bluuebunny/crossref_metadata_2025_split huggingface dataset:
A microswitch for vocalization responses to foster environmental control in children with multiple disabilities
Authors: Lancioni G. E. , O'Reilly M. F. , Oliva D. , Coppa M. M. 
Published: March 2003

AbstractThe present study assessed whether two children with multiple disabilities could learn to successfully operate a special microswitch through vocaliza‐ tion responses so as to obtain environmental stimulation. The study was carried out using a non‐concurrent multiple baseline design across individuals. The treatment phase of the second child started after he had received twice the number of baseline sessions used for the first child. The results were positive, with both children increasing the frequencies of their vocalization responses during the treatment. The importance of using such responses (which do not require excessive efforts and are highly valued) for operating a microswitch with children with multiple disabilities is discussed.
Assessing the need for reactive behaviour management strategies in children with intellectual disability and severe challenging behaviour
Authors: Adams D. , Allen D. 
Published: March 2003

AbstractThe need for reactive behavioural management strategies for aggressive behaviours in adults with intellectual disability has been clearly established, but equivalent information concerning children with challenging behaviour is lacking by comparison. The present study was conducted in order to assess this need. A retrospective study was conducted to ascertain the nature of aggressive behaviours amongst a cohort children referred to a specialist support service. Aggressive behaviours were found to occur at high rates within the study group. Almost 60% of the children displayed aggression that occurred at least daily, and the behaviours resulted in serious consequences for carers in almost one‐third of the group. Physical interventions were already in use in 56% of cases, but were largely improvised by carers. The need to include reactive behavioural training as part of an overall intervention package for carers of children with challenging behaviour appeared to be supported by the present results. The implications for training design are discussed.
Visual impairment in adult people with intellectual disability: Literature review
Authors: Warburg M. 
Published: March 2003

AbstractThe present paper reviews studies on the prevalence of visual impairment (VI) in adults with intellectual disability (ID). Every publication describes an alarming prevalence of blindness and VI. Cataract and keratoconus were common. Many cases of poor distance vision were treatable by ordinary spherical or astigmatic glasses, but few people had had such prescriptions. Elderly residents in community and institutional care often did not receive glasses for near vision. Professional assessments disclosed higher prevalences of VI than questionnaires mailed to the care personnel. The prevalence of VI increased dramatically with the severity of ID and with age. Regular professional assessment of eye disorders, visual acuity and refraction are warranted in residents in both hospital and community care.
Behavioural aspects of Pollitt syndrome: a 32‐year follow‐up of a case described by R. J. Pollitt and colleagues in 1968
Authors: Murrin K. L. , Clarke D. J. 
Published: March 2003

AbstractIn 1968, R. J. Pollitt and colleagues described a syndrome characterized by abnormally brittle, sulphur‐deficient hair (trichothiodystrophy), intellectual disability (ID) and growth retardation. One of the two siblings originally described by the above authors has recently been re‐assessed by the present authors following a referral for advice about ritualistic behaviours. Her current clinical features are described, and the literature concerning trichothiodystrophy and ID is summarized.
Paid employment of mothers and fathers of an adult child with multiple disabilities
Authors: Einam M. , Cuskelly M. 
Published: March 2003

AbstractBackground Paid employment is increasingly undertaken by mothers as their children age, with the majority of women being in employment by the time their offspring are adult. Opportunities to engage in employment appear to be reduced for mothers of children with disabilities; however, little is known about the employment of mothers or fathers of adults with disabilities.Method Data were collected regarding the employment decisions of parents of a young adult with multiple disabilities and contrasted with those of parents whose children were all developing normally. Twenty‐five mothers and 12 fathers of a young adult with multiple disabilities were interviewed, as were 25 comparison mothers and 19 comparison fathers. Data collected included hours of work, reasons for employment status, attitudes towards work and child care, and psychological well‐being.Results Clear differences were found between the two groups. Mothers and fathers of a child with multiple disabilities showed different engagement patterns with the paid workforce from comparison parents. Hours of work for fathers of a young adult with multiple disabilities showed a bi‐modal distribution, with some fathers working fewer hours than usual and others working very long hours. For mothers in both groups, the number of hours in paid employment was negatively associated with reports of psychological problems.Conclusions Increased attention needs to be given to the employment opportunities of parents of children with disabilities since employment appears to play a protective role for mothers, in particular. Services provided to adults with disabilities will need to change if parents are to have the same life chances as parents without adult offspring with a disability.
Difficulties in conducting a randomized controlled trial of health service interventions in intellectual disability: implications for evidence‐based practice
Authors: Oliver P. C. , Piachaud J. , Done J. , Regan A. , Cooray S. , Tyrer P. 
Published: March 2003

