Pediatrics Commons^™

Circulating Fibroblast Growth Factor-2, Hiv-Tat, And Vascular Endothelial Cell Growth Factor-A In Hiv-Infected Children With Renal Disease Activate Rho-A And Src In Cultured Renal Endothelial Cells., Jharna R Das, J Silvio Gutkind, Patricio E. Ray

Pediatrics Faculty Publications

Renal endothelial cells (REc) are the first target of HIV-1 in the kidney. The integrity of REc is maintained at least partially by heparin binding growth factors that bind to heparan sulfate proteoglycans located on their cell surface. However, previous studies showed that the accumulation of two heparin-binding growth factors, Vascular Endothelial Cell Growth Factor-A (VEGF-A) and Fibroblast Growth Factor-2 (FGF-2), in combination with the viral protein Tat, can precipitate the progression of HIV-renal diseases. Nonetheless, very little is known about how these factors affect the behavior of REc in HIV+ children. We carried out this study to determine how …

Do 6-8 Year Old Girls With Central Precocious Puberty Need Routine Brain Imaging?, Paul B. Kaplowitz

Pediatrics Faculty Publications

BACKGROUND: The subject of whether all girls with central precocious puberty (CPP) require brain imaging is controversial.

FINDINGS: A review of the major papers concerning this topic published since 1994 was conducted looking primarily at the frequency of occult intracranial lesions, particularly brain tumors, in girls with CPP. While CNS abnormalities are frequently noted (8-15 %), the proportion of previously unknown findings requiring intervention in 6-8 year old girls is very small, in the range of 0-2 %.

CONCLUSION: While MRI should still be done in boys and in girls with onset of puberty younger than age 6 and in …

Anticipated Resource Utilization For Injury Versus Non-Injury Pediatric Visits To Emergency Departments, Mark R. Zonfrillo, Michelle L. Macy, Lawrence J. Cook, Tomohiko Funai, Rachel Stanley, James M. Chamberlain, For The Pediatric Emergency Care Applied Research Network (Pecarn)

Pediatrics Faculty Publications

Background

Childhood injuries are increasingly treated in emergency departments (EDs) but the relationship between injury severity and ED resource utilization has not been evaluated. The objective of this study was to compare resource utilization for pediatric injury-related ED visits across injury-severity levels and with non-injury visits, using standardized, validated scales.

Methods

A retrospective analysis of 2004-2008 ED visits from the Pediatric Emergency Care Applied Research Network Core Data Project. Maximum Abbreviated Injury Scale severity (MAIS) and Severity Classification System (SCS) scores were calculated and compared. MAIS and SCS are ordinal scales from 1 (minor injury) to 6, and 1 (low …

Molecular And Behavioral Profiling Of Dbx1-Derived Neurons In The Arcuate, Lateral And Ventromedial Hypothalamic Nuclei., Katie Sokolowski, Tuyen Tran, Shigeyuki Esumi, Yasmin Kamal, Livio Oboti, Julieta Lischinsky, Meredith Goodrich, Andrew Lam, Margaret Carter, Yasushi Nakagawa, Joshua G. Corbin

Pediatrics Faculty Publications

BACKGROUND: Neurons in the hypothalamus function to regulate the state of the animal during both learned and innate behaviors, and alterations in hypothalamic development may contribute to pathological conditions such as anxiety, depression or obesity. Despite many studies of hypothalamic development and function, the link between embryonic development and innate behaviors remains unexplored. Here, focusing on the embryonically expressed homeodomain-containing gene Developing Brain Homeobox 1 (Dbx1), we explored the relationship between embryonic lineage, post-natal neuronal identity and lineage-specific responses to innate cues. We found that Dbx1 is widely expressed across multiple developing hypothalamic subdomains. Using standard and inducible fate-mapping to …

"When Should I Tell?": Perspectives On Disclosure To Their Children Among Parents With Perinatally Acquired Hiv., Cynthia D Fair, Hannah Allen, Constance Trexler, Janet Osherow, Lawrence D'Angelo

