Information and communication technology use in daily life among young adults with mild-to-moderate intellectual disability.

BACKGROUND: Information and communication technology (ICT) increases participation in life activities, and young adults are frequent users. Young adults with intellectual disability (ID) do not use ICT as much as their peers, and little is known about how ICT is used by young adults with ID. This study describes the use of ICT from the perspective of young adults with mild to moderate ID in a municipal social care context. METHOD: Semi-structured interviews were used to collect information from 11 young adults with mild-to-moderate ID living in residential care and analysed using a content analysis. RESULTS: ICT was used for family relationships, daily support, interactions based on interests and amusement, and as support for offline activities. Family members were important providers of support for ICT use. CONCLUSION: Young adults with mild-to-moderate ID use ICT in their daily life. The social care context needs to be further investigated due to its influence on the young adults' access to ICT and need of support.

Registered nurses' use of computerised decision support in medication reviews.

Purpose The purpose of this paper is to explore the implications of registered nurses' (RNs) use of a computerized decision support system (CDSS) in medication reviews. Design/methodology/approach The paper employs a quasi-experimental, one-group pre-test/post-test design with three- and six-month follow-ups subsequent to the introduction of a CDSS. In total, 11 RNs initiated and prepared a total of 54 medication reviews. The outcome measures were the number of drug-related problems (DRPs) as reported by the CDSS and the RNs, respectively, the RNs' views on the CDSS, and changes in the quality of drug treatment. Findings The CDSS significantly indicated more DRPs than the RNs did, such as potential adverse drug reactions (ADRs). The RNs detected additional problems, outside the scope of the CDSS, such as lack of adherence. They considered the CDSS beneficial and wanted to continue using it. Only minor changes were found in the quality of drug treatments, with no significant changes in the drug-specific quality indicators (e.g. inappropriate drugs). However, the use of renally excreted drugs in reduced renal function decreased. Practical implications The RNs' use of a CDSS in medication reviews is of value in detecting potential ADRs and interactions. Yet, in order to have an impact on outcomes in the quality of drug treatment, further measures are needed. These may involve development of inter-professional collaboration, such as established procedures for the implementation of medication reviews, including the use of CDSS. Originality/value This is, to the best of the authors' knowledge, the first study to explore the implications of medication reviews, initiated and prepared by RNs who use a CDSS. The paper adds further insight into the RNs' role in relation to quality of drug treatments.

Development and psychometric properties of the stressor scale for emergency nurses.

INTRODUCTION: Emergency department nurses are exposed to specific stressors and report higher stress levels than nurses in other hospital departments. This study aimed to develop and test the psychometric properties of a questionnaire-based instrument for identifying stressors for emergency department nurses. METHODS: The instrument's content and face validities were examined by five experts and nurses in emergency nursing field. The test-retest reliability was examined on 30 emergency department nurses. The construct validity, including an exploratory and a confirmatory factor analysis, was tested on 405 emergency department nurses. Cronbach's alpha values and intra-class coefficients were calculated. RESULTS: The instrument's content and face validities were satisfactory. The exploratory factor analysis provided a five-factor solution, whereas the confirmatory factor analysis provided a final four-factor solution with 25 items distributed among the factors Life and death situations, Patients' and families' actions and reactions, Technical and formal support, and Conflicts. The Cronbach's alpha values ranged from 0.89 to 0.93 per factor, and the intra-class correlation coefficient was 0.89, indicating good homogeneity and stability. CONCLUSIONS: The instrument's content, face, and construct validities were satisfactory, and the internal consistency and test-retest reliability were good. This instrument can be useful in the management of emergency departments.

Family Health Conversations Conducted by Telephone in Heart Failure Nursing Care: A Feasibility Study.

