Objective:To investigate the clinical and genetic characteristics of a Henan Han family with pontine autosomal dominant microangiopathy and leukoencephalopathy (PADMAL), aiming to enhance understanding of this disease.Methods:The proband was first admitted to the Department of Neurology, Henan Provincial People′s Hospital, Fuwai Central China Cardiovascular Hospital in December 2019 due to cerebral infarction and unilateral limb numbness and weakness. Detailed medical history collection, pedigree mapping, whole-exome sequencing screening, and Sanger sequencing validation were performed for the proband and family members. The patients′ clinical manifestations, imaging features, neuropsychological scale assessment results, and pathological changes were summarized, and genetic analysis was conducted on the gene variant site. Relevant literature was reviewed to summarize the characteristics of PADMAL.Results:The proband was a 47-year-old female, with 3 generations of family members affected, including 7 patients, 3 of whom had died. The clinical features of the patients were similar, with the first stroke occurring around the age of 40, without vascular risk factors such as hypertension or diabetes. The main clinical manifestation was unilateral limb numbness and weakness. The proband and her niece sought medical attention due to stroke symptoms. Brain magnetic resonance imaging revealed acute infarct lesions located in the pons, accompanied by multiple oval infarct foci (the "raisin bread sign") and white matter hyperintensity changes. Genetic testing showed that 4 patients carried a heterozygous c. *34GT mutation in the 3′-untranslated region (3′-UTR) of the COL4A1 gene, while the other 4 unaffected family members did not carry this variant, consistent with genotype- phenotype co-segregation in the family. Conclusions:PADMAL is an extremely rare monogenic cerebral small vessel disease caused by pathogenic variants in the 3′-UTR of the COL4A1 gene. The "raisin bread sign" in the pons is a relatively specific imaging feature that distinguishes it from other cerebral small vessel diseases. For patients with this sign, genetic testing for PADMAL should be considered.

Objective:To explore the application indications, clinical characteristics and influencing factors of extracorporeal membrane oxygenation (ECMO) in the treatment of critically ill pregnant and postpartum women in Bao′an district, Shenzhen, and summarize regional experience.Methods:A retrospective analysis was conducted on 5 cases of pregnant and postpartum women who received ECMO treatment at the CUHK Women′s and Children′s Medical Centre (Shenzhen) and the Shenzhen Bao′an People′s Hospital from 2020 to 2024. Baseline characteristics, ECMO parameters, complications and maternal-infant outcomes of the patients were collected.Results:The patients′ age was 29(24, 36) years old, gestational age was 39(31, 39) weeks, and ECMO maintenance time was 8(4, 8) days. ECMO indications included 2 cases of cardiac arrest, 1 case of respiratory and circulatory failure, 1 case of cardiogenic shock, and 1 case of acute respiratory distress syndrome. There were 4 cases of veno-arterial (VA)-ECMO and 1 case of veno-venous (VV)-ECMO. Complications included 3 cases of bleeding, 4 cases of acute renal failure, 2 cases of thrombosis, and 2 cases of infection. Both maternal and infant survival were 3 cases. Successful cases benefited from the multidisciplinary rapid response team and regional transportation cooperation, while failed cases were mostly accompanied by severe bleeding and disseminated intravascular coagulation.Conclusions:ECMO improves the success rate of treating critically ill pregnant and postpartum women in Bao′an District. Amniotic fluid embolism and severe pulmonary hypertension are the main indications. Regional multidisciplinary cooperation, accurate initiation timing and individualized anticoagulation management are the keys. It is recommended to establish a national ECMO registration system for pregnant and postpartum women to optimize treatment strategies and improve maternal and infant prognosis.

