Osteochondroma of the cervical spine—a surprising finding in a liver transplanted patient with polyneuropathy and polyradiculitis: case report

BACKGROUND

Osteochondroma of the spine is a rare condition. We report a case of a patient with a cervical osteochondroma presenting with a polyneuropathy and polyradiculitis simultaneously.

CASE DESCRIPTION

In a liver-transplant patient with progressive neurological deficits a polyneuropathy and a polyradiculitis were diagnosed. Eventually the patient became quadraparetic and an osteochondroma compressing the cervical spinal cord was found. The patient’s neurological symptoms markedly improved after gross total tumor resection and antibiotic therapy.

CONCLUSIONS

Review of the literature reveals this case to be an unusual presentation of a cervical osteochondroma, its diagnosis being delayed because of concomitant neurological diseases.     

LOC283731 promoter hypermethylation prognosticates survival after radiochemotherapy in IDH1 wild‐type glioblastoma patients

MGMT promoter methylation status is currently the only established molecular prognosticator in IDH wild‐type glioblastoma multiforme (GBM). Therefore, we aimed to discover novel therapy‐associated epigenetic biomarkers. After enrichment for hypermethylated fractions using methyl‐CpG‐immunoprecipitation (MCIp), we performed global DNA methylation profiling for 14 long‐term (LTS; >36 months) and 15 short‐term (STS; 6–10 months) surviving GBM patients. Even after exclusion of the G‐CIMP phenotype, we observed marked differences between the LTS and STS methylome. A total of 1,247 probes in 706 genes were hypermethylated in LTS and 463 probes in 305 genes were found to be hypermethylated in STS patients (p values < 0.05, log2 fold change ± 0.5). We identified 13 differentially methylated regions (DMRs) with a minimum of four differentially methylated probes per gene. Indeed, we were able to validate a subset of these DMRs through a second, independent method (MassARRAY) in our LTS/STS training set (ADCY1, GPC3, LOC283731/ISLR2). These DMRs were further assessed for their prognostic capability in an independent validation cohort (n = 62) of non‐G‐CIMP GBMs from the TCGA. Hypermethylation of multiple CpGs mapping to the promoter region of LOC283731 correlated with improved patient outcome (p = 0.03). The prognostic performance of LOC283731 promoter hypermethylation was confirmed in a third independent study cohort (n = 89), and was independent of gender, performance (KPS) and MGMT status (p = 0.0485, HR = 0.63). Intriguingly, the prediction was most pronounced in younger GBM patients (<60 years). In conclusion, we provide compelling evidence that promoter methylation status of this novel gene is a prognostic biomarker in IDH1 wild‐type/non‐G‐CIMP GBMs.     

