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Background: This study investigates the effects of administering intravenous esketamine at a dose of 0.25 mg/kg to pregnant patients receiving epidural anesthesia for emergency cesarean section on both maternal and neonatal outcomes., Methods: Medical records of pregnant patients transitioning from labor analgesia to epidural anesthesia for emergency cesarean sections between January 2020 and December 2022 were analyzed. The patients were categorized based on whether they received esketamine infusions during the incision-to-delivery interval. The variables compared between the groups included hemodynamic parameters, perioperative and postoperative adverse reactions, and neonatal outcomes (gender, weight, Apgar scores at 1 and 5 min, need for neonatal intensive care, and umbilical artery/vein blood gas analysis)., Results: For maternal outcomes, the systolic blood pressure (SBP) in the esketamine group showed a significant increase at 5 and 10 min' post-administration, and the diastolic blood pressure (DBP) significantly increased at 5 min, compared to the control group ( p  < 0.01). No significant differences were observed in heart rate (HR) and oxygen saturation (SpO 2 ) at any time point ( p  > 0.05). The esketamine group experienced a significant rise in the incidence of arrhythmias, dizziness, and nystagmus during the perioperative period, a notable decrease in hypotension incidence, and an increase in postoperative nausea and dizziness. Regarding neonatal outcomes, there were no significant differences in gender, weight, Apgar scores ≤7 at 1 and 5 min, and the need for neonatal intensive care. However, the pH level in the umbilical artery blood of the esketamine group was significantly higher. The levels of PCO 2 and PO 2 in umbilical artery and venous blood did not show significant differences between the groups., Conclusions: In pregnant women undergoing emergency cesarean section, intravenous administration of 0.25 mg/kg esketamine is correlated with favorable maternal and neonatal outcomes.

Objective: To determine the expression and diagnostic value of peripheral blood lymphocytes and functional activation status in non-Hodgkin lymphoma with hemophagocytic lymphohistiocytosis (NHL-HLH) . Methods: We retrospectively analyzed clinical data from 30 newly diagnosed NHL-HLH patients admitted to Jiangsu Province Hospital from September 2022 to September 2023. We assessed peripheral blood lymphocytes and activation status by flow cytometry. Forty newly diagnosed patients with NHL who received treatment at our hospital during the same period and had lymphocyte and functional activation indexes were selected as the control group. The differences in relative and absolute lymphocyte counts and functional activation indexes between the two groups were compared. The optimal cutoff values for continuous variables were calculated from the receiver operating characteristic curve and logistic regression analysis was used to evaluate the risk factors in NHL patients with HLH. Results: A total of 30 NHL-HLH patients were evaluated, including 12 T-cell lymphoma and 18 B-cell lymphoma patients. Forty individuals were in the control group, which included 19 T-cell lymphoma and 21 B-cell lymphoma patients. The absolute counts of CD3(+) T, CD4(+) T, CD8(+) T, and NK cells, along with the relative count of NK cells, were significantly lower in the HLH group compared with that in the control group (all P values<0.01) . The expression of CD38 and HLA-DR on CD8(+) T-cell activated subgroups was significantly higher in the NHL-HLH group compared with that in the control group (CD8(+)CD38(+)/CD8(+) T expression median: 57.4% vs 21.5%, P <0.001; CD8(+)CD38(+)/CD8(+) T expression median: 49.7% vs 33.5%, P =0.028, respectively) . In addition, CD28 expression on CD4(+) and CD8(+) T cells was significantly higher in NHL-HLH patients ( P <0.01) . ROC curve and multivariate logistic regression analyses revealed that absolute NK cell count ≤72.0 cells/μl, CD4(+)CD28(+)/CD4(+) T >94.2%, and CD8(+)CD28(+)/CD8(+) T >38.4% were risk factors for predicting the occurrence of NHL-HLH patients. The sensitivity and specificity of the regression model were 86.7% and 86.1%, respectively, with an area under the curve of 0.94 ( P <0.001) . Conclusions: In NHL patients with HLH, there was a significant reduction in the absolute number of peripheral blood lymphocyte subpopulations, whereas T-cell function was notably activated. Specifically, absolute counts of NK cells ≤72.0 cells/μl, CD4(+)CD28(+)/CD4(+) T >94.2%, and CD8(+)CD28(+)/CD8(+) T >38.4% were identified as risk factors for predicting the development of NHL-HLH patients. This will assist in early clinical diagnosis and treatment.

Objective: To investigate the effect of pre-treatment plasma Epstein-Barr virus (EBV) DNA copy number on the clinical features and prognosis of patients with adult secondary hemophagocytic lymphohistiocytosis(sHLH)., Methods: The clinical characteristics, survival rate, and prognostic factors of 171 patients with adult sHLH treated at Jiangsu Province Hospital from June 2017 to January 2022 were retrospectively analyzed in this study. Patients were divided into three groups, including the EBV DNA-negative group(<5.0×10 2 copies/ml), lower EBV-DNA loads group(5.0×10 2 -8.51×10 4 copies/ml), and higher EBV-DNA loads group(＞8.51×10 4 copies/ml), according to pre-treatment plasma EBV-DNA copy number. Cox regression model was established for screening prognostic factors. Adult sHLH survival prediction model was constructed and realized through the nomogram based on EBV-DNA load after adjusted the factors affecting survival of etiology and treatment strategy.Concordance index (C-index) and calibration curves were calculated to verify model predictive and discriminatory capacity., Results: Among 171 adult sHLH patients, 84 patients were not infected with EBV (EBV DNA-negative group), and 87 with EBV (EBV DNA-positive group, 48 lower EBV-DNA loads group and 39 higher EBV-DNA loads group). Consistent elevations in the levels of liver enzymes (ALT and AST), LDH, TG, β 2 -microglobulin and ferritin across the increasing of EBV-DNA load (all P <0.05), while the levels of fibrinogen decrease ( P <0.001). The median follow-up time was 52 days (range 20-230 days), and 123 patients died. The overall survival (OS) rate of patients in EBV DNA-positive group was lower than that in EBV DNA-negative group (median OS: 40 days vs 118 days, P <0.001). Higher EBV-DNA loads had worse OS (median OS: 24 days vs 45 days vs 118 days, P <0.0001 for trend) compared to lower EBV-DNA loads and EBV DNA-negative group. Multivariate Cox analysis revealed that higher EBV-DNA loads ( P =0.005), fibrinogen≤1.5 g/L ( P ＝0.012), ferritin ( P ＝0.041), associated lymphoma ( P ＝0.002), and anti-tumor based strategy ( P ＝0.001) were independent prognostic factors for OS. The C-indexes of 30 day, 90 days, 365 days survival rate were all greater than 0.8 of the nomogram model and calibration curves provided credibility to their predictive capability. Subgroup analysis showed that patients with higher EBV-DNA loads had a significantly worse prognosis in adult sHLH who were women, ferritin＞5 000 μg/L, β 2 -microglobulin＞7.4 mmol/L and regardless of age, etiologies, HScore points., Conclusion: The EBV-DNA load is a strong and independent predictor for survival in patients with sHLH. The prognostic nomogram based on EBV-DNA loads was dependable and provides a visual tool for evaluating the survival of adult sHLH.

