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.     

出血性玻璃体混浊行玻璃体切除术后假性前房积脓分析

目的介绍一种发生于玻璃体切除术后的非感染性前房积脓现象，即假性前房积脓。方法收集我中心收治的需行玻璃体切除术的连续病例１２５０例，其中各种原因引起的玻璃体积血４１８例。术后发生假性前房积脓者７例，均为玻璃体积血者。主要治疗方法是前房冲洗及其自然吸收。结果假性前房积脓发生于术后３～５天，呈泥沙样沉积，局部抗生素及激素加强治疗无效。眼内穿刺行涂片、细菌和真菌培养未发现病原体。患者无疼痛等自觉症状及刺激征。随访时３例视力在０．０５以上。结论玻璃体积血行玻璃体切除术后可能出现假性前房积脓，须与眼内感染相鉴别。     

Open surgery of giant paraclinoid aneurysms improved by intraoperative angiography and endovascular retrograde suction decompression

Summary In three consecutive cases of giant left sided paraclinoid aneurysms we employed an endovascular retrograde suction decompression technique in combination with intra-operative angiography. A double-lumen balloon catheter was placed in the left internal carotid artery by the transfemoral route. After balloon inflation and placement of a temporary clip distal to the aneurysm blood was aspirated and the aneurysm collapsed. Thus further dissection of the aneurysm could easily be achieved and clips could be placed. Afterwards real-time digital subtraction angiography was performed. Intra-operative angiography led to clip repositioning in all cases either due to a clip induced stenosis of the parent vessel, or because of incomplete aneurysm obliteration. Afterwards successful clipping could be confirmed in all cases. Outcome was excellent in one case, good in the other. The third case, extremely complicated by an accompanying craniopharyngioma, showed a satisfactory outcome, but presented new neurological deficits.     

增生性玻璃体视网膜病变基质金属蛋白酶的定量研究

目的:研究增生性玻璃体视网膜病变(proliferative vitreoretinopathy,PVR)玻璃体中基质金属蛋白酶(matrix metalloproteinases,MMPs)的表达,探讨MMPs在PVR病理过程中的作用。方法:PVR患者采用标准三切口巩膜扁平部玻璃体切割术(pars plana vitrectomy,PPV),取未稀释的玻璃体21只眼,PPV术后复发的玻璃体腔液20只眼,意外死亡的正常人玻璃体10只眼,采用明胶酶谱分析法定量分析MMP-2和MMP-9活性水平。结果:PVR玻璃体有MMP-2活性水平增高,与正常玻璃体比较差异有显著性意义(P<0.05)。21眼PVR玻璃体中13只眼有MMP-9活性水平增高,平均(171.52±13.17)扫描单位。20眼PPV术后PVR复发的玻璃体腔液19只眼有MMP-9活性水平增高,平均(156.01±37.21)扫描单位。正常人玻璃体无MMP-9的表达。结论:PVR玻璃体有MMP-2和MMP-9活性水平增高,MMP-9活性水平增高可能与术后PVR复发有关。眼科学报2003;19:130-132。     

皮肤发疱仪的研制与临床应用

目的：研制皮肤发疱仪，并临床应用于自体表皮移植治疗白癜风，分析治疗效果。方法：根据局部皮肤双控发疱理论，应用负反馈原理，研制出皮肤双控发疱仪；该仪器应用于自体表皮移植治疗白癜风，通过控制局部发疱部位的压力和温度，实现发疱时需要的恒压和恒温。结果：皮肤发疱仪能恒定控制局部皮肤发疱时的压力和温度，使自体表皮移植治疗白癜风的总成活率达到98．01％。结论：皮肤发疱仪性能稳定，操作方便，临床应用效果好，值得推广。     

Endotracheal suctioning causes right upper lobe collapse in intubated children

Objective: Right upper lobe collapse is a common radiographic finding in intubated children. We hypothesized that deep suctioning and uncontrolled negative pressures during endotracheal tube suctioning were significant contributory factors. Methods : The incidence of right upper lobe (RUL) collapse in intubated, ventilated children on a paediatric cardiac intensive care unit was determined over a 3-month period ( n = 102). Graduated suction catheters and suction vacuums of < 165 cm H₂O were then introduced. Another prospective audit was carried out 3 months later ( n = 60). Results : We found that 24% developed RUL collapse and 4 developed an apical pneumothorax. Following the introduction of graduated catheters and controlled vacuums pressures, a significant reduction in the incidence of RUL collapse, to 7%, was observed ( p < 0.05). Conclusions : We conclude that high negative pressure and deep-suctioning causes RUL collapse in children. Any lobar collapse not only prolongs the child's stay in intensive care, but can be associated with further morbidity which may have a serious implication. By improving suctioning technique this morbidity can be significantly reduced.     

