Purpose To investigate the clinicopathological features,diagnostic approaches,and differential diag-nosis of chordoid glioma(CG)and chordoid meningioma(CM)of the central nervous system(CNS).Methods Clinical data from 4 cases of CG and 10 cases of CM were collected.Immunohistochemistry was used to detect the ex-pression of GFAP,EMA,TTF-1,and other markers.Molecular genetic alerations were identified using sequencing techniques and relevant literature was reviewed.Results CG predominantly occurred in the third ventricle but could also arise outside of it.Tumors showed well-defined borders with surrounding tissues.Microscopically,tumor cells were arranged in cords or clusters within a myxoid stroma and expressed GFAP,TTF-1,and other markers.No PRKCA(D463H)mutations were detected in 3 CG cases,however,one case harbored an FLCN ∷ PRKD2 fusion.CM predom-inantly occurred in the supratentorial region but also appeared in the subtentorial area.Histologically,chordoid compo-nents were mixed with classic meningioma features.Chronic inflammatory cell infiltration was noted in the stroma.Tumor cells expressed EMA,PR and SSTR2.One case harbored NF2 mutation and homozygous CDKN2A deletion.Conclusion CG and CM of the CNS shared overlapping morphological characteristics,making histological distinction difficult.Accurate diagnosis required integration of clinical,imaging,immunohistochemical,and molecular pathologi-cal findings.

Objective To investigate the distribution of Streptococcus,sensitivity to commonly used antibiotics,and prognosis of the patients with bloodstream infections,and provide evidence for early diagnosis and selection of antibiotics.Methods The Streptococci i-solated from the blood samples of the First Affiliated Hospital of Nanjing Medical University from 2017 to 2022 were collected and di-vided into α-and β-hemolytic Streptococci according to the type of hemolysis.Their sensitivity to commonly used antibiotics was ana-lyzed.The differences in clinical data and prognosis between the two groups were also compared.Results A total of 314 strains of Streptococcus were isolated from 305 patients,including 270 strains of α-hemolytic Streptococcus and 44 β-hemolytic Streptococcus.The results of the drug sensitivity test showed that the proportion of Streptococcus strains with a diameter of 17-19 mm in the inhibition zone of vancomycin increased year by year.The positive alarm time of blood culture for β-hemolytic Streptococcus was significantly shorter than that for α-hemolytic Streptococcus(P＜0.001).Using 10.5 hours as the diagnostic threshold could assist in distinguishing blood-stream infections caused by α-and β-hemolytic Streptococcus.The overall mortality rate of patients with streptococcal bloodstream infec-tion was 15.1％,and there was no statistically significant difference in the mortality rate between the two groups(P=0.813).Conclu-sion The main Streptococcus causing bloodstream infections in our hospital is α-hemolytic Streptococcus.There are differences in the antimicrobial sensitivity and clinical characteristics among patients with different streptococcal bloodstream infections.The positive a-larm time of blood culture can assist in distinguishing the type of streptococcal bloodstream infections.In addition,the diameter of the inhibition zone of vancomycin against Streptococcus has drifted towards the breakpoint of drug resistance.

Purpose To investigate the clinicopathological features,diagnostic approaches,and differential diag-nosis of chordoid glioma(CG)and chordoid meningioma(CM)of the central nervous system(CNS).Methods Clinical data from 4 cases of CG and 10 cases of CM were collected.Immunohistochemistry was used to detect the ex-pression of GFAP,EMA,TTF-1,and other markers.Molecular genetic alerations were identified using sequencing techniques and relevant literature was reviewed.Results CG predominantly occurred in the third ventricle but could also arise outside of it.Tumors showed well-defined borders with surrounding tissues.Microscopically,tumor cells were arranged in cords or clusters within a myxoid stroma and expressed GFAP,TTF-1,and other markers.No PRKCA(D463H)mutations were detected in 3 CG cases,however,one case harbored an FLCN ∷ PRKD2 fusion.CM predom-inantly occurred in the supratentorial region but also appeared in the subtentorial area.Histologically,chordoid compo-nents were mixed with classic meningioma features.Chronic inflammatory cell infiltration was noted in the stroma.Tumor cells expressed EMA,PR and SSTR2.One case harbored NF2 mutation and homozygous CDKN2A deletion.Conclusion CG and CM of the CNS shared overlapping morphological characteristics,making histological distinction difficult.Accurate diagnosis required integration of clinical,imaging,immunohistochemical,and molecular pathologi-cal findings.

