Risk factors for syncope in children with severe idiopathic pulmonary arterial hypertension / 中华儿科杂志

Objective: To explore the risk factors for syncope in children with severe idiopathic pulmonary arterial hypertension (IPAH). Methods: Forty-four patients (age<18 years) with IPAH admitted to the Department of Pediatric Cardiology, Beijing Anzhen Hospital between May 2011 and October 2021 were retrospectively included. Patients were devided into syncope group and non-syncope group. Clinical manifestation and hemodynamic parameters including echocardiography, blood tests, right heart catheterization and acute pulmonary vascular expansion test were compared between two groups. Comparisons between groups were performed with unpaired Student t test, or Mann-Whitney U test or chi-square test. Logistic regression was used to calculate the odds ratio (OR) for parameters with statistically significant differences between groups and analyze the statistical correlation. Results: Among the 44 patients, 16 were males, the onset age was (7.2±3.9) years. Twenty-four (55%) children presented with 1 to 11 times of episodes of syncope, and 18 cases of whom induced by physical activity. Syncope group had a larger proportion of New York Heart Association (NYHA) heart function class Ⅲ-Ⅳ (67% (16/24) vs. 25% (5/20), χ2=7.59, P=0.006), higher brain natriuretic peptide (BNP) value ((251±39) vs. (61±40) pg/L, t=-2.18, P=0.035), higher pulmonary-to-aorta diameter ratio (1.6±0.4 vs. 1.4±0.2, t=-2.25, P=0.030) and larger pulmonary vascular resistance index ((22±11) vs. (16±7) WU/m2, t=-2.13, P=0.039) compared with non-syncope group. The proportion of patent foramen ovale (4% (1/24) vs. 45% (9/20), χ2=10.36, P=0.001), left ventricular ejection fraction (LVEF) ((68±5)% vs. (72±8)%, t=2.23, P=0.031) and the positive rate of acute pulmonary vascular expansion test (8% (2/24) vs. 35% (7/20), χ2=4.77, P=0.029) of syncope group were significantly lower than those of non-syncope group. Multiple Logistic regression analysis showed that NYHA heart function Ⅲ-Ⅳ (OR=6.787, 95%CI 1.445-31.880), pulmonary vascular resistance index (OR=1.247, 95%CI 1.020-1.525) and BNP (OR=1.036, 95%CI 1.007-1.066) were independent risk factors for syncope. The patent foramen ovale (OR=0.010, 95%CI 0.000-0.586) was a protective factor for syncope. Conclusions: NYHA cardiac function grade, pulmonary vascular resistance index and BNP are independent risk factors for syncope. Patent foramen ovale is a protective factor for syncope. Exercise is the main inducement of syncope in children with IPAH.

Early effect of extracorporeal membrane oxygenation and factors related to early outcome in adult patients with fulminant myocarditis / 中华心血管病杂志

Objective: To evaluate the efficacy within the first 24 h post extracorporeal membrane pulmonary oxygenation (ECMO) and the impact of early efficacy on the prognosis of adult patients with fulminant myocarditis (FM). Methods: This retrospective case analysis study included hospitalized patients (age≥18 years) who were diagnosed with fulminant myocarditis from November 2016 to May 2021 in the First Affiliated Hospital of Zhengzhou University. Patients were divided into survival or non-survival groups according to treatment outcomes. The age, sex, treatments, drug use, ECMO use, clinical and laboratory data (before and 24 h after the use of ECMO) were analyzed. The change rate of clinical and laboratory data after 24 h use of ECMO was calculated to find differences between two groups. Multivariate logistic regression was used to analyze the related factors with in-hospital death and complication between the two groups. Results: A total of 38 FM patients treated with ECMO were included. There were 23 cases (60.5%) in the survival group, aged (39.6±13.7) years, and 17 (73.9%) cases were female. The total ECMO time was (134.4±71.3)h. There were 15 cases (39.5%) in non-survival group, aged (40.0±15.8) years, and there were 12(80.0%) female, the ECMO time was (120.1±72.4) h in this group. The proportion of tracheal intubation and continuous renal replacement therapy in the survivor group and dosage of norepinephrine within 24 h after ECMO implantation were significantly less than in non-survival group (all P<0.05). There was no significant difference in all efficacy related biochemical indexes between two groups before ECMO use. The levels of lactic acid, procalcitonin, creatinine, alanine aminotransferase, aspartate aminotransferase, creatine kinase-MB, cardiac troponin I and N-terminal B-type natriuretic peptide prosoma were significantly less in survival group than in non-survival group at 24 h after the use of ECMO (all P<0.05). Results of multivariate logistic regression analysis showed that the higher 24 h change rate of creatinine (OR=0.587, 95%CI 0.349-0.986, P=0.044) and creatine kinase-MB (OR=0.177, 95%CI 0.037-0.841, P=0.029) were positively correlated with reduced risk of in-hospital mortality. The central hemorrhage and acute kidney injury in survival group were less than in non-survivor group (P<0.05). Conclusions: After 24 h early use of ECMO in FM patients, the improvement of various efficacy related biochemical test indexes in the survival group was better than that in the non-survival group. Faster reduction of creatine kinase-MB and creatinine values within 24 h ECMO use is positively correlated with reduced risk of in-hospital mortality in adult patients with FM.

