Pediatrics Commons^™

X-Linked Agammaglobulinemia Presenting As Polymicrobial Pneumonia, Including Pneumocystis Jirovecii, A. M. Jongco, J. D. Gough, K. Sarnataro, D. W. Rosenthal, J. Moreau, P. Ponda, V. R. Bonagura

Journal Articles

No abstract provided.

Anti-Inflammatory Actions Of Endogenous And Exogenous Interleukin-10 Versus Glucocorticoids On Macrophage Functions Of The Newly Born, K. Kasat, H. Patel, O. Predtechenska, I. Vancurova, D. Davidson

Journal Articles

OBJECTIVE: To determine whether specific macrophage immune functions of the newly born are insensitive to the actions of therapeutic levels of dexamethasone (DEX), previously measured in infants with bronchopulmonary dysplasia (BPD), compared with betamethasone (BETA) and exogenous or endogenous interleukin-10 (IL-10). STUDY DESIGN: Macrophages were differentiated from cord blood monocytes (N=18). A serial dose-response (around 10(-8)M), in vitro study was used to examine the effect of DEX, BETA and IL-10, on proinflammatory (PI) cytokine release, phagocytosis and respiratory burst. RESULT: Exogenous IL-10 (10(-8)M) significantly (P < 0.05) inhibited the endotoxin-stimulated release of IL-6, IL-8 and tumor necrosis factor by 63 to 82% with no significant effect by DEX and BETA. There was no inhibition by these three agents at 10(-8)M on phagocytosis and respiratory burst. Inhibition of endogenous IL-10 with a monoclonal antibody significantly increased endotoxin-stimulated cytokine release by at least fourfold. CONCLUSION: Macrophages were relatively insensitive to therapeutic levels of DEX and BETA with regard to PI cytokine release. This study provides rationale for translational and preclinical research using airway instillation of IL-10 for the treatment of BPD.

Bisphenol A Exposure Is Associated With Decreased Lung Function, A. J. Spanier, E. K. Fiorino, L. Trasande

Journal Articles

Objective To examine the associations of bisphenol A (BPA) exposure with lung function measures and exhaled nitric oxide (FeNO) in children. Study design We performed a cross-sectional analysis of a subsample of US children age 6-19 years who participated in the 2007-2010 National Health and Nutrition Examination Survey. We assessed univariate and multivariable associations of urinary BPA concentration with the predicted pulmonary function measures for age, sex, race/ethnicity and height (forced expiratory volume in 1 second [FEV1], forced vital capacity [FVC], forced expiratory flow 25%-75%, and FEV1 divided by FVC) and with FeNO. Results Exposure and outcome data were available …

Blood Pressure Variability In Children With Primary Vs Secondary Hypertension, D. Leisman, M. Meyers, J. Schnall, N. Chorny, R. Frank, L. Infante, C. B. Sethna

Journal Articles

Increased blood pressure variability (BPV) is correlated with adverse cardiovascular (CV) events in adults. However, there has been limited research on its effect in the pediatric population. Additionally, BPV differences between primary and secondary hypertension (HTN) are not known. Children with primary and secondary HTN underwent 24-hour ambulatory blood pressure monitoring and echocardiography studies. BPV measures of standard deviation (SD), average real variability (ARV), and range were calculated for the 24-hour, daytime, and nighttime periods. Seventy-four patients (median age, 13.5 years; 74% boys) were examined, 40 of whom had primary HTN. Body mass index z score and age were independent …

Characteristics Of Avoidant/Restrictive Food Intake Disorder In Children And Adolescents: A "New Disorder" In Dsm-5, M. M. Fisher, D. S. Rosen, R. M. Ornstein, K. A. Mammel, D. K. Katzman, E. S. Rome, S. T. Callahan, J. Malizio, S. Kearney, B. T. Walsh

Journal Articles

Purpose: To evaluate the DSM-5 diagnosis of Avoidant/Restrictive Food Intake Disorder (ARFID) in children and adolescents with poor eating not associated with body image concerns. Methods: A retrospective case-control study of 8e18-year-olds, using a diagnostic algorithm, compared all cases with ARFID presenting to seven adolescent-medicine eating disorder programs in 2010 to a randomly selected sample with anorexia nervosa (AN) and bulimia nervosa (BN). Demographic and clinical information were recorded. Results: Of 712 individuals studied, 98 (13.8%) met ARFID criteria. Patients with ARFID were younger than those with AN (n = 98) or BN (n = 66), (12.9 vs. 15.6 vs. …

