Pediatrics Commons^™

Presurgical Ct Evaluation Of Congenital Aural Atresia, V. T. Nguyen, G. Paek, J. Hu, L. P. Smith

Journal Articles

Congenital aural atresia occurs in approximately 1 in 10,000‐20,000 births and may be surgically repaired if the middle ear malformation is limited in character. External auditory canal atresia is difficult to repair surgically, with significant risks and complications. Surgical candidacy in congenital aural atresia is based on multiple factors, central to which are the anatomy of the temporal bone and audiometric findings. High-resolution multidetector CT is the imaging technique of choice for anatomy delineation, although there are some specific indications for MR imaging in presurgical assessment. Various CT grading systems have been developed to determine surgical candidacy and are described …

Poly(I:C) Induces Controlled Release Of Il-36gamma From Keratinocytes In The Absence Of Cell Death, A. A. Rana, A. V. Lucs, J. Devoti, L. Blanc, J. Papoin, R. Wu, C. J. Papayannakos, A. Abramson, V. R. Bonagura, B. M. Steinberg

Journal Articles

The epithelium is part of an integrated immune system where cytokines, toll-like receptors and their ligands, and extracellular vesicles play a crucial role in initiating an innate immune response. IL-36gamma is a pro-inflammatory member of the IL-1 family that is mainly expressed by epithelial cells, but regulation of its expression and release are only beginning to be understood. Previous studies reported that IL-36gamma is abundant in recurrent respiratory papillomatosis, a rare but devastating disease caused by human papillomaviruses (HPV) types 6 and 11, in which papillomas recurrently grow in and block the airway. Despite the overexpression of IL-36gamma, papilloma tissues …

2015 Equity And Diversity Report, Children's Mercy Hospital

Equity & Diversity Annual Reports

A report on equity and diversity at Children's Mercy Hospitals & Clinics, in the care it provides, the people it serves, and in its workforce. Specifically discusses the Equity & Diversity Council, The Office of Equity and Diversity,Patient-Provider Communications, Care Delivery and Support Mechanisms, Workforce Diversity and Inclusion, Education and Training, Public Accountability and Quality Improvement, Community Engagement, and Research.

Effects Of Obesity On Health-Related Quality Of Life In Juvenile-Onset Systemic Lupus Erythematosus, R. Mina, M. S. Klein-Gitelman, S. Nelson, B. A. Eberhard, G. Higgins, N. G. Singer, K. Onel, L. Tucker, K. M. O'Neil, H. I. Brunner, +4 Additional Authors

Journal Articles

OBJECTIVE: This study evaluated the effects of obesity on health-related quality of life (HRQOL) measures in juvenile-onset systemic lupus erythematosus (jSLE). METHODS: Obesity was defined as a body mass index (BMI) >/=95th percentile according to the Sex-specific Center for Disease Control BMI-For-Age Charts and determined in a multicenter cohort of jSLE patients. In this secondary analysis, the domain and summary scores of the Pediatric Quality of Life (PedsQL) Inventory and the Child Health Questionnaire (CHQ) of obese jSLE patients were compared to those of non-obese jSLE patients as well as historical obese and non-obese healthy controls. Mixed-effects modeling was performed …

Clinical And Genomic Heterogeneity Of Diamond Blackfan Anemia In The Russian Federation, N. S. Smetanina, I. V. Mersiyanova, M. A. Kurnikova, G. S. Ovsyannikova, L. A. Hachatryan, V. O. Bobrynina, M. A. Maschan, G. A. Novichkova, J. M. Lipton, A. A. Maschan

Journal Articles

BackgroundDiamond Blackfan anemia (DBA) is a genetically and clinically heterogeneous ribosomopathy and inherited bone marrow failure syndrome characterized by anemia, reticulocytopenia, and decreased erythroid precursors in the bone marrow with an increased risk of malignancy and, in approximately 50%, physical abnormalities. MethodsWe retrospectively analyzed clinical data from 77 patients with DBA born in the Russian Federation from 1993 to 2014. In 74 families there was one clinically affected individual; in only three instances a multiplex family was identified. Genomic DNA from 57 DBA patients and their first-degree relatives was sequenced for mutations in RPS19, RPS10, RPS24, RPS26, RPS7, RPS17, RPL5, …

Dissecting Allele Architecture Of Early Onset Ibd Using High-Density Genotyping, D. J. Cutler, M. E. Zwick, D. T. Okou, S. Prahalad, T. Walters, S. L. Guthery, M. Dubinsky, R. Baldassano, J. Markowitz, S. Kugathasan, +24 Additional Authors

