不同诱因噬血细胞综合征患者的临床特征及预后分析

目的: 探讨不同诱因噬血细胞综合征(hemophagocytic syndrome,HPS) 患者临床特征及对预后的影响。方法: 收集35例HPS患者的临床资料,根据原发疾病分为EB病毒感染组（12例）、肿瘤相关组（13例）和免疫疾病组（10例）。将EB病毒感染组患者HPS相关临床特征在2周内全面表现者设为早期表现组(5例),>2周者设为迟发表现组(7例)。EB病毒感染组及肿瘤相关组HPS患者中,对确诊并行干预性治疗120 d内死亡者设为近期死亡组,超过120 d死亡、包括长期存活者为非近期死亡组。分析HPS患者的临床表现、实验室检查和转归等指标。结果： HPS患者以发热、白细胞计数及血小板计数减少、肝功能损伤为初发表现多见,病程中多出现脾肿大、进行性贫血及全身多部位感染,部分患者有胸、腹腔、心包腔积液。EB病毒感染组谷草转氨酶、乳酸脱氢酶水平较肿瘤相关组明显升高,差异均有统计学意义（P<0.05）。免疫疾病组血小板计数较肿瘤相关组和EB病毒感染组升高,谷丙转氨酶、谷草转氨酶和乳酸脱氢酶较EB病毒感染组降低,差异均有统计学意义（P<0.05）。 早期表现组HPS患者乳酸脱氢酶、铁蛋白水平及HPS患者死亡率明显低于迟发表现组患者。近期死亡组HPS患者血清乳酸脱氢酶、铁蛋白水平较非近期死亡组明显升高、而白蛋白水平较非近期死亡组患者明显降低。结论： HPS患者病情发展迅速,死亡率高,尤其是EB病毒感染HPS患者,早期诊断、及时治疗至关重要。     

不同年龄组儿童噬血细胞综合征的临床特点及预后研究

目的 探讨不同年龄组儿童噬血细胞综合征(HPS)的临床特点与预后因素分析.方法 将85例HPS患儿按年龄分为0～2岁(A组)、＞2～8岁(B组)和＞8～14岁(C组),从病因、病理、临床症状、实验室检查及预后等方面进行回顾性分析.结果 A、B和C组病因均以EB病毒感染为主,感染阳性率C组明显高于A、B组;骨髓检查粒细胞红细胞比例(粒红比)小于1.0的发生率,A组高于C组;颈部淋巴结肿大发生在B组中多见,肝脾肿大在C组中最少见;浆膜腔积液发生在B组多见;实验室检查乳酸脱氢酶(LDH)B组高于A组,以上差异均有统计学意义(P＜0.05).其他临床症状和实验室检查结果在组之间差异无统计学意义(P＞0.05).存活与死亡患儿比较,浆膜腔积液、三酰甘油(TG)、CD4/CD8及骨髓检查粒红比比较,差异有统计学意义(P＜0.05),将上述因素进行Logistic分析,显示TG＞3.5 mmol/L、CD4/CD8＜ 1.24、骨髓粒红比小于1.02是影响儿童HPS预后的危险因素(P＜0.05).结论 儿童HPS有部分临床特点与发病年龄有关,TG、CD4/CD8及骨髓粒红比水平是影响预后的危险因素.     

小儿MPP合并EB病毒感染的临床表现及危险因素Logistic回归分析

目的探讨小儿肺炎支原体肺炎(MPP)合并EB病毒感染的临床表现并分析其危险因素。方法选取164例MPP合并EB病毒感染的患儿作为研究组,95例单纯EB病毒感染的上呼吸道感染患儿作为对照组,收集所有患儿的一般资料、临床特征,进行Logistic回归分析危险因素。结果两组患儿在年龄、发病季节、出生低体质量、流行接触史、免疫力低下、高热、呼吸困难、发热时间≥10天、肺部阴影≥2/3肺叶、胸腔积液、血沉≥50 mm/h、C反应蛋白≥8 mg/L上的差异有统计学意义(P＜0.05)；余指标差异无显著性。Logistic回归分析显示流行接触史(OR=2.438)、免疫力低下(OR=3.808)、发热时间≥10天(OR=2.000)、肺部阴影≥2/3肺叶(OR=1.772)、血沉≥50 mm/h(OR=3.062)、C反应蛋白≥8 mg/L(OR=2.192)是MPP合并EB病毒感染的危险因素。结论小儿MPP合并EB病毒感染有明显的临床表现及流行病学特征,降低高危因素有助于MPP合并EB病毒感染的防治。     

