A study of the magazine-reading interests of secondary-school pupils

Thesis (M.A.)--Boston University, 1950. This item was digitized by the Internet Archive

Constructing indices representing supportiveness of the physical environment for walking using the Rasch measurement model

BACKGROUND: The objectives of this study were to use the Rasch model to 1) assess the psychometric properties of a physical environmental audit instrument and 2) to develop indices of interrelated environmental attributes that summarize environmental supportiveness for walking. METHODS: A set of items were derived representing two conceptual physical environmental constructs: 1) functional/safety, and; 2) aesthetics. Ad hoc criteria based on point-biserial and Rasch-based fit statistics were used to examine the construct validity and internal reliability of the two constructs. RESULTS: The Rasch-based fit statistics assisted in identifying 12 items that belonged to the functional/safety construct and 4 items that belonged to the aesthetic construct. The reliability of the two constructs were low to moderate (functional/safety r(β )= 0.19 and aesthetics r(β )= 0.35). CONCLUSION: Given the vast number of built environmental attributes, a means of developing summary indices is essential. Future studies should assess the reliability and validity of indices that summarize physical environmental characteristics conducive to walking before testing them in predictive models of physical activity. More research examining procedures for measuring the built environment and techniques for analyzing environmental data are needed to guide future research in this area

Fatal high-grade skull osteosarcoma 30 years following radiotherapy for Cushing's disease

Cushing’s disease is a rare disorder characterised by excessive cortisol production as a consequence of a corticotroph pituitary tumour. While the primary treatment is surgical resection, post-operative radiation therapy may be used in cases of ongoing inadequate hormonal control or residual or progressive structural disease. Despite improved outcomes, radiotherapy for pituitary tumours is associated with hypopituitarism, visual deficits and, rarely, secondary malignancies. We describe an unusual case of a 67-year-old female with presumed Cushing’s disease diagnosed at the age of 37, treated with transsphenoidal resection of a pituitary tumour with post-operative external beam radiotherapy (EBRT), ketoconazole for steroidogenesis inhibition, and finally bilateral adrenalectomy for refractory disease. She presented 30 years after her treatment with a witnessed generalised tonic-clonic seizure. Radiological investigations confirmed an extracranial mass infiltrating through the temporal bone and into brain parenchyma. Due to recurrent generalised seizures, the patient was intubated and commenced on dexamethasone and anti-epileptic therapy. Resection of the tumour revealed a high-grade osteoblastic osteosarcoma. Unfortunately, the patient deteriorated in intensive care and suffered a fatal cardiac arrest following a likely aspiration event. We describe the risk factors, prevalence and treatment of radiation-induced osteosarcoma, an exceedingly rare and late complication of pituitary irradiation. To our knowledge, this is the longest reported latency period between pituitary irradiation and the development of an osteosarcoma of the skull

Impulse control disorders in dopamine agonist-treated hyperprolactinemia: Prevalence and risk factors

Context: There are growing reports of dopamine agonist (DA)-induced impulse control disorders (ICDs) in hyperprolactinemic patients. However, the magnitude of this risk and predictive factors remain uncertain. Objective: To determine ICD prevalence and risk factors in DA-treated hyperprolactinemic patients compared to community controls. Design, Setting and Participants: Multicenter cross-sectional analysis of 113 patients and 99 healthy controls. Main Outcome Measures: Participants completed a neuropsychological questionnaire consisting of the Depression Anxiety Stress Scale (DASS21), Questionnaire for Impulsive-Compulsive Disorders in Parkinson’s disease (QUIP-S), Hypersexual Behavior Inventory (HBI), Hypersexual Behavior Consequences Scale and Social Desirability Response Set Scale. Demographic and clinical data were collated to determine ICD risk factors. Patients testing positive for an ICD were offered a semi-structured psychological interview. Results: Patients were more likely than controls to test positive by QUIP-S for any ICD (61.1 vs 42.4%, P=0.01), hypersexuality (22.1 vs 8.1%, P=0.009), compulsive buying (15.9 vs 6.1%, P=0.041) and punding (18.6 vs 6.1%, P=0.012), and by HBI for hypersexuality (8.0 vs 0.0%, P=0.004). Independent risk factors were male sex (OR 13.85), eugonadism (OR 7.85), Hardy’s tumor score, and psychiatric comorbidity (OR 6.86) for hypersexuality; and age (OR 0.95) for compulsive buying. DASS21 subset scores were higher in patients vs controls, and in patients with vs without different ICDs. Only 19/51 (37.3%) interviewed patients were aware of the relationship between DAs and ICDs before the study. Conclusions: DA therapy poses a high, previously underestimated risk of ICDs, especially in the form of hypersexuality in eugonadal men

