DNAJB1-PKAc Kinase Is Expressed in Young Patients with Pediatric Liver Cancers and Enhances Carcinogenic Pathways.

Hepatoblastoma (HBL) and fibrolamellar hepatocellular carcinoma (FLC) are the most common liver malignancies in children and young adults. FLC oncogenesis is associated with the generation of the fusion kinase, DNAJB1-PKAc (J-PKAc). J-PKAc has been found in 90% of FLC patients' tumors but not in other liver cancers.

Transcriptional control of a stem cell factor nucleostemin in liver regeneration and aging.

Nucleostemin (NS) plays a role in liver regeneration, and aging reduces its expression in the baseline and regenerating livers following 70% partial hepatectomy (PHx). Here we interrogate the mechanism controlling NS expression during liver regeneration and aging. The NS promoter was analyzed by TRANSFAC.

Re-engagement and retention in HIV care after preventive default tracking in a cohort of HIV-infected patients in rural Malawi: A mixed-methods study.

Loss-to-follow-up (LTFU) in the era of test-and-treat remains a universal challenge, especially in rural areas. To mitigate LTFU, the HIV program in Neno District, Malawi, utilizes a preventive default tracking strategy named Tracking for Retention and Client Enrollment (TRACE). We utilized a mixed-methods descriptive study of the TRACE program on patient's re-engagement and retention in care (RiC).

Increasing Continuous Glucose Monitoring Use for Non-Hispanic Black and Hispanic People With Type 1 Diabetes: Results From the T1D Exchange Quality Improvement Collaborative Equity Study.

Despite the benefits of continuous glucose monitoring (CGM), there is lower use of this technology among non-Hispanic Black and Hispanic people with type 1 diabetes compared with their non-Hispanic White counterparts. The T1D Exchange Quality Improvement Collaborative recruited five endocrinology centers to pilot an equity-focused quality improvement (QI) study to reduce racial inequities in CGM use. The centers used rapid QI cycles to test and expand interventions such as provider bias training, translation of CGM materials, provision of CGM education in multiple languages, screening for social determinants of health, and shared decision-making.

Perturbations of Glutathione and Sphingosine Metabolites in Port Wine Birthmark Patient-Derived Induced Pluripotent Stem Cells.

Port Wine Birthmarks (PWBs) are a congenital vascular malformation on the skin, occurring in 1-3 per 1000 live births. We have recently generated PWB-derived induced pluripotent stem cells (iPSCs) as clinically relevant disease models. The metabolites associated with the pathological phenotypes of PWB-derived iPSCs are unknown, and so we aim to explore them in this study.

Endothelial cells differentiated from patient dermal fibroblast-derived induced pluripotent stem cells resemble vascular malformations of port-wine birthmark.

Lesional induced pluripotent stem cell-derived endothelial cells can resemble pathological vascular phenotypes of port-wine birthmark (PWB). Our data demonstrate that multiple pathways, including Hippo and Wnt, NFκB, TNF, MAPK and cholesterol metabolism, are dysregulated. These data suggest new therapeutics can be developed to target such dysregulated pathways in the treatment of PWB.

Supporting materials: Endothelial cells differentiated from patient dermal fibroblast-derived induced pluripotent stem cells resemble vascular malformations of Port Wine Birthmark.

Background: Port wine birthmark (PWB) is a congenital vascular malformation resulting from developmentally defective endothelial cells (ECs). Developing clinically relevant disease models for PWB studies is currently an unmet need.

Objective: Our study aims to generate PWB-derived induced pluripotent stem cells (iPSCs) and iPSC-derived ECs that preserve disease-related phenotypes.

Perturbations of glutathione and sphingosine metabolites in Port Wine Birthmark patient-derived induced pluripotent stem cells.