Natural human leukocyte antigen (HLA) antibodies refer to preformed antibodies present in the body that are not induced by prior exposure to allogeneic HLA antigens. In healthy individuals without a sensitization history, the detection rate of natural HLA antibodies is approximately 20%-29% when using screening assays with low sensitivity, and can reach up to 63% when more sensitive HLA-specific detection methods are employed. It is therefore inferred that natural HLA antibodies may also be present in transplant candidates with a similar prevalence. This review comprehensively discusses the potential mechanisms of natural HLA antibody generation, the characteristics of the recognized epitopes, detection techniques, clinical relevance in transplantation, their potential to confound therapeutic decisions, and approaches to distinguish and mitigate their impact. The goal is to raise clinician awareness of the objective existence of natural HLA antibodies, provide guidance on evaluating their association with allograft rejection, and inform appropriate clinical management strategies when encountering natural HLA antibody-positive transplant candidates.

Objective To analyze the current practices of the telemedicine collaboration network in Jiangxi Province and provide references for enhancing the efficiency of telemedicine services and strengthening the overall telemedicine system.Meth-ods The research team investigates the progress of the telemedicine service system in Jiangxi Province,using the construction status of the telemedicine collaboration network at Jiangxi Provincial People's Hospital as a case study.The study identifies prob-lems and challenges encountered during the establishment and proposes development strategies.Results Jiangxi has adopted a"holistic approach,"implementing a series of measures including enhanced top-level design,pilot exploration,the establishment of a provincial platform,formulation of supportive policies,and intensified training to promote telemedicine vigorously.In Decem-ber 2015,Jiangxi Provincial People's Hospital established the first provincial teleconsultation center,innovatively advancing the medical consortium construction through the"telemedicine collaboration network"model.By 2024,the hospital completed 20,417 telemedicine service instances and conducted 182 remote surgical demonstrations.Additionally,since its inception,the remote electrocardiogram service has accumulated 1,138,295 instances,effectively enhancing the quality and efficiency of medi-cal services,facilitating tiered diagnosis and treatment,and alleviating access difficulties for grassroots populations.Conclusion Leveraging the National Intelligent Social Governance Pilot Base(Health and Wellness),the study promotes the integration of next-generation information technologies such as big data,artificial intelligence,and cloud computing into the traditional health-care sector.This aims to provide high-quality,efficient,and convenient medical services to the public,achieving comprehensive coverage of the telemedicine collaboration network across provincial,municipal,county,and township levels.In the next steps,the focus will be on strengthening quality control,improving supportive policies,and scientifically managing operations to foster the sustainable development of telemedicine health services,ensuring a"one-stop service and one-stop oversight"system across the province.

Objective:To investigate the relationship between nerve growth factor (NGF) concentration in follicular fluid and abnormal menstrual cycle in infertile patients with polycystic ovary syndrome (PCOS).Methods:A retrospective cohort study was conducted on 100 infertile patients with PCOS who underwent in vitro fertilization and embryo transfer (IVF-ET) at Department of Obstetrics and Gynecology, Peking University Third Hospital from March 2017 to June 2019. For comparison, the 100 patients with PCOS were divided into low NGF group ( n=50) and high NGF group ( n=50) based on the median NGF concentration (1 644.03 ng/L) in follicular fluid. Baseline characteristics, menstrual status and clinical outcomes of assisted reproductive technology were compared. We performed multiple linear regression analysis to examine the effect of NGF in follicular fluid on menstrual cycle length for multivariate analysis. Results:1) PCOS patients in the low NGF group had significantly higher body mass index [(27.24±5.17) kg/m 2] and white blood cell count [7.31(5.99, 8.43)×10 9/L ] than those in the high NGF group [(25.03±4.46) kg/m 2, P=0.024; 5.95(5.08,7.01)×10 9/L, P=0.001], while high-density lipoprotein cholesterol [1.15 (0.98, 1.36) mmol/L] and basic follicle-stimulating hormone level [6.51 (5.10,7.95) U/L] in the low NGF group were significantly lower than those in the high NGF group [1.36 (1.09,1.52) mmol/L, P=0.039;6.51 (5.10,7.95)U/L, P=0.040]. 2) PCOS patients in the low NGF group had significantly higher menstrual cycle length [60.00 (35.00, 180.00) d] than the high NGF group [32.50 (27.00,67.50) d, P=0.001]. 3) Multiple linear regression analysis revealed that after adjustment for body mass index, age, infertility duration, infertility type, and glucose and lipid metabolic parameters, the NGF concentration in the follicular fluid independently and negatively correlated with menstrual cycle length ( P<0.05). 4) The NGF concentration in follicular fluid was not correlated with assisted reproductive outcomes. Conclusion:NGF concentration in follicular fluid is closely related to the degree of menstrual cycle abnormalities in patients with PCOS.

