Keeping Track of My Drinking - Patient Perceptions of Using Smartphone Applications as a Treatment Complement for Alcohol Dependence.

BACKGROUND: Alcohol dependence is common, yet highly undertreated. Smartphone applications (apps) have potential to enhance treatment accessibility and effectiveness, however evidence is limited, especially studies focussing on user experiences. The aim was to describe patient perceptions on the usability and acceptability of self-monitoring apps provided as treatment complement for alcohol dependence. METHODS: Individual semi-structured interviews were conducted through video or phone calls with 21 participants, recruited from a randomized controlled trial at a dependency clinic in Stockholm. The participants had used two specific apps for self-monitoring consumption ("Glasklart" and "iBAC") during 12 wk prior to the interviews. Data was analyzed using Qualitative Content Analysis. RESULTS: Two domains were identified: 1) Smartphone applications as facilitators to treatment, and 2) Barriers to smartphone application use. Using apps within the treatment context was believed to increase the accuracy of the reported consumption. Participants became more aware of their alcohol problem and described the apps as reinforcers that could increase both the motivation to change and the focus on the problem and commitment to treatment. The apps were further described as helpful to control alcohol consumption. However, app usage was constrained by technical problems, unfit app-specific features and procedures, and alcohol-related shame and stigma. DISCUSSION AND CONCLUSIONS: Self-monitoring alcohol apps have several beneficial features that can help assess, track, and control alcohol consumption, and improve communication with clinicians. The results indicate they can be useful complements to treatment for patients with alcohol dependence, but their use can be limited by different, foremost technical, issues.

Smartphone applications for self-monitoring of alcohol consumption may help provide accurate data, increase consumption awareness, focus, motivation, and perceived control;Smartphone applications for self-monitoring of alcohol consumption are considered helpful complements to alcohol treatment;The use of smartphone applications for self-monitoring of alcohol consumption can be constrained by technical problems, and unfit app-specific features and procedures.

Presence of chronic diabetic foot ulcers is associated with more frequent and more advanced retinopathy.

AIMS: To clarify the frequency and severity of diabetic retinopathy in a group of people with Type 2 diabetes and chronic diabetic foot ulcers, and to compare visual acuity, levels of retinopathy and clinical significant macular oedema with a matched control group of people with Type 2 diabetes without a history of chronic diabetic foot ulcers. METHODS: Visual acuity and fundus imaging were evaluated in 90 white people with at least 3 months' duration of full-thickness diabetic foot ulcers below the ankle and the results compared with those in 180 white people with Type 2 diabetes without a history of chronic diabetic foot ulcers (control group). Controls were matched for age, sex and duration of diabetes. RESULTS: Despite similar age and diabetes duration, severe non-proliferative or proliferative diabetic retinopathy was present in 41% of the people in the diabetic foot ulcer group as compared to 15% in the control group (P<0.001). Only 6% in the diabetic foot ulcer group was without any diabetic retinopathy as compared to 34% among controls. Proliferative diabetic retinopathy was more common in the diabetic foot ulcer group (31% vs 8%; P<0.001), but time-to-proliferative diabetic retinopathy did not differ between groups. Clinically significant macular oedema was more frequently present, and the diabetic foot ulcer group exhibited significantly worse results in best and worst eye visual acuity testing. CONCLUSIONS: In this northern European setting almost all people with Type 2 diabetes and chronic diabetic foot ulcers had diabetic retinopathy. Almost one-third had proliferative diabetic retinopathy as compared to <10% in our matched control group. More advanced diabetic retinopathy was linked to worse visual acuity.

