Objective:To further improve the understanding of paroxysmal nocturnal hemoglobinuria (PNH), we retrospectively analyzed and summarized the clinical characteristics, treatment status, and survival status of patients with PNH in Zhejiang Province.Methods:This study included 289 patients with PNH who visited 20 hospitals in Zhejiang Province. Their clinical characteristics, comorbidity, laboratory test results, and medications were analyzed and summarized.Results:Among the 289 patients with PNH, 148 males and 141 females, with a median onset age of 45 (16-87) years and a peak onset age of 20-49 years (57.8% ). The median lactic dehydrogenase (LDH) level was 1 142 (604-1 925) U/L. Classified by type, 70.9% (166/234) were classical, 24.4% (57/234) were PNH/bone marrow failure (BMF), and 4.7% (11/234) were subclinical. The main clinical manifestations included fatigue or weakness (80.8%, 235/289), dizziness (73.4%, 212/289), darkened urine color (66.2%, 179/272), and jaundice (46.2%, 126/270). Common comorbidities were hemoglobinuria (58.7% ), renal dysfunction (17.6% ), and thrombosis (15.0% ). Moreover, 82.3% of the patients received glucocorticoid therapy, 70.9% required blood transfusion, 30.7% used immunosuppressive agents, 13.8% received anticoagulant therapy, and 6.3% received allogeneic hematopoietic stem cell transplantation. The 10-year overall survival (OS) rate was 84.4% (95% CI 78.0% -91.3% ) . Conclusion:Patients with PNH are more common in young and middle-aged people, with a similar incidence rate between men and women. Common clinical manifestations include fatigue, hemoglobinuria, jaundice, renal dysfunction, and recurrent thrombosis. The 10-year OS of this group is similar to reports from other centers in China.

Objective To investigate the role of low dose esketamine in pretreated limb ischemia-reperfusion inju-ry.Methods The rats were divided into sham-operated group(Sham group),ischemia-reperfusion group(I/R group,3 h of ischemia and 2 h of reperfusion),esketamine group(ESK group,ip,5 mg/kg).The plasma con-centrations of creatine kinase(CK)and lactate dehydrogenase(LDH)were measured.The wet/dry weight ratio of skeletal muscle was immediately detected.The gastrocnemius muscle was harvested and the level of malondial-dehyde(MDA)and superoxide dismutase(SOD)was detected by colorimetric assay.The expression of nuclear factor erythroid 2-related factor 2(Nrf2)and heme oxygenase-1(HO-1)were detected by Western blot and im-munohistochemical staining.Results Compared with Sham group,the index of wet/dry weight ratio,MDA,CK,LDH,Nrf2 and HO-1 were all increased,but SOD was decreased in I/R group(P＜0.05).Compared with I/R group,the index of wet/dry weight ratio,MDA,CK and LDH were significantly lower,but SOD and Nrf2 and HO-1 were significantly higher in ESK group(P＜0.05).Conclusions ESK may increase Nrf2 in the nucleus,thereby increase the HO-1 protein as an antioxidant agent.

