A5044685593- Childhood Cancer Survivors' Quality of Life
- Central Venous Catheters and Hemodialysis
- Genetic Syndromes and Imprinting
- Clinical Nutrition and Gastroenterology
- Infection Control in Healthcare
- Genetic and Kidney Cyst Diseases
- Ethics and Legal Issues in Pediatric Healthcare
- Chronic Obstructive Pulmonary Disease (COPD) Research
- Smoking Behavior and Cessation
- Eosinophilic Esophagitis
- Transplantation: Methods and Outcomes
- Cardiac Structural Anomalies and Repair
- Urinary Tract Infections Management
- Palliative Care and End-of-Life Issues
- Vascular Malformations and Hemangiomas
- Eosinophilic Disorders and Syndromes
- Angiogenesis and VEGF in Cancer
- Parathyroid Disorders and Treatments
- Myasthenia Gravis and Thymoma
- Gestational Diabetes Research and Management
- Thermal Regulation in Medicine
- Acupuncture Treatment Research Studies
- Williams Syndrome Research
- Genomics and Rare Diseases
- Opioid Use Disorder Treatment
The University of Texas Southwestern Medical Center
2020-2025
Children's Medical Center
2022-2025
Centers for Disease Control and Prevention
2021-2024
University of Florida
2024
Johns Hopkins Medicine
2002-2022
Johns Hopkins University
2002-2022
University of Colorado Denver
2017-2022
Pediatrics and Genetics
2020
Children's Hospital Colorado
2017-2020
Medical City Children's Hospital
2020
BACKGROUND: In the United States in 2019, there was an outbreak of electronic cigarette, or vaping, product use–associated lung injury (EVALI). The manifestations EVALI adolescents are not well characterized. We describe diagnosis, evaluation, and management hospitalized at a tertiary care, university-affiliated children’s hospital. METHODS: A multidisciplinary committee developed algorithm on basis guidelines from Centers for Disease Control Prevention. retrospective chart review conducted...
Swallowed topical steroids (STS) are the only effective pharmacological therapy for eosinophilic esophagitis (EoE). Thus far, studies of small populations EoE patients have reported conflicting results in relation to adrenal insufficiency (AI). We sought measure AI a clinical setting children taking STS EoE. performed quality improvement study pediatric seen multidisciplinary clinic, who were treated with at least 3 months. Two hundred twenty-five completed questionnaires assess signs AI....
Dravet syndrome is an intractable childhood epilepsy most often associated with SCN1A mutation. In our clinical practice, several patients were noted to have short stature and endocrine dysfunction. This has not been reported in the literature. Our study aim was describe growth measurements abnormalities children syndrome.A retrospective chart review performed at a single institution. Eligibility criteria included genetic (SCN1A) diagnosis of syndrome. Records reviewed for height weight...
Abstract Megalencephaly‐capillary malformation syndrome (MCAP) is a brain overgrowth disorder characterized by cortical malformations (specifically polymicrogyria), vascular anomalies, and segmental secondary to somatic activating mutations in the PI3K‐AKT–MTOR pathway ( PIK3CA ). Cases of growth failure hypoglycemia have been reported patients with MCAP, raising suspicion for unappreciated hormone (GH) deficiency. Here we report an observational multicenter study children MCAP GH Eleven...
To describe the doses of opioids and benzodiazepines administered around time terminal extubation (TE) to children who died within 1 hour TE identify their association with death (TTD).Secondary analysis data collected for Death One Hour After Terminal Extubation study.Nine U.S. hospitals.Six hundred eighty patients between 0 21 years after (2010-2021).Medications included total 24 hours before TE. Correlations drug TTD in minutes were calculated, multivariable linear regression performed...
Abstract GNAS is a complex gene that through use of alternative first exons encodes signaling proteins Gα s and XLαs plus neurosecretory protein NESP55. Tissue‐specific expression these regulated reciprocal genomic imprinting in fully differentiated developed tissue. Mutations account for several human disorders, including McCune‐Albright syndrome Albright hereditary osteodystrophy, further knowledge may provide insights into variable phenotypes disorders. We therefore analyzed , NESP55,...
