Objective:To enhance the understanding of the diagnosis and individualized treatment of myelodysplastic syndrome/myeloproliferative neoplasms with ring sideroblasts and thrombocytosis (MDS/MPN-RS-T).Methods:A retrospective case series study was conducted. The clinical data, diagnosis and treatment process and prognosis of 4 patients with MDS/MPN-RS-T admitted to Affiliated Hospital of Hebei University from September 2015 to May 2021 were retrospectively analyzed, and the related literature was reviewed.Results:All the 4 patients were male, aged 63 to 75 years. Patients 1 and 2 were classified as revised international prognostic scoring system (IPSS-R) high-risk group, combined with ASXL1 mutation and high risk cytogenetic abnormality. The therapeutic effect of various treatment regimens was poor, and they were converted to acute myeloid leukemia (AML) and then died due to disease progression. Patient 3 was classified as IPSS-R medium-risk group. His main manifestation was myelodysplastic syndrome (MDS) combined with ring sideroblasts in the early stage and was transformed into MDS/MPN-RS-T during the treatment, and JAK2 mutation occurred in the subsequent treatment. After lenalidomide treatment, the patient was removed from blood transfusion and the condition was stable at present. Patient 4 was classified as IPSS-R medium-risk group, and lenalidomide showed significant therapeutic effects and he was in stable condition.Conclusions:Lenalidomide can significantly improve transfusion dependence in patients with MDS/MPN-RS-T, and ASXL1 mutation and high-risk cytogenetic abnormality may be associated with AML transformation.

Pyroptosis is a programmed death of inflammatory cells triggered by pathogen invasion,dependent on caspase activation,through both classical and non-classical pyroptosis pathways.Cell pyroptosis is related to the occurrence and development of a variety of animal infectious diseases caused by microbial infection.After microorganisms invading,cells are stimulated by pathology-re-lated molecular patterns,causing strong immune response,stimulating inflammatory signaling pathways,and then activating inflammasome,leading to pyroptosis.The immune system has e-volved multiple mechanisms to fight microbial infections and regulate inflammatory responses.The innate immune system,by recognizing microbial molecules in pathogens and responding quickly by producing inflammasome and activating pyroptosis,helps clear pathogens to prevent infection and maintain the normal functioning of the body.A thorough study of the pathogenesis and immune es-cape mechanism of cell pyroptosis in animal infectious diseases will provide a new direction for the treatment of animal infectious diseases.

This study summarized the nursing experience of 2 pediatric heart transplant surgeries.Perioperative care included preoperative maintenance of cardiac function,volume management,infection prevention measures,and psychological preparation.Early postoperative care involved circulatory monitoring,management of right heart dysfunction,use of immunosuppressants,observation of rejection reactions,control of postoperative infections,nutritional support,psychological care,and home continuity care.The postoperative rehabilitation effect of the children is satisfactory after multi-team special treatment and nursing care.The postoperative hospitalization time for the 2 patients were 20 and 30 days.After discharge,2 patients showed good recovery during follow-up.

