191 results match your criteria: "Nemours A.I. duPont Hospital for Children[Affiliation]"

As curative therapies for pediatric acute myleoid leukemia (AML) remain elusive, identifying potential new treatment targets is vital. We assessed the cell surface expression of CD74, also known as the major histocompatibility complex-II invariant chain, by multidimensional flow cytometry in 973 patients enrolled in the Children's Oncology Group AAML1031 clinical trial (clinicaltrials gov. Identifier: NCT01371981).

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Background And Objectives: The Pediatric Emergency Care Applied Research Network Fluid Therapies Under Investigation in Diabetic Ketoacidosis (DKA) (FLUID) Trial found that rapid fluid infusion does not increase the risk of cerebral injury. Concern persists, however, whether fluid rates should be adjusted for overweight or obese patients. We used the FLUID Trial database to evaluate associations between fluid infusion rate and outcomes in these patients.

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Purpose: Biallelic variants in TARS2, encoding the mitochondrial threonyl-tRNA-synthetase, have been reported in a small group of individuals displaying a neurodevelopmental phenotype but with limited neuroradiological data and insufficient evidence for causality of the variants.

Methods: Exome or genome sequencing was carried out in 15 families. Clinical and neuroradiological evaluation was performed for all affected individuals, including review of 10 previously reported individuals.

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Purpose: Pregnancies affected by maternal or fetal achondroplasia present unique challenges. The optimal route of delivery in fetuses with achondroplasia has not been established. Our objective was to determine whether the route of delivery affects postnatal achondroplasia-related surgical burden.

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Clinical and Laboratory Predictors of Dehydration Severity in Children With Diabetic Ketoacidosis.

Ann Emerg Med

August 2023

Department of Pediatrics, University of California Davis Health, University of California, Davis, School of Medicine, Sacramento, CA; Department of Emergency Medicine, University of California Davis Health, University of California, Davis, School of Medicine, Sacramento, CA.

Article Synopsis
  • The study aimed to assess the dehydration levels in children with diabetic ketoacidosis (DKA) and identify factors (clinical and biochemical) linked to its severity.
  • Analyzed data from 753 children showed that the majority experienced mild to moderate dehydration, with only a small percentage being severely dehydrated.
  • Results indicated that while biochemical factors were more related to dehydration severity, neither physical examination nor biochemical measures were strong enough to guide rehydration strategies effectively.
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Tracheostomy in the extremely premature neonate - Long term outcomes in a multi-institutional study.

Int J Pediatr Otorhinolaryngol

April 2023

Department of Otorhinolaryngology-Head and Neck Surgery, University of Maryland School of Medicine, University of Maryland Children's Hospital, Baltimore, MD, USA. Electronic address:

Objectives: To describe the long-term outcomes related to breathing, feeding, and neurocognitive development in extremely premature infants requiring tracheostomy.

Study Design: Pooled cross-sectional survey.

Setting: Multi-institutional academic children's hospitals.

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Materials with the ability to change properties can expand the capabilities of models of biological processes and diseases as it has become increasingly clear that static, stiff materials with smooth surfaces fall short in recapitulating the cellular microenvironment. Here, we introduce a patterned material that can be rapidly stiffened and softened in response to an external magnetic field through the addition of magnetic inclusions into a soft silicone elastomer with topographic surface patterning. This substrate can be used for cell culture to investigate short-term cellular responses to dynamic stiffening or softening and the interaction with topography that encourages cells to assume a specific morphology.

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Cognitive function following diabetic ketoacidosis in young children with type 1 diabetes.

Endocrinol Diabetes Metab

May 2023

Department of Pediatrics, University of California Davis Health, University of California Davis, School of Medicine, Davis, California, USA.

Introduction: Young children with type 1 diabetes (T1D) may be at particularly high risk of cognitive decline following diabetic ketoacidosis (DKA). However, studies of cognitive functioning in T1D typically examine school-age children. The goal of this study was to examine whether a single experience of DKA is associated with lower cognitive functioning in young children.

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Relationships among biochemical measures in children with diabetic ketoacidosis.

J Pediatr Endocrinol Metab

March 2023

Department of Pediatrics, University of California, Davis Health, University of California Davis, School of Medicine, Sacramento, USA.

Objectives: Investigating empirical relationships among laboratory measures in children with diabetic ketoacidosis (DKA) can provide insights into physiological alterations occurring during DKA. We determined whether alterations in laboratory measures during DKA conform to theoretical predictions.

Methods: We used Pearson correlation statistics and linear regression to investigate correlations between blood glucose, electrolytes, pH and PCO at emergency department presentation in 1,681 pediatric DKA episodes.

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Objective: To determine if removing an ovary for ovarian tissue cryopreservation (OTC) increased rates of primary ovarian insufficiency (POI) in girls undergoing bone marrow transplantation (BMT). Institutional review board approval was obtained from all 3 clinical sites.

Design: Multicenter retrospective cohort study SETTING: Academic children's hospitals PATIENTS: Females aged 2-21 who underwent BMT with or without OTC from 2010 to 2017.

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Genomic and phenotypic characterization of 404 individuals with neurodevelopmental disorders caused by CTNNB1 variants.

