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Background The current increasing trend of new cases of occupational noise-induced deafness indicates that the hearing loss of occupational population has not been effectively controlled in China. It is of great significance to study the characteristics of hearing loss among noise-exposed workers and its related factors. Objective To investigate characteristics and influencing factors of hearing loss among occupational noise-exposed workers in a large machinery maintenance enterprise, and to provide a scientific basis to prevent and control noise-induced hearing loss. Methods A cross-sectional survey was conducted to investigate male Han occupational noise-exposed workers in a large mechanical maintenance enterprise. We acquired demographic characteristics, occupational exposure history, and individual life behavior characteristics of the workers through questionnaires, collected occupational exposure level data from annual occupational disease hazard factor surveillance reports, obtained pure tone hearing threshold test data through occupational health examinations, and estimated individual noise exposure levels using cumulative noise exposure (CNE). According to the results of pure tone air conduction hearing threshold test, the workers were divided into a hearing loss group and a normal hearing group. The chi-square test was employed to compare the occupational exposure characteristics and individual life behavior characteristics between the two groups. Additionally, the trend chi-square test was utilized to analyze the changing trends of age, length of service, CNE, and hearing loss rate within the two groups. The relationship between high-frequency hearing loss in both ears and its related influencing factors was assessed by a multiple logistic regression model. Results The M (P25, P75) of CNE for the 2531 occupational noise-exposed workers was 97.51 (95.39, 99.96) dB(A)·year. The incidence of hearing anomaly, binaural high-frequency hearing anomaly, random ear high-frequency hearing anomaly, binaural low-frequency hearing anomaly, and random ear low-frequency hearing anomaly were 22.48%, 16.59%, 22.13%, 2.77%, and 3.52%, respectively. High-frequency hearing threshold increase was the main reason for hearing anomaly (98.42%). In comparison to the CNE ≤ 97 dB(A)·year group, the 97 dB(A)·year<CNE≤ 100 dB(A)·year group and the CNE>100 dB(A)·year group experienced a 36.4% and 52.3% increase in the risk of bilateral high-frequency hearing loss, respectively. The smoking group exhibited a 43.5% elevated risk of bilateral high-frequency hearing loss when compared to the non-smoking group. Conversely, the group frequently wearing hearing protection equipment demonstrated a 23.6% lower risk of bilateral high-frequency hearing loss in comparison to the group occasionally wearing protective equipment. The data suggested that CNE＞97 dB(A)·year and smoking might be independent risk factors for bilateral high-frequency hearing loss, and frequently wearing hearing protection equipment might be an important protective factor. Conclusion Increased CNE and smoking can elevate the risk of high-frequency hearing loss, while personal hearing protection can effectively reduce the risk of hearing loss.
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Objective:To investigate the clinical characteristics of choledocholithiasis com-bined with periampullary diverticulum and influencing factor for difficult cannulation of endoscopic retrograde cholangiopancreatography (ERCP).Methods:The retrospective case-control study was conducted. The clinical data of 1 920 patients who underwent ERCP for choledocholithiasis in 15 medical centers, including the First Hospital of Lanzhou University, et al, from July 2015 to December 2017 were collected. There were 915 males and 1 005 females, aged (63±16)years. Of 1 920 patients, there were 228 cases with periampullary diverticulum and 1 692 cases without periampullary diverticulum. Observation indicators: (1) clinical characteristics of patients with choledocholithiasis; (2) intraoperative and postoperative situations of patients undergoing ERCP for choledocholithiasis; (3) influencing factor analysis for difficult cannulation in patients undergoing ERCP for choledocholithiasis. Measurement data with normal distribution were represented as Mean±SD, and comparison between groups was conducted using the independent sample t test. Measurement data with skewed distribution were represented as M(range) or M( Q1, Q3), and com-parison between groups was conducted using the Wilcoxon rank sum test. Count data were described as absolute numbers or percentages, and comparison between groups was conducted using the chi-square test or Fisher exact probability. The Logistic regression model was used for univariate and multivariate analyses. Results:(1) Clinical characteristics of patients with choledocholithiasis. Age, body mass index, cases with complications as chronic obstructive pulmonary disease, diameter of common bile duct, cases with diameter of common bile duct as <8 mm, 8?12 mm, >12 mm, diameter of stone, cases with number of stones as single and multiple were (69±12)years, (23.3±3.0)kg/m 2, 16, (14±4)mm, 11, 95, 122, (12±4)mm, 89, 139 in patients with choledocholithiasis combined with periampullary diverticulum, versus (62±16)years, (23.8±2.8)kg/m 2, 67, (12±4)mm, 159, 892, 641, (10±4)mm, 817, 875 in patients with choledocholithiasis not combined with periampullary diver-ticulum, showing significant differences in the above indicators between the two groups ( t=?7.55, 2.45, χ2=4.54, t=?4.92, Z=4.66, t=?7.31, χ2=6.90, P<0.05). (2) Intraoperative and postoperative situations of patients undergoing ERCP for choledocholithiasis. The balloon expansion diameter, cases with intraoperative bleeding, cases with hemorrhage management of submucosal injection, hemostatic clip, spray hemostasis, electrocoagulation hemostasis and other treatment, cases with endoscopic plastic stent placement, cases with endoscopic nasal bile duct drainage, cases with mechanical lithotripsy, cases with stone complete clearing, cases with difficult cannulation, cases with delayed intubation, cases undergoing >5 times of cannulation attempts, cannulation time, X-ray exposure time, operation time were 10.0(range, 8.5?12.0)mm, 56, 6, 5, 43, 1, 1, 52, 177, 67, 201, 74, 38, 74, (7.4±3.1)minutes, (6±3)minutes, (46±19)minutes in patients with choledocholithiasis combined with periampullary diverticulum, versus 9.0(range, 8.0?11.0)mm, 243, 35, 14, 109, 73, 12, 230, 1 457, 167, 1 565, 395, 171, 395, (6.6±2.9)minutes, (6±5)minutes, (41±17)minutes in patients with choledocholithiasis not combined with periampullary diverticulum, showing significant differences in the above indicators between the two groups ( Z=6.31, χ2=15.90, 26.02, 13.61, 11.40, 71.51, 5.12, 