AbstractBackground In an era of evidence‐based medicine, practice is constantly monitored for quality in accordance with the needs of clinical governance (Oyebode et al. 1999). This is likely to lead to a dramatic change in the treatment of those with intellectual disability (ID), in which evidence for effective intervention is limited for much that happens in ordinary practice. As Fraser (2000, p. 10) has commented, the word that best explains ‘the transformation of learning disability practice in the past 30 years is “enlightenment”.’ This is not enough to satisfy the demands of evidence, and Fraser exhorted us to embrace more research‐based practice in a subject that has previously escaped randomized controlled trials (RCTs) of treatment because of ethical concerns over capacity and consent, which constitute a denial of opportunity which ‘is now at last regarded as disenfranchising’.Conclusions The present paper describes the difficulties encountered in setting up a RCT of a common intervention, i.e. assertive community treatment, and concludes that a fundamental change in attitudes to health service research in ID is needed if proper evaluation is to prosper.
Efficacy and safety of systemic recombinant interferon‐alpha in Behcet's disease
Authors: Georgiou  , Monastirli  , Pasmatzi  , Gartaganis  , Goerz  , Tsambaos  
Published: March 2003

Georgiou S, Monastirli A, Pasmatzi E, Gartaganis S, Goerz G, Tsambaos D (Departments of Dermatology and Ophthalmology, University of Patras, Greece and the Department of Dermatology, Heinrich‐Heine University of Düsseldorf, Germany). Efficacy and safety of systemic recombinant interferon‐alpha in Behcet's disease. J Intern Med 1998; 243: 367–72.ObjectivesTo evaluate the therapeutic efficacy and safety of systemic recombinant interferon α‐2a (IFN ‐α) in patients with Behcet's disease (BD) and to determine the incidence of episodes in complete responders during the one‐year pretreatment period and follow‐up.DesignAn open clinical study.SettingDepartments of Dermatology and Ophthalmology, University of Patras, Greece and Department of Dermatology, Heinrich‐Heine University of Düsseldorf, Germany.SubjectsTwelve patients (aged 23–52 years) with active BD who had previously been unsuccessfully treated with systemic steroids and/or immunosuppressives.InterventionsIFN‐α was administered subcutaneously at a dose of 6 × 106 IU per day 3 times per week for 2 months.Main outcome measuresChange of area or number of mucocutaneous lesions, grading score for thrombophlebitis and ocular inflammation, haematological and biochemical parameters and number of episodes during the pretreatment period and the follow‐up. Evaluation of IFN‐α side effects.ResultsNine patients (75.0%) revealed a complete remission, two (16.6%) a partial remission and one patient (8.3%) showed no response. During the follow‐up in five out of the nine complete responders (55.5%) no episodes of BD were seen, whereas, the other four patients (44.5%) had 1–2 episodes, as compared to 5–8 and 5–12 episodes, respectively, during the pretreatment period. An influenza‐like syndrome (fever, nausea and myalgias) appeared during the early phase of therapy in all (but one) patients. No patient had to discontinue IFN‐α because of intolerance.ConclusionsSubcutaneous human recombinant interferon α‐2a appears to be an effective and fairly well tolerated therapy for BD.
Serum 25‐hydroxyvitamin D levels and bone mineral density in 16–20 years‐old girls: lack of association
Authors: Kristinsson J. Ö. , Valdimarsson Ö. , Sigurdsson  , Franzson  , Olafsson  , Steingrimsdottir  
Published: March 2003

