Search PubMed⌕ Search

Biomedical subjects

A Swan

Publications and source records attributed to A Swan.

At least 19 recordsLinked to original sources
PubMed2004

Glucocorticoid corticosteroids for Duchenne muscular dystrophy.

BACKGROUND: Duchenne muscular dystrophy is the most common muscular dystrophy of childhood. This incurable disease is characterised by muscle wasting and loss of walking ability leading to complete wheelchair dependence by 13 years of age. Prolongation of walking is one of the major aims of treatment. OBJECTIVES: The aim of this review was to assess whether glucocorticoid corticosteroids stabilize or improve muscle strength and walking in boys with DMD. SEARCH STRATEGY: We searched the Cochrane Neuromuscular Disease Group specialised register (October 2003) using the term 'Duchenne muscular dystrophy'. We also searched MEDLINE (January 1966 to October 2003), EMBASE (January 1980 to October 2003), CINAHL and LILACS (January 1982 to October 2003). We wrote to authors of published studies and other experts in this disease to help identify other trials, checked the references in the identified trials and handsearched the abstracts of relevant journals. SELECTION CRITERIA: Types of studies: randomised or quasi-randomised trials. TYPES OF PARTICIPANTS: all patients with a definite diagnosis of Duchenne muscular dystrophy. Types of interventions: glucocorticoids such as prednisone, prednisolone, deflazacort or others, with a minimum treatment period of three months. PRIMARY OUTCOME MEASURE: prolongation of walking (independent walking without long leg calipers). SECONDARY OUTCOME MEASURES: strength outcome measures, manual muscle strength testing using Medical Research Council strength scores, functional outcome measures and adverse events. DATA COLLECTION AND ANALYSIS: We identified five randomised controlled trials that met the inclusion criteria for our review. Two reviewers independently selected the trials for the review and assessed methodological quality. Data extraction and inputting were double-checked. MAIN RESULTS PRIMARY OUTCOME MEASURE: data from one small study used prolongation of walking as an outcome measure and did not show significant benefit. SECONDARY OUTCOME MEASURES: The meta-analysis of the results from three randomised controlled trials showed that glucocorticoid corticosteroids improved muscle strength and function over six months. Improvements were seen in time taken to rise from the floor (Gowers' time), nine metres walking time, four-stair climbing time, ability to lift weights, leg function grade and forced vital capacity. One randomised controlled trial showed that glucocorticoid corticosteroids stabilize muscle strength and function for up to two years. The most effective prednisolone regime appears to be 0.75 mg/kg/day. Not enough data were available to compare efficacy of prednisone with deflazacort.Adverse effects: Excessive weight gain, behavioural abnormalities, cushingoid appearance and excessive hair growth were all more common with glucocorticoid corticosteroids than placebo. Long-term adverse effects of glucocorticoid therapy could not be evaluated because of the short-term duration of the randomised studies.Non-randomised studies: a number of non-randomised studies with important efficacy and adverse effects data are tabulated and discussed. REVIEWERS' CONCLUSIONS: There is evidence from randomised controlled studies that glucocorticoid corticosteroid therapy in Duchenne muscular dystrophy improves muscle strength and function in the short-term (six months to two years). The most effective prednisolone regime appears to be 0.75 mg/kg/day. In the short term, adverse effects were significantly more common but not clinically severe. Long-term benefits and hazards of glucocorticoid treatment cannot be evaluated from the currently published randomised studies. Non-randomised studies support the conclusions of functional benefits but also indicate clinically significant adverse effects of long-term treatment. These benefits and adverse effects have implications for future research studies and clinical practice.

Adrenal Cortex Hormones↗
PubMed2002

The molecular epidemiology of tuberculosis in inner London.

