Are epilepsy classifications based on epileptic syndromes and seizure types outdated?
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Epilepsy↗
Biomedical subjects
A Tanner
Publications and source records attributed to A Tanner.
Tracing Sydenham's chorea: historical documents from a British paediatric hospital.
Sydenham's chorea (SC) became a well defined nosological entity only during the second half of the nineteenth century. Such progress was promoted by the availability of large clinical series provided by newly founded paediatric hospitals. This paper analyses the demographic and clinical features of patients with chorea admitted to the first British paediatric hospital (the Hospital for Sick Children, Great Ormond Street, London) between 1852 and 1936. The seasonal and demographic characteristics of SC during this time appear strikingly similar to those observed today, and witness the introduction of modern "statistically averaging" techniques in the approach to complex paediatric syndromes. Great Ormond Street (GOS) hospital case notes provide detailed descriptions of the "typical cases" of SC, and show that British physicians working in the early age of paediatric hospitals succeeded in recognising the most distinctive clinical features of this fascinating condition.
Child↗
Second case report of a metachronous double primary gastric cancer.
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Adenocarcinoma↗
Oxalate decarboxylase requires manganese and dioxygen for activity. Overexpression and characterization of Bacillus subtilis YvrK and YoaN.
The Bacillus subtilis oxalate decarboxylase (EC ), YvrK, converts oxalate to formate and CO(2). YvrK and the related hypothetical proteins YoaN and YxaG from B. subtilis have been successfully overexpressed in Escherichia coli. Recombinant YvrK and YoaN were found to be soluble enzymes with oxalate decarboxylase activity only when expressed in the presence of manganese salts. No enzyme activity has yet been detected for YxaG, which was expressed as a soluble protein without the requirement for manganese salts. YvrK and YoaN were found to catalyze minor side reactions: oxalate oxidation to produce H(2)O(2); and oxalate-dependent, H(2)O(2)-independent dye oxidations. The oxalate decarboxylase activity of purified YvrK was O(2)-dependent. YvrK was found to contain between 0.86 and 1.14 atoms of manganese/subunit. EPR spectroscopy showed that the metal ion was predominantly but not exclusively in the Mn(II) oxidation state. The hyperfine coupling constant (A = 9.5 millitesla) of the main g = 2 signal was consistent with oxygen and nitrogen ligands with hexacoordinate geometry. The structure of YvrK was modeled on the basis of homology with oxalate oxidase, canavalin, and phaseolin, and its hexameric oligomerization was predicted by analogy with proglycinin and homogentisate 1,2-dioxygenase. Although YvrK possesses two potential active sites, only one could be fully occupied by manganese. The possibility that the C-terminal domain active site has no manganese bound and is buried in an intersubunit interface within the hexameric enzyme is discussed. A mechanism for oxalate decarboxylation is proposed, in which both Mn(II) and O(2) are cofactors that act together as a two-electron sink during catalysis.
Amino Acid Sequence↗
An unusual cause of an acute abdomen.
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Abdomen, Acute↗
The accelerations of stars orbiting the Milky Way's central black hole
Recent measurements of the velocities of stars near the centre of the Milky Way have provided the strongest evidence for the presence of a supermassive black hole in a galaxy, but the observational uncertainties poorly constrain many of the black hole's properties. Determining the accelerations of stars in their orbits around the centre provides much more precise information about the position and mass of the black hole. Here we report measurements of the accelerations of three stars located approximately 0.005 pc (projected on the sky) from the central radio source Sagittarius A* (Sgr A*); these accelerations are comparable to those experienced by the Earth as it orbits the Sun. These data increase the inferred minimum mass density in the central region of the Galaxy by an order of magnitude relative to previous results, and localize the dark mass to within 0.05 +/- 0.04 arcsec of the nominal position of Sgr A*. In addition, the orbital period of one of the observed stars could be as short as 15 years, allowing us the opportunity in the near future to observe an entire period.
