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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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Enfant , Mâle , Femelle , Humains , Nourrisson , Enfant d'âge préscolaire , Lymphohistiocytose hémophagocytaire/complications , Études rétrospectives , Interleukine-5 , Hépatomégalie/complications , Immunoglobulines par voie veineuse/effets indésirables , Interleukine-8 , Méthylprednisolone , Hormones corticosurrénaliennes , Diarrhée/complications , Exanthème/complicationsRésumé
Objective: To investigate the clinical presentation and genetic characteristics of malignant infantile osteopetrosis. Methods: This was a retrospective case study. Thirty-seven children with malignant infantile osteopetrosis admitted into Beijing Children's Hospital from January 2013 to September 2022 were enrolled in this study. According to the gene mutations, the patients were divided into the CLCN7 group and the TCIRG1 group. Clinical characteristics, laboratory tests, and prognosis were compared between two groups. Wilcoxon test or Fisher exact test were used in inter-group comparison. The survival rate was estimated with the Kaplan-Meier method and the Log-Rank test was used to compare the difference in survival between groups. Results: Among the 37 cases, there were 22 males and 15 females. The age of diagnosis was 0.5 (0.2, 1.0) year. There were 13 patients (35%) and 24 patients (65%) with mutations in CLCN7 and TCIRGI gene respectively. Patients in the CLCN7 group had an older age of diagnosis than those in the TCIRGI group (1.2 (0.4, 3.6) vs. 0.4 (0.2, 0.6) years, Z=-2.60, P=0.008). The levels of serum phosphorus (1.7 (1.3, 1.8) vs. 1.1 (0.8, 1.6) mmol/L, Z=-2.59, P=0.010), creatine kinase isoenzyme (CK-MB) (457 (143, 610) vs. 56 (37, 82) U/L, Z=-3.38, P=0.001) and the level of neutrophils (14.0 (9.9, 18.1) vs. 9.2 (6.7, 11.1) ×109/L, Z=-2.07, P=0.039) at diagnosis were higher in the CLCN7 group than that in the TCIRG1 group. However, the level of D-dimer in the CLCN7 group was lower than that in the TCIRGI group (2.7 (1.0, 3.1) vs. 6.3 (2.5, 9.7) μg/L, Z=2.83, P=0.005). After hematopoietic stem cell transplantation, there was no significant difference in 5-year overall survival rate between the two groups (92.3%±7.4% vs. 83.3%±7.6%, χ²=0.56, P=0.456). Conclusions: TCIRGI gene mutations are more common in children with osteopetrosis. Children with TCIRGI gene mutations have younger age, lower levels of phosphorus, CK-MB, and neutrophils and higher level of D-dimer at the onset. After hematopoietic stem cell transplantation, patients with CLCN7 or TCIRGI gene mutations have similar prognosis.
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Enfant , Mâle , Femelle , Humains , Ostéopétrose/thérapie , Études rétrospectives , Pronostic , Gènes récessifs , Phosphore , Canaux chlorure/génétique , Vacuolar Proton-Translocating ATPases/génétiqueRésumé
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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Mâle , Femelle , Enfant , Humains , Cytarabine/effets indésirables , Chloro-2 désoxyadénosine/effets indésirables , Études rétrospectives , Protocoles de polychimiothérapie antinéoplasique/effets indésirables , Histiocytose à cellules de Langerhans/traitement médicamenteux , PronosticRésumé
Autoimmune encephalitis (AE) is closely related to epileptic seizure, which is a common clinical manifestation or even the only symptom at acute phase of AE. Most patients do not develop seizures after treatment at acute or subacute stages, and these patients are classified as acute symptomatic seizures secondary to AE (ASSAE). Only a minority of patients will eventually develop autoimmune associated epilepsy (AAE). At present, no unified standard for clinical diagnosis is noted between ASSAE and AAE, but some differences exist in definition, pathogenesis, diagnosis and treatment. This paper summarizes the similarities and differences between ASSAE and AAE in the above aspects, aiming at providing help for clinicians to differentiate the diagnosis of the two diseases.