Objective To investigate the distribution of Streptococcus,sensitivity to commonly used antibiotics,and prognosis of the patients with bloodstream infections,and provide evidence for early diagnosis and selection of antibiotics.Methods The Streptococci i-solated from the blood samples of the First Affiliated Hospital of Nanjing Medical University from 2017 to 2022 were collected and di-vided into α-and β-hemolytic Streptococci according to the type of hemolysis.Their sensitivity to commonly used antibiotics was ana-lyzed.The differences in clinical data and prognosis between the two groups were also compared.Results A total of 314 strains of Streptococcus were isolated from 305 patients,including 270 strains of α-hemolytic Streptococcus and 44 β-hemolytic Streptococcus.The results of the drug sensitivity test showed that the proportion of Streptococcus strains with a diameter of 17-19 mm in the inhibition zone of vancomycin increased year by year.The positive alarm time of blood culture for β-hemolytic Streptococcus was significantly shorter than that for α-hemolytic Streptococcus(P＜0.001).Using 10.5 hours as the diagnostic threshold could assist in distinguishing blood-stream infections caused by α-and β-hemolytic Streptococcus.The overall mortality rate of patients with streptococcal bloodstream infec-tion was 15.1％,and there was no statistically significant difference in the mortality rate between the two groups(P=0.813).Conclu-sion The main Streptococcus causing bloodstream infections in our hospital is α-hemolytic Streptococcus.There are differences in the antimicrobial sensitivity and clinical characteristics among patients with different streptococcal bloodstream infections.The positive a-larm time of blood culture can assist in distinguishing the type of streptococcal bloodstream infections.In addition,the diameter of the inhibition zone of vancomycin against Streptococcus has drifted towards the breakpoint of drug resistance.

This work aims to evaluate a rapid detection method of carbapenem resistance genes in blood cultures based on Xpert Carba-R and preliminarily evaluate its clinical application.Methods:Sixteen strains of Enterobacterales carrying different carbapenem resistance genes were selected to prepare simulated positive blood culture samples and Xpert Carba-R was used to directly detect carbapenem resistance genes in the simulated positive blood culture. From January 2022 to June, a prospective study was conducted on a total of 117 Enterobacteriaceae-positive blood culture samples in the First Affiliated Hospital of Nanjing Medical University. Xpert Carba-R, detecting five kinds of carbapenem resistance genes in these samples, was evaluated in sensitivity and specificity compared to polymerase chain reaction sequencing. Meanwhile clinical data of positive patients was collected for prognostic analysis. Results:Of the 16 simulated specimens, 14 strains had carbapenem resistance genes detected by Xpert Carba-R, including 8 bla KPC, 5 bla NDM and 1 bla IMP, showing 100% agreement with the known results. As of the 117 clinical specimens, 28 cases were determined to be Enterobacterales harboring carbapenem resistance genes, including 24 bla KPC, 2 bla NDM and 2 bla KPC+ bla NDM. In comparison to the PCR sequencing, the sensitivity and specificity of Xpert Carba-R were both 100% for blood culture samples, and furthermore, the detection time was significantly reduced. Of the 25 positive patients, 9 cases were treated with monotherapy and 5 cases were effective, other 16 cases received combined treatment and 12 cases were effective. A total of 17 cases were effective, 8 cases were ineffective and 3 of them died, the mortality rate was 12% (3/25). Conclusion:Xpert Carba-R can rapidly and accurately detect carbapenem resistance genes in blood culture, which can provide evidence for rational drug therapy in early clinical stage.

