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cess and failure, and define core and adaptable components of the SAIA-HTN intervention. The Organizational Readiness for Implementing Change scale will measure facility-level readiness for adopting SAIA-HTN. DISCUSSION SAIA packages user-friendly systems engineering tools to guide decision-making by front-line health workers to identify low-cost, contextually appropriate chronic care improvement strategies. By integrating SAIA into routine hypertension screening and management structures, this pragmatic trial is designed to test a model for national scale-up. TRIAL REGISTRATION ClinicalTrials.gov NCT04088656 (registered 09/13/2019; https//clinicaltrials.gov/ct2/show/NCT04088656).BACKGROUND Hereditary Transthyretin Amyloidosis Polyneuropathy is a rare life-threatening neurologic disease that imposes considerable mortality and it is associated with progressive related disabilities. In this study, we aimed to assess the effect of the disease across health-related quality of life dimensions, in both carriers of the mutation and patients, to compare health-related quality of life with general population, as well as to explore health-related quality of life prognostic factors among patients, including disease progression and treatment. METHODS This study was a multi-institutional, longitudinal, prospective, observational study of hereditary Transthyretin Amyloidosis Polyneuropathy Portuguese adult subjects (621 asymptomatic carriers and 733 symptomatic patients) enrolled in the Transthyretin Amyloidosis Outcomes Survey. Health-related quality of life was captured with the preference-based instrument EQ-5D-3 L. For general population the dataset included all subjects enrolled in a representy Transthyretin Amyloidosis Polyneuropathy symptoms and progressive associated disabilities substantially decrease patient's health-related quality of life. Clinical strategies focused on health-related quality of life preservation such as close follow-up of asymptomatic carriers, prompt diagnosis and adequate, early treatment would benefit patient's long-term outcomes, slowing the progressive decline in health-related quality of life.BACKGROUND Although studies on the effectiveness of the use of ICU diaries on psychiatric disorders and quality of life have been published, the results still seem to be controversial. The study aimed to determine the effects of using an ICU diary on psychiatric disorders, sleep quality, and quality of life (QoL) in adult ICU survivors in China. METHODS One hundred and twenty-six patients who underwent a scheduled cardiac surgery and were expected to stay ≥ 24 h in ICU were randomized to two groups (63 in each group). The patients in the intervention group received the use of ICU diaries during the period of post-ICU follow-up, while the patients in the control group received usual care without ICU diaries. The primary outcome was significant PTSD symptoms (Chinese version of Impact of Event Scale-Revised, IES-R; total score ≥ 35 was defined as significant PTSD symptoms) and its severity in patients 3 months post-ICU. read more The secondary outcomes included memories of the ICU at 1 month, QoL (Medical Outcomes Study 3 months post-ICU, while it significantly improves the survivor's factual memory of ICU and sleep quality, and prevents the hyperarousal symptom. TRIAL REGISTRATION Chinese Clinical Trial Registry, ChiCTR-IOR-16009109, registered on 28 August 2016.BACKGROUND Pathophysiology of life-threatening acute metabolic decompensations (AMD) in propionic acidemia (PA) and isolated methylmalonic acidemia (MMA) is insufficiently understood. Here, we study the metabolomes of PA and MMA patients over time, to improve insight in which biochemical processes are at play during AMD. METHODS Longitudinal data from clinical chemistry analyses and metabolic assays over the life-course of 11 PA and 13 MMA patients were studied retrospectively. Direct-infusion high-resolution mass spectrometry was performed on 234 and 154 remnant dried blood spot and plasma samples of PA and MMA patients, respectively. link2 In addition, a systematic literature search was performed on reported biomarkers. All results were integrated in an assessment of biochemical processes at play during AMD. RESULTS We confirmed many of the metabolite alterations reported in literature, including increases of plasma valine and isoleucine during AMD in PA patients. We revealed that plasma leucine and phenylalanine, and urinary pyruvic acid were increased during AMD in PA patients. 3-hydroxyisovaleric acid correlated positively with plasma ammonia. We found that known diagnostic biomarkers were not significantly further increased, while intermediates of the branched-chain amino acid (BCAA) degradation pathway were significantly increased during AMD. CONCLUSIONS We revealed that during AMD in PA and MMA, BCAA and BCAA intermediates accumulate, while known diagnostic biomarkers remain essentially unaltered. This implies that these acidic BCAA intermediates are responsible for metabolic acidosis. Based on this, we suggest to measure plasma 3-hydroxyisovaleric acid and urinary ketones or 3-hydroxybutyric acid for the biochemical follow-up of a patient's metabolic stability.BACKGROUND High quality cardiopulmonary resuscitation is a key factor in survival with good overall quality of life after out-of-hospital cardiac arrest. Current evidence is predominantly based on studies conducted at low altitude, and do not take into account the special circumstances of alpine rescue missions. We therefore aimed to investigate the influence of physical strain at high altitude on the quality of cardiopulmonary resuscitation. METHODS Alpine field study. Twenty experienced mountaineers of the Austrian