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OBJECTIVE@#To explore the clinical characteristics and genetic etiology of three children with Menkes disease.@*METHODS@#Three children who had presented at the Children's Medical Center, the Affiliated Hospital of Guangdong Medical University from January 2020 to July 2022 were selected as the study subjects. Clinical data of the children were reviewed. Genomic DNA was extracted from peripheral blood samples of the children, their parents and sister of child 1. Whole exome sequencing (WES) was carried out. Candidate variants were verified by Sanger sequencing, copy number variation sequencing (CNV-seq), and bioinformatic analysis.@*RESULTS@#Child 1 was a 1-year-and-4-month male, and children 2 and 3 were monozygotic twin males aged 1-year-and-10-month. The clinical manifestations of the three children have included developmental delay and seizures. WES showed that child 1 has harbored a c.3294+1G>A variant of the ATP7A gene. Sanger sequencing confirmed that his parents and sister did not carry the same variant, suggesting that it was de novo. Children 2 and 3 had carried a c.77266650_77267178del copy number variation. CNV-seq results showed that their mother has carried the same variant. By searching the HGMD, OMIM and ClinVar databases, the c.3294+1G>A was known to be pathogenic. No carrier frequency has been recorded in the 1000 Genomes, ESP, ExAC and gnomAD databases. Based on the Standards and Guidelines for the Interpretation of Sequence Variants: A Joint Consensus Recommendation of the American College of Medical Genetics and Genomics (ACMG), the ATP7A gene c.3294+1G>A variant was predicted to be pathogenic. The c.77266650_77267178del variant has involved exons 8 to 9 of the ATP7A gene. ClinGen online system score for it was 1.8, which was also considered to be pathogenic.@*CONCLUSION@#The c.3294+1G>A and c.77266650_ 77267178del variants of the ATP7A gene probably underlay the Menkes disease in the three children. Above finding has enriched the mutational spectrum of Menkes disease and provided a basis for clinical diagnosis and genetic counseling.
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Humains , Mâle , Nourrisson , Biologie informatique , Copper-transporting ATPases/génétique , Variations de nombre de copies de segment d'ADN , Exons , Maladie de Menkès/génétique , Mutation , Fragments peptidiques , Crises épileptiquesRésumé
This Fructus,study including and aimed to construct a rapid and nondestructive detection flavonoid,model betaine,for and of the content vitamin of(Vit four four quality C).index components Lycium barbarum polysaccharide,of inL ycii rawma total and C Hyperspectral data quantitative of terials modelswere powder developed Lycii using Fructus partial were squares effects collected,regression raw based LSR),on the support content vector the above components,the forest least(P regression compared,(SVR),the and effects random three regression(RFR)were algorithms.also The Four spectral predictive commonly data of the materialsand powder were were applied and of spectral quantitative for models reduction.compared.used were pre-processing screened methods feature to successive pre-process projection the raw algorithm data(SPA),noise competitive Thepre-processed for bands using adaptive reweigh ted sampling howed(CARS),the and maximal effects relevance based and raw minimal materials redundancy and(MRMR)were algorithms Following to optimize multiplicative the models.scatter The correction Based resultss(MS that prediction SPA on feature the powder prediction similar.PLSR C)denoising sproposed and integrated for model,screening the the coefficient bands,determination the effect(R_C~2)of(MSC-SPA-PLSR)coefficient was optimal.of on(R_P~2)thi of of calibration flavonoid,and and of all determination greater prediction0.83,L.barbarum inconte nt prediction of polysaccharide,total mean betaine,of Vit C were than smallest In the compared study,root with mean other prediction content squareserror models of the calibration(RMSEC)residual and deviation root squares was error2.46,prediction2.58,(RMSEP)and were the,and prediction(RPD)2.50,developed3.58,achieve respectively.rapid this the the quality mod el(MSC-SPA-PLSR)fourcomponents based Fructus,on hyperspectral which technology was approach to rapid and effective detection detection of the of Lycii in Lycii provided a new to the and nondestructive of of Fructus.
