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Objective:To evaluate the clinical efficacy of multi-disciplinary single center's CCCG-HB-2016 regimen in the treatment of hepatoblastoma (HB) in children.Methods:Clinical data of 36 HB patients treated with CCCG-HB-2016 program from Aug 2016 to March 2020 were analyzed.Results:These 36 patients included 20 boys and 16 girls. The serum AFP was all higher than 2 792 ng/ml,there was a correlation between AFP and tumor risk stratification ( H=14.973, P<0.05). Twenty eight cases (77.78%) were epithelial type and 8 cases (22.22%) were mixed epithelial mesenchymal type.All children were treated by tumor resection combined with chemotherapy, and there was a correlation between tumor risk stratification and surgical resection of liver lobe ( H=8.847, P<0.05). The probability of bone marrow suppression in the low-risk group was 58.33% (35/60),that in the intermediate-risk group was 73.49% (61/83) and in the high-risk group was 80.23% (69/86).All 36 cases were followed up to March 31, 2020,with an average follow-up of 21.9 months and the median survival was 22.5 months.The overall survival rate (OS) and event-free survival rate (EFS) were 97.2% and 83.3% respectively. Conclusions:The multidisciplinary CCCG-HB-2016 regimen was with a high success rate and along with a high incidence of bone marrow suppression.
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Objective@#To explore the new gene mutation and clinical manifestations of Caroli syndrome in 2 blood brother cases.@*Methods@#The clinical data of the 2 brothers with clinical diagnosis of Caroli syndrome were analyzed, and the related genes were detected by second-generation sequencing technology.@*Results@#The PKHD1 gene of both brothers had the mutation of c. 5059A>T(exon32). c. 4358(exon 32)delC shift mutation, neither of which has been reported in OMIM database.@*Conclusion@#After analysis, c. 4358(exon32) delC and c. 5059A>T(exon32) may be the new pathogenic mutation of this disease, with the same mutant genotype but completely different phenotype.
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Objective To explore the new gene mutation and clinical manifestations of Caroli syndrome in 2 blood brother cases.Methods The clinical data of the 2 brothers with clinical diagnosis of Caroli syndrome were analyzed,and the related genes were detected by second-generation sequencing technology.Results The PKHD1 gene of both brothers had the mutation of c.5059A ＞ T (exon32).c.4358 (exon 32)delC shift mutation,neither of which has been reported in OMIM database.Conclusion After analysis,c.4358 (exon32) delC and c.5059A ＞ T(exon32) may be the new pathogenic mutation of this disease,with the same mutant genotype but completely different phenotype.
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Objective To investigate the application value of ultrasound-guided fine-needle aspiration cytology of lymph nodes in the pre-radiotherapy evaluation of superficial lymph nodes metastases in patients with lung cancer and esophageal cancer. Methods A total of 115 patients with lung cancer and esophageal cancer prepared for thoracic radiotherapy from February 2017 to September 2018 in Cancer Hospital of Huanxing Chaoyang District Beijing were retrospectively analyzed. Ultrasound-guided fine-needle aspiration cytology examination was performed in 166 lymph nodes. Puncture tissues were prepared for cytology production. Then cytological specialist read the film to evaluate if the cancer cells metastasis were present after conventional papanicolaou staining. The effects of short diameter of lymph nodes, blood flow signal and tumor characteristics of patients on the results of cytological examination were analyzed. Results The ultrasound results showed the median short diameter of lymph nodes was 0.6 cm (0.2-3.5 cm), and 25 (15.1％) lymph nodes had blood flow signals, 50 (30.1％) lymph nodes had positive results of puncture tissues cytology. The positive rate of puncture tissues cytology was 10.0％(4/40), 21.6％(21/97) and 86.2％(25/29) in the shortdiameter of lymph nodes <0.5 cm group, ≥0.5 cm and <1 cm group, and ≥1 cm group, respectively (P<0.01). Cytology positive rate of lymph nodes with or without blood flow signal detected by ultrasound was 48.0％ (12/25) and 27.0％ (38/141), and there was a statistical difference (P = 0.034). Location of primary tumor, T stage and chemotherapy history had no significant influence on the cytological results in patients with lung cancer (all P> 0.05). Pathological types had a significant influence on cytological results (χ2= 8.050, P= 0.045). Lymph node metastasis of the upper mediastinum was a risk factor for lymph node metastasis in lower neck and supraclavicular region (χ2= 9.699, P= 0.002). Location of primary tumor, T stage and chemotherapy history had no significant influence on cytological results in patients with esophageal cancer (all P> 0.05). Conclusions Ultrasound-guided fine-needle aspiration cytology is safe and efficient. It can be used to evaluate the metastasis status of superficial lymph nodes in pre-radiotherapy patients with lung cancer and esophageal cancer.
