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Whole-exome sequencing studies have recently identified novel genes implicated in normal- or low-GGT pediatric cholestasis including ubiquitin-specific peptidase 53 (USP53). We identified novel biallelic mutations in the USP53 gene in a 7-month-old infant with pruritus and progressive intrahepatic cholestasis. His liver biopsy showed portal and perivenular fibrosis with bland bilirubinostasis. His parents were asymptomatic heterozygous for the same mutation. He is currently on vitamin supplements and cholestyramine and his family has also been counseled for liver transplantation. Our report confirms that patients with biallelic mutation in USP53 develop cholestatic liver disease.Throughout the COVID-19 pandemic, concerns have been raised about an 'infodemic', with information and misinformation being spread across multiple channels and mediums. Information consumption has also been associated with increased anxiety throughout the pandemic. Thus, the present study investigates the mediating role of state anxiety on the relationship between information consumption (defined as mean frequency of information consumption multiplied by number of information sources) and COVID-19 protective behaviours. We compare results across Australian and United States samples and account for personal risk perception and belief in misinformation about COVID-19. this website Cross-sectional data collected between 28 and 30 April 2020 were analysed using Bayesian structural equation modelling among participants from Australia (N = 201), and the United States (N = 306). State anxiety scores were above the conventional clinical cut-off. Information consumption was positively associated with state anxiety, personal risk perception, and COVID-19 protective behaviours in the Australian and the United States samples. Additionally, the relationship between information consumption and COVID-19 protective behaviours was positively mediated by state anxiety in both nations, suggesting some functional benefits of anxiety. Differences in risk perception and belief in misinformation existed between the Australian and United States sample. Findings provide support for current guidance from organisations such as the WHO, APA, and APS on limiting information consumption to reduce anxiety. To effectively communicate critical public health messaging while minimising potential burdens on mental health, there is a need to develop and test interventions that assist people in calibrating the extent and nature of their information consumption.

Open or endovascular treatment of popliteal artery aneurysms (PAAs) is still debated. Data about the popliteal artery anatomy and its branches are essential to plan a surgical approach. The aim of this study was to report the anatomical variations of the popliteal artery and its branches in a population with aneurysmal disease and compare them with a standard population with non-aneurysmal disease.

A retrospective review of consecutive patients who underwent surgical PAA repair in our center between January 2011 and December 2020 was performed. One-hundred-forty-six limbs in 128 patients underwent PAA treatment (Group 1). Computed tomography angiography images using a 128-section configuration were reviewed for anatomical variations of the popliteal artery and its branches. A control population of 178 limbs in 89 patients with non-aneurysmal disease was used to compare the outcomes (Group 2). All limbs were classified according to Kim's classification. The two groups were analyzed and compared by means ofons of the popliteal artery and its branches is mandatory in case of the surgical approach. Anatomy in PAA patients is not different. Studies with larger population size are needed to validate these outcomes.

To evaluate the predictors of mortality, mortality rate, and causes of death in patients with lupus nephritis (LN) depending on final renal function.

The cohort included 401 Korean patients diagnosed with LN between 1985 and 2019. We retrospectively analyzed the clinical and laboratory indices, treatment response, and the final renal function. The final renal function was defined by the last stable level of eGFR measured in an out-patient department more than 3 times before death occurred and was categorized into five groups depending on CKD stage.

The median follow-up time after the diagnosis of LN was 131months. No difference in baseline demographic characteristics and laboratory findings was found except for the proportion of Hb less than 10mg/dl and baseline eGFR (

= 0.011 and 0.037). We found no significant differences in therapeutic parameters, but all the response parameters including treatment response at 6months (

= 0.004) and 12months (

= 0.004), time to remission (

< 0.001), final determinant of mortality in Korean patients with LN. SLE flare also caused mortality in patients with LN who required maintenance dialysis, suggesting the benefit of KT on lupus activity and survival.

To assess the relationship between the presence of the sonographic finding of chorionic bump (CB) during first trimester and miscarriage rate or other perinatal outcomes.

PubMed, Medline, Embase, Cinahl and Clinicaltrials.gov databases, from inception to April 8, 2021 were explored utilizing combinations of the relevant medical subject heading (MeSH) terms, key words, and word variants for "CB" and "pregnancy." Prospective and retrospective case-control studies were eligible for inclusion. Odds ratios (ORs) comparing obstetrical outcomes among pregnancies with CB and normal pregnancies were determined with 95% confidence intervals (CI) using random-effects models. Primary outcome of interest was miscarriage rate. Secondary outcomes were alive newborns (ANB) rate, adverse pregnancy outcomes (APO) and vaginal bleeding. Quality assessment of the included studies was performed using the Newcastle-Ottawa Scale.

