1.Analysis and summary of clinical characteristics of 289 patients with paroxysmal nocturnal hemoglobinuria in Zhejiang Province
Gaixiang XU ; Weimei JIN ; Baodong YE ; Songfu JIANG ; Chao HU ; Xin HUANG ; Bingshou XIE ; Huifang JIANG ; Lili CHEN ; Rongxin YAO ; Ying LU ; Linjie LI ; Jin ZHANG ; Guifang OUYANG ; Yongwei HONG ; Hongwei KONG ; Zhejun QIU ; Wenji LUO ; Binbin CHU ; Huiqi ZHANG ; Hui ZENG ; Xiujie ZHOU ; Pengfei SHI ; Ying XU ; Jie JIN ; Hongyan TONG
Chinese Journal of Hematology 2024;45(6):549-555
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.
2.Long-term hypomethylating agents in patients with myelodysplastic syndromes: a multi-center retrospective study
Xiaozhen LIU ; Shujuan ZHOU ; Jian HUANG ; Caifang ZHAO ; Lingxu JIANG ; Yudi ZHANG ; Chen MEI ; Liya MA ; Xinping ZHOU ; Yanping SHAO ; Gongqiang WU ; Xibin XIAO ; Rongxin YAO ; Xiaohong DU ; Tonglin HU ; Shenxian QIAN ; Yuan LI ; Xuefen YAN ; Li HUANG ; Manling WANG ; Jiaping FU ; Lihong SHOU ; Wenhua JIANG ; Weimei JIN ; Linjie LI ; Jing LE ; Wenji LUO ; Yun ZHANG ; Xiujie ZHOU ; Hao ZHANG ; Xianghua LANG ; Mei ZHOU ; Jie JIN ; Huifang JIANG ; Jin ZHANG ; Guifang OUYANG ; Hongyan TONG
Chinese Journal of Hematology 2024;45(8):738-747
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.
3. Clinicopathologic and molecular features of cribriform morular variant of papillary thyroid carcinoma
Xiujie CUI ; Haiou ZHAO ; Peng SU ; Jie CHEN ; Renya ZHANG ; Yi PAN ; Xiaoming OUYANG ; Jun LIU ; Jianqiang ZHANG ; Yang YANG ; Rong YANG ; Lan DING ; Zhiyan LIU
Chinese Journal of Pathology 2018;47(5):354-359
Objective:
To investigate the clinicopathologic and molecular features of the rare cribriform morular variant of papillary thyroid carcinoma (CMV-PTC).
Methods:
The clinicopathologic data of 10 patients with CMV-PTC were retrospectively reviewed. Immunohistochemical (IHC) staining was done using LSAB method. DNA sequencing for APC were applied using Sanger method. BRAF V600E mutation was examined using ARMS method. The cytological, morphological, IHC and molecular features were analyzed.
Results:
All patients were female at an average age of 27 years old. The tumors were mostly located in the right lobe of thyroid. Fine needle aspiration cytology was performed in three patients; two were diagnosed as suspicious for PTC and one as PTC. Nine tumors presented as solitary nodule and two as multiple nodules in both lobes. Infiltration was demonstrated in three cases. The average size was 2.6 cm. The neoplastic cells were arranged in papillary, cribriform, solid and glandular patterns, with rare or without colloid inside the lumen. The number of morula varied, ranging from zero to many. The neoplastic cells were variably enlarged, showing round, oval or spindle shape. Nuclear irregularity was identified as irregular membrane, nuclear grooves or pseudoinclusion, but no typical ground glass feature. Peculiar nuclear clearing could be observed in the morular cells. IHC staining showed the neoplastic cells were negative for thyroglobulin and p63, but positive for TTF1, cytokeratin 19 and estrogen receptor. Diffuse staining with cytokeratin was seen in the neoplastic cells and the morula. Specific cytoplasmic and nuclear staining of β-catenin was seen in the neoplastic cells but not the morula. Ki-67 proliferation index was 1%-30%. No recurrence or metastasis was observed. One patient was demonstrated to harbor both somatic and germline mutations of the APC gene, who was found to have adenomatous polyposis and her mother died of colonic carcinoma. No BRAF V600E mutation was detected.
