Objectives:To investigate the clinical and genetic features of young Chinese patients with myeloproliferative neoplasms (MPN) .Methods:In this cross-sectional study, anonymous questionnaires were distributed to patients with MPN patients nationwide. The respondents were divided into 3 groups based on their age at diagnosis: young (≤40 years) , middle-aged (41-60 years) , and elderly (>60 years) . We compared the clinical and genetic characteristics of three groups of MPN patients.Results:1727 assessable questionnaires were collected. There were 453 (26.2%) young respondents with MPNs, including 274 with essential thrombocythemia (ET) , 80 with polycythemia vera (PV) , and 99 with myelofibrosis. Among the young group, 178 (39.3%) were male, and the median age was 31 (18-40) years. In comparison to middle-aged and elderly respondents, young respondents with MPN were more likely to present with a higher proportion of unmarried status (all P<0.001) , a higher education level (all P<0.001) , less comorbidity (ies) , fewer medications (all P<0.001) , and low-risk stratification (all P<0.001) . Younger respondents experienced headache (ET, P<0.001; PV, P=0.007; MF, P=0.001) at diagnosis, had splenomegaly at diagnosis (PV, P<0.001) , and survey (ET, P=0.052; PV, P=0.063) . Younger respondents had fewer thrombotic events at diagnosis (ET, P<0.001; PV, P=0.011) and during the survey (ET, P<0.001; PV, P=0.003) . JAK2 mutations were found in fewer young people (ET, P<0.001; PV, P<0.001; MF, P=0.013) ; however, CALR mutations were found in more young people (ET, P<0.001; MF, P=0.015) . Furthermore, mutations in non-driver genes (ET, P=0.042; PV, P=0.043; MF, P=0.004) and high-molecular risk mutations (ET, P=0.024; PV, P=0.023; MF, P=0.001) were found in fewer young respondents. Conclusion:Compared with middle-aged and elderly patients, young patients with MPN had unique clinical and genetic characteristics.

Zn²⁺ is required for the activity of many mitochondrial proteins, which regulate mitochondrial dynamics, apoptosis and mitophagy. However, it is not understood how the proper mitochondrial Zn²⁺ level is achieved to maintain mitochondrial homeostasis. Using Caenorhabditis elegans, we reveal here that a pair of mitochondrion-localized transporters controls the mitochondrial level of Zn²⁺. We demonstrate that SLC-30A9/ZnT9 is a mitochondrial Zn²⁺ exporter. Loss of SLC-30A9 leads to mitochondrial Zn²⁺ accumulation, which damages mitochondria, impairs animal development and shortens the life span. We further identify SLC-25A25/SCaMC-2 as an important regulator of mitochondrial Zn²⁺ import. Loss of SLC-25A25 suppresses the abnormal mitochondrial Zn²⁺ accumulation and defective mitochondrial structure and functions caused by loss of SLC-30A9. Moreover, we reveal that the endoplasmic reticulum contains the Zn²⁺ pool from which mitochondrial Zn²⁺ is imported. These findings establish the molecular basis for controlling the correct mitochondrial Zn²⁺ levels for normal mitochondrial structure and functions.
Animals ; Caenorhabditis elegans/metabolism* ; Cation Transport Proteins/genetics* ; Homeostasis ; Mitochondria/metabolism* ; Zinc/metabolism*

Autoimmune hepatitis (AIH) is a chronic progressive inflammatory disease of the liver caused by the attack of liver cells by the autoimmune system, with the features of positive serum autoantibodies, high IgG, and/or γ-globulinemia. Current studies on pregnancy in patients with AIH mainly focus on labor complications, and there is still a lack of systematic recommendations for the evaluation, treatment, and management of diseases in the progestational stage, during pregnancy, and after delivery. Although immunity is suppressed during pregnancy, poor disease control within one year before pregnancy and spontaneous drug withdrawal during pregnancy can significantly increase adverse pregnancy outcomes. Therefore, this article describes how to implement multidisciplinary collaboration and management of the whole cycle of pregnancy, so as to improve maternal and fetal safety.

Objective:To carry out prenatal screening and diagnosis for a woman with advanced maternal age.Methods:Non-invasive prenatal testing (NIPT) was carried out to determine the risk of fetal chromosome aneuploidy. Aminiocentesis was proceeded for fetal chromosomal karyotyping and copy number variation sequencing (CNV-seq). The fetus was subjected to systematic ultrasound screening in the second trimester.Results:NIPT has indicated there was a loss of fetal sex chromosome. Karotyping of the amniocyte showed a mosaic sex chromosome abnormality 45, X[53]/46, X, + mar[7]. The result of fetal DNA CNV-seq was seq[GRCh37]del(Yq11.1q12) chrY: g. 13 104 553-28 819 361del, seq[GRCh37]del(Yp11.32p11.2) chrY: g. 10 001-9 873 915del (mosaic ratio: 30%). Ultrasonography discovered that the fetus had renal dysplasia and male external genitalia. The karyotypes of the couple were both normal.Conclusion:Multiple genetic tests should be carried out for fetus with a high risk for chromosome aneuploidies signaled by NIPT. It is difficult to predict the post-natal phenotype for fetuses with mosaic sex chromosomal aneuploidies. The couple should be carefully counseled upon genetic counseling.

