Esen Karamursel Akpek

Johns Hopkins Medicine, Baltimore, Maryland, United States

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Publications (115)385.5 Total impact

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    Anisa I. Gire · Sezen Karakus · Shanna M. Ingrodi · Esen Karamursel Akpek
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    ABSTRACT: Purpose: To study the systemic safety and patient tolerability of frequent dosing of cyclosporine A (CsA) 0.05% eyedrops in the treatment of ocular surface disease. This is a retrospective case series. Patients with significant ocular surface diseases who were treated using topical CsA higher than the usual twice daily dosing (3–8 times daily and over a treatment period of 1–70 months). The main outcome measures are plasma levels of CsA and local tolerability. Methods: Symptom assessment, corneal staining using fluorescein, conjunctival staining using lissamine green, tear film breakup time, and other signs according to the disease process were monitored. Discontinuation of treatment due to intolerability was recorded. CsA levels were measured in the plasma at a clinical laboratory. Results: Plasma levels of CsA were below the level of detection (7 ng/mL) in all the 41 patients included. All patients tolerated the treatment well with none discontinuing due to any treatment-related local adverse effects. Conclusions: This study demonstrates that CsA 0.05% ophthalmic emulsion applied more frequently than the usual twice daily dosing was safe and well tolerated in patients with significant ocular surface diseases.
    Full-text · Article · Jan 2016 · Journal of Ocular Pharmacology and Therapeutics
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    ABSTRACT: The intent of this review is to comprehensively appraise the state of the art with regard to Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), with particular attention to the ocular surface complications and their management. SJS and TEN represent two ends of a spectrum of immune-mediated, dermatobullous disease, characterized in the acute phase by a febrile illness followed by skin and mucous membrane necrosis and detachment. The widespread keratinocyte death seen in SJS/TEN is rapid and irreversible, and even with early and aggressive intervention, morbidity is severe and mortality not uncommon. We have divided this review into two parts. The purpose of Part I is to summarize the epidemiology and immunopathogenesis of SJS/TEN and discuss systemic therapy and its possible benefits. We hope this review will help the ophthalmologist better understand the mechanisms of disease in SJS/TEN, and by extension, better care for patients with this complex and often debilitating disease.
    Full-text · Article · Nov 2015
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    ABSTRACT: Purpose: To compare repeat penetrating keratoplasty (PK) with Boston type I keratoprosthesis (KPro) implantation for full-thickness donor corneal graft failure. Design: Previous donor graft failure is a common indication for both PK and KPro implantation. Selection of the surgical procedure is entirely dependent on the surgeon because there are no studies available for guidance. Therefore, a systematic review was undertaken to examine vision, device retention, graft clarity, and postoperative glaucoma and infection outcomes after repeat PK versus KPro implantation. Methods: Articles with data regarding repeat PK published between 1990 and 2014 were identified in PubMed, EMBASE, the Latin American and Caribbean Health Sciences Literature Database, and the Cochrane Central Register of Controlled Trials and were reviewed. Results were compared with a retrospective review of consecutive, nonrandomized, longitudinal case series of KPro implantations performed at 5 tertiary care centers in the United States. Visual acuity at 2 years was the primary outcome measure. The proportion of clear grafts in the repeat PK group, device retention in the KPro group, and the development of postoperative glaucoma and infection were secondary outcome measures. Results: The search strategy identified 17 128 articles in the PK analysis. After screening, 26 studies (21 case series and 5 cohort studies) were included in the review. Pooled analysis of the 26 unique studies demonstrated a 42% (95% confidence interval [CI], 30%-56%) likelihood of maintaining 20/200 or better at 2 years after repeat PK, compared with an 80% (95% CI, 68%-88%) probability with KPro implantation. The probability of maintaining a clear graft at 5 years was 47% (95% CI, 40%-54%) after repeat PK, whereas the probability of retention of the KPro at 5 years was 75% (95% CI, 64%-84%). The rate of progression of glaucoma at 3 years was 25% (95% CI, 10%-44%) after repeat PK and 30% in the KPro cohort. Conclusions: These results demonstrate favorable outcomes of KPro surgery for donor corneal graft failure with a greater likelihood of maintaining visual improvement without higher risk of postoperative glaucoma compared with repeat donor PK.
