目的 了解老年血流感染(BSI)的病原菌分布和临床特点,分析老年BSI患者死亡相关的危险因素.方法 回顾性分析2016年1月-2020年12月在江西省赣州市兴国县人民医院的住院老年BSI患者的临床特点和死亡危险因素.结果 181例老年BSI患者共分离出197株细菌,其中革兰阴性菌120株(60.91％),革兰阳性菌68株(34.52％),真菌9株(4.57％).最常见的细菌依次为大肠埃希菌(33.50％)、肺炎克雷伯菌(14.21％)、金黄色葡萄球菌(11.68％)、粪肠球菌(4.06％)、表皮葡萄球菌(3.55％)和溶血葡萄球菌(3.55％).呼吸道来源的病原菌最多(32.14％),其次为泌尿道来源(27.86％)和导管相关来源(18.57％).合并肿瘤是老年BSI患者独立预后危险因素(P=0.006).结论 老年BSI患者病原菌以革兰阴性菌为主,大肠埃希菌和肺炎克雷伯菌是最常见的病原菌.合并肿瘤疾病是老年BSI危险因素.

目的:分析3例ANKRD26基因突变所致遗传性血小板减少症2型(THC2)的临床特征与基因突变家系谱,探讨ANKRD26基因突变类型与THC2临床表型之间的关系.方法:回顾性分析2017年5月-10月在医院血液肿瘤科诊治的3例THC2患儿临床资料与一般实验室检查结果,使用二代技术(NGS)进行全外显子检测(WES)、基因数据和致病突变验证三个主要步骤对先证者及其家庭成员ANKRD26基因进行测序、筛查和分析.结果:3例患儿初诊时均PLT<20×109/L,皆有淤点瘀斑,初诊时丙球及激素效果欠佳.基因分析结果均存在ANKRD26基因突变,突变位点分别位于第22号外显子、第1号外显子启动区与第30号外显子,突变类型均为杂合突变.结论:THC2是一组异质性疾病,其不同基因突变类型预后差别很大,ANKRD26基因多态性可能为影响THC2患儿临床表现的关键因素.

目的:探讨3例Wiskott-Aldrich综合征(WAS)的基因检测结果与临床特征.方法:分析3例WAS患儿的临床资料,使用第二代基因测序(NGS)对先证者及其家庭成员WAS蛋白(WASP)基因进行测序、筛查和分析.结果:3例患儿均有湿疹、反复感染及血小板减少,其中仅1例患儿表现为血小板体积减小.3例患儿均行NGS检测,发现WAS基因突变,其中2例突变点突变,1例为插入突变;家系验证结果显示,2例为X连锁遗传,1例为新生突变.结论:对月龄较小的血小板减少症男性患儿,如常规治疗效果欠佳,同时伴发免疫缺陷、湿疹,应及时完善WASP基因检测明确诊断,造血干细胞移植(HSCT)是有效的治疗手段.

目的 对非公立医疗机构助力上海市入境医疗旅游发展的情况进行探讨.方法 回顾性地分析2016-2018年上海市非公立医疗机构收治外籍住院患者的数量、地域分布、疾病构成、手术分级、疾病转归等,并与同期公立医疗机构收治外籍住院患者的情况进行比较.结果 在入境医疗旅游领域,非公立医疗机构具有效率优势和国际化优势,但仍需提升医疗技术、服务品质、品牌美誉度等.结论 非公立医疗机构应通过高质量的诊疗水平和优质的国际化服务管理模式不断提升自身的竞争力,充分利用当前的利好政策,助力上海市入境医疗旅游的发展.

