Xi Jinping Thought on Culture is an important guideline for cultural construction in the new era,providing a new theoretical vision and action guide for the cultural construction of public hospitals.Since the Party's 18th National Congress,a series of reform practices in public hospitals have laid a solid foundation for cultural con-struction and shaped the eight elements of cultural construction in public hospitals.A public cancer specialized hospi-tal in Beijing closely focuses on the spiritual essence of Xi Jinping Thought on Culture,actively explores and practic-es,and builds an all-round hospital culture construction system and action path in the new era.

Objective:To carry out genetic testing and clinical phenotypic characterization on a four-generation Chinese pedigree affected with Stickler syndrome type I and explore its genotype-phenotype correlation.Methods:A child presented at the Second Affiliated Hospital of Guangzhou University of Traditional Chinese Medicine in February 2023 for micrognathia, glossoptosis and cleft palate and his family members were selected as the study subjects. Clinical data were collected from the affected members, and peripheral blood samples were obtained from 17 participants (4 patients and 13 asymptomatic individuals). Whole exome sequencing (WES) was carried out. Candidate variant was verified by Sanger sequencing. Genotype-phenotype correlations were analyzed by integrating the sequencing data with evidence from existing literature. This study has bee granted by the Ethics Committee of Guangdong Provincial Hospital of Traditional Chinese Medicine and Guangzhou Women and Children′s Medical Center (Ethics No.: 2022-406B00).Results:The four-generation pedigree has comprised 19 members. In addition to the proband, 5 affected individuals had manifested high myopia, congenital cataracts, and progressive vision loss. Two deceased members reportedly exhibited similar ocular manifestations. Among the four living patients, two had developed retinal detachment, while two others presented with chronic joint pain onset between 35 ~ 40 years of age. One patient required hip replacement surgery at age 42 secondary to femoral head necrosis. The proband, the youngest affected member, exhibited characteristic phenotypes including congenital micrognathia and cleft palate, consistent with Pierre-Robin syndrome. Genetic analysis revealed a heterozygous nonsense mutation in COL2A1 (NM_001844.5: c. 2668C>T; p. Gln890Ter) segregating with the disease in all four symptomatic patients. This variant was absent in asymptomatic family members and unaffected controls. While the mutation is listed in ClinVar, no clinical case reports has associated it with this phenotypic spectrum. It was not observed in population databases (gnomAD v4.1.0, 1000 Genomes Project, or ExAC), supporting its potential pathogenicity. Conclusion:This study has diagnosed a four-generation Chinese pedigree with Stickler syndrome type I attributed to the pathogenic COL2A1 variant c. 2668C>T (p.Gln890Ter), which is a rare nonsense mutation associated with ocular predominance and variable skeletal involvement. Notably, this family exhibited marked clinical heterogeneity despite sharing the identical genotype, which highlighted challenges in phenotype-genotype correlation. The autosomal dominant transmission pattern observed in this pedigree has provided critical insights into COL2A1-related collagenopathies and underscored the necessity of ultrasonographic monitoring for ocular anomalies in prenatal diagnostics. Above findings have advanced our understanding of pleiotropic effects in type Ⅱ collagen disorders and lay the foundation for precision-based genetic counseling, enabling targeted cascade screening and implementation of tertiary prevention strategies against congenital disabilities in high-risk families.

