Ileal atresia and thrombo-embolic liver calcifications diagnosed after treatment with intrauterine laser coagulation therapy for twin-to-twin transfusion syndrome: report of 2 cases

Ileal atresia is considered to be an acquired anomaly. It is thought to be caused by a mesenteric vascular accident during fetal life which leads to necrosis of downstream tissues. We present a case in which ileal atresia occurred after intrauterine laser coagulation therapy for a twin-to-twin transfusion syndrome. The laser treatment might have been the etiological factor of the atresia. A second case in which thrombo-embolic calcifications were found in the liver is presented as well.     

The multiply injured child

The treatment of multiple traumas in children requires knowledge of common injury patterns, incidence, mortality, and the consequences and differences between these injuries in children and adult patients. However, epidemiological studies concerning pediatric multiple trauma are rare. To address this, data were collected and analyzed from 682 multiple trauma patients treated at a Level I trauma center. The patients were divided into four age-related groups (< 6 years, 6-12 years, 13-18 years, and 18-40 years) and were evaluated for trauma mechanism, injury distribution, and cause of death. Children aged 6 to 17 years mostly were injured as pedestrians and cyclists whereas infants, preschoolers, and adults more commonly were injured as car passengers. Pediatric patients suffered a significantly higher mortality than adults, with a threefold increased risk of death when injured as passengers in car accidents. Injuries to the head and the legs were most common. A lower incidence of thoracic (28% versus. 62%), abdominal (20% versus 36%), pelvic (22% versus 35%), and upper limb (32% versus 43%) trauma was observed in children (< 18 years) than in adults (18-40 years). Nevertheless, trauma to the thorax, abdomen, and head were associated with a high risk of death in all groups. Spinal cord injuries, especially in the cervical region, also carried a high risk of mortality (36.8 in the group of patients younger than 18 years and 18.9 in the group of patients 18-40 years). Children younger than 6 years had the most severe head injuries. The data show that there are important differences in incidence, mortality, and injury patterns between pediatric and adult patients with multiple traumas.     

Pathophysiology of language switching and mixing in an early bilingual child with subcortical aphasia

Acquired aphasia after circumscribed vascular subcortical lesions has not been reported in bilingual children. We report clinical and neuroimaging findings in an early bilingual boy who incurred equally severe transcortical sensory aphasia in his first language (L1) and second language (L2) after a posterior left thalamic hemorrhage. Following recurrent bleeding of the lesion the aphasic symptoms substantially aggravated. Spontaneous pathological language switching and mixing were found in both languages. Remission of these phenomena was reflected on brain perfusion SPECT revealing improved perfusion in the left frontal lobe and left caudate nucleus. The parallelism between the evolution of language symptoms and the SPECT findings may demonstrate that a subcortical left frontal lobe circuity is crucially involved in language switching and mixing.     

The Middelheim Frontality Score: a behavioural assessment scale that discriminates frontotemporal dementia from Alzheimer's disease

BACKGROUND: Despite striking neuropsychological and behavioural differences between Alzheimer's disease (AD) and frontotemporal dementia (FTD), clinical diagnostic criteria failed to discriminate FTD from AD patients. We therefore developed the Middelheim Frontality Score (MFS), a disease-long clinical and behavioural assessment tool that measures frontal lobe features, and set up this prospective study in clinically diagnosed AD and FTD patients to assess discriminatory power and intra- and inter-rater variability. METHODS: Patients with probable AD (n = 400) and FTD (n = 62) were included. The MFS was obtained by summating the scores obtained in a standardized fashion on ten items yielding a total maximal score of 10. Information was obtained through an interview of the patient and her/his caregiver, clinical files and behavioural observation. RESULTS: Comparing mean total MFS scores, FTD patients (6.3 +/- 1.8) had significantly higher scores than AD patients (3.1 +/- 1.8) (p < 0.001). Distribution of scores on individual MFS items was significantly different between both disease groups (chi(2) = 76.2; p < 0.001). A moderately positive and highly significant correlation was shown between the total MFS score and diagnosis FTD (r = 0.478; p < 0.0001). Applying a total MFS score of 5 as discriminatory cut-off, a specificity of 89.0% and a sensitivity of 88.7% were achieved. Intra- and inter-rater variability was calculated in a different study population by means of retest correlation, revealing moderate to strong positive correlations of high statistical significance. CONCLUSIONS: The MFS is a clinical and behavioural assessment scale that measures frontal lobe features and that was shown to reliably discriminate FTD from AD patients.     

