Finally, POP1 mutations should be considered in familial cases with severe short stature even when skeletal dysplasia is not strongly evident. © 2020 Wiley Periodicals, Inc.In the Irish mental healthcare system, voluntarily admitted service users can request to leave hospital at any time. Following engagement with treating clinicians, service users remain as voluntary inpatients, are discharged, discharge themselves against medical advice, or are detained under legislation for the purposes of assessment. While detention conflicts with the principle of autonomy and a human rights-based approach, premature discharge is often associated with negative outcomes including reduced time to readmission, poorer quality of life, increased costs, and suicide. This study is a retrospective review of clinical records to identify factors associated with requests for premature discharge. Considerations of clinicians making the decision to detain the person or to support them through the discharge against medical advice process were also elucidated. Data were collected from clinical records of service users who requested discharge and were subsequently discharged against medical advice or detained involuntarily. Discharge against medical advice represented 3.5% of all discharges. The most frequent reasons for requests for discharge against medical advice were dissatisfaction with treatment, lack of engagement due to addiction, and leaving without notifying staff. Requests for discharge against medical advice frequently occurred out of hours, and nurses were the clinicians most likely to receive such requests. © 2020 Australian College of Mental Health Nurses Inc.OBJECTIVES Pediatric myelofibrosis is a rare entity with the largest reported series of 19 cases. https://www.selleckchem.com/products/homoharringtonine.html We describe here the clinicopathological spectrum and outcomes of 15 cases of pediatric myelofibrosis. METHODS Case files of myelofibrosis of patients less than 18 years were retrieved from January 2016 to January 2019, and patients with idiopathic myelofibrosis after exhaustive work-up were studied. Their clinicopathological profiles were studied and then followed up for resolution and malignant transformation. RESULTS Of the 15 cases of idiopathic myelofibrosis, transfusion-dependent anemia (14/15) was most common presentation. Only one patient showed leukoerythroblastosis with dacryocytes. Myeloid hyperplasia was seen in 13 of 15 patients and megakaryocytic hyperplasia in 10 patients. Dysmegakaryopoiesis was seen in 8 of 15 patients, and only three had small loose megakaryocytic clustering. None showed hyperchromatic megakaryocytes, intrasinusoidal hematopoiesis, or osteosclerosis. One patient with trisomy 8 tested positive for JAK2V617F. Bone marrow biopsy was hypercellular in 13, and 8 had world health organization (WHO) MF-3 fibrosis. None of the patients developed malignancy, one had spontaneous resolution, and one patient required allogenic stem cell transplant. CONCLUSIONS Pediatric myelofibrosis is a distinct entity from primary myelofibrosis in adults and merits mention in the WHO manual as a distinct entity. © 2020 Wiley Periodicals, Inc.Chromosomal instability (CIN) refers to the rate at which cells are unable to properly segregate whole chromosomes, leading to aneuploidy. Besides its prevalence in cancer cells and postulated implications in promoting tumorigenesis, studies in aneuploidy-prone mouse models uncovered an unanticipated link between CIN and aging. Using young to old-aged human dermal fibroblasts, we observed a dysfunction of the mitotic machinery arising with age that mildly perturbs chromosome segregation fidelity and contributes to the generation of fully senescent cells. Here, we investigated mitotic mechanisms that contribute to age-associated CIN. We found that elderly cells have an increased number of stable kinetochore-microtubule (k-MT) attachments and decreased efficiency in the correction of improper k-MT interactions. Chromosome mis-segregation rates in old-aged cells decreased upon both genetic and small-molecule enhancement of MT-depolymerizing kinesin-13 activity. Notably, restored chromosome segregation accuracy inhibited the phenotypes of cellular senescence. link2 Therefore, we provide mechanistic insight into age-associated CIN and disclose a strategy for the use of a small-molecule to inhibit age-associated CIN and to delay the cellular hallmarks of aging. © 2020 The Authors. Published under the terms of the CC BY 4.0 license.Starting from the commercially available dimethyl sulfide-gold(I) chloride complex (DMSAuCl) and diazonium salts in the presence of 2,6-di-tert-butyl-4-methylpyridine as base, symmetric and unsymmetric [C^N^C]AuIII Cl complexes were synthesized in a selective, photosensitizer-free, photochemical reaction using blue LED light. This new protocol provides the first mercury-free synthesis of these types of pincer-complexes in moderate-to-excellent yields, starting from a readily available gold(I) precursor. Owing to the extraordinary properties of the target compounds, like excellent luminescence and high anticancer activities, the synthesis of such complexes is a highly active field of research, which might make its way to an industrial application. Owing to the disadvantages of the known protocols, especially the toxicity and the selectivity issues in the case of unsymmetric complexes, avoiding the use of mercury, should further accelerate this ongoing development. © 2020 The Authors. Published by Wiley-VCH Verlag GmbH & Co. KGaA.OBJECTIVES We assessed the impact of diabetes mellitus (DM) on mortality after percutaneous coronary intervention (PCI) for left main stem (LMS) disease. Second, we compared mortality outcomes between non-insulin treated (NITDM) and insulin treated diabetes (ITDM) in different clinical settings. BACKGROUND There is a paucity of "real world" outcomes data in diabetic patients undergoing LMS PCI. METHODS We undertook a retrospective analysis of consecutive patients undergoing unprotected LMS PCI at 2 high volume tertiary centers. Diabetic status and clinical setting for PCI were