[Objective] To investigate and summarize the clinicopathological features, diagnosis, treatment and prognosis of adrenocortical carcinoma with rhabdoid features. [Methods] The clinical diagnosis and treatment of a case of adrenocortical carcinoma with rhabdoid features admitled to Department of Urology, Weifang People's Hospital were reported.The clinical manifestations, pathological features, diagnosis and prognosis of the disease were analyzed in combination with relevant literature. [Results] A 34-year-old male patient was admitted due to scrotal distension and pain that had persisted for 6 months.Imaging examination showed a huge soft tissue tumor in the left adrenal region of the retroperitoneum with compression displacement of the left kidney, leading to obstruction of venous return in the left spermatic vein, which in turn caused varicose veins.The levels of serum renin, angiotensin, aldosterone, cortisol, and catecholamine were within normal ranges.Surgical resection of the tumor was performed, and postoperative pathological examination revealed that the tumor tissue was predominantly composed of rhabdoid cells, exhibiting positive immunohistochemical staining for INI 1, Syn, Calretinin and Vimentin.Genetic testing did not identify any deletion of SMARCB1 and SMARCA4 mutations.Therefore, the diagnosis was adrenocortical carcinoma with rhabdoid features.At the current 20-month follow-up, no recurrence or metastasis was observed.A review of the literature found that only 7 cases of this disease had been reported. [Conclusion] Adrenocortical carcinoma with rhabdoid features is a rare disease, and a definitive diagnosis is dependent upon pathological examination.Surgical resection remains the primary treatment.Long-term follow-up is essential, and further research is needed to evaluate the impact of adjuvant therapy.

Objective: To investigate the effects of poor sitting postures on muscle fatigue in schoolage children, so as to provide evidence for developing healthy sitting guidelines. Methods: In May 2024,30 children aged 6-12 were recruited from Changping District in Beijing. A combination of surface electromyography (sEMG) and psychophysical scales were used to assess muscle fatigue at varying deviation angles under four sitting postures (forward head tilt, lateral head tilt, uneven shoulder height, and forward trunk inclination). Oneway ANOVA and LSD post hoc multiple comparisons were employed to analyze the differences in electromyographic (EMG) data among various deviation angles under different sitting postures. Results: The mean integrated electromyography (IEMG) of representative muscles showed statistically significant differences (P<0.05) across deviation angles in all postures, with muscle fatigue worsening as deviation angles increased. Forward head tilt:significant IEMG differences were observed in the left/right sternocleidomastoid and left/right splenius capitis (F=13.74, 13.21, 5.43, 6.11,P<0.05). Lateral head tilt:significant differences were found in the right sternocleidomastoid muscle (SCM), right splenius capitis, and left trapezius (F=5.13, 4.73, 12.13, P<0.05). Uneven shoulder height:significant differences occurred in the right SCM, bilateral splenius capitis, and left trapezius (F=12.46, 12.56, 32.49, 5.98, P<0.05).Forward trunk inclination, significant differences were identified in the left/right SCM, left/right splenius capitis, and left/right lumbar muscles (F=4.45, 9.84, 14.49, 26.44, 8.02, 18.34, P<0.01). Temporal analysis revealed varying fatigue onset times across postures:severe fatigue occurred earlier in lateral head tilt and forward trunk inclination, while excessive forward head tilt, lateral head tilt, and uneven shoulder postures predominantly induced mild to moderate fatigue. Conclusions Under different sitting postures, both the integrated IEMG of relevant muscles and subjective evaluations in schoolaged children increase with greater postural deviattion. Head tilt and trunkleaning postures require particular intervention, with emphasis on unevenshoulder alignment and trunk inclination control.

