Myotonic dystrophy type 1(DM1)is a multisystem trinucleotide repeat expansion disorder usually referred to the department of neurology with complaints of progressive muscle weakness and myotonia.This article reported a 33-year-old female patient with DM1 presenting with acute respiratory failure.Muscle bi-opsy in vastus lateralis showed significantly increased internal nuclei.Genetic test show CTG repeat expansions with the size of(847±76)in dystrophia myotonica protein kinase(DMPK)gene on chromosome 19.This case report broadens the clinician's understanding of the atypical clinical manifestations of DM1,so as to avoid missed diagnosis and misdiagnosis.

Objective To establish and validate a Nomogram model for predicting the overall survival(OS)of the patients with intrahepatic cholangiocarcinoma(ICC)based on domestic multicenter data,and screen the beneficiaries of adjuvant chemotherapy based on the prediction model.Methods From December 2011 to December 2017,the data of 278 patients with postoperative pathological diagnosis of ICC from 4 medical centers in our country were collected retrospectively COX regression model was used to screen the independent risk factors of OS and constructed a Nomogram model.This model was used to stratify the risk of OS for all patients and to screen the beneficiaries of adjuvant chemotherapy.Results A total of 278 patients were enrolled,and 23 cases(8.3%)received adjuvant chemotherapy.COX multivariate analysis showed that drinking history,ECOG score,method of hepatectomy,lymph node status,number of tumors,and tumor differentiation were independent risk factors for postoperative OS.The Nomogram model had a C-index of 0.690(95%CI:0.646-0.734)in the training cohort and 0.740(95%CI:0.863-0.617)in the validation cohort.According to risk stratification by Nomogram model,in the high-risk group there was a statistically significant difference in survival between adjuvant chemotherapy and non-adjuvant chemotherapy(P=0.033),whereas in the low-risk group,there was no significant difference in survival(P=0.59).Conclusions Nomogram model based on independent risk factors of OS demonstrated excellent predictive capability for survival and could be used to screen,and identify the patients with ICC who benefit from adjuvant chemotherapy.

Background Tinnitus is a common symptom in workers exposed to noise, and no specific treatment is available yet. Paying attention to the influencing factors of tinnitus is very important to prevent tinnitus. Objective To investigate the situation of tinnitus among noise-exposed workers and its influencing factors. Methods Using a cross-sectional study design, 1011 noise-exposed male workers in Hangzhou were studied. Tinnitus status, age, education, personal protection, smoking history, drinking history, and family history of the participants were surveyed by questionnaires. Binaural pure tone hearing threshold test was performed on all study subjects. The environmental noise in the workplace was measured. The relationships of tinnitus with noise exposure intensity, noise exposure time, noise exposure category, cumulative noise exposure, and wearing hearing protectors among the noise-exposed workers were analyzed by logistic regression. Results The prevalence of tinnitus among the noise-exposed workers was 36.1%. The mean hearing thresholds at 500, 3000, 4000, and 6000 Hz in the right and left ears were higher in the tinnitus group than in the non-tinnitus group (P<0.05); the hearing threshold at 2000 Hz in the right ear was higher in the tinnitus group than in the non-tinnitus group (P<0.05). There was no statistically significant difference between the two groups in the prevalence of monaural speech-frequency hearing loss or monaural high-frequency hearing loss (P>0.05). The on-site noise intensity was divided into three groups: ＜85, 85-＜95, and ≥95 dB(A); the prevalence of tinnitus in the three groups was 19.6%, 40.5%, and 66.7% respectively. The years of noise exposure was divided into three groups: ＜1, 1-＜5, and ≥5 years; the prevalence of tinnitus in the three groups was 17.9%, 34.0%, and 45.4% respectively. The cumulative noise exposure was divided into six groups: ＜80, 80-＜85, 85-＜90, 90-＜95, 95-＜100, and ≥100 dB(A)·year; the prevalence of tinnitus in the six groups was 6.8%, 25.0%, 31.0%, 39.6%, 43.1%, and 46.7% respectively. The prevalence of tinnitus was higher in the non-stationary noise group (42.5%) than in the stationary noise group (26.8%) (χ²=26.18, P<0.01), and the prevalence in the group without or with hearing protection was 39.7% and 35.5% respectively. The results of logistic regression showed that workers exposed to higher intensity, longer noise exposure, higher cumulative noise exposure, and non-stationary noise (reference: stationary noise) resulted in a higher risk of tinnitus (P_trend<0.01 or P<0.01); workers wearing of hearing protection device versus those not showed a lower risk of tinnitus (P<0.05). Conclusion Higher intensity, longer exposure to noise, and more cumulative noise exposure associate with a higher risk of tinnitus. Wearing hearing protective device can reduce the risk of tinnitus. Tinnitus in noise-exposed workers can precede the presentation of abnormal hearing loss.

