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High-ß_{θe} (a ratio of the electron thermal pressure to the poloidal magnetic pressure) steady-state long-pulse plasmas with steep central electron temperature gradient are achieved in the Experimental Advanced Superconducting Tokamak. An intrinsic current is observed to be modulated by turbulence driven by the electron temperature gradient. This turbulent current is generated in the countercurrent direction and can reach a maximum ratio of 25% of the bootstrap current. Gyrokinetic simulations and experimental observations indicate that the turbulence is the electron temperature gradient mode (ETG). The dominant mechanism for the turbulent current generation is due to the divergence of ETG-driven residual flux of current. Good agreement has been found between experiments and theory for the critical value of the electron temperature gradient triggering ETG and for the level of the turbulent current. The maximum values of turbulent current and electron temperature gradient lead to the destabilization of an m/n=1/1 kink mode, which by counteraction reduces the turbulence level (m and n are the poloidal and toroidal mode number, respectively). These observations suggest that the self-regulation system including turbulence, turbulent current, and kink mode is a contributing mechanism for sustaining the steady-state long-pulse high-ß_{θe} regime.
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Objective: To investigate the efficacy and safety of ruxolitinib, liposomal doxorubicin, etoposide, methylprednisolone+/-PEG-asparaginase (RU-DEP+/-L) in the treatment of relapsed/refractory (R/R) pediatric hemophagocytic lymphohistiocytosis (HLH). Methods: The clinical data of R/R pediatric HLH, who accepted the RU-DEP+/-L regimen at Beijing Children's Hospital from January 2018 to December 2019 was retrospectively analyzed. Results: A total of 16 patients were included in this study, including 13 males and 3 females, aged[M(Q1,Q3)] 1 (1, 2) years at diagnosis. Thirteen patients were diagnosed with Epstein-Barr virus (EBV)-HLH, 2 with EBV-induced primary HLH, and 1 with unclear etiology, among which 3 patients were co-infected with CMV. After the first-line treatment, 11 patients had no response, and 5 patients relapsed after complete response. Nine patients received the RU-L-DEP regimen, and 7 patients received the RU-DEP regimen. The overall response rate and complete response of RU-DEP+/-L treatment were 10/16 and 3/16, respectively. The negative conversion rate of plasma EBV-DNA was 7/15. The median follow-up time was 35.1 (2.4, 40.7) months, and 9/16 patients were survival. The 3-year overall survival rate after RU-DEP+/-L treatment in response and accepted hematopoietic stem cell transplantation (HSCT) was higher than that without response and did not receive HSCT (P=0.048). Among the 16 patients, 9 had varying degrees of myelosuppression, and 13 had an infection. Conclusions: RU-DEP+/-L can be used as a salvage treatment in R/R pediatric HLH, which can provide a bridge to HSCT and play an important role in the control of HLH. The main adverse reactions are myelosuppression and infection, which can be tolerated.
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Infecções por Vírus Epstein-Barr , Linfo-Histiocitose Hemofagocítica , Idoso , Asparaginase , Criança , Doxorrubicina/análogos & derivados , Etoposídeo/uso terapêutico , Feminino , Herpesvirus Humano 4 , Humanos , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Linfo-Histiocitose Hemofagocítica/etiologia , Masculino , Metilprednisolona/uso terapêutico , Nitrilas , Polietilenoglicóis , Pirazóis , Pirimidinas , Estudos RetrospectivosRESUMO
Objective: To investigate the association between metabolically healthy obesity and the incident risk of stroke in people aged ≥40 years from rural areas of Henan Province. Methods: During 2007 to 2008, 20 194 residents aged ≥18 years were selected for baseline examination by random cluster sampling and 17 265 participants were followed up during 2013 to 2014. According to the aim of current study, a total of 11 864 eligible subjects were included in this post-hoc analysis. Depending on body mass index and metabolic status, subjects were divided into four groups: metabolically healthy normal weight, metabolically healthy obesity, metabolically abnormal normal weight and metabolically abnormal obesity. Multivariate logistic regression model was used to analyze the relationship between metabolically healthy obesity and the risk of stroke. Results: The median (Q1, Q3) age of study participants was 54(46, 61) years, and 4 526 participants were men. During the mean follow-up of 6 years, the cumulative incidence of stroke was 7.16%. The incidence of stroke in metabolically healthy normal weight, metabolically healthy obesity, metabolically abnormal normal weight, and metabolically abnormal obesity were 3.73%, 4.61%, 8.99% and 9.38%, respectively (χ²=117.458, P<0.001). After adjusting possible confounding factors, compared with metabolically healthy normal weight, the risk of stroke was significantly increased in the metabolically healthy obesity group, metabolically abnormal normal weight group and metabolically abnormal obesity group with the odds ratio (OR) and 95% confidence interval (CI) of 1.52(1.10-2.12), 2.11(1.61-2.77) and 2.78(2.18-3.55), respectively. Stratified analysis showed that the risk of stroke was significantly higher in metabolically healthy obesity people aged 40-59 years compared with metabolically healthy normal weight group (OR=2.12, 95%CI: 1.36-3.30). Conclusion: Metabolically healthy obesity, metabolically abnormal normal weight and metabolically abnormal obesity are positively associated with the risk of stroke.