Diabetic cardiomyopathy (DCM) is a medical condition characterized by cardiac remodeling and dysfunction in individuals with diabetes mellitus. Sarcoplasmic reticulum (SR) and mitochondrial Ca²⁺ overload in cardiomyocytes have been recognized as biological hallmarks in DCM; however, the specific factors underlying these abnormalities remain largely unknown. In this study, we aimed to investigate the role of a cardiac-specific long noncoding RNA, D830005E20Rik (Trdn-as), in DCM. Our results revealed the remarkably upregulation of Trdn-as in the hearts of the DCM mice and cardiomyocytes treated with high glucose (HG). Knocking down Trdn-as in cardiac tissues significantly improved cardiac dysfunction and remodeling in the DCM mice. Conversely, Trdn-as overexpression resulted in cardiac damage resembling that observed in the DCM mice. At the cellular level, Trdn-as induced Ca²⁺ overload in the SR and mitochondria, leading to mitochondrial dysfunction. RNA-seq and bioinformatics analyses identified calsequestrin 2 (Casq2), a primary calcium-binding protein in the junctional SR, as a potential target of Trdn-as. Further investigations revealed that Trdn-as facilitated the recruitment of METTL14 to the Casq2 mRNA, thereby enhancing the m⁶A modification of Casq2. This modification increased the stability of Casq2 mRNA and subsequently led to increased protein expression. When Casq2 was knocked down, the promoting effects of Trdn-as on Ca²⁺ overload and mitochondrial damage were mitigated. These findings provide valuable insights into the pathogenesis of DCM and suggest Trdn-as as a potential therapeutic target for this condition.
Animals ; Diabetic Cardiomyopathies/pathology* ; RNA, Long Noncoding/genetics* ; Myocytes, Cardiac/metabolism* ; Mice ; Calsequestrin/genetics* ; Calcium/metabolism* ; Male ; Sarcoplasmic Reticulum/metabolism* ; Methyltransferases/metabolism* ; Mice, Inbred C57BL ; Mitochondria, Heart/metabolism* ; Disease Models, Animal ; Mitochondria/metabolism*

Ochratoxin A (OTA) is ubiquitous in the food and feed fields. It has strong hepatotoxicity and nephrotoxicity, seriously threatening the health of humans and animals. Enzymatic degradation of mycotoxins is considered to be a promising method to control mycotoxin contaminations. In this study, a new ochratoxin A amidohydrolase from Microbulbifer thermotolerans (MiADH) was obtained. After heterologous expression in Escherichia coli and purification, the recombinant protein was studied regarding the hydrolysis activity, hydrolysis products, enzymatic properties, and substrate binding mode. MiADH can degrade OTA into ochratoxin α (OTα) and phenylalanine, demonstrating a detoxifying ability. It demonstrated the best performance at 70 ℃ and pH 8.0, and Cu²⁺ had the strongest inhibitory effect on the activity of MiADH. MiADH with good thermal stability exhibited huge potential for industrial application. Rational design guided by three-dimensional structural models and substrate docking analysis revealed the important amino acids affecting substrate binding and obtained multiple mutants with improved activity. Among these mutants, V324A had the highest activity, which was 4.2-fold that of the wild type. The identification of MiADH enriches the ochratoxin A degradation enzyme library and provides a new candidate enzyme for the biological detoxification of ochratoxin A in the food and feed industry.
