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1.
Plant Dis ; 2024 Apr 08.
Artigo em Inglês | MEDLINE | ID: mdl-38587799

RESUMO

Salvia splendens is a popular ornamental plant in China with extensive potentials, including value in traditional Chinese medicine and in environmental restoration function (Li et al. 2008). In September 2019, leaf blight disease was observed on road side plants of S. splendens in Bayi park, Nanchang city, Jiangxi province, China. The typical symptoms appeared as irregular necrotic spots or leaf blight, accompanied by extensive scorch necrosis or ultimately defoliation. Small segments cut from diseased leaves were surface sterilized in a 2% sodium hypochlorite solution for 2 min and rinsed three times with sterile distilled water. Then, the samples were placed on potato dextrose agar (PDA) plates incubated at 25°C in darkness. Pure cultures were obtained by the hyphal tip method. Morphologically, all 11 colonies were identical to each other on PDA. Two strains, YZU 191468 and YZU 191481, were selected for further study and deposited in the Fungal Herbarium of Yangtze University (YZU), Jingzhou, Hubei, China. The 7-day-old colonies were circular, 53 to 56 mm in diameter, and consisted of white mycelium with a buff margin, and were cinnamon colored in the center of the reverse side. To examine conidial morphology, the mycelium was transferred onto potato carrot agar (PCA) and incubated at 23°C with a period of 8 h light/16 h dark for 7 days. Conidia were normally solitary or two in a chain, ellipsoid or long ellipsoid, beakless, 10 to 23×30 to 60 µm in size (n=50). Based on morphology, the isolates were consistent with Stemphylium lycopersici (Yamamoto 1960). To confirm the identification, genomic DNA was extracted from both isolates and used to amplify the internal transcribed spacer rDNA region (ITS), glyceraldehydes-3-phosphate dehydrogenase (GAPDH) and calmodulin (CAL) genes with primer pairs ITS5/ITS4, gpd1/gpd2, and CALDF1/CALDR2, respectively (Woudenberg et al. 2017). Sequences were deposited in GenBank with accession numbers OP564983 and OP564984 (ITS), OP892529 and OP892530 (GAPDH), OP584970 and OP584971 (CAL). A neighbor-joining tree was constructed with Mega 7.0 based on the combined dataset with 1,000 bootstrap replicates. The resulting phylogenetic tree showed that the strains from S. splendens clustered with S. lycopersici (CBS 122639 and CBS 124980) supported with 100% bootstrap values. The molecular analyses confirmed that the species causing leaf blight symptoms was S. lycopersici. To test pathogenicity, healthy leaves of S. splendens were surface sterilized and inoculated by mycelium blocks (6 mm in diameter) and spore suspension (1×106 spore/mL) of representative strains YZU 191468 and YZU 191481, respectively. Controls were inoculated with blocks of PDA and sterile water. Each strain was inoculated on three leaves of a plant. One clean plant was used as control. The test was replicated three times. After inoculation, the plants were covered with plastic bags and incubated in a greenhouse (25℃, 80 % relative humidity, 8 h light/16 h dark). After 5 days, the inoculated leaves exhibited dark brown spots with white mycelium, followed by withering of necrotic tissues. There were no symptoms observed on the controls. The fungal isolates inoculated leaves had the same morphological characteristics as the strains used for inoculation. S. lycopersici has been found on eggplant and Zinnia elegans in China (He et al. 2019; Yang et al. 2017). To the best of our knowledge, this is the first report of S. lycopersici causing leaf blight on S. splendens in China. This finding offers a new reference for the management and control of S. splendens leaf diseases in China.

