Early posterior vitreous detachment is associated with LAMA5 dominant mutation

Background: Extracellular matrix molecular components, previously linked to multisystem syndromes include collagens, fibrillins and laminins. Recently, we described a novel multisystem syndrome caused by the c.9418G>A p.(V3140M) mutation in the laminin alpha-5 (LAMA5) gene, which affects connective tissues of all organs and apparatus in a three generation family. In the same family, we have also reported a myopic trait, which, however, was linked to the Prolyl 4-hydroxylase subunit alpha-2 (P4HA2) gene. Results of investigation on vitreous changes and their pathogenesis are reported in the present study.Materials and Methods: Nineteen family individuals underwent complete ophthalmic examination including best-corrected visual acuity (BCVA), fundus examination, fundus photography, intraocular pressure measurement, axial length measurement using ocular biometry, Goldmann visual field examination, standard electroretinogram, SD-OCT. Segregation analysis of LAMA5 and P4HA2 mutations was performed in enrolled members.Results: The vitreous alterations fully segregated with LAMA5 mutation in both young and adult family members. Slight reduction of retinal thickness and peripheral retinal degeneration in only two patients were reported.Conclusions: In this work we showed that PVD is a common trait of LAMA5 multisystem syndrome, therefore occurring as an age-unrelated trait. We hypothesize that the p.(V3140M) mutation results in a reduction of retinal inner limiting membrane (ILM) stability, leading to a derangement in the macromolecular structure of the vitreous gel, and PVD. Further investigations will be necessary to elucidate the role of wild type and mutated LAMA5 in the pathogenesis of PVD.     

复方壳多糖组织工程皮肤的组织学特性

目的评价制备的复方壳多糖组织工程皮肤在组织学方面的特性，为其修复皮肤缺损创面提供实验依据。方法通过苏木精-伊红（HE）染色、高碘酸-希夫（PAS）染色、免疫组织化学染色及电镜，观察组织工程皮肤的组织学特征，包括表皮、真皮及两者的连接结构基底膜。结果制备的复方壳多糖组织工程皮肤表皮细胞增殖活跃，分层分化良好，厚约150μm，真皮层细胞生长正常，排列有序。PAS染色示真表皮间有均匀红染的条带出现，免疫组织化学染色结果显示Ⅳ型胶原、Ⅶ型胶原、层黏蛋白反应阳性，在电镜下可见角质形成细胞间大量桥粒，基底层内侧较多半桥粒，与机体皮肤结构相似。结论复方壳多糖组织工程皮肤组织结构良好，符合新型皮肤替代物在治疗皮肤缺损时的组织学要求。     

Expression of epithelial matrix molecules collagen and laminin and corresponding integrins in chronic wounds

Re-epithelialization of cutaneous wounds is a coordinated process of proliferation and migration of keratinocytes at the wound edge. The study objective was to identify the differences in epidermal morphology, keratinocyte proliferation and matrix molecules (laminin 1, laminin 5, type IV collagen) and their specific integrin (α3, α6) expression in biopsies of meshed split thickness grafted and chronic wounds. The mean mitotic index of keratinocytes (ratio of cell cycle associated antigen Ki-67 expressing keratinocytes to basal keratinocytes) was highest in chronic wounds (38.7%) compared to acute wounds (22.25%, range 5.7% to 54%). The mean thickness of the hyper-proliferative epithelium at the wound edge of chronic wounds was 0.69 mm compared to 0.15 mm at the wound margin of split thickness grafted wounds. Both chronic wounds and skin grafted wounds exhibited strong laminin 5 immunoreactivity at the basal side of the epithelium, which extended under the most forward keratinocytes. Laminin 1 and type IV collagen immunoreactivity did not extend to the wound margin in either skin grafted or chronic wounds. In both transplanted skin and chronic wounds, the integrin sub-units α3 and α6 exhibited a strong pericellular immunoreactivity on the leading keratinocytes of the wound margin. Our data demonstrates that the proliferation of keratinocytes and the expression of associated integrins are not impaired in chronic wounds. Presented at the 33rd Congress of the Association of German Plastic Surgeons, Germany, 18–21 September, 2002.     

汉族、哈萨克族Graves病患者血清CoL IV、LN和HA测定分析

用放免法(单克隆抗体)测定汉、哈萨克族Graves病未经治疗患者66例及正常对照组55例血清中Ⅳ型胶原(CoLⅣ)、板层素(IN)和透明质酸(HA),结果Graves病患者血清CoLⅣ、LN浓度明显升高,HA浓度明显降低,与正常对照组相比均有显著性差异(分别P<0.001、P<0.05、P<0.05),汉族与哈萨克族患者之间、对照组之间相比均无显著性差异(P>0.05),它反映了Graves病患者体内基底膜合成和分解代谢紊乱。     

The development of glial fibrillary acidic protein-positive cells and the appearance of laminin-like immunoreactivity in fetal olfactory bulb transplants

Embryonic rat olfactory bulbs were transplanted into the site vacated by aspiration of an olfactory bulb from a neonatal rat. This paper presents our findings related to the development of glial fibrillary acidic protein (GFAP) positive (+ve) glial cells and the appearance of laminin-like immunoreactivity in these transplants. The GFAP + ve glial cells formed perivascular end-feet on the invading vasculature and formed a glia limitans along the glia surface of the transplant. This reconstituted glia limitans was continuous with that of the host brain, there being no glia limitans at the donor-host interface. Thus, the donor tissue was well-integrated with that of the host brain.     