9.04, 8.92, 9.04, t=?3.89, 2.67, ?3.61, P<0.05). (3) Influencing factor analysis for difficult cannulation in patients undergoing ERCP for choledocholithiasis. Results of multivariate analysis showed total bilirubin >30 umol/L, number of stones >1, combined with periampullary diverticulum were indepen-dent risk factors for difficult cannulation in patients with periampullary diverticulum who underwent ERCP for choledocholithiasis ( odds ratio=1.31, 1.48, 1.44, 95% confidence interval as 1.06?1.61, 1.20?1.84, 1.06?1.95, P<0.05). Results of further analysis showed that, of 1 920 patients undergoing ERCP for choledocholithiasis, the incidence of postoperative pancreatitis was 17.271%(81/469) and 8.132%(118/1 451) in the 469 cases with difficult cannulation and 1 451 cases without difficult cannula-tion, respectively, showing a significant difference between them ( χ2=31.86, P<0.05). In the 1 692 patients with choledocholithiasis not combined with periampullary diverticulum, the incidence of postopera-tive pancreatitis was 17.722%(70/395) and 8.250%(107/1 297) in 395 cases with difficult cannula-tion and 1 297 cases without difficult cannulation, respectively, showing a significant difference between them ( χ2=29.00, P<0.05). In the 228 patients with choledocholithiasis combined with peri-ampullary diverticulum, the incidence of postoperative pancreatitis was 14.865%(11/74) and 7.143%(11/154) in 74 cases with difficult cannulation and 154 cases without difficult cannulation, respectively, showing no significant difference between them ( χ2=3.42, P>0.05). Conclusions:Compared with patients with choledocholithiasis not combined with periampullary divertioulum, periampullary divertioulum often occurs in choledocholithiasis patients of elderly and low body mass index. The proportion of chronic obstructive pulmonary disease is high in choledocholithiasis patients with periampullary diverticulum, and the diameter of stone is large, the number of stone is more in these patients. Combined with periampullary diverticulum will increase the difficult of cannulation and the ratio of patient with mechanical lithotripsy, and reduce the ratio of patient with stone complete clearing without increasing postoperative complications of choledocholithiasis patients undergoing ERCP. Total bilirubin >30 μmol/L, number of stones >1, combined with periampullary diverticulum are independent risk factors for difficult cannulation in patients of periampullary diverticulum who underwent ERCP for choledocholithiasis.
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GATA-binding protein 1 (GATA1), an important hematopoietic transcription factor, specifically regulates the proliferation and differentiation of erythroid and megakaryoid cells at the transcription level, which maintains the normal development and maturation of these two lineages. The functional structure of GATA1 is composed of one N-terminal transactivation domain (N-TAD) and two zinc fingers (NF and CF). GATA1 is highly conserved in different species. Alteration of GATA1 expression or function will lead to transcriptional disorder of erythrocyte and megakaryocyte related genes, resulting in various clinical phenotypes. This article reviews the molecular structure of GATA1, its transcriptional regulation in erythrocyte and megakaryocyte, and the hereditary hematopoietic regulatory disorders of these two lineages caused by GATA1 mutations.
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Objective:To evaluate the performance of an artificial intelligent (AI)-based automated digital cell morphology analyzer (hereinafter referred as AI morphology analyzer) in detecting peripheral white blood cells (WBCs).Methods:A multi-center study. 1. A total of 3010 venous blood samples were collected from 11 tertiary hospitals nationwide, and 14 types of WBCs were analyzed with the AI morphology analyzers. The pre-classification results were compared with the post-classification results reviewed by senior morphological experts in evaluate the accuracy, sensitivity, specificity, and agreement of the AI morphology analyzers on the WBC pre-classification. 2. 400 blood samples (no less than 50% of the samples with abnormal WBCs after pre-classification and manual review) were selected from 3 010 samples, and the morphologists conducted manual microscopic examinations to differentiate different types of WBCs. The correlation between the post-classification and the manual microscopic examination results was analyzed. 3. Blood samples of patients diagnosed with lymphoma, acute lymphoblastic leukemia, acute myeloid leukemia, myelodysplastic syndrome, or myeloproliferative neoplasms were selected from the 3 010 blood samples. The performance of the AI morphology analyzers in these five hematological malignancies was evaluated by comparing the pre-classification and post-classification results. Cohen′s kappa test was used to analyze the consistency of WBC pre-classification and expert audit results, and Passing-Bablock regression analysis was used for comparison test, and accuracy, sensitivity, specificity, and agreement were calculated according to the formula.Results:1. AI morphology analyzers can pre-classify 14 types of WBCs and nucleated red blood cells. Compared with the post-classification results reviewed by senior morphological experts, the pre-classification accuracy of total WBCs reached 97.97%, of which the pre-classification accuracies of normal WBCs and abnormal WBCs were more than 96% and 87%, respectively. 2. The post-classification results reviewed by senior morphological experts correlated well with the manual differential results for all types of WBCs and nucleated red blood cells (neutrophils, lymphocytes, monocytes, eosinophils, basophils, immature granulocytes, blast cells, nucleated erythrocytes and malignant cells r>0.90 respectively, reactive lymphocytes r=0.85). With reference, the positive smear of abnormal cell types defined by The International Consensus Group for Hematology, the AI morphology analyzer has the similar screening ability for abnormal WBC samples as the manual microscopic examination. 3. For the blood samples with malignant hematologic diseases, the AI morphology analyzers showed accuracies higher than 84% on blast cells pre-classification, and the sensitivities were higher than 94%. In acute myeloid leukemia, the sensitivity of abnormal promyelocytes pre-classification exceeded 95%. Conclusion:The AI morphology analyzer showed high pre-classification accuracies and sensitivities on all types of leukocytes in peripheral blood when comparing with the post-classification results reviewed by experts. The post-classification results also showed a good correlation with the manual differential results. The AI morphology analyzer provides an efficient adjunctive white blood cell detection method for screening malignant hematological diseases.
