Outcomes of Childhood Noninfant Acute Lymphoblastic Leukemia With 11q23/ Rearrangements in a Modern Therapy Era: A Retrospective International Study.

Purpose: We aimed to study prognostic factors and efficacy of allogeneic hematopoietic stem-cell transplantation (allo-HSCT) in first remission of patients with noninfant childhood acute lymphoblastic leukemia (ALL) with 11q23/ rearrangements treated with chemotherapy regimens between 1995 and 2010.

Patients And Methods: Data were retrospectively retrieved from 629 patients with 11q23/-rearranged ALL from 17 members of the Ponte-di-Legno Childhood ALL Working Group. Clinical and biologic characteristics, early response assessed by minimal residual disease at the end of induction (EOI) therapy, and allo-HSCT were analyzed for their impact on outcomes.

Fueling the Female Athlete: Auditing Her Representation in Studies of Acute Carbohydrate Intake for Exercise.

Purpose: The aim of this audit was to assess the representation of female athletes within the literature that has led to current guidelines for carbohydrate (CHO) intake in the acute periods surrounding exercise and the quality of this research.

Methods: We conducted a standardized audit of research assessing CHO loading protocols, CHO mouth rinse, and CHO intake before, during, and after exercise.

Results: A total of 937 studies were identified in this audit.

A homozygous truncating mutation of FGL2 is associated with immune dysregulation.

Background: The type II transmembrane protein fibrinogen-like protein 2 (FGL2) plays critical roles in hemostasis and immune regulation. The C-terminal immunoregulatory domain of FGL2 can be secreted and is a mediator of regulatory T (Treg) cell suppression. Fgl2 mice develop autoantibodies and glomerulonephritis and have impaired Treg cell function.

Differential ABC transporter expression during hematopoiesis contributes to neutrophil-biased toxicity of Aurora kinase inhibitors.

Drug-induced cytopenias are a prevalent and significant issue that worsens clinical outcomes and hinders the effective treatment of cancer. While reductions in blood cell numbers are classically associated with traditional cytotoxic chemotherapies, they also occur with newer targeted small molecules and the factors that determine the hematotoxicity profiles of oncologic drugs are not fully understood. Here, we explore why some Aurora kinase inhibitors cause preferential neutropenia.

Improving Discharge Safety in a Pediatric Emergency Department.

Background And Objectives: Discharge from the emergency department (ED) involves a complex series of steps to ensure a safe transition to home and follow-up care. Preventable, discharge-related serious safety events (SSEs) in our ED highlighted local vulnerabilities. We aimed to improve ED discharge by implementing a standardized discharge process with emphasis on multidisciplinary communication and family engagement.

Lipopolysaccharide detection by the innate immune system may be an uncommon defence strategy used in nature.

Since the publication of the Janeway's Pattern Recognition hypothesis in 1989, study of pathogen-associated molecular patterns (PAMPs) and their immuno-stimulatory activities has accelerated. Most studies in this area have been conducted in model organisms, which leaves many open questions about the universality of PAMP biology across living systems. Mammals have evolved multiple proteins that operate as receptors for the PAMP lipopolysaccharide (LPS) from Gram-negative bacteria, but LPS is not immuno-stimulatory in all eukaryotes.

Copy Number Variation Analysis Facilitates Identification of Genetic Causation in Patients with Congenital Anomalies of the Kidney and Urinary Tract.

Background: Congenital anomalies of the kidneys and urinary tract (CAKUT) are the most common cause of chronic kidney disease among children and adults younger than 30 yr. In our previous study, whole-exome sequencing (WES) identified a known monogenic cause of isolated or syndromic CAKUT in 13% of families with CAKUT. However, WES has limitations and detection of copy number variations (CNV) is technically challenging, and CNVs causative of CAKUT have previously been detected in up to 16% of cases.

Clinical, immunological, molecular and therapeutic findings in monogenic immune dysregulation diseases: Middle East and North Africa registry.

Monogenic immune dysregulation diseases (MIDD) are caused by defective immunotolerance. This study was designed to increase knowledge on the prevalence and spectrum of MIDDs, genetic patterns, and outcomes in Middle East and North Africa (MENA). MIDD patients from 11 MENA countries (Iran, Turkey, Kuwait, Oman, Algeria, Egypt, United Arab Emirates, Tunisia, Jordan, Qatar, and Azerbaijan) were retrospectively evaluated.

Tricuspid Valve Blood Cysts Mimicking Thrombus or Vegetation on Echocardiogram in a Neonate.

A 2-month-old male infant, born premature with a birth weight of 865 g, was found to have a tricuspid valve mass mimicking thrombus and vegetation by echocardiogram on the fourth day of life. The patient was treated with antibiotics and anticoagulation with no change in the size of the mass on serial follow-up echocardiography. The patient died of severe pulmonary vein stenosis and complex neurological disability.

Immunologic effects of red blood cell and platelet transfusions in neonates.

Purpose Of Review: Premature neonates are frequently transfused red blood cells (RBCs) or platelets to raise hemoglobin or platelet counts. However, these transfusions may have unintended effects on the immune system. This review will summarize the newest discoveries on the immunologic effects of RBC and platelet transfusions in neonates, and their potential impact on neonatal outcomes.

Reconstruction of the Obliterated Eustachian Tube: A Pilot Case Series.

