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Biomedical subjects

C Adams

Publications and source records attributed to C Adams.

At least 37 records · Page 2Linked to original sources
PubMed2001

Cerebral sinovenous thrombosis in children.

BACKGROUND: Cerebral sinovenous thrombosis in children is a serious disorder, and information is needed about its prevention and treatment. METHODS: The Canadian Pediatric Ischemic Stroke Registry was initiated in 1992 at the 16 pediatric tertiary care centers in Canada. Children (newborn to 18 years of age) with symptoms and radiographic confirmation of sinovenous thrombosis were included. RESULTS: During the first six years of the registry, 160 consecutive children with sinovenous thrombosis were enrolled, and the incidence of the disorder was 0.67 cases per 100,000 children per year. Neonates were most commonly affected. Fifty-eight percent of the children had seizures, 76 percent had diffuse neurologic signs, and 42 percent had focal neurologic signs. Risk factors included head and neck disorders (in 29 percent), acute systemic illnesses (in 54 percent), chronic systemic diseases (in 36 percent), and prothrombotic states (in 41 percent). Venous infarcts occurred in 41 percent of the children. Fifty-three percent of the children received antithrombotic agents. Neurologic deficits were present in 38 percent of the children, and 8 percent died; half the deaths were due to sinovenous thrombosis. Predictors of adverse neurologic outcomes were seizures at presentation and venous infarcts. CONCLUSIONS: Sinovenous thrombosis in children affects primarily neonates and results in neurologic impairment or death in approximately half the cases. The occurrence of venous infarcts or seizures portends a poor outcome.

Adolescent↗
PubMed2001

Severe infantile axonal neuropathy with respiratory failure.

We describe 5 infants (4 male, 1 female) with a severe intractable form of motor-sensory axonal neuropathy. All became ventilator-dependent, 4 have since died and 1 remains static. Diaphragmatic paralysis was an early feature with generalized neuropathy evolving rapidly. Nerve conduction studies and biopsies were consistent with axonal disease. This disorder could be a new condition or part of the spectrum of inherited neuropathies of the axonal degenerative type. It may be that there is a "switching-off" in the infant's Schwann cell-axonal interactions in utero or in the early postnatal period, resulting in severe progressive deterioration and then a static period without recovery.

Biopsy↗
PubMed2001

Mutations in the gene encoding immunoglobulin mu-binding protein 2 cause spinal muscular atrophy with respiratory distress type 1.

Classic spinal muscular atrophy (SMA) is caused by mutations in the telomeric copy of SMN1. Its product is involved in various cellular processes, including cytoplasmic assembly of spliceosomal small nuclear ribonucleoproteins, pre-mRNA processing and activation of transcription. Spinal muscular atrophy with respiratory distress (SMARD) is clinically and genetically distinct from SMA. Here we demonstrate that SMARD type 1 (SMARD1) results from mutations in the gene encoding immunoglobulin micro-binding protein 2 (IGHMBP2; on chromosome 11q13.2-q13.4). In six SMARD1 families, we detected three recessive missense mutations (exons 5, 11 and 12), two nonsense mutations (exons 2 and 5), one frameshift deletion (exon 5) and one splice donor-site mutation (intron 13). Mutations in mouse Ighmbp2 (ref. 14) have been shown to be responsible for spinal muscular atrophy in the neuromuscular degeneration (nmd) mouse, whose phenotype resembles the SMARD1 phenotype. Like the SMN1 product, IGHMBP2 colocalizes with the RNA-processing machinery in both the cytoplasm and the nucleus. Our results show that IGHMBP2 is the second gene found to be defective in spinal muscular atrophy, and indicate that IGHMBP2 and SMN share common functions important for motor neuron maintenance and integrity in mammals.

Amino Acid Sequence↗
PubMed2001

Tumour necrosis factor 5' promoter single nucleotide polymorphisms influence susceptibility to rheumatoid arthritis (RA) in immunogenetically defined multiplex RA families.

