bluuebunny/crossref_metadata_2025_split huggingface dataset:
Mastery motivation in young children with Down's syndrome: relations with cognitive and adaptive competence
Authors: Niccols A. , Atkinson L. , Pepler D. 
Published: March 2003

AbstractBackground  Prior research on relations between motivation and competence have focused on cognitive competence, despite theoretical predictions that mastery motivation spurs behaviour that is effective in meeting the demands of one's environment, i.e. adaptive competence. Issues of adaptive competence are especially relevant for children with developmental delay since functional independence is an important long‐term goal for these children.Methods  In the present study, mastery motivation was examined in relation to both cognitive and adaptive competence in 5‐year‐old children with Down's syndrome (n = 41).Results  Scores on mastery task and parent‐report measures of mastery motivation were generally low, but positively related to scores on standardized measures of cognitive competence and adaptive competence.Conclusions  This is the first study of motivation–competence relations in pre‐school‐age children. The findings have implications for both developmental theory and early intervention efforts, and the authors hope that they will serve as a stimulus for future research on cross‐domain relationships in atypical populations.
Assessment of change with the Developmental Behaviour Checklist
Authors: Clarke A. R. , Tonge B. J. , Einfeld S. L. , Mackinnon A. 
Published: March 2003

AbstractBackground  The Developmental Behaviour Checklist (DBC) is a 96‐item instrument designed for assessing behavioural and emotional problems among young people with intellectual disability.Methods  The present study investigated the validity of the DBC as a measure of change. Changes in individual's DBC scores were correlated with changes in expert clinicians’ ratings of the same subjects.Results  A high correlation was found between expert clinician ratings of change in behaviour and change in the total behaviour problem score of the DBC.Conclusions  The DBC is able to measure changes in behaviour and emotions which are recognized by an experienced clinician who is following the progress of a child with ID.
Radiocontrast‐induced natriuresis associated with increased urinary urodilatin excretion
Authors: Haller  , Meyer  , Scheele  , Koch  , Forssmann  , KÜbler  
Published: March 2003

Haller C, Meyer M, Scheele T, Koch A, Forssmann W‐G and Kübler W (University of Heidelberg; and Lower Saxony Institute for Peptide Research, Hanover; Germany). Radiocontrast‐induced natriuresis associated with increased urinary urodilatin excretion (original article). J Intern Med 1998; 243: 155–62.ObjectiveIntravascular radiocontrast agents induce a pronounced diuresis. The aim of the present study was to investigate the (patho‐)physiological mechanisms of the radiocontrast‐induced diuresis.DesignThe fractional excretion of sodium, the urinary excretion of the renal natriuretic peptide urodilatin and the plasma concentration of atrial natriuretic peptide were measured in 42 unselected patients immediately before and after intravascular radiocontrast administration during coronary angiography.SettingCardiac catheterization laboratory of a university hospital.ResultsAfter angiography both the plasma concentration of atrial natriuretic peptide (median post‐pre difference: 3.9 pmol L−1, quartiles −1.2; 7.0) and the urinary excretion of urodilatin (median post‐pre difference: 67.0 nmol urodilatin/mol creatinine, quartiles 39.7; 152.1) were increased. The urinary urodilatin excretion was correlated with an increase in the fractional excretion of sodium (median post‐pre difference: 1.7%, quartiles 0.6; 3.1). There was no correlation between the serum concentration of atrial natriuretic peptide and urinary sodium excretion. For the radiocontrast‐induced increase in both urodilatin and sodium excretion there was no indication for differences between patients without (31) and with (11) intravenous saline infusion.ConclusionThe radiocontrast‐induced diuresis is a natriuresis which is associated with an increased urinary excretion of urodilatin. The association between natriuresis and urinary urodilatin excretion irrespective of baseline volume status corroborates the hypothesis that urodilatin contributes to the sodium excretion after radiocontrast administration in a paracrine manner. This finding has pathophysiological and potentially therapeutic implications in radiocontrast‐induced nephropathy.
Pathology of MEN‐1: morphology, clinicopathologic correlations and tumour development
Authors: Komminoth  , Heitz  , Klöppel  
Published: March 2003

