Spatial correspondence of cortical activity measured with whole head fNIRS and fMRI: Toward clinical use within subject.

Functional near infrared spectroscopy (fNIRS) and functional magnetic resonance imaging (fMRI) both measure the hemodynamic response, and so both imaging modalities are expected to have a strong correspondence in regions of cortex adjacent to the scalp. To assess whether fNIRS can be used clinically in a manner similar to fMRI, 22 healthy adult participants underwent same-day fMRI and whole-head fNIRS testing while they performed separate motor (finger tapping) and visual (flashing checkerboard) tasks. Analyses were conducted within and across subjects for each imaging approach, and regions of significant task-related activity were compared on the cortical surface.

The Current State of Neonatal Neurodevelopmental Follow-up Programs in North America: A Children's Hospitals Neonatal Consortium Report.

Objective:  This study aimed to determine neonatal neurodevelopmental follow-up (NDFU) practices across academic centers.

Study Design:  This study was a cross-sectional survey that addressed center-specific neonatal NDFU practices within the Children's Hospitals Neonatal Consortium (CHNC).

Results:  Survey response rate was 76%, and 97% of respondents had a formal NDFU program.

Hybrid Closed Loop Systems Improve Glycemic Control and Quality of Life in Historically Minoritized Youth with Diabetes.

We assessed changes in glycemic control and person-reported outcome measures (PROMs) with t:slim X2 insulin pump with Control-IQ technology use among historically minoritized youth who are least likely to access hybrid closed loop (HCL) technology. This single-arm, prospective pilot study enrolled 15 publicly insured, insulin pump-naïve, non-Hispanic Black youth ages 6 to <21 years with type 1 diabetes and hemoglobin A (HbA) ≥10% in a 6-month study of HCL use. The primary outcome was absolute change in time in range (TIR) (70-180 mg/dL).

Knowledge gaps in heart and lung donation after the circulatory determination of death: Report of a workshop of the National Heart, Lung, and Blood Institute.

In a workshop sponsored by the U.S. National Heart, Lung, and Blood Institute, experts identified current knowledge gaps and research opportunities in the scientific, conceptual, and ethical understanding of organ donation after the circulatory determination of death and its technologies.

Real-world treatment patterns and outcomes in patients with primary hemophagocytic lymphohistiocytosis treated with emapalumab.

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening, hyperinflammatory syndrome. Emapalumab, a fully human monoclonal antibody that neutralizes the proinflammatory cytokine interferon gamma, is approved in the United States to treat primary HLH (pHLH) in patients with refractory, recurrent, or progressive disease, or intolerance with conventional HLH treatments. REAL-HLH, a retrospective study, conducted across 33 US hospitals, evaluated real-world treatment patterns and outcomes in patients treated with ≥1 dose of emapalumab between 20 November 2018 and 31 October 2021.

Brain injury and neurodevelopmental outcomes in children undergoing surgery for congenital heart disease.

Objectives: Brain injury is commonly seen on magnetic resonance imaging in infants with complex congenital heart disease. The impact of perioperative brain injury on neurodevelopmental outcomes is not well understood. We evaluate the association of brain injury and other markers on neurodevelopmental outcomes in patients undergoing surgery for congenital heart surgery during infancy.

Continuity Strategies for Long-Stay PICU Patients: Consensus Statements From the Lucile Packard Foundation PICU Continuity Panel.

Objectives: To develop consensus statements on continuity strategies using primary intensivists, primary nurses, and recurring multidisciplinary team meetings for long-stay patients (LSPs) in PICUs.

Participants: The multidisciplinary Lucile Packard Foundation PICU Continuity Panel comprising parents of children who had prolonged PICU stays and experts in several specialties/professions that care for children with medical complexity in and out of PICUs.

Design/methods: We used modified RAND Delphi methodology, with a comprehensive literature review, Delphi surveys, and a conference, to reach consensus.

Outcomes of rheumatic fever in Uganda: a prospective cohort study.

Background: Rheumatic heart disease is the largest contributor to cardiac-related mortality in children worldwide. Outcomes in endemic settings after its antecedent illness, acute rheumatic fever, are not well understood. We aimed to describe 3-5 year mortality, acute rheumatic fever recurrence, changes in carditis, and correlates of mortality after acute rheumatic fever.

The IAAM LTBP4 Haplotype is Protective Against Dystrophin-Deficient Cardiomyopathy.

Background: Dilated cardiomyopathy (DCM) is a major complication of, and leading cause of mortality in Duchenne muscular dystrophy (DMD). Its severity, age at onset, and rate of progression display wide variability, whose molecular bases have been scarcely elucidated. Potential DCM-modifying factors include glucocorticoid (GC) and cardiological treatments, DMD mutation type and location, and variants in other genes.

