Objective To investigate clinical first selected method on the differential diagnosis at early stage between amyotrophic lateral sclerosis(ALS) and cervical spondylosis.Methods The clinical features and EMG in 53 patients with ALS,14 patients with cervical spondylotic myelopathy(CSM) and 23 patients with cervical spondylotic radiculopathy(CSR) were analysed.And then the value of ratio of every probability was calculated.According to the value of ratio, the clinical features and EMG were compared in differential dia gnosis.Results A multiprogram that consisted of high multiple clinical features was used in differential diagnosis, true positive rate is 98%, and true negative rate is 86.5%,EMG still couldn't be used in the differential diagnosis.Conclusion The program is the first selected method, which can be used in the differential diagnosis between ALS and CSM/CSR.

Objective To study how to reduce the incidence rate of skin flap necrosis after the eradicative resection of breast cancer. Methods 45 patients with breast cancer underwent eradicative operation with new methods to prevent the necrosis of skin flap,and they were studied and compared with 1210 cases treated by traditional ways. Results With the new methods adopted, the necrosis rate of skin flap following eradicative operation of breast cancer was reduced from 45%(original)to 2%(present).Statistic analysis showed that there was outstanding difference between them. Conclusion In the eradicative operation of breast cancer,the key to prevent the necrosis rate of skin flap is:prevention of subaxillary lymphatic fistula, two tubular drainage set individually in subcostalis and subaxillaris, appropriate force of chest bandaging, proper thickness of skin flap and perfect suture without tension.

Objective:To evaluate the value of synthetic MRI combined with DWI in the diagnosis of benign and malignant breast lesions.Methods:The data of 184 consecutive patients with suspected breast lesions in Yunnan Cancer Hospital from July to September 2019 were prospectively analyzed. All patients were randomly assigned to training group ( n=110) and validation group ( n=74), and underwent conventional MRI and synthetic MRI respectively before and after contrast injection. At the maximum slice of the lesion, the ROI was drawn along the edge and recorded as "tumor". In the solid area with the most obvious tumor enhancement, the second ROI was drawn and recorded as "local". At the same time, ADC values (ADC local and ADC tumor) and relaxation time values (T local and T tumor) were measured. T and T + represented the relaxation time value of the ROI pre-and post-contrast scanning. ΔT% represented the relative change rate in T value between pre-and post-contrast scanning.The rank sum test was used to test the quantitative parameters of benign and malignant breast lesions in the training group and the validation group, and the variables with P<0.05 were included in the binary logistic regression analysis to screen the independent variables and establish the prediction model. The area under ROC curve was used to evaluate the discrimination of parameters and models. The clinical applicability of model was analyzed by decision curve analysis (DCA). Results:In the training group, univariate analysis showed that there were significant differences in T 1tumor, T 1+tumor, ΔT 1% tumor, T 2local, T 2+local, T 2tumor and T 2+tumor, ADC local, ADC tumor between benign and malignant breast lesions ( P<0.05). Multivariate logistic regression analysis showed that T 1+tumor, ΔT 1% tumor, T 2tumor, ADC local, ADC tumor were independent variables in the diagnosis of breast cancer. The relaxation time model (model A: T 1+tumor, ΔT 1% tumor, T 2tumor) and ADC model (model B: ADC local, ADC tumor) established by combining the above variables had the same diagnostic efficiency (AUC=0.905, 0.914, Z=-1.874, P=0.062), and the multi-parameter combination model (model C: T 1+tumor, ΔT 1% tumor, T 2tumor, ADC local, ADC tumor) had the highest diagnostic efficiency (AUC=0.965). DCA analysis showed that when the threshold probability ranges between 21%-99% (training cohort) and 15%-99% (validation cohort), the net benefit of model C was better than model A and B. Conclusion:The multi-parameter combined prediction model established based on the relaxation time value and ADC can identify breast cancer efficiently and can be used as an auxiliary diagnostic tool.