Objective: To investigate the expression and clinical significance of soluble Fas (sFas) and sFasL in patients with secondary hemophagocytic lymphohistiocytosis (sHLH)., Methods: From September 2015 to December 2020, 86 sHLH patients who met the HLH2004 diagnostic criteria were collected. They were divided into 55 cases in the MAHLH group and 31 cases in the NonMAHLH group according to the etiology. Thirty healthy persons were chosen as the normal control group, and 20 patients with systemic lupus erythematosus (SLE) were chosen as the disease control group. The expression levels of sFas and sFasL in the serum of patients with each group were detected by ELISA, and the clinical data were collected for statistical analysis. The significance of sFas and sFasL in sHLH was analyzed by ROC curve., Results: Serum levels of sFas and sFasL in patients with newly diagnosed sHLH were significantly higher than those in disease control group and normal control group ( P <0.01). The levels of sFas and sFasL in MAHLH group were significantly higher than those in nonMAHLH (infection related HLH and autoimmune disease related HLH) group ( P <0.01). The serum levels of sFas and sFasL in 17 newly treated patients with sHLH (17/86) after treatment were significantly lower than those before treatment ( P <0.01). The serum sFas level in newly diagnosed sHLH patients was positively correlated with SF( r =0.35), sCD25( r =0.79) and sFasL( r =0.73). The serum sFasL level was positively correlated with SF( r =0.39), sCD25( r =0.64) and sFas( r =0.73). Compared with the NonMAHLH group, the area under the ROC curve was 0.707 (95% CI : 0.593-0.821) (P=0.0015). The optimal critical value for diagnosing MAHLH by sFas level was 12 743 pg/ml, and the sensitivity and specificity were 70.9% and 71% respectively. Compared with the NonMAHLH group, the area under the ROC curve was 0.765(95% CI : 0.659-0.87)( P <0.01). The median OS time of sFas high expression group (≥16798.5 pg/ml) and sFasL high expression group (≥4 785 pg/ml) was significantly shorter than that of the low expression group ( P <0.001)., Conclusion: Serum levels of sFas and sFasL can be used for the early diagnosis and differential diagnosis of sHLH disease, and are the factor related to the poor prognosis of sHLH.

Objective: The present study aims to (1) analyze the clinical characteristics and related influencing factors of knee bone infarction in systemic lupus erythematosus (SLE) and (2) improve the understanding of SLE complicated with knee bone infarction., Methods: The data of patients with SLE complicated with knee bone infarction were retrospectively analysed; patients with SLE during the same period who matched in age, gender, and disease duration were selected as control subjects, with a 1 : 1 ratio with the SLE group. The clinical data were collected to analyze the risk factors for SLE complicated with knee bone infarction., Results: In a total of 36 (6.4%) of 563 patients aged 19-33 (25.8 ± 4.8) years who had SLE during the same period, the disease was complicated with knee bone infarction. The diagnosis of knee bone infarction was made at an SLE duration of 7-65 (26.2 ± 15.7) months. During the SLE course, knee bone infarction occurred within 1 year in 6 cases (16.7%), within 1-5 years in 28 cases (77.8%), and in >5 years in 2 cases (5.6%). Raynaud's phenomenon incidence and anti-nRNP antibody positivity were significantly higher in the knee bone infarction group than in the control group ( P < 0.01 and P < 0.05, respectively). The cumulative glucocorticoid dose at 1, 3, and 6 months was significantly higher in the knee bone infarction group than in the control group ( P < 0.05). SLE complicated with knee necrosis had a statistically significant rank correlation with Raynaud's phenomenon ( r  = 0.445, P < 0.001), anti-nRNP antibody ( r  = 0.309, P =0.008), and renal injury ( r  = 0.252, P =0.032). The multivariate analysis of SLE complicated with knee bone infarction showed that Raynaud's phenomenon was an independent influencing factor for the complicated knee bone infarction in SLE patients (OR = 4.938, P =0.004), and the probability of SLE complicated with knee bone infarction in Raynaud's phenomenon positive patients was 4.938 times that of Raynaud's phenomenon negative patients., Conclusions: The risk of knee bone infarction was relatively high in patients with SLE within a 5-year disease course and in young patients. The risk factors were Raynaud's phenomenon, anti-nRNP antibody positivity, and early high-dose glucocorticoid therapy., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2022 Gui-Qi Zhu et al.)

Objectives: To investigate the diversity of peripheral blood T cell receptor (TCR) β chain complementarity-determining region 3 (CDR3) based on immune repertoire sequencing in neonates with sepsis and the possible pathogenesis of neonatal sepsis., Methods: A total of 12 neonates with sepsis were enrolled as the case group, and 9 healthy full-term infants, matched for gestational age, birth weight, and age, were enrolled as the control group. Omega nucleic acid purification kit (SQ blood DNA Kit II) was used to extract DNA from peripheral blood samples, TCR β chain CDR3 was amplified by multiplex PCR, and then high-throughput sequencing was performed for the products to analyze the diversity of TCR β chain CDR3 and the difference in expression., Results: The length and type of TCR β chain CDR3 were similar between the case and control groups, and Gaussian distribution was observed in both groups. With D50 and Shannon-Wiener index as the evaluation indices for diversity, the case group had a significantly lower diversity of TCR β chain CDR3 than the control group ( P <0.05). The frequency of 48 genes in TCR β chain V segment was compared, and the results showed that compared with the control group, the case group had significantly higher frequencies of TRBV10-3 , TRBV2 , and TRBV20-1 ( P <0.05). The frequency of 13 genes in TCR β chain J segment were compared, and the results showed that compared with the control group, the case group had significantly higher frequencies of TRBJ2-3 , TRBJ2-5 , and TRBJ2-7 ( P <0.05)., Conclusions: There is a significant change in the diversity of TCR β chain CDR3 in the peripheral blood of neonates with sepsis, suggesting that it might be associated with the immune pathogenesis of neonatal sepsis.

Objectives: To study the change in regional oxygen saturation (rSO 2 ) of intestinal tissue in preterm infants with hemodynamically significant patent ductus arteriosus (hsPDA) by near-infrared spectroscopy, and the clinical significance of the change in intestinal oxygen level in preterm infants with hsPDA., Methods: The preterm infants with patent ductus arteriosus (PDA) who had gestational age <32 weeks and/or birth weight <1 500 g were prospectively enrolled, who were admitted to the Department of Neonatology, Shenzhen Longgang Central Hospital from October 2017 to October 2020.According to the diagnostic criteria for hsPDA, the preterm infants with patent ductus arteriosus (PDA) were divided into two groups: hsPDA and non-hsPDA. According to closure of the ductus arteriosus after oral administration of ibuprofen, the preterm infants in the hsPDA group were subdivided into two groups: hsPDA closure and hsPDA non-closure. Hemodynamic parameters were measured at diagnosis of PDA and after treatment, and the level of intestinal tissue rSO 2 was monitored continuously to analyze its change., Results: A total of 241 preterm infants with PDA were enrolled, with 55 infants (22.8%) in the hsPDA group and 186 infants (77.2%) in the non-hsPDA group. There were 36 infants (65%) in the hsPDA closure group and 19 infants (35%) in the hsPDA non-closure group. Compared with the non-hsPDA group, the hsPDA group had a significantly higher left atrial diameter/aortic root diameter ratio and significantly lower left ventricular ejection fraction and fractional shortening ( P <0.05). At each time point within 6 hours after diagnosis (1, 2, 4, and 6 hours), the hsPDA group had significantly lower intestinal tissue rSO 2 than the non-hsPDA group ( P <0.05), and intestinal tissue rSO 2 gradually decreased over time in the hsPDA group ( P <0.05), with the lowest level of 0.448±0.014 at 6 hours. Compared with the hsPDA non-closure group, the hsPDA closure group had a significantly lower left atrial diameter/aortic root diameter ratio and significantly higher left ventricular ejection fraction and fractional shortening ( P <0.05). At each time point within 48-96 hours after treatment (48, 72, and 96 hours), the hsPDA closure group had significantly higher intestinal tissue rSO 2 than the hsPDA non-closure group ( P <0.05), and intestinal tissue rSO 2 gradually increased since 24 hours after treatment in the hsPDA closure group ( P <0.05), with the highest level of 0.578±0.031 at 96 hours., Conclusions: hsPDA has an impact on intestinal tissue oxygenation in preterm infants, and continuous monitoring of intestinal tissue rSO 2 by near-infrared spectroscopy can help to guide the clinical management of hsPDA in preterm infants.

