家族性白血病99例临床分析

目的探讨家族性白血病的临床特征和规律。方法收集国内1996～2006年文献26篇共99例家族性白血病病例进行统计分析。结果男女发病比例相近;绝大多数在中年以下年龄段发病;以父(母)子(女)、兄弟姐妹、祖孙之间发病常见;急性白血病占84.85%,慢性白血病占14.14%;化疗完全缓解率61.11%,其中急性白血病化疗完全缓解率62.25%。结论家族性白血病是一类家族或家族聚集性质的白血病,今后在积极开展家系调查,采取早期干预(监测和逆转)研究是努力的方向。     

21-三体综合征相关急性白血病：21例报告

目的总结21-三体综合征（DS）相关急性白血病（AL）患儿的临床、实验室检查特点及疗效,提高诊疗水平。方法分析21 例DS-AL 患儿的临床表现、实验室检查、治疗及预后。结果与非DS-AL 相比,21 例DS-AL 患儿发病年龄1~5 岁为主（占 85.7%）,急性髓细胞白血病多见（占57.1%）,乳酸脱氢酶>2 倍以上者较多（占61.9%）;13 例行心脏彩超检查10 例异常（占 67.9%）,复杂型先天性心脏病常见（占38.5%）;6 例接受化疗,4 例完全缓解（占66.7%）,合并感染死亡2 例,治疗相关死亡率 33.3%;2例减强度化疗者已分别无病生存21月及43月。结论DS-AL以急性髓细胞白血病多见,对化疗敏感,采用减强度化疗 预后较好。     

老年急性白血病31例分析

报道老年急性白血病31例,其中急性非淋巴细胞白血病(ANLL)27例,急性淋巴细胞白血病(ALL)4例.临床表现不典型,起病隐匿,多数以贫血为首发症状,出血及浸润症状较少,常有伴发病.血象非白血性者占16例(5l.6%).骨髓增生低下者并不多,只占9.1%,增生活跃以上者占绝大多数,骨髓象多呈低原始细胞性.联合化疗达完全缓解者共12例(44.4%),其中完全缓解8例,完全缓解率为30%,单用小剂量化疗尽管副作用小但结果不满意.本文对31例老年急性白血病的临床特点及治疗进行了讨论.     

CAG方案治疗老年初治急性髓系白血病17例观察与护理

目的：探讨应用CAG方案治疗老年初治急性髓系白血病的临床观察与护理要点。方法：对17例应用CAG方案行辅助治疗的老年初治急性髓系白血病患者进行系统化整体护理。结果：8例患者1个疗程获得完全缓解（CR）,占47.0%;3例2个疗程获得完全缓解（CR）,占17.6%;3例2个疗程部分缓解（PR）,占17.6%;总有效率为82.3%。无化疗相关性死亡。结论：加强化疗期间有针对性的心理护理,完善各项化疗准备,积极预防和及时处理各种并发症,可有效提高CAG方案治疗老年初治急性髓系白血病患者的治疗效果。     

老年急性白血病的临床特点与疗效观察

目的：研究老年急性白血病的临床特征，寻求治疗老年急性白血病的有效策略，提高老年急性白血病患者的生活质量。方法：回顾性分析69例老年急性白血病的临床表现，实验室检查特征和不同化疗方案的疗效。结果：联合化疗35例，总完全缓解（CR）率42．9％（15／35），其中急性非淋巴细胞性白血病（ANLL）26例，CR率42．3％（11／26）；急性淋巴细胞性白血病（ALL）9例，CR率44．4％（4／9）。小剂量阿糖胞苷（Ara-C)或三尖杉脂碱（H）化疗18例，CR率22．2％（4／18）。全反式维甲酸（ATRA）治疗4例，4例CR。姑息治疗12例。结论：老年急性白血病临床上有其特殊性，对化疗反应差，CR率低。对老年急性白血病应选择积极、合理、个体化的化疗方案，并进行综合治疗，有利于提高老年急性白血病的疗效。     

微小残留白血病泛基因的分子生物学检测

白血病是可发生在任何年龄段的具有较高发病率的造血系统恶性肿瘤,占癌症总发病数的5%.近年来,随着白血病化疗方案的逐步完善,新型抗白血病制剂的应用以及各种造血干细胞移植的开展,使急性白血病联合化疗的完全缓解率达到80%～90%,同种或自身骨髓移植治疗后5年生存率达40%～50%.大多数患者的无病生存期和总生存期显著延长,但仍有许多患者在治疗达完全缓解后复发,因而白血病复发问题仍是当前白血病治疗的主要障碍.目前认为,白血病复发的根源主要是来自体内残留的白血病细胞,这种出现于复发期的肿瘤细胞多数与初诊时的肿瘤细胞来源于同一克隆,因此检测微小残留白血病细胞（MRLC）对指导治疗、评价疗效、判断预后和监测复发都具有十分重要的意义.     

小儿急性白血病的化疗

小儿急性白血病的化疗裴仁治随着化疗方法的不断改进，新的抗白血病药物的不断发现，以及支持疗法的加强，小儿急性白血病（ＡＬ）的疗效不断提高。而今，小儿急性淋巴结胞性白血病（ＡＬＬ）的完全缓解（ＣＲ）率达９５％～１００％，５年以上无病存活率（ＤＦＳ）高达７...     

染色体核型与急性髓系白血病诱导成功的关系

目的 评价染色体核型在急性髓系白血病（AML）WHO 分型系统中的作用。方法 107例AML患者按WHO标准进行分型诊断,并对诊断后各亚型之间的诱导化疗完全缓解率（CR）进行分析。结果 AML中伴有重现染色体异常36例（33.6%）,染色体核型均为低危;伴有多系发育异常24例（22.4%）,染色体核型中危17例、高危7例;不另做分类占47例（43.0%）,染色体核型中危39例、高危8例。伴t（8;21）和伴inv（16）或t（16;16）患者的诱导化疗CR 率显著高于不另做分类的（P〈0.05）。有多系增生异常患者的诱导化疗CR率明显低于无多系增生异常患者（P〈0.05）。结论 AML各亚型之间诱导化疗CR率不同,细胞遗传学是预测急性髓系白血病达到CR最有用的因子之一。     

12例家族性白血病

12例家族性白血病的资料表明:家族性白血病亦有白血病前期(2/12)。该病以急性为多(11/12);细胞以不同类型为多(8/12)。本组8例采用中西医结合治疗,完全缓解率44.44％,最长1例已存活6年余,仍处完全缓解期。作者认为家族性白血病的发病,为遗传因素、环境因素相互影响的结果。     

老年急性白血病25例化疗疗效观察

目的：研究治疗老年性急性白血病临床特点。方法：分析25例老年急性白血病患者临床表现,实验室检查特征及不同化疗方案的疗效。结果：联合化疗的25例患者中完全缓解（CR）率为46％,其中急性非淋巴细胞白血病（AML）CR率为50％,急性淋巴细胞白血病（AML）CR率为37％,结论：临床上老年性急性白血病联合化疗CR率低对老年性急性白血病应选择合理、个体化的治疗方案,以此提高老年性急性白血病患者的生活质量。     

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.