Objective:To explore the association of platelet to lymphocyte ratio (PLR) and neutrophil to lymphocyte ratio (NLR) with early gastric cancer (EGC), and to assess the predictive value of PLR and NLR in EGC diagnosis.Methods:From January 1, 2017 to December 31, 2020, 178 patients with EGC, 129 patients with chronic gastritis (CG), 122 patients with gastric intraepithelial neoplasia (GIN) admitted and treated at Taizhou Hospital of Zhejiang Province were enrolled. According to Rand random function and with the ratio of 7 to 3, the patients were divided into training group ( n=301, 125 cases of EGC, 90 cases of CG, 86 cases of GIN) and validation group ( n=128, 53 cases of EGC, 39 cases of CG, 36 cases of GIN). The age, gender, routine blood test, carcinoembryonic antigen (CEA) level, Helicobacter pylori ( H. pylori) infection status and other data of the patients were collected. The routine blood test and clinical characteristics of EGC, CG and GIN patients of the training group, and the routine blood test of EGC patients and CG+ GIN patients (hereinafter referred to as non-EGC group) of training group were compared to analyzed the independent risk factors of EGC. Receiver operator characteristic curve (ROC) was drawn. The optimal cut-off value, area under the curve (AUC), OR, 95% confidence interval (95% CI) of independent risk factors were analyzed for EGC diagnosis and prediction. A diagnostic prediction model was established, and the model was apply to the validation group for validation. Hosmer-Lemeshow test was used to test the fitting degree of the model. Compared the AUC of the model applied to training group with validation group to evaluate the discrimination of model. Kruskal-Wallis H test, Mann-Whitney U test or Wilcoxon rank sum test, chi square test, and univariate and multivariate logistic regression analysis were used for statistical analysis. Results:In the training group, the proportions of males and females in CG, GIN and EGC patients were 50.0% (45/90) and 50.0% (45/90), 61.6% (53/86) and 38.4% (33/86), 69.6% (87/125) and 30.4% (38/125), respectively, and the difference was statistically significant ( χ2=8.49, P=0.014). The proportion of males in EGC patients was higher than that in CG patients, and the difference was statistically significant ( χ2 =8.48, P=0.004). The H. pylori infection rate, age, PLR, NLR, lymphocyte count, neutrophil count, and CEA level of CG, GIN and EGC patients in the training group were 18.9% (17/90), 18.6% (16/86) and 43.2% (54/125); 54.0 years old (45.5 years old, 64.0 years old), 63.0 years old (58.0 years old, 66.3 years old) and 66.0 years old (58.5 years old, 71.0 years old); 113.70 (84.48, 136.09), 120.00 (97.94, 138.37) and 124.29 (101.97, 173.57), 1.55 (1.17, 2.23), 1.71 (1.44, 2.02) and 2.04 (1.57, 2.62), 2.00×10 9/L (1.50×10 9/L, 2.40×10 9/L), 1.75×10 9/L (1.50×10 9/L, 2.40×10 9/L) and 1.60×10 9/L (1.30×10 9/L, 2.05×10 9/L), 3.00×10 9/L (2.38×10 9/L, 3.90×10 9/L), 3.00×10 9/L (2.48×10 9/L, 3.40×10 9/L) and 3.30×10 9/L (2.60×10 9/L, 4.30×10 9/L), 1.70 g/L (1.10 g/L, 2.50 g/L), 2.05 g/L (1.48 g/L, 2.90 g/L) and 2.50 g/L (1.55 g/L, 3.40 g/L), respectively, and the differences were statistically significant ( χ2=21.26, H=41.00, 11.79, 21.13, 10.82, 8.54 and 14.42; all P<0.05). The H. pylori infection rate of EGC patients was higher than that of CG and GIN patients, the ages of EGC and GIN patients were older than that of CG patients, the NLR and PLR levels of EGC patients were higher than those of CG patients, the NLR level of EGC patients was higher than that of GIN patients, the level of lymphocyte count of EGC patients was lower than that of CG patients, and the levels of neutrophil count and CEA were higher than those of CG patients, and the differences were statistically significant( χ2=13.98 and 13.90, Z=-6.13, -4.15, -4.07, -3.25, -3.40, -3.18, -2.62 and -3.74; all P<0.017). The levels of PLR, NLR, neutrophil count and CEA of EGC patients were all higher than those of non-EGC patients(124.29 (101.97, 173.57) vs. 117.97 (101.57, 137.32); 2.04(1.57, 2.62) vs.1.66(1.25, 2.17); 3.30×10 9/L (2.60×10 9/L, 4.30×10 9/L) vs.3.00×10 9/L(2.40×10 9/L, 3.60×10 9/L); 2.50 g/L (1.55 g/L, 3.40 g/L) vs. 1.90 g/L(1.23 g/L, 2.70 g/L)), and the lymphocyte count level was lower than that of non-EGC patients (1.60×10 9/L(1.30×10 9/L, 2.05×10 9/L) vs. 1.80×10 9/L(1.50×10 9/L, 2.20×10 9/L)), and the differences were statistically significant ( Z=-3.23, -4.45, -2.91, -3.30 and -2.35; all P<0.05). The results of ROC analysis showed that the optimal cut-off value of PLR, NLR, CEA, neutrophil count and lymphocyte count was 138.18, 1.76, 2.70 g/L, 3.40×10 9/L, 1.80×10 9/L, respectively. The results of univariate analysis indicated that the gender, age, H. pylori infection, neutrophil count, PLR, NLR, lymphocyte count and CEA were all related to EGC ( χ2=5.98, 27.73, 21.26, 8.26, 10.26, 22.80, 4.81 and 25.91; all P<0.05). The results of multivariate analysis demonstrated that age≥70 years old( OR=9.267, 95% CI 3.239 to 26.514), H. pylori infection ( OR=3.353, 95% CI 1.862 to 6.037), NLR >1.76 ( OR=2.084, 95% CI 1.190 to 3.648), PLR>138.18 ( OR=2.452, 95% CI 1.325 to 4.539), CEA >2.70 g/L ( OR=2.637, 95% CI 1.490 to 4.667) were independent risk factors for EGC (all P<0.05). The Hosmer-Lemeshow test showed that there was no statistically significant difference between the predicted value of the model and the actual observed value ( P>0.05), which indicated that the fitting degree of the model was good. In the training group, the AUC of the diagnostic prediction model was 0.787 (95% CI 0.737 to 0.832, P<0.001). The model was applied to the validation group for validation, and the result showed that the AUC of the model was 0.664 (95% CI 0.576 to 0.745, P<0.001), which indicated that the discrimination of the model was good. Conclusions:PLR and NLR are independent risk factors of EGC, and may help to identify EGC. In this study the established diagnostic model has good discrimination and fitting degree, which can provide important reference information for early clinical diagnosis of EGC, which may facilitate early treatment and improve prognosis of patients.