Knowledge, risk perception and access to healthcare services for HIV and tuberculosis among university students in Johannesburg, South Africa

background. An increasingly diverse body of students is entering university in South Africa. HIV and tuberculosis (TB) are pressing health issues for this vulnerable population and the university campus offers an opportunity to intervene with health promotion activities. Objectives. This study describes knowledge and risk perception of TB and HIV among high school leavers entering tertiary education. Methods. A cross-sectional survey among first-year students, aged 18-25 years, registered at one of three universities chosen for the study in Johannesburg, South Africa. Informed consent was obtained prior to completing a self-administered, close-ended, structured questionnaire. Factors associated with poor knowledge or high risk perception were identified using modified Poisson regression. Results. In total, 792 students were included; 53.3% (n=438) were categorised as having poor TB knowledge and 52.1% (n=412) poor HIV knowledge, while 43.4% (n=344) were categorised as having high TB risk perception and 39.8% (n=315) high HIV risk perception. Male students were more likely to have poor knowledge of HIV and perceive themselves at risk of acquiring HIV. Low socioeconomic status was associated with a high risk perception of HIV. One in 3 participants (30.6%) stated that they had never had an HIV test. In total, 24students (9 males, 15 female) reported that they were HIV-positive, of whom 15 (62.5%) were on antiretroviral therapy. Only 14.1% had been screened for TB in the past 6 months. Conclusion. The findings indicate a need to enhance health promotion activities among university students so as to aid preventive strategies for reducing the burden of HIV and TB infection

Fixation stability and implication for multifocal electroretinography in patients with neovascular age-related macular degeneration after anti-VEGF treatment.

PURPOSE: To quantify fixation stability in patients with neovascular age-related macular degeneration (nAMD) at baseline, 3 and 6 months after anti-vascular endothelial growth factor (anti-VEGF) treatment and furthermore asses the implications of an unsteady fixation for multifocal electroretinography (mfERG) measurements. METHODS: Fifty eyes of 50 nAMD patients receiving intravitreal anti-VEGF treatment with either bevacizumab or ranibizumab and eight eyes of eight control subjects were included. Fixation stability measurements were performed with the Eye-Link eyetracking system and the retinal area in degrees2 (deg2) containing the 68 % most frequently used fixation points (RAF68) was calculated. MfERG P1 amplitude and implicit time were analyzed in six concentric rings and as a summed response. Patients were examined at baseline, 3 and 6 months. Four different mfERG recordings were performed for the control subjects to mimic an involuntary unstable fixation: normal central fixation, 2.4°, 4.8°, and 7.1° fixation instability. RESULTS: For control subjects, a fixation instability of 2.4° (corresponding to the central hexagon) did not reduce mfERG ring amplitudes significantly, whereas 4.8° and 7.1° fixation instability reduced the amplitudes significantly in rings 1 and 2 (p < 0.001) as well as in the peripheral rings in the 7.1° instability condition (p < 0.001). Fixation stability improved non-significantly for patients at 3 and 6 months. The size of the retinal area of fixation was at baseline, 3 and 6 months negatively correlated to visual acuity (VA) (rbaseline = -0.65, r3 months = -0.60, and r6 months = -0.66 respectively, p < 0.001) and mfERG amplitudes of the three innermost rings (rbaseline = -0.29, p = 0.042, r3 months = -0.43, p = 0.003 and r6 months = -0.31, p = 0.042). The VA cutoff for a fixation area less than 5 deg2 (approximately the central hexagon) was 65, 77, and 68 ETDRS letters (corresponding a maximal Snellen equivalent of 0.31) at baseline, 3 and 6 months, respectively. CONCLUSIONS: MfERG amplitudes in recordings of nAMD patients are at substantial risk of being reduced due to poor fixation as a large number of patients may use a fixation area of more than 5 deg2. Fixation monitoring during recording as well as interpretation of results should be performed with care, especially in patients with poor visual acuity.

Prognosis of schizophrenia in persons with and without a history of cannabis use.

