Faculty Bibliography

Kearns-Sayre syndrome (KSS) is a rare mitochondrial DNA (mtDNA) deletion syndrome that typically presents before 20 years of age and is characterized by chronic progressive external ophthalmoplegia, pigmentary retinopathy, and a combination of cardiac conduction defects, cerebellar ataxia, and elevated cerebrospinal fluid protein levels. The mtDNA defects interfere with oxidative phosphorylation and can affect a number of cellular energy processes in various organs. We report the case of a 15-year-old girl with KSS that was uniquely associated with bilateral, symmetrical exophthalmos.

PURPOSE/OBJECTIVE:To determine whether donor diabetes mellitus (DM) severity is associated with differences in endothelial cell density (ECD) and surgically unsuitable tissue. METHODS:Raw data were obtained from Saving Sight Eye Bank (Kansas City, MO) including 10,454 donated eyes from 5346 eligible donors from July 2014 through May 2017. Donors were grouped into 5 categories by their insulin use and the presence of microvascular end-organ complications. The categories were non-DM (NDM), noninsulin-dependent DM without complications (NIDDMnc), noninsulin-dependent DM with complication (NIDDMc), insulin-dependent DM without complications, and insulin-dependent DM with complication. Outcome variables included ECD and tissue transplant suitability. Mixed effects models were used to adjust for the random effect of repeated measures and fixed effects of donor age, race, lens status, and death to refrigeration and death to preservation times. Interaction effects of DM severity group and donor age and DM severity group and lens status were included in the models. RESULTS:One thousand six hundred eighty-four (32.1%) donors had a diagnosis of DM. Six hundred fifty-eight donors were in the NIDDMnc group, 225 in the NIDDMc group, 404 in the insulin-dependent DM without complication group, and 397 in the insulin-dependent DM with complication group. Compared with non-DM, donors with DM were older (P < 0.001) and more likely to be pseudophakic (P < 0.001). DM severity groups did not affect adjusted ECD at mean donor age. There was no statistically significant ECD interaction between DM severity group and lens status. There was a statistically significant ECD crossover interaction with NIDDMnc and donor age (P < 0.001). In phakic eyes, NIDDMc was associated with a statistically significantly lower odds of transplant suitability (odds ratio 0.62, P = 0.006). CONCLUSIONS:DM severity does not affect lowering adjusted ECD at mean donor age. DM severity and pseudophakia were not associated with lower adjusted ECD. NIDDMnc was associated with an attenuation of the age-dependent decrease in ECD. NIDDMc was associated with decreased transplant suitability in phakic eyes. Future studies should include age, lens status, and interaction effects in their models of ECD and transplant suitability.

PURPOSE/OBJECTIVE:To evaluate possible associations between primary open-angle glaucoma (POAG), dental health, and the oral microbiome. METHODS:Case-control study was conducted at SUNY Downstate. Adult subjects (40 to 87 y) were recruited as POAG cases (n=119) and controls without glaucoma (n=78) based on visual field and optic nerve criteria. Overall 74.6% were African Americans (AA). Information on medical history and oral health was collected and ophthalmologic examinations were performed. Mouthwash specimens (28 AA cases and 17 controls) were analyzed for bacterial DNA amounts. Analyses were limited to AAs as the predominant racial group. Outcome measures included number of natural teeth, self-reported periodontal health parameters, and amounts and prevalence of oral bacterial species. Logistic regression was used to evaluate associated factors and potential interactions. RESULTS:Cases and controls had similar age (mean: 62.2 and 60.9 y, respectively, P>0.48), and frequency of hypertension, diabetes, but cases had a higher proportion of men (P<0.04). On average (±SD), cases had fewer natural teeth than controls [18.0 (±11.1) vs. 20.7 (±9.4)]. Having more natural teeth was inversely associated with POAG, in multivariable analyses, at older ages [eg, odds ratio (95% confidence interval) at age 55: 1.0 (0.95-1.06), P=0.98 vs. at age 85: 0.87 (0.79-0.96), P=0.007]. Amounts of Streptococci were higher in cases than controls (P<0.03) in samples from the subset of subjects analyzed. CONCLUSIONS:The number of teeth (an oral health indicator) and alterations in the amounts of oral bacteria may be associated with glaucoma pathology. Further investigation of the association between dental health and glaucoma is warranted.