1.Short-term results of a multicenter study based on a modified N7 induction regimen combined with arsenic trioxide in the treatment of children with high-risk neuroblastoma
Shu YANG ; Kailan CHEN ; Yunyan HE ; Xiaomin PENG ; Hao XIONG ; Wenguang JIA ; Sha WU ; Xunqi JI ; Yuwen CHEN ; Chuan TIAN ; Zhonglü YE ; Zhen YANG ; Jianjun ZHU ; Aiguo LIU ; Xiaohua TIAN ; Fengjuan PAN ; Ke HUANG ; Dunhua ZHOU ; Jianpei FANG ; Yang LI
Chinese Journal of Pediatrics 2024;62(10):949-955
Objective:To analyze the short-term clinical efficacy and safety of arsenic trioxide (ATO) combined with a modified N7 induction regimen in the treatment of children with high-risk neuroblastoma (NB).Methods:This study was a prospective, single-arm, multicenter phase Ⅱ clinical study. Sixty-seven high-risk NB children from eight units of Sun Yat-sen Memorial Hospital of Sun Yat-sen University, Wuhan Children′s Hospital of Tongji Medical College of Huazhong University of Science and Technology, First Affiliated Hospital of Guangxi Medical University, Hainan General Hospital, Affiliated Hospital of Guangdong Medical University, Kunming Children′s Hospital, Tongji Hospital of Tongji Medical College of Huazhong University of Science and Technology, and Guangdong Provincial Agricultural Reclamation Center Hospital were enrolled from January 2019 to August 2023 and were treated with ATO combined with a modified N7 induction regimen. The efficacy and adverse effects at the end of induction chemotherapy were assessed and analyzed, and the differences in the clinical characteristics were further compared between the treatment-responsive and treatment-unresponsive groups by using the Fisher′s exact test.Results:Among 67 high-risk NB children, there were 40 males (60%) and 27 females (40%), with the age of disease onset of 3.5 (2.6, 4.8) years. Primary NB sites were mostly in retroperitoneum (including adrenal gland) (56/67, 84%) and the common metastases sites at initial diagnosis were distant lymph node in 25 cases (37%),bone in 48 cases (72%),bone marrow in 56 cases (84%) and intracalvarium in 3 cases (4%). MYCN gene amplification were detected in 28 cases (42%). At the end of induction, 33 cases (49%) achieved complete remission, 29 cases (43%) achieved partial remission, 1 case (1%) with stable disease, and 4 cases (6%) were assessed as progressive disease (PD). The objective remission rate was 93% (62/67) and the disease control rate was 94% (63/67). The percentage of central system metastases at the initial diagnosis was higher in the treatment-unresponsive group than in the treatment-responsive group (2/5 vs. 2% (1/62), P=0.013), whereas the difference in MYCN gene amplification was not statistically significant between two groups (3/5 vs.40% (25/62), P=0.786). Grade Ⅲ or higher adverse reactions during the induction chemotherapy period were myelosuppression occurred in 60 cases (90%), gastrointestinal symptoms occurred in 33 cases (49%), infections occurred in 20 cases (30%), hepatotoxicity occurred in 4 cases (6%), and cardiovascular toxicity occurred in 1 case (2%). There were no chemotherapy-related deaths. Conclusion:ATO combined with N7-modified induction regimen had a superiority in efficacy and safety, which deserved further promotion in clinical practice.
2.Research progress on COPD-related fatigue
Jianpei CHEN ; Qinfang ZHANG ; Xiaoyun RUAN ; Meilan LIANG ; Liping TAN
Chinese Journal of Modern Nursing 2020;26(5):685-689
Fatigue is the second most common symptom in patients with chronic obstructive pulmonary disease (COPD) . It has a high incidence and interferes with patients' normal working life. This article reviews the concept and pathogenesis of fatigue, as well as the predisposing factors and treatments of fatigue symptom in patients with COPD. This article provides a reference for medical staff to deepen their understanding of fatigue in order to take effective measures to relieve fatigue and improve patients' quality of life.
