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1.
Zhonghua Fu Chan Ke Za Zhi ; 59(5): 346-352, 2024 May 25.
Article Zh | MEDLINE | ID: mdl-38797563

Objective: To analyze the incidence and clinical phenotype of the concomitant extragenital malformations in the patients with female reproductive tract anomalies. Methods: A retrospective study was conducted using clinical data of hospitalized patients diagnosed with uterine, cervical, or vaginal malformations from January 2003 to December 2022 in Peking Union Medical College Hospital. The malformations were classified according to American Society for Reproductive Medicine müllerian anomalies classification 2021, and in each type, the incidence and specific manifestations of concomitant extragnital malformations were analyzed. Results: A total of 444 patients were included. The overall incidence of concomitant extragenital malformations was 43.5% (193/444), including urinary system, skeletal system, and other system malformations. Renal malformations on the obstructed side were present in all patients with oblique vaginal septum syndrome (100.0%, 78/78). The total incidence of concomitant extragnital malformations was as high as 8/11 in uterus didelphys, 43.5% (10/23) in unicornuate uterus, 33.6% (79/235) in Mayer-Rokitansky-Küster-Hauser syndrome, 18.8% (6/32) in septate uterus and 18.5% (12/65) in cervical agenesis. Urinary system malformations (30.6%, 136/444) and skeletal system malformations (13.5%, 60/444) were the most common concomitant malformations in all types, in which, unilateral renal agenesis and scoliosis were the most common. Conclusions: Urinary and skeletal system malformations are important features of female reproductive tract anomalies. Urologic ultrasonography and spinal roentgenogram are recommended for all patients with female reproductive tract anomalies.


Abnormalities, Multiple , Mullerian Ducts , Urogenital Abnormalities , Uterus , Vagina , Humans , Female , Retrospective Studies , Urogenital Abnormalities/epidemiology , Uterus/abnormalities , Vagina/abnormalities , Mullerian Ducts/abnormalities , Incidence , Abnormalities, Multiple/epidemiology , 46, XX Disorders of Sex Development/epidemiology , Kidney/abnormalities , Cervix Uteri/abnormalities , Cervix Uteri/pathology , Genitalia, Female/abnormalities , China/epidemiology , Congenital Abnormalities/epidemiology , Adult
2.
Zhonghua Zhong Liu Za Zhi ; 46(5): 399-408, 2024 May 23.
Article Zh | MEDLINE | ID: mdl-38742353

Objectives: To investigate the effect of the expression of low-density lipoprotein receptor associated protein (LDLR) on the vascular abnormalities in hepatocellular carcinoma (HCC) and its mechanisms. Methods: Based on the information of Oncomine Cancer GeneChip database, we analyzed the correlation between the expression level of LDLR and the expression level of carcinoembryonic antigen (CEA) and CD31 in hepatocellular carcinoma tissues. Lentiviral transfection of short hairpin RNA target genes was used to construct LDLR-knockdown MHCC-97H and HLE hepatocellular carcinoma cells. The differential genes and their expression level changes in LDLR-knockdown hepatocellular carcinoma cells were detected by transcriptome sequencing, real-time fluorescence quantitative polymerase chain reaction, and protein immunoblotting. The gene-related signaling pathways that involve LDLR were clarified by enrichment analysis. The effect of LDLR on CEA was assessed by the detection of CEA content in conditioned medium of hepatocellular carcinoma cells. Angiogenesis assay was used to detect the effect of LDLR on the angiogenic capacity of human umbilical vein endothelial cells, as well as the role of CEA in the regulation of angiogenesis by LDLR. Immunohistochemical staining was used to detect the expression levels of LDLR in 176 hepatocellular carcinoma tissues, and CEA and CD31 in 146 hepatocellular carcinoma tissues, and analyze the correlations between the expression levels of LDLR, CEA, and CD31 in the tissues, serum CEA, and alanine transaminase (ALT). Results: Oncomine database analysis showed that the expressions of LDLR and CEA in the tissues of hepatocellular carcinoma patients with portal vein metastasis were negatively correlated (r=-0.64, P=0.001), whereas the expressions of CEA and CD31 in these tissues were positively correlated ( r=0.46, P=0.010). The transcriptome sequencing results showed that there were a total of 1 032 differentially expressed genes in the LDLR-knockdown group and the control group of MHCC-97H cells, of which 517 genes were up-regulated and 515 genes were down-regulated. The transcript expression level of CEACAM5 was significantly up-regulated in the cells of the LDLR-knockdown group. The Gene Ontology (GO) function enrichment analysis showed that the differential genes were most obviously enriched in the angiogenesis function. The Kyoto Encyclopedia of Genes and Genomes (KEGG) signaling pathway enrichment analysis showed that the relevant pathways involved mainly included the cellular adhesion patch, the extracellular matrix receptor interactions, and the interactions with the extracellular matrix receptors. The CEA content in the conditioned medium of the LDLR-knockdown group was 43.75±8.43, which was higher than that of the control group (1.15±0.14, P<0.001). The results of angiogenesis experiments showed that at 5 h, the number of main junctions, the number of main segments, and the total area of the lattice formed by HUVEC cells cultured with the conditioned medium of MHCC-97H cells in the LDLR-knockdown group were 295.3±26.4, 552.5±63.8, and 2 239 781.0±13 8211.9 square pixels, which were higher than those of the control group (113.3±23.5, 194.8±36.5, and 660 621.0±280 328.3 square pixels, respectively, all P<0.01).The number of vascular major junctions, the number of major segments, and the total area of the lattice formed by HUVEC cells cultured in conditioned medium with HLE cells in the LDLR-knockdown group were 245.3±42.4, 257.5±20.4, and 2 535 754.5±249 094.2 square pixels, respectively, which were all higher than those of the control group (113.3±23.5, 114.3±12.2, and 1 565 456.5±219 259.7 square pixels, respectively, all P<0.01). In the conditioned medium for the control group of MHCC-97H cells,the number of main junctions, the number of main segments, and the total area of the lattice formed by the addition of CEA to cultured HUVEC cells were 178.9±12.0, 286.9±12.3, and 1 966 990.0±126 249.5 spixels, which were higher than those in the control group (119.7±22.1, 202.7±33.7, and 1 421 191.0±189 837.8 square pixels, respectively). The expression of LDLR in hepatocellular carcinoma tissues was not correlated with the expression of CEA, but was negatively correlated with the expression of CD31 (r=-0.167, P=0.044), the level of serum CEA (r=-0.061, P=0.032), and the level of serum ALT(r=-0.147,P=0.05). The expression of CEA in hepatocellular carcinoma tissues was positively correlated with the expression of CD31 (r=0.192, P=0.020). The level of serum CEA was positively correlated with the level of serum ALT (r=0.164, P=0.029). Conclusion: Knocking down LDLR can promote vascular abnormalities in HCC by releasing CEA.