Meta-analysis and GRADE evaluation of Shuxuetong Injection in treatment of stroke in progressive / 中国中药杂志

This study aims to systematically evaluate the efficacy and safety of Shuxuetong Injection in the treatment of stroke in progressive. Randomized controlled trials of Shuxuetong Injection in the treatment of stroke in progressive were searched from CNKI, Wanfang, VIP, CMB, PubMed and EMbase. After strict literature screening, data extraction and quality evaluation, a total of 22 articles were included for analysis by RevMan 5.3. The Meta-analysis showed that Shuxuetong Injection combined with conventional treatment was superior to the conventional treatment alone in the major outcome indicators including effective rate(RR=1.27, 95%CI[1.20, 1.33], Z=9.18, P<0.000 01), deterioration rate(RR=0.38, 95%CI[0.22, 0.68], Z=3.31, P=0.000 9), NIHSS scores(MD=-3.89, 95%CI[-4.34,-3.43], Z=16.83, P<0.000 01), CSS scores(MD=-5.59, 95%CI[-6.42,-4.76], Z=13.20, P<0.000 01) and activity of daily living scores(MD=12.02, 95%CI[10.31, 13.72], Z=13.83, P<0.000 01), mortality during treatment was not increased(RR=0.40, 95%CI[0.13, 1.26], Z=1.56, P=0.12). Moreover, Shuxuetong Injection combined with conventional treatment further reduced the secondary outcome indicators including fibrinogen(MD=-0.35, 95%CI[-0.58,-0.13], Z=3.09, P=0.002), triglyceride(MD=-0.38, 95%CI[-0.67,-0.10], Z=2.65, P=0.008), low density lipoprotein cholesterol(MD=-0.72, 95%CI[-0.83,-0.61], Z=12.64, P<0.000 01), serum hypersensitive C-reactive protein(MD=-4.41, 95%CI[-6.96,-1.86], Z=3.38, P=0.000 7), and interleukin-6(MD=-5.43, 95%CI[-6.91,-3.96], Z=7.22, P<0.000 01). GRADE evaluation results showed that the major outcome indicators had low quality of evidence. Shuxuetong Injection in the treatment of stroke in progressive can improve the clinical effective rate, reduce the deterioration rate, improve the neurological function and activity of daily living, down-regulate the levels of fibrinogen, triglyceride, low density lipoprotein cholesterol and alleviate the inflammatory response. Although most studies have reported no adverse reactions, there are selective reports. The safety of Shuxuetong Injection needs to be further verified by more high-quality randomized controlled trial.

Meta-analysis of Tanreqing Injection in adjuvant treatment of stroke-associated pneumonia / 中国中药杂志

To systematically evaluate the efficiency and safety of Tanreqing Injection in the treatment of stroke-associated pneumonia(SAP). Seven domestic and foreign databases(CNKI, Wanfang, VIP, CBM, PubMed, Cochrane Library, EMbase) were retrieved from the establishment to July 2020. According to the inclusion and exclusion criteria, randomized controlled trial of the effect of Tanreqing Injection in the treatment of SAP was selected. NoteExpress software was used to screen out literatures. RevMan 5.4 software was used for data analysis. GRADE system was used to evaluate the evidence quality of the outcome indicators. A total of 1 755 cases in 21 studies were retrieved, including 879 cases in experimental group and 876 cases in control group. In general, the quality of stu-dies received was not high. According to Meta-analysis,(1) in terms of shortening the length of hospital stay, Tanreqing Injection combined with conventional western medicine was better than conventional western medicine(MD=-4.04, 95%CI[-4.43,-3.65], P<0.000 01);(2) in terms of increasing effective rate, Tanreqing Injection combined with conventional western medicine was better than conventional western medicine(RR=1.22, 95%CI[1.17, 1.27], P<0.000 01);(3) in terms of reducing inflammation indicators, Tanreqing Injection combined with conventional western medicine was better than conventional western medicine(MD_(CRP)=-10.75, 95%CI[-15.61,-5.88], P<0.000 01; MD_(WBC count)=-1.62, 95%CI[-2.55,-0.69], P=0.000 6; MD_(PCT)=-0.58, 95%CI[-0.89,-0.26], P=0.000 3];(4) in terms of improving symptoms and signs, Tanreqing Injection combined with conventional wes-tern medicine was better than conventional western medicine(MD_(cough)=-2.73, 95%CI[-4.93,-0.53], P=0.02; MD_(antipyretic)=-1.07, 95%CI[-1.17,-0.98), P<0.000 01];(5) in terms of decreasing the NIHSS scores, Tanreqing Injection combined with conventional western medicine was better than conventional western medicine(MD=-3.02, 95%CI[-4.91,-1.13], P=0.002);(6) in terms of adverse reactions, there was no statistically significant difference between Tanreqing Injection combined with conventio-nal western medicine compared with conventional western medicine treatment(RR=1.19, 95%CI[0.61,2.29], P=0.61). GRADE system showed that the evidence levels of above outcome indicators were low and extremely low. The results proved that Tanreqing Injection combined with conventional western medicine had a good advantage in the treatment of SAP, with better observation indicators better than western medicine conventional treatment, and no increase in the incidence of adverse reactions. However, this study had certain limitations. The overall quality of the included studies was low, which affected the reliability of the results. Therefore, the conclusions of this study shall be used cautiously.

