Objective To summarize the pathological morphological features, diagnosis, and differential diagnosis of adult-onset Still disease (AOSD), and to improve clinical understanding of the disease. Methods A retrospective analysis was conducted on the morphological characteristics, immunophenotypes, and molecular detection results of lymph node biopsies from three AOSD patients. Results Case 1: lymph node biopsy tissue showed significant lymphoid follicular hyperplasia, accompanied by parafollicular hyperplasia; the germinal centers exhibited a starry-sky phenomenon, with no obvious histiocyte proliferation foci, plasma cells, or neutrophils; immunohistochemical staining showed that CD3 and CD5 T lymphocyte were positive in the paracortical area, CD20 and CD79α markers showed that B lymphocytes were mainly located in the follicular area, CD21 follicular dendritic cells and CD68 histiocytes were positive. Case 2: lymph node puncture tissue showed paracortical hyperplasia, a decrease in the number of follicles, and a reduction of follicular volume; there were no obvious histiocyte proliferation foci, plasma cells, or neutrophils; immunohistochemical staining showed positive CD3, CD5 T lymphocytes in the paracortical area, and CD20, CD79α B lymphocytes in the follicular region. Case 3: lymph node puncture tissue showed partial preservation of the normal lymph node structure, the paracortical area was diffusely proliferated, and the histiocyte hyperplasia was patchy with partial necrosis, and obvious nuclear debris, scattered plasma cells and eosinophils can be seen and no obvious neutrophil infiltration in the necrotic area; immunohistochemical staining of case 3 showed that CD21 and CD23 follicular dendritic cells were positive, and Bcl-2, Bcl-6, CD3, CD5, CD20, CD79α, and multiple myeloma protein 1 (MUM1) were positively expressed in some lymphocytes; the Ki-67 proliferation index was high, approximately 70%; a few plasma cells were positive for CD138, with individual cells positive for CD1α; CD10 and CyclinD1 were negative; histiocytes were positive for myeloperoxidase (MPO); and EBER was negative for in situ hybridization. The results of TCR gene rearrangement and IG gene rearrangement in the three cases were negative. Conclusion The immunophenotype of AOSD is diversity, and its dignosis depends on the clinical and pathological morphological features and immunophenotype, excluding infectious diseases, malignant tumors and lymphoma, etc.

Objective To investigate the effect of cysteine-rich acidic secretory protein-like protein 1(SPARCL1)on atherosclerosis(AS)plaque formation.Methods A case-control study design was used,394 patients with con-firmed AS were selected as the case group,and 394 healthy medical examiners matched for age and gender were se-lected as the control group.The expression level of serum SPARCL1 was determined by enzyme-linked immunosor-bent assay;immunohistochemistry was used to assess the expression level and localization of SPARCL1 protein in the AS plaque region,and the expression of SPARCL1 protein was also detected in the neutrophils and monocytes of peripheral blood of AS patients and normal controls;SPARCL1 overexpressing and the recombinant adenoviral vec-tors were constructed to inhibit SPARCL1 overexpression and expression,and the effects of SPARCL1 on cell mi-gration were observed in the cell scratch assay using mouse macrophage cells(J774A.1)as target cells.Results Serum SPARCL1 levels in the AS patient group were lower than those in the healthy group(P<0.05);high SPARCL1 expression was detected in AS plaques and was mainly expressed in the cytoplasm of foamy cells;SPARCL1 expression levels in peripheral blood neutrophils and monocytes were lower than those in normal controls in AS patients(P<0.05);recombinant SPARCL1 overexpression and inhibition of expression of adenovirus was successfully constructed;the cell migration rate was decreased in J774A.1 cells that inhibited SPARCL1 expression and increased in J774A.1 cells that overexpressed SPARCL1(P<0.05).Conclusion SPARCL1 is highly ex-pressed in foam cells at the site of AS lesions,which may result from compensatory recruitment of peripheral blood monocytes and neutrophils,and SPARCL1 may be involved as a protective factors for blood vessels in inhibiting the development of AS plaques.