Objective:To investigate the relationship between nerve growth factor (NGF) concentration in follicular fluid and abnormal menstrual cycle in infertile patients with polycystic ovary syndrome (PCOS).Methods:A retrospective cohort study was conducted on 100 infertile patients with PCOS who underwent in vitro fertilization and embryo transfer (IVF-ET) at Department of Obstetrics and Gynecology, Peking University Third Hospital from March 2017 to June 2019. For comparison, the 100 patients with PCOS were divided into low NGF group ( n=50) and high NGF group ( n=50) based on the median NGF concentration (1 644.03 ng/L) in follicular fluid. Baseline characteristics, menstrual status and clinical outcomes of assisted reproductive technology were compared. We performed multiple linear regression analysis to examine the effect of NGF in follicular fluid on menstrual cycle length for multivariate analysis. Results:1) PCOS patients in the low NGF group had significantly higher body mass index [(27.24±5.17) kg/m 2] and white blood cell count [7.31(5.99, 8.43)×10 9/L ] than those in the high NGF group [(25.03±4.46) kg/m 2, P=0.024; 5.95(5.08,7.01)×10 9/L, P=0.001], while high-density lipoprotein cholesterol [1.15 (0.98, 1.36) mmol/L] and basic follicle-stimulating hormone level [6.51 (5.10,7.95) U/L] in the low NGF group were significantly lower than those in the high NGF group [1.36 (1.09,1.52) mmol/L, P=0.039;6.51 (5.10,7.95)U/L, P=0.040]. 2) PCOS patients in the low NGF group had significantly higher menstrual cycle length [60.00 (35.00, 180.00) d] than the high NGF group [32.50 (27.00,67.50) d, P=0.001]. 3) Multiple linear regression analysis revealed that after adjustment for body mass index, age, infertility duration, infertility type, and glucose and lipid metabolic parameters, the NGF concentration in the follicular fluid independently and negatively correlated with menstrual cycle length ( P<0.05). 4) The NGF concentration in follicular fluid was not correlated with assisted reproductive outcomes. Conclusion:NGF concentration in follicular fluid is closely related to the degree of menstrual cycle abnormalities in patients with PCOS.

Objective:To retrieve, evaluate, and integrate evidence on output and input record in patients with heart failure.Methods:Literature related to output and input record for patients with heart failure was systematically searched in national and international guideline networks, professional association websites, and databases. The search period was from database establishment to October 31, 2024. Evidence was extracted, integrated, and graded after two researchers independently conducted a literature quality assessment.Results:A total of 15 papers were included, including six guidelines, two expert consensus, two evidence summaries, one recommended practice, two systematic reviews, and two expert opinions. Twenty-five pieces of best evidence were summarized in five areas of objects and content, fluid measurement, recording and aggregation, evaluation and monitoring, and training and guidance.Conclusions:This study summarizes the best evidence for output and input record in patients with heart failure, which may inform nursing staff in their clinical practice. Nursing staff should judiciously select and apply evidence in a clinical context so as to improve the accuracy of output and input record.