Exploration on rationality evaluation approach of drug combination medication based on sequential analysis and machine learning / 中国中药杂志

Drug combination is a common clinical phenomenon. However, the scientific implementation of drug combination is li-mited by the weak rational evaluation that reflects its clinical characteristics. In order to break through the limitations of existing evaluation tools, examining drug-to-drug and drug-to-target action characteristics is proposed from the physical, chemical and biological perspectives, combining clinical multicenter case resources, domestic and international drug interaction public facilities with the aim of discovering the common rules of drug combination. Machine learning technology is employed to build a system for evaluating and predicting the rationality of clinical drug combinations based on "drug characteristics-repository information-artificial intelligence" strategy, which will be debugged and validated in multi-center clinical practice, with a view to providing new ideas and technical references for the safety and efficacy of clinical drug use.

Study on the influence of risk attitude to hepatitis B vaccination behavior of residents in suburban areas / 中华疾病控制杂志

Objective To explore the influence of risk attitude to hepatitis B vaccination behavior of residents in suburban areas, and provide evidence for improvement of health education. Methods A total of 1 031 adults aged 16-60 years old were selected from 6 villages in Ninghe county and Jinghai county. A questionnaire was used to investigate them. Results Logistic regression analysis showed that respondents’ hepatitis B vaccination behavior was affected by their risk attitude when controlling other factors. The younger (OR=0.94, 95% CI:0.93-0.96, P＜0.001), unmarried (OR=8.24, 95% CI:2.89-23.60, P＜0.001), low self-rated health (OR=1.78, 95% CI:1.53-3.49, P=0.008), the formal sector workers (OR=7.18, 95% CI:2.29-22.54, P=0.001), covered by health insurance (OR=8.46, 95% CI:2.31-30.86, P=0.001), risk aversion (OR=1.65, 95% CI:1.06-2.57, P=0.026) and risk neutral (OR=1.50, 95% CI:1.03-2.17, P=0.032) were more likely to choose hepatitis B vaccination. Conclusions For the risk aversion and risk neutral, health education on disease symptoms and disease economic burden should be enhanced; for the risk seeking, more knowledge on prevalence trend and transmission route need to be improved.

Dynamics of Proliferation and Differentiation after Transplantation of Hematopoietic Cells in Mouse / 中国实验血液学杂志

OBJECTIVE@#To observe the dynamic changes of hematopoietic reconstitution and multiple lineages differentiation at early phase after transplantation.@*METHODS@#Whole bone marrow mononuclear cells (wBMMNC, 5×10) and enriched c-Kit hematopoietic stem/progenitor cells (HSPC, 3×10) from the BM of B6-Ly5.1 mice were transplanted into lethally irradiated B6-Ly5.2 mice, the frequencies and absolute numbers of donor-derived cells (including LKS and LKS) were detected by flow cytometry. The multiple lineages differentiation of donor-derived cells was also monitored by flow cytometry. The homing and early phase proliferations of donor-derived cells were observed by two-photon microscope.@*RESULTS@#The donor-derived cells started to proliferation from 5-7 days after transplantation and reached the peak value at 2-3 weeks after wBMMNC transplantation. The donor-derived cells proliferated from 1-2 weeks and maintained until 4 weeks after c-kitHSPC transplantation. At 1 week after transplantation, the donor-derived cells mainly differentiated into myeloid cells with a few lymphoid cells production (B cells) but the production of T cells was not observed at most in wBMMNC transplanted group, while myeloid cells occupied the majority of donor-derived cells at 2-4 weeks; donor-derived cells almost totally differentiated into myeloid cells at 1-3 weeks after transplantation in c-Kit transplanted group and donor-derived B cells appeared at 4 weeks. The absolute number of donor-derived LKS and LKS cells in the BM of c-Kit transplanted group were much higher than that of wBMMNC group (P＜0.001) at 2 weeks respectively. The clustering proliferation of cKit cells at 4-5 days after transplantation was observed by two photon microscope.@*CONCLUSION@#The dynamical rate of proliferation and reconstitution of donor-derived cells are much earlier and quicker in c-Kit group than those of wBMMNC group. c-Kit cells mainly differentiate into myeloid cells within 1-3 weeks and the lymphoid cell differentiation starts at 4 weeks after transplantation. The immediate proliferation and differentiation of c-Kit cells within 1 week maybe due to the urgent needs of hematopoietic regeneration under the myeloablated hosts.