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Objective:To investigate the effect of sarcopenia on physical performance and nutritional status in elderly patients with gastrointestinal tumors.Methods:a total of 120 eligible in-patients were enrolled from gastrointestinal surgery department of a Grade A tertiary hospital in Ningbo city from September 2021 to January 2022. General clinical data were collected, nutritional risk was screened using Nutritional Risk Screening 2002 (NRS 2002), physical performance was assessed by Short Physical Performance Battery (SPPB), skeletal muscle index at the third lumbar vertebra level (L3 SMI) was calculated using abdominal CT scan, and grip strength/muscle strength, gait speed by 6 Meter Timed Walk Test and calf circumference were measured. Subjects were divided into sarcopenia and non-sarcopenia group according to the diagnostic criteria of the Asian Working Group for Sarcopenia (AWGS). Impacting factors of sarcopenia and the correlation between muscle mass and physical performance in elderly patients with gastrointestinal tumors were analyzed.Results:The overall prevalence rate of sarcopenia in elderly patients with gastrointestinal tumors was 28.3% (34/120) and it was 28.9% (22/76) and 27.3% (12/44) in males and females respectively. There were statistically significant differences in age, tumor stage, nutritional risk, body mass index, calf circumference, L3 SMI, grip strength/muscle strength, gait speed and SPPB score between patients with and without sarcopenia (P<0.05). Muscle strength/grip strength, L3 SMI and gait speed were closely correlated with the occurrence of sarcopenia in elderly patients with gastrointestinal tumors (P<0.05). The L3 SMI was positively correlated with physical performance as assessed with SPPB in both groups.Conclusions:Sarcopenia is a common complication in elderly patients with gastrointestinal tumors with multiple influencing factors. Timely nutritional intervention and exercise intervention should be incorporated into the treatment of elderly gastrointestinal tumor patients with sarcopenia in order to improve nutrition status.
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Objective:To study the clinical and molecular characteristics of a family with familial hypercholesterolemia (FH) with LDLRAP1 and ABCG8 gene abnormality.Methods:In September 2020, one case of FH was included in Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine; peripheral venous blood samples of members of the family were collected to detect serum total cholesterol (TC), triglycerides (TG), and low-density lipoprotein cholesterol (LDL-C), and high-density lipoprotein cholesterol (HDL-C) indicators; use high-performance liquid chromatography to detect serum stigmasterol and sitosterol content; perform second-generation gene sequencing to detect gene mutations in probands and family members; use Pymol software to detect gene mutations point for pathogenicity analysis, and use Uniprot Modelling software to perform protein structure modeling.Results:The patient presented with anemia, multiple xanthomas and early-onset acute coronary syndrome. The coronary angiography showed severe coronary artery lesions; abdominal ultrasound showed splenomegaly; blood smear showed shaped erythrocytes and large platelets. The level of serum TC, LDL-C, stigmasterol and sitosterol was 8.54 mmol/L (2.3-5.7 mmol/L), 4.84 mmol/L (range of normal value 1.3-4.3 mmol/L), 44 μmol/L (1.0-10 μmol/L), 28 μmol/L (1.0-15 μmol/L), respectively; LDLRAP1 gene mutation was found: exon4 c.415C>T:p.Q139X; the truncated protein formed by this homozygous mutation lost multiple stable protein structure regions, which can not have a normal function. At the same time, ABCG8 gene mutations were also found: exon13 c.1895T>C (p.V632A) and exon8 c.1199C>A:p.T400K . Two cases of family members had a mild increase in HDL-C (Ⅱ5: 2.33 mmol/L, Ⅱ6∶2.96 mmol/L), 3 cases carrying the ABCG8 gene mutations had a slight increase in stigmasterol (Ⅱ8: 23 μmol/L, Ⅱ7: 24 μmol/L, Ⅰ2: 18 μmol/L) and sitosterol (Ⅱ8: 41 μmol/L, Ⅱ7: 33 μmol/L, Ⅰ2: 45 μmol/L), suggesting that its association with the concentration of plant sterols. Conclusions:FH patients with LDLRAP1 and ABCG8 gene abnormalities may have abnormal plant sterol concentrations, and their clinical manifestations are more complicated. Therefore, family history, LDL-C, plant sterol levels, and genetic test results should be considered comprehensively.