Journal Article↗
Specialized intestinal metaplasia: analysis of prevalence, risk factors and association with gastro-oesophageal reflux disease
AIMS: There is an increasing awareness that short (less than 3 cm) segments of Barrett's epithelium and macroscopically normal cardia epithelium may harbour specialized intestinal metaplasia (SIM), a premalignant phenotype. This was a prospective study of both the prevalence of SIM in an unselected population of patients attending for endoscopy, and the association of SIM with symptoms, lifestyle, medication, endoscopic oesophagitis and carditis. METHODS: Two hundred consecutive patients underwent endoscopy. Biopsies taken from just below the squamocolumnar junction were stained for SIM, and were analysed for carditis and Helicobacter pylori infection. A detailed questionnaire of symptoms, tobacco consumption and the use of proton pump inhibitors was completed. RESULTS: Forty-two patients (21 per cent) had SIM, 19 of 126 (15 per cent) in an endoscopically normal oesophagus, 15 of 63 (24 per cent) in a short segment of Barrett's epithelium and eight of 11 in classical Barrett's oesophagus. Comparative analysis between the SIM positive and negative groups with respect to potential risk factors is outlined below. Table 1. CONCLUSION: SIM is prevalent in patients undergoing endoscopy, does not correlate with symptoms or with H. pylori infection, but is significantly associated with endoscopic and pathological markers of gastro-oesophageal reflux.
Journal Article↗
The SIBDQ: further validation in ulcerative colitis patients.
OBJECTIVE: The Inflammatory Bowel Disease Questionnaire (IBDQ) is an instrument that assesses quality of life in patients with inflammatory bowel disease. It has 32 items in four domains. The short form of the IBDQ (SIBDQ) was developed in Canadian Crohn's disease patients for use in clinical practice. Patients with ulcerative colitis might require a different form of the SIBDQ. Our aim was to design and validate a SIBDQ for patients with ulcerative colitis and to compare this to the Crohn's SIBDQ. METHODS: We recruited 122 patients with colitis as an initial sample. Using linear regression modeling, the 10 items that best predicted the total IBDQ score were identified. The colitis and Crohn's versions of the SIBDQ were compared by univariate linear regression with the total IBDQ score in two other cohorts of colitis patients. RESULTS: Ten items explained 97% of the variance of the total IBDQ score in our first cohort. These were items 1 and 9 (bowel); 7, 11, 21, 30 (emotional); 2 and 10 (systemic); and 12 and 28 (social). Only three items were shared with the Crohn's SIBDQ. The R2 for both SIBDQs with the total IBDQ score in the other cohorts were very high (> or =0.95), although the Colitis SIBDQ showed better internal consistency. CONCLUSIONS: The development of a SIBDQ for patients with ulcerative colitis did not reveal any clear advantage over the original version of the SIBDQ. Further studies are required to determine the role of the SIBDQ in routine clinical practice.
Attitude to Health↗
Bacillus subtilis YvrK is an acid-induced oxalate decarboxylase.
Bacillus subtilis has been shown to express a cytosolic oxalate decarboxylase (EC 4.1.1.2). The enzyme was induced in acidic growth media, particularly at pH 5.0, but not by oxalate. The enzyme was purified, and N-terminal sequencing identified the protein to be encoded by yvrK. The role of the first oxalate decarboxylase to be identified in a prokaryote is discussed.
Amino Acid Sequence↗
Conversion of 4-hydroxyacetophenone into 4-phenyl acetate by a flavin adenine dinucleotide-containing Baeyer-Villiger-type monooxygenase.
An arylketone monooxygenase was purified from Pseudomonas putida JD1 by ion exchange and affinity chromatography. It had the characteristics of a Baeyer-Villiger-type monooxygenase and converted its substrate, 4-hydroxyacetophenone, into 4-hydroxyphenyl acetate with the consumption of one molecule of oxygen and oxidation of one molecule of NADPH per molecule of substrate. The enzyme was a monomer with an M(r) of about 70,000 and contained one molecule of flavin adenine dinucleotide (FAD). The enzyme was specific for NADPH as the electron donor, and spectral studies showed rapid reduction of the FAD by NADPH but not by NADH. Other arylketones were substrates, including acetophenone and 4-hydroxypropiophenone, which were converted into phenyl acetate and 4-hydroxyphenyl propionate, respectively. The enzyme displayed Michaelis-Menten kinetics with apparent K(m) values of 47 microM for 4-hydroxyacetophenone, 384 microM for acetophenone, and 23 microM for 4-hydroxypropiophenone. The apparent K(m) value for NADPH with 4-hydroxyacetophenone as substrate was 17.5 microM. The N-terminal sequence did not show any similarity to other proteins, but an internal sequence was very similar to part of the proposed NADPH binding site in the Baeyer-Villiger monooxygenase cyclohexanone monooxygenase from an Acinetobacter sp.