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Spectroscopie proche infrarouge/méthodes , Bétaïne , Poudres , Méthode des moindres carrés , Algorithmes , FlavonoïdesRésumé
Objective:To evaluate the effects of peroneal osteotomy combined with arthroscopic knee debridement on perioperative indexes, X-ray indexes, Japanese Orthopaedic Association Assessment score (JOA score), American Hospital for Special Surgery knee score (HSS score) and complication rate of patients with knee osteoarthritis.Methods:A prospective research method was adopted. A total of 110 patients with knee osteoarthritis who were admitted to Chengde Central Hospital from April 2016 to April 2021 were selected as the research objects and divided into the control group (55 cases), the combined group (55 cases) by random number table method. The control group underwent arthroscopic debridement of the knee joint. On the basis of the control group, the combined group was combined with fibula osteotomy. The control group and the combined group were followed up for 6 months after operation. The perioperative indicators, score of JOA before and after 1 month of treatment, X-ray indicators, score of HSS before and after 6 months of treatment, and the incidence of complications during the follow-up period were compared between the control group and the combined group.Results:The operation time of the combined group was longer than that of the control group: (84.36 ± 14.64) min vs. (75.88 ± 12.86) min; compared with before operation, 1 month after operation, the scores of swelling, pain while up and down stairs, walking pain, joint range of motion were increased in the control group and the combined group, and the combined group was higher than the control group: (8.35 ± 0.73) points vs. (6.74 ± 0.67) points, (15.05 ± 1.74) points vs. (13.96±1.66) points, (21.75 ± 2.07) points vs. (18.58 ± 1.73) points, (27.59 ± 3.25) points vs. (25.74 ± 2.83) points; the femoral tibial angle and the range of motion of the knee joint in the control group and the combined group were increased, and the combined group was greater than the control group: (1.94 ± 0.60)° vs. (3.02 ± 0.67)°; the scores of stability, flexion deformity, muscle strength, range of motion, function, and pain in the control group and the combined group increased, and the combined group was higher than the control group: (8.62 ± 0.86) points vs. (6.89 ± 0.78) points, (7.86 ± 0.96) points vs. (6.27 ± 0.68) points, (7.79 ± 0.62) points vs. (6.95 ± 0.57) points, (14.95 ± 2.60) points vs. (12.48 ± 2.33) points, (17.46 ± 2.21) points vs. (14.59 ± 1.76) points, (24.83 ± 3.25) points vs. (18.59 ± 2.57) points, the difference was statistically significant ( P<0.05). During the following up period, there were no significant differences in the incidences of osteofascial compartment syndrome, neurovascular injury, intraarticular adhesion and incision infection between the two groups ( P>0.05). Conclusions:The operation time of fibular osteotomy combined with arthroscopic debridement of knee joint in patients with knee osteoarthritis was longer, but fibular osteotomy combined with arthroscopic debridement of the knee joint can reduce the valgus of the patient and improve the range of motion of the joint. It could effectively reduce the degree of pain and improve the function of knee joint, and the safety was good, with good therapeutic effect.
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OBJECTIVE@#To compare the clinical therapeutic effect between electroacupuncture at "four points of sacral region" and transurethral Erbium laser in treatment of moderate to severe stress urinary incontinence after radical prostatectomy.@*METHODS@#A total of 68 patients of moderate to severe stress urinary incontinence after radical prostatectomy were divided into an electroacupuncture group (34 cases) and an Erbium laser group (34 cases, 3 cases dropped off) according to the settings. In the electroacupuncture group, electroacupuncture was applied at "four points of sacral region", i.e. points of 0.5 cun beside bilateral sacrococcygeal joints and bilateral Huiyang (BL 35), with continuous wave, 2 Hz in frequency, 60 min each time, once every other day, 3 times a week, 12 times as one course of treatment. In the Erbium laser group, transurethral Erbium laser technology was given, once every 4 weeks as one course of treatment. Both groups were treated for 5 courses. The scores of the International Consultation on Incontinence questionnaire-short form (ICI-Q-SF) and the incontinence quality of life questionnaire (I-QOL) were observed before treatment, after each course of treatment and in follow-up after 1 and 2 months of treatment completion, respectively, and the clinical efficacy was evaluated after treatment in the two groups.@*RESULTS@#Compared with those before treatment, the ICI-Q-SF scores were decreased while the I-QOL scores were increased after 5 courses of treatment and in follow-up after 1, 2 months of treatment completion in the two groups (P<0.01). The ICI-Q-SF score in follow-up after 2 months of treatment completion were higher than that after 5 courses of treatment in the Erbium laser group (P<0.05). After 3, 4, 5 courses of treatment and in follow-up after 1 and 2 months of treatment completion, the ICI-Q-SF scores in the electroacupuncture group were lower than those in the Erbium laser group (P<0.05, P<0.01); after 2, 3, 4, 5 courses of treatment and in follow-up after 1 and 2 months of treatment completion, the I-QOL scores in the electroacupuncture group were higher than those in the Erbium laser group (P<0.01). The change ranges of ICI-Q-SF score and I-QOL score between before treatment and after each course of treatment in the electroacupuncture group were lager than those in the Erbium laser group (P<0.01, P<0.05). The total effective rate was 61.8% (21/34) in the electroacupuncture group, which was superior to 19.4 (6/31) in the Erbium laser group (P<0.01).@*CONCLUSION@#Both electroacupuncture at "four points of sacral region" and transurethral Erbium laser can improve the clinical symptoms and the quality of life in patients of moderate to severe stress urinary incontinence after radical prostatectomy. The short-term efficacy and long-term efficacy of electroacupuncture are superior to the Erbium laser technology.