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Objective:To report pediatric endoscopic retrograde cholangiopancreatography (ERCP) intubation techniques and to analyze the influencing factors of pediatric ERCP in China.Methods:Retrospective analysis was performed on 90 cases of pediatric and adult ERCP operations respectively at Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine from January 2016 to June 2020. The anatomic data, intubation time, and endoscopic intubation measures were reviewed. The anatomic differences in duodenal papilla between the children and adults were analyzed to find the factors affecting ERCP intubation time in children.Results:There were 88 cases of successful infantile intubation with the success rate of 97.8%, and 90 cases of successful adult intubation with the success rate of 100.0%. The intubation time in the pediatric group was 187±67 s, and that in the adult group was 247±86 s with significant difference ( t=5.220, P<0.001). The duodenal diameter of pediatric patients was 3.38±1.57 cm, and that of adult patients was 5.94±1.87 cm with significant difference ( t=9.832, P<0.001). The horizontal distance from the duodenal bulb to the papilla in pediatric patients was 2.44±1.15 cm, which was significantly shorter than 4.22±1.43 cm in adult patients ( t=9.077, P<0.001). Most duodenal papillae in children were hemispherical [flat 26.1% (23/88), hemispherical 51.1% (45/88), cylindrical 22.7% (20/88)], while most of those in the adult patients were cylindrical [flat 9.1% (8/88), hemispherical 23.9% (21/88), cylindrical 67.0% (59/88)]. The factors influencing the intubation time of ERCP in children by univariate analysis included the shape of duodenal papilla, duodenal papilla hardness, visual region, distance from junction of duodenal bulb and descending part to duodenal papilla, distance from duodenal papilla to endoscope, and degree of incising. Conclusion:Shorter and stiffer duodenal papillae in children with normal papilla orientation are associated with shorter intubation time. These indicators are favorable factors for intubation.
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Objective:To investigate the epidemiological characteristics, diagnosis, treat-ment and prognosis of gallbladder cancer in China from 2010 to 2017.Methods:The single disease retrospective registration cohort study was conducted. Based on the concept of the real world study, the clinicopathological data, from multicenter retrospective clinical data database of gallbladder cancer of Chinese Research Group of Gallbladder Cancer (CRGGC), of 6 159 patients with gallbladder cancer who were admitted to 42 hospitals from January 2010 to December 2017 were collected. Observation indicators: (1) case resources; (2) age and sex distribution; (3) diagnosis; (4) surgical treatment and prognosis; (5) multimodality therapy and prognosis. The follow-up data of the 42 hospitals were collected and analyzed by the CRGGC. The main outcome indicator was the overall survival time from date of operation for surgical patients or date of diagnosis for non-surgical patients to the end of outcome event or the last follow-up. Measurement data with normal distribu-tion were represented as Mean±SD, and comparison between groups was conducted using the t test. Measurement data with skewed distribution were represented as M( Q1, Q3) or M(range), and com-parison between groups was conducted using the U test. Count data were described as absolute numbers or percentages, and comparison between groups was conducted using the chi-square test. Univariate analysis was performed using the Logistic forced regression model, and variables with P<0.1 in the univariate analysis were included for multivariate analysis. Multivariate analysis was performed using the Logistic stepwise regression model. The life table method was used to calculate survival rates and the Kaplan-Meier method was used to draw survival curves. Log-rank test was used for survival analysis. Results:(1) Case resources: of the 42 hospitals, there were 35 class A of tertiary hospitals and 7 class B of tertiary hospitals, 16 hospitals with high admission of gallbladder cancer and 26 hospitals with low admission of gallbladder cancer, respectively. Geographical distribution of the 42 hospitals: there were 9 hospitals in central China, 5 hospitals in northeast China, 22 hospitals in eastern China and 6 hospitals in western China. Geographical distribution of the 6 159 patients: there were 2 154 cases(34.973%) from central China, 705 cases(11.447%) from northeast China, 1 969 cases(31.969%) from eastern China and 1 331 cases(21.611%) from western China. The total average number of cases undergoing diagnosis and treatment in hospitals of the 6 159 patients was 18.3±4.5 per year, in which the average number of cases undergoing diagnosis and treatment in hospitals of 4 974 patients(80.760%) from hospitals with high admission of gallbladder cancer was 38.8±8.9 per year and the average number of cases undergoing diagnosis and treatment in hospitals of 1 185 patients(19.240%) from hospitals with low admission of gallbladder cancer was 5.7±1.9 per year. (2) Age and sex distribution: the age of 6 159 patients diagnosed as gallbladder cancer was 64(56,71) years, in which the age of 2 247 male patients(36.483%) diagnosed as gallbladder cancer was 64(58,71)years and the age of 3 912 female patients(63.517%) diagnosed as gallbladder cancer was 63(55,71)years. The sex ratio of female to male was 1.74:1. Of 6 159 patients, 3 886 cases(63.095%) were diagnosed as gallbladder cancer at 56 to 75 years old. There was a significant difference on age at diagnosis between male and female patients ( Z=-3.99, P<0.001). (3) Diagnosis: of 6 159 patients, 2 503 cases(40.640%) were initially diagnosed as gallbladder cancer and 3 656 cases(59.360%) were initially diagnosed as non-gallbladder cancer. There were 2 110 patients(34.259%) not undergoing surgical treatment, of which 200 cases(9.479%) were initially diagnosed as gallbladder cancer and 1 910 cases(90.521%) were initially diagnosed as non-gallbladder cancer. There were 4 049 patients(65.741%) undergoing