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Biomedical subjects

L Wachtmeister

Publications and source records attributed to L Wachtmeister.

At least 19 recordsLinked to original sources
PubMed2007

Progressive retinal dystrophy in two sisters with glutathione synthetase (GS) deficiency.

We report the ophthalmological findings of two sisters with severe glutathione synthetase deficiency, an autosomal recessive inborn error of metabolism resulting in very low intracellular levels of the free-radical scavenger glutathione. The patients were investigated because of declining visual acuity. The most prominent finding was progressive retinal dystrophy with hyperpigmentations and maculopathy. Generally disturbed functioning of both the outer and inner layers of the retina resulted in attenuated or nearly abolished electroretinograms. These findings agree with a rod/cone type of retinal dystrophy, and we suggest that this is due to glutathione deficiency. Treatment with antioxidants such as vitamins E and C seems to prevent the progression of CNS damage. We speculate that it might also prevent retinal dystrophy in patients with glutathione synthetase deficiency. We suggest that patients with retinal dystrophy and additional neurological signs should be investigated for a defect in glutathione metabolism. Also, we recommend that patients with low levels of glutathione should be examined for retinal dystrophy. Our results suggest that a decreased capacity for scavenging reactive oxygen species and/or increased oxidative stress may cause retinal dystrophy. If this is the case, the redox state in the retina should be a potentially useful therapeutic target to prevent reduced visual function and blindness.

Antioxidants↗
PubMed2005

Visual function and car driving: longitudinal results 5 years after cataract surgery in a population.

AIMS: To determine visual function in drivers who had cataract surgery 5 years previously, and to analyse longitudinal data, by comparing preoperative and postoperative changes in subjective driving ability and objective visual function. METHODS: All patients (810) who underwent cataract surgery, during a 1 year period, were prospectively studied. Data regarding present driving status were collected from self administered questionnaires and visual acuity (VA) data were measured before and after surgery. All patients who were alive 5 years later were invited to participate with a new eye examination and questionnaire. RESULTS: Before surgery 36 active drivers (16%) did not fulfil the visual requirements for driving; with improved glasses this number could be reduced to 24 (11%). 5 years after surgery, the corresponding figures were 5% and 3% (5/174), respectively. Before surgery 50% stated visual difficulties while driving in daylight and 79% in darkness. A few months and 5 years after surgery the corresponding figures were 6% and 5%, respectively, for daytime driving and 34% and 44%, respectively, for night-time driving. CONCLUSIONS: Long term results regarding cataract surgery in car drivers are beneficial. 5 years after surgery only a few patients drove not fulfilling the requirements, but there were a larger proportion of patients with problems driving in darkness compared with a few months after surgery.

Aged↗
PubMed2001

Topical anesthesia for cataract surgery: a population-based perspective.

PURPOSE: To analyze the anesthetic regimen and sedation in a population-based cohort of unselected cataract surgery cases operated on with the goal of maximizing the percentage of patients with topical anesthesia and no sedation. SETTING: Department of Ophthalmology, Norrlands University Hospital, Umeå, Sweden. METHODS: This prospective observational population-based study comprised all patients having cataract surgery during a 1 year period at 1 institution. Data were collected from the patients' records, which were standardized. Outcome measures were use of preoperative sedation, type of anesthesia, intraoperative complications, and adverse events. RESULTS: The study comprised 890 cases performed by 4 surgeons. Seventy-two percent of patients had no sedation and topical anesthesia only. All patients except 1 who had previous cataract surgery with topical anesthesia chose the same method for their second-eye surgery. The rate of posterior capsule rupture was in the expected range for a population with high incidences of pseudoexfoliation and mature cataract. CONCLUSIONS: It is possible to achieve a high percentage of effective topical anesthesia for cataract surgery in a population-based setting. The findings have implications for cost-containment in health services.

Administration, Oral↗
PubMed2001

Ocular phenotype of bothnia dystrophy, an autosomal recessive retinitis pigmentosa associated with an R234W mutation in the RLBP1 gene.