Komminoth P, Heitz PU, Klöppel G (University of Zürich, Switzerland; and University of Kiel, Germany). Pathology of MEN‐1: morphology, clinicopathologic correlations and tumour development (Minisymposium: MEN & VHL). J Intern Med 1998; 243: 455–64.Multiple endocrine neoplasia type 1 (MEN‐1) is an inherited syndrome which is characterized by the occurrence of neoplastic lesions in the parathyroids, the pancreas, duodenum, anterior pituitary and, less commonly, also in the stomach, thymus and lung. Its genetic defect has recently been identified and appears to involve a new type of tumour suppressor gene called mu on chromosome 11q13. In this overview, we will summarize the morphological features of the MEN‐1 phenotype, discuss its clinicopathologic profile and prognosis and outline the recent findings on the molecular pathology of this syndrome.
Expression of the MEN‐1 gene in a large kindred with multiple endocrine neoplasia type 1
Authors: Burgess  , Greenaway  , Shepherd  
Published: March 2003

Burgess JR, Greenaway TM, Shepherd JJ (Royal Hobart Hospital, and University of Tasmania, Hobart, Tasmania, Australia). Expression of the MEN‐1 gene in a large kindred with multiple endocrine neoplasia type 1 (Minisymposium: MEN & VHL). J Intern Med 1998; 243: 465–70.In 1983 a large family with MEN‐1 (designated Tasman 1) was identified in Tasmania. Kindred screening and case follow‐up over the subsequent 15 years has yielded data on over 160 MEN‐1‐affected patients. Hyperparathyroidism is present in over 60% of gene carriers by age 20 years and 95% by age 30 years. Hyperplasia is the characteristic pathological finding. Kaplan–Meier analysis indicates hyperparathyroidism recurs in the majority of patients despite near‐total parathyroidectomy. Gastrinoma, ‘nonfunctioning’ pancreatic adenoma and insulinoma occur in up to 60, 50 and 10% of patients, respectively. Metastatic gastroenteropancreatic (GEP) tumours develop in up to 35% of family members, being frequent in some branches of Tasman 1, whilst rare in others. Pituitary disease developed in 19% of patients. Prolactinoma and ‘nonfunctioning’ adenoma account for 76 and 24%, respectively, of pituitary abnormalities. Prolactinomas exhibit clustering within branches of the Tasman 1 kindred. Adrenal adenomas occur in 36% of patients. The majority of adrenal lesions are benign and nonsecretory and develop in association with pancreatic neoplasia. Carcinoid tumours are uncommon but important malignancies. Malignant thymic carcinoid occurs in male patients, whereas bronchial carcinoid occurs predominantly in women. Prior to recognition of MEN‐1 in Tasman 1, complications of hyperparathyroidism and malignancy accounted for the majority of patient mortality. Since commencement of prospective screening, malignant GEP tumours and cardiovascular disease have become the most prevalent causes of death amongst MEN‐1‐affected patients.
Abnormal metabolism of free fatty acids in hypertriglyceridaemic men: apparent insulin resistance of adipose tissue
Authors: Mostaza  , Vega  , Snell  , Grundy  
Published: March 2003

Mostaza JM, Vega GL, Snell P, Grundy SM (The Center for Human Nutrition, and the Departments of Clinical Nutrition and Internal Medicine, University of Texas South Western Medical Center, and the Veterans Affairs Medical Center at Dallas, Texas, USA). Abnormal metabolism of free fatty acids in hypertriglyceridaemic men: apparent insulin resistance of adipose tissue. J Intern Med 1998; 243: 265–74.Objective.There is growing evidence that endogenous hypertriglyceridaemia is frequently accompanied by a state of insulin resistance. The present study was performed to determine whether patients with primary endogenous hypertriglyceridaemia commonly have abnormalities in plasma concentrations and turnover rates of free fatty acids (FFA), which could reflect a state of insulin resistance in adipose tissue and could account for raised plasma triglycerides.Design.Hypertriglyceridaemic and normotriglyceridemic control patients underwent measurements of plasma concentrations and turnover rates of FFA. Fat weights in both groups were determined by hydrodensitometry, and fat distribution was assessed by skin‐folds and measurement of waist and hip circumferences. Other measurements included plasma glucose, insulin, lipids, and lipoproteins.Subjects.Fifteen men with normal plasma triglycerides and 21 men with primary endogenous hypertriglyceridaemia were studied. Men in both groups varied in body weights and total fat weights, but total fat weights were entirely overlapping for the two groups. Waist‐to‐hip ratios and waist circumferences also were similar for the two groups.Results.For any total body fat content or waist circumference, most hypertriglyceridaemia patients had higher mean plasma concentrations of FFA and higher turnover rates (flux) for FFA than did normotriglyceridemic patients. Hypertriglyceridaemic patients also had higher fasting insulin concentrations for a given body fat content. In general, both FFA flux and plasma insulin levels were positively correlated with plasma concentrations of triglyceride and inversely with high density lipoprotein (HDL) cholesterol.Conclusions.These studies indicate that many patients with primary endogenous hypertriglyceridaemia have increased flux of FFA and hyperinsulinemia that cannot be explained either by increased total body fat content or by greater waist circumferences than observed in normotriglyceridemic patients.
Role of routine transthoracic echocardiography in evaluation and management of stroke
Authors: Beattie  , Cohen  , Manning  , Douglas  
Published: March 2003