Registered nurses (RNs) in heart failure (HF) nursing care have a key role in providing family support, which positively affects the outcome for the patient. Telephone interventions conducted by RNs have been reported to be successful in HF nursing care, but Family Health Conversations (FamHCs) involving the patient and the family, have not previously been tested. The purpose of the current study was to explore the experiences and feasibility of nurse-led FamHCs conducted by telephone with patients and their family caregivers. A single-group intervention study with a pretest-posttest design was conducted in three regional hospitals that had a nurse-led HF clinic. Five RNs, eight patients, and eight family caregivers participated. Three FamHCs were conducted by telephone with each family every 2 weeks. Qualitative and quantitative data were collected through semistructured interviews and questionnaires. FamHCs improved the nurse-family relationships and relationships within the families and provided RNs with new knowledge about the families. FamHCs conducted by telephone were considered to be feasible for both families and RNs, although RNs preferred fewer and shorter FamHCs. The RNs preferred meeting face-to-face with the families as nonverbal communication between the family members could be missed because of lack of visual input. On the other hand, RNs appreciated to focus entirely on the conversation without the need to perform illness-related routine checks. In conclusion, the advantages of FamHCs conducted by telephone outweighed the disadvantages. Visual contact, provided by video telephony, and a shorter version of the tested FamHC would facilitate the use in HF nursing care.

eHomecare and safety: The experiences of older patients and their relatives.

The study's aim was to extend descriptions of how older patients with granted eHomecare and their relatives understand safety, and further to describe how they experience safety in everyday life. The study was conducted in Sweden. The participants were 12 older patients who had been provided with eHomecare and 8 relatives. Data were collected by semi-structured individual interviews. A conceptual framework of safety was used and a qualitative content analysis was conducted in a deductive and an inductive phase. The deductive results are presented in predefined categories: perceived sense of safety, disturbance and threats, re-establishing safety, and new safety. The inductive analysis resulted in two main categories: safety as part of everyday life and eHomecare as safety. The results show that eHomecare can promote safety for older patients and their relatives. Existing doubts and ethical concerns about the service can be minimized by providing adequate information.

Patterns of everyday functioning in preschool children born preterm and at term.

BACKGROUND/AIM: Children born preterm are at risk of neonatal complications but the long-term consequences for everyday functioning is not well known. The study aimed to identify patterns of everyday functioning in preschool children born preterm and at term in relation to perinatal data, neonatal risk factors, behaviour, and socioeconomic status. Registry data and data from parent rated questionnaires were collected for 331 children. METHOD: A person-oriented approach with a cluster analysis was used. RESULTS: A seven cluster solution explained 65.91% of the variance. Most children (n=232) showed patterns of strong everyday functioning. A minority of the children (n=99), showed diverse patterns of weak everyday functioning. Perinatal characteristics, neonatal risk factors and socio-economics did not predict cluster group membership. Children born preterm were represented in all clusters. CONCLUSION, IMPLICATIONS: Most preschool children are perceived by their parents with strong everyday functioning despite being born preterm. However small groups of children are, for various reasons, perceived with weak functioning, but preterm birth is not the sole contributor to patterns of weak everyday functioning. More critical for all children's everyday functioning is probably the interaction between individual factors, behavioural factors and contextual factors. To gain a broader understanding of children's everyday functioning. Child Health Services need to systematically consider aspects of body function, activity and in addition participation and environmental aspects.

Nurses' attitudes toward family importance in heart failure care.

BACKGROUND: Support from the family positively affects self-management, patient outcomes and the incidence of hospitalizations among patients with heart failure. To involve family members in heart failure care is thus valuable for the patients. Registered nurses frequently meet family members of patients with heart failure and the quality of these encounters is likely to be influenced by the attitudes registered nurses hold toward families. AIMS: To explore registered nurses' attitudes toward the importance of families' involvement in heart failure nursing care and to identify factors that predict the most supportive attitudes. METHODS: Cross-sectional, multicentre web-survey study. A sample of 303 registered nurses from 47 hospitals and 30 primary health care centres completed the instrument Families' Importance in Nursing Care - Nurses' Attitudes. RESULTS: Overall, registered nurses were supportive of families' involvement. Nonetheless, attitudes toward inviting families to actively take part in heart failure nursing care and involve families in planning of care were less supportive. Factors predicting the most supportive attitudes were to work in a primary health care centre, a heart failure clinic, a workplace with a general approach toward families, to have a postgraduate specialization, education in cardiac and/or heart failure nursing care, and a competence to work with families. CONCLUSIONS: Experienced registered nurses in heart failure nursing care can be encouraged to mentor their younger and less experienced colleagues to strengthen their supportive attitudes toward families. Registered nurses who have designated consultation time with patients and families, as in a nurse-led heart failure clinic, may have the most favourable condition for implementing a more supportive approach to families.

