Objective To explore the application effect of action-oriented teaching method in senior midwifery practice teaching.Methods Selecting 30 students from class 1,grade 2011 of the midwifery professional for the experimental group,another 30 students from class 2 for the control group.We selected four core contents for the experimental teaching refbrm such as obstetric abdominal examination,external pelvimetry measurement,prenatal vulva washing disinfection and sterile towel covering before the delivery.The experimental group was taught by action-oriented teaching,in accordance with the procedures:putting forward the projects or work task,set goals and grouping,making work plans,implementing and evaluating.The control group was taught by traditional teaching.We used questionnaire smvey and skills examination to evaluate the teaching effects and conducted teaching satisfaction scores and scores for t test to two groups of students.Results (1)Questionnaires:The resuhs of the 18 issues on the teaching effect evaluation,professional technology level and nursing soft skills,etc.showed that the experimental group's evaluation of the action-oriented teaching is higher than the control group's evaluation of the traditional teaching(P＜0.05),action-oriented teaching is more helptul to improving the professional skill and nursing soft skills(P＜0.05).@The scores of skill examination:The skills scores of the experimental group(80.01 ± 9.36) were significantly higher than those of the control group(71.32 ± 8.85)(t=3.70,P=0.000).The difference was statistically significant (t=3.70,P=0.00).Conclusions Action-oriented teaching helps to raise practice teaching effecr,train students' clinical work ability and nursing soft skills.It is suitable for practice teaching of senior midwifery.

Objective To explore the ultrasonographic features ,diagnostic clues and methods of fetal aortic arch anomalies ,and to improve the diagnosis rate of prenatal ultrasonography for fetal aortic arch anomalies . Methods Fetuses with aortic arch anomalies diagnosed by prenatal ultrasound were selected as the study objects . The prenatal ultrasonographic features of the three views of the aortic arch were carefully observed . The three views included the followings :the transverse view ( the view of three vessels and trachea) ,the sagittal view ( the longitudinal view of the aortic arch) ,and the coronal view ( the coronal view of the descending aorta) . The prenatal diagnostic clues and diagnostic techniques of aortic arch anomalies were summarized and analyzed . Results Eighty-one cases with aortic arch anomalies were followed up . Fifteen cases were left-sided aortic arch and left-sided ductus arteriosus with aberrant right subclavian artery ,22 cases with right aortic arch and left-sided ductus arteriosus with aberrant left subclavian artery ,4 cases were mirror right aortic arch and left-sided ductus arteriosus ,2 cases were mirror right aortic arch and right-sided ductus arteriosus ,11 cases were double aortic arch ,23 cases were coarctation of the aorta ,and 4 cases were interruption of aortic arch . Among them ,27 cases were complicated with other cardiac and extra-cardiac malformations and 13 cases with chromosomal abnormalities . Prenatal ultrasonographic clues for diagnosis of coarctation of the aorta included the three vessels and trachea view showing small diameter and disproportion to the diameter of the ductus arteriosus . And the longitudinal view of the aortic arch was helpful for showing the position and the extent of coarctation of the aorta . Prenatal ultrasonographic clues for diagnosis of interruption of aortic arch were that the three vessels and trachea view and the longitudinal view of the aortic arch showed the interruption between aortic arch and descending aorta . And the abnormal combination cases of the aortic arch segments also had their corresponding diagnostic clues . These clues included that the three vessels and trachea view showed abnormal position ,arrangement and running of the branches of the aortic arch . And the coronal view of the descending aorta also showed abnormal changes in the branches of aortic arch . Conclusions Various types of congenital aortic arch anomalies have characteristic ultrasonography and their corresponding diagnostic clues and methods . The combined use of the three views of the aortic arch is effective in prenatal diagnosis for aortic arch anomalies .