    No preview · Article · Nov 2015 · Ophthalmology
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    ABSTRACT: Purpose: To describe the outcomes and prognostic characteristics of patients who had a repeat Boston type 1 Keratoprosthesis (KPro) implantation. Design: Retrospective case series. Methods: Setting: Data regarding pre-operative clinical and demographic characteristics and postoperative course during initial and repeat KPro placement were collected at multiple centers across the country. Patients: Forty-eight eyes underwent explantation of KPro due to complications between January 2003 and August 2014 at 5 participating tertiary eye care centers in the United States. Of those, 36 eyes that received a subsequent replacement device were included. Main outcome measures: Visual acuity (VA) outcomes and postoperative complications. Results: Ocular surface disease was significantly more common in eyes that required a device explantation, compared to those who retained the device (p<0.001). Sixty-seven percent of eyes (24/36) achieved VA ≥20/200 vision after the repeat KPro. The probability of these 24 eyes maintaining VA ≥20/200 after the repeat KPro was 87% at 1 year and 75% at 2 years. Predictors of the ability to maintain vision ≥20/200 following surgery were a better last-recorded vision before explantation (p=0.0002) and better vision immediately after repeat KPro (p<0.001). Conclusion: Ocular surface disease and its complications were associated with more frequent device removal. In these patients, repeat KPro resulted in restoration of vision. A reasonable visual acuity prior to device removal was associated with favorable long-term post-operative visual acuity and retention.
    No preview · Article · Oct 2015 · American Journal of Ophthalmology
  • Allen O Eghrari · Sumayya Ahmad · Pradeep Ramulu · Nicholas T Iliff · Esen Karamursel Akpek
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    ABSTRACT: Purpose: The Boston keratoprostheses type 1 devices (KPro) are utilized in cases unfavorable to penetrating keratoplasty. The prognosis remains guarded in cases of ocular surface disease due to risk of tissue necrosis. We describe a novel surgical approach using a conjunctival flap with a delayed opening to improve retention. Methods: In three patients with advanced cicatrizing conjunctivitis, a Type 1 keratoprosthesis was stabilized using a full tarsal conjunctival flap. Three months postoperatively, an opening was created in the flap overlying the optical portion of the device. Results: All patients had no device related complications over a mean follow-up period of 17.7 months (range 15-21 months) and vision remained excellent at better than 20/200 for all patients. Conclusions: Utilization of a tarsal flap either primarily as part of a two stage modified technique or secondarily in cases of tissue necrosis and impending device extrusion might maximize retention of the type 1 KPro.
    No preview · Article · Sep 2015 · Ocular immunology and inflammation
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    ABSTRACT: To determine the intra-observer and inter-observer reliability of a novel ocular staining score among trained ophthalmologists. Reliability analysis within a prospective, observational, multi-center cohort study. Those enrolled in the National Institutes of Health-funded Sjögren's International Collaborative Clinical Alliance (SICCA) who presented for follow up at the University of California San Francisco, Aravind Eye Hospital, Johns Hopkins University, and the University of Pennsylvania were included. Study participants were graded using the ocular staining score by at least two masked SICCA-trained ophthalmologists. The primary outcome for this study was the intraclass correlation coefficient (ICC) for the total ocular staining score. ICC's were also calculated for tear break up time (TBUT), conjunctival and corneal staining. Total ocular staining score had an ICC of 0.91 for the right eye (95% CI 0.85 - 0.96) and 0.90 for the left eye (95% CI 0.83 - 0.97). Corneal staining (right eye 0.86, 95% CI 0.76 - 0.93, left eye 0.90, 95% CI 0.81 - 0.95) and conjunctival staining (right eye 0.87, 95% CI 0.80 -0.93, left eye 0.85, 95% CI 0.75 - 0.93) demonstrated excellent agreement. The ICC for TBUT was slightly lower (right eye 0.77, 95% CI 0.64 - 0.89; left eye 0.81, 95% CI 0.68 - 0.90). Previous studies have shown that the ocular staining score is correlated with other diagnostic components of Sjögren syndrome. In this study, we demonstrate high reliability in grading among trained ophthalmologists, completing the validation of this test. Copyright © 2015 Elsevier Inc. All rights reserved.
