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《Survey of ophthalmology》2023,68(5):940-956
Congenital aniridia is a panocular disorder that is typically characterized by iris hypoplasia and aniridia-associated keratopathy (AAK). AAK results in the progressive loss of corneal transparency and thereby loss of vision. Currently, there is no approved therapy to delay or prevent its progression, and clinical management is challenging because of phenotypic variability and high risk of complications after interventions; however, new insights into the molecular pathogenesis of AAK may help improve its management. Here, we review the current understanding about the pathogenesis and management of AAK. We highlight the biological mechanisms involved in AAK development with the aim to develop future treatment options, including surgical, pharmacological, cell therapies, and gene therapies.  相似文献   
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BackgroundRacial disparities in extracorporeal membrane oxygenation (ECMO) outcomes in patients with a broad set of indications are not well documented.MethodsAdults requiring ECMO were identified in the 2016–2019 National Inpatient Sample. Patient and hospital characteristics, including mortality, clinical outcomes, and resource utilization were analyzed using multivariable regressions.ResultsOf 43,190 adult ECMO patients, 67.8% were classified as White, 18.1% Black, and 10.4% Hispanic. Although mortality for Whites declined from 47.5 to 41.0% (P = 0.002), it remained steady for others. Compared to White, Asian/Pacific Islander (PI) race was linked to increased odds of mortalty (AOR = 1.4, 95% CI = 1.1–2.0). Black race was associated with increased odds of acute kidney injury (AOR = 1.4, 95%-CI: 1.2–1.7), while Hispanic race was linked to neurologic complications (AOR 21.6; 95% CI 1.2–2.3). Black and Hispanic race were also associated with increased incremental costs.ConclusionsRace-based disparities in ECMO outcomes persist in the United States. Further work should aim to understand and mitigate the underlying reasons for such findings.  相似文献   
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PurposeTo compare the characteristics of polidocanol (POL) and ethanolamine oleate (EO) sclerosing foams produced by a Shirasu porous glass membrane (SPGM) device with those made using a 3-way stopcock (3WSC).Materials and MethodsFoam half-life times were measured in an ex-vivo benchtop study. Computed tomography (CT) images of each foam were obtained over the time course, and a CT texture analysis was conducted. The bubble size in each foam was measured by an optical microscope.ResultsMedian foam half-life times were longer in the SPGM group than in the 3WSC group (POL: 198 vs 166 s, P = .02; EO: 640 vs 391 s, P < .01). In the CT texture analysis, median standard deviation (SD) and entropy (randomness) were lower, and median energy (uniformity) and gray-level cooccurrence matrix (GLCM) homogeneity were higher in the SPGM group than in the 3WSC group (POL SD: at 30 s and 50–300 s; POL entropy: at 0–60 s; EO SD: at 0–600 s; EO entropy: at 0–460 s; POL energy: at 0–40 s; POL GLCM homogeneity: at 0–250 s; EO energy: at 0–360 s; EO GLCM homogeneity: at 0–480 s; all P < .05). Median bubble diameters in the SPGM group and in the 3WSC group were 69 and 83 μm (P < .01), respectively, in the POL foam; and 36 and 36 μm (P = .45), respectively, in the EO foam.ConclusionsPOL and EO foams had greater uniformity and longer foam half-life time when prepared with an SPGM device than with a 3WSC.  相似文献   
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目的 探讨蜂巢提取物Elixir-X对自然衰老及辐射衰老小鼠的影响,为Elixir-X的进一步研发提供药理学基础。方法 采用小鼠肝脏切除术建立自然衰老小鼠模型,将其分为空白对照组、Elixir-X组、白藜芦醇组及模型组,连续给药7 d后,检测小鼠70%肝脏切除后的再生过程中,衰老细胞、肝脏功能恢复与肝脏再生的变化,观察Elixir-X对自然衰老的影响;采用60CO辐射法建立辐射衰老小鼠模型,将其分为模型组、空白对照组、白藜芦醇组、Elixir-X高、低剂量组,通过检测小鼠肠隐窝干细胞再生、小肠绒毛结构以及衰老细胞的变化,对比不同剂量Elixir-X对辐射后衰老的影响。结果 在肝脏切除术的小鼠模型中,Elixir-X组的肝功能水平明显低于模型组的肝脏自然再生小鼠,显著促进了肝功能恢复(P<0.05);同时与模型组相比,Elixir-X组清除了肝脏切除后再生过程中产生的衰老细胞(P<0.05),Ki-67的着色斑块明显增加,促进肝脏的再生,且肝血窦的扫描电镜影像结果中Elixir-X组肝血窦再生加快。60CO辐照小鼠模型中,与模型组相比,Elixir-X组有效促进辐照后肝脏功能恢复(P<0.05),Elixir-X高剂量组体重也恢复到正常水平,与空白对照组无显著差异;与模型组相比,Elixir-X低剂量组小肠的β-Gal着色细胞减少,Elixir-X高剂量组甚至能更有效地减少衰老细胞(P<0.05);小肠HE染色的结果中,Elixir-X组小肠绒毛结构得到恢复;Ki-67的IHC染色中,与模型组相比,Elixir-X组着色板块更多,且着色区域都在肠隐窝干细胞位置。结论 Elixir-X能有效清除肝脏切除术引起的自然衰老与辐射引起的损伤性衰老细胞,对衰老具有显著地抑制作用。  相似文献   
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Perinatal palliative care has grown out of both an historical necessity in attending to babies in the NICU that face difficult odds of survival, the increasing technology that may avail life-extending, yet technology-dependent, care, and the growth of fetal diagnostic and treatment centers. This review looks ta the history and ethical rationale for making available services from Pediatric and Perinatal Palliative Care to families in the prenatal and postnatal periods caring for a loved one with life-limiting circumstances.  相似文献   
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