阿米巴感染性肠炎是一种较少见的胃肠道感染性疾病，临床症状表现为不同程度的腹痛、腹泻伴血便，暴发型阿米巴肠病为其最严重的并发症，病死率高。本文回顾性分析复旦大学附属华山医院收治的1例以转移性右下腹痛表现起病，最终确诊为暴发型阿米巴肠病患者的临床资料和治疗过程，并结合国内外文献分析该病临床特点、影像学表现、病理特点及治疗。

The nuclear transcription factor twist-related protein 1 (Twist1) is associated with tumor malignant transformation and metastasis in various types of carcinomas. We found that Twist1 was highly expressed in clinical multiple myeloma (MM) cells, and explored its roles in proliferation and apoptosis in human MM cell lines U266 and RPMI-8226. In these cells, Twist1 transcriptionally regulated the miRNA hsa-miR138-5p, which targeted caspase-3 to control apoptosis. Silencing of Twist1 significantly suppressed cell proliferation and increased apoptosis, which was reversed by overexpression of hsa-miR138-5p or simultaneous silencing of caspase-3. This reversion was further substantiated by attenuated apoptotic signaling, including downregulated expression of the cleaved forms of caspase-3 and peroxisome proliferator-activated receptor 1 (PPAR1). We demonstrate here for the first time that the novel Twist1/hsa-miR138-5p/caspase-3 pathway contributes significantly to the proliferation and survival of human MM cells. Our study provides new insight for novel MM treatments by developing Twist1-targeted therapeutics.

[病例] 患者男性,89岁,因反复肺部感染,咳嗽、咳痰加重伴发热1d于2017年9月16日入院.患者既往有高血压、2型糖尿病、脑梗死后遗症(既往口服利伐沙班10 mg,qd)、冠心病、心房颤动、心功能不全、前列腺增生和高脂血症(口服非诺贝特0.16g,qn,控制血三酰甘油在3.63 ～4.52 mmol·L-1);无慢性肝病及肾脏病史,无药物不良反应史.患者在6月9日至23日因肺部感染使用美罗培南+替加环素后出现三酰甘油升高至15.51mmol· L-1,后经停用替加环素、低脂饮食等治疗,三酰甘油下降至4.34 mmol·L-1,感染好转.

IncRNAs play an important role in the regulation of gene expression. The present study profiled differentially expressed lncRNAs (DELs) and mRNAs (DEMs) in myelodysplastic syndrome (MDS) to construct a 4-aminobutyrate aminotransferase (ABAT)-DEL-DEM co-expression network in MDS development using the Agilent human BeadChips and Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway and network analyses. Compared with controls, there were 543 DELs and 2,705 DEMs in MDS patients, among which 285 (52.5%) DELs were downregulated and 258 (47.5%) DELs were upregulated, whereas 1,521 (56.2%) DEMs were downregulated and 1,184 (43.70%) DEMs were upregulated in MDS patients. The ABAT-DEL-DEM co-expression network contained six DELs that were co-expressed with ABAT in MDS. The GO analysis revealed that the co-expression network mainly participated in response to organic cyclic compound, cell proliferation, cell part morphogenesis, regulation of cell proliferation and enzyme-linked receptor protein signaling pathways, while the KEGG database showed that the co-expression network was involved in various pathways, such as phagosome and metabolic pathways. Furthermore, the expression of a selected DEL (lncENST00000444102) and ABAT was shown to be significantly downregulated in MDS patients, and in SKM-1 and THP-1 cells. The selected lncENST00000444102 was then overexpressed and ABAT expression was knocked down in the MDS cell lines using lentiviral transfection. In addition, lncENST00000444102 overexpression reduced the viability and increased the apoptosis of MDS cells, ABAT expression was upregulated by lncENST00000444102.

In our previous study, the 4-aminobutyrate aminotransferase (ABAT) gene was screened and selected as a target gene that may affect the prognosis of myelo-dysplastic syndrome (MDS). The present study aimed to determine the prognostic value of ABAT in 152 patients with MDS, 29 patients with acute myeloid leukemia (AML) and 40 controls, by detecting the expression and methylation levels of the ABAT gene. In patients with MDS, the expression levels of ABAT were significantly reduced compared with in the controls (P<0.0001), and the degree of DNA methylation was increased in MDS subjects (P<0.0001). Age, hemoglobin level, marrow blasts, International Prognostic Scoring System karyotype, and the expression and methylation levels of ABAT were associated with overall survival (OS), as determined by univariate analysis. Multivariate analysis revealed that older age, higher marrow blasts and higher methylation percentage were independent risk factors for OS. In addition, a functional study demonstrated that ABAT gene silencing increased cell apoptosis and blocked the G(1)/S phase in SKM-1 and THP-1 human leukemia cells. A -aminobutyrate aminotransferase inhibitor also blocked the G(1)/S phase; however, it had no effect on cell apoptosis. In conclusion, the present study demonstrated that ABAT methylation served an essential role in the progression of MDS and therefore may be considered an indicator of poor prognosis for hematological malignancies.