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Objective To compare the effect of nutritional support with and without tube feeding on the incidence of adverse reactions in radiotherapy for esophageal cancer.Methods A total of 120 esophageal cancer patients with high-risk factors receiving radiotherapy were selected and randomly assigned into the tube feeding (TF,n=60) and non-tube feeding groups (NTF,n=60) according to the random number method.Nutritional interventions were performed during radiotherapy in both groups.The incidence of esophagitis and myelosuppressioa,aulmonary infectioa,autritional status and the completion of radiotherapy were observed and statistically compared between TF and NTF groups.Results In the TF groua,ahe incidence of ≥ grade 2 esophagitis was lower than that in the NTF group.The incidence of grade 3 esophagitis significantly differed between two groups (P＜ 0.05).The incidence of grade 1-2 myelosuppression did not differ between two groupa,ahereas the incidence of grade 3 myelosuppression in the TF group was significantly lower than that in the NTF group (P＜0.05).In the TF groua,ahe incidence of pulmonary infection was remarkably lower than that in the NTF group (P＜0.05).The changes of BMI and prealbumin in the TF group were better than those in the NTF group (both P＜0.05).One patient in the NTF group failed to complete the radiotherapy due to grade Ⅳ esophagitia,and 5 cases in the NTF group (P＜ 0.05).In the TF groua,ahe length of hospital stay was significantly shortened by 6.2 d on average (P＜ 0.05).Conclusions During radiotherapy for esophageal cancer patients with high-risk factora,autritional support with tube feeding can effectively reduce the incidence of adverse reactiona,amprove the completion rate of treatmena,and shorten the average length of hospital stay.
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Objective To explore the effect of jejunum tube implantation on the reduction of adverse reactions,outcomes improvement and cost reduction in esophageal cancer patients with radiotherapy. Methods Eighty-two esophageal cancer patients with nutritional risk in Beijing Chaoyang District Huanxing Cancer Hospital from August 2014 to December 2016 were analyzed. Forty-one cases received jejunum tube implantation (implantation group) and 41 cases received non-nutrient tube implantation (control group), and data on their nutritional status,clinical efficacy,adverse reactions,and hospitalization costs were collected and compared. Results Compared with the control group, the implantation group has higher body mass index [(21.7±0.5)kg/m2vs. (19.5±0.3) kg/m2,t =2.12,P =0.031], hemoglobin[(120.0±2.1) g/L vs. (115.0±6.3) g/L, t= 2.12, P= 0.031] and clinical efficacy [95.1 % (39/41) vs. 87.8 % (36/41), χ2= 6.11, P= 0.022]. The incidence of adverse reactions in implantation group was significantly lower than that in control group[29.3 % (12/41) vs. 51.2 %(21/41),χ 2=8.12,P=0.002). The total cost in implantation group was lower than that in control group (138 000 yuan vs. 147 000 yuan, t= 2.09, P= 0.037), and the average hospitalization day was significantly reduced (42.1 d vs. 48.4 d, t = 2.27, P = 0.029). Conclusion Enteral nutrition support with jejunal feeding tube can reduce the incidence of adverse reactions, improve the curative effect, shorten hospitalization time and save medical cost for patients with esophageal cancer.
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Objective To study the change and relationship among thrombopoietin (TPO), platelet parameter and megakaryocyte in immune vasculitis of weanling rabbits. The role of platelet parameter, megakaryocyte, thrombopoietin (TPO) in kawasaki disease (KD) was accessed. Methods An experimental model of weanling rabbits for KD was reproduced by bovine serum. The blood platelet counts (PLT), mean platelet volume (MPV), platelet distribution width (PDW), platelet hematocrit (PCT), megakaryocyte and TPO were determined ev-ery four days. Pathological analysis of coronary artery, liver, spleen, kidney, brain was also done. Results PLT, PCT, PDW and the per-centage of total number of megakaryocyte and middle board megakaryocyte in the experimental group had obviously changes at the 12th, 16th, 20th, 24th, 28th day (P <0.05). MPV in the experimental group had obviously changes in the 12th, 24th, 28th day(P <0.05). TPO in the experimental group was higher than that in control group (P < 0.05). TPO in both groups was positively correlated with PLT, PCT, the percentage of total number of megakaryocyte and middle board megakaryocyte (P < 0.05). In experimental group, small arteries showed obvious inflammatory injury at the 17th day, medium-sized arteries showed obvious inflammatory injury at the 28th day, but the aortas showed mild changes. Conclusion Platelet, megakaryocyte and TPO took part in the pathogenesis of KD, and they had interaction and co-ordination in the course of regulation, which suggested that they could be used to monitor the changes in the KD and guide the treatment.