peau d’orange

  • 文章类型: Journal Article
    目的:血管样条纹(AS)是一种罕见的视网膜疾病,当并发脉络膜新生血管(CNV)时,视力受损。它们代表布鲁赫膜水平的裂纹状开裂。这篇客观的叙事综述旨在提供病理生理学的概述,目前的治疗方式,以及在这种情况下的未来前景。材料与方法:使用“PubMed”进行文献检索,\"WebofScience\",\"Scopus\",“科学直接”,“谷歌学者”,\"medRxiv\",和“bioRxiv。“结果:AS可能是特发性的,但它们也与系统条件有关,比如弹性假性黄瘤,遗传性血红蛋白病,或者Paget的病.目前,主要治疗方法是使用抗血管内皮生长因子(anti-VEGF)治疗继发性CNV,这是在这种情况下观察到的主要并发症。如果CNV被及时检测和治疗,ASs患者有很好的机会维持功能性视力。其他治疗方式已经尝试,但显示出有限的益处,因此,没有被更广泛地接受。结论:总之,虽然目前还没有明确的治疗方法,抗VEGF治疗继发性CNV为维持AS患者的功能性视力提供了机会,前提是早期检测和治疗CNV。
    Aim: Angioid streaks (ASs) are a rare retinal condition and compromise visual acuity when complicated with choroidal neovascularization (CNV). They represent crack-like dehiscences at the level of the Bruch\'s membrane. This objective narrative review aims to provide an overview of pathophysiology, current treatment modalities, and future perspectives on this condition. Materials and Methods: A literature search was performed using \"PubMed\", \"Web of Science\", \"Scopus\", \"ScienceDirect\", \"Google Scholar\", \"medRxiv\", and \"bioRxiv.\" Results: ASs may be idiopathic, but they are also associated with systemic conditions, such as pseudoxanthoma elasticum, hereditary hemoglobinopathies, or Paget\'s disease. Currently, the main treatment is the use of anti-vascular endothelial growth factors (anti-VEGF) to treat secondary CNV, which is the major complication observed in this condition. If CNV is detected and treated promptly, patients with ASs have a good chance of maintaining functional vision. Other treatment modalities have been tried but have shown limited benefit and, therefore, have not managed to be more widely accepted. Conclusion: In summary, although there is no definitive cure yet, the use of anti-VEGF treatment for secondary CNV has provided the opportunity to maintain functional vision in individuals with AS, provided that CNV is detected and treated early.
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  • 文章类型: Journal Article
    描述弹性假性黄瘤(PXE)患者布鲁赫膜(BM)钙化的自然史。
    回顾性队列研究。
    120名年龄小于50岁的PXE患者的双眼,其中78人在超过1年后进行了随访成像。
    所有患者都接受了多模态成像,包括彩色眼底摄影,近红外反射(NIR)成像,和后期吲哚菁绿血管造影(ICGA)。我们在NIR上确定了从视盘到橙色中心和时间边界的距离,以水平视神经盘直径(ODD)表示。最长血管样条纹的长度分为5个区域。
    橙色的特定年龄变化,血管样条纹,和ICGA荧光不足作为BM钙化程度的替代标志物。
    在横截面分析中,血管样条纹较长与年龄增长相关(趋势P<0.001).橙色的时间边界显示出与年龄增长的弱关联(β=0.02;95%置信区间[CI],0.00-0.04),而中央边界显示出强关联(β=0.12;95%CI,0.09-0.15)。纵向分析显示,中心边界向外围的中值位移为每年0.08ODD(四分位间距[IQR],0.00-0.17;P<0.001)。这种转变在20岁以下的患者中更为明显(每年0.12ODD[IQR,0.08-0.28])比40岁以上的患者(每年0.07ODD[IQR,-0.05至0.15])。时间边界在随访期间没有移位(P=0.69)。在156只眼中的39只(25%)中检测到新的或正在生长的血管样条纹。ICGA上的低荧光区域仅在第四个或第五个十年中可见,并且与更长的血管样条纹相关。
    在PXE患者中,斑点的BM钙化在一生中缓慢融合。橙色的时间边界的位置保持相当恒定,而中央边界转移到外围。这表明存在用于BM钙化的预定区域。较大的ICGA低荧光区域与年龄较大和较长的血管样条纹相关,这意味着它取决于BM钙化的程度。
    UNASSIGNED: To describe the natural history of Bruch\'s membrane (BM) calcification in patients with pseudoxanthoma elasticum (PXE).
    UNASSIGNED: Retrospective cohort study.
    UNASSIGNED: Both eyes of 120 PXE patients younger than 50 years, 78 of whom had follow-up imaging after more than 1 year.