Objective:To study the neuroprotective effect of Chuanjin Jieyu decoction on a chronic unpredictable stress (CUMS) induced depression rat model.Methods:A total of 48 adult male healthy Sprague-Dawley (SD) rats were randomly selected, with 12 rats as the control group, and the remaining rats were used to establish a CUMS depression model. After successful modeling, the rats were randomly divided into three groups: CUMS group, fluoxetine group, and Chuanjin Jieyu decoction group; 0.9% sodium chloride solution fluoxetine and Chuanjin Jieyu decoction were respectively administered for intervention for 4 weeks. After modeling and intervention, depression levels in rats were measured through open field experiments and sugar water preference experiments; After intervention, real-time fluorescence quantitative polymerase chain reaction (qRT-PCR) was used to detect the expression levels of brain-derived neurotrophic factor (BDNF), tyrosine protein kinase receptor B (TrkB), and cyclic adenosine monophosphate responsive element binding protein (CREB) mRNA in the dentate gyrus of the hippocampus. Immunofluorescence was used to detect the expression levels of BDNF and NeuN. Western blot was used to detect the level of glial fibrillary acidic protein (GFAP).Results:After modeling, the sugar consumption, sugar preference, vertical and horizontal movement results of the CUMS group, fluoxetine group, and Chuanjin Jieyu decoction group were significantly lower than those of the control group (all P<0.01); After intervention, the above indicators in the CUMS group were lower than those in the control group (all P<0.01). The above indicators in the fluoxetine group and the Chuanjin Jieyu decoction group were higher than those in the CUMS group ( P<0.01 or P<0.05), but lower than those in the control group ( P<0.01 or P<0.05). The qRT-PCR results showed that the mRNA expression levels of BDNF, TrkB, and CREB in the CUMS group, the fluoxetine group, and the Chuanjin Jieyu decoction group were significantly lower than those in the control group (all P<0.01); The above indicators in the fluoxetine group and the Chuanjin Jieyu decoction group were significantly higher than those in the CUMS group (all P<0.01). The immunofluorescence results showed that the expression levels of BDNF and NeuN in the CUMS group were significantly lower than those in the control group (all P<0.01); The above indicators in the fluoxetine group and the Chuanjin Jieyu decoction group were higher than those in the CUMS group ( P<0.01 or P<0.05), and all indicators in the Chuanjin Jieyu decoction group and the NeuN expression level in the fluoxetine group were lower than those in the control group ( P<0.01 or P<0.05). Western blot results showed that the expression of GFAP protein in the CUMS group was lower than that in the control group ( P<0.01); The expression of GFAP protein in the fluoxetine group was higher than that in the CUMS group ( P<0.05). Conclusions:Chuanjin Jieyu decoction can improve the behavioral indicators of depression in rats, increase the expression of BDNF, TrkB, CREB, and NeuN in the hippocampal dentate gyrus, suggesting that Chuanjin Jieyu decoction may exert neuroprotective effects through the BDNF/TrkB/CREB pathway.

【Objective:】 To understand the current situation and influencing factors of ethical knowledge, attitude, and practice (KAP) among clinical researchers, enhance their ethical awareness, and improve the quality of clinical researches. 【Methods:】 A self-designed questionnaire was used to conduct a convenient sampling survey of medical staff who have participated in clinical study. 【Results:】 A total of 6,021 subjects were investigated, with males accounting for 28.46% and females accounting for 71.53%. The average age of them was (37.47±11.46) years old. The total score of ethics and the mean scores of the three dimensions of knowledge, attitude and practice were (70.62±12.99), (59.63±12.50), (79.55±19.62) and (74.92±18.29), respectively. Multiple linear regression analysis showed that researchers with male, tertiary hospitals, doctors, ethics-related part-time jobs, postgraduate education, associate senior title or above, and training experience scored higher (P<0.05). 【Conclusion:】 Ethical attitudes have a significant mediating effect between knowledge and practice, accounting for 62.72% of the total effect. Researchers recognize the importance of ethics, but it is urgent to strengthen their knowledge learning and practical application.