Objective:To explore the effect of solution-focused model psychological intervention on disability acceptance of ischemic stroke patients.Methods:From May to July 2020, 200 patients with ischemic stroke who were admitted to the Neurology Department of the Second Hospital of Hebei Medical University were selected as the study subject by convenience sampling. The patients were randomly divided into the control group and the observation group by the envelope method, with 100 cases in each group. The control group was given routine nursing, while the observation group received solution-focused model psychological intervention on the basis of routine nursing. The Acceptance of Disability Scale (ADS) and Stroke Specific Quality of Life Scale (SS-QOL) were used to compare the disability acceptance and quality of life at admission, three months and six months after discharge in the two groups. There were two cases lost in the observation group and two cases lost in the control group, and 98 patients were included in the final observation group and the control group respectively.Results:There was no statistical difference in the scores of ADS and SS-QOL between the two groups at admission ( P>0.05) . At three and six months after discharge, the scores of ADS and SS-QOL of patients in the observation group were higher than those at admission, and the scores in the observation group were higher than those in the control group, with statistically significant differences ( P<0.01) . Conclusions:The solution-focused model psychological intervention can improve the disability acceptance and quality of life of stroke patients, which is worthy of clinical application.

Objective:To explore the effects of Baduanjin combined with resistance exercise in rural elderly.Methods:From May to September 2020, totally 80 aged people from 2 villages in Hebei province were selected by convenient sampling. Patients from one village were randomly included into an intervention group ( n=40) , and patients from the other village were included into a control group ( n=40) . Patients in the control group took daily activities and exercise, and received only health education, while patients in the intervention group practiced Baduanjin combined with resistance exercise in addition to daily activities. The Chinese version of Fried Frailty Phenotype Assessment Components, Time Up and Go Test (TUGT) , Five-Times Sit-to-Stand Test (FTSST) , and Pittsburgh Sleep Quality Index (PSQI) were used to evaluate the frailty, balance ability, lower limb muscle strength, and sleep quality of the two groups of rural elderly before and after the intervention. In this study, totally 80 questionnaires were distributed, and 78 valid questionnaires were recovered, accounting for an effective recovery rate of 97.5%. Results:The Chinese version of Fried Frailty Phenotype Assessment Components and PSQI scores of the rural elderly in the intervention group after the intervention were lower than those in the control group and before the intervention, and the differences were statistically significant ( P<0.05) . The TUGT and FTSST time of the rural elderly in the intervention group after the intervention were shorter than those in the control group and before the intervention, and the differences were statistically significant ( P<0.01) . Conclusions:Baduanjin combined with resistance exercise can delay the debilitating state of rural elderly, improve their lower limb muscle strength, balance ability and sleep quality, and enrich their exercise methods.

Objective:To construct an Omaha system-based remote intervention program for clinical nurses' neck, shoulder and low back pain and evaluate its effect.Methods:From July to October 2020, 94 nurses with neck, shoulder and low back pain in a Class Ⅲ hospital were randomly selected by ward. Using the envelope method, nurses were randomly divided into the intervention group and the control group, with 47 cases in each group, among them, the intervention group dropped 1 and the remaining 46 persons, the control group dropped 4 and the remaining 43 persons. The intervention group conducted a remote intervention program (using Omaha system as the theoretical framework, and using literature review, semi-structured interviews and expert consultation to construct a remote intervention program for clinical nurses' neck, shoulder and low back pain) . The control group used conventional guidance. The General Information Questionnaire, Exercise Compliance Questionnaire, Knowledge-Behavior-Symptoms (K-B-S) and Visual Analog Scale (VAS) were used to evaluate the intervention effect.Results:The exercise compliance of nurses in the intervention group was 78.26% (36/46) and that in the control group was 39.53% (17/43) , the difference was statistically significant ( P<0.01) . After 6 weeks of intervention, the nurses' cognitive and behavior scores in the intervention group were higher than those in the control group, and the difference was statistically significant ( P<0.05) . There was no statistically significant difference in the symptom score between the two groups ( P>0.05) . The VAS score and frequency of neck, shoulder and low back pain were lower than those of the control group, and the difference was statistically significant ( P<0.05) . Conclusions:Based on Omaha system, the remote intervention program for the clinical nurses' neck, shoulder and low back pain is safe, scientific and theoretical. It can improve cognition and behavior of neck, shoulder and low back pain nurses, reduce the intensity and frequency of pain, and the nurses have good compliance.

