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The British Journal of Ophthalmology Oct 1990
Topics: Angioid Streaks; Humans; Osteitis Deformans
PubMed: 2285678
DOI: 10.1136/bjo.74.10.577 -
Genes Aug 2021This study aimed to characterize Korean patients with pseudoxanthoma elasticum (PXE) presenting with angioid streaks. Retinal phenotypes were longitudinally evaluated by... (Observational Study)
Observational Study
This study aimed to characterize Korean patients with pseudoxanthoma elasticum (PXE) presenting with angioid streaks. Retinal phenotypes were longitudinally evaluated by multimodal ophthalmic imaging, and targeted gene panel sequencing for inherited retinal diseases was conducted. Seven subjects from unrelated families (median age, 51.2 years) were enrolled and followed for a median of 3.2 years. Four asymptomatic patients were significantly younger than three symptomatic patients with decreased visual acuity at presentation (mean age; 38.1 vs. 61.5 years, = 0.020). The asymptomatic patients maintained good vision (20/32 or better) and had no choroidal neovascularization (CNV) over the observation period. The symptomatic patients showed additional reduction in visual acuity and bilateral CNV occurrence during the longitudinal follow-up. Pathogenic variants were identified in all patients, leading to a diagnosis of PXE. Heterozygous monoallelic variants were identified in four patients and compound heterozygous variants were detected in three patients. Nine variants were identified, including one novel variant, c.2035G>T [p.Glu679Ter]. This is the first genetic study of Korean patients with PXE.
Topics: Adult; Angioid Streaks; Choroidal Neovascularization; Female; Fluorescein Angiography; Heterozygote; Humans; Male; Middle Aged; Multidrug Resistance-Associated Proteins; Pseudoxanthoma Elasticum; Republic of Korea; Retrospective Studies; Visual Acuity
PubMed: 34440381
DOI: 10.3390/genes12081207 -
Postgraduate Medical Journal May 2004
Topics: Angioid Streaks; Female; Humans; Middle Aged; Pseudoxanthoma Elasticum; Vision Disorders
PubMed: 15138323
DOI: 10.1136/pgmj.2003.009340 -
European Journal of Internal Medicine Aug 2020
Topics: Angioid Streaks; Cerebrovascular Disorders; Humans
PubMed: 32620498
DOI: 10.1016/j.ejim.2020.06.033 -
Ophthalmologica. Journal International... 1988This study examines different clinical parameters and the occurrence of macular degeneration in a series of 110 patients with angioid streaks of the fundus. Among the...
This study examines different clinical parameters and the occurrence of macular degeneration in a series of 110 patients with angioid streaks of the fundus. Among the parameters considered were: age of the patient at the time of ocular diagnosis, associated systemic disease, optic nerve drusen, peau d'orange appearance in the fundus, number of radiating angioid streaks as well as their length, width, and distance from the fovea. Statistically significant correlations were found between the occurrence of macular degeneration (of the exudative hemorrhagic type) and the following: angioid streak length, distance from the fovea, and the diffuse type or 'cracked egg-shell' fundus appearance.
Topics: Adolescent; Adult; Aged; Angioid Streaks; Child; Choroid; Female; Fundus Oculi; Humans; Macular Degeneration; Male; Middle Aged; Neovascularization, Pathologic; Retinal Vessels; Retrospective Studies
PubMed: 2460816
DOI: 10.1159/000309915 -
Archives of Ophthalmology (Chicago,... Aug 1966
Topics: Adult; Angioid Streaks; Child; Ehlers-Danlos Syndrome; Eye Manifestations; Female; Fundus Oculi; Humans; Male; Microscopy; Microscopy, Electron; Middle Aged; Scoliosis
PubMed: 5945174
DOI: 10.1001/archopht.1966.03850010199009 -
Journal Francais D'ophtalmologie Oct 2018
Topics: Adult; Angioid Streaks; Fundus Oculi; Humans; Male; Tomography, Optical Coherence; beta-Thalassemia
PubMed: 30224095
DOI: 10.1016/j.jfo.2018.01.017 -
Eye (London, England) Apr 2016
Topics: Angiogenesis Inhibitors; Angioid Streaks; Antibodies, Monoclonal, Humanized; Choroidal Neovascularization; Fluorescein Angiography; Humans
PubMed: 26795411
DOI: 10.1038/eye.2015.284 -
Ophthalmic Surgery, Lasers & Imaging... Nov 2021To investigate the prevalence of areas of dark without pressure (DWOP) and angioid streaks (AS) in patients with sickle cell disease (SCD).
BACKGROUND AND OBJECTIVES
To investigate the prevalence of areas of dark without pressure (DWOP) and angioid streaks (AS) in patients with sickle cell disease (SCD).
PATIENTS AND METHODS
This was a consecutive series of 77 adults with SCD.
RESULTS
DWOP appeared as multiple patches in 35 of the affected eyes and as a single lesion in 3 eyes. OCT finding demonstrated the ellipsoid layer was hyporeflective in DWOP. AS were identified in six cases (3.9%) and were bilateral in five cases. The prevalence of AS was higher with increasing age, being 67% in the patients older than age 45 years.
CONCLUSION
The prevalence of DWOP in adults with SCD is 25% in this study, which is higher than previously reported, and the prevalence of AS is around 4%, which is midway between prior estimates. Recognition of the clinical examination and imaging features of DWOP reduce the need for additional investigation. .
Topics: Adult; Anemia, Sickle Cell; Angioid Streaks; Fluorescein Angiography; Humans; Middle Aged; Prevalence
PubMed: 34766849
DOI: 10.3928/23258160-20211026-01 -
Eye (London, England) Jun 2023To assess systemic associations of angioid streaks (AS) using a large US healthcare database.
BACKGROUND/OBJECTIVES
To assess systemic associations of angioid streaks (AS) using a large US healthcare database.
SUBJECTS/METHODS
A retrospective cross-sectional study was conducted of patients diagnosed with AS in a large, national US insurer from 2000-2019. Cases were matched 1:5 to controls. The prevalence rates of established associated disease states and other systemic diseases were calculated and compared using logistic regression. Additionally, the rate of anti-VEGF treatment was assessed as a proxy for the incidence of choroidal neovascularization (CNV).
RESULTS
One thousand eight hundred fifty-two cases of AS and 9028 matched controls were included. The rates of association between AS and the well-characterized conditions included: Pseudoxanthoma elasticum (PXE)-228 patients (12.3%), Ehlers-Danlos syndrome-18 patients (1.0%), Paget's disease-6 patients (0.3%), hemoglobinopathies-30 patients (1.6%), and idiopathic-1573 patients (84.9%). There was a statistically higher prevalence of the following less classically associated diseases among patients with AS compared to controls: hereditary spherocytosis (1.7% vs. 0.6%, p < 0.001), connective tissue disease (1.0% vs 0.3%, p < 0.001) and non-exudative age-related macular degeneration (33.9% vs 10.6%, p < 0.001). Among 1442 eligible cases analyzed, 427 (29.6%) received at least 1 anti-VEGF injection with 338 (23.4%) patients having the injection after their AS diagnosis.
CONCLUSIONS
In the largest collection of AS patients to date, the classical teaching of systemic disease associations occur at rates far, far lower than previously reported. The association of AS with other less reported diseases highlights new potential associations and may contribute to the understanding of AS formation.
Topics: Humans; Angioid Streaks; Retrospective Studies; Cross-Sectional Studies; Pseudoxanthoma Elasticum; Choroidal Neovascularization; Delivery of Health Care; Fluorescein Angiography
PubMed: 35915234
DOI: 10.1038/s41433-022-02189-x