Under the national policy background of traditional Chinese medicine（TCM）registration review and approval reform， TCM preparations in medical institutions（hereinafter referred to as medical institution preparations） have human use experience， conform to the characteristics of TCM clinical practice， and have advantages in the research and development of innovative Chinese medicines. Therefore， the research and development mode based on clinical experience prescription-medical institution preparations-innovative Chinese medicines has attracted widespread attention from the industry. However， in the process of development and use of medical institution preparations， there are generally clinical problems that restrict their transformation into innovative Chinese medicines， resulting in a relatively weak collection basis of human use experience and insufficient clinical evidence for supporting the research and development of new TCM medicines. In this paper， on the basis of sorting out the supporting regulations and relevant technical requirements of human use experience， and analyzing the clinical problems restricting the transformation of medical institution preparations， it provides suggestions for medical institutions to carry out high-quality research on human use experience of preparations in the process of TCM clinical practice from the aspects of continuously exploring clinical value， improving the construction of information system， focusing on the TCM clinical practice and giving full play to the advantages of discipline cooperation. By realizing the whole life cycle management of medical institution preparations based on three-combination evaluation evidence system， we can promote the transformation of medical institution preparations into innovative Chinese medicines.

Objective:To investigate the clinicopathological features,immunophenotype,and prognosis of head and neck spindle cell squamous cell carcinoma(SCSCC)to improve the understanding and diagnosis of this tumor.Methods:Clinicopathological data collected from January 2012 to December 2022 at the First Affiliated Hospital,Air Force Military Medical University from 20 patients with head and neck SCSCC were retrospectively reviewed for histological morphology and immunophenotype.In situ and fluorescence in situ hybridization were performed to detect EBV-encoded ribonucleic acid(EBER)status and MDM2 gene amplification,respectively.Results:The median age among the 20 SCSCC cases was 67 years with a male-to-female ratio of 4∶1.Tumor locations were laryngeal(35.0%)and sinonasal(30.0%).SCSCC presented as polypoid or exogenous growths(61.5%),often with surface ulceration(90.0%).Histologically,sarcomatoid growth pat-terns were exhibited in 75.0%of the patients(n=15),while the remainder showed granular tissue-like or angiosarcomatoid patterns.Most tumors(65.0%)displayed components of conventional squamous cell carcinomas,with a predominant occurrence of high to moderate dif-ferentiation(91.7%).In terms of immunohistochemistry,AE1/AE3 was expressed in 83.3%(15/18)of cases,while p63 and p40 expression rates were 62.5%and 66.7%,respectively.All cases were negative for EBER.The Ki-67 proliferation index ranged 10%-70%.Overall,33.3%(1/3)of the cases showed MDM2 gene amplification.Among these,the median follow-up time for 18 patients was 18.3 months(range:1-92 months),with 6 survivors and 12 deaths.Conclusions:Head and neck SCSCC is more prevalent among elderly male smokers,predominantly exhibits a polypoid growth pattern,and does not display human papillomavirus or Epstein-Barr virus infection.Diagnosis requires a compre-hensive evaluation of clinical and pathological features and immunophenotype.Surgical resection is the primary treatment method.

Objective:To explore the clinical manifestations of 4 pedigrees with transthyretin related familial amyloid polyneuropathy (TTR-FAP).Methods:The clinical data were collected and analyzed from 4 pedigrees with TTR-AFP, admitted to our hospital from July 2017 to May 2019; 20 patients and 2 asymptomatic carriers of the TTR mutation gene were included. In particular, the detailed data of the 4 probands affected with TTR-FAP came from the 4 different pedigrees were collected. Results:In these 20 patients, the age of onset ranged from 30 to 65; the first symptoms of diarrhea, constipation, alternating episodes of constipation and diarrhea were found; there were damaged peripheral nerve and inexplicable weight loss; cardiomyopathy was noted in 9 patients; orthostatic hypotension was noted in 9 patients, sexual dysfunction in 5, abnormal urination in 6, and blurred vision or corestenoma in 3. TTR mutation gene was confirmed in 7 patients and pathological diagnosis was found in 3 patients. Diflunisal was used in one patient and tafamidis was used 2 patients. Twelve died and 8 patients survived among 20 patients with disease progression. All the 4 probands were male, with an average age of 49.3 years; all patients had different degrees of sensorimotor peripheral neuropathy, autonomic neuropathy and cardiomyopathy; electrophysiological examination suggested length dependent sensory motor peripheral neuropathy of the extremities, with axonal damage as the evidence; and cardiac hypertrophy was noted in echocardiography. The sural nerve biopsy of the 3 probands showed positive Congo red staining. Medical whole exon sequencing indicated that 2 probands had pathogenic mutations (TTR-E74K and TTR-A140S), and 1 proband had likely pathogenic mutation (TTR-S70R). Two asymptomatic carriers of the TTR gene mutation remained normal condition. Conclusion:The clinical manifestations of TTR-FAP include progressive sensorimotor and autonomic neuropathy, and multi-system disorders, such as combining with gastrointestinal problems, hypertrophic myocardium, inexplicable weight loss and blurred vision or corestenoma, which might be important reminders for diagnosis of TTR-FAP.