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Obesidade Metabolicamente Benigna , Acidente Vascular Cerebral , Adolescente , Adulto , Índice de Massa Corporal , Humanos , Masculino , Pessoa de Meia-Idade , Obesidade/complicações , Obesidade Metabolicamente Benigna/complicações , Obesidade Metabolicamente Benigna/diagnóstico , Obesidade Metabolicamente Benigna/epidemiologia , Fatores de Risco , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/epidemiologiaRESUMO
Metabolism in most organisms can show variations between the day and night. These variations may also affect the composition of products derived from livestock. The aim of the present study was to investigate the difference in composition between the day milk and night milk of dairy cows. Ten multiparous Holstein cows (milk yield = 25.2 ± 5.00 kg/d) were randomly selected during mid lactation. Milk samples were collected at 0500 h ("night milk") and 1500 h ("day milk") and analyzed to determine their composition. Mid-infrared spectroscopy was used to analyze macronutrient content of milk. Metabolomics and lipidomics were used to detect and analyze small molecules and fatty acids, respectively. An automatic biochemical analyzer and ELISA kits were used to determine biochemical indicators, as well as antioxidant and immune parameters in the milk. Though milk fat, protein, lactose, and total milk solids were not different between day milk and night milk, small molecules, metabolites and lipids, and hormones and cytokines differed between day milk and night milk. Regarding biochemical and immune-related indicators, the concentrations of malondialdehyde, HSP70, and HSP90 in night milk were lower than that in day milk. However, interferon-γ levels were higher in night milk. Additionally, night milk was naturally rich in melatonin. Lipidomics analyses showed that the levels of some lipids in night milk were higher than those in day milk. Metabolomics analyses identified 36 different metabolites between day milk and night milk. Higher concentrations of N-acetyl-d-glucosamine, cis-aconitate, and d-sorbitol were observed in day milk. However, the other 33 metabolites analyzed, including carbohydrates, lipids, AA, and aromatic compounds, showed lower concentrations in day milk than in night milk. The present findings show that the composition of night milk differs considerably from that of day milk. Notable changes in the circadian rhythm also altered milk composition. These results provide evidence to support the strategic use and classification of day milk and night milk.
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Ritmo Circadiano , Leite , Animais , Bovinos , Dieta/veterinária , Ácidos Graxos , Feminino , Lactação , LactoseRESUMO
We present the case of a 16-year-old boy presented with 2-week episode of wor-sening lower abdominal pain. Clinically, there was a tender palpable mass on the suprapubic region. Ultrasonography showed an absent spleen at its usual area, instead suprapubic mass suggestive of ectopic spleen was identified. An emer-gency laparotomy revealed a congested spleen in the pelvic cavity. Splenectomy was undertaken as it was non-viable. The patient was discharged uneventfully with triple vaccinations. We describe this unique entity with its literature review. (Folia Morphol 2018; 77, 2: 400-402).
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Abdome Agudo , Esplenectomia , Baço Flutuante , Abdome Agudo/diagnóstico , Abdome Agudo/cirurgia , Adolescente , Feminino , Humanos , Masculino , Baço Flutuante/diagnóstico , Baço Flutuante/cirurgiaRESUMO
Objective: To evalute the clinical outcomes of intracytoplasmic sperm injection(ICSI) for infertility male with severe asthenospermia induced by multiple morphological anomalies of the flagella (MMAF). Methods: The clinical data of 15 patient with MMAF were retrospectively analyzed, who underwent ICSI treatment using hyponotic swelling test the "live" sperm in the Department of Reproductive Medicine of Yantai Yuhuangding Hospital from January 2011 to December 2016. Another 30 obstructive azoospermia (OA)patients are matched strictly who also accepted ICSI in the same treatment time. The two groups were compared in the couples'age, the body mass index(BMI), the duration of infertility, the retrieved oocytes, the number of ICSI oocytes, and the rates of fertilization, cleavage, transferrable embryos, good embryos, embryos implanted, clinical pregncncy, early abortion, singleton and twins. Results: After 27 cycles of ICSI, all of the MMAF patients achieved clinical pregnancy, including 11 cases of live birth, 2 cases of spontaneous abortion, and 2 cases of pregnancy maintenance. There were no significant difference between MMAF and OA groups in the couples'age and BMI, or the numbers of retrieved oocytes and ICSI oocytes(P>0.05), but the differences in the infertility duration had statistical meaning(P<0.001). No statistical differences were observed among groups in ICSI fertility rate(92.0% vs 91.6%), clesvage rate(95.4% vs 96.5%), high-quality embryonic rate(56.5% vs 57.5%), good blastocyst rate(23/61 vs 35/94), embryo implantation rate(20/48 vs 35/75), early abortion rate(4/19 vs 8/36), clinical pregncncy rate(15/27 vs 28/50), singleton rate (10/13 vs 20/25)and twinning rate(3/13 vs 5/25)(P>0.05). Conclusions: MMAF may not affect ICSI treatment outcomes, but genetic defects can be transmitted through ICSI. The affected couples should be informed of the necessity of prenatal genetic diagnosis before embryo implantation and the inevitable vertical transmission of genetic problems to the offspring.