Comparison of Different Staging Systems and Prognostic Analysis in 68 Cases of Primary Intestinal Diffuse Large B Cell Lymphoma / 中国实验血液学杂志

OBJECTIVE@#To compare the prognostic value of different staging systems in primary intestinal diffuse large B cell lymphoma（PI-DLPCL）, and their correlation with clinicopathological characteristics，treatment and prognosis of PI-DLBCL.@*METHODS@#A total of 68 patients with PI-DLBCL were recruited from January 2009 to July 2017. All the patients underwent staging by using TNM, Lugano, Blackledge and Musshoff system, survival curves for the PI-DLBCL patients were plotted using the Kaplan-Meier method and were judged by the log-rank test. The accuracy of each staging system for predicting survival of PI-DLBCL patients was evaluated by calculating the area under curve(AUC) of the receiver operating characteristic(ROC). The correlation of the 4 staging systems, clinical features patients and treatment regimes with PFS and OS were analysed.@*RESULTS@#The median follow-up time was 52 (1-105) months, the median PFS time was 41(1-86) months, patients did not reached the median OS time. The most frequently involved site was ileocecal (30.9%), followed by small intestine (29.4%) and colon (29.4%), multiple sites involvement (7.4%) and rectum (2.94%)．The PFS and OS rates at 5-year were 44.9% and 51.1%, respectively. Kaplan-Meier survival curves and log-rank test results showed that using different staging systems to describe the cumulative retention rates of PFS and OS in PI-DLBCL patients, none of the 4 staging systems can distinguish the survival curves of each stage significantly. The results of ROC curve showed that the prediction ability of the Lugano staging system was better than other staging system for 1 year PFS （AUC=0.826；P=0.015）and 1 year OS（AUC=0-792；P=0.001） in PI-DLBCL patients. The 3 year PFS rate in the operation+chemo or radio-therapy group (62 cases) and the single operation group (6 cases) were 53.9% and 16. 7%,respectively（P=0.116），The 3 year OS rate were 66.7% and 16.7%（P=0.015）,respectively. Patients who received chemotherapy combined with rituximab had a higher 3-year PFS(66.0％ vs 44.0％，P=0.139) and 3.year OS(70.2% vs.39.2%，P=0.148).The patients with ileocecal lesion had higher PFS rate and OS rate than other sites(P<0.05). Multivariate Cox regression analysis indicated that only bone marrow invasion was an independent prognostic factor in patients with PFS.@*CONCLUSION@#Bone marrow invasion is an independent risk factor for PFS in patients with PI-DLBCL , according to this limited preliminary data，Lugano staging system for stratifying and predicting the prognosis of PI-DLBCL patients is better than other staging system.

Life Quality and Its Related Factors of Patients with Multiple Myeloma during Maintenance Therapy / 中国实验血液学杂志

OBJECTIVE@#To evaluate the quality of life (QOL) on patients with multiple myeloma(MM) during maintenance therapy and to explore the related factors important for QOL.@*METHODS@#The demography, clinical and laboratorial data of 66 MM patients during maintenance therapy were collected and explored by using a cross-sectional question naire(EORTC QLQ C30 V 3.0). The statistical analysis was performed using Nowegram normal mode(NM) and reference values(RV) of MM patients which were used as control.@*RESULTS@#In comparison with Nowegran normal mode, the scores of general health status, physical function, role function and social function of patients during maintenance therapy were lower than those of normal mode (61.3, 73.9, 65.4 and 65.2 vs 75.3, 89.9, 83.3 and 85.8 respectively), while the scores of constipation and financial difficulty were higher than those of normal mode(16.7 and 44.4 vs 10.7 and 9.7 respectively) (P<0.05). In comparison with reference values, the scores of general health status, emotional and coguitive functions of patients during maintenance therapy were significantly higher than those of reference values(61.3, 81.7 and 84.3 vs 55.7, 71.3 and 78.1 respectively) (P<0.05). In addition, the maintenance therapy yet decreasd the scores of fatigue, nausea and vomiting, pain, dyspnoea, insomnia, appetite loss and constipation of patients, but increased the score of financial difficulty of patients (P<0.05). The age of initial diagnosis, serum LDH level, peripheral neuropathy, high ratio of own expense and underlying diseases were main factors affecting the general health status of patients (P<0.05), while the decrease of Hb level, increase of blood Ca level and accompanied genetic changes negatively influence the QOL (P<0.05), while the high culture level showed positive effect on QOL (P<0.05). The choise of drugs for maintenace (therapy thalidomide and bortezomib) not had significant effect on QOL of patients.@*CONCLUSION@#The maintenance therapy can improve the QOL of MM patients, the age at initial diagnses, serum LDH level, peripheral neuropathy and high ratio of own expence are the main factors affecting the QOL of MM patients.

