This study reports on three patients with Shwachman-Diamond syndrome (SDS) who underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) at the First Affiliated Hospital of Zhejiang University School of Medicine. Based on relevant literature, the clinical manifestations and genetic mutation characteristics of SDS were summarized, and the efficacy and timing of allo HSCT for such patients were explored. Three SDS patients were all male, with transplant ages of 32, 33, and 32 years old, respectively. All three patients were diagnosed in childhood. Case 1 presented with anemia as the initial clinical manifestation, which gradually progressed to a decrease in whole blood cells; Case 2 and 3 both present with a decrease in whole blood cells as the initial clinical manifestation. Case 1 and 3 have intellectual disabilities, while case 3 presents with pancreatic steatosis and chronic pancreatitis. All three patients have short stature. Three patients all detected heterozygous mutations in the SBDS: c.258+2T>C splice site. The family members of the three patients have no clinical manifestations of SDS. All three patients were treated with a reduced dose pre-treatment regimen (Fludarabine+Busulfan+Me-CCNU+Rabbit Anti-human Thymocyte Globulin). Case 1 and case 2 underwent haploid hematopoietic stem cell transplantation, while case 3 underwent unrelated donor hematopoietic stem cell transplantation. Case 1 was diagnosed with myelodysplastic syndrome transforming into acute myeloid leukemia before transplantation, but experienced early recurrence and death after transplantation; Case 2 is secondary implantation failure, dependent on platelet transfusion; Case 3 was removed from medication maintenance treatment after transplantation, and blood routine monitoring was normal.

Objective:To evaluate the preventive effect of indwelling pancreatic duct guide wire through nasobiliary drainage catheter for post-endoscopic retrograde cholangiopancreatography (ERCP) pancreatitis (PEP) in cholelithiasis patients with small-caliber pancreatic duct.Methods:The clinical data of 127 patients with gall bladder and common bile duct stones undergoing ERCP and elective laparoscopic cholecystectomy in the Cholelithiasis Center of Shanghai East Hospital Affiliated to Tongji University from January 2019 to June 2023 were analyzed retrospectively, including 55 males and 72 females, aged (56.95±10.86) years old. According to the preventive methods of PEP, patients were divided into the guide wire group (indwelling pancreatic duct guide wire through nasobiliary drainage catheter), stenting group (nasobiliary catheter with 5Fr 5 cm single pigtail pancreatic duct stent) and conventional group (nasobiliary catheter). The incidence of post-ERCP hyperamylasemia (PEH) and PEP were compared.Results:The incidence of PEH in the guide wire group was lower than that in the conventional group [17.8% (8/45) vs. 43.5% (10/23), P=0.023], and also lower than that in the stenting group [17.8% (8/45) vs. 32.2% (19/59)], despite no statistical significance ( P=0.337). The incidences of PEH were comparable in the stenting group and conventional group [32.2% (19/59) vs. 43.5% (10/23), P=0.096)]. The incidence of PEP in the guide wire group was lower than that in both the stenting group [6.7% (3/45) vs. 23.7% (14/59), P=0.030]. and conventional group [6.7% (3/45) vs. 30.4% (7/23), P=0.025]. The incidences of PEP were comparable in the stenting and conventional group [23.7% (14/59) vs. 30.4% (7/23), P=0.532]. Conclusion:Compared to the preventive pancreatic duct stenting, indwelling pancreatic duct guide wire through nasobiliary drainage catheter can effectively prevent the PEH and PEP in high-risk patients with a small-caliber pancreatic duct.

LIN28 is an RNA binding protein with important roles in early embryo development, stem cell differentiation/reprogramming, tumorigenesis and metabolism. Previous studies have focused mainly on its role in the cytosol where it interacts with Let-7 microRNA precursors or mRNAs, and few have addressed LIN28's role within the nucleus. Here, we show that LIN28 displays dynamic temporal and spatial expression during murine embryo development. Maternal LIN28 expression drops upon exit from the 2-cell stage, and zygotic LIN28 protein is induced at the forming nucleolus during 4-cell to blastocyst stage development, to become dominantly expressed in the cytosol after implantation. In cultured pluripotent stem cells (PSCs), loss of LIN28 led to nucleolar stress and activation of a 2-cell/4-cell-like transcriptional program characterized by the expression of endogenous retrovirus genes. Mechanistically, LIN28 binds to small nucleolar RNAs and rRNA to maintain nucleolar integrity, and its loss leads to nucleolar phase separation defects, ribosomal stress and activation of P53 which in turn binds to and activates 2C transcription factor Dux. LIN28 also resides in a complex containing the nucleolar factor Nucleolin (NCL) and the transcriptional repressor TRIM28, and LIN28 loss leads to reduced occupancy of the NCL/TRIM28 complex on the Dux and rDNA loci, and thus de-repressed Dux and reduced rRNA expression. Lin28 knockout cells with nucleolar stress are more likely to assume a slowly cycling, translationally inert and anabolically inactive state, which is a part of previously unappreciated 2C-like transcriptional program. These findings elucidate novel roles for nucleolar LIN28 in PSCs, and a new mechanism linking 2C program and nucleolar functions in PSCs and early embryo development.
Animals ; Cell Differentiation ; Embryo, Mammalian/metabolism* ; Embryonic Development ; Mice ; Pluripotent Stem Cells/metabolism* ; RNA, Messenger/genetics* ; RNA, Ribosomal ; RNA-Binding Proteins/metabolism* ; Transcription Factors/metabolism* ; Zygote/metabolism*

