Dominique Prétot, Maria Della Volpe Waizel, Karolina Kaminska, Philippe Valmaggia, Giorgio Placidi, Benedetto Falsini, Fabian N Fries, Nóra Szentmáry, Carlo Rivolta, Hendrik P N Scholl, Giacomo Calzetti
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Main outcome parameters were the mean arterial (A-SO<sub>2</sub>; %), venular (V-SO<sub>2</sub>; %) oxygen saturation, and their difference (A-V SO<sub>2</sub>; %) recorded with the oxygen saturation tool of the Retinal Vessel Analyzer (IMEDOS Systems UG, Germany). Statistical analyses were performed with linear mixed-effects models.</p><p><strong>Results: </strong>Eyes suffering from CHM differed significantly from both RP and control eyes, when the retinal oxygen metabolic parameters were taken into account. While RP showed significantly higher A-SO<sub>2</sub> and V-SO<sub>2</sub> values when compared to controls, CHM showed opposite findings with significantly lower values when compared to both RP and controls (P < 0.001). The A-V SO<sub>2</sub>, which represents the retinal oxygen metabolic consumption, showed significantly lower values in CHM compared to controls.</p><p><strong>Conclusion: </strong>The retina in CHM is a relatively hypoxic environment. The decrease in oxygen levels may be due to the profound choroidal degeneration, leading to decreased oxygen flux to the retina. RO measurements may help understand the pathogenesis of CHM and RP. These findings may provide useful details to inform the planning of clinical trials of emerging therapies for CHM.</p><p><strong>Key messages: </strong>What was known before? Retinal oxygen metabolic function measured with retinal oximetry (RO) shows significant alterations in patients with retinitis pigmentosa.</p><p><strong>What this study adds: </strong>RO function in choroideremia is significantly altered when compared to controls. Furthermore, RO in choroideremia shows opposing findings within different oxygen metabolic parameters to those that were so far known for retinitis pigmentosa. By providing insights into the retinal oxygen metabolic mechanisms, RO can help understand the underlying pathophysiology in choroideremia.</p>","PeriodicalId":12795,"journal":{"name":"Graefe’s Archive for Clinical and Experimental Ophthalmology","volume":" ","pages":"379-385"},"PeriodicalIF":2.4000,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Retinal oxygen metabolic function in choroideremia and retinitis pigmentosa.\",\"authors\":\"Dominique Prétot, Maria Della Volpe Waizel, Karolina Kaminska, Philippe Valmaggia, Giorgio Placidi, Benedetto Falsini, Fabian N Fries, Nóra Szentmáry, Carlo Rivolta, Hendrik P N Scholl, Giacomo Calzetti\",\"doi\":\"10.1007/s00417-024-06659-8\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Purpose: </strong>To measure the retinal oxygen metabolic function with retinal oximetry (RO) in patients with choroideremia (CHM) and compare these findings with retinitis pigmentosa (RP) patients and controls.</p><p><strong>Methods: </strong>Prospective observational study including 18 eyes of 9 molecularly confirmed CHM patients (9♂; 40.2 ± 21.2 years (mean ± SD), 77 eyes from 39 patients with RP (15♀ 24♂; 45.6 ± 14.7 years) and 100 eyes from 53 controls (31♀ 22♂; 40.2 ± 13.4 years). Main outcome parameters were the mean arterial (A-SO<sub>2</sub>; %), venular (V-SO<sub>2</sub>; %) oxygen saturation, and their difference (A-V SO<sub>2</sub>; %) recorded with the oxygen saturation tool of the Retinal Vessel Analyzer (IMEDOS Systems UG, Germany). Statistical analyses were performed with linear mixed-effects models.</p><p><strong>Results: </strong>Eyes suffering from CHM differed significantly from both RP and control eyes, when the retinal oxygen metabolic parameters were taken into account. While RP showed significantly higher A-SO<sub>2</sub> and V-SO<sub>2</sub> values when compared to controls, CHM showed opposite findings with significantly lower values when compared to both RP and controls (P < 0.001). The A-V SO<sub>2</sub>, which represents the retinal oxygen metabolic consumption, showed significantly lower values in CHM compared to controls.</p><p><strong>Conclusion: </strong>The retina in CHM is a relatively hypoxic environment. The decrease in oxygen levels may be due to the profound choroidal degeneration, leading to decreased oxygen flux to the retina. RO measurements may help understand the pathogenesis of CHM and RP. These findings may provide useful details to inform the planning of clinical trials of emerging therapies for CHM.