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Biomedical subjects

R E Kalina

Publications and source records attributed to R E Kalina.

At least 19 recordsLinked to original sources
PubMed1997

Facio-oculo-acoustico-renal (FOAR) syndrome: case report and review.

We report on an 11-year-old boy with distinct facial anomalies, iris coloboma, iris hypoplasia, cataract, high myopia, retinal detachment, moderate sensorineural hearing loss, and proteinuria. He appears to have the facio-oculo-acoustico-renal (FOAR) syndrome, a rare familial disorder reported only 4 times previously. In contrast to the other patients, he has normal intellect.

Abnormalities, Multiple↗
PubMed1997

Pigmentary retinopathy in long chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency.

PURPOSE: To define the ophthalmologic findings in long chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency, an inborn error of mitochondrial beta-oxidation. METHOD: Case report. RESULTS: A 5-year-old girl with long chain 3-hydroxyacyl-CoA dehydrogenase deficiency had a bilateral acquired disturbance of the retinal pigment epithelium consisting of a central macular spot and regularly spaced peripheral spots. Central and peripheral vision and dark adaptation appeared to be mildly compromised. Electroretinography showed abnormalities of the cone system. CONCLUSIONS: An excess of long chain and very long chain fatty acid intermediates has been postulated as the cause of the retinopathy in long chain 3-hydroxyacyl-CoA dehydrogenase deficiency and the biochemically related peroxisomal disorders. Dietary management may slow or halt progression. Ophthalmoscopic detection of regularly spaced pigment spots could help identify long chain 3-hydroxyacyl-CoA dehydrogenase deficiency in future cases.

3-Hydroxyacyl CoA Dehydrogenases↗
PubMed1997

Seeing into the future. Vision and aging.

The leading causes of visual impairment in North Americans are age-related, but appropriate care can preserve useful vision for most older adults. Cataract surgery is highly successful. Early detection and treatment of glaucoma can prevent vision loss. Laser treatment is remarkably effective against diabetic retinopathy. Vision loss due to macular degeneration cannot be delayed in all patients, but low-vision rehabilitation can maximize the usefulness of remaining sight.

Adaptation, Physiological↗
PubMed1996

Recommendations for the use of routine bone marrow aspiration and lumbar punctures in the follow-up of patients with retinoblastoma.

PURPOSE: Patients with metastatic retinoblastoma have a poor outcome. Hope that early detection of extraocular spread will improve survival has led to routine monitoring with bone marrow and cerebrospinal fluid (CSF) examinations. In light of cost and patient morbidity, the clinical utility of this practice is questioned. PATIENTS AND METHODS: We have performed 254 serial bone marrow aspirations and 164 lumbar punctures in 60 children with retinoblastoma. RESULTS: Two patients with extensive intraocular disease at diagnosis developed positive bone marrow aspirations, although no patient died of distant metastasis. Three patients developed positive CSF examinations. All had neurologic symptoms at the time of CSF positivity. CONCLUSIONS: We recommend performing staging bone marrow and CSF evaluations only in patients with clinical, histologic, or radiologic evidence of local or systemic extension (Pratt stage III-IV), or in patients presenting with one Reese-Ellsworth group V eye and retrolaminar or extrascleral extension of their tumor. We recommend limiting follow-up bone marrow and CSF evaluations to patients who develop objective signs and symptoms of metastatic or regionally recurrent disease.

Biopsy, Needle↗
PubMed1994

Visual outcomes of macular retinoblastoma after external beam radiation therapy.

PURPOSE: To determine the risk factors that influence the visual outcomes of patients with macular retinoblastoma who are treated with radiation therapy. METHODS: The medical records of all patients with macular retinoblastoma treated with radiation therapy between 1980 and 1990 were reviewed. Ten patients were entered into the study. Features analyzed included patient age, laterality of eye involvement, location and size of macular tumor(s) at the time of diagnosis, treatment course, and most recent visual acuity. FINDINGS: Ten of 11 eyes (10 patients) were successfully treated with external beam radiation. Eight patients obtained visual acuities ranging from 20/25 to 20/100; two patients had visual acuities of 20/200 or less. The best visual acuities were noted in patients whose tumor(s) did not involve the fovea and were relatively small. The worse visual acuities were noted in patients with binocular vision whose tumors invaded the fovea and were larger in size. In two of three patients in whom both eyes were retained, superimposed amblyopia developed in the eye with macular retinoblastoma. CONCLUSION: The authors' findings indicate that most patients with macular retinoblastoma who are treated with external beam radiation have favorable visual outcomes, but final visual acuity depends on the size of the tumor and involvement of the fovea. Patients in whom both eyes are retained are predisposed to further visual loss from amblyopia.

Eye Neoplasms↗
PubMed1993

Aging of the human photoreceptor mosaic: evidence for selective vulnerability of rods in central retina.

PURPOSE: Because previous studies suggested degeneration and loss of photoreceptors in aged human retina, the spatial density of cones and rods subserving the central 43 degrees of vision as a function of age was determined. METHODS: Cones and rods were counted in 27 whole mounted retinas from donors aged 27 to 90 years with macroscopically normal fundi. Photoreceptor topography was analyzed with new graphic and statistical techniques. RESULTS: Changes in cone density throughout this age span showed no consistent relationship to age or retinal location, and the total number of foveal cones was remarkably stable. In contrast, rod density decreased by 30%, beginning inferior to the fovea in midlife and culminating in an annulus of deepest loss at 0.5 to 3 mm eccentricity by the ninth decade. Space vacated by dying rods was filled in by larger rod inner segments, resulting in a similar rod coverage at all ages. At the temporal equator, cone density declined by 23%, but rods were stable throughout adulthood. CONCLUSIONS: The stability of both rod coverage and rhodopsin content despite decreasing cell number suggests plasticity of the adult rod system and that age-related declines in scotopic sensitivity may be due to postreceptoral factors. There is no evidence for the massive loss of foveal cones required to explain even modest decrements in acuity, consistent with evidence that visual deficits at high photopic levels may be largely due to optical factors. Why the rods of central retina, which share a common support system and light exposure with the neighboring cones, are preferentially vulnerable to aging remains to be determined.

