PEMT).Methods:A total of 272 437 patients with PEMT were enrolled in this study, and all of the patients were received radical surgery. The clinicopathological information of the patients was obtained from the database established by the State Key Laboratory of Esophageal Cancer Prevention & Treatment from September 1973 to December 2020, which included the clinical treatment, pathological diagnosis and follow-up information of esophagus and gastric cardia cancers. All patients were diagnosed and classified by the criteria of esophageal tumor histopathological diagnosis and classification (2019) of the World Health Organization (WHO). The esophageal tumors, which were not included in the WHO classification, were analyzed separately according to the postoperative pathological diagnosis. The χ 2 test was performed by the SPSS 25.0 software on count data, and the test standard α=0.05. Results:A total of 32 histopathological types were identified in the enrolled PEMT patients, of which 10 subtypes were not included in the WHO classification. According to the frequency, PEMT were divided into common (esophageal squamous cell carcinoma, ESCC, accounting for 97.1%), rare (esophageal adenocarcinoma, EAC, accounting for 2.3%) and sparse (mainly esophageal small cell carcinoma, malignant melanoma, etc., accounting for 0.6%). All the common, rare, and sparse types occurred predominantly in male patients, and the gender difference of rare type was most significant (EAC, male∶ female, 2.67∶1), followed with common type (ESCC, male∶ female, 1.78∶1) and sparse type (male∶ female, 1.71∶1). The common type (ESCC) mainly occurred in the middle thoracic segment (65.2%), while the rare type (EAC) mainly occurred in the lower thoracic segment (56.8%). Among the sparse type, malignant melanoma and malignant fibrous histiocytoma were both predominantly located in the lower thoracic segment (51.7%, 66.7%), and the others were mainly in the middle thoracic segment.Conclusion:ESCC is the most common type among the 32 histopathological types of PEMT, followed by EAC as the rare type, and esophageal small cell carcinoma and malignant melanoma as the major sparse type, and all of which are mainly occur in male patients. The common type of ESCC mainly occur in the middle thoracic segment, while the rare type of EAC mainly in the lower thoracic segment. The mainly sparse type of malignant melanoma and malignant fibrous histiocytoma predominately occur in the lower thoracic segment, and the remaining sparse types mainly occur in the middle thoracic segment.

With the development of the COVID-19 epidemic, the psychological impacts and stress-related symptoms associated with it have gradually appeared. The preventative and therapeutic psychological crisis interventions are urgently needed. This article focused on the international psychological crisis intervention models, which included the critical incident stress management, the task model, and the assessment-crisis intervention-trauma treatment intervention model. The aim of the current study was to form the suitable interventions for COVID-19 according to existing intervention methods and the specific characteristics of COVID-19. Possible coping strategies were also proposed for COVID-19 psychological crisis, which provided a theoretical basis for establishing an integrated public health emergency system and mechanism for psychological crisis prevention, evaluation, intervention and follow-up in China.