Macular pigment and lutein supplementation in ABC44-associated retinal degenerations
Macular pigment and lutein supplementation in ABC44-associated retinal degenerations
复制标题
DOI：
10.1167/iovs.06-0764
复制
发表时间：
2007-03-01
影响因子：
4.4
通讯作者：
Jacobson, Samuel G.
中科院分区：
文献类型：
--
作者：
Aleman, Tomas S.;Cideciyan, Artur V.;Jacobson, Samuel G.
目的.测定ABCA 4相关视网膜变性（ABCA 4-RD）患者的黄斑色素（MP）光密度（OD），以及补充叶黄素对MP和视力的影响。包括患有Stargardt病或锥-杆营养不良以及已知或疑似ABCA 4基因致病突变的患者。所有患者均行中心凹固定。用异色闪烁光度法测定MPOD谱。血清类胡萝卜素，视力，中心凹敏感度，视网膜厚度进行定量。在接受口服补充叶黄素6个月的患者亚组中测定MPOD和中心视力的变化。患者的MPOD范围从正常到明显异常。作为一个组，ABCA 4-RD患者的中心凹MPOD降低，并且与视网膜厚度有很强的相关性。平均黄斑中心凹组织浓度的MP，估计除以视网膜厚度的MPOD，是正常的患者，而血清中的叶黄素和玉米黄质浓度显着低于正常。口服叶黄素补充6个月后，91%的患者血清叶黄素显着增加，63%的患者的眼睛显示MPOD显着增加。视网膜反应者倾向于女性，并且在基线时具有较低的血清叶黄素和玉米黄质、较低的MPOD和较大的视网膜厚度。反应眼的基线MP浓度显著低于无反应眼。术后中心视力无明显变化。MP受到ABCA 4疾病阶段的强烈影响，导致异常的中央凹结构。在某些患者中，补充叶黄素可增加MP。叶黄素补充6个月后，中心视力没有变化。这种补充剂对这些黄斑变性自然史的长期影响需要进一步研究。
PURPOSE. To determine macular pigment (MP) optical density (OD) in patients with ABCA4-associated retinal degenerations (ABCA4-RD) and the response of MP and vision to supplementation with lutein.METHODS. Patients with Stargardt disease or cone-rod dystrophy and known or suspected disease-causing mutations in the ABCA4 gene were included. All patients had foveal fixation. MPOD profiles were measured with heterochromatic flicker photometry. Serum carotenoids, visual acuity, foveal sensitivity, and retinal thickness were quantified. Changes in MPOD and central vision were determined in a subset of patients receiving oral supplementation with lutein for 6 months.RESULTS. MPOD in patients ranged from normal to markedly abnormal. As a group, patients with ABCA4-RD had reduced foveal MPOD, and there was a strong correlation with retinal thickness. Average foveal tissue concentration of MP, estimated by dividing MPOD by retinal thickness, was normal in patients, whereas serum concentration of lutein and zeaxanthin was significantly lower than normal. After oral lutein supplementation for 6 months, 91% of the patients showed significant increases in serum lutein, and 63% of the patients' eyes showed a significant augmentation in MPOD. The retinal responders tended to be female and to have lower serum lutein and zeaxanthin, lower MPOD, and greater retinal thickness at baseline. Responding eyes had significantly lower baseline MP concentration than did nonresponding eyes. Central vision was unchanged after, the period of supplementation.CONCLUSIONS. MP is strongly affected by the stage of ABCA4 disease leading to abnormal foveal architecture. MP could be augmented by supplemental lutein in some patients. There was no change in central vision after 6 months of lutein supplementation. Long-term influences of this supplement on the natural history of these macular degenerations require further study.