40例噬血细胞综合征的临床特点及预后分析

目的 探讨噬血细胞综合征(HPS)的临床特点和预后影响因素,提高HPS的诊治水平.方法 时2002年1月～2008年3月收治的40例HPS患者临床资料进行回顾性分析.结果 40例HPS中22例(55%)与感染相关,其中EBV感染14例,7例(17.5%)与肿瘤相关,病因不明12例(30%);临床表现均符合HLH-2004诊断标准;经抗感染、糖皮质激素、化疗等联合治疗,死亡率65.2%(15/23);伴随EB病毒感染、恶性淋巴瘤的HPS预后差.结论 HPS病因复杂,预后不良;应掌握HPS的临床特点,早期诊断和治疗.     

儿童噬血细胞综合征17例临床分析

目的探讨儿童噬血细胞综合征(HPS)的病因、临床特点、治疗及预后。方法对17例HPS患儿的临床资料进行回顾性分析。结果EB病毒感染9例,支原体感染2例,CMV感染2例,郎罕氏组织细胞增生症1例,不明原因3例。经积极治疗原发病,同时给予免疫调节,好转8例,死亡7例,自动出院2例。结论儿童HPS以感染性居多,大部分为EB病毒引起,应及早干预治疗。     

噬血细胞综合征的临床分析

目的分析噬血细胞综合征(hemophagocytic syndrome,HPS)的临床特征,探讨其死亡的危险因素。方法回顾性分析第四军医大学西京医院58例HPS患者的生存资料,分别比较成人与小儿死亡组及存活组患者的临床特点,探讨两组患者死亡的危险因素。结果小儿20例,其中存活12例,死亡8例,病死率40%。小儿死亡组血小板及纤维蛋白原(Fbg)水平明显低于存活组(P<0.05),而乳酸脱氢酶(LDH)水平明显高于存活组(P<0.05);小儿死亡组与存活组年龄及EB病毒感染比较,差异有统计学意义(P<0.05);余项指标(白细胞、血红蛋白、铁蛋白、白蛋白等)比较,差异无统计学意义(P>0.05)。成人38例,其中存活12例,死亡26例,病死率68.4%。成人死亡组血小板及纤维蛋白原水平明显低于存活组(P<0.05),而乳酸脱氢酶水平明显高于存活组(P<0.05);死亡组伴发肿瘤者明显高于存活组(P<0.05);余项指标比较,差异无统计学意义(P>0.05)。结论 HPS临床表现错综复杂,病因多种多样;血小板降低、纤维蛋白原降低、乳酸脱氢酶水平升高的患者预后不佳,小儿EB病毒感染、年龄小于2岁者及成人伴发肿瘤者,死亡率高,存在以上危险因素者,应给予高度重视。     

成人噬血细胞综合征预后危险因素分析

目的总结分析成人噬血细胞综合征的临床特征,探讨对其预后有影响的危险因素。方法回顾性分析30例临床诊断为噬血细胞综合征的患者的临床特征（临床特点、实验室检查及治疗）。比较死亡组与存活组患者的临床特点,并进行多因素Logistic回归分析,探讨患者死亡的危险因素,并计算OR值及OR的95％可信限（95％CI）。结果30例患者存活21例,死亡9例,病死率为30％。多因素Logistic回归分析提示,EB病毒感染、CD4／CD8比例倒置、血小板降低、Hb〈30g／L为死亡危险因素,相对危险度分别为5．421、4．816、3．823及1．097。结论噬血细胞综合征病因复杂,临床表现多样,病死率较高,早期诊断、早期化疗可提高患者的生存率。应重视并针对死亡的危险因素制订相应的防治措施,降低病死率。     

