Objective To investigate the clinical features and gene mutation of a newborn with neonatal-onset ornithine transcarbamylase deficiency (OTCD) and report the multidisciplinary perinatal management of the mother with late-onset OTCD.Methods The clinical features,biochemical data and the treatment of a newborn boy with OTCD and his mother admitted by Xin Hua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine in April,2013,were collected.The ornithine transcarbamylase (OTC) gene in the family was analyzed.Results Serum ammonium in the male newborn gradually increased to 1 020 μ mol/L at 48 h after birth.His blood amino acids level and urine organic acid level showed a pattern indicative of OTCD [blood arginine (97.43 μ mol/L,reference 1.00-25.00 μ mol/L),citrulline (27.43 μ mol/L,reference 4.00-30.00 μ mol/L),ornithine (161.66 μ mol/L,reference 10.00-120.00 μ mol/L) and methionine (70.45 μ mol/L,reference 10.00-50.00 μ mol/L); urine uracil (67.11 μ mol/mol Crea,reference 0.00-7.00 μ mol/mol Crea) and orotic acid (1 372.66 μ mol/mol Crea,reference 0.00-1.50 μ mol/mol Crea)].DNA studies revealed a c.583G ＞ A (G195R) homozygous mutation of the OTC gene.His mother was heterozygous for OTCD and developed acute hyperammonemia during pregnancy.Her blood showed a normal-leveled arginine (8.44 μ mol/L,reference 1.50-25.00 μ mol/L),a normal-leveled citrulline(8.41 μ mol/L,reference 7.00-35.00 μ mol/L),an elevated glutamate(279.15 μ mol/L,reference 45.00-200.00 μ mol/L).Her urine uracil (51.55 μ mol/mol Crea,reference 0.00-7.00 μ mol/mol Crea) and orotic acid (38.75 μ mol/mol Crea,reference 0.00-1.50 μ mol/mol) were elevated.Successful management of her prenatal and postpartum blood ammonia level was achieved after administration of pharmacologic nitrogen scavengers and protein limitation.DNA studies revealed a c.583G ＞ A (G195R) heterozygous mutation in the newborn's mother and grandmother.Conclusions General management on pregnant OTCD women is effective.Male newborn patients often have a poor prognosis.

Objective To study the influences of fenofibrate on oxidative stress and endoplasmic reticulum stress in livers of hyperlipidemic rats. Methods Male SD rats were fed with high-fat diet for 12 weeks to induce a model of hyperlipidemia, then divided into control group, high-fat diet group with another four-week high-fat diet and fenofibrate group, in which rats were treated with fenofibrate [100 mg / (kg·d)] for 4 weeks. Then improvement of insulin resistance was detected in rats with GTT and ITT experiment. The serum levels of glucose (GLU), fasting insulin (FINS),triglyceride (TG) and total cholesterol (TC) were detected. The pathological changes of livers were detected with Oil Red O staining. The oxidative stress indices such as T-SOD, Mn-SOD, GSH and T-AOC were detected with liver homogenate. The expression of GRP78 was detected with real-time quantification RT-PCR and Western blot analysis respectively. Results Compared with rats with high-fat-diet, rats after fenofibrate treatment showed obviously improved insulin resistance, lower serum level of TG, TC and FINS (P < 0.05), decreased number and size of lipid droplets in liver tissue sections. T-SOD level in liver homogenate was significantly increased (P < 0.05), while GSH and T-AOC levels increased but had no obvious differences when compared with control group (P > 0.05). The expression of GRP78 at mRNA and protein levels were increased significantly after fenofibrate treatment (P < 0.05). Conclusions Fenofibrate has significant effects on improving insulin resistance and lipid regulation, which might be related to decreased oxidative stress and subsequent endoplasmic reticulum stress.