BACKGROUND: While emotional distress, encompassing anxiety and depression, has been associated with negative clinical outcomes, its impact across various clinical departments and general hospitals has been less explored. Previous studies with limited sample sizes have examined the effectiveness of specific treatments (e.g., antidepressants) rather than a systemic management strategy for outcome improvement in non-psychiatric inpatients. To enhance the understanding of the importance of addressing mental health care needs among non-psychiatric patients in general hospitals, this study retrospectively investigated the impacts of emotional distress and the effects of early detection and management of depression and anxiety on hospital length of stay (LOS) and rate of long LOS (LLOS, i.e., LOS >30 days) in a large sample of non-psychiatric inpatients. METHODS: This retrospective cohort study included 487,871 inpatients from 20 non-psychiatric departments of a general hospital. They were divided, according to whether they underwent a novel strategy to manage emotional distress which deployed the Huaxi Emotional Distress Index (HEI) for brief screening with grading psychological services (BS-GPS), into BS-GPS ( n = 178,883) and non-BS-GPS ( n = 308,988) cohorts. The LOS and rate of LLOS between the BS-GPS and non-BS-GPS cohorts and between subcohorts with and without clinically significant anxiety and/or depression (CSAD, i.e., HEI score ≥11 on admission to the hospital) in the BS-GPS cohort were compared using univariable analyses, multilevel analyses, and/or propensity score-matched analyses, respectively. RESULTS: The detection rate of CSAD in the BS-GPS cohort varied from 2.64% (95% confidence interval [CI]: 2.49%-2.81%) to 20.50% (95% CI: 19.43%-21.62%) across the 20 departments, with a average rate of 5.36%. Significant differences were observed in both the LOS and LLOS rates between the subcohorts with CSAD (12.7 days, 535/9590) and without CSAD (9.5 days, 3800/169,293) and between the BS-GPS (9.6 days, 4335/178,883) and non-BS-GPS (10.8 days, 11,483/308,988) cohorts. These differences remained significant after controlling for confounders using propensity score-matched comparisons. A multilevel analysis indicated that BS-GPS was negatively associated with both LOS and LLOS after controlling for sociodemographics and the departments of patient discharge and remained negatively associated with LLOS after controlling additionally for the year of patient discharge. CONCLUSION Emotional distress significantly prolonged the LOS and increased the LLOS of non-psychiatric inpatients across most departments and general hospitals. These impacts were moderated by the implementation of BS-GPS. Thus, BS-GPS has the potential as an effective, resource-saving strategy for enhancing mental health care and optimizing medical resources in general hospitals.
Humans ; Retrospective Studies ; Male ; Length of Stay/statistics & numerical data* ; Female ; Middle Aged ; Adult ; Psychological Distress ; Inpatients/psychology* ; Aged ; Anxiety/diagnosis* ; Depression/diagnosis*

OBJECTIVE: To carry out genetic testing and clinical phenotypic characterization on a four-generation Chinese pedigree affected with Stickler syndrome type I and explore its genotype-phenotype correlation. METHODS: A child presented at the Second Affiliated Hospital of Guangzhou University of Traditional Chinese Medicine in February 2023 for micrognathia, glossoptosis and cleft palate and his family members were selected as the study subjects. Clinical data were collected from the affected members, and peripheral blood samples were obtained from 17 participants (including 4 patients and 13 asymptomatic individuals). Whole exome sequencing (WES) was carried out. Candidate variant was verified by Sanger sequencing. Genotype-phenotype correlation was analyzed by integrating the sequencing data with evidence from existing literature. This study has bee granted by the Ethics Committee of Guangdong Provincial Hospital of Traditional Chinese Medicine and Guangzhou Women and Children's Medical Center (Ethics No.: 2022-406B00). RESULTS: The four-generation pedigree has comprised 19 members. In addition to the proband, 5 affected individuals had manifested with high myopia, congenital cataracts, and progressive vision loss. Two deceased members reportedly exhibited similar ocular manifestations. Among the four living patients, two had developed retinal detachment, while two others presented with chronic joint pain onset between 35 ~ 40 years of age. One patient required hip replacement surgery at age 42 secondary to femoral head necrosis. The proband, the youngest affected member, exhibited characteristic phenotypes including congenital micrognathia and cleft palate, consistent with Pierre-Robin syndrome. Genetic analysis revealed a heterozygous nonsense mutation in COL2A1 (NM_001844.5: c.2668C>T; p.Gln890Ter) segregating with the disease in all four symptomatic patients. This variant was absent in asymptomatic family members and unaffected controls. While the mutation is listed in ClinVar, no clinical case report has associated it with this phenotypic spectrum. It was not recorded in population databases (gnomAD v4.1.0, 1000 Genomes Project, or ExAC), supporting its potential pathogenicity. CONCLUSION This study has diagnosed a four-generation Chinese pedigree with Stickler syndrome type I attributed to the pathogenic COL2A1 variant c.2668C>T (p.Gln890Ter), which is a rare nonsense mutation associated with ocular predominance and variable skeletal involvement. Notably, this family exhibited marked clinical heterogeneity despite sharing the identical genotype, which highlighted the challenges in phenotype-genotype correlation. The autosomal dominant transmission pattern observed in this pedigree has provided critical insights into COL2A1-related collagenopathies and underscored the necessity of ultrasonographic monitoring for ocular anomalies during prenatal diagnosis. Above findings have advanced our understanding of the pleiotropic effects in type Ⅱ collagen disorders and laid the foundation for precision-based genetic counseling, enabling targeted cascade screening and implementation of tertiary prevention strategies against congenital disabilities for high-risk families.
Adolescent ; Adult ; Child ; Child, Preschool ; Female ; Humans ; Male ; Middle Aged ; Arthritis/genetics* ; Collagen Type II/genetics* ; Connective Tissue Diseases/genetics* ; Exome Sequencing ; Genetic Association Studies ; Genotype ; Hearing Loss, Sensorineural/genetics* ; Mutation ; Pedigree ; Phenotype ; Retinal Detachment/genetics* ; East Asian People/genetics*

