Automated Assessment of Thoracic-Abdominal Asynchrony in Patients with Morquio Syndrome.

Morquio syndrome is a rare disease caused by a disorder in the storage of mucopolysaccharides that affects multiple organs, including musculoskeletal, respiratory, cardiovascular, and digestive systems. Respiratory failure is one of the leading causes of mortality in Morquio patients; thus, respiratory function testing is vital to the management of the disease. An automated respiratory assessment methodology using the RIP device and a machine-learning algorithm was developed.

Pain Trajectories Following Adolescent Idiopathic Scoliosis Correction: Analysis of Predictors and Functional Outcomes.

Background: A better understanding of early pain trajectories (patterns) following scoliosis surgery and how they relate to baseline patient characteristics and functional outcomes may allow for the development of mitigating strategies to improve patient outcomes.

Methods: This was a prospective cohort study. Adolescents with idiopathic scoliosis were recruited across multiple centers.

Does perioperative ketorolac increase bleeding risk after intracapsular tonsillectomy?

Importance: Conflicting evidence exists regarding the post-tonsillectomy bleed risk associated with perioperative ketorolac use in the pediatric population. Surgical technique for tonsillectomy can further confound this risk.

Objective: The primary objective was to retrospectively quantify the post-tonsillectomy bleed rate after single-dose administration of ketorolac in pediatric patients following intracapsular tonsillectomy.

Linking Lysosomal Enzyme Targeting Genes and Energy Metabolism with Altered Gray Matter Volume in Children with Persistent Stuttering.

Developmental stuttering is a childhood onset neurodevelopmental disorder with an unclear etiology. Subtle changes in brain structure and function are present in both children and adults who stutter. It is a highly heritable disorder, and 12-20% of stuttering cases may carry a mutation in one of four genes involved in intracellular trafficking.

Survey Research With Families in the Context of Pediatric Chronic Health Conditions: Key Considerations and Future Directions.

Self-report family functioning measures play a critical role in advancing our understanding of how families are impacted by, and adapt to, the demands of childhood health conditions. In this article, we present key considerations when conceptualizing, assessing, and analyzing dynamic family processes in research; discuss related implications for selecting instruments; and provide an update on the evidence base of self-report family functioning measures. Researchers need to consider theory, definitions of the family, informants, instruments, and procedural and data analytic issues when designing family research.

Engaging Caregivers and Providers of Children With Sickle Cell Anemia in Shared Decision Making for Hydroxyurea: Protocol for a Multicenter Randomized Controlled Trial.

Background: Sickle cell anemia (SCA) is a genetic blood disorder that puts children at a risk of serious medical complications, early morbidity and mortality, and high health care utilization. Until recently, hydroxyurea was the only disease-modifying treatment for this life-threatening disease and has remained the only option for children younger than 5 years. Evidence-based guidelines recommend using a shared decision-making (SDM) approach for offering hydroxyurea to children with SCA (HbSS or HbS/β0 thalassemia) aged as early as 9 months.

Microcephalic osteodysplastic primordial dwarfism type II is associated with global vascular disease.

Background: Microcephalic osteodysplastic primordial dwarfism type II (MOPDII) is the most common form of primordial dwarfism, caused by bialleic mutations in the pericentrin gene (PCNT). Aside from its classic features, there are multiple associated medical complications, including a well-documented risk of neurovascular disease. Over the past several years, it has become apparent that additional vascular issues, as well as systemic hypertension and kidney disease may also be related to MOPDII.

PICU Passport: Pilot study of a handheld resident curriculum.

Background: To explore the impact of an educational tool designed to streamline resident learning during their pediatric intensive care (PICU) rotations.

Methods: Topics and procedures were chosen for inclusion based on national requirements for pediatric residents. Residents received a PICU Passport at the beginning of their rotations.

Increase in Pediatric Intensive Care Unit Hospitalizations Due to Toxic Ingestions during the COVID-19 Pandemic.

