The outcomes of the arterial switch operation have improved over a period of time with the elimination of coronary artery anatomy as a risk factor for operative mortality in some series. However, cumulatively, when all the series published so far are analyzed, two coronary variations, namely the single sinus coronary artery origin and intramural type, persist as risk factors for an adverse operative outcome.
There are no solid evidence from literature that compare Cox-Maze with pulmonary vein isolation technique for atrial fibrillation in the context of concomitant mitral valve surgery. While the first is perhaps more effective and linked to higher freedom form atrial fibrillation, it is more invasive compared to the pulmonary isolation.
A rapid and convenient strategy to monitor the productivity of biomanufacturing is essential for the research in optimizing relevant bioprocesses. In this work, we have developed a fluorescein-derived probe (FL-DT) that reacts rapidly with thiol groups via 1, 4-Micheal addition reaction of the sulfhydryl to unsaturated ketone and releases fluorescence. FL-DT specifically forms fluorescent adduct with two adjacent thiols in a protein of interest (POI), making the probe a reliable tool for protein quantification. The production of xylanase fused with a short di-Cys tag was then successfully monitored and quantified with FL-DT in E. coli system under different protein expression conditions, providing useful information for optimizing the bioprocess. Our work provides a convenient and efficient strategy for POI labeling and monitoring bioproduction.
Background While open surgical repair continues to be the mainstay option for aortic arch reconstruction, the associated mortality, morbidity, and high turn-down rates have led to a need for the development of minimally invasive options for aortic arch repair. Though RELAY™ Branched (Terumo Aortic, Inchinnan, UK) represents a promising option for complex endovascular aortic arch repair, neurological complications remain a pertinent risk. Herein we seek to present multi-centre data from Europe documenting the neurological outcomes associated with RELAY™ Branched. Methods Prospective data collected between January 2019 and January 2022 associated with patients treated with RELAY™ single-, double-, and triple-branched endoprostheses from centres across Europe was retrospectively analysed with descriptive and distributive analysis. Follow up data from 30 days and 6-, 12-, and 24 months postoperatively was included. Patients follow up was evaluated for the onset of disabling stroke (DS) and non-disabling stroke (NDS). Results Technical success was achieved in 147 (99.3%) cases. Over 24 months period, in total, 6 (4.1%) patients suffered DS and 8 (5.4%) patients suffered NDS after undergoing aortic arch repair with RELAY™. All patients that developed postoperative DS had been treated with the double-branched RELAY™ endoprosthesis. Discussion The data presented herein demonstrates that RELAY™ Branched is associated with favourable neurological outcomes and excellent technical success rates. Key design features of the endoprosthesis and good perioperative management can contribute greatly to mitigating neurological complications following endovascular aortic arch repair.
The most common pediatric extragonadal pelvic cancers include germ cell tumors, sacrococcygeal teratomas, and rhabdomyosarcomas (arising from the urinary bladder, prostate, paratesticular tissues, vagina, uterus, and perineum). This manuscript describes the radiological and nuclear medicine features of these entities and provides consensus-based recommendations for the assessment at diagnosis, during and after treatment.
Preface by Prof. Titia de Lange, Laboratory for Cell Biology and Genetics, The Rockefeller University, New York, NY 10065, USA The 19th Annual Wiley Prize in Biomedical Sciences celebrated a breakthrough in cell biology: how membrane-less cellular compartments are formed. The existence of membrane-less organelles, often called bodies or puncta, have been known for a long time, but what exactly they represented and how they were formed was not known. This problem was solved by a physicist, Clifford Brangwynne, a cell biologist, Anthony Hyman and a chemist, Michael Rosen. Each, synergistically, made groundbreaking contributions to the discovery that membrane-less organelles are liquid-liquid phase-separated entities. The two independent discoveries leading to the principle that multivalent low-affinity interactions between selected sets of macromolecules, some containing intrinsically disordered regions, formed a molecular condensate with unique dynamic properties, gave birth to the large, blossoming field of biomolecular condensates. The implications of those findings have influenced almost all further research of intracellular processes, including RAS signaling, immune synapses, DNA repair, transcriptional activation, and the functions of nuclear pores, the nucleolus and centrosomes. In this Perspective article, the laureates of the award take us on their personal and professional trip that led to their scientific discoveries. Their stories are a celebration of the interdisciplinary essence of Natural Sciences and the potential unlocked when scientists from different fields work together to solve mysteries.
Gastro-oesophageal reflux (GOR) and food allergy (FA) are common conditions, especially during the first 12 months of life. When GOR leads to troublesome symptoms, that affect daily functioning of the infant and family, it is referred to GOR disease (GORD). The role of food allergens as a cause of GORD remains controversial. This European Academy of Allergy and Clinical Immunology (EAACI) position paper aims to review the evidence for FA-associated GORD in young children and translate this into clinical practice that guides healthcare professionals through the diagnosis of suspected FA-associated GORD and the medical and dietary management. The Task Force (TF) on non-IgE mediated allergy consists of EAACI experts in paediatric gastroenterology, allergy, dietetics and psychology from Europe, United Kingdom, United States, Turkey and Brazil. Six clinical questions were formulated, amended and approved by the TF to guide this publication. A systematic literature search using PubMed, Cochrane and EMBASE databases (until June 2021) using a predefined inclusion criteria based on the 6 questions was used. The TF also gained access to the database from the European Society of Paediatric Gastroenterology and Hepatology working group, who published guidelines on GORD and ensured that all publications used within that position paper were included. For each of the 6 questions, practice points were formulated, followed by a modified Delphi method consisting of anonymous web-based voting that was repated with modified practice points where required, until at least 80% consensus for each practice point was achieved. This TF position paper shares the process, the discussion and consensus on all practice points on FA-associated GORD.