Pediatrics Faculty Publications

Adolescents and young adults (AYA) with perinatally acquired HIV (PHIV) engage in developmentally expected behaviors, such as establishing relationships and having children. Previous research has focused on pregnancy management/outcomes of AYA with PHIV. However, little research has focused on the parenting experiences of this emerging cohort and on their views of disclosure to their offspring. This article examines data from a pilot study of five AYA parents with PHIV on disclosure to their child(ren) (n = 7, 6 HIV-negative). Disclosure of their own HIV status to their children is on the minds of parents with PHIV. However, few currently have …

The Clinical Outcome Study For Dysferlinopathy, Elizabeth Harris, Catherine Bladen, Anna Mayhew, Meredith James, Karen Bettinson, Avital Cnaan, The Jain Cos Consortium

Pediatrics Faculty Publications

Objective: To describe the baseline clinical and functional characteristics of an international cohort of 193 patients with dysferlinopathy.

Methods: The Clinical Outcome Study for dysferlinopathy (COS) is an international multicenter study of this disease, evaluating patients with genetically confirmed dysferlinopathy over 3 years. We present a cross-sectional analysis of 193 patients derived from their baseline clinical and functional assessments.

Results: There is a high degree of variability in disease onset, pattern of weakness, and rate of progression. No factor, such as mutation class, protein expression, or age at onset, accounted for this variability. Among patients with clinical diagnoses of Miyoshi …

Incidence Of X And Y Chromosomal Aneuploidy In A Large Child Bearing Population., Carole Samango-Sprouse, Eser Kırkızlar, Megan P Hall, Patrick Lawson, Zachary Demko, Susan M Zneimer, Kirsten J Curnow, Susan Gross, Andrea Gropman

Pediatrics Faculty Publications

BACKGROUND: X&Y chromosomal aneuploidies are among the most common human whole-chromosomal copy number changes, but the population-based incidence and prevalence in the child-bearing population is unclear.

METHODS: This retrospective analysis of prospectively collected data leveraged a routine non-invasive prenatal test (NIPT) using parental genotyping to estimate the population-based incidence of X&Y chromosome variations in this population referred for NIPT (generally due to advanced maternal age).

RESULTS: From 141,916 women and 29,336 men, 119 X&Y chromosomal abnormalities (prevalence: 1 in 1,439) were identified. Maternal findings include: 43 cases of 45,X (40 mosaic); 30 cases of 47,XXX (12 mosaic); 3 cases of …

Safe Infant Sleep Interventions: What Is The Evidence For Successful Behavior Change?, Rachel Y. Moon, Fern R Hauck, Eve R Colson

Pediatrics Faculty Publications

Sudden infant death syndrome (SIDS) and other sleep-related infant deaths, such as accidental suffocation and strangulation in bed and ill-defined deaths, account for >4000 deaths annually in the USA. Evidence-based recommendations for reducing the risk of sleep-related deaths have been published, but some caregivers resist adoption of these recommendations. Multiple interventions to change infant sleep-related practices of parents and professionals have been implemented. In this review, we will discuss illustrative examples of safe infant sleep interventions and evidence of their effectiveness. Facilitators of and barriers to change, as well as the limitations of the data currently available for these interventions, …

Age-Related Effect Of Viral-Induced Wheezing In Severe Prematurity, Geovanny F. Perez, Amisha Jain, Bassem Kurdi, Rosemary Megalaa, Krishna Pancham, Shehlanoor Huseni, Natalia Isaza, Carlos E. Rodriguez-Martinez, Mary C. Rose, Dinesh Pillai, Gustavo Nino

Pediatrics Faculty Publications

Premature children are prone to severe viral respiratory infections in early life, but the age at which susceptibility peaks and disappears for each pathogen is unclear.

Methods: A retrospective analysis was performed of the age distribution and clinical features of acute viral respiratory infections in full-term and premature children, aged zero to seven years.