3.HBB-deficient Macaca fascicularis monkey presents with human β-thalassemia.
Yan HUANG ; Chenhui DING ; Puping LIANG ; Duanduan LI ; Yu TANG ; Wei MENG ; Hongwei SUN ; Hongyu LU ; Yu CHEN ; Xueying CHEN ; Qunshan HUANG ; Jianpei FANG ; Canquan ZHOU ; Shihua YANG ; Junjiu HUANG
Protein & Cell 2019;10(7):538-542
4. Clinical analysis of three cases with beta-thalassemia
Xinyu LI ; Manjiao LIU ; Lyuhong XU ; Honggui XU ; Hailei CHEN ; Jianpei FANG
Chinese Journal of Pediatrics 2018;56(4):294-297
Objective:
To study the diagnostic strategy of β-thalassemia through retrospective analysis of 3 cases of β-thalassemia.
Methods:
Three patients were admitted to the Department of Pediatrics, Sun Yat-sen Memorial Hospital of Sun Yat-sen University from January 2014 to June 2015. The clinical manifestations, hemoglobin electrophoresis and gene detection of these patients and their parents were analyzed, diagnostic ideas and key points were discussed when beta thalassemia gene detection did not explain clinical manifestations or hemoglobin electrophoresis.
Results:
Case 1, boy, 5 years old, was diagnosed as compound heterozygotes of β41-42 and IVS-Ⅱ-654 with hereditary persistence of fetal hemoglobin(HPFH) according to the clinical manifestations of mild anemia, normal size of liver and spleen, 92.8% fetal hemoglobin (HbF) and gene analysis. Case 2, girl, 3 years old, was confirmed the diagnosis of thalassemia intermedia with β41-42 heterozygote compound and αααanti3.7 heterozygote in accordance with the manifestations of severe anemia, hepatosplenomegaly, 8.6% HbF, 4.1% hemoglobin A2(HbA2) and gene analysis. Case 3, girl, 3 years old, with severe anemia, hepatosplenomegaly, 51.2% HbF and 3.7% HbA2, was diagnosed as thalassemia major with compound heterozygotes of PolyA (T→C) and β17 by DNA sequencing.
Conclusion
The diagnosis of β-thalassemia should be confirmed by clinical manifestations of hemolytic anemia, hemoglobin electrophoresis, gene diagnosis and family survey.
5. Simultaneous determination of various volatile organic compounds in urine by headspace GC-MS
Yiran LIN ; Tiandi LI ; Fen LIU ; Jianpei YUN ; Yuxuan XIE ; Jianhui CHEN
Chinese Journal of Industrial Hygiene and Occupational Diseases 2018;36(4):305-307
Objective:
To establish a method for determination of acetone, dichloromethane, hexane, 1, 1, 1-trichloroethane, 1, 2-dichloroethane, benzene, toluene, ethylbenzene etc organic compounds in urine by headspace gas chromatography-mass spectrometry (GC-MS) .
Methods:
Headspace gases of urine samples were injected into GC and determined by mass.
Results:
Determination of urine components were in a good linear range in their concentration range of this method. The correlation coefficients were between 0.996 and 1.000 with the detection limits between 0.1 μg/L and 4.5 μg/L, the precisions were between 1.3% and 4.6%, the recovery rates were between 86.2% and 97.4%.
Conclusion
This method has the advantages of low detection limits, high accuracy, high precision and simple pretreatment, which is suitable for the determination of the content of various volatile organic compounds in urine.