Kristinsson JÖ, Valdimarsson Ö, Sigurdsson G, Franzson L, Olafsson I & Steingrimsdottir L. (Reykjavik Hospital, University of Iceland, Reykjavik, Iceland). Serum 25‐Hydroxyvitamin D Levels and bone mineral density in 16–20 years old girls: lack of association. J of Intern Med 1998; 243: 381–88In conclusion, our results indicate that the vitamin D requirements for normal bone mineral accrual in adolescent and young adult girls may be lower than presumed and suggest that calcium and phosphate concentrations in plasma may be regulated in this age group to some extent by other factors which needs to be further clarified. However, the important question of the effect of repeated, cyclical vitamin‐D nadir on bone mass in young people needs to be explored in a longitudinal survey.
Prophylactic splenectomy and cholecystectomy in mild hereditary spherocytosis: analyzing the decision in different clinical scenarios
Authors: Marchetti  , Quaglini  , Barosi  
Published: March 2003

Marchetti M, Quaglini S, Barosi G (Laboratory of Medical Informatics, IRCCS Policlinico S. Matteo and the Department of Computer Science and Systems, University of Pavia, Pavia, Italy) Prophylactic splenectomy and cholecystectomy in mild hereditary spherocytosis: analyzing the decision in different clinical scenarios. J Intern Med 1998; 244: 217–26. Objectives.Patients with mild hereditary spherocytosis (HS), i.e. with haemolysis without anaemia, have an increased risk of gallstone formation, erythroid aplasia and haemolytic crisis. Since the effect of prophylactic splenectomy on life expectancy has not been established, we conducted a decision analysis comparing prophylactic splenectomy and cholecystectomy with no surgery.Design.The available data on surgery and disease outcomes were modelled to estimate the effects of the different interventions on the quality‐adjusted life expectancy. The early phase outcomes depicted surgery‐related mortality and incorporated compliance and the adverse effects of prophylaxis against post splenectomy infections. The late phase outcomes were framed by a Markov cohort analysis.Results.For patients without gallstones, surgery was of no benefit. For those with gallstones the preferred strategies were found to be splenectomy and cholecystectomy before the age of 39 when asymptomatic, and before 52 when accompanied by occasional biliary colic. Cholecystectomy alone proved to be the preferred strategy in older patients with occasional biliary colic. For patients of up to 52 years of age and candidates for cholecystectomy because of recurrent biliary colic, the best strategy was to combine this procedure with splenectomy. Sensitivity analysis showed that the results were sensitive to the incidence of post cholecystectomy syndrome. Most remarkably an extreme sensitivity to compliance with post splenectomy infection prophylaxis was demonstrated.Conclusions.Our model suggested that combined prophylactic splenectomy and cholecystectomy provide a substantial gain in quality‐adjusted life expectancy for young patients and adults with mild HS and gallstones.
Contributions of studies on uncoupling proteins to research on metabolic diseases
Authors: Ricquier D. , Fleury C. , Larose M. , Sanchis D. , Pecqueur C. , Raimbault S. , Gelly C. , Vacher D. , Cassard‐Doulcier A.‐M. , Lévi‐Meyrueis C. , Champigny O. , Miroux B. , Bouillaud F. 
Published: March 2003

Abstract. Ricquier D, Fleury C, Larose M, Sanchis D, Pecqueur C, Raimbault S, Gelly C, Vacher D, Cassard‐Doulcier A‐M, Lévi‐Meyrueis C, Champigny O, Miroux B, Bouillaud F (Centre National de la Recherche Scientifique, Centre de Recherche sur l’Endocrinologie Moléculaire et le Développement, Meudon, France). Contributions of studies on uncoupling proteins to research on metabolic diseases. (Minisymposium: Genes & Obesity). J Intern Med 1999; 245: 637–642.The coupling of O2 consumption to ADP phosphorylation in mitochondria is partial. This is particularly obvious in brown adipocyte mitochondria which use a regulated uncoupling mechanism generating heat production from substrate oxidation, and catalysing thermogenesis in rodents or infants in response to cold, and arousing hibernators. In the case of brown adipose tissue, the uncoupling mechanism is related to a specific protein in the inner mitochondrial membrane referred to as UCP1. Although the biological importance of UCP1 in human adults is not demonstrated, genetic analysis of various human cohorts suggested a participation of UCP1 to control of fat content and body weight. Very recently, the cloning of UCP2 and UCP3, two homologues of UCP1, has renewed the field of research on the importance of respiration control in metabolic processes and metabolic diseases. UCP2 is widely expressed in organs, whereas UCP3 is mainly present in muscles. These proteins may explain why the coupling of respiration to ADP phosphorylation is less than perfect. Their biological importance should be studied. They also represent new putative targets for drugs against metabolic diseases such as obesity.
Ethical aspects of clinical trials: the attitudes of the public and out‐patients
Authors: Madsen S. , Holm S. , Riis P. 
Published: March 2003

