Publications by authors named "Mott S"

The feasibility and safety of a procedure involving fetal spinal cord tissue transplantation in patients with syringomyelia was assessed using a neurophysiological protocol designed to quantitate peripheral nerve function, spinal cord reflex excitability, and spinal cord conduction pathways essential for somatosensory evoked potentials. We report here data obtained before and for 18 months following the transplantation procedure performed on the first two patients in this study. The neurophysiological assessment protocols included measures of cortical and spinal cord evoked potentials, H-reflex excitability, and peripheral nerve conduction.

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Familial HDL deficiency (FHD) is the heterozygous form of Tangier disease (TD). Mutations of the ABCA1 gene cause FHD and TD. FHD/TD cells are unable to normally efflux cholesterol onto nascent HDL particles, which are rapidly catabolized.

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Background: High density lipoproteins (HDL) are complex lipoprotein particles involved in reverse cholesterol (C) transport and are negatively associated with the risk for coronary artery disease (CAD). We have described a disorder of familial HDL deficiency (FHD) due to abnormal cellular cholesterol efflux. In the present study, we investigated cellular cholesterol efflux on skin fibroblast from 15 probands with moderate to severe hypoalphalipoproteinemia, including one subject with Tangier disease (TD).

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Objective: To identify age-dependent activation patterns of verbal fluency with functional MRI (fMRI).

Background: Few fMRI language studies have been performed in children, and none provide comparison data to adult studies. Normative data are important for interpretation of similar studies in patients with epilepsy.

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Background: A low concentration of HDL cholesterol is the most common lipoprotein abnormality in patients with premature atherosclerosis. We have shown that Tangier disease, a rare and severe form of HDL deficiency characterised by a biochemical defect in cellular cholesterol efflux, is caused by mutations in the ATP-binding-cassette (ABC1) gene. This gene codes for the cholesterol-efflux regulatory protein (CERP).

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Genes have a major role in the control of high-density lipoprotein (HDL) cholesterol (HDL-C) levels. Here we have identified two Tangier disease (TD) families, confirmed 9q31 linkage and refined the disease locus to a limited genomic region containing the gene encoding the ATP-binding cassette transporter (ABC1). Familial HDL deficiency (FHA) is a more frequent cause of low HDL levels.

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Demographic predictions indicate that there will be between 174,300 and 194,200 people in Australia experiencing dementia of the Alzheimer's type by the year 2006. At present, 60% of those who make up the resident population in nursing homes and aged-care units in psychiatric hospitals suffer from dementia. To explore the place of people with dementia in a mental health setting, a comprehensive review of the literature was undertaken and the results compared with the outcomes of an ethnographic study of a unit for confused older people.

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Background: Assessment of language organization is crucial in patients considered for epilepsy surgery. In children, the current techniques, intra-carotid amobarbital test (IAT) for language dominance, and cortical electrostimulation mapping (ESM), are invasive and risky. Functional magnetic resonance imaging (fMRI) is an alternative method for noninvasive functional mapping, through the detection of the hemodynamic changes associated with neuronal activation.

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Objective: To describe the indications for total abdominal hysterectomy for women with nonmalignant disease and to determine the immediate (initial ten days) and medium term outcome.

Design: A prospective cohort study.

Setting: Three district general hospitals in shire counties, two in outer London and one London teaching hospital.

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Encephalopathy, leukoencephalopathy, and secondary parkinsonism occurred in 3 children with refractory leukemia undergoing allogenic bone marrow transplantation (BMT) who were treated with high-dose amphotericin B for pulmonary aspergillosis or sinus aspergillosis that did not involve the nervous system. Treatment included high-dose cytosine arabinoside, cyclophosphamide, and total body irradiation prior to the BMT. The children developed a progressively worsening encephalopathy and parkinsonian features, characterized by resting tremor, cogwheel rigidity, and masklike facies.

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Objective: To describe the neurologic manifestations of cocaine exposure in children and adolescents as the neurologic effects of cocaine have been described in adults and neonates.

Methods: During 1-year period, 41 children between the ages of 2 months and 18 years who had been exposed to cocaine, were examined in the emergency department at the Children's National Medical Center. Cocaine exposure was documented on urine samples; all were confirmed by urine gas chromatographic/mass spectrometric analysis.