Targeting modifiable risk factors in age-related macular degeneration in optometric practice in Sweden.

PURPOSE: The purpose of this study was to investigate the extent to which ophthalmologists and optometrists in Sweden recommend the use of nutritional supplements, changes in diet, or smoking cessation to patients who are at risk of or with signs of age-related macular degeneration (AMD). In addition, this study also examined how these practitioners rate the strength of evidence for nutritional supplements in AMD management and which sources of information they consult to determine supplement recommendations for the prevention or treatment of AMD. METHODS: This study implemented a cross-sectional design using data from a questionnaire. All Swedish optometrists and ophthalmologists who were registered in the membership databases of their respective professional organizations were invited to participate. The questionnaire contained 18 forced choice questions and one free text question and was organized into the following four sections: use of nutritional supplements, dietary advice, smoking and eye diseases, and strength of evidence and the sources of information regarding nutritional supplement interventions. RESULTS: The response rate was 40.3% for optometrists and 5% for ophthalmologists. Optometrists were more likely than ophthalmologists to recommend nutritional supplements in AMD and provided significantly more advice about diet than did the ophthalmologists for both patients at risk for AMD and those with established disease. The ophthalmologists were more likely than the optometrists to rely on the findings from the age-related eye disease studies of AMD regarding treatment with and selection of supplements and to recommend smoking cessation. CONCLUSION: Common evidence-based strategies for AMD management among eye care professionals would presumably be beneficial for AMD patients. Targeted education and implementation strategies may be needed.

Nurses' use of computerised decision support systems affects drug monitoring in nursing homes.

AIM: To describe variations in nurses' perceptions of using a computerised decision support system (CDSS) in drug monitoring. BACKGROUND: There is an increasing focus on incorporating informatics into registered nurses' (RNs) clinical practice. Insight into RNs' perceptions of using a CDSS in drug monitoring can provide a basis for further development of safer practices in drug management. METHOD: A qualitative interview study of 16 RNs. Data were analysed using a phenomenographic approach. RESULTS: The RNs perceived a variety of aspects of using a CDSS in drug monitoring. Aspects of 'time' were evident, as was giving a 'standardisation' to the clinical work. There were perceptions of effects of obtained knowledge and 'evidence' and the division of 'responsibilities' between RNs and physicians of using the CDSS. CONCLUSION: The RNs perceived a CDSS as supportive in drug monitoring, in terms of promoting standardised routines, team-collaboration and providing possibilities for evidence-based clinical practice. IMPLICATIONS: Implementing a CDSS seems to be one feasible strategy to improve RNs' preconditions for safe drug management. Nurse managers' engagement and support in this process are vital for a successful result.

ICT and Intellectual Disability: A Survey of Organizational Support at the Municipal Level in Sweden.

BACKGROUND: Young adults today have grown up in a society where information and communication technology (ICT) support empowerment and social participation. Young adults with mild-to-moderate intellectual disability are at risk for marginalization by the digital divide. The aim was to map and describe how municipal organizations in Sweden organize support in terms of policy and strategies to enable the use of ICT in social care for adults with a mild-to-moderate intellectual disability. METHODS: A quantitative, cross-sectional survey including all municipalities in Sweden (n = 290) was conducted (response rate: 51%, n = 147). Descriptive statistics were used. RESULTS: Findings indicate a lack of organizational support for staff as well as for young adults with mild-to-moderate intellectual disability. CONCLUSION: Municipalities request more knowledge about strategies for making ICT available. Despite the lack of comprehensive strategies for ICT, some Swedish municipalities have taken the initiative in this area.