A 54-year-old, non-smoking woman was diagnosed as stage ⅣB adenocarcinoma with widespread bone metastasis (cT4N2M1c) in the First Affiliated Hospital, Zhejiang University School of Medicine. Immunohistochemistry result showed the presence of anaplastic lymphoma kinase (ALK) gene rearrangement; next-generation sequencing (NGS) indicated EML4-ALK fusion (E6:A20) with concurrent CCDC148-ALK (C1:A20), PKDCC-ALK (Pintergenic:A20)and VIT-ALK (V15:A20) fusions. After 32 weeks of alectinib treatment, the patient complained cough and exertional chest distress but had no sign of infection. Computed tomography (CT) showed bilateral diffuse ground glass opacities, suggesting a diagnosis of alectinib-related interstitial lung disease (ILD). Following corticosteroid treatment and discontinuation of alectinib, clinical presentations and CT scan gradually improved, but the primary lung lesions enlarged during the regular follow-up. The administration of crizotinib was then initiated and the disease was stable for 25 months without recurrence of primary lung lesions and ILD.
Female ; Humans ; Middle Aged ; Carcinoma, Non-Small-Cell Lung/drug therapy* ; Crizotinib/therapeutic use* ; Lung Neoplasms/drug therapy* ; Anaplastic Lymphoma Kinase/therapeutic use* ; Lung Diseases, Interstitial/diagnosis*

Objective:To analyze the factors influencing the success rate of external cephalic version (ECV) and to create a preoperative scoring scale for stratified management of pregnant women who were preparing for ECV.Methods:This prospective study was conducted on singleton pregnant women who underwent ECV without anesthesia in Fujian Maternity and Child Health Hospital from January 1, 2017, to December 31, 2019. Univariate (two independent samples t-test, Mann-Whitney U test, and Chi-square test) and multivariate logistic regression were used to screen the clinical characteristics affecting the success of ECV, and receiver operating characteristic (ROC) curve was used to determine the cut-off value and convert quantitative variables into dichotomous variables. The independent variables were scored according to the regression coefficient in multivariate logistic regression analysis, and then a preoperative scoring scale was created. The ROC curve was used to calculate the cut-off value for the scoring scale. The subjects were divided into low and high score groups according to the cut-off value. The area under the ROC curve was used for evaluating the effectiveness of the scale in predicting the success of ECV. The success rate of ECV, difficulty of the operation and mode of delivery were compared between the two groups. Results:A total of 1 338 pregnant women met the inclusion criteria during the study period. After the exclusion of 885 women, 165 refused ECV in favor of direct cesarean section, 27 spontaneously converted to cephalic position before ECV, 261 who voluntarily accepted ECV were finally enrolled. ECV succeeded in 202 cases and failed in 59. (1) Favorable factors for ECV without anesthesia were the distance between the fetal breech and ischial spine <－3.5 cm ( OR=0.177, 95% CI: 0.071-0.438, P=0.009), the sum of the fundal height and the station of the fetal breech based on the ischial spine <30.25 cm ( OR=0.225, 95% CI: 0.094-0.537, P=0.001), amniotic fluid index ≥12 cm ( OR=0.399, 95% CI: 0.164-0.969, P=0.042), the surgeon's ability to hold the fetal head or breech with one hand ( OR=0.241, 95% CI: 0.098-0.589, P=0.002; OR=0.219, 95% CI: 0.087-0.546, P=0.001), and the fetal head located on the right or left upper abdomen of the mother ( OR=0.184, 95% CI: 0.059-0.568, P=0.003; OR=0.253, 95% CI: 0.084-0.760, P=0.014). (2) The area under the ROC curve of the preoperative score for predicting the success of ECV was 0.881 (95% CI: 0.821-0.941) and the cut-off value was 5.5. The subjects were divided into low (0-5 scores) and high (6-11 scores) score groups and the area under the ROC curve for predicting the success of ECV by grouping was 0.843 (95% CI: 0.774-0.912). Compared with the low score group, the high score group had a shorter ECV duration [2.0 min (0.5-10.0 min) vs 10.0 min (0.9-25.8 min), Z=－6.83, P<0.001], less attempts [1.0 times (1.0-4.0 times) vs 3.0 times (1.0-5.0 times), Z=－8.41, P<0.001], higher success rate [92.7% (190/205) vs 21.4% (12/56), χ2=127.64, P<0.001], higher rate of vaginal birth [75.4% (147/195) vs 18.5% (10/54)] and lower cesarean section rate [24.6% (48/195) vs 81.5% (44/54)] ( χ2=58.70, P<0.001). Conclusions:Preoperative scoring based on the factors influencing the success rate of ECV (the distance between the fetal breech and ischial spine, the sum of the fundal height and the station of the fetal breech based on the ischial spine <30.25 cm, amniotic fluid index ≥12 cm, the surgeon's ability to hold the fetal head or breech with one hand, and the fetal head locating on the right or left upper abdomen of the mother) is conducive to the individualized evaluation of the difficulty and the success rate of ECV as well as the success rate of vaginal delivery after ECV, which can provide a reference for clinical stratified management of ECV patients.