2.
Alzheimers Res Ther ; 16(1): 43, 2024 Feb 20.
Artigo em Inglês | MEDLINE | ID: mdl-38378607

RESUMO

BACKGROUND: Glymphatic dysfunction is a crucial pathway for dementia. Alzheimer's disease (AD) pathologies co-existing with cerebral small vessel disease (CSVD) is the most common pathogenesis for dementia. We hypothesize that AD pathologies and CSVD could be associated with glymphatic dysfunction, contributing to cognitive impairment. METHOD: Participants completed with amyloid PET, diffusion tensor imaging (DTI), and T2 fluid-attenuated inversion-recovery (FLAIR) sequences were included from the Alzheimer's Disease Neuroimaging Initiative (ADNI). White matter hyperintensities (WMH), the most common CSVD marker, was evaluated from T2FLAIR images and represented the burden of CSVD. Amyloid PET was used to assess Aß aggregation in the brain. We used diffusion tensor image analysis along the perivascular space (DTI-ALPS) index, the burden of enlarged perivascular spaces (PVS), and choroid plexus volume to reflect glymphatic function. The relationships between WMH burden/Aß aggregation and these glymphatic markers as well as the correlations between glymphatic markers and cognitive function were investigated. Furthermore, we conducted mediation analyses to explore the potential mediating effects of glymphatic markers in the relationship between WMH burden/Aß aggregation and cognition. RESULTS: One hundred and thirty-three participants along the AD continuum were included, consisting of 40 CN - , 48 CN + , 26 MCI + , and 19 AD + participants. Our findings revealed that there were negative associations between whole-brain Aß aggregation (r = - 0.249, p = 0.022) and WMH burden (r = - 0.458, p < 0.001) with DTI-ALPS. Additionally, Aß aggregation (r = 0.223, p = 0.041) and WMH burden (r = 0.294, p = 0.006) were both positively associated with choroid plexus volume. However, we did not observe significant correlations with PVS enlargement severity. DTI-ALPS was positively associated with memory (r = 0.470, FDR-p < 0.001), executive function (r = 0.358, FDR-p = 0.001), visual-spatial (r = 0.223, FDR-p < 0.040), and language (r = 0.419, FDR-p < 0.001). Conversely, choroid plexus volume showed negative correlations with memory (r = - 0.315, FDR-p = 0.007), executive function (r = - 0.321, FDR-p = 0.007), visual-spatial (r = - 0.233, FDR-p = 0.031), and language (r = - 0.261, FDR-p = 0.021). There were no significant correlations between PVS enlargement severity and cognitive performance. In the mediation analysis, we found that DTI-ALPS acted as a mediator in the relationship between WMH burden/Aß accumulation and memory and language performances. CONCLUSION: Our study provided evidence that both AD pathology (Aß) and CSVD were associated with glymphatic dysfunction, which is further related to cognitive impairment. These results may provide a theoretical basis for new targets for treating AD.


Assuntos
Doença de Alzheimer , Doenças de Pequenos Vasos Cerebrais , Disfunção Cognitiva , Humanos , Doença de Alzheimer/patologia , Imagem de Tensor de Difusão/métodos , Cognição , Encéfalo/metabolismo , Disfunção Cognitiva/metabolismo , Amiloide/metabolismo , Imageamento por Ressonância Magnética , Doenças de Pequenos Vasos Cerebrais/complicações , Doenças de Pequenos Vasos Cerebrais/diagnóstico por imagem , Proteínas Amiloidogênicas/metabolismo
3.
Insect Sci ; 31(1): 13-27, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37231527

RESUMO

Diaphorina citri is a global citrus pest. As a vector insect, it can transmit the causative agents of citrus huanglongbing, causing irreversible losses to the citrus industry. The acquisition of genomic information can provide a molecular genetic basis for effective control of D. citri. Here, the DNBSEQ™ , Oxford Nanopore Technologies, and Hi-C technologies are applied to generate a high-quality chromosome-level genome of D. citri. The genome size of D. citri was 523.78 Mb with a scaffold N50 of 47.05 Mb distributed on 13 chromosomes. A total of 250.64 Mb (47.85%) repeat sequences and 24 048 protein-coding genes were predicted. Genome resequencing of female and male individuals indicated that the sex chromosome system of D. citri is XO. Phylogenetic analysis demonstrated that D. citri and Pachypsylla venusta, which separated from their most recent common ancestor about 336.62 million years ago, were the most closely related. Additionally, we identified genes potentially involved in detoxification metabolism, pathogen transmission, and honeydew secretion for further investigation. The high-quality genome provides an important reference for developing effective management strategies of D. citri.