Expression of MiRNA-19a in Cells of Multiple Myeloma Patients and Its Effect on the Characteristics of Myeloma Cells / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To detect the expression level of miR-19a, one of the oncogenic miR-17-92 cluster, in multiple myeloma cell lines Lp-1 and U266 in vitro and to explore the effects of miR-19a on biological behavior, such as proliferation, migration and apoptosis of Lp-1 and U266 myeloma cells by transfection with miR-19a mimic through Lipofectamine2000.</p><p><b>METHODS</b>The reverse transcription-PCR was applied to detect the expression level of miR-19a in multiple myeloma cell lines Lp-1 and U266 in vitro. The CCK8 was used to assay the effect of miR-19a on the proliferation of Lp-1 and U266 cells in vitro, the transwell migration test was adopted to determine the effect of up-regulation of miR-19a on the migration of Lp-1 and U266 multiple myeloma cells in vitro. The flow cytometry was used to detect the effect of miR-19a on the apoptosis of Lp-1 and U266 cells in vitro.</p><p><b>RESULTS</b>The miR-19a expression was higher in Lp-1 and U266 multiple myeloma cells; compared with the transfected cells with a specific miR-19a NC, those samples transfected with miR-19a mimic displayed significantly higher expression of miR-19a (P<0.05), indicating a higher transfection efficiency; the miR-19a could promote the proliferation of Lp-1 and U266 multiple myeloma cells in vitro. MiR-19a could promote migration ability of Lp-1 and U266 multiple myeloma cell lines in vitro and could inhibit the apoptosis of Lp-1 and U266 cells.</p><p><b>CONCLUSION</b>miR-19a is overexpressed significantly in Lp-1 and U266 multiple myeloma cells, and promots the proliferation and invasion of the myeloma cells, but inhibits their apoptosis.</p>

Clinical Analysis on the Therapeutic Efficacy of Autologous Hematopoietic Stem Cell Transplantation in 56 Multiple Myeloma Patients / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To evaluate the therapeutic efficacy and prognosis of autologous stem Hematopoietic cell transplantation (auto-HSCT) in multiple myeloma (MM) patients.</p><p><b>METHODS</b>A retrospective study was conducted for 56 patients diagnosed with MM and then received auto-HSCT in our hospital from December 2008 to September 2016.</p><p><b>RESULTS</b>All the patients successfully underwent hematopoietic reconstruction without transplantation-related mortality (TRM). The complete response (CR) rate of all the patients after induction chemotherapy was 23.2% (13/56), while the CR rate of these patients with auto-HSCT increased to 78.6% (44/56) (P<0.01). The CR plus VGPR (very good partial response) rates of these 56 patients after induction chemotherapy and auto-HSCT were 53.6%（30/56）and 94.6%（53/56） respectively (P<0.01). The median progression-free survival (PFS) time and median overall survival (OS) time were 37 and 71 months, respectively. The median PFS time in the patients with induction therapy containing bortezomib was 37 months, however, the median OS time did not reach to 71 months; the median PFS (P<0.01) and the median OS (P<0.01) in the patients with the induction chemotherapy without bortezomib was 27 and 51 months, respectively. Univariate analysis demonstrated that the patients maintained CR or VGPR after auto-HSCT or with less than 6 cycles of induction chemotherapy significantly correlated with PFS (P<0.01).</p><p><b>CONCLUSION</b>auto-HSCT can further increase the CR rate, prolong PFS and OS time. Sequential auto-HSCT after bortezomib-based therapy is the first line therapy for the transplant-eligible MM patients. Maintenance treatment is beneficial to the sustained CR+VGPR patients after auto-HSCT.</p>

Clinical Analysis of Maintenance Therapy with Thalidomine and Bortezomib for Multiple Myeloma / 中国实验血液学杂志

