Objective: To summarize the clinical features, management and outcome of superior vena cava syndrome (SVCS) associated with mediastinal malignancy in children. Methods: Clinical data of 42 children of SVSC associated with mediastinal malignancy in Shanghai Children's Medical Center from January 2015 to December 2021 were collected and analyzed retrospectively. The clinical manifestations, pathological diagnosis, disease diagnosis process, and prognosis were summarized. Results: Among 42 children of SVCS associated with mediastinal malignancy, there were 31 males and 11 females. The age at diagnosis was 8.5 (1.9, 14.9) years. Cough and wheezing (33 cases, 79%), orthopnea (19 cases, 45%) and facial edema (18 cases, 43%) occurred most commonly. T-cell lymphoblastic lymphoma (T-LBL) was the most frequent pathological diagnosis (25 cases, 60%), followed by T-cell acute lymphoblastic leukemia (T-ALL) (7 cases, 17%), anaplastic large cell lymphoma (4 cases, 10%) and diffuse large B-cell lymphoma (2 cases, 5%), peripheral T-lymphoma, Hodgkin lymphoma, Ewing's sarcoma and germ cell tumor (1 case each). Pathological diagnosis was confirmed by bone marrow aspiration or thoracentesis in 14 cases, peripheral lymph node biopsy in 6 cases, and mediastinal biopsy in 22 cases. Twenty-seven cases (64%) had local anesthesia. Respiratory complications due to mediastinal mass developed in 3 of 15 cases who received general anesthesia. Of the 42 cases, 27 cases had sustained remission, 1 case survived with second-line therapy after recurrence, and 14 cases died (2 cases died of perioperative complications and 12 cases died of recurrence or progression of primary disease). The follow-up time was 36.7 (1.2, 76.1) months for 27 cases in continuous complete remission. The 3-year overall survival (OS) and events free survival (EFS) rates of 42 children were 59% (95%CI 44%-79%) and 58% (95%CI 44%-77%) respectively. Conclusions: SVCS associated with mediastinal malignancy in children is a life-threatening tumor emergency with high mortality. The most common primary disease is T-LBL. The most common clinical symptoms and signs are cough, wheezing, orthopnea and facial edema. Clinical management should be based on the premise of stable critical condition and confirm the pathological diagnosis through minimal invasive operation.
Child ; China ; Cough ; Edema ; Female ; Humans ; Male ; Mediastinal Neoplasms/diagnosis* ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; Respiratory Sounds ; Retrospective Studies ; Superior Vena Cava Syndrome/therapy*

Objective To explore the diagnostic value of the quantitative detection of serum NF-κB and TNF-α in children with Mycoplasma pneumoniae Pneumonia(MPP).Methods 150 MPP patients were enrolled in this study including 75 with wheeze and 75 without wheeze,and then collected the blood specimens.75 healthy children were recruited to the control group (NC group).The levels of NF-κB and TNF-α in different groups were detected by qRT-PCR.The performance of NF-κB and TNF-α were evaluated by receiver operating characteristic curves (ROC) and the area under the curve (AUC) (95 ％ CI).Results The relative expression of NF-κB and TNF-α in MPP children with wheeze were 3.09±0.15 and 2.14±0.11,respectively.They were significantly higher than NC group (t=11.03,6.987,P＜0.05).NF-κB and TNF-α in MPP children without wheeze were also significantly higher than control group (t =6.873,7.406,P＜ 0.05).The relative expression of NF-κB and TNF-α in children with wheeze were significantly higher than children without wheeze.The detection of NF-κB yielded an area under the curve of ROC of 0.826 5 (95％CI:0.772 8～0.880 2) in discriminating MPP patients from healthy group.The TNF-α was 0.818 3 (95％ CI:0.765 1～0.871 6) in discriminating MPP patients from healthy group.Conclusion The relative expression of NF-κB and TNF-α in children with MPP was significantly higher than that in NC group,NF-κB and TNF-a may prove to be non-invasive biomarkers for the auxiliary diagnosis of MPP.

OBJECTIVETo evaluate the clinical efficacy of Jianpi Liqi Yiliu Formula (JLYF) combined with cytokine-induced killer (CIK) cells for treating patients with advanced hepatocellular carcinoma (HCC).

