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Biomedical subjects

Ronald M Hansen

Publications and source records attributed to Ronald M Hansen.

14 recordsLinked to original sources
PubMed2006

Multifocal ERG in subjects with a history of retinopathy of prematurity.

PURPOSE: Investigate the function of the central retina in subjects with a history of retinopathy of prematurity (ROP). METHODS: Multifocal electroretinogram (mfERG) responses to a scaled array of 103 hexagons were recorded in subjects, aged 11-23 years (N = 11), with a documented history of mild ROP. The amplitude and implicit time of the components (N(1), P(1), N(2)) of the first order kernel for six concentric rings were compared to those of control subjects (N = 9). RESULTS: The amplitude of each component varied significantly with eccentricity in both ROP and control subjects and was significantly smaller in the ROP subjects. The discrepancy between ROP and control subjects was greatest for central rings (1-3) and smaller for peripheral rings (4-6). The slopes of the functions summarizing log response density as a function of log eccentricity (degrees visual angle) were significantly shallower in ROP subjects. The implicit time of each component was longer in ROP subjects at all eccentricities. CONCLUSIONS: ROP associated alterations in neural retinal development may underlie the subtle macular dysfunction disclosed by the mfERG.

Adolescent↗
PubMed2006

The retinal vasculature and function of the neural retina in a rat model of retinopathy of prematurity.

PURPOSE: In a rat model of retinopathy of prematurity (ROP), the retinal vasculature and function of the neural retina were studied longitudinally. Vascular and neural parameters were evaluated for significant relationships. METHODS: Retinopathy was induced by exposing newborn rats to alternating 50% and 10% oxygen until age 14 days. To evaluate the function of the neural retina, electroretinographic (ERG) responses to full-field stimuli were recorded from dark-adapted rats at ages 18 and 31 days. Sensitivity and saturated amplitude of photoreceptor and postreceptor activity were derived from ERG a- and b- waves. To evaluate the retinal vasculature, digital fundus photographs were obtained at the same ages, and the tortuosity indices of the arterioles (TIA) and venules (TIV) were calculated. ROP rats and room-air-raised control animals were compared. Vascular and response parameters were evaluated for significant relationships. RESULTS: In ROP rats, TIA was high at 18 days and decreased in every rat to nearly normal levels by 31 days. TIV was less affected by ROP or age. Deficits in all receptor and post-receptor response parameters were present in 18-day-old ROP rats. Post-receptor sensitivity recovered completely by 31 days. Deficits in other response parameters persisted. No significant correlations between vascular and ERG parameters were found in 18-day-old ROP rats. CONCLUSIONS: Noninvasive, longitudinal measures in this model of ROP showed significant abnormalities in both the retinal vasculature and function of the neural retina that were most marked at age 18 days. However, vascular and neural abnormalities did not correlate.

Animals↗
PubMed2006

Development of the electroretinographic oscillatory potentials in normal and ROP rats.

PURPOSE: To study the development of the electroretinographic (ERG) oscillatory potentials (OPs) in rats and to compare normal OPs with those in a rat model of retinopathy of prematurity (ROP). METHODS: Following a longitudinal design, ERG responses to a greater than 5 log unit range of full-field stimuli were recorded in dark-adapted rats at postnatal day (P) 18, P31, P47, and P67. The ERG records were digitally filtered (60-235 Hz), and the trough-to-peak amplitudes and implicit times of OP2, OP3, OP4, and OP5 were measured. Additionally, rats with oxygen-induced retinopathy, a model of ROP, were studied at P31. RESULTS: Generally, OP amplitude increased and implicit time decreased with increasing stimulus intensity. The shape of the stimulus-response functions changed with age. The amplitudes of OP2, OP3, and OP4 were largest at P31. OP5 was largest at P47. All OPs were significantly affected in ROP rats; OP5 was least affected by ROP. CONCLUSIONS: A prolonged normal course of OP development, which featured waxing and waning of amplitudes, was observed and might have been consequent to maturation and then to final refinements of inner retinal circuitry. In ROP rats, marked attenuation of early OPs was consistent with persistent dysfunction of photoreceptors, and significant attenuation of the late OP5 was evidence of compromised function of inner retinal circuitry.

