AICA ribosiduria is an ultra-rare disorder of de novo purine biosynthesis associated with developmental delay of varying severity, seizures, and varying degrees of visual impairment due to chorioretinal atrophy. Caused by biallelic pathogenic variants in ATIC, accumulation of AICA-riboside is the biochemical hallmark and presumed pathomechanism of the condition. In this study, we report the case of a teenage patient compound-heterozygous for the variants c.
View Article and Find Full Text PDFPurpose: Bacterial keratitis (BK) severity in murine models has traditionally been measured by subjective clinical grading or quantification of ocular bacterial burden. This investigation explores an objective and repeatable quantification of slit lamp photography (SLP) images to measure BK severity.
Methods: BALB/c strain mice underwent three parallel scratches of the right cornea with subsequent inoculation of 107Staphylococcus aureus cells.
Background: To compare the efficacy of intravitreally administered dexamethasone (Dex) and subsequent time-displaced fluocinolone acetonide (FA) on central subfield thickness (CST) in eyes with noninfectious uveitis.
Methods: Retrospective analysis of twenty-three eyes (18 patients) subsequently receiving intravitreal Dex and FA implants. The main outcome measures were CST, best-corrected visual acuity (BCVA), intraocular pressure (IOP), and status of inflammation.
Purpose: To assess clinical applicability of automatic image analysis in microbial keratitis (MK) by evaluating the relationship between biomarker measurements on slit-lamp photography (SLP) and best-corrected visual acuity (BCVA).
Methods: Seventy-six patients with MK with SLP images and same-day logarithm of the minimum angle of resolution (logMAR) BCVA were evaluated. MK biomarkers (stromal infiltrate, white blood cell infiltration, corneal edema, hypopyon, epithelial defect) were segmented manually by ophthalmologists and automatically by a novel, open-source, deep learning-based segmentation algorithm.
Purpose: To evaluate the reliability of manual annotation when quantifying cornea anatomical and microbial keratitis (MK) morphological features on slit-lamp photography (SLP) images.
Methods: Prospectively enrolled patients with MK underwent SLP at initial encounter at 2 academic eye hospitals. Patients who presented with an epithelial defect (ED) were eligible for analysis.
Syphilis is a bacterial infectious disease transmitted by the spirochaete Treponema pallidum. The rate of infection has increased during the last decade. Ocular syphilis is an underestimated presentation of the disease, and it is increased in HIV-positive patients.
View Article and Find Full Text PDFWe propose a fully-automatic deep learning-based algorithm for segmentation of ocular structures and microbial keratitis (MK) biomarkers on slit-lamp photography (SLP) images. The dataset consisted of SLP images from 133 eyes with manual annotations by a physician, P1. A modified region-based convolutional neural network, SLIT-Net, was developed and trained using P1's annotations to identify and segment four pathological regions of interest (ROIs) on diffuse white light images (stromal infiltrate (SI), hypopyon, white blood cell (WBC) border, corneal edema border), one pathological ROI on diffuse blue light images (epithelial defect (ED)), and two non-pathological ROIs on all images (corneal limbus, light reflexes).
View Article and Find Full Text PDFPurpose: To investigate the sources of measurement variability when quantifying the morphology of microbial keratitis (MK) from slit-lamp photography (SLP) images using a semiautomated, image-analysis algorithm.
Methods: Prospectively enrolled patients with MK underwent SLP to obtain images of their epithelial defects (ED). Eyes were stained with fluorescein and imaged multiple times under blue light, at low and high magnifications.
Purpose: To evaluate the influence of active inflammation in uveitis on confocal scanning laser tomography (CSLT) and optical coherence tomography (OCT) measurements.
Methods: Prospective enrollment of patients (64 eyes) with non-infectious uveitis. Retinal nerve fiber layer (RNFL), rim-area and -volume for CSLT and Bruch's-membrane-opening minimum-rim-width (BMO-MRW) and retinal nerve fiber layer thickness (RNFL-O) for OCT were recorded.
Background/aim: To review the long-term outcomes of penetrating keratoplasty (PKP) for corneal complications of herpes zoster ophthalmicus (HZO).
Methods: We reviewed the medical records of 53 eyes of 53 patients who underwent PKP due to corneal complications of HZO at the Kellogg Eye Center.
Results: The mean age of patients at the time of PKP was 68.
Background/aims: To assess the impact of papillary leakage and active inflammation on optical coherence tomography (OCT)-based retinal nerve fibre layer thickness (RNFLT) and Bruch's membrane opening minimum rim width (BMO-MRW) measurements in uveitic eyes with and without secondary glaucoma.
Methods: Prospective, single-centre analysis of patients with uveitis. All patients included received a fluorescein angiography examination and an OCT scan measuring the BMO-MRW and the RNFLT in three concentric peripapillary ring scans.
