Objective To investigate the occurrence of adverse events of lurasidone in the U.S.Food and Drug Administration Adverse Event Reporting System(FAERS)database by using Open Vigil FDA2.1,to enrich the experience and provide the basis for the clinical use of the drug in China.Methods Using Open Vigil FDA2.1,adverse event data were extracted from the FAERS database for a total of 51 quarters from the 4th quarter of 2010 to the 3rd quarter of 2023,and the ratio of reporting ratio(ROR)method and the proportional reporting ratio(PRR)method were used for data mining and analysis.Results A total of 32 728 adverse event reports with lurasidone as the first suspected drug was obtained,with a larger proportion of females(54.26％)and occurring mostly in adults(18 to 59 years).After the screening,326 preferred term(PT)signals were obtained,involving 20 system-organ classifications(injury,poisoning and procedural complications,general disorders and administration site conditions,psychiatric disorders,etc.).Among them,PTs with the higher frequency of occurrence included off label use,feeling abnormal,crying,anxiety,depression,insomnia,etc.PTs with stronger signal strength included activation syndrome,mania,tongue movement disturbance,hypoprolactinaemia,akathisia,etc.Multiple new suspected adverse drug reactions were unearthed,including hypoprolactinaemia,emotional poverty,stiff tongue,etc.Conclusion Lurasidone has a favorable safety profile,and women need to closely monitor prolactin levels when taking this medication.The drug is relatively safe for use in pregnant,puerperal and perinatal women and patients with poor metabolic function.Hypoprolactinaemia and restless leg syndrome are new rare suspected adverse events with lurasidone.

The molecular genetic characteristics of a family with rare -88 C>G (HBB: c.-138 C>G) β-thalassemia gene mutation were studied using cohort study. The cohort study was conducted from June to August 2022 by Prenatal Diagnosis Center of Sanya Women and Children's Hospital Managed by Shanghai Children's Medical Center. The phenotype and genotype were analyzed by hematological cytoanalyzer, automatic electrophoretic analysis system, and next-generation sequencing (NGS). And then, Sanger sequencing was used to verify the rare gene results. The results showed that the proband, her father, her uncle and her younger male cousin had discrete microcytosis (MCV 70.1 fl, 71.9 fl, 73.1 fl and 76.6 fl, respectively) and hypochromia (MCH 21.5 pg,22.0 pg,22.6 pg and 23.5 pg, respectively), elevated hemoglobin A2 level (5.3%, 5.4%, 5.4% and 5.5%, respectively), slightly elevated or normal fetal hemoglobin (Hb F), but no anemia. The proband was identified to have co-inherited ɑ-thalassemia (Hb Westmead gene heterozygous mutation, ɑ^wsɑ/ɑɑ) and β-thalassemia with a rare -88 C>G (HBB: c.-138 C>G) heterozygous mutation (β^{-88 C>G}/β^N). Her mother had the same α-thalassemia as the proband. Her father, her uncle and her younger male cousin had the same rare -88 C>G heterozygous mutations as the proband. While her grandmother and younger brother were not carrier of thalassemia. In conclusion, 4 cases of rare -88 C>G(HBB:c.-138 C>G) heterozygous mutation had been detected in a Chinese family. Carriers of this beta-thalassemia are clinically asymptomatic. This study enriches the knowledge of the thalassemia mutation spectrum in Chinese people and provides valuable information for genetic counseling, prenatal diagnosis, and prevention of thalassemia, providing a scientific basis for improving the quality of birth population and preventing birth defects.
Female ; Humans ; Male ; alpha-Thalassemia/genetics* ; beta-Globins/genetics* ; beta-Thalassemia/diagnosis* ; China ; Cohort Studies ; Genotype ; Molecular Biology ; Mutation