Aging↗
PubMed2005

Development of visual acuity in children with cerebral visual impairment.

OBJECTIVE: To study the development of visual acuity in term-born children with cerebral visual impairment and a history of neonatal hypoxic-ischemic encephalopathy. METHODS: We studied 19 term-born children, aged 6 months to 6 years, with moderate to severe neonatal hypoxic-ischemic encephalopathy and behaviors indicative of cerebral visual impairment. Longitudinal measures of grating acuity were obtained using preferential looking (PL) and visual evoked potential (VEP) procedures. Visual acuities at first and last visits were compared. The courses of acuity development in the 9 children who underwent both VEP and PL acuity assessment at 4 or more ages were compared with normal development. RESULTS: All children had measurable PL and VEP acuity, despite poor visual behavior. In nearly all, both PL and VEP acuity were below normal for age. For both PL and VEP measures, acuity at the last visit was, on average, 1 octave better than at the first visit, with a rate of improvement lower than normal. Although parallel courses of PL and VEP development occurred in many, substantial disparities in PL and VEP acuity were observed in others. CONCLUSIONS: Modest increases in PL and VEP grating acuity occur during early childhood in many of these patients. The rate of increase is lower than normal.

Blindness, Cortical↗
PubMed2005

Recovery of the rod photoresponse in infants.

PURPOSE: To study deactivation of the rod photoresponse in infants using a paired-flash procedure. Rhodopsin content increases and scales the parameters of the activation of rod phototransduction as rods develop. However, little is known about the kinetics of deactivation in the rods of young infants. METHODS: ERG responses to pairs of flashes were used to study the recovery of the rod response in 4- and 10-week-old infants and mature control subjects. The amplitudes of rod isolated a-wave responses to a probe flash (+3.3 log scot td . s) presented 2 to 120 seconds after an equal-intensity test flash were measured. The interstimulus interval (ISI) at which the amplitude was half that of the response to the probe flash alone (t(50)) was determined by linear interpolation. RESULTS: Recovery time (t(50)) was significantly longer in infants than in adults (F = 18.9, df 2, 32; P < 0.01). The shape of the recovery function did not vary with age. The t(50) values were inversely proportional to the parameters of activation of rod phototransduction. CONCLUSIONS: These results are evidence that the kinetics of deactivation in infants are slower and may be set by the proportion of rhodopsin isomerized.

Adolescent↗
PubMed2005

Development of the cone ERG in infants.

PURPOSE: To assess cone photoreceptor and cone-mediated postreceptoral retinal function in infants. METHODS: ERG responses to a 1.8-log unit range of long-wavelength flashes on a white, rod-saturating background were recorded in 4-week-old (n = 22) and 10-week-old (n = 28) infants and control adults and children, 8 to 40 years of age (n = 13). A model of the activation of cone phototransduction was fit to the a-waves. Sensitivity (S(CONE)) and saturated-response amplitude (R(CONE)) were calculated. The amplitude and implicit time of the b-wave were examined as a function of stimulus intensity. The cone photoresponse parameters were compared to the rod photoresponse parameters (S(ROD) and R(ROD)) in the same subjects. RESULTS: S(CONE) and R(CONE) in infants were significantly smaller than in the mature control subjects. The mean S(CONE) was 64% and 68%, and the mean R(CONE) was 63% and 72% in 4- and 10-week-olds, respectively. The mean rod photoresponse parameters were considerably less mature, as the mean S(ROD) was 35% and 46%, and the mean R(ROD) was 39% and 43% of mature values at 4 and 10 weeks. The b-wave stimulus-response functions in the 4- and 10-week-old infants did not show the photopic hill that was characteristic of the children's and adults' photopic b-waves. CONCLUSIONS: Peripheral cone function is relatively more mature than rod function in young infants. The lack of a photopic hill is hypothesized to result from immaturity in the relative contributions of ON and OFF bipolar cell responses.

Adolescent↗
PubMed2003

Rod photoreceptor responses in children with Smith-Lemli-Opitz syndrome.