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Taxa de Gravidez , Injeções de Esperma Intracitoplásmicas , Transferência Embrionária , Feminino , Flagelos , Humanos , Infertilidade Masculina , Masculino , Gravidez , Estudos RetrospectivosRESUMO
Objective: To explore the differential diagnostic role of B cell-activating factor(BAFF) in idiopathic pulmonary fibrosis (IPF) and usual interstitial pneumonia (UIP) associated with autoimmune diseases (AIDs). Methods: Plasma levels of BAFF were measured by ELISA method in 23 patients with AIDs-UIP, 34 patients with IPF, and 21 healthy subjects as control. The correlation between plasma BAFF levels and other clinical results from patients was analyzed. Receiver operating characteristics (ROC) analysis for distinguishing AIDs-UIP from IPF patients was examined and the maximal area under curve (AUC) was found. Results: Plasma levels of BAFF were significantly elevated in AIDs-UIP patients and IPF patients compared to healthy subjects(P<0.001 and P=0.002, respectively). AIDs-UIP patients had higher level of BAFF than IPF patients(P=0.030). Plasma BAFF levels in AIDs-UIP patients were inversely correlated with pulmonary function results, including FVC%(r=-0.435, P=0.040)and TLC%(r=-0.449, P=0.041), as well as DLCO%(r=-0.491, P=0.024). When the cut off value of BAFF was set as 1.5 ng/ml to distinguish AIDs-UIP patients from IPF patients, the sensitivity and the specificity was 64.5% and 90.0%, respectively, and the area under ROC curve reached the maximum of 0.784(P=0.000, 95% CI: 66.3%-90.5%). Conclusions: Plasma BAFF levels were significantly higher and inversely correlated with pulmonary function, reflecting the severity of AIDs-UIP patients. Plasma BAFF levels may be a useful marker for distinguishing AIDs-UIP from IPF.
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Fator Ativador de Células B/sangue , Fibrose Pulmonar Idiopática/diagnóstico , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Autoimunes , Diagnóstico Diferencial , Ensaio de Imunoadsorção Enzimática , Humanos , Fibrose Pulmonar Idiopática/sangue , Pulmão , Doenças Pulmonares Intersticiais/sangueRESUMO
Objective: To evaluate the clinical application value of diffusion tensor imaging (DTI) parameter in evaluating the prognosis of spinal cord injury in acute cervical spinal cord injury. Method: From October 2012 to December 2015, a total of 23 patients with acute cervical spinal cord injury were examined by routine MRI-T2WI high signal DTI examination, according to the American Spinal Injury Association (ASIA) damage standard. One month later according to the functional outcome of the spinal cord, all patients were divided into non improvement group (group A) 4 cases (2 cases of ASIA-A, 2 cases of grade B) and improvement group (group B) 19 cases (13 cases of ASIA-C, 5 cases of grade D, 1 case of grade B), 15 cases of healthy volunteers as the normal group (group C ). The FA value, ADC and axial eigen value[λâ¥=λ1], radial eigen value[λâ¥=(λ2+ λ3)/2]of before and after the treatment in group A and group B were compared by paired t test respectively. Single factor analysis of variance (ANOVA) was used to analyze the differences of DTI among the group A, group B and group C, and LSD test was used to compare the differences among the groups.Before and after treatment, the DTI measurement area was consistent.Changes of nerve fiber bundles in group A and group B before and after treatment were displayed in DTT. Results: After the treatment , the FA value of group A decreased , ADC value and λ⥠value increased than that of before treatment(t=3.037, -4.183, -4.805, all P<0.05), λ⥠value increased(t=-1.160, P>0.05). After the treatment , the FA value and ADC value of group B increased , λ⥠value decreased than that of before treatment(t=-3.344, -3.642, 4.694, all P<0.05), λ⥠value increased(t=-0.596, P>0.05). After treatment, there were statistical differences in FA values, λ⥠value , ADC values and λ⥠values among three groups (F=71.958, 70.432, 75.902, 22.521, all P<0.01). Conclusion: The changes of FA value, λ⥠value are the two most sensitive indicators of acute cervical spinal cord injury of spinal cord function prognosis.It suggests the poor prognosis of spinal cord function when FA value decreases and λ⥠value continues to increase.DTT can visually display subtle changes in the nerve fiber bundle.
