Motor function and respiratory capacity in patients with late‐onset pompe disease

Introduction: The relationship between skeletal muscle strength and respiratory dysfunction in Pompe disease has not been examined by quantitative methods. We investigated correlations among lower extremity proximal muscle strength, respiratory function, and motor performance. Methods: Concentric strength of the knee extensor and flexor muscles was measured with a dynamometer, and pulmonary function was evaluated using spirometry in 7 adult patients. The 6‐minute walk test and the 4‐step stair‐climb test were used for assessing aerobic endurance and anaerobic power, respectively. Results: Anaerobic motor performance correlated with strength of both thigh muscles. Respiratory function did not correlate with either muscle strength or motor function performance. Conclusions: Respiratory and lower extremity proximal muscles could be affected differentially by the disease in individual patients. Motor performance is influenced by thigh muscle strength and is less dependent of respiratory capacity in our cohort of ambulatory patients. Muscle Nerve 49:603–606, 2014     

实时三维超声心动图对两类不同病因心力衰竭患者左心室收缩同步性的临床研究

目的 比较缺血性心脏病与扩张型心肌病两类病因引起的慢性心力衰竭(心衰)患者左室收缩同步性.并分析两组患者不同步性指数(SDI)与左室射血分数(LVEF)的相关性.方法 43例心衰患者分为两组,A组为17例缺血性心脏病引起的心衰患者;B组为26例扩张型心肌病引起的心衰患者.应用实时三维超声心动图(RT一3DE)的全容积显像模式采集左室三维数据库,并用Qlab定量分析软件对其进行分析,获得左室舒张末容积(EDV)、收缩末容积(ESV)和左室射血分数(LVEF)、17节段"牛眼"图,16节段(除外心尖帽)收缩达最小容积时间的标准差并以心率校正后(Tmsv 16-SD/R-R)的百分数作为左室SDI.结果 A、B两组人选患者的年龄、心率、LVEF和EDV差异均无统计学意义(P>0.05);A组的SDI略低于B组,但差异无统计学意义,17节段"牛眼"图显示两组患者左室收缩延迟节段的位置分布各有不同;对数回归分析显示A、B两组的SDI与LVEF均呈良好的负相关(r=-0.83,r=-0.71,P< 0.01).结论 缺血性心脏病与扩张型心肌病心衰患者左室均存在一定程度的收缩不同步,SDI与左室整体收缩功能存在负相关,随收缩功能的减低而增加,两组收缩延迟节段的位置分布各有不同.     

Cryptococcal meningitis accompanying lymphocytic inflammation predominantly in cerebral deep white matter: A possible manifestation of immune reconstitution inflammatory syndrome

Cryptococcal meningitis is rarely complicated by immune‐mediated leukoencephalopathy, but the precise pathomechanism is uncertain. A 72‐year‐old Japanese man treated with prednisolone for Sweet disease developed a subacute progression of meningitis, which was considered as neuro‐Sweet disease. A treatment by methylprednisolone rapidly improved CSF findings with a remarkable decrease in lymphocyte numbers in the blood, but the patient's consciousness still worsened after the cessation of the treatment. The patient developed cryptococcal meningitis and MRI showed abnormal intensities predominantly in the cerebral deep white matter along with the recovery of lymphocyte numbers in the blood, which resulted in death. A postmortem examination of the brain revealed degenerative lesions, especially at the cerebral white matter and cortex adjacent to the leptomeninges abundantly infiltrated by Cryptococcus neoformans. In the affected cerebral deep white matter, perivascular infiltration of lymphocytes was prominent in coexistence with reactive astrocytes and vascular proliferation, but these findings were not observed in the subcortical and cortical lesions. Cryptococcus neoformans was not present within the brain parenchyma. This is the first report of a case suggesting that cryptococcal meningitis can accompany lymphocytic inflammation predominantly in cerebral deep white matter as a possible manifestation of immune reconstitution inflammatory syndrome.     

