This article combs and summarizes the entire process of rare disease selection and priority theme determination,including the application and preliminary review of rare diseases,standardization of disease theme information,the evaluation methods of evidence sorting and disease selection for priority se-lection of disease themes,and other aspects of the content were analyzed in depth.It is expected to provide reference for the subsequent selection of rare diseases,improve the fairness,rationality and scientificity of rare disease selection,and further promote research and decision-making in China's rare disease-related fields.

【Objective】 To analyze the hepatitis B virus (HBV) infection data of blood donors from 18 domestic blood stations, so as to investigate the HBV infection situation of blood donors. 【Methods】 The positive rate of HBV and its distribution characteristics of regions, the percentage of HBsAg+ ELISA in first-time vs repeated blood donors, and the percentage of HBsAg-/HBV DNA+ blood donors of 18 domestic blood stations during 2017 to 2020 were collected from the Working Platform for Practice Comparison of Blood Centers, and the HBV infection among blood donors were statistically analyzed. 【Results】 From 2017 to 2020, the positive rate of HBV in blood donors among 18 domestic blood stations was 13.48/10 000-144.02/10 000, with the average HBV positive rate in eastern, central and western region at 26.14/10 000, 51.98/10 000 and 41.00/10 000, respectively. The HBsAg+ rate by ELISA among first-time and repeated blood donors was 14.55/10 000-305.39/10 000 vs 1.04/10 000-87.43/10 000 The HBsAg-/HBV DNA+ yield was 1.80/10 000-35.31/10 000. 【Conclusion】 The distribution of HBV infection in blood donors has regional characteristics, and HBV prevalence was low in repeated blood donors. HBsAg ELISA combined with HBV DNA detection can better ensure blood safety.

OBJECTIVE To determ ine the contents of main active constituents in Gynura divaricata leaf with different drying methods and at different harvesting time ，so as to confirm the best drying method and harvesting time. METHODS G. divaricata leaf with different drying methods [drying in the shade ，drying in the sun ，oven drying （60℃，70℃，80℃），microwave drying and freeze drying] and different harvesting time （March to October ）were prepared. The content of water-soluble extract was determined by hot dip method. The contents of total flavonoids and polysaccharides were determined by ultraviolet-visible spectrophotometry. The content of astragalin was determined by HPLC. Analytic hierarchy process was used for comprehensive analysis. RESULTS The time of drying in the shade ，drying in the sun ，drying at 60 ℃，drying at 70 ℃，drying at 80 ℃， microwave drying and freeze drying were 7 d，5 d，8 h，5 h，3.5 h，6 min and 1 d respectively. The average contents of water-soluble extract in G. divaricata leaf were 55.98％，60.78％，52.33％，49.54％，46.87％，59.70％ and 58.24％；those of total flavonoids were 3.27％，3.22％，1.99％，1.70％，1.31％，3.92％ and 2.28％；those of polysaccharides were 4.70％，6.09％， 6.48％，5.45％，5.74％，5.76％ and 7.15％；those of astragalin were 0.48％，0.46％，0.24％，0.23％，0.20％，0.48％，0.29％. The comprehensive score of microwave drying was the highest ，being 0.996 3. The average contents of water-soluble extract from March to October were 41.50％，40.57％，39.16％，40.65％，40.68％，43.30％，45.19％ and 40.12％；those of total flavonoids were 2.24％，2.81％，3.87％，3.92％，3.82％，3.93％，3.66％ and 3.25％；those of polysaccharides were 4.41％，4.61％， 4.98％，5.26％，5.75％，5.94％，5.32％ and 4.47％；those of astragalin were 0.20％，0.21％，0.25％，0.26％，0.25％，0.24％， 0.25％ and 0.21％，respectively. The comprehensive scores of samples collected from May to September exceeded 0.92，and the comprehensive score in August was the highest （0.988 6）. CONCLUSIONS Microwave-dried Gynura divaricata leaf has the best quality ，and the best harvesting time is from May to September.