Conclusions
CMV-PTC is rare and shows atypical cytological and clinicopathological features, and it is easily misdiagnosed.TG, TTF1, ER and β-catenin are specific IHC markers for CMV-PTC. The morula is negative for cytokeratin 19, in contrast to squamous metaplasia. Although CMV-PTC has indolent clinical behavior, a definite diagnosis is necessary to rule out the possibility of APC gene mutation and related extra-thyroidal neoplasm, such as FAP and Gardner syndrome.
4.An 8-week group positive psychotherapy intervention on depression associated self-efficacy and subjective well-being in female nursing students
Yufang GUO ; Shuwen LI ; Ouying CHEN ; Qinghua ZHANG ; Xiujie QI ; Jingping ZHANG
Chinese Mental Health Journal 2015;(3):172-177
Objective:To explore the effects of eight-week group positive psychotherapy intervention on de-pression associated self-efficacy and subjective well-being in female nursing students.Methods:Totally 74 female nursing students with mild to moderate depression [the score of Beck Depression Inventory (BDI-Ⅱ)was 14 to 28]were selected from three nursing colleges of Changsha.They were randomly divided into two groups,40 in the control group and 34 in the intervention group.Samples in the intervention group were given the eight-week group positive psychotherapy,while the control group was given nothing (the control group could receive the intervention when the intervention was proved to be effective or the control group needed treatments).The General Self-efficacy Scale(GSES)and Index of Well-being(IWB)were used to get the outcome of female nursing students'self-effica-cy,subjective well-being at baseline,the time after the intervention,3 month follow-up,6 month follow-up.Results:The interactive effect between group and time of self-efficacy,subjective well-being were statistically significant (Ps<0.05).Separate analyses showed that at baseline,there was no significant difference between two groups in all variables (Ps >0.05).At the end of the intervention,3 month follow-up and 6 month follow-up,the GSES score and IWB score in the intervention group were higher than those in the control group (Ps <0.05).Conclusion:Eight-week group positive psychotherapy could improve self-efficacy and subjective well-being of female nursing students who have mild to moderate depressive symptom.This intervention also has a long-term effect.
5.Incidence and risk factors of human papilloma virus type 16 infection
Meng DUAN ; Xiujie CHEN ; Pengpeng QU
Tianjin Medical Journal 2015;(4):379-382
Objective To investigate the incidence and risk factors of human papillomavirus type 16 (HPV16) infec?tion among women through health screenings in Tianjin urban area. Methods A total of 2 000 women from Tianjin urban area were recruited for this study, and potential risk factors were investigated by questionnaire as well as by collecting cervi?cal exfoliated cells from the participants. HPV type was examined using polymerase chain reaction and pyrosequencing tech?nology, then the risk factors of HPV16 infection were examined by chi square test and the multiple factor logistic regression analysis. Results Among those 2 000 women who received health screenings, a total of 271 cases were HPV positive (13.55%). HPV16 accounts for the most common human papillomavirus types (39.5%, 107/271) while HPV58 account for 15.13%(41/271)and HPV18 account for 9.59% (26/271). Factors related to HPV16 infection were investigated include smoking history, initial age of sex (>25 years old or not) , number of pregnancies (≥2 or not), number of sexual partners (≥2 or not) and contraception measures (condom or other ways) with χ2=6.268,8.540,4.951,7.123,6.053,5.019, respectively. (P < 0.05). Age, alcohol intake, education level and previous gynecologial examination did not differ significantly among HPV16 positive patients compared to those with negative HPV. Multiple factor Logistic regression model analysis showed that the independent factors were smoking history and initial age for sex(≤25 years old)(P<0.05). Conclusion HPV16 is the most common type of cervical infection among women in Tianjin urban area. Changing inappropriate sexual behavior and smoking cessation are effective measures to prevent HPV16 infection and reduce incidence of cervical cancer.