    No preview · Article · Aug 2015 · American Journal of Ophthalmology
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    Michelle Hessen · Sezen Karakus · Esen Karamursel Akpek

    Full-text · Article · Jul 2015
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    ABSTRACT: To report two patients with sarcoidosis initially presenting with cicatrizing conjunctivitis. Both patients with chronic conjunctivitis were referred for further management. The first patient had conjunctival granulomas, subepithelial fibrosis, and forniceal foreshortening. The second patient had extensive upper and lower conjunctival scarring with forniceal foreshortening and symblepharon formation of both eyes. Conjunctival biopsy specimens revealed noncaseating granulomas. Immunofluorescein studies were negative for immunoreactant deposition in the basement membrane. Because of further diagnostic evaluations, sarcoidosis was determined to be the final cause of the cicatrizing conjunctivitis. Sarcoidosis should be included in the differential diagnosis of cicatrizing conjunctivitis. Recognition of the characteristic noncaseating granulomas in the conjunctival biopsy and initiation of the appropriate evaluations are essential in establishing the diagnosis and determining the extent of systemic involvement.
    Full-text · Article · Jun 2015 · Optometry and vision science: official publication of the American Academy of Optometry
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    ABSTRACT: To report the ocular complications of primary Sjögren's Syndrome (SS) in men. Retrospective cohort study. Setting: Tertiary-care SS center. 163 consecutive primary Sjögren's Syndrome patients evaluated between January 2007 and March 2013. Frequency of extraglandular ocular and systemic manifestations and serological results in men compared to women. 14 (9%) of the 163 primary Sjögren's Syndrome patients were men. On initial presentation, men were a decade older (61 vs 50 years, p<0.01) and less likely than women to have a prior diagnosis of SS (43% vs 65%, p=0.09). A majority of men reported dry eye on presentation (92%), albeit less chronic compared to women (5.9 vs 10.8 years, p=0.07). Men were more likely to present with serious ocular complications than women (43% vs 11%, p=0.001). Extraglandular systemic complications of SS (i.e. vasculitis, interstitial nephritis) were also more common in men (64% vs 40%, p=0.07). Further, men were more likely to be negative for anti-SSA/Ro, anti-SSB/La, and antinuclear antibodies than women (36% men vs 11% women, p=0.01). Men with primary SS have a higher frequency of serious ocular and systemic manifestations. Although primary Sjögren's Syndrome is typically considered a disease of middle-aged women, it may be underdiagnosed and consequentially more severe in men. Physicians should have a lower threshold to test for SS in men with dry eye. Copyright © 2015 Elsevier Inc. All rights reserved.
    No preview · Article · Jun 2015 · American Journal of Ophthalmology
  • Divya Srikumaran · Beatriz Munoz · Esen Karamursel Akpek

    No preview · Article · May 2015 · Ophthalmology
  • Sang Beom Han · Karen Dunlap · Esen Karamursel Akpek
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    ABSTRACT: Corneal opacity in pediatric patients may be congenital or acquired. Significant corneal opacities in infants can lead to profound deprivation amblyopia. For this reason, early surgical intervention to provide a clear visual axis is recommended. Surgery usually involves a full thickness penetrating keratoplasty (PK). Although PK results are excellent in most adult patients, pediatric cases are not as successful due to the technical difficulty of this surgery, higher rates of graft failure from allograft rejection, and challenges in postoperative visual rehabilitation. The Boston type 1 keratoprosthesis (KPro), once considered a last resort in patients with multiple PK failures, is now considered a reasonable alternative. It even can be the first-line option for cases known to be at high risk for failure with traditional donor corneal transplantation. Thus far, studies of adult patients have shown favorable outcomes with the KPro, with rapid visual recovery and excellent retention rates. KPro may also be useful in the treatment of pediatric corneal opacity as it can restore a clear visual axis quickly and thus may be useful in amblyopia prevention. It does not opacify and cannot be rejected. In addition, the built in refractive rehabilitation even in aphakic patients is advantageous, particularly in pediatric cases. Initial reports from a few centers demonstrate encouraging results from pediatric KPro surgeries.
