Objective:To analyze the clinical features，diagnosis，treatment and prognosis of children with juvenile dermatomyositis(JDM) complicated with interstitial lung disease(ILD).Methods:The clinical manifestations，laboratory examination，treatment and prognosis of 7 children with JDM-ILD who were hospitalized in the Department of Nephrology and Immunology，Women and Children's Hospital Affiliated to Qingdao University from December 2019 to December 2023 were retrospectively analyzed.Results:Among the 7 cases，4 were male and 3 were female.The age of onset was 1.8-10.0 years（mean age 5.6 years），the occurrence time of pulmonary involvement was 0.6-4.0 months（mean time 2.0 months），and the follow-up time was 1.8-4.0 years.All the 7 cases had typical rash and different degrees of myasthenia.Four cases were accompanied by skin mucosal ulceration and 4 cases had fever during the course of the disease.Of the 7 cases，2 were accompanied by macrophage activation syndrome，and 1 of them had nervous system involvement，including convulsion and coma.All the children had increased creatase of varying degrees，and only 1 case had increased creatine kinase.Five cases had positive anti- melanoma differentiation-associated gene 5（MDA5）antibody and 4 cases had positive anti- Ro-52 antibody.Interleukin-6 was increased in 5 cases，interferon-γ was increased in 3 cases，and tumor necrosis factor-α was increased in 2 cases.Electromyography showed myogenic injury，MRI showed different degrees of myositis.Chest high-resolution CT showed ground glass shadow，rope shadow，consolidation shadow，pleural thickening，mesh shadow，etc.Four cases had limited lung function or mixed ventilation function restriction.All 7 cases received methylprednisolone pulse treatment combined with immunosuppressant treatment，and 5 cases received immunoglobulin treatment.Pulmonary lesions improved in 5 cases and partially improved in 1 case.One case died due to macrophage activation and multiple organ failure.Conclusion:The respiratory symptoms of JDM-ILD are obscure，and the incidence of ILD is high in children with anti-MDA5 antibody positive.High-resolution CT contributes to early diagnosis.Reasonable early application of glucocorticoid and immunosuppressants could improve the survival rate and quality of life.

Objective:To analyze the clinical features，diagnosis，treatment and prognosis of children with juvenile dermatomyositis(JDM) complicated with interstitial lung disease(ILD).Methods:The clinical manifestations，laboratory examination，treatment and prognosis of 7 children with JDM-ILD who were hospitalized in the Department of Nephrology and Immunology，Women and Children's Hospital Affiliated to Qingdao University from December 2019 to December 2023 were retrospectively analyzed.Results:Among the 7 cases，4 were male and 3 were female.The age of onset was 1.8-10.0 years（mean age 5.6 years），the occurrence time of pulmonary involvement was 0.6-4.0 months（mean time 2.0 months），and the follow-up time was 1.8-4.0 years.All the 7 cases had typical rash and different degrees of myasthenia.Four cases were accompanied by skin mucosal ulceration and 4 cases had fever during the course of the disease.Of the 7 cases，2 were accompanied by macrophage activation syndrome，and 1 of them had nervous system involvement，including convulsion and coma.All the children had increased creatase of varying degrees，and only 1 case had increased creatine kinase.Five cases had positive anti- melanoma differentiation-associated gene 5（MDA5）antibody and 4 cases had positive anti- Ro-52 antibody.Interleukin-6 was increased in 5 cases，interferon-γ was increased in 3 cases，and tumor necrosis factor-α was increased in 2 cases.Electromyography showed myogenic injury，MRI showed different degrees of myositis.Chest high-resolution CT showed ground glass shadow，rope shadow，consolidation shadow，pleural thickening，mesh shadow，etc.Four cases had limited lung function or mixed ventilation function restriction.All 7 cases received methylprednisolone pulse treatment combined with immunosuppressant treatment，and 5 cases received immunoglobulin treatment.Pulmonary lesions improved in 5 cases and partially improved in 1 case.One case died due to macrophage activation and multiple organ failure.Conclusion:The respiratory symptoms of JDM-ILD are obscure，and the incidence of ILD is high in children with anti-MDA5 antibody positive.High-resolution CT contributes to early diagnosis.Reasonable early application of glucocorticoid and immunosuppressants could improve the survival rate and quality of life.