Decreased Langerhans Cell Responses To Il-36 Gamma: Altered Innate Immunity In Patients With Recurrent Respiratory Papillomatosis, J. Devoti, L. Hatam, A. Lucs, A. Afzal, A. Abramson, B. M. Steinberg, V. Bonagura

Journal Articles

Recurrent respiratory papillomatosis (RRP) is a rare, chronic disease caused by human papillomaviruses (HPVs) types 6 and 11 that is characterized by the polarization of adaptive immune responses that support persistent HPV infection. Respiratory papillomas express elevated mRNA levels of IL-36 gamma, a proinflammatory cytokine in comparison to autologous clinically normal laryngeal tissues; however there is no evidence of inflammation in these lesions. Consistent with this, respiratory papillomas do not contain T(H)1-like CD4(+) T-cells or cytotoxic CD8(+) T-cells, but instead contain a predominance of T(H)2-like and T regulatory cells (Tregs). In addition, papillomas also are infiltrated with immature Langerhans cells …

Helping The Community From The Bottom Up: Distributing Diapers To The Williamsburg Community, Constance A. Hull, Caroline Kelsey

VA Engage Journal

There is a great need for a sustainable supply of clean diapers for low-income residents of Williamsburg since this region is without a diaper bank. Being unable to afford diapers can have a myriad of negative consequences from being unable to send children to child care to increased health risks involved in leaving a baby in a soiled diaper. Our model of a diaper bank differs from most because we mail diapers directly to families, eliminating transportation needs. We hope to partner with community agencies and The College of William & Mary to expand our services and create a more …

Diagnosis And Treatment Of Pediatric Acquired Aplastic Anemia (Aaa): An Initial Survey Of The North American Pediatric Aplastic Anemia Consortium (Napaac), D. A. Williams, C. Bennett, A. Bertuch, M. Bessler, T. Coates, S. Corey, Y. Dror, J. Lipton, A. Vlachos, A. Shimamura, +7 Additional Authors

Journal Articles

BackgroundRandomized clinical trials in pediatric aplastic anemia (AA) are rare and data to guide standards of care are scarce. ProcedureEighteen pediatric institutions formed the North American Pediatric Aplastic Anemia Consortium to foster collaborative studies in AA. The initial goal of NAPAAC was to survey the diagnostic studies and therapies utilized in AA. ResultsOur survey indicates considerable variability among institutions in the diagnosis and treatment of AA. There were areas of general consensus, including the need for a bone marrow evaluation, cytogenetic and specific fluorescent in situ hybridization assays to establish diagnosis and exclude genetic etiologies with many institutions requiring results …

The Treatment-Naive Microbiome In New-Onset Crohn's Disease, D. Gevers, S. Kugathasan, L. A. Denson, Y. Vazquez-Baeza, W. Van Treuren, B. Y. Ren, E. Schwager, D. Knights, J. Markowitz, R. J. Xavier, +22 Additional Authors

Journal Articles

Inflammatory bowel diseases (IBDs), including Crohn's disease (CD), are genetically linked to host pathways that implicate an underlying role for aberrant immune responses to intestinal microbiota. However, patterns of gut microbiome dysbiosis in IBD patients are inconsistent among published studies. Using samples from multiple gastrointestinal locations collected prior to treatment in new-onset cases, we studied the microbiome in the largest pediatric CD cohort to date. An axis defined by an increased abundance in bacteria which include Enterobacteriaceae, Pasteurellacaea, Veillonellaceae, and Fusobacteriaceae, and decreased abundance in Erysipelotrichales, Bacteroidales, and Clostridiales, correlates strongly with disease status. Microbiome comparison between CD patients with …

Randomized, Double-Blind, Placebo-Controlled Trial Of The Efficacy And Safety Of Rilonacept In The Treatment Of Systemic Juvenile Idiopathic Arthritis, N. T. Ilowite, K. Prather, Y. Lokhnygina, L. E. Schanberg, M. Elder, D. Milojevic, J. W. Verbsky, S. J. Spalding, B. S. Gottlieb, C. I. Sandborg, +12 Additional Authors

Journal Articles

OBJECTIVE: To assess the efficacy and safety of rilonacept, an interleukin-1 inhibitor, in a randomized, double-blind, placebo-controlled trial. METHODS: An initial 4-week double-blind placebo phase was incorporated into a 24-week randomized multicenter design, followed by an open-label phase. Seventy-one children who had active arthritis in >/=2 joints were randomized (1:1) to the 2 arms of the study. Patients in the rilonacept arm received rilonacept (loading dose 4.4 mg/kg followed by 2.2 mg/kg weekly, subcutaneously) beginning on day 0. Patients in the placebo arm received placebo for 4 weeks followed by a loading dose of rilonacept at week 4 followed by …