Assuntos
Citrus , Hemípteros , Humanos , Animais , Feminino , Masculino , Hemípteros/genética , Filogenia , Análise de Sequência de DNA , Cromossomos , Citrus/genética
4.
J Pediatr Hematol Oncol ; 46(1): e71-e82, 2024 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-38018972

RESUMO

BACKGROUND: Accurate histologic and molecular genetic diagnosis is critical for the pathogenesis study of pediatric patients with lymphoblastic lymphoma (LBL). Optical genome mapping (OGM) as all-in-one process allows the detection of most major genomic risk markers, which addresses some of the limitations associated with conventional cytogenomic testing, such as low resolution and throughput, difficulty in ascertaining genomic localization, and orientation of segments in duplication, inversions, and insertions. Here, for the first time, we examined the cytogenetics of 5 children with LBL using OGM. METHODS: OGM was used to analyze 5 samples of pediatric LBL patients treated according to the modified NHL-BFM95 backbone regimen. Whole-exon Sequencing (WES) was used to confirm the existence of structural variants (SVs) identified by OGM with potentially clinical significance on MGI Tech (DNBSEQ-T7) platform. According to the fusion exon sequences revealed by WES, the HBS1L :: AHI1 fusion mRNA in case 4 was amplified by cDNA-based PCR. RESULTS: In total, OGM identified 251 rare variants (67 insertions, 129 deletions, 3 inversion, 25 duplications, 15 intrachromosomal translocations, and 12 interchromosomal translocations) and 229 copy number variants calls (203 gains and 26 losses). Besides all of the reproducible and pathologically significant genomic SVs detected by conventional cytogenetic techniques, OGM identified more SVs with definite or potential pathologic significance that were not detected by traditional methods, including 2 new fusion genes, HBS1L :: AHI1 and GRIK1::NSDHL , which were confirmed by WES and/or Reverse Transcription-Polymerase Chain Reaction. CONCLUSIONS: Our results demonstrate the feasibility of OGM to detect genomic aberrations, which may play an important role in the occurrence and development of lymphomagenesis as an important driving factor.


Assuntos
Linfoma não Hodgkin , Leucemia-Linfoma Linfoblástico de Células Precursoras , Humanos , Criança , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Variações do Número de Cópias de DNA , Éxons , Mapeamento Cromossômico
5.
Sci Data ; 10(1): 478, 2023 07 21.
Artigo em Inglês | MEDLINE | ID: mdl-37479750

RESUMO

Asian citrus psyllid (Diaphorina citri, D. citri) is the important vector of "Candidatus Liberibacter asiaticus" (CLas), associated with Huanglongbing, the most devastating citrus disease worldwide. CLas can affect endosymbiont abundance of D. citri. Here, we generated the high-quality gut endosymbiont metagenomes of Diaphorina citri on the condition of CLas infected and uninfected. The dataset comprised 6616.74 M and 6586.04 M raw reads, on overage, from CLas uninfected and infected psyllid strains, respectively. Taxonomic analysis revealed that a total of 1046 species were annotated with 10 Archaea, 733 Bacteria, 234 Eukaryota, and 69 Viruses. 80 unique genera in CLas infected D. citri were identified. DIAMOND software was used for complement function research against various functional databases, including Nr, KEGG, eggNOG, and CAZy, which annotated 84543 protein-coding genes. These datasets provided an avenue for further study of the interaction mechanism between CLas and D. citri.


Assuntos
Hemípteros , Rhizobiaceae , Animais , Hemípteros/genética , Hemípteros/microbiologia , Metagenoma , Rhizobiaceae/genética
6.
Chinese Journal of Pediatrics ; (12): 1118-1123, 2023.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wpr-1013233

RESUMO

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.


Assuntos
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 , Prognóstico
7.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wpr-1004753

RESUMO

【Objective】 To analyze the current situation of direct exemption of clinical blood fess for voluntary blood donors and their family members in Henan Province, in order to improve and fully implement the policy. 【Methods】 According to the policy on blood fees exemption issued by China and Henan Province in 2019,the data of hospitals in 18 prefecture-level cities in Henan from 2020 to 2021 were continuously collected from the system of clinical blood fees exemption,including the way of exemption,the number of people (times) of exemption,exemption amount, the proportion of blood fees exemption and the total exemption rate. The experience gained in the past two years after the implementation of the policy was summarized,and the existing problems and causes were analyzed. 【Results】 The rates of direct exemption of blood fees in Henan Province in 2020 and 2021 were 34.53% (8 709/25 221) and 71.68%(23 587/32 906) (P<0.05) ,respectively. In 2021, the direct exemption rate of blood fees in 18 cities was 6.20% (83/1 370) to 88.50% (1 332/1 505) [ (47.35±41.15)%],and increased month by month from 43.19% (1 183/2 507) in January to 83.15% (2 097/2 522) in August, then remained stable at a similar level to August from September to December, with 83.43% (2 744/3 289) in December as the highest for the year. 【Conclusion】 The implementation of the policy of blood fees exemption showed significant effectiveness, which has effectively promoted the development of voluntary blood donation in Henan. However, there is still room for improving the policy in some cities, which is expected to further increase the direct exemption rate of the city and the whole province.

