关键词: Bruch’s membrane Calcification Peau d’orange Pseudoxanthoma elasticum natural history

来  源:   DOI:10.1016/j.xops.2020.100001   PDF(Pubmed)

Abstract:
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.
摘要:
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。
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