surgical treatment, of which 2 303 cases(56.878%) were initially diagnosed as gallbladder cancer and 1 746 cases(43.122%) were initial diagnosed as non-gallbladder cancer. Of the 1 746 patients who were initially diagnosed as non-gallbladder cancer, there were 774 cases(19.116%) diagnosed as gallbladder cancer during operation and 972 cases(24.006%) diagnosed as gallbladder cancer after operation. Of 6 159 patients, there were 2 521 cases(40.932%), 2 335 cases(37.912%) and 1 114 cases(18.087%) undergoing ultrasound, computed tomography (CT) or magnetic resonance imaging (MRI) examination before initial diagnosis, respec-tively, and there were 3 259 cases(52.914%), 3 172 cases(51.502%) and 4 016 cases(65.205%) undergoing serum carcinoembryonic antigen, CA19-9 or CA125 examination before initially diagnosis, respectively. One patient may underwent multiple examinations. Results of univariate analysis showed that geographical distribution of hospitals (eastern China or western China), age ≥72 years, gallbladder cancer annual admission of hospitals, whether undergoing ultrasound, CT, MRI, serum carcinoembryonic antigen, CA19-9 or CA125 examination before initially diagnosis were related factors influencing initial diagnosis of gallbladder cancer patients ( odds ratio=1.45, 1.98, 0.69, 0.68, 2.43, 0.41, 1.63, 0.41, 0.39, 0.42, 95% confidence interval as 1.21-1.74, 1.64-2.40, 0.59-0.80, 0.60-0.78, 2.19-2.70, 0.37-0.45, 1.43-1.86, 0.37-0.45, 0.35-0.43, 0.38-0.47, P<0.05). Results of multivariate analysis showed that geographical distribution of hospitals (eastern China or western China), sex, age ≥72 years, gallbladder cancer annual admission of hospitals and cases undergoing ultrasound, CT, serum CA19-9 examination before initially diagnosis were indepen-dent influencing factors influencing initial diagnosis of gallbladder cancer patients ( odds ratio=1.36, 1.42, 0.89, 0.67, 1.85, 1.56, 1.57, 0.39, 95% confidence interval as 1.13-1.64, 1.16-1.73, 0.79-0.99, 0.57-0.78, 1.60-2.14, 1.38-1.77, 1.38-1.79, 0.35-0.43, P<0.05). (4) Surgical treatment and prognosis. Of the 4 049 patients undergoing surgical treatment, there were 2 447 cases(60.435%) with complete pathological staging data and follow-up data. Cases with pathological staging as stage 0, stage Ⅰ, stage Ⅱ, stage Ⅲa, stage Ⅲb, stage Ⅳa and stage Ⅳb were 85(3.474%), 201(8.214%), 71(2.902%), 890(36.371%), 382(15.611%), 33(1.348%) and 785(32.080%), respectively. The median follow-up time and median postoperative overall survival time of the 2 447 cases were 55.75 months (95% confidence interval as 52.78-58.35) and 23.46 months (95% confidence interval as 21.23-25.71), respectively. There was a significant difference in the overall survival between cases with pathological staging as stage 0, stage Ⅰ, stage Ⅱ, stage Ⅲa, stage Ⅲb, stage Ⅳa and stage Ⅳb ( χ2=512.47, P<0.001). Of the 4 049 patients undergoing surgical treatment, there were 2 988 cases(73.796%) with resectable tumor, 177 cases(4.371%) with unresectable tumor and 884 cases(21.833%) with tumor unassessable for resectabi-lity. Of the 2 988 cases with resectable tumor, there were 2 036 cases(68.139%) undergoing radical resection, 504 cases(16.867%) undergoing non-radical resection and 448 cases(14.994%) with operation unassessable for curative effect. Of the 2 447 cases with complete pathological staging data and follow-up data who underwent surgical treatment, there were 53 cases(2.166%) with unresectable tumor, 300 cases(12.260%) with resectable tumor and receiving non-radical resection, 1 441 cases(58.888%) with resectable tumor and receiving radical resection, 653 cases(26.686%) with resectable tumor and receiving operation unassessable for curative effect. There were 733 cases not undergoing surgical treatment with complete pathological staging data and follow-up data. There was a significant difference in the overall survival between cases not undergoing surgical treatment, cases undergoing surgical treatment for unresectable tumor, cases undergoing non-radical resection for resectable tumor and cases undergoing radical resection for resectable tumor ( χ2=121.04, P<0.001). (5) Multimodality therapy and prognosis: of 6 159 patients, there were 541 cases(8.784%) under-going postoperative adjuvant chemotherapy and advanced chemotherapy, 76 cases(1.234%) under-going radiotherapy. There were 1 170 advanced gallbladder cancer (pathological staging ≥stage Ⅲa) patients undergoing radical resection, including 126 cases(10.769%) with post-operative adjuvant chemotherapy and 1 044 cases(89.231%) without postoperative adjuvant chemo-therapy. There was no significant difference in the overall survival between cases with post-operative adjuvant chemotherapy and cases without postoperative adjuvant chemotherapy ( χ2=0.23, P=0.629). There were 658 patients with pathological staging as stage Ⅲa who underwent radical resection, including 66 cases(10.030%) with postoperative adjuvant chemotherapy and 592 cases(89.970%) without postoperative adjuvant chemotherapy. There was no significant difference in the overall survival between cases with postoperative adjuvant chemotherapy and cases without postoperative adjuvant chemotherapy ( χ2=0.05, P=0.817). There were 512 patients with pathological staging ≥stage Ⅲb who underwent radical resection, including 60 cases(11.719%) with postoperative adjuvant chemotherapy and 452 cases(88.281%) without postoperative adjuvant chemotherapy. There was no significant difference in the overall survival between cases with postoperative adjuvant chemo-therapy and cases without post-operative adjuvant chemo-therapy ( χ2=1.50, P=0.220). Conclusions:There are more women than men with gallbladder cancer in China and more than half of patients are diagnosed at the age of 56 to 75 years. Cases undergoing ultrasound, CT, serum CA19-9 examination before initial diagnosis are independent influencing factors influencing initial diagnosis of gallbladder cancer patients. Preoperative resectability evaluation can improve the therapy strategy and patient prognosis. Adjuvant chemotherapy for gallbladder cancer is not standardized and in low proportion in China.
