Hereditary hemochromatosis is a rare autosomal genetic disorder that can cause multi-organ dysfunction in the liver,pancreas,spleen,heart and pituitary gland,with diverse clinical manifestations,make the diagnosis difficult.In recent years,with the deepening of clinical understanding and the development of genetic diagnosis tools,the diagnostic rate of this disease has increased significantly.In this paper,we report a case of hereditary hemochromatosis type 3 involving multiple organs and complicated by severe heart failure,aiming to improve the clinicians'understanding of this disease and reduce the leakage and misdiagnosis.

Objective:To explore the predictive value of multi-slice spiral CT (MSCT) arterial enhancement fraction (AEF) in the risk degree of gastrointestinal stromal tumors (GIST).Methods:The clinical data of 178 patients with GIST in Shanxi Province Cancer Hospital from January 2013 to May 2021 were retrospectively analyzed. According to tumor size, primary site, mitotic figure and whether the tumor was ruptured or not, the patients were divided into extremely low risk group (24 cases), low risk group (44 cases), medium risk group (48 cases), and high risk group (62 cases). One-way ANOVA was used to compare MSCT AEF value and conventional plain and enhanced scan values among the 4 groups. The receiver operating characteristics (ROC) curve was drawn to assess the predictive efficacy of AEF value, conventional plain value, enhanced scan value, Ki-67 and AEF value combined with Ki-67 for high-risk GIST.Results:Except for CT value in the venous phase and CT value in added arterial phase, the comparison of AEF value, plain scan CT value, arterial phase CT value, and added venous phase CT value of GIST patients in the 4 groups showed statistically significant differences (all P < 0.05). In the predictive efficacy evaluation of high-risk GIST, the area under the curve (AUC) of the ROC curve for AEF value was 0.753, which was higher than that for plain scan CT value (0.593), arterial phase CT value (0.592) and added venous phase CT value (0.631), lower than AEF combined with Ki-67 (0.799). Kappa consistency test showed that the AEF value, plain scan CT value and arterial phase CT value were consistent with the pathological examination results (Kappa values were 0.375, 0.168 and 0.168, respectively), however, the added venous phase CT value was inconsistent with the pathological examination results (Kappa = -0.224). The AUC of AEF value combined with Ki-67 for predicting high-risk GIST was 0.799, and the Kappar value was 0.528. Conclusions:MSCT AEF value varies in GIST with different risk degree, and its preoperative efficacy in predicting high-risk GIST is superior to that of conventional plain scan and enhanced scan; especially the combination of AEF value with Ki-67 shows a higher predictive efficacy.

OBJECTIVE To explore the implementation strategy of China ’s drug patent linkage system from the perspective of the system design ，in order to encourage drug innovation and improve the development level of high -quality generic drugs . METHODS The current progress and challenges were summarized from the current situation of the implementation of China ’s drug patent linkage system by sorting out the relevant policy documents of the system . On this basis ，the successful experience and lessons of foreign countries were summarized to provide strategic suggestions for the implementation of China ’s drug patent linkage system in combination with China ’s basic national conditions . RESULTS& CONCLUSIONS With the introduction of the revised Patent Law of the People ’s Republic of China in 2020，China had basically completed the top -level design of the system ，but some rules and regulations had not been improved . There were still some problems ，such as the lack of a “fake infringement ”system in China，the lack of review and correction mechanisms for patent information registration ，poor links between judicial and administrative channels ，the dispute over the length of waiting period ，and the lack of clarity of relevant rules for the exclusivity period system of the first generic drug market . It is recommended that China establish a “fake infringement ”system and a judicial decision procedure for patent invalidation ，adjust the length of the waiting period or the basis for market approval ，further clarify the definition of the first generic drug ，the circumstances of jointly challenging patents and the reasons for the elimination of the market exclusivity period ，and strengthen the functional link procedures of relevant departments and implement the responsibility mechanism.