Objective:To explore the relationship and mechanism among job stressors, perceived professional benefits and humanistic practice ability in midwives, so as to provide reference for the training and management of midwives.Methods:Used the convenient sampling method to conduct a cross-sectional survey on 216 midwives in 6 comprehensive or specialized hospitals in Zhejiang Province by using Stressor Scale, Perceived Professional Benefits Questionnaire and Humanistic Practice Ability Scale from May to September 2020. The mediating effect was analyzed on the basis of the current situation analysis and correlation analysis.Results:The total scores of humanistic practice ability, job stressors and perceived professional benefits of midwives were (102.13 ± 25.91), (72.77 ± 14.49) and (138.23 ± 18.71) respectively. The job stressors of midwives were negatively correlated with humanistic practice ability ( r=-0.206, P<0.01), perceived professional benefits were positively related to humanistic practice ability ( r=0.289, P<0.001) and were negatively correlated with job stressors ( r=-0.507, P<0.001). Perceived professional benefits played a major mediating role in the influence of stressors on humanistic practice ability (the total standardized effect was -0.233, and the indirect effect was -0.144). Conclusions:Perceived professional benefits act as a main mediator between job stressors and humanistic practice ability in midwives. Managers should reduce job stressors, enhance perceived professional benefits, and promote the formation, development and practice of humanistic practice ability of midwives.

Objectives To provide genetic counseling and prenatal molecular diagnosis for two families with megalencephalic leukoencephalopathy with subcortical cysts (MLC).Methods Two MLC patients (probands 1 and 2) were admitted to the Department of Pediatrics of Peking University First Hospital in June 2011 and June 2009,respectively.Peripheral blood was collected and DNA sequencing was performed for genetic analysis for the two MLC patients and their parents.Amniotic fluid and villus of two fetuses (fetus 1 and 2) were collected at 21+4 and 12+3 weeks of gestational age from their mothers when they were pregnant again.The genomic DNA of the two fetuses was extracted and corresponding sites of MLC1 gene were sequenced.Haplotype analysis using a combination of 3 microsatellite markers (AR,DXS6807 and DXS6797) on chromosome X and sex determining region of Y chromosome was performed to detect maternal cell contamination.Verification of the prenatal molecular diagnosis and follow up study after birth were conducted for both fetuses.Results Macrocephaly,motor development delay and typical findings on brain MRI were identified in the two probands,and were clinically diagnosed with MLC.Compound heterozygous mutations were detected in proband 1 [c.353C＞T (p.T118M) and c.803C＞G (p.T268R)] and proband 2 [c.353C＞T (p.T118M) and c.836T＞C(p.L279P)],respectively.MLC was genetically diagnosed.Heterozygous variation in c.353[c.353C＞T (p.T118M)] and wild c.803C were identified in fetus 1,and both wild c.353C and c.836T were found in fetus 2.No maternal cell contamination was detected in both fetuses.Sequencing the corresponding sites after birth confirmed the prenatal diagnosis,and the head circumference and motor development were normal in fetus 1 at 5 months old.No macrocephaly was found and no DNA sequencing was done in fetus 2 at one month old.Conclusions Genetic counseling and prenatal molecular diagnosis for MLC families combined with clinical and genetic diagnosis are important in preventing MLC.Haplotype analysis with a combination of three microsatellite markers on chromosome X and sex determining region of Y chromosome is useful in detecting maternal cell contamination and avoiding its influence on prenatal diagnosis,and confirming the reliability of prenatal diagnosis.

