BMJ No. 7063 Volume 313 Saturday 26 October 1996
Design - Clinical and laboratory data were collected from patients diagnosed as having homocystinuria due to cystathionine synthase deficiency, with special reference to the ages at which the patients had their first major signs of the disease, ectopia lentis was established, and homocystinuria was diagnosed.
Setting - University hospital in the Netherlands.
Subjects - 34 patients (18 males) in whom homocystinuria due to cystathionine synthase deficiency was diagnosed in the period 1970-94.
Results - Among 34 consecutively detected homocystinuria patients the mean age at diagnosis of homocystinuria was 24 (range 1-61) years. Despite frequent ocular manifestations, serious complications in the vascular, skeletal, and central nervous systems, and repeated examinations performed in these patients by clinicians of various disciplines, there was a mean delay of 11 (0-43) years between the first major signs of the disease (at mean age 13 (1-40) years) and the ultimate diagnosis of homocystinuria. Even when ectopia lentis was diagnosed (in 26 (76%)patients, mean age l8 (1-50) years), this did not lead to adequate biochemical analysis for homocystinuria at the time of detection,causing a mean diagnostic delay of 8 (0-24) years in these patients.
Conclusions - Three factors should have precipitatedthe diagnosis of homocystinuria: early recognition that unusual myopia (high, very high, abnormal progressive, or at young age) was caused by subluxation of the ocular lenses; awareness that the occurrence of myopia combined with systemic complications ("myopia plus") might be due to homocystinuria; and appropriate biochemical investigations carried out in patients with ectopia lentis and in their siblings.
University Hospital Nijmegen,
PO Box 9101,
6500 HB Nijmegen,
Netherlands
Johan R M Cruysberg, ophthalmologist
Godfried H J Boers,internist for metabolic diseases
J M Frans Trijbels, professor of clinical biochemistry
August F Deutman, professor of ophthalmology
Correspondence to: Dr Cruysberg.
Design - Prospective and cross sectional study of a large randomly selected population sample.
Setting - Part of the Bruneck study 1990-5 (Bolzano province, Italy).
Subjects - 820 healthy non-diabetic women and men aged 40-79 years.
Main outcome measure - Concentrations of fasting and post-glucose insulin, cholesterol, apolipoproteins, triglycerides, Lp(a) lipoprotein, glucose, fibrinogen, and antithrombin III; blood pressure; insulin resistance estimated by the homeostasis model assessment.
Results - Fasting insulin concentrations in those who did not drink alcohol and subjects reporting low (1-50 g/day), moderate (51-99 g/day), and heavy (100 g/day or more) alcohol intake were 12.4, 10.0, 8.7, and 7.1 mU/l (P < 0.001). Likewise, post-glucose insulin concentrations and estimates for insulin resistance assessed by the homeostasis model assessment decreased significantly with increasing amounts of regular alcohol consumption. These trends were independent of sex, body mass index, physical activity, cigarette smoking, medication, and diet (P < 0.001). Regular alcohol intake predicted multiple changes in vascular risk factors over a five year period including increased concentrations of high density lipoprotein cholesterol and apolipoprotein A I; higher blood pressure; and decreased concentration of antithrombin III. These associations were in part attributable to the decrease in insulin concentrations observed among alcohol consumers.
Conclusions - Low to moderate amounts of alcohol, when taken on a regular basis, improve insulin sensitivity. Insulin is a potential intermediate component in the association between alcohol consumption and vascular risk factors (metabolic syndrome).
Department of Neurology,
University of Innsbruck,
Innsbruck,
Anichst 35,
A-6020 Innsbruck,
Austria
Stefan Kiechl, lecturer in neurology
Johann Willeit, associate professor
Werner Poewe, professor
Department of Internal Medicine,
Bruneck Hospital,
I-39031 Bruneck,
Italy
Georg Egger, consultant cardiologist
Friedrich Oberhollenzer, head of hospital
Department of Endocrinology and Metabolism,
Ospedale Civile Maggiore,
I-37126 Verona,
Italy
Michele Muggeo, professor
Enzo Bonora, associate professor
Correspondence to: Dr Kiechl.
Design - Cross sectional surveys in 1987 and 1992 determined mean total cholesterol concentrations in the population. A random sample of respondents in the 1992 survey completed a nutrition questionnaire that included questions on diet in the previous 24 hours.
Setting - Mauritius.
Intervention - In 1987 the government of Mauritius changed the composition of the commonly used cooking oil from being mostly palm oil (high in saturated fatty acids) to being wholly soya bean oil (high in unsaturated fatty acids).
