bluuebunny/crossref_metadata_2025 huggingface dataset:
‘Change to cup’: an audit to determine parental awareness and practices in changing from bottle to cup
Authors: Avery A. , Baxter A.
Published: March 2003

Background

It is recommended that infants from the age of 6 months should be introduced to drinking from a cup and the use of a bottle should be actively discouraged after the age of 1 year. The number of young children using a feeder bottle after the age of 1 year is not known but it is recognized by health professionals as a ‘growing’ concern.

Aims

To obtain local information about the number of children using a bottle past the age of 1 year, what fluids were being offered and to assess parental awareness about when a cup can be introduced.

Methods

The study data was collected by questionnaire from 407 parents between June 1998 and January 1999. Socially disadvantaged groups were targetted for the sample group as well as care being taken to include a representative ethnic population.

Results

Ninety‐nine per cent of the study infants were below the age of 2 years, with the age range being 12–28 months. Thirty per cent were using only a cup/beaker, 8% of infants still used a bottle only, and 62% used both a bottle and a cup. Drinks/food other than milk and water had been offered from the bottle by 47% of respondents. More than 60% of the parents thought that the age at which the use of a bottle should be discontinued was older than that recommended. Only 18% thought a cup/beaker could be introduced as early as 6 months.

Conclusion

The results show that there is a need to actively encourage the use of a cup at an earlier age and to discourage the inappropriate and extended use of a feeder bottle. Barriers to change need to be addressed.
Dietary interventions in Finland, Norway and Sweden: nutrition policies and strategies
Authors: Roos G. , Lean M. , Anderson A.
Published: March 2003

Aims

To describe the organization and implementation of nutrition policies, and examine intervention strategies for dietary change in three Scandinavian countries.

Methods

Descriptions of nutrition policies and dietary intervention strategies are based on published nutrition policy research and reports.

Results

All countries studied have adopted formal nutrition policies. Norway issued its first white paper in 1976, the Finnish National Nutrition Council published an action plan in 1989, and the Swedish Government issued an official action plan in 1995. Norway has a centralized National Nutrition Council with a permanent administration whereas the responsibilities and administration are more spread out between several authorities and groups in Finland and Sweden. Amongst the dietary intervention strategies employed, a Norwegian nutrition campaign, symbol labelling of foods in Sweden, the community‐based North Karelia Project in Finland, and mass catering in Finland and Sweden have been selected as potentially transferable.

Conclusions

Policy documents serve as guidelines for activities and assist in achieving dietary targets. A responsible administrative body with advice from a standing expert committee is valuable for implementation. Guidelines, recommendations or voluntary labelling standards can be incentives to product development and changes to food production. Regional demonstration projects may also encourage action and collaboration.
Socioeconomic differences in food purchasing behaviour and suggested implications for diet‐related health promotion
Authors: Turrell G. , Hewitt B. , Patterson C. , Oldenburg B. , Gould T.
Published: March 2003

Background  The relationship between socioeconomic position (SEP) and diet has been examined mainly on the basis of food and nutrient intake. As a complement to this work, we focused on the socioeconomic patterning of food purchasing, as many educational dietary messages emphasize behaviours such as food choice when shopping. Also, the type of food people buy influences the quality of their nutrient intake.

Methods  A probability sample of households in Brisbane City, Australia (n = 1003, 66.4% response rate). Data were collected using face‐to‐face interviews. SEP was measured using education, occupation and household income. Food purchasing was examined on the basis of grocery items (including meat and chicken) and fruit and vegetables.

Results  Significant associations were found between each socioeconomic indicator and food purchasing. Persons from socioeconomically disadvantaged backgrounds were less likely to purchase grocery foods that were comparatively high in fibre and low in fat, salt and sugar. The least educated, those employed in blue‐collar (manual) occupations and residents of low income households purchased fewer types of fruit and vegetables, and less regularly, than their higher status counterparts.

Conclusions  Health promotion efforts aimed at narrowing socioeconomic differences in food purchasing need to be designed and implemented with an understanding of, and a sensitivity to, the barriers to nutritional improvement that difficult life circumstances can impose.
Review of methods used to estimate non‐milk extrinsic sugars
Authors: Kelly S. A. M. , Moynihan P. J. , Rugg‐Gunn A. J. , Summerbell C. D.
Published: March 2003

Background  Those sugars in foods, which are potentially damaging to dental health, were classified by the Committee on Medical Aspects of Food Policy (COMA) as non‐milk extrinsic sugars (NMES). The NMES include sugars outside the cellular structure of a food, excluding the sugars naturally present in milk and milk products. The NMES should contribute no more than 10% of energy intake (Department of Health, 1991). A number of studies have been published where NMES content of foods has been estimated. The purpose of this study was to carry out a comprehensive literature review using a methodical search strategy in order to identify the different methods that have been used for NMES estimation.

Methods  Databases searched were MEDLINE, EMBASE, Health‐CD and Health Management Information Consortium (HMIC) (as sources of UK government and other official publications).

Results  In total, 32 publications were found in which NMES values were reported and five different methods to estimate NMES were identified. No published method provided sufficient information to clearly differentiate between methods and inadequate detail was given to support replication of any of the methods. Of these five methods, The Ministry of Agriculture, Fisheries and Food (MAFF) have published three different descriptions of methods of NMES estimation used in UK national dietary surveys published since 1989. However, one method has been described consistently in the National Diet and Nutrition surveys published since 1994.