Iron-refractory iron-deficiency anaemia (IRIDA) is a rare autosomal recessive disease that presents in childhood. We report the case of fraternal twins presenting with severe hypochromic microcytic anaemia and hypoferritinemia. Two missense mutations affecting the TRMPSS6 gene were identified, consistent with IRIDA. Subsequent parenteral iron therapy improved clinical and blood parameters.
Since pemphigus blisters are intraepidermal, scarring should induce at most a post-inflammatory hyperpigmentation. We describe a very atypical and unusual course of pemphigus vulgaris with extensive keloid formation despite high systemic steroids. This could be promoted by the severe flare of the disease, the delay of scarring and the superinfection.
Pediatric thyroid cancer is rare in children, however incidence is increasing. Papillary thyroid cancer and follicular thyroid cancer are the most common subtypes, comprising about 90% and 10% of cases respectively. This manuscript provides consensus imaging recommendations for evaluation of pediatric patients with thyroid cancer at diagnosis and during follow-up.
Objective Functional tricuspid regurgitation(FTR) levels can vary over time and its longitudinal changing patterns may predict right ventricular dysfunction(RVD) risk. We aim to identify different trajectories of FTR in those who received mitral valve replacement(MVR) and investigate the association between longitudinal trajectory groups and RVD risk in a cohort study. Methods and results A prospective cohort study, reported usual FTR levels at baseline in 2005–2015 and the participants of MVR have been followed up for 5~6 years, approximately every one years, and so far, the data have been collected across five subsequent phases. Five-year longitudinal trajectories of FTR were identified using group-based trajectory modelling(GBTM). We identified 3 distinct trajectories using a GBTM, labeled by initial value and changing pattern: stable group(258/378, 68.2%), increasing-slow group(67/378, 17.6%) and increasing-fast group(53/378, 14.2%). Treating the stable group as the reference, the age- and sex-adjusted odds ratio(OR) was 25.84 (95% confidence interval, 11.78-56.65) for the increasing-slow group and 139.94(95% confidence interval, 45.47-430.68) for the increasing-fast group by logistic regression model. After adjustment for every potential confounding factors, the OR is 14.21(95% confidence interval, 4.36-46.33)、49.34(95% confidence interval, 8.88-273.87) respectively. Conclusions The longitudinal trajectories of worsening FTR were mostly associated with increased risk of RVD outcomes, which is independent of other factors including FTR levels. These findings have implications for intervention and prevention of RVD among individuals who received MVR.
The present paper is dedicated to the mechanical and fracture characterisation of a specific earthen material, that is, the shot-earth 772. Although such a material has been recently characterised from a microstructural, chemical and physical point of view, the knowledge of its mechanical and fracture properties (essential for extending its use in construction industry) is still lacking. Such characterisations are here performed both experimentally, through laboratory tests, and numerically, through a FE model. The experimental tests (i.e. flexural, compression and fracture tests) are carried out on shot-earth specimens according to Recommendations available for concrete and a method proposed by some of the present authors, named Modified Two-Parameter Model. The numerical analyses are performed by employing a micromechanical model (implemented in a non-linear 2D FE homemade code), which allows to simulate both flexural and fracture behaviour of the shot-earth examined. Finally, the obtained numerical results are compared with the experimental ones.
Introduction: Bronchopulmonary dysplasia (BPD) is characterized by lung injury with varying degrees of disrupted alveolarization, vascular remodeling, inflammatory cell proliferation, and pulmonary edema. Diuretics are often used to ameliorate the symptoms or progression of BPD. Our primary objective was to use lung ultrasound (LUS) to determine if diuretics decrease pulmonary edema in infants with BPD. The secondary objective was to assess changes in respiratory support during the first week after initiation of diuretics. Methods: Premature infants requiring non-invasive respiratory support and starting diuretic therapy for evolving BPD were compared with a similar group of infants not receiving diuretics (control). For the diuretic group, LUS exams were performed before and on days 1, 3 and 6 after initiation of treatment. For the control group, LUS was performed at equivalent time points. A composite pulmonary edema severity (PES) score of 0 to 5 was calculated based on the total number of B-lines in 6 scanned areas. Respiratory support parameters (FiO2, nasal cannula flow or CPAP) were also recorded. Results: Infants in the diuretic (n=28) and control (n=23) groups were recruited at median corrected gestational ages of 34.2 (33.3-35.9) and 34.0 (33.4-36.3) weeks, respectively ( p=0.82). PES scores, FiO2, and respiratory flow support decreased significantly from day 0 to 6 ( p<.0001, p=0.001, and p=0.01, respectively) in the diuretic group, but not in the control group. Conclusion: Diuretic use is associated with decreased pulmonary edema and improved oxygenation in infants with BPD during the first week of treatment.
Although COP is idiopathic by definition, it is important to investigate each diagnosed case for potential causes, such as iatrogenic from radiation or known causative medications , connective tissue diseases, inflammatory bowel disease, malignancies, history of lung transplant or bone marrow graft. OP may present weeks to months before other signs of connective tissue disorders and therefore the patient should undergo serologic testing for these diseases.  In the case presented, the specific etiology was unyielding and so remains cryptogenic in nature. This patient’s symptoms quickly improved following the use of steroids and tolerated tapering off completely without relapse one year out from initial diagnosis.