Objective To analyze the current practices of the telemedicine collaboration network in Jiangxi Province and provide references for enhancing the efficiency of telemedicine services and strengthening the overall telemedicine system.Meth-ods The research team investigates the progress of the telemedicine service system in Jiangxi Province,using the construction status of the telemedicine collaboration network at Jiangxi Provincial People's Hospital as a case study.The study identifies prob-lems and challenges encountered during the establishment and proposes development strategies.Results Jiangxi has adopted a"holistic approach,"implementing a series of measures including enhanced top-level design,pilot exploration,the establishment of a provincial platform,formulation of supportive policies,and intensified training to promote telemedicine vigorously.In Decem-ber 2015,Jiangxi Provincial People's Hospital established the first provincial teleconsultation center,innovatively advancing the medical consortium construction through the"telemedicine collaboration network"model.By 2024,the hospital completed 20,417 telemedicine service instances and conducted 182 remote surgical demonstrations.Additionally,since its inception,the remote electrocardiogram service has accumulated 1,138,295 instances,effectively enhancing the quality and efficiency of medi-cal services,facilitating tiered diagnosis and treatment,and alleviating access difficulties for grassroots populations.Conclusion Leveraging the National Intelligent Social Governance Pilot Base(Health and Wellness),the study promotes the integration of next-generation information technologies such as big data,artificial intelligence,and cloud computing into the traditional health-care sector.This aims to provide high-quality,efficient,and convenient medical services to the public,achieving comprehensive coverage of the telemedicine collaboration network across provincial,municipal,county,and township levels.In the next steps,the focus will be on strengthening quality control,improving supportive policies,and scientifically managing operations to foster the sustainable development of telemedicine health services,ensuring a"one-stop service and one-stop oversight"system across the province.

Objective:To retrieve, evaluate, and integrate evidence on output and input record in patients with heart failure.Methods:Literature related to output and input record for patients with heart failure was systematically searched in national and international guideline networks, professional association websites, and databases. The search period was from database establishment to October 31, 2024. Evidence was extracted, integrated, and graded after two researchers independently conducted a literature quality assessment.Results:A total of 15 papers were included, including six guidelines, two expert consensus, two evidence summaries, one recommended practice, two systematic reviews, and two expert opinions. Twenty-five pieces of best evidence were summarized in five areas of objects and content, fluid measurement, recording and aggregation, evaluation and monitoring, and training and guidance.Conclusions:This study summarizes the best evidence for output and input record in patients with heart failure, which may inform nursing staff in their clinical practice. Nursing staff should judiciously select and apply evidence in a clinical context so as to improve the accuracy of output and input record.

Objective:To investigate the clinical and genetic characteristics of a Henan Han family with pontine autosomal dominant microangiopathy and leukoencephalopathy (PADMAL), aiming to enhance understanding of this disease.Methods:The proband was first admitted to the Department of Neurology, Henan Provincial People′s Hospital, Fuwai Central China Cardiovascular Hospital in December 2019 due to cerebral infarction and unilateral limb numbness and weakness. Detailed medical history collection, pedigree mapping, whole-exome sequencing screening, and Sanger sequencing validation were performed for the proband and family members. The patients′ clinical manifestations, imaging features, neuropsychological scale assessment results, and pathological changes were summarized, and genetic analysis was conducted on the gene variant site. Relevant literature was reviewed to summarize the characteristics of PADMAL.Results:The proband was a 47-year-old female, with 3 generations of family members affected, including 7 patients, 3 of whom had died. The clinical features of the patients were similar, with the first stroke occurring around the age of 40, without vascular risk factors such as hypertension or diabetes. The main clinical manifestation was unilateral limb numbness and weakness. The proband and her niece sought medical attention due to stroke symptoms. Brain magnetic resonance imaging revealed acute infarct lesions located in the pons, accompanied by multiple oval infarct foci (the "raisin bread sign") and white matter hyperintensity changes. Genetic testing showed that 4 patients carried a heterozygous c. *34GT mutation in the 3′-untranslated region (3′-UTR) of the COL4A1 gene, while the other 4 unaffected family members did not carry this variant, consistent with genotype- phenotype co-segregation in the family. Conclusions:PADMAL is an extremely rare monogenic cerebral small vessel disease caused by pathogenic variants in the 3′-UTR of the COL4A1 gene. The "raisin bread sign" in the pons is a relatively specific imaging feature that distinguishes it from other cerebral small vessel diseases. For patients with this sign, genetic testing for PADMAL should be considered.