Results: The study comprised of a total of 630 hospitalizations (n = 580 children). Sixty-seven percent of these hospitalizations occurred in children born full-term (>37 weeks), 12% in preterm (32–37 weeks) and 21% in severely premature children (<32 weeks). The most common viruses identified were rhinovirus (RV; 60%) and respiratory syncytial virus (RSV; 17%). Age-distribution analysis of each virus identified that severely premature children had a higher relative frequency of RV and RSV in their first three years, relative to preterm or full-term children. Additionally, the probability of RV- or RSV-induced wheezing was higher overall in severely premature children less than three years old.

Conclusions: Our results indicate that the vulnerability …

Endocrine Dysfunction In Diamond-Blackfan Anemia (Dba): A Report From The Dba Registry (Dbar), A. Lahoti, Y. T. Harris, P. W. Speiser, E. Atsidaftos, J. M. Lipton, A. Vlachos

Journal Articles

BACKGROUND: Diamond-Blackfan anemia (DBA) is a rare inherited bone marrow failure syndrome. The mainstays of treatment involve chronic red cell transfusions, long-term glucocorticoid therapy, and stem cell transplantation. Systematic data concerning endocrine function in DBA are limited. We studied patients in the DBA Registry (DBAR) of North America to assess the prevalence of various endocrinopathies. PROCEDURE: In a pilot study, retrospective data were collected for 12 patients with DBA. Subsequently, patients with DBA aged 1-39 years were recruited prospectively. Combined, 57 patients were studied; 38 chronically transfused, 12 glucocorticoid-dependent, and seven in remission. Data were collected on anthropometric measurements, systematic …

Development Of A Novel Renal Activity Index Of Lupus Nephritis In Children & Young Adults, H. I. Brunner, M. Bennett, K. Abulaban, M. Klein-Gitelman, K. O'Neil, L. Tucker, S. Ardoin, K. Rouster-Stevens, B. A. Eberhard, P. Devarajan, +8 Additional Authors

Journal Articles

BACKGROUND: Noninvasive estimation of the degree of inflammation seen on kidney biopsy with lupus nephritis (LN) remains difficult. The objective of this study was to develop a Renal Activity Index for Lupus (RAIL) that, based solely on laboratory measures, accurately reflects histological LN activity. METHODS: We assayed traditional LN laboratory tests and 16 urine biomarkers (UBMs) in children (n=47) at the time of kidney biopsy. Histological LN activity was measured by the NIH Activity Index (NIH-AI) and the Tubulointerstitial Activity Index (TIAI). High LN-activity status (vs. moderate/low) was defined as NIH-AI scores > 10 (vs.5 (vs.92% accuracy and LN-activityTIAI status with …

Increased Hepcidin In Transferrin-Treated Thalassemic Mice Correlates With Increased Liver Bmp2 Expression And Decreased Hepatocyte Erk Activation, H. Chen, T. Choesang, H. Li, S. Sun, P. Pham, W. Bao, M. Feola, M. Westerman, L. Blanc, Y. Z. Ginzburg, +4 Additional Authors

Journal Articles

Iron overload results in significant morbidity and mortality in beta-thalassemic patients. Insufficient hepcidin is implicated in parenchymal iron overload in beta-thalassemia and approaches to increase hepcidin have therapeutic potential. We have previously shown that exogenous apo-transferrin markedly ameliorates ineffective erythropoiesis and increases hepcidin expression in Hbb(th1/th1) (thalassemic) mice. We utilize in vivo and in vitro systems to investigate effects of exogenous apo-transferrin on Smad and ERK1/2 signaling, pathways that participate in hepcidin regulation. Our results demonstrate that apo-transferrin increases hepcidin expression in vivo despite decreased circulating and parenchymal iron concentrations and unchanged liver Bmp6 mRNA expression in thalassemic mice. Hepatocytes …

Pericardiocentesis Followed By Thoracotomy And Repair Of Penetrating Cardiac Injury Caused By Nail Gun Injury To The Heart, Vasu Chirumamilla, Kartik Prabhakaran, Patrizio Petrone, John A. Savino, Corrado Marini, Zobair Zoha

NYMC Faculty Publications

INTRODUCTION: Work site injuries involving high projectile tools such as nail guns can lead to catastrophic injuries. Generally, penetrating cardiac injuries are associated with a high mortality rate.

