Long-term surgical outcomes of cervical myelopathy with athetoid cerebral palsy

Purpose

To understand the long-term surgical outcomes and prognostic factors for the operative treatment of cervical myelopathy (CM) in patients with athetoid cerebral palsy (ACP).

Methods

We retrospectively reviewed 24 patients with ACP who underwent surgery for CM at our hospital between March 2002 and June 2008. All patients had more than 5 years follow-up. Anterior fusion (11 patients), posterior fusion (1 patient), or combined anterior and posterior (AP) fusion (7 patients) and C1-2 fusion (5 patients) surgeries were performed. Surgical outcomes (average follow-up 102 months), as assessed using modified JOA (mJOA) scores, the Neck Disability Index (NDI), and a visual analog scale (VAS) were compared between the preoperative and postoperative states.

Results

Preoperatvie cervical kyphosis decreased mJOA scores significantly. Long-term follow-up clinical outcomes demonstrated that 10 patients showed favorable (excellent and good) outcomes and 11 patients had non-favorable (fair and worse) outcomes. According to the mJOA scores, patients showed postoperative improvement (7.10–10.45). NDI decreased from 68.46 to 31.66. A second operation was done in seven cases due to instrument failure, progressive kyphotic deformities and adjacent segment degeneration. A preoperative botulinum toxin injection significantly decreased (p < 0.05) the incidence of a second operation.

Conclusions

Patients with ACP have high incidence of instrument failure. Strong surgical fixation, bone fusion and perioperative immobilizations using botulinum toxin injection should be carefully planned preoperatively.     

Conversion of Aβ43 to Aβ40 by the successive action of angiotensin‐converting enzyme 2 and angiotensin‐converting enzyme

The longer and neurotoxic species of amyloid‐β protein (Aβ), Aβ42 and Aβ43, contribute to Aβ accumulation in Alzheimer's disease (AD) pathogenesis and are considered to be the primary cause of the disease. In contrast, the predominant secreted form of Aβ, Aβ40, inhibits amyloid deposition and may have neuroprotective effects. We have reported that angiotensin‐converting enzyme (ACE) converts Aβ42 to Aβ40 and that Aβ43 is the earliest‐depositing Aβ species in the amyloid precursor protein transgenic mouse brain. Here we found that Aβ43 can be converted to Aβ42 and to Aβ40 in mouse brain lysate. We further identified the brain Aβ43‐to‐Aβ42‐converting enzyme as ACE2. The purified human ACE2 converted Aβ43 to Aβ42, and this activity was inhibited by a specific ACE2 inhibitor, DX600. Notably, the combination of ACE2 and ACE could convert Aβ43 to Aβ40. Our results indicate that the longer, neurotoxic forms of Aβ can be converted to the shorter, less toxic or neuroprotective forms of Aβ by ACE2 and ACE. Moreover, we found that ACE2 activity showed a tendency to decrease in the serum of AD patients compared with normal controls, suggesting an association between lower ACE2 activity and AD. Thus, maintaining brain ACE2 and ACE activities may be important for preventing brain amyloid neurotoxicity and deposition in Alzheimer's disease. © 2014 Wiley Periodicals, Inc.     

来氟米特联合糖皮质激素治疗IgA肾病的临床研究

目的 观察应用来氟米特（Leflunomide,LEF）联合激素治疗IgA肾病的疗效和安全性.方法 选择IgA肾病患者63例,分为LEF合并激素治疗组（LEF组）及大剂量激素治疗组（激素组）,观察治疗前和治疗6个月、12个月后的24 h尿蛋白定量、血清白蛋白、肾小球率过滤（eGFR）、收缩压、舒张压等实验室指标的变化,并进行疗效评价.结果 LEF组和激素组治疗6个月和12个月后的24 h尿蛋白定量、收缩压、舒张压均较治疗前显著下降（P＜0.05）,血清白蛋白水平较治疗前显著升高（P＜0.05）,2组eGFR变化均无统计学差异（P＞0.05）.治疗12个月后LEF组的总有效率明显高于激素组（P＜0.05）,而完全缓解率和部分缓解率的差异无统计学意义（P＞0.05）,2组不良反应无显著性差异（P＞0.05）.结论 LEF联合激素可以作为治疗IgA肾病的选择之一,且安全、有效.     

