Understanding frames: A qualitative study of young people's experiences of using standing frames as part of postural management for cerebral palsy.

BACKGROUND: Consensus opinion supports standing frame use as part of postural management for nonambulant young people with cerebral palsy. Although the rationale for standing frame use and the associated challenges have been described, little attention has been given to the users' experiences. The aim of the current study was to explore young people's positive and negative experiences, and attitudes regarding standing frame use. METHODS: Framework analysis informed an open exploration of young people's opinions of standing frames. Using semistructured interviews, 12 young people with cerebral palsy (6 female) were interviewed, providing the data set for transcription and thematic analysis. FINDINGS: The first theme "attitudes to standing frames" describes the young people's understanding of why they use standing frames. Although standing frames can be painful, some young people believe they should be endured to improve their body structure and function. There were mixed views about the impact standing frames have socially, with some young people feeling excluded from their peers, and others feeling as though standing frames helped them "fit in." Some young people are not offered a choice about how and when they use their standing frame. The second theme "challenges of standing frame use" highlights the issues with standing frame use such as manual handling, interference from siblings, and the lack of aesthetically pleasing standing frame designs. CONCLUSIONS: Young people report benefits related to choice, pain relief, and participation but can also cause pain, discomfort, and reduced independence and participation. Healthcare professionals should have open, informative conversations about potential benefits and challenges of standing frames on all aspects of the young people's lives, including participation and activity.

Understanding frames: A UK survey of parents and professionals regarding the use of standing frames for children with cerebral palsy.

BACKGROUND: Standing frames are used for children with cerebral palsy (CP). They may improve body structure and function (e.g., reducing risk of hip subluxation, and improving bladder and bowel function), improving activity (e.g., motor abilities) and participation (e.g., interaction with peers), but there is little evidence that they do. We aimed to identify current UK standing frame practice for children with CP and to understand stakeholder views regarding their clinical benefits and challenges to use. METHOD: Three populations were sampled: clinicians prescribing standing frames for children with CP (n = 305), professionals (health and education) working with children with CP who use standing frames (n = 155), and parents of children with CP who have used standing frames (n = 91). Questionnaires were developed by the co-applicant group and piloted with other professionals and parents of children with CP. They were distributed online via clinical and parent networks across the UK. RESULTS: Prescribing practice was consistent, but achieving the prescribed use was not always possible. Respondents in all groups reported the perceived benefits of frames, which include many domains of the International Classification of Functioning Disability and Health for Children and Youth. Challenges of use are related to physical space and child-reported pain. CONCLUSIONS: These survey findings provide information from key stakeholders regarding current UK standing frame practice.

Comparing medical versus surgical termination of pregnancy at 13-20 weeks of gestation: a randomised controlled trial.

OBJECTIVE: To compare the psychological impact, acceptability and clinical effectiveness of medical versus surgical termination of pregnancy (TOP) at 13-20 weeks of gestation. DESIGN: Randomised trial. SETTING: Large UK tertiary centre. SAMPLE: Women accepted for TOP at 13-20 weeks of gestation. METHODS: Medical TOP (MTOP) using mifepristone and misoprostol or surgical TOP (STOP) by vacuum aspiration at <15 weeks of gestation, and by dilatation and evacuation at 15 or more weeks of gestation. MAIN OUTCOME MEASURES: Distress 2 weeks after TOP, measured by the impact of events scale (IES), and acceptability, measured by the proportion of women who would opt for the same procedure again. RESULTS: One hundred and twenty two women were randomised: 60 to the MTOP group and 62 to the STOP group. Twelve women opted to continue their pregnancy. Follow-up rates were low (n=66/110; 60%). At 2 weeks post-procedure there was no difference in total IES score between groups. However, compared with women undergoing STOP, women undergoing MTOP had a higher score on the IES intrusion subscale (mean difference 6.6; 95% CI 1.4-11.8), and a higher score on the general health questionnaire (GHQ) (P=0.033). Women found STOP more acceptable: compared with MTOP, more women would opt for the same procedure again (100% versus 53%, P≤0.001), and fewer women found the experience to be worse than expected (0% versus 53%, P=0.001). Women who had MTOP experienced more bleeding (P=0.003), more pain on the day of the procedure (P=0.008), and more days of pain (P=0.020). Of the 107 women who declined to participate, 58 (67%) preferred a STOP. CONCLUSIONS: Randomised trials of women requesting midtrimester TOP are challenging. Women found STOP less painful and more acceptable than MTOP.