Carcinoma, Hepatocellular , Liver Neoplasms , Neovascularization, Pathologic , Receptors, LDL , Humans , Liver Neoplasms/metabolism , Liver Neoplasms/genetics , Carcinoma, Hepatocellular/metabolism , Carcinoma, Hepatocellular/genetics , Carcinoma, Hepatocellular/pathology , Carcinoma, Hepatocellular/blood supply , Receptors, LDL/metabolism , Receptors, LDL/genetics , Cell Line, Tumor , Neovascularization, Pathologic/metabolism , Carcinoembryonic Antigen/metabolism , Carcinoembryonic Antigen/genetics , Human Umbilical Vein Endothelial Cells/metabolism , Signal Transduction , Gene Expression Regulation, Neoplastic , Gene Knockdown Techniques , Transcriptome , Platelet Endothelial Cell Adhesion Molecule-1/metabolism , Platelet Endothelial Cell Adhesion Molecule-1/genetics
3.
Zhonghua Yi Xue Za Zhi ; 104(11): 793-798, 2024 Mar 19.
Article Zh | MEDLINE | ID: mdl-38462357

The impact of human leukocyte antigen (HLA) on hematopoietic stem cell transplantation (HSCT) necessitates high precision in HLA genotyping. Confirmatory typing for patients and their related or unrelated donors before HSCT is critical. This study seeks to standardize HLA confirmatory typing in laboratories by examining the current state of HLA genotyping in the country, building upon the National Standards and Industrial Standards for HLA, and highlighting the significance of confirmatory typing for patients and potential donors prior to HSCT. A retrospective analysis over a decade reveals initial typing errors, indicating potential issues and critical considerations in pre-analytical, analytical, and post-analytical stages. Problems are attributed to three main causes: (1) random human errors, including technical mistakes, sample mix-up, and transcription inaccuracies; (2) limitations of technical methods, such as the varied sequence ranges between confirmatory and initial typing; (3) patient factors, involving high tumor burden, the influence of certain drugs on HLA genotyping results, and the second transplantation. Solutions are proposed for these problems, along with recommendations to standardize HLA confirmatory typing.