Objective:To explore the necessity and feasibility of introducing the courses of dental practice management in the undergraduate education of stomatology under the background of the undergraduate entrepreneurship education reform.Methods:In the School of Stomatology of Zhejiang Chinese Medical University, the entrepreneurial course dental practice management was set-up for the junior undergraduate students. CBL, PBL, teacher's lecture, student's lecture on the stage and PPT demonstration, role-play, and team cooperation were taken in the course. And the questionnaires were used to investigate the course setting, necessity, teaching forms and satisfaction from multiple groups. SPSS was performed for data analysis, and the teaching effect was evaluated through the data results.Results:The scores of 108 students in the study [(75.99±5.76) points] were roughly distributed as normal functions. The results of the survey showed that the number of students who planned to have their own private clinics after the course increased by 41 (37.96%) compared with that before the course. All instructors and 33 (78.57%) active dentists thought that the course was reasonable in the undergraduate courses.Conclusion:It is necessary and feasible to introduce the dental practice management course in undergraduate education of stomatology, which will have a certain guiding effect on the long-term development of dentists, and is also beneficial to the reform of innovation and entrepreneurship courses in medical universities.

Objective:To explore the effect of phased nursing based on the compliance curve in pelvic floor function rehabilitation training for patients with stress urinary incontinence.Methods:From January 2019 to June 2020, convenience sampling was used to select 70 patients with stress urinary incontinence in the Urology Surgery, the Second Affiliated Hospital of Zhejiang Chinese Medical University as the research object. Patients were randomly divided into the control group and the intervention group with the random number table method, each with 35 cases. The control group conducted conventional nursing, and the intervention group carried out phased nursing based on the compliance curve on this basis. The treatment effects of the two groups after intervention were compared, and the self-efficacy and quality of life of the two groups of patients before and after the intervention were compared through the Pelvic Floor Muscle Training Self-Efficacy Scale and the Incontinence Quality of Life Questionnaire (I-QOL) .Results:After the intervention, the treatment effect of the intervention group was better than that of the control group, and the difference was statistically significant ( P<0.05) . After the intervention, the self-efficacy score of patients in the intervention group was (1 328.8±276.8) , which was higher than that (1 150.5±324.8) of the control group, and the difference was statistically significant ( P<0.05) . After the intervention, the total score and the scores of each dimension of the I-QOL of the intervention group were higher than those of the control group, and the differences were statistically significant (all P<0.05) . Conclusions:The phased nursing based on the compliance curve applied to the pelvic floor function rehabilitation training for patients with stress urinary incontinence can improve the treatment effect, self-efficacy and quality of life of the patients.