The vitamin D binding protein axis modifies disease severity in Lymphangioleiomyomatosis

Background: Lymphangioleiomyomatosis (LAM) is a rare disease of women. Decline in lung function is variable making appropriate targeting of therapy difficult. We used unbiased serum proteomics to identify markers associated with outcome in LAM. Methods: 101 women with LAM and 22 healthy controls were recruited from the National Centre for LAM (Nottingham, UK). 152 DNA and serum samples with linked lung function and outcome data were obtained from patients in the NHLBI LAM Registry (USA). Proteomic analysis was performed on a discovery cohort of 50 LAM and 20 control sera using a SCIEX SWATH mass spectrometric workflow. Protein levels were quantitated by ELISA and SNPs in GC encoding Vitamin D Binding Protein (VTDB) genotyped. Results: Proteomic analysis showed VTDB was 2.6 fold lower in LAM than controls. Serum VTDB was lower in progressive compared with stable LAM (p=0.001) and correlated with diffusing capacity (p=0.01). Median time to death or lung transplant was reduced by 46 months in those with CC genotypes at rs4588 and 38 months in those with non-A containing haplotypes at rs7041/4588 (p=0.014 and 0.008 respectively). Conclusions: The VTDB axis is associated with disease severity and outcome, and GC genotype could help predict transplant free survival in LAM

Prosthetics services in Uganda : a series of studies to inform the design of a low cost, but fit-for-purpose, body-powered prosthesis

The majority of people with upper limb absence (PWULA) live in lower, or middle-income countries (LMICs). However, efforts to develop improved prostheses have largely focused on electrically powered devices, sustainable deployment of which, in LMICs, is difficult. In the ‘Fit-for-purpose, affordable body-powered prostheses’ project, teams from the UK, Uganda and Jordan are developing mechanically-operated prostheses, optimised for LMICs, and establishing local methods for fabrication, fitting and evaluation. Here we first report on preliminary studies aimed at grounding the project in the reality of current prosthetics services and the experiences of people with limb absence in Uganda. Finally, we outline our ongoing work in the context of our findings. In our first two studies we reviewed current prosthetics and associated repair services. An issue which came up repeatedly was the difficulty faced by orthopaedic technologists in accessing componentry/materials. All specialised prosthetics components and materials are imported, often at a high cost. Purchasing does not appear to be well coordinated between centres, meaning potential economies of scale are not being fully exploited. Although there is supposed to be government funding for prosthetics, in practice budgets are often inadequate and a reliance on donations is common. The resource limitations mean Orthopaedic Technologists often resort to ad-hoc solutions; unsurprisingly perhaps, failures in prostheses were reported. In particular, lamination-based socket manufacture is very difficult, given the complexity (and cost) of the processes involved. Repair services are also limited, in part also due to problems accessing materials/components. Despite (or in part, as a result of) these challenges, the orthopaedic technologists are generally an extremely resourceful and multi-skilled group and there is genuine enthusiasm to see services improve. Further, there is a growth in interest and capabilities in the area of medical device innovation. In the third of our studies, we interviewed 17 PWULA and present preliminary results from the analysis of a subset of five participants. Firstly, we found that only 2 of the participants reported experience with using an upper limb prosthesis, again supporting the picture which emerged from the other studies. The findings illustrate the emergence of four key themes: a) attitude towards disability; b) barriers to prosthesis use; c) coping without a prosthesis; and d) communication with other PWULA. Although attitudes to those with limb loss varied, participants reported impacts in terms of social isolation and a mixed experience of emotions that appeared predominantly negative; barriers to prosthesis use were broader than just cost and functionality, and included a lack of training and psychological support; given that it is difficult to access an upper limb prosthesis, PWULA have found ways to perform daily life activities without relying on one; finally, most PWULA find the suggestion of communicating with other people with the same experience appealing. In our project we are addressing some of the issues found in the preliminary studies. To make socket manufacture less dependent on access to imported materials and specialised equipment, we are investigating the development of lattice-style, adjustable sockets, made from locally available materials. We are also investigating alternatives to the traditional harness-controlled, body-powered prosthetic hands. Given that clinicians have no objective means of evaluating the value of the prosthesis to their clients, we are testing the use of low-cost digital monitoring tools. We are also exploring the potential value of using mobile-phones to reduce the isolation of PWULA. Finally, we are exploring how these innovations may be translated into the Ugandan health setting