8.
Chinese Journal of Radiology ; (12): 542-548, 2022.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wpr-932537

RESUMO

Objective:To develop a multimodal MRI-based radiomics model for the differential diagnosis of benign and malignant lung lesions, and to compare the discriminative abilities of different models.Methods:Totally 114 patients with 115 lesions (44 benign and 71 malignant) in Nantong First Peoples′s Hospital from January 2014 to October 2019 were included in the study. All patients underwent non-enhanced MR examination, and textural features from T 1WI,T 2WI and apparent diffusion coefficient (ADC) imaging were extracted. The feature selection methods included L1 based, mutual information, tree based, recursive feature elimination and F-test. Then we constructed a prediction model by using logistic regression (LR), support vector machine (SVM), random forest (RF) and k-nearest neighbor (KNN) respectively. In order to control the number of modeling features and reduce the ininterpretability of the model, the new model was obtained by manually modifying some parameters of the hyperparameter model. One hundred and fourteen cases were rotated as training and validation sets. The performance of each model was evaluated by confounding matrix and receiver operating characteristic (ROC) curve. Results:The area under the curve (AUC) of T 2WI based LR model for the differential diagnosis of benign and malignant pulmonary nodules/masses was 0.71 and the F1 score was 0.57. Based on T 1WI images, LR and SVM model could be used to identify benign and malignant pulmonary nodules, the AUC before parameter adjustment were 0.77 and 0.78, the accuracy after parameter adjustment (LR a,SVM a) was 0.67, 0.70, and both the AUC were 0.72. However, no matter which feature or classifier was selected, both the AUC and accuracy of ADC-based model were less than 0.70. Conclusion:Multimodal MRI-based radiomics model is valuable for the differential diagnosis of benign and malignant pulmonary nodules/masses, and T 1WI-based model shows the best discrimination.

9.
JOURNAL OF RARE DISEASES ; (4): 311-317, 2022.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wpr-1005020

RESUMO

Langerhans cell histiocytosis(LCH)and Langerhans cell sarcoma(LCS)are characterized by clone proliferation of Langerhans-type cells, which may occur concurrently or sequentially with T-cell acute lymphoblastic leukemia (T-ALL) and other Lymphoid neoplasms. A 15-year old female patient diagnosed with T-ALL developed LCH involving multiple systems during maintenance chemotherapy of T-AL. After treated with chemotherapy with improved result, the patient showed progression of the illness and refractory to the second-line treatment. We found c.G35A (p.G12D)mutation in the KRAS gene and used the targeted drug Trametinib for treatment. The treatment proved effective, leading to partial remission within a week. Three months after Trametinib treatment, the patient developed new lymphadenopathy. Biopsy revealed the existence of LCS. The disease progressed quickly, and the patient died 7 days after diagnosis of LCS. The case of patients with T-ALL then developing LCH and LCS sequentially is extraordinarily rare. The causes of the case is unclear and may be related to cell transdifferentiation, clonal evolution, and chemotherapy. Targeted drugs can contain this disease for a short time.

10.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wpr-928244

RESUMO

Background Ultrasound-guided continuous thoracic paravertebral block can provide pain-relieving and opioid-sparing effects in patients receiving open hepatectomy. We hypothesize that these effects may improve the quality of recovery (QoR) after open hepatectomy. Methods Seventy-six patients undergoing open hepatectomy were randomized to receive a continuous thoracic paravertebral block with ropivacaine (CTPVB group) or normal saline (control group). All patients received patient-controlled intravenous analgesia with morphine postoperatively for 48 hours. The primary outcome was the global Chinese 15-item Quality of Recovery score on postoperative day 7, which was statistically analyzed using Student's t-test. Results Thirty-six patients in the CTPVB group and 37 in the control group completed the study. Compared to the control group, the CTPVB group had significantly increased global Chinese 15-item Quality of Recovery scores (133.14 ± 12.97 vs. 122.62 ± 14.89, P = 0.002) on postoperative day 7. Postoperative pain scores and cumulative morphine consumption were significantly lower for up to 8 and 48 hours (P < 0.05; P = 0.002), respectively, in the CTPVB group. Conclusion Perioperative CTPVB markably promotes patient's QoR after open hepatectomy with a profound analgesic effect in the early postoperative period.