The study used DNA fingerprint typing (spoligotyping and Heminested-lnverse-PCR) of Mycobacterium tuberculosis from all culture-confirmed inner London patients over a 12-month period to describe transmission. The methodology was evaluated by comparison with standard IS6110 typing and by examining its ability to identify known household clusters of cases. Isolates sharing indistinguishable typing patterns using both techniques were defined as clustered. Clusters were investigated to identify epidemiological links. The methodology showed good discriminatory power and identified known household clusters of cases. Of 694 culture-confirmed cases, 563 (81%) were typed. Eleven (2%) were due to laboratory cross-contamination and were excluded. Of the remaining 552 isolates 148 (27%) were clustered. Multivariate analysis indicated that clustering was more common in those with pulmonary smear positive disease (P < 0.02); those born in the United Kingdom (P < 0.0003) and in patients living in south London (P = 0.02). There was also a trend towards clustering being more common in those not known to have HIV infection (P = 0.051). The results suggest that in inner London, recent local transmission makes an important contribution to notification rates.

Adolescent↗
PubMed2002

The value of synovial fluid assays in the diagnosis of joint disease: a literature survey.

OBJECTIVE: To carry out a critical appraisal of the literature in an attempt to assess the current value of synovial fluid (SF) analysis in the diagnosis of joint disease. METHODS: A literature search was undertaken using the Medline, Biomed, Bids, Pubmed, and Embase electronic databases using the keywords: synovial fluid (SF) analysis, SF crystals, joint sepsis, acute arthritis, and SF cell counts, cytology, biomarkers, and microbiology. RESULTS: Publications fell into three main categories. Firstly, reports assessing the value of the three traditional assays (microbiology, white blood cell counts, and microscopy for pathogenic crystals). For these quality control evidence was found to be sparse, and tests for sensitivity, specificity, and reliability showed worrying variations. These poor standards in SF analysis may be due to lack of inclusion of some tests within routine pathology services. Secondly, claims for the usefulness of "new" assays (cytology and biochemical markers). For cytology, the supporting evidence was mainly anecdotal and there were no reports on specificity, sensitivity, and reliability. Interpretation difficulties are a major hindrance to the clinical use of biochemical assays, which remain primarily research tools. Finally, work on the diagnostic value of SF analysis in general. The appraisal confirmed that SF analysis remains of major diagnostic value in acute arthritis, where septic arthritis or crystal arthropathy is suspected, and in intercritical gout. CONCLUSIONS: Given the importance of SF tests, rationalisation of their use, together with improved quality control, should be immediate priorities. Further investigation is recommended into the contribution of SF inspection and white cell counts to diagnosis, as well as of the specificity and sensitivity of SF microbiological assays, crystal identification, and cytology.

Acute Disease↗
PubMed2001

Making vaccines more acceptable--methods to prevent and minimize pain and other common adverse events associated with vaccines.

The growing abundance of highly immunogenic vaccines has arrived with a burden of pain, distress, and common adverse reactions that in turn may interfere with parental compliance and aggravate anti-vaccine sentiment. In a study of 150 children in each of 2 age-groups, we found that approximately 20% of the subjects suffered serious distress or worse. During the procedural phase, approximately 90% of the 15-to-18 month old children and 45% of the 4-to-6 year old children showed serious distress or worse. To address non-adherence with pediatric vaccine schedules, we must consider all of the possible issues that might prevent a parent from taking a child to a health care provider for vaccination. In that same study we identified useful predictors for both preparatory and procedural distress - predictors that might be used in identifying children who might benefit from preventive interventions. Vaccine providers might consider a variety of interventions. Attitude, empathy, instruction, and practice have all been shown to have a salutatory effect upon pain and anxiety with medical procedures in general and specifically with vaccinations. Distraction has also been found to be an effective method for distress and pain prevention in children. More formal methods of clinical hypnosis which combine a deep state of relaxation with focused imagery and suggestion have also been found to be effective in helping children and adolescents prepare for, cope with, and tolerate the pain and anxiety associated with medical procedures. So-called 'sugar nipples' delivering small amounts of sucrose orally at the time of a painful procedure in an infant has been not been shown to decrease vaccination pain and studies on refrigerant topical anesthetics are mixed. Studies have found a eutectic mixture of 2.5% lidocaine and 2.5% prilocaine (EMLA) effective in providing adequate local anesthesia in children, but it suffers from problems in practical application. Studies with various injection techniques have not identified ready solutions, and although jet injection appears to provoke less anxiety and cause less immediate pain, studies also indicate a somewhat greater incidence of delayed local reactogenicity including soreness and edema. Other measures to prevent or rapidly treat other common adverse events have been shown effective and should be considered as well.