Acute pancreatitis(AP)is a frequently encountered acute abdominal syndrome in clinical settings,and the integrated model of traditional Chinese and Western medicine(TCM-WM)has demonstrated notable advantages in the diagnosis and treatment of AP.To systematize and standardize clinical practices related to develop clinical pathway for integrated TCM-WM diagnosis and treatment of AP,which enhances the efficiency and quality of patient care.This pathway focuses on AP,a common acute and life-threatening disease within the digestive system,and outlines that the central pathological mechanism involves pancreatic injury and localized inflammation resulting from the abnormal activation of pancreatic enzymes.It has the characteristics of rapid onset,multiple causes,and complex manifestations.Severe cases can be life-threatening.At present,conventional treatments encompass a diverse range of modalities.Moreover,traditional Chinese medicine(TCM)holds distinct advantages in alleviating relevant symptoms,and TCM-WM is gaining increasing prevalence.To enhance the standardization and consistency of diagnostic and therapeutic practices,this clinical pathway clearly delineates the target patient population,which includes individuals diagnosed with abdominal pain disorder according to TCM and with AP in accordance with WM criteria,as well as the corresponding inclusion standards.The diagnostic framework integrates both TCM and WM guidelines,and further incorporates disease staging,severity grading,and syndrome differentiation to support a comprehensive and integrated diagnostic strategy.The treatment integrates approaches from both TCM and WM.Within the WM framework,interventions consist of basic supportive care,infection control,nutritional support,and the management of complications.In the context of TCM,the protocol includes syndrome differentiation and corresponding therapeutic strategies(Distinct syndrome patterns are identified and managed during the acute and convalescent phases),such as acupuncture and retention enema.This clinical pathway addresses multiple key components,including preventive strategies,post-treatment follow-up,criteria for evaluating therapeutic efficacy,admission and discharge,admission examination protocols,discharge criteria,and the rationale for deviations or withdrawal from the pathway.It is designed to provide a systematic and standardized reference framework for relevant clinical practices.

Objective:To identify different subtypes of patients with preeclampsia(PE)through clinical liver function index data-driven the cluster analysis,to explore the correlation between liver function of different sub-types and pregnancy complications.Methods:From January 2012 to December 2022,the general data of 2230 sin-gleton pregnant women with PE who underwent prenatal examination and delivered in Fujian Maternity and Child Health Hospital were collected.Using 13 liver function indexes before delivery as baseline variables,all included subjects were classified into subtypes by cluster method.The clinical characteristics of different subtypes of PE patients were compared.Single-factor Logistic regression was used to analyze the risk of pregnancy complications among subtypes.Results:PE patients were divided into 3 subgroups that represented different characteristics of patients' liver function.The first subtype(n=1065)exhibited abnormal liver enzymology index characterized by in-creased alkaline phosphatase(ALP)level.The second subtype(n=648)showed abnormal bilirubin metabolism index with the highest levels of total bilirubin(TBIL),direct(DBIL)and indirect bilirubin(IBIL).The third subtype(n=517)had abnormal liver enzymology indexes with elevated alanine aminotransferase(ALT)and aspartate aminotransferase(AST)levels,abnormal bile acid detection indexes with elevated total bile acid(TBA)levels,and abnormal liver synthesis function indexes with decreased total protein(TP),albumin(ALB),and globulin lev-els(GLB).Significant differences were observed among the three subtypes in age,severe PE,anemia,cardiac dysfunction,and renal dysfunction(P＜0.05).Single-factor Logistic regression demonstrated that the third sub-type had significantly higher risks of intrahepatic cholestasis of pregnancy,fetal growth restriction,premature rup-ture of membranes,and preterm birth compared to the first and second subtypes(P＜0.05),as well as a higher risk of placental abruption than the second subtype(P＜0.05).The first subtype had higher risks of placental ab-ruption and fetal growth restriction than the second subtype(P＜0.05).Conclusions:Cluster analysis could be used to subclassify PE patients by liver function characteristics,so as to identify the occurrence of pregnancy complications.The results had significance for understanding the heterogeneity of PE and promoting individualized management.

