Bilateral ptosis and gaze palsies following radioactive seed treatment of tectal plate tumours.
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Biomedical subjects
Publications and source records attributed to J Ashworth.
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OBJECTIVE: To evaluate whether the traction force indicator in a ventouse device gave an accurate estimation of the force applied. METHODS: The study was carried out at the Derby City Hospital. An estimate of measurement errors was made by analyzing the standard deviation of the residuals for 30 Kiwi OmniCup devices (Clinical Innovations, Abingdon, Oxfordshire, England) and a standard industrial spring balance. RESULTS: The measurement errors for the traction forces were very small and acceptable for the OmniCup. The overall estimate of measurement errors was 0.45 kg (1.35 lb). CONCLUSION: The overall measurement error for the traction force indicator of the OmniCup was found to be very small. Injuries associated the ventouse, such as cephalohematomas and subgaleal hemorrhages, may be minimized if the recommended limits for a safe traction force are not exceeded.
Linear epidermal naevus (LEN) in the genital area is quite rare. It may present at birth or appear later on in life, in infancy or childhood and occasionally for the first time in adult life. There are several variants of epidermal naevi (EN), which, to the less experienced, can be mistaken for warts. When extensive, it can be associated with abnormalities in other organ systems (epidermal naevus syndrome). The definitive treatment of LEN is surgical ablation with excision of underlying dermis, but this frequently leads to scarring. Laser therapy is an alternative treatment modality and good results have been shown. We report an unusual case of LEN in the genital area in a 60-year-old man presenting as genital warts.
Vohwinkel syndrome (VS) is a family of genodermatoses which exhibits extensive clinical and genetic heterogeneity. Here, we studied a pedigree originating from the UK with typical features of the ichthyotic variant of VS and identified a recurrent insertion mutation in the loricrin gene resulting in a mutant polypeptide with an unusual C terminus. Functional studies in transgenic mice have shown that the accumulation of mutant loricrin in the nucleus appears to interfere with the later stages of epidermal differentiation, thereby explaining the clinical manifestations of ichthyosis, keratoderma and pseudoainhum. Our findings extend the body of evidence implicating mutations in the loricrin gene as the underlying cause of VS.
Fibrillin-rich microfibrils are a unique class of extensible connective tissue macromolecules. Their critical contribution to the establishment and maintenance of diverse extracellular matrices was underlined by the linkage of their principal structural component fibrillin to Marfan syndrome, a heritable connective tissue disorder with pleiotropic manifestations. Microscopy and preparative techniques have contributed substantially to the understanding of microfibril structure and function. The supramolecular organisation of microfibrillar assemblies in tissues has been examined by tissue sectioning and X-ray diffraction methods. Published findings are discussed and new information reported on the organisation of microfibrils in the ciliary zonular fibrils by environmental scanning electron microscopy. This review summarises microscopy and X-ray diffraction studies that are informing current understanding of the ultrastructure of fibrillin-rich microfibrils.
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BACKGROUND: Chronic idiopathic urticaria is a disabling condition that does not always respond to antihistamine drugs and other agents are sometimes needed to control disease activity. Warfarin has demonstrated efficacy in single unblinded case studies [1] but has been dismissed by others [2]. OBJECTIVE: We investigated the effect of warfarin treatment in eight patients with chronic idiopathic urticaria unresponsive to antihistamines in an open study. Six of the eight patients responded to treatment and three had a dramatic response. These three were included in a double-blind placebo-controlled trial of warfarin therapy to confirm significant benefit from treatment. METHODS: The three warfarin responders had their stable warfarin dose encapsulated and placebo capsules were provided. A double-blind placebo-controlled crossover trial was performed on each patient. Visual analogue scores recorded disease activity. RESULTS: Comparison of visual analogue scores showed a significant benefit while on warfarin with a reduction in pruritus and angio-oedema. CONCLUSION: This is the first double-blind placebo-controlled study to show a response of chronic idiopathic urticaria to warfarin. The mechanisms of action are unclear and require further study.
Microfibrils are ubiquitous fibrillin-rich polymers that are thought to provide long-range elasticity to extracellular matrices, including the zonular filaments of mammalian eyes. X-ray diffraction of hydrated bovine zonular filaments demonstrated meridional diffraction peaks indexing on a fundamental axial periodicity (D) of approximately 56 nm. A Ca2+-induced reversible change in the intensities of the meridional Bragg peaks indicated that supramolecular rearrangements occurred in response to altered concentrations of free Ca2+. In the presence of Ca2+, the dominant diffracting subspecies were microfibrils aligned in an axial 0.33-D stagger. The removal of Ca2+ caused an enhanced regularity in molecular spacing of individual microfibrils, and the contribution from microfibrils not involved in staggered arrays became more dominant. Scanning transmission electron microscopy of isolated microfibrils revealed that Ca2+ removal or addition caused significant, reversible changes in microfibril mass distribution and periodicity. These results were consistent with evidence from x-ray diffraction. Simulated meridional x-ray diffraction profiles and analyses of isolated Ca2+-containing, staggered microfibrillar arrays were used to interpret the effects of Ca2+. These observations highlight the importance of Ca2+ to microfibrils and microfibrillar arrays in vivo.