Conclusions  A single, uniform approach to the estimation of NMES for application in nutritional surveys is essential for cross‐comparison between surveys. The results show that there is a clear need for one standardized approach for the estimation of NMES in foods.
‘As required’ neuroleptics: Have these drugs a place in the management of challenging behaviour in intellectual disability?
Authors: Bouras N. , Suresh T.
Published: March 2003

The present study examined the prescription pattern and usage of ‘as required’ neuroleptics (PRNs) in the management of challenging behaviours in people with intellectual disability in a hospital setting. The prescription cards and drug administration records of residents were scrutinized to see how many of them were prescribed PRNs and how many had actually received these drugs during the previous 3 months. The results were compared in the following groups: (1) males and females; (2) the behavioural and nonbehavioural sectors; (3) intensive treatment wards (ITWs) for severe challenging behaviour which had a better staff:resident ratio than the rest of the behavioural wards; and (4) those prescribed PRNs together with the daily administration of neuroleptics and those without. It was found that more females used PRNs and that the prescription rate was higher in the behavioural sector. A better staff:resident ratio did not bring about any reduction in the usage of PRNs. The use of these drugs was not influenced by whether PRNs were prescribed along with regular neuroleptics or not. Further research is required to delineate the precise role of PRNs for challenging behaviours in this population, and to determine if PRNs can be used along with other modes of therapeutic interventions, instead of administering neuroleptics on a regular basis for this indication.
Economic impact of feeding a phenylalanine‐restricted diet to adults with previously untreated phenylketonuria
Authors: Brown M. C. J. , Guest J. F.
Published: March 2003

The aim of the present study was to estimate the direct healthcare cost of managing adults with previously untreated phenylketonuria (PKU) for one year before any dietary restrictions and for the first year after a phenylalanine‐ (PHE‐) restricted diet was introduced. The resource use and corresponding costs were estimated from medical records and interviews with health care professionals experienced in caring for adults with previously untreated PKU. The mean annual cost of caring for a client being fed an unrestricted diet was estimated to be ??83 996. In the first year after introducing a PHE‐restricted diet, the mean annual cost was reduced by ??20 647 to ??63 348 as a result of a reduction in nursing time, hospitalizations, outpatient clinic visits and medications. However, the economic benefit of the diet depended on whether the clients were previously high or low users of nursing care. Nursing time was the key cost‐driver, accounting for 79% of the cost of managing high users and 31% of the management cost for low users. In contrast, the acquisition cost of a PHE‐restricted diet accounted for up to 6% of the cost for managing high users and 15% of the management cost for low users. Sensitivity analyses showed that introducing a PHE‐restricted diet reduces the annual cost of care, provided that annual nursing time was reduced by more than 8% or more than 5% of clients respond to the diet. The clients showed fewer negative behaviours when being fed a PHE‐restricted diet, which may account for the observed reduction in nursing time needed to care for these clients. In conclusion, feeding a PHE‐restricted diet to adults with previously untreated PKU leads to economic benefits to the UK's National Health Service and society in general.
Association of a balanced chromosomal translocation (4; 12)(q21.3; q15), affective disorder and autism
Authors: Nasr A. , Roy M.
Published: March 2003

This case report describes a set of monozygotic twins with severe intellectual disability, autism and affective disorder with a balanced translocation between chromosomes 4 and 12. Their mother, who carries the same balanced translocation, had severe postnatal depression. The association between autism affective disorder and these chromosome break points has not been reported previously. The implications are discussed.
Sexual abuse in children and adolescents with intellectual disability
Authors: Balogh R. , Bretherton K. , Whibley S. , Berney T. , Graham S. , Richold P. , Worsley C. , Firth H.
Published: March 2003

The present authors conducted a study of the occurrence of victimization and the perpetration of sexual abuse among 43 in‐patients with intellectual disability aged between 9 and 21 years who were admitted to a child and adolescent psychiatric in‐patient department over a period of 5 years. A retrospective case‐note review was employed that explored the nature and severity of abuse in relation to the age, gender and level of disability. The prevalence of abuse or abusive behaviour, i.e. 14% of 300 admissions, did not change over time. In 13 out of the 43 cases, the issue of sexual abuse was identified after admission. Victimization alone occurred in 21 cases, perpetration alone in six cases, and both victimization and perpetration in 16 cases. Fifty per cent of the victims had been abused by a member of their close or extended family. Most cases (62%) were adolescents. There was only one instance of a victim being abused by a female. However, there were five girls who were perpetrators, all of whom had previously been victims. By contrast, 11 out of the 17 male perpetrators had been victims. Despite difficulties of disclosure, it was possible to establish that severely disabled patients had suffered sexual abuse. The present data support theories which (1) recognize gender differences in sexual abuse patterns and (2) have a developmental perspective, incorporating the influence of adolescence.
Association between perceived social support and strain, and positive and negative outcome for adults with mild intellectual disability
Authors: Lunsky Y. , Benson B. A.
Published: March 2003