BACKGROUND: The aim of the study was to determinate whether schizophrenia patients with a history of cannabis use have a different prognosis, with regards to readmission and hospital duration, compared with those without a history of cannabis use. METHOD: The present investigation was a cohort study of 50,087 Swedish men with data on cannabis use at the ages of 18-20 years. A total of 357 cases of schizophrenia were identified from in-patient care and followed up from 1973 to 2007. RESULTS: Schizophrenia patients with a history of cannabis use had a higher median duration of first hospital episode (59 days v. 30 days). Patients with a history of cannabis use had a higher median rate of readmission (10 times v. four times). Also, total number of hospital days was higher in patients with a history of cannabis use compared with those without (547 days v. 184 days). Patients with a history of cannabis use had an increased odds of having more than 20 hospital readmissions compared with non-users [3.1, 95% confidence interval (CI) 1.3-7.3] as well as an increased odds of hospital admission lasting more than 2 years (2.4, 95% CI 1.1-7.4) after controlling for diagnosis of personality disorders, family socio-economic position, IQ score, civil status, place of residence, risky use of alcohol and use of other drugs. Patients with a history of cannabis use were less likely to have paranoid schizophrenia compared with never users (8% v. 17%) in the first admission. CONCLUSIONS: Schizophrenia patients with a history of cannabis use had a significantly higher burden of lifetime in-patient care than non-cannabis users. Not only does cannabis increase the risk of schizophrenia, but also our findings indicate that the course and prognosis of schizophrenia may be more severe than schizophrenia cases in general.

Alcohol, mortality and cardiovascular events in a 35 year follow-up of a nationwide representative cohort of 50,000 Swedish conscripts up to age 55.

AIMS: To assess the association between drinking patterns and mortality, and cardiovascular disease in a large cohort of young- and middle-aged men and to assess whether the net balance of harm and protective effect implies protective effect or not. METHODS: Information from health examinations, psychological assessments and alcohol use background in a nationally representative birth cohort of 49,411 male military conscripts aged 18-20 years in 1969/1970, were linked to mortality and hospitalization data through 2004. Cox regression analyses were conducted and attributable proportions (APs) calculated. Confounders (baseline social status, intelligence, personality and smoking) were taken into account. RESULTS: Increasing alcohol use was associated with increasing mortality (2614 deceased) and with decreasing risk for myocardial infarction (MI). The hazard ratio (HR) for mortality was 1.42 [95% confidence interval (CI) 1.10-1.82] with a consumption corresponding to 30 g 100% ethanol/day or more in multivariate analysis. The risk for non-fatal MI was significantly reduced at 60 g 100% ethanol/day (HR 0.37, 95% CI 0.15-0.90), not reduced for fatal MI, and non-significantly reduced for total MI. There was a marked association between alcohol use at conscription and mortality and hospitalization with alcohol-related diagnosis. APs indicate that alcohol caused 420 deaths, 61 cases of non-fatal stroke and protected from 154 cases on non-fatal MI. CONCLUSION: Many more deaths were caused by alcohol than cases of non-fatal MI prevented. From a strict health perspective, we find no support for alcohol use in men below 55 years.

Cannabis, schizophrenia and other non-affective psychoses: 35 years of follow-up of a population-based cohort.

BACKGROUND: There is now strong evidence that cannabis use increases the risk of psychoses including schizophrenia, but the relationship between cannabis and different psychotic disorders, as well as the mechanisms, are poorly known. We aimed to assess types of psychotic outcomes after use of cannabis in adolescence and variation in risk over time. METHOD: A cohort of 50 087 military conscripts with data on cannabis use in late adolescence was followed up during 35 years with regard to in-patient care for psychotic diagnoses. RESULTS: Odds ratios for psychotic outcomes among frequent cannabis users compared with non-users were 3.7 [95% confidence interval (CI) 2.3-5.8] for schizophrenia, 2.2 (95% CI 1.0-4.7) for brief psychosis and 2.0 (95% CI 0.8-4.7) for other non-affective psychoses. Risk of schizophrenia declined over the decades in moderate users but much less so in frequent users. The presence of a brief psychosis did not increase risk of later schizophrenia more in cannabis users compared with non-users. CONCLUSIONS: Our results confirm an increased risk of schizophrenia in a long-term perspective, although the risk declined over time in moderate users.

Electrophysiological studies in newly onset type 2 diabetes without visible vascular retinopathy.