Objective To observe and compare the curative effects of zoledronic acid (ZA) combined with radiotherapy and ZA combined with chemotherapy in the treatment of bone metastasis of non-small cell lung cancer (NSCLC). Methods Seventy-eight patients with NSCLC bone metastasis treated by radiotherapy or chemotherapy were taken in our hospital from January 2010 to June 2014, including 39 cases of ZA combined conventional fractionation radiotherapy (radiotherapy combined group), and 39 cases of ZA combined with chemotherapy (paclitaxel liposome + cisplatin) (chemotherapy combined group). Each group had 39 cases. WHO objective evaluation standard, efficacy evalulation of solid tumor metastasis and curative effect standard grading of pain, anticancer agent toxicity classification standard, Karnofsky standard were used for evaluating and analyzing the patients with primary lung tumor, bone metastasis, degree of pain, adverse reactions and functional status (once before and after the treatment). Results The efficiency rate of primary lung tumor, the efficiency rate of bone metastasis, the total effective rate of pain relief and the improvement rate of functional status (Karnofsky score increased by 10 points or more)in the radiotherapy combined group and chemotherapy combined group were 82.05 % (32/39) vs. 79.49 % (31/39), 48.72 % (19/39) vs. 51.28 %(20/39), 82.05 % (32/39) vs. 84.62 % (33/39), 66.67 % (26/39) vs. 71.79 % (28/39) respectively, and the differences were not statistically significant (the values of x2 were 0.224, 0.237, 0.195, 0.259 respectively, all P> 0.05); Although the two groups showed low-grade fever, bone marrow suppression, esophagitis, liver and kidney damage, gastrointestinal reactions and other adverse reactions, the adverse reactions of two groups were close to [28.21 % (11/39) vs. 30.77 % (12/39)] (x2 = 0.314, P> 0.05). Fortunately, these reactions were controlled well after symptomatic treatment. Conclusion ZA combined with radiotherapy or chemotherapy is a safe and effective way for bone metastasis of NSCLC, which should be taken based on the individual condition of the patients.

This study investigated the changes of CD4(+) CD25(+) regulatory T cells (Tregs) in peripheral blood of patients with hepatocellular carcinoma before and after transcatheter arterial chemoembolization (TACE). The proportion of CD4(+) CD25(+) Tregs among CD4(+) T lymphocytes in peripheral blood of 33 patients with hepatocellular carcinoma was determined by flow cytometry before, 1 week and 1 month after TACE. And 25 healthy volunteers served as control. One month after TACE, the patients were divided into two groups: 22 in group A, who were in stable condition or getting better; and 10 in group B, who were deteriorating. One patient died and was excluded. The results showed that the percentage of CD4(+)CD25(+) Tregs among CD4(+) T lymphocytes did not significantly change in the 33 patients 1 week after TACE as compared with that before TACE, however, the difference was significant (P<0.01) between the patients with hepatocellular carcinoma and the healthy subjects. The percentage of CD4(+) CD25(+) Tregs among CD4(+) T lymphocytes in group A 1 month after TACE was decreased significantly in comparison with that before and 1 week after TACE (P<0.01), whereas, that in group B was increased significantly 1 month after TACE (P<0.01). It was concluded that patients with hepatocellular carcinoma had a higher proportion of CD4(+)CD25(+) Tregs in peripheral blood. TACE did not significantly affect the level of CD4(+) CD25(+) Tregs within short time (such as 1 week). The proportion of CD4(+)CD25(+) Tregs in peripheral blood 1 month after TACE was related to the prognosis of hepatocellular carcinoma.
Antineoplastic Agents/administration & dosage ; Carcinoma, Hepatocellular/*immunology ; Carcinoma, Hepatocellular/therapy ; Chemoembolization, Therapeutic/*methods ; Liver Neoplasms/*immunology ; Liver Neoplasms/*therapy ; T-Lymphocytes, Regulatory/*immunology

Objective To investigate the initial clinical value of shear wave elastography ( SWE ) combined with shear wave dispersion ( SWD) in preoperatively differentiating diagnosis of focal liver lesions ( FLLs) . Methods Eighty‐three patients diagnosed as FLLs were prospectively enrolled from January to April 2018 in Zhongshan Hospital of Fudan University . M easurements of SWD and SWE both inner FLLs and surrounding liver parenchyma 2 cm away from the board lines of lesions were performed . ROIs were placed within the lesions and surrounding parenchyma 2 cm away from the lesions . In each ROI , measurements were performed 13 times . Histopathological results after operation were regarded as gold standard ,and the values of viscoelasticity in differentiating diagnosis of FLLs were analyzed . Results The final histopathological results showed that of all 83 cases ,20 cases were diagnosed as benign lesions ( 5 cases of focal nodular hyperplasia ,15 cases of hemangioma) ,and 63 cases as malignant lesions ( 12 cases of liver metastases ,51 cases of hepatocellular carcinoma) . T he SWE of benign lesions and malignant lesions were ( 9 .94 ± 8 .22) kPa ,and ( 13 .68 ± 7 .80) kPa( P ＜0 .05 ) . T he SWD of benign lesions and malignant lesions were ( 13 .47 ± 2 .76 ) ( m/s)/kHz ,and ( 15 .00 ± 3 .82 ) ( m/s)/kHz ( P ＜0 .05 ) . T he SWE ratio and SWD ratio of benign lesions to surrounding liver parenchyma were higher than those of malignant lesions( all P ＜

