A5021705160- Congenital heart defects research
- Neonatal Respiratory Health Research
- Ultrasound in Clinical Applications
- Congenital Heart Disease Studies
- Central Venous Catheters and Hemodialysis
- Congenital Diaphragmatic Hernia Studies
- Cardiovascular Conditions and Treatments
- Neuroscience of respiration and sleep
- Hemodynamic Monitoring and Therapy
- Neonatal and fetal brain pathology
- Respiratory Support and Mechanisms
- Child and Adolescent Health
- Developmental Biology and Gene Regulation
- Eosinophilic Esophagitis
- Genomics and Chromatin Dynamics
- Genomic variations and chromosomal abnormalities
- Infant Development and Preterm Care
- Genetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities
- Phonocardiography and Auscultation Techniques
- Inflammatory Bowel Disease
- Cerebral Palsy and Movement Disorders
- Cancer-related gene regulation
- Gastrointestinal Bleeding Diagnosis and Treatment
- Genetics and Neurodevelopmental Disorders
- Airway Management and Intubation Techniques
Children's Hospital of Philadelphia
2014-2025
University of Pennsylvania
2012-2023
Philadelphia University
2008-2019
RELX Group (United States)
2019
Philadelphia Fire Department
2018
Cardiovascular Institute Hospital
2008
Hôpital Charles-Nicolle
2004
University of Washington
2000
Notch signaling is vital for proper cardiovascular development and function in both humans animal models. Indeed, mutations either JAGGED or NOTCH cause congenital heart disease are associated with adult valvular disease. typically functions to mediate developmental interactions between adjacent tissues. Here we show that absence of the ligand Jagged1 inhibition second field tissues results murine aortic arch artery cardiac anomalies. In mid-gestation, these mutants displayed decreased Fgf8...
TBX1 encodes a DNA binding transcription factor that is commonly deleted in human DiGeorge syndrome and plays an important role heart development. Mechanisms of Tbx1 function, such as interacting regulatory proteins transcriptional target specificity, are largely unknown. Ash2l the mammalian homolog Drosophila Ash2 (absent small homeotic 2) core component multimeric histone methyltransferase complex epigenetically regulates via methylation lysine residues. We undertook unbiased yeast...
DiGeorge syndrome (DGS) is the most common human chromosomal deletion and frequently associated with deletions on chromosome 22q11. Approximately 17% of patients phenotypic features this have no detectable genomic deletion. Animal studies using mouse models implicated Tbx1 as a critical gene within commonly deleted region, several mutations in TBX1 been identified recently non-deleted patients, including missense frameshift mutations. The mechanisms by which these cause disease remained...
The ductus arteriosus (DA) shunts blood away from the lungs during fetal life, but at birth this shunt is no longer needed and vessel rapidly constricts. Postnatal persistence of DA, patent (PDA), predominantly a detrimental condition for preterm infants simultaneously required to maintain systemic flow born with certain severe congenital heart defects. Although PDA in associated significant morbidities, there controversy regarding whether truly causative. Despite advances our understanding...
Failure of the ductus arteriosus (DA) to close at birth can lead serious complications. Conversely, certain profound congenital cardiac malformations require DA be patent until corrective surgery performed. In each instance, clinicians have a very limited repertoire therapeutic options their disposal - indomethacin or ibuprofen (PDA) and prostaglandin E1 maintain patency DA. Neither treatment is specific both may deleterious off-target effects. Therefore, more specifically targeted should...
The family of Ap2 transcription factors comprises five members with highly conserved DNA-binding domains. Among the members, Ap2delta is most divergent, because it lacks residues within transactivation domain (TAD) and has weak affinity for known binding sites. To identify specific coactivators/regulators during development, we performed a yeast two-hybrid screen, using Ap2delta's TAD. We identified trithorax superfamily member, Ash2l, as partner that interacts exclusively Ap2delta. showed...
OBJECTIVES: Umbilical venous cannulation is the favored approach to perinatal central access worldwide but has a failure rate of 25–50% and insertion technique not evolved in decades. Improving success this procedure would have broad implications, particularly where peripherally inserted catheters are easily obtained neonates with congenital heart disease, whom umbilical facilitates administration inotropes blood products while sparing vessels essential for later cardiac interventions. We...
Abstract Background Pulmonary artery acceleration time measured by echocardiography inversely correlates with pulmonary pressures in adults and children older than 1 year of age. There is a paucity data investigating this relationship young children, particularly among preterm infants. Objective To characterize the between ( PAAT ) Design/Methods Patients ≤ age at Children's Hospital Philadelphia 2011 2017 were reviewed. Infants congenital heart disease excluded, except those patent ductus...
Jun is a highly conserved member of the multimeric activator protein 1 transcription factor complex and plays an important role in human cancer where it known to be critical for proliferation, cell cycle regulation, differentiation, death. All these biological functions are also crucial embryonic development. Although all null mouse embryos die at mid-gestation with persistent truncus arteriosus, severe cardiac outflow tract defect seen congenital heart disease, developmental mechanisms...
<b><i>Background:</i></b> Prophylactic indomethacin reduces the risk of severe intraventricular hemorrhage (IVH) but does not reduce death or neurodevelopmental impairment (NDI) among extremely low birth weight (ELBW) infants. Some investigators have suggested that prophylactic may a greater treatment effect on IVH infants at high for IVH. <b><i>Objective:</i></b> To determine whether relative effects and composite outcome NDI vary based...
Background Intraventricular hemorrhage (IVH) is a common complication of prematurity and has been associated with adverse short long term outcomes. Despite the identification risk factors for IVH, overall rate remained stable during last decade. Objectives Our aim was to reduce incidence severe IVH in preterm infants (<30 weeks) our NICU from 8% 4% between October 2014 December 2015. Methods A multidisciplinary team drafted charter using Model Improvement (fig 1). Implementation began...
There has been a large amount of recent literature suggesting that ω-3 unsaturated fatty acids found in fish oils should be incorporated into the diet for purpose decreasing serum cholesterol levels. Inclusion these shown to decrease total as well low-density lipoprotein cholesterol. Some trials have complicated by fact many subjects are afflicted with non-insulin-dependent diabetes mellitus. Unfortunately, effects on insulin secretion not characterized. In this study, we examined effect...