多普勒超声测定左心室Max（dp／dt）的新方法

心室内压力变化率峰值Ｍａｘ（ｄｐ／ｄｔ）是一项评价心肌收缩功能的重要指标。但其测定常需借助于心导管术，临床应用受到限制。依据流体力学基本定律，本文提出：等容收缩期内表现为压力的势能将在射血期部分地转化为以１／２ｐＶ２为形式的动能，因而势能的最大变化率Ｍａｘ（ｄｐ／ｄｔ）将在动能的最大变化率Ｍａｘ（ｄｋ／ｄｔ）上得到反映。应用特制计算机软件将主动脉血流速度曲线转化为４Ｖ２曲线，该曲线的一级导数峰值即为Ｍａｘ（ｄｋ／ｄｔ）。应用脉冲多普勒超声测定２３例先天性心脏病患儿的主动脉血流频谱参数并与心导管测定的左心室Ｍａｘ（ｄｐ／ｄｔ）进行对比分析。结果显示：主动脉血流频谱参数Ｍａｘ（ｄｋ／ｄｔ）与左心室Ｍａｘ（ｄｐ／ｄｔ）呈良好相关（ｒ＝０．８１７，Ｐ＜０．００１），直线回归方程为：Ｍａｘ（ｄｐ／ｄｔ）＝０．４７Ｍａｘ（ｄｋ／ｄｔ）＋６７．９（Ｋｐａ／ｓ），平均加速度ｍＡ及Ｖｐ２／ＡＴ与Ｍａｘ（ｄｐ／ｄｔ）亦有较好的相关性。表明由多普勒超声获得的主动脉血流频谱参数Ｍａｘ（ｄｋ／ｄｔ）能较准确地估侧左室Ｍａｘ（ｄｐ／ｄｔ），因而可作为评价心脏收缩功能的可靠指标     

电影MR对测定心功能价值的探讨

目的探讨电影MR测定心功能的价值。材料和方法；通过对21例健康成人电影MR成像，采用双盲法，分析和测量左心功能指标，并与超声心动图（UCG）测值进行相关性t检验。结果：电影MR测定左室短径、左室后壁／室间隔厚度、左室舒末／缩末容积等指标，与UCG比较，具有良好的相关性（r＝0．87－0．99）。结论：电影MR是又一检测心功能准确、无创的影像新技术，具有较大发展潜力。     

Roux-en-Y吻合重建对胃窦癌合并T2DM患者血糖及胰岛功能的影响

目的:探讨Roux-en-Y吻合重建术对胃窦癌合并2型糖尿病(T2DM)患者血糖水平、胰岛功能及术后生存质量的影响。方法:选取2010年1月—2014年1月收治的68例I~II期胃窦癌合并T2DM患者为研究对象,根据患者手术方式将其分为胃十二指肠吻合组(对照组)32例,Roux-en-Y吻合重建组(观察组)36例,对比分析2组患者术前、术后6个月体质量指数(BMI)、空腹血糖(FPG)、糖化血红蛋白(Hb A1c)、空腹胰岛素(FINS)、空腹C肽(FCP)水平。术前、术后6个月对2组患者行口服葡萄糖耐量试验(OGTT),检测及比较2组餐后2h血糖(2h FPG)、餐后2 h胰岛素(2h INS)及餐后2 h C肽(2h CP),计算2组胰岛素抵抗指数(HOMA-IR)。术前及术后6个月采用肿瘤生存质量量表(QOL)对2组患者生存质量进行评价。结果:对照组术后6个月BMI、FPG、Hb A1c、FINS、FCP、2h FPG、2h INS、2h CP、HOMA-IR与术前相比差异无统计学意义(P0.05)。观察组术后6个月FPG、Hb A1c、2h FPG、HOMA-IR水平显著低于术前,而FINS、FCP、2h INS、2h CP较术前显著升高(P0.05)。术后6个月观察组食欲、睡眠、自理能力、心理能力、疾病认识以及总生存质量评分均高于对照组(P0.05)。结论:Roux-en-Y吻合术能有效提高胃窦癌合并T2DM患者胰岛β细胞功能,降低血糖水平,提高患者生存质量。     

艾滋病合并新型隐球菌脑膜炎临床分析

目的通过对25例艾滋病合并新型隐球菌脑膜炎的临床分析,提高对艾滋病合并新型隐球菌脑膜炎的认识。方法对本院2000年8月—2006年2月收治的25例艾滋病合并新型隐球菌脑膜炎病例进行回顾性分析。结果25例新型隐球菌脑膜炎患者均为艾滋病晚期患者,T细胞亚群检测11例:CD4<50/mm39例,CD450~100/mm32例;平均26.18/mm3。在确诊时已合并多种机会感染。结论艾滋病患者合并新型隐球菌脑膜炎病情重,预后差,实验室病原学检查结果是诊断新型隐球菌脑膜炎的主要依据。