1.Application of E- learning in training of related knowledge about public health emergencies in Emergency department
Junhua CHEN ; Youzhen HU ; Hui XIONG ; Huijun HUANG
Chinese Journal of Practical Nursing 2015;(30):2331-2333
Objective To apply E- learning in training of related knowledge about public health emergencies in emergency department in order to improve the treatment level of medical staff in public health emergencies. Methods The medical staff was divided into the observation group and the control group with 50 persons in each group according to the odd and even grouping method. The control group was given conventional training method, while the observation group received E- learning based on conventional training method. The theoretical test results and training effect were compared between two groups. Results The scores of the observation group were obviously higher than that of the control group [ (90.88 ± 4.09) points vs. (84.36± 4.92) points ], Z= -5.895, P<0.01. Conclusions The application of E- learning in the training of public health emergencies is beneficial to help the medical staff to obtain and share information resources more conveniently, to enhance the ability of the clinic medical workers of understanding the condition of emergent public health affairs, to elevate the grades of theoretical learning of the relevant knowledge and to improve the management level of training, training effect and efficiency.
2.The clinical and laboratory characteristics of congenital pyruvate kinase deficiency
Lin SONG ; Yuan LI ; Guangxin PENG ; Li ZHANG ; Liping JING ; Kang ZHOU ; Yang LI ; Lei YE ; Jianping LI ; Huihui FAN ; Xin ZHAO ; Wenrui YANG ; Yang YANG ; Yuping ZHAO ; Youzhen XIONG ; Zhijie WU ; Fengkui ZHANG
Chinese Journal of Internal Medicine 2018;57(7):511-513
Clinical data of 19 patients with congenital pyruvate kinase deficiency were analyzed. Insufficient pyruvate kinase confirmed the diagnosis. Laboratory parameters of hemolysis were summarized. In cases of neonatal hyperbilirubinemia and unexplained hemolytic anemia, pyruvate kinase activity and next generation sequencing test may help the early diagnosis.
3. Correlation of the degree of band 3 protein absence on erythrocyte membrane by eosin-5′-maleimide binding test and clinical phenotype in hereditary spherocytosis
Guangxin PENG ; Wenrui YANG ; Liping JING ; Li ZHANG ; Kang ZHOU ; Yang LI ; Lei YE ; Yuan LI ; Jianping LI ; Huihui FAN ; Lin SONG ; Xin ZHAO ; Zhijie WU ; Yang YANG ; Youzhen XIONG ; Huijun WANG ; Fengkui ZHANG
Chinese Journal of Hematology 2017;38(6):537-541
Objective:
To investigate the relationship between the eosin-5′-maleimide (EMA) binding test and the clinical severity of hereditary spherocytosis (HS).
Methods:
A total of 258 un-splenectomize HS patients were consecutively enrolled. Correlation of hemoglobin concentration, hemolytic parameters, compensating erythropoiesis and the EMA binding test were evaluated.
Results:
258 (128 male and 130 female) patients were included in this study, including 91 compensatory hemolysis patients, 53 patients with mild anemia, 78 patients with moderate anemia and 36 patients with severe anemia. The median age at diagnosis was 23 (2-70) years. The median decreased fluorescence intensity of EMA binding test was 29.97% (16.09%-47.34%) and the average intensity was (29.70±6.28) % of 258 HS patients. The decreased EMA binding fluorescence intensity correlated with MCV (
4. The characteristic of hereditary spherocytosis related gene mutation in 37 Chinese hereditary spherocytisis patients
Guangxin PENG ; Wenrui YANG ; Xin ZHAO ; Liping JIN ; Li ZHANG ; Kang ZHOU ; Yang LI ; Lei YE ; Yuan LI ; Jianping LI ; Huihui FAN ; Lin SONG ; Yang YANG ; Youzhen XIONG ; Zhijie WU ; Huijun WANG ; Fengkui ZHANG
Chinese Journal of Hematology 2018;39(11):898-903
Objective:
To reveal the genetic characteristics of erythrocyte membrane protein in hereditary spherocytosis (HS) in China.
Methods:
Next-generation sequencing technology was used to detect mutations in genes of erythrocyte membrane proteins in 51 clinically diagnosed HS patients. The relationship between gene mutations and clinical phenotypes was analyzed.
Results:
Mutations in erythrocyte membrane protein genes were detected in 37 patients, including 17 with ANK1 mutations (17/37, 45.9%), 14 with SPTB mutations (14/37, 37.8%), and 5 with SLC4A1 mutations (5/37, 13.5%). One patient carried both heterozygous ANK1 mutation and SPTB mutation (1/37, 2.7%). SPTA1 and EPB42 mutation was not fou nd in any patient. Nonsense mutations (36.8%) and missense mutations (31.6%) were most common. Of the 38 mutations detected, 34 were novel mutations and have not been reported elsewhere (89.5%). Sixteen HS patients underwent parental genetic validation, 6 patients (37.5%) inherited gene mutation from parents and 10 (62.5%) were
5. Using target next-generation sequencing assay in diagnosing of 46 patients with suspected congenital anemias
Yuan LI ; Guangxin PENG ; Qingyan GAO ; Yang LI ; Lei YE ; Jianping LI ; Lin SONG ; Huihui FAN ; Yang YANG ; Youzhen XIONG ; Zhijie WU ; Wenrui YANG ; Kang ZHOU ; Xin ZHAO ; Liping JING ; Fengkui ZHANG ; Li ZHANG
Chinese Journal of Hematology 2018;39(5):414-419
Objective:
To evaluate the impact of the targeted next-generation sequencing (NGS) assay for difficult congenital anemias.