Tumour necrosis factor (TNF) plays a pivotal role in the pathogenesis of rheumatoid arthritis (RA) and it has been shown that the TNF-lymphotoxin (TNF-LT) region influences susceptibility to RA. To investigate the role of the TNF-LT locus further, inheritance of TNF 5' promoter alleles was determined in multiplex RA families. Six previously defined TNF promoter single nucleotide polymorphisms (SNPs) (-238, -308, -376, -857, -863, -1031) were observed in these families and in addition, a heretofore undocumented adenine (A) to cytosine (C) substitution at position -572 relative to the transcription start site was defined. TNF 5' promoter SNPs were found to co-segregate with specific TNF microsatellite haplotypes. In particular, the SNP -308A allele was found to be inherited with the TNF a2, b3, c1, d1, e3 (H2) microsatellite haplotype (P < 0.001) which had previously been found to be associated with RA in individuals heterozygous for the HLA-DR 'shared epitope' (SE). When the data were stratified by the presence of the SE with further stratification according to SE DR subtypes and analysed by transmission disequilibrium test (TDT) for which offspring were assumed independent, the -308A and -857T alleles were found to be associated with RA in patients carrying the SE (P = 0.0076 and 0.0063 respectively). The data were further stratified to analyse for association in individuals homozygous or heterozygous for SE alleles. Results showed that the -308A allele was significantly associated with RA susceptibility in individuals heterozygous for the SE (P < 0.001) with the significance only occurring in patients carrying HLA-DR4 (P < 0.001), while the -857T allele was significant in individuals homozygous for the SE (P = 0.0039). Further analysis using the pedigree disequilibrium test (PDT) which conservatively adjusts for all sources of familial correlation except that conferred by linkage disequilibrium still indicated a significant role for the -308A and -857T alleles. These data provide evidence that TNF promoter SNPs may play an independent role in RA susceptibility in specific immunogenetically-defined groups of RA patients.

Arthritis, Rheumatoid↗
PubMed2001

Risky business: stories from the field of rural community nurses' work in domestic violence.

This paper reports on a descriptive study into family violence in rural Victoria. Focus groups were held in a number of areas across rural Victoria with a total of 24 community nurse participants. The focus groups were audio-taped and the tapes transcribed to enable the clustering of themes. The dominant themes were: picking up cues, helping and helplessness, holding secrets and quiet resistance. Underpinning all these themes however, was the notion of 'risky business'. All nurses in the study gave examples of situations that they encountered; their ways of helping; of working around a system that is unhelpful; and the ways in which their work while skilled, thoughtful and wise, is also costly in terms of the emotional wounds they carry. Rural nurses work with considerable risk and courage as they engage in the care and support of women experiencing family violence.

Anecdotes as Topic↗
PubMed2001

What is the optimal therapy of brain metastases?

The conclusions of a symposium held in London in October 1999 and devoted to the optimal management of brain metastatic disease were: 1. Prognostic factors are: size and number of metastases (and the presence of mass effect); the status of the systemic cancer outside the central nervous system; performance/neurological status; the age of the patient; and the type of cancer. 2. Surgical management of the single, superficially located brain metastasis with symptomatic mass effect is recommended in good performance status patients. Many would follow this routinely by whole brain radiotherapy. 3. Whole brain radiotherapy is often not followed by durable control of the disease and carries morbidity; better management plans are required. In poor prognosis patients the delivery of radiotherapy may not always be indicated. 4. The current literature demonstrates that stereotactic radiosurgery can enhance the likelihood of sterilizing individual brain metastases compared with whole brain radiotherapy alone. 5. The results of questionnaire showed that the histological diagnosis and latency to onset made little difference to the opinion of neuroscience clinicians, who generally favoured stereotactic radiation therapy over whole brain radiotherapy (with or without a conventionally delivered boost) for all patients with less than four metastases. The opinions of oncologists differed. For bronchial and breast cancer patients, whole brain radiotherapy, with or without a boost, was favoured by the majority, particularly in oat cell cancer. However, with a long latency to 'isolated' brain metastasis, oncologists favoured focal radiation therapy. There was a strong preference amongst oncology experts to reserve stereotactic radiation therapy for apparently isolated brain metastasis; this opinion applied to bronchus and breast cancer, and also to melanoma. 6. Whole brain radiotherapy followed by positron emission tomography scanning to determine what viable metastatic disease remained (and potentially treatable by stereotactic/focal technology) was favoured by most of delegates who answered this question.