OBJECTIVE: To explore the genetic basis for 7 families with gonadal mosaicism for Duchenne muscular dystrophy (DMD). METHODS: For the 7 families presented at the CITIC Xiangya Reproductive and Genetic Hospital from September 2014 to March 2022, clinical data were collected. Preimplantation genetic testing for monogenic disorders (PGT-M) was carried out for the mother of the proband from family 6. Peripheral venous blood samples of the probands, their mothers and other patients from the families, amniotic fluid samples from families 1 ~ 4 and biopsied cells of embryos cultured in vitro from family 6 were collected for the extraction of genomic DNA. Multiplex ligation-dependent probe amplification (MLPA) was carried out for the DMD gene, and short tandem repeat (STR)/single nucleotide polymorphism (SNP)-based haplotypes were constructed for the probands, other patients, fetuses and embryos. RESULTS: The results of MLPA showed that the probands and the fetuses/probands' brothers in families 1 ~ 4, 5, 7 had carried the same DMD gene variants, whilst the probands' mothers were all normal. The proband in family 6 carried the same DMD gene variant with only 1 embryo (9 in total) cultured in vitro, and the DMD gene of the proband's mother and the fetus obtained through the PGT-M were normal. STR-based haplotype analysis showed that the probands and the fetuses/probands' brothers in families 1 ~ 3 and 5 have inherited the same maternal X chromosome. SNP-based haplotype analysis showed that the proband from family 6 has inherited the same maternal X chromosome with only 1 embryo (9 in total) cultured in vitro. The fetuses in families 1 and 6 (via PGT-M) were both confirmed to be healthy by follow up, whilst the mothers from families 2 and 3 had chosen induced labor. CONCLUSION Haplotype analysis based on STR/SNP is an effective method for judging gonad mosaicism. Gonad mosaicisms should be suspected for women who have given births to children with DMD gene variants but with a normal peripheral blood genotype. Prenatal diagnosis and reproductive intervention may be adapted to reduce the births of further affected children in such families.
Male ; Pregnancy ; Child ; Humans ; Female ; Muscular Dystrophy, Duchenne/diagnosis* ; Dystrophin/genetics* ; Mosaicism ; Exons ; Prenatal Diagnosis/methods* ; Nucleotides

OBJECTIVE To optimize the molding process of Shuangye pipa granules based on the concept of quality by design （QbD） and analyze its physical fingerprint. METHODS The dry extract of Shuangye pipa granules was used as the main drug. The retention rate of total flavonoid， moisture absorption rate， dissolution rate， angle of repose and molding rate of the granules were selected as evaluation indexes. The single-factor test combined with the entropy weight method and Box-Behnken response surface design was used to optimize the molding process， and validation test was conducted. The physical fingerprints of 10 batches of Shuangye pipa granules prepared by the optimal process were comprehensively analyzed by eight secondary physical indexes （relative homogeneity， moisture， moisture absorption rate， Hausner ratio， angle of repose， bulk density， tap density and porosity）. RESULTS The optimal molding process of Shuangye pipa granules was as follows： soluble starch-maltodextrin-mannitol was 1∶1∶1 （m/m/m）， 95% ethanol was as wetting agent and the amount of it was 37%， the drug-assisted ratio was 1∶0.8 （m/m）， the drying temperature was 59 ℃， drying time was 28 min. The results of 3 validation tests showed that the average comprehensive score was 0.879 6， the RSD of which with prediction value （0.881 9 score） was 1.97%. The similarity between the physical fingerprints of 10 batches of Shuangye pipa granules and the control physical fingerprint was higher than 0.99. CONCLUSIONS The optimized molding process of Shuangye pipa granules is stable and feasible， and the physical property of Shuangye pipa granules is stable and controllable.