    No preview · Article · Jan 2015
  • E.K. Akpek · S.D. Cassard · K. Dunlap · S. Hahn · P.Y. Ramulu
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    ABSTRACT: Purpose: To compare short-term outcomes of repeat penetrating keratoplasty (PK) to those of Boston type 1 keratoprosthesis (KPro). Our hypothesis was that visual outcomes were superior for KPro compared to PK. Methods: This is a retrospective, nonrandomized, intermediate-term case series. Consecutive adults with one or more failed PKs who underwent either PK or KPro between January 2008 and December 2010 were included. Demographics, indication for the initial PK, comorbidities, concomitant procedures, and complications were considered. Only one procedure in each eye was included. All KPro procedures were retained in the analyses. Results: Fifty-three patients underwent PK and 27 received KPro. Mean follow-up was 19.5 months in the PK group and 16.5 months in the KPro group. KPro eyes had worse mean preoperative vision (hand motions vs counting fingers, P=.01) and more comorbidities. In the postoperative period, 35% of PK eyes and 45% of KPro eyes attained best-ever visual acuity of 20/70. Forty-seven percent of PK eyes vs 40% of KPro eyes were able to retain this visual acuity. Two-year rate of failure to retain visual acuity better than the baseline was higher for PK eyes, though not at a statistically significant level (hazard ratio [HR]=1.67; 95% CI, 0.78-3.60; P=.19). Two-year cumulative rate of graft failure (loss of clarity for PK and removal/replacement for KPro) was higher for PK eyes (HR=3.23; 95% CI, 1.12-9.28; P=.03). Retinal detachment, endophthalmitis, and glaucoma rates were similar (P=.6 for all). These results demonstrate less frequent graft failure, greater visual improvement, and greater likelihood of maintaining the visual improvement in KPro eyes vs PK.
    No preview · Article · Jan 2015 · Transactions of the American Ophthalmological Society
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    ABSTRACT: To identify predictors of visual outcomes following Boston type 1 keratoprosthesis (KPro) implantation. Retrospective chart review. Data regarding pre-operative clinical and demographic characteristics and postoperative course were collected. Patients: Fifty-nine eyes of 59 adult patients who underwent KPro implantation between January 2006 and March 2012 at a single tertiary care center. Main Outcome Measures: Pre-operative factors associated with all-cause and glaucoma-related loss of visual acuity from the best postoperative visual acuity noted. Fifty two of 59 eyes (88%) achieved improved vision post implantation, with 7 eyes failing to gain vision as a result of pre-existing glaucoma (n=4) or retino-choroidal disease (n=3). Twenty one eyes (21/52, 40%) maintained their best ever visual acuity at last visit (mean follow-up period was 37.8 months). The likelihood of maintaining best ever vision was 71% at 1 year, 59% at 2 years and 48% at 3 years. Primary KPro implantation was associated with a higher likelihood of losing best ever vision as compared to KPro implantation as a repeat corneal procedure (Hazard Ratio [HR]=3.06; p=.006). The main reasons for post-implantation vision loss was glaucoma (12/31, 39%), and the risk of glaucomatous visual acuity loss was 15% at 2 years and 27% at 3 years. Prior trabeculectomy was associated with a higher rate of vision loss from glaucoma (HR=3.25, p=.04). Glaucoma is the primary reason for loss of visual acuity after KPro implantation. Conditions necessitating primary KPro surgery are associated with more frequent all-cause vision loss. Prospective trials are necessary to better determine which clinical features best predict KPro success. Copyright © 2015 Elsevier Inc. All rights reserved.