Administration, Intranasal↗
PubMed2001

Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy.

This multicenter, randomized, double-blind, crossover trial compared a six week course of oral prednisolone tapering from 60 mg to 10 mg daily with intravenous immunoglobulin (IVIg) 2.0 g/kg given over one to two days for treating chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Twenty-four of the thirty-two randomized patients completed both treatment periods. Both treatments produced significant improvements in the primary outcome measure, change in an 11-point disability scale two weeks after randomization. There was slightly, but not significantly, more improvement after IVIg than with prednisolone, the mean difference between the groups in change in disability grade being 0.16 (95% CI = -0.35 to 0.66). There were also slightly, but not significantly, greater improvements favoring IVIg in the secondary outcome measures: time to walk 10 meters after two weeks and improvement in disability grade after six weeks. Results may have been biased against IVIg by the eight patients who did not complete the second arm of the trial. A serious adverse event (psychosis) attributable to treatment occurred in one patient while on prednisolone and in none with IVIg.

Administration, Oral↗
PubMed2001

The utilization of synovial fluid analysis in the UK.

OBJECTIVE: To ascertain what use is being made of synovial fluid analysis in the UK, who is carrying out polarized light microscopy (PLM), and what confidence clinicians have in the results. Subjects and methods. A postal survey was developed, piloted, adjusted and then posted to 535 people, 90% of whom were senior rheumatologists and 10% orthopaedic surgeons, whose names had been obtained from professional lists. RESULTS: Three hundred and eleven replies (59%) were obtained after 1 month. Analysis of the replies showed that only microbiological tests and PLM are used regularly, that these are used mainly for the diagnosis of acute arthritis, and that the majority of respondents would like data from these assays to be available within 24 h. The majority of the respondents (95%) had access to PLM and 80% said that clinicians should be able to use it. However, PLM is currently being undertaken by a variety of people: non-specialist technicians (34% of respondents), specialist technicians (35%) and clinicians (31%). Respondents were confident in microbiological assays but not in cell counts or PLM, unless they were undertaking it themselves. CONCLUSIONS: There is an urgent need for guidelines, standardization and education about the use of synovial fluid assays in the UK.

Crystallization↗
PubMed2001

Bladder rupture after blunt trauma: guidelines for diagnostic imaging.

PURPOSE: The purpose of this study was to establish guidelines for diagnostic imaging for bladder rupture in the blunt trauma victim with multiple injuries, in whom the delay caused by unnecessary testing can hamper the trauma surgeon and threaten outcome. METHODS: We undertook chart review (1995-1999) of patients with blunt trauma and bladder rupture at our four institutions and performed focused literature review of retrospective series. RESULTS: Of our 53 patients identified, all had gross hematuria and 85% had pelvic fracture. Literature review revealed similar rates. CONCLUSION: The classic combination of pelvic fracture and gross hematuria constitutes an absolute indication for immediate cystography in blunt trauma victims. Existing data do not support lower urinary tract imaging in all patients with either pelvic fracture or hematuria alone. Clinical indicators of bladder rupture may be used to identify atypical patients at higher risk. Patients with isolated hematuria and no physical signs of lower urinary tract injury may be spared the morbidity, time, and expense of immediate cystographic evaluation.

Abdominal Injuries↗
PubMed2001

Identification of new X-chromosomal genes required for Drosophila oogenesis and novel roles for fs(1)Yb, brainiac and dunce.