Adolescent↗
PubMed1992

Effects of povidone-iodine chemical preparation and saline irrigation on the perilimbal flora.

PURPOSE: To analyze the effects of 5% povidone-iodine preparation and saline irrigation on the species composition of perilimbal flora. METHODS: Cultures were taken from the perilimbal conjunctiva in 100 eyes before preparation for ophthalmic surgery, after instillation of povidone-iodine solution, and after saline irrigation. RESULTS: Bacteria were isolated in 75% of eyes before preparation, in 28% after povidone-iodine instillation, and in 24% after saline irrigation. Fifty-one culture-positive eyes became negative with povidone-iodine, while only four culture-negative eyes became culture-positive (P < 0.001). The number of eyes yielding coagulase-negative staphylococci, Staphylococcus aureus, and Propionibacterium were significantly decreased after povidone-iodine instillation. Twenty-three culture-positive eyes became negative after saline irrigation, while 19 culture-negative eyes became culture-positive (P > 0.25). CONCLUSION: Povidone-iodine solution is effective in reducing bacterial recovery from the perilimbal conjunctiva, where most incisions for intraocular surgery occur. Saline irrigation after povidone-iodine preparation has no significant effect.

Bacteria↗
PubMed1991

An evaluation of saline irrigation and comparison of povidone-iodine and antibiotic in the surface decontamination of donor eyes.

We evaluated the effect of saline irrigation on 38 pairs of donor eyes and determined the relative efficacy of antibiotic rinse/storage (neomycin-polymyxin B-gramicidin) and povidone-iodine immersion. Microbial growth was found in 49 (64.5%) of 76 eyes from which cultures were taken before irrigation, and only four (8.2%) became culture negative after irrigation. Of 27 eyes culture negative before irrigation, 15 (55.6%) became positive after irrigation. One eye of each pair was then assigned randomly to antibiotic rinse/storage and the other eye to 3-minute immersion in 1% povidone-iodine. Both antimicrobial treatments decreased the numbers of positive cultures, with povidone-iodine showing an advantage that did not reach significance. These results demonstrate that saline irrigation of donor eyes in situ does not reduce surface contamination and that povidone-iodine immersion should be considered as an alternative for donor eye decontamination.

Anti-Bacterial Agents↗
PubMed1990

Human photoreceptor topography.

We have measured the spatial density of cones and rods in eight whole-mounted human retinas, obtained from seven individuals between 27 and 44 years of age, and constructed maps of photoreceptor density and between-individual variability. The average human retina contains 4.6 million cones (4.08-5.29 million). Peak foveal cone density averages 199,000 cones/mm2 and is highly variable between individuals (100,000-324,000 cones/mm2). The point of highest density may be found in an area as large as 0.032 deg2. Cone density falls steeply with increasing eccentricity and is an order of magnitude lower 1 mm away from the foveal center. Superimposed on this gradient is a streak of high cone density along the horizontal meridian. At equivalent eccentricities, cone density is 40-45% higher in nasal compared to temporal retina and slightly higher in midperipheral inferior compared to superior retina. Cone density also increases slightly in far nasal retina. The average human retina contains 92 million rods (77.9-107.3 million). In the fovea, the average horizontal diameter of the rod-free zone is 0.350 mm (1.25 degrees). Foveal rod density increases most rapidly superiorly and least rapidly nasally. The highest rod densities are located along an elliptical ring at the eccentricity of the optic disk and extending into nasal retina with the point of highest density typically in superior retina (5/6 eyes). Rod densities decrease by 15-25% where the ring crosses the horizontal meridian. Rod density declines slowly from the rod ring to the far periphery and is highest in nasal and superior retina. Individual variability in photoreceptor density differs with retinal region and is similar for both cones and rods. Variability is highest near the fovea, reaches a minimum in the midperiphery, and then increases with eccentricity to the ora serrata. The total number of foveal cones is similar for eyes with widely varying peak cone density, consistent with the idea that the variability reflects differences in the lateral migration of photoreceptors during development. Two fellow eyes had cone and rod numbers within 8% and similar but not identical photoreceptor topography.

Cell Count↗
PubMed1990

Cone dysfunction in a subgroup of patients with autosomal dominant cerebellar ataxia.

Four patients with autosomal dominant cerebellar ataxia and retinal degeneration underwent neurologic and ophthalmologic examinations and computer-assessed corneal electroretinography. Previous reports described progressive panretinal degeneration initially involving the cones and subsequently spreading to the rods. By contrast, all our patients displayed evidence of selective dysfunction of the cone system in one or two successive electroretinograms, irrespective of their age or duration of visual symptoms. Color vision testing, funduscopy, and fluorescein angiography suggested a cone dystrophy. Within the period of observation only one patient showed progression of cone dysfunction. In patients with hereditary ataxias, quantitative electroretinography provides an objective and sometimes early indication of retinal degeneration and helps to characterize it.

Adult↗