Utility of apical four-chamber longitudinal strain in the assessment of childhood cancer survivors: A multicenter study.

Background: A previous multicenter study showed that longitudinal changes in standard cardiac functional parameters were associated with the development of cardiomyopathy in childhood cancer survivors (CCS). Evaluation of the relationship between global longitudinal strain (GLS) changes and cardiomyopathy risk was limited, largely due to lack of quality apical 2- and 3-chamber views in addition to 4-chamber view. We sought to determine whether apical 4-chamber longitudinal strain (A4LS) alone can serve as a suitable surrogate for GLS in this population.

Dysfunctional Postnatal Mitochondrial Energy Metabolism in a Patient with Neurodevelopmental Defects Caused by Intrauterine Growth Restriction Due to Idiopathic Placental Insufficiency.

We report the case of a four-year-old male patient with a complex medical history born prematurely as the result of intrauterine growth restriction due to placental insufficiency. His clinical manifestations included severe neurodevelopmental deficits, global developmental delay, Pierre-Robin sequence, and intractable epilepsy with both generalized and focal features. The proband's low levels of citrulline and lactic acidosis provoked by administration of Depakoke were evocative of a mitochondrial etiology.

Navigating the post-Dobbs landscape: ethical considerations from a perinatal perspective.

Restrictive abortion laws have impacts reaching far beyond the immediate sphere of reproductive health, with cascading effects on clinical and ethical aspects of neonatal care, as well as perinatal palliative care. These laws have the potential to alter how families and clinicians navigate prenatal and postnatal medical decisions after a complex fetal diagnosis is made. We present a hypothetical case to explore the nexus of abortion care and perinatal care of fetuses and infants with life-limiting conditions.

Cholesteatoma: Canalplasty for External Auditory Stenosis in a Pediatric Patient.

External auditory canal (EAC) stenosis is the narrowing of the external auditory meatus to less than 4 mm. Severe stenosis of the EAC may inhibit the ability to conduct sound and may lead to the formation of a cholesteatoma. While most cases of EAC stenosis may be managed nonoperatively, the significant impact that the associated symptoms can have on patients may require surgical intervention.

DON in pediatric cerebral malaria, a phase I/IIA dose-escalation safety study: study protocol for a clinical trial.

Background: Despite treatment with highly effective antimalarial drugs, malaria annually claims the lives of over half a million children under 5-years of age in sub-Saharan Africa. Cerebral malaria (CM), defined as Plasmodium falciparum infection with coma, is the severe malaria syndrome with the highest mortality. Studies in the CM mouse model suggest that a T cell-mediated response underlies CM pathology, opening a new target for therapy in humans.

Challenges and opportunities to bridge translational to clinical research for personalized mitochondrial medicine.

Mitochondrial disorders are a group of rare and heterogeneous genetic diseases characterized by dysfunctional mitochondria leading to deficient adenosine triphosphate synthesis and chronic energy deficit in patients. The majority of these patients exhibit a wide range of phenotypic manifestations targeting several organ systems, making their clinical diagnosis and management challenging. Bridging translational to clinical research is crucial for improving the early diagnosis and prognosis of these intractable mitochondrial disorders and for discovering novel therapeutic drug candidates and modalities.

variants in are associated with hearing impairment, ocular pathology, and cardiac defects.

Phospholipase C isozymes (PLCs) hydrolyze phosphatidylinositol 4,5-bisphosphate into inositol 1,4,5-trisphosphate and diacylglycerol, important signaling molecules involved in many cellular processes. encodes the PLCγ1 isozyme that is broadly expressed. Hyperactive somatic mutations of are observed in multiple cancers, but only one germline variant has been reported.

Characteristics of Health Care Settings Where Adolescents and Young Adults Receive Care for ALL.

Purpose: Individuals diagnosed with cancer between 15 and 39 years (adolescent and young adult [AYA]) face unique vulnerability. Detail is lacking about care delivery for these patients, especially those with ALL. We address these knowledge gaps by describing AYA ALL care delivery details at National Cancer Institute Community Oncology Research Program (NCORP) (sub)affiliates by model of care.

Computer Simulation to Assess Emergency Department Length of Stay in Pediatric Traumatic Brain Injury.

Objectives: Our study aimed to identify how emergency department (ED) arrival rate, process of care, and physical layout can impact ED length of stay (LOS) in pediatric traumatic brain injury care.

Methods: Process flows and value stream maps were developed for 3 level I pediatric trauma centers. Computer simulation models were also used to examine "what if" scenarios based on ED arrival rates.