OBJECTIVES: To investigate the clinical characteristics of patients with listeriosis and to provide a basis for diagnosis, treatment, prevention and control of hospital infection. METHODS: A total of 10 inpatients, who suffered from the listeriosis in Xiangya Hospital, Central South University from January 2013 to June 2019, were retrospectively collected for this study. The characteristics of the patients' age, gander, basic information, case type, clinical manifestations, first consultation department, days of diagnosis, infection indicator, specimen type, results of drug sensitivity, treatment plan, hospital infection or not, outcome, follow-up data were analyzed. RESULTS: Two cases were pregnant women and other were non-pregnant adults among 10 patients with listeriosis. Among them, there were 3 cases with hospital acquired infection. The age of patient onset was 27-71 years old, and the time from onset to diagnosis was 5-36 days. Five cases had fever, and other 5 cases had not fever. There were headache, fatigue, local pain, and other specialized symptoms in the 10 patients.The white blood cell count,the neutrophil ratio, the inflammatory index C-reactive protein, the procalcitonin were all increased, and the erythrocyte sedimentation was accelerated in the 10 patients.All the patients were sensitive to ampicillin, penicillin G, meropenem, and compound sinomine. CONCLUSIONS Listeriosis often affects the patients with low immunity, which often leads to misdiagnosis or missed diagnosis in clinic.So early prevention, early diagnosis, and early treatment can reduce mortality; it is important for departments of nosocomial infection management to manage patients' diet for avoiding outbreaks of listeriosis in hospital.
Adult ; Aged ; Female ; Humans ; Listeria monocytogenes ; Listeriosis/epidemiology* ; Meropenem ; Middle Aged ; Pregnancy ; Pregnancy Complications, Infectious ; Retrospective Studies

Clostridioides difficile is a key pathogen of antibiotic related diarrhea and hospital associated infection, causing several outbreaks in Europe and North Americans and resulting in severe disease burden. However, the standardized diagnostic principle and detection specifications in C. difficile infection (CDI) survey are limited in China, and the infection rate and disease burden of CDI in China are unclear. Therefore, National Institute for Communicable Disease Control and Prevention,National Institute for Communicable Disease Control and Prevention, Chinese Center for Disease Control and Prevention, together with another 11 institutions, draft the group standard entitled "Diagnosis of Clostridium difficile infection (T/CPMA 008-2020)" of Chinese Preventive Medicine Association. Based on the principle of "legality, scientificity, advancement, and feasibility", this standard clarifies risk factors, diagnosis principles, diagnoses and differential diagnoses in order to improve the accuracy of CDI diagnosis in clinical practice, guide the surveillance for CDI, and understand the infection rate and disease burden of CDI in China .

Objective: To study the feasibility, safety and aesthetics of laparoscopic cholecystectomy using a single-port through the umbilicus in patients with chronic cholecystitis with cholecystolithiasis. Methods: From Jan 2007 to Jun 2017, the clinical data of 4790 patients who underwent single-port, 4 426 patients conventional three-ports, and 3 304 patients two-ports laparoscopic cholecystectomy were retrospectively studied. Results: There were no death in all the three groups. There were no significant differences in the incidences of postoperative bleeding, bile leakage, biliary tract injury and other significant short-term complications (all P>0.05). The operation time of transumbilical single-port laparoscopic cholecystectomy was significantly longer than that of two-ports and three-ports laparoscopic cholecystectomy [(35.3±9.0)min vs. (28.4±7.2)min vs. (23.3±6.4)min, P<0.05]. Looking only in a single year, there was no significant difference in the operation time of the three groups in the year 2017 (P>0.05). There were no significant differences among the three groups in intraoperative blood loss, first postoperative passing of flatus, postoperative pain, and length of hospital stay (all P>0.05). The satisfaction score of surgical scar in the single-port group was higher than that in the two-ports and three-ports groups [(9.6±1.6) vs. (7.6±1.9) vs. (6.7±2.0), P<0.05]. Conclusions Transumbilical single-port laparoscopic cholecystectomy was safe and feasible, with good aesthetics and other advantages. It was more difficult than the traditional laparoscopic technique. After the learning curve, the operation time could be comparable to the three-ports technique.