Hematopoietic Stem Cell Transplantation , Humans , Retrospective Studies , HLA Antigens , Histocompatibility Testing/methods , Tissue Donors , Histocompatibility Antigens Class I
4.
Zhonghua Yi Xue Za Zhi ; 104(11): 834-842, 2024 Mar 19.
Article Zh | MEDLINE | ID: mdl-38462359

Objective: To establish prediction models for human leukocyte antigen (HLA) haplotypes and HLA genotypes, and verify the prediction accuracy. Methods: The prediction models were established based on the characteristic of HLA haplotype inheritance and linkage disequilibrium (LD), as well as the invention patents and software copyrights obtained. The models include algorithm and reference databases such as HLA A-C-B-DRB1-DQB1 high-resolution haplotypes database, B-C and DRB1-DQB1 LD database, G group alleles table, and NMDP Code alleles table. The prediction algorithm involves data processing, comparison with reference data, filtering results, probability calculation and ranking, confidence degree estimation, and output of prediction results. The accuracy of the predictions was verified by comparing them with the correct results, and the relationship between prediction accuracy and the probability distribution and confidence degree of the predicted results was analyzed. Results: The HLA haplotypes and genotypes prediction models were established. The prediction algorithm included the prediction of A-C-B-DRB1-DQB1 haplotypes according to HLA-A, B, DRB1, C, DQB1 genotypes, the prediction of C and DQB1 high-resolution results according to A, B and DRB1 high-resolution results, and the prediction of A, B, DRB1, C and DQB1 high resolution results according to the A, B and DRB1 intermediate or low resolution results. Validation results of "Predicting A-C-B-DRB1-DQB1 haplotypes basing on HLA-A, B, DRB1, C, DQB1 genotypes" model: for 787 data, the accuracy was 94.0% (740/787) with 740 correct predictions, 34 incorrect predictions, and 13 instances with no predicted results. For 847 data, the accuracy was 100% (847/847). The 2 411 and 2 594 haplotype combinations predicted from 787 and 847 data were grouped according to confidence degree, the accuracy was 100% (48/48, 114/114) for a confidence degree of 1, 96.2% (303/315) and 97.8% (409/418) for a confidence degree of 2 respectively. Validation results of "Predicting A, B, DRB1 and C, DQB1 high-resolution genotypes basing on HLA-A, B, DRB1 high, intermediate, or low resolution genotypes" model: when predicting C and DQB1 high resolution genotypes basing on A, B, and DRB1 high resolution genotypes, 89.3% (1 459/1 634) of the predictions were correct. The accuracy for the top 2 predicted probability (GPP) ranking was 79.2% (1 156/1 459), and for the top 10, it was 95.0% (1 386/1 459). Furthermore, when GPP≥90% and GPP 50%-90%, the prediction accuracy was 81.3% (209/257) and 72.8% (447/614) respectively. The accuracy of predicting C and DQB1 high resolution genotypes basing on the results of A, B, and DRB1 high resolution genotypes from the China Marrow Donor Program was 87.0% (20/23). The accuracy of predicting A, B, DRB1, C, and DQB1 high resolution genotypes basing on the results of A, B, and DRB1 intermediate or low-resolution genotypes was 70.0% (7/10) and 52.5% (21/40) respectively. When predicting whether the patient is likely to have a HLA 10/10 matched donor, the accuracy of the top 2 GPP combinations with a proportion of ≥50% was 85.7% (6/7). Conclusions: When using A, B, DRB1, C, DQB1 genotypes to predict A-C-B-DRB1-DQB1 haplotype combinations, the results with a confidence degree of 1 and 2 are reliable. When predicting C and DQB1 genotypes according to A, B and DRB1 genotypes, the top 10 results ranked by GPP are reliable, and the top 2 results with GPP≥50% are more reliable.


HLA-B Antigens , HLA-C Antigens , Humans , Haplotypes , HLA-B Antigens/genetics , HLA-C Antigens/genetics , Gene Frequency , HLA-DQ beta-Chains/genetics , HLA-DRB1 Chains/genetics , Histocompatibility Antigens Class I/genetics , Genotype , HLA-A Antigens/genetics , Alleles
5.
Zhonghua Yi Xue Za Zhi ; 104(11): 857-864, 2024 Mar 19.
Article Zh | MEDLINE | ID: mdl-38462362