OBJECTIVE: To describe the phenotype of Bothnia dystrophy, an autosomal recessive retinal dystrophy with an R234W mutation in the RLBP1 gene encoding cellular retinaldehyde-binding protein. DESIGN: Medical records were reviewed retrospectively. Ophthalmologic examination, including kinetic perimetry and, in selected cases, adaptometry, color vision tests, fluorescein angiography, and electrophysiologic studies, was performed. The study included 24 individuals, all homozygous for an R234W mutation in the RLBP1 gene. RESULTS: Patients typically show night blindness from early childhood. In young adults, retinitis punctata albescens was observed, followed by macular degeneration and a decrease in visual acuity that led to legal blindness in early adulthood. Dark adaptometry and electrophysiologic testing showed an initial loss of rod function followed by a progressive reduction of the cone responses in older ages. CONCLUSIONS: Bothnia dystrophy is a unique retinal dystrophy belonging to the rod-cone dystrophies and has a high prevalence in northern Sweden. Fifty-seven cases of Bothnia dystrophy have been diagnosed, indicating a prevalence as high as 1 per 4500 population in the geographic area studied. A defect ability of mutated cellular retinaldehyde-binding protein to bind retinoid probably explains the defect rod function followed by central and peripheral degeneration. CLINICAL RELEVANCE: Retinal dystrophies associated with other mutations of the RLBP1 gene, including retinitis pigmentosa of Bothnia type, might account for a considerable number of cases of autosomal recessive retinitis pigmentosa in other geographic areas as well.

Adolescent↗
PubMed1999

Dissatisfaction with cataract surgery in relation to visual results in a population-based study in Sweden.

PURPOSE: To evaluate dissatisfied patients and those with subjectively worse visual ability after cataract surgery and to analyze how these factors relate to poor visual acuity results (worse than 20/40). SETTING: Department of Ophthalmology, Norrlands University Hospital, Umeå, Sweden. METHODS: A prospective, population-based study of cataract surgery outcome was conducted. All patients (459 surgeries) who had cataract surgery during a 1 year period were studied using self-administered questionnaires and data from patient records. Outcome measures were (1) patient degree of satisfaction with the result, (2) subjective visual ability after surgery of visually demanding tasks such as reading, television viewing, orientation in unfamiliar surroundings, and distance estimation far and near, and (3) visual acuity results. RESULTS: After surgery, 37 of the 459 cases (8%) were dissatisfied. These patients had a significantly lower age-corrected visual acuity in the operated eye than the satisfied patients (P < .0001). Ten percent said 1 or more visual ability was worse after their cataract surgery. Logistic regression analysis revealed that the visual acuity in the better eye before surgery and age-related maculopathy were the most significant risk factors. Poor visual acuity after surgery in the operated eye was found in 22%, mostly as a result of concurrent age-related maculopathy, diabetes, or glaucoma. Four percent had no improvement in visual acuity in the operated eye. CONCLUSIONS: Dissatisfaction seemed to rely mostly on visual acuity in the operated eye. Low visual acuity in the better eye before surgery and age-related maculopathy were the most significant risk factors for subjectively worse visual ability after surgery. The degree of satisfaction after cataract surgery and changes in visual ability differed in important ways from visual acuity as a measure of visual function.

Activities of Daily Living↗
PubMed1999

Impact of cataract surgery on visual acuity and subjective functional outcomes: a population-based study in Sweden.

PURPOSE: First, to determine the effects of cataract surgery on subjectively experienced visual function and visual acuity in a defined population, at a specific frequency of surgery. Secondly, to validate questionnaire data regarding the visual function of cataract patients. METHODS: A prospective population-based investigation of the subjective visual functional and visual acuity outcomes of cataract surgery over a 1 year time interval at one institution was conducted. All operated cases (n = 459) were grouped into three levels of visual impairment, according to the preoperative visual acuities of their better eyes. Subjective reading, TV watching, distance estimation and ability to orientate in unfamiliar surroundings, before and after surgery, were assessed using self-administered questionnaires. The subjective outcomes were related to the subjects' post-operative visual acuities. The statistical evaluations comprised analyses of variance, Yates'-corrected chi-squared tests, weighted kappa and correlation statistics. RESULTS: The pre-operative subjective visual disabilities of the patients were significantly correlated with the pre-operative visual acuities of the patients' better eyes. There was an improvement in subjective reading ability, distance estimation and ability to orientate in unfamiliar surroundings for most patients at all three pre-operative visual acuity levels. After surgery there was a stronger correlation between the subjective functional improvement and the increase in visual acuity for the operated eye than for the better eye. CONCLUSIONS: An incidence of cataract surgery of 3.3 per 1000 population for the year the present study was conducted seems not to be an over-utilisation of resources. Irrespective of the visual acuity level before cataract surgery, the vast majority of patients gain better subjective visual function and better acuity after surgery. It is possible to gain valid information from cataract surgery patients using a short questionnaire.