Objective:To investigate the expression of replication factor C5 (RFC5) in cervical cancer, and its effect on the prognosis and immune regulation as well as its related mechanism.Methods:RFC5 mRNA expression data and the clinical data of 280 cervical cancer patients were downloaded from the Cancer Genome Atlas (TCGA) database in May 2023. The difference of RFC5 mRNA expression between cervical cancer tissues and paracancerous normal tissues was compared and the expression of RFC5 mRNA in patients with different clinicopathological characteristics was analyzed. According to the median expression level of RFC5 mRNA in cancer tissues, 280 patients were divided into the high expression group and the low expression group. Kaplan-Meier method was used for survival analysis, and log-rank test was used for comparison. Gene Expression Profiling Interactive Analysis (GEPIA) 2 online tool was used to verify the relationship between RFC5 gene expression and the prognosis of cervical cancer. Univariate and multivariate Cox proportional risk models were used to analyze the factors influencing the overall survival (OS) of patients with cervival cancer in TCGA database. LinkedOmics database was used to screen the co-differentially expressed genes (DEG) related to RFC5 in cervical cancer. Gene Ontology (GO) and Kyoto Encyclopedia of Genesand Genomes (KEGG) pathway enrichment analysis of DEG related to RFC5 were performed based on the DAVID database. Based on the Tumor Immune Estimation Resource (TIMER) database, the relationship between RFC5 expression and tumor infiltrating immune cells in cervival cancer was analyzed by using Spearman method. The relationship between RFC5 expression and immunomodulatory factors in cervical cancer was analyzed based on tumor-immune system interactions database (TISIDB). The expression of RFC5 protein in cervical cancer tissues was analyzed based on Human Protein Atlas (HPA) database. The expression of RFC5 in pan-cancer and its correlation with OS were analyzed based on GEPIA2 online tool.Results:In TCGA database, the relative expression level of RFC5 mRNA in cervical cancer tissues (277 cases) was higher than that in paracancerous normal tissues (3 cases), and the difference was statistically significant ( P < 0.001), while there were no significant differences in the relative expression level of RFC5 mRNA in cancer tissues of patients with different age, pathological type and clinical staging (all P > 0.05). The OS of cervival cancer patients in high RFC5 expression group (139 cases) was better than that of patients in low RFC5 expression group (138 cases), and the difference was statistically significant ( P = 0.027). GEPIA tool verification indicated that expression of RFC5 in cervical cancer and its relationship with OS showed the same results. GO and KEGG enrichment analysis showed that RFC5-related genes were mainly involved in DNA replication, cell cycle, Fanconi anemia, mismatch repair, base excision repair, nucleotide excision repair and other signaling pathways. Multivariate Cox regression analysis showed that age ≥ 65 years, clinical staging Ⅳ and the low expression of RFC5 were independent risk factors of poor OS in patients with cervical cancer (all P < 0.05). TIMER database analysis showed that the expression of RFC5 was positively correlated with tumor purity ( rho = 0.198, P < 0.001), while weakly correlated or not correlated with the infiltration levels of B cells ( rho = 0.062, P = 0.306), CD8 + T cells ( rho = 0.168, P = 0.005), CD4 + T cells ( rho = -0.049, P = 0.418), macrophages ( rho = 0.034, P = 0.577), neutrophils ( rho = 0.169, P = 0.005) and bone marrow dendritic cells ( rho = 0.026, P = 0.667). Analysis of TISIDB data showed that RFC5 expression was mostly negatively correlated with immunosuppressive factors and immunostimulatory factors. HPA database showed that the expression level of RFC5 in cervical cancer tissues was higher than that in normal cervical tissues. GEPIA online tool database analysis showed that RFC5 expression was up-regulated in a variety of malignant tumors. The OS of thymoma patients with high RFC5 expression was better than that of those with low RFC5 expression, while the OS of acute myeloid leukemia patients with high RFC5 expression was worse than those with low RFC5 expression, and the differences in OS were statistically significant (both P < 0.05). Conclusions:RFC5 is highly expressed in cervical cancer and its expression is associated with prognosis of patients with cervival cancer. Overexpression of RFC5 may inhibit the expression of immunomodulatory factors, and it may regulate the development and progression of cervical cancer through DNA replication, cell cycle, mismatch repair, base excision repair and other related pathways.

