bluuebunny/crossref_metadata_2025_split huggingface dataset:
A review of the assessment and treatment of anger and aggression in offenders with intellectual disability
Authors: Taylor J. L. 
Published: March 2003

AbstractRates 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

AbstractThe 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

AbstractIn 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

AbstractBackground 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

AbstractBackground 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

AbstractBackground 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

AbstractBackgroundTraditionally, 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

AbstractBackground  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.ObjectivesTo 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.DesignA 20‐day intervention study.SettingMakita general hospital.SubjectsFive male and five female healthy participants, mean age 20.4 years, range 19–24 years.InterventionsTwenty days of bed rest.Main outcome measuresLipid, lipoprotein, lipoprotein lipase, hepatic triglyceride lipase, cholesterol ester transfer protein and lecithin–cholesterol acyltransferase.ResultsFasting 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).ConclusionsTwenty 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

AbstractRossi 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.ObjectivesThe aim of this study is to report our experience on myocardial infarction (MI) in patients with essential thrombocythemia (ET) and polycythemia vera (PV).DesignPatients with PV and ET consecutively diagnosed and followed in authors' Department between 1 July 1986 and 30 June 1996.SubjectsOver 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).InterventionsIn 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 measuresFrequency of MI in patients with ET and PV with and without ASA therapy.Results9.4% 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.ConclusionsMI 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.