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Medula Cervical , Traumatismos da Medula Espinal , Imagem de Tensor de Difusão , Humanos , Imageamento por Ressonância Magnética , Lesões do Pescoço , PrognósticoRESUMO
Microglia mediated neuroinflammation plays a crucial role in intracerebral hemorrhage (ICH). Raf kinase inhibitor protein (RKIP), a member of the phosphatidylethanolamine-binding protein (PEBP) family, is a negative regulator of inflammatory responses. However, the expression and anti-inflammatory effects of RKIP in microglia after ICH have not been reported. Therefore, in the current study, we investigated the effects of RKIP on inflammatory responses in erythrocyte lysate-treated BV2 microglia. Furthermore, we analyzed the detailed molecular mechanisms underlying the anti-inflammatory effects of RKIP in microglia. Our results showed that the expression level of RKIP was significantly decreased by erythrocyte lysate treatment in BV2 microglia. Overexpression of RKIP inhibited the production of pro-inflammatory molecules. In addition, overexpression of RKIP attenuated neuronal cell death induced by activated microglia. Moreover, RKIP suppressed the activation of NF-κB signaling pathway in erythrocyte lysis-treated BV2 cells. In conclusion, these data suggest that overexpression of RKIP attenuated microglia inflammation through inhibiting the NF-κB signaling pathway in erythrocyte lysis-treated BV2 cells. The present study provides evidence that RKIP may be used as an effective molecular target for the treatment of ICH.
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Hemorragia Cerebral/metabolismo , Hemorragia Cerebral/patologia , Inflamação/patologia , Microglia/metabolismo , Microglia/patologia , Modelos Biológicos , Proteína de Ligação a Fosfatidiletanolamina/metabolismo , Animais , Morte Celular , Linhagem Celular , Citocinas/metabolismo , Eritrócitos/metabolismo , Hemólise , Humanos , Mediadores da Inflamação/metabolismo , Camundongos , NF-kappa B/metabolismo , Neurônios/metabolismo , Neurônios/patologia , Fagocitose , Espécies Reativas de Oxigênio/metabolismo , Transdução de Sinais , TransfecçãoRESUMO
The purpose of this study was to evaluate single nucleotide polymorphism (SNP) variants of the estrogen receptor 1 gene (ESR1) at rs2234693 and rs9340799, as well as to investigate the relationship between ESR gene polymorphisms and postmenopausal osteoporosis (OP) of the spine in Chinese women. We recruited 198 postmenopausal women with OP and 276 healthy women between May 2012 and September 2015 in Zhongshan Hospital. Dual energy x-ray absorptiometry was used to measure the bone mineral density (BMD) of the lumbar vertebrae in all subjects. In addition, PCR-restriction fragment length polymorphism based analysis was conducted to identify the genotypes of ESR1. The distribution of ESR1 in the osteoporosis group and the control group was determined; the relationship between ESR polymorphisms and BMD was analyzed. The distributions of BMD were: TT < TC < CC, GG < AG < AA. The TT, TTGG, and TCGG genotypes were found to be lower as compared to the other genotypes. Stratified analysis suggested that the TT genotype and the combined genotypes TTGG and TCGG were significantly higher in the OP group as compared to the control group (P < 0.01). Therefore, ESR1 polymorphisms at rs2234693 and rs9340799 may be associated with OP, and could be used as markers to screen those with high risks to postmenopausal OP in Chinese women.