OBJECTIVE: To summarize the genetic characteristics of 671 Chinese pedigrees affected with Duchenne/Becker muscular dystrophy (DMD/BMD). METHODS: Clinical data of the pedigrees were collected. Multiplex PCR, multiple ligation dependent probe amplification (MLPA), next generation sequencing (NGS), Sanger sequencing and long read sequencing were used to detect the variant of DMD gene in the probands and their mothers, and prenatal diagnosis was provided for high risk pregnant women. RESULTS: Among 178 pedigrees analyzed by multiplex PCR, 44 variants of the DMD gene were detected, with the genetic diagnosis attained in 110 pedigrees. Among 493 pedigrees analyzed by MLPA in combination with NGS or Sanger sequencing, 294 pathogenic/possible pathogenic variants were identified, among which 45 were unreported previously, and the genetic diagnosis attained in 484 pedigrees. Structural variants of the DMD gene were identified in two pedigrees by long-read sequencing. Among 444 probands, 341 have inherited the DMD gene variant from their mothers (76.8%). Among 390 women with a high-risk, 339 have opted to have natural pregnancy and 51 chose preimplantation genetic testing for monogenetic disease (PGT-M). The detection rate of neonatal patients and carriers following natural pregnancy was significantly higher than that for PGT-M. CONCLUSION Combined application of MLPA, NGS, Sanger sequencing and long-read sequencing is an effective strategy to detect DMD/BMD. PGT-M can effectively reduce the risk of fetuses. Above finding has expanded the spectrum of DMD gene variants and provided a basis for reproductive intervention for pregnancies with a high risk for DMD/BMD.
China ; Dystrophin/genetics* ; Exons ; Female ; Genetic Testing ; Humans ; Infant, Newborn ; Multiplex Polymerase Chain Reaction ; Muscular Dystrophy, Duchenne/genetics* ; Mutation ; Pedigree ; Pregnancy ; Prenatal Diagnosis

Objective: To explore the molecular mechanism of resistance to rituximab in diffuse large B-cell lymphoma (DLBCL) during treatment with R-CHOP (rituximab+cyclophosphamide+ doxorubicin+vincristine+prednisone). Methods: FCM was used to detect the expression level of CD20 and the three membrane complement regulatory proteins CD46, CD55 and CD59 in parental cells LY8 [germinal center B-cell-like (GCB) subtype] and NU-DUL-1 [activated B-cell-like (ABC) subtype], rituximab (R) resistance cells LY8-R and NU-DUL-1-R, chemotherapy drug cyclophosphamide+doxorubicin+ vincristine (CHO) resistance cells LY8-CHO and NU-DUL-1-CHO, and rituximab combined with CHO resistance (RCHO) LY8-RCHO and NU-DUL-1-RCHO cells. The expression level of CD20 protein and mRNA were detected by Western blotting and real-time fluorescent quantitative PCR, respectively. The expression levels of CD20, CD46, CD55 and CD59 in SRY (sex determining region Y)-box 2 (SOX2) overexpression of parental LY8 and NU-DUL-1 cells as well as SOX2 gene silencing of LY8-RCHO and NU-DUL-1-RCHO cells were detected by FCM method. Furthermore, in tumor tissues of clinical 25 DLBCL patients with at least 6 circles of R-CHOP regimen treatment, immunohistochemical were used to detect the expression levels of CD20 and SOX2 before and after the treatment. Results: Compared with the parental cells, in the LY8-R, LY8-RCHO and NU-DUL-1-R and NU-DUL-1-RCHO cells that developed rituximab resistance, not in LY8-CHO and NU-DUL-1-CHO cells which resistant to chemotherapy, the expression level of CD20 was significantly reduced (all P < 0.001), while the expression levels of CD46, CD55 and CD59 were also decreased (all P < 0.05). The expression level of CD20 protein and mRNA were significantly reduced (P < 0.001and P < 0.01). Furthermore, in the parental LY8 and NU-DUL-1 cells, overexpression of SOX2 significantly reduced the expression level of CD20 (P < 0.000 1), while knockdown of SOX2 in LY8-RCHO and NU-DUL-1-RCHO cells significantly increased the expression level of CD20 (P < 0.0001). It was further found that the expression level of CD20 in tumor tissues of DLBCL patients was significantly decreased after R-CHOP treatments (P < 0.01), and the expression level of SOX2 was significantly increased (P < 0.001). After comprehensive analysis, it was found that there was a negative correlation between CD20 and SOX2 expression in tumor tissues (P < 0.000 1). Conclusion: SOX2 induces DLBCL resistance to rituximab by reducing the expression level of CD20.