OBJECTIVE: To study the molecular pathogenesis of SLC26A4 mutations associated with inner ear malformations including large vestibular aqueduct syndrome (LVAS), Mondini dysplasia and inner ear malformations but not accompanied with LVAS. METHOD: DNA sample and clinical material were obtained from 14 sporadic LVAS probands, six Mondini dysplasia probands and seven inner ear malformations excluding IVAS probands. SLC26A4 gene mutation was analyzed by direct sequencing for its 20 coding exons. GJB2 gene and also mt12SrRNA were analyzed by direct sequencing. RESULT: In 14 cases of LVAS, two mutations were detected in 12 patients (85.7%, either homozygous or compound heterozygous mutations), and one mutation was found in two patients (14.3%). In six cases of Mondini dysplasia, two mutations were detected in all of patients (100%). No mutation could be found in the seven cases of other inner ear abnormalities not accompanied with LVAS. No pathogenic mutation was detected in all of these 27 probands in GJB2 gene and mt12SrRNA 1555/1494T. CONCLUSION We have shown that LVAS and Mondini dysplasia closely correlate with SLC26A4 gene. No mutation was detected in seven probands of inner ear malformations not accompanied with LVAS. We should study the molecular pathogenesis of this disease in depth.
Adolescent ; Adult ; Child ; Child, Preschool ; Connexins ; Ear, Inner ; abnormalities ; Exons ; Female ; Genome ; Humans ; Infant ; Male ; Membrane Transport Proteins ; genetics ; Mutation ; Sulfate Transporters ; Syndrome ; Vestibular Aqueduct ; abnormalities ; Young Adult

Nonalcoholic fatty liver disease is a metabolic stress liver injury, including simple hepatic steatosis, nonalcoholic steatohepatitis (NASH), cirrhosis, and hepatocellular carcinoma (HCC). Liver fibrosis in NASH patients progressed by an average of 7 to 10 years, and the incidence of cirrhosis in NAH patients was only 0.6％to 3.0％after 10 to 20 years of follow-up. This case reports a 21-year-old man with pituitary dysfunction and cirrhosis, whose condition develops rapidly. The mechanism may be that growth hormone deficiency affects liver signaling transduction pathways to make the liver metabolism disordered, causing nonalcoholic fatty liver disease. In this report, the pathogenesis, diagnosis and treatment of this case of cirrhosis duo to hypopituitarism in adolescence are reviewed retrospectively to improve the understanding of the diagnosis and therapy of this disease.

Objectives It was a preliminary exploration on rare disease management of comprehensive hospital based on the"Center-Multidisciplinary Team"structure.Methods By applying interrupted time series analysis,the characteristics and related medical service of rare disease patients discharged from sample hospitals from 2021 to 2023 were measured.Results The results showed that the hospital served 19 825 rare disease patients from 2021 to 2023.The proportion of patients whose main diagnosis were rare diseases was approximately 27％.The interrupted time series technique indicated that the expected proportion of rare disease patients increased instantly by 1.89 thousand points.The capability to diagnose has significantly improved,with a growing trend on volume of bioinformatics analysis services for rare diseases patients.Conclusion The rare disease management based on the"Center-Team"structure can increase the attention of medical staff to rare diseases,strengthen the diagnostic capacity of rare diseases,and make patient management more continuous and disciplinary co-operation more efficient without significantly increasing the input burden on hospitals.

Objectives It was a preliminary exploration on rare disease management of comprehensive hospital based on the"Center-Multidisciplinary Team"structure.Methods By applying interrupted time series analysis,the characteristics and related medical service of rare disease patients discharged from sample hospitals from 2021 to 2023 were measured.Results The results showed that the hospital served 19 825 rare disease patients from 2021 to 2023.The proportion of patients whose main diagnosis were rare diseases was approximately 27％.The interrupted time series technique indicated that the expected proportion of rare disease patients increased instantly by 1.89 thousand points.The capability to diagnose has significantly improved,with a growing trend on volume of bioinformatics analysis services for rare diseases patients.Conclusion The rare disease management based on the"Center-Team"structure can increase the attention of medical staff to rare diseases,strengthen the diagnostic capacity of rare diseases,and make patient management more continuous and disciplinary co-operation more efficient without significantly increasing the input burden on hospitals.