OBJECTIVE@#To evaluate the therapeutic effect and adverse reactions of the maintenance therapies with Thalidomine or Bortezomib in the patients with newly diagnosed multiple myeloma (MM), so as to provide a reference for clinical treatment.@*METHODS@#A retrospective analysis was conducted to compare the progression-free survival (PFS), overall survival (OS) and adverse reaction rate of 23 MM patients received the maintenance therapies of Bortezomib and of 68 MM patients received maintenance therapy of Thalidomine.@*RESULTS@#The maintenance therapy with Bortezomib could extend the PFS of MM patients as compared with Thalidomine (PFS rate of patients on the maintenance therapy of Bortezomib in 12th, and 24th month was 100%, 88.89%, and that of Thalidomine-treated group was 72.31%, 47.54%). What's more, some specific patients could get better 2-year PFS rate in Bortezomib group than that in Thalidomine group, such as older than 65 years old, after autologous hematopoietic stem cell transplantation(ASCT), having genetic changes, extramedullary lesions, poor renal function, low serum free light chain ratio, high β2-MG, anemia, high LDH, VGPR of induction and consolidation therapy. The OS rate of Bortezomib on 18th, 24th and 30th month was 100%, 88.89%, 80% verus 91.52%,83.63%,72.90% of the group with thalidemide at the same time. As for 2-year OS rate, the Bortezomib group was higher than Thalidomine without statistical differences. However, the patients such as older than 65 years old, poor renal function and with extramedullary lesions, would also get higher 2-year OS rate from Bortezomi. Bortezomib and thalidomide could cause bone marrow suppression, peripheral neuritis and other adverse reactions.@*CONCLUSION@#The efficacy of maintenance therapy with Bortezomib is superior to thalidomide. As a conclusion, bortezomib is a better option for maintenance therapy of MM patient.

Role of Whole-exome Sequencing in Phenotype Classification and Clinical Treatment of Pediatric Restrictive Cardiomyopathy / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Restrictive cardiomyopathy (RCM) is the least common cardiomyopathy in which the walls are rigid and the heart is restricted from stretching and filling properly. Cardiac troponin I (cTnI) mutation-caused myofibril Ca2+ hypersensitivity has been shown to be associated with impaired diastolic function. This study aimed to investigate the linkage between the genotype and clinical therapy of RCM.</p><p><b>METHODS</b>Five sporadic pediatric RCM patients confirmed by echocardiography were enrolled in this study. Whole-exome sequencing (WES) was performed for the cohort to find out candidate causative gene variants. Sanger sequencing confirmed the WES-identified variants.</p><p><b>RESULTS</b>TNNI3 variants were found in all of the five patients. R192H mutation was shared in four patients while R204H mutation was found only in one patient. Structure investigation showed that the C terminus of TNNI3 was flexible and mutation on the C terminus was possible to cause the RCM. Catechins were prescribed for the five patients once genotype was confirmed. Ventricular diastolic function was improved in three patients during the follow-up.</p><p><b>CONCLUSIONS</b>Our data demonstrated that TNNI3 mutation-induced RCM1 is the most common type of pediatric RCM in this study. In addition, WES is a reliable approach to identify likely pathogenic genes of RCM and might be useful for the guidance of clinical treatment scheme.</p>

Differentially expressed genes in heads and tails of Angelica sinensis diels: Focusing on ferulic acid metabolism / 中国结合医学杂志

<p><b>OBJECTIVE</b>To explore the scientific connotation of the discrepant pharmaceutical activities between the head and tail of Angelica sinensis diels (AS), an important herb extensively utilized in Chinese medicine, by the approach of transcriptome sequencing.</p><p><b>METHODS</b>Ten samples of AS were randomly collected in Min County, Gansu Province of China. Transcriptome sequencing of AS was accomplished in a commercial ILLumina HiSeq-2000 platform. The transcriptome of each head and tail of AS were fixed in a gene chip, and detected under the procedure of Illumina HiSeq-2000. Differentially expressed unigenes between the heads and tails of AS were selected by Shanghai Biotechnology Corporation (SBC) online analysis system, based on Gene Ontology, Kyoto Encyclopedia of Genes and Genomes and relevant bioinformatic database.</p><p><b>RESULTS</b>Totally 63,585 unigenes were obtained from AS by high-throughput sequencing platform. Among which 3359 unigenes were identified as differentially expressed unigenes between the heads and tails of AS by SBC analysis system scanning. Of which 15 differentially expressed unigenes participate in the metabolic regulation of phenylpropanoid biosynthesis (PB) pathway and ferulic acid metabolites, in response to the distinguished pharmaceutical actions of the heads and tails of AS.</p><p><b>CONCLUSION</b>Different content of ferulic acid in the heads and tails of AS is related to the differentially expressed genes, particularly involved in the PB pathway.</p>

Effect of Rigorous Staging at the First Diagnosis on Prognosis of Patients with Follicular Lymphoma / 中国实验血液学杂志

