scholarly journals Identification of Retinal Vascular Lesions Using Ultra-Widefield Angiography in Hereditary Hemorrhagic Telangiectasia Patients

2019 ◽  
Vol 3 (6) ◽  
pp. 510-515
Author(s):  
Sampat Sindhar ◽  
Bliss E. O’Bryhim ◽  
Jordan Licata ◽  
Jay F. Piccirillo ◽  
Rajendra S. Apte
Keyword(s):  
Hereditary Hemorrhagic Telangiectasia ◽  
Vascular Lesions ◽  
Retinal Vascular Lesions

scholarly journals Reduced Levels of Drp1 Protect against Development of Retinal Vascular Lesions in Diabetic Retinopathy

Cells ◽  
2021 ◽  
Vol 10 (6) ◽  
pp. 1379
Author(s):  
Dongjoon Kim ◽  
Hiromi Sesaki ◽  
Sayon Roy
Keyword(s):  
Diabetic Retinopathy ◽  
Cell Loss ◽  
Vascular Lesions ◽  
Protective Effects ◽  
Diabetic Mice ◽  
Wild Type ◽  
Apoptotic Genes ◽  
Retinal Vascular Lesions ◽  
Acellular Capillaries ◽  
Pericyte Loss

High glucose (HG)-induced Drp1 overexpression contributes to mitochondrial dysfunction and promotes apoptosis in retinal endothelial cells. However, it is unknown whether inhibiting Drp1 overexpression protects against the development of retinal vascular cell loss in diabetes. To investigate whether reduced Drp1 level is protective against diabetes-induced retinal vascular lesions, four groups of mice: wild type (WT) control mice, streptozotocin (STZ)-induced diabetic mice, Drp1+/− mice, and STZ-induced diabetic Drp1+/− mice were examined after 16 weeks of diabetes. Western Blot analysis indicated a significant increase in Drp1 expression in the diabetic retinas compared to those of WT mice; retinas of diabetic Drp1+/− mice showed reduced Drp1 level compared to those of diabetic mice. A significant increase in the number of acellular capillaries (AC) and pericyte loss (PL) was observed in the retinas of diabetic mice compared to those of the WT control mice. Importantly, a significant decrease in the number of AC and PL was observed in retinas of diabetic Drp1+/− mice compared to those of diabetic mice concomitant with increased expression of pro-apoptotic genes, Bax, cleaved PARP, and increased cleaved caspase-3 activity. Preventing diabetes-induced Drp1 overexpression may have protective effects against the development of vascular lesions, characteristic of diabetic retinopathy.

Retinal vascular lesions

2017 ◽  
Vol 53 (1) ◽  
pp. 89-89 ◽  
Author(s):  
Koushik Tripathy ◽  
Yog Raj Sharma
Keyword(s):  
Vascular Lesions ◽  
Retinal Vascular Lesions

scholarly journals Hereditary Hemorrhagic Telangiectasia with Hepatic Vascular Malformations

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Yujiro Nishioka ◽  
Nobuhisa Akamatsu ◽  
Yasuhiko Sugawara ◽  
Junichi Kaneko ◽  
Junichi Arita ◽  
...  
Keyword(s):  
Hereditary Hemorrhagic Telangiectasia ◽  
Vascular Malformations ◽  
Hereditary Disease ◽  
Vascular Lesions ◽  
Mild Anemia ◽  
Asymptomatic Patients ◽  
Japanese Male ◽  
Old Japanese ◽  
History Of ◽  
Enhanced Computed Tomography

Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant hereditary disease. Early diagnosis is important to avoid complications from vascular lesions, but diagnosis is difficult in asymptomatic patients. A 69-year-old Japanese male patient was referred to our hospital for evaluation of hepatic vascular malformations. He had mild anemia with iron deficiency, and dynamic contrast-enhanced computed tomography revealed significant arteriovenous and arterioportal shunts throughout the liver. Telangiectasia from the pharynx to the duodenum was confirmed by gastrointestinal endoscopy. The patient history revealed episodes of epistaxis as well as a family history of epistaxis. He was diagnosed with HHT, although no other family member had been diagnosed with definite HHT. A diagnosis of HHT must be considered in patients with hepatic vascular malformations.