Social strain has been identified as a trigger for both depression and physical health problems, but has not been well researched in people with intellectual disability (ID). The present study contrasted the effects of social support with social strain on depressive symptoms, somatic complaints and quality of life over time in adults with mild ID. The level of social support explained a significant proportion of variance in quality of life 6 months later, but not depressive symptoms or somatic complaints. In contrast, the level of social strain accounted for a significant proportion of variance in depressive symptoms and somatic complaints 6 months later, but not quality of life. The results suggest that interpersonal relationships can be both positively and negatively associated with physical and mental health for people with ID.
Mental disorder in adults with intellectual disability. 1: Prevalence of functional psychiatric illness among a community‐based population aged between 16 and 64 years
Authors: Deb, S. , Thomas M. , Bright C.
Published: March 2003

The reported prevalence of psychiatric illness among adults with intellectual disability (ID) varies widely between 10 and 39%; however, many methodological problems exist. The aims of the present study were to establish the prevalence of functional psychiatric illness among adults with ID who live in the community, in order to compare the overall rate and types of psychiatric illness between the population with ID and the general population without ID, and to establish the risk factors associated with psychiatric illness in adults with ID. The study was done in two stages. In the first part, a trained psychiatrist interviewed 101 randomly selected adults with ID and their carers using the Mini Psychiatric Assessment Schedule for adults with Developmental Disability (Mini PAS‐ADD) to screen for psychiatric caseness. Out of these 101 adults, 90 had sufficient communicative abilities that made the administration of Mini PAS‐ADD possible. A second trained psychiatrist interviewed 19 out of the 20 adults who were diagnosed as psychiatric cases according to the initial Mini PAS‐ADD interview. This psychiatrist interviewed patients and their carers in line with the full PAS‐ADD interview. The second psychiatrist was blind to the initial diagnoses made according to the Mini PAS‐ADD questionnaire. A final psychiatric diagnosis was made according to International Classification of Diseases – 10th Revision (ICD‐10) criteria. Some 14.4% (95% confidence interval = 7.4–21.4%) of the cohort had a psychiatric diagnosis according to ICD‐10 criteria: 4.4% had schizophrenia, 2.2% depressive disorder, 2.2% generalized anxiety disorder, 4.4% phobic disorder and 1% delusional disorder. The overall rate of functional psychiatric illness (point prevalence) was similar to that found in the general population (16%). However, the rates of schizophrenic illness and phobic disorder were significantly higher in the study cohort compared with those in the general population (0.4% and 1.1%, respectively). Increasing age and the presence of physical disability were significantly associated with the occurrence of psychiatric illness. Out of the 11 remaining adults with severe ID, two (18%) had a diagnosis of a psychiatric illness (one mania and one anxiety disorder) according to the Diagnostic Assessment for the Severely Handicapped (DASH) questionnaire.
A review of the assessment and treatment of anger and aggression in offenders with intellectual disability
Authors: Taylor J. L.
Published: March 2003

Rates of aggression amongst people with intellectual disability (ID) have been found to be high in studies conducted on several continents across a number of service settings. Aggression is the primary reason for people with ID to be admitted or re‐admitted to institutional settings, and it is also the main reason for individuals in this client group to be prescribed behaviour‐control drugs. Anger is a significant activator of aggression, but little is known about the emotional aspects of the lives of people with ID. There are many reasons for this, but a lack of reliable and validated assessment measures is chief among them. The present review found that very little work has been conducted to date concerning the development of robust tools for assessing anger and aggression in this population. A narrative review of interventions for reducing aggression and anger in people with ID showed that there is virtually no evidence to support the use of psychotropic medications. Research has shown that behavioural interventions can be effective; however, they are intrusive and have not been tested in naturalistic settings with higher‐functioning clients and low‐frequency aggression. More recently, cognitive–behavioural interventions have shown promise, but the mechanisms for effective change have yet to be delineated. Priority research questions relating to assessment, treatment and therapeutic skills in working with anger and aggression problems are offered by the present review.
Communication profiles of individuals with Down's syndrome, Angelman syndrome and pervasive developmental disorder
Authors: Duker P. C , Driel S. van , Bercken J. van de
Published: March 2003

The communication profiles of individuals with Down's syndrome (DS), Angelman syndrome (AS) and pervasive developmental disorder (PDD) were investigated and contrasted. Seventy‐seven individuals participated in the study. A within‐group analysis revealed that those with DS performed better on tacting or labelling and echoing than on manding or requesting. No other effects were found, apart from an absence of echoing in those with AS, a result that is hardly surprising. A between‐groups analysis revealed no differences between the aetiological groups in terms of their use of mands or requests and tacts. Individuals with DS and PDD did not differ in their scores on echoic functioning. The implications of these findings for the study of behavioural phenotypes and for communication intervention are discussed.
Environmental, medical, behavioural and disability factors associated with Helicobacter pylori infection in adults with intellectual disability
Authors: Wallace R. A , Webb P. M , Schluter P. J
Published: March 2003