Registered Nurses' Perceptions about the Situation of Family Caregivers to Patients with Heart Failure - A Focus Group Interview Study.

INTRODUCTION: Heart failure is a growing public health problem associated with poor quality of life and significant morbidity and mortality. The majority of heart failure care is provided by family caregivers, and is associated with caregiver burden and reduced quality of life. Research emphasizes that future nursing interventions should recognize the importance of involving family caregivers to achieve optimal outcomes. AIMS: The aims of this study are to explore registered nurses' perceptions about the situation of family caregivers to patients with heart failure, and registered nurses' interventions, in order to improve family caregivers' situation. METHODS: The study has a qualitative design with an inductive approach. Six focus group interviews were held with 23 registered nurses in three hospitals and three primary health care centres. Data were analysed using qualitative content analysis. RESULTS: Two content areas were identified by the a priori study aims. Four categories and nine sub-categories emerged in the analysis process. The content area "Family caregivers' situation" includes two categories: "To be unburdened" and "To comprehend the heart failure condition and its consequences". The content area "Interventions to improve family caregivers' situation" includes two categories: "Individualized support and information" and "Bridging contact". CONCLUSIONS: Registered nurses perceive family caregivers' situation as burdensome, characterized by worry and uncertainty. In the PHCCs, the continuity and security of an RN as a permanent health care contact was considered an important and sustainable intervention to better care for family caregivers' worry and uncertainty. In the nurse-led heart failure clinics in hospitals, registered nurses can provide family caregivers with the opportunity of involvement in their relative's health care and address congruence and relationship quality within the family through the use of "Shared care" and or Family-centred care. Registered nurses consider it necessary to have a coordinated individual care plan as a basis for collaboration between the county council and the municipality.

Nurses in municipal care of the elderly act as pharmacovigilant intermediaries: a qualitative study of medication management.

OBJECTIVE: To explore registered nurses' experience of medication management in municipal care of the elderly in Sweden, with a focus on their pharmacovigilant activities. DESIGN: A qualitative approach using focus-group discussions was chosen in order to provide in-depth information. Data were analysed by qualitative content analysis. SETTING: Five focus groups in five different long-term care settings in two regions in Sweden. SUBJECT: A total of 21 registered nurses (RNs), four men and 17 women, aged 27-65 years, with 4-34 years of nursing experience. RESULTS: The findings reveal that RNs in municipal long-term care settings can be regarded as "vigilant intermediaries" in the patients' drug treatments. They continuously control the work of staff and physicians and mediate between them, and also compensate for existing shortcomings, both organizational and in the work of health care professionals. RNs depend on other health care professionals to be able to monitor drug treatments and ensure medication safety. They assume expanded responsibilities, sometimes exceeding their formal competence, and try to cover for deficiencies in competence, experience, accessibility, and responsibility-taking. CONCLUSION: The RNs play a central but also complex role as "vigilant intermediaries" in the medication monitoring process, including the issue of responsibility. Improving RNs' possibility to monitor their patients' drug treatments would enable them to prevent adverse drug events in their daily practice. New strategies are justified to facilitate RNs' pharmacovigilant activities. KEY POINTS: This study contributes to the understanding of registered nurses' (RNs') role in medication management in municipal care of the elderly (i.e. detecting, assessing, and preventing adverse drug events or any drug-related problems). RNs can be considered to be "vigilant intermediaries" in elderly patients' drug treatments, working at a distance from staff, physicians, and patients. RNs occasionally take on responsibilities that exceed their formal competence, with the patients' best interests in mind. In order to prevent adverse drug events in municipal care of the elderly, new strategies are justified to facilitate RNs' pharmacovigilant activities.

Informal Caregivers' Experiences and Needs When Caring for a Relative With Heart Failure: An Interview Study.