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Epilepsy is a common and severe brain disease affecting >65 million people worldwide. Recent studies have shown that kinesin superfamily motor protein 17 (KIF17) is expressed in neurons and is involved in regulating the dendrite-targeted transport of N-methyl-D-aspartate receptor subtype 2B (NR2B). However, the effect of KIF17 on epileptic seizures remains to be explored. We found that KIF17 was mainly expressed in neurons and that its expression was increased in epileptic brain tissue. In the kainic acid (KA)-induced epilepsy mouse model, KIF17 overexpression increased the severity of epileptic activity, whereas KIF17 knockdown had the opposite effect. In electrophysiological tests, KIF17 regulated excitatory synaptic transmission, potentially due to KIF17-mediated NR2B membrane expression. In addition, this report provides the first demonstration that KIF17 is modified by SUMOylation (SUMO, small ubiquitin-like modifier), which plays a vital role in the stabilization and maintenance of KIF17 in epilepsy.
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Animals , Mice , Epilepsy/metabolism , Kinesins/metabolism , Neurons/metabolism , Receptors, N-Methyl-D-Aspartate/metabolism , Seizures/metabolismABSTRACT
The detection of coagulation factor Ⅷ activity plays an important role in the diagnosis, typing, efficacy monitoring and detection of inhibitor titer in hemophilia A, acquired hemophilia A and von Willebrand disease. However, due to the diversity of detecting systems, the difference of reagent composition, the existence of interfering substances and other influence factors, the detection of coagulation factor Ⅷ activity in the laboratories in China still needs to be improved.
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Objective:To investigate the value of radiotherapy in patients with stage Ⅳ B thoracic esophageal squamous cell carcinoma (ESCC) at initial diagnosis. Methods:A total of 199 patients with stage Ⅳ B thoracic ESCC at initial diagnosis (according to UICC/AJCC Eighth Edition Esophageal and Esophagogastric Junction Cancer TNM Staging) who were treated in the Fourth Hospital of Hebei Medical University between January 2010 to December 2016 were recruited. Winthin the whole group, 130 patients (65.3%) had distant lymph node metastases alone, 51 cases (25.6%) of solid organ metastases alone and 18 cases (9.0%) of solid organ complicated with distant lymph node metastases. Among them, 16 patients (8.0%) were treated with chemotherapy alone, 50 cases (25.1%) of radiotherapy alone, 133 cases (66.8%) of radiochemotherapy (81 patients treated with concurrent radiochemotherapy and 52 patients treated with sequential radiochemotherapy). The survival rate was calculated by Kaplan-Meier method and the difference was analyzed by log-rank test. Clinical prognosis was assessed by multivariate Cox regression model. Results:The median overall survival (OS) of the entire cohort was 12.3 months (95% CI: 10.6-15.4m), and the 1-, 2-, 3-and 5-year OS rates were 52.1%, 25.2%, 19.1%, and 11.5%, respectively. Multivariate analysis showed that tumor length, the number of metastatic organs, and treatment modalities were the independent prognostic factors for OS. There was no significant difference in OS between concurrent radiochemotherapy and sequential radiochemotherapy ( P=0.955). The OS of patients in the radiotherapy dose of ≥6000 cGy group was significantly longer than that of their counterparts in the 4500-5039 cGy and 5040-6000 cGy groups (both P<0.001). Conclusions:For stage Ⅳ B thoracic ESCC patients at initial diagnosis, tumor length ≤3cm, single organ metastasis, and radiochemotherapy strategy are significantly correlated with longer OS. For stage Ⅳ ESCC patients with good physical status, radiotherapy can be supplemented on the basis of systemic chemotherapy. Concurrent or sequential radiochemotherapy needs to be individualized. If patients are tolerable, radiochemotherapy is recommended to the primary tumor or non-regional metastatic lymph nodes, aiming to prolong the OS of patients.
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Objective:To study the relationship between gastric fundus size and postoperative gastroparesis and to find effective ways to prevent postoperative gastroparesis in high-risk patients.Methods:We retrospectively reviewed the clinical data of 276 gastric cancer patients undergoing radical gastrectomy from 2015 to 2016. The gastric fundus volume/total gastric volume (FV/TV) ratio was measured by computed tomography (CT) and comparative study between the gastroparesis group and the non-gastroparesis group was carried out in terms of postoperative gastroparesis. Receiver operating characteristic (ROC) curve analysis was used to evaluate the predictive performance. Single-factor and multiple-factor analyses were performed to filter clinically significant predictive factors of gastroparesis. Then, we increased the sample size to 304 patients whose FV/TV ratio was >19.4%. The different surgical methods and perioperative management of these patients were analysed. The chi-square test and logistic regression analysis were performed to identify effective independent factors for preventing gastroparesis.Results:The FV/TV ratio in the gastroparesis group was significantly higher than that in the non-gastroparesis group ( P<0.05). A cut-off value of 19.4% was selected by ROC curve analysis, at which the FV/TV ratio had a sensitivity of 76.2% and a specificity of 53.7%. In 304 patients in the second retrospective study, the incidence of gastroparesis was 9.2%. Gastroparesis was significantly reduced in patients with residual gastric size <1/3 ( P<0.05) and early postoperative gastrointestinal decompression ( P<0.05). Conclusions:The FV/TV ratio can effectively predict the risk of postoperative gastroparesis preoperatively. Small residual stomach and early postoperative gastrointestinal decompression are effective measures to prevent gastroparesis in high-risk patients.