In institutionalized adults with intellectual disability (ID), Helicobacter
pylori infection occurs at approximately twice the rate it appears in the general
population, and it may be responsible for the twofold higher rates of peptic ulcer
disease and gastric cancer in this population. Medical, behavioural and additional
environmental factors, as well as level of ID, may be related to the risk of infection
with H. pylori. One hundred and sixty‐eight adults with ID who were currently,
had previously been or had never been institutionalized underwent a biopsychosocial
evaluation. This included assessment of: level of ID using the Adaptive Behaviour
Scale (ABS) Part I; levels of maladaptive behaviour using the ABS Part II; demographic,
medical and environmental factors; as well as H. pylori tests using serology
and faecal antigen. The overall rates of past or current infection with H. pylori
in institutionalized and previously institutionalized participants were about twice
that of the overall group of never‐institutionalized participants, i.e. 87%
and 79% compared to 44%, respectively (P < 0.001). The
rates of H. pylori infection appeared to increase with age in the never‐institutionalized
group, but were consistently high across all ages in the other groups. The rate of
infection was higher in those institutionalized for more than 5 years (95%
versus 76%, P = 0.02), in those with flatmates with excessive oral
secretions (65% versus 21%, P < 0.001) or faecal incontinence
(67% versus 27%, P < 0.001), and in those with more chronic
illness and medications. All mean domain scores of the ABS Part I (Intellectual Disability)
were significantly lower (indicating more severe ID) in the group currently infected
with H. pylori compared to their non‐infected counterparts. The majority of
mean domain scores of the ABS Part II (Behaviour) were also worse, with half of these
score differences reaching statistical significance in the currently infected group.
The presence of alarm symptoms (e.g. vomiting, weight loss, haematemesis and melena),
iron deficiency and body mass index were not significantly different in currently
infected subjects. Adults with ID appear to be particularly at risk of infection
with H. pylori. Environmental associations with infection include past or current institutionalization, a longer period of institutionalization, living with flatmates with excessive oral secretions and faecal incontinence. Medical associations include chronic disease and more medications, but not alarm symptoms or body mass index. Demographic associations may include increasing age in never‐institutionalized adults, but no age effect in currently or previously institutionalized individuals. Psychosocial associations include more severe ID and maladaptive behaviour with current infection.
Healthcare costs of intellectual disability in the Netherlands: a cost‐of‐illness perspective
Authors: Polder J. J. , Meerding W. J. , Bonneux L. , Maas P. J. van der
Published: March 2003

Background Healthcare costs are continuously increasing, and impose a strong responsibility on governments for an adequate allocation of resources among healthcare provisions and patients.

Objectives The aims of the present study were to describe the healthcare costs of intellectual disability (ID) and other mental disorders in the context of the total costs of all other diseases, and to determinate the future need of healthcare resources, especially for ID and mental disorders.

Methods The present authors performed a top‐down cost‐of‐illness study comprising all healthcare costs of the Netherlands in 1994. Data on healthcare use were obtained for all 22 healthcare sectors, and used to ascribe costs to disease groups, age and sex.

Results Costs of mental disorders are by far the largest in the Dutch healthcare system. Some 25.8% of total disease‐specific costs could be ascribed to mental disorders: psychiatric conditions, 10.6%; ID, 9.0%; and dementia, 6.2%. There are large differences between age and sex groups. The costs of ID and schizophrenia are higher among men, and the costs of dementia and depression are higher among women. The age pattern shows two peaks: the first occurs at 25–35 years of age (ID and psychiatric conditions); and the second at 75–85 years of age (dementia). Time trends between 1988 and 1994 show an average annual growth rate of 5.2% for total healthcare costs: psychiatric conditions, 4.8%; ID, 5.4%; and dementia, 9.4%. Demographic projections suggest a less‐than‐average cost increase for ID and psychiatric disorders (with annual growth rates of 0.2% and 0.4%, respectively) compared to the costs of dementia and total healthcare (with annual growth rates of 1.6% and 0.9%, respectively).

Conclusions Intellectual disability and mental disorders represent a large part of healthcare use in the Netherlands. The costs will inevitably increase because of the ageing of the population and increasing life expectancy among people with disabilities. Non‐specific cost containment measures may endanger the quality of care for vulnerable people at younger and older ages.
The relationship between levels of mood, interest and pleasure and ‘challenging behaviour’ in adults with severe and profound intellectual disability
Authors: Ross E. , Oliver C.
Published: March 2003

Background Research on affective disorders in adults with intellectual disability (ID) suggests that depression may not present a ‘classic picture’ in individuals with severe and profound ID, but may include challenging behaviours, which are referred to as ‘atypical symptoms’, such as self‐injury, aggression and irritability. The aim of the present study was to explore whether there is an association between constructs relating closely to the core symptoms of depression and challenging behaviours in adults with severe and profound ID.

Method Mood and levels of interest and pleasure were measured in 53 adults with severe or pro‐found ID using the Mood, Interest and Pleasure Questionnaire (MIPQ).

Results Two groups of adults were identified based on MIPQ scores: (1) a ‘low mood’

group (lowest score = 12); and (2) a comparison group (highest scoring = 12). The

groups were clearly differentiated on the MIPQ (P < 0.0001), but were comparable on age, gender and medication use. The Challenging Behaviour Interview showed no difference between the two groups in self‐injury, aggression or disrupting the environment. A secondary analysis revealed that participants who showed challenging behaviour scored significantly lower on the MIPQ than those who did not show challenging behaviour.

Conclusions Possible reasons for these results and considerations for future studies are discussed.
Social timing, clock genes and autism: a new hypothesis
Authors: Wimpory D. , Nicholas B. , Nash S.
Published: March 2003

Background Timing and social timing deficits are fundamental in autism and may play a developmental role in its manifestation. Sleep problems are associated with this disorder, as is a reduction or loss of Purkinje cells associated with regions of the brain which co‐ordinate fine motor movements. Genetic studies suggest that a number of genes of limited effect lead to autism and that the genes are epistatic.

