Surgery on the Internet. Part 2: The World Wide Web.
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Biomedical subjects
Publications and source records attributed to A Richards.
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We present a case of rupture and herniation of cartilaginous tracheal rings into the lumen of the trachea that was noticed as an incidental finding during bronchoscopy after percutaneous dilatational tracheostomy. While percutaneous methods of tracheostomy formation gain popularity in intensive care settings, the number of reported problems associated with this technique continue to grow. We propose that fibreoptic bronchoscopy used routinely with percutaneous tracheostomy formation will identify a number of complications involving the tracheal skeleton.
We previously localised the gene (LAMA4) encoding a novel laminin alpha 4 chain to chromosome 6q21. In this study, we describe the complete coding sequence and compare the protein with the other three known human laminin alpha chains. Although closely linked to LAMA2, the LAMA4 product most closely resembles laminin alpha 3, a constituent of laminin 5. Like laminin alpha 3A, the alpha 4 chain is a truncated version of the alpha 1 and alpha 2 chains, with a much reduced short arm. While the alpha 4 molecule is most similar to alpha 3, it shares some features of the C-terminal domains G4 and G5 in common with alpha 2. Unlike the LAMA3 gene, LAMA4 appears to encode only a single transcript, as determined by 5' rapid amplification of cDNA ends. The cDNA sequence encodes 1816 amino acids, which include a 24-residue signal peptide. The gene is expressed in skin, placenta, heart, lung, skeletal muscle, and pancreas. We have also shown that the mRNA can be readily reverse transcribed and amplified from cultured dermal fibroblasts.
A polymerase chain reaction (PCR) method was developed for detecting measles virus (MV) RNA in a variety of clinical samples using primer pairs in the nucleocapsid (N) and matrix (M) genes in one reaction (dual target-PCR). The dual target-PCR detected MV RNA in tissue culture fluid containing 1 TCID50 of the MV Loss strain, and was as sensitive as a single target-PCR. Specificity was confirmed by the failure of the dual target-PCR to amplify products from the infected tissue culture containing related paramyxoviruses. Thirty-two of 35 (91.4%) samples collected from 23 patients with confirmed measles infection by the detection of measles specific IgM were found to be positive for MV RNA by PCR. Direct sequencing of the PCR amplicons revealed three different genotypes among the MV strains that were detected in 12 patients. The dual target-PCR method is suitable for the diagnosis of measles infection and, based on the sequencing of the PCR product DNA, for investigating the molecular epidemiology of MV strains.
Craniometaphyseal and craniodiaphyseal dysplasia are rare genetic disorders of bone due to modelling errors of long bones and skull bones. These syndromes present with multiple ENT symptomatology from an early age. The diagnostic distinction can now be made radiologically by serial skeletal survey which is important for prognosis. We review the clinical, radiological, computed tomography (CT) scan, otological, audiological and histopathological findings in two cases with craniodiaphyseal, and two cases with craniometaphyseal dysplasia, and report our experiences of medical and surgical treatment to date. In the craniodiaphyseal dysplasia, the hearing abnormality progressed from an initial conductive to a mixed loss on serial audiometric follow up. Temporal bone CT scans showed narrowing of the middle ear cavity, internal auditory meatus, and facial nerve canal at the geniculate ganglion. Benefits from choanal stenosis surgery, craniofacial remodelling and dacrocystorhinostomy were shortlived. Calcitriol therapy with a low calcium diet did not alter the clinical course of progression in our cases. The underlying defect, causing net bone formation in these phenotypically similar syndromes, appears to be different when based on the differing biochemical responses to calcitriol and bone biopsy findings. Increased numbers of osteoblasts were found in bone biopsies from both cases with craniodiaphyseal dysplasia. Early recognition is crucial in these conditions as therapy directed at the underlying bony defect has the best chance of success if initiated in infancy.
An experiment was conducted to examine processing strategy differences in anxiety. The sentence-picture verification task was modified to incorporate a block of threat-related trials (involving a knife and a rifle) and a block of neutral trials (involving a truck and a chair). It was predicted that the high-trait, relative to low-trait, anxious individuals would prefer to use a linguistic strategy for threat-related as compared to neutral trials. In addition, the idea that an image-based strategy is more important for processing emotional material was examined. There was some support for both of these hypotheses.
Two experiments examined the functional equivalence of memory in the rat (Rattus norvegicus) with memory in humans for serially presented items. Memory was assayed with an 8-arm radial maze, in which rats were allowed access to 5 arms of the maze and were then removed. Following a retention interval of 16 min, the rats were replaced in the maze and allowed to retrieve pellets from the 3 unvisited arms. The errors in reentering previously visited arms were noted. Both primacy and recency effects were found as with humans. Presenting a stimulus change after entry to 1 of the maze arms improved recall for that arm relative to when no change occurred. This effect was found using both handling and tone cues, and irrespective of whether the change consisted of presentation or nonpresentation of the cue. These results suggest that rats are subject to a von Restorff-like effect similar to that in humans.