The previous pharmacokinetic methods can be only limited to drug analysis in vitro, which provide less information on the distribution and metabolismof drugs, and limit the interpretation and assessment of pharmacokinetics, the determination of metabolic principles, and evaluation of treatment effect. The objective of the study was to investigate the pharmacokinetic characteristics of gene recombination angiogenesis inhibitor Kringle 5 in vivo. The SPECT/CT and specific 131I-Kringle 5 marked by Iodogen method were both applied to explore the pharmacokinetic characteristics of 131I-Kringle 5 in vivo, and to investigate the dynamic distributions of 131I-Kringle 5 in target organs. Labeling recombinant angio-genesis inhibitor Kringle 5 using 131I with longer half-life and imaging in vivo using SPECT instead of PET, could overcome the limitations of previous methods. When the doses of 131I-Kringle 5 were 10.0, 7.5 and 5.0 g/kg, respectively, the two-compartment open models can be determined within all the metabolic process in vivo. There were no significant differences in t1/2α, t1/2β, apparent volume of distribution and CL between those three levels. The ratio of AUC(0 ? 1) among three different groups of 10.0, 7.5 and 5.0 g/kg was 2.56:1.44:1.0, which was close to the ratio (2:1.5:1.0). It could be clear that in the range of 5.0–10.0 g/kg, Kringle 5 was characterized by the first-order pharmacokinetics. Approximately 30 min after 131I-Kringle 5 was injected, 131I-Kringle 5 could be observed to concentrate in the heart, kidneys, liver and other organs by means of planar imaging and tomography. After 1 h of being injected, more radionuclide retained in the bladder, but not in intestinal. It could be concluded that 131I-Kringle 5 is mainly excreted through the kidneys. About 2 h after the injection of 131I-Kringle 5, the radionuclide in the heart, kidneys, liver and other organs was gradually reduced, while more radionuclide was concentrated in the bladder. The radionuclide was completely metabolized within 24 h, and the distribution of radioactivity in rats was similar to normal levels. In our study, the specific marker 131I-Kringle 5 and SPECT/CT were suc-cessfully used to explore pharmacokinetic characteristics of Kringle 5 in rats. The study could provide a new evaluation platform of the specific, in vivo and real-time functional imaging and pharmacokinetics for the clinical application of 131I-Kringle 5.