Objective:To observe the effect of gait training assisted by an end-effector-based robot on the gait, balance, walking and general lower limb motor functioning of stroke survivors.Methods:Thirty-nine stroke survivors were randomized into a training group ( n=21) and a control group ( n=18). In addition to routine hemiplegia rehabilitation, the control group was given medium frequency electrical stimulation while the training group underwent 20 minutes of gait training assisted by an end-effector-based robot six days a week for 4 weeks. Before and after the intervention, both groups were evaluated using the functional ambulatory categories (FAC), the Fugl-Meyer lower extremity assessment, the Wisconsin Gait Scale and the Berg balance scale (BBS). Results:There were no significant differences between the groups before the intervention. Afterward significant improvement was observed in both groups in all measurements, with the training group scored better in terms of FAC and BBS.Conclusion:Using an end-effector-based robot in gait training might be more advantageous for improving the gait, balance, walking and general lower limb functioning of stroke survivors.

Acute lung injury/acute respiratory distress syndrome (ALI/ARDS) is an inflammatory lung injury induced by a variety of factors, and these diseases are associated with high rates of mortality due to the lack of effective treatments. Based on the latest research in ALI/ARDS, it is widely accepted that generalized inflammatory responses play a critical role in initiating and developing process of ALI/ARDS. We make a brief review on the immune-pathogenesis and the signaling pathways of ALI/ARDS from the perspective of inflammation, thereby helping develop novel therapeutic strategy for treatment of patients with ALI/ARDS.

Objective To investigate the relationship between inflammatory bowel disease (IBD) activity and choroidal thickness,and evaluate the utility of a choroidal thickness measurement in assessing IBD activity.Methods A total of 100 eyes of 50 patients of IBD with different disease activity,including 23 patients of ulcerative colitis,27 patients of Crohn's disease (CD).Ninety-six eyes of 48 healthy volunteers were recruited as control group.Choroidal thickness was measured using enhanced depth imaging(EDI)optical coherence tomography.Results Compared with the subfoveal choroidal thickness (294.37 ± 35.04) μm in healthy volunteers,the subfoveal choroidal thickness (349.28 ± 76.57) μm in UC patients with severe disease activity,the subfoveal choroidal thickness (326.71 ± 59.71) μm and (354.24 ± 66.34) μm,respectively,in CD patients with moderate and severe disease activity were found to be increased significantly (all P ＜ 0.05).Conclusion Choroidal thickness should be considered as a potential marker to assess the disease activity in patients with IBD,especially in patients with CD.

Objective To evaluate the effect of ischemic postconditioning on myocardial injury in the patients undergoing cardiac valve replacement under cardiopulmonary bypass (CPB).Methods Thirty patients of both sexes,aged 21-59 yr,of American Society of Anesthesiologists physical status Ⅱ or Ⅲ (New York Heart Association Ⅱ or Ⅲ),with left ventricular ejection fraction ≥ 40％,scheduled for elective cardiac valve replacement under CPB,were divided into 2 groups (n=15 each) using a random number table:control group (C group) and ischemic postconditioning group (P group).In group P,ischemic postconditioning was induced by 3 cycles of 30 s aortic unclamping followed by 30 s cross-clamping starting from 5 min before complete opening of the ascending aorta.After induction of anesthesia and before operation (T1),at 1 h after opening of the ascending aorta (T2),at the end of operation (T3),and at 12,24 and 48 h after opening of the ascending aorta (T4-6),blood samples were taken from the radial artery for determination of plasma concentrations of heart-type fatty acid-binding protein (H-FABP),ischemia-modified albumin (IMA),and cardiac troponin Ⅰ (cTnI).Myocardial specimens in the right auricle were obtained at T2,and the pathological changes were examined using an optical microscope.Results The concentrations of plasma cTnI at T2-6 and H-FABP and IMA at T2-5 were significantly higher than those at T1 in the two groups (P＜0.05).Compared with group C,the concentrations of plasma cTnI at T2-6,H-FABP at T2-4,and IMA at T2,3 were significantly decreased (P＜0.05),and the pathological changes of myocardial tissues were significantly reduced in group P.Conclusion Ischemic postconditioning can reduce myocardial injury in the patients undergoing cardiac valve replacement under CPB.

