Objective:To comprehensively understand the disease burden of liver cirrhosis and other chronic liver diseases caused by alcohol use in China from 1990 to 2019, as well as to predict the trends in disease burden from 2020 to 2030.Methods:The analysis utilized data from the Global Burden of Disease study in 2019 (GBD2019). Key indicators such as incidence rate, mortality rate, disability-adjusted life years (DALY), years of life lost due to premature mortality, and years lived with disability were selected to describe the disease burden of alcohol-related liver cirrhosis and other chronic liver diseases in China from 1990 to 2019. The estimated annual percentage change (EAPC) was used to depict the temporal trends in disease burden. Furthermore, a Bayesian age-period-cohort (BAPC) model was constructed using R software to predict the age-standardized incidence rate (ASIR) and age-standardized mortality rate (ASMR) of alcohol-related liver cirrhosis and other chronic liver diseases in China from 2020 to 2030.Results:From 1990 to 2019, the incidence of alcohol-related liver cirrhosis and other chronic liver diseases in China showed an upward trend, with an EAPC of 0.31% (95% CI: 0.10%-0.52%). However, the DALY declined, with an EAPC of -2.81% (95% CI: -2.92% - -2.70%). The ASMR showed a downward trend, with an EAPC of -2.55% (95% CI: -2.66% - -2.45%). The highest incidence of cirrhosis of liver caused by alcohol and other chronic liver diseases was reported in the age group of 35-49 years, while the ASMR increased gradually with age, with a significant rise after the age of 30. The age-standardized DALY rate peaked between the ages of 55 and 64. The disease burden indicators for males were consistently higher than those for females during the same period. According to the predictions of the BAPC model, from 2020 to 2030, the ASIR for cirrhosis of liver caused by alcohol and other chronic liver diseases in the entire population of China was projected to increase from 3.45/100 000 in 2020 to 3.78/100 000 in 2030, a growth of 9.57%. Conversely, the ASMR was expected to decrease from 1.45/100 000 in 2020 to 1.24/100 000 in 2030, a reduction of 14.48%. Conclusions:The disease burden of cirrhosis of liver caused by alcohol and other chronic liver diseases remained serious in China, especially in men and the middle-aged to elderly population. There is a pressing need to prioritize attention and resources towards these groups. Despite the projected decrease in ASMR, the ASIR continued to rise and is expected to persist in its upward trend until 2030.

Objective:To explore the genetic basis and clinical phenotype of a Chinese pedigree affected with Focal segmental glomerulosclerosis (FSGS).Methods:A male patient who was admitted to the First Affiliated Hospital of Zhengzhou University on July 26, 2018 was selected as the study subject. Clinical data of the patient was collected. Next generation sequencing and Sanger sequencing were carried out to detect the variant sites. Bioinformatic software was used to simulate the effect of candidate variants on the protein functions.Results:Ultrasound exam of the patient showed enhanced echo for the renal parenchyma. Kidney biopsy had confirmed the pathological diagnosis of FSGS (non-specific). Electronic microscopy displayed segmental sclerosis of the glomeruli, mild hyperplasia of mesangial cells and matrix. The proband was found to harbor two novel variants of the PLCE1 gene, namely c. 3199delA (p.N1067Mfs*15) and c.4441_4443delATC (p.1481_1481del), which were respectively inherited from his mother and father. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), both variants were rated as pathogenic (PVS1+ PM2_Supporting+ PP3; PM2_Supporting+ PM3+ PP3). Bioinformatic simulation suggested that both variants could significantly affect the tertiary structure of the PLCE1 protein. Conclusion:The c. 4441_4443delATC and c. 3199delA variants of the PLCE1 gene probably underlay the pathogenesis of the FSGS in this pedigree.

