A 20-year-old male patient presented to the Department of Dermatology of Peking Union Medical College Hospital with complaints of an 8-year history of facial scarring, swelling of the lower limbs, and a 4-year history of scalp thickening. Physical examination showed thickening furrowing wrinkling of the skin on the face and behind the ears, ciliary body hirsutism, blepharoptosis, and cutis verticis gyrate. Both lower limbs were swollen, especially the knees and ankles. The skin of the palms and soles of the feet was keratinized and thickened. Laboratory examination using bone and joint X-ray showed periostosis of the proximal middle phalanges and metacarpals of both hands, distal ulna and radius, tibia and fibula, distal femurs, and metatarsals.Genetic testing revealed two variants in SLCO2A1, c.1658T > A and c.96+4A > C.Through multidisciplinary consultation, we confirmed the diagnosis of pachydermoperiostosis.

BACKGROUND:Anthocyanin is one of the most important active components in Lycium ruthenicum Murr,which has antioxidant and immunomodulatory effects.CD146+human adipose-derived pericytes/perivascular cells(CD146+hAD-PCs)are the progenitors of bone marrow mesenchymal stem cells,which can promote the proliferation and differentiation of hematopoietic stem/progenitor cells in vitro.The support effect of anthocyanin in combination with CD146+hAD-PCs on umbilical cord blood hematopoietic stem/progenitor cells remains to be studied. OBJECTIVE:To investigate the supporting effect of anthocyanins in Lycium ruthenicum Murr(ALRM)combined with CD146+hAD-PCs on umbilical cord blood CD34+hematopoietic stem/progenitor cells(UCB CD34+HSPCs)in vitro. METHODS:The CCK-8 assay was used to detect the effect of different concentrations(0,200,400,600,800,1 000 mg/L)of ALRM on the proliferation of CD146+hAD-PCs.Flow cytometry was used to detect the effect of ALRM on the cell cycle of CD146+hAD-PCs.The co-culture experiments were divided into blank group,ALRM group,CD146+hAD-PCs group,and ALRM+CD146+hAD-PCs group to analyze the in vitro supporting effect of ALRM combined with CD146+hAD-PCs on UCB CD34+HSPCs.The number of expanded cells and the number of colony-forming units were compared at 1,2,and 4 weeks of co-culture.The immunophenotype of cells was detected by flow cytometry.The level of cytokines was detected by enzyme-linked immunosorbent assay. RESULTS AND CONCLUSION:(1)The cell viability of CD146+hAD-PCs was highest at an ALRM concentration of 200 mg/L,the proportion of G0/G1 phase cells decreased and the proportion of S and G2/M phase cells increased in CD146+hAD-PCs(P<0.01).(2)The change in number of UCB CD34+HSPCs cells in the ALRM+CD146+hAD-PCs group was higher than that in the ALRM group at 1,2,and 4 weeks of co-culture(all P<0.05),and higher than that in CD146+hAD-PCs group at 2 and 4 weeks of co-culture(all P<0.05).The number of cells in the ALRM group and blank group decreased gradually with the extension of co-culture time.(3)Colony forming capacity and immunophenotype analysis:The number of colony-forming units in the ALRM+CD146+hAD-PCs group was higher than that in the CD146+hAD-PCs group and ALRM group at 1 and 2 weeks of co-culture(P<0.05).The proportion of CD45+and CD34+CD33-cells in the ALRM+CD146+hAD-PCs group was higher than that in the CD146+hAD-PCs group at 1 and 2 weeks of co-culture(all P<0.01).(4)Changes in cytokines:Interleukin-2 level in the ALRM+CD146+hAD-PCs group was higher than that in the ALRM and CD146+hAD-PCs groups(P<0.05).The interleukin-3 content of the ALRM+CD146+hAD-PCs group was higher than that of the CD146+hAD-PCs group at 2 and 4 weeks(P<0.05).The expression level of granulocyte colony-stimulating factor in the ALRM+CD146+hAD-PCs group was higher than that in the CD146+hAD-PCs group at 1 week,and higher than that in the ALRM group and CD146+hAD-PCs group at 2 weeks(P<0.01).Interferon-γ content in the ALRM group and ALRM+CD146+hAD-PCs group was lower than that in the CD146+hAD-PCs group at 1,2,and 4 weeks of co-culture(P<0.01).(5)Due to the absence of stromal cells in the blank group,UCB CD34+HSPCs could not be counted after 1 week of co-culture and were not subjected to immunophenotyping,colony analysis,or cytokine assays.(6)In summary,ALRM can promote the expansion of UCB CD34+HSPCs in vitro by promoting CD146+hAD-PCs proliferation and cell cycle transformation,which is of great value in hematopoietic stem cell transplantation.