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2019 novel coronavirus(2019-nCoV) outbreak is one of the public health emergency of international concern.Since the 2019-nCoV outbreak, China has been adopting strict prevention and control measures, and has achieved remarkable results in the initial stage of prevention and control.However, some imported cases and sporadic regional cases have been found, and even short-term regional epidemics have occurred, indicating that the preventing and control against the epidemic remains grim.With the change of the incidence proportion and the number of cases in children under 18 years old, some new special symptoms and complications have appeared in children patients.In addition, with the occurrence of virus mutation, it has not only attracted attention from all parties, but also proposed a new topic for the prevention and treatment of 2019-nCoV infection in children of China.Based on the second edition, the present consensus further summarizes the clinical characteristics and experience of children′s cases, and puts forward recommendations on the diagnostic criteria, laboratory examination, treatment, prevention and control of children′s cases for providing reference for further guidance of treatment of 2019-nCoV infection in children.
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With continuous discovery of tumor immune targets and continuous changes in antibody research and development technology, antibody drugs are becoming more and more widely used in clinical practice. However, some targets are not only expressed on tumor cells, but also on red blood cells. Therefore, the clinical application of antibodies against the corresponding targets may interfere with the detection of blood transfusion compatibility, resulting in difficulty in blood matching or delay of blood transfusion. This consensus summarizes the current solutions for the interference of CD38 monoclonal antibody (CD38 mAb) in transfusion compatibility testing. After analyzing the advantages and disadvantages of different methods, polybrene and sulfhydryl reducing agents [dithiothreitol (DTT) or 2-mercaptoethanol (2-Me)], as a solution for CD38 mAb interference in blood compatibility testing, are recommended for Chinese patients, so as to eliminate blood transfusion interference produce by CD38 mAb and further provide a pre-transfusion workflow for clinicians and technicians in Department of Blood Transfusion.
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【Objective】 To investigate and analyze the clinical blood use and full-time professional technical personnel of Blood Transfusion Departments in tertiary hospitals in the Yangtze River Delta, in order to provide basis for health administration and medical institutions to make decisions on clinical blood transfusion management and standardize the construction of Blood Transfusion Departments. 【Methods】 According to the Clinical Blood Quality Control Index (2019 Edition), 435 tertiary hospitals in the Yangtze River Delta were selected for statistical analysis. 【Results】 Among the 435 tertiary hospitals, 316 (72.64%, 316/435) set up independent Blood Transfusion Departments, but 119 (27.36%, 119/435) did not. 295 (67.82%, 295/435) met the national standards for Blood Transfusion Department, and 263 (60.46%, 263/435) met the local standards. Only 5 of the top 10 tertiary hospitals with the most consumption of blood in the Yangtze River Delta met the national standards for the allocation of blood transfusion professional and technical personnel, and 17 hospitals met the local standards. Nine of the top 10 tertiary hospitals with the most beds met the national standards for blood transfusion professional and technical personnel, and 19 met the local standards. Among the full-time professional technicians in Blood Transfusion Department, medical technicians, physicians and nurses accounted for 91.03%, 6.46% and 2.52%; doctor, master, bachelor and below accounted for 1.74%, 12.4% and 85.86%; senior, intermediate, junior and below titles accounted for 18.82%, 38.61% and 42.58%, respectively. 【Conclusion】 Standardized construction of Blood Transfusion Department of tertiary hospitals in the Yangtze River Delta, including organization and talent team construction, needs to be strengthened to improve scientific and rational use of clinical blood.
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【Objective】 To compare the transfusion effects of ABO homotype leukocyte depleted suspended RBC and washed RBC in patients, who present major and minor cross-match incompatibility, DAT+, IAT+ and autoantibody+ . 【Methods】 The hemoglobin and total bilirubin of patients before and after transfusion were detected, and statistical analysis was conducted by IBM SPSS Statistics 22.0 software. 【Results】 34 transfusions were performed in 17 patients with major and minor cross-match incompatibility. Both leukocyte depleted suspended RBC and washed RBC significantly increased Hb level(P0.05), with similar transfusion efficacy(P>0.05). After t, Hb levels(g/L) increased by 11.35±8.07 and 13.94±9.017, and TBIL(μmol/L) decreased by 25.76±88.63 and 6.91±9.39, respectively, after the transfusion of leukocyte depleted suspended RBC and washed RBC per unit. 【Conclusion】 For blood recipients with major and minor cross-match incompatibility, both ABO homotype leukocyte depleted suspended RBC and washed RBC, given in time, were effective. However, washed RBC is secondary choice due to long preparation time and short storage time.
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Rare bleeding disorders (RBD) are autosomal recessive inherited diseases caused by one or more coagulation factor defects, including the deficiency of fibrinogen (FG), prothrombin, factor (F)V, Ⅶ, Ⅹ，Ⅺ, Ⅷ and so on. Due to the low prevalence of RBD, and lack of large-scale randomized controlled studies in the world, where are great challenges to clinicians in diagnosis and treatment of this series of diseases. Facing in the heterogeneity of clinical phenotype and laboratory characteristics, it is more necessary to strengthen the communication and cooperation between the clinical and laboratory, realizing comprehensive management.