We performed a screen for female sterile mutations on the X chromosome of Drosophila melanogaster and identified new loci required for developmental events in oogenesis as well as new alleles of previously described genes. We present mapping and phenotypic characterization data for many of these genes and discuss their significance in understanding fundamental developmental and cell biological processes. Our screen has identified genes that are involved in cell cycle control, intracellular transport, cell migration, maintenance of cell membranes, epithelial monolayer integrity and cell survival or apoptosis. We also describe new roles for the genes dunce (dnc), brainiac (brn) and fs(1)Yb, and we identify new alleles of Sex lethal (Sxl), ovarian tumor (otu), sans filles (snf), fs(1)K10, singed (sn), and defective chorion-1 (dec-1).

Animals↗
PubMed2000

A survey of the microbiological quality of private water supplies in England.

Results from statutory testing of private water supplies in nine Public Health Laboratories in England were compiled, and the effects of supply class, source, treatment and location on water quality were examined. A total of 6551 samples from 2911 supplies was examined, over a 2-year period, of which 1342 (21%) samples, and 949 (33%) supplies on at least one occasion, failed current regulations for Escherichia coli. Total coliforms, including E. coli, were detected in 1751 (27%) samples from 1215 (42%) supplies. The percentage of samples positive for E. coli was highest in summer and autumn, and lowest in winter. Samples taken from larger supplies and from boreholes were less frequently contaminated than those from other sources. Chlorination, filtration or UV light treatment improved the bacteriological quality of supplies, but still resulted in a low level of compliance with the regulations. The public health implications of the study are discussed.

Chlorine↗
PubMed1999

Drosophila Lissencephaly-1 functions with Bic-D and dynein in oocyte determination and nuclear positioning.

Here we show that the Drosophila homologue of Lissencephaly-1, DLis-1, acts together with Bicaudal-D (Bic-D), Egalitarian (Egl), dynein and microtubules to determine oocyte identity. DLis-1 is further required for nurse-cell-to-oocyte transport during oocyte growth, and for the positioning of the nucleus in the oocyte. Immunostaining of DLis-1 protein reveals a cortical localization that is independent of microtubules. DLis-1 may function in this position as a cortical anchor for the other nuclear-localization factors. DLis-1 and Bic-D are further required for nuclear localization in the developing nervous system, indicating that homologues of Bic-D, dynein and Egl-like proteins may also be involved in vertebrate neural migration and that their absence may cause a Miller-Dieker-like lissencephaly.

Amino Acid Sequence↗
PubMed1999

Olanzapine in dementia with Lewy bodies: a clinical study.

OBJECTIVES: Dementia with Lewy bodies (DLB) is now a well-recognized form of dementia in which psychosis and behavioural disturbance are common. Treatment with conventional neuroleptics is often very poorly tolerated. Olanzapine, a newly introduced atypical neuroleptic which binds to multiple receptor types with relatively low affinity for D2 receptors, may be a useful treatment option in DLB. MAIN OUTCOME MEASURES: The Behavioural Pathology in Alzheimer's Disease Rating Scale, The Neuropsychiatric Inventory, Unified Parkinson's Disease Rating Scale and The Webster Disability Scale. DESIGN: We present the results of eight DLB patients with associated psychotic and behavioural difficulties. All patients were given olanzapine 2.5-7.5 mg. Their psychotic phenomena and behavioural and extrapyramidal symptoms were monitored at 2-weekly intervals. RESULTS: Three out of the eight patients could not tolerate olanzapine even at the lowest available dose. Two patients had clear improvement in psychotic and behavioural symptoms. Three patients were able to tolerate olanzapine but gained only minimal benefit. CONCLUSIONS: Olanzapine at the doses used conferred little advantage over conventional neuroleptics and should only be given with great caution to patients with DLB. The utility of smaller doses deserves further evaluation.

Aged↗
PubMed1998

Renin-aldosterone system can respond to furosemide in patients with hyperkalemic hyporeninism.