Purpose: This study evaluated the efficacy and safety of transcatheter radiofrequency ablation (RFCA) in treating ventricular premature contractions (PVCs) in children, summarized the countermeasures during intraoperative ventricular fibrillation (VF), and improved the safety of ventricular premature treatment., Methods: A retrospective analysis was conducted on 75 children with PVCs who received RFCA in the Second Affiliated Hospital of Wenzhou Medical University from January 2010 to April 2019. Data including age, sex, body weight, ejection fraction, left ventricular end diastolic diameter, burden and number of PVCs/24 h, origin of PVCs, and its complications were collected. Paired t test was used to compare changes in cardiac function before and after surgery., Results: Among the 75 cases treated with RFCA, 68 were successfully ablated, giving a success rate of 90.67%. After ablation, the left ventricular ejection fraction (LVEF) of the children was 69.13 ± 3.81%, which was significantly higher than that before surgery (69.13 ± 3.81% vs. 66.21 ± 3.22%, P = 0.012). One of the patients experienced VF during the operation, with no other complications. The initial locus of origin was the anterior septum of the right ventricular outflow tract, but VF occurred during the ablation process. Mean follow-up time was 39 ± 33 months, with two recurrent cases (2.94%)., Conclusions: Performing RFCA in children is safe and effective, with a low recurrence rate and few complications. VF is not an indication to cease surgery; the key to eliminating complications is repositioning the catheter and finding a more accurate origin point.

Stable isotopes are increasingly used to detect and understand the impacts of environmental changes on riverine ecological properties. The δ 13 C and δ 15 N signatures of fish with different feeding habits were measured in a large subtropical river to evaluate how fish isotopic niches respond to environmental gradients and human disturbance. From basal resources to fish consumers, the high values of epilithic periphyton (biofilm) δ 13 C and suspended particulate organic matter δ 15 N concurrently determined the niche ranges and space (e.g., convex hull area) of fish communities. Along a longitudinal gradient (except in the industrial zone), the number of fish trophic guilds identified by Bayesian ellipses continuously increased; meanwhile, higher trophic diversity and less redundancy were observed near the lower reaches and estuary. Variance inflation factors were estimated to detect the multicollinearity of 40 environmental variables, 14 of which were selected as indicators. Relative importance (RI) analysis was used to evaluate the explanatory power of these indicators for the spatial variation in isotopic niche metrics; the results showed that riffle habitat area, water nitrate concentration, gravel-cobble substrate, and riparian buffer width were the 4 key environmental indicators (average RI > 12%) that determined the longitudinal pattern of fish isotopic niches. These findings suggested that community-level δ 13 C signatures are more responsive to changes in habitats (e.g., riffle) and substrates (e.g., gravel-cobble) supporting the productivity of autochthonous diatoms while δ 15 N signatures respond to water quality altered by nitrogen pollution from manure-fertilized farming and poultry livestock effluent. Furthermore, δ 15 N may be more robust and interpretable than δ 13 C as an isotopic indicator of ecosystem change in rivers exposed to multiple or complex anthropogenic stressors., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2020 Elsevier B.V. All rights reserved.)

This study aimed to examine the association between low T3 syndrome and overall survival (OS) in patients with secondary hemophagocytic lymphohistiocytosis (sHLH). The study consisted of 111 consecutive patients hospitalized due to newly diagnosed sHLH with detailed thyroid hormone profiles on admission. Low T3 syndrome was found in 75.7% of the studied sHLH population. After a median follow-up of 83 (interquartile range 25-365) days, there were 60 (71.4%) cumulative deaths in the low T3 syndrome group and 13 (48.1%) in the euthyroid group. Survival analysis showed a lower survival probability for patients with low FT3 than for those with normal FT3 (median OS, 60 vs. 365 days, p  = .011). In the multivariate analysis, low T3 syndrome was an independent prognostic factor for OS (HR = 2.474; 95% CI 1.351-4.532, p  = .003). Low T3 syndrome is frequently found and associated with worse outcomes in patients with sHLH.

Objective: To investigate the significance of plasma pentraxin 3 (PTX3) in patients with secondary hemophagocytic lymphohistiocytosis (sHLH). Methods: Plasma PTX3 levels were tested by ELISA in 48 newly diagnosed sHLH patients, 18 healthy volunteers and 9 lymphoma controls in the First Affiliated Hospital of Nanjing Medical University from January 2017 to July 2019. Clinical parameters were collected, and the correlations with PTX3 levels were analyzed. Results: PTX3 level in newly diagnosed group was significantly higher than that of healthy control group [16.29(1.17-66.00) vs. 0.76(0.01-7.86) μg/L, P< 0.01]. Patients with lymphoma-associated HLH(LHLH) had higher plasma level of PTX3 than Fhose with infection-associated HLH (IHLH) [24.29(3.36-66.00) vs. 9.56(1.17-36.50)μg/L, P< 0.05]. Plasma PTX3 levels in 48 sHLH patients were positively correlated with serum ferritin ( P< 0.05). Receiver operating characteristic (ROC) curve for plasma PTX3 levels of sHLH and healthy controls produced a cutoff value at 3.9 μg/L, with its 86.7% sensitivity and 94.4% specificity. And ROC analysis showed that PTX3 17.5 μg/L was the critical value for diagnosis of LHLH from non-LHLH group, that the sensitivity and specificity were 63.0% and 76.2% respectively. The 1-year overall survival (OS) rate in patients with PTX3≥17.5 μg/L was significantly lower in those with PTX3<17.5 μg/L (18.5% vs. 75.8%, P< 0.01). Conclusion: These results indicate the potential of PTX3 as a biomarker for diagnosis and prognosis in patients with sHLH.

Objective: To investigate the expression and clinical significance of soluble B7-H3 (sB7-H3) in patients with secondary hemophagocytic lymphohistiocytosis (sHLH)., Methods: The plasma samples of 85 newly diagnosed sHLH patients from December 2012 to April 2018 were collected. The patients were divided into lymphoma-related HLH（LHLH）group and infection-related HLH（IHLH）group. The expression of sB7-H3 in plasma was detected by ELISA, and the clinical data were collected for analysis. Fifteen healthy people were chosen as control group., Results: The expression level of sB7-H3 in lymphoma-related HLH and infection-related HLH group significant increased as compared with the control group, (P＜0.05), and the expression level of sB7-H3 in lymphoma-related HLH group was significant higher than that in infection-related HLH group [(35.75± 9.90) vs (28.70±8.95) ng/ml)] (P＜0.001). There were no significant statistical difference in the expression of some clinical factors (including age, fever, splenomegaly, ANC, Plt, FIB, calcium ion, serum albumin, LDH, serum ferritin, sCD25) in lymphoma-related HLH and infection-related HLH group (P＞0.05). The evaluation of expression and significance of sB7-H3 in sHLH by using ROC curve, showed that the area under ROC curve comparison of patients in lymphoma-related HLH group and infection-related HLH group was 0.718 (95% CI 0.610-0.810) (P=0.0002), and predicting the sensitivity and specificity of the lymphoma-related HLH patients were 77.36% and 59.38%, respectively. The best cut-off value of patients in sB7-H3 was 29.81 ng/ml, the overall survival time of sB7-H3 high-expression group (≥29.81 ng/ml) was significant shorter than that in low-expression group (＜29.81 ng/ml) (24 vs 440 d) (P＜0.001). The clinical factors affecting the survival status of sHLH patients were neutrophils, albumin, serum ferritin, serum calcium ions and sB7-H3 levels., Conclusion: sB7-H3 associates with poor prognosis of sHLH patients, and may be involved in disease progression.