目的:回顾性分析国内文献报道的系统性肥大细胞增生症(SM)的临床特征,探讨SM的诊断,不同分型的治疗及预后特点.方法:回顾性分析2000-01—2016-12国内文献报道的30例及我院收治的1例SM患者.结果:共检索到文献26篇,其中24篇文献报道了30例诊断明确、资料完整的SM.其不同分型临床表现具有异质性,主要依靠病理诊断.根据2016年WHO诊断及分型标准,10例诊断为惰性肥大细胞增生症(ISM),11例诊断为侵袭性肥大细胞增生症(ASM),3例SM相关的非肥大细胞系单克隆性血液病(SM-AHN),7例肥大细胞白血病(MCL).SM的治疗目标在于消除肥大细胞释放介质引起的症状和控制肥大细胞增生.ISM患者给予抗组胺药物、糖皮质激素、γ-干扰素、甲磺酸伊马替尼等治疗,疗效佳,患者基本都可以获得缓解.而ASM的疗效较差,11例ASM患者中4例治疗无效,包括2例死亡.SM-AHN、MCL疾病进展较快,确诊时已有多器官、多系统累及,无有效的治疗方案,死亡率极高.结论:SM是一种罕见疾病,不同分型具有较强的异质性,其诊断主要依赖于病理学检查.目前尚无根治方法,治疗目标在于控制症状和改善生活质量.ISM预后相对较好,ASM、SM-AHN、MCL多数诊断时已累及到其他器官、系统,病情较重,预后较差,死亡率高.

Objective To investigate the quantitative relationship between serum erythropoietin (sEPO)levels and hemoglobin(Hb)in iron deficiency anemia(IDA)patients.Methods A total of 170 patients with mild,moderate and severe IDA were collected.The levels of sEPO and Hb were measured, the correlation between sEPO and Hb levels was analyzed and a regression equation was established. Results In 170 patients with IDA,there was a strong negative correlation between sEPO and Hb (r= -0.810,P<0.001).The regression equation between sEPO and Hb was sEPO=1 084.00-10.68×Hb. In mild IDA group,the correlation between sEPO and Hb was weak(r= -0.313,P=0.010).The regression equation was sEPO=299.30 -2.39 ×Hb and the predictable range was 12.5-84.2 IU/L.In moderate IDA group,sEPO and Hb showed obvious negative correlation(r= -0.519,P<0.001).The regression equation was sEPO =1 063. 07 -11. 25 × Hb and the predictable range was 50. 57-388. 07 IU/L. The correlatio n between sEPO and Hb in severe IDA group was significantly negatively correlated (r= -0.495,P = 0. 006). The regression equation was sEPO = 1 310. 97 - 13. 44 × Hb and the predictable range was 504.57-750.00 IU/L. Conclusion There is a quantitative correlation between sEPO and Hb levels in patients with IDA,which provides evidence for differential diagnosis and treatments by predicting the sEPO level of IDA patients.

