Pediatric Transplant and Cellular Therapy Consortium RESILIENT Conference on Pediatric Chronic Graft-versus-Host Disease Survivorship after Hematopoietic Cell Transplantation: Part II. Organ Dysfunction and Immune Reconstitution Considerations for children with chronic GVHD after Hematopoietic Cell Transplantation.

While highly morbid forms of chronic graft versus host disease (cGVHD) and severe late effects of allogeneic hematopoietic cell transplant (HCT) can impact children and adults alike, unique considerations arise in pediatric cases regarding diagnosis, monitoring, treatment, and likelihood of resolution. As children can present with atypical features of cGVHD, and with more significant disease due to inability to communicate symptoms, they may be at increased risk for highly morbid forms of cGVHD and incur greater subsequent late effects, which may be more pronounced in those with underlying chromosomal breakage syndromes, with higher prevalence in pediatric HCT recipients. The long-term effects of cGVHD and its therapies include impaired immune reconstitution, leading to increased risks of infection and secondary malignant neoplasms.

Finding Value in Pediatric Liver Transplantation: When Does Volume Matter?

Introduction: Pediatric liver transplantation provides substantial survival benefit. An emphasis on value-based practices has become a central theme in many surgical fields, but have not been well-studied in pediatric transplantation. Given an increasing focus on optimizing outcomes while containing costs, defining value in pediatric liver transplantation warrants investigation.

MicroRNA Expression in High-Grade B-Cell Lymphoma With 11q Aberration.

Mature aggressive B-cell lymphomas, such as Burkitt lymphoma (BL) and Diffuse large B-cell lymphoma (DLBCL), show variations in microRNA (miRNA) expression. The entity of High-grade B-cell lymphoma with 11q aberration (HGBCL-11q) shares several biological features with both BL and DLBCL but data on its miRNA expression profile are yet scarce. Hence, this study aims to analyze the potential differences in miRNA expression of HGBCL-11q compared to BL and DLBCL.

The ACTION VAD registry: A collective five-year experience.

Background: The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) began in 2018 as a collaborative learning health system committed to improving outcomes in pediatric heart failure, including children and adults with congenital heart disease, supported with ventricular assist devices (VADs). This report describes patient and device characteristics, and outcomes through 1-year post-implant.

Methods: The ACTION VAD registry report was created from data submitted to the ACTION learning network from April 2018 to June 2023.

Comparison of two point-of-care lung ultrasound techniques and their associated outcomes for bronchiolitis in the pediatric emergency department.

Background: Acute bronchiolitis (AB) is the most common lower respiratory tract infection in infants. Clinician diagnosis and management vary due to limited objective assessment tools. Point-of-care lung ultrasound (LUS) offers a promising diagnostic and prognostic tool in the emergency department (ED), however, the time to perform LUS is of concern in the emergency setting.

Six-Month Outcomes in the Long-Term Outcomes After the Multisystem Inflammatory Syndrome in Children Study.

Importance: Multisystem inflammatory syndrome in children (MIS-C) is a life-threatening complication of COVID-19 infection. Data on midterm outcomes are limited.

Objective: To characterize the frequency and time course of cardiac dysfunction (left ventricular ejection fraction [LVEF] <55%), coronary artery aneurysms (z score ≥2.

Prevention and Management of Infectious Complications in Pediatric Patients With Cancer: A Survey Assessment of Current Practices Across Children's Oncology Group Institutions.

Introduction: While clinical practice guidelines (CPGs) for pediatric oncology infection prophylaxis and management exist, few data describe actual management occurring at pediatric oncology centers.

Methods: An electronic survey querying infection management practices in nontransplant pediatric oncology patients was iteratively created by the Children's Oncology Group (COG) Cancer Control and Supportive Care Infectious Diseases Subcommittee and sent to leaders at all COG institutions, limiting each site to one response to represent their institution.

Results: The response rate was 57% (129/227 institutions).

Addressing Late-arriving Surgeons in Support of First-case On-time Starts.

Introduction: First-case on-time starts (FCOTS) is an established metric of perioperative efficiency, impacting global perioperative throughput. Late-arriving surgeons are a common cause of late operating room (OR) starts. This project reflects a quality improvement effort to reduce late surgeon arrivals by 30% for 24 months and improve FCOTS.

Evaluation of Swallowing Dysfunction With Flexible Endoscopic Evaluation of Swallowing in the Neonatal Unit.