Objective: To establish the threshold value of human leukocyte antigen (HLA) mixed antigen reagent screening test results, and to verify it by HLA single antigen reagent confirmation test results. Methods: The results of 2 255 serum samples tested for HLA antibodies by HLA mixed antigen reagent in the department of HLA Laboratory, the First Affiliated Hospital of Soochow University from October 2017 to December 2021 were retrospectively analyzed. Among them, 1 139 samples were also tested by single antigen HLA Class-Ⅰ reagent and 1 116 samples were also tested by single antigen HLA Class-Ⅱ reagent. Based on the same antigens coated with both reagents, the Mean Fluorescence Intensity (MFI) and Nomalized Background ratio (NBG ratio) of 12 HLA Class-Ⅰ beads and 5 HLA Class-Ⅱ beads in the HLA mixed antigen reagent and the MFI of 77 anti-HLA class-Ⅰ antibodies and 35 anti-HLA class-Ⅱ antibodies detected by HLA single antigen reagent were recorded. The MFI and NBG ratio of HLA mixed antigen reagent beads in 1 139 or 1 116 samples were segmented according to the positive rate of antibodyies detected by the single antigen reagent corresponding to the antigens coated with each HLA mixed antigen reagent bead, and the results of the HLA mixed antigen screening test were verified by the HLA single antigen reagent confirmation test. Results: The threshold values of MFI and NBG ratio of HLA mixed antigen reagent's 17 beads were established. The MFI of No. 1 to No. 17 beads of HLA mixed antigen reagent ranged from 26.86 to 21 925.58, and the NBG ratio ranged from 0 to 434.65. According to the positive detection rate of HLA single antigen reagent corresponding to the coated antigens, the MFI and NBG ratio of the beads of HLA mixed antigen reagent were divided into positive interval, suspicious positive interval, suspicious negative interval and negative interval. The positive rates of anti-HLA class-Ⅰ antibodies by HLA mixed antigen reagent and single antigen HLA Class-Ⅰ reagent were 87.5% (997/1 139) and 66.3% (755/1 139). The positive rates of anti-HLA class-Ⅱ antibodies were 63.4% (707/1 116) and 44.9% (501/1 116). In the samples with suspicious negative, suspicious positive and positive results of HLA class-Ⅰ、Ⅱ antibodies detected by HLA mixed antigen reagent, the positive detection rates of single antigen HLA Class-Ⅰ reagent were 14.9% (17/114), 41.3% (145/351) and 91.3% (590/646), respectively. The positive detection rates of single antigen HLA Class-Ⅱ reagent were 15.5% (58/375), 26.5% (81/306) and 88.8% (356/401), respectively. Conclusions: In this study, the threshold values of MFI and NBG ratio of HLA mixed antigen reagent screening test are established, and the threshold values are verified by the results of HLA single antigen reagent confirmation test. HLA mixed reagent screening test can be used for screening of HLA antibodies, and if necessary, it should be combined with HLA single antigen confirmatory test for clinical detection of HLA antibodies.


HLA Antigens , Histocompatibility Antigens Class II , Humans , Indicators and Reagents , Retrospective Studies , Histocompatibility Testing/methods , Histocompatibility Antigens Class I , Isoantibodies , Graft Rejection
6.
Zhonghua Kou Qiang Yi Xue Za Zhi ; 59(3): 295-300, 2024 Mar 09.
Article Zh | MEDLINE | ID: mdl-38432665

The success of clear aligner treatment relies on rational treatment planning, favorable patients' compliance and high-quality clinical dental monitoring. High-quality clinical dental monitoring involves not only evaluation of real-time tooth position and treatment-designed tooth position, but also the assessment of the root-alveolar bone relationship. However, fluent imaging tests with ionizing radiation are not realistic in clinical practice. Regarding the rapid advances in digital techniques, biomechanism based method, image processing algorithm-based method and multimodal data fusion-based method all exhibit great potential in monitoring tooth movement. This paper introduced the progress and clinical application in those methods for clinicians' reference.


Orthodontic Appliances, Removable , Tooth Movement Techniques , Humans
7.
Bull Exp Biol Med ; 176(1): 19-25, 2023 Nov.
Article En | MEDLINE | ID: mdl-38087140

We studied the effect of TFP5 on MIN6 cells (cultured mouse islet ß cells) treated with different concentrations of glucose (5 or 25 mM). The results were verified in C57BL/6J mice (control; n=12) and db/db mice with type 2 diabetes mellitus (n=12). To synthesize TFP5, peptide p5 (a derivative of p35 protein, activator of cyclin-dependent kinase 5, Cdk5) was conjugated with a FITC tag at the N-terminus and an 11-amino acid TAT protein transduction domain at the C-terminus. TFP5 was employed to inhibit Cdk5 activity and then to evaluate its efficiency in treating experimental type 2 diabetes mellitus. TFP5 effectively inhibited the pathological hyperactivity of Cdk5, enhanced insulin secretion, and protected pancreatic ß cells from apoptosis in vitro and in vivo. In addition, TFP5 inhibited inflammation in pancreatic islets by reducing the expression of inflammatory cytokines TGF-ß1, TNFα, and IL-1ß. These novel data indicates that TFP5 is a promising candidate for treatment of type 2 diabetes mellitus.