6.The Analysis of HPV Genotypes and Related High Risk Factors in 459 Women of Annual Health Examination
Xiujie CHEN ; Yanling LI ; Pengpeng QU
Tianjin Medical Journal 2014;(2):123-126
Objective To investigate the cervical human papillomavirus (HPV) infection, genotypes and related high risk factors in 459 women of annual health examination. Methods A total of 459 cervical cytology specimens were de-tected with nest PCR and pyrosequencing methods. Questionnaires were collected simultaneously and the risk factors of HPV infection were analyzed. Results The detection rate of HPV-positive samples was 17.9%. Seven kinds of high-risk genotypes of HPV were found. The detection of HPV 16 was the most common (9.8%), followed by HPV58 (7.0%) and HPV18 (5.2%). The detection rates of simplex infection, dual infections and multiple infections were 9.6%, 4.8%and 1.5%respectively. The risk factors for HPV infection included alcohol consumption,income <3 000 yuan/ month, sexual part-ners>1, frequency of sexual activity>4 times/month and cervical erosion (P<0.05). Conclusion HPV DNA genotyping can provide important reference for HPV screening at early time and the application of HPV vaccines, which also provide sig-nificant evidence for the prevention, diagnosis and treatment of cervical cancer at early stage for the high risk population.
7.The lowest dosages of mifepristone and misoprostol to terminate ultra-early pregnancy
Cuilan LI ; Dunjin CHEN ; Xiujie SHENG ; Mingxing LIU ; Huinan WENG ; Peili DU ; Min WEI ; Qian LIU
Chinese Journal of Obstetrics and Gynecology 2012;47(10):764-768
Objective To explore the lowest effective dosage of mifepristone combined with misoprostol in terminating ultra-early pregnancy.Methods All the cases of ultra-early pregnancy classified by amenorrhea days,β-hCG and vaginal B-ultrasonic were randomly divided into two groups.One hundred cases in G1 group (minimized dosage) were orally administered 25 mg mifepristone once a day for 2 days and combined with 200 μg misoprostol 48 hours later,while 150 mg mifepristone combined with 600 μg misoprostol 48 hours later were given to 100 cases in G2 group (normal dosage).All cases were observed for 6 hours after taking misoprostol and returned for assessment three days later.Results None missing.Expulsion of conceptus:G1 and G2 group were 22 (22.0%,22/100) and 25 (25.0%,25/100;P > 0.05).Failure rate:cases with incomplete abortion were 1 (1.0%,1/100) and 2 (2.0%,2/100) in G1 and G2 group,hospitalization for suspected ectopic pregnancies both was 1 (1.0%).Bleeding:bleeding cases during the administration of mifepristone in G1 and G2 group were 71 (71.0%,71/100) and 78 (78.0%,78/100; P>0.05); the mcan bleeding time were (5.3 ± 1.4) days and (6.0± 1.5) days (P <0.01).Other side effects:in G1 group,majority showed light nausea (7.0%,7/100) and light abdominal pain (20.0%,20/100).Menses recovery:99 (99.0%,99/100) for G1 group and 98 (98.0%,98/100) for G2 group to recovery on scheduled time.Satisfactions:both were 99 (99.0%,99/100).Except mean bleeding days and side-effects,the differences above showed no significance (P > 0.05).Conclusion It is safe and effective treatment with the lowest dosages of mifepristone and misoprostol to terminate ultra-early pregnancies.