儿童重型噬血细胞综合征的临床特征及预后相关因素分析

目的探讨儿童重型噬血细胞综合征(HPS)的临床特征、预后危险因素及有效治疗方法, 为该病的临床诊治提供参考。方法收集2014年1月至2021年4月遵义医科大学附属医院收治的83例重型HPS患儿的临床资料, 分析其临床特征、转归及预后危险因素。根据合并中枢神经系统(CNS)功能障碍与否将患儿分为CNS功能障碍组及非CNS功能障碍组, 根据接受血液净化与否将患儿分为血液净化组及非血液净化组, 比较生存差异。结果 83例患儿中, 男43例, 女40例, 年龄[M(Q1, Q3)]36(15, 27)个月。51例由感染诱发, 其中EB病毒感染41例(80.4%)。所有患儿均伴多器官功能障碍(MODS), 以血液系统(72.3%)、肝脏(71.1%)、呼吸系统(53.0%)及CNS(37.3%)功能障碍常见。至随访截止, 存活40例(48.2%), 死亡38例(45.8%), 失访5例(6.0%)。CNS功能障碍是影响患儿预后的危险因素(HR=3.358, 95%CI:1.445～7.803, P=0.005), 血液净化治疗是影响患儿预后的保护因素(HR=0.362, 95%CI:0.179～...     

小儿噬血细胞综合征的发病机制及预后危险因素的分析

目的:探讨儿童噬血细胞综合征(white blood cell syndrome,HPS)的临床特点?诊断标准?治疗过程以及预后的相关危险因素?方法:回顾分析2009—2013年南京市儿童医院血液肿瘤科收治的72例儿童HPS的临床表现?辅助检查?治疗及转归,采用Logistic方法分析患儿的预后危险因素?结果:72例中42例(58.3%)是感染相关性HPS,其中EB病毒感染相关性HPS最多;其余的30例中4例(5.56%)是非感染相关HPS,26例(36.1%)病因不明?临床表现为发热(91.7%)?肝脏肿大(83.3%)?脾脏肿大(66.7%)?外周血血常规三系检查结果为两系减低(72.2%),三系低下(27.8%),血红蛋白浓度减低(91.7%),血小板数量减少(77.8%);血生化检查血清甘油三酯升高(38.9%);凝血常规检查功能障碍(41.7%);血清铁蛋白升高(55.6%);骨髓细胞形态学检查大部分找到了噬血细胞?72例中死亡10例(13.8%),好转或者基本痊愈24例(33.3%),出院后失访14例(19.4%)?年龄<3岁?乳酸脱氢酶(LDH)>2 500 U/L是不利的危险因素?结论:年龄小和LDH水平增高是儿童HPS预后不良的危险因素,应该及早诊断治疗,降低病死率?     

8例成人噬血细胞综合征临床分析

目的:分析8例噬血细胞综合征的临床表现及实验室检查特点.方法:回顾性分析2017年1月—2019年6月在德阳市人民医院初步诊断为噬血细胞综合征患者8例临床资料,对患者性别、年龄、临床表现、实验室检查结果及归转情况进行分析.结果:本组8例噬血细胞综合征患者,7男1女,患者平均年龄为(46.13±16.65)岁;临床表现主要以发热(100％)、淋巴结大(50％)、肝脏肿大(50％)等为主;实验室检查结果以血细胞减少(100％)、铁蛋白升高(100％)、乳酸脱氢酶升高(87.5％)、甘油三酯升高(75％)、纤维蛋白原降低(37.5％)、可溶性CD25升高(87.5％)、NK细胞活性降低(75％).比较肿瘤、感染2组HPS患者初诊时各实验室指标HB、Ferr有统计学意义(P<0.05).比较EB病毒感染与非EB病毒感染2组HPS患者初诊时各实验室指标WBC、HB、PLT、Ferr、LDH、TG、FBG的差异均无统计学意义(P>0.05).比较死亡组与生存组2组HPS患者初诊时各实验室指标Ferr有统计学意义(P<0.05).结论:HPS临床表现错综复杂,病因多种多样;高铁蛋白水平的患者预后不佳.     