Objective To investigate the value of contrast‐enhanced ultrasound ( CEUS ) in the differential diagnosis of pancreatic neoplasia ( SPN ) before operation . Methods Forty‐six cases of SPN confirmed by operation and histopathological results from January 2012 to June 2018 were enrolled in the study . According to the European Ultrasound Association ( EFSUMB) guidelines for CEUS in 2018 ,the enhancement pattern of pancreatic lesion with normal surrounding pancreatic parenchyma was used for reference . T he enhancement pattern of SPN were observed during the arterial phase ,venous phase and delayed phase . CEUS pattern of 16 cases with pancreatic ductal adenocarcinoma ( PDAC ) with cystic changes proved by histopathology were observed and compared with SPN . Results T he mean size of 46 cases of SPN was ( 32 .72 ± 25 .51) mm . Fifteen SPN lesions located in the head of pancreas ,31 cases located in the body and tail of the pancreas . Most of SPN were solidcystic lesions with thin separation on conventional B mode ultrasoud ,without communication with the main pancreatic duct . Color flow signals could be detected in 78 .3% ( 36/46) SPN lesions . After the injection of 2 .4 ml ultrasound contrast agent , the substantial part of all SPN showed hyperenhancement ( n＝ 44 ,99 .7% ) or isoenhancement ( n ＝ 2 , 4 .3% ) during the arterial phase ,venous phase and late phase . However ,93 .8% ( 15/16 ) of the PDAC lesion with cystic changes showed consistent hypo enhancement throughout the arterial ,venous and late phase ( P ＜0 .05) . T he accuracy of preoperative diagnosis of CEUS was 95 .6% . Conclusions Depending on its unique advantages such as real‐time observation ,high‐resolution imaging ,and no radiation ,CEUS is helpful for early detection ,accurate localization and preoperative diagnosis of SPN . CEUS has potential role for clinical decision‐making before treatment .

Objective:To analyze the changes and efficacy of antiviral treatment regimens in patients with chronic hepatitis C.Methods:This was a single center retrospective study. A total of 157 patients with chronic hepatitis C in Huashan Hospital, Fudan University from January 2014 to February 2019 were included. Clinical informations of antiviral treatment and follow-up were collected. The sustained virologic response (SVR) rate and adverse events in patients receiving different antiviral regimens were compared. Chi-square test was used for statistical analysis.Results:Among the 157 patients, 133 patients had sufficient follow-up data. Seventy-one patients received treatment before 2017, among which 63 patients received interferon regimens and the SVR rate was 74.65%(53/71). Sixty-two patients received treatment after 2017, among which 61 patients received direct-acting antiviral agents (DAA) regimens and the SVR rate was 98.39%(61/62). The difference in SVR rate between the two groups was statistically significant ( χ2=15.230, P<0.01). In 69 patients who received DAA regimens from 2014 to 2019, the SVR at post-treatment week 12 (SVR12) was 95.65%(66/69). Among 43 patients who received DAA regimens containing sofosbuvir, the SVR12 rates of patients with hepatitis C virus genotype 1, 3 and other genotypes were 15/15, 5/6 and 90.91%(20/22), respectively. All the 26 patients who received DAA regimens non-containing sofosbuvir achieved SVR12. The SVR12 rates of patients with different hepatitis C virus genotypes and DAA regimens were not significantly different ( χ2=5.243, P=0.263). The incidences of adverse events in pre-2017 group and post-2017 group were 84.62%(77/91) and 6.06% (4/66), respectively. The difference was statistically significant ( χ2=94.520, P<0.01). The most common adverse events were decreases in neutrophil cell count, decreases in hemoglobin level and decreases in platelet count. Treatment was ceased in six patients due to adverse events. Conclusions:After 2017, the majority of patients with chronic hepatitis C received DAA regimens instead of interferon regimens. The SVR rate increases and the incidence of adverse events decreases along with the changes of leading treatment regimens.The SVR12 rate is higher in patients receiving DAA regimens, regardless of hepatitis C virus genotypes.