METHODSBetween January 2011 and January 2014, 60 advanced HCC patients were enrolled in this study, who were assigned to the treatment group and the control group according to their willingness for taking JLYF, 30 cases in each group. All patients received CIK cell treatment: 1 x 10⁹-3 x 10⁹ each time, by intravenous dripping from the 1st day to the 3rd day, once per day. Besides, patients in the treatment group took JLYF decoction, while those in the control group took Chinese medical decoction by syndrome typing. All patients received treatment of at least two cycles. The time to progression (TTP) , overall survival (OS), disease control rate (DCR), performance status scale (PS), Child-Pugh scale, and adverse reactions were observed, and subgroup analyzed.

RESULTSTo May 31, 2014, all patients reached the clinical endpoint. TTP was 3.5 months (95% Cl: 3.30-4.10) in the treatment group, better than that (2.5 months, 95% CI: 2.32-2.68) of the control group (P < 0.05). DCR was 36.7% in the treatment group and 30.0% in the control group (P > 0.05). OS was 5.2 months (95% CI: 4.53-5.87) in the treatment group and 4.6 months (95% CI: 4.06-5.14) in the control group (P > 0.05). The PS scale was 1.60 ± 0.10 after treatment, lower than that (1.80 ± 0.09) before treatment in the treatment group (P < 0.05). When the PS scale was 0-2 or Child-Pugh scale was class A, TTP was longer in the treatment group than in the control group (P < 0.05). No adverse reaction occurred in the two groups during the treatment course.

CONCLUSIONSThe combination of JLYF with ClK cell treatment could prolong advanced HCC patients' TTP, improve PS scale, as compared with syndrome typed Chinese medical decoction treatment group. Besides, when the PS scale was 0-2 or Child-Pugh scale was class A, it was a better treatment program for advanced HCC patients.

Carcinoma, Hepatocellular ; therapy ; Cell- and Tissue-Based Therapy ; Cytokine-Induced Killer Cells ; cytology ; Disease Progression ; Drugs, Chinese Herbal ; therapeutic use ; Humans ; Liver Neoplasms ; therapy

Objective To know the plasma lipids level and influencing factors among adults in Tongxiang city,and to provide evidence for prevention and intervention of dyslipidemia.Methods Multistage stratified cluster sampling method was conducted to select participants in Tongxiang city.Questionnaire interview,physical examination and blood lipid detection were performed among the selected subjects.Models of the univariate and multivariate unconditional logistic regressions were used to analyze the influencing factors of dyslipidemia.Results A total of 600 people were involved in this study.The prevalence and standardized rates of dyslipidemia were 36.33% and 36.11 %,and abnormal rates of HDL -C,TG,TC and LDL -C were 27.33%,15.67%,4.83% and 4.83%,which standardized were 29.17%,15.1 3%,3.1 8% and 3.12%, respectively.The abnormal rate of LDL -C in females was higher than that in males (P <0.05).Borderline abnormal rate of TC and dyslipidemia rates were increased with age (P <0.05),and the abnormal rate of HDL -C were decreased with age (P <0.05).Multivariate logistic regression analysis showed that BMI (OR =3.398),diabetes mellitus(OR =1.694), hypertension (OR =1.717)and total static time(OR =1.712)were the risk factors for dyslipidemia.Conclusion The prevalence of dyslipidemia was high among the adults in Tongxiang city,especially the abnormity of HDL -C and TG. Community comprehensive prevention and control of dyslipidemia should be carried out according to its risk factors.

Objective To exam the influence of psychological factors,occupational factors and dietary habits on malignant tumors.Methods A total of 2 471 patients with malignant tumor were selected from 2010 to 2012 and 1 to 1 matching method was used controlling sex and age distribution in the case and control groups.Data collected by interviewing was analyzed by univariate and multivariate conditional logistic regression.Results 12 related factors including 6 psychological factors were found in relation to malignant tumor after univariate logistic regression analysis.The multivariate logistic regression analysis showed that harmful occupational history (OR =1.731,95%CI:1.337 ~2.242),often having fried food (OR =1.889,95%CI:1.398 ~2.551),heavy salty diet(OR =1.457,95%CI:1.169 ~1.818),psychological problems (OR =1.274,95%CI:1.003 ~1.617),poor marital life (OR =2.007,95%CI:1.111 ~3.623)and lack of self -regulation (OR =1.460,95%CI:1.189 ~1.793 )were the risk factors.Conclusion Malignant tumor was related to a variety of exposure factors and psychological factor has become an important risk factor for malignant tumor in Tongxiang city.