Clinical phenotypes and gene characteristics of a patient diagnosed with Mowat-Wilson syndrome (MWS) with Hirschsprung′s disease (HSCR) and vaginal atresia in the Department of Neonatal Surgery, Beijing Children′s Hospital, Capital Medical University in March 2021 were analyzed retrospectively.The eight-month-old girl was admitted to the hospital with symptoms of constipation for nine days and abdominal distension for two days.Lower digestive tract radiography and rectal mucosa biopsy results suggested HSCR.The child also had specific facial features and motor development delay.Whole exome test showed a de novo heterozygous mutation, ZEB2 gene c. 2761C>T (p.R921*). After laparoscopic-assisted Soave procedure, the child had normal bowel movements, and no surgery-related compli-cations occurred during the follow-up period.The child′s motor development improved after rehabilitation treatment.According to literature review, 2 female cases show similar clinical manifestations to this girl, but the genotypes were different.This patient expands the clinical phenotype of ZEB2 gene pathogenicity.

Objective:To explore the effects of health education based on evidence-based practice using the knowledge to action model (KTA) on patients with osteoporosis and lumbar vertebral fractures.Methods:Totally 108 patients with osteoporosis and lumbar vertebral fractures admitted in the the Xingtai General Hospital of North China Medical and Health Group from October 2017 to October 2019 were selected and divided into an observation group and a control group according to the random number table, with 54 patients in each group. Patients in both groups received routine treatment and care. Patients in the control group received routine health education, while patients in the observation group received health education received health education based on evidence-based practice using KTA. The rehabilitation status [Oswestry dysfunction index (ODI) , Barthel index] between the two groups of patients, as well as the knowledge level (osteoporosis knowledge questionnaire) , living habits and quality of life [The World Health Organization Quality of Life Brief Version (WHOQOL-BREF) ] before and after the intervention were compared.Results:After 3 months of intervention, the ODI of the two groups decreased while the Barthel index increased ( P< 0.05) ; and the ODI of the observation group was lower than that of the control group, while the Barthel index score was higher than that of the control group, with statistically significant differences ( P< 0.05) . After 3 months of intervention, the risk factors, exercise knowledge, calcium knowledge scores and total scores of the osteoporosis knowledge questionnaire in the two groups increased, with statistically significant differences ( P< 0.05) , which were higher in the observation group than in the control group, with statistically significant differences ( P< 0.05) . After 3 months of intervention, the ratio of smoking and drinking in the observation group was lower than that in the control group, and the ratio of exercise and calcium supplementation was higher than that in the control group, with statistically significant differences ( P< 0.05) . After 3 months of intervention, the scores in all dimensions of the WHOQOL-BREF of the two groups increased ( P< 0.05) , and the scores in the physical, psychological and social dimensions of the observation group were higher than those of the control group, with statistically significant differences ( P< 0.05) . Conclusions:The health education based on evidence-based practice using KTA in patients with osteoporosis and lumbar vertebral fractures helps to improve patients' related knowledge levels, living habits, rehabilitation effects, and quality of life.

Objective In a new hospital,the construction of the ethics committee is also gradually evolving.This article aims to describe the current status of the ethics committee,analyze and summarize identified problems in the operation of the ethics committee,to propose a solution for better construction of ethics committee.At the same time,it can also provide certain reference for the new establishment of Ethics Committee of other institutions.Methods This paper analyzes the construction and current situation of operation of the ethics committee in detail.Results The ethics committee is running well,but there are still some problems such as management mode to be further optimized and ethical review system to be further strengthened.Conclusions The construction and capacity building of the EC is a continuing effort.On-going improvement of identified problems plays significant role in better construction of the ethics committee.

Hypertrophic scar is a pathological lesion during the repair of skin wound, involving fibroblast, extracellular matrix and cytokines. The prevention and treatment of hypertrophic scar has been a clinical problem, but its exact mechanism is still unclear. The renin angiotensin system (RAS) in the skin is proved to directly involve in wound healing and hypertrophic scar formation. After local RAS activating during wound repair, the expression of angiotensinogen Ⅱ (Ang Ⅱ) and angiotensin converting enzyme (ACE) have increased. They can lead to pathological scarring by stimulating the proliferation of fibroblasts in the skin, affecting collagen metabolism, promoting skin fibrosis and angiogenesis. This review will focus on the role of Ang Ⅱ and ACE in hypertrophic scar formation, which will help to further understand the mechanism of scar formation, and summarize the potential application of RAS antagonists in hypertrophic scar prevention and treatment, so as to provide a new direction for the prevention and treatment of hypertrophic scar.