Background: Preterm and low-birth-weight infants often experience discoordination of the suck-swallow-breathe pattern, leading to dysrhythmic feeding, inefficient feeding skills, and swallowing dysfunction, increasing the risk of aspiration and respiratory morbidity. While videofluoroscopic swallowing study is commonly utilized to assess swallow function in neonates, flexible endoscopic evaluation of swallowing (FEES) has been an emerging modality and has been utilized routinely at our institution since 2018.

Method: A single-center, retrospective study including 90 infants admitted to the neonatal unit between 2018-2023 who underwent FEES procedure.

The role of brainstem biopsy and targeted therapies in pediatric diffuse midline glioma/diffuse intrinsic pontine glioma.

Pediatric diffuse midline glioma (DMG), including diffuse intrinsic pontine glioma (DIPG), are aggressive brainstem tumors with a dire prognosis, traditionally diagnosed based on MRI characteristics. The recognition that molecular characteristics may determine prognosis and response to therapy has led to a reevaluation of biopsy necessity. This comprehensive review addresses the evolving role of brainstem biopsies in diagnosing and managing these tumors - both within the context of a clinical trial and in routine clinical care.

Influence of Weather on Sickle Cell Disease Vaso-Occlusive Episodes and Acute Chest Syndrome: A Nationwide Sample Analysis.

Background: The clinical manifestations of sickle cell disease (SCD) result in significant morbidity and healthcare costs. The effect of weather as a risk factor for the development of vaso-occlusive episodes (VOEs) has been previously studied, although with variable results. The aim of our retrospective, nationwide study was to determine the association between weather patterns and pediatric VOE and acute chest syndrome (ACS).

Genetic profiling of Wilson disease reveals a potential recurrent pathogenic variant of ATP7B in the Jordanian population.

Objectives: Wilson disease (WD) is an autosomal-recessive disorder that disrupts copper homeostasis. ATPase copper transporting beta (ATP7B) gene is implicated as the disease-causing gene in WD. The common symptoms associated with WD include hepatic, neurological, psychiatric, and ophthalmic manifestations.

The role of MEK inhibition in pediatric low-grade gliomas.

Pediatric low-grade gliomas (pLGGs) are the most common brain tumors in children. Many patients with unresectable tumors experience recurrence or long-term sequelae from standard chemotherapeutics. This mini-review explores the emerging role of MEK inhibitors in the management of pLGGs, highlighting their potential to transform current treatment paradigms.

Wyburn-Mason disease: Management of a Spetzler-Martin grade 5 arteriovenous malformation with predominant thalamic involvement.

Wyburn-Mason disease (WMD) is a rare congenital phakomatosis known for its complex arteriovenous malformations (AVMs) predominantly affecting the brain and ocular structures. We present the case of a 19-year-old female with an unruptured Spetzler-Martin grade 5 left thalamic AVM, who initially exhibited progressive visual impairment and migraines. Following diagnosis, she was treated with trametinib, a MEK inhibitor; however, nine months later, she developed acute complications, including left monocular blindness and right hemisensory loss.

Community Engagement in the BE SAGE Project: Reducing COVID-19 in Hispanic and Low-Income Preschoolers via Testing and Open-Air Garden-Based Education.

Objective: Back to Early Care and Education Safely With Sustainability via Active Garden Education (BE SAGE) involved COVID-19 testing and a free garden-based physical activity and nutrition program at early care and education centers with primarily Hispanic/Latino enrollment. This article describes the project community engagement plan, process, and outcomes focusing on deliberate and intentional staffing, an extensive online presence, and focused outreach.

Methods: BE SAGE purposefully hired bilingual (English/Spanish) and bicultural staff; developed and maintained a large bilingual online presence (website, newsletters, social media), and fostered community partnerships with community health workers (CHWs) and dedicated staff.

A Guide to Pediatric Antibiotic Allergy Testing: A Report From the US Drug Allergy Registry.

Pediatric antibiotic labels are common, and unnecessary antibiotic avoidance is associated with negative personal and public health outcomes; as a result, there is an increasing emphasis on the importance of pediatric antibiotic allergy evaluations. Different testing strategies have been advised, including skin testing and challenge testing with varied doses and duration. Established consensus testing protocols are lacking.

Utility of color Doppler imaging in patients with retinoblastoma treated by intra-arterial chemotherapy.

Background: There are no clinical parameters that predict response to intra-arterial chemotherapy (IAC), which is a first-line treatment for primary and recurrent unilateral retinoblastoma. We evaluated the utility of ophthalmic ultrasound with color Doppler imaging to predict retinoblastoma response to IAC treatment.

Methods: The medical records of 14 retinoblastoma patients (20 eyes) treated with IAC were reviewed retrospectively.