Mountain Rescue Service trained in Basic Life Support (BLS) performed BLS on a manikin in groups of two for 16 min. The scenario was executed at baseline altitude and immediately after a quick ascent over an altitude difference of 1200 m at 3454 m above sea level. link3 The sequence of scenarios was randomised for a cross over analysis. Quality of CPR and exhaustion of participants (vital signs, Borg-Scale, Nine hole peg test) were measured and compared between high altitude and baseline using random-effects linear regression models. RESULTS The primary outcome of chest compression depth significantly decreased at high altitude compared to baseline by 1 cm (95% CI 0.5 to 1.3 cm, p less then 0.01). There was a significant reduction in the proportion of chest compressions in the target depth (at least 5 cm pressure depth) by 55% (95% CI 29 to 82%, p less then 0.01) and in the duration of the release phase by 75 ms (95% CI 48 to 101 ms, p less then 0.01). No significant difference was found regarding hands-off times, compression frequency or exhaustion. CONCLUSION Physical strain during a realistic alpine rescue mission scenario at high altitude led to a significant reduction in quality of resuscitation. Resuscitation guidelines developed at sea level are not directly applicable in the mountain terrain.The long-term effect of average volume-assured pressure support (AVAPS) on health-related quality of life (HRQOL) in chronic obstructive pulmonary disease (COPD) patients with chronic hypercapnic respiratory failure (CHRF) remains unclear. The objective of this study is to identify the long-term effect of AVAPS in COPD patients with CHRF through assessment of HRQOL, exercise tolerance after six months duration. METHODS In this randomized, controlled, parallel-group study, 40 stable hypercapnic COPD patients were randomized in a 11 ratio to receive either spontaneous timed AVAPS (ST/AVAPS) (intervention) or Bilevel positive airway pressure (ST/BiPAP) (control). HRQL was measured with the Short Form 12 Health Survey Questionnaire (SF-12). Exercise tolerance assessed by 6 min walking distance. Analyses were done between groups from baseline to the average of six months measurements. RESULTS AVAPS led to significant 6 months improvements in several domains of (SF-12) compared to the control group, with the greatest improvement seen in general health [treatment effect of 8.2 points (95% confidence interval [95% CI 3.2 to 11.7; p = 0.001)], vitality (treatment effect 5.4 points [95% CI 1.4 to 9.3]; p = 0.001), physical functioning 5.5 points [95% CI 1.1 to 9.8]; p = 0.001) and bodily pain 5.1 points [95% CI 3.4 to 8.8]; p = 0.002). The physical health summary score improved by 3.7 points (95% CI 1.2 to 5.8; p = 0.001), but no significant improvement in the emotional or social role functioning, mental health subscale was noted. AVAPS also resulted in improvement 6 min walking distance 9.2 points (95% CI - 1 to - 15];p = 0.001). A significant reduction in the daytime (PaCO2) was observed after 6 months in those treated with AVAPS. CONCLUSIONS In COPD patients with hypercapnic respiratory failure, AVAPS improved exercise tolerance and multiple domains of HRQOL over six months of follow-up, with the significant improvement observed in general health.BACKGROUND District- and county-level maternal and child health hospitals (MCHHs) are positioned to provide primary maternal and child healthcare in rural and urban areas of China. Their efficiencies and productivity largely affect the equity and accessibility of maternal and child health care. This study aimed to assess the efficiency of district- and county-level MCHHs in China and identify their associated factors. METHODS Thirty-three district- and 84 county-level MCHHs were selected from Shanxi Province in 2017. At the first stage, bootstrapping data envelopment analysis (DEA) models were established to calculate the technical efficiency (TE), pure technical efficiency (PTE) and scale efficiency (SE) of district- and county-level hospitals. At the second stage, the estimated efficiency scores were regressed against external and internal hospital environmental factors by using bootstrap truncated regression to identify their determinants. RESULTS The average TE, PTE and SE scores for district-level MCHHs HHs. CONCLUSION The operational inefficiency of district- and county-level MCHHs in Shanxi Province is severe and needs to be substantially improved, especially in terms of TE and PTE. Hiring additional medical personnel and ensuring the stability of the workforce should be prioritised. The Chinese government must provide sufficient financial subsidy to compensate for service costs.Kaposi's sarcoma-associated herpesvirus (KSHV), also designated human herpesvirus 8 (HHV-8), has been linked to Kaposi's sarcoma, as well as to primary effusion lymphoma (PEL), and a subset of multicentric Castleman's disease. KSHV genomes are maintained as episomes within infected cells and the virus exhibits a biphasic life cycle consisting of a life-long latent phase during which only a few viral genes are expressed and no viral progeny are produced and a transient lytic reactivation phase, in which a full repertoire of ~ 80 lytic genes are activated in a temporally regulated manner culminating in the release of new virions. Lytic replication is initiated by a single viral protein, K-Rta (ORF50), which activates more than 80 viral genes from multiple resident viral episomes (i.e., viral chromosomes). One of the major targets of K-Rta is a long non-coding nuclear RNA, PAN RNA (polyadenylated nuclear RNA), a lncRNA that accumulates to exceedingly high levels in the nucleus during viral reactivation. K-Rta directly binds to the PAN RNA promoter and robustly activates PAN RNA expression.