Assuntos
Humanos , Anestésicos Locais/uso terapêutico , Método Duplo-Cego , Hepatectomia/efeitos adversos , Morfina/uso terapêutico , Medição da Dor , Dor Pós-Operatória/etiologia , Ultrassonografia de Intervenção
11.
New Phytol ; 229(5): 2827-2843, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-33206385

RESUMO

Atmospheric CO2 concentrations exert a strong influence on the susceptibility of plants to pathogens. However, the mechanisms involved in the CO2 -dependent regulation of pathogen resistance are largely unknown. Here we show that the expression of tomato (Solanum lycopersicum) ß-CARBONIC ANHYDRASE 3 (ßCA3) is induced by the virulent pathogen Pseudomonas syringae pv. tomato DC3000. The role of ßCA3 in the high CO2 -mediated response in tomato and two other Solanaceae crops is distinct from that in Arabidopsis thaliana. Using ßCA3 knock-out and over-expression plants, we demonstrate that ßCA3 plays a positive role in the activation of basal immunity, particularly under high CO2 . ßCA3 is transcriptionally activated by the transcription factor NAC43 and is also post-translationally regulated by the receptor-like kinase GRACE1. The ßCA3 pathway of basal immunity is independent on stomatal- and salicylic-acid-dependent regulation. Global transcriptome analysis and cell wall metabolite measurement implicate cell wall metabolism/integrity in ßCA3-mediated basal immunity under both CO2 conditions. These data not only highlight the importance of ßCA3 in plant basal immunity under high CO2 in a well-studied susceptible crop-pathogen system, but they also point to new targets for disease management strategies in a changing climate.


Assuntos
Anidrases Carbônicas , Imunidade Vegetal , Solanum lycopersicum , Dióxido de Carbono/metabolismo , Anidrases Carbônicas/genética , Regulação da Expressão Gênica de Plantas , Solanum lycopersicum/genética , Solanum lycopersicum/metabolismo , Doenças das Plantas , Pseudomonas syringae/metabolismo
12.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wpr-882858

RESUMO

Objective:To understand the clinical characteristics and prognosis of Langerhans cell histiocytosis (LCH) with skin-limited lesion.Methods:A retrospective analysis was performed on clinical characteristics and prognosis of 16 skin-limited LCH patients, out of 578 LCH patients who were hospitalized in Beijing Children′s Hospital during December 2013 to June 2018.Results:A total of 16 skin-limited LCH cases, accounted for 2.7% of all 578 cases, were included.Among which, sex ratio (male vs.female) was 1.28∶1.00.Median ages of skin eruption occurrence and of diagnosis of the disease were 3.5 months (3 days to 2 years and 5 months) and 6 months (2 months 14 days to 2 years and 8 months) in this group.Among the 16 cases, seborrheic dermatitis-like lesions(11 cases, 68.7%) was the most common, and the trunk was most frequently involved[75.0% (12 cases)]. Serine/threonine protein kinase gene V600E [ BRAF (p.V600E)] mutation was detected in pathological specimens from 10 skin-limi-ted cases, with 9 cases being positive.Plasma samples from 5 positive cases were further detected for BRAF (p.V600E) mutation, and 4 positive results were gained.Of all 16 patients, 11 cases (68.7%) were treated.Remission were achieved in 3-6 months from treatment start in patients treated whether according to the Histiocyte Society′s LCH-2009 protocol for 25 weeks(6 cases, 37.5%), or with topical mometasonefuroate for 3 months (3 cases, 18.8%). Two patients(12.5%) with solitary cutaneous lesions underwent excision biopsy (one face and one prepuce) and were considered to be in remission immediately after surgery.None of these patients suffered from the recurrence of the disease.The remaining 5 patients (31.3%) with skin-limited LCH were just evaluated regularly, and achieved remission in 3-6 months of commencing observation.Among these untreated patients, 1 with consistently positive BRAF (p.V600E) mutation in plasma had bone involvement in the 24 th month of assessment, and was then treated based on the Histiocyte Society′s LCH-2009 Protocol.No clinical or imageological evidence supporting disease progression was found on this patient.Median follow-up period was 32.8 months (2.9-63.9 months). Except one patient, none of the rest cases had active disease till follow-up ended.Two-year event free survival(EFS) of this research was (92.3± 7.4)%.There was no significant difference between EFS of treated group and that of observation group( χ2=1.250, P=0.264). Conclusions:Skin-limited LCH often occurs in infants and newborns, with strong heterogeneity in clinical manifestations, laboratory indicators, and pathogenesis.Seborrheic dermatitis-like lesions were the most common cutaneous type.The prognosis of the patients is excellent despite progressing into multisystem involvement can be seen in a few patients.

