[Analysis and summary of clinical characteristics of 289 patients with paroxysmal nocturnal hemoglobinuria in Zhejiang Province].

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.

[Long-term hypomethylating agents in patients with myelodysplastic syndromes: a multi-center retrospective study].

Objective: To evaluate the efficacy and safety of hypomethylating agents (HMA) in patients with myelodysplastic syndromes (MDS) . Methods: A total of 409 MDS patients from 45 hospitals in Zhejiang province who received at least four consecutive cycles of HMA monotherapy as initial therapy were enrolled to evaluate the efficacy and safety of HMA. Mann-Whitney U or Chi-square tests were used to compare the differences in the clinical data. Logistic regression and Cox regression were used to analyze the factors affecting efficacy and survival. Kaplan-Meier was used for survival analysis. Results: Patients received HMA treatment for a median of 6 cycles (range, 4-25 cycles) . The complete remission (CR) rate was 33.98% and the overall response rate (ORR) was 77.02%. Multivariate analysis revealed that complex karyotype (P=0.02, OR=0.39, 95%CI 0.18-0.84) was an independent favorable factor for CR rate. TP53 mutation (P=0.02, OR=0.22, 95%CI 0.06-0.77) was a predictive factor for a higher ORR. The median OS for the HMA-treated patients was 25.67 (95%CI 21.14-30.19) months. HMA response (P=0.036, HR=0.47, 95%CI 0.23-0.95) was an independent favorable prognostic factor, whereas complex karyotype (P=0.024, HR=2.14, 95%CI 1.10-4.15) , leukemia transformation (P<0.001, HR=2.839, 95%CI 1.64-4.92) , and TP53 mutation (P=0.012, HR=2.19, 95%CI 1.19-4.07) were independent adverse prognostic factors. There was no significant difference in efficacy and survival between the reduced and standard doses of HMA. The CR rate and ORR of MDS patients treated with decitabine and azacitidine were not significantly different. The median OS of patients treated with decitabine was longer compared with that of patients treated with azacitidine (29.53 months vs 20.17 months, P=0.007) . The incidence of bone marrow suppression and pneumonia in the decitabine group was higher compared with that in the azacitidine group. Conclusion: Continuous and regular use of appropriate doses of hypomethylating agents may benefit MDS patients to the greatest extent if it is tolerated.

[Knowledge, attitude and practice about first aid and influencing factors in key populations in Beijing: a large cross-sectional study].

Objective: To investigate the status of knowledge, attitude and practice about first aid and influencing factors in key population, those who have frequent contacts with people and might be "first aid" promoter in general population of Beijing, and provide evidences for the promotion of first aid. Methods: This study was a cross-sectional survey based on the "Emergency Rescue Capacity Training Project". Using cluster sampling, we recruited 9 591 participants after the training in Beijing for survey with a self-designed questionnaire from October 2021 to March 2022. Results: In 9 591 participants, 98.58% knew the emergency call number, while only 32.00% knew how to identify sunstroke. The awareness rate of the definition of first aid and legal disclaimer were lower (63.61%, 75.91%). People's willingness to give first aid to others, including relative, acquaintance, stranger and vagrant, were different (χ2=822.82, P<0.001). People were more likely to give first aid to relative, acquaintance and stranger compared with vagrant (P<0.05). With the increase of psychological distance, the willingness to give first aid decreased (Z=-26.57, P<0.001). According to the results of Logistic regression analysis, older age, higher annual family income and higher awareness of first aid related knowledge were contributing factors for giving first aid. In 1 529 people who met emergency, only 66 people (4.32%)did not give any first aid. Conclusions: The key population in Beijing has high awareness of first aid related knowledge and positive attitude of first aid after training. A high proportion of people performed first aid to others. Age, annual family income and awareness of first aid related knowledge were contributing factors for giving first aid.

Analysis of high-resolution HLA-A, -B, -Cw, -DRB1, and -DQB1 alleles and haplotypes in 718 Chinese marrow donors based on donor-recipient confirmatory typings.

High-resolution human leucocyte antigen (HLA)-A, -B, -Cw, -DRB1, and -DQB1 alleles and haplotype frequencies were analysed from 718 Chinese healthy donors selected from the Chinese Marrow Donor Program registry based on HLA donor-recipient confirmatory typings. A total of 28 HLA-A, 61 HLA-B, 30 HLA-Cw, 40 HLA-DRB1 and 18 HLA-DQB1 alleles were identified, and HLA-A*1101, A*2402, A*0201, B*4001, Cw*0702, Cw*0102, Cw*0304, DRB1*0901, DRB1*1501, DQB1*0301, DQB1*0303 and DQB1*0601 were found with frequencies higher than 10% in this study population. Multiple-locus haplotype analysis by the maximum-likelihood method revealed 45 A-B, 38 Cw-B, 47 B-DRB1, 29 DRB1-DQB1, 24 A-B-DRB1, 38 A-Cw-B, 23 A-Cw-B-DRB1, 33 Cw-B-DRB1-DQB1 and 22 A-Cw-B-DRB1-DQB1 haplotypes with frequencies >0.5%. The most common two-, three-, four- and five-locus haplotypes in this population were: A*0207-B*4601 (7.34%), Cw*0102-B*4601 (8.71%), B*1302-DRB1*0701 (6.19%), DRB1*0901-DQB1*0303 (14.27%), A*3001-B*1302-DRB1*0701 (5.36%), A*0207-Cw*0102-B*4601 (7.06%), A*3001-Cw*0602-B*1302-DRB1*0701 (5.36%), Cw*0602-B*1302-DRB1*0701-DQB1*0202 (6.12%) and A*3001-Cw*0602-B*1302-DRB1*0701-DQB1*0202 (5.29%). Presentation of the high-resolution alleles and haplotypes data at HLA-A, -B, -Cw, -DRB1 and -DQB1 loci will be useful for HLA matching in transplantation as well as for other medical and anthropological applications in the Chinese population.

Effect of topical aclacinomycin on glaucoma filtration surgery in rabbits.

A prospective, randomized, double-masked and placebo-controlled study was performed to compare the effects of a single 5-minute intraoperative exposure to aclacinomycin (AMC) 0.4 mg/ml or 0.8 mg/ml with control eyes treated with saline solution on the success of glaucoma filtration surgery in 26 rabbits. Intraocular pressure (IOP), bleb survival, fistula patency and complications were evaluated. The results showed that IOP in the eyes treated with AMC was significantly lower than that in the control eyes from days 5-40 in the 0.4 mg/ml group and from days 5-20 in the 0.8 mg/ml group. The bleb survival lasted significantly longer in the two treated groups than in the control group and in the AMC 0.4 mg/ml group than in the AMC 0.8 mg/ml group. At 40 days, the rate of sclera fistula occlusion was 0% in the AMC 0.4 mg/ml eyes, 43.8% in the AMC 0.8 mg/ml eyes, and 100% in the control eyes. Significant complications, such as anterior chamber inflammation, hyphema, moderate and severe corneal haze, dense corneal neovascularization and mild cataract occurred only in the eyes treated with AMC 0.8 mg/ml. The results indicated that intraocular treatment with AMC at a dose of 0.4 mg/ml had a markedly beneficial effect on IOP, bleb appearance and fistula patency after experimental filtration surgery in rabbits.

Identification of a novel HLA-B allele HLA-B*4070 in a Chinese donor.

Identification of a novel human leukocyte antigen-B allele HLA-B*4070.