Diabetes Mellitus, Type 2 , Insulin-Secreting Cells , Animals , Mice , Cyclin-Dependent Kinase 5/genetics , Cyclin-Dependent Kinase 5/metabolism , Diabetes Mellitus, Type 2/drug therapy , Glucose/toxicity , Glucose/metabolism , Insulin-Secreting Cells/metabolism , Mice, Inbred C57BL , Peptides/pharmacology , Nerve Tissue Proteins/metabolism , Nerve Tissue Proteins/pharmacology
8.
Cell Rep ; 42(11): 113364, 2023 11 28.
Article En | MEDLINE | ID: mdl-37922312

Erythro-myeloid progenitors of the yolk sac that originates during early embryo development has been suggested to generate tissue-resident macrophage, mast cell, and even endothelial cell populations from fetal to adult stages. However, the heterogeneity of erythro-myeloid progenitors (EMPs) is not well characterized. Here, we adapt single-cell RNA sequencing to dissect the heterogeneity of EMPs and establish several fate-mapping tools for each EMP subset to trace the contributions of different EMP subsets. We identify two primitive and one definitive EMP subsets from the yolk sac. In addition, we find that primitive EMPs are decoupled from definitive EMPs. Furthermore, we confirm that primitive and definitive EMPs give rise to microglia and other tissue-resident macrophages, respectively. In contrast, only Kit+ Csf1r- primitive EMPs generate endothelial cells transiently during early embryo development. Overall, our results delineate the contribution of yolk sac EMPs more clearly based on the single-cell RNA sequencing (scRNA-seq)-guided fate-mapping toolkit.


Endothelial Cells , Yolk Sac , Microglia , Myeloid Progenitor Cells , Sequence Analysis, RNA , Cell Lineage , Hematopoiesis/genetics
9.
Zhonghua Er Ke Za Zhi ; 61(11): 989-994, 2023 Nov 02.
Article Zh | MEDLINE | ID: mdl-37899338

Objective: To explore the clinical and imaging features of acute encephalopathy with biphasic seizures and late reduced diffusion(AESD) in children. Methods: For the case series study, 21 children with AESD from Peking University First Hospital, Provincial Children's Hospital Affiliated to Anhui Medical University, Children's Hospital of Fudan University, and Shanxi Children's Hospital who were diagnosed and treated from October 2021 to July 2023 were selected. Clinical data were collected to summarize their clinical information, imaging, and laboratory tests, as well as treatment and prognostic characteristics. Descriptive statistical analysis was applicated. Results: Of the 21 cases with AESD, 11 were males and 10 were females, with the age of onset of 2 years and 6 months (1 year and 7 months, 3 years and 6 months). Of the 21 cases, 18 were typical cases with biphasic seizures. All typical cases had early seizures within 24 hours before or after fever onset. Among them, 16 cases had generalized seizures, 2 cases had focal seizures, and 7 cases reached the status epilepticus. Of the 21 cases, 3 atypical cases had late seizures in biphasic only. The late seizures in the 21 cases occurred on days 3 to 9. The types of late seizures included focal seizures in 12 cases, generalized seizures in 6 cases, and both focal and generalized seizures in 3 cases. Diffusion-weighted imaging (DWI) test on days 3 to 11 showed reduced diffusion of subcortical white matter which was named "bright tree sign" in all cases. The diffuse cerebral atrophy predominantly presented in the front-parietal-temporal lobes was found in 19 cases between day 12 and 3 months after the onset of the disease. Among 21 cases, 20 had been misdiagnosed as autoimmune encephalitis, central nervous system infection, febrile convulsions, posterior reversible encephalopathy syndrome, acute disseminated encephalomyelitis, and hemiconvulsion-hemiplegia-epilepsy syndrome. All the cases received high-dose gammaglobulin and methylprednisolone pulse therapy with poor therapeutic effect. By July 2023, 18 cases were under follow-up. Among them, 17 cases were left with varying degrees of neurologic sequelae, including 11 cases with post-encephalopathic epilepsy; 1 recovered completely. Conclusions: AESD is characterized by biphasic seizures clinically and "bright tree sign" on DWI images. Symptomatic and supportive treatments are recommended. The immunotherapy is ineffective. The prognosis of AESD is poor, with a high incidence of neurological sequelae and a low mortality.