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The growth of Listeria monocytogenes , Aeromonas hydrophila and Yersinia enterocolitica on sliced roast beef packaged under vacuum or saturated CO controlled atmosphere conditions was measured. At -1.5°C, the pathogens declined in numbers in the controlled atmosphere packs but were able to grow under vacuum packaging.

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Parkinsonism is an uncommon movement disorder in childhood. Six unusual cases of acquired parkinsonism in hospitalized children are described. Clinical manifestations included an akinetic-rigid syndrome with and without tremor, the combination of parkinsonism and dystonia, and a parkinsonism-plus syndrome.

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A total of 182 Aeromonas hydrophila strains isolated from environmental (food and water) and clinical (stool and other sources) samples taken in mainland Australia, Tasmania and New Zealand were assigned to one of three DNA/DNA hybridization groups (HGs) on the basis of biochemical characteristics, and tested with regard to their ability to produce virulence factors. Strains from HG2 were rarely isolated; strains from HG1 were most commonly isolated from clinical sources; and strains from HG3 formed the majority of environmental strains. There was no correlation of HG to geographic source.

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One hundred ovine and 100 bovine carcasses in two abattoirs were sampled just after dressing for the presence of Yersinia enterocolitica, Listeria monocytogenes and motile aeromonads. Yersinia enterocolitica was not isolated and only two samples were positive for Listeria spp. In both cases, the Listeria species were not normally pathogenic to man.

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A reference or type strain and a food derived-strain of the cold-tolerant pathogens Listeria monocytogenes, Aeromonas hydrophila and Yersinia enterocolitica were individually inoculated into samples of commercial pâté and incubated at 4 and 10 degrees C. The organisms were periodically estimated by presumptive counts, then values for the lag and generation times were calculated. Both strains of L.

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Environmental swab samples collected on three occasions from a supermarket delicatessen were examined for the presence of Listeria monocytogenes, motile aeromonads and Yersinia enterocolitica. Strains of Y. enterocolitica belonging to a pathogenic biotype were not recovered, but isolates of L.

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This paper is based upon findings from a study carried out by the Institute of Nursing Research between 1989 and 1990. The major objective of the study was to determine the impact of staffing mix on nursing home residents' quality of care and life as measured against the standards set out in 'Living in a Nursing Home'. An additional objective was to identify if there are any factors which may constrain or influence optimality.

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This paper derives from a study conducted by the Deakin Institute of Nursing Research between 1988 and 1990, whose major objective was to determine the impact of staffing mix on nursing resident's quality of care and life. Resident satisfaction with life in the nursing home is a key element in determining the quality of care and quality of life provided. Both the literature review and the study objectives supported the view that resident outcome can be collected through assessing the quality of care and the quality of life, through assessment by informed observers using instruments derived from explicitly stated standards, and through eliciting the perceptions of residents themselves.

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This study investigated the relationships between skills mix, resident dependency and the quality of care and life of residents in a stratified random sample of 200 non-government homes in four Australian states. The study considered various measures of skills mix including the qualifications of the staff (staffing mix), the exposure of staff to in-service training and the leadership style of the director of nursing. The only relationships found between staffing mix (that is, the proportion of qualified and unqualified staff) related to the positive effect of therapy staff on the variety of experience of residents.

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A survey for the presence of Listeria spp., Yersinia enterocolitica and motile aeromonads in 203 samples of ready-to-eat fleshfoods purchased from retail outlets was conducted. Overall, 39.

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We reviewed cranial sonographic studies done on 108 normal newborn infants to determine the prevalence and variability in size of the cavum septi pellucidi (CSP). Infants were classified according to gestational age by 2-week intervals. At 24 weeks, only four normal scans were identified.

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This paper is based on the outcomes of a study undertaken by Deakin Institute of Nursing Research for the Department of Community Services and Health between 1988 and 1990 (1). The study, which looked at the relationship between skills mix and resident outcomes, involved gathering data from 200 nursing homes in four Australian states using questionnaires and a case study approach. One of the major findings related to the importance of the management and leadership style on the outcome of quality of care and life within the nursing home.

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Study Objective: To report a 9-year experience with the treatment of posthemorrhagic hydrocephalus (PHH) with the use of an easily inserted external ventricular drain.

Design: A case series with a retrospective review of hospital records and cranial ultrasound results, from 1981 through 1989, in all infants with PHH.

Intervention: A previously defined method of identification and bedside management of PHH was applied.

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