Objective To understand the correlation between psychological counseling behaviors of accompanying family members and anxiety and depression of inpatients with hepatocellular carcinoma. Methods Used self-designed questionnaires to investigate the behaviors of 45 accompanying family members psychological counseling for their relative hospitalized patients with hepatocellular carcinoma, at the same time, evaluated the anxiety and depression among those 45 hospitalized patients with hepatocellular carcinoma, then analyzed the correlation between the accompanying family members' psychological counseling behaviors and hepatocellular carcinoma in patients' anxiety and depression. Results Among the accompanying family members,53.3%(24/45) were the spouses of the patients. During the accompanying nursing time, there were 20.0%(9/45) accompanying families told to their relative patients less than 2 hours per day, and 80.0%(36/45) told to the patients over 2 hours per day. The proportion of anxiety and depression in the hospitalized patients with hepatocellular carcinoma was 28.9%(13/45) and 77.8% (34/45) respectively. Whether the relationship between the accompanying family members and the patients with hepatocellular carcinoma were spouse relationship was related to the incidence of depression in the patients (χ2=10.470, P=0.005), and whether the time accompanying family members spent in talking with the patients over 2 hours every day was correlative with the incidence of anxiety and depression in the patients (χ2=7.012, P =0.030 andχ2=6.438, P=0.040). The patients who accompanying families told to them less than 2 hours per day suffered a higher depression rate (8/9), and among the patients who accompanying families told to them over 2 hours per day, the lowest rate of anxiety (1/12) was found in the patients who accompanying families told to them around 4.1-6.0 hours per day. Conclusions The patients who are accompanied and cared by their spouses have lower depression rate and lesser depression extent. The accompanying families talk to the patients total 4.1-6.0 hours per day could most favorably reduce the incidence of anxiety and depression in the hospitalized patients with hepatocellular carcinoma.

Objective To investigate the prevalence rate of healthcare-associated infection(HAI)in Xiangya Hospital,and provide reference for preventing and controlling HAI.Methods The cross sectional surveys on preva-lence rates of HAI,cross-sectional antimicrobial use,and bacterial detection among all hospitalized patients on the given days in 2000-2014 (except 2006)were carried out by combination of bedside investigation and medical record reviewing.Results The prevalence rates of HAI in 2000-2014 decreased from 6.30% to 3.91%,difference was statistically significant (χ2 = 35.14,P < 0.001 );prevalence rates of community-associated infection(CAI)were 15.61%-15.76%,there was no significant difference among each year.General intensive care unit (ICU)had the highest prevalence rate;respiratory tract was the most common site of both HAI and CAI;urinary catheterization rate showed a decreased tendency,arteriovenous catheterization rate showed a increased tendency,difference were both significant(χ2 = 5.21,96.24,respectively,both P <0.001).In 2008 - 2014,pathogenic detection rates for specimens from patients receiving therapeutic antimicrobial agents were 36.37%-44.51%,from patients with HAI were 34.00%-44.99%,detection rate of pathogens causing HAI were 41.57%-68.48%,all showed a increased tendency,difference was significant (χ2 = 22.78,10.03,26.49,respectively,all P < 0.001 ).Gram-negative bacteria were the main pathogens causing infection;both cross sectional and combination antimicrobial usage rates declined (P < 0.05 ).Conclusion Prevention and control of HAI,and antimicrobial management has achieved preliminary success,prevalence rate of HAI and cross sectional antimicrobial usage rate declined obviously,the main pathogen is gram-negative bacteria,and the major infection site is lower respiratory tract.

Objective To understand the current situation and existing problems in the training of healthcare-asso-ciated infection(HAI)management,and provide scientific basis for strengthening the management of HAI preven-tion and control system.Methods A questionnaire survey was adopted to investigate situation of training on HAI in 15 provincial-level HAI training agencies in China during the past 30 years,and basic condition of training on HAI management in recent 5 years.Results Among 15 provincial-level training agencies,66.67%(n=10)were respon-sible by HAI management quality control centers,80.00% have already conducted training in each city,53.33%carried out training for 10 to 20 times,33.34% performed training for ≤2 times per year.Of 33 728 trainees in 2011-2015,41.30% were 41-50 years old,61.82% were nursing staff,50.56% had bachelor degree,43.96%were with the intermediate professional title.Most trainers were HAI prevention and control experts in their respec-tive province,accounting for 68.07%,the curriculums were mainly designed on professional course,and only 26.78% were involved in management.Conclusion Professional structure of HAI management personnel is not reasonable,faculty is imbalance,knowledge update is lacking,and HAI training and education system need to be improved further.

Objective To investigate the impact of applying patient-centered care concept on easing the negative moods among the parents of the neonates hospitalized in NICU.Methods The parents of neonates (76 people) hospitalized in NICU from March to May in 2012 were selected as the control group,they conducted normal nursing.The parents of neonates hospitalized in NICU during June and August in 2012 were named as the observation group (81 people).In addition to the normal nursing,they were given nursing intervention according to patient-centered care concept.The anxiety and depression moods of the two groups was analyzed.Results Before the patient-centered care concept intervention,there was no statistical difference of SAS,SDS scores for both groups.For the observation group,the SAS,SDS scores after intervention were significantly lower than those before the intervention.While for the control group,there was no statistical difference of SAS,SDS scores.After intervention,the SAS、SDS scores of the observation group were significantly lower than those of the control group,and the difference was statistically significant.Conclusions The negative moods of the parents of hospitalized neonates can be eased significantly by applying patient-centered care concept intervention.