Abstract. Madsen S, Holm S, Riis P (Copenhagen University Hospital, Herlev, and University of Copenhagen, Blegdamsvej, Denmark). Ethical aspects of clinical trials: the attitudes of the public and out‐patients. J Intern Med 1999; 245: 571–579.Objectives.  To investigate attitudes to clinical research amongst potential research participants.Design.  Questionnaire‐survey.Setting.  Two medical out‐patient clinics and the background population.Subjects.  A total of 508 randomly selected citizens in Copenhagen County (64% responded) and 200 consecutive patients attending the out‐patient clinics (64% responded).Outcome measures. Attitudes toward different aspects of clinical research.Results.  Positive attitudes toward medical research were disclosed. The majority found scientific testing necessary, although only a minority considered participation a moral obligation. Both personal benefits and altruistic motives for participation were highly rated, whereas former positive experiences from trial participation had only minor impact on decisions. Several respondents stated former trial participation had changed their attitudes negatively. Lack of feedback of results was of major importance for this change. Attitudes are significantly influenced by the presence of independent research ethics committees, whereas trial technicalities such as drawing lots and blinding was found problematic by only a few respondents. Altruistic motives of physicians to conduct trials were highly rated by a majority of respondents, but the motive of promoting doctors’ careers was also judged important. Respondents rated nondiscomforting procedures as acceptable or having only a small impact or strain on their lives.Conclusion.  Attitudes toward medical research are positive amongst out‐patients and the general public. Altruistic and nonaltruistic motives both concerning trial participation and concerning the motives of physicians to conduct medical research were rated highly. Lack of feedback concerning results of trials to participants was important for a negative change in attitude toward participation.
Total homocysteine and cardiovascular disease
Authors: Nygård O. , Vollset S. E. , Refsum H. , Brattström L. , Ueland P. M. 
Published: March 2003

Abstract. Nygård O, Vollset SE, Refsum H, Brattström L, Ueland PM (University of Bergen, Norway; County Hospital, Kalmar, Sweden). Total homocysteine and cardiovascular disease (Review). J Intern Med 1999; 246: 425–454.Recent data have shown that an elevated plasma level of the amino acid homocysteine (Hcy) is a common, independent, easily modifiable and possibly causal risk factor for cardiovascular disease (CVD) which may be of equal importance to hypercholesterolemia, hypertension and smoking. This paper reviews the biochemical, clinical, epidemiological and experimental data underlying this conclusion and is critically questioning whether elevated tHcy is a causal factor.
Factors predicting lower extremity amputations in patients with type 1 or type 2 diabetes mellitus: a population‐based 7‐year follow‐up study
Authors: Hämäläinen H. , Rönnemaa T. , Halonen J.‐P. , Toikka T. 
Published: March 2003

Objectives. The aim of the study was to find factors predicting lower extremity amputation in patients with type 1 or type 2 diabetes mellitus through a 7‐year follow‐up period.Design. Follow‐up study.Subjects. Altogether 733 diabetic patients, aged 10–79 years, were drawn from the national drug reimbursement register.Methods. At baseline, the patients underwent a podiatric, circulatory and neurophysiological examination. Seven years later a follow‐up study was performed based on clinical and register data. Patient data for those who died during the follow‐up were collected from hospital records and death certificates. All amputations were recorded. The patients with amputation were compared with the other patients and also, in a case‐control manner, by taking three nonamputated patients matched by sex, type of diabetes, and age for each patient with amputation.Results. The number of amputations was 25 in the sample. Compared with all patients without amputation, patients with amputation differed in altogether 24 variables concerning diabetes and its complications. Compared with the matched nonamputated patients, the amputated patients had longer duration of diabetes, lower ankle/brachial pressure index (ABI), more often history of retinopathy, nephropathy, and hypertension, more often visual handicap, elevated serum creatinine level, abnormal neurophysiological indices and electrophysiological findings. In the logistic regression analysis, vibration perception threshold, low ABI, history of retinopathy, visual handicap, and male sex were independently associated with lower extremity amputation.Conclusions. Lower extremity amputations were strongly associated with retinopathy, nephropathy, and neuropathy. The presence of any of these complications should lead to intensified actions in order to prevent amputations. As far as arterial circulation is concerned, claudication or absent peripheral pulses were not good predictors of amputation, whereas low ABI, despite its known weaknesses, was a reliable indicator of future amputation.
Serum cholesterol is a risk factor for myocardial infarction in elderly men and women: the Rotterdam Study
Authors: Houterman S. , Verschuren W. M. M. , Hofman A. , Witteman J. C. M. 
Published: March 2003