Acute disseminated encephalomyelitis（ADEM）is a common inflammatory demyelinating disease of the central nervous system in children.The etiology is not yet clear，but it is currently mainly believed to be related to the activation of autoimmune reactions following infection.ADEM is typically a monophasic disease，unlike other demyelinating diseases such as multiple sclerosis and neuromyelitis optica spectrum disorders.With the increasing recognition，understanding，and detection of myelin oligodendrocyte glycoprotein antibodies in recent years，the diagnosis and treatment of ADEM may further evolve.The diagnosis of ADEM is clinical and requires exclusion of other infectious，inflammatory，neoplastic，and genetic conditions.The acute phase treatment methods include intravenous injection of high-dose corticosteroids，therapeutic plasma exchange，and intravenous immunoglobulin，with a generally good prognosis.This review summarizes the current knowledge on the pathogenesis，epidemiology，clinical features，diagnosis and differentiation，treatment，and prognosis of ADEM in children.

Through the reform and practice of the "four-in-one" cultivation model of clinical medical talents, the training mode of applied high-quality medical and health talents with different orientations is established, and the curriculum teaching system suitable for the development of modern medical education is constructed to promote the reform of education and teaching. It solves the problems of single medical talents training mode, fragmentation of curriculum system, single teaching and training in teaching, and insufficient support for talent training. This reform established a set of curriculum system, teaching methods and evaluation methods to strengthen the cultivation of medical students' post competency, and achieved the goal of improving the quality of clinical medical personnel training in local medical colleges.

Objective:To investigate the application effect of the best evidence for prevention of perioperative delirium in elderly patients with hip fracture.Methods:Using the convenient sampling method, a total of 62 elderly patients with hip fractures admitted to the Trauma Department of Provincial Hospital Affiliated to Shandong First Medical University from January to August 2021 were selected as the control group, and 62 elderly patients with hip fractures admitted from September 2021 to April 2022 were selected as the observation group. At the same time, 17 nurses from the same department were selected as the research objects. According to the standard procedures and clinical conversion system application methods of the Evidence Based Clinical Evidence Practice System at the Joanna Briggs Institute in Australia, the best evidences were applied to the prevention of delirium in elderly hip fracture patients during the perioperative period. Before and after the application of evidence, the compliance rates of the review indicators, the scores of knowledge and attitude on prevention of delirium in elderly patients with hip fracture and the occurrence of delirium in patients were compared.Results:After the application of evidence, the compliance rates of review indicators were higher than those before the application, and the differences were statistically significant ( P < 0.05). The knowledge accuracy and attitude scores of delirium prevention in elderly nurses with hip fractures during perioperative period were higher than those before application, and the differences were statistically significant ( P<0.05). The number of delirium cases in the observation group was lower than that in the control group, and the duration of delirium was shorter than that in the control group, and the differences were statistically significant ( P<0.05) . Conclusions:The clinical application of the best evidence for perioperative delirium prevention in elderly hip fractures can improve nurses' awareness and attitude towards perioperative delirium prevention in elderly hip fractures, and reduce the occurrence of delirium in patients.

Objective:To explore the effect of the Internet combined pre-rehabilitation intervention model in patients with chronic hepatitis B liver cirrhosis.Methods:From June 2019 to December 2020, convenience sampling was used to select 140 patients with chronic hepatitis liver cirrhosis in Henan Provincial People's Hospital. The patients were randomly divided into the observation group and the control group according to the order of entry, 70 cases in each group.The observation group carried out the Internet combined pre-rehabilitation model and the control group implemented conventional nursing intervention. We compared the disappearance time of clinical symptoms, mastery of rehabilitation knowledge and the quality of life of the two groups of patients.Results:The disappearance time of abdominal distension, ascites, anorexia, fatigue, and lower extremity edema of the observation group was shorter than that of the control group, and the differences were statistically significant ( P<0.05) .After the intervention, the disease knowledge such as reasonable work and rest, reasonable diet, emotional control, exercise and medical treatment of observation group was better than that of the control group, and the differences were statistically significant ( P<0.05) . After the intervention, the quality of life scores of patients in the observation group were higher than those in the control group, and the differences were statistically significant ( P<0.05) . Conclusions:The Internet combined pre-rehabilitation intervention model can shorten the disappearance time of clinical symptoms in patients with chronic hepatitis B liver cirrhosis, improve the patient's knowledge of disease rehabilitation and the quality of life, which is worthy of clinical application.