Value of D-Dimers in patients with acute aortic dissection

Objective: To evaluatel the value of D-dimers in patients with acute aortic dissection (AAD). Methods: This study consisted of 16 patients with AAD and 27 non-AAD patients. Serum D-dimets were measured by Sta-Liatest D-DI immunoturbidimetric assay. Results: D-dimer level was higher (P ＜ 0.001) in patients with AAD(7.91 ± 5.52 μg/ml) than that in non- AAD group(1.57±1.24 μg/ml). D-dimer was positive (＞0.4 μg/ml) in all patients with AAD and in 10 control group patients (37%). Among patients with acute AAD, D-dimers tended to be higher in Stanford A than in Stanford B (8.67 ± 4.31 μg/ml vs. 3.24±1.27 μg/ml, P ＜0.01). D-dimer values tended to be higher in more extended disease(3.84 ± 1.65 μg/ml, 8.57 ± 3.58 μg/ml and 11.87 ± 5.69 μg/ml in thoracic aorta, thoracic and abdominal aorta, thoracic and abdominal aorta and iliacal arteries, respectively, P ＜ 0.05 for both 8.57 ± 3.58 and 11.87 ± 5.69 vs. 3.84 ± 1.65 ). Including the control group into the analysis, we found a sensitivity of 100%, a negative predictive value of 100%, and a specificity of 66% and a positive predictive value of 64% for D-dimer in diagnosis of AAD in our patients with suspected AAD. Conclusion: D-dimer was elevated in patients with AAD. A negative D-dimer test result could be useful in excluding AAD.     

Research of combined liver-kidney transplantation model in rats

Objective： To set up a simple and reliable rat model of combined liver-kidney transplantation. Methods： SD rats served as both donors and recipients. 4℃ sodium lactate Ringer＇s was infused from portal veins to donated livers,and from abdominal aorta to donated kidneys, respectively. Anastomosis of the portal vein and the inferior vena cava （IVC） inferior to the right kidney between the graft and the recipient was performed by a double cuff method, then the superior hepatic vena cava with suture. A patch of donated renal artery was anastomosed to the recipient abdominal aorta. The urethra and bile duct were reconstructed with a simple inside bracket. Results： Among 65 cases of combined liver-kidney transplantation, the success rate in the late 40 cases was 77.5%. The function of the grafted liver and kidney remained normal. Conclusion： This rat model of combined liver-kidney transplantation can be established in common laboratory conditions with high success rate and meet the needs of renal transplantation experiment.     

BLOOD PRESSURE CHANGE WITH AGE IN SALT-SENSITIVE TEENAGERS

Objective To observe blood pressure change with age in salt-sensitive teenagers whose salt sensitivity were determined by repeated testing.Methods Salt sensitivity was determined through intravenous infusion of normal saline combined with volume-depletion by oral diuretic furosemide in 55 teenagers. After five years, salt sensitivity was re-examined and subject blood pressure was followed up. Blood pressure changes in salt-sensitive teenagers were compared to that of non-salt sensitive teenagers over five years.Results After 5 years, the repetition rate of salt sensitivity determined by intravenous saline loading is 92.7%. In teenagers with salt sensitivity on the baseline, both the systolic blood pressure increments and increment rates were much higher than non-salt sensitive teenagers (12.7±12.1 mmHg vs. 2.8±5.2 mmHg, P＜ 0.01; 12.2%± 12.0% vs. 2.5% ±4.4%, P＜ 0.001,respectively). There was a similar trend for diastolic blood pressure (8.4 ± 6.4 mmHg vs. 3.7 ± 6.4 mmHg, P = 0.052; 13.2% ±10.6 % vs. 6.8%± 10.1%, P = 0.053, respectively).Conclusions Salt sensitivity determined by intravenous saline loading showed good reproducibility. Blood pressure increments with age were much higher in salt-sensitive teenagers than non-salt sensitive teenagers, especially in terms of systolic blood pressure.     