Journal Articles

BACKGROUND: The inflammatory bowel diseases (IBD) are common, complex disorders in which genetic and environmental factors are believed to interact leading to chronic inflammatory responses against the gut microbiota. Earlier genetic studies performed in mostly adult population of European descent identified 163 loci affecting IBD risk, but most have relatively modest effect sizes, and altogether explain only ~20% of the genetic susceptibility. Pediatric onset represents about 25% of overall incident cases in IBD, characterized by distinct disease physiology, course and risks. The goal of this study is to compare the allelic architecture of early onset IBD with adult onset in …

The Impact Of Disease Duration On Quality Of Life In Children With Nephrotic Syndrome: A Midwest Pediatric Nephrology Consortium Study, D. T. Selewski, J. P. Troost, S. F. Massengill, R. A. Gbadegesin, L. A. Greenbaum, I. F. Shatat, Y. Cai, G. Kapur, C. B. Sethna, D. S. Gipson, +13 Additional Authors

Journal Articles

BACKGROUND: The Patient Reported Outcomes Measurement Information System (PROMIS) II is a prospective study that evaluates patient reported outcomes in pediatric chronic diseases as a measure of health-related quality of life (HRQOL). We have evaluated the influence of disease duration on HRQOL and, for the first time, compared the findings of the PROMIS measures to those of the PedsQL 4.0 Generic Scales (PedsQL) from the PROMIS II nephrotic syndrome (NS) longitudinal cohort. METHODS: This was a prospective study in which 127 children (age range 8-17 years) with active NS from 14 centers were enrolled. Children with active NS defined as …

Mitochondrial Dysfunction In Peripheral Blood Mononuclear Cells In Pediatric Septic Shock, S. L. Weiss, M. A. Selak, F. Tuluc, J. Perales Villarroel, V. M. Nadkarni, C. S. Deutschman, L. B. Becker

Journal Articles

OBJECTIVES: Mitochondrial dysfunction in peripheral blood mononuclear cells has been linked to immune dysregulation and organ failure in adult sepsis, but pediatric data are limited. We hypothesized that pediatric septic shock patients exhibit mitochondrial dysfunction within peripheral blood mononuclear cells which in turn correlates with global organ injury. DESIGN: Prospective observational study. SETTING: Academic PICU. PATIENTS: Thirteen pediatric patients with septic shock and greater than or equal to two organ failures and 11 PICU controls without sepsis or organ failure. INTERVENTIONS: Ex vivo measurements of mitochondrial oxygen consumption and membrane potential (DeltaPsim) were performed in intact peripheral blood mononuclear cells …

Non Alcoholic Fatty Liver Disease In A Case Of Kabuki Syndrome, A. Jain, M. See

Journal Articles

No abstract provided.

Population Pharmacokinetics Of Darbepoetin Alfa In Conjunction With Hypothermia For The Treatment Of Neonatal Hypoxic-Ischemic Encephalopathy, J. K. Roberts, C. Stockmann, R. M. Ward, J. Beachy, M. C. Baserga, M. G. Spigarelli, C. M. Sherwin

Journal Articles

AIM: The aim of this study was to determine the population pharmacokinetics of darbepoetin alfa in hypothermic neonates with hypoxic-ischemic encephalopathy treated with hypothermia. METHODS: Neonates >/=36 weeks gestation andrandomized, multicenter, investigational, new drug pharmacokinetic study. Two intravenous darbepoetin alfa treatment groups were evaluated: 2 and 10 microg/kg. Serum erythropoietin concentrations were measured using an enzyme-linked immunosorbent assay. Monolix 4.3.1 was used to estimate darbepoetin alfa clearance and volume of distribution. Covariates tested included: birthweight, gestational age, postnatal age, postmenstrual age, sex, Sarnat score, and study site. RESULTS: Darbepoetin alfa pharmacokinetics were well described by a one-compartment model with exponential …

Protocolized Sedation Vs Usual Care In Pediatric Patients Mechanically Ventilated For Acute Respiratory Failure: A Randomized Clinical Trial, M. A. Curley, D. Wypij, R. S. Watson, M. J. Grant, L. A. Asaro, I. M. Cheifetz, B. L. Dodson, L. S. Franck, R. G. Gedeit, J. Schneider, +2 Additional Authors

