P型ATP酶阻滞剂和激动剂对肝豆状核变性成纤维细胞胞浆铜的影响

探讨P型ATP酶阻滞剂、激动剂对肝豆状核变性患儿离体培养皮肤成纤维细胞胞浆铜含量的影响。方法采用高浓度铜及P型AFP酶阻滞剂（矾酸钠）、激动剂（长春新碱）孵育细胞，分析孵育6、12、24小时后，肝豆状核变性患儿、杂合子及正常儿童成纤维细胞胞浆中铜含量的变化。结果铜及长春新碱孵育后，患儿成纤维细胞胞浆钢水平在各时点均高于杂合子及正常儿童。铜及矾酸钠孵育后，患儿、杂合子及正常儿童胞浆铜含量在各时点均明显少于其他条件孵育后。结论患儿钢转运P型ATP酶对P型ATP酶阻滞剂的敏感性与杂合子及正常儿童相同，对激动剂的敏感性异常。P型ATP酶能减少患儿成纤维细胞对铜的摄取，在肝豆状核变性的治疗方面有一定的提示意义。     

新型的治疗阿尔茨海默氏症药物(1-二甲基磷酰基-2,2,2-三氯)-乙基-1-醇烟酸酯对体外神经细胞的作用

目的：观察本实验室合成的一种治疗阿尔茨海默氏症(AD)的药物(1-二甲基磷酰基-2，2，2-三氯)-乙基-1-醇烟酸酯(NMF)，对体外培养的皮层神经细胞活性的影响以及对海人藻酸(KA)所致的神经损伤的保护作用。方法：采用体外培养皮层神经元的方法，解剖分离15d胚胎小鼠皮层神经细胞，接种于96孔板，48h后加药并培养72h，以MTT法观察NMF对小鼠皮层神经细胞活性的影响；同时将接种于24孔板的细胞预先给予NMF，d3时加或不加KA处理后，以台盼蓝染色鉴别并计数死、活细胞，可得出细胞的存活率。结果：NMF明显促进胎鼠皮层神经元活性，其中NMF 1、0．1、10 nmol·L~(-1)促进神经元活性增殖率分别高达34．7％、37．4％、36．7％。NMF明显促进正常胎鼠皮层神经元存活率，与对照组比较，10、1nmol·L~(-1)NMF对皮层神经元的存活率分别提高39．3％、73．5％。NMF能显著对抗KA所致的神经元损伤，与KA损伤组相比，NMF0．1、10、1nmol·L~(-1)对损伤皮层神经元的保护率分别为77．30％、80．10％、84．15％。结论：NMF明显促进胎鼠皮层神经元的活性、提高正常皮层神经元的存活率，并能有效地保护KA所致的神经元损伤，提示NMF是一种很有潜力的治疗AD的药物。     

多频稳态听觉诱发反应测试的临床应用

目的：利用多频稳态诱发反应(MFSSR)测试聋儿的残余听力，比较该方法与听性脑干反应(ABR)之间阳性率的差异来说明这种客观测试法所具有的优点。方法：在睡眠状态下测试了278例聋儿的MFSSR及ABR，分析了MFSSR不同频率阳性反应出现率及阈值分布范围，并与ABR阳性反应出现率进行比较。结果：左右耳MFSSR在0．5kHz、1．0kHz、2．0kHz和4．0kHz的阳性率分别为33．0g％、70．50％、44．96％、19．42％和33．81％、66．91％、41．07％、17．63％，而ABR的阳性率为16．91％和17．27％，MFSSR后3个频率的阳性反应出现率要明显高于ABR。结论：MFSSR作为一种客观测试方法具有频率特性好、刺激强度高、结果由计算机自动判断等特点，是一种有价值的客观测试方法。     

小儿纵隔肿瘤的诊治

目的 探讨胸部CT确诊纵隔肿瘤后的早期诊治。方法 回顾性分析近年本院收治的早期无明显胸部症状，经胸部CT检查确诊及定位的19例纵膈肿瘤的病例资料。结果 19例全部采用患侧后外侧切口入路，手术视野暴露充分，良性肿瘤能完整切除。结论 小儿纵隔内肿瘤一旦明确诊断，治疗以手术切除为首选方法。     

