To have better clinical understanding of Schimke immuno-osseous dysplasia(SIOD) through analyzing the clinical features, treatment, and prognosis of four patients with SIOD.

Mutations in the SAMD9 and SAMD9L genes are associated with MIRAGE syndrome and ATXPC syndrome, respectively. This study reports the clinical characteristics and genetic analysis of one case with SAMD9 mutation (c.3809T > A, p.F1270Y) and two cases with SAMD9L mutations (c.2675T > G, p.M892R; c.1096T > G, p.F366V and c.4517T > C, p.L1506p), none of which have been previously reported. All patients presented with recurrent infections, growth retardation, and myelodys-plasia, with varying degrees of involvement of multiple systems, including respiratory, gastrointestinal, immune, endocrine, neurological, and reproductive systems. In terms of treatment, two patients underwent regular intravenous immunoglobulin and their clinical symptoms improved, while one patient had a favorable recovery following hematopoietic stem cell transplantation at the age of 1 year and 5 months of age. SAMD9/SAMD9L gene mutations, therefore, chould be considered in children with recurrent infections, myelodysplasia, pancytopenia, and growth retardation. Early genetic testing is crucial for timely diagnosis and treatment, which may improve patient outcomes.

Purpose/Significance To study and develop an intervention plan for the utilization barriers of health information services for empty nest elderly,in order to provide a basis for improving their knowledge,attitude,and action abilities in health information serv-ices.Method/Process Selecting residents of Yan'an community in Bengbu as research subjects,who are randomly divided into a control group and an intervention group.The intervention group use a pre-constructed intervention plan.After the intervention study,the con-trol group is provided with the same health education content as the intervention group.Result/Conclusion The intervention programs can improve the knowledge level and behavioral ability of empty nest elderly in utilizing information services.

Purpose/Significance To understand the influencing factors of health information literacy of elderly discharged patients receiving continuous nursing services,and to provide theoretical basis for related health education and health promotion.Method/Process The selective quota sampling method is used to conduct a questionnaire survey on elderly discharged patients aged 60 and above receiving continuous nursing services in Bengbu city,and a structural equation model is constructed to analyze the effect pathway of influencing fac-tors.Result/Conclusion The level of health information literacy of the discharged elderly patients receiving continuous nursing services is average.Healthy lifestyle has direct influence on health information literacy of them,and social support and psychological status have in-direct influence on them through self-efficacy and healthy lifestyle.It is suggested that family members and medical staffs should pay positive attention to patients,help patients maintain a good psychological state,promote the recovery of diseases,and improve the level of health information literacy.

Objective:To investigate the differences between the mental clips placed intraoperatively and the tumor bed's target volume delineation of seroma based on CT scanning during radiotherapy for breast cancer patients who received breast-conserving surgery in the persuit of a better solution to determine the tumor bed position.Methods:The clinical data of 13 patients with early breast cancer who received postoperative radiotherapy after breast-conserving surgery at Beijing Shijingshan Hospital and Beijing Shijitan Hospital of Capital Medical University from December 2020 to January 2022 were retrospectively analyzed. They all had surgical clips implanted during the surgery. The following methods were used to delineate the target volume of tumor bed, including gross target volume delineation of tumor bed based on the mental clips (GTVtb-Clip), the tumor bed's gross target volume delineation of seroma based on CT scanning (GTVtb-Seroma), and the combination of both (GTVtb-C+S). The volume, diameter on three coordinate axis, neutral point displacement and conformability of these delineation methods were compared.Results:The volume of GTVtb-Clip, GTVtb-Seroma and GTVtb-C+S was (25±10) cm 3, (38±17) cm 3, (49±20) cm 3, and the differences were statistically significant (all P<0.05). The diameter on X axis was (4.7±1.2) cm, (5.3±1.4) cm, (5.7±1.6) cm, respectively in GTVtb-Clip, GTVtb-Seroma and GTVtb-C+S; the diameter on Y axis was (4.6±1.7) cm, (5.0±1.6) cm, (5.7±1.7) cm, respectively in GTVtb-Clip, GTVtb-Seroma and GTVtb-C+S; the diameter on Z axis was (4.4±1.5) cm, (5.2±1.4) cm, (5.6±1.4) cm in GTVtb-Clip, GTVtb-Seroma and GTVtb-C+S. The differences in the diameter of GTVtb-Clip and GTVtb-C+S on X,Y, Z axis were statistically significant (all P<0.05); the differences in the diameter of GTVtb-Seroma and GTVtb-C+S on X, Z axis were statistically significant (all P<0.05); the difference in the diameter of GTVtb-Clip and GTVtb-Seroma on X axis was statistically significant ( P<0.05) .Neutral point displacement was (5.8±1.6) cm, (5.5±1.9) cm, (6.0±1.7) cm, respectively of GTVtb-Clip, GTVtb-Seroma, GTVtb-C+S, and the difference was not statistically significant ( P>0.05). Conformability of GTVtb-Clip and GTVtb-Seroma, GTVtb-Clip and GTVtb-C+S, GTVtb-Seroma and GTVtb-C+S was 0.412±0.112, 0.525±0.095, 0.774±0.112,respectively, and the differences were statistically significant (all P<0.05). Conclusions:During radiotherapy after breast-conserving surgery for breast cancer, compared with the single method, the combination of GTVtb-Clip and GTVtb-Seroma can better cover the real tumor bed, thus reducing the omission of tumor bed and recurrence rate. CT position should better take place at 4 to 8 weeks for patients receiving radiotherapy after breast-conserving surgery, and target volume of tumor bed will be delineated based on the postoperative changes of both mental clips and seroma.