A randomized, double blind, placebo-controlled study to investigate the use of conscious sedation in conjunction with paracervical block for reducing pain in termination of first trimester pregnancy by suction evacuation

BACKGROUND: This study evaluated the role of conscious sedation in pain relief during termination of first trimester pregnancy by suction evacuation (SE) under local anaesthesia. METHODS: A hundred women undergoing SE before 12 weeks gestation were randomized by computer using the sealed envelope method to receive placebo (saline) or conscious sedation (2 mg midazolam and 25 microg fentanyl) i.v. 5 min before cervical dilatation. Paracervical block was given to all patients, 2 min later. Pain scores during and after SE, post-operative side-effects and satisfaction level were compared. RESULTS: No statistically significant differences in pain scores were found between the two groups. Post-operative side-effects such as dizziness (P = 0.015) and drowsiness (P < 0.001) were significantly more severe in the conscious sedation group. However, patients in the conscious sedation group reported better satisfaction levels than the control group (P = 0.003). CONCLUSION: The use of conscious sedation significantly improved patient satisfaction during termination of first trimester pregnancy by SE under local anaesthesia, despite a lack of improvement in pain relief and the presence of increased severe dizziness/drowsiness in the post-operative period.     

Low resting potentials in single isolated heart cells due to membrane damage by the recording microelectrode

Single myocytes from adult rat hearts were prepared following the method of Powell and co-workers (9, 10, 11). Low resting potentials (E_m) could be improved by three techniques. (i) Elevation of Ca_o to 7.2 mM which, however, mostly resulted in spontaneity and irreversible contracture. (ii) Pre-incubation in a KB medium (6). (iii) Use of suction pipettes instead of tapered microelectrodes for intracellular recordings (2). It is concluded that low E_m measured previously (11) were due to membrane damage upon microelectrode impalement accopanied by insfufficient healing of the membrane around the electrode insertion.     

兔视网膜细胞问水肿及玻璃体收缩性状改变的相关研究

目的探讨玻璃体收缩与视网膜细胞问水肿的相关性。方法色素性兔17只17只眼，激光光凝诱发视网膜静脉阻塞(RVO)后1个月，视网膜血管分布区域作病理观察，对出现视网膜细胞间水肿者，测定其玻璃体内凝胶比重及重力下伸展度的改变，并通过II型胶原蛋白免疫印迹分析其相关的分子学机制。结果RVO后1个月，13只实验眼视网膜出现细胞问水肿，占76．5％；玻璃体出现凝缩、液化，收缩性增强；玻璃体内II型胶原的a单链相互交连成大分子的p和7重链结构，破坏了胶原支架的稳定性。结论视网膜细胞间水肿与玻璃体收缩有相关性，玻璃体胶原的相互交连并导致支架稳定性的破坏是其主要原因。 （中华眼底病杂志，2004，20：29-32）     

PVR B 级的裂孔性视网膜脱离术后复发与玻璃体病变关系临床分析

目的回顾分析近10年PVR B级的裂孔性视网膜脱离术后复发与玻璃体病变的关系.方法对429例(435只眼)PVR B级的裂孔性视网膜脱离施行巩膜扣带术治疗,其中41只眼术后复发(9.43%),分析术后复发与玻璃体病变的关系.结果术前玻璃体正常组与浓缩组的复发率(3.19%与12.33%)之间差异有显著性(P＜0.05);正常组与条索牵引组间的复发率(3.19%与14.29%)之间差异有极显著性(P＜0.01);正常组与总体组的复发率(3.19%与9.43%)之间差异有显著性(P＜0.05);术前无玻璃体后脱离组与玻璃体后脱离组间的复发率(5.56%与12.90%)之间差异有显著性(P＜0.05).视网膜脱离术前各种玻璃体的情况在视网膜脱离复发时均有不同程度地加重.结论PVR B级的裂孔性视网膜脱离术前玻璃体病变和玻璃体后脱离对术后复发影响明显.重视玻璃体-视网膜界面动态变化,可进一步提高手术治愈率.