1.Four Cases of Atypical Teratoid/Rhabdoid Tumor of Lateral Ventricles in Children
Jiaqi FENG ; Xinyao WANG ; Lei BAO ; Wenbin GUAN ; Yabing ZHOU ; Xiaoqiang WANG
Medical Journal of Peking Union Medical College Hospital 2024;15(3):655-660
Atypical teratoid/rhabdoid tumor (AT/RT) is a rare malignancy located primarily in infratentorial or subcortical areas with a poor prognosis, and rarely in the lateral ventricle with a very poor prognosis. So far, only 6 cases of AT/RT in lateral ventricle have been reported in China. This article reports the diagnosis and treatment of four children with AT/RT in the lateral ventricle, and discusses the clinical manifestations, differentiation and diagnosis, treatment and prognosis of the disease through literature review, in order to improve clinicians' understanding of the disease and reduce missed diagnosis and misdiagnosis.
2.Chinese expert consensus on the diagnosis and treatment of traumatic supraorbital fissure syndrome (version 2024)
Junyu WANG ; Hai JIN ; Danfeng ZHANG ; Rutong YU ; Mingkun YU ; Yijie MA ; Yue MA ; Ning WANG ; Chunhong WANG ; Chunhui WANG ; Qing WANG ; Xinyu WANG ; Xinjun WANG ; Hengli TIAN ; Xinhua TIAN ; Yijun BAO ; Hua FENG ; Wa DA ; Liquan LYU ; Haijun REN ; Jinfang LIU ; Guodong LIU ; Chunhui LIU ; Junwen GUAN ; Rongcai JIANG ; Yiming LI ; Lihong LI ; Zhenxing LI ; Jinglian LI ; Jun YANG ; Chaohua YANG ; Xiao BU ; Xuehai WU ; Li BIE ; Binghui QIU ; Yongming ZHANG ; Qingjiu ZHANG ; Bo ZHANG ; Xiangtong ZHANG ; Rongbin CHEN ; Chao LIN ; Hu JIN ; Weiming ZHENG ; Mingliang ZHAO ; Liang ZHAO ; Rong HU ; Jixin DUAN ; Jiemin YAO ; Hechun XIA ; Ye GU ; Tao QIAN ; Suokai QIAN ; Tao XU ; Guoyi GAO ; Xiaoping TANG ; Qibing HUANG ; Rong FU ; Jun KANG ; Guobiao LIANG ; Kaiwei HAN ; Zhenmin HAN ; Shuo HAN ; Jun PU ; Lijun HENG ; Junji WEI ; Lijun HOU
Chinese Journal of Trauma 2024;40(5):385-396
Traumatic supraorbital fissure syndrome (TSOFS) is a symptom complex caused by nerve entrapment in the supraorbital fissure after skull base trauma. If the compressed cranial nerve in the supraorbital fissure is not decompressed surgically, ptosis, diplopia and eye movement disorder may exist for a long time and seriously affect the patients′ quality of life. Since its overall incidence is not high, it is not familiarized with the majority of neurosurgeons and some TSOFS may be complicated with skull base vascular injury. If the supraorbital fissure surgery is performed without treatment of vascular injury, it may cause massive hemorrhage, and disability and even life-threatening in severe cases. At present, there is no consensus or guideline on the diagnosis and treatment of TSOFS that can be referred to both domestically and internationally. To improve the understanding of TSOFS among clinical physicians and establish standardized diagnosis and treatment plans, the Skull Base Trauma Group of the Neurorepair Professional Committee of the Chinese Medical Doctor Association, Neurotrauma Group of the Neurosurgery Branch of the Chinese Medical Association, Neurotrauma Group of the Traumatology Branch of the Chinese Medical Association, and Editorial Committee of Chinese Journal of Trauma organized relevant experts to formulate Chinese expert consensus on the diagnosis and treatment of traumatic supraorbital fissure syndrome ( version 2024) based on evidence of evidence-based medicine and clinical experience of diagnosis and treatment. This consensus puts forward 12 recommendations on the diagnosis, classification, treatment, efficacy evaluation and follow-up of TSOFS, aiming to provide references for neurosurgeons from hospitals of all levels to standardize the diagnosis and treatment of TSOFS.