The purpose of this study was to investigate the early alterations of retinal function, assessed with electrophysiology, in newly onset type 2 diabetes patients without vascular retinopathy. Seventeen patients with newly diagnosed type 2 diabetes (duration 7±3 months), without any vascular retinopathy in fundus photographs, were examined with full-field electroretinogram (ERG) and multifocal ERG (mfERG). The results were compared with those of age-matched subjects without diabetes. In the dark-adapted full-field ERG, the a-wave and the 30-Hz flicker implicit times were delayed in diabetes patients compared to controls, P=0.001 and P=0.020. In the first-order kernel of the mfERG, the first positive wave, P1, was delayed in all areas measured. The electrophysiological examinations demonstrate early alterations of retinal function characterised by a delayed a-wave implicit time in the dark-adapted full-field ERG, representing the rod signalling, and alterations in the multifocal ERG reflecting cone and/or postreceptoral function.

Long-term effects of surgical correction of adrenal hyperplasia and adenoma causing primary aldosteronism.

PURPOSE: The purpose of this is to study long-time results of surgery for primary aldosteronism. MATERIALS AND METHODS: Thirty patients operated on for primary aldosteronism were followed for an average of 7 years. All but five required potassium substitution. Systolic as well as diastolic hypertension (mean 157/93 mmHg) was present necessitating one to five antihypertensive drugs daily (mean 2.33). Preoperative indications for surgery included presumed adenoma (aldosterone-producing adenoma (APA)) or in one case unilateral dominance of hyperplasia. RESULTS: Histopathology was classified into adenoma (n = 9), dominant nodule (n = 16), and general hyperplasia without dominating nodules (n = 5), demonstrating a higher frequency of hyperplasia than anticipated. Long-term results revealed well-controlled blood pressure (BP; mean 134/80 mmHg). Antihypertensive medication was reduced (average of 1.78 per day), but only 36% of the patients were taken off these drugs completely. S-Aldosterone was normalized. All but one (a recurrence) were normokalemic without potassium substitution at follow-up. The APA group needed less medication (median 0.5 vs. 1.5 and 2 per day) and more patients in this group were totally medication free (50%). Two recurrences occurred in the group with general hyperplasia without dominating nodules. CONCLUSION: Nodular hyperplasia is more common than anticipated. Hypersecretion of aldosterone may be released from a large nodule identified as an adenoma, as well as from a generally hyperplastic gland that has not been identified as such. Nevertheless, surgery for lateralized disease results in good long-term control of BP with less antihypertensive medication. However, patients with dominant nodule or general hyperplasia without dominating nodules need more postoperative treatment than patients with APA. The majority of patients do not achieve normotension without medications, but they do become normokalemic.

Peritonectomy with high voltage electrocautery generates higher levels of ultrafine smoke particles.

BACKGROUND: To adequately perform peritonectomy, the use of an electrocautery device at a high voltage is recommended. The aim of this study was to analyse the amount of airborne and ultrafine particles (UFP) generated during peritonectomy and to compare this with standard colon and rectal cancer surgery (CRC). METHOD: UFP was measured approximately 2-3 cm from the breathing area of the surgeon (personal sampling) and 3 m from where the electrocautery smoke was generated (stationary sampling) from 14 consecutive peritonectomy procedures and 11 standard CRC resections. The sampling was by P-Trak UFP counter that has the capacity to detect particle size ranging from 0.02 to 1 microm. RESULTS: The cumulative level of UFP of personal sampling in the peritonectomy group was higher (9.3 x 10(6) particle/ml/h (pt/ml/h)) than in the control group (4.8 x 10(5) pt/ml/h). A higher cumulative level of UFP in stationary sampling was observed in the PC group (2.6 x 10(6) pt/ml/h) than in the control group (3.9 x 10(4)pt/ml/h). CONCLUSION: Peritonectomy procedure with high voltage electrocautery generates elevated levels of UFP than standard CRC surgery does. The level of UFP produced by a peritonectomy is comparable to cigarette smoking. More efficient smoke evacuator systems are needed in order to reduce the levels of UFP generated during electrocautery surgery.

Retinal function and histopathology in rabbits treated with Topiramate.