    No preview · Article · Dec 2014 · American Journal of Ophthalmology
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    ABSTRACT: Purpose To report vision-threatening ocular manifestations of primary Sjögren’s syndrome (SS). Design Retrospective review. Participants Consecutive patients evaluated at an SS center between January 2007 and May 2011. Methods Data collection was completed in March 2013. The 2002 American-European consensus criteria were used for diagnosis of SS. Main Outcome Measures Frequency of extraglandular ocular findings and timing of their diagnosis relative to that of SS and dry eye were assessed. Results One hundred sixty-three patients were included. Almost all patients (98%) had a history of dry eye for an average of 10.4 years (median, 7.9 years) before presentation. One or more extraglandular ocular manifestations were present in 40 patients (25%), and vision-threatening findings were present in 22 patients (13%). Twelve patients (55%) with a vision-threatening ocular finding did not have a diagnosis of SS at presentation. Sixty-eight patients (42%) had extraglandular systemic manifestations of SS. Patients with vision-threatening ocular findings were 3.9 times more likely to have systemic involvement (95% confidence interval, 1.4–11.0; P = 0.010). Peripheral neuropathy, interstitial nephritis, and vasculitis were more common in those with vision-threatening ocular findings compared with patients without (P < 0.05 for all). Conclusions These results from a tertiary referral-based cohort demonstrate that primary SS frequently is associated with ocular and systemic complications. Dry eye precedes these findings on average by 1 decade. Therefore, ophthalmologists should consider assessing for SS in patients with clinically significant dry eye.
    No preview · Article · Dec 2014 · Ophthalmology
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    ABSTRACT: The aim of this study was to evaluate the clarity of gamma-irradiated sterile corneal donor lenticules. Broadband UV, visible, and near-infrared (200-850 nm) light transmission was measured through gamma-irradiated, sterile partial-thickness, and full-thickness donor lenticules and fresh corneal tissues and compared with standard acrylic intraocular lens (IOL) implants using a conventional spectrophotometer technique. All tissues had high light transmission (≥ 90%) in the visible and near-infrared regions and very low (<2%) transmission below 290 nm. Differences in light transmission between irradiated and fresh cornea types were observed between 300 and 450 nm, which mirrored differences in light transmission through their respective storage solutions. Light transmission through partial-thickness irradiated donor lenticules was greatest across all wavelengths. All corneal tissues exhibited higher transmission than acrylic IOL implant across all wavelengths. Gamma-irradiated donor lenticules are comparable with fresh corneas regarding light transmission, with both partial-thickness and full-thickness lenticules having greater transmission than standard IOL. We would expect the optical performance of gamma-irradiated donor lenticules to be comparable to fresh cornea if used for lamellar corneal procedures that do not require a viable endothelium.
    No preview · Article · Dec 2014 · Cornea
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    ABSTRACT: Individuals who have failed one or more full thickness penetrating keratoplasties (PKs) may be offered repeat corneal surgery using an artificial or donor cornea. An artificial or prosthetic cornea is known as a keratoprosthesis. Both donor and artificial corneal transplantations involve removal of the diseased and opaque recipient cornea (or the previously failed cornea) and replacement with another donor or prosthetic cornea.
    No preview · Article · Nov 2014 · Cochrane database of systematic reviews (Online)
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    ABSTRACT: Purpose: To study the long-term outcomes of Boston type 1 keratoprosthesis (KPro) surgery. Design: Retrospective, multicenter case series. Participants: A total of 158 eyes of 150 patients underwent KPro implantation at 5 participating tertiary centers in the United States between January 2003 and December 2006. Of those, 139 eyes of 133 patients were included in the analyses. Methods: The medical records of consecutive adult patients who received KPro surgery were reviewed. All patients with at least 1 postoperative visit were retained in the outcomes analyses. In eyes in which a repeat KPro procedure was performed, only the outcomes of the initial surgery were analyzed. Main Outcome Measures: Visual acuity (VA) outcomes, postoperative complications, and device retention. Results: The mean follow-up was 46.7 +/- 26 months with all but 4 eyes having at least 6 months of follow-up. Preoperatively, only 10.8% of the eyes had VA of >= 20/200. Postoperatively, the VA in 70% of eyes improved to >= 20/200. The probability of maintaining VA of >= 20/200 at 7 years was 50%. The device retention rate was estimated at 67% at 7 years. The 7-year cumulative incidence of complications was 49.7% for retroprosthetic membrane formation, 21.6% for glaucoma surgery, 18.6% for retinal detachment, and 15.5% for endophthalmitis. Conclusions: Although the risk for complications with longer follow-up seemed to increase, this large multicenter cohort demonstrates favorable outcomes with KPro, with a large number of patients achieving and retaining useful vision over a 7-year period. (C) 2014 by the American Academy of Ophthalmology.