Acetophenones↗
Combined subcutaneous recombinant alpha-interferon and interleukin-2 in metastatic renal cell cancer: results of the Multicentre All Ireland Immunotherapy Study Group.
OBJECTIVE: To analyse the toxicity and efficacy of combined interferon-alpha and interleukin-2, administered subcutaneously in a general multicentre setting, as treatment for metastatic renal cell carcinoma. METHODS: Thirty-three patients with metastatic renal cell carcinoma were scheduled to receive 2 cyclical doses of subcutaneous interferon-alpha (week 1: 5 MU x 3 days) and interleukin-2 (week 2: 36 MU x 2 days, 9 MU x 3 days; weeks 3-5: 9 MU daily). Karnofsky scores ranged from 80 to 100 (median 90). Metastases occurred in multiple organs (lung 63%, retroperitoneal 39%, liver 24%). Patients were categorised according to the risk of disease progression. Treatment toxicity, therapeutic response and actuarial survival were analysed. RESULTS: All patients received recommended doses of treatment, but 6 received less than 2 cycles. Most were treated as outpatients, although hospitalisation was usual during the 1st week of a cycle. All complained of mild flu-like symptoms. Severe side effects developed in 13 patients (39%), and treatment was discontinued in 3 of these patients. No deaths occurred as a result of treatment. The overall median survival was 10 months. The overall actuarial survival rate at 3 years was 22%. On statistical analysis, actuarial survival rates were not influenced by either response to treatment or risk group category. CONCLUSION: Subcutaneously administered, combined interferon-alpha and interleukin-2 therapy achieves durable survival rates in a minority of patients with renal cell carcinoma. Toxicity is remedial, and not fatal, when subcutaneous therapy is administered by multiple medical disciplines at a variety of centres.
Carcinoma, Renal Cell↗
Identification of oral Actinomyces species using DNA probes.
Oral Actinomyces comprise a major segment of both the supra- and subgingival microbiota; however, little is known about the distribution of individual species in different sites or clinical conditions. The purpose of the present investigation was to develop DNA probes for suggested species and genotypes of oral Actinomyces. Whole genomic DNA probes to 12 human oral species and/or serotypes were labeled with digoxigenin and used to seek cross-reactions among the taxa using the checkerboard DNA-DNA hybridization assay. The Actinomyces formed three distinct groups: 1) Actinomyces georgiae, Actinomyces meyeri and Actinomyces odontolyticus serotypes I and II; 2) Actinomyces viscosus and Actinomyces naeslundii serotypes I, II, III and WVA 963; and 3) Actinomyces gerencseriae and Actinomyces israelii. Cross-reactions among taxa were detected and minimized by increasing the temperature of the post-hybridization high-stringency wash to 80 degrees C. Despite the elevation in high stringency wash temperature, cross-reactions among strains of the A. naeslundii/A. viscosus group persisted. Probes for two of the three currently recognized genospecies in this group were prepared by removing the DNA in common between cross-reacting species using subtraction hybridization and polymerase chain reaction. Nine species and genospecies could be clearly separated by a combination of whole genomic and subtraction hybridization probes and by increasing the high-stringency wash temperature. A total of 195 fresh isolates of Actinomyces were grouped in a blind study using DNA probes and separately by SDS-PAGE protein profiles. Concordance between the two methods was 97.3%. The probes and hybridization conditions were tested for their ability to detect the Actinomyces species and genospecies in samples of supragingival and subgingival plaque from periodontitis subjects using checkerboard DNA-DNA hybridization. The probes detected the species in samples of supragingival and subgingival plaque. We concluded that whole genomic and subtraction hybridization DNA probes facilitate the detection and enumeration of species and genospecies of Actinomyces in plaque samples.