PURPOSE: To evaluate retinal function and histopathology in rabbits treated orally with the anti-epileptic drug topiramate. METHODS: Six rabbits were treated with a daily oral dose of topiramate during a period of eight months. Six rabbits receiving water served as controls. Blood samples were analyzed for determination of topiramate serum levels in order to ensure successful drug exposition. Standardized full-field electroretinograms (ERGs) were performed before treatment and then at 2, 3 and 8 months during the treatment period. After terminating treatment the rabbits were sacrificed and the morphology of the sectioned retina was studied. RESULTS: After eight months of treatment the full-field ERG demonstrated normal rod function in treated and control rabbits, but the light adapted 30 Hz flicker b-wave amplitude was significantly reduced in the treated rabbits. This was the case for both the light adapted (Wilcoxon signed ranks test, P = 0.046) and the dark adapted (Wilcoxon signed ranks test, P = 0.028) 30 Hz flicker response from the treated rabbits. Retinal immunohistology revealed a severe accumulation of GABA in amacrine cells and in the inner plexiform layer in 4 of 6 treated rabbits compared to the controls. CONCLUSIONS: Topiramate, orally administrated to rabbits, may cause a significant reduction of the retinal function demonstrated by the reduced b-wave amplitude in the full-field ERG, as well as changes in immunohistology characterized by a severe accumulation of GABA in the inner retina. The retinal dysfunction and the morphological changes indicate that topiramat may damage the retina, similarly to vigabatrin (another anti-epileptic drug).

Retinal dysfunction and anterior segment deposits in a patient treated with rifabutin.

PURPOSE: To describe the clinical and electrophysiological findings in a young boy with decreased vision possibly due to retinal damage by rifabutin. METHODS: An 8-year-old boy with osteomyelitis was referred due to visual disturbance. During a period of 4 years, the boy was examined six times with electroretinography. Ophthalmological examination included testing of visual acuity, slit-lamp inspection, fundus inspection, fundus photography and kinetic perimetry. Two electrophysiological methods were performed for objective evaluation of retinal function, namely full-field electroretinography and multifocal electroretinography. RESULTS: We found a slightly reduced visual acuity, a slowly increasing amount of yellow-white deposits on the posterior surface of the cornea and on the anterior part of the lens, a normal fundus appearance, and normal visual fields. However, the electroretinogram was abnormal on several occasions during therapy with rifabutin, but returned to normal 3 months after withdrawal of the medication. The multifocal electroretinogram returned to normal after the full-field electroretinogram had done so. The anterior chamber deposits still remain. CONCLUSION: Long-term treatment with rifabutin may have a reversible and previously undescribed side-effect on retinal function. The drug may also accumulate irreversibly on the posterior surface of the cornea and on the anterior surface of the lens. We suggest that objective evaluation of retinal function with electrophysiological methods should be performed in patients with visual disturbance during treatment with rifabutin.

Different clinical expressions in two families with Stargardt's macular dystrophy (STGD1).

PURPOSE: To describe the clinical expressions, with emphasis on electrophysiological examinations, in two Swedish families with Stargardt's macular dystrophy (STGD1). METHODS: Two pairs of siblings with STGD1, for whom diagnosis had been confirmed by genetic linkage to the ABCA4 gene region, were examined regarding visual acuity, kinetic perimetry, fundus photography, full-field ERG and multifocal ERG (MERG). Possible disease-causing mutations were screened for by DNA sequencing of selected regions of the ABCA4 gene. RESULTS: All STGD1 patients had visual acuity 0.07-0.1. The two families presented different fundus appearances, MERGs and implicit times on 30 Hz flicker white light full-field ERGs. Genetic analysis revealed one unique sequence variation in exon 19 of the ABCA4 gene, in one allele from the patients of one of the families. This point mutation causes the amino acid substitution T972N in the ABCR protein. CONCLUSION: Two pairs of siblings with STGD1 presented two different expressions of the disease regarding the distribution of the retinal dysfunction. One possible molecular explanation to the different clinical expressions may be the T972N substitution present in the ABCR protein in one of the STGD1 families investigated.

CNGA3 mutations in hereditary cone photoreceptor disorders.