Objective To review the clinical characteristics of prostate mucinous adenocarcinoma cases and update literatures,and recommend the corresponding clinical treatment strategy.Methods From October 2010 to March 2018,36 cases of prostate mucinous adenocarcinoma were involved from 5 urinary centers in China,including 9 cases from Shanghai Changhai Hospital,4 cases from Wuhan Tongji Hospital,13 cases from Shanghai Renji Hospitals,8 cases from the First Affiliated Hospital of Nanjing Medical University,and 2 cases from Sichuan West China Hospitals.The patients' age were (66.8 ±7.2) years (53-83 years) and the median PSA was 22.89 ng/ ml (2.67-1786 ng/ ml).Prostate biopsy confirmed Gleason score 3 + 3 points in 6 cases,3 + 4 points in 9 cases,4 + 3 points in 5 cases,8 points in 11 cases,and 9 to 10 points in 5 cases.According to D'Amico risk stratification,2 patients were in the low-risk group,9 in the intermediate-risk group,and 25 in the high-risk group.Eight cases underwent radical retroperitoneal prostatectomy,13 cases underwent laparoscopic radical prostatectomy,and 12 cases underwent robotic laparoscopic radical prostatectomy.Twenty-three cases underwent pelvic lymphadenectomy,including 12 cases of bilateral obturator lymph node dissection,and 11 cases of bilateral obturator + intraorbital + para-vascular para-aortic lymphadenectomy.Results All 36 operations were completed successfully.Twenty-three cases underwent pelvic lymphadenectomy,including 12 of bilateral obturator lymph node dissection,and 11 of bilateral obturator,intraorbital,and para-aortic lymphadenectomy.Pathological examination showed 9 cases of prostate mucinous adenocarcinoma,26 cases of mucinous adenocarcinoma with acinar adenocarcinoma,and 1 case of mucinous adenocarcinoma with neuroendocrine and immunohistochemical positive of MUC2 (+).Among 33 cases undergoing radical surgery,the pathological stage of ≤T2b in 12 cases (36.3％),T2c in 7 cases (21.2％),T3a in 7 cases (21.2％),T3b in 6 cases (18.2％),and T4 in 1 case (3.0％).Four cases had positive pelvic lymph nodes and 9 cases had positive margin.The median follow-up period was 26 months (6-48 months).The biochemical recurrence occurred in 6 patients one year after surgery,including 3 cases in the intermediaterisk group and 3 cases in the high-risk group.Six cases with postoperative biochemical recurrence and 19 cases with PSA ＞ 0.2 ng/ml after radical or palliative resection underwent adjuvant androgen deprivation therapy(ADT),no postoperative adjuvant radiotherapy or chemotherapy was administered,and 4 cases progressed to castration-resistant prostate cancer.Four cases with CRPC were in the high-risk group and had underwent radical surgery,and the median period progressed to CRPC was 26 months(3-37months)with 2 cases of death.However,there was no significant difference in the rate of biochemical recurrence and the incidence of CRPC in the low-risk group,the intermediate-risk group and the high-risk group.In addition,2 cases had metastases,with pelvic MRI presenting pelvic multiple nodular mass in one case which was consistent with recurrence and metastasis at the 5th month after radical surgery,and pathological examination presenting the mucinous adenocarcinoma being neurosecretory in another case and mestastasis being detected on glans at the 3rd months after radical surgery.The recovery rate of urinary continience at 6 and 12 months after radical surgery was 86.2％ (31/36) and 89.7％ (32/36) respectively.Conclusions Prostate mucinous adenocarcinoma is a variant of acinar adenocarcinoma.This study clarifies prostate mucinous adenocarcinoma of Chinese patients with high Gleason scores,advanced pathological stage,variant in prognosis,and prone to recurrence and metastasis.For treatment strategy,the low-risk and intermediate-risk mucinous adenocarcinoma is recommended undergoing radical surgery,and the prognosis maybe good.High-risk mucinous adenocarcinoma could treated with radical surgery or palliative surgery with adjuvant ADT,and most high-risk patients can benefite,with a small number of poor prognosis.

Objective To evaluate opioids utilization of cancer ached inpatients in the integrative traditional Chinese and western medicine hospital and provide suggestion for the rational utilization of opioids .Method DDDs ,DUI ,the distribution of cancer pain ,pain scores of the discharged patients and the utility of opioids were evaluated and analyzed by retrieving the medi-cal records from January in 2013 to December in 2014 .Results 292 medical records were selected and analyzed .Among them , 89 patients′pain score≥3 .The top opioids of DDDs were sufentanil citrate injection and fentanyl derivatives ,which is the main medication in treating the cancer pain patients .And the irrationaluseof fentanyl transdermal system was a common phenomenon among different departments .Conclusion Theutilization of opioids was basically rational ,but there still had some deficiencies . The intervention and management of narcotic drugs should be strengthened and deepened .

Objective:To evaluate the efficacy and safety of peroral endoscopic myotomy (POEM) in achalasia of cardia (AC) patients with the long course.Methods:A total of 159 AC patients who received POEM from January 2015 to March 2022 in the First Affiliated Hospital of Soochow University were divided into the long course group (≥10 years) and the non-long course group (<10 years). The baseline information, POEM procedure and postoperative recurrence were compared and the differences between the recurrent patients and non-recurrent patients in the long course group were explored.Results:The age (57.09±14.30 years VS 42.08±15.68 years, t=5.569, P<0.001), the rate of treatment history [28.9% (13/45) VS 9.6% (11/114), χ2=9.319, P=0.020], the proportion of Henderson grade Ⅲ esophagus [17.8% (8/45) VS 6.1% (7/114), χ2=7.020, P=0.030] in the long course group were significantly higher than those in the non-long course group. The recurrence rate in the long course group was significantly higher than that in the non-long course group [33.3% (15/45) VS 14.9% (17/114), χ2=6.811, P=0.009]. In the long course group, the age (62.50 ± 16.94 years VS 53.77 ± 12.95 years, t=-2.121, P=0.040), and the rate of treatment history [53.3% (8/15) VS 16.7% (5/30), χ2=6.544, P=0.016] in the recurrent patients were higher than those in the non-recurrent patients. Conclusion:POEM is safe and effective for long-course AC patients. In patients with the long course, the aged patients with previous treatment are more likely to relapse.