OBJECTIVETo analyze clinical and cytogenetic features of hematological disorders associated with 20q- and t (20;21) (q11;q11) abnormalities.

METHODSFollowing short-term culture of bone marrow cells, karyotypic analysis was carried out with R-banding. 20q- and t(20;21) (q11;q11) was detected by fluorescence in situ hybridization (FISH) using dual-color 20q11/12 probe, ST 20qter /ST 21qter probes, SE20(D20Z1)/SE 13/21 probes, and WC20/WC21 probes.

RESULTSSix (2.3%) of the 257 patients with 20q- detected by conventional karyotypic analysis were found to have t(20;21) (q11;q11) abnormality. Five cases had myelodysplastic syndrome, 1 had acute lymphoblastic leukemia. Above results were all confirmed by FISH.

CONCLUSIONi (20q-), t(20;21) (q11;q11) seems to be a rare but recurrent chromosomal abnormality which is specifically associated with myeloid disease, late occurrence and poor prognosis. The translocation between chromosome 20q11 and 21q11 may form a novel fusion gene which has an important role in the pathogenesis of the disease.

Aged ; Chromosome Deletion ; Chromosomes, Human, Pair 20 ; Chromosomes, Human, Pair 21 ; Female ; Humans ; In Situ Hybridization, Fluorescence ; Male ; Middle Aged ; Myelodysplastic Syndromes ; genetics ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; genetics ; Translocation, Genetic

OBJECTIVETo explore the distribution of Chinese medicine (CM) syndrome types in primary liver cancer (PLC) and their differences of the survival time.

METHODSFrom May 2007 to March 2009, recruited were 151 PLC inpatients at Department of Tumor, Guangdong Provincial Hospital of Traditional Chinese Medicine. Their survival time were statistically calculated. Patients' average survival time and median survival time were calculated using Kaplan-Meier method. The Log-rank test was used to analyze their differences of survival time among different CM syndrome types.

RESULTSThe proportion of CM syndrome types in PLC patients were ranked from high to low as follows: mutual accumulation of dampness and blood stasis syndrome [MADBSS, 43.0% (65/151)], Gan-stagnation Pi-deficiency syndrome [GSPDS, 34.4% (52/151)], qi stagnation blood stasis syndrome [QSBSS, 9.3% (14/151)], retention of damp-heat syndrome [RDHS, 8.6%(13/151)], and Gan-Shen yin deficiency syndrome [GSYDS, 4.6% (7/ 151)]. The median survival time of different CM syndrome types were ranked from longer to shorter as follows: GSPDS (14.77 months), QSBSS (6.13 months), RDHS (5.27 months), MADBSS (4.78 months), and GSYDS (0.80 months). The mean survival times were ranked from longer to shorter as follows: GSPDS (12.40 months), QSBSS (8.84 months), MADBSS (6.99 months), RDHS (7.08 months), and GSYDS (0.72 months). There was statistical difference in the difference of the survival time among different CM syndrome types (P < 0.05).

CONCLUSIONSGSPDS and MADBSS were the most common CM syndrome types in PLC patients. There was difference in the survival time between GSPDS and MADBSS/between RDHS and GSYDS. There was difference in the survival time between MADBSS and GSYDS. Patients of GSPDS might get the best prognosis, while patients of GSYDS might get the poorest prognosis.

Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Liver Neoplasms ; diagnosis ; mortality ; pathology ; Male ; Medicine, Chinese Traditional ; Middle Aged ; Neoplasm Staging ; Prognosis ; Retrospective Studies ; Survival Rate ; Yang Deficiency ; Yin Deficiency

OBJECTIVETo explore the morphology, immunophenotype, cytogenetics and clinical features of TCF3-PBX1 fusion gene positive adult acute lymphoblastic leukemia (ALL).

METHODSR banding was used to analyze conventional cytogenetics (CC), interphase fluorescence in situ hybridization (iFISH) and RT-PCR to detect the TCF3-PBX1 fusion gene, and flow cytometry to immunophenotype. The clinical and laboratory features and long-term follow-up of the patients were analyzed.

RESULTSThe incidence of 19 TCF3-PBX1-positive adult ALL was 3.13% of total ALL patients. Of them, 12 and 7 cases were diagnosed as L(1) and L(2) morphology respectively; 7 cases with balanced translocation of chromosome 1 and 19; 10 with der(19) t(1;19) formed from unbalanced translocation and 2 with normal karyotypes. TCF3-PBX1 fusion gene was detected by RT-PCR in 9 cases, and by iFISH in 17. 16 cases were B-phenotype and the other 2 T-phenotype; 17 cases had lymph node, spleen or liver infiltration. Of 18 patients received chemotherapy, 17 (94.7%) achieved complete remission (CR); the median relapse-free survival (RFS) and median overall survival was 3.2 months and 7.2 months, respectively.