OBJECTIVES: To investigate the correlation of serum levels of bridging integrating factor 1 (BIN1) with acute myocardial infarction (AMI) and Killip class of the patients. METHODS: We retrospectively collected the data from 94 patients with AMI and 30 healthy individuals for analysis of the correlations of serum BIN1 levels with Killip class, TIMI scores, and neutrophil-to-lymphocyte ratio (NLR). We also assessed the diagnostic value of BIN1 combined with NLR for AMI. RESULTS: Serum BIN1 levels were significantly lower in AMI patients than in the healthy individuals (P=0.032). The AMI patients with Killip class I had significantly lower serum BIN1 levels than the healthy individuals (P=0.008). Serum BIN1 level was an independent predictor of AMI with a predictive value of 0.630 (95% CI: 0.513-0.748) at the optimal cutoff level of 0.341 ng/mL, a specificity of 50%, and a sensitivity of 78.5%. Serum BIN1 level was also an independent predictor for Killip class I group in the AMI patients with a predictive value of 0.672 (95% CI: 0.548-0.797) at the optimal cutoff level of 0.287 ng/mL, a specificity of 74.1%, and a sensitivity of 60%. For AMI diagnosis, the combination of NLR and serum BIN1 level had a predictive value of 0.811 (95% CI: 0.727-0.895) at the optimal cutoff level of 0.548 ng/mL, with a specificity of 92.6% and a sensitivity of 62.2%. There was a positive correlation between serum BIN1 level and TIMI score in AMI patients (r=0.186, P=0.003). CONCLUSIONS BIN1 is correlated with AMI and can be helpful for predicting short-term prognosis of the patients, and BIN1 combined with NLR has a high diagnostic value for AMI.
Humans ; Myocardial Infarction/diagnosis* ; Tumor Suppressor Proteins/blood* ; Adaptor Proteins, Signal Transducing/blood* ; Retrospective Studies ; Nuclear Proteins/blood* ; Lymphocytes/cytology* ; Neutrophils/cytology* ; Female ; Male ; Prognosis ; Middle Aged

Objective:To explore the expression levels of adrenomedullin (ADM) in follicular fluid of patients with polycystic ovary syndrome (PCOS) and its correlation with ovarian function and inflammation.Methods:To conduct a cohort study, the data on infertile couples who received an antagonistic regimen of in vitro fertilization (IVF)/intracytoplasmic sperm injection (ICSI) to promote ovulation from March to December 2023 at the Reproductive Medicine Center of Shanxi Children's Hospital (Shanxi Maternal and Child Health Hospital) were collected. PCOS patients were selected as the PCOS group, and patients who underwent IVF/ICSI assisted pregnancy solely due to tubal and/or male factors during the same period were selected as control group. The general clinical data of two groups of patients were analyzed, and the expression of ADM, interleukin 1β (IL-1β), IL-18, transforming growth factor β (TGF-β) in the follicular fluid were compared between the two groups of patients. And taking the concentration of ADM in follicular fluid as the main research indicator, correlation and multiple linear regression analysis were conducted with other indicators. Simultaneously the ADM mRNA expression, cell cycle and cell apoptosis of granulosa cells were compared between the two groups. Results:This study included 20 cases in the PCOS group and 20 cases in control group. Compared with control group, the expression of ADM in follicular fluid and granulosa cells of patients with PCOS were significantly lower (both P<0.001), while its testosterone, the ratio of luteinizing hormone and follicle-stimulating hormone, antral follicle count (AFC), number of retrieved eggs, ovarian sensitivity index, as well as IL-1β, IL-18 and TGF-β in follicular fluid were higher and negatively correlated with ADM ( r=-0.37, P=0.019; r=-0.32, P=0.047; r=-0.50, P<0.001; r=-0.38, P=0.017; r=-0.38, P=0.016; r=-0.44, P=0.005; r=-0.37, P=0.018; r=-0.54, P<0.001). Multiple linear regression showed that AFC, gonadotropin initiation dose, IL-1β and TGF-β were the independent related factors that affect local ADM levels ( r=-0.37, P=0.008; r=-0.27, P=0.035; r=-0.28, P=0.028; r=-0.45, P<0.001). There was no statistically significant difference in the cell cycle of granulocytes between the two groups ( P>0.05), but the apoptosis rate (AR) of granulocytes was higher in the PCOS group than in control group (median AR in the PCOS group was 46.07%, median AR in control group was 28.57%, n=10, P=0.036). Conclusion:The decreased expression of ADM in follicles of PCOS patients is related to ovarian endocrine disorders, multiple vesicles, high ovarian responsiveness and local inflammatory status.