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Objective:To analyze the phenotype and genotype of a Chinese pedigree with congenital dysfibrinogenemia and investigate the molecular mechanism of the disease.Methods:Pedigree analysis. Peripheral blood samples were collected from 7 members of the pedigree and routine coagulation tests were conducted. The activity of fibrinogen was measured using Clauss method, and fibrinogen antigen was measured by immunoturbidimetry. All the exons and exon-intron boundaries of FGA, FGB and FGG genes were amplified using PCR, which was followed by direct sequencing. Electrophoretic and immunological analysis of fibrinogen, fibrinogen clottability measurement, fibrin polymerization measurement and scanning electron microscopy were used to investigate the pathogenesis of this disease. Results:The proband showed normal activated partial thromboplastin time (APTT) , prolonged prothrombin time(PT), thrombin time (TT),and reptilase time (RT).The antigen level of fibrinogen in the proband (1.6 g/L) decreased slightly, while the activity level of fibrinogen (0.7 g/L) decreased significantly. His father and grandmother showed normal APTT and PT, prolonged TT and RT. The antigen levels of fibrinogen in both of them were normal (2.0 g/L and 2.2 g/L, respectively), while the activity levels of fibrinogen were low (1.0 g/L and 1.1 g/L, respectively). The results of other members from the pedigree were all within the normal range. Genetic analysis revealed a heterozygous A>G mutation at nucleotide 4774 in exon 6 of FGG gene in the proband, which was predicated to be a novel Gln195Arg mutation. The mutation was also found in his father and grandmother.Western blot results showed that no abnormal bands of plasma fibrinogen were found in the proband, his father and grandmother. The fibrinogen clottability in the proband was 49.3%, while that in the heathy control was 98.9%. Both thrombin-induced fibrin polymerization and reptilase-induced fibrin polymerization were significantly impaired in the proband, compared to that in the heathy control. Scanning electron microscopy revealed that compared with the heathy control, the average fiber diameters of the fibrin clot in the proband increased significantly ( P<0.001), while the density of fibers decreased and the arrangement of fibers was sparse. Conclusions:The heterozygous Arg19Gly mutation, which probably damages functions of fibrinogen, should be responsible for the congenital dysfibrinogenemia in this pedigree. This mutation has not been reported.
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Objective:To establish clinical detection of coagulation factor Ⅷ activity by chromogenic substrate assay and evaluate its clinical application.Methods:A total of 40 hemophilia Apatients, 20 acquired hemophilia A patients, 26 patients with positive lupus anticoagulant and 60 apparently healthy people were enrolled from January 2018 to May 2019 in Ruijin Hospital of Medical College, Shanghai Jiaotong University. According to Clinical and Laboratory Standards Institute(CLSI), the accuracy, within-run and between-run imprecision, lower detection limit, linear range, carryover rate, reference range, and reportable range of chromogenic substrate assay for detecting coagulation factor Ⅷ activity was evaluated, and compared with coagulation assay. The clinical application was evaluated by detecting F Ⅷ activity in acquired hemophilia A patients and patients with lupus anticoagulant by chromogenic substrate assay.Results:The results of two constant quality control products were within range provided by the manufacturer(the bias was 3.93%-6.79%). The within-run imprecision was 1.86%-2.06%(≤5%). The between-run imprecision was 4.83%-6.90%(≤15%). The chromogenic substrate assay had a good performance in sensitivity(CV=11.23%<20%). The recommended reference range was appropriate for our laboratory. The maximum dilution was 1∶16. The linear range was 5.00%-193.50% (a=1.0243, R 2=1.000). The clinical reportable range was 0.50%-387.00%. The method had a low carryover rate (0.04%). The chromogenic substrate assay had good consistency with coagulation assay in detecting coagulation factor Ⅷactivity (R 2=0.961) as well as the titer of FⅧ inhibitor(R 2=0.973).The difference of FⅧ activity in patients with lupus anticoagulant between these two assays was statistically significant(t=9.232,P<0.05). Conclusion:The chromogenic substrate assay has a good performance in clinical detection of coagulation factor Ⅷactivity with wider clinical application and less interference.
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ObjectiveTo investigate the clinical value of ultrasound-guided real-time percutaneous transhepatic-cholangial or transhepatic-cholecyst drainage (PTCD) in the treatment of patients with acute obstructive cholangitis in primary hospitals. MethodsA retrospective analysis was performed for the clinical data of 38 patients with acute obstructive cholangitis who underwent ultrasound-guided real-time PTCD in Tengchong People’s Hospital from February 2012 to June 2018, and among these patients, 4 had malignant biliary obstruction and cholangitis and 34 had acute obstructive suppurative cholangitis caused by hepatolithiasis. ResultsOf all patients, 30 underwent puncture of the bile duct in the right lobe of liver, 6 underwent puncture of the bile duct in the left lobe of liver, and 2 underwent puncture of the gallbladder. The success rate of first-time needle insertion was 91.2% (35/38), and the overall success rate of puncture was 100%. There were no complications such as massive hemorrhage, biliary peritonitis, and biliary-vascular fistula. At 2 weeks after surgery, 89.5% (34/38) of the patients had a bilirubin level reduced by ≥1/2, and 32 patients (84.2%) had a level of alanine aminotransferase reduced to normal. As for the 34 patients with hepatolithiasis, second-stage surgeries including bile duct exploration/cholecystolithotomy and hepatic lobectomy were performed after the infection was under control, or they were referred to superior hospitals with tube indwelling for further treatment. The 4 patients with neoplastic bile duct obstruction were referred to superior hospitals with tube indwelling, among whom 3 underwent radical surgery and 1 underwent biliary stent placement via the former deep blind fistula. ConclusionUltrasound-guided real-time PTCD can be used as an effective, simple, and safe biliary drainage technique for the early treatment of acute biliary obstruction in primary hospitals. This technique has a high success rate and low requirements for medical equipment, and therefore, it holds promise for clinical application in primary hospitals after related technical procedures are standardized.