Objective To investigate the changes of hypothalamic-pituitary-adrenal axis (HPA)function, volume of the hypothalamic, pituitary and adrenal glands in elderly patients with depression. Methods Twenty-five cases of elderly patients with depression and 19 healthy elderly patients were enrolled into the patient group and the healthy control group, respectively. Enzyme linked immunosorbent assay was used to measure the level of the main hormone related with HPA axis in blood. The low dose dexamethasone suppression test was used to assessassessment the negative feedback regulation of HPA axis. The volume of the hypothalamus, pituitary and adrenal glands were determined by the magnetic resonance imaging. Results The Beck Depression Scale (BDI-II) and Montgomerie Rating Scale (MADRs) score of the elderly depression patients were significantly higher than those in the healthy control group (P < 0.01). The values of main hormone related with HPA axis in the patient group, such as CRH, ACTH and CORT, were higher than those in the healthy control group (P<0.01). The non- suppression rate of low dose dexamethasone suppression test in the patient group was significantly higher than that in the healthy control group (P < 0.05). The hypothalamus, pituitary and adrenal gland volume of patients group were 4.7%, 18.3% and 17.5% ,respectively. Significant the differences of pituitary and adrenal volume were observed between two groups (P < 0.05). Conclusion The levels of main hormone related with HPA axis in the elderly patients with depression were significantly increased, the non-suppression rate of low dose dexamethasone suppression test was also increased. Meanwhile, the volume of pituitary and adrenal gland in the elderly patients with depression increased.

Objective To explore and evaluate the effects of participatory teaching approach in the teaching of medical research methods. Methods The students of clinical medicine of Grade 2007 who took the elective course of medical scientific research method were taken as traditional teaching group, taught by teachers only, while the students of clinical medicine of Grade 2008 and 2009 as participatory teaching group, adopting the way of teachers' lectures and students' participation. At the end of the course, the questionnalre survey method combining interviews was used to investigate the teaching effectiveness, using descriptive analysis and chi-square test to compare the effectiveness between the traditional teaching methods and the participatory teaching methods. Results Compared with the traditional 'teachers centered' method, the 'participatory teaching approach' had better teach-ing results. Through the study of this course, 54 students (42.9%) learned how to conduct scientific research topics, 44 students (34.9%) mastered the design of the questionnalre, 59 students (46.8%) enhanced their ability of data analysis. Students' learning motivation and satisfaction were higher, and 117 student's participation (92.8%) were satisfied with the curriculum setting. Conclusion In medical research method teaching, the effect of participatory teaching method teaching is superior to the tradi-tional teaching method, and students' motivation and satisfaction are higher.

BACKGROUND:Gene-modified stem cel s can increase the secretion of peptides and ful-length proteins to protect spinal cord injury and promote recovery of neuronal function, which thus become a research hotspot in recent years. OBJECTIVE:To investigate the effect of nerve growth factor-modified adipose derived stem cel s in repairing spinal cord injury in rats. METHODS:Adipose derived stem cel s were primarily cultured by adherent culture method and cel surface markers were detected by immunofluorescence method, while spinal cord injury models were set by modified Al en method. Nerve growth factor plasmid was transfected into adipose derived stem cel s with Lipofectamine2000 and the expression of nerve growth factor was detected by real-time PCR and western blot. The modified adipose derived stem cel s intervened by nerve growth factor were injected into the injured part of spinal cord injury rat models. Basso-Beattie-Bresnahan score was used to evaluate the repairing effect. Models rats were sacrificed at 3 weeks after cel transplantation. Real-time PCR and western blot were used to testify nerve growth factor expression in the injured spinal segment after cel transplantation. RESULTS AND CONCLUSION:Adipose derived stem cel s were successful y cultured primarily, and positive for CD29 and CD44;the mRNA and protein expression of nerve growth factor was elevated after plasmid transfection. The Basso-Beattie-Bresnahan score was elevated after transplantation of adipose derived stem cel s intervened by nerve growth factor modification compared to control group;and the expression of nerve growth factor in the injured segment of the spinal cord was up-regulated detected by real-time PCR and western blot. These findings indicate that the nerve growth factor-modified adipose derived stem cel s have repairing effects on spinal cord injury in rats.