Biomedical subjects
Publications and source records attributed to A B Fulton.
A patient had continued active giant cell arteritis proved on biopsy four years after the initial diagnosis of temporal arteritis. The patient had had erythrocyte sedimentation rates within the normal range and had been receiving corticosteroids without interruption for four years. Central nervous system symptoms occurred three and four years after the initial diagnosis.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Scotopic b-wave stimulus/response (S/R) functions are abnormal in several human retinal degenerative disorders. However, the mechanisms by which diseases affect the S/R parameters are not yet fully known. Three experiments were done to simulate functional pathologies known to occur in retinal degenerations: 1) attenuated sensitivity of retinal units, 2) loss of rhodopsin, 3) loss of sensitivity with little or no loss of rhodopsin. None of the experimental perturbations of normal function replicated the pattern of S/R abnormalities caused by retinal degenerations. Thus, in the retinal degenerative disorders intrinsic abnormalities of cellular processing must affect the organization of distal retinal function indexed by the b-wave.
Ametropias, particularly myopia, and mild retinal dysfunction are found in eyes with a history of retinopathy of prematurity. The retina is an important controller of refractive development. The aims of this study were to find out whether altered measures of retinal function and ametropias are associated and to consider mechanisms by which the retina might control refractive development. Nine infants and children with a history of stage 1, 2 or 3 retinopathy of prematurity and known courses of refractive development were studied. Spherical equivalents at the time of the electroretinogram ranged from +5.50 to -9.00 diopters. Rod photoresponse characteristics were derived from the a-wave, and postreceptoral components were also analyzed with calculation of the sensitivity and saturated amplitude of the b-wave, the sensitivity of oscillatory wavelet OP2, and average amplitudes of OP3 and OP4. In hyperopic and myopic patients alike, the saturated amplitude and gain of the rod cell response were attenuated. In all patients, b-wave sensitivity was low, but in most there was little effect on saturated b-wave amplitude. In patients with courses toward myopia, the amplitude of OP4, an 'OFF' signal, is relatively more attenuated than that of OP3, an 'ON' signal. OP4 is relatively larger in patients with courses toward hyperopia. The OP results suggest that an imbalance of 'ON' and 'OFF' activity in the retina is associated with development of ametropias in retinopathy of prematurity.
This paper reviews the course of development of visual acuity in human infants and young children. Researchers have devised methods based on optokinetic nystagmus, visually evoked cortical potentials and preferential looking to assess visual acuity in infants and preverbal youngsters. During the first postnatal year and early childhood, acuity, measured by any of these methods, improves with increasing age. Each of these methods, has now been applied to the evaluation of clinical cases. Also presented are the results of a quick and simple test based on preferential looking, which has been incorporated into our clinical evaluation of infants. The data so far indicate that infants with normal eyes pass the test while infants with ocular problems which would interfere with vision fail. For evaluation of large numbers of infants this test appears to have the potential to assist nonspecialized personnel in the early identification of ophthalmic abnormalities.
Dark-adapted adults' electroretinographic b-wave thresholds are approximately 2 log units below a-wave thresholds and approximately 3 log units above the perceptual threshold, and their perceptual and the visually evoked cortical potential (VEP) thresholds are similar. Dark-adapted infants' scotopic a- and b-wave thresholds for full-field stimuli are both about 0.5 log units above those of adults, but their scotopic VEP thresholds for such stimuli have not been studied. We obtained scotopic VEP thresholds for brief, full-field stimuli from dark-adapted, infants and adults to consider the relationships of the cortical responses to the responses of more distal structures, namely the rod photoreceptors (scotopic a-wave) and ON-bipolars (scotopic b-wave). The median VEP threshold of infants is 0.5 log unit above that of adults. Thus, the relationships of a- and b-wave and VEP thresholds in infants are similar to those in adults. These results are consistent with rod cell immaturities being the primary determinant of the difference between infants' and adults' thresholds.
We tested the hypothesis that the dark-adapted sensitivity of the near central retina is lower than that of peripheral retina in 10-week-old infants. A spatial two alternative forced-choice psychophysical method was used to estimate the threshold for 2-deg spots presented 10 deg and 30 deg from the center of a screen. Each 10-week-old infant (n = 11) and adult (n = 5) subject was tested at both eccentricities. Every infant's threshold at 10 deg is higher than that at 30 deg. The median difference between the infants' thresholds at 10 deg and 30 deg is about 0.5 log unit. Whereas the scotopic thresholds of adults at 10 deg and 30 deg are virtually identical, the thresholds of infants' near central retina are higher than those in more peripheral retina. Delayed maturation of the near central rod outer segments with consequent lower quantum catch may be the primary determinant of this infantile sensitivity pattern.