BACKGROUND: Informal caregivers play an important role for persons with heart failure in strengthening medication adherence, encouraging self-care, and identifying deterioration in health status. Caring for a relative with heart failure can affect informal caregivers' well-being and cause caregiver burden. OBJECTIVE: The objective of this study was to explore informal caregivers' experiences and needs when caring for a relative with heart failure living in their own home. METHODS: The study has a qualitative design with an inductive approach. Interviews were conducted with 14 informal caregivers. Data were analyzed using qualitative content analysis. RESULTS: Two themes emerged: "living in a changed existence" and "struggling and sharing with healthcare." The first theme describes informal caregivers' experiences, needs, and ways of moving forward when living in a changed existence with their relative. Informal caregivers were responsible for the functioning of everyday life, which challenged earlier established roles and lifestyle. They experienced an ever-present uncertainty related to the relative's impending sudden deterioration and to lack of knowledge about the condition. Incongruence was expressed between their own and their relative's understanding and acceptance of the heart failure condition. They also expressed being at peace with their relative and managed to restore new strength and motivation to care. The second theme describes informal caregivers' experiences, needs, and ways in which they handled the healthcare. They felt counted upon but not accounted for, as their care was taken for granted while their need to be seen and acknowledged by healthcare professionals was not met. Informal caregivers experienced an ever-present uncertainty regarding their lack of involvement with healthcare. The lack of involvement with healthcare had a negative impact on the relationship between informal caregivers and their relative due to the mutual loss of important information about changes in medication regimens and the relative's symptoms and well-being. Another cause of negative impact was the lack of opportunity to talk with healthcare professionals about the emotional and relational consequences of heart failure. Healthcare professionals had provided them neither with knowledge on heart failure nor with information on support groups in the municipality. Informal caregivers captured their own mandate through acting as deputies for their relative and claiming their rights of involvement in their relative's healthcare. They also felt confident despite difficult circumstances. The direct access to the medical clinic was a source of relief and they appreciated the contacts with the registered nurses specialized in heart failure. Informal caregivers' own initiatives to participate in meetings were positively received by healthcare professionals. CONCLUSIONS: Informal caregivers' daily life involves decisive changes that are experienced as burdensome. They handled their new situations using different strategies to preserve a sense of "self" and of "us." Informal caregivers express a need for more involvement with healthcare professionals, which may facilitate informal caregivers' situation and improve the dyadic congruence in the relation with their relative.

Nurses' self-reported medication competence in relation to their pharmacovigilant activities in clinical practice.

RATIONALE, AIMS AND OBJECTIVES: Adverse drug reactions (ADRs) represent a major health problem and previous studies show that nurses can have an active role in promoting medication safety. The aim of this study was to describe and evaluate nurses' self-reported competence and pharmacovigilant activities in clinical practice and also to explore the impact of age, education, workplace and nursing experience on these matters. METHODS: This cross-sectional study was based on a questionnaire covering areas related to nurses' medication competence, including knowledge, assessment and information retrieval, and pharmacovigilant activities within these areas, for example, the detection and assessment of ADRs. A 45-item questionnaire was 2013 sent out to 296 nurses in different settings and counties in Sweden. They were selected on the basis of having applied to a university course including pharmacovigilance during 2008-2011. One hundred twenty-four had participated in the courses (exposed) and 172 had applied to the courses but not participated (unexposed). RESULTS: Completed questionnaires were obtained from 75 exposed (60%) and 93 unexposed (54%) nurses. Overall nurses rated themselves high in medication competence but low in pharmacovigilant activities. Significant (P ≤ 0.001) differences between groups were observed regarding medication competence. The exposure of completed dedicated courses in pharmacovigilance was the strongest factor for self-reported medication competence when adjusted for age, other education, workplace and experience. No significant differences between the groups were found regarding the number of pharmacovigilant activities during the 6 months prior to answering the questionnaire. CONCLUSION: Dedicated university courses improved nurses' self-reported competence in pharmacovigilance but did not increase the number of related activities. Education per se seems to be not sufficient to generate pharmacovigilant activities among nurses.