<p><b>BACKGROUND</b>A survey of early stage follicular lymphoma(FL) revealed that the rigorously staged FL patients at first diagnosis had a better outcome as compared with non-rigorous staged FL patients, but there were no similar reports in China.</p><p><b>OBJECTIVE</b>To explore the relationship between the rigorous staging at first diagnosis and the prognosis of FL patients at different stages.</p><p><b>METHODS</b>The clinical data of 111 patients with newly diagnosed FL from 2008 to 2014 year were collected and analyzed. The rigorous staging included: (1) bone marrow aspiration and biopsy, (2) imaging examination of whole body including CT and ultrasounic scan, or PET/CT, either or both is defined as rigorous staging, or else as non-rigorous staging.</p><p><b>RESULTS</b>The FL patients at I-II stages by rigorous staging showed a superior progression-free survival(PFS) compared with non-rigorous staging patients(P=0.048). For all the patients, the age, serum LDH, bone marrow lesion and more than 3 foci of diameter larger than 3 cm correlated with prognosis in univariate analysis, and multivariate analysis revealed that the age, serum LDH and bone marrow imolvement were the independent prognostic factors.</p><p><b>CONCLUSION</b>Rigorous staging leads to better outcomes, suggesting that accurate and appropriate testing is important for the patients at the first treatment. The close correlation of bone marrow with prognosis indicates that the evaluation of bone marrow is very important for the daily clinical practice.</p>

Balloon Angioplasty as a Modality to Treat Children with Pulmonary Stenosis Secondary to Complex Congenital Heart Diseases / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Pulmonary stenosis is common in children with complex congenital heart diseases. Proper management of this problem, especially postoperatively, is still controversial. This study was designed to assess the rate and determinants of success or failure of balloon angioplasty for such lesions.</p><p><b>METHODS</b>Clinical and hemodynamic data from 40 pediatric patients (24 boys and 16 girls) with complex congenital heart diseases who underwent balloon angioplasty were reviewed retrospectively from January 2012 to December 2016. Patients were divided into four groups according to the site of stenosis, which included pulmonary valve stenosis (PVS), valved conduit stenosis, pulmonary artery stenosis (PAS), and supravalvular pulmonary stenosis (SVPS). Success rates were calculated according to defined criteria for initial success and favorable clinical impacts, and comparison between the successful subgroup and the unsuccessful subgroups was analyzed.</p><p><b>RESULTS</b>Grouped by the site of stenosis, initial success rates varied from 40.0% to 52.4% with the greatest success being seen in the PVS group, followed by the PAS group and SVPS group. In the PVS group and the PAS group, there was no statistical difference among age at dilation, postoperative interval, balloon/stenosis ratio, or pressure gradient predilation between the successful and the unsuccessful subgroups. Favorable clinical impacts included success rates of balloon angioplasty in the SVPS group, which was best (100%), followed by the PVS group (90.9%) and the PAS group (85.7%). There were a total of two transient complications (5.0%).</p><p><b>CONCLUSIONS</b>Balloon angioplasty was proven to be a safe and useful modality in children with complex congenital heart diseases and postoperative pulmonary stenosis, which should be the initial therapeutic modality in selected patients.</p>

Analysis of Clinical Characteristics and Prognosis of 46 Elderly Patients with Peripheral T Cell Lymphoma (PTCL) / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To investigate the clinical characteristics and prognostic factors of patients with peripheral T cell lymphoma (PTCL).</p><p><b>METHODS</b>The clinical data of 46 elderly PTCL patients admitted in Tianjin Medical University Cancer Hospital from April 2008 to August 2014 were collected, the clinical features, prognostic factors and treatments, as well as followed-up outcome were analyzed retrospectively. Survival analysis was performed by Kaplan-Meier method, and the COX proportional hazard model was used to perform multivariate analysis.</p><p><b>RESULTS</b>The median survival time was 11 months, and the expected 1-year, 2-year and 3-year overall survival rate (OS) was 50%, 36% and 33%, respectively. Univariate analysis showed that the age, ECOG score, Charlson Comorbidity Index Score, the efficacy and course of chemotherapy were all the prognostic indicators affecting the OS and progression free survival (PFS) in this cohort of elderly patients. Multivariate analysis indicated that ECOG score and course of chemotherapy were the independent prognostic indicators affecting the OS and PFS (P < 0.05).</p><p><b>CONCLUSION</b>ECOG score and course of chemotherapy are of great significance for predicting the prognosis in elderly PTCL patients. The elderly patients's general condition and completion of a certain intensity of chemotherapy are an important measure to prolong survival time in elderly PTCL patients.</p>