Objective: The aim of this study was to estimate the diagnostic efficiency of aberrant immunophenotypes by flow cytometry (FCM) and generate a new scoring system of FCM for differential diagnosis between refractory cytopenia with multilineage dysplasia (RCMD) and aplastic anemia (AA). Methods: This prospective study with immunophenotypes of RCMD (including HRCMD) and AA bone marrows was analyzed by FCM, in a blinded fashion and compared with normal controls, to identify aberrant immunophenotypes. Diagnostic efficiency of aberrant immunophenotypes was evaluated by single and multi-parameter diagnostic tests. Based on comprehensive analysis of the diagnostic values of each aberrant immunophenotype, a new scoring system of FCM was generated. Results: In single parameter diagnostic tests between RCMD and AA, the specificity was 75 similar to 100%, but showed a very low sensitivity from 5.4%-50%. Only the parameters of CD34(+) >= 1% and myeloblasts >= 3% in myeloblasts showed diagnostic significance, with an AUC >0.7. Similar results were observed between HRCMD and AA. In multi-parameter diagnostic tests, the optimal combination was CD34+ cells >= 1%, myeloblasts >= 3% in myeloblasts, and CD117 aberrancy in granulocytes with less parameters and with a comparatively better diagnostic value of sensitivity of 63.1%, specificity of 92.2%, and AUC of 0.79. AUC of the new scoring system of FCM was 0.836 +/- 0.02 (95% CI: 0.79-0.88) for differential diagnosis between RCMD and AA, with 0.8129 +/- 0.03, 95% CI: 0.69-0.86, between HRCMD and AA. Conclusion: The immunophenotypes of CD34+ cells >= 1%, myeloblasts >= 3%, and CD117 aberrant expressions were the most important in differential diagnosis between RCMD and AA. The new scoring system of FCM was an independent predictor for differential diagnosis between RCMD and AA and between HRCMD and AA.

Acute megakaryocytic leukemia (AMKL) is a rare subtype of acute myeloid leukemia (AML), which is challenging to diagnose due to frequent myelofibrosis (MF) and a low percentage of blast cells. In the present study, clinical characteristics and experimental observations in 9 adult patients diagnosed with AMKL, who were recruited by the Sino-U.S. Shanghai Leukemia Co-operative Group, were analyzed in order to summarize the diagnostic experience and provide recommendations on diagnosing AMKL. All the patients were diagnosed according to the 2008 World Health Organization diagnostic criteria. The mean age of the patients with AMKL was 59 years (range, 53-68 years). A total of 8 patients had different degrees of anemia, and 2 patients had <5% marrow blasts present in the bone marrow; however, the percentage of positive cells with cluster of differentiation (CD)41 and CD61 expression was >20%, as demonstrated by flow cytometry. A total of 6 patients were positive for platelet-specific antigens, as indicated by immunocytochemistry. Furthermore, 7 patients presented with moderate or marked MF, as demonstrated by a bone marrow biopsy. Karyotypic analysis indicated that 6 patients had abnormal karyotypes. Only 1 patient exhibited the Janus kinase 2V617F mutation. Treatment efficiency was notably poor, with a median survival time of 6.0 months (range, 1.1-24.0 months). In conclusion, the diagnosis of AMKL requires a combination of the results of bone marrow smears and bone marrow biopsy, immunophenotype or immunohistochemistry. We recommend that routine immunophenotypic analysis should include the CD41 and CD61 markers for diagnosing acute leukemia when bone marrow morphology does not indicate the diagnosis.

The aim of the present study was to investigate the predictive value of baseline serum microRNA (miRNA)-125b for nucleos(t)ide analogues (NAs) in patients with chronic hepatitis B (CHB). A total of 66 patients with Be antigen (HBeAg)-positive CHB received NAs therapy for 144 weeks. Serum miRNA-125b levels were measured at the baseline, while hepatitis B virus (HBV) DNA, hepatitis B surface antigen (HBsAg) and alanine aminotransferase (ALT) levels were measured throughout treatment. Stepwise logistic regression analysis was performed to identify predictors of treatment response. The results indicated that baseline serum miR-125b (OR=4.377; P=0.006), HBsAg (OR=0.120; P=0.010), ALT >5× upper limit of normal (ULN; OR=11.726; P=0.018) and undetectable HBV DNA at week 24 (OR=7.828; P=0.021) were independent predictors of complete response (CR) at 144 weeks (CR is defined as HBV DNA <500 IU/ml and HBeAg seroconversion). The baseline serum miRNA-125b combined with baseline HBsAg level yielded an area under the receiver-operating curve of 0.852 in discriminating CR and non-CR at 144 week. The combination of baseline miRNA-125b ≥1.7 and ALT >5× ULN had a positive predictive value 80% for CR at 144 weeks. The combination of baseline miRNA-125b ≥1.7 and HbsAg ≤4.4 (log10 IU/ml) had a negative predictive value of CR at 144 weeks of 100%. Together, these results suggest that baseline miRNA-125b is a reliable predictor of HBeAg seroconversion following NAs treatment. The present study may be used as a basis for the use of baseline miRNA-125b to optimize treatment prior to NAs therapy.

