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Biomedical subjects

A J Franks

Publications and source records attributed to A J Franks.

At least 19 recordsLinked to original sources

Scleroderma "en coup de sabre": pathological evidence of intracerebral inflammation.

Linear scleroderma "en coup de sabre" (LScs) is associated with neurological complications, the pathogenesis of which is uncertain. A 27 year old woman is reported on who developed epilepsy and focal neurological signs in association with LScs. Brain MRI demonstrated predominantly ipsilateral relapsing and remitting grey and white matter lesions. Analysis of CSF and pathology obtained at brain biopsy provides evidence of an inflammatory process which may be amenable to immunosuppressive treatment.

Adult↗

Temporal artery biopsy: is there any value in examining biopsies at multiple levels?

AIMS: To analyse the cost-effectiveness of three strategies for examining temporal artery biopsies based on data from cases examined over the past 10 years. METHODS: Of a total of 172 temporal artery biopsies, five were unsuitable for further analysis, 47 had already had levels cut, and 120 had levels cut as part of the study. All the biopsies were examined blind before and after levels. A tree with eventual diagnostic outcomes for different strategies was constructed and economic and sensitivity analyses performed. Welcan units were used to assess technical workload. RESULTS: Only one of the 132 initially normal cases and two of 14 diagnosed with periarterial lymphocytic infiltration (PALI) revealed giant cell arteritis after examining the tissue at multiple levels. Fifteen cases (8.9%) showed PALI not previously observed. The marginal cost for each extra case of giant cell arteritis detected was 83.5 Welcan units for a strategy of routine levels on all sections, and 21 Welcan units for a strategy of only cutting levels if PALI was present on the initial section. These costs were sensitive to the frequency of giant cell arteritis in cases with PALI and to the relative extra cost of moving from cutting single section to routine levels. CONCLUSIONS: Routinely examining a temporal artery biopsy at multiple levels does not increase the diagnostic yield of the test, although selective further examination may be indicated in some cases. The significance of PALI is uncertain. The cost-benefit of the different strategies in terms of clinical decision making revolve around the perceived risk inherent in not making a diagnosis of giant cell arteritis.

Aged↗

Meningioangiomatosis: a case report and review of the literature.

A case of cerebral meningioangiomatosis in an adult man without any stigmata of neurofibromatosis is reported. A 22 year-old man with no previous neurological history, presented with adversive seizures; clinical examination and electro-encephalogram were normal. CT showed nodular calcified masses in the left frontal lobe and in the left posterior parietal area. A craniotomy was performed and the frontal lesion was excised. Histological examination showed a predominantly intracerebral tumour involving both grey and white matter, with a complex picture of variable cellularity, dense calcification and prominent perivascular arrangements of reticulin-rich spindle cell fascicles with palisade formation widely involving the brain tissue. Immunohistochemistry for S100 protein varied between different parts of the tumour; there was focal positivity for S100 protein and smooth muscle actin in the lesion with no reaction for GFAP or EMA. Immunostaining for Factor 8-related antigen highlighted the perivascular arrangement of lesional cells and demonstrated an increase in the number of small vessels in other areas. Electron microscopy of the main mass showed elongated spindle cells with formation of pericellular basal lamina. The literature on meningioangiomatosis was reviewed. The evidence for meningeal, perivascular neural plexus or pericyte origin does not appear to be well founded. The present case further illustrates the difficulty in identifying an exact histogenetic cell and probably reflects an origin from a primitive perivascular mesenchymal cell.

Adult↗

Malignant transformation in pleomorphic xanthoastrocytoma--a report of two cases.

Pleomorphic xanthoastrocytomas (PXA) with malignant transformation are reported in two adult men with a long history of seizures, recent onset of neurological symptoms and superficially located, temporal lobe lesions. Although PXA is generally described as having relatively benign behaviour, this report adds two further cases of malignant transformation to the literature.

Adult↗

Major trauma workload within an English Health Region.

The incidence, distribution and clinical patterns of life-threatening and multiple injuries were evaluated within an English Regional Health Authority area. Cases of major injury were identified retrospectively for the 12 month period October 1988 to September 1989 using data from the 16 Accident and Emergency (A&E) units within the Yorkshire Health Region, and coroners' records. There were 968 cases of fatal and serious injury, meeting the criterion of an injury severity score greater than 15, 67 per cent (645) being due to road traffic incidents. Thirty-five per cent (337) died at the scene or before reaching hospital, whilst 65 per cent (631) survived to reach an A&E unit (0.082 per cent of the Region's annual A&E case load), 75 per cent arriving outside of normal office hours. Eleven per cent (72) died prior to ward admission and 34 per cent (213) were immediately transferred to a secondary medical referral centre. Three hundred and sixty-seven patients (38 per cent) survived to be discharged from acute hospital care whilst 188 (19 per cent) died as in-patients. Major injuries were found to be distributed throughout the Authority area in rough proportion to district population density with a regional incidence of 27 cases per 100,000. This study has quantified a group of patients with very specific and specialized needs, but further research and debate is required to decide how these needs are best met.

Accidents, Traffic↗

Health and health care of rural populations in the UK: is it better or worse?