Conclusions We suggest that anomalies in clock genes operating as timing genes in high frequency oscillator systems may underlie the timing deficits of autism. We outline how anomalies in methylation‐related genes may also be implicated.
Responses of staff towards people with mild to moderate intellectual disability who behave aggressively: a cognitive emotional analysis
Authors: Wanless L. K. , Jahoda A.
Published: March 2003

BackgroundTraditionally, research into interactions between staff and adults with an intellectual disability who engage in challenging behaviour has been carried out within a behavioural paradigm. Recent work has begun to examine cognitive factors. The majority of this research has drawn upon Weiner's attributional model of helping behaviour. Moreover, staff responses have usually been investigated in relation to client vignettes of challenging behaviour.

MethodThe present study involved 38 staff who worked with frequently aggressive clients and its aims were twofold: (1) to compare different methods of examining the cognitive and emotional responses of staff to aggression; namely, descriptive vignettes and real incidents of aggression which staff could recall; and (2) to attempt to replicate previous findings supportive of Weiner's model with a new carer group (day centre staff).

ResultsWhen staff responses were compared across the hypothetical and real scenarios, using a series of one‐way analyses of variance, it was revealed that staff experienced more negative emotions in response to the real incidents of aggression. The present study produced little evidence in support of Weiner's model. However, it was found that staff perceptions of the aggressive clients were linked to their cognitive and emotional responses to the aggression.

ConclusionThe theoretical and clinical implications of these findings are considered, and the possible role played by interpersonal attributions in mediating staff responses to aggression is discussed.
Prevalence, aetiology and comorbidity of severe and profound intellectual disability in Finland
Authors: Arvio M. , Sillanpää M.
Published: March 2003

Background  The aim of the present study was to describe the aetiology, associated impairments and prevalence of severe and profound intellectual disability (SPID) in Finland.

Methods  The number of people with SPID in the catchment area of the Pääjärvi Centre for the Mentally Retarded, Lammi, Finland, (total population = 341 227) was calculated from the client register of this centre. Aetiological factors and background diagnoses for all subjects with SPID were analysed retrospectively.

Results  The number of people with SPID was 461, giving a prevalence of 0.13%. The aetiology of their SPID was genetic or congenital in 235 (50.9%) individuals, acquired in 89 (19.3%), genetic and/or acquired in 84 (18.3%), and unknown in 53 (11.5%) subjects. Out of the 53 individuals with an SPID of unknown origin, 48 (90.6%) had an associated impairment; the remaining five were the only members of the study group showing normal growth, and having neither dysmorphic features, physical abnormalities nor family members with ID. Out of the 461 subjects, 422 (91.5%) had between one and six associated impairments (total = 954), and the remaining 39 (8.5%) had SPID as their only impairment. Uncomplicated SPID was mainly of genetic or congenital origin, whereas all subjects with acquired encephalopathy had multiple disabilities. Speech defects, epilepsy and cerebral palsy were the most common associated impairments.

Conclusions  Severe and profound ID almost always occurs concomitantly with other severe neurological or psychiatric impairments. The proportion of people with SPID described in the present study is similar to that found in Finland in 1966. The aetiology of SPID in the vast majority of cases is biopathological.
Effect of 20 days' bed rest on the reverse cholesterol transport system in healthy young subjects
Authors: Yanagibori Ryoko , Kondo Kazuo , Suzuki Yoji , Kawakubo Kiyoshi , Iwamoto Tamami , Itakura Hiroshige
Published: March 2003

Ryoko Yanagibori, Kazuo Kondo, Yoji Suzuki, Kiyoshi Kawakubo, Tamami Iwamoto, Hiroshige Itakura, Atsuaki Gunji (Aichi Prefectural College of Nursing and Health, Aichi: National Institute of Health and Nutrition, Tokyo; and University of Tokyo, Japan). Effects of 20 days' bed rest on the reverse cholesterol transport system in healthy young subjects J Intern Med 1998; 243: 307–12.

Objectives

To study the effects of 20 days of bed rest on HDL cholesterol, lipoprotein lipase, hepatic triglyceride lipase, cholesterol ester transfer protein and lecithin–cholesterol acyltransferase.

Design

A 20‐day intervention study.

Setting

Makita general hospital.

Subjects

Five male and five female healthy participants, mean age 20.4 years, range 19–24 years.

Interventions

Twenty days of bed rest.

Main outcome measures

Lipid, lipoprotein, lipoprotein lipase, hepatic triglyceride lipase, cholesterol ester transfer protein and lecithin–cholesterol acyltransferase.

Results

Fasting HDL, HDL2 and HDL3 cholesterol levels decreased from 1.748 to 1.404 mmol L−1 (P < 0.01), from 0.807 to 0.628 mmol L−1 (P < 0.01) and from 0.939 to 0.784 mmol L−1 (P < 0.05), respectively, while VLDL triglyceride levels increased from 0.365 to 0.754 mmol L−1 (P < 0.05). Plasma post‐heparin lipoprotein lipase activity decreased from 0.494 to 0.418 μmol mL−1 h−1 (P < 0.01), but plasma post‐heparin hepatic triglyceride lipase activity and cholesterol ester transfer protein activity did not change during bed rest. Lecithin–cholesterol acyltransferase activity increased from 72.5 to 84.8 nmol mL−1 h−1 (P < 0.001).