Objective To investigate the effect of preoperative application of leuprolide acetate on hormone levels,menstrual and uterine fibroids recurrence in patients with laparoscopic myomectomy.Methods A total of 100 patients with hysteromyoma were randomly divided into experimental group (n=50) and control group (n=50).The control group was treated with laparoscopic myomectomy,while the experimental group was treated with leuprorelin acetate before laparoscopic myomectomy.The levels of serum hormones,menstruation,fibroid volume and recurrence rate of uterine fibroids were observed.Results In the experimental group,after use of leuprolide acetate,the luteinizing hormone,follicle stimulating hormone and serum estradiol were significantly lower than those before treatment,and menstrual blood volume,uterine volume,leiomyoma volume were significantly less than those before treatment (P<0.05).Compared with the control group,the amount of bleeding in operation and pelvic drainage were significantly lower,and ambulation time,hospitalization time were significantly shorter in experimental group (P<0.05).Conclusion Before the laparoscopic myomectomy,the clinical efficacy and safety of leuprolide acetate is great,which can reduce the serum hormone level,improve the volume of leiomyoma.

Objective To investigate the effect of preoperative application of leuprolide acetate on hormone levels,menstrual and uterine fibroids recurrence in patients with laparoscopic myomectomy.Methods A total of 100 patients with hysteromyoma were randomly divided into experimental group (n=50) and control group (n=50).The control group was treated with laparoscopic myomectomy,while the experimental group was treated with leuprorelin acetate before laparoscopic myomectomy.The levels of serum hormones,menstruation,fibroid volume and recurrence rate of uterine fibroids were observed.Results In the experimental group,after use of leuprolide acetate,the luteinizing hormone,follicle stimulating hormone and serum estradiol were significantly lower than those before treatment,and menstrual blood volume,uterine volume,leiomyoma volume were significantly less than those before treatment (P<0.05).Compared with the control group,the amount of bleeding in operation and pelvic drainage were significantly lower,and ambulation time,hospitalization time were significantly shorter in experimental group (P<0.05).Conclusion Before the laparoscopic myomectomy,the clinical efficacy and safety of leuprolide acetate is great,which can reduce the serum hormone level,improve the volume of leiomyoma.

Objective To investigate the status and related factors of chronic functional constipation in community elders.Methods Totally 892 elders of 11 communities at Lucheng district of Wenzhou from August 2013 to December 2015 were investigated by sample survey; methods as household survey, survey in community public health station and outpatient survey, etc. were used to develop a unified questionnaire to collect information. The chronic functional constipation was evaluated by Rome III diagnostic criteria, and statistical processing was analyzed by single-factor analysis and multi-factor logistic regression analysis.Results Totally 257 elders acquired functional constipation, and the morbidity was 28. 81%, and bowel movements were less than 3 times per week. Defecation laborious and poor stool were the main symptoms of functional constipation in elders. Independent risk factors of functional constipation in community elderly patients were: too much meat intake, few vegetables and fruit intake, drinking little water, few daily activities and depression (P<0.05).Conclusions Eating habits of partial fine diet fatness, little fiber and water intake, little exercise etc. and depression are closely related to chronic functional constipation in community elderly patients.