Objective To investigate the curative effect of suture anchor(SA)repair combined with open reduction and internal fixation(ORIF)on patients with ankle joint fracture(AF)combined with deltoid ligament injury(DLI).Methods A total of 138 patients with AF combined with DLI admitted to our hospital from January 2020 to September 2022 were selected and divided into the control group(69 cases)and the observation group(69 cases)according to the random number table method.Patients in the control group were treated with ORIF,while patients in the observation group were treated with SA repair on the basis of the control group.The clinical efficacy,American Orthopedic Foot and Ankle Society(AOFAS)score,visual analogue scale(VAS)score,talus inclination angle,medial malleolar space of affected side,bone metabolic indexes[serum bone gla protein(BGP),β-collagen degradation product(β-CTX)]levels and the incidence of complications before and 3 months after treatment were compared between the two groups.Results The total effective rate in the observation group was higher than that in the control group,and the difference was statistically significant(P<0.05).Compared with before treatment,the talus inclination angle,medial malleolar space of affected side,VAS score,β-CTX level 3 months after treatment of patients in the two groups were reduced,while the AOFAS score and BGP level were increased,and the differences were statistically significant(P<0.05).After treatment,the AOFAS score and BGP level in the observation group were higher than those in the control group,while the talus inclination angle and medial malleolar space of affected side were smaller than those in the control group,and the VAS score and β-CTX level were lower than those in the control group,with statistically significant differences(P<0.05).The total incidence of complications in the observation group was lower than that in the control group,and the difference was statistically significant(P<0.05).Conclusion SA repair has a definite therapeutic effect on AF combined with DLI,which can improve patients' symptoms and promote the recovery of ankle joint function and bone metabolism.

Intramedullary needle(IMN)has the advantages of high healing rate and low incidence of complications in treatment of tibial fracture,and has become one of the most commonly used fixation methods for the treatment of tibial fracture.However,due to the patient's own factors,fracture location and fracture type,infection and surgical treatment,bone nonunion after IMN still occurs in clinic.Bone nonunion leads to the increase of medical cost and prolonged the hospitalization time of patients,which causes great pain to patients,and also brings great challenges to the treatment of orthopedic surgeons.Therefore,this paper reviews the influencing factors of bone nonunion after IMN for tibial fracture,in order to provide reference for clinical treatment.

Objective To compare the efficacy of renal proximal renal artery denervation(pRDN)and full-length renal artery denervation(fRDN)for treatment of hypertension.Methods Fifty-six hypertensive patients were enrolled and randomly assigned to full-length renal artery denervation group(n=25)and proximal renal artery denervation group(n=31).After the procedure,24-hour ambulatory blood pressure monitoring(24 h-ABPM)at 6 months and office blood pressure at 12 months was recorded for statistical analysis.Results The blood pressure at follow-up reduced significantly in both groups,while there was no significant difference between groups.The baseline office blood pressure in fRDN group and pRDN group was(180±15)/(104±10)mmHg and(180±12)/(103±8)mmHg,respectively,which decreased to(142±9)/(82±7)mmHg and(143±10)/(83±6)mmHg at 12 months postoperatively(P＜0.001 within groups and P＞0.05 between groups).The baseline 24 h-ABPM in the two groups was(162±13)/(95±8)mmHg and(160±12)/(94±8)mmHg,respectively,which decreased to(142±11)/(83±7)mmHg and(141±8)/(81±7)mmHg at 6 months postoperatively(P＜0.001 within groups and P＞0.05 between groups).However,there was no significant difference in the reduction of office blood pressure and ambulatory blood pressure between the two groups.No treatment-related adverse events were observed.Conclusions pRDN has similar antihypertensive effect to fRDN.