An electroretinographic (ERG) study was undertaken to test the hypothesis that scotopic retinal function is altered in transfused thalassemics on chronic Deferoxamine (DFO). ERG a- and b-wave responses and dark adapted visual thresholds were obtained from 11 patients with beta-thalassemia major, ages 7 to 38 (median 17) years. A quantitative model of the activation of phototransduction was fitted to the a-waves to estimate the gain of the transduction processes and the saturated amplitude of the rod photoresponse. From b-wave stimulus/response functions. the saturated b-wave amplitude and an index of b-wave sensitivity (log sigma ) were calculated. The patients' data were compared to those of normal subjects. The relations of the ERG parameters to age. average ferritin level, and duration of transfusion without DFO as well as other clinical parameters were examined. Longitudinal measures of b-wave responses and dark adapted visual thresholds. available for nine of the patients, were examined for significant change over time. For all patients both the gain and saturated amplitude of the rod response are normal. In two patients log sigma is below the 99% prediction interval for normal. One has low scotopic visual sensitivity. The duration of transfusion therapy unprotected by DFO chelation therapy was correlated with log a. These results suggest iron accumulation rather than DFO toxicity underlies scotopic dysfunction in older thalassemics. some of whom may have had extended periods of transfusion without the protection of chelation. Thus, monitoring of retinal function is recommended in such patients.
Clathrin-coated vesicles, identified by negative staining with uranyl acetate, were purified from Chlamydomonas reinhardtii. Isolated coated vesicles had diameters ranging from 70 to 140 nm (mean diameter +/- SD of 95 +/- 17 nm, n = 300). These vesicles were markedly heterogeneous in both density and surface charge, as indicated by equilibrium density sedimentation and elution from anion-exchange columns. Highly-purified coated-vesicle fractions contained 2 major polypeptides, identified as the clathrin heavy chain (185 kDa) and the clathrin light chain (40 kDa). Chlamydomonas clathrin heavy chain cross-reacts weakly with an antibody against bovine brain clathrin heavy chain. Coat stability in several buffers was compared to that of bovine brain coated vesicles. Stability was similar, except for a greater stability of Chlamydomonas coated vesicles in 0.5 M Tris at pH 7.0.
An initial exposure to high concentrations of free fatty acid increased the transfer of albumin across cultured endothelial monolayers. The rate and amount of albumin transfer was dependent on the oleic acid concentration to which the cultures were initially exposed, with 300 microM producing the maximum transfer. The albumin transfer also increased with the increasing time of exposure to oleic acid, the maximum effect occurring during the first 24 hours. An exposure to 300 microM linoleic acid produced an even greater increase in albumin transfer than did 300 microM oleic acid. The increased albumin transfer observed when cells were exposed to high concentrations of free fatty acid was largely reversible after reincubation of the cell monolayers in free fatty acid-poor media. In parallel experiments, radioactive oleic acid incorporation into cell triglycerides increased linearly as the fatty acid concentration was raised, with cell triglyceride content increasing up to sevenfold after incubation in a medium containing 300 microM oleic acid. A significant amount of oleic acid was incorporated into phospholipids, and the fatty acid composition of the endothelial triglycerides and phospholipids was modified. All these effects of oleic occurred without altering the incorporation of leucine into the cell protein. These results indicate that exposure to high concentrations of free fatty acid can alter endothelial cell lipid composition, and that this increases the albumin transfer across endothelium. This process might permit more macromolecules to enter the arterial wall.
The development of the outer plexiform layer (OPL) at the posterior pole was studied in albino rats, ages 1 to 18 days. Light microscopic observations were consistent with previous reports that within the first postnatal week neuroepithelial cells migrate into outer and inner nuclear layers with formation of the intervening OPL. The relation of neural and glial cells was consistent with the suggestion of others that guidance by glial cells influences the radial organization of the developing neural retina. The new observations presented in this paper raise the possibility that the horizontal orientation of neural processes in the outer plexiform layer depends upon guidance of glial cells.
The retinal function of the delayed amelanotic (DAM) chicken that has progressive retinal degeneration, was investigated electroretinographically (ERG). Subjects showing a gradation of abnormalities of retinal morphology and impairment of visual behaviors were tested. Dark-adapted a-, b-, and c-wave responses to full-field stimuli were studied. In general, the more severe the anatomical abnormalities, the greater the compromise of sensitivity and amplitude of ERG responses. The DAM diseases appears to affect c-waves more than a-waves, and a-waves more than b-waves. Dark-adapted responses are affected more than light-adapted responses. In DAM's with moderately to severely impaired visual behaviors, ERG studies should permit analysis of the distal retinal pathophysiology caused by this disease.
We studied the application of preferential looking (PL) procedures to evaluation of visual performance of 59 prematurely born infants and young children. Neurological abnormalities were present in 29 of the 59 patients. Ophthalmic examination revealed normal eyes in 20 of the patients; retinopathy of prematurity (20 patients) and strabismus (13 patients) were the most prevalent ophthalmological disorders; the remaining 6 patients had a variety of ophthalmic anomalies. Results indicate that PL testing of such patients is best accomplished after age 2 months. Patients with developmental delays can be tested, but they may have poor PL performance that is not accounted for by ophthalmic abnormalities. Differences in PL acuities between right and left eyes were indicative of amblyopia in some of the strabismic patients; sequential PL acuities monitored occlusion therapy in these pre-verbal youngsters.
The electroretinographic (ERG) responses of normal human infants have been studied in scotopic conditions. The relation of stimulus intensity to the amplitude of the b-wave of the ERG undergoes developmental changes during infancy. The maximum amplitude that can be obtained gradually increases to approach adult values by age 12 months. Sensitivity, however, is equivalent to that of adults at age five to six months. Latencies reach adult values at the end of the first year. Some results of ERG testing of infant patients with retinal disorders are compared to the normal results. As previously reported for animals and human adults with retinal disorders, amplitude, sensitivity and latency are not always equally compromised by the retinal diseases of infants.