Objective:To explore the current situation of work stress and job burnout of nursing staff in the Department of Infectious Diseases, and analyze its influencing factors.Methods:From January to June 2020, 63 nurses from the Department of Infectious Diseases of Zhejiang Hospital and the Affiliated Hospital of Chengde Medical University were selected as research subjects. Questionnaires were used to investigate general information, work stress, and job burnout.Results:63 questionnaires were issued and 60 were recovered. There was a statistically significant difference in work stress scores of nurses with different working hours, daily number of patients admitted, public recognition and respect, and training times ( P<0.05) . There were statistically significant differences in the scores of the personal accomplishment dimension of nurses with or without administrative positions and different professional titles, and in the scores of the depersonalization tendency dimension of nurses with different education levels, public recognition and respect, and training times, and in the scores of the emotional exhaustion dimension of nurses with different working hours, daily number of patients admitted, public recognition and respect, and training times ( P<0.05) . Conclusions:Nursing staff in the Department of Infectious Diseases are under high work stress, overall burnout is mild, and the personal sense of accomplishment of the nursing staff is low. The main reasons for this are closely related to the daily number of patients admitted, public recognition and respect, and the training times.

Objective:To evaluate the treatment efficacy of children with T-cell acute lymphoblastic leukemia (T-ALL) and to explore the prognostic risk factors.Methods:The clinical and laboratory data of children with newly diagnosed T-ALL in Children's Hospital of Fudan University and Children's Hospital of Shanghai from January 2002 to December 2014 were retrospectively analyzed and compared with children with newly diagnosed B-cell acute lymphoblastic leukemia (B-ALL) in the same period. The treatment protocols were based on the combination of the Berlin-Frankfurt-Münster (BFM)-ALL regimen with chemotherapy. The treatment response and infection of the children were observed. Cox proportional hazard regression model single-factor and multifactor analysis were used to evaluate the prognostic factors.Results:Seventy-one children with T-ALL and 333 children with B-ALL were enrolled. The clinical features including gender, age, central nervous system leukemia as well as the white blood cell count at first diagnosis were significantly different between the two groups (all P < 0.05). The prednisone good response rates of children with T-ALL were lower than that of B-ALL [78.9% (56/71) vs. 93.4% (311/333), P < 0.01], and the complete remission rates were lower than that of [94.4% (67/71) vs. 99.1% (330/333), P= 0.023]. By the end of follow-up, the relapse rates of children with T-ALL and B-ALL were 20.9% (14/67) and 16.4% (54/330) ( P= 0.369). The children with T-ALL had a shorter time to relapse compared with children with B-ALL [64.3% (9/14) vs. 35.2% (19/54), P= 0.049]. The 5-year overall survival (OS) rates of children with T-ALL and B-ALL were (62.1±6.4)% and (81.3±2.4)% (P < 0.05), and the 5-year event free survival (EFS) rates were (61.0±6.3)% and (71.0±2.7)% (P < 0.05). There was no significant difference in OS and EFS among pro/pre T-ALL, cortical T-ALL and mature T-ALL (both P > 0.05). The difference of EFS curves between children with early T-precursor (ETP)-ALL and non-ETP ALL was statistically significant ( P= 0.044). The most common infection site was respiratory tract [63.9% (186/291)], and the gram-negative bacteria accounted for 43.5% (20/46). Cox univariate analysis showed that prednisone poor response, bone marrow non-remission on day 33 of induction-therapy, relapse and sepsis were prognostic risk factors for children with T-ALL (all P < 0.05), and Cox multivariate analysis showed that the latter three were independent prognostic risk factors (all P < 0.05). Conclusions:The prognosis of children with T-ALL is worse than children with B-ALL, and T-ALL patients are prone to early relapse. The EFS of children with ETP-ALL is poor. Non-remission at the end of induction-therapy, relapse and sepsis are independent risk factors for prognosis.