Conclusions

Twenty days of bed rest induced a decline in HDL cholesterol levels and an increase in VLDL triglyceride levels. When considering lipoprotein lipase, hepatic triglyceride lipase, cholesterol ester transfer protein and lecithin–cholesterol acyltransferase as factors in the decreased HDL cholesterol, the contribution of lipoprotein lipase is suggested.
Acute coronary disease in essential thrombocythemia and polycythemia vera
Authors: Rossi C. , RandI M. L. , Zerbinati P. , Rinaldi V. , Girolami A.
Published: March 2003

Rossi C, Randi ML, Zerbinati P, Rinaldi V, Girolami A (University of Padua Medical School, Padua, Italy). Acute coronary disease in essential thrombocythemia and polycythemia vera. J Intern Med 1998; 244:49–53.

Objectives

The aim of this study is to report our experience on myocardial infarction (MI) in patients with essential thrombocythemia (ET) and polycythemia vera (PV).

Design

Patients with PV and ET consecutively diagnosed and followed in authors' Department between 1 July 1986 and 30 June 1996.

Subjects

Over the past 10 years we have followed 170 patients with ET and 149 with PV, diagnosed according to the Polycythemia Vera Study Group (PVSG) criteria. The patients were divided into 3 groups on the basis of the age at diagnosis (group A < 40, B 41–65, C > 65 years).

Interventions

In all patients with PV phlebotomies and/or myelosuppressive therapy were used to keep haematocrit level lower than 45%. Hydroxyurea was given to patients with ET with a positive history for major vascular complications or with an extreme thrombocytosis. Aspirin therapy (ASA) (100 mg per day) was administered in patients with microvascular disturbances or previous thrombosis (in patients with PV also in the presence of atherosclerotic risk factors).

Main outcome measures

Frequency of MI in patients with ET and PV with and without ASA therapy.

Results

9.4% of patients with ET and 11.4% of those with PV had MI. 17.6% of patients with PV were younger than 40 years at the moment of MI in contrast to 0% of those with ET. 75% of patients with ET and 70.6% of those with PV with MI had atherosclerotic risk factors such as smoking, hypertension, diabetes, dyslipidaemia. All patients with MI received ASA 100 mg daily after thrombosis and four of the ET group developed a transient ischaemic attack (TIA) afterwards. Four subjects with PV during the follow‐up had TIAs and two peripheral arteriopathy in spite of ASA treatment.

Conclusions

MI is less common in patients with ET younger than 40 years than in older patients. Association of MI and cardiovascular risk factors is frequent in patients with ET and PV. A low dose of ASA could be able to reduce the number of coronary thrombosis without increasing bleeding complications in patients with elevated platelet count and common atherosclerotic risk factors. However, a larger population must be evaluated to confirm our hypothesis.
Functions of the von Hippel–Lindau tumour suppressor protein
Authors: Kaelin  , Iliopoulos  , Lonergan  , Ohh
Published: March 2003

Kaelin WG, Iliopoulos O, Lonergan KM, Ohh M (Dana‐Farber Cancer Institute, Brigham and Women's Hospital, Boston, MA, USA). Functions of the von Hippel–Lindau tumour suppressor protein (Minisymposium: MEN & VHL). J Intern Med 1998; 243: 535–9.

Von Hippel–Lindau disease (VHL) is caused by germline mutations in the VHL tumour suppressor gene. Tumour development in this setting is due to loss or inactivation of the remaining wild‐type VHL allele. The VHL gene product (pVHL) resides primarily in the cytoplasm. A frequently mutated region of pVHL can bind to complexes containing elongin B, elongin C and Cul2. Loss of pVHL leads to an inappropriate accumulation of hypoxia‐inducible mRNAs, such as the mRNA encoding vascular endothelial growth factor (VEGF), under normoxic conditions. This finding is most likely to account for the hypervascular nature of VHL‐associated neoplasms. Current studies are focussed on understanding if and how binding to elongins and Cul2 is linked to the ability of pVHL to regulate hypoxia‐inducible mRNAs. In this regard, it is perhaps noteworthy that elongin C and Cul2 are homologous to yeast proteins Skp1 and Cdc53. These latter proteins participate in the formation of complexes that target certain proteins for ubiquitination.
Carney complex and the familial lentiginosis syndromes: link to inherited neoplasias and developmental disorders, and genetic loci
Authors: Chrousos  , Stratakis
Published: March 2003

Chrousos GP, Stratakis CA (National Institutes of Health, Bethesda, MD, USA). Carney complex and the familial lentiginosis syndromes: link to inherited neoplasias and developmental disorders, and genetic loci (Minisymposium: MEN & VHL). J Intern Med 1998; 243: 573–9.

Lentigines, synonymous but not identical to freckles or ephelides, are common skin lesions. In a small number of patients, however, these lesions constitute part of genetic syndromes that are associated with inherited forms of neoplasias or other pathologic processes of the cardiovascular, endocrine, and gastrointestinal systems. The familial lentiginoses, as these syndromes are collectively known, include Carney complex, the LEOPARD and Peutz–Jeghers syndromes, and the newly described ‘syndrome of arterial dissections with lentiginosis’; isolated, familial lentiginosis has also been described. In the majority of the reported kindreds with these syndromes, the lesions were inherited in an autosomal dominant manner. The specific genes that are responsible for these disorders have remained elusive, but the genetic loci of Carney complex and Peutz–Jeghers syndromes were recently identified on chromosomes 2p 16 and distal 19p, respectively. Cytogenetic studies of tumours from patients with Carney complex suggest that the gene responsible for most patients with this syndrome may not have a tumour suppression function. We suggest that the genes responsible for the lentiginosis syndromes are important regulators of melanocyte function, they participate in the pleiotropy of human pigmentation, and are involved in the function, growth and proliferation of neural crest and mesenchymal cells.
A 50 year perspective endocrine neoplasia: clinic to genes and back
Authors: Jackson
Published: March 2003

Jackson CE (Henry Ford Hospital, Detroit, MI, USA). A 50 year perspective on endocrine neoplasia: clinic to genes and back (Minisymposium: MEN & VHL). J Intern Med 1998; 243: 419–23.

