Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive hematological malignancy, there is no standard treatment and the prognosis is very poor. Affiliated Zhongshan Hospital of Dalian University report a case of 85-year-old BPDCN male patient treated with DVT regimen (decitabine combined with Venetoclax and thalidomide) and achieved complete remission. The patient with skin nodules and the pathology diagnosed BPDCN, the next generation sequencing of skin nodules showed mutations of IDH2 and ASXL1. DVT (decitabine combined with Venetoclax and thalidomide) has significant efficacy with rapid and deep remission for BPDCN, and the adverse effects is less, especially suitable for elderly patients who cannot tolerate intense chemotherapy.

Objective:To investigate the clinical features and family gene mutation results of the adult onset Gaucher disease patient.Methods:The diagnosis and treatment of a patient with Gaucher disease who was admitted to the Affiliated Zhongshan Hospital of Dalian University in March 2020 were retrospectively analyzed, and the literature was reviewed.Results:The patient (the proband) was a 34-year-old female, the anemia, thrombocytopenia and splenomegalia were found in 2005. After regular physical examination, the decrease of leukocyte, red blood cell and platelet was gradually aggravated, and the progressive enlargement of the spleen was aggravated. She visited the clinic on March 19, 2020 due to worsening of fatigue. The physical examination revealed hepatomegaly, splenomegaly; blood routine examination showed pancytopenia, and bone marrow morphology showed a large number of Gaucher cells. Peripheral blood β- glucocerebrosidase (GBA) of the proband was 1.8 nmol·mg -1·h -1 (reference value 10-25 nmol·mg -1·h -1), and the GBA of her father, mother, brother, and daughter was normal. The second-generation sequencing results showed that the proband had a mutation in the BCL2 gene at locus c.127G>A p.A43T, with a mutation frequency of 49.53%. GBA gene testing showed that the proband had a heterozygous mutation at c.1448T>C (a pathogenic mutation), heterozygous mutations at c.1026A>G and c.1038C>T (homozygous mutations), and a heterozygous mutation at c.1075G>T (mutation of unknown clinical significance). The proband's father had c.1448T>C heterozygous mutation, and he was a carrier; the proband's mother had c.1075G>T heterozygous mutation; the proband's brother had no gene mutation; the proband's daughter had c.1448T>C heterozygous mutation, and she was a carrier. The diagnosis was type Ⅰ Gaucher disease (non-neuropathic). The patient was proposed to participate in the clinical trial of imiglucerase, and during the follow-up, the blood routine was relatively stable, and the spleen remained progressively enlarged. Conclusions:Gaucher disease is rare, which is easy to be misdiagnosed and underdiagnosed. It is necessary to be alert to the possibility of Gaucher disease when there are patients with unexplained hemocytopenia, liver and spleen enlargement and multiple organs involvement, and enzyme and genetic tests should be performed as soon as possible.

Objective:To summarize the experience of brain death determination under veno-arterial extracorporeal membrane oxygenation (VA-ECMO) assisted circulatory support, especially apnea test (AT) precautions, and to provide references for brain death determination in this scenario.Methods:In 78 patients who had VA-ECMO at Organ Transplant Center, Zhengzhou People's Hospital from October 2019 to December 2023, 8 organ donors had brain death determination under VA-ECMO assisted circulatory support. Baseline data, clinical data, and VA-ECMO data during AT trial were collected from these 8 patients to summarize the process of brain death determination.Results:Six of the 8 donors met the criteria of brain death; 10 EEG, 12 evoked potentials and 15 ATs were performed. Complications in ATs, including hypotension, decreased oxygenation and arrhythmia, were alleviated after timely improved VA-ECMO flow and applied cardiotonic and pressor drugs.Conclusion:AT is key for successful brain death determination in organ donors under VA-ECMO assisted circulatory support; therefore, complications should be closely monitored and managed.

BACKGROUND: Health literacy is a public health goal which can be used as an independent factor of health outcomes. This study aimed to assess the association between health literacy and health status, as well as the two mediating factors of behavior and self-efficacy among residents aged 15-69 years in Qingdao. METHODS: A cross-sectional survey was implemented among residents aged 15-69 years (N = 3793) in Qingdao, China. A combination of stratified cluster random and proportional probability sampling methods was used to select subjects for this study. Data were collected using "The Chinese Citizen Health Literacy Questionnaire (2019)". We proposed a hypothetical model for the relationship between sociodemographic characteristics, health literacy, self-efficacy, health behavior, and health status, and used path analysis to validate the hypothesis. RESULTS: The path analysis showed that higher education (β = 0.293) and income (β = 0.135) are positively and directly associated with greater health literacy, which was positively associated with health status (β = 0.057). Health literacy is a direct influencing factor of health behavior (β = 0.070) and self-efficacy (β = 0.099). Health behavior (β = 0.041) and self-efficacy (β = 0.173) exerted a positive direct effect on health status. The model explained 14.1% of variance for health literacy, 3.8% for self-efficacy, 5.7% for health behavior, and 15.0% for health status. CONCLUSIONS Health literacy was identified to be a critical factor in health status. The results emphasized that the dissemination of health knowledge, development of healthy behavior, and cultivation of self-efficacy should be jointly promoted to reinforce the level of health status among residents in future work.
Adolescent ; Adult ; Aged ; China ; Cross-Sectional Studies ; Female ; Health Behavior ; Health Knowledge, Attitudes, Practice ; Health Literacy/statistics & numerical data* ; Health Status ; Humans ; Male ; Middle Aged ; Young Adult