应用人自体血清培养人口腔黏膜上皮的实验研究

目的 研究人自体血清培养人口腔黏膜移植生长的生物学特性，为组织工程化尿道提供新材料。方法 将人自体血清培养黏膜移植于裸鼠体内，分别于移植后2、3、4、6周观察培养黏膜生长与转归，应用anti—HLA免疫荧光鉴定成活黏膜组织属性，应用抗人Ⅳ型胶原及抗人层黏蛋白为基底膜形成指标。结果 裸鼠体内移植培养黏膜成活生长分化良好，anti—HLA免疫荧光证实为移植的培养人黏膜组织；免疫组化发现移植后3周开始形成基底膜，4周形成完整的基底膜。结论 自体血清培养的人口腔黏膜可形成功能完整的上皮组织。     

人T淋巴母细胞（Jurkat）移动性的研究

利用人Ｔ淋巴母细胞（Ｊｕｒｋａｔ）细胞系作为研究对象。企图探探索细胞外基质成份及肌醇磷脂细胞信息传递系统与细胞移动的关系。研究结果表明：当细胞被伏波醇酯（ＰＭＡ）处理过后，其粘连性与移动件对纤维连结蛋白（ＦＮ）底物的反应在已是高水平的基础上有更进一步提高的作用。与ＦＮ刊相反的是，未经ＰＭＡ处理处的Ｊｕｒｋａｔ细胞对层粘连蛋白（Ｌｍ）底物却无明显的粘连性及移动性增加的反应，尽管在ＰＭＡ处理过４小时内亦无任何的增强。但是，当ＰＭＡ作用于细胞２４一４８小时后则明显池增加其Ｌｍ底物的粘连性及移动性反应，表明了其明显的协同作用。分别用抗ＦＮ、抗Ｌｍ及蛋白激酶Ｃ抑制剂Ｓｔａｕｒｏｓｐｒｉｎｅ（ＳＰ）时均起到特异性的抑制作用。可见，协同作用的产生是通过蛋白激酶Ｃ激活后的－段时间内，使细胞表面Ｌｍ受体密度增加而对底物Ｌｍ反应增强所致。     

肺癌细胞外基质的表达与临床病理的关系

目的探讨原发性肺癌层粘蛋白（Ｌａｍｉｎｉｎ,ＬＮ）、纤维连接蛋白（ｆｉｂｒｏｎｅｃｔｉｎ,ＦＮ）和Ⅳ型胶原的表达与临床病理的关系。方法采用免疫组化ＬＳＡＢ法检测１８４例肺癌标本中ＬＮ、ＦＮ和Ⅳ型胶原的表达。结果ＬＮ及Ⅳ型胶原在中、高分化的肺鳞癌中的表达均明显强于低分化组（Ｐ＜００１）,在有无淋巴结转移两组间的表达差异均具显著性（Ｐ＜００５）;肺鳞癌５年以上生存组ＬＮ、Ⅳ型胶原的表达明显高于０５年内死亡组（Ｐ＜００１）,ＦＮ的表达亦有差异（Ｐ＜００５）。肺腺癌的５年以上生存组中ＦＮ、Ⅳ型胶原的表达均强于０５年内死亡组（Ｐ＜００５,００１）。结论ＬＮ和Ⅳ型胶原的表达与肺鳞癌的组织学分级和淋巴结转移有关;ＬＮ、ＦＮ、Ⅳ型胶原的表达均可作为评估肺鳞癌预后的指标,而腺癌的预后仅与ＦＮ、Ⅳ型胶原的表达有关。     

缺血性股骨头坏死患者血清透明质酸及层粘连蛋白的研究

为探讨细胞外间质主要成分透明质酸（ＨＡ）与层粘连蛋白（ＬＮ）在缺血性股骨头坏死（ＩＮＦＨ）中的生理病理过程及临床价值，采用放射免疫分析法对４５例不同病因的ＩＮＦＨ患者进行了血清ＨＡ与ＬＮ的定量分析，并与３０例正常人对照。结果显示，ＩＮＦＨ患者血清ＨＡ含量极显著地高于对照组（ｔ＝３－２９；Ｐ＜０－０１）。尤以激素性ＩＮＦＮ增高最为显著（ｔ＝３－６２；Ｐ＜０－０１）。ＩＮＦＨ患者ＬＮ含量亦明显高于对照组（ｔ＝２－８４；Ｐ＜０－０１）。同时，ＨＡ与ＬＮ含量增高与病程发展密切相关。故定量检测ＨＡ与ＬＮ可作为ＩＮＦＨ早期诊断及判断预后的良好指标     

Immunohistochemical differentiation between lymphangiographically verified lymphatic vessels and blood vessels

Summary In order to investigate the antigen profile in human lymphatic vessels when compared with blood vessels, postmortem retrograde lymphangiography was done via the thoracic duct on six patients. Formalin fixed, paraffin embedded tissue was stained immunohistochemically for Factor VIII-Related antigen (F VIII R:Ag), with Ulex Europaeus 1 lectin (UEA-1) and for laminin. The results show that the endothelium of blood vessels and lymphatics at all levels of the lymphatic system react positively following staining for Factor VIII-R:Ag and with UEA-1 lectin. The staining for F VIII R:Ag was generally weaker in the endothelial cells lining lymphatic vessels. Staining for the basement membrane component laminin can be used to distinguish lymphatic capillaries and smaller lymphatic collecting vessels from blood vessels.