Objective:To investigate the current situation and underlying problems of English courses in general education for eight-year M.D. Program and to put forward possible solutions.Method:s We investigated and interviewed teachers from West China Medical Center who teaches bilingual courses and teachers from College of Foreign Languages and Cultures along with faculty in Education Ministry of Sichuan University, analyzed the problems in English courses and explored measures for improvement.Result:s The emphasis students put on English course is satisfactory, but teachers still claim that few students participate actively in class. Therefore, the efficiency of English course should be improved.Conclusion:The current status of English course is not ideal, which can be attributed to both teachers and students, so future schemes should be based on these two perspectives.

Objective:To investigate the effects of deep learning-based AiContour ??versus atlas-based Raystation ?? automatic contouring methods on the contouring of organs-at-risk on CT images of patients with rectal cancer who undergo radiotherapy, providing evidence for clinical application. Methods:Fifty patients with rectal cancer who received treatment during January to June 2020 in Zhejiang Provincial People's Hospital (Affiliated Hospital of Hangzhou Medical College) were included in this study. The CT images from 20 patients with rectal cancer that had been contoured by experienced radiotherapist were selected as target images and automatically contoured using the data template library of AiContour ?? and Raystation ?? automatic contouring methods. Hausdorff distance, mean distance to agreement, dice similarity coefficient, Jaccard coefficient were used to quantitatively evaluate the accuracy of the volume of contour of organs-at-risk automatically sketched by the two methods. Results:There was no significant difference in Hausdorff distance in left femoral head [(6.81 ± 2.66) vs. (7.24 ± 2.10)], right femoral head [(7.38 ± 3.91) vs. (8.14 ± 3.71)], pelvis [(24.00 ± 9.01) vs. (24.66 ± 9.67)] between AiContour ?? and Raystation ?? automatic contouring methods ( tleft femoral head = -0.831, tright femoral head = -0.821, tpelvis = -0.357, all P > 0.05). Significant differences were observed in mean distance to agreement, dice similarity coefficient and Jaccard coefficient of organs-at-risk (all P < 0.05). The mean values of dice similarity coefficient automatically sketched by AiContour ?? method were > 0.7. The DSC of left kidney, right kidney, rectum and bladder automatically sketched by Raystation ?? method were < 0.7, and the dice similarity coefficient values of other organs-at-risk automatically sketched by Raystation ?? method were > 0.7. In addition, Hausdorff distance, mean distance to agreement and Jaccard coefficient values of organs-at-risk automatically sketched by AiContour ?? method were superior to those automatically sketched by Raystation ??. Conclusion:After slight modification, the organs-at-risk automatically sketched by AiContour ?? and Raystation ?? methods can meet clinical requirement. The contouring effects provided byAiContour ?? method were superior to those provided by Raystation ?? method.

OBJECTIVETo study the metabolic changes of pancreatic extracts from insulin-resistant rats induced by fructose feeding using nuclear magnetic resonance ¹H spectroscopy (¹H NMR).

METHODSSixteen Wistar rats were divided equally into control group and model group and given water and 10% fructose water for 8 weeks, respectively. The pancreatic tissues were then obtained for H NMR spectra analysis and principal component analysis (PCA).

RESULTSCompared with the control rats, the rats in the model group showed significantly increased creatine, betaine/TMAO, taurine, glycine and myo-inositol and decreased levels of lipids, lactate, glutamate, choline and GPC/PC.

CONCLUSION¹H NMR and pattern recognition can define the metabolic characteristics of the pancreatic tissue extracts from insulin-resistant rats and provide reliable metabolic evidence for studying the mechanisms of insulin resistance at the molecular level.

Animals ; Fructose ; Insulin Resistance ; Metabolomics ; Pancreatic Extracts ; chemistry ; Principal Component Analysis ; Proton Magnetic Resonance Spectroscopy ; Rats ; Rats, Wistar