1,298 results match your criteria: "Nemours - Alfred I. duPont Hospital for Children[Affiliation]"

Background: Pediatric hospital medicine (PHM) became a subspecialty of the American Board of Pediatrics (ABP) in 2016. Starting in 2019, residency graduates are required to complete fellowship training to qualify for PHM board eligibility. These requirements pose unique challenges to internal medicine-pediatrics (med-peds) residents interested in practicing combined adult hospital medicine (HM) and PHM.

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Background: The Long-terM OUtcomes after the Multisystem Inflammatory Syndrome In Children (MUSIC) study aims to characterize the frequency and time course of acute and long-term cardiac and non-cardiac sequelae in multisystem inflammatory syndrome in children associated with COVID-19 (MIS-C), which are currently poorly understood.

Methods: This multicenter observational cohort study will enroll at least 600 patients <21 years old who meet the Centers for Disease Control and Prevention case definition of MIS-C across multiple North American centers over 2 years. The study will collect detailed hospital and follow-up data for up to 5 years, and optional genetic testing.

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Article Synopsis
  • The IKZF1 gene alterations are linked to B-cell acute lymphoblastic leukemia (B-ALL) but are not consistently useful for determining patient risk levels due to variable outcomes.
  • Researchers identified a specific deletion at 22q11.22 that correlates with poor prognosis in B-ALL patients with IKZF1 changes after studying multiple cohorts of pediatric patients over ten years.
  • The study, which included over 1300 patients, revealed that focal deletions in 22q11.22 are common and could serve as important indicators for predicting relapse and survival rates among high-risk B-ALL patients.
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Study Design: Multi-center, prospective, observational cohort.

Objective: To compare myelopathic vs. non-myelopathic ambulatory patients in short- and long-term neurologic function, operative treatment, and patient-reported outcomes.

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A Quality Improvement Initiative to Provide Timely Central Vascular Access in a Neonatal Intensive Care Unit.

Adv Neonatal Care

June 2022

Newborn/Infant Intensive Care Unit, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania (Ms Kristoff and Wade, Ms Wang, and Drs Munson and Dysart); Neonatal Intensive Care Unit, Nemours Alfred I. DuPont Hospital for Children, Wilmington, Delaware (Ms Stracuzzi); and Center for Pediatric Nursing Research & Evidence-Based Practice, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania (Dr Birnbaum).

Background: Timely central venous access is essential in the care of critically ill neonates. Peripherally inserted central catheters (PICCs) are the preferred form of central venous access when umbilical venous catheters cannot be placed or are discontinued. However, time delays increase risk for injury from peripheral intravenous lines and may contribute to inconsistent delivery of necessary fluids and medications.

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Background: This prospective study used instrumented gait analysis, patient-reported outcomes, and portable accelerometers to examine walking activity in adults with cerebral palsy (CP).

Research Question: This study aimed to provide objective data and evaluate factors associated with walking activity in adults with CP.

Methods: Participants with CP (ages 25-45 years) completed instrumented gait analysis and patient-reported outcomes, including the Patient Reported Outcome Measurement Information System (PROMIS) and Satisfaction with Life Score (SWLS), and wore a StepWatch for 8 days.

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Challenges with Fassier-Duval rod exchanges in congenital pseudarthrosis of the tibia: explant roadblock and solution.

J Pediatr Orthop B

January 2022

International Center for Limb Lengthening, Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore, Baltimore, Maryland.

Congenital pseudarthrosis of the tibia (CPT) is characterized by anterolateral tibial bowing and hamartomatous periosteum that predisposes it to fracture. Fassier-Duval telescopic rods can improve the structural integrity of bone segments after reconstruction. We present our experience treating CPT with the Fassier-Duval rod and a novel technique for Fassier-Duval exchange that was developed after extraction failed in one patient.

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A Case Report of Myelopathy Following Heroin Overdose in a Child.

Child Neurol Open

July 2021

Section of Neurology, Department of Pediatrics, St. Christopher's Hospital for Children, Drexel University College of Medicine, Philadelphia, PA, USA.

Neurologic complications secondary to heroin abuse in the adult population have been widely described in the literature. With the recent opioid epidemic and increasing rates of heroin abuse in adolescents, pediatricians are now encountering the diagnostic and management challenge of similar complications in children. We report a case of a 16-year-old girl who presented with complete paraplegia after a heroin overdose.

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Purpose: Osteogenesis imperfecta (OI) is a genetic disorder that causes skeletal fragility. For the most fragile infants and young children with OI, intravenous (IV) bisphosphonate administration is essential, but IV access attempts often cause fractures. Port-a-caths help prevent these events, but some surgeons are hesitant to insert these devices in these infants due to lack of data on their safety.

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Objectives: Diabetic ketoacidosis (DKA) is typically characterized by low or low-normal serum sodium concentrations, which rise as hyperglycemia resolves. In retrospective studies, researchers found associations between declines in sodium concentrations during DKA and cerebral injury. We prospectively investigated determinants of sodium concentration changes and associations with mental status alterations during DKA.

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Objectives: There is little research on the rate and risk factors for revision tonsillectomy after primary intracapsular tonsillectomy. Our study aimed to determine the revision rate following intracapsular tonsillectomy, identify patient characteristics that may increase the probability of revision surgery, and report the tonsillar hemorrhage rate after revision.

Study Design: Level III, retrospective case-control study.