Clinical Characteristics and Therapeutic Efficacy Evaluation of 14 Cases of Solitary Myeloid Sarcoma / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To investigate the clinical characteristics, treatment and prognosis of solitary myeloid sarcoma (MS).</p><p><b>METHODS</b>The clinical data of 14 solitary MS patients were retrospectively analysed, including their clinical features and treatment, and were evaluated.</p><p><b>RESULTS</b>A total of 14 cases of solitary MS mainly occurred in middle-aged population with the median age 41 years old (17-62 years old). The involved sites were more extensive, including breast, testis, spinal canal, skin, gastrointestinal system, nose and so on. The poorly differentiated cells of small to medium size showed diffuse distribution, relatively consistent morphology and a higher ratio of cytoplasm. The nucleus is in round or oval shape with fine and dense chromatin. Pathological mitosis was easily observed. Expression of MPO, lysozyme, CD43, myeloid-derived cells were positive. Treatment methods included surgery, chemotherapy and stem cell transplantation. Median survival time of 14 patients was 22.5 months; overall survival (OS) was 35.7% (5/14), median disease-free survival reached to 10.4 months on averge (3.5 months to 16 months), and 2-year overall survival (OS) was 50.3%.</p><p><b>CONCLUSION</b>The incidence of solitary MS is low, with a tendency progressing to leukemia, the chemotherapy regimen of anthracycline+cytarabine combined with radiotherapy can achieve better clinical efficacy.</p>

Analysis of Clinical Characteristics and Prognostic Factors of 223 Newly Diagnosed Hodgkin's Lymphoma Patients / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To analyze the clinical characteristics and prognostic factors of the newly diagnosed Hodgkin's lymphoma (HL) patients.</p><p><b>METHOD</b>The clinical data of 223 patients with pathologically confirmed and newly diagnosed HL (since January 2001 to December 2012) were collected from Tianjin Medical University Cancer Hospital. The patients' route features, laboratory examination results, clinical manifestations and short-term efficacy were analyzed. Furthermore, the Log-rank test and Cox proportional hazard model were applied to analyze the prognosis. All data were analyzed with SPSS software version 1.0 and graphPad Prism 5.</p><p><b>RESULTS</b>Among the 223 newly diagnosed HL patients, the ratio of male to female was approximately 1.25:1 and the median age was 40 years old (range 3-84 years). The median survival period is 62 months (range 8-189 months). The 5-year overall survival (OS) rate was 92.8% for all 223 HL patients. Multivariate analysis showed that HL originated from mediastinal lymph node region, the extranodal sites were involved, lactate dehydrogenase (LDH) level increased and CR2 was unable to fulfil the CR/CRu which are independent prognostic factors of HL patients (P < 0.05). Therefore, according to the above-mentioned adverse prognostic factors, 223 cases of HL patients were regrouped as low, intermediate and high risk. The HL 5-year OS rate of three groups were 100%, 96.9% and 42.9%, OS in each group was significantly different (P < 0.05).</p><p><b>CONCLUSION</b>HL displays a high incidence in young male patients but the curative efficacy is optimal and the survival period is long. The HL originated at mediastinal lymph node region, involved extranodal sites, lactate dehydrogenase (LDH) level increased and CR2 is unable to fulfil the CR/CRu which are independent prognostic factors of HL patients. According to the above-mentioned factors for the initial treatment of patients with HL, risk discrimination can predict the prognosis of patients with HL to a certain extent.</p>

A Retrospective Study of 1,526 Cases of Transcatheter Occlusion of Patent Ductus Arteriosus / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Patent ductus arteriosus (PDA) is one of the most common congenital heart diseases and began to get treated by transcatheter occlusion since 1997 in China. Since then, several devices have been invented for occluding PDA. This study aimed to evaluate the technical feasibility, safety, and efficacy of transcatheter occlusion of PDA with different devices.</p><p><b>METHODS</b>One thousand five hundred and twenty-six patients (537 boys, 989 girls) with PDA from January 1997 to September 2014 underwent descending aortogram and transcatheter occlusion procedure. We retrospectively analyzed data of these patients, including gender, age, weight, size and morphology of PDA, and devices used in transcatheter occlusion, outcomes, and postoperational complications.</p><p><b>RESULTS</b>Median age and median weight were 4.0 years (range: 0.3-52.0 years old) and 15.3 kg (range: 4.5-91.0 kg), respectively. Mean ductal diameter, aortic ductal diameter, ductal length, and pulmonary artery pressure were 3.50 ± 2.15 mm, 10.08 ± 2.46 mm, 7.49 ± 3.02 mm, and 30.21 ± 17.28 mmHg, respectively. Morphology of PDA assessed by descending aortogram was of type A in 1428 patients, type B in 6 patients, type C in 79 patients, type D in 4 patients, and type E in 9 patients according to the classification of Krichenko. Of all the 1526 patients, 1497 patients underwent transcatheter PDA closure, among which 1492 were successful. Devices used were Amplatzer duct occluder I (ADO I, 1280, 85.8%), Cook detachable coils (116, 7.8%), ADO II (ADO II, 68, 4.6%), muscular VSD occluder (12, 0.8%), and Amplatzer vascular plug (16, 1.0%).</p><p><b>CONCLUSIONS</b>Excellent occlusion rates with low complication rates were achieved with all devices regardless of PDA types. With transcatheter occlusion technique and devices developing, more patients with PDA can be treated with transcatheter closure both safely and efficiently.</p>