Objective. To investigate the associations of serum total and HDL cholesterol with the risk of myocardial infarction in men and women of 55 years and over.Design. The Rotterdam Study is a population‐based prospective cohort study. In total 2453 men and 3553 women of 55 years and older were included in this study. The mean duration of follow‐up was 4 years.Main outcome measures. Relative risks were estimated with Cox’s proportional‐hazard analysis. Cholesterol was analysed as a continuous variable and in sex‐specific quartiles.Results. In subjects aged 55 years and older the relative risk of myocardial infarction was 1.9 in men (95% confidence interval 1.1–3.3) and 3.2 in women (1.5–6.4) in the highest compared to the lowest serum total cholesterol quartile (Q4 vs. Q1). In men and women of 70 years and older, total cholesterol remained an important risk factor for myocardial infarction (Q4 vs. Q1 relative risk 3.2; 1.3–7.7 and 2.9; 1.3–6.6, respectively). For HDL cholesterol, the relative risk in the highest compared to the lowest quartile (Q4 vs. Q1) was 0.5 in men (0.3–0.9) and 0.4 in women (0.2–0.9). HDL cholesterol was a weaker predictor in men after the age of 70 (Q4 vs. Q1 0.8; 0.3–2.1). In women of 70 years and older the relative risk was also not significant (Q4 vs. Q1 0.6; 0.3–1.3), although the trend over the quartiles was still significant.Conclusion. Serum total cholesterol remains an important risk factor for myocardial infarction in men and women aged 70 years and older, whilst HDL cholesterol at older age remains important in women only.
A decision analysis of thrombolytic therapy compared with standard therapy in acute ischaemic stroke
Authors: Hallan S. , Åsberg A. , Indredavik B. , Widerøe T. E. 
Published: March 2003

Abstract.

Hallan S, Åsberg A, Indredavik B, Widerøe TE (University Hospital of Trondheim, Trondheim, Norway). A decision analysis of thrombolytic therapy compared with standard therapy in acute ischaemic stroke. J Intern Med 1999; 246: 549–559.Objectives. Experts draw different conclusions on whether thrombolysis can be recommended or not for acute ischaemic stroke. A major problem is weighing the improvement in functional ability against the risk of increased mortality. We wanted to examine this uncertainty regarding thrombolysis using a systematic approach and with a strong emphasis on the patient’s point of view.Methods. We performed a decision analysis where the base case focused on an average stroke patient. We used published probabilities for different functional outcomes after standard supportive care and after adding tissue plasminogen activator (tPA), and we tried to estimate corresponding long‐term survival. We interviewed 158 subjects with the standard gamble method to elicit their preference values (utility) for these outcomes.Results. When using the baseline data for an average stroke patient, thrombolysis with tPA was the better choice, with 48 extra quality‐adjusted living days; tPA was also superior in 117 individual decision analyses , giving from 10 to 173 extra days. However, sensitivity analysis showed that these results were highly susceptible to changes in utility for major disability, probability of early death, and long‐term survival after thrombolysis. To increase the gain as well as the margin of safety regarding the treatment choice, thrombolysis should be restricted to patients who assign low utility values < 0.6–0.7 to major poststroke disability (death = 0.0, good health = 1.0).Conclusion. Evaluated by decision analysis, thrombolysis with tPA is on average superior to standard therapy for the few patients fulfilling the strict medical inclusion criteria. Individual incorporation of the patient’s point of view narrows the indication even further.