Port Wine Birthmark (PWB) is a congenital vascular malformation in the skin, occurring in 1-3 per 1,000 live births. We recently generated PWB-derived induced pluripotent stem cells (iPSCs) as clinically relevant disease models. The metabolites associated with the pathological phenotypes of PWB-derived iPSCs are unknown, which we aimed to explore in this study.

Autoimmune Lymphoproliferative Syndrome (ALPS) Disease and ALPS Phenotype: Are They Two Distinct Entities?

Autoimmune lymphoproliferative syndrome (ALPS) is an inherited disorder of lymphocyte homeostasis classically due to mutation of FAS, FASL, and CASP10 genes (ALPS-FAS/CASP10). Despite recent progress, about one-third of ALPS patients does not carry classical mutations and still remains gene orphan (ALPS-U, undetermined genetic defects). The aims of the present study were to compare the clinical and immunological features of ALPS-FAS/CASP10 versus those of ALPS-U affected subjects and to deepen the genetic characteristics of this latter group.

Intravenous lidocaine simultaneously infused with sufentanil to accelerate gastrointestinal function recovery in patients after thoracolumbar surgery: a prospective, randomized, double-blind controlled study.

Purpose: Postoperative gastrointestinal dysfunction is one of the common complications of surgery, especially after surgery for a thoracolumbar spinal fracture. Intravenous lidocaine is a potential method to improve postoperative gastrointestinal function in surgical patients, reduce opioid use and shorten hospital stays. The purpose of this study is to explore the effect of intravenous lidocaine on the recovery of gastrointestinal function in patients after thoracolumbar surgery.

Effect of lidocaine perioperative infusion on chronic postsurgical pain in patients undergoing thoracoscopic radical pneumonectomy.

Background: Thoracoscopic radical pneumonectomy is associated with a high incidence of postoperative chronic pain. Studies on the benefits of lidocaine intravenous infusion during the perioperative period were still controversial in thoracoscopic surgery.

Methods: Sixty-four lung cancer patients scheduled for thoracoscopic radical pneumonectomy were randomly divided into two groups: normal saline group (control group) or lidocaine group.

Isolation and Characterization of Tissue Resident CD29-Positive Progenitor Cells in Livestock to Generate a Three-Dimensional Meat Bud.

The current process of meat production using livestock has significant effects on the global environment, including high emissions of greenhouse gases. In recent years, cultured meat has attracted attention as a way to acquire animal proteins. However, the lack of markers that isolate proliferating cells from bovine tissues and the complex structure of the meat make it difficult to culture meat in a dish.

Does central nervous system dysfunction underlie patellofemoral pain in young females? Examining brain functional connectivity in association with patient-reported outcomes.

Patellofemoral pain (PFP) is defined as retro- or peri-patellar knee pain without a clear structural abnormality. Unfortunately, many current treatment approaches fail to provide long-term pain relief, potentially due to an incomplete understanding of pain-disrupted sensorimotor dysfunction within the central nervous system. The purposes of this study were to evaluate brain functional connectivity in participants with and without PFP, and to determine the relationship between altered brain functional connectivity in association with patient-reported outcomes.

Building consensus on definition and nomenclature of hepatic, pancreatic, and biliary organoids.

Hepatic, pancreatic, and biliary (HPB) organoids are powerful tools for studying development, disease, and regeneration. As organoid research expands, the need for clear definitions and nomenclature describing these systems also grows. To facilitate scientific communication and consistent interpretation, we revisit the concept of an organoid and introduce an intuitive classification system and nomenclature for describing these 3D structures through the consensus of experts in the field.

Effect of intravenous administration of lidocaine on the ED50 of propofol induction dose during gastroscopy in adult patients: A randomized, controlled study.

What Is Known And Objective: Propofol provides a prominent sedation effect in gastroscopy. However, sedation with propofol alone during gastroscopy might result in circulatory and respiratory depression. This study aimed to test the hypothesis that the addition of intravenous lidocaine to propofol-based sedation could decrease the ED50 of propofol induction dose during gastroscopy in adult patients while the respiratory and haemodynamic stability were not compromised.

