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PURPOSE: Birdshot chorioretinitis (BSCR) is an ocular HLA-related disease with variable clinical progression. We examine the quality of life (QOL) of BSCR individuals aged ≥80 years, providing insights into the long-term disease impact. METHODS: We utilized data from the CO-BIRD cohort (ClinicalTrials.gov Identifier: NCT05153057) conducted at Hôpital Cochin in Paris, France, focusing on BSCR patients aged ≥80. The main outcome was vision-related QOL using the National Eye Institute Visual Function Questionnaire-25 (NEI VFQ-25). We used Spearman correlation to explore the impact of better BCVA and MD on the composite score, and the Wilcoxon test to evaluate specific visual symptoms' effects. RESULTS: The study included 35 patients with a mean age of 83.9 ± 3.7 years, 74.3% of whom received systemic immunosuppression. The mean composite score was 58 ± 30, with a median of 75 (23-79). The lowest subscores were driving capacity (38 ± 38), mental health (49 ± 33), and role difficulties (50 ± 35), while the highest were for ocular pain (70 ± 25) and social function (70 ± 38). Decimal BCVA below 0.5 and MD below -6 dB were associated with lower subscores. BCVA and MD were strongly correlated with the composite score (R = 0.67). Symptoms of poor color and blurry vision were significantly associated with lower composite score (p < 0.005). CONCLUSION: Most BSCR patients over 80 in our cohort maintained sufficient vision for daily activities. The high standard deviation and wide range of VFQ-25 results reflect the heterogeneity of visual outcomes among elderly BSCR patients.
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OBJECTIVE: Ectopic lipid accumulation in the liver and kidneys is a hallmark of metabolic diseases leading to non-alcoholic fatty liver disease (NAFLD) and chronic kidney disease (CKD). Moreover, recent data have highlighted a strong correlation between NAFLD and CKD incidences. In this study, we use two mouse models of hepatic steatosis or CKD, each initiated independently of the other upon the suppression of glucose production specifically in the liver or kidneys, to elucidate the mechanisms underlying the development of CKD in the context of NAFLD-like pathology. METHODS: Mice with a deletion of G6pc, encoding glucose-6 phosphatase catalytic subunit, specifically in the liver (L.G6pc-/- mice) or the kidneys (K.G6pc-/- mice), were fed with either a standard diet or a high fat/high sucrose (HF/HS) diet during 9 months. These mice represent two original models of a rare metabolic disease named Glycogen Storage Disease Type Ia (GSDIa) that is characterized by both NAFLD-like pathology and CKD. Two other groups of L.G6pc-/- and K.G6pc-/- mice were fed a standard diet for 6 months and then treated with fenofibrate for 3 months. Lipid and glucose metabolisms were characterized, and NAFLD-like and CKD damages were evaluated. RESULTS: Lipid depot exacerbation upon high-calorie diet strongly accelerated hepatic and renal pathologies induced by the G6pc-deficiency. In L.G6pc-/- mice, HF/HS diet increased liver injuries, characterized by higher levels of plasmatic transaminases and increased hepatic tumor incidence. In K.G6pc-/- mice, HF/HS diet increased urinary albumin and lipocalin 2 excretion and aggravated renal fibrosis. In both cases, the worsening of NAFLD-like injuries and CKD was independent of glycogen content. Furthermore, fenofibrate, via the activation of lipid oxidation significantly decreased the hepatic or renal lipid accumulations and prevented liver or kidney damages in L.G6pc-/- and K.G6pc-/- mice, respectively. Finally, we show that L.G6pc-/- mice and K.G6pc-/- mice developed NAFLD-like pathology and CKD independently. CONCLUSIONS: This study highlights the crucial role that lipids play in the independent development of both NAFLD and CKD and demonstrates the importance of lipid-lowering treatments in various metabolic diseases featured by lipid load, from the "rare" GSDIa to the "epidemic" morbid obesity or type 2 diabetes.
