๐๐ต๐ข๐ณ๐จ๐ข๐ณ๐ฅ๐ตโ๐ด ๐๐ช๐ด๐ฆ๐ข๐ด๐ฆ ๐๐ฏ๐ท๐ฆ๐ด๐ต๐ช๐จ๐ข๐ต๐ช๐ฐ๐ฏ๐ด ๐ข๐ฏ๐ฅ ๐๐ข๐ฏ๐ข๐จ๐ฆ๐ฎ๐ฆ๐ฏ๐ต โฃ
โฃ
๐๐ก๐ช๐ค๐ง๐๐จ๐๐๐๐ฃ ๐ผ๐ฃ๐๐๐ค๐๐ง๐๐ฅ๐๐ฎ:โฃ
โฃ
โ Dark choroid sign- Not exclusive to Stargardt disease.โฃ
โ Due to a lack of early choroidal hyperfluorescence, which isโฃ
blocked by high-grade lipofuscin accumulation in the RPE, thus improving visualization of the small retinal capillaries that become easily evident over the dark, non-fluorescent and high-contrast choroid (blockhead arrow)โฃ
โ Atrophic fovea- choroidal vessels become visibleโฃ
โ Fundus flecks are seen as small irregular hyperfluorescent lesionsโฃ
(arrow). The clinically visible flecks do not necessarily correspond to the window-defects in the FFA.โฃ

โฃ
๐๐ช๐ฃ๐๐ช๐จ ๐ผ๐ช๐ฉ๐ค๐๐ก๐ช๐ค๐ง๐๐จ๐๐๐ฃ๐๐:โฃ
โฃ
โ Abnormally increased FAF represents excessive lipofuscin accumulation in the RPE.โฃ
โ Inversely, decreased areas of FAF relate to low level RPE metabolic activity which normally underlies local atrophy with secondary photoreceptor loss.โฃ
โ Abnormally high FAF intensity with all other normal parameters suggests that RPE lipofuscin deposition may be the first pathophysiological event in ABCA4-related disease.โฃ
โฃ
๐๐๐ :โฃ
โฃ
โ Normal in early disease. Reduced scotopic and photopic responses in advanced cases.โฃ
โ Slow dark adaptationโฃ
โฃ
๐๐พ๐ :โฃ
โฃ
โ Shows atrophic changes and disorganisation in photoreceptorsโฃ
โ RPE and lipofuscin deposits maybe detected within the parafoveal RPE.โฃ

โฃ
๐๐๐ฃ๐๐๐๐ข๐๐ฃ๐ฉ :โฃ
โฃ
โ No proven treatment.โฃ
โ Avoid Vitamin A and its supplements- makes the disease worse.โฃ
โ PUFA such as Docosahexaenoic acid (DHA) shown to reduce toxicity ofโฃ
A2E ( In autosomal dominant Stargardt)โฃ
โ Gene therapyโฃ
โ RPE precursor cells derived from embryonic stem cells injectedโฃ
subretinally.โฃ
โฃ
โฃ
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