Objective:To analyze the clinical efficacy and prognostic factors of comprehensive treatment for atypical teratoid / rhabdomyoma tumor (AT/RT).Methods:Clinical data of children diagnosed with AT/RT who underwent radiotherapy in Department of Oncology of Xinhua Hospital affiliated to Shanghai Jiao Tong University School of Medicine from November 2012 to September 2022 were retrospectively analyzed. Pearson Chi-square test or continuous-corrected Chi-square test or Fisher's exact probability method were used for inter-group comparison of categorical variables. Independent sample t-test or Wilcoxon rank-sum test were used for inter-group comparison of continuous variables. Kaplan-Meier method was used to calculate the 1-year and 2-year overall survival (OS) rate and progression free survival (PFS) rate. Univariate and multivariate Cox regression analyses were employed to determine relevant prognostic factors. Results:A total of 45 patients were included, with a male/female ratio of 1.65:1, including 27 children aged ≥3 years old. All patients received surgery and radiotherapy in which 39 patients received chemotherapy, 41 craniospinal irradiation (CSI), and 4 whole brain or focal radiation therapy. The median follow-up was 28 (13.5, 49) months. A total of 14 patients died after comprehensive treatment. The 1-year OS rate was 80.0% and the PFS rate was 71.1%. The 2-year OS rate was 75.5% and the PFS rate was 65.7%. Survival prognostic analysis showed negative imaging assessment after radiotherapy ( HR=0.087, 95% CI: 0.011-0.697, P=0.022) was a favorable factor for PFS. The primary tumor<4.8 cm ( HR=0.221, 95% CI: 0.052-0.935, P=0.040) and CSI ( HR=0.085, 95% CI: 0.011-0.651, P=0.018) were favorable factors for OS. In subgroup analysis, CSI also improved OS in children aged ≥3 years ( HR=0.014, 95% CI: 0-0.470, P=0.017), but there was no significant difference in PFS. In children without cerebrospinal fluid dissemination, negative radiographic results after radiotherapy ( HR=0.066, 95% CI: 0.009-0.481, P=0.007; HR=0.076, 95% CI: 0.008-0.695, P=0.024, respectively) and CSI (HR=0.105, 95% CI: 0.012-0.937, P=0.044; HR=0.054, 95% CI: 0.005-0.629, P=0.020, respectively) were favorable factors for PFS and OS in children, and the primary tumor<4.8 cm also suggested a longer OS ( HR=0.094, 95% CI: 0.013-0.690, P=0.020). Conclusions:Comprehensive treatment including radiotherapy improves clinical prognosis of children with AT/RT. Our study shows that negative imaging results after radiotherapy are associated with PFS improvement. The primary tumor<4.8 cm and CSI are favorable factors for OS. CSI is also a significantly positive prognostic factor in children aged ≥3 years and those without cerebrospinal fluid dissemination.

Objective To improve clinicians'understanding of the diagnosis and treatment of neonatal hyperthyroidism which is rare in clinical practice.Methods The clinical data of a neonatal hyperthyroidism case diagnosed and treated was retrospectively analyzed,and the relevant literature was reviewed.Results A female infant,borned at 32 weeks and 6 days of gestation,exhibited dyspnea,a rapid heart rate,hepatosplenomegaly,thrombocytopenia,cholestasis,and abnormal liver function that continued to deteriorate after birth.She was later diagnosed with neonatal hyperthyroidism after undergoing thyroid function and antibody tests.After treated with methimazole,propranolol and other symptomatic supportive therapies,the infant's symptoms improved and she was discharged in good health.Conclusions The clinical manifestations of neonatal hyperthyroidism are non-specific and the symptoms are atypical.Therefore,when encountering symptoms such as growth retardation,tachycardia,cardiac insufficiency,tachypnea,thrombocytopenia,liver injury,hepatosplenomegaly,and a small head circumference,it is essential to consider the possibility of congenital hyperthyroidism.Timely thyroid function and antibody tests should be conducted to confirm the diagnosis,followed by early drug intervention to enhance the prognosis.