</p><p><strong>Key messages: </strong>What was known before? Retinal oxygen metabolic function measured with retinal oximetry (RO) shows significant alterations in patients with retinitis pigmentosa.</p><p><strong>What this study adds: </strong>RO function in choroideremia is significantly altered when compared to controls. Furthermore, RO in choroideremia shows opposing findings within different oxygen metabolic parameters to those that were so far known for retinitis pigmentosa. 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引用次数: 0
摘要
目的：通过视网膜血氧仪（RO）测量脉络膜血症（CHM）患者的视网膜氧代谢功能，并将这些结果与视网膜色素变性（RP）患者和对照组进行比较：前瞻性观察研究，包括9名经分子证实的络氨酸血症患者的18只眼睛（9♂；40.2 ± 21.2岁（平均 ± SD））、39名RP患者的77只眼睛（15♀ 24♂；45.6 ± 14.7岁）和53名对照组患者的100只眼睛（31♀ 22♂；40.2 ± 13.4岁）。主要结果参数是使用视网膜血管分析仪（IMEDOS Systems UG，德国）的氧饱和度工具记录的平均动脉（A-SO2；%）、静脉（V-SO2；%）氧饱和度及其差值（A-V SO2；%）。统计分析采用线性混合效应模型：结果：考虑到视网膜氧代谢参数，CHM 患者的视力与 RP 和对照组的视力有显著差异。RP患者的A-SO2和V-SO2值明显高于对照组，而CHM患者的A-SO2和V-SO2值明显低于RP和对照组（P 2代表视网膜氧代谢消耗，CHM患者的P 2值明显低于对照组）：结论：CHM 患者的视网膜处于相对缺氧的环境中。结论：CHM 患者的视网膜处于相对缺氧的环境中，氧含量的降低可能是由于脉络膜严重变性，导致进入视网膜的氧通量减少。RO 测量有助于了解 CHM 和 RP 的发病机制。这些发现可能会为CHM新兴疗法的临床试验规划提供有用的细节信息：以前知道些什么？用视网膜血氧仪（RO）测量的视网膜氧代谢功能在视网膜色素变性患者中显示出明显的改变：与对照组相比，脉络膜血症患者的视网膜氧代谢功能明显改变。此外，脉络膜血症患者的视网膜氧代谢参数与迄今已知的视网膜色素变性患者的氧代谢参数截然相反。通过深入了解视网膜氧代谢机制，RO有助于理解脉络膜血症的潜在病理生理学。
Retinal oxygen metabolic function in choroideremia and retinitis pigmentosa.
Purpose: To measure the retinal oxygen metabolic function with retinal oximetry (RO) in patients with choroideremia (CHM) and compare these findings with retinitis pigmentosa (RP) patients and controls.
Methods: Prospective observational study including 18 eyes of 9 molecularly confirmed CHM patients (9♂; 40.2 ± 21.2 years (mean ± SD), 77 eyes from 39 patients with RP (15♀ 24♂; 45.6 ± 14.7 years) and 100 eyes from 53 controls (31♀ 22♂; 40.2 ± 13.4 years). Main outcome parameters were the mean arterial (A-SO2; %), venular (V-SO2; %) oxygen saturation, and their difference (A-V SO2; %) recorded with the oxygen saturation tool of the Retinal Vessel Analyzer (IMEDOS Systems UG, Germany). Statistical analyses were performed with linear mixed-effects models.
Results: Eyes suffering from CHM differed significantly from both RP and control eyes, when the retinal oxygen metabolic parameters were taken into account. While RP showed significantly higher A-SO2 and V-SO2 values when compared to controls, CHM showed opposite findings with significantly lower values when compared to both RP and controls (P < 0.001). The A-V SO2, which represents the retinal oxygen metabolic consumption, showed significantly lower values in CHM compared to controls.
Conclusion: The retina in CHM is a relatively hypoxic environment. The decrease in oxygen levels may be due to the profound choroidal degeneration, leading to decreased oxygen flux to the retina. RO measurements may help understand the pathogenesis of CHM and RP. These findings may provide useful details to inform the planning of clinical trials of emerging therapies for CHM.
Key messages: What was known before? Retinal oxygen metabolic function measured with retinal oximetry (RO) shows significant alterations in patients with retinitis pigmentosa.
What this study adds: RO function in choroideremia is significantly altered when compared to controls. Furthermore, RO in choroideremia shows opposing findings within different oxygen metabolic parameters to those that were so far known for retinitis pigmentosa. By providing insights into the retinal oxygen metabolic mechanisms, RO can help understand the underlying pathophysiology in choroideremia.
期刊介绍：
Graefe''s Archive for Clinical and Experimental Ophthalmology is a distinguished international journal that presents original clinical reports and clini-cally relevant experimental studies. Founded in 1854 by Albrecht von Graefe to serve as a source of useful clinical information and a stimulus for discussion, the journal has published articles by leading ophthalmologists and vision research scientists for more than a century. With peer review by an international Editorial Board and prompt English-language publication, Graefe''s Archive provides rapid dissemination of clinical and clinically related experimental information.
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