13.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wpr-874352

RESUMO

Purpose@#We sought to investigate the effectiveness and safety of dabrafenib in children with BRAFV600E-mutated Langerhans cell histiocytosis (LCH). @*Materials and Methods@#A retrospective analysis was performed on 20 children with BRAFV600E-mutated LCH who were treated with dabrafenib. @*Results@#The median age at which the patients started taking dabrafenib was 2.3 years old (range, 0.6 to 6.5 years). The ratio of boys to girls was 2.3:1. The median follow-up time was 30.8 months (range, 18.9 to 43.6 months). There were 14 patients (70%) in the risk organ (RO)+ group and six patients (30%) in the RO– group. All patients were initially treated with traditional chemotherapy and then shifted to targeted therapy due to poor control of LCH or intolerance to chemotherapy. The overall objective response rate and the overall disease control rate were 65% and 75%, respectively. During treatment, circulating levels of cell-free BRAFV600E (cfBRAFV600E) became negative in 60% of the patients within a median period of 3.0 months (range, 1.0 to 9.0 months). Grade 2 or 3 adverse effects occurred in five patients. @*Conclusion@#Some children with BRAFV600E-mutated LCH may benefit from monotherapy with dabrafenib, especially high-risk patients with concomitant hemophagocytic lymphohistiocytosis and intolerance to chemotherapy. The safety of dabrafenib is notable. A prospective study with a larger sample size is required to determine the optimal dosage and treatment duration.

14.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wpr-934464

RESUMO

Objective:To address a new classification method in term of the three-dimensional space of orbit and to present the different surgical approaches correspondingly.Methods:In a retrospect study from April of 2015 to June of 2018, 102 patients were performed lower eyelid blepharoplasty, including 20 males and 82 females, aged 21-65 years, with an average of 45.2 years. These patients were divided into five groups, which were described in term of three-dimensional structure of orbit, based on the following points: the presence and extent of herniated orbital fat, the presence of inferior orbitopalpebral sulcus, amount of excess skin, and the skin wrinkles in the lower eyelid. And then patients in different group were treated with different kinds of blepharoplasty. All patients in this study ranged in follow-up from 1 month to 12 months. With patients' permit, photographs and clinical information were taken to evaluate the preoperative and postoperative outcome.Results:In type 1, all the 32 cases healed well, no complications such as hematoma, infection and ectropion occurred. During the follow-up of more than 1 months, the overall effect was good, and the pouch-shaped appearance of lower eyelid pouch was significantly improved. In types 2, 8 cases had no complications, and the incision healed well; the patients were followed up for more than 3 months, the lower eyelid bag and lower eyelid skin relaxation were significantly improved, and the lower eyelid skin was tighter than before. In types 3, there were no complications in these 19 cases, and the incision was healed well. The patients were followed up for more than 3 months, the deformity of lower eyelid bag was improved obviously, and no obvious local bulge was found under static and dynamic expression. In type 4, there were no complications in all 34 cases, and the incision healed well; during the follow-up of more than 3 months, the lower eyelid pouch deformity and lower eyelid skin relaxation were greatly improved, except for 1 case with mild bulge (untreated). In type 5, 9 cases had no complications, and the incision healed well; during the follow-up of more than 3 months, the fold of the lower eyelid skin disappeared.Conclusions:Little information is available about classification of lower eyelid bags. And the exact surgical approach remains controversial and largely dependent upon surgeon preference and a patient's stated cosmetic desire. In this study, an objective classification based on clinical appearance combined with forming reasons of lower eye bags is little available, and the appropriate surgical approach remains controversial as well.

15.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wpr-905381

RESUMO

Objective:To apply 13N-ammonia PET/CT cerebral blood perfusion imaging combined with methazolamide challenge for cerebrovascular reserve (CVR) evaluation in ischemic cerebrovascular diseases. Methods:From January, 2014 to December, 2016, 56 ischemic stroke patients with serious stenosis of unilateral internal carotid artery or middle cerebral artery accepted basal and stress PET/CT with methazolamide challenge. The patients were divided into normal-CVR group (n = 29) and reduced-CVR group (n = 27) according to the results of CVR, and followed up for 24 months. The ischemic cerebrovascular events and cerebral blood flow were observed. Results:The incidence of transient ischemic attack was more in the reduced-CVR group than in the normal-CVR group (χ2 = 4.389, P < 0.05), while the incidence of ischemic stroke increased a little with no significant difference between the two groups (P > 0.05). The CBF was improved in normal-CVR group after treatment (t = 2.409, P < 0.05), and the improvement was not significant in reduced-CVR group (t = 0.648, P > 0.05). Conclusion:13N-ammonia PET/CT cerebral blood flow perfusion imaging combined with methazolamide challenge can be used to evaluate CVR to predict the outcome for patients with cerebral ischemic disease, which is helpful for early intervention.

