面部肉芽肿：66例患者的临床病理学研究

背景：以往对面部肉芽肿（GF）的研究仅局限于病例报道或小样本研究。目标：通过对GF的大样本研究，描述其临床病理特征。方法：对66例患者和73份皮肤组织标本进行回顾性分析。结果：GF通常发生在中年人，表现为淡红色斑块或结节，1／3患者有多个皮损，5例患者有面部以外皮损。只有10例患者临床诊断为GF；其主要鉴别诊断包括：肉样瘤病、淋巴瘤、狼疮及基底细胞癌。GF最常见的组织病理学特征为一条grenz带、中性粒细胞浸润及毛细血管扩张。尽管坏死性血管炎罕见，但血管改变常见。通常皮损同时存在急性和慢性炎症，提示GF为急性复发的慢性过程，而不是一个明显连续的急性和慢性阶段。     

肾上腺脑白质营养不良

肾上腺脑白质营养不良(ALD)是由于饱和极长链脂肪酸(VLCFAs)代谢障碍，使VLCFAs积聚于大脑白质及肾上腺皮质等处，导致一系列进展性神经精神症状和肾上腺皮质功能不全。本病为x连锁隐性遗传，目前已发现数百种ALD基因突变类型。与临床表现型无关，临床呈多样化表现，以神经系统症状为主，部分仅表现为单纯肾上腺皮质功能不全。在原发性肾上腺皮质功能不全的男性患者中，约占13％。本文介绍其病因学及临床特征、诊断学和治疗进展。     

321例特发性血小板减少性紫癜患者的临床特征及治疗反应的回顾性评估

特发性血小板减少性紫癜(ITP)是一种自身免疫性疾病,其治疗手段有多种,本文回顾性评估了321例ITP患者的初次临床特征、对治疗的反应及临床过程。     

踝关节软组织撞击综合征临床研究

目的:总结与探讨踝关节软组织撞击综合征发生的相关因素、关节镜下表现和组织病理学特点。方法:自2000年11月至2005年4月,本所共收治21例踝关节撞击综合征患者,其中男性16例,女性5例,平均年龄24·7岁。运动员7例,非运动员14例。左踝9例,右踝12例。对所有患者均进行关节镜探查,镜下切除造成撞击的组织,同时对其进行病理检查。结果:21例患者中有20例发生于踝关节扭伤后,其中16例为内翻伤。单纯前外侧撞击9例,单纯前内侧撞击2例,单纯外侧撞击1例,前内和前外撞击同时存在9例。关节镜下发现撞击组织为滑膜组织的20例,下胫腓前韧带远侧束5例,纤维瘢痕组织4例,距腓前韧带组织3例,半月板样组织3例。合并关节软骨损伤16例。病理检查确认造成撞击的组织为慢性炎症性滑膜组织、韧带组织、肉芽组织。半月板样组织病理表现为慢性滑膜炎或致密结缔组织伴软骨化生,或慢性滑膜炎伴肉芽组织形成。结论:踝关节软组织撞击综合征多发生在创伤后,尤其内翻伤后较常见。部位以踝关节前外侧多见,内侧也可发生。撞击组织以滑膜组织最常见,其次为下胫腓前韧带远侧束增厚、纤维瘢痕组织和损伤的距腓前韧带组织。软组织撞击部位与软骨损伤部位符合程度不显著(χ~2=2·524,P=0·112)。     

功能性消化不良的突出症状与临床特征及病理生理学机制的关系

背景与目的：功能性消化不良（FD）被认为是一类由不同病理生理原因导致的多种机能紊乱而促发的症状。Rome Ⅱ委员会建议将具有同种病理生理学及临床特征的FD患划分为感觉明显疼痛组和感觉不适组两个亚组。该研究旨在分析显疼痛或感觉不适与病理生理学机制间的关系，评价是否考虑到个体的显性症状将会产生更好的结果。方法：持续性FD患（n=720；489例女性；年龄41．3±0．6岁）填写消化不良调查问卷并确定出最令人烦恼的症状。分析此显症状在人口统计学、临床、病理生理学特点上的相互联系（研究592例患幽门螺杆菌感染、胃排空情况，对332例患行胃敏感性、顺应性试验）。结果：根据Rome Ⅱ标准，22％疼痛明显，78％不适感明显。疼痛明显的患超敏性的发生率较高（44％ vs 25％），且在这些病例中观察到的胃排空迟缓较少出现（16％ vs 26％），但是有较多的重叠。详细分析表明可能有8种消化不良症状其中之一较为突出。     

Effect on peripheral nerve vivo with human insulin-like regeneration by transgene in growth factor-1