Hypertrophic scar is a pathological lesion during the repair of skin wound, involving fibroblast, extracellular matrix and cytokines. The prevention and treatment of hypertrophic scar has been a clinical problem, but its exact mechanism is still unclear. The renin angiotensin system (RAS) in the skin is proved to directly involve in wound healing and hypertrophic scar formation. After local RAS activating during wound repair, the expression of angiotensinogen Ⅱ (Ang Ⅱ) and angiotensin converting enzyme (ACE) have increased. They can lead to pathological scarring by stimulating the proliferation of fibroblasts in the skin, affecting collagen metabolism, promoting skin fibrosis and angiogenesis. This review will focus on the role of Ang Ⅱ and ACE in hypertrophic scar formation, which will help to further understand the mechanism of scar formation, and summarize the potential application of RAS antagonists in hypertrophic scar prevention and treatment, so as to provide a new direction for the prevention and treatment of hypertrophic scar.

Asthma and chronic obstructive pulmonary disease overlap (ACO) is a hot topic in recent years. Although the precise definition of ACO is still controversial, scholars have found that the patients with overlapping features of asthma and COPD have a worse prognosis and increased medical cost than those with asthma or COPD alone. Thus, there is a big challenge to have a better understanding and to manage these patients in clinical practice. To strengthen awareness of this type of patients, the Global initiative for Chronic Obstructive Lung Disease (GOLD) and Global initiative for asthma (GINA) in 2014 named them as " Asthma Chronic Obstructive Lung Overlap Syndrome" (ACOS), which was further revised to the Asthma Chronic Obstructive Lung Overlap (ACO) in the follow-up GINA guidelines (2017). In the updated version, it emphasized that ACO was not a separate disease. However, although the research on ACO has increased significantly in recent years, there are still many controversies, which includes the definition, diagnostic criteria and treatment therapies. In this review, we provide the recent advances in the diagnosis and an overview of available treatment for of ACO.

Objective To explore the dynamic change and clinical signiticance of plasma D-damer level in patients with cerebral hemorrhage and acute craniocerebral injury.Methods 50 patients with cerebral hemorrhage and 40 patients with acute craniocerebral injury were selected,The enzyme-linked immunosorbent assay (ELISA) was used to measure plasma D-dimer level in two groups of patients after onset,and the results were compared with 40 healthy controls.Results The levels of plasma D-dimer in the patients with cerebral hemorrhage were 1.59mg/L,2.10mg/L,1.03 mg/L,0.82mg/L at 3 h,6h,12h,2d after onset,which in the patients with acute craniocerebral injury were 1.61mg/L,2.02mg/L,1.01mg/L and 0.67mg/L,respectively.And the plasma D-dimer levels were 0.50mg/L,0.49mg/L,0.47mg/L,0.48mg/L in the control group at 3h,6h,12h and 2d after onset.The levels of plasma D-dimer in the patients with acute craniocerebral injury were significantly higher than those in the control group,and the differences were statistically significant (t =9.35,12.17,4.03,3.05,all P ＜ O.05).At 7d after onset,the D-dimer levels in the cerebral hemorrhage group and acute craniocerebral injury group were 0.53mg/L,0.55mg/L,respectively,which of the control group was 0.47mg/L,there was no statistically significant difference among the three groups(P ＞ 0.05).Conclusion Cerebral hemorrhage patients and acute craniocerebral injury patients have high coagulation and fibrinolytic activity in brief increase trend,dynamic observation of plasma D-dimer level in patients with cerebral hemorrhage and acute craniocerebral injury is helpful to determine courses,condition and evaluate prognosis.