PRESENTATION OF CASE: A construction worker was brought to the emergency room having sustained a nail gun injury to the chest. The patient was hypotensive, tachycardic with prominent jugular venous distention, and had a profound lactic acidosis. Bedside ultrasound confirmed the presence of pericardial fluid. Pericardiocentesis was performed twice using a central venous catheter inserted into the pericardial space, resulting in improvement in the patient's hemodynamics. Thereafter he underwent left anterolateral thoracotomy …

Family-Centered Rounds And Medical Student Performance On The Nbme Pediatrics Subject (Shelf) Examination: A Retrospective Cohort Study, Tiffany N. Kimbrough, Victor Heh, N. Romesh Wijesooriya, Michael S. Ryan

Pediatrics Publications

Objective

To determine the association between family-centered rounds (FCR) and medical student knowledge acquisition as assessed by the National Board of Medical Examiners (NBME) pediatric subject (shelf) exam.

Methods

A retrospective cohort study was conducted of third-year medical students who graduated from Virginia Commonwealth University School of Medicine between 2009 and 2014. This timeframe represented the transition from ‘traditional’ rounds to FCR on the pediatric inpatient unit. Data collected included demographics, United States Medical Licensing Examination (USMLE) Step 1 and 2 scores, and NBME subject examinations in pediatrics (PSE), medicine (MSE), and surgery (SSE).

Results

Eight hundred and sixteen participants …

Continuous Eeg For Diagnosis Of Electrographic Seizures In The Pediatric Cardiac Critical Care Unit: Using A Precious Resource Wisely, Saptharishi Lalgudi Ganesan

Data and Test Instruments

No abstract provided.

2015-2016 Pediatric Subspecialty Trainees, Children's Mercy Hospital

Residents and Fellows Portraits

Photo composite of the graduate pediatric trainees in allergy/immunology, child abuse pediatrics, child neurology, clinical child psychology, clinical cytogenetics, clinical pharmacology, developmental-behavioral pediatrics, neonatal-perinatal medicine, pediatric adolescent gynecology, pediatric & congenital cardiac perfusion, pediatric cardiology, pediatric critical care, pediatric dentistry, pediatric dermatology, pediatric emergency medicine, pediatric endocrinology, pediatric gastroenterology, pediatric hematology/oncology, pediatric hospital medicine, pediatric infectious diseases, pediatric nephrology, pediatric ophthalmology, pediatric optometry, pediatric otolaryngology, pediatric ophthalmology/optometry, pediatric otolaryngology, pediatric radiology, pediatric rehabilitation, pediatric surgery, pediatric residency and med/peds residency

Examination Of Reticulocytosis Among Chronically Transfused Children With Sickle Cell Anemia., Megha Kaushal, Colleen Byrnes, Zarir Khademian, Natalie Duncan, Naomi L.C. Luban, Jeffery L Miller, Ross M. Fasano, Emily Riehm Meier

Pediatrics Faculty Publications

Sickle cell anemia (SCA) is an inherited hemolytic anemia with compensatory reticulocytosis. Recent studies have shown that increased levels of reticulocytosis during infancy are associated with increased hospitalizations for SCA sequelae as well as cerebrovascular pathologies. In this study, absolute reticulocyte counts (ARC) measured prior to transfusion were analysed among a cohort of 29 pediatric SCA patients receiving chronic transfusion therapy (CTT) for primary and secondary stroke prevention. A cross-sectional flow cytometric analysis of the reticulocyte phenotype was also performed. Mean duration of CTT was 3.1 ± 2.6 years. Fifteen subjects with magnetic resonance angiography (MRA) -vasculopathy had significantly higher …

Graduate Medical Education 2015-2016 Annual Report, Children's Mercy Hospital

GME Annual Reports

Annual report of the Children's Mercy Hospital Graduate Medical Education department, including pediatric residency, internal medicine/pediatrics residency, fellowship programs, medical students, continuing medical education, and library services.