Actinomyces↗
Patient pathways: solving the problem of institutional mortality in London during the later nineteenth century.
This paper confronts a major problem in relation to the metropolitan and urban mortality declines in Britain during the later nineteenth century: the extent to which cause-specific death rates at district level were distorted by an official failure to redistribute deaths occurring in institutions back to "normal" place of residence. Describing and analysing the manner in which hospital and infirmary provision in the capital during this period determined the geographical incidence of mortality, the article develops methodologies that bring the researcher progressively closer to more accurate spatial facts of demographic and epidemiological experience. Devoting separate sections to general voluntary and fever hospitals as well as to institutions administered by the Metropolitan Asylums Board, the paper also engages with similar problems in relation to workhouses and Poor Law infirmaries. By way of conclusion, it shows that, far from generating demographically trivial results, the process of redistribution radically revises the epidemiological history of London in the crucial years between the 1860s and the mid-1880s and provides a dataset that will allow controlled interrogation of the metropolitan mortality decline during this period.
History, 19th Century↗
Lesions of the reflection pulley of the long biceps tendon. MR arthrographic findings.
RATIONALE AND OBJECTIVES: The purpose of this study was to assess the diagnostic value of MR arthrography in detecting a lesion of the common insertion of the coracohumeral and the superior glenohumeral ligaments and the superior border of the subscapularis tendon (pulley lesion), which predisposes to biceps tendon subluxation and subsequent degeneration. METHODS: Parasagittal T1-weighted turbo spin-echo and axial gradient-echo (three-dimensional fast imaging with steady state-precession [FISP]) MR arthrographic images were obtained in 14 patients with surgically confirmed pulley lesions and in 10 patients with an intact pulley. Various MR arthrographic signs potentially associated with a pulley lesion were evaluated separately and independently in a blinded fashion by two radiologists. RESULTS: Abnormalities of the superior border of the subscapularis tendon on axial and parasagittal images, extra-articular contrast collection, and biceps tendon subluxation were the MR findings of a pulley lesion. The coracohumeral and superior glenohumeral ligaments were not readily visible in all patients and were not helpful in diagnosing pulley lesions in this study. The overall sensitivity for detecting a pulley lesion by MR arthrography was 86% and 93% for readers 1 and 2, with a specificity of 100% and 80% and an accuracy of 92% and 87% (kappa = 0.75). CONCLUSIONS: Based on the authors' experience, MR arthrography is valuable in detecting lesions of the reflection pulley of the long biceps tendon, although differentiation from an isolated lesion of the superior border of the subscapularis tendon may not be possible.
Adult↗
Neuronal ceroid lipofuscinosis (nclf), a new disorder of the mouse linked to chromosome 9.
The neuronal ceroid lipofuscinoses (NCLs) comprise a set of at least 6 distinct human and an unknown number of animal diseases characterized by storage of proteolipids in lysosomes of many cell types. By unknown mechanisms, this accumulation leads to or is associated with severe neuronal and retinal degeneration. The genes for 3 human NCLs, infantile, late infantile, and juvenile, have been cloned. The first murine form of NCL, the motor neuron degeneration (mnd) mouse, has been described and mapped to proximal Chromosome 8. Here we describe a second genetic variant of NCL in the mouse, neuronal ceroid lipofuscinosis, nclf. These mice exhibited a phenotype that was almost exactly the same as that observed in mnd/mnd mice. Homozygous nclf mice developed progressive retinal atrophy early in life and become paralyzed at around 9 months of age. They accumulated luxol fast blue staining material in cytoplasm of neurons and many other cell types. Ultrastructurally, affected lysosomes had a "finger print pattern" with membranous material arranged in "pentalaminar" patterns. Affected mice developed severe cerebral gliosis in late stages of their disease. They also had severe Wallerian degeneration of long tracts in spinal cord and brain stem, lesions that accounted for the distinctive upper motor neuron signs displayed by both nclf/nclf and mnd/mnd mice. By crossing nclf/nclf mice with CAST/Ei mice, linkage analysis of nclf with respect to SSLP markers was performed, showing that nclf is located on Chromosome 9 between D9Mit164 and D9Mit165, in a region that is homologous with human Ch 15q21, where the gene for one variant of late infantile NCL, CLN6, recently has been mapped. The genes for two proteolipids known to be stored in lysosomes of animals and people with NCL were also mapped in this study and found not to map to the mnd or nclf loci nor to any mouse locus homologous to any known human NCL disease locus.