Objective: To investigate the clinical value of lymph node dissection (LND) in the radical resection of intrahepatic cholangiocarcinoma (ICC). Methods: The propensity score matching and retrospective cohort study was conducted. The clinicopathological data of 448 patients with ICC who were admitted to 12 medical centers from December 2011 to December 2017 were collected, including 279 in the Eastern Hepatobiliary Surgery Hospital of Navy Medical University, 32 in the Mengchao Hepatobiliary Hospital of Fujian Medical University, 21 in the First Hospital Affiliated to Army Medical University, 20 in the Cancer Hospital Chinese Academy of Medical Science and Peking Union Medical College, 19 in the West China Hospital of Sichuan University, 18 in the Second Hospital Affiliated to Zhejiang University School of Medicine, 18 in the Renji Hospital Affiliated to Shanghai Jiaotong University School of Medicine, 16 in the Beijing Friendship Hospital Affiliated to Capital Medical University, 10 in the Xuanwu Hospital Affiliated to Capital Medical University, 7 in the Tongji Hospital Affiliated to Tongji Medical College of Huazhong University of Science and Technology, 5 in the Beijing Tiantan Hospital Affiliated to Capital Medical University, and 3 in the Affiliated Hospital of North Sichuan Medical College. There were 281 males and 167 females, aged from 22 to 80 years, with a median age of 57 years. Of the 448 patients, 143 with routinely intraoperative LND were divided into LND group and 305 without routinely intraoperative LND were divided into control group, respectively. Observation indicators: (1) the propensity score matching conditions and comparison of general data between the two groups after matching; (2) intraoperative and postoperative situations; (3) follow-up; (4) survival analysis. Patients were followed up by outpatient examination, telephone interview and email to detect survival of patients and tumor recurrence up to October 31, 2018 or death. Follow-up was conducted once every 3 months within postoperative 1-2 years, once every 6 months within postoperative 2-5 years, and once a year after 5 years. The propensity score matching was realized using the nearest neighbor method with 1∶1 ratio. Measurement data with normal distribution were represented as Mean±SD, and comparison between groups was analyzed using the t test. Measurement data with skewed distribution were represented as M (range), and comparison between groups was analyzed using the Mann-Whitney U test. Count data were described as absolute numbers, and comparison between groups was analyzed using the chi-square test or Fisher exact probability. Kaplan-Meier method was used to calculate survival rates and draw survival curve, and Log-rank test was used to perform survival analysis. Univariate analysis and multivaraiate analysis were conducted using the Log-rank test and COX regression model, respectively. Results: (1) The propensity score matching conditions and comparison of general data between the two groups after matching: 286 of 448 patients had successful matching, including 143 in each group. The number of males and females, cases with hepatitis, cases with grade A and B of Child-Pugh classification, cases with distance from margin to tumor <1 cm and ≥1 cm, cases with highly, moderately, and poorly differentiated tumor, cases with anatomical hepatectomy and nonanatomical hepatectomy before propensity score matching were 77, 66, 33, 96, 47, 70, 73, 105, 38, 79, 64 in the LND group, and 204, 101, 121, 165, 140, 207, 98, 251, 54, 124, 181 in the control group, respectively, showing significant differences in the above indices between the two groups (χ²=7.079, 11.885, 7.098, 14.763, 5.184, 8.362, P<0.05). After propensity score matching, the above indices were 77, 66, 33, 96, 47, 70, 73, 105, 38, 79, 64 in the LND group, and 79, 64, 29, 88, 55, 71, 72, 112, 31, 74, 69 in the control group, respectively, showing no significant difference in the above indices between the two groups (χ²=0.056, 0.329, 2.158, 0.014, 0.936, 1.400, P>0.05). (2) Intraoperative and postoperative situations: the operation time, volume of intraoperative blood loss, cases with intraoperative blood transfusion, cases with postoperative complications, and duration of postoperative hospital stay were 265 minutes (range, 160-371 minutes), 300 mL (range, 200-500 mL), 37, 46, 12 days (range, 9-17 days) for the LND group, and 59 minutes (range, 46-250 minutes), 200 mL (range, 100-400 mL), 24, 25, 9 days (range, 7-11 days) for the control group, respectively. There was no significant difference in the volume of intraoperative blood loss or cases with intraoperative blood transfusion between the two groups (Z=1.700, χ²=3.520, P>0.05). There were significant differences in the operation time, cases with postoperative complications, and duration of postoperative hospital stay (Z=6.520, χ²=8.260, Z=4.270, P<0.05). (3) Follow-up: 143 patients in the LND group was followed up for 18-26 months. The 1-, 3-, 5-year survival rates were 66.9%, 32.8%, 25.0%, and the median survival time was 22.0 months (range, 18.0-26.0 months). In the control group, 143 patients were followed up for 11-32 months. The 1-, 3-, 5-year survival rates were 71.7%, 38.0%, 31.0%, and the median survival time was 22.0 months (range, 11.0-32.0 months). There was no significant difference in the overall survival between the two groups (χ²=0.466, P>0.05). (4) Survival analysis: results of univariate analysis showed that distance from margin to tumor, tumor diameter, the number of tumors, microvascular tumor thrombus, and lymph node metastasis by pathological examination were related factors for prognosis of patients undergoing radical resection of ICC (χ²=10.662, 9.477, 16.944, 6.520, 11.633, P<0.05). Results of multivariate analysis showed that distance from margin to tumor<1 cm, tumor diameter>5 cm, multiple tumors, microvascular tumor thrombus, positive lymph node metastasis by pathological examination were independent risk factors for prognosis of patients undergoing radical resection of ICC (hazard ratio=0.600, 1.571, 1.601, 1.750, 1.723, 95% confidence interval: 0.430-0.837, 1.106-2.232, 1.115-2.299, 1.083-2.829, 1.207-2.460, P<0.05). Conclusion Lymph node metastasis is an independent risk factor affecting the prognosis of ICC patients, and it is necessary to perform LND in radical resection for patients with ICC and lymph node metastasis.