Abstract. Beattie JR, Cohen DJ, Manning WJ, Douglas PS (Harvard Medical School, Harvard‐Thorndike Laboratory, and Department of Health Policy and Management, Harvard School of Public Health, Boston, MA, USA). Role of routine transthoracic echocardiography in evaluation and management of stroke. J Intern Med 1998; 243: 281–91.ObjectiveTo determine the value of routine transthoracic echocardiography in patients with cerebral ischemia without known cardiac disease.DesignThe literature from 1990 to 1995 was searched and relevant bibliographies from these papers reviewed. Articles reporting the prevalence and/or risk of stroke for myxoma, vegetation, mitral stenosis, left atrial thrombus, left ventricular thrombus or cardiomyopathy, patent foramen ovale, and atrial septal aneurysm were used. Prevalences and recurrent stroke risk were examined for adult patients less than and greater than 45 years.ResultsBoth the prevalence of transthoracic echocardiographic findings and recurrent risk of stroke differ by age. The finding of a patent foramen ovale can be expected in nearly half of younger patients with stroke, whilst transthoracic echo can be expected to yield no relevant lesion in three quarters of patients > 45 years. Warfarin anticoagulation appears beneficial in patients with mitral stenosis, left atrial thrombus, left ventricular thrombus, and left ventricular dysfunction, but is of unproven benefit for patent foramen ovale, atrial septal aneurysm, or in the absence of a risk‐associated abnormality.ConclusionFor most transthoracic echo findings in stroke, optimal management strategies have not been well defined. Future research is needed to evaluate the appropriate therapeutic approaches.
The relations of microalbuminuria to ambulatory blood pressure and myocardial wall thickness in a population
Authors: Nilsson T. , Svensson A. , Lapidus L. , Lindstedt G. , Nyström E. , Eggertsen R. 
Published: March 2003

Abstract. Nilsson T, Svensson A, Lapidus L, Lindstedt G, Nyström E, Eggertsen R (Mölnlycke Primary Health Care and Research Centre; Göteborg University, Göteborg, Sweden). The relations of microalbuminuria to ambulatory blood pressure and myocardial wall thickness in a population. J Intern Med 1998; 244: 55–9.ObjectivesTo examine the relationship between microalbuminuria (20–200 μg min−1) and 24 h ambulatory blood pressure and heart wall thickness, in a representative population sample of men and women aged 56–65 years.DesignEvery second individual aged 56–65 years (n= 488) in the district, was invited for a health examination, which included determination of urinary albumin and creatinine (overnight sample). The highest and lowest decentile of urinary albumin/creatinine ratio were compared.SettingThe district of the Primary Health Care and Research Centre of Mölnlycke, Sweden.SubjectsAfter excluding 2 individuals with a urinary albumin excretion exceeding 200 μg min−1, 26 subjects (group 1) could be compared with 27 subjects in the lowest decentile (group 2).Main outcome measuresComparison between the determinations of the ambulatory blood pressure and echocardiographic variables in the two groups.ResultsGroup 1 had significantly higher 24 104 ambulatory blood pressure, and heart septum and posterior wall thickness as well as significantly higher fasting blood glucose and serum triglyceride concentrations. The differences in blood pressure (P &lt; 0.05) but not heart wall thickness remained significant (P &lt; 0.05) after excluding subjects with hypertension, angina pectoris, treated diabetes mellitus, and/or history of heart or cerebrovascular disease. When excluding individuals with both treated and untreated diabetes mellitus, fasting blood glucose concentration was higher in group 1. The waist‐hip ratio, weight and body mass index did not differ between the groups.ConclusionsThe findings indicate that microalbuminuria is related to signs of cardiovascular and metabolic influence and therefore could be a valuable tool for grading the risk of later cardiovascular morbidity.
Differential effects of antihypertensive drugs on neurohormonal activation: insights from a population‐based sample
Authors: Schunkert  , Hense  , Bröckel  , Luchner  , Muscholl  , Holmer  , Danser  , Mayer  , Riegger  
Published: March 2003