Subjects - 5,080 and 5,162 subjects in 1987 and 1992 cross sectional surveys. 2,059 subjects aged 30-64 years were randomly selected from the respondents of the 1992 survey to take part in the nutrition survey
Main outcome measures - Fatty acid composition of phospholipids in pooled serum samples from men and women from the two surveys; measured and predicted change in serum cholesterol concentration.
Results - From 1987 to 1992 total cholesterol concentrations fell significantly by 0.79 mmol/l (P < 0.001) in men and 0.82 mmol/l (P < 0.001) in women. The estimated intake of saturated fatty acids decreased by 3.5% of energy intake in men and by 3.6% in women, and the intake of polyunsaturated fatty acids increased by 5.5% and 5.6% of energy intake, respectively. These changes were reflected in changes in the fatty acid composition of serum phospholipids, and according to Keys' formula these changes explained much of the decrease in serum cholesterol concentrations (predicted decrease of 0.38 mmol/l in men and by 0.40 mmol/l in women).
Conclusion - Dietary changes that entailed a reductionin the saturated fat content of a ubiquitous cooking oil explained mostof the observed decrease in serum cholesterol concentration over fiveyears in the population of Mauritius.
National Public Health Institute,
Diabetes and Genetic Epidemiology Unit,
Department of Epidemiology and Health Promotion
Mannerheimintie 166,
FIN-00300 Helsinki,
Finland
Ulla Uusitalo, research nutritionist
Jaakko Tuomilehto, professor
National Institute of Public Health and the Environment,
Department of Chronic Disease and Environmental Epidemiology,
3720 Bilthoven,
Netherlands
Edith J M Feskens, senior epidemiologist
Correspondence to: Dr Tuomilehto.
Design - Computer simulation of 100 fund years assuming Poisson distribution of referrals.
Setting - British general practices that have opted to become total purchasing sites. Referral rates and price estimates were supplied by South and West Devon Health Commission.
Main outcome measures - Variation in referral costs to purchasers in relation to size of risk pool (person years at risk).
Results - Random variation in referral costs increased as the size of the risk pool decreased. Variation increased greatly below 30 000 person years. The mean simulated cost of the referral categories considered was 2.8% of total NHS hospital and community service costs, and the maximum simulated cost for 7000 person years was 6.8%. Simulated variation was robust to assumptions about prices and referral rates for specific types of referral.
Conclusion - Rare costly referrals seem unlikely to bankrupt total purchasing sites. The management of risk is not in itself justification for total purchasing to be based in several general practices in order to generate large populations. There are other ways of managing risk. Sites can easily explore options by simulations using local referral rates and prices.
Department of Social Medicine,
University of Bristol,
Bristol BS8 2PR
Max O Bachmann, lecturer in public health medicine
Gwyn Bevan, senior lecturer in health economics
Correspondence to: Dr Bachmann.
Design - Randomised controlled trial with blind follow up six months after recruitment.
Setting - Community of south east London.
Subject - 69 people aged 65 or over who received home care and were depressed according to criteria of the standardised automatic geriatric examination for computer assisted taxonomy (AGECAT). 33 were randomly allocated to an intervention group and 36 to a control group.
Intervention - Members of the intervention group received an individual package of care that was formulated by the community psychogeriatric team in their catchment area and implemented by a researcher working as a member of that team. The control group received normal general practitioner care.
Main outcome measures - Recovery from depression (AGECAT case at recruitment but non-case at follow up).
Results - Data were analysed on an intention to treat basis. 19 (58%) of the intervention group recovered compared with only nine (25%) of the control group, a difference of 33% (95% confidence interval 10% to 55%). This powerful treatment effect persisted after controlling for possible confounders in logistic regression analysis, with members of the intervention group more likely than members of the control group to have recovered at follow up (odds ratio 9.0 (2.0 to 41.5)). This did not seem to be a simple effect of antidepressant prescription: use of antidepressants at follow up did not have a significant effect (multiply adjusted odds ratio 0.3 (0.0 to 1.9)).
Conclusions - Depression is treatable in elderly people receiving home care. Therapeutic nihilism based on an assumed poor response to treatment in these socially isolated, disabled elderly people in the community is not supported.
Section of Epidemiology and General Practice,
Institute of Psychiatry,
London SE5 8AF
Sube Banerjee, lecturer in psychiatry
Anthony H Mann, professor of epidemiological psychiatry
Section of Old Age Psychiatry,
United Medical and Dental Schools of Guy's and
St Thomas's Hospitals,
Guy's Campus,
Guy's Hospital,
London SE1 9RT
Kim Shamash, senior registrar
Alastair I D Macdonald, senior lecturer in old age psychiatry
Correspondence to: Dr Banerjee