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Receptor alfa de Estrogênio/genética , Estudos de Associação Genética , Predisposição Genética para Doença , Osteoporose Pós-Menopausa/genética , Absorciometria de Fóton , Densidade Óssea/genética , China , Feminino , Frequência do Gene , Genótipo , Humanos , Osteoporose Pós-Menopausa/patologia , Polimorfismo de Nucleotídeo Único , Pós-MenopausaRESUMO
The low-light tolerance index was investigated in a set of 123 F2:3 lines during the seedling stage across 2 seasons, and the heredity of low-light tolerance was assessed via different ge-netic analysis methods. The results of the classical analysis showed that low-light tolerance is controlled by an additive-dominant poly-gene, and the polygenic inheritance rate of separate generations was >30%. In addition, 5 quantitative trait loci (QTLs) exhibited a low-light tolerance index across both seasons, including 2 QTLs (Llti1.1 and Llti1.2) on the 1st linkage group (variances of 6.0 and 9.5%) and 3 QTLs (Llti2.1, Llti2.1, and Llti2.1) on the 2nd linkage group (variances of 10.1-14.0%). The classical analysis method and QTL information on the heredity of low-light tolerance showed that it is controlled by several major genes and a mini-polygene. The results will facilitate the breeding of resistance to low-light stress in cucumber.
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Adaptação Fisiológica/genética , Cucumis sativus/genética , Genes de Plantas , Herança Multifatorial , Locos de Características Quantitativas , Adaptação Fisiológica/efeitos da radiação , Mapeamento Cromossômico , Cromossomos de Plantas/química , Cucumis sativus/crescimento & desenvolvimento , Cucumis sativus/efeitos da radiação , Bases de Dados Genéticas , Ligação Genética , Luz , Fenótipo , Melhoramento Vegetal , Estações do Ano , PlântulaRESUMO
This study aimed to determine whether feeding betaine to cows elevates their production performance during summer heat stress. Thirty-two lactating Holstein cows were randomly divided into 4 groups: the control group, which received a total mixed ration (TMR), and 3 experimental groups that received TMR blended with 10 g/day (group I), 15 g/day (group II), and 20 g/day (group III) betaine for 8 weeks. Milk and blood were sampled throughout the experimental period. The average maximum and minimum air temperatures were 28.3 and 24.1°C, respectively. The average temperature-humidity index was 78.6 units. The results showed that feeding betaine to cows increased feed intake, milk yield, milk lactose, milk protein, plasma cortisol, glutathione peroxidase, superoxide dismutase, and malondialdehyde levels (P<0.05); however, it caused HSP70 levels to decrease (P<0.05). The milk performance of group II was significantly affected. These results indicate that supplementing betaine to the diet of dairy cows increases their milk performance and improves their antioxidant capacity; these processes help relieve the cow from heat stress. In conclusion, supplementing dairy cows with 15 g/day betaine generated the most positive influence on performance and productivity, and hence caused the greatest reduction in heat stress.
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Ração Animal , Betaína , Laticínios , Suplementos Nutricionais , Leite , Característica Quantitativa Herdável , Estresse Fisiológico , Animais , Antioxidantes/metabolismo , Temperatura Corporal , Bovinos , Clima , Frequência Cardíaca , Temperatura Alta , Lactação , Metaboloma , Metabolômica , Taxa Respiratória , Estações do AnoRESUMO
A 0.65 THz Solid-state Source Interferometer-Polarimeter (SSIP) with vertical viewing has been installed for line-integrated density and Faraday rotation measurements on Experimental Advanced Superconducting Tokamak (EAST). The SSIP utilizes three independent solid-state diode sources based on frequency multiplier (X48) to provide the probing beam at a fixed frequency of 0.65 THz with â¼2.5 mW output power, which provide an Intermediate Frequency (IF) with wide range, and the highest IF is up to â¼10 MHz. The mixers optimized for high sensitivity, â¼750 mV/mW, are used in the SSIP system, which permits multichannel interferometer-polarimeter on EAST with a low phase noise. The resolution of the integrated density is 3.3 × 1017 m-2, and the Faraday rotation is about 1.5°. The density measurement has been implemented with IF â¼0.85 MHz, in a plasma with plasma current â¼300 kA on EAST. The line-averaged density, 3.5 × 1019 m-3, measured by the SSIP shows good agreement with the results measured by the POlarimeter-INTerferometer (POINT) system and the CO2 dispersion interferometer system in the same discharge.