    UNASSIGNED: All patients underwent multimodal imaging, including color fundus photography, near-infrared reflectance (NIR) imaging, and late phase indocyanine green angiography (ICGA). We determined the distance from the optic disc to the central and temporal border of peau d\'orange on NIR, expressed in horizontal optic disc diameter (ODD). The length of the longest angioid streak was classified into 5 zones.
    UNASSIGNED: Age-specific changes of peau d\'orange, angioid streaks, and ICGA hypofluorescence as surrogate markers for the extent of BM calcification.
    UNASSIGNED: In cross-sectional analysis, longer angioid streaks were associated with increasing age (P < 0.001 for trend). The temporal border of peau d\'orange showed a weak association with increasing age (β = 0.02; 95% confidence interval [CI], 0.00-0.04), whereas the central border showed a strong association (β = 0.12; 95% CI, 0.09-0.15). Longitudinal analysis revealed a median shift of the central border to the periphery of 0.08 ODD per year (interquartile range [IQR], 0.00-0.17; P < 0.001). This shift was more pronounced in patients younger than 20 years (0.12 ODD per year [IQR, 0.08-0.28]) than in patients older than 40 years (0.07 ODD per year [IQR, -0.05 to 0.15]). The temporal border did not shift during follow-up (P = 0.69). New or growing angioid streaks were detected in 39 of 156 eyes (25%). The hypofluorescent area on ICGA was visible only in the fourth or fifth decade and correlated with longer angioid streaks.
    UNASSIGNED: In PXE patients, the speckled BM calcification slowly confluences during life. The location of the temporal border of peau d\'orange remains rather constant, whereas the central border shifts to the periphery. This suggests the presence of a predetermined area for BM calcification. A larger ICGA hypofluorescent area correlates with older age and longer angioid streaks, which implies that it depends on the degree of BM calcification.
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  • 文章类型: Journal Article
    Purpose: To evaluate the fundus phenotype of young patients affected with Pseudoxantoma Elasticum (PXE). Materials and Methods: Retrospective case series of five young PXE patients. Clinical data, ultra-widefield imaging (color, red-free (RF), choroidal (Ch) and fundus autofluorescence (FAF)) and OCT examination were collected. Diagnosis was confirmed by the characteristic histopathological abnormalities in skin biopsies and genetic testing results. Results: Five patients, 2 males and 3 females (mean age 16 years, range 12-20 years) were included in our study. The visual acuity was 20/20 in all subjects. Fundus evaluation revealed peau d\'orange in all patients: multiple, yellowish/white round lesions, scattered from the posterior pole to the mid-peripheral retina of each eye. Ultra-wide field imaging allows us to capture and describe the entire area of coquille d\'oeuf/peau d\'orange in a single picture, facilitating their identification and discrimination. Angiod streaks were visible in both eyes of four patients. In one patient optic disc drusen were detected in both eyes. All patients presented comet lesions. Conclusions: PXE-related retinopathy findings: peau d\'orange/coquille d\'oeuf, angioid streaks, comet lesions and drusen of the optic disc were present early in PXE patients. The early detection of coquille d\'oeuf/peau d\'orange revealed a preferable area into midperiphery where Bruch\'s membrane will be more likely to be affected.
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  • 文章类型: Journal Article
    我们回顾了2009年至2015年的200例患者记录,这些患者在作者办公室进行面部皱纹减少以及进行身体检查时患有皱纹。peaud\'橙色,或薄的真皮,并接受了新的脂肪浆注射。在第2、1、3和6个月对患者进行了评估,发现患者的皱纹和真皮密度改善了80%,60%和40%,分别。我们提出了由自体脂肪组成的制造填充物的合理替代或辅助方案和描述,干细胞,和生长因子浆料。在办公室设置的床边容易产生和注入浆液,在肤色方面有显著和可重复的积极结果,颜色,纹理,和由患者和医生判断的韵律。
    方法:本期刊要求作者为每篇文章分配一定程度的证据。对于这些循证医学评级的完整描述,请参阅目录或在线作者说明www。springer.com/00266.
    We reviewed 200 patient records from 2009 to 2015 of individuals who presented at the authors offices for facial wrinkle reduction and who on physical examination had rhytides, peau d\'orange, or thin dermis and underwent novel adipose slurry injections. The patients were evaluated at week 2, 1, 3 and 6 months revealing an 80, 60 and 40% improvement in rhytides and dermis density, respectively. We present the protocol and description of a reasonable alternative or adjunct to manufactured fillers that is comprised of autologous adipose, stem cells, and growth factor slurry. The slurry is easily produced and injected at the bedside in the office setting, and has remarkable and reproducible positive outcomes in skin tone, color, texture, and rhytides as judged by both patient and physician.
    METHODS: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .
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