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LONG-TERM MULTIMODAL IMAGING OF OCULAR FINDINGS ASSOCIATED WITH THIAMINE-RESPONSIVE MEGALOBLASTIC ANEMIA

Tsui, Edmund; Tauber, Jenna; Barbazetto, Irene; Gelman, Susan K
PURPOSE/OBJECTIVE:To report on 5-year multimodal imaging of ocular findings in a patient with thiamine-responsive megaloblastic anemia. METHODS:Observational case report. RESULTS:A 20-year-old-man with a history of thiamine-responsive megaloblastic anemia demonstrated a symmetric bull's eye maculopathy. Spectral domain optical coherence tomography revealed disruption of the parafoveal ellipsoid zone, fundus autofluorescence demonstrated foveal hypoautofluorescence, and full-field electroretinogram testing revealed a decreased photopic and scotopic response consistent with cone-rod dystrophy. His best-corrected visual acuity remained stable over 5 years at 20/50 in the right eye and 20/40 in the left eye, and visual field testing remained stable over time. CONCLUSION/CONCLUSIONS:Ocular manifestations in thiamine-responsive megaloblastic anemia are uncommon and variable. In this case, multimodal imaging and electroretinogram findings are consistent with cone-rod degeneration. The patient is taking daily thiamine supplementation, and visual acuity, funduscopic examination, spectral domain optical coherence tomography, and autofluorescence remained stable over a 5-year period.
PMID: 29210962
ISSN: 1937-1578
CID: 4457922

THE PEARL NECKLACE SIGN: A Novel Spectral Domain Optical Coherence Tomography Finding in Exudative Macular Disease

Gelman, Susan K; Freund, K Bailey; Shah, Vinnie P; Sarraf, David
PURPOSE:: To report a novel spectral domain optical coherence tomography finding in exudative macular disease, called the pearl necklace sign. METHODS:: A retrospective case series of 21 eyes (20 patients) with chronic exudative maculopathy resulting from age-related macular degeneration, diabetic macular edema, branch retinal vein occlusion, retinal arterial macroaneurysm, and Coats disease. Spectral domain optical coherence tomography images were carefully evaluated and correlated with color fundus photography, near-infrared reflectance, and fluorescein angiography. RESULTS:: A unique spectral domain optical coherence tomography macular finding of hyperreflective dots in a contiguous ring around the inner wall of cystoid spaces in the outer plexiform layer of the retina that the authors refer to as the pearl necklace sign was seen in all patients. Visual acuity ranged from 20/30 to hand motions. The cystoid spaces and the hyperreflective dots resolved in certain cases after anti-vascular endothelial growth factor therapy and/or focal macular laser, but tended to recur. CONCLUSION:: Because the pearl necklace configuration can be found adjacent to hard exudates in the outer plexiform layer, the authors speculate that the hyperreflective material is composed of lipoproteins or lipid-laden macrophages. This novel spectral domain optical coherence tomography sign gives further insight into the development and progression of hard lipoprotein exudates in exudative maculopathy.
PMID: 25020214
ISSN: 0275-004x
CID: 1075032

Cross-cultural differences in children's beliefs about the objectivity of social categories

Diesendruck, Gil; Goldfein-Elbaz, Rebecca; Rhodes, Marjorie; Gelman, Susan; Neumark, Noam
The present study compared 5- and 10-year-old North American and Israeli children's beliefs about the objectivity of different categories (n = 109). Children saw picture triads composed of two exemplars of the same category (e.g., two women) and an exemplar of a contrasting category (e.g., a man). Children were asked whether it would be acceptable or wrong for people in a different country to consider contrasting exemplars to be the same kind. It was found that children from both countries viewed gender as objectively correct and occupation as flexible. The findings regarding race and ethnicity differed in the two countries, revealing how an essentialist bias interacts with cultural input in directing children's conceptualization of social groups.
PMCID:3714352
PMID: 23581723
ISSN: 1467-8624
CID: 1758942

Combined arteriovenous sheathotomy and intraoperative intravitreal triamcinolone acetonide for branch retinal vein occlusion