目的 探讨非维生素K拮抗剂口服抗凝药(non-vitamin K antagonist oral anticoagulants,NOAC)在合并非瓣膜性心房颤动(non valvular atrial fibrillation,NVAF)等多重合并症的复杂情况下脑卒中患者中应用的有效性及安全性.方法 对复旦大学附属华山医院国际医疗中心2015年8月1日～2016年8月31日收治的14例合并NVAF等多重合并症的复杂情况下脑卒中患者的临床资料进行随访分析.其中合并NVAF且同时合并颅颈动脉狭窄(颅内动脉、颈动脉)、颅外系统性栓塞、肿瘤、肥厚型心肌病等共10例,不合并NVAF但合并肿瘤、偏头痛、房间隔缺损、卵圆孔未闭等共4例.14例患者中8例用达比加群110 mg 2次/d,4例用利伐沙班20 mg1次/d,2例用利伐沙班15 mg 1次/d.结果 14例患者中合并心房颤动10例,CHA2DS2-VASc评分均≥2分,不合并心房颤动4例.所有患者随访时间1～14个月,平均随访8个月.1例患者确诊2周后再发急性脑梗死.1例患者确诊5d后短暂性脑缺血发作(transient ischemic attack,TIA).1例患者确诊2周后再发TIA,3个月后死于肠系膜动脉栓塞.1例患者确诊半年后因情绪障碍跳楼身亡.其余患者随访中未再有脑卒中事件复发或死亡.改良的Rankin量表评分4～5分2例(14.29％),2～3分2例(14.29％),0～1分8例(57.14％),死亡2例(14.29％).结论 NOAC对合并NVAF等多重合并症的复杂情况下脑卒中患者的预防和治疗可能安全有效,但需要进一步的临床随机试验来研究证实.

目的:运用磁敏感加权成像(SWI)研究高血压患者高血压分级与脑内微出血灶(CMB)之间的相关性以及心血管危险因素与CMBs之间的关系.方法:回顾性分析复旦大学附属华山医院国际医疗中心收治的28例在沪境外多国籍原发高血压患者,根据高血压患者血压情况及临床情况等影响预后的因素确定高血压分级及心血管危险分层.采用3.0T超导型磁共振成像仪采集常规MRI SWI图像后由本院两名影像科高级职称医师对SWI的校正后图像进行分析,识别脑内微出血,并确定微出血的数量.应用SPSS 15统计软件包,Spearman相关分析研究研究心血管危险因素、高血压分级与脑内微出血灶之间的相关性.Mann-Whitney检验比较低危-中危组与高危-很高危组SWI序列微出血阳性率差异.结果:SWI显示18例患者出现脑微出血灶,阳性率64％.高血压分级与脑内微出血灶之间Spearman相关分析结果:rs=0.623,P＜0.001,提示高血压患者随着血压级别的升高,CMBs数量显著性增加.不同心血管危险分层高血压患者脑内微出血灶显示,高危-很高危组SWI上微出血灶数量较低危-中危组明显增加(P＜0.005).其中,血清总胆固醇、血清三酰甘油分别与CMBs数量呈正相关(rs=0.5615、0.5562,P＜0.005).结论:高血压患者随着血压分级及心血管危险分层升高,脑内微出血灶数量显著增加.控制血压及血清总胆固醇、血清三酰甘油对预防CMBs发生有重要意义.

