Retinal disease in the C3 glomerulopathies and the risk of impaired vision

Background: Dense deposit disease and atypical hemolytic uremic syndrome are often caused by Complement Factor H (CFH) mutations. This study describes the retinal abnormalities in dense deposit disease and, for the first time, atypical haemolytic uremic syndrome. It also reviews our understanding of drusen pathogenesis and their relevance for glomerular disease. Methods: Six individuals with dense deposit disease and one with atypical haemolytic uremic syndrome were studied from 2 to 40 years after presentation. Five had renal transplants. All four who had genetic testing had CFH mutations. Individuals underwent ophthalmological review and retinal photography, and in some cases, optical coherence tomography, and further tests of retinal function. Results: All subjects with dense deposit disease had impaired night vision and retinal drusen or whitish-yellow deposits. Retinal atrophy, pigmentation, and hemorrhage were common. In late disease, peripheral vision was restricted, central vision was distorted, and there were scotoma from sub-retinal choroidal neovascular membranes and atypical serous retinopathy. Drusen were present but less prominent in the young person with atypical uremic syndrome due to a heterozygous CFH mutation. Conclusions: Drusen are common in forms of C3 glomerulopathy caused by compound heterozygous or heterozygous CFH mutations. They are useful diagnostically but also impair vision. Drusen have an identical composition to glomerular deposits. They are also identical to the drusen of age-related macular degeneration, and may respond to the same treatments. Individuals with a C3 glomerulopathy should be assessed ophthalmologically at diagnosis, and monitored regularly for vision-threatening complications.     

人皮质骨矿化基质中骨盐框架结构

目的：研究人皮质骨矿化基质中骨盐的框架结构及框架中骨微间隙。方法：应用透射电镜、场发射扫描电镜观察、电脑图像分析及能谱分析，分析无骨病成人长骨、扁骨２００例骨盐分布特征。结果：骨盐框架结构由微柱、微梁、微小梁、弓状梁、致密点、隔板和骨微间隙构成。骨微间隙由洞、内衬和壁组成，洞平均直径为８４．４±７５．６ｎｍ，与骨小管相比有显著差异（Ｐ值＜０．００１），平均密度为１１～１７个／μｍ２，与骨小管之比超过１０：１。骨盐分针形结晶和微颗粒结晶。结论：骨盐框架结构及骨微间隙是骨盐在人皮质骨矿化基质中的存在形式，可能与骨盐吸收、沉着有关。     

Studies on fine structure and location of lipids in quick-freeze replicas of atherosclerotic aorta of WHHL rabbits

Summary The fine structure of intracellular and extracellular lipids in the atherosclerotic aorta of Watanabe-heritable hyperlipidemic (WHHL) rabbits was demonstrated by a quick-freeze etching technique. Many lipid droplets, with and without a membrane, were observed in the foam cells. Membrane-free droplets were observed as onionlike structure with a concentric lamellar structure surrounded by 10 nm filaments. Droplets surrounded by a limited membrane probably correspond to lipid-laden lysosomes.In the extracellular connective tissue space, marked accumulation of lipids with a vesicular structure was seen among collagen fibers. The appearance of these lipids was similar to that of lipids in lysosomes of foam cells.     

Intramembranous fine deposit disease associated with collagen disorders: a new morphological entity?

Summary A distinct, hitherto unknown renal histopathological appearance, consisting of diffuse thickening of the glomerular basement membrane (GBM) with fine intramembranous electron-dense deposits, was observed in the renal biopsies from three patients with collagen diseases. In each case, proteinuria was mild with normal urinary sediment. On light microscopy there were no particular abnormalities but a mild thickening of the glomerular capillary wall. Immunofluorescence studies revealed faint linear or extremely fine granular IgG deposition along the capillary wall. On electron microscopy, the GBM was diffusely thickened with fine intramembranous electron-dense deposits without spike formation. No other deposits were seen in the glomerulus. These histological features resembled those of membranous glomerulonephritis (MGN), although the possibility of the early change of MGN is excluded by specific findings in these cases. Other GBM-thickening diseases such as diabetic glomerulosclerosis were ruled out clinically and histologically. Our cases have a singular renal histopathology which differs from any of the previously established classifications of glomerular lesions. It may be a specific change associated with some type of collagen disease.     

