听神经病临床表现特征分析

目的 探讨听神经病患者的临床表现特点.方法 总结分析18例(36耳)听神经病患者的一般情况、听力学及电生理检查结果.结果 听神经病患者均为双耳发病,青少年多见,平均发病年龄17.94岁;纯音测听表现为双耳基本对称、上升型感音神经性听力损失,以低频250Hz、500Hz损失为主;鼓室压力图多为A型,声反射均消失;畸变产物耳声发射(DPOAE)正常,对侧声抑制消失;听性脑干反应(ABR)异常,阈值均≥90dBnHL.结论 ABR严重异常、声反射消失、低频纯音听阈升高而DPOAE正常,多见于青少年、双耳发病等是听神经病的临床特征.     

单侧听神经病1例

目的 分析单侧听神经病的听力学特征,探讨其病损部位.方法 对单侧听神经病患儿的临床资料、纯音测听、声导抗测试、畸变产物耳声发射（distortion product otoacoustic emission, DPOAE） 、听性脑干反应（auditory brain stem response ,ABR）的听力学测试结果进行分析.结果 患耳（右耳）纯音听力图为缓降型,听力损失为重度感音神经性聋,健耳（左耳）听力正常;患耳的同侧及对侧镫骨肌声反射未能引出,健耳的同、对侧声反射均能引出 .患耳的听性脑干反应各波未能引出,健耳的ABR各波潜伏期正常 .双耳 DPOAE各频率均正常引出.颞骨高分辨率CT未见异常.结论 单侧听神经病的听力学特征与双侧听神经病的听力学特征基本相同.但单侧听神经病以重度聋较多见,应对单侧感音神经性聋患者进行全面的听力学检测 ,需注意与一般单侧感音神经性聋相鉴别.DPOAE 及ABR 对诊断、鉴别诊断具重要意义.     

单侧听神经病1例

目的 分析单侧听神经病的听力学特征,探讨其病损部位.方法 对单侧听神经病患儿的临床资料、纯音测听、声导抗测试、畸变产物耳声发射(distortion product otoacoustic emission, DPOAE) 、听性脑干反应(auditory brain stem response ,ABR)的听力学测试结果进行分析.结果 患耳(右耳)纯音听力图为缓降型,听力损失为重度感音神经性聋,健耳(左耳)听力正常;患耳的同侧及对侧镫骨肌声反射未能引出,健耳的同、对侧声反射均能引出 .患耳的听性脑干反应各波未能引出,健耳的ABR各波潜伏期正常 .双耳 DPOAE各频率均正常引出.颞骨高分辨率CT未见异常.结论 单侧听神经病的听力学特征与双侧听神经病的听力学特征基本相同.但单侧听神经病以重度聋较多见,应对单侧感音神经性聋患者进行全面的听力学检测 ,需注意与一般单侧感音神经性聋相鉴别.DPOAE 及ABR 对诊断、鉴别诊断具重要意义.     

听神经病的听力学特征分析

目的 研究听神经病的听力学特点.方法 回顾分析23例听神经病患者的病史、纯音测听、言语识别率、镫骨肌反射、听性脑干反应、畸变耳声发射及对侧白噪声抑制试验.结果 纯音测听示全部患者均为轻至中度低频感音性聋,听力损失最严重的频率是0.25 kHz和0.5 kHz,听力图上升型38耳,鞍型7耳,平坦型1耳.声导抗鼓室图全部正常,45耳镫骨肌反射消失,仅1耳引出且反射阈升高.全部病例可引出DPOAE,不能被对侧噪声抑制.ABR不能引出,或仅出现波Ⅰ和(或)波Ⅴ.23例言语识别率差,与纯音听阈不成比例.结论 ABR缺失或严重异常而DPOAE正常引出,纯音听力图多呈上升型以低频损失为主,镫骨肌反射及OAE交叉抑制异常,言语分辨率差与纯音听阈不成比例,是听神经病的主要特征,应与一般感音神经性聋和中枢性聋相鉴别.     

听神经病

目的 :介绍一种特殊的感音神经性听力疾患—听神经病 ,探讨其临床特征及听力学特点。方法 :报道 5例听神经病患者 ,2例为成人 ;3例为儿童。记录患者的临床资料 ,并对患者进行纯音测听、脑干电反应测听、耳声发射、耳蜗电图及语言辨别率等听力学检查。结果 :5例均主诉听力下降 ,听力学检查纯音听阈为轻、中度感音神经性聋 ,与纯音测听不相符的语言辨别率明显下降 ,不能引出脑干诱发电位 (ABR) ,耳蜗电图基本正常 ,畸变产物耳声发射 (DPOAE)基本正常。提示外毛细胞功能正常 ,病变可能在听神经。结论 :听神经病是一种主诉听力下降 ,纯音听阈为轻、中度感音神经性聋 ,不能引出脑干诱发电位 (ABR) ,畸变产物耳声发射正常的听力疾患 ,临床上应与其他感音神经性聋区别     