Thirty-four patients (65.3+/-3.3 years of age, mean+/-SEM) with hyperkalemia (serum potassium >5.0 mEq/L) had measurement of their renin-aldosterone system. Nineteen patients (56%) had plasma renin activity (PRA) >1.5 ng/mL/h, which was not low, while 15 (44%) had PRA <1.5. Twelve of the 15 hyporeninemic hyperkalemic patients were studied to determine whether their renin-aldosterone system responded to 2 weeks of furosemide, 20 mg daily. Four were nonresponders: PRA averaged 0.3+/-0.1 ng/mL/h, and it did not increase with furosemide or respond to captopril before or after furosemide. Eight patients were responders: PRA averaged 0.6+/-0.2 ng/mL/h and increased with furosemide to 5.5+/-3.4 ng/mL/h. Captopril failed to increase PRA before furosemide, but PRA increased to 15.3+/-8.4 ng/mL/h after furosemide. Plasma aldosterone was low in both nonresponders and responders (3.5+/-1.2 ng/dL vs 5.8+/-2.5 ng/dL) and did not increase significantly with furosemide (4.3+/-1.7 ng/dL vs 8.7+/-2.5 ng/dL). Serum potassium did not fall and therefore did not limit the rise in aldosterone. Renin responders had greater body weight, were predominantly female (6/8 vs 2/4) and were more likely to have diabetes mellitus (7/8 vs 0/4). Plasma atrial natriuretic peptide (ANP) fell with furosemide in 8 of 8 responders and in 1 of the 2 nonresponders in whom it was measured. Neither group had suppressed plasma prorenin levels, indicating no suppression of renin gene expression. These results indicate that many hyperkalemic patients do not have suppressed PRA. Further, a majority of patients with suppressed PRA have high levels of ANP and can respond to diuretic therapy with a rise in PRA and a fall in ANP, suggesting physiologic suppression of the renin system by volume expansion. A minority of hyperkalemic patients with suppressed PRA had PRA that did not increase under these study conditions.

Adult↗
PubMed1998

vasa is required for GURKEN accumulation in the oocyte, and is involved in oocyte differentiation and germline cyst development.

The Drosophila gene vasa is required for pole plasm assembly and function, and also for completion of oogenesis. To investigate the role of vasa in oocyte development, we generated a new null mutation of vasa, which deletes the entire coding region. Analysis of vasa-null ovaries revealed that the gene is involved in the growth of germline cysts. In vasa-null ovaries, germaria are atrophied, and contain far fewer developing cysts than do wild-type germaria; a phenotype similar to, but less severe than, that of a null nanos allele. The null mutant also revealed roles for vasa in oocyte differentiation, anterior-posterior egg chamber patterning, and dorsal-ventral follicle patterning, in addition to its better-characterized functions in posterior embryonic patterning and pole cell specification. The anterior-posterior and dorsal-ventral patterning phenotypes resemble those observed in gurken mutants. vasa-null oocytes fail to efficiently accumulate many localized RNAs, such as Bicaudal-D, orb, oskar, and nanos, but still accumulate gurken RNA. However, GRK accumulation in the oocyte is severely reduced in the absence of vasa function, suggesting a function for VASA in activating gurken translation in wild-type ovaries.

Animals↗
PubMed1996

Role of Bicaudal-D in patterning the Drosophila egg chamber in mid-oogenesis.

The Bicaudal-D (Bic-D) gene is required early in Drosophila oogenesis for the differentiation of an oocyte from one of a cluster of 16 interconnected germarial cells. To analyze the role of Bic-D later in oogenesis, we have constructed Drosophila lines in which Bic-D expression is under the control of the hsp70 promoter. In these flies, Bic-D activity can be induced early in oogenesis, allowing an oocyte to be made. Then, by shifting females to non-inducing conditions, Bic-D levels are depleted for the remainder of oogenesis. Using this system, we find that Bic-D is indeed required in the later stages of oogenesis. In ovaries from mutant females, oocyte growth is reduced, apparently due to defects in nurse-cell-to-oocyte transport. Smaller oocyte size results in the misalignment of follicle cells and the underlying germ line, leading to ventralization of dorsal follicle cells and to defects in centripetal cell migration. In addition, we show that Bic-D is required for the localization of specific mRNAs at both the anterior and posterior of the oocyte.

Animals↗