Objective:To identify different subtypes of patients with preeclampsia(PE)through clinical liver function index data-driven the cluster analysis,to explore the correlation between liver function of different sub-types and pregnancy complications.Methods:From January 2012 to December 2022,the general data of 2230 sin-gleton pregnant women with PE who underwent prenatal examination and delivered in Fujian Maternity and Child Health Hospital were collected.Using 13 liver function indexes before delivery as baseline variables,all included subjects were classified into subtypes by cluster method.The clinical characteristics of different subtypes of PE patients were compared.Single-factor Logistic regression was used to analyze the risk of pregnancy complications among subtypes.Results:PE patients were divided into 3 subgroups that represented different characteristics of patients' liver function.The first subtype(n=1065)exhibited abnormal liver enzymology index characterized by in-creased alkaline phosphatase(ALP)level.The second subtype(n=648)showed abnormal bilirubin metabolism index with the highest levels of total bilirubin(TBIL),direct(DBIL)and indirect bilirubin(IBIL).The third subtype(n=517)had abnormal liver enzymology indexes with elevated alanine aminotransferase(ALT)and aspartate aminotransferase(AST)levels,abnormal bile acid detection indexes with elevated total bile acid(TBA)levels,and abnormal liver synthesis function indexes with decreased total protein(TP),albumin(ALB),and globulin lev-els(GLB).Significant differences were observed among the three subtypes in age,severe PE,anemia,cardiac dysfunction,and renal dysfunction(P＜0.05).Single-factor Logistic regression demonstrated that the third sub-type had significantly higher risks of intrahepatic cholestasis of pregnancy,fetal growth restriction,premature rup-ture of membranes,and preterm birth compared to the first and second subtypes(P＜0.05),as well as a higher risk of placental abruption than the second subtype(P＜0.05).The first subtype had higher risks of placental ab-ruption and fetal growth restriction than the second subtype(P＜0.05).Conclusions:Cluster analysis could be used to subclassify PE patients by liver function characteristics,so as to identify the occurrence of pregnancy complications.The results had significance for understanding the heterogeneity of PE and promoting individualized management.

To provide scientific guidance and standardization for perioperative clinical nursing practice in patients undergoing percutaneous renal biopsy, relevant evidence on percutaneous renal biopsy nursing care was systematically retrieved and synthesized through an evidence-based approach. After two rounds of Delphi expert consultation and expert panel discussions, expert consensus on perioperative clinical nursing standards for patients undergoing percutaneous renal biopsy (hereinafter referred to as the "Consensus") was developed. The Consensus includes three primary themes: preoperative care, intraoperative care, and postoperative care, encompassing 21 secondary themes. It is characterized by scientific rigor, practical applicability, and comprehensiveness, and serves as a valuable reference and guide for clinical nursing professionals across medical institutions.

Xi Jinping Thought on Culture is an important guideline for cultural construction in the new era,providing a new theoretical vision and action guide for the cultural construction of public hospitals.Since the Party's 18th National Congress,a series of reform practices in public hospitals have laid a solid foundation for cultural con-struction and shaped the eight elements of cultural construction in public hospitals.A public cancer specialized hospi-tal in Beijing closely focuses on the spiritual essence of Xi Jinping Thought on Culture,actively explores and practic-es,and builds an all-round hospital culture construction system and action path in the new era.

To provide scientific guidance and standardization for perioperative clinical nursing practice in patients undergoing percutaneous renal biopsy, relevant evidence on percutaneous renal biopsy nursing care was systematically retrieved and synthesized through an evidence-based approach. After two rounds of Delphi expert consultation and expert panel discussions, expert consensus on perioperative clinical nursing standards for patients undergoing percutaneous renal biopsy (hereinafter referred to as the "Consensus") was developed. The Consensus includes three primary themes: preoperative care, intraoperative care, and postoperative care, encompassing 21 secondary themes. It is characterized by scientific rigor, practical applicability, and comprehensiveness, and serves as a valuable reference and guide for clinical nursing professionals across medical institutions.