Minimally Invasive Surgery In Pediatric Trauma: One Institution's 20-Year Experience, Gustavo Stringel, Min Li Xu, Joseph Lopez

NYMC Faculty Publications

BACKGROUND: Minimally invasive surgery (MIS) for trauma in pediatric cases remains controversial. Recent studies have shown the validity of using minimally invasive techniques to decrease the rate of negative and nontherapeutic laparotomy and thoracotomy. The purpose of this study was to evaluate the diagnostic accuracy and therapeutic options of MIS in pediatric trauma at a level I pediatric trauma center.

METHODS: We reviewed cases of patients aged 15 years and younger who had undergone laparoscopy or thoracoscopy for trauma in our institution over the past 20 years. Each case was evaluated for mechanism of injury, computed tomographic (CT) scan findings, …

Rac1 Signaling Is Required For Anterior Second Heart Field Cellular Organization And Cardiac Outflow Tract Development, Carmen Leung, Yin Liu, Xiangru Lu, Mella Kim, Thomas A. Drysdale, Qingping Feng

Paediatrics Publications

Background-The small GTPase Rac1 regulates diverse cellular functions, including both apicobasal and planar cell polarity pathways; however, its role in cardiac outflow tract (OFT) development remains unknown. In the present study, we aimed to examine the role of Rac1 in the anterior second heart field (SHF) splanchnic mesoderm and subsequent OFT development during heart morphogenesis. Methods and Results-Using the Cre/loxP system, mice with an anterior SHF-specific deletion of Rac1 (Rac1SHF) were generated. Embryos were collected at various developmental time points for immunostaining and histological analysis. Intrauterine echocardiography was also performed to assess aortic valve blood flow in embryos at embryonic …

Is Testosterone Detrimental To Renal Function?, Guido Filler, Amanda Ramsaroop, Robert Stein, Claire Grant, Raanan Marants, Aaron So, Christopher Mcintyre

Paediatrics Publications

No abstract provided.

Children's Mercy Hospital Annual Report 2015, Children's Mercy Hospital

Children's Mercy Annual Reports

Annual report for The Children's Mercy Hospital in Kansas City MO, a pediatric medical center.

A Comparison Of Right Ventricular Volumes In Children And Adults With Repaired Tetralogy Of Fallot - Including And Excluding Right Ventricular Trabeculations, James D. Enos, Jacob Hartz, Rachel Quinn, Lasya Gaur, Russell Cross, Laura J. Olivieri

Pediatrics Posters and Presentations

No abstract provided.

Supersilac Quantitative Proteome Profiling Of Murine Middle Ear Epithelial Cell Remodeling With Nthi, Stephanie Val, Katelyn Burgett, Kristy J. Brown, Diego A. Preciado

Pediatrics Faculty Publications

Background

Chronic Otitis Media with effusion (COME) develops after sustained inflammation and is characterized by secretory middle ear epithelial metaplasia and effusion, most frequently mucoid. Non-typeable Haemophilus influenzae (NTHi), the most common acute Otitis Media (OM) pathogen, is postulated to promote middle ear epithelial remodeling in the progression of OM from acute to chronic. The goals of this study were to examine histopathological and quantitative proteomic epithelial effects of NTHi challenge in a murine middle ear epithelial cell line.