Amidohydrolases/chemistry* ; Ochratoxins/metabolism* ; Substrate Specificity ; Escherichia coli/metabolism* ; Recombinant Proteins/metabolism* ; Actinomycetales/genetics*

Objective:To investigate the effect of immune checkpoint inhibitors on reducing residual lymph node metastasis in patients with gastric cancer.Methods:The cohort of this retrospective study comprised patients from Nanfang Hospital of Southern Medical University and the First Affiliated Hospital of Xiamen University who had undergone systemic treatment prior to gastrectomy with D2 lymphadenectomy and had achieved Grade 1 primary tumor regression (TRG1) from January 2014 to December 2023. After exclusion of patients who had undergone preoperative radiotherapy, data of 58 patients (Nanfang Hospital: 46; First Affiliated Hospital of Xiamen University: 12) were analyzed. These patients were allocated to preoperative chemotherapy (Chemotherapy group, N=36 cases) and preoperative immunotherapy plus chemotherapy groups (Immunotherapy group, N=22 cases). There were no significant differences between these groups in sex, age, body mass index, diabetes, tumor location, pathological type, Lauren classification, tumor differentiation, pretreatment depth of invasion by primary tumor, pretreatment lymph node stage, pretreatment clinical stage, mismatch repair protein status, number of preoperative treatment cycles, or duration of preoperative treatment (all P>0.05). The primary outcome measure was postoperative lymph node downstaging. Secondary outcomes included postoperative depth of invasion by tumor, number of lymph nodes examined, and factors affecting residual lymph node metastasis status. Results:Lymph node downstaging was achieved significantly more often in the Immunotherapy group than the Chemotherapy group (pN0: 90.9% [20/22] vs. 61.1% [22/36]; pN1: 4.5% [1/22] vs. 36.1% [13/36]; pN2: 4.5% [1/22) vs. 0; pN3: 0 vs. 2.8% [1/36], Z=-2.315, P=0.021). There were no significant difference between the two groups in number of lymph nodes examined (40.5±16.3 vs. 40.8±17.5, t=0.076, P=0.940) or postoperative depth of invasion by primary tumor (pT1a: 50.0% [11/22] vs. 30.6% [11/36]; pT1b: 13.6% [3/22] vs. 19.4% [7/36]; pT2: 13.6% [3/22] vs. 13.9% [5/36]; pT3: 13.6% [3/22] vs. 25.0% [9/36]; pT4a: 9.1% [2/22] vs. 11.1% [4/36], Z=-1.331, P=0.183). Univariate analysis revealed that both preoperative treatment regimens were associated with residual lymph node metastasis status in patients whose primary tumor regression was TRG1 (χ 2=6.070, P=0.014). Multivariate analysis incorporated the following factors: pretreatment depth of invasion by primary tumor, pretreatment lymph node stage, pretreatment clinical stage, number of preoperative treatment cycles, and preoperative treatment duration. We found that a combination of immunotherapy and chemotherapy administered preoperatively was an independent protective factor for reducing residual lymph node metastases in study patients whose primary tumor regression was TRG1 (OR=0.147, 95%CI: 0.026–0.828, P=0.030). Conclusion:Compared with preoperative chemotherapy alone, a combination of preoperative immunotherapy and chemotherapy achieved greater reduction of residual lymph node metastases in the study patients who achieved TRG1 tumor regression in their primary lesions.

Objective:To investigate the effect of immune checkpoint inhibitors on reducing residual lymph node metastasis in patients with gastric cancer.Methods:The cohort of this retrospective study comprised patients from Nanfang Hospital of Southern Medical University and the First Affiliated Hospital of Xiamen University who had undergone systemic treatment prior to gastrectomy with D2 lymphadenectomy and had achieved Grade 1 primary tumor regression (TRG1) from January 2014 to December 2023. After exclusion of patients who had undergone preoperative radiotherapy, data of 58 patients (Nanfang Hospital: 46; First Affiliated Hospital of Xiamen University: 12) were analyzed. These patients were allocated to preoperative chemotherapy (Chemotherapy group, N=36 cases) and preoperative immunotherapy plus chemotherapy groups (Immunotherapy group, N=22 cases). There were no significant differences between these groups in sex, age, body mass index, diabetes, tumor location, pathological type, Lauren classification, tumor differentiation, pretreatment depth of invasion by primary tumor, pretreatment lymph node stage, pretreatment clinical stage, mismatch repair protein status, number of preoperative treatment cycles, or duration of preoperative treatment (all P>0.05). The primary outcome measure was postoperative lymph node downstaging. Secondary outcomes included postoperative depth of invasion by tumor, number of lymph nodes examined, and