Distal femoral extension osteotomy and patellar tendon advancement or shortening in ambulatory children with cerebral palsy: A modified Delphi consensus study and literature review.

Purpose: In children with cerebral palsy, flexion deformities of the knee can be treated with a distal femoral extension osteotomy combined with either patellar tendon advancement or patellar tendon shortening. The purpose of this study was to establish a consensus through expert orthopedic opinion, using a modified Delphi process to describe the surgical indications for distal femoral extension osteotomy and patellar tendon advancement/patellar tendon shortening. A literature review was also conducted to summarize the recent literature on distal femoral extension osteotomy and patellar tendon shortening/patellar tendon advancement.

Utilizing Social Media to Identify Potential Living Donors: Learning from US Living Donor Programs.

Purpose Of Review: Living donor transplantation provides the best possible recipient outcomes in solid organ transplantation. Yet, identifying potential living donors can be a laborious and resource intensive task that heavily relies on the recipient's means and social network. Social media has evolved to become a key tool in helping to bring recipients and potential living donors together given its ease of utilization, widespread access, and improved recipient's comfort with public solicitation.

A Roadmap for Potential Improvement of Newborn Screening for Inherited Metabolic Diseases Following Recent Developments and Successful Applications of Bivariate Normal Limits for Pre-Symptomatic Detection of MPS I, Pompe Disease, and Krabbe Disease.

The mucopolysaccharidoses (MPS), Pompe Disease (PD), and Krabbe disease (KD) are inherited conditions known as lysosomal storage disorders (LSDs) The resulting enzyme deficiencies give rise to progressive symptoms. The United States Department of Health and Human Services' Recommended Uniform Screening Panel (RUSP) suggests LSDs for inclusion in state universal newborn screening (NBS) programs and has identified screening deficiencies in MPS I, KD, and PD NBS programs. MPS I NBS programs utilize newborn dried blood spots and assay alpha L-iduronidase (IDUA) enzyme to screen for potential cases.

Hearing outcomes in children with Congenital Cytomegalovirus: A multi-center, single-enterprise experience.

Background: Cytomegalovirus (CMV) is the most common cause of non-genetic sensorineural hearing loss (SNHL) in the United States; yet screening for congenital CMV (cCMV) remains controversial. CMV related SNHL can be present at birth, or develop in a delayed manner, and it is a consistent feature in children with either symptomatic or asymptomatic disease. A retrospective chart review was performed to determine the characteristics of patients diagnosed with cCMV and SNHL.

Sex Difference Leads to Differential Gene Expression Patterns and Therapeutic Efficacy in Mucopolysaccharidosis IVA Murine Model Receiving AAV8 Gene Therapy.

Adeno-associated virus (AAV) vector-based therapies can effectively correct some disease pathology in murine models with mucopolysaccharidoses. However, immunogenicity can limit therapeutic effect as immune responses target capsid proteins, transduced cells, and gene therapy products, ultimately resulting in loss of enzyme activity. Inherent differences in male versus female immune response can significantly impact AAV gene transfer.

Regulation of Molecular Targets in Osteosarcoma Treatment.

The most prevalent malignant bone tumor, osteosarcoma, affects the growth plates of long bones in adolescents and young adults. Standard chemotherapeutic methods showed poor response rates in patients with recurrent and metastatic phases. Therefore, it is critical to develop novel and efficient targeted therapies to address relapse cases.

Treatment of a Lisfranc Injury in a 7-Year-Old Boy: Case Report and Surgical Technique.

Case: This is a case of a 7-year-old boy with a Lisfranc injury identified on weight-bearing films who was treated successfully with closed reduction with a clamp and smooth pin fixation. This patient returned to sports without pain or radiographic signs of arthritis at 1 year.

Conclusion: Lisfranc fractures and ligamentous injuries are rare and can occur in skeletally immature children.

Evaluation of the pan-class I phosphoinositide 3-kinase (PI3K) inhibitor copanlisib in the Pediatric Preclinical Testing Consortium in vivo models of osteosarcoma.

Copanlisib is a pan-class I phosphoinositide 3-kinase (PI3K) inhibitor, with activity against all four PI3K class I isoforms (PI3Kα, PI3Kβ, PI3Kγ, and PI3Kδ). Whole-genome and RNA sequencing data have revealed several PI3K aberrations in osteosarcoma tumor samples. The in vivo anticancer effects of copanlisib were assessed in a panel of six osteosarcoma models.

Fellows in Training Wellness in Allergy and Immunology: AAAAI Workgroup Report.

Physician health and wellness can be negatively affected by burnout, which in turn can lead to multiple potential professional and personal issues. Burnout issues can start in medical school and progress during residency and fellowship, and throughout a physician's career. A previous survey of allergists and immunologists reported a burnout rate of 35%.

Minimally invasive endoscopic approaches to pediatric skull base pathologies.

Objective: Endoscopic endonasal approaches to pediatric skull base lesions are rare, challenging, and present distinct considerations from adult pathology. In this study, we describe our endoscopic technical approach to various skull base lesions demonstrating the efficacy and nuances of these approaches in pediatric patients.

Methods: Pediatric patients underwent endoscopic approach for skull base lesions from January of 2015 to April of 2021 were included in the study.

Skeletal Outcomes in Children and Young Adults with Glomerular Disease.