Journal Articles

IMPORTANCE: Protocolized sedation improves clinical outcomes in critically ill adults, but its effect in children is unknown. OBJECTIVE: To determine whether critically ill children managed with a nurse-implemented, goal-directed sedation protocol experience fewer days of mechanical ventilation than patients receiving usual care. DESIGN, SETTING, AND PARTICIPANTS: Cluster randomized trial conducted in 31 US pediatric intensive care units (PICUs). A total of 2449 children (mean age, 4.7 years; range, 2 weeks to 17 years) mechanically ventilated for acute respiratory failure were enrolled in 2009-2013 and followed up until 72 hours after opioids were discontinued, 28 days, or hospital discharge. INTERVENTION: Intervention …

Use Of Serogroup B Meningococcal Vaccines In Adolescents And Young Adults: Recommendations Of The Advisory Committee On Immunization Practices, 2015, J. R. Macneil, L. Rubin, T. Folaranmi, I. R. Ortega-Sanchez, M. Patel, S. W. Martin

Journal Articles

At its June 2015 meeting, the Advisory Committee on Immunization Practices (ACIP) recommended that adolescents and young adults aged 16-23 years may be vaccinated with a serogroup B meningococcal (MenB) vaccine to provide short-term protection against most strains of serogroup B meningococcal disease. This report summarizes the deliberations of ACIP, the rationale for its decision, and recommendations for use of MenB vaccines in adolescents and young adults. Two MenB vaccines have recently been licensed by the Food and Drug Administration (FDA) for use in the United States and approved for use in persons aged 10-25 years: MenB-FHbp (Trumenba, Wyeth Pharmaceuticals, …

Cancer Care Annual Report 2014-2015, Children's Mercy Hospital

Cancer Center Annual Reports

Annual report of cancer care and oncology services at the Children's Mercy Hospital, Kansas City MO, a pediatric medical center. This report has a focus on germ cell tumors.

A Fatal Twist: Volvulus Of The Small Intestine In A 46-Year-Old Woman, Jared Klein, Kathryn Baxstrom, Stephen Donnelly, Patrick Feasel, Paul Koles

Pediatrics Publications

A 46-year-old woman presented to two emergency departments within 12 hours because of acute abdominal pain. Physical exam demonstrated tenderness and epigastric guarding. An ultrasound was interpreted as negative; she was discharged home. Later that evening, she was found dead. Postmortem exam revealed acute hemorrhagic necrosis of a segment of jejunum secondary to volvulus. Clinical clues suggesting presentations of small bowel volvulus are usually nonspecific; the diagnosis is typically confirmed at surgery. Her unremitting abdominal pain, persistent vomiting, and absolute neutrophilia were consistent with an acute process. The etiology of this volvulus was caused by an elastic fibrous band at …

Γ-Secretase Inhibitor Alleviates Acute Airway Inflammation Of Allergic Asthma In Mice By Downregulating Th17 Cell Differentiation, Weixi Zhang, Xueya Zhang, Anqun Sheng, Cuiye Weng, Tingting Zhu, Wei Zhao, Changchong Li

Pediatrics Publications

T helper 17 (Th17) cells play an important role in the pathogenesis of allergic asthma. Th17 cell differentiation requires Notch signaling. γ-Secretase inhibitor (GSI) blocks Notch signaling; thus, it may be considered as a potential treatment for allergic asthma. The aim of this study was to evaluate the effect of GSI on Th17 cell differentiation in a mouse model of allergic asthma. OVA was used to induce mouse asthma model in the presence and absence of GSI. GSI ameliorated the development of OVA-induced asthma, including suppressing airway inflammation responses and reducing the severity of clinical signs. GSI also significantly …

Exosomes In Cancer Research, S. R. Vaiselbuh

Journal Articles

No abstract provided.

Hiv Status Non-Disclosure In Hiv- Infected Families, Saptharishi Lalgudi Ganesan

Data and Test Instruments

No abstract provided.

Maintenance Intravenous Fluids For Children With Central Nervous System Infections, Saptharishi Lalgudi Ganesan, Sunit C. Singhi, Karthi N.

Data and Test Instruments

No abstract provided.

Proven Family-Based Interventions In The Treatment Of Pediatric Obesity, Sarah Winter

Physician Assistant Scholarly Project Posters

• Childhood obesity continues to be ever expanding.

• This is leading to increasing health care needs and costs.

• Despite numerous interventions, the rates continue to climb.

• The purpose of this study is to identify family-based childhood obesity interventions that are proven to decrease the rates of childhood obesity.

• A literature review explores multiple different family-based childhood obesity interventions.

• The effective interventions are examined closely to discover commonalities between the interventions.

• Family-based interventions that decrease childhood obesity share similar attributes.

• The proven interventions may indicate the need to focus coordination of care with the …

10 Steps To Collaborating With Pediatric Medical Providers, American Dental Association

Action for Dental Health

No abstract provided.