Objectives:To explore health-related quality of life (HRQoL) and identify its associated variables in Chinese patients with Philadelphia-negative myeloproliferative neoplasms (MPNs) .Methods:In this cross-sectional study, anonymous questionnaires were distributed to adult patients with MPNs to assess symptom burden measured by MPN-10 and HRQoL measured by Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36) and the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire Core 30 (EORTC QLQ-C30) .Results:The data from 1405 respondents with MPNs, including 645 (45.9%) with essential thrombocythemia (ET) , 297 (21.1%) with polycythemia vera (PV) , and 463 (33.0%) with myelofibrosis (MF) , were analyzed. 646 (46.0%) respondents were male. The median age was 56 (range, 18-99) years. The mean MPN-10 scores were 13.0±12.7, 15.0±14.7, and 21.0±16.6 ( P<0.001) , and the physical component summary (PCS) and mental component summary (MCS) scores were 48.0±8.5, 47.0±9.0, and 42.0±10.0 ( P<0.001) and 51.0±11.0, 50.0±10.8, and 49.0±11.1 ( P=0.002) for respondents with ET, PV, and MF, respectively. Respondents with MF reported the lowest score of physical functioning, role functioning, emotional functioning, cognitive functioning, social function, and global health status (all P<0.01) and the highest score of fatigue, pain, dyspnea, appetite loss, diarrhea, and financial problems (all P<0.05) in EORTC QLQ-C30. Multivariate analyses revealed that higher MPN-10 scores were significantly associated with lower PCS (-0.220 to -0.277, P<0.001) and MCS (-0.244 to -0.329, P<0.001) scores; increasing age (-1.923 to -4.869; all P<0.05) , lower PCS score. Additionally, comorbidity (ies) , symptom at diagnosis, splenomegaly, anemia, unknown driver gene, and higher annual out-of-pocket cost were significantly associated with lower PCS and/or MCS scores. However, age ≥ 60 years, urban household registration, concomitant medication, and receiving ruxolitinib therapy in respondents with MF were associated with higher MCS scores. Weak correlations were found between MPN-10 score (except the subscale of appetite loss and constipation) and EORTC QLQ-C30 score in majority of subscales in respondents with ET (| r| = 0.193-0.457, all P<0.001) , PV (| r| = 0.192-0.529, all P<0.01) , and MF (| r| = 0.180-0.488, all P<0.001) , respectively. Conclusions:HRQoL in patients with MPN was significantly reduced, especially in patients with MF. Sociodemographic and clinical variables were significantly associated with the HRQoL in patients with MPNs.

Objective:To evaluate the MAPO index of work-related musculoskeletal disorders (WMSDs) among ward nurses who manually handled patients, and to explore the influencing factors of WMSDs in the workplace.Methods:From March to May 2019, convenience sampling method was used to select 317 nurses from 26 wards of a ClassⅢ Grade A hospital. The General Information Questionnaire, MAPO index assessment, and the meter ruler were used to comprehensively evaluate the situation of WMSDs in the workplace of nurses who manually handled patients inwards with the method of on-site investigation and measurement. According to the score of the MAPO index, there were 3 risk levels of wards, namely red light, yellow light, and green light. The yellow light meant that it was necessary to implement the medium-term and long-term intervention plan, and the red light meant that the short-term intervention plan must be implemented. Multivariate Logistic regression was used to analyze the influencing factors of WMSDs in nurses who manually handled patients in the ward.Results:A total of 317 questionnaires were distributed and 265 valid questionnaires were returned with an effective response rate of 83.6%. In the 265 ward nurses, there were 76.2% (202/265) of nurses with WMSDs. There were 13 (50.0%, 13/26) wards with red lights, including 158 (59.6%, 158/265) nurses, and 6 (23.1%, 6/26) wards with yellow lights, including 92 (34.7%, 92/265) nurses, and 7 (26.9%, 7/26) wards with green lights, including 15 (5.7%, 15/265) nurses. Multivariate Logistic regression analysis showed that MAPO index and the ward score were the influencing factors for the occurrence of WMSDs among ward nurses with statistical differences ( OR=1.056, 1.571; P<0.05) . Conclusions:The risk of WMSDs in the workplace of ward nurses is high. It is recommended starting with the types of beds, bed spacing, auxiliary equipment, nursing human resource allocation, and occupational protection training to reduce the occurrence of WMSDs in nurses.

Objective:To explore effects of breathing meditation training on fatigue and anxiety state of nurses.Methods:Using the convenient sampling method, 32 neurological nurses from a Class Ⅲ Grade A hospital were selected as the research objects from June 1 to September 1, 2019. A breathing meditation training program was developed and breathing meditation training was performed 5 times a week, 25 minutes each time, for 6 consecutive weeks. Before and after the intervention, Multidimensional Fatigue Scale (MFI-20) and Self-rating Anxiety Scale (SAS) were used to evaluate patients in the two groups.Results:After the intervention, the total score of MFI-20 and the scores of all dimensions were reduced. Except for the reduction of activities, the differences were statistically significant ( P<0.05) . After intervention, SAS scores of 32 nurses were (33.90±2.54) , which was lower than that (38.78±2.23) before intervention, and the difference was statistically significant ( t=7.956, P<0.05) . Conclusions:Breathing meditation training can effectively improve the anxiety level of nurses and relieve their fatigue state.