Objective:To explore the expression levels of adrenomedullin (ADM) in follicular fluid of patients with polycystic ovary syndrome (PCOS) and its correlation with ovarian function and inflammation.Methods:To conduct a cohort study, the data on infertile couples who received an antagonistic regimen of in vitro fertilization (IVF)/intracytoplasmic sperm injection (ICSI) to promote ovulation from March to December 2023 at the Reproductive Medicine Center of Shanxi Children's Hospital (Shanxi Maternal and Child Health Hospital) were collected. PCOS patients were selected as the PCOS group, and patients who underwent IVF/ICSI assisted pregnancy solely due to tubal and/or male factors during the same period were selected as control group. The general clinical data of two groups of patients were analyzed, and the expression of ADM, interleukin 1β (IL-1β), IL-18, transforming growth factor β (TGF-β) in the follicular fluid were compared between the two groups of patients. And taking the concentration of ADM in follicular fluid as the main research indicator, correlation and multiple linear regression analysis were conducted with other indicators. Simultaneously the ADM mRNA expression, cell cycle and cell apoptosis of granulosa cells were compared between the two groups. Results:This study included 20 cases in the PCOS group and 20 cases in control group. Compared with control group, the expression of ADM in follicular fluid and granulosa cells of patients with PCOS were significantly lower (both P<0.001), while its testosterone, the ratio of luteinizing hormone and follicle-stimulating hormone, antral follicle count (AFC), number of retrieved eggs, ovarian sensitivity index, as well as IL-1β, IL-18 and TGF-β in follicular fluid were higher and negatively correlated with ADM ( r=-0.37, P=0.019; r=-0.32, P=0.047; r=-0.50, P<0.001; r=-0.38, P=0.017; r=-0.38, P=0.016; r=-0.44, P=0.005; r=-0.37, P=0.018; r=-0.54, P<0.001). Multiple linear regression showed that AFC, gonadotropin initiation dose, IL-1β and TGF-β were the independent related factors that affect local ADM levels ( r=-0.37, P=0.008; r=-0.27, P=0.035; r=-0.28, P=0.028; r=-0.45, P<0.001). There was no statistically significant difference in the cell cycle of granulocytes between the two groups ( P>0.05), but the apoptosis rate (AR) of granulocytes was higher in the PCOS group than in control group (median AR in the PCOS group was 46.07%, median AR in control group was 28.57%, n=10, P=0.036). Conclusion:The decreased expression of ADM in follicles of PCOS patients is related to ovarian endocrine disorders, multiple vesicles, high ovarian responsiveness and local inflammatory status.