Measurement of Exocytosis in Genetically Manipulated Mast Cells.

The hallmark of mast cell activation is secretion of immune mediators by regulated exocytosis. Measurements of mediator secretion from mast cells that are genetically manipulated by transient transfections provide a powerful tool for deciphering the underlying mechanisms of mast cell exocytosis. However, common methods to study regulated exocytosis in bulk culture of mast cells suffer from the drawback of high signal-to-noise ratio because of their failure to distinguish between the different mast cell populations, that is, genetically modified mast cells versus their non-transfected counterparts.

SUNCT/SUNA in children and adolescents: Application of ICHD-3 criteria and treatment response: Case series of 13 SUNCT/SUNA pediatric cases.

Introduction: Trigeminal autonomic cephalalgias (TACs) are characterized by paroxysmal attacks of unilateral primary headaches associated with ipsilateral craniofacial autonomic symptoms. In this pediatric case series, 13 cases of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)/short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA), including children ages 3-18 years, are discussed. This paper reviews the application of International Classification of Headache Disorders, 3rd edition (ICHD-3) criteria especially in children presenting with SUNCT or SUNA.

Comparison of total body irradiation non-total body irradiation containing regimens for de novo acute myeloid leukemia in children.

With limited data comparing hematopoietic cell transplant outcomes between myeloablative total body irradiation (TBI) containing and non-TBI regimens in children with de novo acute myeloid leukemia, the aim of this study was to compare transplant-outcomes between these regimens. Cox regression models were used to compare transplant-outcomes after TBI and non-TBI regimens in 624 children transplanted between 2008 and 2016. Thirty two percent (n=199) received TBI regimens whereas 68% (n=425) received non-TBI regimens.

Motor Behavior Literature Fails to Translate: A Preliminary Investigation into Coaching and Focus of Attention in Recreational Distance Runners.

The benefits of using an external focus relative to an internal focus for endurance activities are well documented. However, literature has revealed that internally focused instructions are predominantly adopted in the field, and existing data are limited to highlyskilled level populations. Moreover, athletes' focus of attention during fatigue invoking activities is unknown.

Volumetric quantification of lung MR signal intensities using ultrashort TE as an automated score in cystic fibrosis.

Objectives: The study aimed to validate automated quantification of high and low signal intensity volumes using ultrashort echo-time MRI, with CT and pulmonary function test (PFT) as references, to assess the severity of structural alterations in cystic fibrosis (CF).

Methods: This prospective study was performed in a single center between May 2015 and September 2017. Participants with CF completed clinical examination, CT, MRI, and PFT the same day during routine clinical follow-up (M0), and then 1 year after (M12) except for CT.

The genetics of macrophage activation syndrome.

Macrophage activation syndrome (MAS), or secondary hemophagocytic lymphohistiocytosis (HLH), is a cytokine storm syndrome associated with multi-organ system dysfunction and high mortality rates. Laboratory and clinical features resemble primary HLH, which arises in infancy (1 in 50,000 live births) from homozygous mutations in various genes critical to the perforin-mediated cytolytic pathway employed by NK cells and cytotoxic CD8 T lymphocytes. MAS/secondary HLH is about ten times more common and typically presents beyond infancy extending into adulthood.

Modified paediatric preoperative risk prediction score to predict postoperative ICU admission in children: a retrospective cohort study.

Objective: To integrate intrinsic surgical risk into the paediatric preoperative risk prediction score (PRPS) model to construct a more comprehensive risk scoring system (modified PRPS) and improve the prediction accuracy of postoperative intensive care unit (ICU) admission in paediatric patients.

Design: This was a retrospective study conducted between 1 January and 30 December 2016. Data on age, American Society of Anaesthesiology physical status (ASA-PS), oxygen saturation, prematurity, non-fasted status, severity of surgery and immediate transfer to the ICU after surgery were collected.