晕可宁颗粒对急、慢性梅尼埃病模型的影响

目的　探讨晕可宁颗粒的主要药效学 ,为临床提供药效学资料及治疗学基础。方法　采用三氯甲烷破坏豚鼠一侧膜迷路感受器模型 ,探讨受试药对眼震颤、摆头及旋转的影响 ;采用内淋巴囊和内淋巴管阻塞手术复制豚鼠膜迷路实验性膜迷路积水模型 ,研究内耳组织平均中阶面积 (SMA)增加率及形态学的变化。结果　抑眩宁阳性对照组、晕可宁颗粒 (8、16g/kg)模型给药组豚鼠眼球震颤次数减少 ,差异有显著意义 (P <0 .0 5 )。成功复制了不同程度膜迷路积水豚鼠模型 ,表现为前庭膜重度膨出 ,前庭阶缩小 ,膜蜗管增大 ,SMA增加率变大 ,差异有显著意义 (P <0 .0 1) ;晕可宁颗粒灌胃后可减轻豚鼠实验性膜迷路积水的程度 ,差异有显著意义 (P <0 .0 1) ;但与空白对照组比较SMA增加率差异无显著意义 (P >0 .0 5 )。结论　晕可宁颗粒可以减轻内淋巴囊积水程度 ,对梅尼埃病症状有对抗治疗作用。     

Relative frequency of solitary melanocytic lesions of the oral mucosa

Background: Solitary pigmented lesions of melanocytic origin are uncommon in the oral mucosa. These lesions include the oral and labial melanotic macule, oral melanocytic nevus, oral melanoacanthoma, oral melanoma and atypical melanocytic proliferation. The purpose of the study was twofold: to report a large series of solitary melanocytic lesions from one source, and to determine the relative frequency of these lesions. Methods: The study was based on a systematic search of the files of the Pacific Oral and Maxillofacial Pathology Laboratory, University of the Pacific, San Francisco for solitary pigmented melanocytic lesions (benign and malignant) accessed during the years 1984–2002. Results: Of the 89 430 biopsies accessed during the 19‐year period, 773 (0.83%) cases of solitary pigmented melanocytic lesions in the oral mucosa were identified. Oral and labial melanotic macules were the most common melanocytic lesions comprising 86.1% of the entire group and 0.7% of the total number of accessed biopsies. The vermilion border and gingiva were the most common sites (31.1% and 31.0% respectively). Oral melanocytic nevi comprised 11.8% of the entire melanocytic group and 0.1% of the total number of biopsies. The most common site was the palate (44%). Intramucosal nevi were the most common (64%), followed by compound nevi (16.5%) and common blue nevi (16.5%). Junctional nevi were uncommon (3.0%). Oral melanoacanthoma comprised only 0.9% of the entire melanocytic group and 0.008% of the total number of biopsies. Oral melanoma and atypical melanocytic proliferation were the least common lesions each comprising 0.6% of the entire melanocytic group and 0.006% of the total number of biopsies. The most common site for oral melanoma was the palate (60%). Conclusion: The palate was the most common location for both melanocytic nevi and oral melanoma. Thus, all melanocytic lesions in the palate should be viewed with caution and biopsy is recommended to rule out melanoma. Further studies are required to elucidate the entity of oral atypical melanocytic proliferation.     

Morel-Lavallée损伤诊疗进展

Morel-Lavallée(MLL)损伤是由剪切力等暴力使皮肤、皮下组织与深筋膜分离而引起的复杂的软组织闭合性撕脱损伤,疾病初期具有复杂性和隐蔽性,常因延迟诊断而导致感染、截肢、多器官衰竭甚至死亡等。目前,MLL的治疗尚无统一指南,需根据损伤情况及患者意愿综合考虑。在合并开放伤、骨折、神经血管肌腱损伤时,处理的优先顺序尚存在很大的争议。本文对MLL的发病机制、临床表现、影像学检查、治疗等进行综述,以提高临床对该类疾病的关注和认识。     