Animals↗
Clinical investigation of the pressure and shear stress on the trans-tibial stump with a prosthesis.
A system for measuring pressures and bi-axial shear stresses at the body support interfaces has been developed. This system has been used, in five unilateral trans-tibial amputees, to investigate the stresses at multiple points on the residual limb and prosthetic socket interface during standing and walking. The subjects investigated regularly used a patellar-tendon-bearing socket. The maximum peak pressure at the measured points was 320 kPa over the popliteal area during walking. The maximum shear stress was 61 kPa over the medial tibia area. Variable wave-forms of stress during walking were observed at the different measured points. The influence of the angular alignment on the stresses was investigated on one subject. It was found that a miss-alignment of +/- 8 degrees produced a change in peak longitudinal shear stress of between 8% and 11.5%.
Adult↗
A prospective study comparing intravenous tenoxicam with rectal diclofenac for pain relief in day case surgery.
In a prospective, randomized, double-blind study, we compared intravenous tenoxicam with rectal diclofenac for post-operative pain relief after day case arthroscopy or laparoscopic sterilization. Intravenous tenoxicam (40 mg) was administered as a single bolus at induction, or rectal diclofenac (100 mg) was administered immediately after induction. Both groups were similar with respect to age, weight, sex of the patients, the operation performed and the operative time. There were no significant differences observed between the groups for pain scores at 30 min, 60 min and 24 h post-operatively. The time to first analgesic requirement, the dose of pethidine administered and total analgesic requirements in the first 24 h post-operatively were equivalent in both groups. In view of the similar efficacy of both of these drugs, patient preference and ease of administration, the use of tenoxicam is appropriate in many patients undergoing day case surgery.
Administration, Rectal↗
Microbiota of health, gingivitis, and initial periodontitis.
This study compared the subgingival microbiota in periodontal health, gingivitis and initial periodontitis using predominant culture and a DNA probe, checkerboard hybridization method. 56 healthy adult subjects with minimal periodontal attachment loss were clinically monitored at 3-month intervals for 12 months. More sites demonstrated small increments of attachment loss than attachment gain over the monitoring period. Sites, from 17 subjects, showing > or = 1.5 mm periodontal attachment loss during monitoring were sampled as active lesions for microbial analysis. Twelve subjects demonstrated interproximal lesions, and 5 subjects had attachment loss at buccal sites (recession). Cultural studies identified Bacteroides forsythus, Campylobacter rectus, and Selenomonas noxia as the predominant species associated with active interproximal lesions (9 subjects), whereas Actinomyces naeslundii, and Streptococcus oralis, were the dominant species colonizing buccal active sites. A. naeslundii, Campylobacter gracilis, and B. forsythus (at lower levels than active sites) were the dominant species cultured from gingivitis (10 subjects). Health-associated species (10 subjects) included Streptococcus oralis, A. naeslundii, and Actinomyces gerencseriae. DNA probe data identified higher mean levels of B. forsythus and C. rectus with active (7 subjects) compared to inactive periodontitis sites. Porphyromonas gingivalis and Actinobacillus actinomycetemcomitans were detected infrequently. Cluster analysis of the cultural microbiota grouped 8/9 active interproximal lesions in one subcluster characterized by a mostly gram-negative microbiota, including B. forsythus and C. rectus. The data suggest that B. forsythus C. rectus and S. noxia were major species characterizing sites converting from periodontal health to disease. The differences in location and microbiota of interproximal and buccal active sites suggested that different mechanisms may be involved in increased attachment loss.
Adult↗