Ocular manifestations and visual functions in patients with Fanconi anaemia.

PURPOSE: Fanconi anaemia (FA) is a multisystemic disorder with ocular implications. This study aims to describe visual function, ocular characteristics and visual processing skills in patients with FA after haematopoietic stem cell transplantation. METHODS: Ten patients with FA, ages 8-17 years, underwent ophthalmological examinations, corneal, periocular and fundus photography, visual evoked potentials (VEPs), visual fields and ocular motor and visual processing information tests. RESULTS: Best-corrected decimal visual acuity was ≥0.65 in all 20 eyes. Microcornea was present in 18 of 18 eyes, short axial lengths in six of six eyes, steep corneal curvatures in four of six eyes, ptosis in 14 of 16 eyes, short palpebral fissures in 15 of 16 eyes and hypotelorism in three of seven patients. Optic disc areas were smaller in patients with FA compared to controls (p = 0.0003 right, p = 0.0003 left eye). Visual fields were abnormal in 4 of 18 eyes, while VEP was abnormal in 1 of 20 eyes. Eight patients had ocular motor dysfunction, while nine of 10 patients showed delay in visual processing skills. CONCLUSION: Patients with FA can present with microcornea, microphthalmia, ptosis, steep corneal curvatures, small optic discs, ptosis and delay in visual processing skills. Detailed ophthalmological and visual processing skills assessments and developmental investigations are important to detect impairments and facilitate appropriate support.

Optic nerve hypoplasia in children and adolescents; prevalence, ocular characteristics and behavioural problems.

PURPOSE: To report prevalence, ocular characteristics and coexisting behavioural problems in children and adolescents with optic nerve hypoplasia (ONH), which is a common cause of visual impairment in children in western countries, often associated with neurological or endocrinological problems and where autism has been reported in severe cases with blindness. METHODS: This is a population-based cross-sectional study of patients <20 years of age who had been diagnosed with ONH and lived in the county of Stockholm in December 2009. Ophthalmological assessments including fundus photographs with optic disc analyses were made. A questionnaire was used to screen for behaviour and development. RESULTS: The prevalence of ONH in all living children <18 years of age in Stockholm was 17.3/100 000 with a prevalence of visual impairment (<0.3) of 3.9/100 000. In total, 66 patients, median age 9.3 years (0.6-19.4), 36 with bilateral and 30 with unilateral ONH, were included in the current study; 53 were re-examined clinically, group A, and 13 agreed to retrospective analyses of existing medical records, group B. Analyses of the optic discs were made in fundus photographs from 53 patients comparing a semi-automated (Retinal Size Tool) and a manual method (Zeki). There was a strong curvilinear correlation (r(S)  = -0.91 p < 0.0001 for both eyes). Behavioural problems were more common (p < 0.05) in bilateral ONH. CONCLUSION: Optic nerve hypoplasia is a common ocular malformation with a prevalence of 17.3/100 000 children and adolescents <18 years of age in Stockholm. Unilateral ONH seems as common as bilateral.

Test-retest variation in macular thickness measurements with the Heidelberg Retina Tomograph.