Objective:To study the urinary iodine levels of children in Jiangxi Province and provide a basis for prevention and treatment of iodine deficiency disorders and scientific iodine supplementation.Methods:From March to July in 2022, a systematic sampling method was used in 99 counties (cities, districts, abbreviated as counties) in Jiangxi Province. One township (street) was selected from each county in five directions: east, west, south, north and middle. One primary school was selected from each township (street), and 40 non boarding children aged 8 - 10 (half male and half female) were selected from each primary school. One random urine sample was collected to test urinary iodine, and the distribution of urinary iodine in different regions, genders, and ages were compared.Results:A total of 19 842 urine samples were collected from children, with a median urinary iodine level of 181.90 μg/L. The median urinary iodine levels of children in different cities ( H = 1 014.05, P < 0.001), genders ( Z = 6.44, P < 0.001) and ages groups ( H = 29.82, P < 0.001) were compared, and the differences were statistically significant. Conclusion:The urinary iodine level of children in Jiangxi Province is at an appropriate level, but the distribution of urinary iodine is uneven among different regions, genders, and ages, indicating that scientific and precise iodine supplementation is necessary for the prevention and treatment of iodine deficiency disorders.

Objective:To evaluate the clinical efficacy of multi-disciplinary single center's CCCG-HB-2016 regimen in the treatment of hepatoblastoma (HB) in children.Methods:Clinical data of 36 HB patients treated with CCCG-HB-2016 program from Aug 2016 to March 2020 were analyzed.Results:These 36 patients included 20 boys and 16 girls. The serum AFP was all higher than 2 792 ng/ml,there was a correlation between AFP and tumor risk stratification ( H=14.973, P<0.05). Twenty eight cases (77.78%) were epithelial type and 8 cases (22.22%) were mixed epithelial mesenchymal type.All children were treated by tumor resection combined with chemotherapy, and there was a correlation between tumor risk stratification and surgical resection of liver lobe ( H=8.847, P<0.05). The probability of bone marrow suppression in the low-risk group was 58.33% (35/60),that in the intermediate-risk group was 73.49% (61/83) and in the high-risk group was 80.23% (69/86).All 36 cases were followed up to March 31, 2020,with an average follow-up of 21.9 months and the median survival was 22.5 months.The overall survival rate (OS) and event-free survival rate (EFS) were 97.2% and 83.3% respectively. Conclusions:The multidisciplinary CCCG-HB-2016 regimen was with a high success rate and along with a high incidence of bone marrow suppression.

Objective To investigate the clinical manifestations,imaging and pathological features and treatment prognosis of primary renal sarcoma in adults.Methods A retrospective analysis was performed on the clinical data of 48 patients with primary renal sarcoma from January 2009 to December 2018 in the first affiliated hospital and cancer center of Sun Yat-Sen university.There were 30 males and 18 females.Their aged ranged from 27 to 76 yrs with an average age of 50 yrs.A total of 24 patients presented with lumbar and abdominal pain.Abdominal mass was found in 2 cases.Gross hematuria was noticed in 4 cases.Febrile was recorded in 2 cases and 21 cases were diagnosed by physical examination.31 tumors located in the left kidney and 17 tumors located in the right kidney.The tumor diameter ranged from 3 to 16 cm with an average diameter of 8 cm.All patients underwent ultrasound or CT/MRI examination of the urinary system before surgery.The ultrasound showed the undistributed echo inside the tumor with the undistinguished border.The image of necrosis and liquefaction could be seen in some cases.The CT/MRI examination showed the lesion site with the necrosis,liquefaction or cystic changing.The mass exhibited the unregular enhancement with undistinguished border line.The mean diameter of liposcarcoma was 10.2 cm.CT scan demonstrated the relative low density of tumor,which was hard to be identified with AML.The average diameter of leiomyosarcoma was 6.5 cm.The enhanced CT scan showed the low density of tumor,compared with renal parenchyma.46 patients underwent radical nephrectomy,2 patients underwent renal tumor biopsy.And postoperative follow-up was performed.Results Pathological diagnosis revealed that 19 cases with liposarcoma,9 cases with leiomyosarcoma and 4 cases with synovial sarcoma,especially 4 cases with Ewing' s sarcoma and 12 cases with other sarcoma.36 cases were followed up and survived for 4 to 64 months.The average survival time was 28 months.The longest mean survival time was seen in patients with liposarcoma,which was 32 months (ranging 11 to 64 months).The mean survival time of synovial sarcoma group was 25 months (ranging 5-58 months).The mean survival time of Ewing's sarcoma group was 22 months(ranging 12-46 months).and the survival time of leiomyosarcoma group was the shortest 20 months (ranging 4-36 months).Conclusion Renal sarcoma is rare and highly malignant.It needs to be diagnosed with clinical manifestations,imaging and pathological data together.

