POEMS综合征22例临床特点分析

目的 探讨POEMS综合征的临床特点,提高诊断率.方法 对22例POEMS综合征临床资料进行回顾性分析.结果 POEMS综合征多见于中年男性,22例患者均以多发性周围神经病为首发症状,内分泌改变59.1％ (13/22),皮肤改变50.0％(11/22),异常球蛋白血症阳性率63.6％(14/22).22例患者激素或免疫抑制剂均有效.结论 POEMS综合征临床表现各异,易误诊,常规检查、内分泌改变、肌电图、血清M蛋白检测有重要意义,激素及免疫抑制剂是有效的治疗方法.     

POEMS综合征20例临床特点分析

目的 分析POEMS综合征的临床特征,以提高对该病的诊治水平.方法 回顾分析20例POEMS综合征的临床资料,并与国外文献(Mayo组99例)比较,总结其临床特点.结果 临床表现为多发性神经病20例(100%,100%),皮肤色素沉着19例(95%,46%),脾肿大19例(95%,22%),阳痿11例(69%,71%),甲状腺功能减低9例(45%,17%);M蛋白检出率较低,骨髓浆细胞增生及骨损害明显低于国外病例;20例早期均误诊;15例经皮质类固醇、三苯氧胺等治疗后,10例症状缓解.结论 该组病例临床特征与国外组基本相似,但有其特点;对不明原因的多发性神经病病例,应及时行血清、尿液蛋白免疫固定电泳等检查;皮质类固醇治疗对该病有效.     

POEMS综合征1例报道及相关文献复习

POEMS综合征是一种与浆细胞异常有关的多系统疾病,主要表现为:多发性神经病变(poly-neuropathy)、脏器肿大(organomegaly)、内分泌病变(endocrinopathy)及M蛋白血症(M protein)、皮肤改变(skin changes),取各种病变英文首字命名为POEMS综合征.POEMS综合征的病因目前尚不明确,诊断标准尚有争议,治疗方法未能标准化.     

POEMS综合征6例临床分析

【目的】总结POEMS综合征的临床特点,以提高对该病的认识。【方法】回顾性分析6例POEMS综合征患者的一般情况、临床表现、实验室检查结果及治疗方案。【结果】6例POEMS综合征患者中,男女比例为1.5:1,平均年龄(47±4)岁,平均病程(24±4)个月;主要临床表现发生率分别为周围神经病变和内分泌异常100%,脏器肿大和皮肤改变80%,M蛋白阳性50%;经激素和免疫抑制剂治疗,4例患者好转。【结论】POEMS综合征是一种少见的多脏器损害疾病,诊断较难;临床上对可疑病例需进行密切观察及必要的检查,以免误诊;应用糖皮质激素和免疫抑制剂治疗,可使POEM综合征的预后得到明显的改善。     

POEMS综合征20例临床特征分析

目的分析总结POEMS综合征临床特征,以提高医师认识水平,减少误诊。方法回顾性分析2005年1月—2012年4月我院收治的20例POEMS综合征患者的临床表现、辅助检查、治疗及预后,并复习相关文献。结果 20例患者均有多发性周围神经病变和皮肤改变;单克隆浆细胞增生18例,血清M蛋白阳性率为90.0%,免疫固定电泳阳性率高达100.0%(17/17),12例行骨髓穿刺,骨髓细胞涂片浆细胞平均比例为3.2%;脏器肿大16例,内分泌异常19例;水负荷增多19例。本组肾功能受累较常见,其中β2微球蛋白增高12例、肌酐增高4例。其他特征肺动脉压增高8例、贫血10例、血小板增多5例、多发骨骼异常2例及顽固性腹痛1例。本组误诊率为95.0%,误诊为格林巴利综合征8例,甲状腺功能减退症4例,其他内科疾病7例。病死率为5.0%(1/20)。结论 POEMS综合征以各种症状为首发,临床表现复杂,误诊率极高,导致病情发展,临床医师应加深认识,对疑诊患者应进行细致的检查以避免误诊。     