Objective To construct an intervention programme for breastfeeding among mothers of infants with congenital heart disease and to verify its effect.Methods Taking the capability-opportunity-motivation-behaviour model as the theoretical basis,the first draft of the intervention programme was formed through literature search and semi-structured interviews,and 8 experts in the relevant fields were selected to conduct expert validation and determine the content of the program.By the consecutive sampling method,infants and their mothers who attended the cardiothoracic disease clinic of a tertiary-level children's specialist hospital in Shanghai from 4 May to 24 June 2023,with a day-old age of≤14 d and a confirmed diagnosis of CHD,were selected as the study subjects.They were divided into an experimental group and a control group,with 16 cases in each group,by the method of randomized grouping by district.The experimental group received breastfeeding intervention for mothers of infants with CHD on the basis of routine care;the control group received routine care and basic disease education and breastfeeding counselling.At 1 and 3 months of the infants with CHD,the 2 groups were compared in terms of exclusive breastfeeding rate,daily human milk as a percentage of total feeds,weight for age Z-score and height for age Z-score.Results A total of 1 round of expert validation was conducted,with a valid questionnaire recovery rate of 100％and an expert authority coefficient of 0.94.The breastfeeding intervention programme for mothers of infants with CHD is a comprehensive approach that encompasses 3 key dimensions,including capability,opportunity,and motivation.The programme is designed to enhance mothers'ability to recognize feeding signals,master breastfeeding techniques,and maintain successful breastfeeding practices.It also addresses common breastfeeding challenges and provides effective solutions.Additionally,it includes strategies for breastmilk management and fosters family support for breastfeeding.The programme offers medical information support on breastfeeding and aims to elevate mothers'understanding of its benefits.It involves systematic monitoring and recording of breastfeeding volumes,as well as thorough assessments and guidance on infant growth and development,including but not limited to the 10 specific entries related to the infant's progress.The results of the generalized estimating equations and repeated measures analysis of variance revealed statistically significant between-group differences(P＜0.05)in the rates of exclusive breastfeeding,the proportion of daily human milk as a percentage of total feeds,and the weight-for-age(WAZ)and height-for-age(HAZ)z-scores of infants across the 2 groups at various time points.The results of simple effect analysis showed that the differences in exclusive breastfeeding rate,the proportion of daily human milk as a percentage of total feeds,WAZ and HAZ between the 2 groups before intervention were not statistically significant(P＞0.05).At the age of 3 months of the infants after the intervention,the rate of exclusive breastfeeding in the experimental group was higher than that in the control group,and the difference was statistically significant(P=0.003).The proportion of daily human milk as a percentage of total feeds for infants in the intervention group at 1 and 3 months of age was higher than that in the control group,with a statistically significant difference(P＜0.05).At 3 months of age,WAZ of the experimental group was higher than that of the control group,and the difference was statistically significant(P=0.037);HAZ of the experimental group was higher than that of the control group at 1 and 3 months of age after intervention,and the difference was statistically significant(P＜0.05).Conclusion The breastfeeding intervention programme for mothers of infants with CHD,constructed on the basis of the COM-B model in this study,was comprehensive,feasible and acceptable.Implementation of the programme has potential positive effects on increasing exclusive breastfeeding rates and the daily human milk as a percentage of total feeds,and may also have a positive impact on WAZ and HAZ of infants.

自噬是细胞内一种高度保守的生理过程，可通过溶酶体系统降解过量或受损的细胞器、有毒的蛋白聚集体和病原体等。最新研究表明，海马钙素样1（HPCAL1）可作为特异性自噬受体和铁死亡的正调节因子。HPCAL1可选择性降解钙粘素2（CDH2），加速脂质过氧化，促进癌细胞铁死亡。iHPCAL1是抑制HPCAL1的小分子化合物，可抑制Erastin诱导的肿瘤细胞铁死亡。此外，它还可以抑制铁死亡诱导的急性胰腺炎。本文通过对HPCAL1在铁死亡中的具体作用机制进行概述，为HPCAL1作为铁死亡相关疾病的潜在治疗靶点提供新思路和理论依据。
Ferroptosis ; Cell Line, Tumor ; Autophagy

Objective:To investigate the relationship between job burnout, job stress, and depression in perimenopausal women.Methods:A total of 1 208 postmenopausal women from Wenzhou were randomly selected from August 2020 to August 2022 as research subjects for this study. Job burnout, job stress, social support, and depression were measured using the personal general condition questionnaire, the Chinese community nurse stress scale, Maslach Burnout Inventory-General Survey, Center for Epidemiologic Studies Depression Scale. The Pearson correlation coefficient was used to analyze the correlation between job burnout, job stress, and depression.Results:The scores of the Chinese community nurse stress scale and Maslach Burnout Inventory-General Survey in married people were (47.54 ± 4.54) points and (34.69 ± 4.12) points, respectively, which were significantly lower than those in unmarried, divorced, separated, and widowed people ( F = 9.49, 51.86, all P < 0.001). The Social Support Questionnaire score in married people was (33.28 ± 8.94) points, which was significantly higher than that in unmarried, divorced, separated, or widowed people ( F = 17.55, P < 0.001). There was no significant difference in the Center for Epidemiologic Studies Depression Scale score among married, unmarried, divorced,separated, or widowed people (all P > 0.05). The scores for the Chinese community nurse stress scale, Maslach Burnout Inventory-General Survey, Center for Epidemiologic Studies Depression Scale, and Social Support Questionnaire in people who received middle school-level education were (55.41 ± 6.15) points, (44.31 ± 6.13) points, (21.24 ± 4.11) points, and (40.76 ± 10.44) points, respectively, which were significantly higher than those in people who received education of primary school and below, college and above ( F = 147.29, 106.46, 70.14, 13.08, all P < 0.001). The scores of stressors for individuals engaged in other non-service professions, the score of the Maslach Burnout Inventory-General Survey, and the score of the Center for Epidemiologic Studies Depression Scale were (44.21 ± 3.84) points, (37.78 ± 5.24) points, (17.53 ± 3.42) points, and (34.27 ± 6.97) points, respectively, which were significantly lower than that of stressors for individuals engaged in nursing, teaching, and other service professions ( F = 207.75, 102.47, 37.24, 137.35, all P < 0.001). The results of correlation analysis showed that the score of the stressor scale was positively correlated with the scores of the Maslach Burnout Inventory-General Survey, Center for Epidemiologic Studies Depression Scale, and Social Support Questionnaire ( r = 0.58, 0.62, 0.52, all P < 0.05). The score of the Maslach Burnout Inventory-General Survey was positively correlated with the scores of the Center for Epidemiologic Studies Depression Scale and Social Support Questionnaire ( r = 0.59, 0.68, both P < 0.05). The score of the depression scale was positively correlated with the score of the Social Support Questionnaire ( r = 0.65, P < 0.05). Conclusion:Job burnout and job stress are positively correlated with depression and related to perimenopausal women's marital status, social support, occupation, and education level.