The study aimed to investigate the role of oxidised low-density lipoprotein (oxLDL)/lectin-like-oxLDL receptor-1 (LOX-1) in coronary artery lesions (CALs) in Kawasaki disease (KD) and of plasma oxLDL concentration in the early prediction of CALs in KD. This prospective study included 80 KD patients, 20 febrile and 20 healthy children. oxLDL, LOX-1 and other parameters were analysed in the acute phase. Plasma oxLDL concentration and LOX-1 mRNA expression in peripheral blood mononuclear cells (PBMCs) were significantly increased in KD patients compared with febrile and healthy children (P < 0.001 and P = 0.022, respectively), particularly in the group with CALs (P < 0.001 and P = 0.027, respectively). Coronary Z-score was significantly correlated with plasma oxLDL concentration and LOX-1 mRNA expression (r = 0.739 and 0.637, respectively; P < 0.01). The sensitivity and specificity of predicting CALs were 71.4% and 77.2%, respectively, at plasma oxLDL concentration ≥ 12.38 mU/L. oxLDL/LOX-1 may be involved in CAL development. The plasma oxLDL concentration in the acute phase is a potentially useful biological indicator for predicting CAL in KD patients.

Objective: To explore the mediation mechanism of coronary artery lesion among both male and female Kawasaki disease (KD) children. Methods: Children with KD that hospitalized in the Wenzhou Medical University affiliated Yuying Children's Hospital from January 2009 to December 2014, were included in this study. Differences on demographical characteristics, clinical manifestations, laboratory indicators, regimen and time of treatment, results from pre/post echocardiography and treatment between male and female patients, were compared. The independent effect of gender on the risk of coronary artery lesions (CAL) was evaluated, and the mediating effect of BMI, visiting time and KD type on the association between gender and CAL were also studied. Results: The average BMI level of male patients was higher than that of female patients. The difference was statistically significant ( P <0.001). The prevalence of overweight among male patients (20.9 % ) was higher than female (14.1 % ). The difference was statistically significant ( P =0.011). Data from the multivariate logistic regression analysis confirmed that the incidence of CAL in male patients was higher than that in female patients (a OR =1.50, 95 %CI : 1.06-2.12) but the CAL was mainly different before on the immunoglobulin therapy. Results from the mediation analysis showed that BMI was an important mediator in the association between gender and CAL, with the indirect effect as 1.05 (95 %CI : 1.01- 1.10) and the proportion mediated as 13.0 % . Conclusions: Male patients presented higher incidence of CAL but was mainly reflected in the difference of CAL before the treatment. BMI was probably an important mediator related to the association between gender and CAL.

Objective: To investigate the clinical characteristics of secondary hemophagocytic lymphohistiocytosis (sHLH) complicated with capillary leak syndrome (CLS) . Methods: The clinical and laboratory data of 87 sHLH patients, who were treated in our hospital between January 2015 and December 2017, were retrospectively analyzed. Depending on whether they were complicated with CLS, 21 sHLH patients were classified as the CLS-sHLH group, while 66 were classified as the non-CLS-sHLH group. The differences of clinical manifestations, laboratory tests, treatment and prognosis between the two groups were compared. Results: There was no significant difference in the etiology of sHLH between the CLS-sHLH group and the non-CLS-sHLH group ( P >0.05) . The neutrophil, fibrinogen and albumin levels in the CLS-sHLH group were lower than those in the non-CLS-sHLH group, while the triacylglycerol levels were higher than those in the non-CLS-sHLH group ( P <0.05) . Varying degrees of edema, weight gain, hypotension, hypoproteinemia, oliguria and multiple serous effusions were observed in the CLS-sHLH group. Among them, there were 15 patients that CLS get improved, and the medial time of improvement was 7 (5-14) days. The other 6 patients did not get remission, while they died within 6-30 days. The median overall survival of the CLS-sHLH group was lower than that of the non-CLS-sHLH group (75 days vs not reached, P =0.031) . Conclusions: There may be no correlation between the cause of sHLH and the occurrence of CLS. Severity of neutropenia, fibrinogen and albumin levels, and triglyceride levels may be accompanied for sHLH patients complicated with CLS. Patients with sHLH who complicated with CLS have a poor prognosis. Active treatment of HLH and its primary disease, reasonable fluid replacement and oxygen supply are crucial, which can effectively control disease progression.

Objective: To investigate the clinical significance of serum calcium concentrations and albumin levels in patients with secondary hemophagocytic lymphohistiocytosis (sHLH) . Methods: The clinical and laboratory data of 68 patients with newly diagnosed sHLH from April 2013 to April 2016, and 20 healthy controls were retrospectively analyzed. The patients were classified as the clinical remission group and the progression group according to the response criteria. To explore the changes of serum calcium concentrations and albumin levels in the patients, its correlations with other laboratory indexes and the impact on survival. Results: ①The serum calcium concentrations and albumin levels in lymphoma-related HLH group and non-tumor HLH group were lower than control group ( P <0.05) ; The serum calcium concentrations and albumin levels in the clinical remission group after treatment was higher than that before treatment ( P <0.01) , while in the progression group the serum calcium concentrations after treatment decreased ( P =0.002) , the albumin levels did not changed significantly ( P =0.086) .②The serum calcium concentrations in newly diagnosed sHLH patients was positively correlated with ANC, PLT, HGB ( P <0.05) , and negatively with ferritin ( P <0.05) ; The overall survival (OS) was significantly longer in patients with serum calcium concentration≥1.92 mmol/L than that<1.92 mmol/L (266 d vs 95 d, P =0.006) . ③The serum albumin levels in newly diagnosed sHLH patients was positively correlated with ANC, HGB ( P <0.05) , and negatively with ferritin ( P <0.05) ; The overall survival was longer in patients with serum albumin level ≥ 26.7 g/L than that<26.7 g/L (263 d vs 95 d, P =0.023) . ④The serum calcium concentrations in patients before treatment was positively correlated with serum albumin levels ( P <0.001) , and no significant correlation was found in control group ( P =0.079) . Conclusion: The serum calcium concentrations and albumin levels have clinical significance for disease diagnosis, decision-making therapy and treatment outcomes in the patients with sHLH.