CONCLUSIONSTCF3-PBX1-positive adult ALL had unique clinical and pathological features with high remission rate, high relapse rate and short survival time and should be considered to receive intensified treatment strategies. iFISH combined with CC and RT-PCR can increase the detection rate of t(1;19)/TCF3-PBX1 fusion gene.

Adult ; Chromosomes, Human, Pair 1 ; Humans ; In Situ Hybridization, Fluorescence ; Oncogene Proteins, Fusion ; genetics ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; genetics ; Translocation, Genetic

OBJECTIVETo examine whether the polymorphisms of endothelial nitric oxide synthase (eNOS) gene are associated with the susceptibility to high altitude pulmonary edema (HAPE) in Chinese railway construction workers at Qinghai-Tibet where the altitude is over 4 500 m above sea level.

METHODSA case-control study was conducted including 149 HAPE patients in the construction workers and 160 healthy controls randomly recruited from their co-workers, matching the patients in ethnicity, age, sex, lifestyle, and working conditions. Three polymorphisms of eNOS gene, T-786C in promoter, 894G/T in exon 7, and 27bp variable number tandem repeat (VNTR) in intron 4, were genotyped using polymerase chain reaction (PCR) and confirmed with DNA sequencing.

RESULTSThe frequencies of 894T allele and heterozygous G/T of the 894G/T variant were significantly higher in HAPE patients group than in the control group (P=0.0028 and P=0.0047, respectively). However, the frequencies of the T-786C in promoter and the 27bp VNTR in intron 4 were not significantly different between the two groups. Haplotypic analysis revealed that the frequencies of two haplotypes (H3,T-T-b, b indicates 5 repeats of 27 bp VNTR; H6, C-G-a, a indicates 4 repeats of 27 bp VNTR) were significantly higher in HAPE patients (both Pü0.0001). On the contrary, the frequencies of H1 (T-G-b) and H2 (T-G-a) were lower in HAPE patients than in healthy controls (both Pü0.001).

CONCLUSIONSTwo haplotypes (T-T-b and C-G-a) may be strongly associated with susceptibility to HAPE. Compared with the individual alleles of eNOS gene, the interaction of multiple genetic markers within a haplotype may be a major determinant for the susceptibility to HAPE.

Adolescent ; Adult ; Altitude ; Base Sequence ; Case-Control Studies ; DNA Primers ; Genotype ; Haplotypes ; Humans ; Male ; Middle Aged ; Nitric Oxide ; blood ; Nitric Oxide Synthase Type III ; genetics ; Occupational Diseases ; enzymology ; genetics ; Polymorphism, Genetic ; Pulmonary Edema ; enzymology ; genetics ; Tibet ; Young Adult

OBJECTIVETo develop a rapid and simple multiplex polymerase chain reaction (PCR) method which discriminates extended-spectrum beta-lactamases (ESBLs) genes in sporadic Shigella isolates from 1998 to 2007 in Hangzhou city, China.

METHODSAfter ESBLs screening according to the Clinical and Laboratory Standards Institute (CLSI) method, CTX-M, TEM, SHV and OXA-1 encoding genes were detected by using a multiplex PCR method, and the results were verified by 8 single gene PCR amplification.

RESULTSSeventeen isolates harbored ESBLs genes among 195 Shigella isolates (8.72%). Genes encoding CTX-M (17 strains), TEM (2 strains), OXA-1 (10 strains) and SHV (0 strains) were discriminated with multiplex PCR analysis, which coincided with eight single gene PCR analysis at 94.12%.

CONCLUSIONMultiplex PCR should be a suitable tool for initial rapid screening and discriminating ESBLs genes in Shigella isolates. With similar trend of national surveillance data, the proportion of sporadic Shigella isolates harbouring ESBLs genes might probably be on increase.

DNA, Bacterial ; analysis ; Genes, Bacterial ; Genotype ; Humans ; Microbial Sensitivity Tests ; Polymerase Chain Reaction ; methods ; Shigella ; genetics ; isolation & purification ; beta-Lactamases ; genetics