Altered circadian locomotor activity in APP23 mice: a model for BPSD disturbances

Over the past decade, clinical Alzheimer's disease research has been challenged with an increased interest in noncognitive symptomatology, commonly referred to as behavioural and psychological signs and symptoms of dementia (BPSD). In accordance, major attention is being paid to behavioural alterations in the phenotyping of transgenic mouse models. Besides an age-dependent decline of cognitive functions, the APP23 model was previously shown to exhibit cage activity disturbances, reminiscent of diurnal rhythm disturbances in Alzheimer patients. To further scrutinize these observations, circadian patterns of horizontal locomotor activity were assessed in 3-, 6- and 12-month-old APP23 mice and wild-type littermates in a test paradigm continuously recording cage activity over a period ranging from 1 to 3 days. At the age of 3 months, APP23 profiles resembled the wild-type pattern to a large extent, although minor differences were already noticeable. Six-month-old APP23 mice displayed an altered activity profile with a first indication of increased activity during the second half of the active phase, reminiscent of sundowning behaviour in Alzheimer patients. This bimodal overnight activity pattern became even more evident at the age of 12 months. The APP23 model was therefore shown to display an age-dependent development of cage activity disturbances and sundowning-like behaviour. A comparison is made with actigraphic recordings of human Alzheimer patients exhibiting sundowning behaviour. This first report of diurnal rhythm disturbances and sundowning-like phenomena in a transgenic mouse model greatly adds to the validity of the APP23 model.     

Voxel-based morphometry in hypocretin-deficient narcolepsy

STUDY OBJECTIVES: Recent studies suggest that narcolepsy is caused by degeneration of hypocretin (orexin) producing neurons. To find evidence for this hypothesis, we aimed to detect structural changes in the hypothalamus and/or hypocretin projection areas of patients with narcolepsy. DESIGN: We used voxel-based morphometry (VBM), an unbiased MRI morphometric method with a high sensitivity for subtle changes in gray and white matter volumes. SETTING: Image acquisition was carried out in the department of Radiology at Leiden University Medical Center; image post-processing was performed in the Wellcome Department of Cognitive Neurology, London. PARTICIPANTS: Fifteen narcoleptic patients were studied, all having cataplexy and typical findings on Multiple Sleep Latency Testing. All patients were HLA-DQB1*0602 positive and hypocretin-1 deficient. The control group consisted of 15 age and sex matched healthy subjects. MEASUREMENTS AND RESULTS: We found no differences in global gray or white matter volumes between patients and controls. Furthermore, regional gray or white matter volumes in the hypothalamus and hypocretin projection areas did not differ between patients and controls. CONCLUSIONS: VBM failed to show structural changes in the brains of patients with narcolepsy. This suggests that narcolepsy either is associated with microscopic changes undetectable by VBM or that functional abnormalities of hypocretin neurons are not associated with structural correlates.     

Dietary zinc reduces uptake but not metallothionein binding and elimination of cadmium in the springtail,Orchesella cincta

In metal-polluted environments, high concentrations of metals such as zinc, cadmium, copper, and lead are present. Many studies have been conducted to determine uptake and binding of the nonessential, toxic metal cadmium. Effects of essential metals on this uptake and binding remain unclear. The aim of the present study was to determine the effect of zinc on cadmium kinetics and metallothionein induction in the springtail Orchesella cincta. Exposure experiments were conducted with cadmium only, zinc only, and cadmium + zinc. Metallothionein was isolated to determine zinc binding to or induction of this cadmium-binding protein. In animals exposed to cadmium only and to cadmium + zinc, metallothionein was induced. In animals exposed to zinc only, no metallothionein was induced, and zinc did not bind to the metallothionein induced by cadmium. Cadmium uptake rate was significantly lower in animals exposed to cadmium + zinc than in animals exposed to cadmium only. No significant pattern of uptake and elimination of zinc was observed. Zinc concentrations in the animals were not significantly different for the different exposures, demonstrating that this element is regulated by O. cincta. These results suggest a protective role of zinc in the uptake of cadmium but no interference of zinc with the internal cadmium distribution and elimination.     

Nursing science and related sciences. Questions about centralization of nursing science and interdisciplinary relations

The thesis of this paper is that nursing science draws a lot of its innovative potential from the knowledge that has been gathered in related disciplines. This knowledge is specified and reformulated with regard to nursing practice. The thesis is developed on two levels: first under aspects of methodology and the philosophy of science, then under aspects of the adoption of interdisciplinary knowledge. The integration of various related sciences that takes place in nursing science will finally be demonstrated in an example. This example shows which qualifications the practitioners need to deal with patients who suffer from a stroke.