16.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wpr-864193

RESUMO

Objective:To evaluate the prognostic value of Epstein-Barr virus (EBV)-DNA level in plasma and whole blood in treatment of children with EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH).Methods:Clinical data of 66 children with EBV-HLH, who were admitted to the Hematology and Oncology Center of Beijing Children′s Hospital, Capital Medical University from January 2016 to December 2017 were retrospectively reviewed and analyzed.The data included the dynamic changes of the EBV-DNA level in plasma (P-EBV-DNA) and whole blood (W-EBV-DNA) at the time of admission, 2 and 4 weeks after treatment.P-EBV-DNA was divided into the positive group and the negative group according to the copy number of EBV-DNA, and W-EBV-DNA was divided into the high and the low level group by the receiver operating characteristic curve(ROC); the incidence of poor prognosis was compared between different groups by Chi- Square test; the event-free survival (EFS)was evaluated by the Log- Rank test to identify its prognostic significance. Results:The analysis showed that both P-EBV-DNA and W-EBV-DNA at admission could not be associated with a poor outcome; P-EBV-DNA (≥500 copies/mL) or W-EBV-DNA [>(5.04-5.09)×10 5 copies/mL]after 2 and 4 weeks of treatment could be a good marker of a poor outcome and progression-free survival ( P<0.001). Besides, central nervous system (CNS) involvement ( P=0.025), sever leukopenia(WBC≤3×10 9/L, P=0.031), neutropenia (ANC ≤0.5×10 9/L, P=0.041), albumin reduction (≤26 g/L, P=0.012) and hemoglobin decrease (≤90 g/L, P=0.023) at diagnosis are also associa-ted with worse outcomes.In multivariate analysis, only P-EBV-DNA at 4 th week and CNS involvement were indepen-dent prognostic factors ( HR=7.139, P=0.032 and HR=6.455, P=0.042, respectively). The prognostic value of W-EBV-DNA at different time points and P-EBV-DNA after 2 weeks of treatment had a lower prognostic value. Conclusions:The P-EBV-DNA level after 4 weeks of treatment is a promising risk indicator for early diagnosis of disease and early recognition of poor prognosis in EBV-HLH children, so it provides the guidance for optimal treatment.

17.
Chinese Journal of Neurology ; (12): 935-938, 2020.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wpr-870899

RESUMO

Recently, neuromyelitis optica spectrum disorders (NMOSD) appear to be a multi-organ disorder, however, the involvement of myocardium in NMOSD is extremely rare. In the present article, we present a young girl who manifested bilateral optic neuritis, area postrema syndrome, brainstem syndrome and transverse myelitis, as well as tachycardia, abnormal electrocardiograph, moderate elevation of myocardial biomarker and regional wall movement abnormalities, which confirmed the diagnosis of Takotsubo cardiomyopathy associated with NMOSD. The neurological deficits along with myocardial injury were recovered soon after the administration of intravenous methylprednisolone and intravenous immunogloblin. This is a rare case that should be paid attention and by which can not only broaden the symptom spectrum of NMOSD, but can also provide novel visions for further investigating the mechanism of organs damage.

18.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wpr-879291

RESUMO

Objectives Transmuscular quadratus lumborum block (TQLB) may provide postoperative analgesia in patients undergoing intraperitoneal surgeries. The purpose of this study was to examine the potential efficacy of TQLB among patients undergoing retroperitoneal procedures, such as the laparoscopic partial nephrectomy (LPN). Methods This prospective, randomized, controlled study was conducted from August 2017 to November 2018 at Peking Union Medical College Hospital (Beijing, China). Patients who were scheduled for a LPN, aged 18-70 years old with an ASA physical status score of I - II were randomly assigned to receive either TQLB with 0.6 ml/kg of 0.5% ropivacaine plus general anesthesia (TQLB group) or general anesthesia alone (control group). Patient-controlled intravenous analgesia with morphine was initiated immediately upon surgery completion. The primary outcome was the cumulative consumption of morphine within 8 h after surgery. The secondary outcome included postoperative consumptions of morphine at other time points, pain score at rest and during activity, postoperative nausea and vomitting (PONV), and recovery related parameters. Results Totally 30 patients per group were recruited in the study. The 8 h consumption of morphine was lower in the TQLB group than in the control group (median, 0.023 mg/kg