OBJECTIVE: To analyze the clinical efficacy of haploidentical hematopoietic stem cell transplantation (haplo-HSCT) by using parental donors on thalassemia patients. METHODS: The 13 thalassemia patients treated by haplo-HSCT using parental donors in our hospital from July 1, 2016, to July 1, 2020 were retrospectively reviewed. Hematopoiesis reconstitution, the incidence of GVHD, infections and the long-term survival of the patients were analyzed. RESULTS: Twelve of the 13 patients were successfully implanted, the success rate of implantation was 92.3%. The median time of neutrophil and platelet engraftment was 12.5 days (range, 9-22 days) and 21 days (range,12-34 days), respectively. One patient achieved primary graft failure. Three (25%) patients developed to acute GVHD (aGVHD) and achieved complete remission after treatment. Chronic GVHD developed in three (25%) patients, one of them was extensive and under treatment, while one patient developed to severe bacterial infection (7.7%). CMV viremia was diagnosed in two patients (15.4%). There were no patients developed to CMV disease. Three (23.1%) patients achieved EB viremia after transplantation, one of them developed to EBV-related lymphocytic proliferative disease, while there were no patients showed invasive fungal infection. At the last follow-up, all patients survived, twelve of them were free from transfusion dependency. There were no transplant-related deaths. Projected overall and thalassemia-free survival at three years was 100% and 92.3%, respectively. CONCLUSION The transplant protocol of haplo-HSCT by using parental donors in patients with thalassemia has reliable source of donors, high incidence of successful implantation and low incidence of GVHD, which can be used as an effective way to increase the source of donors in children with thalassemia.
Child ; Cytomegalovirus Infections ; Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation ; Humans ; Parents ; Retrospective Studies ; Thalassemia/therapy* ; Transplantation Conditioning/methods* ; Treatment Outcome ; Viremia

OBJECTIVE: To investigate the difference of therapeutic effects on children with thalassemia at different age after hematopoietic stem cell transplantation. METHODS: The clinical data of children with thalassemia treated in our hospital were retrospectively analyzed. The children were divided into 2-5 years old group and 6-12 years old group. The success rate of implantation, transplant-related mortality, GVHD incidence, and other transplant-related complications, as well as thalassemia-free survival (TFS) were compared between the two groups. RESULTS: The incidence of GVHD, hemorrhagic cystitis and severe oral mucositis after transplantation in the 2-5 years old group were significantly lower than those in the 6-12 years old group, while there was no statistically significant difference in the TFS between the two groups. CONCLUSION Children in the low age (2-5 years old) group show fewer complications and higher quality of life after transplantation, therefore, stem cell transplantation at 2-5 years old is more conducive to rehabilitation of the children with thalassemia.
Child ; Child, Preschool ; Graft vs Host Disease/complications* ; Hematopoietic Stem Cell Transplantation ; Humans ; Quality of Life ; Retrospective Studies ; Thalassemia/therapy* ; beta-Thalassemia/therapy*

OBJECTIVE: To retrospectively analyze the efficacy and safety of ruxolitinib therapy for children with thalassemia after unrelated or haploidentical stem cell transplantation. METHODS: From March 2020 to March 2021, 22 patients received successfully allogeneic hematopoietic stem cell transplantation in the Zhongshan Hospital of Xiamen University, from +30 to 100 days,those patients received ruxolitinib therapy (2.5 mg, twice daily) and all adverse reactions were observed, include aGVHD, cGVHD, CMV and EBV infection. RESULTS: 22 patients underwent allogeneic stem cell transplantation, 5 patients were diagnosed as aGVHD, 3 patients had grade I－II skin GVHD and 2 patients had grade II intestinal GVHD, those patients were cured. All patients were followed up for more than 21 weeks, 4 cases developed cGVHD, including 3 cases of localized liver GVHD and 1 case of pulmonary GVHD, those were relieved after active treatment. 8 patients had elevated EBV copies (>3×10³/ml), and 3 patients had increased CMV copies, the patients recovered after immunosuppressant and antiviral treatment. There was no CMV infection and EBV related post-transplantant lymphoproliferative disorders(PTLD), and no transplant related deaths. CONCLUSION Ruxolitinib can effectively reduce the incidence and severity of GVHD without affecting the hematopoietic recovery, and improve the survival status of thalassemia children after transplantation.
Antiviral Agents/therapeutic use* ; Child ; Graft vs Host Disease/prevention & control* ; Hematopoietic Stem Cell Transplantation/adverse effects* ; Humans ; Immunosuppressive Agents/therapeutic use* ; Nitriles ; Pyrazoles ; Pyrimidines ; Retrospective Studies ; Thalassemia