TRAF6通过内源性凋亡通路促进卡介苗诱导的巨噬细胞凋亡

目的 探讨肿瘤坏死因子受体相关因子6（TRAF6）对卡介苗（BCG）诱导巨噬细胞凋亡的调控作用。方法 Q-PCR检测活动性结核患者外周血中TRAF6的表达后利用不同感染复数的BCG感染RAW264.7细胞,在感染的不同时间通过Q-PCR和Western blot检测巨噬细胞中Caspase 3和TRAF6的表达。时间梯度试验处理组分为5组：对照组（未感染,C）、BCG感染6 h组、12 h组、18 h组和24 h组;感染复数试验处理组分为5组：对照组（未感染,C）、5 MOI组、10 MOI组、15 MOI组和20 MOI组。随后,通过小干扰RNA技术敲减RAW264.7细胞中TRAF6的表达,并结合BCG感染建立TRAF6敲减模型。TRAF6敲减实验分为4组：阴性对照组（NC）、BCG单独感染组（BCG）、TRAF6小干扰RNA单独处理组（siRNA）和TRAF6小干扰RNA与BCG共处理组（siRNA+BCG）。利用平板涂布法检测巨噬细胞菌载量变化。通过流式细胞术检测巨噬细胞凋亡率和线粒体膜电位变化。利用Western blot检测TRAF6、Caspase 3、PARP、Bcl-2,BAX的表达。使用免疫荧光技术检测TRAF6与Caspase 3的表达。结果与健康人群相比,活动性结核患者外周血中TRAF6高表达（P<0.001）。BCG感染显著上调RAW264.7中TRAF6与Caspase 3的表达,且当感染时间为18 h（P=0.03,P=0.04）,感染复数为15时二者表达量最高（P<0.001,P<0.001）。敲减TRAF6上调BCG感染的巨噬细胞内的菌载量（P=0.05）。与BCG单独处理组相比,敲减TRAF6显著抑制BCG感染的巨噬细胞凋亡率（P<0.001）,并显著下调Caspase 3（P=0.002）和PARP的表达（P<0.001）。同时,BCG感染RAW264.7细胞的线粒体膜电位上升（P<0.001）,而敲减TRAF6抑制BCG感染的巨噬细胞线粒体膜电位（P<0.001）。敲减TRAF6显著下调BCG感染巨噬细胞中BAX表达（P=0.005）的同时上调Bcl-2的表达（P=0.04）。结论 TRAF6通过内源性凋亡通路促进BCG感染的巨噬细胞凋亡。     

A severe form of Noonan syndrome and autosomal dominant café-au-lait spots – evidence for different genetic origins

Aim: The clinical overlap among Noonan syndrome (NS), cardio-facio-cutaneous (CFC), LEOPARD and Costello syndromes as well as Neurofibromatosis type 1 is extensive, which complicates the process of diagnosis. Further genotype–phenotype correlations are required to facilitate future diagnosis of these patients. Therefore, investigations of the genetic cause of a severe phenotype in a patient with NS and the presence of multiple café-au-lait spots (CAL) spots in the patient and four members of the family were performed.
Methods: Mutation analyses of candidate genes, PTPN11 , NF1, SPRED1 and SPRED2, associated with these syndromes, were conducted using DNA sequencing.
Results: A previously identified de novo mutation, PTPN11 F285L and an inherited NF1 R1809C substitution in the index patient were found. However, neither PTPN11 F285L, NF1 R1809C, SPRED1 nor SPRED2 segregated with CAL spots in the family. The results indicate that the familial CAL spots trait in this family is caused by a mutation in another gene, distinct from previous genes associated with CAL spots in these syndromes.
Conclusion: We suggest that the atypical severe symptoms in the index patient may be caused by an additive effect on the F285L mutation in PTPN11 by another mutation, for example the NF1 R1809C or alternatively, the not yet identified gene mutation associated with CAL spots in this family.     

卡介菌多糖核酸治疗小儿咳嗽变异性哮喘

目的：观察卡介菌多糖核酸注射液在小儿咳嗽变异性哮喘治疗应用的疗效。方法：将本科40例咳嗽变异性哮喘患儿，随机分为治疗组和对照组（各20例）。治疗组给予BCG—PSN＋必可酮气雾剂，对照组：给予单用必可酮气雾剂吸入，连续吸入6周，观察疗效，并进行统计学处理。结果：治疗组总有效率为95％，对照组总有效率为70％，统计学处理总有效率有显著差异（P〈O．01）。结论：卡介菌多糖核酸（polysaccharide nucleic acidfraction of BCG，BCG—PSN）能有效地控制咳嗽变异性哮喘（CVA）的呼吸道的反复感染，提高免疫功能；与必可酮配合，能有效地控制CVA的复发。     