Objective:To investigate the therapeutic efficacy and prognosis of multiple myeloma (MM) patients with early relapse and the influencing factors of early relapse.Methods:The clinical data of 164 patients with newly diagnosed MM admitted to Affiliated Hospital of Hebei University from January 2018 to January 2021 were retrospectively analyzed, and 53 cases (32.3%) relapsed at the end of the follow-up. According to the recurrence within 12 months or not, the patients were divided into early relapse group and advanced relapse group; the clinical characteristics, overall response rate (ORR) and overall survival (OS) of both groups were compared. Logistic regression was used to analyze if the following indexes including age, gender, albumin, lactate dehydrogenase (LDH), β 2-microglobulin (β 2-MG), hemoglobin, creatinine, serum calcium, bone marrow plasma cell ratio, extramedullary disease, high-risk fluorescent in situ hybridization (FISH) were the influencing factors of the early relapse. Based on 7 published clinical trials, simplified early relapse MM (S-ERMM) scoring system was constructed to subgroup all relapsed patients. The difference in risk stratification between early relapsed patients and advanced relapsed patients was compared. Results:The median follow-up time of 164 newly diagnosed MM patients was 26 months (12-48 months). Among 53 relapsed MM patients, 24 cases had early relapse and 29 cases had advanced relapse. The ORR of patients with early relapse was decreased compared with that of those with advanced relapse [70.8% (17/24) vs. 89.7% (26/29), χ2 = 3.04, P = 0.001]. The median OS of the early relapse group was shorter than that of the advanced relapse group (24 months vs. not reached, P < 0.001). The OS of patient in the early relapse group with the best response ≥ complete remission (CR), ≥ very good partial remission (VGPR) and ≥ partial remission (PR) during initial induction therapy was worse than that of those in the advanced relapse group, and the differences were statistically significant ( P values were 0.008, 0.011, 0.012, respectively). Multivariate Logistic regression analysis showed low albumin (<35 g/L vs. ≥35 g/L: OR = 1.644, 95% CI 1.076-2.511, P = 0.022) and high LDH (< the upper limit of normal value vs. ≥ the upper limit of normal value: OR = 0.998, 95% CI 0.985-1.011, P = 0.030) were independent influencing factors of early relapse. Among 24 early relapse patients, there were 5 cases (20.8%), 13 cases (54.2%), 6 cases (25.0%), respectively in the S-ERMM scoring system low-risk, middle-risk, high-risk groups; among 29 advanced relapse patients, there were 18 cases (62.1%),9 cases (31.0%), 2 cases (6.9%), respectively in the S-ERMM scoring system low-risk, middle-risk, high-risk groups; the difference in risk stratification of the S-ERMM scoring system between the early relapse group and the advanced relapse group was statistically significant ( χ2 = 9.09, P = 0.003). Conclusions:MM patients with early relapse have poor therapeutic efficacy and prognosis. The prognosis is not affected by the depth of remission to first-line therapy. Low albumin and high LDH may be independent risk factors of MM patients with early relapse.