Activities of Daily Living↗
PubMed1999

Ophthalmic health care in diabetes mellitus: a cross-sectional study in northern Sweden.

The purpose of this study was to estimate the prevalence of diabetic retinopathy, visual impairment/blindness and previous surgical eye treatments in patients with older-onset vs. younger-onset diabetes mellitus. A cross-sectional study of patients with diabetes mellitus in the area of Umeâ, Sweden (135,000 inhabitants) was performed during 1994-1995, comprising 1,805 diabetic patients. Diabetic patients of younger ages were referred more frequently than older for fundus examination. With age at diagnosis <30 years retinopathy was seen in 68% of the patients; with age at diagnosis 30 years or older retinopathy was seen in 66% of the insulin-treated, 30% of the oral agent-treated and 7% of the diet-treated patients. Retinopathy that had previously been treated or needed treatment was seen in about one-third of the patients in both the younger-onset and the insulin-treated older-onset groups. The corresponding values for the oral agent-treated and diet-treated older-onset diabetic patients were 9.4% and 0.6%, respectively. No older-onset diabetic patient with acceptable glycemic control and with only dietary treatment had received or needed ophthalmic treatment. In the Umeâ area, patients with a relatively short duration of diabetes had a lower prevalence of diabetic retinopathy than that found in Wisconsin in 1979-1980. We conclude that demand for ophthalmic care in general is much higher for older-onset diabetic patients because of the greater number of patients with older-onset than younger-onset diabetes. In addition, older-onset patients should be examined regularly for treatable retinopathy. Diet-treated older-onset diabetic patients with acceptable glycemic control need little ophthalmic screening.

Adolescent↗
PubMed1998

Oscillatory potentials in the retina: what do they reveal.

This chapter is an overview of current knowledge on the oscillatory potentials (OPs) of the retina. The first section describes the characteristics of the OPs. The basic, adaptational, pharmacological and developmental characteristics of the OPs are different from the a- and b-waves, the major components of the electroretinogram (ERG). The OPs are most easily recorded in mesopic adaptational conditions and reflect rapid changes of adaptation. They represent photopic and scotopic processes, probably an interaction between cone and rod activity in the retina. The OPs are sensitive to disruption of inhibitory (dopamine, GABA-, and glycine-mediated) neuronal pathways and are not selectively affected by excitatory amino acids. The earlier OPs are associated with the on-components and the late OPs with the off-components in response to a brief stimulus of light. The postnatal appearance of the first oscillatory activity is preceded by the a- and b-waves. The earlier OPs appear postnatally prior to, and mature differently from, the later ones. The second section deals with present views on the origin of the OPs. These views are developed from experimental studies with the vertebrate retina including the primate retina and clinical studies. Findings favor the conclusion that the OPs reflect neuronal synaptic activity in inhibitory feedback pathways initiated by the amacrines in the inner retina. The bipolar (or the interplexiform) cells are the probable generators of the OPs. Dopaminergic neurons, probably amacrines (or interplexiform cells), are involved in the generation of the OPs. The earlier OPs are generated in neurons related to the on-pathway of the retina and the later ones to the off-channel system. Peptidergic neurons may be indirectly involved as modulators. The individual OPs seem to represent the activation of several retinal generators. The earlier OPs are more dependent on an intact rod function and the later ones on an intact cone system. Thus, the OPs are good indicators of neuronal adaptive mechanisms in the retina and are probably the only post-synaptic neuronal components that can be recorded in the ERG except when structured stimuli are used. The last section describes the usefulness of the oscillatory response as an instrument to study the postnatal development of neuronal adaptation of the retina. In this section clinical examples of of the sensitivity of the OPs for revealing early disturbance in neuronal function in different retinal diseases such as pediatric, vascular and degenerative retinopathies are also given.

Animals↗
PubMed1998

Cataract surgery from a gender perspective--a population based study in Sweden.

PURPOSE: To determine the presence of any gender-related differences of preoperative subjective visual functional problems that may explain the higher frequency of cataract surgery found in women. METHODS: All patients (n=453) who, during a one-year period, underwent cataract surgery at Norrlands University Hospital, Umeå, Sweden, were included in the study. Incidence, age-distribution, visual acuity, self-estimated ability to perform vision dependent tasks as well as subjective symptoms from the cataractous eyes preoperatively and visual acuity after surgery were analyzed separately in males and females. RESULTS: Before surgery there were no statistically significant differences between men and women in visual acuity of the eyes to be operated on and the fellow eyes. Preoperatively women had significantly more subjective problems with distance-estimation for near and far (57% and 60%, respectively) compared to males (45% and 43%, respectively). Women had significantly larger problems orientating in unfamiliar surroundings. CONCLUSION: There are gender-related differences in self-assessed visual function before surgery that might contribute to the higher incidence of cataract surgery in women. Preoperatively women experience a higher degree of visual functional problems than men. Women were not found to demand cataract surgery earlier than men when preoperative visual acuities were compared.