The coronavirus disease 2019 (COVID-19) pandemic and related community mitigation measures had a significant psychosocial impact. We suspected that more patients were admitted to our pediatric intensive care unit (PICU) for toxic ingestions since the start of the pandemic. We therefore investigated if PICU admissions related to toxic ingestions were higher in 2020 as a result of COVID-19 compared with previous years.

Navigating the perils and pitfalls of pediatric forensic postmortem imaging in the United States.

Postmortem CT is widely used in the general adult and military populations. It is used extensively in pediatric death investigations in Europe and Asia, but distinctive challenges are encountered when launching a postmortem imaging program in the United States. We describe the issues we have encountered specific to establishing a pediatric postmortem imaging service in this country and propose potential solutions.

Unravelling neuroinflammation in abusive head trauma with radiotracer imaging.

Abusive head trauma (AHT) is a leading cause of mortality and morbidity in child abuse, with a mortality rate of approximately 25%. In survivors, the prognosis remains dismal, with high prevalence of cerebral palsy, epilepsy and neuropsychiatric disorders. Early and accurate diagnosis of AHT is challenging, both clinically and radiologically, with up to one-third of cases missed on initial examination.

Orthopaedic Manifestations of Transverse Myelitis in Children.

Background: Transverse myelitis (TM) is a rare inflammatory disorder of the spinal cord. It can have a heterogeneous presentation with sensory, motor, and autonomic dysfunction. Neurological sequelae of TM include autonomic dysfunction, motor weakness, and/or spasticity.

Transcriptional analysis of muscle tissue and isolated satellite cells in spastic cerebral palsy.

Aim: To analyze transcriptomes from muscle tissue and cells to improve our understanding of differences in gene expression and molecular function in cerebral palsy (CP) muscle.

Method: In this case-control study, eight participants with CP (five males, three females; mean [SD] age 14y 2mo [1y 8mo]) and 11 comparison individuals (eight males, three females; mean [SD] age 14y 0mo [2y 6mo]) were enrolled after informed consent/assent and skeletal muscle was obtained during surgery. RNA was extracted from tissue and from primary satellite cells grown to form myotubes in vitro.

The culture microenvironment of juvenile idiopathic arthritis synovial fibroblasts is favorable for endochondral bone formation through BMP4 and repressed by chondrocytes.

Background: We examined influences of conditioned media from chondrocytes (Ch) on juvenile idiopathic arthritis synovial fibroblasts (JFLS) and potential for JFLS to undergo endochondral bone formation (EBF).

Methods: Primary cells from three control fibroblast-like synoviocytes (CFLS) and three JFLS were cultured in Ch-conditioned media and compared with untreated fibroblast-like synoviocytes (FLS). RNA was analyzed by ClariomS microarray.

Effect of gestational age at birth, sex, and race on foveal structure in children.

Purpose: Premature birth, race, and sex are contributing risk factors for retinopathy of prematurity (ROP) and have long-term impact on children's retinal structure. Few studies investigate impact of race and sex on macular structure in children born preterm. This study compared foveal structure in preterm and full-term children.

CEBPA-bZip mutations are associated with favorable prognosis in de novo AML: a report from the Children's Oncology Group.

Biallelic CEBPA mutations are associated with favorable outcomes in acute myeloid leukemia (AML). We evaluated the clinical and biologic implications of CEBPA-basic leucine zipper (CEBPA-bZip) mutations in children and young adults with newly diagnosed AML. CEBPA-bZip mutation status was determined in 2958 patients with AML enrolled on Children's Oncology Group trials (NCT00003790, NCT0007174, NCT00372593, NCT01379181).

Early and late hospital readmissions in adolescent idiopathic scoliosis.

Study Design: Retrospective review of a prospectively collected multicenter database.

Objectives: To identify risk factors for early and late readmission of surgically treated patients with adolescent idiopathic scoliosis (AIS). Specific risk factors associated with readmission in patients with AIS remain poorly understood.

Mesothelin is a novel cell surface disease marker and potential therapeutic target in acute myeloid leukemia.