OBJECTIVE: To test the hypothesis that the kinetics of activation and deactivation of rod phototransduction are altered in children with Smith-Lemli-Optiz syndrome (SLOS), a common genetic disorder caused by an inborn error in cholesterol biosynthesis. METHODS: Thirteen patients with SLOS (median age, 4 years) were studied by means of scotopic full-field electroretinography. The kinetics of activation and deactivation of rod phototransduction were derived from the electroretinographic a-wave. Postreceptoral electroretinographic components were also evaluated. RESULTS: The kinetics of activation were below normal limits in all but 3 of the 13 patients. Rod cell recovery (deactivation) in SLOS was slower than normal in all 8 patients in whom it was studied. Postreceptoral sensitivity was below normal limits in all but 1 of the 13 patients. CONCLUSIONS: The kinetics of phototransduction are slow in children with SLOS. This is likely a consequence of altered sterol composition in the cell membranes of the rod photoreceptors. To our knowledge, this is the first demonstration of altered kinetics of a membrane-bound signaling system in SLOS. Investigation of other membrane-bound signaling systems may be warranted in the quest to understand development and phenotype of individuals with SLOS.

Adolescent↗
PubMed2003

Retinal function in carriers of Bardet-Biedl syndrome.

OBJECTIVE: To test the hypothesis that carriers of Bardet-Biedl syndrome have abnormal rod-mediated responses. METHODS: Parents (n = 26) of children with Bardet-Biedl syndrome (BBS), who are obligate carriers of BBS, consented to scotopic, full-field electroretinography (ERG). Responses were recorded over a 4 to 5 log unit range, up to a maximum stimulus of approximately +3.6 log scotopic troland seconds. The parameters of activation of the rod photoresponse, S (sensitivity parameter) and Rmp3(amplitude of the saturated rod response), were derived by fitting a transduction model to the ERG a-waves. For the b-wave, the stimulus producing a half maximum response (log sigma) and the saturated amplitude (Vmax) were determined. The model of the rod photoresponse was subtracted from the intact ERG to demonstrate a corneal positive potential (P2), and log kP2 and P2max were determined. The carriers' ERG responses were compared with those of healthy control subjects (n = 26). MAIN OUTCOME MEASURES: Sensitivity (S, log sigma, and log kP2) and saturated amplitude (Rmp3, Vmax, and P2max) of receptoral and postreceptoral response components. RESULTS: All parents had decreased P2 sensitivity, and most (15 [60%] of 26) had decreased b-wave sensitivity. The rod photoresponse sensitivity and the saturated amplitudes of the rod cell response, b-wave and P2, did not differ significantly between carriers and controls. CONCLUSIONS: Diminished P2 sensitivity is characteristic of the carriers of BBS. The site of the primary defect in the BBS rod pathway appears to be proximal to the outer segments, most likely before the rod-bipolar cell synapse.

Adolescent↗
PubMed2003

Recovery of the rod photoresponse in infant rats.

We tested the hypothesis that the kinetics of recovery of the rod photoresponse differ between mature and immature rods. A paired flash paradigm was used. The effect of a test flash on the ERG a-wave response to a probe flash presented 60 to 2 s after the test flash was studied. The functions summarizing the interaction between the test and probe flash did not differ significantly between infants and adults if the stimuli were equated for estimated proportion of rhodopsin isomerized/rod/flash. The kinetics of rod cell recovery are likely the same in infants and adults.

Adaptation, Ocular↗
PubMed2003

Development of ERG responses: the ISCEV rod, maximal and cone responses in normal subjects.

PURPOSE: Summarize ISCEV ERG responses from normal infants and children. METHODS: The amplitudes and implicit times of the ISCEV rod, maximal dark-adapted and cone responses from a total of 409 normal infants (n = 128), children and adult controls were compiled. The subjects, aged 1 week to 52 years, were divided into seven age groups, including four in infancy (< 52 weeks). The response parameters for each age group were summarized as percentiles. RESULTS: In each ISCEV condition, the youngest infants (1-5 weeks) had significantly smaller amplitudes and longer implicit times than adults. Amplitude increased and implicit time decreased systematically with age. CONCLUSIONS: The developmental changes in ERG responses are significant. The medians and ranges herein provide provisional norms against which the ERG responses from pediatric patients can be compared.