自主研制镍钛记忆合金左心耳封堵器封闭左心耳对实验动物左心功能的影响

目的:应用经胸彩色多普勒超声技术评价自主研制的镍钛记忆合金左心耳封堵器封闭左心耳对实验动物猪左心房、左心室功能的影响。方法:实验于2005-09/2006-08在南京医科大学第一附属医院江苏省实验动物中心完成。①实验分组:选用苏钟小型种猪17只,随机分为实验组12只和对照组5只。②实验干预:实验组12只苏钟小型种猪使用自主研制的左心耳封堵器(发明专利号码:200610037789.3,公开号CN1799521,由镍钛合金骨架、多聚四氟乙烯膜和传送连接部分等构成。其外观呈单盘状,封堵器的左心房面呈圆盘状,直接连接放入心耳内的圆柱体结构)行左心耳封堵,对照组5只手术步骤相同而不采用封堵器行左心耳封堵。③实验评估:两组动物分别于术前、术后1周、2周、4周采用经胸超声心动图检查观察心功能的改变,测量左心房内径、最大及最小容积、左房射血分数、左心房搏出量、血流分数等左房功能参数以及左室射血分数、左室短轴缩短率、Tei指数、E/A比值等指标。结果:①实验动物数量分析:在施行左心耳封堵后,1头猪于术中出血过多并出现室颤后死亡,1头猪因封堵器脱入左房,卡在二尖瓣口导致死亡。其余动物封堵效果良好。②两组动物术后1,2,4周左房功能指标各参数与术前比较无明显变化(P>0.05);与术前相比,实验组术后1周、2周左室射血分数、左心室短轴缩短率、E/A比值分别由术前的0.70±0.04、0.39±0.03、1.33±0.28降低至术后1周的0.59±0.05、0.31±0.03、0.95±0.11(P<均0.01)及术后2周的0.62±0.05、0.33±0.05、0.90±0.05(P<均0.01);Tei指数由术前的0.48±0.02增加至术后1周的0.59±0.03(P<0.01)及术后2周的0.58±0.04(P<0.01)。对照组手术前后左室功能指标差异无显著性。结论:自主研制左心耳封堵器可以有效的封堵左心耳;左心耳封堵后短期内对实验动物左房功能无明显影响;封堵后短期内对左心室功能具有短期的减弱,更长期的安全性有待于进一步研究。     

应用经胸超声心动图评估快速左心耳起搏心房颤动模型心脏结构及功能的变化

目的:应用经胸超声心动图观察高频率左心耳起搏致猪慢性心房颤动模型心脏结构和功能的变化。方法:实验于2005-09/2006-08在南京医科大学第一附属医院江苏省实验动物中心完成。①12只苏钟种猪随机分为实验组及对照组各6只,所有动物均开胸,将起搏电极固定在左心耳根部,高频率脉冲发生器植入左侧胸部囊袋。实验组术后恢复1周后起搏器以500次/min的频率快速起搏左心耳8周;对照组始终不起搏。②术后心电图定期监测起搏、心房颤动的发生情况;于术前、起搏后1周、起搏后4,8周超声心动图观察实验动物左房内径、心室收缩及舒张末左房面积、左房和左室射血分数、左室舒张及收缩末内径、左心室短轴缩短率等变化。结果:①实验组5只完成了实验,术后2周复查心电图,1只动物发生房颤,起搏8周3只发生阵发性房颤,1只未发生房颤;对照组则未发生任何心律失常情况。②左心房相关指标:起搏后1周,实验组左房内径、收缩末期左心房面积和舒张末期左房容积均较起搏前增加[(2.70±0.12),(2.50±0.12)cm;(6.78±0.81),(6.21±0.93)cm2;(4.66±0.53),(3.78±0.57)mL;P均<0.05],左房射血分数较起搏前下降[(55.6±6.0)%,(63.8±4.0)%,P<0.01],至起搏4,8周,左房射血分数进一步降低,左房内径等指标则继续增大。③左心室相关指标:起搏后1周,实验组左室舒张、收缩末期内径较起搏前增加[(3.64±0.13),(3.46±0.15)cm;(2.48±0.08),(2.14±0.09)cm;P均<0.01],左心室短轴缩短率和左室射血分数较起搏前下降[(31.6±2.0)%,(37.8±3.0)%;(60.8±2.0)%,(69.2±4.0)%;P均<0.01];至起搏4,8周,左心室短轴缩短率和射血分数进一步降低,左室舒张、收缩末期内径则继续增大,与对照组比较也差异显著(P<0.05,0.001)。结论:①超声心动图是监测房颤模型建立过程中心房、心室结构和功能变化的有效手段。②高频起搏左心耳是建立猪心房颤动模型的有效方法,快速心房起搏可导致左心房左心室增大及心功能减退。     