Objective To evaluate the safety and effectiveness of the treatment by selective β 1 receptor blockers on patients with chronic obstructive pulmonary disease (COPD).Methods Eighty cases of COPD Ⅲ (stable period) inpatient with or without coronary heart disease were collected in The Second People's Hospital of Shanghai from September 2012 to November 2013.The patients were randomly divided into testing group (Metoprolol treatment group) and control group (regular treatment group) with 40 cases for each group.Metoprolol group therapy based on the use of conventional metoprolol tablets,an initial dose of metoprolol 12.5 mg/d,titrated to the appropriate dose based on heart rate and tolerance of the morning resting heart rate of 55 to 60 times/min that reached the target dose of metoprolol continuous medication for 12 months.Blood gas analysis were recorded before and after treatment,pulmonary function,and 6 min walk test (6MWT) and were chronic lung disease Assessment Test (CAT) Rating.The control group was administrated regular treatment while the testing group added small dose of Metoprolol with titration to an appropriate dose on this basis.12 months in a row,and assessed the end stage.Results (1) After the application of selective β receptor blockers on testing group,no statistically significant difference (P＞0.05) in the values of FEV1 in anticipation value％ (testing group:(45.45 ± 4.68) ％ vs.(43.32 ± 4.84) ％;control group:(44.23 ± 4.68) ％ vs.(42.58 ±4.24)％),PaO2(testing group:(75.92± 10.78) mmHg vs.(74.86± 11.21) mmHg;control group:(70.23 ±6.45) mmHg vs.(72.36±7.28) mmHg) and PaCO2(testing group:(46.28±8.28) mmHg vs.(47.46±10.22) mmHg);control group:(44.54 ± 8.89) mmHg vs.(42.36 ± 7.45) mmHg) before and after treatment.But the 6MWD (testing group:(287 ± 23) m vs.(384± 34) m;control group:(284 ± 25) m vs.(295 ±21) m) and COPD appraisal test(CAT) (testing group:(21±7) score vs.(17±6) score);control group:(22 ±5) score vs.(20± 6) score) had improved significantly compared with that before treatment,with significant difference(t=4.903,4.784;P＜0.05).Conclusion Selective β receptor blockers have no effect on the airway resistance of COPD patients and reduction on pulmonary function.It can also increase the exercise tolerance and enhance the living quality for improving clinical prognosis.

OBJECTIVE: Differences in clinical features, especially facial nerve canal leision between cholesteatoma in external auditory meatus and middle ear were compaired. METHOD: A retrospective clinical analysis was made. Clinical data included 125 cases of middle ear cholesteatoma with facial nerve canal leision and 28 cases of cholesteatoma occurred in external auditory canal from 2003-01-2014-08 in our hospital. RESULT: Clinical course of cholesteatoma in external auditory canal was 4.97 ± 7.51 years, course of middle ear cholesteatoma was 16.60 ± 14.42 years (P < 0.01). 21 cases (75%) of external auditory canal cholesteatoma were manifested as pneumatic mastoid and 110 cases (88%) of middle ear cholesteatoma were manifested as diploic mastoid respectively. 22 cases (78.6%) of facial nerve canal damage-in mastoid segment in cholesteatoma of external auditory meatus and 76 cases (60.8%) of facial nerve canal damage in tympanic segment in cholesteatoma of middle ear were observed (P < 0.01). The incidence rate of ossicular errosion in middle ear chol-esteatoma was significantly higher than that in external auditory meatus (P < 0.01). The incidence of semicircular canal defects in middle ear cholesteatoma (30.4%), was significantly higher when comparing to the incidence (10.7%) in cholesteatoma of external auditory meatus (P < 0.05). CONCLUSION The site of facial nerve canal lesion in middle ear cholesteatoma and cholesteatoma of external auditory meatus were different. More attention should be paid before and during operation to avoid facial nerve injury, including physical examinations, especial otologic exams, radiological reading and careful operation.
Cholesteatoma, Middle Ear ; diagnosis ; pathology ; Ear Canal ; pathology ; Ear, Middle ; pathology ; Facial Nerve Injuries ; complications ; Humans ; Incidence ; Mastoid ; pathology ; Retrospective Studies ; Semicircular Canals ; pathology