Objective: To investigate the safety and early efficacy of a procedure for reconstruction of the obliterated Eustachian tube (ET).

Study Design: Retrospective case series.

Methods: Patients with total obliteration of the cartilaginous ET, with intractable mucoid effusion causing repeated occlusion of tympanostomy tubes were included.

Encoding of inflammatory hyperalgesia in mouse spinal cord.

Inflammation modifies the input-output properties of peripheral nociceptive neurons such that the same stimulus produces enhanced nociceptive firing. This increased nociceptive output enters the superficial dorsal spinal cord (SDH), an intricate neuronal network composed largely of excitatory and inhibitory interneurons and a small percentage of projection neurons. The SDH network comprises the first central nervous system network integrating noxious information.

Skeletal Muscle Dysfunction in Experimental Pulmonary Hypertension.

Pulmonary arterial hypertension (PAH) is a serious, progressive, and often fatal disease that is in urgent need of improved therapies that treat it. One of the remaining therapeutic challenges is the increasingly recognized skeletal muscle dysfunction that interferes with exercise tolerance. Here we report that in the adult rat Sugen/hypoxia (SU/Hx) model of severe pulmonary hypertension (PH), there is highly significant, almost 50%, decrease in exercise endurance, and this is associated with a 25% increase in the abundance of type II muscle fiber markers, thick sarcomeric aggregates and an increase in the levels of FoxO1 in the soleus (a predominantly type I fiber muscle), with additional alterations in the transcriptomic profiles of the diaphragm (a mixed fiber muscle) and the extensor digitorum longus (a predominantly Type II fiber muscle).

Intraoperative Stent Placement for the Treatment of Congenital Pulmonary Vein Atresia.

A 2-month-old infant presenting with failure to thrive was found to have right upper and right lower pulmonary vein atresia and underwent 2 failed transcatheter recanalization attempts. A hybrid intervention including pulmonary vein identification, ostial resection, venoplasty, and intraoperative stent placement is described and 2.5 years of subsequent follow-up is presented.

Risk Factors Associated with Severe Macromastia among Adolescents and Young Women.

Background: Macromastia is common among adolescents and young women and has well-documented negative physical and psychosocial effects. The pathogenesis of idiopathic macromastia has been attributed to increased end organ sensitivity to circulating gonadal hormones. Despite the known negative effects of macromastia, there is a paucity of literature examining the clinical risk factors associated with macromastia severity in this age group.

Carbon footprint of the 2021 and 2022 AAPOS annual meetings.

The COVID-19 pandemic necessitated a virtual annual meeting of the American Association for Pediatric Ophthalmology and Strabismus (AAPOS) in 2021, thus eliminating carbon emissions from travel to and from the planned meeting venue in Boston, Massachusetts. We found that the reduced carbon footprint of the virtual meeting saved 1,282 metric tonnes of CO emissions compared with estimated CO emissions for travel if the meeting had taken place in person, or 880 metric tonnes relative to the projected emissions associated with the in-person 2022 annual meeting in Scottsdale, Arizona. An entirely virtual or hybrid AAPOS meeting would reduce its environmental footprint and increase the opportunity for national and international participation and education.

Retinal Ganglion Cell Survival and Axon Regeneration after Optic Nerve Injury: Role of Inflammation and Other Factors.

The optic nerve, like most pathways in the mature central nervous system, cannot regenerate if injured, and within days, retinal ganglion cells (RGCs), the neurons that extend axons through the optic nerve, begin to die. Thus, there are few clinical options to improve vision after traumatic or ischemic optic nerve injury or in neurodegenerative diseases such as glaucoma, dominant optic neuropathy, or optic pathway gliomas. Research over the past two decades has identified several strategies to enable RGCs to regenerate axons the entire length of the optic nerve, in some cases leading to modest reinnervation of di- and mesencephalic visual relay centers.

Are methods of estimating fat-free mass loss with energy-restricted diets accurate?

Background/objectives: Fat-free mass (FFM) often serves as a body composition outcome variable in weight loss studies. An important assumption is that the proportions of components that make up FFM remain stable following weight loss; some body composition models rely on these "constants". This exploratory study examined key FFM component proportions before and following weight loss in two studies of participants with overweight and obesity.

Impact of tight glycemic control and hypoglycemia after pediatric cardiac surgery on neurodevelopmental outcomes at three years of age: Findings from a randomized clinical trial.

Background: Studies examining the impact of randomization As per standard instruction, city is required for affiliations; however, this information is missing in affiliation 6. Please check if the provided city is correct and amend if necessary. to tight glycemic control (TGC) and resultant hypoglycemia on later neurodevelopmental outcomes have produced mixed results.

Inflammatory Bowel Disease and Cardiovascular Diseases.

Background: Emerging data showed patients with chronic inflammatory disorders, including inflammatory bowel disease, are more likely to develop atherosclerotic cardiovascular diseases, heart failure, and atrial fibrillation. This article aims to review the evidence of those associations.

Methods: PubMed was searched from inception to January 2022 using the keywords, including inflammatory bowel diseases, Crohn disease, ulcerative colitis, atherosclerotic cardiovascular disease, coronary artery disease, cardiovascular disease, atrial fibrillation, heart failure, and premature coronary artery disease.