Brain Neoplasms↗
PubMed2001

Severe intermittent intraprosthetic regurgitation after mitral valve replacement with subvalvular preservation.

Preservation of the subvalvular apparatus during mitral valve replacement preserves left ventricular function and improves long-term survival. Complications of subvalvular preservation include left ventricular outflow tract obstruction and prosthesis impingement. We report a case of severe intermittent intraprosthetic mitral regurgitation detected by transesophageal echocardiography after mitral valve replacement by a bileaflet mechanical prosthesis with subvalvular preservation. Intravalvular prosthetic valve regurgitation was caused by remnants of the subvalvular apparatus, which were shown at reoperation to interfere with prosthetic leaflet motion and which were excised. Postoperative transesophageal echocardiography showed neither abnormal mitral regurgitation nor residual mass. The use of intraoperative transesophageal echocardiography could enable the detection of this rare complication.

Aged↗
PubMed2001

A time course study of the protective effect of mesenteric lymph duct ligation on hemorrhagic shock-induced pulmonary injury and the toxic effects of lymph from shocked rats on endothelial cell monolayer permeability.

BACKGROUND: We have previously documented that lymphatic duct division protects against shock-induced lung injury when tested 3 hours post-shock and that lymph collected at 3 hours post-shock increases endothelial cell monolayer permeability. However, whether lymph collected at other time points post-shock also increases endothelial cell permeability is not known. We tested the protective effects of lymphatic division on lung permeability at 6, 12, and 24 hours post-shock and the ability of lymph collected before, during, and hourly (up to 6 hours) after shock to increase endothelial cell monolayer permeability. METHODS: At 3, 6, 12, or 24 hours after sham or actual shock (30 mm Hg for 90 min), lung permeability was measured by using Evans blue dye in rats subjected to sham or actual mesenteric duct ligation. In separate experiments, the ability of lymph collected from rats subjected to shock or sham shock to increase human umbilical vein endothelial cell (HUVEC) monolayer permeability to a 40 kd dextran rhodamine permeability probe. Lymph was tested at 10% and 1% concentrations. RESULTS: Hemorrhagic shock induced a 3- to 4-fold increase in lung permeability compared with sham-shock rats when tested at 3, 6, 12, or 24 hours post-shock. Lymphatic division prevented this increase in lung permeability at each of these time points. Sham shock lymph did not increase HUVEC permeability, while lymph from the shocked rats did, whether tested at 1% or 10%. Lymph samples collected during the shock period and hourly for 6 hours post-shock all increased HUVEC permeability; however, the greatest relative increase in HUVEC permeability was observed in the 3- and 6- hour post-shock samples. CONCLUSIONS: Lung injury after hemorrhagic shock appears to be caused by toxic factors carried in the mesenteric lymph, and factors capable of increasing HUVEC permeability initially appear in the lymph during the shock period and increase over time.

Animals↗
PubMed2001

The benefits of integrated nursing teams in primary care.

This article reports a study in which a literature search, questionnaire study and interviews were used to measure the costs and benefits to patients and professionals when nurses work in integrated nursing teams in primary care. Our study showed that there are significant benefits for the nurses' employers, the primary health-care team, the nurses and the patients. Compared with traditional models, delivery by integrated nursing teams can offer a much higher quality service. Patients can expect to be cared for by a team in which morale is high, communication is good and care is efficient and effective. The team can also offer many specialist services that were not previously available. Integrated nursing teams are most effective when the management savings are used by the teams to meet their professional and client needs. Financial savings, therefore, cannot be expected. The difficulties that arise when such teams are set up should be short-term and surmountable, but will be influenced by the motivation of the team members and the efficiency of the human resource management.