3.Efficacy and safety of rituximab in the treatment of pediatric myasthenia gravis
Yunong TONG ; Cuijie WEI ; Xiaoling YANG ; Taoyun JI ; Yao ZHANG ; Ye WU ; Xingzhi CHANG ; Xinhua BAO ; Yuwu JIANG ; Hui XIONG ; Yuehua ZHANG
Chinese Journal of Pediatrics 2024;62(11):1050-1055
Objective:To evaluate the efficacy and safety of rituximab in pediatric myasthenia gravis (MG).Methods:Case series study. The clinical manifestations, laboratory tests, treatment plans and prognosis of 27 pediatric MG patients treated with rituximab from June 2013 to June 2023 at Children′s Medical Center of Peking University First Hospital were retrospectively collected.Results:There were 5 males and 22 females in 27 MG children. The onset age was 2.1 (1.6, 4.8) years, ranging from 8 months to 11 years. The clinical classification included 20 children (74%) of ocular MG and 7 children (26%) of generalized MG. Seventeen children (63%) had positive MG-related pathogenic antibodies, including 17 children of anti-AchR antibody and 1 of them also had anti-MuSK antibody. Rituximab was used as first-line immunosuppressant in 13 children, second-line immunosuppressant in 13 children and third-line immunosuppressant in 1 child. Immunosuppressants used before rituximab including 8 children of cyclosporine, 3 children of tacrolimus, 1 child of azathioprine, 1 child of mycophenolate mofetil and 1 child of cyclosporine combined with azathioprine. Rituximab was used for at least half a year with a follow-up period of more than 12 months. At the last follow-up after rituximab treatment, all children achieved improved or above, 14 children (52%) achieved complete stable remission, 7 children (26%) achieved pharmacologic remission, 1 child (4%) achieved minimal manifestations, and 5 children (18%) improved. After rituximab treatment, 27 children all could reduce the immunomodulation therapy and shorten the course of glucocorticoid therapy, and 22 children (81%) had stopped the glucocorticoid therapy. Among the 14 children with poor efficacy of other immunosuppressants, rituximab had complete stable remission of 7 children. The most common adverse reaction was respiratory infection (4 children (15%)). Only 2 children had allergic reaction to rituximab and got better after symptomatic treatment.Conclusions:Rituximab has good efficacy and tolerance in pediatric MG. Early application of rituximab can improve the prognosis and shorten the course of glucocorticoid treatment.
4.Association between remnant cholesterol levels and incident diabetic retinopathy in middle-aged and older patients with diabetes
Rong YU ; Xuhong HOU ; Xiangning WANG ; Lili JIA ; Xinhua DU ; Qiang WU ; Yuqian BAO ; Weiping JIA
Chinese Journal of Internal Medicine 2023;62(12):1430-1435
Objective:To investigate the association between remnant cholesterol (RC) and the risk of diabetic retinopathy (DR) in middle-aged and older individuals with diabetes.Methods:Based on the Shanghai Nicheng Cohort Study database, the data of 1 255 individuals with diabetes aged 55-70 years at baseline (2013-2014) with complete fundus photographs and serum cholesterol data in Nicheng, Shanghai, were analyzed. Multinomial logistic regression models were used to evaluate risk ratios ( RRs) and their 95% confidence intervals ( CIs) between baseline RC level and incident DR. Results:The median age of the subjects was 61.9 years, and 60.4% were women. After a 4.6-year follow-up, 79 (6.3%) patients developed DR, including 50 (4.0%) mild non-proliferative DR and 29 (2.3%) referable DR (RDR). Multivariable logistic regression showed that each mmol/L increase of RC was associated with a 40% higher risk of RDR ( RR=1.40, 95% CI 1.03-1.90). Compared with the lowest tertile of RC (<0.63 mmol/L), the risk of RDR in the highest tertile (≥0.85 mmol/L) increased by 4.59 times ( RR=5.59, 95% CI 1.51-20.73). Conclusion:The RC level may help identify individuals at high risk of incident RDR in middle-aged and older Chinese adults with diabetes.
5.Naoxintong Capsule for Secondary Prevention of Ischemic Stroke: A Multicenter, Randomized, and Placebo-Controlled Trial.
Xiao-Fei YU ; Xu-Ying ZHU ; Can-Xing YUAN ; Dan-Hong WU ; Yu-Wu ZHAO ; Jia-Jun YANG ; Chang-de WANG ; Wei-Wen WU ; Xue-Yuan LIU ; Zhen-Guo LIU ; Zhi-Yu NIE ; Ben-Qiang DENG ; Huan BAO ; Long-Xuan LI ; Chun-Yan WANG ; Hong-Zhi ZHANG ; Jing-Si ZHANG ; Ji-Han HUANG ; Fan GONG ; Ming-Zhe WANG ; Yong-Mei GUO ; Yan SUN ; Ding-Fang CAI
Chinese journal of integrative medicine 2022;28(12):1063-1071
OBJECTIVE:
To examine whether the combination of Naoxintong Capsule with standard care could further reduce the recurrence of ischemic stroke without increasing the risk of severe bleeding.