    No preview · Article · Jul 2014 · Ophthalmology
  • Claudia F Henrich · Pradeep Y Ramulu · Esen K Akpek
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    ABSTRACT: Purpose: The aim of this study was to review the frequency and types of inflammatory systemic diseases in a cohort of patients with dry eye, and identify clinical features suggesting the presence of these. Methods: Consecutive new patients with a primary diagnosis of dry eye evaluated at a tertiary dry eye center between January 2010 and December 2011 were reviewed retrospectively. Standardized questionnaires were used to obtain data regarding systemic symptoms, previous medical diagnoses, and family history. Dry eye evaluations included Schirmer testing, tear film break-up time, corneal fluorescein staining, and bulbar conjunctival lissamine green staining. Clinically significant dry eye was defined as having a Schirmer test score without anesthesia of ≤10 mm or conjunctival lissamine green staining of ≥1 using the Oxford scale. Results: A total of 228 new patients were analyzed. Of these, 47.4% (108/228) presented with a known diagnosis of inflammatory disease. Based on a review of systems and ocular examination, 81 patients (81/228) underwent a further work-up that revealed 25 additional diagnoses that were not known on presentation. The most common newly identified conditions included occult thyroid eye disease (n = 20), primary Sjögren Syndrome (4), and Sjögren Syndrome suspect (1). Female gender, family history of autoimmune disease, self-reported joint pain or dry mouth, external signs of orbital inflammation, and conjunctival chemosis were more common in patients with inflammatory systemic disease as compared with that in patients with no identifiable condition (P < 0.05 for all). Conclusions: Systemic inflammatory diseases are frequently associated with dry eye in patients evaluated at a tertiary academic center. Diagnostic evaluations may help uncover previously undiagnosed significant conditions in about one-third of tested patients.
    No preview · Article · Jun 2014 · Cornea
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    ABSTRACT: Extranodal Rosai-Dorfman disease is a rare benign condition recently reported to sometimes show features of IgG4-related disease. The purpose of this study was to describe the corneal-limbal manifestation of the entity and to investigate whether numerous IgG4-positive plasma cells are associated with the disease at this site. This is an interventional retrospective small case series. Two patients presenting with painless limbal mass lesions underwent total excisional biopsy with anterior lamellar keratoplasty for diagnostic and therapeutic purposes. Histopathologic evaluation of the specimens revealed inflammatory lesions containing atypical S100-immunoreactive histiocytes diagnostic of Rosai-Dorfman disease, but not an increase in the IgG4-positive plasma cells. Point mutations (V600E) in the BRAF oncogene were absent. Rosai-Dorfmann disease should be considered in the differential diagnosis of limbal mass lesions. Involvement at this site was not associated with BRAF mutation or IgG4 abnormalities in the cases examined.
    No preview · Article · May 2014 · Cornea

  • No preview · Article · Apr 2014 · Ocular immunology and inflammation

Publication Stats

2k Citations
385.50 Total Impact Points


  • 2000-2015
    • Johns Hopkins Medicine
      • Wilmer Eye Institute
      Baltimore, Maryland, United States
  • 1999-2015
    • Johns Hopkins University
      • • Wilmer Eye Institute
      • • Department of Medicine
      Baltimore, Maryland, United States
  • 2001
    • University of Maryland, Baltimore
      Baltimore, Maryland, United States
  • 1999-2001
    • Harvard University
      Cambridge, Massachusetts, United States
  • 1999-2000
    • Harvard Medical School
      • Department of Medicine
      Boston, Massachusetts, United States
  • 1997-1999
    • Massachusetts Eye and Ear Infirmary
      • • Ocular Immunology and Uveitis Service
      • • Department of Ophthalmology
      Boston, Massachusetts, United States
  • 1996
    • Ankara Numune Training and Research Hospital
      Engüri, Ankara, Turkey