Breast cancer is one of the most malignant tumors and is associated with high mortality rates among women. Lycium barbarum polysaccharide (LBP) is an extract from the fruits of the traditional Chinese herb, L. barbarum. LBP is a promising anticancer drug, due to its high activity and low toxicity. Although it has anticancer properties, its mechanisms of action have not been fully established. Ferroptosis, which is a novel anticancer strategy, is a cell death mechanism that relies on iron-dependent lipid reactive oxygen species (ROS) accumulation. In this study, human breast cancer cells (Michigan Cancer Foundation-7 (MCF-7) and MD Anderson-Metastatic Breast-231 (MDA-MB-231)) were treated with LBP. LBP inhibited their viability and proliferation in association with high levels of ferroptosis. Therefore, we aimed to ascertain whether LBP reduced cell viability through ferroptosis. We found that the structure and function of mitochondria, lipid peroxidation, and expression of solute carrier family 7 member 11 (SLC7A11, also known as xCT, the light-chain subunit of cystine/glutamate antiporter system X_c^-) and glutathione peroxidase 4 (GPX4) were altered by LBP. Moreover, the ferroptosis inhibitor, Ferrostatin-1 (Fer-1), rescued LBP-induced ferroptosis-associated events including reduced cell viability and glutathione (GSH) production, accumulation of intracellular free divalent iron ions and malondialdehyde (MDA), and down-regulation of the expression of xCT and GPX4. Erastin (xCT inhibitor) and RSL3 (GPX4 inhibitor) inhibited the expression of xCT and GPX4, respectively, which was lower after the co-treatment of LBP with Erastin and RSL3. These results suggest that LBP effectively prevents breast cancer cell proliferation and promotes ferroptosis via the xCT/GPX4 pathway. Therefore, LBP exhibits novel anticancer properties by triggering ferroptosis, and may be a potential therapeutic option for breast cancer.
Breast Neoplasms/drug therapy* ; Drugs, Chinese Herbal/pharmacology* ; Female ; Ferroptosis ; Glutathione/metabolism* ; Humans ; Iron/metabolism*

【Objective】 To evaluate the efficacy and safety of human coagulation factor Ⅷ developed by Shenzhen Weiguang Biological products Co, Ltd in the treatment of patients with hemophilia A. 【Methods】 A prospective, multi-center, open, single-group clinical study was conducted. A total of 65 subjects with hemophilia A were enrolled, and human coagulation factor Ⅷ(FⅧ) was injected according to the patients’ bleeding severity. The improvement score of bleeding symptoms and signs after the first infusion of the first bleeding event and the transfusion efficiency of FⅧ activity at 10 min and 1 hour after infusion were taken as the main efficacy indexes. The improvement scores of bleeding symptoms and signs after the first infusion and the increase of FⅧ activity at 10 min and 1 hour after infusion were the secondary efficacy indexes. 【Results】 The 65 subjects were enrolled in safety analysis set (SS) and full analysis set (FAS), and 58 of them were enrolled in protocol analysis set (PPS). Ten minutes and one hour after the first infusion, the level of factor Ⅷ activity in the subjects increased significantly, and the FⅧ activity increased by 100% or more in more than 79% of the subjects. The average infusion efficiency of FⅧ activity in all subjects was more than 100%. In 70% of the subjects, the pain was relieved rapidly and /or the bleeding symptoms were significantly improved 8 hours after each bleeding infusion, and the improvement rate of bleeding symptoms and signs reached 100% 72 hours after infusion. 【Conclusion】 After infusion of human coagulation factor Ⅷ, the activity level of factor Ⅷ in patients with hemophilia A significantly increased. The infusion efficiency can reach a optimal level, and the bleeding symptoms can be significantly improved.

【Objective】 To investigate the main causes of blood donor deferral in domestic blood center. 【Methods】 The causes of donor deferral were classified into 12 categories as previous medical history, drug use, alcohol consumption, menstrual period, underweight, abnormal blood pressure, abnormal body temperature, abnormal hemoglobin (Hb), lipemic blood, positive hepatitis B surface antigen (HBsAg), elevated alanine aminotransferase (ALT) and others according to the comparison indicators of Asia-Pacific Blood Network (APBN) and the national standard Blood Donor Health Examination Requirements. The relevant data of the top 3 causes of donor deferral, voluntarily reported by the members of Practice Comparison Working Group of China’s Mainland Blood Collection and Supply Institutions from 2014 to 2019, were collected and a histogram was generated. 【Results】 The median donor deferral rate of 20 domestic blood centers from 2014 to 2019 was 12.14%, with the lowest at 0.18% and highest at 32.32%, respectively. The top three causes for donor deferral were elevated ALT, abnormal Hb and abnormal blood pressure in year 2014, 2015, 2018 and 2019; elevated ALT, lipemic blood and abnormal blood pressure in 2016; elevated ALT, abnormal Hb, and lipemic blood in 2017. 【Conclusion】 The main causes of donor deferral were elevated ALT, abnormal Hb, abnormal blood pressure and lipemic blood.

With the development of society, women’s attention to their external genitals is gradually increasing, genitalia plastic surgery has also achieved a dramatic of development.The labia majora, as one of the most obvious structures of female external genitalia, occupies an important part of external genital aesthetics. After reviewing the literature, we found that there are few reports on plastic surgery of labia majora, so we make a review on this aspect to discusse the anatomy of the labia majora, the treatments of atrophy and hypertrophy of labia majora, and the complications of these therapies.

With the development of society, women’s attention to their external genitals is gradually increasing, genitalia plastic surgery has also achieved a dramatic of development.The labia majora, as one of the most obvious structures of female external genitalia, occupies an important part of external genital aesthetics. After reviewing the literature, we found that there are few reports on plastic surgery of labia majora, so we make a review on this aspect to discusse the anatomy of the labia majora, the treatments of atrophy and hypertrophy of labia majora, and the complications of these therapies.