Objective:To observe and analyze the clinical features and prognosis of proliferative diabetic retinopathy (PDR) with chronic myeloid leukemia.Methods:A retrospective case series study. From May 2011 to December 2020, 5 patients (10 eyes) were included in this study in Eye-ENT Hospital of Fudan University. Basic information about the patient's age, gender, diabetes history and CML history were collected. The endocrine and hematological indexes of all patients were evaluated. All the patients were undertaken visual acuity, intraocular pressure, slit lamp and fundus examination and other examinations to observe the eye conditions. Ophthalmic treatments included panretinal laser photocoagulation, intravitreal injection of anti-vascular endothelial growth factor, vitrectomy. During the follow up period from 5 months to 6 years, prognosis was observed at each office visit. During the follow up period, patients' vision, intraocular pressure, anterior segment and retinal status were observed.Results:There were 4 males and a female in 5 patients. The ages were from 27 to 49 years, with the mean age of 39 years. All patients were bilateral. All patients suffered type 2 diabetes for 3 months to 13 years. Four of them were diagnosed as chronic myeloid leukemia before visiting to ophthalmologists, while the other visited to ophthalmology first due to poor vision. The initial visual acuity ranged from light perception to 0.4 and 6 eyes were less than 0.1. In addition to the typical manifestations of diabetic retinopathy, such as venous tortuous dilation, exudation, microaneurysm and neovascularization, patients also presented with Roth spot as leukemic fundus manifestations. All eyes developed to PDR stage. Abnormal thickening of the neovascular membranes may occur in the lower part of the retina, with secondary traction retinal detachment. All the eyes were treated with pan retinal photocoagulation and 9 eyes underwent pars plana vitrectomy. After treatment, retina of 8 eyes kept flat. The best corrected visual acuity ranged from no light perception to 1.0, and only 4 eyes reached more than 0.2. Unfortunately, one eye lost vision because of secondary neovascular glaucoma.Conclusions:PDR patients with CMLof fundus not only have venous tortuous dilation, exudation, microaneurysm and neovascularization, also present with Roth spot as leukemic fundus manifestations. Diabetic retinopathy combined with CML could progress rapidly, and its aggravating complications such as hyperplastic membrane, vitreous hemorrhage and traction retinal detachment may result in poor visual prognosis. Early screening and treatment can help improve the prognosis of patients.

Objective　To explore the clinical manifestations,pathological characteristics and therapeutic responses of lipid storage myopathy (LSM).Methods　The clinical information of 22 LSM patients were collected and analyzed retrospectively.Results　Proximal limb muscle weakness and motor intolerance were observed in all patients.The concentrations of creatine kinase in blood were found increased to various degrees in 20 cases.EMG examinations showed myogenic damage in 18 cases.Muscle pathology examination of 22 cases showed increased lipid droplets in the muscle fibers.Follow-up data was available for 20 of all the patients after treatment.Nineteen cases were treated with low-doses prednisone,18 cases with riboflavin and 6 cases with L-Carnitine.All patients showed distinct degree of improvement of clinical symptoms within a month.After treatment for 3 months,8/22 patients were recovered except for 2 patients who were relapsed.And repeated treatment was effective.Conclusions　LSM is a curable disease.It is recommended to avoid fever,fatigue and other triggering factors.In the absence of genetic testing,riboflavin,L-carnitine,and prednisone should be used empirically.In the presence of genetic testing,targeted therapy should be performed based on gene mutations.