Objective: To investigate the effects of interferon inducible protein 16 (IFI16), a cytosolic DNA sensor, on the expression of human T-cell leukemia virus type 1 (HTLV-1) proteins and pro-inflammatory cytokines in adult HTLV-1-positive T cells. Methods: IFI16 expression in different HTLV-1-positive T cell lines was detected by immunoblot assay. Specific siRNA targeting the IFI16 gene was constructed and the gene silencing efficiency was detected by immunoblot assay. Expression of HTLV-1 Tax protein at mRNA and protein levels was respectively detected by real-time PCR and immunoblot assay after knocking down the expression of IFI16 in HTLV-1-positive T cells with siRNA. Expression of interferon (IFN)-α, IFN-γ, tumor necrosis factor (TNF)-α, Tax and Env were detected by real-time PCR. Results: Compared with the HTLV-1-negative T cell line Jurkat, IFI16 expression was enhanced in the HTLV-1-positive T cell lines MT2, MT4 and C8166. Tax expression was increased, while that of IFN-α, IFN-γ and TNF-α was decreased in MT2 and MT4 cells after silencing the expression of IFI16 with siRNA. Conclusions IFI16 expression was increased in HTLV-1-positive MT2 and MT4 cells. Meanwhile, IFI16 promoted the production of interferon and pro-inflammatory cytokines and inhibited the expression of HTLV-1 proteins.

Objective: Develop a kind of portable negative pressure calibrator by the design and implement of method. Methods:By the working principle of negative pressure sensor and gas flow sensor, the negative pressure aspirator realizes the calibration function and stability of the monitoring function of the pressure and flow. Results:Stating from the design concept, this portable negative pressure can satisfy the requirement of the quality control and test work. Conclusion:Guarantee the accuracy and efficiency in the clinical first aid and field battle when using attractor. This avoids attractor of iatrogenic injury accident caused by negative pressure fall off.

Objective Arrhythmias are one of the main causes of postoperative morbidity after thoracic surgery.The aim of this study was to evaluate whether video-assisted thoracoscopic surgery decreases the risk of postoperative arrhythmias compared with traditional open lung lobectomy.Methods 138 consecutive patients were enrolled from January 2011 to February 2012,after five age and risk factors matched,68 patients undergoing video-assisted thoracic surgery and 70 patients undergoing traditional open lung lobectomy were eligible for analysis.The rhythm was documented preoperatively and postoperatively with standard electrocardiogram (ECG) recording and ECG monitoring.All patients followed 3 months after hospital admission.Results There was a 17.6％ incidence (12/68) of postoperative new-onset arrhythmias undergoing video-assisted thoracic surgery and 18.6％ of patients (13/70) undergoing thoracotomy,but the difference was not statistically significant.In both groups,atrial fibrillation was the most common arrhythmia (60％).There was no significant difference in the other morbidity (P ＞0.05) and mortality rate(P =0.57,1.5％ vs 2.9％) between the two groups.Conclusion New-onset arrhythmias,most frequently atrial fibrillation,are common after lung lobectomy.Regardless of surgical approach,postoperative arrhythmias after lobectomy occurred with equal frequency.New insights in the pathophysiology of postoperative thoracic arrhythmias and advances in prevention and therapy are need future study.

Medical equipment quality control is the core content of hospital management. It runs through the whole process of medical equipment management, including the purchase, the installation and acceptance, the use and maintenance, the abandonment. It is the absolute duty for a hospital to assure and control the quality of the equipment. This paper presents the establishment of medical equipment quality control system, as well as a detailed description and analysis in the implementation of this system. Finally, discuss a few experiences in the quality control work.
Equipment Safety ; Equipment and Supplies, Hospital ; Models, Theoretical ; Quality Control