Objective: To evaluate the expression of p-AKT and p-mTOR, the key proteins in PI3K/AKT/mTOR pathway in pediatric Burkitt lymphoma (BL), and to investigate the clinical and prognostic significance. Methods: Fifty-eight cases of pediatric BL and thirty cases of reactive hyperplastic lymphadenitis (RH) were collected at Children′s Hospital of Fudan University from September 2011 to July 2018. Paraffin sections of tissues were immune stained for p-AKT and p-mTOR, and the expression was assessed and correlated with the clinical features and prognosis. Results: A total of 58 cases were diagnosed and 6 cases lost the follow-up. Of the remaining 52 BL patients including 43 males and 9 females, the median age was 5 years (range: 2 to 14 years). Regarding to the correlation between the two biomarkers, Spearman test showed that p-mTOR was positively associated with the expression of p-AKT (r=0.759, P<0.001). Of all BL patients, the positive rates of p-AKT and p-mTOR were 62.1% (36/58) and 60.3%(35/58) respectively, both significantly higher than control group (P=0.011, P=0.035 respectively). The presence of p-AKT was significantly associated with higher lactate dehydrogenase (LDH≥573 IU/L) level in patients of the disease (P=0.006), while p-mTOR was increased both in the higher LDH and lower ratio of albumin to globulin (A/G) group (P=0.006, P=0.034 respectively). Expression of p-AKT and p-mTOR did not show any statistical correlation with sex, age, St.jude stage, tumor size, B-symptom present or not, number of extra-nodal sites or international prognostic index (IPI) (P>0.05). Fifty-two patients had a median follow-up of 40 months (range: 5-87 months). Univariate analysis showed that p-AKT expression was significant in predicting both inferior OS (5-year estimate, 72.7% vs. 94.7%, χ²=4.123, P=0.042) and PFS (5-year estimate, 66.7% vs. 94.7%, χ²=5.822, P=0.016). The 5-year OS rate was 71.0% (22/31) for the p-mTOR positive cohort of patients compared to 95.2% (17/21) for p-mTOR negative group (χ²=4.881, P=0.027); however, there was no statistical significance in 5-year PFS rate (P>0.05). Especially, the 5-year OS and PFS rate of p-AKT/p-mTOR double-positive group were significantly lower than negative control group (including absence of single p-AKT or p-mTOR expression, and absence of both) (OS: 69.0% vs. 95.7%, χ²=6.285, P=0.012; PFS: 65.5% vs. 91.3%, χ²=5.405, P=0.020). The results of multivariate COX proportional risk regression analysis indicated that p-AKT/p-mTOR double-positive, higher LDH and IPI score 3-5 were independent prognostic factors for both OS and PFS, and the bulky tumor (>10 cm) for PFS of pediatric BL. Conclusion The expression of p-AKT and p-mTOR may be a potential reference for diagnosis and the independent prognostic indicators of pediatric BL.

Objective:To explore the effect of project improvement on improving early out-of-bed activity of patients after total knee arthroplasty (TKA) .Methods:From June 2018 to March 2019, a project improvement team was established in Department of Joint Surgery of a ClassⅢ Grade A hospital in Shandong province. The 56 patients undergoing TKA before the implementation of the project improvement were set as the pre-improvement group, and 58 patients undergoing TKA after the implementation of the project improvement were set as the post-improvement group. Based on the analysis of project improvement methods, the improvement was made from the following aspects, including formulating and implementing the standardized plan of early postoperative out-of-bed activity plan for TKA patients, perfecting management strategy of cluster pain and perfecting the implementation standard of drainage tube management in the department. The incidence of postoperative complications, knee joint function recovery and postoperative hospital stay were compared between the two groups.Results:After the implementation of project improvement, out-of-bed activity time for the first time was shortened to (21.49±4.94) h, the time for postoperative straight leg elevation was shortened to (32.10±11.48) h, time for 90°knee flexion was shortened to (23.08±5.93) h, and the postoperative hospital stay was shortened to (4.12±1.03) d. The differences were statistically significant ( t=-26.06, -5.37, -6.88, -10.37; P<0.01) . There was no statistically significant difference in the incidence of incision bleeding before and after the implementation of the project improvement ( P>0.05) . The incidence of postoperative constipation decreased from 17.9% (10/56) before improvement to 3.4% (2/58) , and the postoperative erection intolerance increased from 5.4% (3/56) before improvement to 20.7% (12/58) . The differences were statistically significant (χ 2=6.28, 4.36; P<0.05) . Conclusions:The implementation of the project improvement can shorten early out-of-bed activity of patients after TKA, reduce the incidence of postoperative constipation, accelerate the rehabilitation process of knee function, and shorten the postoperative hospital stay.