factors affecting residual lymph node metastasis status. Results:Lymph node downstaging was achieved significantly more often in the Immunotherapy group than the Chemotherapy group (pN0: 90.9% [20/22] vs. 61.1% [22/36]; pN1: 4.5% [1/22] vs. 36.1% [13/36]; pN2: 4.5% [1/22) vs. 0; pN3: 0 vs. 2.8% [1/36], Z=-2.315, P=0.021). There were no significant difference between the two groups in number of lymph nodes examined (40.5±16.3 vs. 40.8±17.5, t=0.076, P=0.940) or postoperative depth of invasion by primary tumor (pT1a: 50.0% [11/22] vs. 30.6% [11/36]; pT1b: 13.6% [3/22] vs. 19.4% [7/36]; pT2: 13.6% [3/22] vs. 13.9% [5/36]; pT3: 13.6% [3/22] vs. 25.0% [9/36]; pT4a: 9.1% [2/22] vs. 11.1% [4/36], Z=-1.331, P=0.183). Univariate analysis revealed that both preoperative treatment regimens were associated with residual lymph node metastasis status in patients whose primary tumor regression was TRG1 (χ 2=6.070, P=0.014). Multivariate analysis incorporated the following factors: pretreatment depth of invasion by primary tumor, pretreatment lymph node stage, pretreatment clinical stage, number of preoperative treatment cycles, and preoperative treatment duration. We found that a combination of immunotherapy and chemotherapy administered preoperatively was an independent protective factor for reducing residual lymph node metastases in study patients whose primary tumor regression was TRG1 (OR=0.147, 95%CI: 0.026–0.828, P=0.030). Conclusion:Compared with preoperative chemotherapy alone, a combination of preoperative immunotherapy and chemotherapy achieved greater reduction of residual lymph node metastases in the study patients who achieved TRG1 tumor regression in their primary lesions.

Objective:To analyze the clinicopathological features of solitary papillary thyroid isthmus carcinoma (SPTIC) and the therapeutic effect and prognosis of different surgical methods.Methods:A total of 161 patients with SPTIC who underwent surgical treatment in the Department of Thyroid Surgery of the First Hospital of Jilin University from Dec. 2012 to Oct. 2021 were selected. Gender, age, body mass index and pathology of the patients were collected and the clinicopathological characteristics of SPTIC were analyzed. They were divided into three groups according to different surgical methods: group A underwent isthmic excision, group B underwent extended isthmic excision, and group C underwent total thyroidectomy. There were 47 patients in group A (8 males and 39 females with an average age of 42.6±9.1 years), 50 patients in group B (11 males and 39 females with an average age of 45.3±11.3 years), and 64 patients in group C (10 males and 54 females with an average age of 46.9±11.4 years). The clinicopathological features and therapeutic effect of the three groups were compared by ANOVA, multiple local rank sum test, χ2 test or Fisher's exact probability method, and the recurrence rate and recurrence free survival (RFS) of the three groups were compared after a follow-up of 6 to 126 months. Results:Among the 161 patients with SPTIC, 132 (82.0%) were female, 130 (80.7%) were younger than 55 years old, BMI (25.1±3.6) kg/m 2, 124 (77.0%) were combined with capsule invasion. There were 53 cases (32.9%) with central lymph node metastasis (CLNM). Subgroup analysis showed that the proportion of males in patients with tumor diameter > 1 cm, the proportion of patients with BMI≥26.0 kg/m 2, the rate of capsular invasion, the rate of extrandular invasion and the rate of CLNM were higher (30.0% vs. 14.0%, P=0.023; 60.0% vs. 33.9%, P=0.004; 97.5% vs. 70.2%, P< 0.001; 42.5% vs. 9.9%, P<0.001; 50.0% vs. 27.3%, P=0.008) ; The CLNM rate was higher in male patients with BMI≥26.0 kg/m 2 and tumor diameter > 1 cm (28.3% vs. 13.0%, P=0.017; 52.8% vs. 34.3%, P=0.024; 37.7% vs. 18.5%, P=0.008). Compared with groups A and B, group C had longer hospitalization days, higher hospitalization costs, longer operation time, more postoperative drainage flow and higher incidence of postoperative hypoparathyroidism, and the differences were statistically significant (all P< 0.05). Groups A and B were similar in all aspects, with no statistical significance ( P> 0.05 for all). During the follow-up, 3 patients in both group A and B relapsed, while no patients in group C relapsed, and there were no statistically significant differences in the recurrence rate ( P=0.059) or RFS ( P=0.082) among the three groups. Conclusions:The majority of SPTIC were microcarcinomas, but the incidence of tumor combined with capsule invasion was higher, and the tumor size of more than 1 cm was more invasive (capsule invasion rate and extrathyroid invasion rate were higher). SPTIC should be treated as conservatively as possible (isthmic excision or enlarged isthmic excision) .