Chicken IgL gene rearrangement involves deletion of a circular episome and addition of single nonrandom nucleotides to both coding segments

Chicken immunoglobulin light chain (IgL) gene rearrangement has been characterized. Rearrangement of the single variable (VL) segment with the single joining (JL) segment within the chicken IgL locus results in the deletion of the DNA between VL and JL from the genome. This deletion is accomplished by a molecular mechanism in which a precise joining of the IgL recombination signal sequences leads to the formation of a circular episomal element. The circular episome is an unstable genetic element that fails to be propagated during B cell development. Evidence was obtained that the formation of the circular episome is accompanied by the addition of a single nonrandom base to both the VL and JL coding segments. The subsequent joining of the VL and JL segments appears to occur at random, as we observed at least 25 unique V-J junction sequences, 11 of which are out-of-frame. A novel recombination mechanism that accounts for the observed features of chicken IgL gene rearrangement is discussed.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/28019/1/0000455.pd

The vitamin D binding protein axis modifies disease severity in lymphangioleiomyomatosis

Background: Lymphangioleiomyomatosis (LAM) is a rare disease of women. Decline in lung function is variable making appropriate targeting of therapy difficult. We used unbiased serum proteomics to identify markers associated with outcome in LAM.Methods: 101 women with LAM and 22 healthy controls were recruited from the National Centre for LAM (Nottingham, UK). 152 DNA and serum samples with linked lung function and outcome data were obtained from patients in the NHLBI LAM Registry (USA). Proteomic analysis was performed on a discovery cohort of 50 LAM and 20 control sera using a SCIEX SWATH mass spectrometric workflow. Protein levels were quantitated by ELISA and SNPs in GC encoding Vitamin D Binding Protein (VTDB) genotyped.Results: Proteomic analysis showed VTDB was 2.6 fold lower in LAM than controls. Serum VTDB was lower in progressive compared with stable LAM (p=0.001) and correlated with diffusing capacity (p=0.01). Median time to death or lung transplant was reduced by 46?months in those with CC genotypes at rs4588 and 38?months in those with non-A containing haplotypes at rs7041/4588 (p=0.014 and 0.008 respectively).Conclusions: The VTDB axis is associated with disease severity and outcome, and GC genotype could help predict transplant free survival in LAM

Patient/family views on data sharing in rare diseases: study in the European LeukoTreat project.: Survey assessing data sharing in leukodystrophies

International audienceThe purpose of this study was to explore patient and family views on the sharing of their medical data in the context of compiling a European leukodystrophies database. A survey questionnaire was delivered with help from referral centers and the European Leukodystrophies Association, and the questionnaires returned were both quantitatively and qualitatively analyzed. This study found that patients/families were strongly in favor of participating. Patients/families hold great hope and trust in the development of this type of research. They have a strong need for information and transparency on database governance, the conditions framing access to data, all research conducted, partnerships with the pharmaceutical industry, and they also need access to results. Our findings bring ethics-driven arguments for a process combining initial broad consent with ongoing information. On both, we propose key item-deliverables to database participants