Achieving Language Justice in Pediatric Hematology-Oncology: A Multinational Perspective for Language-Concordant Equitable Patient- and Family-Centered Care and Research Inclusion.

Language-discordant healthcare encounters-when the patient/caregiver and clinician are not able to communicate directly in the patient's/caregiver's preferred language-are associated with worse quality of care, increased adverse events, and research exclusion. Here, we describe the current state of language justice in clinical practice and research in the United States, Canada, and Spain, discuss the role of social determinants of health and language, in patient safety and health outcomes and review an example of culturally and linguistically concordant interventions to increase research participation. We close with practical and global strategies to increase multilingual research participation and to provide equitable patient- and family-centered care in pediatric hematology-oncology.

Genomic and phenotypic correlates of mosaic loss of chromosome Y in blood.

Mosaic loss of Y (mLOY) is the most common somatic chromosomal alteration detected in human blood. The presence of mLOY is associated with altered blood cell counts and increased risk of Alzheimer disease, solid tumors, and other age-related diseases. We sought to gain a better understanding of genetic drivers and associated phenotypes of mLOY through analyses of whole-genome sequencing (WGS) of a large set of genetically diverse males from the Trans-Omics for Precision Medicine (TOPMed) program.

Effects of 24-Hour Diet- or Exercise-Induced Energy Availability Manipulations on Substrate Utilization and Performance.

Purpose: To examine sex-based differences in substrate oxidation, postprandial metabolism, and performance in response to 24-hour manipulations in energy availability (EA), induced by manipulations to energy intake (EI) or exercise energy expenditure (EEE).

Methods: In a Latin Square design, 20 endurance athletes (10 females using monophasic oral contraceptives and 10 males) undertook five trials, each comprising three consecutive days. Day one was a standardized period of high EA; EA was then manipulated on day two; post-intervention testing occurred on day three.

Socioeconomic, Race-Ethnicity, Household, and Infrastructure Disparities of Hematologic Cancer Outcomes in the US.

Although social determinants of health (SDoH) investigations have shown limited analyses of socioeconomic and race-ethnic status on certain hematologic malignancies, the impact of factors beyond those across a fuller scope of hematologic cancers remains unknown. The Social Vulnerability Index (SVI), a tool for assessing varied US-census derived sociodemographic factors, allows the specific quantification of SDoH in dynamic, regional contexts for their associations with hematologic-malignancy inequities. To assess the summative influence of varied SDoH-factors on hematologic malignancy outcomes and discern which SDoH-factors contributed the largest associations towards disparities 796,005 adults with hematologic malignancies between 1975-2017 were identified for this retrospective cohort study.

Neurodevelopmental Outcomes after Multiple General Anesthetic Exposures before 5 Years of Age: A Cohort Study.

Background: The general anaesthesia or awake-regional anaesthesia in infancy (GAS) trial demonstrated evidence that most neurodevelopmental outcomes at 2 and 5 yr of age in infants who received a single general anesthetic for elective inguinal herniorrhaphy were clinically equivalent when compared to infants who did not receive general anesthesia. More than 20% of the children in the trial had at least one subsequent anesthetic exposure after their initial surgery. Using the GAS database, this study aimed to address whether multiple (two or more) general anesthetic exposures compared to one or no general anesthetic exposure in early childhood were associated with worse neurodevelopmental outcomes at 5 yr.

Formulation of Quality Improvement Methodology for Risk Mitigation in Congenital Cardiac Catheterization.

The C3PO collaborative, with a history of successful quality improvement (QI) initiatives, leveraged registry participants to develop a multi-center QI initiative to reduce adverse events (AEs) in congenital cardiac catheterization. A 32-person, interdisciplinary working group analyzed audited data for all congenital cardiac catheterization cases from 2014-2017. The primary outcome was the occurrence of any high-severity (level 3/4/5) AE.

Outcomes for infants with BRUE diagnosed with oropharyngeal dysphagia or gastroesophageal reflux disease: a multicenter study from the Pediatric Health Information System Database.

We aimed to determine the prevalence of gastroesophageal reflux disease (GERD) and oropharyngeal dysphagia as explanatory diagnoses, risk factors for acid suppression treatment, and risk factors for repeat hospital visit in infants hospitalized after brief resolved unexplained event (BRUE) using a multicenter pediatric database. We performed a multicenter retrospective database study of infants admitted with BRUE in the Pediatric Health Information System between 2016 and 2021. Data included diagnostic testing, explanatory diagnoses, treatment with acid suppression, and related repeat hospital visits within 6 months.

Skeletal surveys for suspected infant abuse: patient-specific radiation dose estimation using a hybrid computational phantom.

Background: Radiographic skeletal survey plays an important role in the diagnosis of infant abuse. Some practitioners have expressed concerns about the radiation exposure from this examination.

Objective: To utilize state-of-the-art hybrid computational phantoms to more accurately estimate radiation doses of skeletal surveys performed for suspected infant abuse.