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Objective: To analyze the clinical features,treatment and prognosis of drug induced hypersensitivity syndrome related hemophagocytic lymphohistiocytosis (DIHS-HLH). Methods: This was a retrospective case study. Clinical characteristics, laboratory results, treatment and prognosis of 9 patients diagnosed with DIHS-HLH in Beijing Children's hospital between January 2020 and December 2022 were summarized. Kaplan-Meier survival analysis was used to calculate the overall survival rate. Results: Among all 9 cases, there were 6 males and 3 females, with the age ranged from 0.8 to 3.1 years. All patients had fever, rash, hepatomegaly and multiple lymph node enlargement. Other manifestations included splenomegaly (4 cases), pulmonary imaging abnormalities (6 cases), central nervous system symptoms (3 cases), and watery diarrhea (3 cases). Most patients showed high levels of soluble-CD25 (8 cases), hepatic dysfunction (7 cases) and hyperferritinemia (7 cases). Other laboratory abnormalities included hemophagocytosis in bone marrow (5 cases), hypofibrinogenemia (3 cases) and hypertriglyceridemia (2 cases). Ascending levels of interleukin (IL) 5, IL-8 and interferon-γ (IFN-γ) were detected in more than 6 patients. All patients received high dose intravenous immunoglobulin, corticosteroid and ruxolitinib, among which 4 patients were also treated with high dose methylprednisolone, 2 patients with etoposide and 2 patients with cyclosporin A. After following up for 0.2-38.6 months, 7 patients survived, and the 1-year overall survival rate was (78±14)%. Two patients who had no response to high dose immunoglobulin, methylprednisolone 2 mg/(kg·d) and ruxolitinib died. Watery diarrhea, increased levels of IL-5 and IL-8 and decreased IgM were more frequently in patients who did not survive. Conclusions: For children with fever, rash and a suspicious medication history, when complicated with hepatomegaly, impaired liver function and high levels of IL-5 and IL-8, DIHS-HLH should be considered. Once diagnosed with DIHS-HLH, suspicious drugs should be stopped immediately, and high dose intravenous immunoglobulin, corticosteroid and ruxolitinib could be used to control disease.
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Exantema , Linfo-Histiocitose Hemofagocítica , Criança , Masculino , Feminino , Humanos , Lactente , Pré-Escolar , Linfo-Histiocitose Hemofagocítica/etiologia , Linfo-Histiocitose Hemofagocítica/complicações , Estudos Retrospectivos , Interleucina-5 , Hepatomegalia/complicações , Imunoglobulinas Intravenosas/efeitos adversos , Interleucina-8 , Metilprednisolona , Corticosteroides , Diarreia/complicações , Exantema/complicaçõesRESUMO
Objective: To analyze the efficacy and safety of the L-DEP regimen (asparaginase, liposome doxorubicin, etoposide and methylprednisolone) as a salvage therapy for the refractory primary hemophagocytic lymphohistocytosis triggered by Epstein-Barr virus infection (EBV-pHLH) in children. Methods: In this retrospective case study, clinical and laboratory data before and after L-DEP regimen of 4 children diagnosed with EBV-pHLH in Beijing Children's hospital between January 2016 and June 2022 were collected, and the efficacy and safety of L-DEP regimen for the treatment of EBV-pHLH were analyzed. Results: Among 4 patients, there were 3 females and 1 male with the age ranged from 0.8 to 7.0 years. Two of them showed compound heterozygous mutations of PRF1, one with a heterozygous mutation of UNC13D, one homozygous mutation of ITK. Before the L-DEP therapy, all of them had anemia and a soaring level of soluble CD25, 3 patients had neutropenia and thrombopenia, 3 patients had a high level of ferritin, 3 patients had hypofibrinogenemia and 1 patient had hypertriglyceridemia. After receiving 1 or 2 cycles of L-DEP treatment, three achieved remission, including complete remission (1 case) and partial remission (2 cases), and the other one had no remission. The levels of blood cell counts, soluble CD25, triglyceride, fibrinogen and albumin were recovered gradually in 3 patients who got remission. All four patients underwent hematopoietic stem cell transplantation (HSCT) after L-DEP regimen, and three survived. All patients had no severe chemotherapy related complications. The main side effects were bone marrow suppression, infection and pancreatitis, which recovered after appropriate treatments, apart from one who died from severe infection after urgent HSCT. Conclusion: L-DEP regimen could be served as an effective and safe salvage treatment for refractory pediatric EBV-pHLH, and also provide an opportunity for patients to receive HSCT.