人巨细胞病毒UL140基因在临床低传代分离株中的多态性研究

目的　研究人巨细胞病毒HCMV UL14 0基因在临床低传代分离株中的多态性及其与致病性的关系。方法　对 4 0株HCMV临床低传代分离株进行UL14 0基因全序列的PCR扩增 ,对 12株进行了测序及结果分析。结果　 4 0株HCMV UL14 0基因PCR扩增均阳性。测序的 12株HCMV UL14 0开放阅读框架 (ORF)均在Toledo株UL14 0 ORF的第 174位核苷酸处 ,插入一个胞嘧啶核苷酸C ,造成移码突变。与Toledo株相比 ,临床分离株新增了ScAMP磷酸化SPS和酪蛋白激酶Ⅱ磷酸化CKP两种重要功能位点 ,且其所位于的氨基酸位点可能是UL14 0蛋白的蛋白质作用位点。结论　临床分离株UL14 0基因的ORF较Toledo株多出 2 31个核苷酸 ,故两者的核苷酸及其编码产物氨基酸序列明显不同。临床分离株存在SPS及CKP两种新的重要功能位点 ,可能在HCMV UL14 0编码蛋白的生物学功能方面起重要作用。     

脱髓鞘疾病51例

目的分析小儿脱髓鞘疾病的临床特点,以提高对该疾病的诊断与治疗。方法对51例小儿脱髓鞘疾病的发病年龄、前驱症状、临床表现及辅助检查特点等进行回顾性分析。结果51例脱髓鞘疾病患儿中学龄期38例,有前驱感染史32例,疫苗接种史3例,大多以急性起病。周围神经脱髓鞘疾病32例以双下肢或四肢瘫痪为首发;中枢神经脱髓鞘疾病以视力障碍、肢体无力、发热、抽搐、头痛等为首发。周围神经脱髓鞘疾病患儿中15例行肌电图检查,均显示为神经源性改变;18例中枢神经脱髓鞘疾病患儿中17例行CT或MRI,均发现异常信号。结论小儿脱髓鞘疾病的临床表现复杂多变,通过病史及辅助检查,进行综合分析,不难得出诊断,预后良好。     

Effects of GM-CSF, IL-3, and GM-CSG/IL-3 fusion protein on apoptosis of human myeloid leukemic cell line Tf-1 induced by irradiation

AIM: To observe the effects of three cytokines on the apoptosis of Tf-1 cells induced by y irradiation and investigate the relationship between apoptosis and caspase-3 activity. METHODS: Different cytokines GM-CSF, IL-3 and GM-CS/IL-3 fusion protein were added into the irradiated Tf-1 cells. MTT assay, morphology, flow cytometry, and DNA fragmentation assay were used to observe the effects of cytokines on apoptosis. The caspase-3 activity was determined with a fluorocytometer. RESULTS: Irradiated Tf-1 cells showed typical morphological characteristic of apoptosis demonstrated by transmission electron microscopy and were accumulated in G0/G1 phase. In the groups treated with growth factors after irradiation, three cytokines significantly increased the viability rate, distinctly decreased the apoptosis rate and the proportion of DNA fragmentation. When Tf-1 cells were irradiated by y     

Multi‐center Retrospective Study of Factors Affecting Perioperative Transfusion of Packed Red Blood Cells for Pelvic Fracture Patients