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Mâle , Humains , Qualité de vie , Incontinence urinaire d'effort/thérapie , Région sacrococcygienne , Électroacupuncture , Erbium , Prostatectomie/effets indésirablesRésumé
Immunoglobulin (IgG) glycosylation affects the effector functions of IgG in a myriad of biological processes and has been closely associated with numerous autoimmune diseases, including systemic lupus erythematosus (SLE), thus underlining the pathogenic role of glycosylation aberration in autoimmunity. This study aims to explore the relationship between IgG sialylation patterns and lupus pregnancy. Relative to that in serum samples from the control cohort, IgG sialylation level was aberrantly downregulated in serum samples from the SLE cohort at four stages (from preconception to the third trimester of pregnancy) and was significantly associated with lupus activity and fetal loss during lupus pregnancy. The type I interferon signature of pregnant patients with SLE was negatively correlated with the level of IgG sialylation. The lack of sialylation dampened the ability of IgG to suppress the functions of plasmacytoid dendritic cells (pDCs). RNA-seq analysis further revealed that the expression of genes associated with the spleen tyrosine kinase (SYK) signaling pathway significantly differed between IgG- and deSia-IgG-treated pDCs. This finding was confirmed by the attenuation of the ability to phosphorylate SYK and BLNK in deSia-IgG. Finally, the coculture of pDCs isolated from pregnant patients with SLE with IgG/deSia-IgG demonstrated the sialylation-dependent anti-inflammatory function of IgG. Our findings suggested that IgG influences lupus activity through regulating pDCs function via the modulation of the SYK pathway in a sialic acid-dependent manner.
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Humains , Grossesse , Femelle , Lupus érythémateux disséminé/anatomopathologie , Transduction du signal , Acide N-acétyl-neuraminique/métabolisme , Immunoglobuline G , Cellules dendritiques/anatomopathologieRésumé
Aim To evaluate the protective effect of α-asarone on microglials with cerebral ischemia/reperfusion injury by measuring the expression of polar transformation and related inflammatory proteins in BV2 cells in vitro and its mechanisms.Methods The cerebral ischemia/reperfusion injury BV2 cells were pretreated by α-asarone in vitro and simulated by OGD/R model.The effect of α-asarone on the viability of damaged cells in OGD/R model was determined by CCK-8; the morphological changes of cells were observed to analyze the general morphology of cells; the levels of proinflammatory factor IL-1β, IL-18 and anti-inflammatory factor IL-10, IL-4, and ROS activity secreted by BV2 cells were detected by ELISA; the protein expressions of TGF-β, TNF-α and inflammatory related protein NLRP3, caspase 1, p-NF-κB were detected by Western blot.Results The results of in vitro experiments were as follows: the activity of damaged cells in OGD/R model was significantly increased by α-asarone, with the increase of administration dose, the cells in the low, medium and high dose groups of α-asarone decreased, and the "amoeba-like" cells and the cell body were gradually became stereoscopic and full.From the results of cell morphology, it could be seen that α-asarone had a certain proliferative effect on normal cells; the release was significantly reduced of proinflammatory factor IL-1β, IL-18 and TNF-α in OGD/R injured BV2 cells pretreated with α-asarone, also increased the release of IL-10, IL-4 and TGF-β, with a dose-effect relationship, and the high dose(16 μmol·L-1)was the best; the expressions of inflammatory related protein NLRP3, caspase 1, NF-κB and ROS activity in injured cells of OGD/R model were significantly reduced after pretreatment with α-asarone.Conclusions α-asarone has a significant protective effect on cerebral ischemia/reperfusion injury, mainly by regulating ROS activity and inhibiting phosphorylation of NF-κB, in order to reduce the excessive activation of NLRP3 inflammatory corpuscles reducing the secretion of proinflammatory factor IL-1β and IL-18, promoting the secretion of anti-inflammatory factor IL-10 and IL-4, so as to protect cerebral ischemia/reperfusion injury by anti-inflammatory reaction.