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Asparaginase , Infecções por Vírus Epstein-Barr , Etoposídeo , Linfo-Histiocitose Hemofagocítica , Terapia de Salvação , Humanos , Linfo-Histiocitose Hemofagocítica/terapia , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Masculino , Feminino , Infecções por Vírus Epstein-Barr/tratamento farmacológico , Infecções por Vírus Epstein-Barr/complicações , Estudos Retrospectivos , Terapia de Salvação/métodos , Criança , Lactente , Pré-Escolar , Etoposídeo/administração & dosagem , Asparaginase/administração & dosagem , Doxorrubicina/administração & dosagem , Metilprednisolona/administração & dosagem , Mutação , Proteínas de Membrana/genética , Resultado do Tratamento , Perforina/genética , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Lipossomos , Herpesvirus Humano 4/genéticaRESUMO
Objective: To analyze the efficacy, safety, and long-term prognosis of intermediate-dose cytarabine (Ara-c) regimen in the treatment of children with refractory risk organ involvement Langerhans cell histiocytosis (LCH). Methods: Clinical data of 17 children with multisystem and risk organ involvement LCH who failed the first-line therapy and were treated with intermediate-dose Ara-c (250 mg/m2, twice daily) regimen in the Hematology Center, Beijing Children's Hospital from January 2013 to December 2016 were analyzed retrospectively. In addition to the basic treatment of vindesine and dexamethasone, the patients received two regimens: regimen A: the intermediate-dose Ara-c combined with cladribine and regimen B: the intermediate-dose Ara-c alone. The efficacy, safety and prognosis of the two regimens were analyzed. Results: Among all 17 patients, there were 11 males and 6 females, with the diagnosis age of 2.1 (1.6, 2.7) years. Ten children received regimen A, all of them achieved active disease-better (AD-B) after 8 courses of induction therapy. The disease activity scores (DAS) decreased from 5.5 (3.0, 9.0) to 1.0 (0, 2.3). Seven children received regimen B, and 6 of them achieved AD-B after 8 courses of induction therapy. The DAS decreased from 4.0 (2.0, 4.0) to 1.0 (0, 2.0). The follow-up time was 6.2 (4.9,7.2) and 5.2 (3.7,5.8) years in group A and B. The 5-year overall survival rate was 100.0% in both groups, and the 5-year event free survival rate was (88.9±10.5)% and (85.7±13.2)% in group A and B. Grade 3 or 4 myelosuppression was observed in 8 patients in group A and 2 patients in group B. Conclusions: The intermediate-dose Ara-c regimen (with or without cladribine) is effective and safe for patients with refractory high-risk LCH, with a good long-term prognosis.
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Citarabina , Histiocitose de Células de Langerhans , Masculino , Feminino , Criança , Humanos , Citarabina/efeitos adversos , Cladribina/efeitos adversos , Estudos Retrospectivos , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Histiocitose de Células de Langerhans/tratamento farmacológico , PrognósticoRESUMO
Objective: To evaluate the efficacy and safety of Beijing Children's Hospital (BCH) modified hemophagocytic lymphohistiocytosis (HLH) 04 regimen in the treatment of childhood HLH. Methods: A retrospective cohort study was conducted. From January 2016 to December 2017, 110 children with HLH who were treated with the modified HLH-04 regimen (replacing dexamethasone with methylprednisolone during the induction period, reducing the dose and frequency of etoposide, and not using cyclosporine except for autoimmune-related HLH) at the Hematology Oncology Center of Beijing Children's Hospital were selected as the modified group, while 102 children treated with the standard HLH-04 regimen from January 2012 to December 2015 were selected as the control group. The early remission rate, survival rate and adverse reactions of two groups were compared. Rank sum test and chi square test were used for comparison between groups. Results: The age of onset in the modified group was 1.9 (1.1, 3.5) years, with 65 males and 45 females. The age of onset in the control group was 2.0 (1.2, 4.6) years, with 47 males and 55 females. No significant difference was found in age and gender between 2 groups (both P>0.05). Except for fibrinogen (1.3 (1.0, 1.7) vs. 1.1 (0.8, 1.4) g/L, Z=-2.67, P=0.008) and natural killer cell activity (13.9 (13.4, 16.3) % vs.14.9 (12.0, 16.1) %, Z=-2.34, P=0.028), there were no statistically significant differences in etiology, disease duration, first clinical presentation, or laboratory tests between 2 groups (all P>0.05). At 2 months and 3 years, there were no statistically significant differences in overall survival between 2 groups (84.5% (93/110) vs.76.5% (78/102), 78.2% (86/110) vs. 67.6% (69/102), χ2=2.28, 3.07, P=0.131, 0.080). The first 3 weeks were the most common time for bone marrow suppression in the modified group, with a lower incidence than in the control group (47.3% (52/110) vs. 62.7% (64/102), χ2=5.11, P=0.024). The modified group had a lower rate of fungal infections than the control group (3.6% (4/110) vs. 13.7% (14/102), χ2=6.93, P=0.008). Compared with the control group, fewer children in the modified group died as a result of side effects from chemotherapy (8.0% (2/25) vs.30.3% (10/33), χ2=4.31, P=0.038). Conclusion: The BCH modified HLH-04 regimen reduced the intensity of chemotherapy, with overall efficacy no worse than the standard HLH-04 regimen, and significantly reduced the rate of chemotherapy-related myelosuppression, fungal infection and mortality.