安心颗粒对急诊经皮冠状动脉介入术后生活质量影响的研究

目的：评价使用安心颗粒对急诊经皮冠状动脉介入术（PPCI）术后生活质量的影响.方法：将160例接受PPCI的急性ST段抬高型心肌梗死患者随机分为安心颗粒组（术前顿服安心颗粒8.8g,术后安心颗粒4.4 g/次,每日2次）和对照组（仅接受基础药物治疗）.所有患者均服用阿司匹林、氯吡格雷和阿托伐他汀.分别在入院时、出院前1d、出院后180 d时,应用心肌梗死多维度量表（MIDAS）、中文版SF-36评价量表对患者生活质量评分.并观察术后30 d以内的出血并发症、血小板减少症发生情况.结果：入院时和出院前1d,两组患者的心肌梗死MIDAS、SF-36量表评分比较无差异（P＞0.05）;出院后180 d时,与对照组比较,安心颗粒组MIDAS、SF-36评分明显减低（P＜0.05）;组内与入院时比较,两组出院前1d、出院后180 d时,MIDAS、SF-36评分均降低（P＜0.05）.两组患者在随访期间均无大量出血、少量出血、重度和极重度血小板减少症发生,安心颗粒组有4例、对照组有7例发生不明显出血（P＞0.05）.两组发生轻度血小板减少症的患者数比较无差异（P＞0.05）.结论：PPCI使用安心颗粒,能改善急性ST段抬高型心肌梗死患者的生活质量,且不增加出血风险.     

The comparative studies of the influences of Urapidil and Nicardipine on sino-atrial node function, atrio-ventricular node function and hemodynamics

Objective:To investigate the influences of urapidil and nicardipine on rabbit sinus function,atrio-ventricular node function and hemodynamics.Methods:Thirty-two Angora's rabbits were selected and randomly divided into four groups.U1 group:urapidil 0.25 mg/kg;U2 group:urapidil 0.5 mg/kg;N1 group:nicardipine 10 μg/kg;N2 group:nicardipine 20 μg/kg.All these medicine were administrated within 30 seconds.Measurements were taken before and after the administration of urapidil or nicardipine for the following data:mean blood pressure(MAP),heart rate(HR),sino-atrial conduction time(SACT),maximal sinoatrial recovery time(SNRTmax)corrected sinus node recovery time(CSNRT),index of sinus node recovery time(SNRTI),Wenckebach A-V conduction frequency (WB),and P-R interval.Results:Significant MAP and HR changes were identified in all of the four groups before and after administration of both urapidil and nicardipine.No significant changes could be found in the rest of the parameters.Intergroup analysis showed that SACT and CSNRT of N1 and N2 groups were shorter than those of the U2 group(P＜0.01);the MAP decreased(P＜0.01)and the HR increased drastically(P＜0.01).Conclusions:Neither urapidil(0.25 mg/kg,0.5 mg/kg)nor nicardipine(10μg/kg,20μg/kg)has any significant influence on rabbit sinus function or rabbit atrio-ventricular node function.Nicardipine could be a better choice than urapidil for parafunctional sinus node patients.     

Expression of OPGmRNA and ODFmRNA in the patients of hip fracture due to osteoporosis

Objective：To investigate the gene expression of osteoprotegerin（OPG） and osteoclast differentiation factor（ODF） in the bone tissue of patients with hip fracture due to osteoporosis. Methods：OPGmRNA and ODFmRNA in the bone tissue in 50 cases of osteoporosis sufferers（over 50 years old） with hip fracture（Observer Group） and 30 cases of hip facture sufferers with no osteoporosis（Control group） were analyzed with the Semi-Quantitative RT-PCR method. Results：The mRNA expressed of ODF, OPG were both high in the patients with hip fracture. In the control group, the expression of OPG mRNA was observed, while the expression of ODF mRNA was very slight. Conclusion：Aged patients contained all signals including OPG, ODF that are essential for inducing osteoclastogenesis and promoting bone resorption.     