8.Effect of the phagocytosis function on NR8383 ceil exposed to cigarette smoke extracts
Wenjuan ZHANG ; Xiujie PAN ; Zhihua YANG ; Wangshun HU ; Jiangjin LIU ; Feng CHEN ; Maoxiang ZHU
Chinese Journal of Microbiology and Immunology 2011;(3):261-264
Objective To investigate the phagocytosis function of cigarette smoke extracts (CSE)on the NR8383 cells. Methods The concentration of CSE and the optimal time was defined by cell counting kit-8 assay, Annexin V/PI cell apoptosis assay and CFSE cell proliferation assay. The cell was gained after exposed to the different concentration of CSE for 24 h and mixed with fluorescein-labeled Escherichia coli in 37℃ for 2 h. The fluorescence intensity was used to assay the phagocytosis function of NR8383 cells.Results The phagocytosis function of NR8383 cells may be changed by the concentration of CSE. In the concentration of 100 μg/ml, the phagocytosis function of NR8383 was enhanced 0.5 times than the normal cell when NR8383 cell was exposed to CSE, and the specific activity is the highest. When NR8383 cells were exposed to CSE and LPS, the phagocytosis function of NR8383 cells was enhanced 2 times than the normal cell. In the concentration of 200 μg/ml, the phagocytosis function of NR8383 cells was damaged, the rate of apoptosis is the 54. 1%. Conclusion Low concentration of CSE enhanced the phagocytosis function of NR8383 cells, but high concentration of CSE damaged the phagocytosis function of NR8383 cells. This study reveals a new role of CSE as an activator of macrophage function.
9.Hearing the impact of MP3 on a survey of middle school students.
Zhan XU ; Zonghua LI ; Yang CHEN ; Ya HE ; Xiujie CHUNYU ; Fangyuan WANG ; Pengzhi ZHANG ; Lei GAO ; Shuping QIU ; Shunli LIU ; Li QIAO ; Jianhua QIU
Journal of Clinical Otorhinolaryngology Head and Neck Surgery 2011;25(4):151-153
OBJECTIVE:
To understand the usage of MP3 and effects on hearing of middle school students in Xi'an, and discuss controlling strategies.
METHOD:
Stratified random cluster sampling method was used in the 1567 middle school students in Xi'an through questionnaire survey, ear examination and hearing examination, data were analysed by the SPSS13.0 statistical software.
RESULT:
1) The rate of holding MP3 in the middle school students was 85.2%. Average daily use time was (1.41 +/- 1.11) h. 2) The noise group of pure tone hearing threshold was significantly higher compared with the control group (P<0.01), and increased the detection rate of hearing loss with the increasing use of MP3. 3) The detection rate of symptoms increased with the increasing use of MP3.
CONCLUSION
The usage of MP3 can harm hearing in middle school students, which can result in neurasthenic syndrome.
Adolescent
;
Audiometry, Pure-Tone
;
Auditory Threshold
;
Data Collection
;
Female
;
Hearing Loss
;
etiology
;
Hearing Tests
;
Humans
;
MP3-Player
;
Male
;
Students
;
Surveys and Questionnaires
10.Association study between depression and tryptophan hydroxylase 2 gene(rsl386494,G1463A)polymorphism in Yunnan Han population
Jing CAO ; Zhuangfei CHEN ; Xiujie ZHANG ; Fang LIU ; Yong ZENG
Chinese Journal of Behavioral Medicine and Brain Science 2010;19(2):140-142
Objective To investigate the association of the two single nucleotide polymorphisms(SNPs) rs1386494 and G1463A of tryptophan hydroxylase 2 gene with depression in Yunnan Han population. Methods A case-control study Was designed by collecting 102 patients and 102 controls.Polymerase chain reaction-restriction fragment length polymorphisms(PCR-RFLP)technique Was used to detect the rs1386494 and G1463A polymorphisms.All patients were evaluated with Hamilton Rating Scale for Depression(HAMD),and scores were compared according to different genotype.Results 1.There were differences in genotype and allele frequency of rsl386494 in Yunnan Han population(X~2=4.300,P=0.038;X~2=4.067,P=0.044).The A allele frequency of rsl386494 in controls Was higher than in patients(P=0.044).2.No genotype of G1463A polymorphism was observed in all samples.3.No significant association genotype of rsl386494 with scores of HAMD Was observed(F=2.461,P=0.120).Conclusion The polymorphism of rsl386494 may be associated with the vulnerability of Yunnan Han population,and the A allele maybe the protective gene for depression.

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