Objective To summarize the 18F-fluorodeoxyglucose (FDG) PET/CT findings of pediatric rhabdomyosarcoma (RMS),and to explore the value of PET/CT in the diagnosis of RMS.Methods PET/CT images and clinical data of 29 RMS patients (15 males,14 females,age range:1-14 years) histopathologically confirmed from July 2011 to September 2017 were evaluated retrospectively.The imaging features were analyzed,and the maximum standardized uptake value (SUVmax) ratios of tumors to the liver (TLR) in different pathological types of RMS were calculated and compared.Mann-Whitney u test was used to analyze the data.Results There were 19 patients with embryonic RMS,7 with alveolar RMS,and 3 with unknown types.The median SUVmax of primary lesion was 6.8(4.3,8.9).There were no statistically significant differences in SUVmax(6.2(4.3,8.8) vs 5.3(4.0,9.0)) and TLR (3.6(2.6,8.1) vs 3.0(2.2,6.3)) between embryonic RMS and alveolar RMS (u values:60.00 and 48.50,both P ＞ 0.05).PET/CT detected lymph nodes lesions with increased metabolism in 15 patients,lung lesions in 6 patients,bone or bone marrow lesions in 4 patients and breast involvement in 2 patients.Conclusion 18 F-FDG PET/CT can indicate the extent and metabolic activity of RMS,thus providing information for clinical practice.

Androgen receptor (AR) is a ligand-activated transcription factor that plays a pivotal role in the development and progression of many severe diseases such as prostate cancer, muscle atrophy, and osteoporosis. Binding of ligands to AR triggers the conformational changes in AR that may affect the recruitment of coactivators and downstream response of AR signaling pathway. Therefore, AR ligands have great potential to treat these diseases. In this study, we searched for novel AR ligands by performing a docking-based virtual screening (VS) on the basis of the crystal structure of the AR ligand binding domain (LBD) in complex with its agonist. A total of 58 structurally diverse compounds were selected and subjected to LBD affinity assay, with five of them (HBP1-3, HBP1-17, HBP1-38, HBP1-51, and HBP1-58) exhibiting strong binding to AR-LBD. The IC values of HBP1-51 and HBP1-58 are 3.96 µM and 4.92 µM, respectively, which are even lower than that of enzalutamide (Enz, IC = 13.87 µM), a marketed second-generation AR antagonist. Further bioactivity assays suggest that HBP1-51 is an AR agonist, whereas HBP1-58 is an AR antagonist. In addition, molecular dynamics (MD) simulations and principal components analysis (PCA) were carried out to reveal the binding principle of the newly-identified AR ligands toward AR. Our modeling results indicate that the conformational changes of helix 12 induced by the bindings of antagonist and agonist are visibly different. In summary, the current study provides a highly efficient way to discover novel AR ligands, which could serve as the starting point for development of new therapeutics for AR-related diseases.
Androgen Receptor Antagonists ; pharmacology ; Androgens ; metabolism ; pharmacology ; Biological Assay ; Cell Line, Tumor ; Drug Discovery ; methods ; Humans ; Ligands ; Male ; Molecular Docking Simulation ; Molecular Dynamics Simulation ; Phenylthiohydantoin ; analogs & derivatives ; pharmacology ; Principal Component Analysis ; Prostatic Neoplasms ; drug therapy ; Protein Binding ; physiology ; Protein Conformation ; drug effects ; Receptors, Androgen ; metabolism

BACKGROUND: Surgery was not standard-of-care of patients with advanced lung cancer. However, a serial of retrospective studies demonstrated that thoracic dissemination (M1a) patients could benefit from contraindicated surgery. After non-standard treatment, how should these patients choose following treatment approaches? Herein, we conducted this retrospective study to explore subsequent optimal treatment approaches. METHODS: Different therapeutic approaches were evaluated by comparing progression-free survival (PFS), overall survival (OS), time to treatment interval (TTI) using the Kaplan-Meier method and Log-rank test. A Cox proportional hazards regression model was used for multivariate analysis. RESULTS: 141 eligible were enrolled. The median PFS of chemotherapy group, targeted therapy group and observation group were 14.7, 41.0 and 31.0 months, respectively (95%CI: 19.01-26.01; P<0.001). There was no significantly statistically difference between median PFS of targeted group and observation group (P=0.006). The median OS were 39.0, 42.6 and 38.1 months (95%CI: 32.47-45.33; P=0.478). The median PFS and OS of TTI<3 months and TTI ≥3 months were 15.2 months versus 31.0 months (95%CI: 19.01-26.06; P<0.001) and 41.7 months versus 38.7 months (95%CI: 32.47-45.33; P=0.714). Multivariate analyses revealed gender (P=0.027), lymph node status (P=0.036) and initial therapy (P<0.001) were independent prognostic factors for PFS. CONCLUSIONS Observation did not shorten survival of thoracic dissemination patients with lung adenocarcinoma or squamous carcinoma, therefore, it could be an favorable option. But prospective randomized controlled study was needed to confirm its validity.
Adenocarcinoma ; drug therapy ; mortality ; pathology ; surgery ; Adenocarcinoma of Lung ; Adult ; Aged ; Aged, 80 and over ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Carcinoma, Squamous Cell ; drug therapy ; mortality ; pathology ; surgery ; Disease-Free Survival ; Female ; Humans ; Lung Neoplasms ; drug therapy ; mortality ; pathology ; surgery ; Male ; Middle Aged ; Neoplasm Staging ; Retrospective Studies ; Young Adult