Community Health Nursing↗
PubMed2001

Child'sTalk--for children with autism and pervasive developmental disorder.

A research assessment protocol and early intervention approach designed for use by multi-disciplinary professionals with children who have the severe social communication deficits of early autism and pervasive developmental disorder (PDD) are described. The assessment analyses the specific pattern of social communication impairment in each child and defines the characteristics of the dyadic communication between parent and child. The intervention aims to identify facilitative strategies, using video feedback, which lead to close interpersonal interaction between the child and their parents. Parents can reflect on their own interaction and identify which strategies successfully engage their child. Child'sTalk aims to facilitate adaptations to the child's level of communication by sensitively and finely tuning the interaction and mutual sharing of intentions as a fundamental agent for the emergence of communication.

Autistic Disorder↗
PubMed2000

Zuclopenthixol acetate in psychiatric emergencies: looking for evidence from clinical trials.

BACKGROUND: Case series and reviews have suggested the effectiveness of zuclopenthixol acetate in the acute management of disturbed behaviour caused by serious mental illnesses. This review investigates the trial-based evidence for these suggestions. METHODS: All randomized clinical trials comparing zuclopenthixol acetate to other 'standard' treatments for the acute management of those with serious mental illnesses were identified and, if possible, their results summated. RESULTS: Six trials were identified. All had methodological problems and one did not meet the minimal methodological inclusion criteria. The summary data do not demonstrate that zuclopenthixol acetate is better than 'standard care' for altering behaviour, decreasing the need for supplementary medication, avoiding side-effects, or postponing early discharge against medical advice. One trial, however, presented data that suggested an earlier, more intense level of sedation. CONCLUSIONS: Recommendations of reviews and open studies for the use of zuclopenthixol acetate in preference to 'standard' treatments in the psychiatric emergency are not supported by evidence from randomized controlled trials.

Adolescent↗
PubMed2000

Tel induces a G1 arrest and suppresses Ras-induced transformation.

The Tel gene is a major target of translocations in leukemia and loss of heterozygosity is regularly observed for the non-translocated allele, thus supporting the notion that Tel is a tumor suppressor. Most tumor suppressors influence cellular proliferation, differentiation and cell death and thereby prevent oncogenic transformation and genetic instability. We found that overexpression of Tel retards proliferation of many cell types, primary cells and immortalized cells, by inducing a G1 arrest. Tel's block of cellular proliferation is rescued by high seeding densities. Furthermore, Tel suppressed Ras-mediated colony growth in soft agar and tumor formation in nude mice. The Pointed and DNA binding (DB) domains of Tel were required for all Tel-induced phenotypes.

3T3 Cells↗
PubMed2000

Pol kappa: A DNA polymerase required for sister chromatid cohesion.

Establishment of cohesion between sister chromatids is coupled to replication fork passage through an unknown mechanism. Here we report that TRF4, an evolutionarily conserved gene necessary for chromosome segregation, encodes a DNA polymerase with beta-polymerase-like properties. A double mutant in the redundant homologs, TRF4 and TRF5, is unable to complete S phase, whereas a trf4 single mutant completes a presumably defective S phase that results in a failure of cohesion between the replicated sister chromatids. This suggests that TRFs are a key link in the coordination between DNA replication and sister chromatid cohesion. Trf4 and Trf5 represent the fourth class of essential nuclear DNA polymerases (designated DNA polymerase kappa) in Saccharomyces cerevisiae and probably in all eukaryotes.

Chromatids↗
PubMed2000

Smoking. Baccy to front.

The government is making 60m pounds available to develop smoking cessation services over three years. This year's funding is concentrated on health action zones where progress has been slow. The cost of nicotine replacement patches is significantly more than many smokers spend on cigarettes.

Financing, Government↗