Objective:To examine the potential of combining the blood urea nitrogen(BUN)/albumin(ALB)ratio(BAR)with soluble growth stimulation expressed gene 2(sST2)as an early warning indicator for myocardial injury in elderly patients with sepsis in the emergency intensive care unit(EICU).Methods:The clinical data of elderly patients with sepsis admitted to the EICU of the Emergency Medicine Department at Harrison International Peace Hospital of Hebei Medical University from August 2018 to August 2022 were prospectively analyzed.The patients were divided into two groups based on the presence or absence of myocardial injury: the myocardial injury group and the non-myocardial injury group.The general clinical data and laboratory indexes of the two groups were compared, and the BAR was calculated.The correlation between BAR and myocardial injury in elderly patients with sepsis was analyzed.Binary Logistic regression analysis was used to identify the risk factors for myocardial injury in elderly patients with sepsis in the EICU.MedCalc software was employed to determine the early warning value of the combined sST2 and BAR for myocardial injury in elderly patients with sepsis in the EICU.Results:A total of 165 cases were analyzed, with 106 cases(64.24%)showing myocardial injury.It was found that elderly sepsis patients with lung and abdominal infection were more likely to experience myocardial injury( P<0.05 for all).In comparison to the group without myocardial injury, the levels of inflammatory markers such as white blood cell count(WBC), neutrophil to lymphocyte ratio(NLR), lactic acid, and procalcitonin(PCT), as well as combined markers BAR and sST2, were higher in elderly sepsis patients with myocardial injury upon admission.Correlation analysis results revealed significant positive correlations between BAR and lactic acid, PCT, and C-reactive protein(CRP)within 24 hours of admission to EICU in elderly sepsis patients with myocardial injury.Among these correlations, the strongest was observed between BAR and PCT( r=0.417, P<0.001).Additionally, BAR exhibited a positive correlation with acute physiology and chronic health evaluation scoring system(APACHEⅡ)scores( r=0.241, P=0.002).Furthermore, BAR showed positive correlations with myocardial injury markers sST2 and cTnI( r=0.327, 0.307, P<0.05 for all).Logistic regression analysis revealed that septic shock( OR=2.406, P=0.008), decreased left ventricular ejection fraction(EF)( OR=0.939, P=0.015), BAR( OR=2.205, P=0.044), lactic acid( OR=1.137, P=0.014), and sST2 elevation( OR=1.016, P=0.020)were identified as independent risk factors for predicting myocardial injury in elderly patients with sepsis.The results of ROC analysis indicated that BAR had a high early warning value for the occurrence of myocardial injury in elderly patients with EICU sepsis[area under curve(AUC)0.651, P<0.05], with an optimal cut-off value of 0.32(sensitivity 77.4%, specificity 60.2%).Furthermore, the combined detection of BAR and sST2 demonstrated a higher early warning value for the occurrence of myocardial injury in elderly patients with sepsis(AUC 0.697, P<0.05).The mortality rate of patients with myocardial injury below a cut-off value of 0.32 was 36.00%(9/25), while the mortality rate of patients with myocardial injury equal to or above 0.32 was 66.67%(54/81).The difference between the two groups was statistically significant( χ2=8.624, P=0.003). Conclusions:Both BAR and sST2 are considered independent risk factors for myocardial injury in elderly patients with sepsis.The combined detection of BAR and sST2 provides a more accurate prediction for the occurrence of myocardial injury in these patients.