瑞芬太尼联合硝酸甘油控制性降压在鼻内镜手术中的应用

目的比较瑞芬太尼和瑞芬太尼联合硝酸甘油在全麻下鼻内镜手术中控制性降压效果。方法鼻内镜下鼻息肉、鼻窦炎手术病人共40例,ASAⅠ或Ⅱ级,用随机数字表分为瑞芬组和瑞硝组,每组20例,咪达唑仑、丙泊酚、瑞芬太尼和维库溴铵静脉复合全麻气管插管,2组术中均以瑞芬太尼0.1～0.2μg·kg~(-1)·min~(-1)、丙泊酚2～5 mg·kg~(-1)·h~(-1)持续泵输,维库溴铵4 mg·h~(-1),静脉注射。瑞硝组加用硝酸甘油静脉滴注,开始剂量5μg·min~(-1),调节滴速使收缩压维持在12 kPa(90 mmHg)左右。观察降压前(T_0)、降压后5 min(T_1)、30 min(T_2)、60 min(T_3)和停止降压后5 min(T_4)的收缩压和心率,计算心肌氧耗指数,瑞芬太尼、丙泊酚用量,手术时间和病人苏醒时间。结果2组病人年龄、性别和体重间差别无统计学意义(P>0.05)。T_1时2组血压明显下降,与T_0比较,均P<0.01,且瑞硝组降压程度明显大于瑞芬组(P<0.01),降压后的T_1,T_2瑞硝组心率加快,与瑞芬组相比有非常显著意义(P<0.01);降压期的氧耗指数,瑞硝组低于瑞芬组(P<0.05,P<0.01);停止降压后,瑞芬组血压和心率变化与T_3比,无统计学意义(P>0.05),但瑞硝组血压和心率均明显增加(P<0.01);瑞芬组的瑞芬太尼、丙泊酚用量和手术时间明显大于瑞硝组(P<0.01),2组病人的苏醒时间差异无统计学意义(P>0.05)。结论鼻内镜手术瑞芬太尼在常用剂量范围内作控制性降压虽比较平稳,但难以达到目标血压,联合硝酸甘油后效果明显,并可减少麻醉药用量和手术时间,减少病人医疗费用。     

中药灯盏细辛中酚酸类化合物的结构与活性研究

目的 研究中药灯盏细辛的化学成分。方法 利用硅胶柱层析等分离手段从灯盏细辛[Erigeron breviscapus(Vant.)Hand-Mazz]的醋酸乙酯部位分离得到2个化合物，采用波谱方法（IR，MS，^1H-NMR,^13C-NMR,2D-NMR)鉴定了它们的结构。结果 2个酚酸类化合物分别为：5－O－咖啡酰基－奎宁酸甲酯（I）和4－O－咖啡酰基－奎宁酸甲酯（Ⅱ），结论 化合物Ⅰ，Ⅱ为首次从灯盏细辛中分得的酚酸类化合物。药理活性研究显示，化合物Ⅰ，Ⅱ可明显抑制VEGF诱导的血管高通透性（P＜0．001）。     

A Homozygous MSH6 Mutation in a Child with Café-au-Lait Spots, Oligodendroglioma and Rectal Cancer

Hereditary nonpolyposis colorectal cancer (HNPCC) is an autosomal dominant condition due to heterozygous germline mutations in DNA mismatch repair genes, in particular MLH1, MSH2 and MSH6. Recently, a syndrome was recognized in which children develop haematological malignancies, solid tumours and signs of neurofibromatosis type 1 due to bi-allelic MMR gene mutations in MLH1, MSH2 and PMS2. Here we describe the child of healthy consanguineous parents who had café-au-lait spots, oligodendroglioma, and rectal cancer. The patient was homozygous for the MSH6 mutation c.3386_3388delGTG in exon 5 which has a predicted pathogenic effect. Germline NF1 gene mutation testing was negative. The rectal tumour showed microsatellite instability and absence of MSH6 staining, whereas the brain tumour was MSI stable and showed normal immunohistochemical expression of MSH6. Apparently, not only MLH1, MSH2 and PMS2, but also MSH6 is involved in the syndrome of childhood cancer and signs of neurofibromatosis type 1.