Magnetic Resonance Imaging/Angiography And Transcranial Doppler Velocities In Sickle Cell Anemia: Results From The Switch Trial, K. J. Helton, R. J. Adams, K. L. Kesler, A. Lockhart, B. Aygun, C. Driscoll, M. M. Heeney, S. M. Jackson, L. Krishnamurti, R. E. Ware, +3 Additional Authors

Journal Articles

The Stroke With Transfusions Changing to Hydroxyurea (SWiTCH) trial compared standard (transfusions/chelation) to alternative (hydroxyurea/phlebotomy) treatment to prevent recurrent stroke and manage iron overload in children chronically transfused over 7 years before enrollment. Standardized brain magnetic resonance imaging/magnetic resonance angiography (MRA) and transcranial Doppler (TCD) exams were performed at entry and exit, with a central blinded review. A novel MRA vasculopathy grading scale demonstrated frequent severe baseline left/right vessel stenosis (53%/41% >/=Grade 4); 31% had no vessel stenosis on either side. Baseline parenchymal injury was prevalent (85%/79% subcortical, 53%/37% cortical, 50%/35% subcortical and cortical). Most children had low or uninterpretable …

A New System For Naming Ribosomal Proteins, N. Ban, R. Beckmann, J. H. D. Cate, J. D. Dinman, F. Dragon, S. R. Ellis, D. L. J. Lafontaine, L. Lindahl, J. M. Lipton, M. Yusupov, +15 Additional Authors

Journal Articles

A system for naming ribosomal proteins is described that the authors intend to use in the future. They urge others to adopt it. The objective is to eliminate the confusion caused by the assignment of identical names to ribosomal proteins from different species that are unrelated in structure and function. In the system proposed here, homologous ribosomal proteins are assigned the same name, regardless of species. It is designed so that new names are similar enough to old names to be easily recognized, but are written in a format that unambiguously identifies them as 'new system' names.

Newborn Screening For Scid In New York State: Experience From The First Two Years, B. H. Vogel, V. Bonagura, G. A. Weinberg, M. Ballow, J. Isabelle, L. Diantonio, A. Parker, A. Young, C. Cunningham-Rundles, M. Caggana, +8 Additional Authors

Journal Articles

PURPOSE: To describe the process and assess outcomes for the first 2 years of newborn screening for severe combined immunodeficiency (SCID NBS) in New York State (NYS). METHODS: The NYS algorithm utilizes a first-tier molecular screen for TRECs (T-cell receptor excision circles), the absence of which is indicative of increased risk of immunodeficiency. RESULTS: During the first 2 years, 485,912 infants were screened for SCID. Repeat specimens were requested from 561 premature and 746 non-premature infants with low or borderline TRECs. A total of 531 infants were referred for diagnostic evaluation leading to identification of 10 infants with SCID and …

Newborn Screening For Severe Combined Immunodeficiency In 11 Screening Programs In The United States, A. Kwan, R. S. Abraham, R. Currier, A. Brower, K. Andruszewski, J. K. Abbott, M. Baker, M. Ballow, L. E. Bartoshesky, V. R. Bonagura, +70 Additional Authors

Journal Articles

IMPORTANCE: Newborn screening for severe combined immunodeficiency (SCID) using assays to detect T-cell receptor excision circles (TRECs) began in Wisconsin in 2008, and SCID was added to the national recommended uniform panel for newborn screened disorders in 2010. Currently 23 states, the District of Columbia, and the Navajo Nation conduct population-wide newborn screening for SCID. The incidence of SCID is estimated at 1 in 100,000 births. OBJECTIVES: To present data from a spectrum of SCID newborn screening programs, establish population-based incidence for SCID and other conditions with T-cell lymphopenia, and document early institution of effective treatments. DESIGN: Epidemiological and retrospective …

Teens Texting, Archana Srinivas, Megan White, Hatim A. Omar

Pediatrics Faculty Publications

In this chapter we summarize the current literature on texting use amongst adolescents. A brief overview will be presented on the prevalence of texting among teenagers, the use of texting for sexting and cyberbullying as well as the dangers of texting while driving. This chapter will serve as a brief overview of these topics in order to bring to light the emerging challenges that texting presents to the mental and physical well-being of adolescents in an effort to evoke further discussion regarding the need for increasing awareness and education to parents, educators, law makers and health care providers concerning these …