Objective: To investigate the prevalence and Influence factors of work-related musculoskeletal disorders (WMSDs) among dental nurses in tertiary stomatology hospitals. Methods: From June to July 2020, 1321 dental nurses from 18 tertiary stomatology hospitals in 18 provinces of China were enrolled as research subjects through a phased sampling method. The cross-sectional study was performed with validated instruments including the basic information questionnaire, Effort-Reward Imbalance Questionnaire, Nurse-Physician Collaboration Scale, and Chinese Musculoskeletal Questionnaire, so as to get the information about the social demography information, nursing-physician cooperation, work stress and the incidence of WMSDs. The influenang factors of WMSDs was analyzed. Results: The past prevalence, annual prevalence, and weekly prevalence of WMSDs were 80.8% (1067/1321) , 68.7% and 43.7% (575/1321) . 58.4% (771/1321) of nurses had WMSDs in three or more body parts. Neck (51.4%) , waist (42.2%) and shoulder (41.6%) were the top three body regions in the past year. The absenteeism rate due to musculoskeletal disorders ranged from 3.5% to 9.0%, waist (8.9%) and neck (8.0%) were the top two body regions. Working more than 40 hours per week (OR=1.74, P<0.001) and effect/reward>1 (OR=1.50, P=0.002) were risk factors of WMSDs (OR=1.74, 95% CI: 1.32~2.30; OR=1.50, 95%CI: 1.17~1.92, P<0.05) , while regular physical exercise every week (≥30 min per times) was the protective factor (OR=0.74, 95%CI: 0.56~0.97, P=0.030) . Conclusion: The prevalence rate of WMSDs of dental nurses in China was high, and most of the nurses suffered with many body parts. Thus, we should reduce controllable occupational stresso, ensuring adequate rest and strengthening physical exercise to reduce the incidence of WMSDs.
Humans ; Prevalence ; Cross-Sectional Studies ; Occupational Diseases/epidemiology* ; Dental Assistants ; Musculoskeletal Diseases/epidemiology* ; Surveys and Questionnaires ; Risk Factors

OBJECTIVE: To investigate the clinical correlation of expression level changes of miR-181b and miR-194 to the pathogenesis of acute graft-versus-host disease (aGVHD), and determine plasma miR-181b and miR-194 as the potential biomarkers for aGVHD. METHODS: The plasma samples were collected from 31 patients who underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) at before HSCT, 15 days after HSCT and onset of aGVHD. The expression levels of miR-181b and miR-194 were detected by quantitative real-time PCR. Receiver-operating characteristic (ROC) curves and the area under the ROC curve (AUC) were used to assess the sensitivity and specificity of miRNA biomarkers for the diagnosis of aGVHD. RESULTS: MiR-181b and miR-194 downregulated after treatment were significantly upregulated in the plasma at onset of aGVHD (P<0.05), and there was no significant difference in comparison with the level of before HSCT (P>0.05). The expressions of plasma miR-181b and miR-194 collected on day 15 after HSCT were significantly upregulated in the patients with aGVHD in comparison with non-GVHD patients (P<0.05). Moreover, these elevated miRNAs were detected before aGVHD. The AUC of miR-181b predicting aGVHD was 0.91±0.05 (specificity was 0.94, sensitivity was 0.69). The AUC of miR-194 predicting aGVHD was 0.91±0.06 (specificity was 0.94, sensitivity was 0.77). CONCLUSION MiR-181b and miR-194 may serve as early biomarkers for the diagnosis and prognosis of aGVHD.
Acute Disease ; Biomarkers ; Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation ; Humans ; MicroRNAs ; Transplantation, Homologous

OBJECTIVE: To investigate the clinical characteristics, laboratorial and bone marrow pathological features of primary thrombocytopenia (ET) patients with different mutations of CALR, JAK2 and MPL genes. METHODS: The chinical data of 120 cases of ET in Jiangsu provincial people's hospital/ The First Affiliated Hospital of Nanjing Medical University from January 2015 to December 2017 were collected and analyzed, including 76 cases with JAK2 gene mutation, 40 cases with CALR gene mutation, 2 cases with MPL gene mutations, 2 cases without gene mutation. RESULTS: Among the ET patients, compared with the JAK2 gene mutation, CALR gene mutation showed statistically significant deareament of white blood cells and hemoglobin (P=0.001, P=0.01) and the male platelets in CALR group showed significant increament (P=0.04). Fourthermore, the average number of megakaryocytes and its cluster numbers in each hight power field of vision showed statistically significant decreament in CALR group as compared with JAK2 group (P=0.001, P=0.001), and thrombotic events in CALR group were signicantly lower than those in JAK2 group (7.5% vs 18.4%) (P=0.03). CONCLUSION Mutations of CALR, JAK2 have different clinical characteristics and blood pathological changes of Chinese ET patients, and their clinical significance is worth to explore.
Bone Marrow ; Calreticulin ; genetics ; China ; Humans ; Janus Kinase 2 ; genetics ; Male ; Mutation ; Receptors, Thrombopoietin ; genetics ; Thrombocythemia, Essential