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OBJECTIVE@#To assess the efficacy of GelMA hydrogel loaded with bone marrow stem cell-derived exosomes for repairing injured rat knee articular cartilage.@*METHODS@#The supernatant of cultured bone marrow stem cells was subjected to ultracentrifugation separate and extract the exosomes, which were characterized by transmission electron microscopy, particle size analysis and Western blotting of the surface markers. The changes in rheology and electron microscopic features of GelMA hydrogel were examined after loading the exosomes. We assessed exosome release from the hydrogel was detected by BCA protein detection method, and labeled the exosomes with PKH26 red fluorescent dye to observe their phagocytosis by RAW264.7 cells. The effects of the exosomes alone, unloaded hydrogel, and exosome-loaded hydrogel on the polarization of RAW264.7 cells were detected by q-PCR and immunofluorescence assay. We further tested the effect of the exosome-loaded hydrogel on cartilage repair in a Transwell co-culture cell model of RAW264.7 cells and chondrocytes in a rat model of knee cartilage injury using q-PCR and immunofluorescence assay and HE and Masson staining.@*RESULTS@#GelMA hydrogel loaded with exosomes significantly promoted M2-type polarization of RAW264.7 cells (P < 0.05). In the Transwell co-culture model, the exosome-loaded GelMA hydrogel significantly promoted the repair of injured chondrocytes by regulating RAW264.7 cell transformation from M1 to M2 (P < 0.05). HE and Masson staining showed that the exosome-loaded hydrogel obviously promoted cartilage repair in the rat models damage.@*CONCLUSION@#GelMA hydrogel loaded with bone marrow stem cell-derived exosomes can significantly promote the repair of cartilage damage in rats by improving the immune microenvironment.
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Animaux , Rats , Cellules de la moelle osseuse , Cartilage , Chondrocytes , Exosomes , Hydrogels/métabolismeRésumé
OBJECTIVE@#To investigate the prognostic significance of dynamic detection of minimal residual disease (MRD) in patients with acute myeloid leukemia (AML) by 8-color flow cytometry.@*METHODS@#MRD of 282 AML patients who achieved remission after initial therapy was detected by 8-color flow cytometry. MRD threshold for predicting recurrence was determined by receiver operating characteristic (ROC) curve, and time from MRD-positive to clinical recurrence was analyzed. The differences in overall survival (OS) time and relapse-free survival (RFS) time of patients with different MRD-changes were compared, and the related factors of recurrence in patients with MRD-negative were analyzed by univariate and logistic regression analysis.@*RESULTS@#ROC curve determined that the MFC-MRD threshold for predicting the recurrence of AML was 0.105%, and the recurrence rate of MRD-positive patients was significantly higher than that of MRD-negative patients [52.45% (75/143 cases) vs 35.97% (50/139 cases), P=0.005]. The patients in MRD persistent positive group and negative to positive group recurred earlier than those in positive to negative group and negative-positive fluctuation group (P<0.005). Survival analysis showed that OS and RFS time of patients with MRD persistent positive were significantly shorter than those of patients with MRD persistent negative, positive to negative, and negative-positive fluctuation (P<0.005). There was no significant difference in OS and RFS between MRD negative to positive group and MRD persistent positive group (P>0.005), either between MRD persistent negative group and MRD positive to negative group (P>0.005). Among 139 MRD-negative patients, 50 recurred. Univariate and logistic regression analysis showed that the risk of recurrence increased with the increase of white blood cells level (95%CI: 1.000-1.013, P=0.045). The risk of recurrence in patients without hematopoietic stem cell transplantation (HSCT) was 9.694 times higher than that in patients who received HSCT (95%CI: 1.720-54.651, P=0.010), and in the high-risk group was 5.848 times higher than that in the low-risk group (95%CI: 1.418-24.121, P=0.015).@*CONCLUSION@#The prognosis of AML patients with different MRD changes is significantly different. No matter MRD-positive or MRD-negative at the initial remission, dynamic detection of MRD after treatment is more helpful to accurately guide treatment.