Background: Children with glomerular disease have unique risk factors for compromised bone health. Studies addressing skeletal complications in this population are lacking.

Methods: This retrospective cohort study utilized data from PEDSnet, a national network of pediatric health systems with standardized electronic health record data for more than 6.

The Temporal Association of the COVID-19 Pandemic and Pediatric Cardiopulmonary Resuscitation Quality and Outcomes.

Objectives: The COVID-19 pandemic resulted in adaptations to pediatric resuscitation systems of care. The objective of this study was to determine the temporal association between the pandemic and pediatric in-hospital cardiac arrest (IHCA) process of care metrics, cardiopulmonary resuscitation (cardiopulmonary resuscitation) quality, and patient outcomes.

Design: Multicenter retrospective analysis of a dataset comprising observations of IHCA outcomes pre pandemic (March 1, 2019 to February 29, 2020) versus pandemic (March 1, 2020 to February 28, 2021).

Minimally Displaced Lateral Humeral Condyle Fractures: Optimizing Follow-up and Minimizing Cost.

Background: Minimally displaced fractures of the lateral humeral condyle (LHC) may be treated nonoperatively in a long arm cast, but there is not a standardized evidence-based protocol. The aim of this study was to evaluate our nonoperatively treated LHC fractures, determine the risk of subsequent displacement, analyze our practice variability, and develop an evidence-based protocol to safely manage children with LHC fractures and to evaluate potential savings related to this analysis.

Methods: We retrospectively reviewed clinical and radiographic parameters of all patients with LHC fractures at our institution from 2009 to 2015.

Publication timeline of chest imaging reporting in children with coronavirus disease 2019 (COVID-19): a systematic review spanning 2020.

Amid the coronavirus disease 2019 (COVID-19) pandemic, numerous publications of imaging findings in children have surfaced in a very short time. Publications discuss populations of overlapping age groups and describe different imaging patterns. We aim to present an overview of the quantity and type of literature available regarding COVID-19 chest imaging findings in children according to a 2020 publication timeline.

The Immunoglobulin M Response to Pneumococcal Polysaccharide Vaccine Is Sufficient for Conferring Immunity.

In mice, pneumococcal polysaccharide (PPS) vaccines generate antigen-specific immunoglobulin M (IgM) and immunoglobulins G1, G2, and G3. Antibody and complement-dependent opsonophagocytosis correlates with the protection induced by PPS vaccines in vivo. Since IgM is a very efficient immunoglobulin isotype in activating the complement system, we evaluated whether anti-PPS IgM alone is sufficient to confer protective immunity to Streptococcus pneumoniae.

Vitamin D supplementation in children and young adults with persistent proteinuria secondary to glomerular disease.

Background: Vitamin D deficiency is common in glomerular disease. Supplementation may be ineffective due to ongoing urinary losses of vitamin D binding protein. We sought to determine if daily cholecalciferol supplementation would increase vitamin D concentrations in children with glomerular disease and persistent proteinuria, without adverse effects.

Spontaneous Lumbar Curve Correction Following Vertebral Body Tethering of Main Thoracic Curves.

Background: Growth modulation through anterior vertebral body tethering (AVBT) has emerged as a fusionless option for the treatment of progressive scoliosis. When tethering the main thoracic curve, the compensatory thoracolumbar/lumbar curve must correct indirectly as a result. The present study evaluated the response of these lumbar curves following AVBT of the main thoracic curves.

The modified Kocher criteria for septic hip: Does it apply to the knee?

Background: Pediatric septic arthritis is a surgical emergency and timely diagnosis prevents serious complications. To differentiate between septic hip and transient synovitis, the predictive value of four original Kocher criteria (fever, inability to bear weight, elevated serum white blood cell count, and elevated erythrocyte sedimentation rate) plus Caird's addition of elevated C-reactive protein have been studied, termed the modified Kocher criteria. These criteria have not been tested extensively on septic knee.

Pedicle Screw Plowing in Adolescent Idiopathic Scoliosis: How Common Is It and Is It a Problem?

Study Design: Multicenter retrospective review.

Objective: To calculate overall incidence of pedicle screw "plowing" in adolescent idiopathic scoliosis (AIS) patients who underwent posterior spinal fusion (PSF). To identify risk factors for pedicle screw plowing and associated postoperative outcomes, including loss of correction and revision rate.

Glucosamine amends CNS pathology in mucopolysaccharidosis IIIC mouse expressing misfolded HGSNAT.

The majority of mucopolysaccharidosis IIIC (MPS IIIC) patients have missense variants causing misfolding of heparan sulfate acetyl-CoA:α-glucosaminide N-acetyltransferase (HGSNAT), which are potentially treatable with pharmacological chaperones. To test this approach, we generated a novel HgsnatP304L mouse model expressing misfolded HGSNAT Pro304Leu variant. HgsnatP304L mice present deficits in short-term and working/spatial memory 2-4 mo earlier than previously described constitutive knockout Hgsnat-Geo mice.

Starting and Sustaining an Extracorporeal Membrane Oxygenation Program.

The use of extracorporeal membrane oxygenation (ECMO) is growing rapidly in all patient populations, especially adults for both acute lung or heart failure. ECMO is a complex, high risk, resource-intense, expensive modality that requires appropriate planning, training, and management for successful outcomes. This article provides an optimal approach and the basic framework for initiating a new ECMO program, which can be tailored to meet local needs.