主题综合疗法辅导戒毒患者的个案报告

目的:中国戒毒心理辅导工作起步较晚,还未形成有效的方法体系,本个案采用主题综合疗法对戒毒人员进行心理辅导,旨在探索有效的戒毒心理辅导模式。方法:心理辅导于2004-11/12在广州三九脑科医院进行,戒毒患者,男,25岁,吸毒史8年,采用主题综合疗法。根据戒毒人员普遍存在的心理问题和患者所存在的特殊问题,围绕培养积极生活态度,促进自我发展;增强戒毒信心,防止复吸的总目标,确定咨访关系建立;发现自我,建立戒毒信心;家庭关系及人际关系辅导;约定辅导4个主题,采用了半结构式的综合策略,贯穿着人本主义理念,以行为矫正法为主,综合了精神分析,认知疗法的具体技术。共进行了12次辅导,每次约50min,每周3次,总时程1个月。在心理辅导前后对患者进行药物依赖程度量表,抑郁自评量表和焦虑自评量表的测试,比较前后得分情况。结果:整个辅导获得了一定的成效,与患者建立了良好的咨询关系,在金钱管理、拒绝毒友、家庭沟通、共同约定几个主题效果较显著。患者主观上对海洛因依赖程度减轻,客观上药物依赖程度量表得分较辅导前降低(18,24);抑郁自评量表和焦虑自评量表得分均降低(抑郁量表前测粗分为40,后测为34,焦虑量表前测粗分为42,后测为32)。结论:主题综合戒毒疗法对于培养戒毒患者的积极生活态度,增强戒毒信心有明显效果,认知行为矫正技术是针对戒毒心理辅导的有效疗法。     

Hairy cell leukemia: a tumor of pre-plasma cells

Monoclonal antibodies defining B-, T-, and myeloid-restricted cell surface antigens were used to characterize the lineage and state of differentiation of tumor cells isolated from 22 patients with hairy cell leukemia (HCL). These tumors were shown to be of B lineage because they strongly expressed the B cell-restricted antigens B1 and B4 and lacked T cell- and monocyte-restricted antigens. Moreover, the strong expression of the plasma cell-associated PCA-1 antigen on the majority of hairy cells suggested that these tumors correspond to later stages of B cell ontogeny. Dual fluorescence experiments further confirmed that HCL splenocytes that coexpressed B1 and PCA-1 demonstrated both the morphology and tartrate-resistant acid phosphatase positivity of hairy cells. The observation that some hairy cells either spontaneously produce immunoglobulin (Ig) or could be induced to proliferate and secrete Ig provides complementary support for the view that HCL is a pre-plasma cell tumor. However, staining of hairy cells with anti-IL2R1 monoclonal antibody, which is directed to the T cell growth factor receptor and/or with the anti-Mo1 reagent, directed to C3bi complement receptor, distinguish these cells from currently identified B cells.     

Long-term in vivo expression of a murine adenosine deaminase gene in rhesus monkey hematopoietic cells of multiple lineages after retroviral mediated gene transfer into CD34+ bone marrow cells

Retroviral mediated gene transfer into stem cells has been proposed as therapy for many inherited hematopoietic diseases. Deficiency of the enzyme adenosine deaminase (ADA) results in depletion of T lymphocytes, causing severe combined immunodeficiency syndrome (SCIDS). In this report, we describe retroviral mediated gene transfer of a murine ADA cDNA into Rhesus monkey hematopoietic stem cells. Immunoselected CD34+ bone marrow cells were exposed to medium containing the ADA retrovirus during culture on a stromal cell line engineered to express the transmembrane form of stem cell factor. After infusion of autologous, transduced cells into irradiated recipients, gene transfer was observed in all three monkeys. The ADA provirus was detected in 2% of circulating granulocytes and T cells from 100 days post-transplantation to longer than 1 year and in B cells from 250 days post-transplantation and beyond. Mouse ADA activity was detected in peripheral blood cells at approximately 3% the activity of monkey ADA. Thus, we have shown gene transfer into repopulating cells that contribute to all hematopoietic lineages with persistent gene expression. These data provide support for the use of stem cell targeted gene transfer for therapy of ADA deficiency.