Objectives It was a preliminary exploration on rare disease management of comprehensive hospital based on the"Center-Multidisciplinary Team"structure.Methods By applying interrupted time series analysis,the characteristics and related medical service of rare disease patients discharged from sample hospitals from 2021 to 2023 were measured.Results The results showed that the hospital served 19 825 rare disease patients from 2021 to 2023.The proportion of patients whose main diagnosis were rare diseases was approximately 27％.The interrupted time series technique indicated that the expected proportion of rare disease patients increased instantly by 1.89 thousand points.The capability to diagnose has significantly improved,with a growing trend on volume of bioinformatics analysis services for rare diseases patients.Conclusion The rare disease management based on the"Center-Team"structure can increase the attention of medical staff to rare diseases,strengthen the diagnostic capacity of rare diseases,and make patient management more continuous and disciplinary co-operation more efficient without significantly increasing the input burden on hospitals.

Objectives It was a preliminary exploration on rare disease management of comprehensive hospital based on the"Center-Multidisciplinary Team"structure.Methods By applying interrupted time series analysis,the characteristics and related medical service of rare disease patients discharged from sample hospitals from 2021 to 2023 were measured.Results The results showed that the hospital served 19 825 rare disease patients from 2021 to 2023.The proportion of patients whose main diagnosis were rare diseases was approximately 27％.The interrupted time series technique indicated that the expected proportion of rare disease patients increased instantly by 1.89 thousand points.The capability to diagnose has significantly improved,with a growing trend on volume of bioinformatics analysis services for rare diseases patients.Conclusion The rare disease management based on the"Center-Team"structure can increase the attention of medical staff to rare diseases,strengthen the diagnostic capacity of rare diseases,and make patient management more continuous and disciplinary co-operation more efficient without significantly increasing the input burden on hospitals.

Objectives It was a preliminary exploration on rare disease management of comprehensive hospital based on the"Center-Multidisciplinary Team"structure.Methods By applying interrupted time series analysis,the characteristics and related medical service of rare disease patients discharged from sample hospitals from 2021 to 2023 were measured.Results The results showed that the hospital served 19 825 rare disease patients from 2021 to 2023.The proportion of patients whose main diagnosis were rare diseases was approximately 27％.The interrupted time series technique indicated that the expected proportion of rare disease patients increased instantly by 1.89 thousand points.The capability to diagnose has significantly improved,with a growing trend on volume of bioinformatics analysis services for rare diseases patients.Conclusion The rare disease management based on the"Center-Team"structure can increase the attention of medical staff to rare diseases,strengthen the diagnostic capacity of rare diseases,and make patient management more continuous and disciplinary co-operation more efficient without significantly increasing the input burden on hospitals.

Objectives It was a preliminary exploration on rare disease management of comprehensive hospital based on the"Center-Multidisciplinary Team"structure.Methods By applying interrupted time series analysis,the characteristics and related medical service of rare disease patients discharged from sample hospitals from 2021 to 2023 were measured.Results The results showed that the hospital served 19 825 rare disease patients from 2021 to 2023.The proportion of patients whose main diagnosis were rare diseases was approximately 27％.The interrupted time series technique indicated that the expected proportion of rare disease patients increased instantly by 1.89 thousand points.The capability to diagnose has significantly improved,with a growing trend on volume of bioinformatics analysis services for rare diseases patients.Conclusion The rare disease management based on the"Center-Team"structure can increase the attention of medical staff to rare diseases,strengthen the diagnostic capacity of rare diseases,and make patient management more continuous and disciplinary co-operation more efficient without significantly increasing the input burden on hospitals.