6.Risk factors analysis of hair loss in obese patients afer laparoscopic sleeve gastrectomy
Haijun GUO ; Jiangfan ZHU ; Yingzhang MA ; Bhagat SACHIN ; Dongliang CAO ; Lin TANG ; Jie CHEN ; Jianpei PENG
Chinese Journal of Digestive Surgery 2017;16(6):592-595
Objective To investigate the related risk factors of hair loss in obese patients after laparoscopic sleeve gastrectomy (LSG).Methods The retrospective case-control study was conducted.The clinical data of 54 obese patients who underwent LSG in the East Hospital of Tongji University between November 2013 and June 2015 were collected.All the patients received LSG,and postoperative hair loss of patients was observed.Factors affecting postoperative severe hair loss were analyzed,including gender,age,preoperative body mass index (BMI),postoperative excess weight loss (EWL),total bilirubin (TBil),albumin (Alb),hemoglobin (Hb),iron,zinc,copper,folic acid,vitamin B12 and vitamin D.Observation indicators:(1) follow-up and postoperative hair loss situations:cases with follow-up,follow-up time,cases with hair loss,severity of hair loss,time of hair loss,treatment of hair loss;(2) univariate analysis affecting severity of hair loss after LSG;(3) multivariate analysis affecting severity of hair loss after LSG.Follow-up using outpatient examination and Wechat was performed to detect the changes of BMI and hair loss up to September 2016.Measurement data with normal distribution were represented as (x)±s and comparison between groups was done by the t test.Comparison of count data was analyzed by the chi-square test.Multivariate analysis was done using the Logistic regression model.Results (1) Follow-up and postoperative hair loss situations:all the 54 patients were followed up for 15 months.Forty-two patients had hair loss,including 21 with slight hair loss,10 with moderate hair loss and 11 with severe hair loss.A proportion of hair loss was 6/11 in male and 36/43 in female.The onset time and end time of hair loss were (3.4± 1.4) months and (9.0± 3.6) months,respectively.Of 42 patients,15 took oral medication (6 with ferrous sulfate,5 with decavitamin and 4 with zinc gluconate oral solution) against hair loss,with no obvious improvement.During the follow-up,42 patients stopped hair loss and gradually grow new hair.(2) Univariate analysis affecting severity of hair loss after LSG:gender,postoperative EWL and folic acid were factors affecting severity of hair loss after LSG (x2 =5.161,t =-5.114,4.266,P<0.05).(3) Multivariate analysis of affecting severity of hair loss after LSG:postoperative EWL and folic acid were independent factors affecting severity of hair loss after LSG (OR=1.039,0.499,95% confidence interval:1.011-1.068,0.300-0.802,P<0.05).A prediction accuracy of severity of hair loss after LSG was 85.2%.Conclusion Postoperative EWL and folic acid are independent factors affecting severity of hair loss after LSG.
7.Effect of self-management education based on nurse-leading on the quality of life in patients with chronic heart failure
Aixue YUE ; Liqin ZHU ; Jiaqian HE ; Ping XU ; Jia YOU ; Zhiying WANG ; Jianmei CHEN ; Jianpei PENG
Chinese Journal of Modern Nursing 2017;23(1):45-48
Objective To evaluate the effect of self-management education based on nurse-leading on the quality of life in patients with chronic heart failure (CHF).Methods Eighty patients with CHF in a tertiary hospital were enrolled in this study,and were divided into the intervention group and the control group randomly,with 40 cases in each group. Patients in the control group received conventional health education, while patients in the intervention group received the self-management education based on nurse-leading. The score of self-management behavior and the quality of life in two groups were evaluated and compared after six months.Results After six months,the score of self-management in the intervention group was (70.83±12.02), and was significantly higher than that in the control group (55.58±6.46) (t=9.675,P<0.05). The score of total quality of life and self-management in the intervention group was significantly higher than that in the control group (P<0.05).Conclusions Self-management education based on nurse-leading can improve the self-management and the quality of life of patients with CHF.
8.Combination antifungal therapy for invasive fungal disease in children with hematologic disease.
Kunyin QIU ; Lanlan DENG ; Ke HUANG ; Haixia GUO ; Jianpei FANG ; Honggui XU ; Hongman XUE ; Yang LI ; Chun CHEN ; Dunhua ZHOU
Chinese Journal of Hematology 2015;36(11):912-917
OBJECTIVETo evaluate antifungal combination strategy in children with hematologic diseases and invasive fungal disease( IFD).