Having completed medical school and internship in 1947 when effective therapeutic interventions were quite limited (mostly to surgery), I became impressed with the importance of diagnosing the then known treatable diseases. As a young army medical officer, I was exposed to those who emphasized pheochromocytomas and parathyroid adenomas as treatable conditions. After completing internal medicine training, I continued my search for these conditions but it was not until 1958 that I first encountered my first patient with hyperparathyroidism (HPT) who happened to be in a family with many others affected. After leaving the small clinic in Indiana where I practiced for 19 years, in 1970 I came to Henry Ford Hospital where I continued my search for hereditary HPT and encountered families of MEN‐2 by applying calcitonin studies to the families of our cases of medullary thyroid cancer. Early genetic linkage studies suggested that progress in the field would benefit from a workshop on MEN‐2 and Nancy Simpson agreed to host the first workshop in Kingston in 1984. This and the five subsequent workshops brought together those interested in the field and resulted in the remarkable advances being made in the diagnosis and treatment of endocrine neoplasias and in the understanding of these conditions and of neoplasia in general.
Identification and characterization of the multiple endocrine neoplasia type 1 (MEN1) gene
Authors: Guru  , Manickam  , Crabtree  , Olufemi  , Agarwal  , Debelenko  , Zhuang  , Lubensky  , Kester  , Kim  , Heppner  , Weisemann  , Boguski  , Wang  , Roe  , Burns  , Liotta  , Spiegel  , Emmert‐Buck  , Marx  , Collins  , Chandrasekharappa
Published: March 2003

Guru SC, Manickam, Crabtree JS, Olufemi S‐E, Agarwal SK, Debelenko LV, Zhuang Z, Lubensky IA, Kester MB, Kim YS, Heppner C, Weisemann JM, Boguski MS, Wang Y, Roe BA, Burns AL, Liotta LA, Speigel AM, Emmert‐Buck MR, Marx SJ, Collins FS, Chandrasekharappa SC (National Institutes of Health, Bethesda; University of Oklahoma, Norman; USA). Identification and characterization of the multiple endocrine neoplasia type 1 (MEN1) gene (Minisymposium: MEN & VHL). J Intern Med 1998; 243: 433–9.

For nearly a decade since the mapping of the multiple endocrine neoplasia type 1 (MEN1) locus to 11q13 and the suggestion that it is a tumour suppressor gene, efforts have been made to identify the gene responsible for this familial cancer syndrome. Recently, we have identified the MEN1 gene by the positional cloning approach. This effort involved construction of a 2.8‐Mb physical map (D11S480–D11S913) based primarily on a bacterial clone contig. Using these resources, 20 new polymorphic markers were isolated which helped to reduce the interval for candidate genes by haplotype analysis in families and by loss of heterozygosity (LOH) studies in approximately 200 tumours, utilizing laser‐assisted microdissection to obtain tumour cells with minimal or no admixture by normal cells. The interval was narrowed by LOH to only 300 kb, and nearly 20 new transcripts that map to this region of 11q13 were isolated and characterized. One of the transcripts was found by dideoxyfingerprinting and cycle sequencing to harbour deleterious germline mutations in affected individuals from MEN‐1 kindreds and therefore identified as the MEN1 gene. The type of germline mutations and the identification of mutations in sporadic tumours support the Knudson's two‐hit model of tumorigenesis for MEN‐1. Efforts are being made to identify the function of the MEN1 gene‐encoded protein, menin, and to study its role in tumorigenesis.
Effects of atorvastatin on serum lipids of patients with familial hypercholesterolaemia
Authors: Hoogerbrugge
Published: March 2003

Hoogerbrugge N (University Hospital Dijkzigt, Rotterdam, the Netherlands). Effects of atorvastatin on serum lipids of patients with familial hypercholesterolaemia. J Intern Med 1998; 244: 143–7.

Objectives

The effects of atorvastatin, a new synthetic HMG‐CoA reductase inhibitor, were investigated in patients with familial hypercholesterolaemia (FH), with high LDLc levels whilst on standard treatment.

Design

Open treatment with 40 mg atorvastatin daily for 6 weeks, followed by another 6 weeks with 80 mg atorvastatin.

Setting

Outpatient lipid clinic of a tertiary referral centre.

Subjects

FH was diagnosed when the untreated LDLc concentration was higher than 6 mmol L−1, tendon xanthomas were present at the participant or a first degree relative, and the family history for hypercholesterolaemia was positive. The FH patients were selected for an LDLc above 5.0 mmol L−1 whilst on standard therapy for at least 3 months. Standard therapy consisted of a diet and 40 mg simvastatin, either alone (n= 17), or in combination with 8–12 g colestyramin (n= 12), or 1800 mg nicotinic acid (n= 12).

