非孕期子宫肌瘤红色变性40例分析

目的 了解非孕期子宫肌瘤红色变性的临床特征。方法 本组回顾分析了1993年1月～2001年2月间住院手术治疗40例非孕期子宫肌瘤红色变性的临床病理特征。结果 非孕期子宫肌瘤红色变性发生率为2.3%。生育年龄多见，绝经后也可发生。有典型表现者2例，占5%，非典型下腹隐痛而无发热者7例，占17.5%，肌瘤结节局部压痛者12例，占30%，无任何症状者30例，占75%，白细胞升高占10例，25%。90%的红色变性肌瘤直径在5cm以上，77.5%为壁间肌瘤。68.6%有典型牛肉样改变，54.8%旋涡状结构存在。65%为单纯红色变性，余35%可合并其他类型变性。结论 非孕期子宫肌瘤红色变性临床过程缓和，症状不典型，易漏诊，不作为手术指征，但因子宫肌瘤往往较大，多需手术治疗。     

肝豆状核变性的治疗进展

阐述肝豆状核变性（HLD）在药物、饮食、外科和分子生物学方面的治疗进展，介绍了HLD的药物治疗、外科治疗和分子生物学治疗中的新方法或新技术：以DMPS等为主的药物治疗仍是治疗HLD的主体方法，肝移植等是治疗HLD中的暴发性肝功能衰竭的首选方法，基因等治疗为HLD的彻底治疗提供了可能。     

白内障超声乳化人工晶体术后视网膜脱离临床分析

目的 探讨超声乳化白内障人工晶体术后视网膜脱离的特点及手术疗效。方法 对16例视网膜脱离患者做常规巩膜扣带术或玻璃体切割联合眼内填充进行回顾性分析。结果 视网膜脱离手术后完全复位14例，一次复位率为87．5％，另2例患者经二次手术后完全复位，视力均有不同程度提高。结论 对该类患者术前应仔细检查，选择合适的手术方式尽早施行手术，以提高疗效。     

CT scan and threshold vibrometry in the diagnosis of spinocerebellar degenerations

45 patients with spinocerebellar degeneration (SCD) underwent through quantitative investigation of the ventricular and cisternal systems by CT scanning and threshold vibrometry in the limbs to find out whether these parameters could be used for distinguishing mainly spinal from cerebellar forms or from olivopontoce-rebellar atrophy. The increase in the vibration sense threshold and the mild atrophy in the posterior cranial fossa proved typical of spinal forms and the reverse for cerebellar forms. In olivopontocerebellar atrophy enlargement of the cisterns and dilatation of the ventricles always exceeded 85% of normal values.

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Sommario 45 pazienti affetti da degenerazione spinocerebellare (SCD) sono stati sottoposti ad uno studio approfondito di esplorazione quantitativa del sistema ventricolare e cisternale alla TAC e della soglia di percezione vibratoria agli arti per valutare la possibilità di distinguere con questi parametri le forme prevalentemente spinali da quelle cerebellari o delle atrofie olivopontocerebellari. L'aumento della soglia vibratoria e la modesta atrofia in fossa posteriore è risultata tipica delle forme spinali, mentre l'inverso si verifica per le forme cerebellari. Nelle atrofie olivopontocerebellari l'allargamento delle cisterne e la dilatazione dei ventricoli supera quasi sempre l'85% rispetto ai casi normali.

     

Ocular findings in familial adenomatous polyposis

Background: Retinal pigment epithelium (RPE)lesions are predictive congenital phenotypic markersfor familial adenomatous polyposis (FAP). Thisprospective screening study aims at assessing theincidence and significance of these lesions in FAPpatients and their family members.Methods: Sixty-two members from three familiesincluding five patients with the diagnosis of FAP havebeen ophthalmologically surveyed. All RPE lesions weredocumented with fundus photography and fluoresceinangiography was performed in 13 subjects.Sigmoidoscopy and/or radiological examination wereperformed annually in 9 family members with typicalRPE lesions during 4 years to allow early diagnosis ofFAP.Results: Typical RPE lesions were present infive FAP patients and 15 family members.Telangiectatic dilatations in the retinal peripherywith small dot-like hemorrhages were detected in 6subjects from 3 families These lesions wereparticularly evident on fluorescein angiography.Annual colon analysis showed polyps in 3 out of 9subjects who were positive for RPE lesions.Conclusion: RPE lesions are valuable as aclinical marker in predicting FAP. The co-existingperipheral vascular alterations which have not beenreported before, are probably related to FAP.     

Development of neurochemical separation of ON and OFF channels at retinal ganglion cells

Anatomical and physiological segregation of neurons into ON (brightening detector) and OFF (darkening detector) channels in the retina and subsequent visual system ensure the high sensitivity required for contrast detection and spatial discrimination. This segregation is finest at the visual axis. Neurochemically, ON and OFF ganglion cells at the visual axis seem to be distinguished by different inhibitory transmitters but not excitatory transmitters. Microiontophoretic studies of inhibitory transmitters on the retinal ganglion cells in kittens and adult cats suggest that this neurochemical distinction is poor in immature ganglion cells at the visual axis. Initially both ON and OFF cells seem to be supplied by GABAergic, glycinergic, and catecholaminergic amacrine cells, but in adults, ON cells remain supplied only by GABAergic amacrines, while OFF cells are supplied by glycinergic amacrines. Postnatal elimination of multiple inputs and strengthening of the appropriate inputs, as seen in the central nervous system, also seem to occur at the retinal neurotransmitter synapses during development.     