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Humains , Cytométrie en flux , Transplantation de cellules souches hématopoïétiques , Leucémie aigüe myéloïde/traitement médicamenteux , Maladie résiduelle/diagnostic , Pronostic , Récidive , Transplantation homologueRésumé
Objective To measure the anatomical morphology of coracoclavicular ligament based on fresh cadavers, and to provide anatomical basis for anatomical reconstruction of coracoclavicular ligament. Methods A total of 52 the acromioclavicular joints (fresh body specimen) was dissected, and the anatomical characteristics of coracoclavicular ligament were observed by dissecting acromioclavicular joint specimens. The anatomic data of which the length of the conoid ligament (QR), the length of the trapezoid ligament (ST) ; the distance from the conoid ligament attachment on coracoid to coracoid tip (RV), the distance from the trapezoid ligament attachment on coracoid to coracoid tip (TV), the distance form conoid ligament attachment on the clavicular to acromioclavicular joint (QU), the distance form the trapezoid ligament attachment on the clavicular to acromioclavicular joint (SU), the distance from supraclavicular plane to subcoracoid plane (WX) were measured ; and the mean diameter of the trapezoid ligament attachment on the clavicular (ā), the mean diameter of the conoid ligament attachment on the clavicular(b¯), the mean diameter of the trapezoid ligament attachment on the coracoid (c¯), the mean diameter of the conoid ligament attachment on the coracoid (d¯) were calculation. The measurement result underwent statistical analysis. Results The minimum diameter of the trapezoid ligament attachment on the clavicular and coracoid on the left and right sides, respectively. There were no significant differences in those anatomical morphology of the coracoclavicular ligament (P>0.05). The anatomical morphology of the diameter of coracoclavicular ligament attachment on the male and female had no significant difference (P>0.05). The maximum length of the conoid ligament was (14.19±2. 43/15. 87±2. 99) mm on the left and right sides, respectively. The distance from the conoid ligament attachment on coracoid to coracoid tip was ( 36. 66 ± 4. 25/33. 61 ± 3. 45 ) mm on the left and right sides, respectively. The mean diameter of the conoid ligament attachment on the clavicular and coracoid was (11.95± 1.43/ 11.23± 1.12)mm and (9.20± 1.60/7.90±0.76) mm on the left and right sides, respectively. There were significant differences in those anatomical morphology of the coracoclavicular ligament(P 0.05). The anatomical morphology of the coracoclavicular ligament on the male and female had no significant difference (P>0.05). Conclusion The comprehensive measurement of anatomical morphology of coracoclavicular ligament can provide an anatomical basis for shoulder joint diseases related to coracoclavicular ligament injury. It will help surgical staff perform a complete anatomic reconstruction of coracoclavicular ligaments for the surgical treatment of dislocation of the acromioclavicular joint.
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Background: Visceral hypersensitivity is considered as a key pathophysiological mechanism involved in functional gastrointestinal disorders (FGIDs). Visceral nociception and hyperalgesia is existed extensively following exposure to post-traumatic stress disorder (PTSD), however, its molecular mechanism in intestinal tract is unclear. Aims: To explore the potential role of N-Myc downstream-regulated gene 2 (NDRG2) in intestinal tract for mediating visceral hypersensitivity following exposure to PTSD. Methods: PTSD model was established by single prolonged stress (SPS). SD rats were divided into normal control group, CTX group, PTSD group and PTSD+CTX group. Mice were divided into normal control group, PTSD group, NDRG2
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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.