Objective:To evaluate the value of contrast-enhanced ultrasound (CEUS) in the diagnosis and differential diagnosis of bladder urothelial carcinoma(BUC).Methods:A comparative analysis of 138 patients with bladder lesions (123 cases of BUC and 15 cases of other benign lesions) who were hospitalized in Union Hospital, Fujian Medical University from January 2019 to May 2021 were confirmed by pathology. All patients underwent two-dimensional ultrasound, color Doppler ultrasound, CEUS examination, the time intensity curve (TIC) of the region of interest(ROI) before operation was drawn, the ultrasound examination results with the pathological diagnosis results were compared and its diagnostic efficiency was analyzed.Results:Among the 138 cases of bladder lesions, 98 cases were single lesions and 40 cases were multiple lesions. In all single lesions, 95 cases were found by two-dimensional ultrasound and 3 cases were missed, while in all multiple lesions, 24 cases were found by two-dimensional ultrasound and 16 cases were missed, but all cases could be shown by CEUS. The sensitivity of CEUS to multiple bladder lesions was higher than that of two-dimensional ultrasound ( P<0.05). Besides, the differences between BUC and benign lesions in color blood flow distribution intensity and CEUS performance were significant (both P<0.05). Malignant lesions were mostly "less- to -rich" blood flow signals, and benign lesions were mainly "less- to- no" blood flow signals.In addition, in the CEUS examination, 83.7% (103/123) of BUC were high enhancement, and only 33.3% (5/15) of benign lesions were high enhancement. The diagnostic accuracy, specificity and negative predictive value of CEUS(89.9%, 46.7%, 53.8%) were higher than that of two-dimensional ultrasound(67.4%, 13.3%, 13.3%). The area under the curve, the rising slope and the peak intensity of BUC were all higher than those of benign lesions, and the differences were significant(all P<0.05), but none of them was independent risk factor for BUC ( P>0.05). Conclusions:CEUS can significantly improve the diagnostic sensitivity of bladder multiple lesions and help to improve the diagnostic accuracy of BUC, while the area under the curve, the rising slope and the peak intensity of TIC were not the independent risk factors for BUC.

OBJECTIVE: To explore the genetic etiology of Vici syndrome in a Chinese family. METHODS: Whole exome sequencing (WES) technology was used to detect gene variants in a fetus of abnormal ultrasonic structure without abnormalities in routine chromosome karyotype analysis and SNP-array. Sanger sequencing and bioinformatics prediction were performed for the suspected variants of the fetus and parents. RESULTS: The fetus and the elder sister have carried c. 2427delC (p.T809fs) and c.1886A>T (p.E629V) compound heterozygous variants of the EPG5 gene, which were respectively inherited from their mother and father. Neither variant was reported previously. According to ACMG guidelines, the c.2427delC variant was predicted as pathogenic, while the c.1886A>T variant was of uncertain significance. PolyPhen-2 and PROVEAN software indicated that c.1886A>T variant was probably damaging. CONCLUSION The c.2427delC and c.1886A>T variants of the EPG5 gene probably underlie the pathogenesis of the Vici syndrome in this family. Above finding has enriched the variational spectrum of EPG5 gene and provided a basis for genetic counseling and prenatal diagnosis for the family.
Aged ; Agenesis of Corpus Callosum ; Autophagy-Related Proteins ; Cataract ; Female ; Humans ; Mutation ; Pregnancy ; Vesicular Transport Proteins/genetics* ; Whole Exome Sequencing