Protective effect of protodioscin or methyl protodioscin against inflammation had been reported in various inflammation diseases. This study aimed to investigate the effect of protodioscin against Complete Freund's adjuvant (CFA)-induced arthritis rats. Rats randomly divided into model groups were injected with CFA, companied with different dose of protodioscin (50, 100, and 200 mg/kg body weight). The histology, changes in biochemical parameters and inflammatory cytokines expression were detected for anti-inflammation effect evaluation of protodioscin. CFA treatment induced arthritic rats with swelling paw, ankle inflammation, and area of lymphocyte infiltration, upregulated inflammatory cytokines (IL-1β, TNF-α, cyclo-oxygenase 2, and IL-6 as well as prostaglandin E2), articular elastase, myeloperoxidase, lipid peroxidase and nitrite oxide levels, downregulated glutathione, catalase, and superoxide dismutase. In contrast, protodioscin ameliorated all the changes induced by CFA in rats, suggesting the anti-inflammatory effect of protodioscin. We concluded that protodioscin administration into CFA-induced arthritis rats protected against CFA-induced oxidative stress, neutrophil infiltration, and inflammation, suggesting the anti-inflammatory effect and the therapeutic potential of protodioscin for arthritis.

The aim of the present study was to identify key genes that may be involved in the pathogenesis of Tetralogy of Fallot (TOF) using bioinformatics methods. The GSE26125 microarray dataset, which includes cardiovascular tissue samples derived from 16 children with TOF and five healthy age‑matched control infants, was downloaded from the Gene Expression Omnibus database. Differential expression analysis was performed between TOF and control samples to identify differentially expressed genes (DEGs) using Student's t‑test, and the R/limma package, with a log2 fold‑change of >2 and a false discovery rate of <0.01 set as thresholds. The biological functions of DEGs were analyzed using the ToppGene database. The ReactomeFIViz application was used to construct functional interaction (FI) networks, and the genes in each module were subjected to pathway enrichment analysis. The iRegulon plugin was used to identify transcription factors predicted to regulate the DEGs in the FI network, and the gene‑transcription factor pairs were then visualized using Cytoscape software. A total of 878 DEGs were identified, including 848 upregulated genes and 30 downregulated genes. The gene FI network contained seven function modules, which were all comprised of upregulated genes. Genes enriched in Module 1 were enriched in the following three neurological disorder‑associated signaling pathways: Parkinson's disease, Alzheimer's disease and Huntington's disease. Genes in Modules 0, 3 and 5 were dominantly enriched in pathways associated with ribosomes and protein translation. The Xbox binding protein 1 transcription factor was demonstrated to be involved in the regulation of genes encoding the subunits of cytoplasmic and mitochondrial ribosomes, as well as genes involved in neurodegenerative disorders. Therefore, dysfunction of genes involved in signaling pathways associated with neurodegenerative disorders, ribosome function and protein translation may contribute to the pathogenesis of TOF.

Objective: To investigate the effect of premature rupture of the membrane (PROM) on neonatal complications in premature infants., Methods: The registration information of 7684 preterm infants with gestational age <37 weeks were collected from the cooperative units in the task group between January 1, 2014 to December 31, 2014. Specially trained personnel from each cooperative units filled in the unified form in a standardized format to record the gender, gestational age, birth weight, PROM, placental abruption, antenatal corticosteroid, Apgar score, amniotic fluid pollution, and complications of the infants. The data were analyzed comparatively between the cases with PROM and those without (control)., Results: The preterm mortality rate was significantly lower but the incidences of ICH, NEC, ROP and BPD were significantly higher in PROM group than in the control group (P<0.05). The 95% confidence interval of the OR value was <1 for mortality, and was >1 for ICH, NEC, ROP and BPD. After adjustment for gestational age, birth weight, gender, mode of delivery, placental abruption, placenta previa, prenatal hormones, gestational diabetes mellitus (GDM), gestational period hypertension and 5-min Apgar score <7, the incidences of NEC, ROP and BPD were significantly different between the two groups (P<0.05) with 95% confidence interval of OR value >1, but the mortality rate and incidence of ICH were not significantly different between the two groups (P>0.05)., Conclusion: PROM is a risk factor for NEC, ROP and BPD in preterm infants, and adequate intervention of PROM can reduce the incidences of such complications as NEC, ROP and BPD in the infants.

With the advent of antibiotic resistance, pathogenic bacteria have become a major threat in cases of neonatal sepsis; however, guidelines for treatment have not yet been standardized. In this study, 15 cases of neonatal Streptococcus agalactiae sepsis from our hospital were retrospectively analyzed. Of these, nine cases showed early-onset and six cases showed late-onset sepsis. Pathogens were characterized by genotyping and antibiotic sensitivity tests on blood cultures. Results demonstrated that in cases with early-onset sepsis, clinical manifestations affected mainly the respiratory tract, while late-onset sepsis was accompanied by intracranial infection. Therefore, we suggest including a cerebrospinal fluid examination when diagnosing neonatal sepsis. Bacterial genotyping indicated the bacteria were mainly type Ib, Ia, and III S. agalactiae. We recommend treatment with penicillin or ampicillin, since bacteria were resistant to clindamycin and tetracycline. In conclusion, our results provide valuable information for the clinical treatment of S. agalactiae sepsis in neonatal infants.

Objective: To investigate the expression levels and clinical significance of serum high mobility group box 1 (HMGB-1) in patients with secondary hemophagocytic lymphohistiocytosis (sHLH)., Methods: Serum HMGB-1 levels were determined by using enzyme linked immunosorbent assays (ELISA) in 51 sHLH patients and 15 healthy contrlols. Other laboratory data, including soluble interleukin-2 receptor (sCD25), white blood cells (WBC), hemoglobin (Hb), platelet (Plt), fibrinogen (FIB), lactate dehydrogenase (LDH), triglyceride (TG), alanine transaminase (ALT), aspartate aminotransferase (AST), serum ferritin (SF), C reactive protein (CRP), and blood sedimentation rate (ESR) were also collected., Results: Serum HMGB-1 levels in the newly diagnosed group were significantly higher than that in the control group (P<0.01). Serum HMGB-1 levels in the newly diagnosed lymphoma-associated HLH (LHLH) group were significantly higher than that in non-HLH group, including infection-associated HLH (IHLH) and autoimmune-associated HLH (AHLH) group (P<0.05); The serum HMGB-1 levels in the clinical remission group were significantly lower than that in the newly diagnosed group (P<0.05), however, serum HMGB-1 was not decreased significantly in the progression/relapsed group, compared with the newly diagnosed group (P>0.05). Serum HMGB-1 levels in newly diagnosed sHLH patients positively correlated with sCD25 (r=0.62, P<0.01) and ESR (r=0.55, P<0.05). The receiver operating characteristic curves (ROC) for serum HMGB-1 levels of sHLH patients and healthy controls produced a cutoff value at 15.3 µg/L, with its 90% sensitivity and 99% specificity, respectively. In addition, an optimal cutoff value for HMGB-1 was 27.4 µg/L in the patients LHLH and non-HLH (AHLH+IHLH) with 96% sensitivity and 81% specificity, separately., Conclusion: Serum HMGB-1 levels possesses an important clinically significance for disease diagnosis, differential diagnosis, evaluation of nosographic activity and treatment efficacy in the patients with sHLH.

Many Chinese patients with hematologic diseases, who need allogeneic hematopoietic stem cell transplantation (HSCT), lack a human leukocyte antigen-matched donor. To save these patients and to avoid collecting donor bone marrow graft, we adopted haploidentical peripheral blood HSCT with granulocyte colony stimulating factor (G-CSF) mobilized peripheral blood stem cells as the grafts without ex vivo T cell depletion. Thirty-eight patients were enrolled, and they received myeloablative preconditioning. Thirty-five patients attained a successful neutrophil and platelet recovery. The median time for the neutrophil recovery was 16 days (range of 10-23 days), and the median time for the platelet recovery was 19 days (range of 10-66 days). During the follow-up at a median time of 33.1 weeks (range of 1.1-412.6 weeks), eleven (28.9 %) patients developed aGVHD grade I-II and seven (18.4 %) patients developed aGVHD grade III-IV. The incidence of cGVHD was 27.6 %, and nine (23.7 %) patients died within the first 100 days after transplantation. The cumulative survival proportions at 1 and 2 years were 52.51 ± 8.57 % and 43.76 ± 9.11 %, respectively. These results suggested that the G-CSF-primed peripheral blood stem cell grafts, without in vitro T cell depletion, could be an appropriate stem cell source for Haplo-HSCT.