Schunkert H, Hense H‐W, Bröckel U, Luchner A, Muscholl M, Holmer SR, Danser AHJ, Mayer B, Riegger GAJ (University of Regensburg, Regensburg; University of Münster, Münster, Institut für Epidemiologie, Munich‐Neuherberg, Germany; Erasmus University, Rotterdam, The Netherlands). Differential effects of antihypertensive drugs on neurohormonal activation: insights from a population‐based sample.J Intern Med1998;244: 109–19.ObjectivesThe clinical course of hypertension or heart failure may be modified by the extent of concurrent neurohormonal activation. Factors that regulate neurohormones in patients with these conditions are complex. In the present study, we examined the relative contribution of antihypertensive therapy to the variability of neurohormonal levels in a well defined population based sample.Design and settingCross‐sectional study of a mixed urban and rural population.SubjectsMiddle‐aged individuals (n= 646) were analysed in order to elucidate determinants of neurohormone levels by uni‐ and multivariate comparisons. The assessment included anthropometric, echocardiographic and, if appropriate, genotype information.ResultsThe intake of antihypertensive drugs was related to significant alterations of neurohormone levels that, in part, exceeded the contribution of all other variables studied. Multivariate analyses revealed that renin levels were independently related to the intake of beta blockers (n= 80; –8.4 mU L−1;P= 0.001), angiotensin‐converting enzyme (ACE)‐inhibitors (n= 39; +15.9 mU L−1;P= 0.0001), diuretics (n= 62; +14.3 mU L−1;P= 0.0001), and calcium channel blockers (n= 45; +5.9 mU L−1;P= 0.05). Aldosterone levels were related to ACE‐inhibition (–156.5 pmol L−1;P= 0.04) and diuretic treatment (+422.4 pmol L−1;P= 0.0001) in an opposite fashion whereas beta blockers and calcium channel blockers had no significant independent effects. The levels of the atrial natriuretic peptide were significantly related to the use of beta blockers (+3.9 pmol L−1;P= 0.002) and calcium channel blockers (+3.1 pmol L−1;P= 0.05). Finally, serum angiotensinogen levels and ACE activity were not found to be significantly affected by antihypertensive medication but were rather related to gender or genotype.ConclusionsThe data emphasize that antihypertensive treatment with different classes of drugs may modulate serum levels of neurohormones substantially resulting in distinct patterns of activation. These drug‐related effects may require consideration when neurohormonal activation is of functional relevance or when neurohormones serve as prognostic predictors in patients with cardiovascular disorders.
Patients hospitalized because of small vessel vasculitides with renal involvement in the period 1975–95: organ involvement, anti‐neutrophil cytoplasmic antibodies patterns, seasonal attack rates and fluctuation of annual frequencies
Authors: Tidman  , Olander  , Svalander  , Danielsson  
Published: March 2003

Tidman M, Olander R, Svalander C, Danielsson D (Örebro Medical Center Hospital, Örebro and Sahlgrenska Hospital, Gothenburg, Sweden). Patients hospitalized because of small vessel vasculitides with renal involvement in the period 1975–95: organ involvement, anti‐neutrophil cytoplasmic antibodies patterns, seasonal attack rates and fluctuation of annual frequencies. J Intern Med 1998; 244: 133–41.Objectives: To study organ involvement, anti‐neutrophil cytoplasmic antibodies (ANCA) patterns, trends in yearly frequencies and seasonal variations of symptom onset in patients hospitalized because of small vessel vasculitides during a 21 year period (1975–95).DesignA retrospective investigation was conducted of 138 patients hospitalized with a diagnosis of small vessel vasculitides, as defined by the Chapel Hill Consensus Conference, within the County of Örebro, a mixed urban and rural area of central Sweden.SettingÖrebro Medical Center Hospital, Örebro, Sweden and two district hospitals within the County of Örebro, Sweden.ResultsDuring the studied period there were 19 patients with a diagnosis of Wegener's granulomatosis (WG), 70 patients with microscopic polyangiitis (MPA), 36 patients with renal limited vasculitis (RLV), two with Churge‐Strauss vasculitis (C‐S), seven with Henoch‐Schönleins purpura (HSP) and four with essential cryoglobulinemic vasculitis (ECV). Renal involvement was present in 123 patients (89.1%). A positive c‐ and/or pANCA was found in nearly 90% of the 111 patients where sera were available. Calculations of frequency data, restricted to the primary catchment area for patients with ANCA associated vasculitis and renal involvement (WG, MPA, RLV) during a 21‐year period (1975–95) gave a mean annual frequency of 1.6 per 100 000 adults (95% CI: 1.2–3.1); for this group of patients with the inclusion of those with C‐S, HSP and ECV during the last 10 year period (1986–95) gave a mean annual frequency of 2.5 per 100 000 adults (95% CI: 1.7–3.4), for male adults 3 per 100 000 (95% CI: 1.6–4.4), and female adults 1.9 (95% CI: 0.9–2.8). A frequency peak of 6.3 per 100 000 was seen for men aged 55–64. A periodic fluctuation of the frequencies with peaks every 3–4 years was noted for patients with ANCA related vasculitis (WG, MPA, RLV) during the 21‐year period 1975–95. Onset of symptoms was predominantly noticed during the winter months (December– February) for patients with a positive cANCA.ConclusionThe observed frequencies in our study of patients with small vessel vasculitides were higher than those previously documented. We also showed a periodic fluctuation of the annual frequencies and a seasonal variation of symptom onset.
Idiopathic oedema with increased cytokine production: a pathogenetic link?
Authors: Hoffmann  , Fontana  , Steurer  , Bollinger  
Published: March 2003

