[Unilateral segmental keratotic papules].
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Biomedical subjects
Publications and source records attributed to L Weber.
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Lichen amyloidosis (LA) is a chronic, pruritic skin disorder characterized by brownish-grey papules on extensor surfaces of legs and rarely on the trunk. Thioflavin T-positive amyloid deposits are found in the papillary dermis of affected skin, which is the only organ involved. A variety of therapeutic regimens for lichen amyloidosis have been described; however, in many cases with only limited effect. We report on two patients with lichen amyloidosis with typical clinical symptoms not responding to local treatment. A combined regimen with bath psoralen ultraviolet A (PUVA) and oral acitretin was initiated, resulting in nearly complete resolution of the papules and impressive relief from the severe pruritus. The beneficial response has persisted for 8 months. The suggested combined therapy with bath PUVA photochemotherapy and oral acitretin represents an efficacious and practical treatment modality for lichen amyloidosis with long-lasting effects.
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An 80-year-old man presented with a distinctive reticular form of elastolytic giant cell granuloma (EGCG). This represents a rare subset of EGCG, thus belonging to a larger group of granulomatous skin diseases with poorly understood pathogenesis. The patient exhibited numerous erythematous to brownish, sharply demarcated patches of reticulate appearance with partial central atrophy and nonelevated margins, which involved the trunk and especially chest, shoulders, upper back and arms. Skin biopsy revealed a granulomatous infiltrate with multinucleate histiocytes containing remnants of elastic fibres in conjunction with a significant loss of elastic fibres throughout the dermis. There is little published information on treatment, which remains unsatisfactory.
Lung transplant recipients exhibit a high incidence of invasive aspergillosis. The inhalation of lipid complex amphotericin-B (Abelcet; ABLC) offers a possible prophylactic strategy. The goals of this study were to select the optimal nebulizer delivery system for ABLC and to measure deposited aerosol dose in 12 lung transplant recipients. In vitro testing was performed to select a nebulizer delivery system, and an empirical model was used to estimate lung deposition. Estimated pulmonary doses varied by as much as 2-fold between different nebulizers. Aerosol deposition testing was performed in six single and six double lung recipients, each of whom received one 7 mL (35 mg) nebulized dose of Technetium-labeled ABLC using the selected nebulizer. In single lung recipients, the average deposited doses were 3.9 +/- 1.6 mg (mean +/- S.D.) in the allograft versus 2.1 +/- 1.1 mg in the native lung. Double lung recipients deposited on average 2.8 +/- 0.8 mg (left lung) and 4.0 +/- 1.3 mg (right lung). The drug was well distributed throughout the lungs, but delivery to the native lung was in some cases suboptimal. These studies provide an important precursor to studies of the efficacy of inhaled ABLC as a prophylaxis of invasive aspergillosis after lung transplant.
Primary aorto-oesophageal fistula, secondary to an aneurysm of the thoracic aorta, are almost fatal. In the literature, only twenty six successfully operated cases have been reported. We report the case of a 78-year-old man with a thoracic aortic aneurysm eroded into the mid oesophagus. Prompt diagnosis of an aorto-oesophageal fistula resulted from clinical history, CT-imaging and oesophagoscopy. The patient was successfully operated by exclusion of the thoracic aneurysm (insertion of a straight cryopreserved arterial allograft), oesophagectomy and cervical oesophagostomy and jejunostomy. The continuity of the digestive tube was later restored after preliminary aortic valve remplacement (stenosis of 0.8 cm2). This case report is the second in which a cryopreserved allograft was successfully implanted in the management of a primary aorto-oesophageal fistula.
The authors describe four unrelated girls with a distinctive neurologic disorder with early-onset progressive ataxia and hypodontia with a characteristic pattern of delayed dentition. Cerebral MRI shows hypomyelinated white matter and cerebellar atrophy; 1H-MRS of white matter reveals a marked elevation of myo-inositol.
Lake trout fry (Salvelinus namaycush) were exposed in laboratory experiments to two wastewater treatment polymers, one anionic (MagnaFloc 156) and one cationic (MagnaFloc 368; Ciba Speciality Chemical), to determine if these chemicals which are used and discharged by mining operations in Canada's North pose a significant hazard to juvenile fishes. The cationic polymer was substantially more toxic to lake trout fry than the anionic polymer, with 96-h LC50 estimates of 2.08 and >600 mg/l, respectively. Separate 30-d exposure experiments yielded no observed and lowest observed effect concentrations, respectively, of 0.25 and 0.5mg/l for MagnaFloc 368, and 75 and 150 mg/l for MagnaFloc 156. In both cases, behavioural responses, especially startle response, were the most sensitive test endpoints. Histopathological assessment revealed that gill pathology appeared within a few days of exposure to both polymers, apparently as a result of localized hypoxia. Acute (4 d) effects included cloudy swelling of epithelial cells, increased gill vascularization, and thickening and shortening of the gill lamella. Chronic (30 d) polymer exposure produced only slightly greater pathological effects than acute exposure, with comparable responses observed only at >1.0mg/l MagnaFloc 368 and 150 mg/l MagnaFloc 156, suggesting that the fish displayed some level of both behavioural and physiological adaptation to the respiratory stress imposed by the two polymers.