Current screening and detection of asymptomatic aortic aneurysms is based largely on uniform cut-point diameters. The aims of this study were to define normal aortic diameters in asymptomatic men and women in a community-based cohort and to determine the association between aortic diameters and traditional risk factors for cardiovascular disease. Measurements of the diameters of the ascending thoracic aorta (AA), descending thoracic aorta (DTA), infrarenal abdominal aorta (IRA), and lower abdominal aorta (LAA) were acquired from 3,431 Framingham Heart Study (FHS) participants.
View Article and Find Full Text PDFObjectives: This study sought to determine the accuracy of plaque pattern assessment by coronary computed tomography angiography (CCTA) to differentiate between early and advanced atherosclerotic lesions as defined by histology.
Background: A ringlike attenuation pattern of coronary atherosclerotic plaques termed as napkin-ring sign (NRS) was described in CCTA of patients who had acute coronary syndrome.
Methods: All procedures were performed in accordance with local and federal regulations and the Declaration of Helsinki.
Purpose: Orbitofrontal and insular epilepsy are difficult to recognize because clinical presentations are variable and surgical approaches remain difficult.
Methods: Literature review and review of our own case series including selected cases regarding the clinical manifestation and diagnostic utility of diagnostic tests in orbitofrontal and insular epilepsy.
Results: Orbitofrontal epilepsy presents with either frontal lobe type seizures with hypermotor automatism or temporal lobe type seizures with oroalimentary and manual automatisms depending on the spread pattern.
Radiology
November 2012
Purpose: To establish an ex vivo experimental setup for imaging coronary atherosclerosis with coronary computed tomographic (CT) angiography, intravascular ultrasonography (US), and optical frequency domain imaging (OFDI) and to investigate their ability to help differentiate early from advanced coronary plaques.
Materials And Methods: All procedures were performed in accordance with local and federal regulations and the Declaration of Helsinki. Approval of the local Ethics Committee was obtained.
Objective: Pericardial adipose tissue (PAT) is a pathogenic fat depot associated with coronary atherosclerosis and cardiovascular events. We hypothesized that higher PAT is associated with coronary high-risk lesions as determined by cardiac CT.
Methods: We included 358 patients (38% female; median age 51 years) who were admitted to the ED with acute chest pain and underwent 64-slice CT angiography.
Background: Main pulmonary artery diameter (mPA) and ratio of mPA to ascending aorta diameter (ratio PA) derived from chest CT are commonly reported in clinical practice. We determined the age- and sex-specific distribution and normal reference values for mPA and ratio PA by CT in an asymptomatic community-based population.
Methods And Results: In 3171 men and women (mean age, 51±10 years; 51% men) from the Framingham Heart Study, a noncontrast, ECG-gated, 8-slice cardiac multidetector CT was performed.
Twelve-lead surface electrocardiography (ECG) and computed tomography (CT) are used to evaluate for myocardial ischemia and coronary artery disease (CAD), respectively. We aimed to determine features on resting ECG that predict coronary artery stenosis by cardiac CT. In 309 acute chest pain patients, we compared the initial triage resting ECG to contrast-enhanced 64-slice cardiac CT angiography.
View Article and Find Full Text PDFIntroduction: The 12-lead surface electrocardiogram (ECG) is commonly used as a noninvasive modality to assess for left atrial enlargement (LAE), but data comparing ECG against cardiac computed tomography (CT) for LAE is lacking. We aimed to determine the diagnostic performance of 6 ECG criteria for LAE as compared with CT left atrial volume (LAV) and index to body surface area (LAVI) as the reference standard.
Materials And Methods: In 339 patients (age: mean ± mean, 53 ± 12 years; 63% male), we evaluated the quantitative ECG parameters of P duration, P to PR segment ratio, P wave area, and P terminal force in lead V1.
Objective: Cardiac computed tomography (CT) is a state-of-the-art technology that provides an accurate noninvasive method to quantify left ventricular mass for analysis of left ventricular hypertrophy (LVH). We aimed to examine seven ECG-based LVH criteria against two CT indexation criteria for LVH: a CT-specific body surface area cutoff and the obesity-independent height criteria.
Methods: In 333 patients (mean age 53 +/- 12 years, 61% men), 64-slice contrast-enhanced CT was performed and 12-lead surface ECG within 24 h.
Objectives: We sought to describe the progression of coronary atherosclerotic plaque over time by computed tomography (CT) angiography stratified by plaque composition and its association with cardiovascular risk profiles.
Background: Data on the progression of atherosclerosis stratified by plaque composition with the use of noninvasive assessment by CT are limited and hampered by high measurement variability.
Methods: This analysis included patients who presented with acute chest pain to the emergency department but initially showed no evidence of acute coronary syndromes.