scholarly journals Opa1 Deficiency Promotes Development of Retinal Vascular Lesions in Diabetic Retinopathy

2021 ◽  
Vol 22 (11) ◽  
pp. 5928
Author(s):  
Dongjoon Kim ◽  
Marcela Votruba ◽  
Sayon Roy
Keyword(s):  
Diabetic Retinopathy ◽  
Caspase 3 ◽  
Vascular Lesions ◽  
Apoptotic Cells ◽  
Diabetic Mice ◽  
Cytochrome C Release ◽  
Retinal Vascular Lesions ◽  
Acellular Capillaries ◽  
Pericyte Loss ◽  
Retinal Capillaries

This study investigates whether reduced optic atrophy 1 (Opa1) level promotes apoptosis and retinal vascular lesions associated with diabetic retinopathy (DR). Four groups of mice: wild type (WT) control mice, streptozotocin (STZ)-induced diabetic mice, Opa1+/− mice, and diabetic Opa1+/− mice were used in this study. 16 weeks after diabetes onset, retinas were assessed for Opa1 and Bax levels by Western blot analysis, and retinal networks were examined for acellular capillaries (AC) and pericyte loss (PL). Apoptotic cells were detected in retinal capillaries using TUNEL assay, and caspase-3 activity was assessed using fluorometric analysis. Opa1 expression was significantly downregulated in retinas of diabetic and Opa1+/− mice compared with those of WT mice. Inducing diabetes further decreased Opa1 expression in retinas of Opa1+/− mice. Increased cytochrome c release concomitant with increased level of pro-apoptotic Bax and elevated caspase-3 activity were observed in retinas of diabetic and Opa1+/− mice; the number of TUNEL-positive cells and AC/PL was also significantly increased. An additional decrease in the Opa1 level in retinas of diabetic Opa1+/− mice exacerbated the development of apoptotic cells and AC/PL compared with those of diabetic mice. Diabetes-induced Opa1 downregulation contributes, at least in part, to the development of retinal vascular lesions characteristic of DR.

A case of hereditary hemorrhagic telangiectasia with dilatation of intrahepatic vessels without vascular lesions in other organs

Kanzo ◽  
2015 ◽  
Vol 56 (11) ◽  
pp. 596-602
Author(s):  
Kiyohiro Izumi ◽  
Kojiro Michitaka ◽  
Tomoe Kawamura ◽  
Toshihiko Aibiki ◽  
Tomonari Okudaira ◽  
...  
Keyword(s):  
Hereditary Hemorrhagic Telangiectasia ◽  
Vascular Lesions

scholarly journals Potential Second-Hits in Hereditary Hemorrhagic Telangiectasia

2020 ◽  
Vol 9 (11) ◽  
pp. 3571
Author(s):  
Carmelo Bernabeu ◽  
Pinar Bayrak-Toydemir ◽  
Jamie McDonald ◽  
Michelle Letarte
Keyword(s):  
Hereditary Hemorrhagic Telangiectasia ◽  
Arteriovenous Malformations ◽  
Somatic Mutations ◽  
Autosomal Dominant ◽  
Genetic Disorder ◽  
Vascular Lesions ◽  
Mechanical Trauma ◽  
Internal Organs ◽  
Second Hit ◽  
Functional Mechanisms

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant genetic disorder that presents with telangiectases in skin and mucosae, and arteriovenous malformations (AVMs) in internal organs such as lungs, liver, and brain. Mutations in ENG (endoglin), ACVRL1 (ALK1), and MADH4 (Smad4) genes account for over 95% of HHT. Localized telangiectases and AVMs are present in different organs, with frequencies which differ among affected individuals. By itself, HHT gene heterozygosity does not account for the focal nature and varying presentation of the vascular lesions leading to the hypothesis of a “second-hit” that triggers the lesions. Accumulating research has identified a variety of triggers that may synergize with HHT gene heterozygosity to generate the vascular lesions. Among the postulated second-hits are: mechanical trauma, light, inflammation, vascular injury, angiogenic stimuli, shear stress, modifier genes, and somatic mutations in the wildtype HHT gene allele. The aim of this review is to summarize these triggers, as well as the functional mechanisms involved.

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