OBJECTIVE--To review available evidence on the problems facing rural health care in the UK. In particular, to determine whether the health of rural populations is worse than that of town dwellers and how the quality of health care is influenced by rurality. CRITERIA FOR INCLUSION AND EXCLUSION OF ARTICLES--A wide variety of publications and data sources were used. A number of computerised databases with different specialisations (for example medical, health care management) were used to identify relevant published articles. In addition, reports, reviews, and surveys produced by agencies for local circulation were identified by approaching academic, service, and voluntary bodies thought likely to have an interest in rural health. Although this "grey" literature is not subject to peer review, the relative lack of relevant UK publications made it a useful data source for illustrative purposes. Similarly, published articles based on rural health in other developed countries were used when UK data were lacking. CONCLUSIONS--Although the evidence concerning the health and health care of the UK rural populations is suggestive, it is very general and further research is needed. Levels of urban health seem to be generally worse than in rural areas, but contradictions do exist. The evidence on quality of care suggests that service accessibility is a central problem, and rural populations have poorer access than others. Within rural populations, such disadvantage is not uniformly experienced--it affects some groups more than others. In addition, the NHS does not seem to have a consistent policy about whether rurality should influence resource allocation, and how it should be incorporated.

Health Care Rationing↗

Extracorporeal photochemotherapy in progressive systemic sclerosis.

BACKGROUND: Extracorporeal photochemotherapy, an immune-modulating form of therapy, has been shown to be effective in the treatment of autoimmune diseases. We evaluated the effects of extracorporeal photochemotherapy in the treatment of patients with progressive systemic sclerosis (PSS). METHODS: Nine patients with active progressive systemic sclerosis were treated with extracorporeal photochemotherapy on 2 successive days monthly. The duration of therapy ranged from 6 to 21 months. RESULTS: A significant improvement was noted in the skin, musculoskeletal system, functional index, and symptoms including Raynaud's phenomenon, dyspnea, fatigue, dysphagia, and arthralgias, as well as improvement of cutaneous ulcers. Stabilization of the pulmonary function studies was also noted in the majority of patients over the course of therapy. No serious side effects were noted throughout the course of therapy in the 9 patients. CONCLUSIONS: The results suggest that photopheresis may be beneficial in selected early cases of progressive systemic sclerosis.

Activities of Daily Living↗

Audit and research.

Explore the source record for details and available documents.

Management Audit↗

Mast cells, nerves and fibrosis in the appendix: a morphological assessment.

Mast cells are closely associated with nerves in the mucosa of the appendix vermiformis, and obliteration of the appendiceal lumen by fibrous tissue is accompanied by neurogenous hyperplasia. However, changes in the density of mast cells in this process have not been reported. Accordingly, fibrosis was graded in haematoxylin and eosin sections from 46 samples of human appendix. This was compared with mast cell number in toluidine blue-stained slides and nerve density in PGP9.5-immunoreactive sections. In the mucosa, the mast cell number in the samples with minimal fibrosis was three times greater than in those classified as normal (P less than 0.0001), and this declined in the more fibrotic samples. The mucosal nerve scores paralleled the mucosal mast cell changes, and stereological analysis revealed a correlation of mast cell number and nerve density within the lamina propria of the same specimens (r = 0.49-0.90). In the submucosa, mast cell numbers and nerve scores were not significantly different in the different histological grades and obliterated samples resembled normal submucosa, except that a dense axial block of nerve staining was often present. The progressive fibrotic changes in appendices provide a human model for studying the relationships of nerves, mast cells, and fibrosis in the gastrointestinal tract.

Appendix↗

Development of the cerebellum with particular reference to cellular differentiation in the external granular layer.

Immunocytochemical evidence of differentiation in developing human cerebellum is presented in this study. Antibodies to neuron specific enolase, neurofilament protein, glial fibrillary acidic protein, vimentin, cytokeratin, epithelial membrane antigen and lymphoid markers, DLC and Leu 7 were used. The external granular layer showed positivity with neuronal markers between 27 weeks gestation and 4 months postnatal, but was negative for all other markers including glial fibrillary acidic protein. Characteristic staining reactions were noted in the other cerebellar layers. Monoclonal antibodies, UJ13A (pan-neuroectodermal marker) and G10 (localising microtubule-associated protein MAP1x) were also used in a limited number of cryostat sections and were positive and negative, respectively, in the external granular layer. The results of this study are discussed in relation to the theory that the external granular layer may be one source of medulloblastomas.

Biomarkers↗

Immunohistochemical localisation of tissue plasminogen activator in human brain tumours.

The distribution of tissue plasminogen activator (t-PA) has been studied in a series of 38 human brain tumours and two specimens of cerebral cortex, using the monoclonal antibody ESP6. t-PA was localised in vascular endothelium in the majority of tumours and both the cortical specimens, confirmed by double staining with Ulex europaeus lectin (Uel) and Factor 8-related antigen. Nineteen out of 22 high grade astrocytomas showed strong endothelial staining whereas staining was weak or absent in the four low grade astrocytomas studied. No consistent relationship was found between the pattern of staining and tumour grade in the other tumours, although strong staining of the three metastatic lesions with Uel was observed. Among the astroglial tumours only one glioblastoma showed any tumour cell staining for t-PA, which raises questions concerning the origin of t-PA producing cells derived from human gliomas in vitro. Studies of t-PA in brain tumours should take account of this vascular localisation before concluding that the activity is derived from neoplastic cells.

Brain Neoplasms↗

Status epilepticus complicating migraine.

We describe 2 patients who developed status epilepticus in association with migraine attacks. One patient died and at the autopsy had ischaemic changes confined to the occipital lobes. The second patient made an uneventful recovery.

Adult↗