This study was aimed to enhance clinical understanding the effect of nilotinib on CML patients with V299L mutation who were resistant to imatinib. Bone marrow specimens from 2 cases of CML with V299L mutation were collected before and after the treatment with nilotinib. ABL mutation was detected by nested reverse transcription polymerase chain reaction (PCR) followed by direct sequencing. The clinical characteristics of the two cases were analyzed. The results showed that both cases were resistant to imatinib presented V299L mutation. Out of them 1 case achieved complete haematological response (CHR) after treatment with nilotinib for 6 months and another case abstained obvious molecular response after using nilotinib for 7 month. V299L mutation of both cases was turned into negative after the treatment with nilotinib. It is concluded that the nilotinib can safely and effectively override tyrosine kinase inhibitor (TKI) resistance mediated by the V299L mutation. The safety and efficacy of nilotinib for treatment of CML patients with TKI resistance and V299L mutation are satisfactory.

The optimal post-remission therapy (PRT) for acute myeloid leukemia (AML) remains uncertain. We reported 32 AML patients in first complete remission (CR1) undergoing autologous hematopoietic stem cell transplantation (ASCT) with a characteristic conditioning regimen, termed I-Bu, based on high-dose idarubicin plus busulfan, which considerably strengthened antileukemic activity. Most patients were in better or intermediate-risk group except that cytogenetic or molecular risk information was missing for 18.7 % of the patients. Unpurged peripheral blood stem cells were used in all the cases. The adverse effects were mild and reversible. Only one case of transplant-related mortality was observed. All the patients in this study acquired hematopoietic reconstitution after ASCT. After a median follow-up of 30 (6-119) months, 24 patients (75.0 %) were alive in which 20 (62.5 %) patients were in continuous CR. There were 11 (34.4 %) patients who relapsed after HSCT. Cumulative relapse probability was about 40 % after 24 months. Median OS and DFS have not been reached. Patients in the better and intermediate-risk group had different clinical outcomes, but the differences were not statistically significant. ASCT with I-Bu regimen is possibly promising PRT for better and intermediate-risk AML patients in CR1.

Objective: To investigate SRSF2 mutations in patients with chronic myelomonocytic leukemia (CMML) and the clinical characteristics of patients with SRSF2 mutants., Methods: In this study, the frequency of SRSF2 mutation in a cohort of 20 patients with CMML was detected by polymerase chain reaction (PCR) followed by direct sequencing to couple with their clinical features., Results: Of 20 patients, 4 patients were found harboring SRSF2 mutations, including 2 P95L, 1 P95H and 1 P95R point mutations. There were no significantly statistical differences in terms of their clinical characteristics between mutant and wild type group., Conclusion: SRSF2 mutation was not frequently occurred in CMML patients and might associated with poor prognosis. It might be a practically diagnostic maker and therapeutic target in CMML.

This study was purposed to investigate the molecular mechanism of 4-1BBL reverse signals in the human acute monocytic leukemia cell line of U937. The U937 cell line was used as target cells, and stimulated by the mouse anti-human 4-1BBL monoclonal antibody 1F1. The nuclear translocation of NF-κB and the co-location of 4-1BBL and CD28i molecules in U937 cells were observed with confocal laser scanning microscopy. The protein and m-RNA expression levels of 4-1BBL and CD28i were detected by flow cytometry and RT-PCR respectively. The results showed that the significant nuclear translocation of NF-κB and co-localization of 4-1BBL and CD28i on membrane of U937 cells appeared after being stimulated by mAb1F1. It is concluded that the 4-1BBL reverse signals transduction mediating the growth of U937 cells relates with the nuclear translocation of NF-κB. CD28i may be involved in intracellular 4-1BBL reverse signaling pathways.

This study was aimed to detect the peripheral blood serum neopterin (Npt) level in the patients with hemophagocytic lymphohistiocytosis (HLH) and to explore its significance in HLH. The enzyme-linked immunosorbent assay (ELISA) was applied to detect the serum Npt level and sCD25 level in 20 HLH patients before and after treatment and 15 healthy controls. The results indicated that the serum Npt and sCD25 levels in HLH patients were significantly higher than those in healthy controls (P < 0.0001). The serum Npt and sCD25 levels in the HLH group decreased significantly after treatment, respectively (P < 0.0001). The correlation analysis of Npt with sCD25 before and after treatment showed that they had significant correlation (r = 0.81, P < 0.05 before treatment; r = 0.65, P < 0.05 after treatment). Meanwhile, the level of serum Npt and ferritin had a significant correlation in newly diagnosed HLH patients (r = 0.55, P < 0.05). It is concluded that the serum Npt may play an important role in the HLH pathogenesis, the enhancement of Npt levels has an important significance for diagnosis and evaluation for HLH.

This study was purposed to investigate the incidence of mixed lineage leukemia (MLL) gene rearrangement and partner gene types as well as the clinical features and prognosis of acute leukemia (AL) with this rearrangement through detection in adult AL using combination of 3 techniques, and to evaluate the clinical value of this combination detection. The MLL gene rearrangement in 183 cases of adult AL was detected by combination of conventional cytogenetics, split signal FISH and multiplex nested PCR. The results showed that the incidence of MLL rearrangements in adult patients with AL was low (8.2%), and MLL-AF4 fusion gene was most common and predominant in acute lymphoblastic leukemia (ALL), while the MLL-AF6 and MLL-AF9 were most frequent in acute myeloid leukemia (AML). Extramedullary involvements were found in 40% of MLL-rearranged AL patients, and 33.3% of patients with MLL-rearranged AL reached to complete remission within 30 days during induction chemotherapy. In addition, in this cohort of MLL-rearranged adult AL patients, the 3-month relapse rate and 6-month overall survival rate were 50.0% and 50.0% respectively. It is concluded that the rate of missed diagnosis of CC technique for patients with MLL-rearranged AL reached to 60% in this study, while the combination of CC, FISH and multiplex nested PCR has been confirmed to have important significance for evaluating prognosis and conducting clinical therapy of patients with MLL-rearranged AL.

Objective: To observe the expression of urotensin II (UII) on the lung of patients with pulmonary hypertension (PH) with congenital heart disease and investigate the meaning of this phenomenon., Method: Thirty eight patients with CHD were divided into three groups according to pulmonary arterial systolic pressure (PASP) measured in cardiac catheterization and surgery: normal pulmonary pressure group (N group, PASP < 30 mm Hg, n = 10), mild PH group (M group, PASP ≥ 30 mm Hg, n = 15), severe or moderate PH group (S group, PASP ≥ 50 mm Hg, n = 13). The expression of UII protein and UII mRNA in pulmonary arterioles were measured separately by immunohistochemical (IHC) analysis and in situ hybridization (ISH) analysis., Result: (1) The results of UIIIHC staining: The UII protein expression of group M was higher than that of group N (20.22 ± 3.58 vs. 14.34 ± 2.18, P < 0.01), but less than group S (20.22 ± 3.58 vs. 28.92 ± 3.22, P < 0.05). (2) The results of UIIISH mRNA staining were similar to IHC staining, the A value of group M was higher than group N (12.51 ± 2.02 vs. 8.85 ± 1.41, P < 0.05), less than that of group S(12.51 ± 2.02 vs. 25.35 ± 4.33, P < 0.01). (3) Correlation study: there was a positive correlation between the A values of UIIIHC and pulmonary hypertension (r = 0.64, P < 0.01, n = 38), a positive correlation between the A values of UIIISH and pulmonary hypertension (r = 0.58, P < 0.01, n = 38)., Conclusion: There was the expression of Urotensin II protein and mRNA in the lung of pulmonary hypertension patients with congenital heart disease, and these expression may involve the formation of pulmonary hypertension of congenital heart disease.