Assuntos
Linfo-Histiocitose Hemofagocítica , Criança , Ciclosporina/uso terapêutico , Etoposídeo , Feminino , Hospitais , Humanos , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Linfo-Histiocitose Hemofagocítica/etiologia , Masculino , Estudos RetrospectivosRESUMO
Vertical position stability plays a crucial role in maintaining safe and reliable plasma operation for long-pulse fusion devices. In general, the vertical position is measured by using inductive magnetic coils installed inside the vacuum vessel; however, the integration drift effects are inherent for steady-state or long-pulse plasma operation. Developing a non-magnetic approach provides a fusion reactor-relevant steady-state solution that avoids the negative impact of integration drift. In this paper, we compare the non-inductively determined vertical position achieved by line-integrated interferometer and polarimeter measurements to that employing an inductive flux loop for a 1056 s discharge recently achieved on EAST (Experimental Advanced Superconducting Tokamak). Experimental results show that the non-inductive measurement is more robust than flux loops after 300 s if the integrator is not reset to suppress integrator drift. Real-time vertical position control using the non-inductive system is proposed for the next EAST experimental campaign.
RESUMO
BACKGROUND: It has been reported that some single nucleotide polymorphisms (SNPs) of the angiotensin converting enzyme (ACE) gene and the endothelial nitric oxide synthase (eNOS) gene are associated with the development of systemic lupus erythematosus (SLE) and the progression of nephropathy. The aim of this study was to evaluate the possible association between six SNPs (A-5466C, T-3892C, A-240T, C1237T, G2215A and A2350G) of the ACE gene and two SNPs (T-786C and G894T) of the eNOS gene with lupus nephropathy in a northern Chinese population. METHODS: In this study, 225 patients with lupus nephropathy were compared to 232 healthy controls, matched by gender, age and ethnicity. Following the extraction of genomic DNA from the leukocytes in the peripheral blood, the genotypes of the eight selected SNPs were determined by the method of PCR-RFLP; the haplotypes were inferred using PHASE 2.1. The associations between the SNPs and the risk of lupus nephropathy were analyzed using Chi-square test and Logistic regression with SPSS13.0 software. RESULTS: Statistically significant differences of the allele frequency distribution of three SNPs (A-5466C, A2350G and G894T) were observed between cases and controls (P<0.05). Among the 53 haplotypes identified, the frequencies of five haplotypes (CTTCGA, ACTTAA, ACATGG, ACACGG and ATTCGA) were significantly different between cases and controls (P<0.05). CONCLUSIONS: Our study indicated an association between the risk of lupus nephropathy and the sequence variations of both the ACE gene and the eNOS gene, which may play an important role in the pathogenesis of lupus nephropathy in the northern Chinese population. Further studies are warranted to validate our findings.
Assuntos
Povo Asiático/genética , Lúpus Eritematoso Sistêmico/genética , Óxido Nítrico Sintase Tipo III/genética , Peptidil Dipeptidase A/genética , Polimorfismo Genético , Alelos , Progressão da Doença , Frequência do Gene , Genótipo , Haplótipos , Humanos , Reação em Cadeia da Polimerase , Polimorfismo de Fragmento de Restrição , Polimorfismo de Nucleotídeo Único , Grupos Populacionais/genéticaRESUMO
OBJECTIVE: On December 8, 2019, many cases of pneumonia with unknown etiology were first reported in Wuhan, China, subsequently identified as a novel coronavirus infection aroused worldwide concern. As the outbreak is ongoing, more and more researchers focused interest on the COVID-19. Therefore, we retrospectively analyzed the publications about COVID-19 to summarize the research hotspots and make a review, to provide reference for researchers in the world. MATERIALS AND METHODS: We conducted a search in PubMed using the keywords "COVID-19" from inception to March 1, 2020. Identified and analyzed the data included title, corresponding author, language, publication time, publication type, research focus. RESULTS: 183 publications published from 2020 January 14 to 2020 February 29 were included in the study. The first corresponding authors of the publications were from 20 different countries. Among them, 78 (42.6%) from the hospital, 64 (35%) from the university and 39 (21.3%) from the research institution. All the publications were published in 80 different journals. Journal of Medical Virology published most of them (n=25). 60 (32.8%) were original research, 29 (15.8%) were review, 20 (10.9%) were short communications. 68 (37.2%) epidemiology, 49 (26.8%) virology and 26 (14.2%) clinical features. CONCLUSIONS: According to our review, China has provided a large number of research data for various research fields, during the outbreak of COVID-19. Most of the findings play an important role in preventing and controlling the epidemic around the world. With research on the COVID-19 still booming, new vaccine and effective medicine for COVID-19 will be expected to come out in the near future with the joint efforts of researchers worldwide.