This study was aimed at studying the phenotypic and molecular characteristics of a Salmonella Grumpensis isolate from a patient with diarrhea in Shanghai,to provide evi-dence for the prevention of salmonellosis.Biochemical identifi-cation,serum agglutination testing,antimicrobial susceptibility testing,and whole genome sequencing(WGS)were performed on isolate 2023JD76.Global Salmonella Grumpensis genome sequences were searched and downloaded for serotyping predic-tion,multilocus sequence typing(MLST),prediction of anti-microbia resistance genes and virulence genes,and phylogenetic analysis of 2023JD76.The 2023JD76 strain was identified as Salmonella Grumpensis(13,23:d:1,7)with ST2060,and was susceptible to 20 antimicrobial agents.Strain 2023JD76 carried the aminoglycoside resistance gene aac(6')-Iaa and five types of virulence genes:the adhesion genes csg and rat;the secretion and transport genes sip and inv;the typhoid toxin genes cdt and plt;the invasive gene nutrient metabolism factor mgt;and the antimicrobial peptide resistance factor mig.Global S.Grumpensis strains harbored ten types of antimicrobial resistance genes whose prevalence ranged from 58.33％to 100％.The global genome sequences of S.Grumpensis were divided into two lineages.Lineage I was dominated by ST751(88.89％,16/18),and lineage Ⅱ was dominated by ST2060(89.47％,17/19).The genome sequence of strain 2023JD76 belonged to lineage Ⅱ,and was closely related to the genome sequences from human fecal and human cerebrospinal fluid.This study provides the first report of a S.Grumpensis isolate from the stool of a patient with diarrhea in China.Considerable variability in antimicrobial resistance genes was observed among genome sequences from different sources,and the strains harbored a substantial number of virulence genes.Enhanced surveillance should be emphasized to prevent a potential risk of global dissemination.

Objective:To investigate the therapeutic effect of Xueshuantong injection combined with urinary kallindino-genase and butylphthalide on acute cerebral infarction(ACI)and its influence on the levels of matrix metalloproteinases(MMP)and neuronal factors.Methods:A total of 92 ACI patients treated in our department from January 2022 to June 2022 were selected,divided into control group(n=46)and study group(n=46)using random number table method.The control group was treated with urinary kallindinogenase+butylphthalide,and the study group was treated with Xueshuan-tong injection on the basis of the control group.After 3-week treatment,therapeutic effect,hemodynamics,levels of MMP and neuronal factors were compared between two groups.Results:Compared with the control group after treatment,there were significant rise in total effective rate(82.61％vs.97.83％),mean blood velocity(Vm)[(25.91±2.94)cm/s vs.(29.88±2.18)cm/s],levels of nerve growth factor(NGF)[(69.91±6.54)pg/ml vs.(79.07±8.72)pg/ml]and neurotrophic factors(NTF)[(4.12±1.30)ng/ml vs.(4.85±0.93)ng/ml],and significant reductions in peripheral re-sistance(Rv)[(89.33±14.89)kPa·s/m vs.(79.89±12.32)kPa·s/m],dynamic resistance(DR)[(37.81±5.18)kPa vs.(33.98±4.6)kPa],levels of MMP-2[(248.63±12.06)μg/L vs.(207.06±12.06)μg/L],MMP-9[(234.99±12.86)μg/L vs.(200.30±11.78)μg/L]and neuron specific enolase(NSE)[(16.14±2.81)μg/L vs.(12.66±2.77)μg/L]in study group(P＜0.05 or＜0.01).Conclusion:Xueshuantong injection combined with urinary kallindinogenase and butylphthalide can help to improve the therapeutic effect,promote the improvement of cerebral he-modynamics,and further regulate the levels of matrix metalloproteinases and neuronal factors in patients with acute cerebral infarction,which is worthy of promotion.