Genet Med

November 2022

Adelaide Medical School, The University of Adelaide, Adelaide, South Australia, Australia; Robinson Research Institute, The University of Adelaide, Adelaide, South Australia, Australia. Electronic address:

Article Synopsis
  • Germline loss-of-function variants in the CTNNB1 gene are linked to neurodevelopmental disorders that include spastic diplegia and visual issues, making them a common genetic cause of cerebral palsy (CP).
  • A study analyzed genetic data from 404 individuals with pathogenic CTNNB1 variants, including newly detailed phenotypes for 52 cases, to explore how these variants relate to CP and other traits.
  • Findings showed that individuals with CTNNB1 variants exhibited similar clinical features, suggesting that CP is part of the neurodevelopmental disorder spectrum rather than a separate condition; two specific variants were found to disrupt WNT signaling processes.
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Background: International medical graduates (IMGs) have less burnout than U. S. medical school graduates (USMGs) during residency training.

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Background: Studies of pedal cyclist injuries have largely focused on individual injury categories, but every region of the cyclist's body is exposed to potential trauma. Real-world injury patterns can be complex, and isolated injuries to one body part are uncommon among casualties requiring hospitalization. Latent class analysis (LCA) may identify important patterns in heterogeneous samples of qualitative data.

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Background: Prior studies of early antibiotic use and growth have shown mixed results, primarily on cross-sectional outcomes. This study examined the effect of oral antibiotics before age 24 months on growth trajectory at age 2-5 years.

Methods: We captured oral antibiotic prescriptions and anthropometrics from electronic health records through PCORnet, for children with ≥1 height and weight at 0-12 months of age, ≥1 at 12-30 months, and ≥2 between 25 and 72 months.

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Structural mapping of GABRB3 variants reveals genotype-phenotype correlations.

Genet Med

March 2022

Department of Epilepsy Genetics and Personalized Treatment, The Danish Epilepsy Centre "Filadelfia", Dianalund, Denmark; Department of Regional Health Research, University of Southern Denmark, Odense, Denmark; Department of Medicine, University of Genoa, Genoa, Italy; Department of Clinical Neurophysiology, The Danish Epilepsy Centre "Filadelfia", Dianalund, Denmark. Electronic address:

Purpose: Pathogenic variants in GABRB3 have been associated with a spectrum of phenotypes from severe developmental disorders and epileptic encephalopathies to milder epilepsy syndromes and mild intellectual disability (ID). In this study, we analyzed a large cohort of individuals with GABRB3 variants to deepen the phenotypic understanding and investigate genotype-phenotype correlations.

Methods: Through an international collaboration, we analyzed electro-clinical data of unpublished individuals with variants in GABRB3, and we reviewed previously published cases.

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Objective: Headaches are common among youth and are associated with significant negative outcomes. Despite advances in interdisciplinary treatments for youth with chronic pain, research suggests disparities in access to these services.

Methods: A total of 186 youth (M = 14.

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Introduction: Viral bronchiolitis is a leading cause of admissions to pediatric intensive care unit (PICU). A literature review indicates that there is limited information on fluid overload and the use of diuretics in mechanically ventilated children with viral bronchiolitis. This study was conducted to understand diuretic use concerning fluid overload in this population.

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Statistical kinematic modelling: concepts and model validity.

Comput Methods Biomech Biomed Engin

July 2022

Department Human Structure & Repair, University Ghent, Ghent, Belgium.

Data reduction techniques are applied to reduce the volume of data while maintaining its integrity. For cyclic motion data, a reliable overview comparing these methods is lacking. Therefore, this study aims to evaluate the features of the different data reduction techniques by applying them to large public data sets.

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Background: Obesity rates continue to rise among children and adolescents across the globe. A multicenter research consortium composed of institutions in the Southern US, located in states endemic for childhood obesity, was formed to evaluate the effect of obesity on pediatric musculoskeletal disorders. This study evaluates the effect of body mass index (BMI) percentile and socioeconomic status (SES) on surgical site infections (SSIs) and perioperative complications in patients with adolescent idiopathic scoliosis (AIS) treated with posterior spinal fusion (PSF).

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Morquio syndrome (mucopolysaccharidosis IV/MPS IV) is a genetic disorder leading to skeletal abnormalities and gait deviations. Research on the gait patterns and lower extremity physical characteristics associated with skeletal dysplasia in children with MPS IV is currently limited. This research aimed to provide baseline gait patterns and lower limb skeletal alignment of children with MPS IV utilizing three-dimensional instrumented gait analysis.

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Article Synopsis
  • Subcellular membranes are rich in dolichol, important for protein glycosylation, but its exact role in organelle function and the endosomal-lysosomal pathway is still unclear.
  • Variants in the DHDDS gene, which is essential for dolichol production, are linked to a form of retinitis pigmentosa and various neurodevelopmental disorders, causing symptoms like epilepsy and movement issues in affected patients.
  • Clinical studies showed that patients with DHDDS mutations experienced neurological decline, cognitive issues, and changes in their lysosomal function, suggesting that these variants primarily affect the enzyme's active site and disrupt normal cell processes.
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Background: Despite being at high risk for depression, patients with childhood-onset systemic lupus erythematosus (c-SLE) are infrequently and inconsistently screened for depression by their pediatric rheumatologists. We aimed to systematically increase rates of formal depression screening for c-SLE patients in an academic Pediatric Rheumatology clinic.

Methods: Our multi-disciplinary quality improvement (QI) team used electronic health record (EHR) documentation to retroactively calculate baseline rates of documented depression screening using the Patient Health Questionnaire-9 (PHQ-9).

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Objective: This study examined the association between youth, parent, and family characteristics in mother-father agreement of youth externalizing behaviors among ethnically diverse families.

Method: Eighty-eight mother-father dyads of youth (44% Latino, 38% European-American, 17% African American) 6-16 years participated.

Results: Overall associations between parent's reports of youth behavior problems were positively correlated.

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