PURPOSE: The aim was to evaluate the test-retest variation of macular thickness measurements using the Heidelberg Retina Tomograph (HRT3) Retina Module and compare it with the Stratus optical coherence tomography (OCT). As no normative data has been published for the HRT Retina Module, a secondary purpose was to obtain reference values for this technique. METHODS: Thirty healthy subjects and nine patients with macular oedema were examined. The healthy subjects underwent five examinations by two experienced examiners at two different occasions. The patients with maculopathy were examined twice by one examiner on one occasion. All measurements were made with both the HRT3 and the Stratus OCT and the macular thickness measurements were used for calculations of the test-retest variation. RESULTS: In healthy subjects the coefficient of variation (CV) ranged from 4.5 to 8.8 per cent with the HRT3 and from 0.6 to 1.5 per cent with the Stratus OCT. In the nine patients with macular oedema, the HRT CV ranged from 7.9 to16.6 per cent and for OCT from 1.1 to 2.5 per cent. The intra-class correlation in the central area for intra-observer/intra-visit in healthy subjects was 0.78 with the HRT3 and 0.93 with the Stratus OCT. Corresponding values for the patients with macular oedema were 0.64 with the HRT3 and 1.0 with the Stratus OCT. Mean macular thickness was significantly larger in all areas measured with the HRT3 compared with the Stratus OCT. No significant correlations were found between the thickness values from the different instruments. CONCLUSION: In this study, macular thickness was measured with two different techniques. The HRT3 showed lower repeatability and reproducibility as well as a higher CV compared with the Stratus OCT in both healthy subjects and patients with macular oedema. This difference might be of clinical relevance, even though the nine patients with maculopathy in the current study showed abnormal results with both methods.

Visual function, ocular motility and ocular characteristics in patients with mitochondrial complex I deficiency.

PURPOSE: The aims of the present study were to investigate visual function, ocular motility and ocular characteristics in children and young adults with complex I deficiency. MATERIAL AND METHODS: In a prospective study with longitudinal follow-up, the visual and ocular outcome in 13 patients with deficiency in complex I [nicotine-amide adenine dinucleotide (NADH) dehydrogenase] in the mitochondrial respiratory chain is presented. The patients were diagnosed during 1995-2007 and assessed during 1997-2009 at a median age of 12.8 years (range 3.1-23.4). RESULTS: Twelve of 13 patients had visual impairment and/or ocular pathology. Four of 10 patients who co-operated in visual assessment had a best corrected decimal visual acuity of ≤ 0.5 in one or both eyes. Cataract surgery was performed in one patient and another patient showed retinal pigmentations and ptosis. Eleven patients demonstrated ocular motility problems, mainly saccade deficiencies. Five patients had optic atrophy (OA), which was bilateral in four patients. In four siblings, the OA showed a similarity to Leber's Hereditary Optic Neuropathy. These patients also had the 11778 G → A mutation in mitochondrial DNA. Only one patient had normal visual acuity and ocular outcome including refraction and visual fields. Follow-up time was median 3.0 years (range 0-11). CONCLUSION: Visual impairment, ocular motility problems and OA are common in children and young adults with complex I deficiency and should prompt the paediatric ophthalmologist to consider mitochondrial disorders.

Macular structure and function and the development of retinopathy in diabetes.

BACKGROUND: The aim of this study was to evaluate the relationship between structural and functional changes identified with the Rarebit fovea test (RFT) in diabetic patients over two years. In addition, we evaluated whether the RFT changes at baseline can predict vascular changes detectable by conventional screening methods four to six years later. METHODS: Forty-two patients with diabetes and 42 age-matched healthy subjects underwent an initial examination. Two years later, 25 of the diabetic patients and 20 of the controls were re-examined in the same way. Four to six years later, 40 of the diabetic patients underwent a standard screening examination including fundus photography. After two years, all subjects were examined with the RFT and visual acuity (VA) was assessed. In addition, optical coherence tomography (OCT) and fundus photography were performed on the diabetic patients. At a screening examination of the diabetic patients after four to six years, fundus photographs were reviewed. RESULTS: After two years, a marked difference in RFT results was observed between the 20 normal subjects and the 25 patients with diabetes. Results from other tests (VA and central retinal thickness, as measured with OCT) were unchanged compared with the initial examinations. Fundus photography four to six years later of 40 of the 42 diabetic patients showed an increased incidence of retinopathy unrelated to the RFT findings at baseline. CONCLUSIONS: The findings in the current study indicate that the RFT might detect macular dysfunction in diabetic eyes without microvascular changes. This dysfunction increased during a two-year period and presumably reflects neural impairment in diabetes mellitus but did not predict development of retinopathy during the four to six year period.