Cytochrome c oxidase deficient muscle fibres: substantial variation in their proportions within skeletal muscles from patients with mitochondrial myopathy.

Mitochondrial DNA (mtDNA) disease is a common cause of myopathy and the presence of histochemically demonstrated cytochrome c oxidase (COX) deficiency is an extremely useful diagnostic feature. However, there is currently no quantitative information regarding the variability of COX deficiency within or between muscles. This study addresses this issue by studying a number of skeletal muscle samples obtained at post-mortem from three patients with mitochondrial disease due to established mitochondrial DNA defects. COX deficient muscle fibres were enumerated in sections of these muscles and analysed according to patient, individual muscle, position within a particular muscle and sample size. Descriptive statistics were generated followed by an analysis of variance (ANOVA) to assess the effect of these parameters on the mean percentage of COX deficient fibres. We observed statistically significant variation in the percentage of COX deficient fibres within individual muscles from each patient for samples sizes of between 100 and 400 fibres. Our results have implications for the way in which biopsies of skeletal muscle are used for the assessment of disease severity, progression and response to treatment.

Does long term residency near industry have an impact on the body burden of polychlorinated dibenzo-p-dioxins, furans, and polychlorinated biphenyls in older women?

BACKGROUND: For the retrospective study of environment and health linkages biomarkers of exposure are required. Polychlorinated dibenzo-dioxins and furans (PCDD/F) and polychlorinated biphenyls (PCBs) have been useful markers in some settings. This is the first study of PCDD/F body burden in a population based sample from the UK. AIMS AND METHODS: The authors aimed to investigate whether long term residents close to a heavy chemical industrial complex (Teesside, UK) had a higher body burden and distinct pattern of PCDD/F and PCBs. We measured current levels of PCDD/F and PCBs in a population based sample of older women (mean 64 years, range 42-79 years). Forty women were recruited, 20 living near (zone A: 0.1-2.7 km) and 20 distant (zone C: 5-40 km) from industry during 2000-03. The authors ascertained occupational exposure to lung carcinogens, residential history, consumption of local produce, breast feeding, diet, and height and weight. RESULTS: The mean body burden measured on lipid basis in ng/kg for the whole sample was: WHO-TEQ (PCDD/Fs): 29.9, 2378TCDD: 4.0, PCB 118:16200, PCB156: 13100. Body burdens were similar to others reported from industrialised countries, except that mean 2378TCDD was slightly higher. Mean ages, body mass index, and lifelong dietary patterns were similar in both zones. The authors found no significant difference in mean body burden levels between zones A and C before or after adjustment for covariates. All congener patterns were consistent with an urban background pattern, and there was no significant difference between congener compositions in the two zones. The TCDD body burden increased with age with accelerated increments above age 70. CONCLUSION: Long term residency near heavy and chemical industry did not have an effect on women's body burden of PCDD/Fs and PCBs on Teesside, UK. The body burden of PCDD/F and PCBs was not a suitable biomarker for chronic, non-occupational exposure to industrial air pollution.

Consultations of children living near open-cast coal mines.

Because of local concerns, general practitioner consultation rates in children living in communities close to and away from open-cast mines were compared. Information on consultations was collected on 2,442 children 1-11 years of age living in five socioeconomically matched pairs of open-cast and control communities in northern England. The data collection periods were 6 weeks each during 1996-1997 and the 52-week periods preceding these weeks. Consultations were categorized as respiratory, skin and eye conditions (possibly exacerbated by particulate matter), or other conditions. Over the 6-week periods, children in 4/5 pairs of open-cast and control communities had similar consultation rates for all conditions combined [2.7 vs. 2.4 per person-year; odds ratio (OR) = 1.1; 95% confidence interval (CI), 0.96-1.3). Consultations were higher in the open-cast communities for respiratory, skin, and eye conditions (2.1 vs. 1.5 per person-year; OR = 1.4; 95% CI, 1.2-1.7), and respiratory conditions alone (1.5 vs. 1.1 per person-year; OR = 1.5; 95% CI, 1.2-1.8). However, increases in consultation rates in open-cast communities were generally not seen over the portions of the 52-week periods when the open-cast sites were either active or inactive.