Main outcome measure

Effects on LDLc concentration.

Results

LDLc concentration significantly decreased during treatment with 80 mg atorvastatin as compared to LDLc levels on 40 mg simvastatin alone or in combination with 8–12 g colestyramin, by 24 ± 14% (P < 0.01) and 19 ± 22% (P < 0.01), respectively. LDLc concentration was comparable during treatment with 80 mg atorvastatin or 40 mg simvastatin in combination with 1800 mg nicotinic acid. Atorvastatin was tolerated well, no side‐effects were observed.

Conclusions

Atorvastatin is a valuable addition to the treatment possibilities of patients with serious hypercholesterolaemia, like FH.
The benefit of appropriate empirical antibiotic treatment in patients with bloodstream infection
Authors: Leibovici  , Shraga  , Drucker  , Konigsberger  , Samra  , Pitlik  , Pitlik
Published: March 2003

Abstract. Leibovici L, Shraga I, Drucker M, Konigsberger H, Samra Z, Pitlik SD (Rabin Medical Center, Petah‐Tiqva, and Tel‐Aviv University, Tel‐Aviv, Israel). The benefit of appropriate empirical antibiotic treatment in patients with bloodstream infection. J Intern Med 1998; 244: 379–86.

Objectives

To test whether empirical antibiotic treatment that matches the in vitro susceptibility of the pathogen (appropriate treatment) improves survival in patients with bloodstream infections; and to measure the improvement.

Design

Observational, prospective cohort study.

Setting

University hospital in Israel.

Subjects

All patients with bloodstream infections detected during 1988–94.

Interventions

None.

Main outcome measures

In‐hospital fatality rate and length of hospitalization.

Results

Out of 2158 patients given appropriate empirical antibiotic treatment, 436 (20%) died, compared with 432 of 1255 patients (34%) given inappropriate treatment (P = 0.0001). The median durations of hospital stay for patients who survived were 9 days for patients given appropriate treatment and 11 days for patients given inappropriate treatment. For patients who died, the median durations were 5 and 4 days, respectively (P < 0.05), for both comparisons.

In a stratified analysis, fatality was higher in patients given inappropriate treatment than in those given appropriate treatment in all strata but two: patients with infections caused by streptococci other than Streptococcus gr. A and Streptoccocus pneumoniae (odds ratio (OR) of 1.0, 95% confidence interval (95% CI) 0.4–2.5); and hypothermic patients (OR = 0.9, 95% CI = 0.3–2.4). Even in patients with septic shock, inappropriate empirical treatment was associated with higher fatality rate (OR = 1.6, 95% CI = 1.0–2.7). The highest benefit associated with appropriate treatment was observed in paediatric patients (OR = 5.1, 95% CI = 2.4– 10.7); intra‐abdominal infections (OR = 3.8, 95% CI = 2.0–7.1); infections of the skin and soft tissues (OR = 3.1, 95% CI = 1.8–5.6); and infections caused by Klebsiella pneumoniae (OR = 3.0, 95%  CI = 1.7–5.1) and S. pneumoniae (OR = 2.6, 95%  C = 1.1–5.9).

On a multivariable logistic regression analysis, the contribution of inappropriate empirical treatment to fatality was independent of other risk factors (multivariable adjusted OR = 1.6, 95% CI = 1.3–1.9).

Conclusion

Appropriate empirical antibiotic treatment was associated with a significant reduction in fatality in patients with bloodstream infection.
Atorvastatin compared with simvastatin in patients with severe LDL hypercholesterolaemia treated by regular LDL apheresis
Authors: Geiss H. C. , Parhofer K. G. , Schwandt P.
Published: March 2003

Objectives. Atorvastatin is a new potent HMG‐CoA reductase inhibitor. We evaluated whether patients with coronary heart disease and severe hypercholesterolaemia showing insufficient LDL (low‐density lipoprotein) cholesterol reduction despite combined therapy with simvastatin and regular LDL apheresis will benefit from atorvastatin therapy.

Setting. Tertiary care centre, university hospital.

Methods. In 21 patients treated by LDL apheresis, concomitant simvastatin therapy (40 mg day−1) was replaced by atorvastatin (40 mg day−1) and increased to 60 and 80 mg day−1 (each for 3 months) if no side‐effects were reported and NCEP treatment goals were not reached.

Results. In 20 of 21 patients (95%), atorvastatin resulted in significant reduction of LDL cholesterol compared with simvastatin (by 10%, additional 8% and additional 1%, with 40, 60 and 80 mg day−1, respectively). In four patients, NCEP treatment goals were reached (in three by atorvastatin alone, and in one by atorvastatin and apheresis). Patients with little reduction in LDL cholesterol to 40 mg day−1 atorvastatin benefited most by increasing the dose to 60 mg day−1 (additional 13% reduction), whilst those responding to atorvastatin 40 mg day−1 benefited less (additional 1.9% reduction). During atorvastatin therapy, significantly less plasma had to be treated during apheresis resulting in shorter apheresis time. Eight patients (38%) reported side‐effects, resulting in discontinuation of atorvastatin in three (14%) and dose reduction in five patients (24%), whilst no elevation of biochemical markers was observed.

Conclusion. Concomitant atorvastatin therapy is superior to simvastatin therapy in patients with severe hypercholesterolaemia treated with regular LDL apheresis, but is associated with a high rate of subjective side‐effects.