Home Health Care Research for Children With Disability and Medical Complexity.

Pediatric home health care represents a vital system of care for children with disability and medical complexity, encompassing services provided by family caregivers and nonfamily home health care providers and the use of durable medical equipment and supplies. Home health care is medically necessary for the physiologic health of children with disability and medical complexity and for their participation and function within home, school, and community settings. While the study of pediatric home health care in the United States has increased in the last decade, its research remains primarily methodologically limited to observational studies.

Perioperative Management of Patients with Preeclampsia: A Comprehensive Review.

Preeclampsia is a common condition of pregnancy characterized by hypertension complicated by cerebral, cardiac, hepatic, renal, hematologic, and placental dysfunction. Patients with preeclampsia frequently undergo cesarean delivery, the most common major surgical procedure in the world. They represent a high-risk perioperative cohort suffering significant preventable morbidity and mortality.

Blended phenotype of TECPR2-associated hereditary sensory-autonomic neuropathy and Temple syndrome.

Uniparental isodisomy (UPiD) can cause mixed phenotypes of imprinting disorders and autosomal-recessive diseases. We present the case of a 3-year-old male with a blended phenotype of TECPR2-related hereditary sensory and autonomic neuropathy (HSAN9) and Temple syndrome (TS14) due to maternal UPiD of chromosome 14, which includes a loss-of-function founder variant in the TECPR2 gene [NM_014844.5: c.

Outcomes following resective and disconnective strategies in the treatment of epileptic spasms: a systematic review of the literature and individual patient data meta-analysis.

Epileptic spasms (ES) are a unique seizure type typically presenting in the form of infantile epileptic spasms syndrome (IESS) with characteristic hypsarrhythmia on scalp EEG and a preponderance with developmental delay or regression. While pharmacotherapy is the mainstay of treatment, surgical options, including disconnective or resective procedures, are increasingly recognized as viable therapeutic options for recurrent or persistent ES. However, limited data on safety, effectiveness, and prognostic factors hinder informed decision-making regarding surgery indications, timing, and intervention type.

Identifying risk factors for vomiting during diarrhea: A secondary analysis of a randomized trial of zinc supplementation.

Objectives: Supplemental zinc during acute diarrhea reduces illness duration but also increases vomiting. In a recent trial, we found that children receiving lower daily doses of zinc (5 mg or 10 mg vs. 20 mg) had lower rates of vomiting with comparable stool output and duration of diarrhea.

Predictive equation derived from 6,497 doubly labelled water measurements enables the detection of erroneous self-reported energy intake.

Nutritional epidemiology aims to link dietary exposures to chronic disease, but the instruments for evaluating dietary intake are inaccurate. One way to identify unreliable data and the sources of errors is to compare estimated intakes with the total energy expenditure (TEE). In this study, we used the International Atomic Energy Agency Doubly Labeled Water Database to derive a predictive equation for TEE using 6,497 measures of TEE in individuals aged 4 to 96 years.

Specialized pericyte subtypes in the pulmonary capillaries.

Pericytes are essential for capillary stability and homeostasis, with impaired pericyte function linked to diseases like pulmonary arterial hypertension. Investigating pericyte biology has been challenging due to the lack of specific markers, making it difficult to distinguish pericytes from other stromal cells. Using bioinformatic analysis and RNAscope, we identified Higd1b as a unique gene marker for pericytes and subsequently generated a knock-in mouse line, Higd1b-CreERT2, that accurately labels pericytes in the lung and heart.

Tenecteplase for the Treatment of Pediatric Arterial Ischemic Stroke: A Safety Surveillance Report.

Objectives: Intravenous tenecteplase (TNK) is increasingly used to treat adult patients with acute arterial ischemic stroke, but the risk profile of TNK in childhood stroke is unknown. This study aims to prospectively gather safety data regarding TNK administration in children.

Methods: Since December 2023, a monthly email survey was sent to participants recruited from the International Pediatric Stroke Study and Pediatric Neurocritical Care Research Group querying recent experience with TNK in childhood stroke.

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.

Safety and Efficacy of IV Onasemnogene Abeparvovec for Pediatric Patients With Spinal Muscular Atrophy: The Phase 3b SMART Study.

Background And Objectives: Safety and efficacy of IV onasemnogene abeparvovec has been demonstrated for patients with spinal muscular atrophy (SMA) weighing <8.5 kg. SMART was the first clinical trial to evaluate onasemnogene abeparvovec for participants weighing 8.

Treatment of focal anaplastic Wilms tumor: A report from the Children's Oncology Group AREN0321 and AREN03B2 studies.

Background: In the fifth National Wilms Tumor Study, patients received vincristine and dactinomycin (VA) without radiation for stage I focal anaplastic Wilms tumor (FAWT) and VA plus doxorubicin (DD4A) and radiation for stage II-IV FAWT. Four-year event-free survival (EFS) and overall survival (OS) for stage I FAWT were 67.5% and 88.