METHODS:
A total of 23 Chinese medical centers participated in this trial. Adult patients with a history of ischemic stroke were randomly assigned in a 1:1 ratio using a block design to receive either Naoxintong Capsule (1.2 g orally, twice a day) or placebo in addition to standard care. The primary endpoint was recurrence of ischemic stroke within 2 years. Secondary outcomes included myocardial infarction, death due to recurrent ischemic stroke, and all-cause mortality. The safety of drugs was monitored. Results were analyzed using the intention-to-treat principle.
RESULTS:
A total of 2,200 patients were enrolled from March 2015 to March 2016, of whom 143 and 158 in the Naoxintong and placebo groups were lost to follow-up, respectively. Compared with the placebo group, the recurrence rate of ischemic stroke within 2 years was significantly lower in the Naoxintong group [6.5% vs. 9.5%, hazard ratio (HR): 0.665, 95% confidence interval (CI): 0.492-0.899, P=0.008]. The two groups showed no significant differences in the secondary outcomes and safety, including rates of severe hemorrhage, cerebral hemorrhage and subarachnoid hemorrhage (P>0.05).
CONCLUSION
The combination of Naoxintong Capsule with standard care reduced the 2-year stroke recurrence rate in patients with ischemic stroke without increasing the risk of severe hemorrhage in high-risk patients. (Trial registration No. NCT02334969).
Adult
;
Humans
;
Secondary Prevention/methods*
;
Ischemic Stroke
;
Stroke/prevention & control*
;
Cerebral Hemorrhage/complications*
;
Double-Blind Method
;
Platelet Aggregation Inhibitors
6.Population Pharmacokinetics of Teicoplanin in Septic Patients from Intensive Care Unit
Zhi-jie HE ; Yao-wei ZHI ; Yan-pin GUAN ; Fang-yi LI ; Zi-jun ZOU ; Qiang BAO ; Xiao-xia YU
Journal of Sun Yat-sen University(Medical Sciences) 2022;43(6):1005-1011
ObjectiveTo establish a population pharmacokinetic (PPK) model of teicoplanin in septic patients from Intensive Care Unit (ICU) and to explore its pharmacokinetic characteristics and related covariates. MethodsThe study included 66 septic patients hospitalized in the department of critical care medicine from November 2017 to February 2020. After intravenous dosing of teicoplanin in septic patients, the trough concentration of teicoplanin was determined by high performance liquid chromatography (HPLC) and the concentration-time data was analyzed by non-linear mixed effect model. The pharmacokinetic parameters and residual errors were evaluated. The influence of covariates on model parameters was tested by forward addition and backward elimination. The predictive performance of the final model was assessed by internal validation. ResultsA two-compartment model best described the teicoplanin concentration-time data. The PPK parameter estimates were central clearance of 0.45 L/min, peripheral clearance of 0.72 L/min, central volume of distribution of 39.74 L and peripheral volume of distribution of 152.41 L. Creatinine, total protein, and lactate were found to significantly affect central clearance of teicoplanin (P<0.05). ConclusionThe two-compartment PPK model of teicoplanin established in this study could be used for individualized treatment in septic patients from ICU due to its good stability and high predictive accuracy.
7.Mid-term efficacy of China Net Childhood Lymphoma-mature B-cell lymphoma 2017 regimen in the treatment of pediatric Burkitt lymphoma.
Meng ZHANG ; Pan WU ; Yan Long DUAN ; Ling JIN ; Jing YANG ; Shuang HUANG ; Ying LIU ; Bo HU ; Xiao Wen ZHAI ; Hong Sheng WANG ; Yang FU ; Fu LI ; Xiao Mei YANG ; An Sheng LIU ; Shuang QIN ; Xiao Jun YUAN ; Yu Shuang DONG ; Wei LIU ; Jian Wen ZHOU ; Le Ping ZHANG ; Yue Ping JIA ; Jian WANG ; Li Jun QU ; Yun Peng DAI ; Guo Tao GUAN ; Li Rong SUN ; Jian JIANG ; Rong LIU ; Run Ming JIN ; Zhu Jun WANG ; Xi Ge WANG ; Bao Xi ZHANG ; Kai Lan CHEN ; Shu Quan ZHUANG ; Jing ZHANG ; Chun Ju ZHOU ; Zi Fen GAO ; Min Cui ZHENG ; Yonghong ZHANG
Chinese Journal of Pediatrics 2022;60(10):1011-1018
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
8.Expert consensus on rational usage of nebulization treatment on childhood respiratory system diseases.