Objective: To explore the effectivity and safety of single high-dose (HD) etoposide (Vp16) with granulocyte colony-stimulating factor (G-CSF) for mobilization of autologous peripheral blood stem cells (PBSC) in patients with hematologic malignancies., Methods: 80 patients of hematologic malignancies including 20 patients with acute leukemia (AL), 23 with multiple myeloma (MM), 35 with non-Hodgkin's lymphoma (NHL) and 2 with Hodgkin's lymphoma (HL) received Vp16 (1.6 g/m(2)) continuous intravenous infusion for 10 hrs on day 1. G-CSF at 10 µg/kg once daily subcutaneous injection began to use on day of ANC lower than 1×10(9)/L and continued until PBSC collection was completed. Autologous PBSC (APBSC) was collected on day of WBC greater than 5×10(9)/L and continuing until the collection goal was met (target value: MNC ≥ 6.0×10(8)/kg and CD34(+) ≥ 2.0×10(6)/kg). The patients received APBSC after conditioning regimen. The number of the cells collection, time of hematopoietic reconstruction, adverse effect and so on were observed during the course of stem cell mobilization and collection., Results: PBSC was collected on day 11 (range: 7 - 25 days) of after Vp16 administration with a median collection time of 2 (range 1 - 5). 3/80 patients with AML got stem cell mobilization failure. 5 of 6 patients who failed to mobilize before got successful stem cell mobilization, 1/6 patient with AML-M(5) got a second failure after the mobilization of VP16 whose first time's mobilization using Ara-C did not succeed. The median number of CD34(+) cells collected in 77 patients who got successful mobilization was 4×10(6)/kg \[range (1.59 - 24.68)×10(6)/kg\]. The collection of 20 patients with AL and 23 with MM were got detection for minimal residual disease, no pollution of tumor cells were happened. All patients could tolerate the whole course of stem cell mobilization. 29/80 (36.25%) patients got a 4 grade leucopenia, 19/80 (23.75%) patients got infection., Conclusion: Single high-dose etoposide with G-CSF for mobilization of APBSC has a higher achievement ratio, a controllable adverse effect, a promising hematopoiesis recovery, which is an effective and safe mobilizing regimen for patients with hematologic malignancies.

This study was aimed to investigate the significance of 18F-FDG PET/CT in secondary hemophagocytic lymphohistiocytosis (sHLH) patients. A total of 18 patients received 18F-FDG PET/CT scan at initial diagnosis. All patients (18/18) had at least 3 organs involved, with increased FDG metabolism in different degrees. Fifteen cases (15/18) had definite underlying diseases, including infections (IAHLH), rheumatosis (RAHLH), or malignancy (MAHLH). The SUVmax of patients in MAHLH group was significantly higher than patients in IAHLH group or RAHLH group (P = 0.015, P = 0.045). Furthermore, the SUVmax of patients in IAHLH group was significantly higher than patients of RAHLH group (P = 0.043). Therefore, we concluded that 18F-FDG PET/CT may especially play important role in differential diagnosis of sHLH.

NPM1 mutation is the most common molecular abnormality in patients with acute myeloid leukemia (AML), especially normal karyotype AML (NK-AML), and is associated with a favorable prognosis in the absence of concomitant FLT3-ITD. Like other molecular abnormalities such as FLT3-ITD, C/EBPα and c-Kit mutation, NPM1 mutation normally presents as a recurrent molecular abnormality. The NPM1 mutation is generally used as a molecular marker in the prognosis evaluation of a patient with AML. Here, we report a different case. He was first diagnosed with NPM1 mutation-positive acute monocytic leukemia. However, he achieved no remission, but the NPM1 mutation dramatically became negative after induction chemotherapy. Finally, he achieved complete remission after salvage chemotherapy and the NPM1 mutation was still negative. To our knowledge, this is a rare case according to the worldwide published literature.

This study was aimed to detect the level of soluble interleukin-2 receptor (sCD25) and cytotoxic activity of NK lymphocytes in patients with hemophagocytic lymphohistiocytosis (HLH), and to explore their clinical significance in HLH. The enzyme-linked immunosorbent assay was used to detect the sCD25 level in serum of 20 patients with HLH, 15 healthy controls, 20 cases of acute myeloid leukemia and 20 cases of systemic lupus erythematosus. The NK cell cytotoxicity in peripheral blood of patients with HLH and controls were detected by flow cytometry with CD107a antibody labeling and LDH release assay. The results indicated that the level of sCD25 in HLH patients was significantly higher than that in healthy controls and disease groups (P < 0.001). The NK cell cytotoxicity in peripheral blood detected by both methods in patients with HLH were lower than that in healthy controls (P < 0.05), and the results detected by flow cytometry correlated significantly with those by LDH release assay (r = 0.73, P < 0.05). It is concluded that detection of sCD25 levels and NK cell activity in peripheral blood in HLH is of great value. Using flow cytometry following CD107a antibody labeling to measure NK activity is a simple, stability, reproducibility method and can be used for clinical diagnosis of HLH.

Treatment regimens of secondary hemophagocytic lymphohistiocytosis (sHLH) are complicated and individualized. CHOP regimen is well known for the treatment of adult sHLH, but it was not so effective for the 56-year-old male patient in our study. Splenomegaly, one of clinical manifestations of HLH, has urged us to investigate the role of splenectomy in HLH patients. Splenectomy is not only beneficial to confirm the underlying diseases, but also beneficial for the treatment of HLH. Here, we present a case diagnosed as sHLH who has recovered from HLH following comprehensive treatment based on splenectomy. The therapeutic value of splenectomy in sHLH needs further study., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

The aim of this study was to explore cytogenetic characteristics of acute promyelocytic leukemia (APL) and compare the interphase fluorescence in situ hybridization (I-FISH) with conventional cytogenetic (CC) analysis. A total number of 157 APL patients were recruited in this study, and the I-FISH and CC were applied to analyze cytogenetic features. Chromosome samples of bone marrow cells were prepared by short-term culture. Out of all 157 cases, 136 were observed with CC assay, 66 with I-FISH, of which 45 samples were analyzed with both methods. The results showed that among all 136 CC samples, t(15;17)(q22;q21) was found in 120 cases, of which 107 cases was isolated t(15;17)(q22;q21) abnormality, 13 cases was complex abnormalities and 12 case without mitotic figure. Among all 66 cases of I-FISH group, PMI/RARα fusion gene was found in 64 cases (97.0%), suggesting that I-FISH group was more sensitive than CC group (p = 0.041). It is concluded that combination of I-FISH and CC techniques plays a pivotal role for diagnosis and detection of minimal residual disease in APL.