OBJECTIVE To establish a myelodysplastic syndrome transformed to leukemia cell line stably expressing green fluorescent protein (GFP), and to evaluate its biological characteristics and applications. METHODS SKM-1 cells were transfected by lentiviral particles with vector of GFP. The GFP positive single cell clone was isolated by limiting dilution and continued being cultured. The cells were injected into mice subcutaneously and were screened in vivo. Then SKM-1/GFP cells were obtained after tumour plaque was separated and cultivated. The cell morphology was observed by fluorescence microscopy. The GFP expression was further detected by flow cytometry. The cell proliferation was analysed by CCK-8 assay. SKM-1/GFP cells were inoculated to subcutaneous tissue of the immunodeficiency mice. The growth and invasion of the tumour were observed after tumour formation. RESULTS No differences in cell morphology and growth characteristics were observed between SKM-1 cells and SKM-1/GFP cells. The rate of GFP expression was 100%. No differences in cell proliferation were observed between SKM-1 cells and SKM-1/GFP cells. The tumour mass was observed after 14 days of subcutaneous vaccination in NOD/SCID mice. Spontaneous fluorescence from plaque was observed by living fluorescence microscopy at 30th day after vaccination. Homogenous GFP positive cells were observed by fluorescence microscopy in the frozen section of tumour mass. The invasion of SKM-1/GFP cells was also detected in heart, liver, stomach and kidney of mice. CONCLUSION A myelody-splastic syndrome transformed to leukemia cell line stably expressing green fluorescent protein has been established successfully, which can track tumor cell sensitively and can be applied to the research of minimal residual leukemia. The establishment of SKM-1/GFP cells may serve as a powerful means for studing myelodysplastic syndrome transformation.

Objective: To evaluate the effects of care bundle on relevant complications and postoperative recovery in patients with traumatic brain injury during nasal feeding. Methods: One hundred and sixty patients with traumatic brain injury were randomly divided into intervention group (I Group) and control group (C group) with 80 cases in each one. The I Group patients were treated with some care bundle measures: adjusting patients' position before nasal feeding, increasing the supply step by step and performing moderate massage after nasal feeding, while the C Group adopted the routine care. The incidence of complications, conditions of clinical recovery, nutritional status and satisfaction degree of care during nasal feeding were observed and compared between the two groups. Results: The overall incidence of complications during nasal feeding, such as vomiting, diarrhea, food reflux, constipation, gastric retention and tube slippage, in I Group was lower than that in C Group (P < 0.001). The number of cured patients in I Group was more than that in C Group, and the number of patients that did not response to the care in I Group was less than that in C Group. The overall effective rate of clinical recovery in I Group was 96.25%, which was better than that in C Group (P=0.004). The nutritional status of patients in I Group was better than that in the C Group after the care (P < 0.001). The overall satisfaction degree of care in I Group was 87.50%, which was higher than that in C Group (60%, P < 0.001). Conclusion: By applying care bundle to patients with traumatic brain injury, incidence of complications of nasal feeding can be decreased, recovery ability of patients can be strengthened, nutritional status of patients can be improved and the satisfaction degree of patients can be increased.

A multi-center study from the French Myelodysplastic Syndrome (MDS) Group confirmed that iron chelation therapy is an independent prognostic factor that can increase the survival rate of patients who are suffering from transfusion-dependent low-risk MDS. In this study, we aimed to explore this clinical phenomena in vitro, by exploring the synergistic effect of the iron chelator Deferasirox (DFX) and the DNA methyl transferase inhibitor Decitabine (DAC) in the leukemia cell lines SKM-1, THP-1, and K-562. Treatment with both DFX or DAC promoted apoptosis, induced cell cycle arrest, and inhibited proliferation in all three of these cell lines. The combination of DFX and DAC was much greater than the effect of using either drug alone. DFX showed a synergistic effect with DAC on cell apoptosis in all three cell lines and on cell cycle arrest at the G0/G1 phase in K-562 cells. DFX decreased the ROS levels to varying degrees. In contrast, DAC increased ROS levels and an increase in ROS was also noted when the two drugs were used in combination. Treatment of cells with DAC induced re-expression of ABAT, APAF-1, FADD, HJV, and SMPD3, presumably through demethylation. However the combination of DAC and DFX just had strong synergistic effect on the re-expression of HJV.