Objective To evaluate and analyze the panel composition and conflict of interest management of clinical practice guidelines of rehabilitation.Methods Clinical practice guidelines of rehabilitation were systematically retrieved from PubMed, EMBASE, China Biology Medicine disc, CNKI, Wanfang Data and Medlive database, the National Institute for Health and Care Excellence, National Guideline Clearinghouse, The Scottish Intercollegiate Guidelines Network, World Health Organization (WHO) and Guidelines International Network until January 11, 2020. Two researchers independently screened and cross-checked the literatures, and extracted the basic information of included guidelines, including the title, formulating institution, published journol, as well as the expert group, the number of expert group, the specific division of labor and their discipline, affiliation and the geographic location, the statement and management of conflict of interest, types of conflicts of interest, and whether to accept fund and the source of the fund.Results A total of 84 guidelines were finally included, 17 domestic ones and 67 foreign ones, in which, 52 (61.9%) reported the expert panel; 22 (26.2%) reported the number of expert panel groups, 21 (25.0%) indicated the specific division of work among the members of the expert panel, 74 (88.1%) reported the names of the members of the expert panel, 47 (56.0%) reported the subjects and specialties of the panelists, 70 (83.3%) reported the affiliation and location of panelists, 14 (16.7%) mentioned the management of conflicts of interest, and 25 (29.8%) reported if there were conflicts of interest. Only five of the 16 funded guidelines stated that there was no conflict of interest between the funding and the development of the guidelines. Among them, the reporting rate of expert panel was significantly higher in foreign countries than in China (χ2 = 9.542, P < 0.01), the reporting rate of name of expert panel members and specific division of labor were higher in foreign countries than in China ( χ2 > 4.155, P < 0.05), and the reporting rate of conflict of interest management was also higher in foreign countries than in China ( P < 0.05). There was no significant difference in whether there was a conflict of interest, whether it was funded and the type of funding at home and abroad ( P > 0.05). Conclusion In gerenal, clinical practice guidelines of rehabilitation published at home and abroad are necessary to be improved in the reporting quality of expert group formulation and division of labor, conflict of interest reporting and management. It is proposed that future guideline developers should follow the WHO Handbook for Guideline Development, assign roles of experts, strengthen the management and reporting of conflicts of interest, and standarderize the development process and reporting of the guidelines.

Objective To analyze the contents of the recommendations of stroke clinical rehabilitation guidelines in order to provide advice for the development and implementation of stroke rehabilitation guidelines.Methods PubMed, EMBASE, CNKI, China Biology Medicine disc, Wanfang databases and guideline-related websites were retrieved from the date of establishment to January 2020, to collect stroke clinical rehabilitation guidelines. The main content, recommendations based on evidence, the type of evidences, and the scope of evidence classification had been explored based on World Health Organization Handbook for Guideline Development-2nd Edition.Results A total of twelve guidelines were included in this study, one in Chinese and eleven in English. They were from the United States (3 guidelines), United Kingdom (3 guidelines), Canada (3 guidelines), Australia (2 guidelines) and China (1 guidelines), and published from September, 2005 to February, 2019. Three articles (25.0%) used the Appraisal of Guidelines for Research and Evaluation (AGREE) for quality evaluation, and two articles (16.7%) used Grading of Recommendations Assessment, Development and Evaluation (GRADE) as the grading system. The recommendations covered the areas such as: cognitive dysfunction (4 dimensions) with a recommendation of cognitive function assessment at most (7 guidelines, 66.7%), language and swallowing dysfunction (5 dimensions) with a recommendation of swallowing-related complications at most (10 guidelines, 83.3%), motor dysfunction (6 dimensions) with a recommendation of spasm treatment at most (10 guidelines, 83.3%), and psychological and behavioral dysfunction (4 dimensions) with a recommendation for assessment or monitoring at most (6 guidelines, 50.0%). There were many recommendations in these areas, but the types of evidence were different, and observational studies and/or randomized controlled trials accounted for most.Conclusion There are different types of evidence and levels of recommendation strength. It is proposed for future guideline developers in stroke rehabilitation to follow World Health Organization Handbook for Guideline Development-2nd Edition to improve the quality and the overall implementation of the rehabilitation guidelines, and to improve the quality and safety of rehabilitation.