We recently showed that mutations in the CNGA3 gene encoding the alpha-subunit of the cone photoreceptor cGMP-gated channel cause autosomal recessive complete achromatopsia linked to chromosome 2q11. We now report the results of a first comprehensive screening for CNGA3 mutations in a cohort of 258 additional independent families with hereditary cone photoreceptor disorders. CNGA3 mutations were detected not only in patients with the complete form of achromatopsia but also in incomplete achromats with residual cone photoreceptor function and (rarely) in patients with evidence for severe progressive cone dystrophy. In total, mutations were identified in 53 independent families comprising 38 new CNGA3 mutations, in addition to the 8 mutations reported elsewhere. Apparently, both mutant alleles were identified in 47 families, including 16 families with presumed homozygous mutations and 31 families with two heterozygous mutations. Single heterozygous mutations were identified in six additional families. The majority of all known CNGA3 mutations (39/46) are amino acid substitutions compared with only four stop-codon mutations, two 1-bp insertions and one 3-bp in-frame deletion. The missense mutations mostly affect amino acids conserved among the members of the cyclic nucleotide gated (CNG) channel family and cluster at the cytoplasmic face of transmembrane domains (TM) S1 and S2, in TM S4, and in the cGMP-binding domain. Several mutations were identified recurrently (e.g., R277C, R283W, R436W, and F547L). These four mutations account for 41.8% of all detected mutant CNGA3 alleles. Haplotype analysis suggests that the R436W and F547L mutant alleles have multiple origins, whereas we found evidence that the R283W alleles, which are particularly frequent among patients from Scandinavia and northern Italy, have a common origin.

Clinical variables and pro-inflammatory activation in paediatric heart surgery.

OBJECTIVES: The first aim was to analyse the role of preoperative characteristics and perioperative variables in predicting the inflammatory response during and early after operations for congenital heart malformations of moderate to severe complexity. The second aim was to correlate complement and cytokine activation during the same period with clinical variables reflecting the postoperative course. METHODS: Prospective descriptive clinical study that involved 22 consecutive children (1-28 months). Five children had Down's syndrome. Concentrations of C3a, C5b-9 and IL-6 were measured. RESULTS: C3a, C5b-9 and IL-6 increased significantly during the study period (ANOVA: C3a, p = 0.001; C5b-9, p = 0; IL-6, p = 0). C3a correlated with preoperative haemoglobin (r = 0.71, p = 0.0002) and CPB time (r = 0.72, p=0.0005). C5b-9 correlated with CPB time (r= 0.58, p=0.004). IL-6 related to presence of Down's syndrome (p=0.0001) and correlated with preoperative haemoglobin (r=0.55, p=0.02), preoperative weight deviation (r = -0.52, p = 0.03) and time in the ventilator (r = 0.68, p = 0.002). CONCLUSIONS: Preoperative and perioperative characteristics predict the inflammatory response during open heart surgery in infants and small children. IL-6 response is related to the postoperative course.

Electrophysiological findings in two young patients with Bothnia dystrophy and a mutation in the RLBP1 gene.

PURPOSE: To characterize the clinical phenotype, with emphasis on electrophysiology, of two children with suspected Bothnia dystrophy. METHODS: Two unrelated affected patients, 10 and 11 years old, were studied. Ophthalmological examination included testing of visual acuity, fundus inspection and fundus photography, kinetic perimetry, full-field electroretinogram (ERG), and multifocal ERG. The presence of a mutation in exon 7 of the RLBP1 gene was investigated by DNA sequencing. RESULTS: Both patients were homozygous for the Arg234Trp-causing mutation in the RLBP1 gene, but the resulting disease phenotype appeared to vary somewhat between them. Visual acuity was moderately reduced in one patient and normal in the other. Fundus inspection at this age revealed no pathology in either patient and there were no signs of retinitis punctata albescens, which has been described previously as a frequent clinical feature of Bothnia dystrophy. The result of kinetic perimetry was normal. The final rod threshold was moderately elevated. Full-field ERG demonstrated the uncommon combination of absent rod response and normal cone response after 40 minutes of dark adaptation. However, after prolonged dark adaptation (20-24 h), both the rod response and the dark adaptation threshold became normal. Multifocal ERG was performed in one of the patients (the one with normal visual acuity and normal fundus appearance) and showed a reduced cone response in the central region of the tested area. There was no improvement of the multifocal ERG result after 20-24 h of dark adaptation. CONCLUSION: Patients with mutations in the RLBP1 gene (Arg234Trp) may have a normal fundus appearance early in the disease course. Multifocal ERG can be used for the objective documentation of the disturbed macular function, especially when the patient's visual acuity and fundus appearance are normal. The rod response is absent in the electroretinogram; however, after prolonged dark adaptation (20-24 hours), the rods recover completely. The central cones do not seem to recover.

