Bone scintigraphy in hereditary mutiple exostoses

A case of hereditary mutiple exostoses is presented. ^99mTc-MDP imaging demonstrated uptake of radiotracer in the lesions.     

Aesthetic refinements in prophylactic mastectomy with immediate reconstruction

Immediate reconstruction of more than 1000 breasts was performed on high-risk patients on whom a prophylactic mastectomy was done. The mastectomy removes as much breast tissue as possible while leaving sufficient skin, and possibly the nipple-areola complex, to enable immediate reconstruction. The creation of symmetrical, well-balanced muscle pockets for the implant is the most important factor in producing satisfactory results in these cases.     

Sensory axonopathy in hereditary distal spinal muscular atrophy

A girl of 14 year is presented with a distal spinal muscular atrophy (SMA) with autosomal recessive inheritance. The technical findings are in agreement with the diagnosis. Light microscopical examination of sural nerve biopsy, including teased fiber studies and morphometry, showed no abnormalities. Electron microscopical investigation however demonstrated axonal pathology. The question arises if distal SMA is a distal axonopathy mainly of motor nerves, but to some extent also of sensory nerves.     

盐酸法舒地尔治疗缺血性心肌病心力衰竭

目的用6 min步行试验和多普勒超声心动图评价盐酸法舒地尔治疗缺血性心肌病心力衰竭的疗效。方法将60例因缺血性心肌病而导致慢性心力衰竭的患者分为2组各30例,均采用常规治疗,治疗组同时行静脉滴注盐酸法舒地尔60 mg每日1次。评估2组治疗前后6 min步行距离、左心室收缩末期内径(LVESD)、左心室收缩末期容积(LDESV)、左心室射血分数(LVEF)情况。结果用药3个月后治疗组6 min步行距离、LVESD、LVESV、VLEF较对照组均明显改善(P均<0.05)。结论在常规治疗基础上加用盐酸法舒地尔可提高运动耐量,改善患者心脏收缩功能,且耐受性良好。     

Usefulness of PCR Strategies For Early Diagnosis of Chagas'' Disease Reactivation and Treatment Follow-Up in Heart Transplantation

Heart transplantation (HTx) is a useful therapy for end‐stage Chaga? cardiomyopathy; however, Chagas reactivation remains a mayor complication. Parasitological methods offer poor diagnostic sensitivity, and use of more sensitive tools such as the Polymerase chain reaction (PCR) is usually necessary. In the present study, reactivation incidence and PCR usefulness for early reactivation diagnosis, as well as for treatment response evaluation during follow‐up, were analyzed using Strout parasite detection test, in 10 of 222 consecutive HTx patients suffering Chagas cardiomyopathy. PCR strategies targeted to minicircle sequences (kDNA, detection limit 1 parasite/ 10 mL blood) and miniexon genes (SL‐DNA, 200 parasite/10 mL) were performed to compare parasite burdens between samples. No patients received prophylactic antiprotozoal therapy (benznidazole). Five patients (50%) exhibited clinical reactivation within a mean period of 71.6 days; positive Strout results were observed in most cases presenting clinical manifestations. kDNA‐PCR was positive 38–85 days before reactivation, whereas SLDNA‐PCR became positive only 7–21 days later, revealing post‐HTx parasitic load enhancement present prior to clinical reactivation development. Reactivations were successfully treated with benznidazole and generated negative PCR results. Results observed in this study indicate the value of PCR testing for an early diagnosis of Chagas reactivation as well as for monitoring treatment efficacy.     