Methods

NTHi lysates were generated and used to stimulate murine epithelial cells (mMEEC) cultured at air-liquid interface over 48 hours– …

Sexual Minority (Lgbtq) Youth And Role Of Health Care Provider, Mandakini Sadhir, Stephanie J. Stockburger, Hatim A. Omar

Pediatrics Faculty Publications

Adolescence is a time period of physical, psychological and cognitive changes. It is an important phase for an individual to discover who they really are (self-identity), how they are perceived (social identity) and how they fit into the environment they live in.¹ One of the major tasks of adolescence is sexual maturation and sexual and /or gender identity development.² Adolescents who are developing as gay, lesbian, bisexual or transgender face a tremendous challenge of establishing their identity which is often complex.³ They cope with the feelings of being different and are in constant dilemmas about revealing their …

Role Of Elosulfase Alfa In Mucopolysaccharidosis Iva., Debra S. Regier, Pranoot Tanpaiboon

Pediatrics Faculty Publications

Mucopolysaccharidosis type IVA (MPS IVA or Morquio A) is an autosomal recessive lysosomal storage disease which results in a striking skeletal phenotype, but does not negatively impact the intellect of the patient. MPS IVA has a phenotypic continuum that ranges from a severe and rapidly progressing form to a slowly progressive form. The clinical diagnosis is often made in the preschool years based on abnormal bone findings on physical examination and dysplasia on radiographic imaging. Supportive care has been the mainstay in caring for patients. Orthopedic physicians often form the core of the care team due to the early and …

Caring For Children With Intrauterine Drug Exposure, The Rees-Jones Center For Foster Care Excellence

White Papers and Policy Briefs

Overview of national statistics regarding in utero substance exposure and the impacts on health care for children involved with the child welfare system.

For questions about this publication or others, please email [email protected]

childrens.com/fostercare

Addressing The Health And Safety Of Children In Foster Care, The Rees-Jones Center For Foster Care Excellence

White Papers and Policy Briefs

The Texas foster care system is an integral part of the safety net for the state’s most vulnerable children, and is charged with the daunting task of providing safe homes to thousands of children who are in crisis. Children and youth in foster care still face persistent barriers when they interact with the health system, which blunt the effectiveness of reform initiatives and potentially create lifelong harms for children in the program. Addressing disparities in the areas of physical health, mental health, educational attainment and special health care needs is integral to improving safety, stability and permanency for every child …

Effect Of Provision Of An Integrated Neonatal Survival Kit And Early Cognitive Stimulation Package By Community Health Workers On Developmental Outcomes Of Infants In Kwale County, Kenya: Study Protocol For A Cluster Randomized Trial, Lisa G. Pell, Diego G. Bassani, Lucy Nyaga, Isaac Njagi, Catherine Wanjiku, Thulasi Thiruchselvam, William Macharia, Ripudaman S. Minhas, Patricia Kitsao-Wekulo, Amyn Lakhani, Zulfiqar Ahmed Bhutta, Robert Armstrong, Shaun K. Morris

Paediatrics and Child Health, East Africa

Background: Each year, more than 200 million children under the age of 5 years, almost all in low- and middle-income countries (LMICs), fail to achieve their developmental potential. Risk factors for compromised development often coexist and include inadequate cognitive stimulation, poverty, nutritional deficiencies, infection and complications of being born low birthweight and/or premature. Moreover, many of these risk factors are closely associated with newborn morbidity and mortality. As compromised development has significant implications on human capital, inexpensive and scalable interventions are urgently needed to promote neurodevelopment and reduce risk factors for impaired development.

Method/Design: This cluster randomized trial aims at …

Self-Reported Practices Among Traditional Birth Attendants Surveyed In Western Kenya: A Descriptive Study, Sherri Bucher, Olive Konana, Edward Liechty, Ana Garces, Peter Gisore, Irene Marete, Constance Tenge, Evelyn Shipala, Linda Wright, Fabian Esamai

Paediatrics and Child Health, East Africa

Background: The high rate of home deliveries conducted by unskilled birth attendants in resource-limited settings is an important global health issue because it is believed to be a significant contributing factor to maternal and newborn mortality. Given the large number of deliveries that are managed by unskilled or traditional birth attendants outside of health facilities, and the fact that there is on-going discussion regarding the role of traditional birth attendants in the maternal newborn health (MNH) service continuum, we sought to ascertain the practices of traditional birth attendants in our catchment area. The findings of this descriptive study might help …