Hwang, John C; Gelman, Susan K; Fine, Howard F; Chang, Stanley; Del Priore, Lucian V
PURPOSE: To describe the treatment of macular oedema secondary to branch retinal vein occlusion (BRVO) with concomitant pars plana vitrectomy/arteriovenous sheathotomy and intraoperative intravitreal triamcinolone acetonide. METHODS: Retrospective case series. RESULTS: Four eyes of four patients were identified. Patients improved from a mean best-corrected visual acuity of 20/124 at baseline to 20/122 at month 1, 20/83 at month 3, 20/74 at month 6, 20/59 at month 9 and 20/44 at month 12 (p = 0.01). All patients demonstrated improvement in visual acuity and macular oedema on fluorescein angiography and/or optical coherence tomography (OCT). CONCLUSION: For patients with persistent macular oedema due to BRVO refractory to pharmacotherapy and/or laser photocoagulation, concomitant pars plana vitrectomy with arteriovenous sheathotomy and intravitreal triamcinolone may improve best-corrected acuity and reduce macular oedema on fluorescein angiography and OCT.
PMID: 20829319
ISSN: 0007-1161
CID: 1155052

Plus disease in retinopathy of prematurity: quantitative analysis of standard published photograph [Letter]

Gelman, Susan K; Gelman, Rony; Callahan, Alison B; Martinez-Perez, M Elena; Casper, Daniel S; Flynn, John T; Chiang, Michael F
PMID: 20837812
ISSN: 0003-9950
CID: 161737

Retinopathy of prematurity management using single-image vs multiple-image telemedicine examinations

Lajoie, Alexandra; Koreen, Susan; Wang, Lu; Kane, Steven A; Lee, Thomas C; Weissgold, David J; Berrocal, Audina M; Du, Yunling E; Coki, Osode; Flynn, John T; Starren, Justin; Chiang, Michael F
PURPOSE: To compare performance of single-image vs multiple-image telemedicine examinations for retinopathy of prematurity (ROP) diagnosis. DESIGN: Prospective comparative study. METHODS: A total of 248 eyes from 67 consecutive infants underwent wide-angle retinal imaging by a trained neonatal nurse at 31 to 33 weeks and/or 35 to 37 weeks postmenstrual age (PMA) at a single academic institution. Data were uploaded to a web-based telemedicine system and interpreted by three masked retinal specialists. Diagnoses were provided based on single images, and subsequently on multiple images, from both eyes of each infant. Findings were compared to a reference standard of indirect ophthalmoscopy by a pediatric ophthalmologist. Primary outcome measures were recommended follow-up interval, presence of plus disease, presence of type-2 or worse ROP, and presence of visible peripheral ROP. RESULTS: Among the three graders, mean sensitivity/specificity for detection of infants requiring follow-up in less than one week were 0.85/0.93 by single-image examination and 0.91/0.88 by multiple-image examination at 35 to 37 weeks PMA. Mean sensitivity/specificity for detection of infants with type-2 or worse ROP were 0.82/0.95 by single-image examination and 1.00/0.91 by multiple-image examination at 35 to 37 weeks PMA. Mean sensitivity/specificity for detection of plus disease were 1.00/0.86 by single-image examination and 1.00/0.87 by multiple-image examination at 35 to 37 weeks PMA. There were no statistically-significant intragrader differences between accuracy of single-image and multiple-image telemedicine examinations for detection of plus disease. CONCLUSIONS: Single-image and multiple-image telemedicine examinations perform comparably for determination of recommended follow-up interval and detection of plus disease. This may have implications for development of screening protocols, particularly in areas with limited access to ophthalmic care.
PMCID:2580058
PMID: 18547536
ISSN: 0002-9394
CID: 1155012

Variation in appearance of severe zone 1 retinopathy of prematurity during wide-angle contact photography [Letter]

Koreen, Susan; Lopez, Robert; Jokl, Danny H-Kauffmann; Flynn, John T; Chiang, Michael F
PMID: 18474793
ISSN: 0003-9950
CID: 1155022

Evaluation of a computer-based system for plus disease diagnosis in retinopathy of prematurity