Best's vitelliform macular dystrophy caused by a new mutation (Val89Ala) in the VMD2 gene.

PURPOSE: To describe the clinical phenotype in a family with Best's vitelliform macular dystrophy (BMD) and a new mutation (Val89Ala) in the VMD2 gene. METHODS: The genotype was determined by direct sequence analysis of the individual exons of VMD2. Nine members of a family with BMD were examined. The examination included best-corrected visual acuity, electro-oculography (EOG), fundus examination, and photography. Four of the patients were also examined with full-field ERG and three with multifocal ERG. RESULTS: A T-to-C substitution was identified at position 370 in the cDNA of VMD2, leading to a Val89Ala change in the protein. Six patients, five with the Val89Ala mutation and a nine-year-old boy without the mutation, presented with a pathological Arden ratio on EOG examination. Most of the patients with BMD in this family had an onset of visual failure by the age of 40-50 years. The older patients in the family demonstrated atrophic macular dystrophy. CONCLUSIONS: Patients with BMD and the Val89Ala mutation in the VMD2 gene can present with a phenotype of a mostly late-onset visual failure. These BMD patients, who present with visual failure and macular degeneration in middle age, can be misdiagnosed as being affected with adult-onset macular dystrophies instead of BMD, because the latter is often regarded as a disease of childhood and adolescence.

Multifocal ERG and full-field ERG in patients on long-term vigabatrin medication.

PURPOSE: To examine the retinal function with different electrophysiological methods in twelve Swedish patients on long-term treatment (2-10 years) with the anti-epileptic drug vigabatrin. METHODS: Ophthalmological examination of twelve consecutive patients included testing of visual acuity, fundus inspection and fundus photography, kinetic perimetry, full-field ERG and multifocal ERG. RESULTS: All patients had a visual acuity of 0.7 or better. Fundus inspection revealed no pathology except in one patient who had a pallor of the optic disc. All patients had a normal appearance of the macula. The result of kinetic perimetry was normal in five patients while seven patients had a concentric defect of the visual field. The 30 Hz flicker cone b-wave amplitude in the full-field ERG was abnormal in all of the seven patients with a visual field defect. None of the patients with normal visual fields had a reduction of the 30 Hz flicker cone b-wave amplitude. Six of the twelve patients had a reduced multifocal ERG response but without any correlation with visual field defect. CONCLUSION: Long-term treatment with vigabatrin seems to selectively reduce retinal cone function. The visual field defects in patients taking vigabatrin correlate with pathology in the full-field ERG (reduction of the cone b-wave amplitude). The results from this study indicate that electroretinography can be used for monitoring patients taking vigabatrin in a more objective manner than with visual field testing.

Perioperative morbidity and mortality in chronic alcoholic patients.

This article represents the proceedings of a symposium at the 2000 ISBRA Meeting in Yokohama, Japan. The chairs were Claudia Spies and Hanne Tønnesen. The presentations were (1) Relevance of alcohol misuse in surgical patients, by Hanne Tønnesen; (2) Diagnosis of alcohol abuse and alcohol dependence, by Sven Andreasson; (3) Diagnosis of acute alcohol misuse, by Anders Helander; (4) Preoperative intervention for excessive alcohol consumption, by Kate Conigrave; and (5) Prevention and treatment of perioperative complications in chronic alcoholics, by Claudia Spies.