Infantile hepatic hemangiomas (IHHs) are the most common tumors of infancy and occur in approximately 5% children younger than age 1 year (1–4). Hemangiomas postulated to arise from dysregulated angiogenesis (3,5), vascular endothelial growth factor (VEGF) may play a role development these tumors; thus, serum levels VEGF can be used for diagnostic therapeutic guidance (2). could different proliferative versus involuting (6). A recently published study has shown that corticosteroids suppress...
Objective: To evaluate the impact of type and dose swallowed topical steroids (STS) concurrent steroid therapy on development resolution adrenal insufficiency (AI) in pediatric eosinophilic esophagitis (EoE). Methods: We performed a retrospective case-control study EoE subjects single tertiary care center, who were treated with STS for at least 3 months diagnosed AI based peak stimulated cortisol level <18 µg/dL (500 nmol/L). Steroid forms doses, endoscopy data collected time diagnosis or...
Focal dermal hypoplasia (FDH) is a rare genetic disorder caused by mutations in the PORCN gene located on X chromosome. Short stature was previously noted to be common finding FDH, however etiology of this unclear. The present study sought elucidate specific causes for short assessing growth charts, determining bone ages and auxologic measurements, examining laboratory data failure, dietary intake, performing hormone stimulation test. Sixteen patients with FDH between 3 18 years age...
Aims & Objectives: Our recent Association of Enteral Nutrition and Dexmedetomidine Usage for Critically Ill Children Requiring Non-Invasive Ventilation (NIV Dex EN) study aimed to investigate the relationship between enteral nutrition (EN) sedation in critically ill children acutely supported with non-invasive ventilation (NIV). Here we aim describe current feeding practices on NIV United States a large sample. Methods: Retrospective patients at least 12 hours over one-year period fourteen...
Aims & Objectives: There is growing pediatric safety evidence for enteral nutrition (EN) during non-invasive ventilation (NIV) as well dexmedetomidine the primary sedative of choice to improve tolerance NIV. Research exploring impact two practices lacking. We aimed describe in a multicenter study potential association EN with requirements children supported NIV respiratory compromise, hypothesizing that those fed would require less sedation. Methods: Retrospective one-year sample 14 U.S....
Infections cause substantial morbidity and mortality among patients receiving care in outpatient hemodialysis facilities. We describe comprehensive infection prevention assessments by US public health departments using standardized interview observation tools. Results demonstrated how facility layouts can undermine that clinical practices often fall short of policies.
Expression of the human hsp70 gene is cell cycle regulated and inducible by both serum adenovirus E1a protein (K. Milarski R. Morimoto, Proc. Natl. Acad. Sci. USA 83:9517-9521, 1986; M. C. Simon, K. Kitchener, H.-T. Kao, E. Hickey, L. Weber, Voellmy, N. Heintz, J. Nevins, Mol. Cell. Biol. 7:2884-2890, 1987; B. Wu, H. Hurst, Jones, 6:2994-2999, Wu 82:6070-6074, 1985). This expression predominantly controlled CCAAT element at position -70 relative to transcriptional initiation site (G....
Abstract Context: Patients with Albright hereditary osteodystrophy (AHO) have defects in stimulatory G protein signaling due to loss of function mutations GNAS. The mechanism by which these lead the AHO phenotype has been difficult establish inaccessibility affected tissues. Objective: objective study was gain insight into downstream consequences abnormal human epithelial and Design: We assessed transcription GNAS Gsα-stimulated activation cystic fibrosis transmembrane conductance regulator...
Terminal extubation (TE) and terminal weaning (TW) during withdrawal of life-sustaining therapies (WLSTs) have been described defined in adults. The recent Death One Hour After Extubation study aimed to validate a model developed predict whether child would die within 1 hour after discontinuation mechanical ventilation for WLST. Although TW has not children, pre-extubation known occur before WLST, though what extent is unknown. In this preplanned secondary analysis, we aim describe/define TE...