ObjectiveTo analyze the use of packed red blood cells (PRBCs) for patients with pelvic fracture and evaluate factors associated with PRBC transfusion for patients with pelvic fracture.MethodsThis retrospective cohort study collected 551 patients with pelvic fractures from six hospitals between September 1, 2012, and June 31, 2019. The age span of patients varied from 10 to 95 years old, and they were classified into two groups based on high‐energy pelvic fractures (HE‐PFs) or low‐energy pelvic fractures (LE‐PFs). The study''s outcome was the use of PRBCs, fresh frozen plasma (FFP), and albumin. Demographic data, characteristics, laboratory tests, clinical treatment details, and clinical outcomes were compared between the two groups. Factors that were statistically associated with perioperative PRBCs in univariate analyses were included to conduct an optimal scale regression to determine the independent factors for perioperative PRBCs.ResultsA total of 551 patients were screened from six hospitals, and after inclusion and exclusion, 319 were finally included and finished the follow‐up from admission to discharge, while four patients died during hospitalization. Three hundred and nineteen patients were classified into two groups by their injury mechanisms. A total of 230/319 (72.1%) patients were classified into the HE‐PF group, and 89/319 (27.8%) patients were classified into the LE‐PF group. Patients in the HE‐PF group were transfused with 4.5 (3–8) units of PRBCs, 300 (0–600) ml of FFP, and 0 (0–30) g of albumin, while patients in the LE‐PF group were transfused with 3.5 (2–4.5) units of PRBCs, 0 (0–295) ml of FFP, and 0 (0–0) g of albumin (all P < 0.001). There were higher proportions of male patients and patients under 65 in the HE‐PF group (all P < 0.001). HE‐PF group patients were more severely injured and likely to take external fixation. The optimal scale regression revealed four significant factors associated with perioperative transfused PRBCs, which were patients on admission with hemorrhagic shock (importance = 0.283, P = 0.004), followed by fracture types identified by Tile classification (importance = 0.156, P < 0.001), hemoglobin levels below 70 g/L on admission (importance = 0.283, P = 0.004), followed by fracture types identified by Tile classification (importance = 0.156, P < 0.001), hemoglobin levels below 70 g/L on admission (importance = 0.148, P = 0.039), and methods of pelvic fixation (importance = 0.008, P = 0.026), ranked by the importance.ConclusionPatients with HE‐PFs had increased transfusions of PRBCs, FFP, and albumin, and hemorrhagic shock on admission, Tile classification, Hb levels, and stabilization methods were found to be associated with perioperative PRBCs.     

Selenium substituted axitinib reduces axitinib side effects and maintains its anti-renal tumor activity

Axitinib is a potent vascular endothelial growth factor receptor (VEGFR) inhibitor, which has a strong inhibitory effect on the three isoforms of VEGFR 1–3. Having strong therapeutic efficacy, its broad use is limited by its side effects such as hypertension, proteinuria, cardiovascular damage, and liver and kidney dysfunction. Selenium compounds are broadly reported to have a good protective effect on cardiovascular disease, inflammation, infection, and immune function. In this study, a selenium substitute of axitinib was synthesized, and its anti-renal cell carcinoma activity and side effects were investigated. The results of the study indicated that Se-axitinib had potent antitumor activity on renal cell carcinoma (RCC), alleviated vascular hyperpermeability, and also alleviated axitinib-related side effects including hypertension, liver dysfunction and kidney dysfunction significantly. Therefore, we suggest that Se-axitinib could be a solution to the severe side effects of VEGFR inhibitors and provide evidence to improve the outcome of RCC treatment.

Se-axitinib is a selenium substitution of sulfur in axitinib, which reduced the side effect of VEGFR inhibitors and maintained the potent anticancer activity of the original drug.     

印迹基因Snrpn在人类卵母细胞及植入前胚胎mRNA表达

目的了解印迹基因Snrpn在人类卵母细胞和植入前胚胎的表达,以分析Snrpn基因表达对人类卵母细胞和早期胚胎发育的影响。方法采用单细胞巢式逆转录多聚酶链式反应(Single Cell Nested RT-PCR)方法,检测人类卵子和植入前各个阶段胚胎的印迹基因Snrpn mRNA表达情况。结果Snrpn在人类卵母细胞GV、MI、MII和植入前胚胎2、4、6、8细胞期都有表达,单卵母细胞扩增成功率为87.5%,单个胚胎为75.8%,平均扩增成功率为79.1%。单个卵母细胞和单个卵裂球扩增成功率无显著性差异(P>0.05),但单卵母细胞较单个卵裂球高。结论建立了比较稳定、可靠的单细胞巢式RT-PCR技术检测基因表达。印迹基因Snrpn在人类卵细胞和植入前胚胎的表达对卵子生长和胚胎发育有着重要意义,为早期胚胎阶段该基因表达正常图谱提供实验依据