Brain Diseases , Posterior Leukoencephalopathy Syndrome , Seizures, Febrile , Status Epilepticus , Male , Female , Child , Humans , Infant , Child, Preschool , Posterior Leukoencephalopathy Syndrome/complications , Seizures/diagnostic imaging , Seizures/etiology , Brain Diseases/diagnostic imaging , Seizures, Febrile/diagnostic imaging
11.
Zhonghua Kou Qiang Yi Xue Za Zhi ; 58(9): 887-892, 2023 Sep 09.
Article Zh | MEDLINE | ID: mdl-37659845

The health of dental tissue is an important guarantee to obtain good orthodontic effect, and also the basis to achieve beauty, stability and function after orthodontic treatment. The classification of dental diseases is complex and diverse, which has different effects on orthodontic treatment. It is necessary to make clear diagnosis before orthodontic treatment, and take preventive and therapeutic measures according to the etiology and clinical symptoms, so as to achieve the goal of health treatment. Orthodontic treatment might cause a variety of changes in the teeth and pulp tissue. Such changes have certain rules and are complex and variable. Orthodontist should master relevant knowledge to minimize the side effects of orthodontic treatment.

12.
Zhonghua Er Ke Za Zhi ; 61(6): 515-519, 2023 Jun 02.
Article Zh | MEDLINE | ID: mdl-37312462

Objective: To analyze the short-time efficacy of empagliflozin in the treatment of glycogen storage disease type Ⅰb (GSD Ⅰb). Methods: In this prospective open-label single-arm study, the data of 4 patients were collected from the pediatric department in Peking Union Medical College Hospital from December 2020 to December 2022. All of them were diagnosed by gene sequencing and had neutropenia. These patients received empagliflozin treatment. Their clinical symptoms such as height and weight increase, abdominal pain, diarrhea, oral ulcer, infection times, and drug applications were recorded at 2 weeks, 1 month, 2 months, 3 months, 6 months, 9 months, 12 months, and 15 months after treatment to assess the therapeutic effect. The liquid chromatography-tandem mass spectrometry method was used to monitor the changes in 1, 5-anhydroglucitol (1, 5AG) concentration in plasma. At the same time, adverse reactions such as hypoglycemia and urinary tract infection were closely followed up and monitored. Results: The 4 patients with GSD Ⅰb were 15, 14, 4 and 14 years old, respectively at the beginning of empagliflozin treatment, and were followed up for 15, 15, 12 and 6 months, respectively. Maintenance dose range of empagliflozin was 0.24-0.39 mg/(kg·d). The frequency of diarrhea and abdominal pain decreased in cases 2, 3, and 4 at 1, 2 and 3 months of treatment, respectively. Their height and weight increased at different degrees.The absolute count of neutrophils increased from 0.84×109, 0.50×109, 0.48×109, 0.48×109/L to 1.48×109, 3.04×109, 1.10×109, 0.73×109/L, respectively. Granulocyte colony-stimulating factor was gradually reduced in 1 patients and stopped in 3 patient. Plasma 1, 5 AG levels in 2 children were significantly decreased after administration of empagliflozin (from 46.3 mg/L to 9.6 mg/L in case 2, and from 56.1 mg/L to 15.0 mg/L in case 3). All 4 patients had no adverse reactions such as hypoglycemia, abnormal liver or kidney function, or urinary system infection. Conclusion: In short-term observation, empagliflozin can improve the symptoms of GSD Ⅰb oral ulcers, abdominal pain, diarrhea, and recurrent infection, also can alleviate neutropenia and decrease 1, 5AG concentration in plasma, with favorable safety.


Glycogen Storage Disease Type I , Hypoglycemia , Neutropenia , Humans , Child , Child, Preschool , Adolescent , Prospective Studies , Glycogen Storage Disease Type I/drug therapy , Abdominal Pain , Diarrhea/drug therapy
13.
Clin Oncol (R Coll Radiol) ; 35(9): e516-e527, 2023 09.
Article En | MEDLINE | ID: mdl-37230875

AIMS: Endometrial cancer is one of the most widely known gynaecological malignancies that lacks a prognostic prediction model. This study aimed to develop a nomogram to predict progression-free survival (PFS) in patients with endometrial cancer. MATERIALS AND METHODS: Information for endometrial cancer patients diagnosed and treated from 1 January 2005 to 30 June 2018 was collected. The Kaplan-Meier survival analysis and multivariate Cox regression analysis were carried out to determine the independent risk factors and a nomogram was constructed by R based on analytical factors. Internal and external validation were then carried out to predict the probability of 3- and 5-year PFS. RESULTS: In total, 1020 patients with endometrial cancer were included in the study and the relationship between 25 factors and prognosis was analysed. Postmenopause (hazard ratio = 2.476, 95% confidence interval 1.023-5.994), lymph node metastasis (hazard ratio = 6.242, 95% confidence interval 2.815-13.843), lymphovascular space invasion (hazard ratio = 4.263, 95% confidence interval 1.802-10.087), histological type (hazard ratio = 2.713, 95% confidence interval 1.374-5.356), histological differentiation (hazard ratio = 2.601, 95% confidence interval 1.141-5.927) and parametrial involvement (hazard ratio = 3.596, 95% confidence interval 1.622-7.973) were found to be independent prognostic risk factors; these factors were selected to establish a nomogram. The consistency index for 3-year PFS were 0.88 (95% confidence interval 0.81-0.95) in the training cohort and 0.93 (95% confidence interval 0.87-0.99) in the verification set. The areas under the receiver operating characteristic curve for the 3- and 5-year PFS predictions are 0.891 and 0.842 in the training set; the same conclusion also appeared in the verification set [0.835 (3-year), 0.803(5-year)]. CONCLUSIONS: This study established a prognostic nomogram for endometrial cancer that provides a more individualised and accurate estimation of PFS for patients, which will help physicians make follow-up strategies and risk stratification.