D二聚体与原发性肾病综合征临床及病理的相关分析

目的 对血清D二聚体（D-Dimer,简称D-D）与原发性肾病综合征（primary nephrotic syndrome,PNS）临床及病理资料相关性进行分析,探讨血清D-D在PNS中的临床意义.方法 符合肾病综合征的诊断标准及本研究排除标准的肾病综合征患者为PNS组,健康体检者为对照组,收集一般临床指标和患者的病理资料,进行病例对照研究与临床及病理资料分析.结果 （1）120例PNS患者血清D-D水平较60例正常人的血清D-D水平明显升高,差异有统计学意义（P＜0.05）.（2）临床资料显示120例PNS患者中,血清D-D水平与C-反应蛋白（CRP）、24 h尿蛋白之间存在正相关（r=0.429,P=0.000;r=0.481,P=0.000）,但与白蛋白（Alb）、血尿素氮（BUN）、血肌酐（SCr）、血清三酰甘油（TG）、血清总胆固醇（CHOL）之间无相关性（P＞0.05）.（3）微小病变型肾病、系膜增生性肾小球肾炎、系膜毛细血管性肾小球肾炎、膜性肾病及局灶性节段性肾小球硬化5种病理类型间血清D-D水平相比有统计学意义（P＜0.05）,其中微小病变型肾病和膜性肾病患者的血清D-D水平均较其他3种病理类型患者的血清D-D水平明显升高,有统计学意义（P＜0.05）,而微小病变型肾病和膜性肾病患者间及其余3种病理类型患者间的血清D-D水平相比无统计学意义（P＞0.05）.结论 PNS存在高凝状态,血清D-D水平可间接提示蛋白尿严重程度,并且可能间接预测PNS病理的类型.     

IFN-γ对胆囊癌荷瘤小鼠IL-10和单核-巨噬细胞的影响

目的探讨IFN-γ对胆囊癌小鼠模型中肿瘤内IL-10和单核-巨噬细胞的影响。方法20只BALB／C裸鼠通过皮下接种人胆囊癌细胞株GBC—SD构建胆囊癌小鼠模型,按照随机数字表法分为IFN-γ组和对照组（每组各10只）。IFN．叮组小鼠在肿瘤内注剃、鼠重组IFN-γ 0．1mL（1×10^5kU／L,生理盐水溶解）,对照组注射同等剂量的生理盐水,通过ELISA法检测肿瘤中鼠源性IL-10的表达情况,通过免疫组织化学染色法计数CDl4’细胞（单核-巨噬细胞）、CD64^＋细胞（M1型巨噬细胞）和CD206’细胞（M2型巨噬细胞）,并以Student’st检验对所得数据进行分析。结果接种肿瘤细胞1周后,裸鼠皮下全部成瘤,固定于左前肢腋下。IFN-γ组存活9只,对照组存活7只。IFN-γ组裸鼠肿瘤质量为（518±138）mg,对照组为（669±128）mg;IFN．1组肿瘤体积为（456±172）mm。,对照组为（505±146）mm。,两组裸鼠肿瘤质量和体积比较,差异无统计学意义（t=2．240,1．503,P〉0．05）。IFN-γ组裸鼠肿瘤中鼠源性IL-10浓度为（58±16）μg／g,显著低于对照组的（102±45）“g／g,两组比较,差异有统计学意义（t=2．796,P〈0．05）。IFN-γ组中单核-巨噬细胞计数为814-16,显著高于对照组的50-e21;M1型巨噬细胞计数为66±12,显著高于对照组的94-4;M2型巨噬细胞计数为154-4,显著低于对照组的40±14,两组细胞计数比较,差异均有统计学意义（t=3．214,13．127,6．914,P〈0．05）。结论IFN-γ能够降低肿瘤微环境中的IL-10浓度。IFN-γ能够诱导单核-巨噬细胞浸润至胆囊癌细胞问质中,主要分化为M1型巨噬细胞。