The cyclic GMP-AMP synthase(cGAS)-stimulator of interferon genes signaling pathway plays a central role in cells'ability to sense the presence of abnormal double-stranded DNA within the cytoplasm.Modulation of the function of the cytoplasmic DNA recognition receptor cGAS allows regulation of this signaling pathway.In recent years,research has identified protein post-translational modifications(PTMs),including phosphorylation,acetylation,ubiquitination,methylation,and SUMOylation,as having significant roles in regulating innate immune signaling pathways.This paper reviews how PTMs influence the function of cGAS and its downstream signaling pathways through various mechanisms.

Objective To investigate the incidence rate,clinical characteristics,and prognosis of neonatal stroke in Shenzhen,China.Methods Led by Shenzhen Children's Hospital,the Shenzhen Neonatal Data Collaboration Network organized 21 institutions to collect 36 cases of neonatal stroke from January 2020 to December 2022.The incidence,clinical characteristics,treatment,and prognosis of neonatal stroke in Shenzhen were analyzed.Results The incidence rate of neonatal stroke in 21 hospitals from 2020 to 2022 was 1/15 137,1/6 060,and 1/7 704,respectively.Ischemic stroke accounted for 75％(27/36);boys accounted for 64％(23/36).Among the 36 neonates,31(86％)had disease onset within 3 days after birth,and 19(53％)had convulsion as the initial presentation.Cerebral MRI showed that 22 neonates(61％)had left cerebral infarction and 13(36％)had basal ganglia infarction.Magnetic resonance angiography was performed for 12 neonates,among whom 9(75％)had involvement of the middle cerebral artery.Electroencephalography was performed for 29 neonates,with sharp waves in 21 neonates(72％)and seizures in 10 neonates(34％).Symptomatic/supportive treatment varied across different hospitals.Neonatal Behavioral Neurological Assessment was performed for 12 neonates(33％,12/36),with a mean score of(32±4)points.The prognosis of 27 neonates was followed up to around 12 months of age,with 44％(12/27)of the neonates having a good prognosis.Conclusions Ischemic stroke is the main type of neonatal stroke,often with convulsions as the initial presentation,involvement of the middle cerebral artery,sharp waves on electroencephalography,and a relatively low neurodevelopment score.Symptomatic/supportive treatment is the main treatment method,and some neonates tend to have a poor prognosis.

Objective:The purpose of this study is to test and assess the model of modeling data of TaiChi accelerator in the Raystation Treatment Planning System(RayStation system)according to the test method and item of TG119 report of American Association Physicians Medicine(AAPM).Methods:The intensity-modulated radiation therapy(IMRT)and volumetric-modulated arc therapy(VMAT)plans of the test cases of different clinical situations,which included the simulated multi target region,prostate target region,head and neck target region,easy type C-shape target region plan and difficult type C-shape target region plan,were designed according to the AAPM TG119 report in the treatment planning system.The deviations of the doses of point and area of the two kinds of plans were measured,and the measured results were compared and analyzed with the recommended standards of AAPM TG119 report.The IBA CC13 ionization chamber and the ArcCHECK matrix ionization chamber were used respectively to verify the point dose and area dose,and the assessment standard was γ passing rate under 3%3mm.The confidence interval was adopted to judge the consistency between the measured dose and the calculated dose.Results:The accuracies of plan dose target,point dose deviation and area dose distribution of tested cases could meet the requirement of the TGl19 report.The deviations of mean doses for the high-dose points of IMRT plan and VMAT plan of tested cases were respectively(0.39±1.02)%and(1.27±0.64)%,and the confidence intervals of them were respectively 2.39%and 2.52%.The average dose deviations of low doses of organ at risk(OAR)of IMRT plan and VMAT plan were respectively(0.53±1.73)%and(0.88±1.11)%,and the confidence intervals were respectively 3.92%and 3.06%.The average γ passing rate under 3%/3mm of IMRT plan and VMAT plan were respectively(99.52±0.366)%and(99.86±0.136)%,and the confidence intervals of them were respectively 1.196%and 0.406%.Conclusion:The TaiChi accelerator performance and the accuracy of Raystation system 6MV FFF model fitting can meet the standard of TG119 report,and the subsequent standards of the quality control of equipment and patients were established according to these tested results,which would provide reference for the improvement of the performance of subsequent accelerator.