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Biomedical subjects

D Younger

Publications and source records attributed to D Younger.

18 recordsLinked to original sources

Myopathy with tubulin-reactive crystalline inclusions.

A 61-year-old man with muscle aches and persistently elevated serum creatine kinase had aggregates of randomly oriented, rhomboidal or rectangular protein crystalline inclusions in the sarcoplasm of type II fibers. Immunochemical studies showed strong reactivity of the inclusions to tubulin antibodies, suggesting that these unique crystalline inclusions may be a consequence of altered synthesis, processing, or degradation of tubulin.

Crystallization↗

Person-centred care for people with dementia: a quality audit approach.

This paper addresses the concept of person-centred care for people with dementia by consideration of an audit process using dementia care mapping as the audit tool. It is argued that this tool is best for identifying the lived experiences of the people in receipt of care. As a result it is able to identify the overall culture of care and its level of 'person-centred' approach. The audit was conducted on 12 units, half of which were day units and the others catering for inpatients. Five patients were mapped on each day for a 4-day period. The results give some idea of the quality of care and identify where improvement is necessary. Scores such as well-being values and the Dementia Care Index give clear signposts to the level of person-centred care and highlight where staff development is necessary. Recommendations are given to aid on-going planning.

Dementia↗

Dementia care mapping: an approach to quality audit of services for people with dementia in two health districts.

The audit reported in this paper and submitted to the Psychiatry of Old Age Management group, assessed six units within each of two health districts in the UK. Using a nonparticipatory observation method in the units selected, the aim was to measure quality and the environment of care. Dependency levels of the clients/residents were also estimated to give a clearer picture of the setting and the care requirements. This was intended to establish a baseline for the units mapped and to enable care developments to be focussed upon intended outcomes. Results led to a number of observations related to the levels of interaction between staff and clients/residents, the need for a wider range of activities to promote person-centred care, and a suggested route to the improvement in quality of life for this vulnerable group of people. Assessment of dependency levels linked to the results of the mapping showed that high dependency does not lead automatically to a lower quality of person centred care.

Activities of Daily Living↗

Anti oppressive practice: a route to the empowerment of people with dementia through communication and choice.

This short study looks at the issue of anti oppressive practice and the way that it relates to the care of people with dementia. In particular it considers ways in which people can be empowered and given choices around aspects of day-to-day living in a care setting. The study took place over a period of 6 months and utilized the Dementia Care Mapping (DCM) observation process to assess the level of well being of residents in the setting studied. The results can only be seen as a first stage in the process of understanding ways in which people with dementia may be empowered. It is demonstrated that a change of practice developed over the 6 months between the two observation periods. Areas such as communications and day-to-day activities are examined to identify both problems and strategies for such change. The conclusion highlights the need to continue the mapping exercise as a way of ensuring that change is not only maintained but also advanced.

Activities of Daily Living↗

Expression of HLA-DR in peripheral nerve of amyotrophic lateral sclerosis.

To investigate the possibility of local antigen presentation within the peripheral nerve in amyotrophic lateral sclerosis (ALS), cryostat sections of 83 peripheral nerve biopsies were stained for the demonstration of HLA-DR using a monoclonal antibody. Forty samples showed increased expression of HLA-DR in endoneurium. The phenotypic characteristics of the HLA-DR positive cells are chiefly Schwann cells, using S-100 protein as a marker. We did not detect any co-expression between HLA-DR and NF (axons) and HLA-DR and myelin marker. We also detected co-expression between HLA-DR and NGFr in a majority of HLA-DR positive cells. Inflammatory cells were infrequent, being detected only in 11 cases, predominantly around epineurial blood vessels. Motor and sensory nerve biopsies performed simultaneously showed higher expression of HLA-DR in motor nerves in 2 out of 4 patients. The significance of these findings is not clear. The presence of endoneurial cells expressing HLA-DR suggests that an autoimmune mechanism may be involved in ALS having Schwann as the main target.

Adolescent↗

Familial amyloidotic polyneuropathy presenting with carpal tunnel syndrome and a new transthyretin mutation, asparagine 70.

We report familial amyloidotic polyneuropathy in a pedigree of German ancestry residing in New Jersey. Eight affected subjects presented in the third to seventh decade with carpal tunnel syndrome (CTS) and one subject presented with vitreous opacification. Transmission was autosomal dominant and survival was prolonged. Affected subjects were heterozygous for a novel mutation in serum transthyretin (TTR), resulting in an asparagine for lysine substitution at residue 70 of the TTR monomer. We report two methods for rapid identification of the mutation based on the polymerase chain reaction. This pedigree further emphasizes the evolving phenotypic and genotypic heterogeneity of the transthyretinopathies. Familial or sporadic CTS or unexplained vitreous opacification suggest the possibility of TTR amyloidosis and should prompt a search for TTR mutations.

Adult↗

Seronegative myasthenia gravis.

Of 221 patients with myasthenia gravis, 18.5% had no detectable antibodies to acetylcholine receptor. Seven of 14 patients (50%) with only ocular symptoms for more than 2 years were seronegative, and 25 of 145 (17%) patients with generalized myasthenia were seronegative. The clinical characteristics of seronegative patients did not differ from patients with high antibody titers. No seronegative patient had a thymoma, but that difference did not reach statistical significance. Lack of serum antibodies did not preclude favorable response to thymectomy or plasmapheresis.

Adult↗

Neurologic disorders and aspiration.

Many neurologic disorders impair normal swallowing. In this article, the spectrum of neurologic disease in which aspiration can be a complication is reviewed.

Central Nervous System Diseases↗

Aspects of the natural history of myasthenia gravis: crisis and death.

In an analysis of myasthenia crises during the past 20 years, the incidence of crisis remained fairly constant at 12-16%, but the fatality rate of crises declined from 42% to 6%, and the overall mortality of myasthenia declined from 12% to 3.3%. Most crisis episodes were caused by aspiration pneumonia or occurred in patients with severe dysphagia, who were at risk of aspiration. Cholinergic drug therapy clearly was not useful during crisis, and should probably be discontinued while the patient is on a respirator. Fifteen of 28 patients with thymectomy had 21 episodes of crisis months or years after surgery. Ultimately, 42% of crisis survivors achieved a state of improved myasthenia or remission after one or more crises.

Adolescent↗