PURPOSE: Mohs micrographic surgery has been advocated as the optimal management of nonmelanoma skin cancer in the periocular region. It is a technique that is ideally suited to the removal of skin tumours with a contiguous growth pattern such as basal cell carcinoma and squamous cell carcinoma, allowing examination of 100% of the surgical margin. As a result of this total margin control, the technique offers an unsurpassed curve rate combined with maximal preservation of normal tissue. Following excision of a periocular tumour by a Mohs surgeon, the resulting defect usually requires reconstruction. Our objective was to determine whether the size of defect produced by Mohs surgery and the type of reconstruction required differed from the results we would have expected from standard surgery with a 3 mm excision margin. METHODS: A Mohs surgery service with a combined dermatological and oculoplastic approach was set up in Manchester in 1994. We reviewed 60 of our patients who underwent Mohs surgery and compared the size of defect produced as well as the type of reconstruction required with the results we would have predicted for standard excisional surgery with a 3 mm margin. RESULTS: Although a minority of patients required larger reconstructions than would have been anticipated (20%), many had smaller reconstructions than we had predicted (37%). This latter group often had important structures preserved, and therefore had the benefit of less extensive reconstructive surgery. CONCLUSIONS: Excision of a periocular tumour by Mohs surgery may occasionally identify extensive subclinical tumour extension and so produce an unexpectedly large defect for reconstruction. Many patients, however, require less extensive reconstructive surgery than would have been predicted. This produces benefits in terms not only of improved cosmesis and eyelid function, but also reduced operating theatre costs.
PURPOSE: The hydroxyapatite orbital implant has been introduced as a buried, integrated implant for use in the anophthalmic patient. The second stage of the procedure involves drilling the implant and inserting a motility peg that allows direct coupling of the artificial eye to the implant. Other authors have advocated an assessment of implant vascularity by imaging prior to drilling. We aimed to see whether our practice of drilling after a predetermined time interval without assessment of implant vascularity would result in a higher complication rate. We also aimed to determine how successful the drilling procedure was in improving both the motility and the stability of the artificial eye. METHODS: The notes of 41 consecutive patients who underwent drilling were studied to determine the time interval between implantation with a hydroxyapatite sphere and drilling, the nature of any complications and any further surgical procedures undertaken. A postal questionnaire was sent to all patients asking them to grade the motility of their implant before and after drilling, and to state whether or not there had been any improvement in the stability of their artificial eye. RESULTS: The most frequent complication encountered was extrusion of the motility peg, which occurred in 3 patients. A total of 5 patients required redrilling. There was an 80% response rate to the questionnaire. Ninety-one per cent of patients felt that there had been an improvement in the motility of their artificial eye and 76% felt that the stability of their artificial eye had been improved. CONCLUSION: Pegging of the hydroxyapatite implant improves both the motility and the stability of the artificial eye in the majority of cases. Complications are infrequent and minor. If sufficient time is allowed after implantation for full implant vascularity to occur, it is not necessary to perform imaging studies. This practice does not result in an increase in complications, and significantly reduces the expense of the procedure.
UNLABELLED: Desflurane is a potentially useful anesthetic for ambulatory surgery, but it has had limited evaluation in spontaneously breathing patients. After the induction of anesthesia with propofol and laryngeal mask insertion, 90 patients were randomized to receive isoflurane (0.25%-1%), propofol (50-200 microg x kg(-1) x min(-1)), or desflurane (1.4%-6%) for anesthetic maintenance. Respiratory complications were uncommon; only six patients coughed (three who received isoflurane, one who received propofol, and two who received desflurane), and no anesthetic produced significant respiratory depression. Purposeful movement was significantly more common with propofol (19 patients; 63%) compared with isoflurane (7 patients; 23%) or desflurane (2 patients; 6.7%), but no patient had recall. Emergence times were similar in the isoflurane, propofol, and desflurane groups (5.1 +/- 2.3, 5.6 +/- 3.1, and 4.4 +/- 1.4 min, respectively). Later recovery end points and pain and sedation visual analog scale scores did not differ among groups. Overall, 85 patients (94%) were free from postoperative nausea and vomiting. Desflurane produced few respiratory complications in spontaneously breathing ambulatory patients but offered no improvement in emergence or recovery compared with isoflurane. Propofol also did not reduce recovery times or side effects; however, it was more difficult to maintain an adequate depth of anesthesia. We conclude that neither desflurane nor propofol offered any major advantages over the older anesthetic, isoflurane, under the conditions of our study. IMPLICATIONS: The new inhaled anesthetic desflurane is acceptable in spontaneously breathing outpatients despite its known ability to irritate the airway. The i.v. anesthetic propofol was associated with more patient movement (without awareness) during surgery. Neither anesthetic conferred any clinically significant advantages over the older inhaled drug, isoflurane.