In an effort to identify acute myeloid leukemia (AML)-restricted targets for therapeutic development in AML, we analyzed the transcriptomes of 2051 children and young adults with AML and compared the expression profile with normal marrow specimens. This analysis identified a large cohort of AML-restricted genes with high expression in AML, but low to no expression in normal hematopoiesis. Mesothelin (MSLN), a known therapeutic target in solid tumors, was shown to be highly overexpressed in 36% of the AML cohort (range, 5-1077.

Artificial intelligence in child abuse imaging.

There have been rapid advances in artificial intelligence (AI) technology in recent years, and the field of diagnostic imaging is no exception. Just as digital technology revolutionized how radiology is practiced, so these new technologies also appear poised to bring sweeping change. As AI tools make the transition from the theoretical to the everyday, important decisions need to be made about how they will be applied and what their role will be in the practice of radiology.

Rapid Growth of Left Atrial Thrombus in a Pediatric Heart Transplant Recipient.

We report on a 10-year-old female patient who rapidly developed a left atrial (LA) mass 2 months after orthotopic heart transplant. Nine days prior to detection of the mass, she received high-dose corticosteroids for acute cellular rejection (grade 2). Despite negative echocardiogram 5 days prior to detection, a large echogenic mass was noted in the LA (18 x 12 x 24 mm); it was surgically resected after unsuccessful anticoagulation treatment.

What happens to the unfused upper thoracic curve after posterior spinal fusion for adolescent idiopathic scoliosis?

Objective: Spontaneous lumbar curve correction after selective thoracic fusion in surgery for adolescent idiopathic scoliosis (AIS) is well described. However, only a few articles have described the course of the uninstrumented upper thoracic (UT) curve after fusion, and the majority involve a hybrid construct. In this study, the authors sought to determine the outcomes and associated factors of uninstrumented UT curves in patients with AIS.

Clinical Presentation and Characteristics of Hand and Wrist Ganglion Cysts in Children.

Purpose: Ganglion cysts are the most common mass of the hand or wrist. In adults, ganglions have a female predilection and are commonly located in the dorsal wrist. However, their presentation in children has not been well reported.

Comparing telehealth with office-based visits for common pediatric otolaryngology complaints.

Objective: The objective of this study was to evaluate the feasibility of telehealth visits and compare office-based visits for pediatric patients undergoing evaluation of recurrent acute otitis media or sleep-disordered breathing.

Methods: A retrospective cohort study compared telehealth patients with matched controls seen in the office. The feasibility of a thorough patient evaluation in a single telehealth visit without a follow-up office visit was assessed.

Height Versus Body Surface Area to Normalize Cardiovascular Measurements in Children Using the Pediatric Heart Network Echocardiographic Z-Score Database.

Normalizing cardiovascular measurements for body size allows for comparison among children of different ages and for distinguishing pathologic changes from normal physiologic growth. Because of growing interest to use height for normalization, the aim of this study was to develop height-based normalization models and compare them to body surface area (BSA)-based normalization for aortic and left ventricular (LV) measurements. The study population consisted of healthy, non-obese children between 2 and 18 years of age enrolled in the Pediatric Heart Network Echo Z-Score Project.

Neointimal Hyperplasia after Carotid Transection and Anastomosis Surgery is Associated with Degradation of Decorin and Platelet Derived Growth Factor Signaling.

Objective: Intimal hyperplasia (IH) is the expansion of the vascular intimal region after intervention, which can lead to stenosis and eventual failure of vascular grafts or interventional procedures such as angioplasty or stent placement. Our goals were to investigate the development of IH in a rabbit open surgical model and to evaluate the associated pathophysiological processes involving decorin and the platelet derived growth factor-BB / platelet derived growth factor receptor-β / mitogen activated protein kinase (PDGF/PDGFR-β/MAPK) pathway.

Methods: We conducted carotid transection and primary anastomosis on five New Zealand White rabbits to induce IH and examined the associated pathophysiological changes.