AIM: To report a case of pseudoxanthoma elasticum (PXE) in a 48 year old woman that presented with bilateral blurry vision. METHODS: A case report RESULTS: A 48-year-old woman presented with bilateral blurry vision an...AIM: To report a case of pseudoxanthoma elasticum (PXE) in a 48 year old woman that presented with bilateral blurry vision. METHODS: A case report RESULTS: A 48-year-old woman presented with bilateral blurry vision and right eye metamorphopsia. The patient had a history of angioid streaks in the left eye ten years ago for which she had received laser surgery and had poor residual vision. Visual acuity was 20/60 in the right eye and count fingers at 6 feet in the left. Fundus examination showed subretinal hemorrhage and macular thickening on the right and a disciform macular scar with focal atrophic pigment epithelial lesions on the left. Both eyes had angioid streaks and peau d’orange pigmentary pattern of the retina. External examination showed several, yellow skin papules and plaques on the lateral and posterior neck, as well as prominent mental creases. Pathologic examination of skin biopsy confirmed the diagnosis of PXE, showing calcium deposition and fragmented, clumped elastic fibers in the deep reticular dermis. She responded well to intravitreal bevacizumab injections and visual acuity improved to 20/25 OD. Preventative care was emphasized and the patient was referred to cardiology, gastroenterology and human genetics for counseling. CONCLUSION: PXE is a multisystem disorder affecting the dermatologic, ocular, and cardiovascular systems. Ophthalmic findings of angioid streaks and choroidal neovascularization in the presence of stereotypical skin changes and prominent mental creases should prompt evaluation for PXE.展开更多
Pseudoxanthoma elasticum (PXE) is a hereditary disorder that affects primarily the elastic tissues of skin, eyes and blood vessels. Its estimated prevalence is thought to be 1:25,000 - 100,000. PXE patients also carry...Pseudoxanthoma elasticum (PXE) is a hereditary disorder that affects primarily the elastic tissues of skin, eyes and blood vessels. Its estimated prevalence is thought to be 1:25,000 - 100,000. PXE patients also carry an increased risk for cardiovascular and gastrointestinal diseases. Early diagnosis can play a crucial role in preventing early loss of vision and systemic complications in these patients however despite having characteristic clinical features;it is still to a large extent being under-diagnosed. Here we present two cases of PXE with reduced vision.展开更多
It is challenging to make an ultrafast diagnosis of the temporal evolution of small and short-lived plasma in two dimensions. To overcome this difficulty, we have developed a well-timed diagnostic utilizing an x-ray s...It is challenging to make an ultrafast diagnosis of the temporal evolution of small and short-lived plasma in two dimensions. To overcome this difficulty, we have developed a well-timed diagnostic utilizing an x-ray streak camera equipped with a row of multi-pinhole arrays. By processing multiple sets of one-dimensional streaked image data acquired from various pinholes, we are capable of reconstructing high-resolution two-dimensional images with a temporal resolution of 38 ps and a spatial resolution of 18 μm. The temporal fiducial pulses accessed from external sources can advance the precise timing and accurately determine the arrival time of the laser. Moreover, it can correct the nonlinear sweeping speed of the streak camera. The effectiveness of this diagnostic has been successfully verified at the Shenguang-II laser facility,providing an indispensable tool for observing complex physical phenomena, such as the implosion process of laser-fusion experiments.展开更多
文摘AIM: To report a case of pseudoxanthoma elasticum (PXE) in a 48 year old woman that presented with bilateral blurry vision. METHODS: A case report RESULTS: A 48-year-old woman presented with bilateral blurry vision and right eye metamorphopsia. The patient had a history of angioid streaks in the left eye ten years ago for which she had received laser surgery and had poor residual vision. Visual acuity was 20/60 in the right eye and count fingers at 6 feet in the left. Fundus examination showed subretinal hemorrhage and macular thickening on the right and a disciform macular scar with focal atrophic pigment epithelial lesions on the left. Both eyes had angioid streaks and peau d’orange pigmentary pattern of the retina. External examination showed several, yellow skin papules and plaques on the lateral and posterior neck, as well as prominent mental creases. Pathologic examination of skin biopsy confirmed the diagnosis of PXE, showing calcium deposition and fragmented, clumped elastic fibers in the deep reticular dermis. She responded well to intravitreal bevacizumab injections and visual acuity improved to 20/25 OD. Preventative care was emphasized and the patient was referred to cardiology, gastroenterology and human genetics for counseling. CONCLUSION: PXE is a multisystem disorder affecting the dermatologic, ocular, and cardiovascular systems. Ophthalmic findings of angioid streaks and choroidal neovascularization in the presence of stereotypical skin changes and prominent mental creases should prompt evaluation for PXE.
文摘Pseudoxanthoma elasticum (PXE) is a hereditary disorder that affects primarily the elastic tissues of skin, eyes and blood vessels. Its estimated prevalence is thought to be 1:25,000 - 100,000. PXE patients also carry an increased risk for cardiovascular and gastrointestinal diseases. Early diagnosis can play a crucial role in preventing early loss of vision and systemic complications in these patients however despite having characteristic clinical features;it is still to a large extent being under-diagnosed. Here we present two cases of PXE with reduced vision.
基金supported by the Strategic Priority Research Program of the Chinese Academy of Sciences (Grant Nos. XDA25030700 and XDA25030500)the National Key R&D Program of China (Grant Nos. 2022YFA1603200 and 2022YFA1603203)the National Natural Science Foundation of China (Grant Nos. 12175018, 12135001, 12075030, and 11903006)。
文摘It is challenging to make an ultrafast diagnosis of the temporal evolution of small and short-lived plasma in two dimensions. To overcome this difficulty, we have developed a well-timed diagnostic utilizing an x-ray streak camera equipped with a row of multi-pinhole arrays. By processing multiple sets of one-dimensional streaked image data acquired from various pinholes, we are capable of reconstructing high-resolution two-dimensional images with a temporal resolution of 38 ps and a spatial resolution of 18 μm. The temporal fiducial pulses accessed from external sources can advance the precise timing and accurately determine the arrival time of the laser. Moreover, it can correct the nonlinear sweeping speed of the streak camera. The effectiveness of this diagnostic has been successfully verified at the Shenguang-II laser facility,providing an indispensable tool for observing complex physical phenomena, such as the implosion process of laser-fusion experiments.
