Psychometric Analysis of the Adult Sickle Cell Quality of Life Measurement Information System (ACSQ-Me) in a UK Population

Background: The Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-Me) has been shown to be a reliable and valid questionnaire measuring health-related quality of life (HRQoL) in the US sickle cell disease (SCD) population. The study objective was to test the validity and reliability of the ASCQ-Me for use in the UK. Methods: The US ASCQ-Me, Hospital Anxiety and Depression Scale (HADS), self-reported symptoms, and Medical Outcome Survey Short Form 36 (SF-36) were administered to 173 patients with SCD. Clinical severity was assessed by the number of painful episodes indicated by hospital admissions. Results: The results showed that the item banks of the UK ASCQ-Me had good internal consistency. Anxiety and depression were strongly correlated with the emotional, and social item banks of the UK ASCQ-Me, with moderate correlations between the UK ASCQ-Me item banks and SF-36 components suggesting convergent validity. A confirmatory factor analysis confirmed the conceptual framework of the scale as being the same as the US ASCQ-Me, indicating construct validity. Known groups validity was found, with the ASCQ-Me being able to differentiate by SCD severity groups. Conclusion: The analysis of the sample shows evidence of both validity and reliability of the ACCQ-Me for use in the UK SCD population

Non-invasive or minimally invasive autopsy compared to conventional autopsy of suspected natural deaths in adults: a systematic review

Objectives: Autopsies are used for healthcare quality control and improving medical knowledge. Because autopsy rates are declining worldwide, various non-invasive or minimally invasive autopsy methods are now being developed. To investigate whether these might replace the invasive autopsies conventionally performed in naturally deceased adults, we systematically reviewed original prospective validation studies. Materials and methods: We searched six databases. Two reviewers independently selected articles and extracted data. Methods and patient groups were too heterogeneous for meaningful meta-analysis of outcomes. Results: Sixteen of 1538 articles met our inclusion criteria. Eight studies used a blinded comparison; ten included less than 30 appropriate cases. Thirteen studies used radiological imaging (seven dealt solely with non-invasive procedures), two thoracoscopy and laparoscopy, and one sampling without imaging. Combining CT and MR was the best non-invasive method (agreement for cause of death: 70 %, 95%CI: 62.6; 76.4), but minimally invasive methods surpassed non-invasive methods. The highest sensitivity for cause of death (90.9 %, 95%CI: 74.5; 97.6, suspected duplicates excluded) was achieved in recent studies combining CT, CT-angiography and biopsies. Conclusion: Minimally invasive autopsies including biopsies performed best. To establish a feasible alternative to conventional autopsy and to increase consent to post-mortem investigations, further research in larger study groups is needed. Key points: • Health care quality control benefits from clinical feedback provided by (alternative) autopsies. • So far, sixteen studies investigated alternative autopsy methods for naturally deceased adults. • Thirteen studies used radiological imaging modalities, eight tissue biopsies, and three CT-angiography. • Combined CT, CT-angiography and biopsies were most sensitive diagnosing cause of death

Batch Scheduling for Energy-Efficient Sensing in Smartphones

Sensing in smartphones consumes a significant amount of energy and leads to quick depletion of the battery. Most of the existing solutions to overcome the short battery lifetime caused by periodic sensing are personalized. They tend to learn and predict the user activities. Thus, fewer samples are required to recognize user state and sensing intervals can be extended. However, such methods require a training phase and any change in the user pattern causes a need for a new training phase. Therefore, in addition to personalized learning methods, we also need user-agnostic techniques that guarantee instant energy savings independently of the context to be recognized. In this thesis a user-agnostic method that seeks to provide energy efficiency in sensing is proposed. Our approach is based on the observation that an energy overhead occurs every time the CPU is woken up to perform a sensor sampling task. Hence, our goal is to decrease the number of CPU wake-ups incurred due to periodic sampling by combining multiple sensing actions into one joint activity. Our contribution is a mechanism that batches the execution of periodic tasks. BASS (BAtch Scheduler for Sensing) uses the greatest common divisor of the time intervals defined for the sensor sampling tasks. It also introduces a flexibility factor that implies the time delay tolerance regarding the execution of a task. Moreover, our tool implements a detection method for CPU wake-ups caused by any other application or the user. Based on the above, BASS applies batch scheduling to execute the sensor sampling tasks in batches and result in fewer CPU wake-ups. We evaluated our mechanism using a sensing application for monitoring patients that suffer from Rheumatic Arthritis. We conducted a number of experiments on an HTC Sensation phone, which showed that the efficient exploit of CPU wake-ups cuts down the energy consumption in mobile sensing. The BASS tool achieved an average power reduction of up to 44% and 18% in laboratory and real-world experiments respectively, in our application scenario, without compromising sensing time accuracy.Embedded SystemsSoftware and Computer TechnologyElectrical Engineering, Mathematics and Computer Scienc

Bone Marrow Failure in Fanconi Anemia: Clinical and Genetic Spectrum in a Cohort of 20 Pediatric Patients

Prognostic refinement in Fanconi anemia (FA) is needed, especially when considering allogeneic hematopoietic stem cell transplantation (HCT). We studied 20 children with FA and bone marrow failure from a single center. According to Hôpital Saint-Louis risk classification for FA, patients were classified in stage A (no or mild cytopenia/dysplasia), B (single non-high-risk cytogenetic abnormality), C (severe cytopenia and/or significant dysplasia and/or high-risk cytogenetic abnormality), and D (myelodysplastic syndrome with excess of blasts/acute myeloid leukemia) in 4, 2, 13, and 0 cases, respectively. Nine patients received androgens +/- steroids, with a response rate of 30%, and 11 patients underwent HCT. Ten-year cumulative incidence (CI) of myelodysplastic syndrome/acute myeloid leukemia and overall survival (OS) were 21.9% and 45.3%, respectively, in the entire cohort, whereas cumulative incidence of transplantation-related mortality and OS were 27% and 63%, respectively, in patients who underwent HCT. Patients with significant dysplasia at diagnosis (stages C and D) had significantly shorter OS post-HCT as compared with patients without dysplasia. All patients in stages C and D at diagnosis or during evolution died from their disease. HCT in recent years was associated with more favorable outcomes. Larger cohorts could validate homogenous reporting of risk and help decision-making, particularly for HCT. © 2019 Wolters Kluwer Health, Inc. All rights reserved