听神经病的临床听力学特征分析

目的 探讨听神经病的听力学基本特点.方法 回顾性分析14例28耳听神经病患者临床病史、纯音测听(puretone audiometry)、镫骨肌反射(stapedial muscle reflex)、听性脑干反应(auditory brainstem response)、40 Hz相关电位(40 Hz audi-tory event related potential)、畸变耳声发射(distortion product otoacoustic emission)及瞬态耳声发射(transient-evoked otoacousticemission)等听力学相关资料.结果 纯音测听示患者中24耳以轻至中度低频感音性聋为主(听力图上升型),2耳为中重度全频听力下降(听力图平坦型),2耳为低、高频听力下降(听力图鞍型).声导抗鼓室图全部正常,镫骨肌反射消失,全部病例可引出畸变耳声发射及瞬态耳声发射,听性脑干反应各波不能引出,40 Hz相关电位检查只有1例未引出,其余均引出.14例患者中有13例能闻其声,不能辨其语.结论 异常听性脑干反应、正常畸变耳声发射及瞬态耳声发射、纯音听阈升高(以低频为主)的感音神经性聋、镫骨肌声反射未引出、听性脑干反应阈值与纯音听阈不符均是听神经病的主要特征,应与一般感音神经性聋和中枢性聋相鉴别.     

听神经病的临床特点分析

目的探讨听神经疾病的临床特点及听力学特征，以指导临床诊断与诊治。方法分析28例听精神病患者临床表现、纯音测听、声导抗、听性脑干反应（ABR）和畸变产物耳声发射（DPOAE）检查，并与耳蜗性聋对照。结果听神经病多发于青少年，多为双侧发病，听力损失呈轻、中、重度，以低频区为重，听阈图以低频区阈值升高的上升型为主。同、对侧镫骨肌反射消失。ABR引不出或严重异常，DPOAE幅值均在正常范围。结论听神经病发病以青少年为多，听力损失与语言辨别情况不相符。低频区听阈升高的上升型听阈图对听神经病的早期发现和明确诊断有重要意义。     

听神经病相关基因OTOF研究进展

引言 听神经病（auditory neuropathy,AN）是1996年Starr等首次提出,定义为第Ⅷ脑神经的听支（自耳蜗至进入脑干之前的耳蜗神经）受损而引起的一种临床表现特殊的感音神经性聋.其听力学特征包括：听性脑干反应（ABR）自Ⅰ波起缺失或严重异常,诱发性耳声发射（EOAE）正常,言语分辨率不成比例的明显差于纯音听阈,听力图多以低频听阈升高为主,镫骨肌声反射消失或阈值升高等.AN可独立发生,仅有听力损失,不伴有其他神经系统的症状,称为非综合征型;也可伴随其他神经系统症状的听力损失,称为综合征型.     

一遗传性耳聋家系临床特征分析及候选致病基因的突变筛查

目的：分析一遗传性耳聋大家系的听力学特征及对候选致聋基因进行筛查。方法：通过家系调查,整理分析家系资料,绘制系谱图;对家系成员行听力学检测及全身体格检查并抽取外周血。对2例家系患者进行已知致聋基因的全外显子测序及线粒体DNA测序。结果：该家系可追溯6代97人,耳聋患者18例。系谱特征表现为世代连续传递,男女均可发病。家系分支Ⅲ2及其后代、Ⅲ4、Ⅳ20听力学表现为迟发性、渐进性的听力损失,发病年龄均在28岁左右,逐渐加重。Ⅳ20子女（V36、V37、V38、V39）幼时听力言语正常,3、4岁左右表现出听力损失,非进行性,氨基糖甙类抗生素用药史不详。经纯音测听、声导抗、听性脑干反应、耳声发射等检查,发现该家系患者均表现为中重度感音神经性听力损失（V10、V35除外,其言语交流正常,听力检测表现为高频感音神经性听力损失）。对该家系进行已知的耳聋致病基因全外显子及线粒体DNA测序结果分析发现Ⅳ21及其后代为线粒体DNA A1555G突变携带者,其余患者均无阳性发现。结论：该耳聋家系为一个非综合征型、双耳对称性感音神经性听力损失;初步分子遗传学筛查提示该家系致病基因复杂,可能为新基因突变或多基因协同作用致病。     

听神经病的研究进展

听神经病（Auditory neuropathy,AN）是1996年Starr首次提出,定义为第Ⅷ脑神经的听支（自耳蜗至进入脑干之前的耳蜗神经）受损而引起的一种临床表现特殊的感音神经性聋。其听力学特征包括：听性脑干反应（ABR）自Ⅰ波起缺失或严重异常,诱发性耳声发射（EOAE）正常,言语分辨率不成比例的明显差于纯音听阈,听力图多以低频听阈升高为主,镫骨肌声反射消失或阈值升高及EOAE对侧抑制消失等。这一组具有听力学特征的症侯群,与一般感音神经性聋的诊断和治疗完全不同。近年来已逐渐被人们所认识和关注,     

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.     

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.     

Scientific principles and rigorous processes should be followed in developing clinical guidelines for therapeutic interventions of integrative medicine

A clinical guideline for the therapeutic interventions of integrative medicine may be defined as a written document which states a series of recommendations on therapeutic interventions of integrative medicine for a special disease or condition. The guideline may provide assistance to medical professionals in making clinical decisions aimed at improving the clinical outcome of patients and reducing the costs of medical care（~＇4~. Recommendations issued by a guideline should be based on the best available evidence in both Western and Chinese medicine. For fulfilling this purpose, the development of clinical guidelines for therapeutic interventions in the field of integrative medicine should follow scientific principles and undergo a rigorous processes.