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Objective:To explore the effects of childhood trauma on depression of college students and the role of depletion sensitivity and self-control in it.Methods:A total of 1 678 freshmen and sophomores from a medical university in Guangzhou were investigated by childhood trauma questionnaire(CTQ), Beck depression inventory-Ⅱ(BDI-Ⅱ), depletion sensitivity scale(DSS) and self-control scale(SCS) through random cluster sampling. The mediating effect of depletion sensitivity and moderating effect of self-control were examined by SPSS 26.0 macro program PROCESS V2.13.Results:(1)The total scores of CTQ and BDI-Ⅱ in college students were (34.56±7.03) and ( M( QR): 5.00(7.00)) respectively, and the scores of DSS and SCS were (41.78±12.21) and (40.02±8.15) respectively. (2)Childhood trauma was positively correlated with depletion sensitivity ( r= 0.20, P<0.01) and depletion sensitivity was positively correlated with depressive symptoms ( r=0.48, P<0.01). There was a negative correlation between self-control and depressive symptoms ( r=-0.49, P<0.01). (3)Depletion sensitivity partly mediates the relationship between childhood trauma and depressive symptoms. The direct effect (effect size=0.28) and mediating effect (effect size=0.07) accounted for 80% and 20% of the total effect (effect size=0.35), respectively. (4)Self-control moderated the path between depletion sensitivity and depressive symptoms. Conclusion:Childhood trauma can indirectly affect college students′ depressive symptoms through depletion sensitivity, and the partial mediatory role of depletion sensitivity is also moderated by self-control.
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Objective To investigate the effect of alkaline phosphatase liver / bone / kidney (ALPL) overexpression on ventricular remodeling after acute myocardial infarction (MI). Methods Thirty six 8-week-old male C57BL / 6 J mice were randomly divided into sham group(sham), MI group (MI+Adv-EGFP) and ALPL overexpression group (MI+ Adv-ALPL) with 12 mice in each group. Two weeks after MI, cardiac function of mice was detected by echocardiography, pathological changes was detected by HE staining. ALPL mRNA expression in mouse heart was detected by Real-time PCR. The levels of ALPL, α- smooth muscle actin (α-SMA),collagen I (ColI) and collagen III (ColIII) protein were detected by Western blotting. The collagen volume fraction (CVF) and the ratio of Col I / Col III were measured by polarizing method with Sirius red staining. Results Compared with MI+Adv-EGFP group, the left ventricular ejection fraction (LVEF) (%) and left ventricular fractional shortening (LVFS) (%) of the heart in MI+Adv-ALPL group were reduced significantly (P < 0. 05). Hearts in the ALPL overexpression group showed myocardial fiber broken, and disordered and fibrous scars were obvious in myocardial infarction area. The expression of ALPL and the expression of α-SMA, ColI and ColIII protein in MI+Adv-ALPL group increased significantly (P<0. 05), and the proportion of ColI / ColIII in MI+Adv-ALPL group increased significantly (P<0. 05).Compared with MI+Adv-EGFP group, the CVF and the proportion of the ratio of ColI / ColIII in the MI+Adv-ALPL group increased significantly (P<0. 05). Conclusion ALPL overexpression can promote the ventricular pathological remodeling after acute myocardial infarction in mice.
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OBJECTIVE@#To investigate the killing effect of NK-92MI cells modified by chimeric antigen receptor (CD7-CAR) and specifically targeting CD7 to CD7 hematological malignant cells.@*METHODS@#Three types of hematological malignant tumor cells, including 5 cases of CD7 acute T-lymphoblastic leukemia (T-ALL), 10 cases of acute myeloid leukemia (AML) and 6 cases of T-cell lymphoma were collected, centrifuged, cultured and used to detect the expression levels of tumor cell surface targets; 7-AAD, CD56-APC, CD3-FITC, IgG Fc-PE flow cytometry were used to detected the transfection efficiency of NK-92MI and CD7-CAR-NK-92MI cells, killing efficiencies of CD7-CAR-NK-92MI cells to CD7 hematological tumor cells in vitro were determined by flow cytometry using PE Annexin V Apoptosis Detection Kit. Secretion differences of NK-92MI and CD7-CAR-NK-92MI cytokines interleukin (IL)-2, interferon (IFN)-γ, and granzyme B detection were estimated by using CBA kit.@*RESULTS@#The killing efficiencies of CD7-CAR-modified NK-92MI cells to CD7 T-ALL, AML, T-cell lymphoma tumor cells were significantly higher than those of NK-92MI cells without genetical modification. The difference showed statistically significant (P<0.05). The level of IFN-γ and granzyme B were significantly increased among cytokines secreted by CD7-CAR-modified NK-92MI cells as compared with those of NK-92MI cells without genetical modification (P<0.05) .@*CONCLUSION@#CD7-CAR-modified NK-92MI cells have significantly improved killing efficiency against CD7 T-ALL, AML and T lymphoma cells, and shows specific targeting effects, which provides a clinical basis for the treatment of CD7 hematological malignancies.