Endometrial Neoplasms , Nomograms , Female , Humans , Progression-Free Survival , Prognosis , Risk Factors , Endometrial Neoplasms/pathology
14.
J Endocrinol Invest ; 46(9): 1843-1854, 2023 Sep.
Article En | MEDLINE | ID: mdl-37020103

OBJECTIVE: Silent corticotroph adenomas (SCAs) are a subtype of nonfunctioning pituitary adenomas that exhibit more aggressive behavior. However, rapid and accurate preoperative diagnostic methods are currently lacking. DESIGN: The purpose of this study was to examine the differences between SCA and non-SCA features and to establish radiomics models and a clinical scale for rapid and accurate prediction. METHODS: A total of 260 patients (72 SCAs vs. 188 NSCAs) with nonfunctioning adenomas from Peking Union Medical College Hospital were enrolled in the study as the internal dataset. Thirty-five patients (6 SCAs vs. 29 NSCAs) from Fuzhou General Hospital were enrolled as the external dataset. Radiomics models and an SCA scale to preoperatively diagnose SCAs were established based on MR images and clinical features. RESULTS: There were more female patients (internal dataset: p < 0.001; external dataset: p = 0.028) and more multiple microcystic changes (internal dataset: p < 0.001; external dataset: p = 0.012) in the SCA group. MRI showed more invasiveness (higher Knosp grades, p ≤ 0.001). The radiomics model achieved AUCs of 0.931 and 0.937 in the internal and external datasets, respectively. The clinical scale achieved an AUC of 0.877 and a sensitivity of 0.952 in the internal dataset and an AUC of 0.899 and a sensitivity of 1.0 in the external dataset. CONCLUSIONS: Based on clinical information and imaging characteristics, the constructed radiomics model achieved high preoperative diagnostic ability. The SCA scale achieved the purpose of rapidity and practicality while ensuring sensitivity, which is conducive to simplifying clinical work.


ACTH-Secreting Pituitary Adenoma , Adenoma , Pituitary Neoplasms , Humans , Female , ACTH-Secreting Pituitary Adenoma/surgery , Adenoma/diagnostic imaging , Adenoma/surgery , Pituitary Neoplasms/diagnostic imaging , Pituitary Neoplasms/surgery , Magnetic Resonance Imaging , Retrospective Studies
15.
Zhonghua Gan Zang Bing Za Zhi ; 39(1): 36-38, 2023 Jan 15.
Article Zh | MEDLINE | ID: mdl-36776013

Central venous lesion is a difficult problem in the vascular access complications of hemodialysis, which can cause serious clinical symptoms and affect the quality of hemodialysis and life of patients. We established arteriovenous fistula of the contralateral graft blood vessel with the used vein on the diseased side of the central vein of the patient. The arteriovenous fistula of the graft blood vessel was successfully punctured and hemodialysis was performed 2 weeks later. In this way, we not only solved the problem of venous hypertension and subsequent vascular access in the patient, but also reserved more vascular resources.


Arteriovenous Fistula , Arteriovenous Shunt, Surgical , Blood Vessel Prosthesis Implantation , Humans , Arteriovenous Shunt, Surgical/adverse effects , Treatment Outcome , Renal Dialysis
16.
Zhonghua Kou Qiang Yi Xue Za Zhi ; 58(2): 158-164, 2023 Feb 09.
Article Zh | MEDLINE | ID: mdl-36746449