The clinicopathological and immuohistochemical analysis of solid-pseudopapillary tumor of the pancreas： report of 9 cases

Objective：To investigate the clinical features, pathological characteristics and immunophenotype of solid-pseudopapillary tumor of the pancreas（SPTP）. Methods：Nine surgically treated cases of SPTP were retrospectively reviewed. Hematoxylin and Eosin（HE） staining and immunohistochemical staining were used to analyze all cases, and the general clinical data was collected. Results：Six patients were asymptomatic except for a palpable mass. Two patients complained of vague-epigastric pain. One patient appeared jaundice. The tumor was encapsulated and solid tissues alternately with cystic tissues. Histologically, the histological structure of solid portion was pseudopapillary with a fibrovascular core. Tumor cells were uniform and medium-sized which were arranged in sheets ets or nests or pseudopapillary patterns. Immunohistochemical studies demonstrated that SPTP proved positive in vimentin（9/9 cases）, AAT（9/9 cases）, NSE（9/9 cases）, ACT（7/9 cases）, CK20（2/9 cases）, CgA（1/9 cases）, S-100（3/gcases）, PR（4/gcases）, Syn（3/9 cases） and CD56（5/9cases）, negative in CEA and ER. Conclusion：SPTP is a tumor predominantly occurring in young women frequently without special symptoms. This tumor has various characteristical histological patterns with different immunophenotype.     

Dynamic Changes in Serum Estradiol and Progesterone levels in Patients of Premenstrual Syndrome with Adverse Flow of Liver-qi

Objective:To probe into the influence of changes of ovarian hormones on the pathogenesis of the specific sub-type premenstrual syndrome(PMS)and reveal partial microcosmic mechanisms of adverse flow of liver-qi.Methods:Estradiol(E2)and progesterone(P)levels in serum were determined at different phases of menstrual cycle by radioimmunoassay.Results:In the group of PMS with adverse flow of liver-qi.the secretive peak value Of E2 and P at the follicular phase significantly decreased,and the secretive peak value at the luteal phase did not come into being.Conclusions:Low E2 and P secretive peak at the follicular phase and absence of secretive peak at the luteal phase is one of the microcosmic mechanisms of PMS with adverse flow of liver-qi.One of the pathophysiologic mechanisms of specific sub-type PMS is probably the continuous low level of E2and P.     

Real-time Three-dimensional Echocardiography in Assessment of Left Ventricular and Right Ventricular Volumes

Real-time three-dimensional echocardiography (RT3DE)is a new ultrasound technique that enables dynamic threedimensional visualization and quantification of the heart in real time. Investigation of feasibility and methodology of RT3DE in determining left ventricular (LV) and right ventricular (RV) volumes, RT3DE was performed in 35 normal adults using Philips SONOS 7500 system with a 2-4 MHz matrix array transducer. The 60°×60° "pyramid" volume database was obtained and analyzed on a TomTec echo workstation. Both LV and RV volumes were calculated with four 3DE methods (i.e. apical 2, 4, 8, and 16-plane) through manually tracing ventricular endocardial borders in end diastole and end systole. Stroke volumes were then calculated. LV volume was also measured by 2DE Simpson's rule using GE VIVID 7 ultrasound machine.     

SCREENING FOR A 21-CHROMOSOME ABNORMALITY IN PREIMPLANTED EMBRYOS OF ELDERLY WOMEN

Increasing maternal age is the only etiological factor unequivocally linked to Down's syndrome in humans. The occurrence rate of newborns with Down's syndrome is about 1/220 in women over 35 years old. However, the occurrence rate in embryos fertilized in vitro, of the elder woman is unclear. Using FISH we screened the number of chromosome 21 in preimplanted embryos of 5 elderly women (average age, 38.4 years) to study the feasibility and necessity of screening trisomy 21 in embryos in patients over 35 years old at the in vitro fertilization (IVF) center.