Objective: To observe the effects of carvedilol on the expression of Bcl-2, Bax and Fas in autoimmune myocarditis (AM)., Methods: A total of 60 inbred male BALB/C mice 4 - 5 weeks of age were divided at random into 3 groups as follows: AM group (n = 20), carvedilol group (n = 20) and control group (n = 20). The mice were sacrificed after gathering blood specimens by taking out the eyeballs and hearts tissue. The histological and ultrastructural changes were observed under light microscope and electron microscope. The concentrations of cardiac troponin I (cTn I) were detected by chemiluminescence immunoassay (CLIA). Immunohistochemistry (IHC) was performed to analyze the contents of Bcl-2, Bax and Fas, TUNEL to detect the apoptotic index in myocardial cells., Results: There were large number of lymphocyte and monocyte infiltrates under light microscope and karyopyknosis and chromatin gathered along the nuclear membrane under electron microscope in AM group. There were no inflammations and chromatin gathering in group C. Compared with control group, the Bcl-2, Bax and Fas protein expression significantly elevated in AM group (23.48 ± 2.24 vs. 6.64 ± 1.60, 26.15 ± 2.02 vs. 5.09 ± 0.85, 21.22 ± 3.62 vs. 5.86 ± 1.37, P < 0.01). The histopathologic scores (2.60 ± 0.31 vs. 2.02 ± 0.26, P < 0.05) and karyopyknosis of carvedilol group decreased as compared with AM group. The Bcl-2, Bax and Fas protein expression (17.13 ± 1.94 vs. 23.48 ± 2.24, 17.66 ± 2.62 vs. 26.15 ± 2.02, 16.79 ± 2.83 vs. 21.22 ± 3.62, P < 0.05), AI [(16.61 ± 4.67)% vs. (24.51 ± 4.70)%, P < 0.05] and contents of cTnI [(1.878 ± 0.48) ng/ml vs. (1.102 ± 0.23) ng/ml, P < 0.05] also decreased in carvedilol group compared with AM group., Conclusion: Carvedilol could protect against AM by alleviating cardiomyocyte apoptosis.

Cytogenetic features have an important role in the definition of distinct disease subsets in CLL. The deletion of 6q is known to occur at a relatively low frequency in CLL, and the detailed analysis of hematologic and clinical features of patients with CLL with 6q deletion is limited. To verify the incidence and prognostic significance of 6q deletion in Chinese patients with CLL, fluorescence in situ hybridization (FISH) was used in 240 patients with CLL. del(6q23) was found in 18 patients (7.5%), and only five patients had deletion in 6q23 as the sole abnormality. Strong correlations between del(6q23) and clinical parameters were not found. A difference in terms of survival in patients with del(6q23) as compared with patients without this anomaly was not able to be demonstrated. However, a significant difference was found when comparing the del(6q23) group with the del(17p13) or del(11q22.3) group (p = 0.023), or isolated del(13q14) group (p = 0.019). Our findings place the del(6q23) cytogenetic subset of CLL in an intermediate prognosis position between patients with del(11q22.3) or del(17p13), and patients with isolated del(13q14). FISH probes to detect deletions of 6q might be useful in clinical practice in the work-up of patients with CLL.

Optical coherence tomography is an appropriate imaging method for biomedical science, due to its advantages of noninvasive nature, high resolution and fast imaging speed. Because most biological tissues have the characteristic of high scattering coefficient, OCT system can just obtain the structural images several millimeters below the surface of the tissues. The superficial depth of OCT's penetration limits application in dermatology field. As a common disease, the port wine stain (PWS) is a indication of OCT, because of its superficial lesion and significant expansion of blood vessels. To get deeper penetration in the skin, the authors employed 1 310 nm superluminescent diode as light source, optimized the light intensity ratio of reference delay arm and sample arm and control polarization, and the research of PWS imaging in vivo was accomplished. Besides, OCT is able to gather clear image and key characteristic parameters, such as the depth of epidermis layer, the diameter of blood vessel, etc. OCT will play an important role in the diagnosis and therapy of PWS.

This study was aimed to investigate the therapeutic efficacy and adverse events of bortezomib-based chemotherapy for 40 patients with multiple myeloma. 16 newly diagnosed patients and 11 patients with refractory/relapse myeloma were treated with bortezomib, dexamethasone and thalidomide; 7 newly diagnosed patients and 4 patients with refractory/relapse myeloma were treated with bortezomib and dexamethasone; 2 newly diagnosed patients were treated with bortezomib, melphalan and thalidomide. Cycles were repeated every 28 or 35 days, all the patients were treated for 2 to 8 cycles. The therapeutic efficacy and adverse events were evaluated according to International Myeloma Working Group Uniform Response Criteria. The results indicated that the median follow-up duration was 13 months, the total response rate was 72.5%, among which 16 patients achieved complete response (CR), 13 achieved partial response (PR). The main side effects included gastrointestinal symptoms, peripheral neuropathy, thrombocytopenia, respiratory infection, herpes zoster and urinary retention and so on. The adverse events were ameliorated by treatment and decrease of the bortezomib dose. It is concluded that bortezomib-based chemotherapy is effective in the treatment of either newly diagnosed or refractory/relapse MM patients and the adverse events are tolerable and manageable for patients.

This study was aimed to investigate the therapeutic efficacy of HLH-2004 chemotherapy in patients with secondary hemophagocytic lymphohistiocytosis (sHLH). 10 cases of sHLH treated with HLH-2004 regimen at our department were analyzed retrospectively. The results showed that 7 patients had clinical response to HLH-2004 regimen, while other 3 patients had no clinical response. 5 cases did not complete initial therapy of 8 weeks. Out of 5 cases, 4 died in the process of chemotherapy, 1 patient abandoned for serious side effects but finally acquired remission following 4 cycles of CHOP regimen. 5 cases underwent the whole courses of initial therapy. Out of 5 cases, 3 patients acquired remission, and other 2 were not well controlled. Out of the 3 patients who had achieved remission, one died of relapse, and other 2 patients kept remission. Out of the 2 patients who were not well controlled, one patient died, but another patient acquired remission after being discharged. It is concluded that patients with infection-associated hemophagocytic syndrome (IAHS) have high rates of remission after receiving HLH-2004 regimen combining with effective antibiotics. However, patients with HLH secondary to EBV (EBV-HLH) or lymphoma (LAHS) have low rates of remission or are easy to get relapse after remission.

Objective: Previous studies have shown that hydrogen sulfide (H2S) plays key roles in a number of biological processes, including vasorelaxation, inflammation, apoptosis, ischemia/reperfusion and oxidative stress, which are involved in the pathogenesis of myocarditis. This study aimed to examine the expression of cystathionine-γ-lyase(CSE)/H2S pathway in mice with viral myocarditis., Methods: Six-week-old inbred male mice were randomly assigned to control (n=25) and myocarditis group (n=30). The myocarditis and the control groups were inoculated intraperitoneally with 0.1 mL 10-5.69TCID50/mL CVB3 or vehicle (PBS) alone respectively. Ten mice were sacrificed 4 and 10 days after injection. Blood and heart specimens were harvested for measuring the content of serum H2S and the H2S production rates in cardiac tissues. Heart sections were stained with hematoxylin and eosin. Immunohistochemisty was used to detect the CSE protein expression in the heart., Results: In the myocarditis group, the serum H2S content and H2S production rates in cardiac tissues were significantly higher than those in the control group 4 and 10 days after injection (P<0.05). The expression of CSE protein in the heart in the myocarditis group was also significantly higher than that in the control group (P<0.05)., Conclusions: CSE and its downstream production H2S increase in mice with acute viral myocarditis. The increased expression of CSE/H2S pathway might be involved in the pathogenesis of viral myocarditis.