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Humains , Lignée cellulaire tumorale , Cellules tueuses naturelles , Leucémie aigüe myéloïde , Récepteurs chimériques pour l'antigène , Lymphocytes TRésumé
Objective:To investigate the correlation between EOS level and hormone therapy effect and prognosis of patients with acute exacerbation of chronic obstructive pulmonary disease (AECOPD).Methods:From January 2016 to June 2018, 120 patients with AECOPD were selected in the Fifth People's Hospital of Datong.According to EOS levels, all patients were divided into two groups, including EOS ≥2% group(56 cases) and EOS<2% group(64 cases). The general clinical data and treatment related indicators of patients with different EOS levels were compared, and the clinical value of EOS level in predicting the risk of severe AECOPD recurrence and death of patients were evaluated.Results:The levels of WBC, N%, NLR and CRP of the EOS≥2% group were significantly lower than those of the EOS<2% group[(6.89±1.16)×10 9/L, (69.08±12.79)%, (3.54±1.16), (5.30±1.18)mg/L vs.(8.45±1.85)×10 9/L, (76.42±16.58)%, (6.08±1.42), (7.43±1.77)mg/L]( t=5.27, 4.81, 4.65, 2.58, all P<0.05). The used time of antibiotics of the EOS≥2% group was significantly shorter than that of the EOS<2% group[8.0(6.0, 10.0)d vs.9.0(7.0, 11.0)d]( U=2.46, P<0.05). The time of hormone therapy and hospitalization time of the EOS≥2% group were significantly shorter than those of the EOS<2% group[9.0(7.0, 11.0)d, 10.0(9.0, 12.0)d vs.11.0(7.0, 13.0)d, 12.0(10.0, 13.0)d]( U=2.79, 2.56, all P<0.05). The proportion of CAT score decreased ≥2 points at 7d after treatment of the EOS≥2% group was significantly higher than that of the EOS<2% group[86.84% vs.68.18%](χ 2=2.84, P<0.05). Logistic regression analysis showed that EOS≥2% was the independent risk factor for severe AECOPD recurrence and death( OR=2.84, 95% CI: 1.49~5.03, P<0.05). There was no relationship between EOS level and death risk ( P>0.05). Conclusion:Serum EOS level can independently predict the clinical effect of hormone therapy and prognosis in patients with AECOPD, and clinicians can make more reasonable clinical treatment plan accordingly.
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Chuanxiong Chatiaosan was first recorded in Taiping Huimin Heji Jufang, which was made up of 8 herbs, including Chuanxiong Rhizoma, Menthae Haplocalycis Herba, Asari Radix et Rhizoma, Schizonepetae Herba, Saposhnikoviae Radix, Angelicae Dahuricae Radix, Notopterygii Rhizoma et Radix and Glycyrrhizae Radix et Rhizoma. This prescription mainly contains a variety of alkaloids, flavonoids, phenylpropanoids, volatile oils and other compounds, which play the biological activity of promoting blood circulation and relieving pain. Modern pharmacological studies have confirmed that Chuanxiong Chatiaosan can reduce blood viscosity, improve cerebral circulation, and has central analgesic effect to treat migraine effectively. However, the mechanism for treating migraine of this prescription is still unclear. The author elaborated the research status of Chuanxiong Chatiaosan from four aspects, including quality control method, chemical composition, preparation technology and pharmacological research, hoping to provide references for rational clinical application and explanation of pharmacological mechanism of this prescription.