Woon Cho Kim

Exacerbation of Avellino corneal dystrophy after LASIK in North America.

Purpose: To report the first case of Avellino corneal dystrophy exacerbation after LASIK in a white or North American patient. Methods: Case report and literature review. Results: A 25-year-old white female developed progressive corneal opacities after LASIK. Preoperative examination had revealed only subtle white corneal opacities in each eye. The patients mother had similar corneal opacities. DNA analysis of the patient revealed a heterozygous mutation at the R124H location in the BIGH3 gene. Conclusions: LASIK can exacerbate Avellino corneal dystrophy and should be avoided in patients with this condition. A careful history and genetic analysis can identify affected patients and those at risk.

Prevalence of Granular Corneal Dystrophy Type 2 (Avellino Corneal Dystrophy) in the Korean Population

Purpose: This study investigates the prevalence of granular corneal dystrophy type 2 (GCD2; Avellino corneal dystrophy) in the Korean population. Methods: GCD2 homozygotes were identified through a collaboration of Korean referral centers for corneal disease. The genetic status of the patients and their immediate families were verified by DNA analysis. A lower bound for the gene prevalence was calculated using a model based on the Hardy-Weinberg principle. A second population-based model was developed to correct for known underestimation in the primary model. The corrected model used population data from the 2005 Korean census and fertility rates from historical Korean census data. Results: We identified 21 individuals homozygous for GCD2 (R124H mutation) from 16 Korean families. From this, we estimate that the overall prevalence (combining heterozygotes and homozygotes) is at least 8.25 affected persons/10,000 persons. Our corrected estimate for overall prevalence is 11.5 affected persons/10,000 persons. Conclusion: We present the first estimate of the prevalence of GCD2. Although uncommon, the prevalence of GCD2 in Korea is greater than anticipated. We believe that our approach could potentially be applied to estimating the prevalence of other rare diseases.

Avellino corneal dystrophy exacerbated after LASIK: scanning electron microscopic findings.

Purpose: To evaluate the ultrastructure of the cornea of Avellino corneal dystrophy (ACD) exacerbated by LASIK. Methods: Three ACD patients with exacerbation of granular corneal deposits after LASIK underwent surgical removal of the corneal flap. The corneal flap was processed for scanning electron microscopy (SEM). Results: SEM of all patients showed abnormal granular clusters in the fibrils of the corneal flap. Conclusion: Laser in situ keratomileusis induces corneal collagen abnormalities and adhesions of granular material in ACD patients.

Quality of diabetes care in low- and middle-income Asian and Middle Eastern countries (1993-2012): 20-year systematic review.

OBJECTIVE To assess the extent to which people with diabetes in low- and middle-income countries (LMIC) of Asia and the Middle East met evidence-based care recommendations through a systematic review of published literature. METHODS Electronic searches of Medline and Embase were carried out for studies assessing quality of care among people with diabetes in Asia and the Middle East between 1993 and 2012. Benchmarking against American Diabetes Association guidelines, we reported level and proportions meeting recommended risk factor control (glycated hemoglobin [HbA1c], blood pressure, and low density lipoprotein-cholesterol [LDL]) and preventive care processes across different settings. RESULTS One hundred and fifteen publications met eligibility for inclusion (91 reported risk factor control, 7 reported preventive processes, and 17 reported both). Only China, Thailand, Malaysia and Philippines had nationally representative data. Mean HbA1c (6.5-11% or 48-97 mmol/mol), SBP (120-152 mm Hg), and LDL (2.4-3.8 mmol/l) varied greatly. Despite variation in availability of data, studies consistently showed that recommended care goals were not being achieved. CONCLUSIONS The practice of auditing and benchmarking against evidence-based guidelines appears to be uncommon in Asia and the Middle East and there was heterogeneity of reporting across studies, populations, and methods used. The available data showed inadequate care.

Effects of corneal neovascularization on the manifestations of Avellino corneal dystrophy (granular corneal dystrophy type II).

Purpose: To evaluate the corneal deposits of Avellino corneal dystrophy (ACD) in patients with corneal neovascularization from pterygium or phthisis bulbi as a way of understanding the pathogenesis of ACD. Methods: Five patients with ACD with pterygium, 10 patients with ACD (age >50 years) without pterygium, 1 patient with ACD with phthisis bulbi with corneal neovascularization, and 1 patient with ACD with phthisis bulbi without corneal neovascularization were examined. The corneal deposits of all patients were assessed by slit-lamp examination and reviewed with biomicroscopic photographs. The distance between the limbus and the nearest corneal opacities was measured. Results: In eyes with vascularized nasal pterygia, there was a granule-free zone adjacent to the advancing edge of the pterygium so that the distance between the nasal limbus and the most nasally located granule exceeds that of the distance between the limbus and the closest granule elsewhere on the cornea. In patients with ACD with phthisis bulbi, no granular deposits were observed in the cornea with neovascularization, but there were deposits in the cornea without neovascularization. Conclusion: Corneal neovascularization prevents the deposition of corneal opacities in patients with ACD.

Developing a conceptual and practical framework for outcomes measurement to support a quality improvement project in Rwanda: an observational study

Abstract Background A landmark study in 2009 demonstrated that a WHO surgical safety checklist decreased mortality and complications in surgical patients. Attempts to replicate this in the developing world have been mixed. We piloted the implementation of a surgical safety checklist with outcomes measurement in one district hospital in Rwanda, with the goal of creating a scalable model to use in all public hospitals in the country. Methods We sought to develop a conceptual and practical framework for ongoing outcomes measurement that can be applied to quality improvement projects in resource-poor settings. We accomplished this through interviews with global checklist implementation teams and Rwandan hospital staff, as well as observation. Findings We developed the following framework with specific examples from our experience. (1) Define the elements of data collection and who will perform each. In particular, the data audit function must be carefully outlined. (2) Understand the potential motivators for each participant in data collection and align incentives; these may be financial or non-financial. (3) Choose a data collection training method including initiation, maintenance, and troubleshooting. (4) Select a database software considering the following: availability of consistent or intermittent internet access, user interface complexity, flexibility of software for Mac and PC operating systems, need for mobile access, initial and ongoing costs, and technical support. Based on these criteria, we chose to pilot a new offline version of REDCap, which may have important implications for global health projects in settings without consistent internet access. (5) Be flexible. The framework provides a best first guess at the methods that will work for high-quality data collection, but a willingness to change course is critical. Interpretation Monitoring outcomes in quality improvement projects in resource-poor settings is difficult, but a framework makes it possible to effectively plan, initiate, and sustain data collection. Funding Lifebox Foundation, London, UK.