Adult↗
PubMed1997

The impact of cataract surgery on low vision patients. A population based study.

PURPOSE: To assess the impact of cataract surgery on low vision patients. METHODS: Self-estimated ability to perform visually demanding tasks before and after cataract surgery and expectations and satisfaction with the surgery were analysed in all low-vision patients (n = 52), who during a one-year period underwent surgery at Norrlands University Hospital in Umeå, Sweden. Self-administered questionnaires were used and the records of the patients were studied. RESULTS: The majority (81%) of the low vision patients reported an improved ability to perform visually demanding tasks after surgery. The number of patients who were unable to read newspaper-size print decreased significantly, from 44% to 21% after surgery. The subjectively improved visual ability as well as the augmentation of VA of the operated eye were both significantly related to satisfaction with the outcome of surgery. CONCLUSION: Most low-vision patients with significant cataract benefit from surgery, both subjectively by increased ability to perform visually demanding tasks as well as by an improved visual acuity. Particularly patients with loss of peripheral visual fields subjectively gain in orientation ability. It is important to reduce unreasonable expectations about the outcome of the surgery.

Activities of Daily Living↗
PubMed1997

Impact of cataract surgery on car driving: a population based study in Sweden.

AIMS: To investigate the outcome of cataract surgery on the patients' self estimation of visual function while driving. Furthermore, the benefit of surgery to the car driving population was determined. METHODS: A total of 208 consecutive patients (211 cases) with driving licences, who underwent cataract surgery with intraocular lenses, were studied prospectively using self administered questionnaires. Their self estimated degree of visual functional problems while driving were analysed before and after surgery. RESULTS: Visual problems while driving declined from 82% preoperatively to 5% after surgery. Remaining visual problems with the operated eye were present in only seven patients. Problems in estimating distance while driving decreased from 37% before surgery to 6% after surgery. Twenty three per cent of the patients drove with a visual acuity below the requirements for driving in Sweden before surgery and only 4% after surgery. There was no correlation between the degree of visual problems while driving and visual acuity before surgery. CONCLUSION: The car driving population greatly benefited from cataract surgery in terms of subjectively improved visual function and distance estimation while driving. Functional visual problems while driving should be considered as an indication for cataract surgery. These findings also indicated that the second eye should be operated on, if necessary, to achieve optimal ability to estimate distance and give best possible road safety.

Adult↗
PubMed1995

A gene for autosomal dominant progressive cone dystrophy (CORD5) maps to chromosome 17p12-p13.

Inherited retinal dystrophy is a common cause of visual impairment. Cone dystrophy affects the cone function and is manifested as progressive loss of the central vision, defective color vision, and photophobia. Linkage was demonstrated between progressive cone dystrophy (CORD5) and genetic markers on chromosome 17p12-p13 in a five-generation family. Multipoint analysis gave a maximum lod score of 7.72 at the marker D17S938. Recombinant haplotypes in the family suggest that the cone dystrophy locus is located in a 25-cM interval between the markers D17S926/D17S849 and D17S804/D17S945. Furthermore, one recombination was detected between the disease locus and a microsatellite marker in the candidate gene RCV1, encoding the retinal protein recoverin. Two additional candidate genes encoding retinal guanylate cyclase (GUC2D) and pigment epithelium-derived factor (PEDF) are located at 17p13.1. Moreover, loci for retinitis pigmentosa and Leber congenital amaurosis have been mapped to the same region. Identification of the cone dystrophy locus may be of importance not only for identifying functional genes in the cone system, but also for identifying genes for other retinal disorders.

Chromosome Mapping↗
PubMed1993

The postnatal development of the oscillatory potentials of the electroretinogram V. Relation to the double peaked a-wave.