19.
China Medical Equipment ; (12): 125-128, 2019.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wpr-744956

RESUMO

Objective:To discuss application effect of risk management in medical device management of hospital and its impact on management quality.Methods:9800 medical devices of hospital were selected and divided into routine management group (the corresponding medical devices came from the term of 2014 year to 2015 year, n=4900) and risk management group (the corresponding medical devices came from the term of 2014 year to 2015 year, n=4900) according to different management method.The arrangement and placement of device, the specification and quantity of device, the placement of accessories and the intact situation of widgets of the two groups were recorded and calculated as statistics method.A self-designed questionnaire was adopted to assess these situations included classification error, incompetence, loss of device, undersupply, instrument damage, the timeliness degree of supply, the timeliness degree of withdrawing and quality of packaging in the management process for medical devices.And the management effects of two groups and the corresponding influences of them for curative effect were further compared.Results:The arrangement and placement of medical device, the different specification and quantity of device, the placement of accessories, intact rate of widgets of risk management group were significantly higher than those of■timeliness degree of supply, the timeliness degree of withdrawing and quality of packaging of risk management group post management were significantly higher than those of routine group (t=20.394, t=21.847, t=16.481, P<0.05) .Conclusion:The effect of risk management that is used in medical equipment management of hospital is significant, and it contribute to improve the management quality for medical apparatus and instruments and reduce the error rate of instruments, and it can ensure the safety usage of them in hospital.

20.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wpr-781666

RESUMO

To explore the values of minimal apparent diffusion coefficient(ADC),difference between ratios of apparent diffusion coefficients(ADC),and dynamic contrast-enhanced magnetic resonance imaging(DCE-MRI)in the treatment of breast ductal carcinoma in situ with microinvasion(DCIS-Mi). Totally 27 patients with DCIS-Mi and 31 patients with breast ductal carcinoma in situ(DCIS)were collected in our hospital from October,2016 to June,2018.Philips Ingenia 3.0T superconducting magnetic resonance scanner and dedicated phase-controlled array surface coil were used for breast examinations.ADC and maximum apparent diffusion coefficient(ADC)were selected from multiple regions of interest(ROI)in the apparent diffusion coefficients(ADC)figure,and ADC was calculated.In addition,DCE-MRI characteristics were analyzed. The ADC of DCIS-Mi was significantly lower than that of DCIS[(1.15±0.03)×10 mm /s .(1.34±0.04)×10 mm /s,=-7.192,=0.002],the ADC was significantly higher than that of DCIS[(0.32±0.03)×10 mm /s .(0.18±0.08)×10 mm /s,=-10.228,<0.001],and the early enhancement rate of DCIS-Mi was higher than that of DCIS[159.71(157.82,162.49)% .147.29(143.59,160.22)%,=-3.578,=0.007].The background parenchymal enhancement of DCIS-Mi was moderate,severe,and non-lump-like,mainly segmental,and the internal enhancement was heterogeneous or clustered circular.Multivariate Logistic regression analysis showed that non-internal characteristics of the mass,the edge of the mass,internal enhancement characteristics of the mass,time-intensity curve,early enhancement rate,ADC and ADC were the optimal variables for the diagnosis of DCIS-Mi,and the optimal variables were shown by receiver operating characteristic(ROC)curve analysis:the area under curve,sensitivity and specificity of ADC,ADC,non-tumor internal enhancement,and tumor internal enhancement were higher,with the critical values being 1.12×10 mm /s,0.31×10 mm /s,1.50,and 1.50,respectively. DCE-MRI combined with ADC value(especially ADC,ADC,non-mass internal enhancement,and mass internal enhancement)is helpful in differentiating breast DCIS-Mi and DCIS.


Assuntos
Humanos , Mama , Neoplasias da Mama , Diagnóstico por Imagem , Carcinoma Intraductal não Infiltrante , Diagnóstico por Imagem , Imagem de Difusão por Ressonância Magnética , Imageamento por Ressonância Magnética
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