Matrix Metalloproteinase-2 and Tissue Inhibitor of Metalloproteinase-1 in the Retinal Pigment Epithelium and Interphotoreceptor Matrix: Vectorial Secretion and Regulation

Matrix metalloproteinases (MMPs) and the tissue inhibitors of metalloproteinases (TIMPs) play an essential role in both normal and pathological extracellular matrix degradation, and a TIMP has been associated with at least one type of retinal degeneration. We have studied expression of MMP-2 and TIMP-1 by zymography, immunocytochemistry, and immunoblotting in the retinal pigment epithelium (RPE) from normal, aged and diseased retinas. MMPs and TIMPs were found in the rat RPE, interphotoreceptor matrix (IPM), and in media conditioned by human and rat RPE in culture. In other polarized cells, MMPs and TIMP-2 are secreted vectorially towards the basal lamina. In the RPE, however, MMP-2 and TIMP-1 were secreted preferentially from the apical surface, the surface bordering the IPM. These findings provide new evidence that MMPs and TIMPs could play a role in the turnover of IPM components.Cell homogenates and conditioned media from RPE isolated from mutant Royal College of Surgeons (RCS) rats with inherited retinal dystrophy had similar amounts of MMP-2 and TIMP-1 as those from congenic control rats. The secretion of MMP-2 and TIMP-1 from RPE cell cultures isolated from young and aged human donors varied widely. However, with increasing cell passage number, secretion of MMPs and TIMPs from human RPE increased dramatically. Also, growing human RPE on bovine corneal endothelial cell-generated extracellular matrix instead of plastic reduced the secretion of both MMPs and TIMPs. These data suggest that the integrity of Bruch's membrane may serve to regulate RPE functions in MMP and TIMP secretion and that extracellular matrices contain signals that regulate MMP and TIMP synthesis and/or secretion by the RPE.     

Histological comparison of patellar cartilage degeneration between chondromalacia in youth and osteoarthritis in aging

The histological findings of the patellar cartilage were compared between cases of chondromalacia, which occurs predominantly in young persons (22 patients, average age 19.8 years) and cases of osteoarthritis, which is common among the elderly (21 patients, average age 65.4 years). The histological findings of cartilage in the chondromalacia were characterized by increased density and vigorous fibrous metaplasia of chondrocytes. These findings may be considered to represent a reactive change in the chondrocyte. Cartilage degeneration in osteoarthritis, by contrast, is regressive and presents a clearly different histological picture from that of chondromalacia patellae. We conclude that chondromalacia does not easily lead to osteoarthritis. On the other hand, the cartilage was characteristically softened, as observed by gross inspection, and showed rarefaction of the cartilage matrix. It should be noted that the change was not observed in aging, but showed a pattern of cartilage degeneration peculiar to young patients with chondromalacia patellae.     

干性型老年黄斑变性60例随访观察

探刊干性型老年黄斑变性（age-related macular deganeration，AMD)的病变发展特征并 寻找监测AMD病情进展的较敏感指标。 方法：用眼底彩色照相和眼底荧光血管造影(FFA)、明视视网膜电囤(ERG)、FM100-hue 色彩试验及视力检查，对60例(111眼)50~80岁、视力≤1.0的干性型AMD患者进行3～74个月(平均30.2个月)的随访观察。 结果：本组68眼经FFA检查、平均25.6个月随访，其中25%的患眼病灶损害有快慢不一的发展．全部111眼视力在平均30.2个月随访中有18%患眼的视力下降2行以下，且与病灶进展相符，FM100-hue 色彩试验的被随访者中，随访2年以后，其总错误分明显的增加(P<0.01)．63眼的明视ERG在随访过程中无明显变化。 结论：大部分干性AMD患眼的病灶损害和视力可以在较长时间内维持稳定。FM100—hue的总错误分可能为监测AMD病情进展的较敏感指标． （中华眼底病杂志，1997，13：150-152）     

Effects of intravenous L-acetylcarnitine on retinal oscillatory potentials

L-acetylcarnitine is a compound with cholinergic properties and putative action on the visual system and the glucose metabolism. Ten healthy, emmertropic volunteers (age range: 21 to 28 years) were studied. Each subject was administered 5, 10, and 30 mg/kg acute intravenous doses of L-acetylcarnitine and matching placebo. Retinal oscillatory potentials to full-field flash stimulation were recorded before and 30, 60, and 120 min after administration. A systematic reduction of the implicit time of the P2 and N2 oscillatory potential components was observed after administration of the 10 and 30 mg/kg doses: significant changes were not evident at the 5 mg dose or after placebo. The latency reduction was significantly correlated with the postdrug increment of the L-acetylcarnitine plasma concentration. No other systematic modification in latency of amplitude was observed.The results were presented in part at the XXV I.S.C.E.V. Symposium, Sarasota (Florida), April 26–30, 1987.