Five studies including 173 pregnant women with CB (study group) and 1,263 pregnant women without Cquires a close surveillance throughout pregnancy to prevent long-term complications and provide adequate counseling to the patient.

The presence of CB at first trimester ultrasound significantly increases the risk of miscarriage and APO, and intact the rate of ANB. This article is protected by copyright. All rights reserved. Key message The presence of CB on early first trimester ultrasound increases three-fold the risk of miscarriage and adverse pregnancy outcomes and reduces the rate of alive newborns. It is important to consider CB as an ultrasound marker that requires a close surveillance throughout pregnancy to prevent long-term complications and provide adequate counseling to the patient.An efficient stereoselective total synthesis of (3R, 4S)-4-hydroxylasiodiplodin has been accomplished starting from known starting materials. The key steps involved in this synthesis are Stille cross coupling, alkylation of 1,3-dithiane and Yamaguchi macrolactonization.Self-management support has been identified as an effective nursing intervention for improving outcomes for people with chronic conditions, yet this concept lacks a clear definition. Furthermore, the concept has not been used in school nursing literature despite the clear connection between school nursing practice and tenets of self-management support. Additionally, the concept has not been explored in the context of difficult-to-manage mental health concerns, such as psychogenic nonepileptic seizures. A conversion disorder in which seizure events in the absence of abnormal brainwave activity result from stress, psychogenic nonepileptic seizures affect the quality of life and school experience for students experiencing them and could be addressed through self-management support. This hybrid concept analysis included a review of extant literature and semi-structured interviews with school nurses to ascertain a definition of self-management support in the context of school nursing using care of students with psychogenic nonepileptic seizures as an exemplar.

Moral distress, defined as moral suffering or a psychological imbalance, can affect nursing students. However, many new instruments or adaptations of other scales that are typically used to measure moral distress have not been used for nursing students.

This study aimed to translate, culturally adapt and evaluate the psychometric properties of an Italian version of the Moral Distress Scale for Nursing Students (It-ESMEE) for use with delayed nursing students (students who could not graduate on time or failed the exams necessary to progress to the next level).

The study used a cross-sectional research design.

Incidental sampling resulted in a sample of 282 delayed nursing students (mean age = 26.73 ± 4.43 years, 73% female) enrolled between May and August 2020 in a University of central Italy.

The research protocol was approved by the internal review board of the university, and all participants provided their written informed consent.

The study confirmed a multidimensional second-order factorial s, and to ensure the quality of nurse education.Eight phenolic compounds were isolated from Eugenia pyriformis leaves fraction by semi-preparative HPLC and characterized by Nuclear Magnetic Resonance (NMR) and mass spectrometry (ESI-MS). Five compounds were isolated and identified for the first time in E. pyriformis species, while this is the first report of the accumulation of isoquercitrin, quercitrin, and the aglycone quercetin in its leaves. E. pyriformis leaves and fruits extracts, as well as the compounds isolated from the leaves most active fraction, were evaluated for their antiglycation and antioxidant activities. The mixture of myricetin-3-O-(2″-O-galloyl)-α-L-rhamnoside and myricetin-3-O-(4″-O-galloyl)-α-L-rhamnoside showed the highest antiglycation activity. These results suggest that this species is a promising source of bioactive compounds. Further studies to investigate the inhibition of the glycation process in vivo are necessary to evaluate its use in the treatment and/or prevention of advanced glycation end-products (AGEs)-associated diseases.Background Apixaban pharmacokinetic properties and some clinical reports suggest cessation 48 hours prior to surgery is safe, but this has not been demonstrated in a naturalistic setting. We sought to measure the residual apixaban exposure in patients who had apixaban held as part of standard of care perioperative management. Methods This was a prospective, observational study of patients in whom apixaban plasma concentration and anti-Xa activity were measured while at steady state apixaban dosing and again immediately prior to surgery. Clinical management of cessation and resumption of apixaban was at the discretion of the treating physician. Results Paired blood samples were provided by 111 patients. Ninety-four percent (104/111) of patients had measured apixaban concentrations of ⩽ 30 ng/mL. Only one patient had a value > 50 ng/mL. The median time between the self-reported last dose and presurgery blood sampling was 76 hours (range 32-158) for those who achieved concentrations ⩽ 30 ng/mL and 59 hours (range 49-86) for those > 30 ng/mL. Measured anti-Xa activity correlated well with apixaban exposure. Clinically significant nonmajor bleeding was reported in one patient at 1 week postsurgery. There was one venous thromboembolic event and one stroke in the perioperative period. Conclusion In a naturalistic setting with a heterogeneous patient population, apixaban discontinuation for at least 48 hours before a procedure resulted in a clinically insignificant degree of anticoagulation prior to a surgical procedure. ClinicalTrials.gov Identifier NCT02935751.

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