Han Min LIU ; Zhou FU ; Xiao Bo ZHANG ; Hai Lin ZHANG ; Yi Xiao BAO ; Xing Dong WU ; Yun Xiao SHANG ; De Yu ZHAO ; Shun Ying ZHAO ; Jian Hua ZHANG ; Zhi Min CHEN ; En Mei LIU ; Li DENG ; Chuan He LIU ; Li XIANG ; Ling CAO ; Ying Xue ZOU ; Bao Ping XU ; Xiao Yan DONG ; Yong YIN ; Chuang Li HAO ; Jian Guo HONG
Chinese Journal of Pediatrics 2022;60(4):283-290
9.Hepatopancreatoduodenectomy for advanced biliary malignancies
Xiangsong WU ; Maolan LI ; Wenguang WU ; Xu’an WANG ; Huaifeng LI ; Runfa BAO ; Yijun SHU ; Jun SHEN ; Jun GU ; Xuefeng WANG ; Wei GONG ; Shuyou PENG ; Yingbin LIU
Chinese Medical Journal 2022;135(23):2851-2858
Background::Hepatopancreatoduodenectomy (HPD) has been considered the only curative treatment for metastatic cholangiocarcinoma and some locally advanced gallbladder cancers (GBCs). However, HPD has not yet been included in treatment guidelines as a standard surgical procedure in consideration of its morbidity and mortality rates. The aim of this study was to evaluate the safety and effectiveness of HPD in treating biliary malignancies.Methods::The medical records of 57 patients with advanced biliary cancer undergoing HPD from January 2009 to December 2019 were retrospectively retrieved. A case-control analysis was conducted at our department. Patients with advanced GBC who underwent HPD (HPD-GBC group) were compared with a control group (None-HPD-GBC group). Baseline characteristics, preoperative treatments, tumor pathologic features, operative results, and prognosis were assessed.Results::Thirteen patients with cholangiocarcinoma and 44 patients with GBC underwent HPD at our department. Significant postoperative complications (grade III or greater) and postoperative pancreatic fistula were observed in 24 (42.1%) and 15 (26.3%) patients, respectively. One postoperative death occurred in the present study. Overall survival (OS) was longer in patients with advanced cholangiocarcinoma than in those with GBC (median survival time [MST], 31 months vs. 11 months; P < 0.001). In the subgroup analysis of patients with advanced GBC, multivariate analysis demonstrated that T4 stage tumors ( P = 0.012), N2 tumors ( P = 0.001), and positive margin status ( P = 0.004) were independently associated with poorer OS. Patients with either one or more prognostic factors exhibited a shorter MST than patients without those prognostic factors ( P < 0.001). Conclusion::HPD could be performed with a relatively low mortality rate and an acceptable morbidity rate in an experienced high-volume center. For patients with advanced GBC without an N2 or T4 tumor, HPD can be a preferable treatment option.
10.The effectiveness of left atrial appendage occlusion during off-pump coronary artery bypass grafting in elderly patients with coronary artery disease and atrial fibrillation: A retrospective cohort study
Zhaolei JIANG ; Min TANG ; Ju MEI ; Hao LIU ; Nan MA ; Saie SHEN ; Chunrong BAO ; Fangbao DING
Chinese Journal of Clinical Thoracic and Cardiovascular Surgery 2021;28(02):186-190
Objective To investigate the technique and efficacy of left atrial appendage (LAA) occlusion during off-pump coronary artery bypass grafting (OPCABG) in elderly patients with coronary artery disease (CAD) and atrial fibrillation (AF). Methods From 2013 to 2018, 84 elderly patients with CAD and AF with reduced left ventricular ejection fraction (LVEF< 50%) underwent OPCABG in our department. There were 54 males and 30 females at age of 70-82 years. They were divided into a left atrial appendage (LAA) occlusion group (n=56) and a non-LAA occlusion group (n=28). Postoperative antithrombotic therapy: the LAA occlusion group was given warfarin + aspirin + clopidogrel “triple antithrombotic therapy” for 3 months after operation, then was changed to aspirin + clopidogrel “dual antiplatelet” for long-term antithrombotic; the non-LAA occlusion group was given warfarin + aspirin + clopidogrel “triple antithrombotic” for long-term antithrombotic after operation. The clinical effectiveness of the two groups was compared. Results All patients underwent the surgery successfully. There were 56 patients in the LAA occlusion group, including 44 patients of LAA exclusion and 12 patients of LAA clip. The time of LAA occlusion was 3 to 8 minutes. There was no injury of graft vessels and anastomotic stoma. Early postoperative death occurred in 2 patients (2.4%). There was no statistical difference between the two groups in postoperative hospital stay (P=0.115). Postoperative LVEF of the two groups significantly improved compared with that before operation (P<0.05). There was no stroke or bleeding in important organs during hospitalization. During follow-up of 1 year, no cerebral infarction occurred in both groups, but the incidence of bleeding related complications in the LAA occlusion group was significantly lower than that in the non-LAA occlusion group (3.6% vs. 18.5%, P=0.036). Conclusion For elderly patients with CAD and AF with reduced LVEF, LAA occlusion during OPCABG can effectively reduce the risk of stroke and bleeding related complications, and without increasing the risk of surgery.

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