关键词: OCTA early-treated adults with phenylketonuria pRNFL phenylketonuria radial peripapillary capillary plexus retinal imaging

来  源:   DOI:10.3389/fneur.2024.1305984   PDF(Pubmed)

Abstract:
UNASSIGNED: To compare radial peripapillary capillary (RPC) vascular plexus parameters and peripapillary retinal nerve fiber layer (pRNFL) thickness between Early-Treated Adults with Phenylketonuria (ETPKU) and controls.
UNASSIGNED: This observational study was a monocentric, case control study including 36 eyes of 36 participants. Among these, 18 were early-treated PKU (ETPKU) and 18 were controls. A SD-OCTA (XR Avanti AngioVue OCTA; Optovue Inc., Fremont, CA) was employed to assess the OCT and OCTA parameters of all the participants. The main outcome measures were the RPC vessels density (VD) %, and the pRNFL thickness.
UNASSIGNED: The average pRNFL thickness was significantly reduced in ETPKU (110.78 ± 12.48 μm) compared to controls (113.22 ± 13.95 μm), p = 0.046. The mean VD% of the small vessels of the RPC plexus was 52.31 ± 2.2 in ETPKU and 50.71 ± 3.2 in controls (p = 0.049), while the VD% of all the radial peripapillary capillary plexus (RPCP) was 58.5 ± 2.2 in ETPKU and 55.08 ± 3.4 in controls (p < 0.001). By contrast, there were no differences in age, sex, and IOP between the two groups.
UNASSIGNED: Through structural OCT and OCTA, we observed thinning of the nerve fibers accompanied by an increase in perfusion of the RPC plexus. Thus, our conclusions suggest that OCTA may serve as a noninvasive method to identify novel retinal biomarkers in ETPKU.
摘要:
比较早期治疗的苯丙酮尿症(ETPKU)成人和对照组之间的放射状乳头周围毛细血管(RPC)血管丛参数和乳头周围视网膜神经纤维层(pRNFL)厚度。
这项观察性研究是单中心的,病例对照研究,包括36名参与者的36只眼。其中,18例为早期治疗的PKU(ETPKU),18例为对照。ASD-OCTA(XRAvantiAngioVueOCTA;OptovueInc.,弗里蒙特,CA)用于评估所有参与者的OCT和OCTA参数。主要结果指标为RPC血管密度(VD)%,和pRNFL厚度。
与对照组(113.22±13.95μm)相比,ETPKU(110.78±12.48μm)的平均pRNFL厚度显着降低,p=0.046。在ETPKU和对照组中,RPC丛小血管的平均VD%为52.31±2.2,在50.71±3.2(p=0.049),而ETPKU中所有放射状乳头状周围毛细血管丛(RPCP)的VD%为58.5±2.2,对照组为55.08±3.4(p<0.001)。相比之下,年龄没有差异,性别,两组之间的IOP。
通过结构OCT和OCTA,我们观察到神经纤维变薄,伴有RPC丛灌注增加。因此,我们的结论提示,OCTA可作为一种非侵入性方法,用于鉴定ETPKU中的新型视网膜生物标志物.
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