METHODSA retrospective clinical study was performed based on 67 childhood patients with hematologic diseases and IFD who firstly accepted combination antifungal therapy for ≥ 7 days during January 2012 and December 2014. Of them, 11 cases received combination of echinocandin with azole, 10 cases received combination of echinocandin with amphotericin B, and 46 cases received combination of azole with amphotericin B.
RESULTSOverall response rate was 79.1%. Univariate analysis revealed that granulocyte recovery (P=0.031), status of underling disease (P=0.023) and the duration of the therapy (P=0.046) were significantly associated with efficacy. Multivariate analysis showed that the independent prognostic factor was the duration of combination antifungal therapy (OR=0.229, 95% CI 0.061- 0.863, P=0.029). The response rates of echinocandin combined with azole, echinocandin combined with amphotericin B and azole combined with amphotericin B were 81.8%, 60.0% and 82.6%, respectively (P>0.05), and 12-week survival rates were 81.8%, 80.0% and 86.5%, respectively (P>0.05). The drug- related adverse reactions occurred 59 times in 34 patients. BUN increasing, hypokalemia and abnormal liver functions were considered the main side effects.
CONCLUSIONFor IFD in children with hematologic disease, to extend the duration of treatment (≥ 14 days) could significantly improve the curative effect. Combinations of echinocandin with azole, echinocandin with amphotericin B and azole with amphotericin B can be used as a combination treatment options. Combination of Azole with amphotericin B is efficacious, safe and economic treatment option considering efficacy, survival rate, cost and dosage form.
Amphotericin B ; administration & dosage ; therapeutic use ; Antifungal Agents ; administration & dosage ; therapeutic use ; Child ; Drug Therapy, Combination ; Echinocandins ; administration & dosage ; therapeutic use ; Hematologic Diseases ; microbiology ; Humans ; Mycoses ; drug therapy ; Retrospective Studies ; Survival Rate ; Treatment Outcome
9.Liver and heart iron deposition status in patients with β thalassemia major: a multicenter study.
Changgang LI ; Sixi LIU ; Ying WANG ; Feiqiu WEN ; Hongying GAO ; Guangfu CHEN ; Chunfu LI ; Xuedong WU ; Jianpei FANG ; Wenge HAO ; Riyang LIU ; Xinhua ZHANG ; Chiuwing Winnie CHU ; WingYan AU
Chinese Journal of Pediatrics 2014;52(2):90-93
OBJECTIVETo observe the status of iron deposition in patient with β thalassemia major, and to formulate appropriate treatment strategies.
METHODThe data of status of transfusion and chelation in 135 patients aged from 6 years and 4 months to 17 years and 11 months with β thalassemia major were collected and analyzed. Serum ferritin levels were determined and cardiac and hepatic iron deposition was determined using MRI T2(*) technology.
RESULTOf the 135 cases studied, 66 were male, and 69 were female, their average age was 12.1 years. Serum ferritin (SF) was determined for 111 cases, it varied from 1 086.8 µg/L to 15 011.5 µg/L. Among them, 16 cases had SF level <2 000 µg/L (14.5%) , in 41 cases SF were between 2 000 and 4 000 µg/L (36.0%) ;in 54 cases SF >4 000 µg/L (48.7%) . Liver MRI T2(*) results showed that in only 8 cases (5.9%) iron content in the liver was in normal range, 19 cases (14.9%) showed mild liver iron deposition;34 (25.2%) moderate and 74 (54.8%, the youngest one was only 6 years and 4 months of age) had severe iron deposition respectively. Cardiac MRI T2(*) showed that in 89 cases (65.9%) iron content in the heart was in normal range;19 cases (14.1%) had mild cardiac iron deposition and 27 (20.0%) presented severe iron deposition (the youngest one was only 9 years and 3 months of age) . SF level was obviously related to liver and cardiac iron deposition (MRI T2(*)) r and P value were -0.284, 0.003 and -0.374, 0.000 respectively. In 108 cases regular transfusion and chelation were delayed due to financial problem. The late and insufficient dosage administered and irregular chelation caused the higher SF level and the severe iron deposition.