The postnatal development of the double peaked a-wave and its relation to the oscillatory potentials of the rat electroretinogram was studied. The split a-wave appeared one day later than the first oscillatory activity. The appearance and maturation of the positivity between the two a-waves (a-hump) was found to be similar to that of the first oscillatory potential, and the development of the second a-wave showed similarities to that of the second oscillatory potential. Thus, we conclude that the double peaked a-wave is closely related to the appearance of the two first oscillatory potential. Secondly, the division of the a-wave in the immature animal may be caused by the intrusion of the first oscillatory potential. Thirdly, the presence of a split a-wave before spontaneous opening of the lid seems to indicate a state of comparatively progressed maturity of neuronal adaptation.

Aging↗
PubMed1992

The postnatal development of the oscillatory potentials of the electroretinogram. IV. Mesopic characteristics.

The postnatal development of the oscillatory potentials (OP) of the rat's electroretinogram (ERG) was studied during relatively mesopic adaptational conditions. The behaviour of the OPs induced at an interstimulus interval (ISI) of 30 sec entailed photopic qualities at an individual level, but when summated conformed to a scotopic course of postnatal maturation. An ISI of 1 min elicited OPs with individual scotopic characteristics. The total oscillatory activity culminated at 17 days of age at both ISIs, similar to more scotopically induced oscillations. These results show that relatively mesopic states of adaptation do not promote any earlier appearance of the OPs, and that the immature mesopically induced OPs seem to contain both scotopic and photopic qualities reflecting a postnatal change of balance between these components. Furthermore, photopic mechanisms seem to govern the oscillatory activity in the mature rat retina. We suggest that the differences in relation to adaptational conditions between the immature and mature OPs may reflect the postnatal status of the dopamine and its synthetizing enzyme, tyrosine-hydroxylase.

Adaptation, Ocular↗
PubMed1992

Efficacy of cryotherapy for retinopathy of prematurity.

This is a retrospective study of the benefit of transconjunctival cryotherapy for retinopathy of prematurity (ROP) in 43 eyes of 22 infants (birth weight 716-1675 g) from July 1985 to January 1988. Nine eyes were treated at ROP stage 4A (early), 14 eyes at stage 3 plus disease, and 20 eyes at stage 3. Cryotherapy was applied to the retinal avascular zone. In 13 eyes the ridge of extraretinal fibrovascular proliferation was also treated. Two eyes with peripheral exudative retinal detachment in two quadrants had additional treatment of the area of elevated retina. Reexamination was performed at the age of 26 to 52 months (average 38 months). Regression of the ROP was found in all eyes. Seven eyes showed a lateral traction of the temporal vascular arcades, six of these eyes had a slight macular ectopia. None of the eyes progressed to stage 4B or 5. Visual acuity was 6/60 or better. Twelve children (60%) achieved 6/18 or better and 5 (25%) 6/9 or more. Our data indicate that cryotherapy for ROP grade 3 and early stage 4A prevents the progression to advanced stages. The results also imply a functional benefit of cryotreatment in infants with progressive severe ROP.

Child, Preschool↗
PubMed1991

The postnatal development of the oscillatory potentials of the electroretinogram. II. Photopic characteristics.

The postnatal development of the oscillatory potentials (OPs) of the rat's electroretinogram (ERG) was studied during more extreme photopic conditions. The appearance of the OPs was further delayed, by 1 to 3 days, compared to previously studied less photopic conditions. A rapid phase of development of the total OP activity occurred at the age of 15 days, which coincided with the physiological opening of the eye-lids. During maturation the first two OPs were enhanced while the later OPs were attenuated. The total OP activity culminated at the age of 30 days. These results strongly indicate that the rapid neuronal adaptive system appears and develops later than the slow photochemical one. Secondly, the fast phase of maturation of the neuronal adaptive process, as reflected in the OPs, seems to be dependent on environmental light. Thirdly, the findings support previous indications that two different systems seem to be responsible for the OPs in the rat and thus the earlier OPs to have different origins from those of the later ones.

Adaptation, Physiological↗
PubMed1991

The postnatal development of the oscillatory potentials of the electroretinogram. III. Scotopic characteristics.

The postnatal development of the oscillatory potentials (OPs) of the rat electroretinogram (ERG) was studied during more extreme scotopic conditions. Enhancement of scotopic conditions did not facilitate any earlier appearance of the OPs, including the later ones, compared to previously studied less scotopic conditions. The oscillatory activity appeared at Days 12 to 15, and increased rapidly up to Day 17, which coincided with the major period of development of the photoreceptors. After the physiological opening of the eyelids there was a decline of the OPs. We propose that the decline of the oscillatory activity induced during more extreme scotopic conditions is related to early cell death in the distal retina and/or to developmental neuronal plasticity in the proximal retina.

Animals↗