Hoffmann U, Fontana A, Steurer J, Bollinger a (University Hospital, Zurich, Switzerland). Idiopathic oedema with increased cytokine production: a pathogenetic link? (Case Report). J Intern Med 1998; 244: 179–82.We describe four patients with idiopathic oedema and elevated cytokines. All patients shared increased serum concentrations of soluble interleukin‐2 receptors (sIL‐2R). In three patients tumour necrosis factor alpha (TNF‐alpha), gamma interferon (IFN‐gamma) and interleukin‐2 (IL‐2) were transiently elevated. There was no evidence for an underlying disease. Based on experimental and clinical data it is hypothesized that oedema formation in our patients is the consequence of cytokine induced alteration of endothelial cells.
Metabolic abnormalities related to cardiovascular risk in primary hyperparathyroidism: effects of surgical treatment
Authors: Valdemarsson  , Lindblom  , Bergenfelz  
Published: March 2003

Valdemarsson S, Lindblom P, Bergenfelz A (Departments of Internal medicine and surgery, Lund University Hospital, Lund Sweden). Metabolic abnormalities related to cardiovascular risk in primary hyperparathyroidism: effects of surgical treatment. J Intern Med 1998; 244: 241–49.ObjectivesUntreated primary hyperarathyroidism (pHPT) is accompanied by an excessive morbidity in circulatory disorders, associated with blood pressure and diabetes. The aim of the present study was to further penetrate the impact of pHPT on glucose, urate, lipid and lipoprotein concentrations, known to be interrelated metabolic cardiovascular risk factors.DesignLongitudinal study of patients with pHPT before and 1 year after surgical treatment.SettingDepartments of Internal Medicine and Surgery, Lund University Hospital.SubjectsOne hundred and seventeen consecutive patients with pHPT referred to surgical treatment. At presentation, 11 patients had previously diagnosed diabetes mellitus.InterventionAll patients were successfully operated for pHPT.Main outcome measuresFasting blood glucose and serum concentrations of cholesterol, triglyceride and urate were determined before and 1 year after surgery. The concentration of LDL‐ and HDL‐cholesterol was separately analyzed in 21 cases. These data as well as the systolic and diastolic blood pressure were related to intact PTH and ionized calcium at presentation. Glomerular filtation was separately measured pre‐operatively and related to the urate values.ResultsWhile the mean value for glucose remained unchanged among 11 patients with previously diagnosed diabetes at presentation, a significant decrease of glucose from 5.03 ± 0.13 to 4.71 ± 0.08 mmol/L (P < 0.05) was found among patients without known diabetes. Out of these patients, eight had diabetic glucose values at presentation, decreasing from 8.35 ± 0.54 to 5.10 ± 0.35 mmol/L (P < 0.05), and 12 had glucose values indicating impaired glucose tolerance, decreasing from 5.94 ± 0.06 to 5.10 ± 0.38 mmol/L (P < 0.05) after surgery. Total cholesterol and trigylceride concentrations were not changed. However, male patients had significantly lower triglyceride levels at follow‐up, 1.16 ± 0.09 mmol/L compared to 1.57 ± 0.14 mmol/L before surgery (P < 0.05). Significantly lower triglyceride values were also found among patients with glucose values indicating impaired glucose tolerance at presentation. The LDL/HDL cholesterol ratio remained normal. The serum level of urate decreased in both male and female patients after surgery, and was positively correlated to the PTH and ionized calcium values and inversely correlated to renal function before treatment. There was no significant correlation between calcium or PTH and the other metabolic variables studied.