Comprehensive Analysis Of Human Cytomegalovirus Microrna Expression During Lytic And Quiescent Infection, Zhang-Zhou Shen, Xing Pan, Ling-Feng Miao, Han-Qing Ye, Stéphane Chavanas, Christian Davrinche, Michael Mcvoy, Min-Hua Luo

Pediatrics Publications

Background

Human cytomegalovirus (HCMV) encodes microRNAs (miRNAs) that function as post-transcriptional regulators of gene expression during lytic infection in permissive cells. Some miRNAs have been shown to suppress virus replication, which could help HCMV to establish or maintain latent infection. However, HCMV miRNA expression has not been comprehensively examined and compared using cell culture systems representing permissive (lytic) and semi-permissive vs. non-permissive (latent-like) infection.

Methods

Viral miRNAs levels and expression kinetics during HCMV infection were determined by miRNA-specific stem-loop RT-PCR. HCMV infected THP-1 (non-permissive), differentiated THP-1 (d-THP-1, semi-permissive) and human embryo lung fibroblasts (HELs, fully-permissive) were examined. The impact of …

Compliance With Referrals For Non-Acute Child Health Conditions: Evidence From The Longitudinal Asenze Study In Kwazulu Natal, South Africa, O. T. Uwemedimo, S. M. Arpadi, M. K. Chhagan, S. Kauchali, M. H. Craib, F. Bah, L. L. Davidson

Journal Articles

BACKGROUND: Caregiver compliance with referrals for child health services is essential to child health outcomes. Many studies in sub-Saharan Africa have examined compliance patterns for children referred for acute, life-threatening conditions but few for children referred for non-acute conditions. The aims of this analysis were to determine the rate of referral compliance and investigate factors associated with referral compliance in KwaZulu Natal, South Africa. METHODS: From September 2008-2010, a door-to-door household survey was conducted to identify children aged 4-6 years in outer-west eThekwini District, KwaZulu-Natal, South Africa. Of 2,049 identified, informed consent was obtained for 1787 (89%) children who were …

Adolescence And Human Sexuality, Donald E. Greydanus, Hatim A. Omar

Pediatrics Faculty Publications

Human sexuality is a complex phenomenon involving the interaction of biology, sex, core gender identity, and gender role behavior. Successful completion of normal stages of sexuality development is important for children and adolescents to allow for optimal life as an adult. Controversies arise for clinicians as they work with their pediatric patients regarding health care sexuality issues. It is important that clinicians help these patients in an unbiased and neutral manner. As adults, these children and adolescents will function in a number of sexuality roles, whether heterosexual, homosexual, or bisexual. This chapter reviews many of these complex and critical issues …

Emerging Biomarkers And Metabolomics For Assessing Toxic Nephropathy And Acute Kidney Injury (Aki) In Neonatology, M. Mussap, A. Noto, V. Fanos, John N. Van Den Anker

Pediatrics Faculty Publications

Identification of novel drug-induced toxic nephropathy and acute kidney injury (AKI) biomarkers has been designated as a top priority by the American Society of Nephrology. Increasing knowledge in the science of biology and medicine is leading to the discovery of still more new biomarkers and of their roles in molecular pathways triggered by physiological and pathological conditions. Concomitantly, the development of the so-called “omics” allows the progressive clinical utilization of a multitude of information, from those related to the human genome (genomics) and proteome (proteomics), including the emerging epigenomics, to those related to metabolites (metabolomics). In preterm newborns, one of …

Autosomal Recessive Polycystic Kidney Disease: The Prototype Of The Hepato-Renal Fibrocystic Diseases, Lisa Guay-Woodford

Pediatrics Faculty Publications

Autosomal recessive polycystic kidney disease (ARPKD) is a severe, typically early onset form of renal cystic disease. The care of ARPKD patients has traditionally been the purview of pediatric nephrologists for management of systemic hypertension and progressive renal insufficiency. However, the disease has multisystem manifestations and a comprehensive care strategy frequently requires a multidisciplinary team. In severely affected infants, the diagnosis often is first suspected by obstetricians when enlarged, echogenic kidneys and oligohydramnios are detected on prenatal ultrasounds. Neonatologists are central to the care of these infants, who may have respiratory compromise due to pulmonary hypoplasia and massively enlarged kidneys. …