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Objective: To examine relationships amongst parental post-traumatic stress symptoms, parental post-traumatic growth, overprotective parenting, and child emotional/behavioural problems in families of children with critical CHD.

Method: Sixty parents (15 fathers) of children aged 1-6 completed online questionnaires assessing parental post-traumatic stress symptoms and post-traumatic growth, overprotective parenting, and child emotional/behavioural problems. Bivariate correlations and mediational analyses were conducted to evaluate overprotective parenting as a mediator of the association between parental post-traumatic stress symptoms and child emotional/behavioural problems.

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Background Recent evaluation of rheumatic heart disease (RHD) mortality demonstrates disproportionate disease burden within the United States. However, there are few contemporary data on US children living with acute rheumatic fever (ARF) and RHD. Methods and Results Twenty-two US pediatric institutions participated in a 10-year review (2008-2018) of electronic medical records and echocardiographic databases of children 4 to 17 years diagnosed with ARF/RHD to determine demographics, diagnosis, and management.

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Expanding the KIF4A-associated phenotype.

Am J Med Genet A

December 2021

Medical Genetics, Institute of Medical Genetics and Pathology, University Hospital Basel, Basel, Switzerland.

Kinesin super family (KIF) genes encode motor kinesins, a family of evolutionary conserved proteins, involved in intracellular trafficking of various cargoes. These proteins are critical for various physiological processes including neuron function and survival, ciliary function and ciliogenesis, and cell-cycle progression. Recent evidence suggests that alterations in motor kinesin genes can lead to a variety of human diseases, including monogenic disorders.

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Background: Methyl-CpG binding protein 2 (MECP2) disorders, including Rett syndrome and MECP2 duplication syndrome, are typified by profound intellectual disability, spasticity, and decline in gross motor function. Unlike scoliosis, linked to disease severity, little has been reported regarding the hip. The aim of this study was to report the prevalence and risk factors of hip displacement (HD) in MECP2 disorders.

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Background: Managing patients with coronal imbalance (CI) and shoulder height asymmetry following scoliosis surgery can be challenging. Little is known about the course of findings over time and whether they improve or persist. The aim was to report the rate of suboptimal radiographic CI or shoulder asymmetry (SA) at 5 years in patients who were already reported to have CI or SA 2 years after surgery for adolescent idiopathic scoliosis (AIS).

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Background: Compressions given during cardiopulmonary resuscitation generate small, ineffective passive ventilations through oscillating waves. Positive end-expiratory pressure increases the volume of these passive ventilations; however, its effect on passive ventilation is unknown. Our objective was to determine if increasing positive end-expiratory pressure during cardiopulmonary resuscitation increases passive ventilation generated by compressions to a clinically significant point.

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Case: Cortical atrophy, or stress shielding, secondary to a large-diameter femoral intramedullary rod was noted over almost a decade in a now 14-year-old girl with osteogenesis imperfecta (OI). After an initial minimally invasive unsuccessful revision, we downsized the left femur rod with realignment and noted restoration of the left femur cortical thickness.

Conclusion: We demonstrate the significant functional impact of stress shielding and its evolution over a protracted period and outline treatment principles.

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Human Growth and Growth Hormone: From Antiquity to the Recominant Age to the Future.

Front Endocrinol (Lausanne)

February 2022

Pediatrics/Endocrinology, University of Virginia, Charlottesville, VA, United States.

Since antiquity Man has been fascinated by the variations in human (and animal) growth. Stories and art abound about giants and little people. Modern genetics have solved some of etiologies at both extremes of growth.

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Background: Children with cerebral palsy (CP) often present with a stiff knee gait pattern because of rectus femoris (RF) spasticity and/or contracture. Rectus femoris transfers (RFTs) and resections are surgical procedures aimed at reducing muscle stiffness, thereby improving knee flexion during the swing phase of gait. Previous research has consistently demonstrated objective benefits of rectus transfer using instrumented gait analysis (IGA).

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Objective: To examine the association between electrocardiographic (ECG) evidence of carditis at the time of Lyme disease evaluation and a diagnosis of Lyme disease.

Study Design: We performed an 8-center prospective cohort study of children undergoing emergency department evaluation for Lyme disease limited to those who had an ECG obtained by their treating clinicians. The study cardiologist reviewed all ECGs flagged as abnormal by the study sites to assess for ECG evidence of carditis.

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This clinical consensus document addresses the assessment, selection, and fitting considerations for non-surgical bone conduction hearing devices (BCHD) for children under the age of 5 years identified as having unilateral or bilateral, permanent conductive or mixed hearing losses. Children with profound unilateral sensorineural hearing losses are not addressed. The document was developed based on evidence review and consensus by The Paediatric Bone Conduction Working Group, which is composed of audiologists from North America who have experience working with BCHDs in children.

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X-linked lymphoproliferative syndrome type 2 (XLP2) is a rare genetic primary immunodeficiency disease caused by mutations in the gene that lead to deficiency of the X-linked inhibitor of apoptosis protein. XLP2 is characterized by dysregulated immune responses and can result in an inflammatory bowel disease (IBD)-like phenotype, a form of monogenic IBD. Patients with XLP2 often succumb to fulminant hemophagocytic lymphohistiocytosis or Epstein-Barr virus infections.

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Alcohol and cannabis use frequently co-occur, which can result in problems from social and academic impairment to dependence (i.e., alcohol use disorder [AUD] and/or cannabis use disorder [CUD]).

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