French and Richards (1993) found that subjects asked to draw from memory a clock that had Roman numerals on its face typically represented the number four on the clock face as "IV" rather than the correct "IIII", whereas those merely asked to copy it typically drew "IIII". The current experiments followed the methodology of French and Richards, but then went on to examine the subsequent memorial representation of the number four. Subjects drew a clock with Roman numerals on its face, either from memory (with or without forewarning) or while the clock remained in full view. Subsequently, subjects were asked to recall the exact form in which the numbers were represented on the clock (Experiment 1) or were asked to recognise which of two clocks had been presented earlier (Experiment 2). Findings supported the idea that subjects in the copy condition were more likely than subjects in other conditions to draw the clock without invoking schematic knowledge of Roman numerals. The basic effect reported by French and Richards was replicated in both experiments. Furthermore, in both experiments, subjects who correctly drew the clock with the number four represented as "IIII" were more likely to misrepresent it as "IV" in the subsequent memory task if they were in the copy condition rather than the two memory conditions. The results are interpreted in terms of schema theory.
The Ehlers-Danlos syndrome (EDS) is a heterogeneous group of inherited connective tissue disorders in which cutaneous fragility and ligamentous laxity often combine with vascular, gastrointestinal, and skeletal deformities. There is considerable phenotypic overlap between the more common forms of EDS (types I and II), in which specific molecular defects have not yet been identified. Recently, genetic linkage has been demonstrated between the COL5A1 gene, which encodes the alphal chain of type V collagen, and EDS type II in a large British kindred. Using a polymorphic intragenic simple sequence repeat at the COL5A1 locus, we now demonstrate tight linkage to EDS type I/II in a three-generation family, giving a LOD score (log10 of the odds for linkage) of 4.07 at zero recombination. The variation in expression in this family suggests that EDS types I and II are allelic, and the linkage data support the hypothesis that mutation in COL5A1 can cause both phenotypes.
Bentall & Thompson (1990) selected participants on the basis of high, medium and low scores on the Hypomanic Personality Scale. In an emotional Stroop test, the high hypomanic participants showed interference of colour naming for depression-related but not euphoria-related words. The current study tests the hypothesis that the effects found were mediated by anxiety, and not hypomania as claimed. Bentall & Thompson's study was repeated but measures of state of trait anxiety were also taken. When analysis was restricted to three subgroups selected in the same manner as in Bentall & Thompson's (1990) study, the findings were very similar to those found in that study and the hypomania-related interference effects were found to occur even when anxiety levels were taken into account. Further analyses of data from the full sample of participants supported the idea that hypomanic personality might reflect a partially successful means of coping with depressive tendencies.
OBJECTIVE: To assess the recently proposed preliminary criteria for the classification of Sjögren's syndrome (SS) in a multicentre European study of a new series of clinically defined cases. METHODS: The criteria included six items: I = ocular symptoms; II = oral symptoms; III = evidence of keratoconjunctivitis sicca; IV = focal sialoadenitis by minor salivary gland biopsy; V = instrumental evidence of salivary gland involvement; VI = presence of autoantibodies. Each centre was asked to provide five patients with primary SS, five with secondary SS, five with connective tissue diseases (CTD) but without SS, and five controls (patients with ocular or oral features that may simulate SS). The preliminary six item classification criteria set was applied to both the SS patients and the non-SS controls, and the performance of the criteria in terms of sensitivity and specificity was tested. RESULTS: The criteria set was tested on a total of 278 cases (157 SS patients and 121 non-SS controls) collected from 16 centres in 10 countries. At least four of the six items in the criteria set (limiting item VI to the presence of Ro(SS-A) or La(SS-B) antibodies) were present in 79 of 81 patients initially classified as having primary SS (sensitivity 97.5%), but in only seven of 121 non-SS controls (specificity 94.2%). When the presence of item I or II plus any two of items III-V of the criteria set was considered as indicative of secondary SS, 97.3% (71 of 73) of the patients initially defined as having this disorder and 91.8% (45 of 49) of the control patients with CTD without SS were correctly classified. CONCLUSION: This prospective study confirmed the high validity and reliability of the classification criteria for SS recently proposed by the European Community Study Group.
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In order to evaluate the effect of sodium fluoride (NaF) on bone biomechanical competence, iliac crest biopsies were taken before and after one year of treatment in 12 osteoporotic patients, and before and after five years of treatment in 14 patients. Bone fluoride content had increased significantly after both one and five years of treatment, indicating that the administered fluoride had been ingested. After one year of treatment, no difference was observed in iliac crest trabecular bone ash content. A general trend for decreased bone strength and bone quality was observed, but this was insignificant. After five years of fluoride treatment, an insignificant decrease in iliac crest trabecular bone ash content was observed. A significant reduction of 45% was found in trabecular bone strength (p < 0.05), and an even more pronounced reduction of 58% was found in trabecular bone quality (p < 0.01). The results of this study indicate that long-term administration of sodium fluoride may be detrimental to bone quality, at least as measured in non-loaded iliac crest trabecular bone.
High- and low-trait anxious subjects participated in a divided visual field emotional Stroop task. Subjects identified the colour of unilaterally presented threat-related, positive and neutral words. The high- and low-trait groups differed in terms of their accuracy scores but not in their reaction times. Analysis of the accuracy data revealed that the high-trait group exhibited right hemisphere interference for emotional relative to neutral trials whereas the low-trait showed right hemisphere interference for threat-related trials only. The idea that the right hemisphere is differentially specialised for the processing of emotional information was supported.
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