BACKGROUND: Human insulin-like growth factor (hIGF-1) has been successful in treating peripheral nerve injury, but it is still unclear whether hIGF-1 after transgene in vivo has the effect on promoting the regeneration of peripheral nerve. OBJECTIVE: To observe the effect of hIGF-1 on the regeneration of peripheral nerve by transgene in vivo with electrophysiology, histological morphology and ultromicro morphology. DESIGN: A univariate design. SETTINGS: Jilin Institute of Surgery, China-Japan Friendship Hospital Affiliated to Jilin University; School of Basic Medical Sciences, Jilin University. MATERIALS: Thirty male adult Wistar rats of grade Ⅱ, weighing 200-250 g, were provided by the Animal Experimental Center of Jilin University [certification number: SCXK-(Ji)20030001]. The rats were raised in the environment at the temperature of 25 ℃ and humidity of 70%. All the rats were randomly divided into hIGF-1-treated group, treatment control group and blank control group, 10 rats in each group. Positive liposomes (mass concentration of 2 g/L) and pcDNA3.1 (mass concentration of 1 g/L) were purchased from Beijing Yuanpinghao Company; pcDNAhIGF-1 (mass concentration of 1 g/L) was provided by Dr. Shen from the School of Public Health of Jilin University. The liposomes were mixed with plasmids with the mass ratio of 1.5 to 10.Operative microscope was made by Jiangsu Zhenjiang Microsurgical Instrument Factory; EMB-5304K electromyogram (EMG) evoked potential meter by Nihon Kohden Corporation. HPIAS-1 000 high-acuity color pathological imaging analytical system (Japan) and JEM-1200EX transmission electron microscope (Japan) were also used. METHODS: The experiments were carried out in Jilin Institute of Surgery from April to June in 2004. ① All the rats were anesthetized, and the right sciatic nerve was exposed, and it was clipped with a clip at 5 mm below the piriform muscle for 3 times, 10 s for each time. The pressed width was 3 mm, and formed as membrane under operating microscope (×6). Rats in the hIGF-1-treated group were subepineurially injected with the mixture of pcDNAhIGF-1 and positive liposomes (10 μL) immediately, those in the treatment control group were injected with the mixture of pcDNA3.1, positive liposomes and distilled water (10 μL), and those in the blank control group were not given any injection. ② The sciatic nerve functional indexes (SFI) were measured within 56 days postoperatively according to the methods used by Shen et al. ISFI=0 was taken as normal, and ISFI=-100 as completely damaged. EMG evoked potential meter was used to record the electrophysiological changes of the regenerated nerve fibers. The indexes of histological morphology in 5 randomly selected sights were determined with the color pathological imaging analytical system, and the ultrostructures of the regenerated nerve fibers were also observed. MAIN OUTCOME MEASURES: ① Comparison of the SFI within 56 days postoperatively; ② Comparison of the electrophysiology, histological morphology and ultrastructure of the regenerated nerve fibers 56 days postoperatively. RESULTS: All the 30 Wistar rats were involved in the analysis of results. ① SFI: The SFI values were gradually increased as time prolonged in all the three groups, and the changes were more obvious after 24 days, the SFI values recovered better at each time point in the hIGF-1-treated group than in the other two groups. ② Eelectrophysiological results of right sciatic nerve: The latency of motor evoked potential (MEP) was close between the treatment control group and the blank control group [(2.55±0.36), (2.65±0.55) ms, P > 0.05], but higher in the hIGF-1-treated group [(2.14±0.22) ms] than in the blank control group (P < 0.01). The amplitude and conduction velocity of MEP in the treatment control group [(6.67±0.69) mV, (29.57±4.06) m/s] were close to those in the blank control group [(6.60±0.59) mV, (29.22±3.20) m/s, P > 0.05], but those in the hIGF-1-treated group [(7.81±0.84) mV, (36.91±4.37) m/s] were larger or faster than those in the blank control group (P < 0.01). ③ Results of the pathological image analysis of the regenerated nerve fibers: The axonal diameter, thickness of myelin sheath of the regenerated nerve fiber and the number of myelinated nerve fiber in the treatment control group [(2.28±0.33) μm, (1.08±0.18) μm2, (71.80±8.25) fibers] were close to those in the blank control group [(2.18±0.29) μm, (1.03±0.15) μm2, (68.60±8.55) fibers] (P > 0.05), and those in the hIGF-1-treated group [(3.03±0.35) μm, (1.65±0.24) μm2, (88.20±8.82) fibers] were obviously larger or more than those in the blank control group (P < 0.01). ④ Ultrastructure of the regenerated nerve fibers of sciatic nerve: In the hIGF-1-treated group, the regenerated fibers of sciatic nerve were more and mature, manifested by thicker nerve fibers, thicker and evener myelin sheath, which were better than those in the other two groups. CONCLUSION: The results of the quantitative parameters of the electrophysiology, gross histological morphology and ultrostructural changes in the process of repairing damaged peripheral nerve indicate that transgene in vivo with hIGF-1 can promote the neural regeneration after peripheral nerve injury.