POEMS综合征1例报告

POEMS综合征是可导致多系统多器官受到损害的浆细胞异常增生性疾病。其主要表现为：多发性神经病（Polygneuropathy）、脏器肿大（Organomegaly）、内分泌病（Endocrinopathy）、M蛋白（M—protein）和皮肤改变（Skin—changes）。取其英文之首字母，称为POEMS综合征，通常义称为Crow—Fukase综合征”尽管在国内外已有不少报道，但仍有很多方面不甚了解，临床漏诊、误诊屡见不鲜。最近我院收治1例，特报告如下。     

M蛋白血症90例临床分析

目的提高对M蛋白血症的认识。方法分析90例M蛋白血症的M蛋白类型及原发疾病病种分布,以及多发性骨髓瘤(MM)的M蛋白类型和M蛋白含量。结果90例M蛋白血症病例中,IgG型40例(占44.4%)、IgA型15例(占16.7%)、IgM型12例(占13.3%)、轻链型14例(占15.6%)、IgD型4例(占4.4%)、双克隆型5例(占5.6%);90例M蛋白血症病种分布,MM59例、华氏巨球蛋白血症(WM)4例,B细胞非霍奇金淋巴瘤(NHL)4例,意义未明的单克隆免疫球蛋白血症(MGUS)8例,其余包括慢性肾炎、骨转移癌、POEMS综合征等。59例MM中,IgG型30例(占50.8%)、IgA型11例(占18.6%)、轻链型12例(占20.4%)、IgD型和双克隆型各3例(占5.1%);相应的免疫球蛋白显著升高。结论M蛋白血症常见于MM、WM等恶性浆细胞,也可见于MGUS、POEMS综合征、NHL等多种疾病。     

POEMS综合征研究进展

POEMS综合征是一种与浆细胞病有关的多系统病变，临床上以多发性周围神经病(polyeuropathy)、脏器肿大(organpomegaly)、内分泌障碍(endocrinopathy)、M蛋白血症(M-protein)和皮肤病变(skin change)为特征。又称Crow综合征、Crow Fukase综合征、PEP综合征、Takatsuki综合征、Shimpo综合征、Nakanishi综合征，1980年Bardwish取本病的五大临床特征的英文首字母，命名为POEMS综合征^[1]。我国自1987年开始陆续报道，近年病例日渐增多，涉及到神经、内分泌、血液和皮肤科等各专业领域，常易误诊。现将近年来研究进展综述如下，以引起警惕。     

免疫固定电泳在多发性骨髓瘤诊断中的应用

目的:探讨免疫固定电泳对于多发性骨髓瘤的应用意义。方法:对115例建议进行免疫电泳检测的患者检测血清M蛋白和浓缩尿中的M蛋白,并根据M蛋白的结果进行分型。结果:115例患者中血清M蛋白阳性为45例,浓缩尿M蛋白阳性结果47例,两者均阳性的患者为38例,经调阅病例临床确诊为多发性骨髓瘤的是34例,其中IgG为22例(64.7%),IgA为8例(23.53%),IgM为2例(5.88%),轻链型为2例(5.88%)。结论:免疫固定电泳对于多发性骨髓瘤的诊断、病情监测、预后的判断具有重要意义。     

POEMS综合征12例临床特征分析

目的：探讨POEMS综合征的临床特征。方法回顾性分析12例POEMS综合征患者的临床资料。结果以四肢麻木无力为首发者9例（75％）,表现为周围神经病者12例（100％）,单克隆浆细胞增生性异常10例（83.3％）,器官肿大者8例（66.7％）,内分泌异常者11例（91.7％）,皮肤改变者11例（91.7％）,水负荷增多者9例（75％）。血清 M 蛋白阳性4例（33.3％）;血清免疫固定电泳阳性12例（100％）;12例患者均有脑脊液蛋白-细胞分离现象;10例（83.3％）患者做骨髓穿刺,骨髓浆细胞〈5％者9例;12例（100％）患者肌电图检查均有周围神经受损表现。结论 POEMS综合征临床表现复杂,并累及多个系统,临床上易误诊,临床医生应加强对本病的认识,以达到早诊断、早治疗的目的。     

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.