Objective To evaluate the clinical effects of color Doppler ultrasound in perforating vein closure by foam sclerotherapy for the treatment of venous leg ulcer.Methods Choosing the patients with venous ulcers (classified as C6) that underwent superficial and perforating veins closure by ultrasound-guided foam sclerotherapy in the Department of Vascular Surgery,First Affiliated Hospital of Chongqing Medical University,during December 2014 to August 2016.All patients were followed up by color Doppler scanning for veins closure,and the postoperative healing of skin ulcers were observed at the same time.Results A total of 114 patients with 119 limbs were enrolled,which were confirmed 1-3 perforating vein lesions by ultrasound.An average of 2.1 perforators were closed per leg (1-3 perforators).The average follow-up period was 11.3 months (1-21 months).The healing rate is 100％ and all the insufficient perforating veins were occlusion 1 month postoperative.Three cases of ulcer recurrence,and 8 cases of insufficient perforator recanalization were found.Serious complications did not appear.Conclusions Color doppler ultrasound can improve the safety and efficacy of perforating vein closure by foam sclerotherapy for the treatment of venous leg ulcer,and it has a high clinical value.

Objective To investigate the incidences, risk factors, genotypes and epidemiology of community-acquired blood stream infection caused by extended spectrum β-lactamases (ESBLs)-producing Escherichia coli and Klebsiella pneumonia strains and to analyze the sensitivity of those ESBLs producing strains to commonly used antibiotics. Methods Forty-two patients who were diagnosed with community-ac-quired blood stream infection caused by Escherichia coli or Klebsiella pneumonia strains in Sichuan Provincial People′s Hospital were recruited in this study. Disc diffusion method was used for the phenotypic confirmato-ry test of ESBLs. Agar dilution method was performed to measure the antimicrobial susceptibility of the ESBLs-producing strains to 13 clinically commonly used antibiotics. Genotypes of the ESBLs-producing strains were identified by polymerase chain reaction (PCR). Multilocus sequence typing (MLST) was used to analyze the epidemiology of ESBLs-producing strains. Logistic regression analysis was performed to analyze the risk factors for community-acquired blood stream infection. Results The ESBLs-producing Escherichia coli strains accounted for 56. 3% (18 / 32) and the ESBLs-producing Klebsiella pneumoniae strains accounted for 20% (2 / 10). All of the 20 ESBLs-producing strains were sensitive to imipenem, meropenem, ertapen-em, nitrofurantoin and moxalactam. The ESBLs-producing strains sensitive to amikacin, piperacillin-tazobactam and fosfomycin accounted for 95% , 90% and 85% , respectively. Drug resistance rates of the 20 strains to cefotaxime, levofloxacin, ciprofloxacin and cefepime were relatively high accounting for 100% , 80% , 80% and 75% , respectively. Among the 20 ESBLs-producing strains, 7 strains only carried the CTM gene, while the other 13 strains were all positive for two genotypes of ESBLs, mainly identified as TEM+CTM-M-14 and TEM+CTM-15 genotypes. The 18 Escherichia coli strains were classified into 10 ST types, most of which were ST131 type, followed by ST10 and ST38 types. This study indicated that malignant tumor might be a possible risk factor. Conclusion The prevalence of community-acquired blood stream infection caused by ESBLs-producing Escherichia coli strains was becoming increasingly serious. Malignant tumor might be the risk factor associated with the producing of ESBLs in Escherichia coli and Klebsiella pneumonia strains. TEM+CTX-M-14 was the predominant genotype of ESBLs-producing strains and the prevalent clone was ST131 type. Carbapenems and enzyme inhibitor compounds were ideal drugs for the treatment of commu-nity-acquired blood stream infection caused by ESBLs-producing Escherichia coli and Klebsiella pneumonia strains. This study was limited by the small sample size. Therefore, it is necessary to conduct further resear-ches based on a large number of samples.