Objective:To analyze the neurocognitive abnormalities and related emotional and behavioral problems in 410 adolescent patients with Turner syndrome (TS) managed in Henan Children′s Hospital in the past 5 years, and to explore the relationship between neurocognitive abnormalities and chromosome karyotype, pubertal development, hormone replacement therapy.Methods:A retrospective case series study.A total of 410 adolescent patients who were diagnosed with TS by karyotype or fluorescence in situ hybridization in the outpatient or inpatient Department of Endocrinology, Genetics and Metabolism at Henan Children′s Hospital from June 2018 to June 2023 were selected and divided into 2 groups according to age: < 12 years old and 12-18 years old.Neurocognitive assessments were performed based on the results of the Wechsler Intelligence Scale (4 th edition) for children and behavior scales for children, SPSS 22.0 software was used for data processing and statistical analysis, and chi-square test was used to analyze the correlation between chromosome karyotype, intelligence development level, pubertal development status, hormone therapy status and the occurrence of neuropsychiatric diseases. Results:Among the 410 TS patients, 207 cases had the karyotype of 45, X0/46, XX, accounting for 50.49%, 94 cases had the monosomic karyotype of 45, X0, accounting for 22.93%.Forty-six patients completed the Wechsler intelligence test, with the intelligence quotient (IQ) score ranging from 70 to 105, with high verbal comprehension and perceptual reasoning scores and low processing speed and working memory scores on all assessments.Fifty-two patients completed the hyperactivity scale assessment, and 43 cases had a predisposition to attention deficit hyperactivity disorder (ADHD).There were no significant differences in total IQ, perceptual reasoning and processing speed among the children with karyotype 45, X0, chimeric, and X chromosome structural abnormalities ( H=3.161, 1.955, 5.890, all P>0.05), while there were significant differences in verbal comprehension and working memory among the three groups ( H=7.697, 9.694, all P<0.05).Among TS patients 12-18 years old, 68 cases completed the depression scale self-assessment, of which 23 cases had depressive tendencies.There was no correlation between depressive tendency and chromosome karyotype, pubertal development and hormone replacement therapy ( P>0.05). Conclusions:TS patients generally have low intelligence levels and tend to have ADHD in childhood.TS patients in the pubertal development have a high incidence of depression.Pubertal development status and hormone replacement therapy show no correlation with the occurrence of neuropsychiatric diseases in TS patients.

ObjectiveTo investigate the role of traditional Chinese medicine （TCM） constitution in the progression of abnormal glucose metabolism among community population. MethodsA total of 393 community residents who participated in community diabetes screening from 2019 to 2021 and had complete physical examination data at baseline and at the 2nd year follow-up were selected. According to the results of glucose tolerance tests， community residents were divided into the abnormal glucose metabolism progression group and non-progression group， and were matched at the ratio of 1∶2 using the propensity score matching. The influencing factors related to diabetes at baseline were collected for both groups. The traditional Chinese medicine constitution was determined among population in two groups. The differences of influencing factors and traditional Chinese medicine constitution between the two groups were compared. Logistic regression analysis was used to analyze the risk factors for the progression of abnormal glucose metabolism. ResultsFinally， 131 patients were included in the progression group and 262 patients in the non-progression group. Compared to the non-progression group， the progression group had significantly higher proportion of hyperlipidemia history ， hyperglycemia history， phlegm-dampness constitution， and blood stasis constitution （P＜0.05 or P＜0.01）. Univariate logistic regression analysis revealed that the history of hyperlipidemia （P=0.011）， history of hyperglycemia （P＜0.001）， the family history of diabetes （P＜0.001）， yin-deficiency constitution （P=0.047）， phlegm-dampness constitution （P=0.011） and blood-stasis constitution （P=0.008） were risk factors for the progression of abnormal glucose metabolism. Multivariate logistic regression analysis indicated that the history of hyperglycemia （P＜0.001）， yin-deficiency constitution （P=0.026） and blood-stasis constitution （P=0.032） were independent risk factors for the progression of abnormal glucose metabolism. ConclusionThe history of hyperglycemia， yin-deficiency constitution and blood-stasis constitution may be the risk factors for the progression of abnormal glucose metabolism.