Adolescent↗
PubMed2002

Rod photoreceptor function in children with mitochondrial disorders.

OBJECTIVE: To test the hypothesis that function of the rod photoreceptors is abnormal in pediatric patients with mitochondrial disorders. METHODS: Patients (n = 22; median age, 5 years) with a deficiency of 1 or more of the mitochondrial enzyme complexes, or a mutation in mitochondrial DNA, were studied by means of scotopic, full-field electroretinography (ERG). The conditions of ERG testing allowed derivation of the parameters of the activation of rod phototransduction from the ERG a-wave, and postreceptoral function from b-wave and P(2) stimulus-response functions. The deactivation of phototransduction was studied in 5 patients. The patients' ERG responses were compared with those of healthy control subjects (n = 25). RESULTS: Responses from 19 patients were sufficient for analysis of rod photoreceptor and postreceptoral function. Saturated amplitudes of the rod photoresponse and b-wave sensitivity were significantly depressed in the patients. Saturated amplitudes of rod cell and P(2) responses were correlated. The kinetics of deactivation of phototransduction were slowed even if the kinetics of activation were normal. CONCLUSIONS: In patients with mitochondrial disorders, some abnormalities of the scotopic ERG responses originate in the rod photoreceptors, but postreceptoral processes may also be abnormal. From a practical perspective, ERG testing can contribute to diagnosis of mitochondrial disorders.

Adolescent↗
PubMed2002

Background adaptation in a rat model of retinopathy of prematurity.

Low dark-adapted, scotopic retinal and visual sensitivity in retinopathy of prematurity (ROP) could be due to disease of the inner retina, or the recently described rod photoreceptor abnormalities. Receptoral disease decreases catch of quanta from both test flashes and steady background lights; increment threshold functions are shifted up and right. In diseases with normal receptors but low retinal sensitivity due to abnormal post receptoral processing, the increment threshold functions are shifted up with no horizontal translation. Herein we test the hypothesis that the rod photoreceptors are the site of ROP disease which causes low dark adapted b-wave sensitivity. The effect of steady background light on the ERG b-wave in a rat model of ROP is studied. ERG stimulus/response functions were obtained using full-field stimuli in the dark-adapted state, and in the presence of a steady background light. In each adaptation condition, log sigma, the test flash intensity that produced a half-maximum b-wave amplitude, was calculated. In pilot experiments, the background light selected had raised log sigma about a log unit in controls. In dark-adapted ROP rats log sigma was significantly higher, 0.35 log unit, than in controls. In the presence of the background light, log sigma in ROP and control rats did not differ significantly indicating a relative shift, up and right, of the increment sensitivity function for the less sensitive ROP rats. The effect of the background light is consistent with receptoral disease causing low dark adapted b-wave sensitivity in ROP rats.

Adaptation, Ocular↗
PubMedDate not supplied

ERG oscillatory potentials in infants.

PURPOSE: Study the scotopic and photopic oscillatory potentials (OPs) of the electroretinogram (ERG) in 10-week old infants. METHODS: Term-born 10-week old infants (n = 15) and adults (n = 12) were tested. Full-field ERGs were recorded under scotopic and photopic conditions. The records were filtered (75-300 Hz) to demonstrate the oscillatory wavelets. The amplitudes and implicit times of the infants' OPs were compared to those in adults and also to amplitudes of the saturated photoreceptor responses. RESULTS: In infants, the mean OP amplitudes are similar in scotopic and photopic conditions and do not vary significantly with OP number. Infants' OPs are significantly smaller than in adults, with scotopic OPs averaging 19% of that in adults and photopic OPs averaging 47%, whereas the amplitudes of the saturated photoreceptor responses are 43% and 66% of those in adults. Mean interpeak intervals are similar in infants and adults, indicating oscillatory behavior at a frequency of 155 Hz in scotopic conditions and 135 Hz in photopic conditions. CONCLUSIONS: In young infants, the OPs are relatively immature compared to the photoreceptor responses, with the immaturity of the scotopic responses being more marked than that of the photopic responses.

Adaptation, Ocular↗