CONCLUSIONThe survival status of β thalassemia major in China is worrisome. Majority of them had not received regular transfusion and chelation. Liver and cardiac iron deposition occur early and had a high incidence.
Adolescent ; Child ; Female ; Ferritins ; blood ; Humans ; Iron ; metabolism ; Iron Chelating Agents ; adverse effects ; therapeutic use ; Iron Overload ; epidemiology ; etiology ; Liver ; metabolism ; Magnetic Resonance Imaging ; Male ; Myocardium ; metabolism ; Radiography ; Retrospective Studies ; Transfusion Reaction ; beta-Thalassemia ; diagnostic imaging ; metabolism ; therapy
10.Preoperative location of colorectal polyps in laparoscopic colectomy
Jianpei LIU ; Pinjie HUANG ; Tufeng CHEN ; Zonghen ZHENG ; Hongbo WEI
Chinese Journal of Digestive Surgery 2014;13(8):621-624
Objective To investigate the efficacies of preoperative location with titanium clip and methylene blue staining in laparoscopic colectomy.Methods The clinical data of 31 patients with colorectal polyps which could not be resected under endoscope were admitted to the Third Affiliated Hospital of Sun Yat-Sen University from August 2006 to September 2012 were retrospectively analyzed.According to the methods of preoperative location of colorectal polyps,all patients were divided into the titanium clip group (18 patients) and the methylene blue group (13 patients).Titanium clip group:enteroscopic and pathological examination were firstly performed,and then 1 or 2 titanium clips were placed at the superior and inferior part of the polyps.After enteroscopic examination,abdominal X ray examination was performed to detect the position of polyps according to the positions of the titanium clips.Methylene blue group:after colonoscopy,methylene blue of 1 mL was injected into the adjacent mucosa of the polyps,and 4 positions around the polyps were selected for the injection of methylene blue.If the 2 locating methods were failed,intraoperative enteroscopy was performed.Laparoscopic resection for intestine or mesenterium was performed according to the treatment principle of colorectal neoplasms,and the location efficacy of the 2 methods and the treatment of the 2 groups were analyzed.The measurement data and the count data were analyzed using the t test and chi-square test,respectively.Results The success rates of the titanium clip group and the methylene blue group were 15/18 and 8/13,with no significant difference between the 2 groups (x2=0.284,P >0.05).The polyps in 8 patients which were failed to be localized by titanium clip or methylene blue were localized by intraoperative enteroscopy,while the operation time was prolonged to (44 ± 13)minutes.No positioning errors were detected in all the 31 patients.Laparoscopic right colectomy was performed on 4 patients,laparoscopic left colectomy on 11 patients,laparoscopic sigmoid colectomy on 9 patients,laparoscopic anterior resection of rectum on 7 patients.No morbidity or mortality was detected in the 2 groups.The length of specimen,distance between the proximal margin and the superior margin of the tumor,distance between the distal margin and the inferior margin of the tumor (patients with colonic polyps),distance between the distal margin and the inferior margin of the tumor (patients with high rectal polyps),and the number of lymph node resected were (20 ± 7) cm,(11 ± 4) cm,(8.6 ± 3.1) cm,4.2 ± 1.1,8 ± 5 in the titanium clip group,and (20 ± 5) cm,(9 ± 3) cm,(9.1 ± 2.8) cm,4.6 ± 0.5,7 ± 6 in the methylene blue group,with no significant difference between the 2 groups (t =0.053,0.918,0.213,1.486,0.267,P >0.05).Fifteen patients had cancerization of the polyps,including 8 patients with TNM Ⅰ stage,6 with TNM Ⅱ] stage and 1 with TNM Ⅲ stage.Conclusions For patients with colorectal polyps located at the intestine above the descending colon,titanium clip locating is easy,safe and effective,and it could be the first choice for locating the colorectal polyps.Endoscopic methvlene blue staining is simple but high technique demanding.Intraoperative enteroscopy is precise for locating the polyps at the intestine below the descending colon.

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