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We report the rare case of a patient with leukaemia cutis first presenting only on the hand and fingers and then subsequently spreading over the trunk and face. The lesions heralded the transformation of a previously undiagnosed myelodysplastic syndrome type RAEB (refractory anaemia with blast excess) into frank myeloid leukaemia. The haematological disease was first detected by the dermatohistopathologist. This case underlines the need to look meticulously for skin changes and perform early skin biopsies in haematological patients, as the skin can reveal the first clinical signs of an otherwise not evident bone marrow disorder. Leukaemia cutis as the initial clinical presentation of a transforming myelodysplastic syndrome type RAEB into acute myeloid leukaemia has been reported only very rarely.
An extensive set of benchmark measurement of PDDs and beam profiles was performed in a heterogeneous layer phantom, including a lung equivalent heterogeneity, by means of several detectors and compared against the predicted dose values by different calculation algorithms in two treatment planning systems. PDDs were measured with TLDs, plane parallel and cylindrical ionization chambers and beam profiles with films. Additionally, Monte Carlo simulations by means of the PENELOPE code were performed. Four different field sizes (10 x 10, 5 x 5, 2 x 2, and 1 x 1 cm2) and two lung equivalent materials (CIRS, p(w)e=0.195 and St. Bartholomew Hospital, London, p(w)e=0.244-0.322) were studied. The performance of four correction-based algorithms and one based on convolution-superposition was analyzed. The correction-based algorithms were the Batho, the Modified Batho, and the Equivalent TAR implemented in the Cadplan (Varian) treatment planning system and the TMS Pencil Beam from the Helax-TMS (Nucletron) treatment planning system. The convolution-superposition algorithm was the Collapsed Cone implemented in the Helax-TMS. The only studied calculation methods that correlated successfully with the measured values with a 2% average inside all media were the Collapsed Cone and the Monte Carlo simulation. The biggest difference between the predicted and the delivered dose in the beam axis was found for the EqTAR algorithm inside the CIRS lung equivalent material in a 2 x 2 cm2 18 MV x-ray beam. In these conditions, average and maximum difference against the TLD measurements were 32% and 39%, respectively. In the water equivalent part of the phantom every algorithm correctly predicted the dose (within 2%) everywhere except very close to the interfaces where differences up to 24% were found for 2 x 2 cm2 18 MV photon beams. Consistent values were found between the reference detector (ionization chamber in water and TLD in lung) and Monte Carlo simulations, yielding minimal differences (0.4%+/-1.2%). The penumbra broadening effect in low density media was not predicted by any of the correction-based algorithms, and the only one that matched the experimental values and the Monte Carlo simulations within the estimated uncertainties was the Collapsed Cone Algorithm.
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Common ivy (Hedera helix L.) is a ubiquitous plant in Europe whose major allergen falcarinol has moderate allergic potential. It is not related to poison ivy (Toxicodendron spp.). There are no cross reactions between the allergens of common ivy (falcarinol) and poison ivy (urushiol). Contact with common ivy or falcarinol may lead to sensitization and then a delayed hypersensitivity reaction. There are only few cases described in the literature. We report on a male hobby gardener with appropriate clinical history and positive patch test. The pathogenic mechanism is a type IV reaction following a sensitization exposure. Gardeners and landscape architects with frequent exposure to common ivy and thus a high risk of sensitization should wear appropriate protective clothing.
A method is presented for studying fibre damage in continuous fibre reinforced composites. It is based on contrasting the transmission of light through intact translucent fibres with the light through fractured or dead-ended fibres. The method is applied in order to detect processing-induced fibre fractures in aluminium reinforced with continuous alumina fibres.
We determined whether digital photographs of skin lesions could enhance diagnostic accuracy in dermatohistological evaluations. Two dermatohistopathologists examined 375 unsorted consecutive cases. On a standardized questionnaire they recorded whether the final diagnostic interpretation would be improved by the availability of digital images of the skin lesions. In 101 cases (27%) they said that digital photographs would be helpful. Subsequently, 30 histological analyses were performed with and without digital photographs of the skin lesions. Presentation of digital photographs reduced the number of differential diagnoses significantly, from a median of 3 to 2. Ratings of ability to make a single definitive diagnosis increased significantly with the presentation of digital photographs. Enhancement of information given by the digital images was scored a median of 6 (on a scale of 0-10, with higher scores reflecting greater enhancement). Digital photographs of skin lesions are likely to refine diagnostic accuracy in histopathology.
Nocardia species is a well-known pathogen in immunocompromised hosts, including renal transplant recipients. Primary pulmonary infection can disseminate to other organs and recommended first-line therapy is high-dose trimethoprim/sulfamethoxazole (TMP/SMX). We report two cases of primary pulmonary Nocardia sp. in immunosuppressed patients who were treated with minocycline, a second-line drug. During treatment with minocycline, both patients developed central nervous system (CNS) lesions of Nocardia sp. and were then treated with TMP/SMX with resolution of disease. The literature on Nocardia and treatment with minocycline is reviewed. Treatment of pulmonary Nocardia sp. with 200 mg minocycline daily is not adequate to prevent disseminated CNS disease.