recorded. The primary outcome measure was all-cause 30-day and long-term mortality (up to 36 months) post index PCI. RESULTS Between 2003 and 2017, 2,675 patients undergoing index LMS PCI were analyzed. Of those, 77.1% were non-DM, 15.8% NITDM, and 7.1% ITDM. Overall, DM status was not associated with higher 30-day mortality (OR 1.39, 95% CI 0.89-2.16, p = .15). During a median follow-up of 36 months, there was a borderline statistical association of DM with long-term mortality in all PCI settings (HR 1.31, 95% CI 1.00-1.71, p = .05). Compared to non-DM, ITDM but not NITDM was associated with short- and long-term mortality in all clinical presentations. CONCLUSIONS Overall, DM did not impact on 30-day mortality and had only a borderline statistical association with long-term mortality. It did not have an influence on mortality in non-emergency LMS PCI. The impact of DM on mortality outcomes following LMS PCI was only significant in the insulin treated patients. © 2020 The Authors. Catheterization and Cardiovascular Interventions published by Wiley Periodicals, Inc.It is highly desired to improve the visible-light activity of g-C 3 N 4 for H 2 evolution by constructing closely-contacted heterojunctions with conductive polymers. Herein, a polymer nanocomposite photocatalyst with high visible-light activity was successfully fabricated by coupling nano-sized polypyrrole (NPPy) particles onto g-C 3 N 4 nanosheets through a simple wet-chemical process and its visible-light activity is further improved by constructing Mg-O bridges between the NPPy and g-C 3 N 4 . The amount-optimized bridged nanocomposite displays a ~9-time visible-light activity improvement compared to g-C 3 N 4 . link3 Based on transient-state surface photovoltage responses and photoluminescence spectra, •OH amount evaluation and photoelectrochemical curves, it is concluded that the exceptional photoactivity is attributed to the significantly promoted charge transfer and separation along with visible photosensitization from NPPy. Interestingly, it is confirmed that the promoted charge separation mainly depends on the excited high-level electron transfer from g-C 3 N 4 to NPPy by single-wavelength photocurrent action spectra. This work provides a feasible strategy for designing polymer nano-heterojunction photocatalysts with exceptional visible-light activities. © 2020 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.BACKGROUND Biliary atresia is the number one cause of cirrhosis and liver transplantation in children. Hyponatremia is the most important electrolytic disturbance observed in decompensated cirrhosis. Studies of hyponatremia in cirrhotic children are scarce and those that exist have defined hyponatremia as serum sodium  less then  130 mEq/L lasting for at least 7 days. METHODS We evaluated transplant-free survival (Kaplan-Meier) of children with cirrhosis due to biliary atresia and serum sodium  less then  130 mEq/L persisting for 1, 2-6, and ≥7 days. This was a single-center, historical cohort that included all patients aged ≤ 18 years on a liver transplantation waiting list. RESULTS We studied 128 patients. The overall frequency of hyponatremia was 30.5% (39/128). Thirteen patients (10.2%) had hyponatremia when put on the list, and 20.3% developed it during follow-up. The Kaplan-Meier overall transplant-free survival rate was 83.3%. Patients with persistent hyponatremia for at least two days had the lowest transplant-free survival. Glomerular filtration rate (P = .00, RR = 0.96, IC 95% = 0.94-0.99), BMI/age Z-score (P = .02, RR = 0.59, IC 95% = 0.39-0.91), INR (P = .00, RR = 1.43, IC 95% = 1.17-1.74), and serum sodium (P = .04, RR = 0.91, IC 95% = 0.84-0.99) were independently associated with transplant-free survival. We did not observe any difference in mortality prediction after adding sodium to the original PELD score. CONCLUSIONS We conclude that persistent hyponatremia lasting at least two days may herald poor prognosis for children with cirrhosis due to biliary atresia. © 2020 Wiley Periodicals, Inc.In teaching of experiments for medical students, by using the isolation of mononuclear cells and identification of B lymphocytes, an experiment was developed that integrated biochemistry, cytology, and immunology techniques, from which the students performed a series of operations including spleen separation, isolating mononuclear cells, and identifying B lymphocytes. From the immunocytochemistry detection, we included advanced equipment-a flow cytometer-in the experiment, which detected the B lymphocytes more accurately. Moreover, we applied modern information technology to the teaching of experiments by using an internet study platform, micro-lectures, and adopting "formative teaching and evaluation." This lab practice aims to increase the ability of medical students to solve problems using flexible scientific methods, to initiate them thinking about clinical and research application. © 2020 International Union of Biochemistry and Molecular Biology.The presence of bilateral brain injury in patients with unilateral cerebral palsy (CP) may impact neuroplasticity in the ipsilateral hemisphere; however, this pattern of injury is typically under-analyzed due to the lack of methods robust to severe injury. In this study, injury-robust methods have been applied to structural brain magnetic resonance imaging (MRI) data of a cohort of 91 children with unilateral CP (37 with unilateral and 54 with bilateral brain injury, 4-17 years) and 44 typically developing controls (5-17 years), to determine how brain structure is associated with concurrent motor function, and if these associations differ between patients with unilateral or bilateral injury. Regression models were used to associate these measures with two clinical scores of hand function, with patient age, gender, brain injury laterality, and interaction effects included. Significant associations with brain structure and motor function were observed (Pearson's r = .494-.716), implicating several regions of the motor pathway, and demonstrating an accurate prediction of hand function from MRI, regardless of the extent of brain injury.