板蓝根醇沉物的药理作用

目的：观察板蓝根醇沉物与板蓝根颗粒剂的差异。方法：对比研究板蓝根醇沉物与板蓝根颗粒剂前浸膏的药理作用。结果：板蓝根醇沉物的热解、抗炎及抗茵作用与同剂量板蓝根颗粒剂前浸膏相同，其靛蓝含量高于板蓝根颗粒剂前浸膏。结论：板蓝根醇沉物有明显的解热、抗炎作用，板蓝根制剂传统制备工艺有待改进。     

Paraaortic dissection in “total mesopancreas excision” and “mesopancreas-first resection” pancreaticoduodenectomies for pancreatic cancer: Useless,optional, or necessary?A systematic review

The mesopancreas does not have well-defined boundaries but is continuous and connected through its components with the paraaortic area. The mesopancreatic resection margin has been indicated as the primary site for R1 resection after PD in pancreatic head cancer and total mesopancreas excision has been proposed to achieve adequate retropancreatic margin clearance and to minimize the likelihood of R1 resection. However, the anatomy of the mesopancreas requires extended dissection of the paraaortic area to maximize posterior clearance. The artery-first surgical approach has been developed to increase local radicality at the mesopancreatic resection margin. During PD, the artery-first approach begins with dissection of the connective tissues around the SMA. However, the concept of the mesopancreas as a boundless structure that includes circumferential tissues around the SMA, SMV, and paraaortic tissue highlights the need to shift from artery-first PD to mesopancreas-first PD to reduce the risk of R1 resection. From this perspective the “artery-first” approach, which allows for the avoidance of R2 resection risk, should be integrated into the “mesopancreas-first” approach to improve the R0 resection rate.In total mesopancreas excision and mesopancreas-first pancreaticoduodenectomies, the inclusion of the paraaortic area and circumferential area around the SMA in the resection field is necessary to control the tumour spread along the mesopancreatic resection margin rather than to control or stage the spread in the nodal basin.     

Complement inhibition in C3 glomerulopathy

C3 glomerulopathy (C3G) describes a spectrum of glomerular diseases defined by shared renal biopsy pathology: a predominance of C3 deposition on immunofluorescence with electron microscopy permitting disease sub-classification. Complement dysregulation underlies the observed pathology, a causal relationship that is supported by well described studies of genetic and acquired drivers of disease. In this article, we provide an overview of the features of C3G, including a discussion of disease definition and a review of the causal role of complement. We discuss molecular markers of disease and how biomarkers are informing our evolving understanding of underlying pathology. Research advances are laying the foundation for complement inhibition as a targeted approach to treatment of C3G.     

Photoablation of meningiomas and neurinomas by Holmium Yag laser radiation

Abstract

Cranial rat bone was irradiated by 2.1 pm Holmium Yag laser radiation. Quantitative edge rates were calculated. Histologic sections were investigated by light and electron microscopy. Eighteen cases of hard fibrous or calcified spinal and cranial meningiomas and neurinomas were operated upon using pulsed laser beam, in rat cranial bone ablation rate ranged between 0.3-0.5 mm per pulse. Perifocal thermal damage was observed in a zone of20-90 pm around the lesion. In all human cases tumors could be removed totally without additional neurological deficit. In vivo heat development was measured by an i.r.-camera. [Neurol Res 1999; 21: 96–98]     

Acute lymphocytic leukemia presenting as a single brain mass

Hematologic malignancies most commonly spread to the central nervous system via leptomeningeal infiltration. We present a unique case of a woman who presented with a right parietal mass as the initial manifestation of B cell acute lymphocytic leukemia. Because the diagnosis was unclear at the time of presentation she underwent surgical debulking of the mass prior to treatment with chemotherapy. Unfortunately, she relapsed several months after treatment and ultimately entered hospice care. We review the literature surrounding management considerations in patients with intracranial leukemic involvement.