Methods:
Blood Disease Hospital Anemia Panel 2014 (BDHAP-2014) including 217 known genes of congenital anemias was developed. NGS and parental verification were performed for patients who were suspected diagnosed with congenital anaemia from August 2014 to July 2017.
Results:
A total of 46 patients were enrolled in this study, the clinical suspection were 11 cases Fanconi anemia (FA), 8 cases congenital dyserythropoietic anemia (CDA), 6 cases congenital sideroblast anemia (CSA), 12 cases congenital hemolytic anemia (CHA), 1 case dyskeratosis congenital (DC), 4 cases iron-refractory iron deficiency anemia and 4 cases unexplained cytopenia (Uc), respectively. 28 (60.9%) of 46 patients became confirmed cases after targeted NGS, corresponding to 44 mutations of which 33 were new. 26(56.5%) patients with results of the assay matching to clinical suspection, including FA (5/11, 45.5%), CSA (6/6, 100.0%), CDA (3/8, 37.5%) and CHA (12/12, 100.0%). 2 (4.3%) cases not matching to clinical suspection, including dyskeratosis congenital (DC) was made in 1(2.2%) patients with suspected FA and familial hemophagocytic lymphohistiocytosis (FHL) was made in 1(2.2%) patients with suspected unexplained cytopenia (Uc). In 12 CHA patients, the hemolytic type was further clarified by the NGS. The remaining 18 cases were not clearly diagnosed.
Conclusion
Targeted NGS assay is of major impact on congenital anemias. The assay should be used routinely in congenital anemias.
6.Clinical characteristics of aplastic anemia patients with abnormal autoantibodies and the impact of autoantibodies on immunosuppressive therapy response
Weiru LIANG ; Rui KANG ; Xin ZHAO ; Li ZHANG ; Liping JING ; Wenrui YANG ; Yuan LI ; Lei YE ; Kang ZHOU ; Jianping LI ; Huihui FAN ; Yang YANG ; Youzhen XIONG ; Fengkui ZHANG
Chinese Journal of Internal Medicine 2023;62(10):1200-1208
Objective:To investigate the clinical characteristics of patients with acquired aplastic anemia (AA) accompanied by abnormal antinuclear antibody (ANA) and autoantibodies and their effects on the efficacy of immunosuppressive therapy (IST).Method:A retrospective case-control study was conducted, analyzing the clinical data of 291 patients with AA who underwent IST and were screened for autoantibodies at initial diagnosis between January 2018 and December 2019 at Blood Diseases Hospital, Chinese Academy of Medical Sciences. According to the titer of ANA at the initial diagnosis, extracted nuclear antigen antibodies (ENAs) abnormality and the change of ANA titer after treatment, the treatment responses of 3 months and 6 months after IST were compared. The correlation between clinical features and ANA abnormality was analyzed by univariate and multivariate logistic regression analysis. The parameters of univariate analysis P<0.1 were included in multivariate analysis, stepwise regression analysis and subgroup analysis. Results:A total of 291 patients were included in the study, of which 145 (49.83%) were male. Among all patients, 147 (50.52%) tested positive for ANA at initial diagnosis, with titers of 1∶100, 1∶320, and 1∶1 000 observed in 94, 47, and 6 cases, respectively. Female gender, older age, presence of paroxysmal nocturnal hemoglobinuria (PNH) clone, and higher levels of IgG, IgA, and thyroid hormone were significantly associated with ANA positivity at initial diagnosis, while white cell counts, reticulocytes, and free triiodothyronine were significantly lower than that of ANA-negatively patients (all P<0.05). Furthermore, logistic regression analyses revealed that female gender ( OR=1.980, 95% CI 1.206-3.277), older age ( OR=1.017, 95% CI 1.003-1.032), and presence of PNH clone ( OR=1.875, 95% CI 1.049-3.408) were independent risk factors for ANA positivity at initial diagnosis. Subgroup analysis indicated that the risk of ANA positivity at initial diagnosis was even higher in PNH clone-positive patients in the subgroups of females ( OR=1.24, 95% CI 1.02-1.51), severe AA ( OR=1.26, 95% CI 1.07-1.47), and age≥40 years ( OR=1.26, 95% CI 1.05-1.52) (all P<0.05). However, ANA titers at initial diagnosis, presence of other abnormal ENAs, and changes in ANA titers after treatment with IST were not correlated with treatment response (all P>0.05). Conclusions:Approximately 50% of patients with AA had abnormal ANA, and their presence was significantly associated with female gender, older age, and presence of PNH clone at initial diagnosis. However, the presence of abnormal ANA and changes in ANA titers after treatment did not affect the efficacy of IST in patients with AA.