Objective:To explore the efficacy of venetoclax plus azacitidine (VA) in the treatment of patients with newly diagnosed chronic myelomonocytic leukemia (CMML).Methods:The clinical data of 4 newly diagnosed CMML-2 patients treated with VA regimen in the Affiliated Hospital of Hebei University from February 2022 to March 2023 were retrospectively analyzed, and the related literature was reviewed.Results:All 4 CMML-2 patients achieved the effect of ≥ partial bone marrow remission (PMR) after 1 course of treatment, and with the deepened extension of treatment course, the overall response rate and complete remission (CR) rate was 100% and 50%, respectively. In terms of dose adjustment, the dose and usage day of venetoclax were determined by using dynamic frailty assessment and adverse events. Among the 2 patients who achieved CR, 1 patient initially received venetoclax 200 mg for 14 days, and 1 patient received venetoclax 400 mg for 28 days and then the usage reduced to venetoclax 200 mg for 14 days due to hematological adverse events. All 4 patients maintained CR status. The most common grade 3 and 4 adverse events were neutropenia and thrombocytopenia.Conclusions:The first-line application of VA regimen in the treatment of newly diagnosed CMML-2 patients may achieve faster remission and better safety compared with traditional HMA monotherapy.

Objective Interleukin 6(IL-6)is an important cytokine featuring pleiotropic activity in the cardiac microenviron-ment and improves cardiomyocyte regeneration to favor cardiac repair after injury.Thereby this study aimed to investigate the effects of IL-6 on murine embryonic cardiomyocyte proliferation and unveil the possible developmental changes.Methods Single cardiomyocyte was obtained from mouse through enzyme dissociation at the early embryonic developmental stage(EDS)and late embryonic developmental stage(LDS).The cells were cultured for 24 h in 20％DMEM and then treated with 10 ng/mL IL-6 or 50 ng/mL IL-6 for 24 h.The effect of IL-6 on cardiomyocyte proliferation was comprehensively studied by tracing BrdU+α-acti-nin+nuclei and BrdU+α-actinin+mononuclear cardiomyocyte with immunofluorescence staining.Western blot was performed to unveil the changes in phosphorylation levels of the key proteins under IL-6 treatments.Results The 10 ng/mL IL-6 did not change the proliferation capacity of EDS myocardium,but significantly promoted the proliferation capacity of LDS myocardi-um.The 50 ng/mL IL-6 significantly inhibited the proportion of EDS BrdU+α-actinin+monocytes,and increased the proportion of LDS BrdU+α-actinin+nuclei and BrdU+α-actinin+monocytes.This might be related to the various effects of IL-6 at differ-ent concentrations on signal transduction pathways of EDS and LDS cardiomyocytes:10 ng/mL IL-6 and 50 ng/mL IL-6 inhibi-ted the phosphorylation of Akt in EDS cardiomyocytes,and had no significant effect on the phosphorylation of STAT3 and ERK.In LDS,10 ng/mL IL-6 and 50 ng/mL IL-6 inhibited phosphorylation of Akt and increased the phosphorylation of STAT3,but had no significant effect on the phosphorylation of ERK.Finally,the basic phosphorylation states of these three proteins were detected and the total protein amount and phosphorylation levels of STAT3,Akt and ERK showed a development-dependent decline.Conclusion IL-6 influenced the fetal cardiomyocyte proliferation in a developmental-and dose-dependent manner.It might be related to the developmental changes in the basic status of the target signaling pathways.These observa-tions provided new knowledge of cardiac proliferation and might propose new clues for the microenvironment in cell therapy.

NKX6-2 encodes a transcription factor involving the genesis and development of oligodendrocytes.Mutations in the NKX6-2 gene are related to a severe variant of hypomyelinating leukodystrophy, namely the NKX6-2-related spastic ataxia type 8 (SPAX8). As of March 24, 2020, there are 36 cases of SPAX8 involving 11 mutations of the NKX6-2 gene, which has not been reported in Chinese population.This article for the first time reported a 31-month-old Chinese boy diagnosed as SPAX8 caused by a novel mutation of the NKX6-2 gene at c. 234dup(p.Leu79Alafs*? ) admitted to the Department of Rehabilitation, Guangdong Women and Children Hospital in October 2019 retrospectively.He presented severe global psychomotor delay, spasticity, nystagmus, hearing impairment and trichiasis, ametropia of both eyes, and retinal degeneration of the right eye, which have not been reported in previous cases.His elder sister presented relevant manifestations, but did not perform the genetic testing.Through this case report and literature review, the genotype and phenotype of SPAX8 were expanded.