Prevalence of asthma and other respiratory symptoms in children living near and away from opencast coal mining sites.

BACKGROUND: Public concern about respiratory conditions prompted the investigation of asthma and other respiratory diseases in children living near and away from opencast coal mining sites. METHODS: We selected all 4860 children aged 1--11 years from five socioeconomically matched pairs of communities close to (OC) and away from (CC) active opencast sites. A postal questionnaire collected data on health and lifestyle. Outcomes were the cumulative and period prevalence (2 and 12 months) of wheeze, asthma, bronchitis and other respiratory symptoms. RESULTS: The cumulative prevalence of wheeze varied from 30% to 40% across the ten communities, it was 36% in OC and 37% in CC. The cumulative prevalence of asthma was 22% in both OC and CC, varying between 12% and 24%. We found little evidence for associations between living near an opencast site and an increased prevalence of respiratory illnesses, or asthma severity. Some outcomes such as allergies, hayfever, or cough varied little across the study communities. Others, such as the use of asthma medication, the number of severe wheezing attacks in the past year or tonsillitis showed large variation. These similarities and variations were not explained by differences in lifestyle factors or differences in health services delivery and remain unexplained. CONCLUSIONS: There was little evidence of an association between residential proximity to opencast mining sites and cumulative or period prevalence of respiratory illness, or asthma severity. Some variations in health outcomes between communities remained unexplained.

Incidence and prevalence of primary biliary cirrhosis in the city of Newcastle upon Tyne, England.

OBJECTIVE: To describe the incidence and prevalence of primary biliary cirrhosis in an urban population between 1987 and 1994, using stringent inclusion criteria and a well-defined study area and population. DESIGN: Descriptive study based on a case register compiled by a retrospective and prospective case-finding exercise and examination of case notes. SETTING: The city of Newcastle upon Tyne. MAIN INCLUSION CRITERIA: (1) Definite cases: fulfilling all three of the following diagnostic criteria: positive antimitochondrial antibody (AMA) > or = 1:40; cholestatic liver function tests (LFT); diagnostic or compatible liver histology. (2) Probable cases: fulfilling two of these criteria. SUBJECTS: All cases of primary biliary cirrhosis identified by multiple case-finding methods, alive from 1 January 1987 to 31 December 1994, in the defined area. MAIN OUTCOME MEASUREMENTS: Incidence and point prevalence rates by age and sex. RESULTS: In all, 202 potential cases were identified, of whom 160 met at least two inclusion criteria. In definite cases annual incidence varied from 14 to 32 (mean 22) per million whole population (with no clear trend) and point prevalence rose from 180 per million in 1987 to 240 in 1994. Mean age at diagnosis in cases incident during the study period was 63.2 years (S.D. 11.1 years, range 39.8-85.7 years). CONCLUSIONS: Primary biliary cirrhosis is much more common in Newcastle than has previously been reported anywhere in the world, and prevalence appears to be rising.

A simple test for the random arrangement of muscle fibres.

The random arrangement of a given muscle fibre class has been assessed by estimating 'mean cluster size' in transverse sections of skeletal muscle. The method was found to be useful when the proportion of the fibre class of interest was low. The statistical distribution of this measure was investigated by computer simulation using a hexagonal lattice model of muscle fibre arrangement. An approximate significance test was developed by considering the extreme points of the distribution. Minor changes to the hexagonal lattice model were incorporated to give a more realistic representation of fibre arrangement and these were found to give very similar results to the simpler model.

Clinical progression of mitochondrial myopathy is associated with the random accumulation of cytochrome c oxidase negative skeletal muscle fibres.

We studied the accumulation of cytochrome c oxidase (COX)-negative skeletal muscle fibres in six patients with a myopathy due to a mitochondrial DNA (mtDNA) defect. Each patient was biopsied on two or more occasions over a period of 3-15 years. Progressive proximal weakness was associated with an increase in the proportion of COX-negative fibres. These fibres were arranged randomly, indicating that each fibre became COX negative independently of the status of neighbouring fibres. The clinical progression of mtDNA myopathy is therefore a consequence of a biochemical defect that develops independently within individual muscle fibres. It is likely that this is due to the clonal expansion of mutant mtDNA.