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Health-related quality of life (HRQoL) is difficult to measure in rare diseases, especially in paediatric populations, yet capturing HRQoL is critical to evaluating treatment, including the cost-effectiveness of treatments. Given the ultra-rare nature of AADC deficiency indirect elicitation of HRQoL data through proxy caregiver/parent ratings is not feasible. In these circumstances, HRQoL data may be derived through vignette studies using the general population. The aim of the study was to generate health utility values specific for France for AADC deficiency using vignettes.

The study was completed online by panel participants from a French representative sample. Five health state vignettes, reflecting key milestones in the eladocagene exuparvovec clinical trials and economic model, were presented to the participants "bedridden", "head control", "sitting unsupported", "standing with assistance" and "walking with assistance". The vignettes had been previously developed with input from parents of patients with AADC deficiency, patients and expert opinion. Participants also completed the Health Utilities Index-3 for the "bedridden" health state.

A total of 1001 participants (51% females; mean age 46 years) completed the vignettes. Utilities increased linearly as the health state improved for both the time trade-off (TTO) 0.47 (standard deviation, SD 0.36) to 0.54 (SD 0.36) and standard gamble (SG) 0.61 (SD 0.29) to 0.67 (SD 0.27). A significant minority had incongruent responses (high utilities for the bedridden compared to walking health states) for the vignette (27%). When these were removed, the TTO health utilities (N=729) ranged from 0.39 (SD 0.36) to 0.56 (SD 0.38) and 0.61 (SD 0.30) to 0.69 (SD 0.27) for the SG.

Health utilities were derived for AADC deficiency which will be used for a cost-effectiveness model of an AADC deficiency treatment.
Health utilities were derived for AADC deficiency which will be used for a cost-effectiveness model of an AADC deficiency treatment.
Poor sleep quality is a common problem among middle-aged women. Few studies, however, have assessed differences in sleep quality among premenopausal, perimenopausal, and postmenopausal women and related risk factors in Korea women. The aim of this study was to assess sleep quality and factors associated therewith according to menopausal status in Korean women.

This study was based on the 2016 Korean Study of Women's Health Related Issues (K-Stori), a cross-sectional survey employing nationally representative random sampling. In total, 3000 Korean women aged 45 to 64 years completed the Pittsburgh Sleep Quality Index (PSQI). Comparison of demographic characteristics and sleep quality among pre-, peri-, and postmenopausal women was conducted.

Among the participants, 26% suffered from poor sleep quality based on the PSQI. The prevalence of poor sleep quality increased with later menopausal stage (from 18.8% in the premenopausal stage to 29.5% in the postmenopausal stage
<0.001). Multivariate logisticopausal transition is required.
The effects of sleep restriction on subjective alertness, motivation, and effort vary among individuals and may explain interindividual differences in attention during sleep restriction. We investigated whether individuals with a greater decrease in subjective alertness or motivation, or a greater increase in subjective effort (versus other participants), demonstrated poorer attention when sleep restricted.

Fifteen healthy men (M±
, 22.3±2.8 years) completed a study with three nights of 10-hour time in bed (baseline), five nights of 5-hour time in bed (sleep restriction), and two nights of 10-hour time in bed (recovery). selleck chemicals llc Participants completed a 10-minute psychomotor vigilance task (PVT) of sustained attention and rated alertness, motivation, and effort every two hours during wake (range 3-9 administrations on a given day). Analyses examined performance across the study (first two days excluded) moderated by per-participant change in subjective alertness, motivation, or effort from baseline to sleep restrm poorly during sleep restriction, while those reporting additional effort demonstrate impaired vigilance.
Vigilance declines during sleep restriction regardless of change in subjective alertness or motivation, but individuals with reduced motivation exhibit poorer inhibition. Individuals with preserved subjective alertness still perform poorly during sleep restriction, while those reporting additional effort demonstrate impaired vigilance.
Given the scarcity of data on sleep insufficiency in developing countries and its emerging importance in public health, this study aims to provide the prevalence and factors associated with insufficient sleep among working Malaysian adults.

A total of 11,356 working adults (≥18 years) were recruited from a cross-sectional online health and work survey. Sleep duration was self-reported and was categorised as insufficient sleep (<7 h) and sufficient sleep (≥7 h) based on the National Sleep Guidelines' daily sleep time recommendation for adults. The 10-item Kessler scale was used to assess psychological distress levels (cutoff score ≥20). Bivariate and multivariable regression analyses were performed to examine the factors associated with insufficient sleep.

The mean age of the participants was 35.7 years (standard deviation, SD±9.2). There were more female (n=6613; 58.2%) respondents. The mean sleep duration was 6.49 h (SD±1.10). A total of 6212 individuals (54.7%) reported getting <7 h of sleep daig, and high levels of psychological distress were significantly associated with insufficient sleep. Our findings recognise sleep as an emerging public health issue. Smoking cessation programmes and addressing high psychological distress may be beneficial to improve sleep.
To evaluate the clinical efficacy of hybrid surgery for Stanford type A aortic dissection.

Twenty-two patients with Stanford type A aortic dissection were selected. All patients had completed or undergone hybrid surgery, including extracorporeal circulation, treatment of proximal anastomosis of ascending aorta and the distal anastomosis of the ascending aorta, management of the branch vessels on the arch, aortic endovascular repair. This study analyzed the time of surgery and awake, blood transfusion during surgery, patient's drainage, complications and CTA of aorta was re-examined about one month after operation during patients follow-up.

All patients underwent the operation successfully. One patient died of renal failure after the operation. Two patients experienced postoperative neurological complications (anxiety and delirium). Renal function was abnormal in two patients, and one patient needed bedside blood filtration. The serum creatinine levels temporarily increased in seven patients. No stent migration was found during patient follow-up.
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