Twenty-one node-negative breast cancer patients were interviewed shortly after completing adjuvant chemotherapy and asked about side effects they had experienced, expectation of side effects, and strategies for coping with the side effects. Eighteen of the women were interviewed 6 months later to determine their feelings about the chemotherapy experience and ending treatment and what side effects persisted or developed after chemotherapy. Hair loss, fatigue, treatment-related problems, nausea and infections/low blood counts were the most frequently described problems during the first interviews. Patients used coping strategies suggested by physicians and nurses. Six months later, hair problems, fatigue, weight gain, menopausal problems, emotional problems and nail problems were most often reported. Most patients (16/18) did not expect to be experiencing chemotherapy-related problems 6 months after ending treatment. Fatigue interfered with daily lives and weight gain caused concern. A total of 35% of participants experienced fear or anxiety at the end of chemotherapy, but most (62%) recalled at least some positive feelings 6 months later. Given the same circumstances, all but two would make the same decision to undergo adjuvant chemotherapy. Support groups would be especially useful for patients completing chemotherapy who would lose continued frequent support from clinic personnel.
The use of amino acid dialysate (AAD) has been shown to improve the nutritional status of malnourished continuous ambulatory peritoneal dialysis (CAPD) patients. We report on a randomized, prospective, cross-over study evaluating the effects of a single, daily, postprandial 2-L exchange of 1.1% AAD (Nutrineal) on a nutritionally unselected group of 18 stable CAPD patients. Patients in group A (n = 10) were randomized to receive AAD in the initial six months, whereas group B (n = 8) patients received AAD in the final six months of the study. Regular biochemical, hematological, and anthropometric measurements were made. A computerized nutrition score(1) combined anthropometry, serum albumin, and total lymphocyte count. Improved nutritional status was indicated by a decreased score. Mean serum albumin and transferrin did not show a significant rise in either group. However, patients in group A, with a mean serum albumin of less than 30 g/L, showed a significant rise at two months, which persisted at six months (26.8 g/L on entry, 29.0 g/L at two months, 30.1 g/L at six months; p < 0.05 and p < 0.01, respectively). Treatment with AAD showed a trend towards improvement in midarm muscle circumference in both groups (22.9 -23.5 cm, group A; 22.9-23.7 cm, group B). The nutrition score improved in both groups but was significant only in group A (14.6 to 13.1; p = 0.02). These effects of AAD on the nutritional status of CAPD patients need validation in a long-term study to evaluate the effects on morbidity and mortality.
Facial inflammatory dermatoses can be difficult to diagnose in their early stages; skin biopsy may help but often shows indeterminate features. The aims of our study were to identify patients presenting with a facial dermatosis requiring skin biopsy, in whom the initial histological features were classified as "non-specific", to determine the eventual diagnostic outcome, and to attempt to identify subtle early histologic features. We located 79 such cases with indeterminate histology from 45,000 dermatopathology reports 1972-1987. The original biopsies were assessed and scored using a check-list of 20 features and the patients' case-records up to 1992 were examined. Follow-up information was available in 64 cases and an eventual clinical diagnosis was made in 54 (84%) of these; the commonest diagnoses were rosacea (26%), chronic discoid lupus erythematosus (24%) and seborrheic or unclassified dermatitis (17%). Histological differences were seen in the original biopsies of these three diagnostic categories, although none was predictive. We conclude that clinical follow-up leads to the diagnosis of most facial rashes initially showing "non-specific" histology.
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A detailed survey of skin complaints amongst 114 airline employees working in a new warehouse revealed 26 cases of skin problems which originated during the 2 1/2 year operation. A clinical survey of broadly the same population confirmed 14 cases from 98 employees as chronic irritant contact dermatitis of the hands. The work involved the reception, unpackaging, inspection, repackaging and dispatch of aircraft parts. The source of the skin irritation was not to be found in the work itself. Rather, the presence among the employees of two severe cases of non-occupational eczema, combined with the idea that incoming aircraft parts from foreign countries might be 'dirty' in some way, had caused a heightened perception of a risk of skin disease, and the frequency of hand washing had increased as a result. Over-frequent hand washing in a few employees had resulted in precisely what the warehouse staff had been trying to avoid.