Objective: To explore the effect of scanning methods on finish line trueness of the full crown preparation. Methods: The standard full crown preparation model of the right maxillary first molars was prepared by using the maxillary standard resin dentition model. The standard preparation was scanned by imetric scanner and data were used as the true value. CEREC Omnicam and 3Shape TRIOS were used to scan the standard preparation. According to the scanning methods, they were divided into parallel scanning group, occlusal wave scanning group and buccolingual wave scanning group. Each group was scanned repeatedly 6 times. The data were imported into Geomagic Studio 2013 software, and the local finish line image data of the mesial, distal, buccal and lingual regions of the full crown preparation were extracted respectively. Three-dimensional deviation analysis was performed with the reference true value, and the root- mean-square error (RMSE) was the evaluation index of scanning trueness. The statistical method was one-way ANOVA. Results: Parallel scanning group: in general, the RMSE value of complete finish lines of scanner B [(35±6) µm] was significantly lower than that of scanner A [(44±7) µm](P<0.05). After scanner A occlusal wave scanning, the RMSE values of the mesial and distal finish lines [(33±5) and (50±12) µm] were significantly lower than those of parallel scanning group (P<0.05). After buccal and lingual wave scanning, the RMSE values of local finish lines in the mesial, distal, buccal and lingual regions [(37±3), (50±6), (28±6) and (29±8) µm] were significantly lower than those in parallel scanning group [(45±9), (63±7), (38±3) and (40±3) µm] (P<0.05). No significant difference was found in the RMSE values of the mesial, distal, buccal and lingual regions of scanner B between parallel scanning group, occlusal wave scanning group and buccolingual scanning group (P>0.05). Conclusions: The scanning trueness of the full crown preparation finish line obtained by the active triangulation scanning equipment can be improved by changing the scanning method to wave scanning.


Dental Impression Technique , Imaging, Three-Dimensional , Humans , Computer-Aided Design , Dental Care , Crowns
17.
Climacteric ; 26(1): 25-33, 2023 02.
Article En | MEDLINE | ID: mdl-35674253

OBJECTIVE: This study aimed to examine the effects of SIRT1 agonist resveratrol on bone mass in ovariectomized (OVX) rats and the SIRT1 single-nucleotide polymorphism (SNP) rs7896005 on bone mass in women during menopause and early postmenopause. METHODS: An animal experiment was conducted on rats that were sham-operated (SHAM), OVX or OVX and different administered doses of resveratrol. Serum markers and femur microstructure and staining were assessed. A cross-sectional study was conducted in women undergoing menopause. SIRT1 protein and SIRT1 SNP rs7896005 were evaluated. RESULTS: OVX rats administered resveratrol, especially high doses, showed lower bone loss than OVX rats. Serum osteoprotegerin (OPG) and femur SIRT1, ß-catenin and bone mineral density (BMD) were significantly increased, whereas receptor activator of NF-κB ligand (RANKL) was significantly decreased. Serum SIRT1 levels were significantly lower in women with low bone mass (p < 0.01). Women with the CA genotype of rs7896005 had lower bone mass than those with the CC genotype. The A allele showed a significant negative effect on bone loss risk (odds ratio = 3.48; p = 0.025). CONCLUSIONS: Resveratrol stimulated SIRT1 expression and Wnt/ß-catenin signaling to promote bone mass in rat femurs. Among women in perimenopause and early postmenopause, SIRT1 protected bone mass, and the A allele of SIRT1 rs7896005 was a risk factor for reduced bone mass.


Bone Density , beta Catenin , Animals , Female , Humans , Rats , beta Catenin/genetics , beta Catenin/metabolism , Cross-Sectional Studies , Femur , Osteoprotegerin/genetics , Ovariectomy , Perimenopause , Polymorphism, Single Nucleotide , Postmenopause , RANK Ligand/genetics , Rats, Sprague-Dawley , Resveratrol/pharmacology , Resveratrol/metabolism , Sirtuin 1/genetics , Sirtuin 1/metabolism
18.
Immunooncol Technol ; 20: 100409, 2023 Dec.
Article En | MEDLINE | ID: mdl-38192614

Background: Glioblastoma (GBM) is an aggressive brain tumor giving a poor prognosis with the current treatment options. The advent of chimeric antigen receptor (CAR) T-cell therapy revolutionized the field of immunotherapy and has provided a new set of therapeutic options for refractory blood cancers. In an effort to apply this therapeutic approach to solid tumors, various immune cell types and CAR constructs are being studied. Notably, macrophages have recently emerged as potential candidates for targeting solid tumors, attributed to their inherent tumor-infiltrating capacity and abundant presence in the tumor microenvironment. Materials and methods: In this study, we developed a chemically defined differentiation protocol to generate macrophages from human pluripotent stem cells (hPSCs). A GBM-specific CAR was genetically incorporated into hPSCs to generate CAR hPSC-derived macrophages. Results: The CAR hPSC-derived macrophages exhibited potent anticancer activity against GBM cells in vitro. Conclusion: Our findings demonstrate the feasibility of generating functional CAR-macrophages from hPSCs for adoptive immunotherapy, thereby opening new avenues for the treatment of solid tumors, particularly GBM.

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