Objective To analyze the clinical and genetic features of immunodeficiency,centromeric instability,and facial anomalies (ICF) syndrome with a case report and literature review.Methods The clinical data and genetic test of a girl diagnosed with ICF syndrome in the Department of Nephrology and Immunology in Qingdao Women and Children's Hospital in December 2016 were extracted and analyzed."ICF syndrome" "immunodeficiency,centromeric instability and facial anomalies syndrome" "ICF syndrome and DNMT3B" were used as key words to search Chinese databases and Pubmed for literature until March 2018,and the literature was reviewed.Results A female patient aged 22 months old with ocular hypertelorism and low-set ears was admitted due to recurrent infection over one year.Laboratory tests showed humoral immune deficiency with IgG＜ 1.34 g/L,IgA＜0.060 g/L,and IgM＜0.179 g/L,but normal cellular immunity (total T lymphocyte 0.503,hepler T lymphocyte 0.328,cytotoxic T lymphocyte 0.166,natural killer cell 0.184,total B lymphocyte 0.276).Whole-exome sequencing revealed a de novo heterozygous splice site mutation c.922-2A＞G in intron 8,and a de novo heterozygous missense mutation c.2477G＞A in exon 23 of DNMT3B gene.Chromosome karyotype analysis showed 46,XX,with 64 out of 100 karyotypes showing centromere instability in chromosome 1.Five papers were found which were all in English,with total of 29 patients.Forty-three mutations were reported,including 34 missense,2 deletion,1 insertion,6 splice site mutations.Eleven patients had complex heterozygosis mutations.All patients had centromere instability,humoral immune deficiency and facial dysplasia which were mainly ocular hypertelorism and low-set ears.Most patients had language and motor development delay,and a few were combined with mental retardation.Conclusions ICF syndrome is a rare autosomal recessive primary immunodeficiency with classic clinical triad manifestations.De novo mutation of DNMT3B gene is one of etiologies according to genetic test.

Objective: To investigate the changes of brain gray matter volume in patients with occupational noise-induced hearing loss by voxel based morphometry (VBM) . Methods: 16 age-and education-matched healthy controls and 42 patients with occupational noise induced hearing loss, including 27 in mild group and 15 in severe group, received MRI 3D-FSPGR sequence T1WI sagittal scan, and then underwent VBM of brain gray matter volume data analysis. Results: The brain gray matter volume of the left occipitotemporal lateral gyrus, the anterior cingulate gyrus, the bilateral angular gyrus, the precuneus and the near midline area of cerebellum differed between experimental group and control group (P<0.01) . Conclusion The volume of gray matter in specific brain areas of patients with occupational noise-induced hearing loss was changed, and the effect of noise on brain structure was revealed from the perspective of imaging.

This article elaborated the connotation of public hospital medical insurance payment system and the importance of reform,summarizing and analyzing the practical exploration of promoting medical insurance payment system reform in the localities.Then it moved on to introduce new progress of medical insurance payment system reform abroad and the emerging mode of medical insurance payment, such as pay for performance,payment by results,and bundled payment.In the end,the authors put forward policy suggestions to improve medical insurance payment system at public hospitals in China, namely to build a modern healthcare payment system in line with the needs of medical service system.Such a system should be guided by comprehensive performance,restrained by cost budgeting, based on a diversified payment mode,and supported by information technology.In addition,it should have scientific payment standard and modern governance mechanism,and keep interactive development with commercial health insurance.

BACKGROUND:The repair method of proximal humeral comminuted fractures in the elderly remains controversial. OBJECTIVE:To compare the recovery of shoulder joint function using the hemiarthroplasty of shoulder and locking proximal humeral plate fixation for complex proximal humeral fracture in the elderly. METHODS:A total of 22 elderly patients with complex proximal humeral fracture, who were treated from October 2012 to October 2014, were retrospectively analyzed. 12 cases underwent hemiarthroplasty, and 10 cases received locking proximal humeral plate fixation. Al patients were folowed up after treatment. X-ray films were rechecked during the folow-up. The recovery of shoulder function was evaluated with Neer score. RESULTS AND CONCLUSION:During final folow-up, no prosthetic loosening or avascular necrosis of humeral head was found in the shoulder hemiarthroplasty group, and the Neer score was (81±5) points. In the locking proximal humeral plate fixation group, there was fixator loosening in four cases and avascular necrosis of humeral head in three cases, and the Neer score was (69±5) points (P < 0.05). During repair, mean operation time was shorter in the shoulder hemiarthroplasty group than in the locking proximal humeral plate fixation group. The amount of bleeding was higher in the shoulder hemiarthroplasty group than in the locking proximal humeral plate fixation group (P > 0.05). These data suggest that locking proximal humeral plate fixation and shoulder hemiarthroplasty are effective repair methods for complex proximal humerus fractures, but the hemiarthroplasty had the advantage of early functional exercise and good recovery of shoulder joint.