Koreen, Susan; Gelman, Rony; Martinez-Perez, M Elena; Jiang, Lei; Berrocal, Audina M; Hess, Ditte J; Flynn, John T; Chiang, Michael F
OBJECTIVE: To measure accuracy and reliability of the computer-based Retinal Image Multiscale Analysis (RISA) system compared with those of recognized retinopathy of prematurity (ROP) experts, for plus disease diagnosis. DESIGN: Evaluation of diagnostic test or technology. PARTICIPANTS: Eleven recognized ROP experts and the RISA image analysis system interpreted a set of 20 wide-angle retinal photographs for presence of plus disease. METHODS: All experts used a secure Web site to review independently 20 images for presence of plus disease. Images were also analyzed by measuring individual computer-based system parameters (integrated curvature [IC], diameter, and tortuosity index) for arterioles and venules and by computing linear combinations and logical combinations of those parameters. Performance was compared with a reference standard, defined as the majority vote of experts. MAIN OUTCOME MEASURES: Diagnostic accuracy was measured by calculating sensitivity, specificity, and receiver operating characteristic area under the curve (AUC) for plus disease diagnosis by each expert, and by each computer-based system parameter, compared with the reference standard. Diagnostic agreement was measured by calculating the mean kappa value of each expert compared with all other experts and the mean kappa value of each computer-based system parameter compared with all experts. RESULTS: Among the 11 experts, sensitivity ranged from 0.167 to 1.000, specificity ranged from 0.714 to 1.000, AUC ranged from 0.798 to 1.000, and mean kappa compared with all other experts ranged from 0.288 to 0.689. Among individual computer system parameters, arteriolar IC had the highest diagnostic accuracy, with sensitivity of 1.000; specificity, 0.846; and AUC, 0.962. Arteriolar IC had the highest diagnostic agreement with experts, with a mean kappa value of 0.578. CONCLUSIONS: A computer-based image analysis system has the potential to perform comparably to recognized ROP experts for plus disease diagnosis.
PMID: 18054630
ISSN: 0161-6420
CID: 161741

Inaccuracy of stated versus measured parental heights

Teitelbaum, Jonathan E; Koreen, Susan; Hightower, Kimberly; Rajaraman, Raj; Jaeger, Joseph
Routine measurement of children's height is essential in monitoring for deviations in normal growth velocity. Target adult height of children is estimated by determining an adjusted midparental height. Such determinations are dependent on the accurate acquisition of parental height. Incorrect assessment of parental height will result in inaccurate expectations for the child's height. To observe the difference between stated and measured parental heights within a United States subspecialty setting, prospective acquisition of parental stated and measured heights during the summer months of 2000 and 2001 was examined. Two hundred and thirty-eight parents, 185 mothers and 53 fathers, were measured. The mean values of the stated heights for mothers and fathers were 163.7 cm +/- 6.3 cm and 177.1 cm +/- 8.6 cm, respectively. The corresponding measured heights were 163.0 cm +/- 5.9 cm and 175.2 cm +/- 6.6 cm, respectively. The mean height difference for mothers was 0.69 cm (p<0.001) while the mean height difference for fathers was 1.90 cm (p<0.001). Parents significantly overestimate their height in a clinical setting. Fathers overestimate to a greater degree than mothers. These findings emphasize the need to obtain height measurement of parents along with that of their children in assessing for linear growth delay.
PMID: 15864367
ISSN: 0009-9228
CID: 1155032

Effects of repeated Valsalva maneuver straining on cardiac and vasoconstrictive baroreflex responses

Convertino, Victor A; Ratliff, Duane A; Doerr, Donald F; Ludwig, David A; Muniz, Gary W; Benedetti, Erik; Chavarria, Jose; Koreen, Susan; Nguyen, Claude; Wang, Jeff
INTRODUCTION: We hypothesized that repeated respiratory straining maneuvers (repeated SM) designed to elevate arterial BPs (arterial baroreceptor loading) would acutely increase baroreflex responses. METHODS: We tested this hypothesis by measuring cardiac baroreflex responses to carotid baroreceptor stimulation (neck pressures), and changes in heart rate and diastolic BP after reductions in BP induced by a 15-s Valsalva maneuver in 10 female and 10 male subjects at 1, 3, 6, and 24 h after performing repeated SM. Baroreflex responses were also measured in each subject at 1, 3, 6, and 24 h at the same time on a separate day without repeated SM (control) in a randomized, counter-balanced cross-over experimental design. RESULTS: There was no statistical difference in carotid-cardiac and peripheral vascular baroreflex responses measured across time following repeated SM compared with the control condition. Integrated cardiac baroreflex response (deltaHR/ deltaSBP) measured during performance of a Valsalva maneuver was increased by approximately 50% to 1.1 +/- 0.2 bpm x mm Hg(-1) at 1 h and 1.0 +/- 0.1 bpm x mm Hg(-1) at 3 h following repeated SM compared with the control condition (0.7 +/- 0.1 bpm x mm Hg(-1) at both 1 and 3 h, respectively). However, integrated cardiac baroreflex response after repeated SM returned to control levels at 6 and 24 h after training. These responses did not differ between men and women. CONCLUSIONS: Our results are consistent with the notion that arterial baroreceptor loading induced by repeated SM increased aortic, but not carotid, cardiac baroreflex responses for as long as 3 h after repeated SM. We conclude that repeated SM increases cardiac baroreflex responsiveness which may provide patients, astronauts, and high-performance aircraft pilots with protection from development of orthostatic hypotension.
PMID: 12650267
ISSN: 0095-6562
CID: 1155042