Objective: To study changes of ultrastructure of atrial endothelial cells during acute atrial fibrillation (AF) in canines, and explore the possible mechanism of AF left atrial thrombosis. Methods: A total of 16 healthy adult mongrel canines were randomly and equally divided into blank control group (only received thoracotomy without pacing) and rapid atrial pacing (RAP) group (established acute AF model). Myocardial tissue of left and right appendage were taken from two groups and received hematoxylin eosin (HE) staining, then myocardial cell morphological changes was observed under ordinary light microscope; morphological changes of appendage endothelial cells was observed under electron microscope. Results: (1) Paroxysmal AF was successfully induced in all canines of RAP group; (2) There were no significant difference in morphology of appendage and endocardial tissue under ordinary light microscope between two groups; 3. Under transmission electron microscope, endothelium cell of appendage tissue presented defect of different extent, and some shedding in RAP group; while endothelial cell layer was complete with absence of necrosis and shedding in blank control group. Compared with blank control group, there was significant rise in endothelial cell incompleteness (12.5% vs. 75.0%) in RAP group, P=0.041. Conclusion: When acute atrial fibrillation occurs, endothelial cell ultrastructure has already changed, which may be related to thrombosis adhered to wall during atrial fibrillation.

Objective: To determine the national norm of Chinese questionnaire of quality of life in Chinese patients with cardiovascular diseases (CQQC) according patients with hypertension, coronary artery disease（CAD）, and congestive heart failure（CHF）and to analyze the influence factors. Methods: The quality of life was estimated by CQQC to evaluate 7937 persons normal and with cardiovascular diseases（contain with hypertension, CAD, and CHF ） in 28 hospitals of 19 cities across China. The data were compared in gender, age, exercise, psychologic condition, education, number of family member and medical cost. Results: 1、The mean scores（national norms）of normal persons were （95.74±23.21）; 2、The scores of hypertension patients were （73.75±23.62）, the scores of hypertension stage 3 group （69.06±21.78）were least compared with other stages（P<0.01）; 3、The scores of CAD patients were（64.67±20.59）, the scores of unstable angina group（55.37±18.62）were least compared with other types in CAD patients（P<0.01）; 4、The scores of CHF patients were （40.36±17.24）, it was least compared with those of hypertension and CAD patients （P<0.01）; 5、Influence factor: （1）Sex, age: The mean scores were （71.41±27.8）for 7937 persons, the scores of male persons were significantly higher than those of female (P=0.000). In normal persons, along with age increased, the scores decreased （P<0.05） ; except ≥70 ages group, the scores of male persons were higher than those of female（P<0.05） ; （2）Exercise: The scores of patients participating exercise were significantly higher than those of no participating exercise [（72.64±21.87）vs.（66.91±23.13）, P=0.000]; （3）Psychological condition: The scores of patients with healthy psychological condition were significantly higher than those of with psychologic disorder [（70.13±25.66）vs.（68.91±20.44）, P=0.001]; （4）Other: patients with better education, more than 1 family members and medical insurance or public expense had more scores (P<0.05～0.001). Conclusions： The sample size is large and the datum is reliable for Chinese questionnaire of quality of life in Chinese patients with cardiovascular diseases. It can reflect influence of age, sex, exercise, psychologic condition, family, education background, medical condition and disease, its item is brief, clear, and operation is easy, so it may well be spread.

Objective: To analyze the clinical characteristics of children with Burkitt lymphoma (BL) and to summarize the mid-term efficacy of China Net Childhood Lymphoma-mature B-cell lymphoma 2017 (CNCL-B-NHL-2017) regimen. Methods: Clinical features of 436 BL patients who were ≤18 years old and treated with the CNCL-B-NHL-2017 regimen from May 2017 to April 2021 were analyzed retrospectively. Clinical characteristics of patients at disease onset were analyzed and the therapeutic effects of patients with different clinical stages and risk groups were compared. Survival analysis was performed by Kaplan-Meier method, and Cox regression was used to identify the prognostic factors. Results: Among 436 patients, there were 368 (84.4%) males and 68 (15.6%) females, the age of disease onset was 6.0 (4.0, 9.0) years old. According to the St. Jude staging system, there were 4 patients (0.9%) with stage Ⅰ, 30 patients (6.9%) with stage Ⅱ, 217 patients (49.8%) with stage Ⅲ, and 185 patients (42.4%) with stage Ⅳ. All patients were stratified into following risk groups: group A (n=1, 0.2%), group B1 (n=46, 10.6%), group B2 (n=19, 4.4%), group C1 (n=285, 65.4%), group C2 (n=85, 19.5%). Sixty-three patients (14.4%) were treated with chemotherapy only and 373 patients (85.6%) were treated with chemotherapy combined with rituximab. Twenty-one patients (4.8%) suffered from progressive disease, 3 patients (0.7%) relapsed, and 13 patients (3.0%) died of treatment-related complications. The follow-up time of all patients was 24.0 (13.0, 35.0) months, the 2-year event free survival (EFS) rate of all patients was (90.9±1.4) %. The 2-year EFS rates of group A, B1, B2, C1 and C2 were 100.0%, 100.0%, (94.7±5.1) %, (90.7±1.7) % and (85.9±4.0) %, respectively. The 2-year EFS rates was higher in group A, B1, and B2 than those in group C1 (χ²=4.16, P=0.041) and group C2 (χ²=7.21, P=0.007). The 2-year EFS rates of the patients treated with chemotherapy alone and those treated with chemotherapy combined with rituximab were (79.3±5.1)% and (92.9±1.4)% (χ²=14.23, P<0.001) respectively. Multivariate analysis showed that stage Ⅳ (including leukemia stage), serum lactate dehydrogenase (LDH)>4-fold normal value, and with residual tumor in the mid-term evaluation were risk factors for poor prognosis (HR=1.38,1.23,8.52,95%CI 1.05-1.82,1.05-1.43,3.96-18.30). Conclusions: The CNCL-B-NHL-2017 regimen show significant effect in the treatment of pediatric BL. The combination of rituximab improve the efficacy further.
Adolescent ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Burkitt Lymphoma/drug therapy* ; Child ; Disease-Free Survival ; Female ; Humans ; Lactate Dehydrogenases ; Lymphoma, B-Cell/drug therapy* ; Male ; Prognosis ; Retrospective Studies ; Rituximab/therapeutic use* ; Treatment Outcome

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disease caused by abnormal expression of glycosylphosphatidylinositol (GPI) on the cell membrane due to mutations in the phosphatidylinositol glycan class A(PIGA) gene. It is commonly characterized by intravascular hemolysis, repeated thrombosis, and bone marrow failure, as well as multiple systemic involvement symptoms such as renal dysfunction, pulmonary hypertension, swallowing difficulties, chest pain, abdominal pain, and erectile dysfunction. Due to the rarity of PNH and its strong heterogeneity in clinical manifestations, multidisciplinary collaboration is often required for diagnosis and treatment. Peking Union Medical College Hospital, relying on the rare disease diagnosis and treatment platform, has invited multidisciplinary clinical experts to form a unified opinion on the diagnosis and treatment of PNH, and formulated the Expert Consensus of Multidisciplinary Diagnosis and Treatment for Paroxysmal Nocturnal Hemoglobinuria (2024), with the hope of promoting the standardization of PNH diagnosis and treatment.