不同免疫剂治疗扩张型心肌病患儿心功能和临床预后评价

目的观察比较泼尼松联用硫唑嘌呤和大剂量静脉用免疫球蛋白(IVIG)对扩张型心肌病(DCM)患儿心功能及预后的影响。方法将DCM患儿20例分为治疗Ⅰ和Ⅱ组,分别在传统抗心衰治疗基础上加泼尼松联用硫唑嘌呤和大剂量IVIG,治疗1个疗程后评价心功能,检测治疗前后左房横径(LA)、左室舒张期内径(LV)、左室射血分数(LVEF)、心脏指数(CI),并计算其治疗前后变化。所有病例随访1年,计算1年存活率。结果治疗Ⅱ组心功能明显优于治疗Ⅰ组(P均<0.05);两组LA、LV、LVEF和CI治疗后较治疗前均有明显改善(P均<0.05);治疗Ⅰ组各参数变化明显低于治疗Ⅱ组(P均<0.05),其1年存活率明显低于治疗组Ⅱ(P<0.05)。结论泼尼松联用硫唑嘌呤和大剂量IVIG均能改善DCM患儿心功能,但IVIG疗效优于泼尼松联用硫唑嘌呤。     

糖心乐对糖尿病性心肌病大鼠血糖、血脂代谢的实验研究

目的:观察糖心乐(TXL)对糖尿病性心肌病(DCMP)大鼠血糖、血脂的影响。方法:SD大鼠随机分为正常对照组、模型对照组、达美康组及糖心乐大、小剂量组。将除正常对照组外的各组大鼠腹腔注射STZ建立糖尿病模型,造模后8w分别给予生理盐水、达美康、TXL大小剂量灌胃,连续4w,正常对照组不做处理。结果:与模型对照组比较,达美康组、糖心乐大、小剂量组大鼠的血糖、甘油三酯(TG)、总胆固醇(TC)显著降低(P<0.05,P<0.01),高密度脂蛋白(HDL-C)显著升高(P<0.05,P<0.01)。TXL大剂量组的降低血糖、TC,升高HDL-C作用与达美康组相当,降低TG作用优于达美康组(P<0.05)。结论:TXL可通过降低血糖、TG、TC水平,升高HDL-C水平来改善DCMP大鼠心肌的功能。     

厄贝沙坦治疗缺血性心肌病临床评价

目的:评价厄贝沙坦治疗缺血性心肌病心力衰竭的临床疗效。方法:缺血性心肌病病人随机分为治疗组和对照组,其中治疗组22例(厄贝沙坦150mg,qd,PO,加常规处理),对照组20例(常规处理)。详细记录入院时和治疗4周临床症状体征、心脏彩超左室射血分数(LVEF)值及药物不良反应,观察治疗4周心功能恢复情况。结果:治疗组临床心功能改善程度较对照组大(P<0.01),治疗组LVEF改善较对照级更明显(P<0.01),两组均无咳嗽及其他显著不良反应发生。结论:厄贝沙坦能够明显改善缺血性心肌病患者心功能,可明显提高LVEF,不良反应少。     

遗传性小脑型共济失调症(附55例报告)

本文分析了55例小脑型遗传性共济失调的临床资料，男女之比为4：1，2/3患者年龄在37～50之间，并发症状多为行走不稳。主要临床表现为兼有躯干和肢体共济失调（78.2％），眼震（56.4），构音障碍（69.l％），约1/4病人有病理征及家族史。讨论了本病与其他疾病所致共济失调的鉴别诊断，以及本病的病理学基础。     

常规应用螺内酯联合依那普利治疗老年扩张型心肌病心力衰竭

目的评价螺内酯联合依那普利治疗老年人扩张型心肌病（DCM）心力衰竭的有效性和安全性。方法以148例老年DCM心力衰竭患者作研究对象,按随机化原则分为观察组（74例）和对照组（74例）。对照组为依那普利,加上基础用药（地高辛、双氢克尿噻）;观察组为对照组用药加上螺内酯。治疗6周后复查两组的动态心电图、超声心动图。结果治疗后两组的左室射血分数（LVEF）均有增加,左室收缩末期容积（LVESV）和左室舒张末期容积（LVEDV）均有减少,24 h室性早搏（VA）数也均有减少;观察组与对照组比较,LVEF显著增加。两组均未发现高血钾及肝肾功能损害。结论在老年DCM心力衰竭常规用药的基础上,常规加用螺内酯联合依那普利的治疗有效、安全,可显著改善左室重构和防治猝死。