Medicine and Health Sciences Commons^™

Prevalence, Morbidity, And Mortality Of Men With Sex Chromosome Aneuploidy In The Million Veteran Program Cohort, Shanlee Davis, Craig Teerlink, Julie Lynch, Bryan Gorman, Meghana Pagadala, Aoxing Liu, Matthew Panizzon, Victoria Merritt, Giulio Genovese, Judith Ross, Richard Hauger

Department of Pediatrics Faculty Papers

IMPORTANCE: The reported phenotypes of men with 47,XXY and 47,XYY syndromes include tall stature, multisystem comorbidities, and poor health-related quality of life (HRQOL). However, knowledge about these sex chromosome aneuploidy (SCA) conditions has been derived from studies in the less than 15% of patients who are clinically diagnosed and also lack diversity in age and genetic ancestry.

OBJECTIVES: To determine the prevalence of clinically diagnosed and undiagnosed X or Y chromosome aneuploidy among men enrolled in the Million Veteran Program (MVP); to describe military service metrics of men with SCAs; and to compare morbidity and mortality outcomes between men with …

The Child Healthcare At Mater Pediatric Study (Champs): A 2-Arm Cluster Randomized Control Trial Of Group Well Child Care For Mothers In Treatment For Opioid Use Disorder And Their Children, Vanessa L. Short, Diane J. Abatemarco, Erica Sood, Dennis J. Hand, Meghan Gannon, Jobayer Hossain, Neera K. Goyal

Department of Pediatrics Faculty Papers

BACKGROUND: Studies suggest that group-based well child care-a shared medical appointment where families come together as a group to receive pediatric primary care-increases patient-reported satisfaction and adherence to recommended care. Evidence supporting the use of group well child care for mothers with opioid use disorder, however, is lacking. The overall objective of the Child Healthcare at MATER Pediatric Study (CHAMPS) trial is to evaluate a group model of well child care for mothers with opioid use disorder and their children.

METHODS: CHAMPS is a single-site 2-arm cluster randomized controlled trial. A total of 108 mother-child dyads will be enrolled into …

A Training Curriculum For An Mhealth Supported Peer Counseling Program To Promote Exclusive Breastfeeding In Rural India, Roopa M. Bellad, Niranjana S. Mahantshetti, Umesh S. Charantimath, Tony Ma, Yukiko Washio, Vanessa L. Short, Katie Chang, Parth Lalakia, Frances J. Jaeger, Patricia J. Kelly, Geetanjali Mungarwadi, Chandrashekar C. Karadiguddi, Shivaprasad S. Goudar, Richard J. Derman

Department of Pediatrics Faculty Papers

BACKGROUND: Despite strong evidence about the benefits of exclusive breastfeeding, that is the baby receiving only breast milk, no other foods or liquids, rates have remained relatively unchanged over the past two decades in low- and middle-income countries. One strategy for increasing exclusive breastfeeding is through community-based programs that use peer counselors for education and support. The use of mobile health applications is also gaining increasing applicability in these countries. Minimal information is available about training peer counselors in the use of mobile technologies to support exclusive breastfeeding. The present article describes our curriculum in the state of Karnataka, India …

Active Vs Traditional Methods Of Recruiting Children For A Clinical Trial In Rural Primary Care Clinics: A Cluster-Randomized Clinical Trial, Paul M Darden, Ann M Davis, Jeannette Y Lee, Milan Bimali, Alan E Simon, Andrew M Atz, Crystal S Lim, Thao-Ly Phan, James R Roberts, Russell J Mcculloh, Lee Pyles, Michelle Shaffer, Jessica N Snowden

Department of Pediatrics Faculty Papers

Importance: To our knowledge, there are no published randomized clinical trials of recruitment strategies. Rigorously evaluated successful recruitment strategies for children are needed.

Objective: To evaluate the feasibility of 2 recruitment methods for enrolling rural children through primary care clinics to assess whether either or both methods are sufficiently effective for enrolling participants into a clinical trial of a behavioral telehealth intervention for children with overweight or obesity.

Design, setting, and participants: This cluster-randomized clinical trial of 2 recruitment methods was conducted at 4 primary care clinics in 4 separate states. Each clinic used both recruitment methods in random order. …

Genetic Testing To Inform Epilepsy Treatment Management From An International Study Of Clinical Practice, Dianalee Mcknight, Ana Morales, Kathryn E. Hatchell, Sara L. Bristow, Joshua L. Bonkowsky, Michael Scott Perry, Anne T. Berg, Felippe Borlot, Edward D. Esplin, Chad Moretz, Katie Angione, Loreto Ríos-Pohl, Robert L. Nussbaum, Swaroop Aradhya, Chad R. Haldeman-Englert, Rebecca J. Levy, Venu G. Parachuri, Guillermo Lay-Son, David J. Dávila-Ortiz De Montellano, Miguel Angel Ramirez-Garcia, Edmar O. Benítez Alonso, Julie Ziobro, Adela Chirita-Emandi, Temis M. Felix, Dianne Kulasa-Luke, Andre Megarbane, Shefali Karkare, Sarah L. Chagnon, Jennifer B. Humberson, Melissa J. Assaf, Sebastian Silva, Katherine Zarroli, Oksana Boyarchuk, Gary R. Nelson, Rachel Palmquist, Katherine C. Hammond, Sean T. Hwang, Susan B. Boutlier, Melinda Nolan, Kaitlin Y. Batley, Devraj Chavda, Carlos Alberto Reyes-Silva, Oleksandr Miroshnikov, Britton Zuccarelli, Louise Amlie-Wolf, James W. Wheless, Syndi Seinfeld, Manoj Kanhangad, Jeremy L. Freeman, Susana Monroy-Santoyo, Natalia Rodriguez-Vazquez, Monique M. Ryan, Michelle Machie, Patricio Guerra, Muhammad Jawad Hassan, Meghan S. Candee, Caleb P. Bupp, Kristen L. Park, Eric Muller, Pamela Lupo, Robert C. Pedersen, Amir M. Arain, Andrea Murphy, Krista Schatz, Weiyi Mu, Paige M. Kalika, Lautaro Plaza, Marissa A. Kellogg, Evelyn G. Lora, Robert P. Carson, Victoria Svystilnyk, Viviana Venegas, Rebecca R. Luke, Huiyuan Jiang, Tetiana Stetsenko, Milagros M. Dueñas-Roque, Joseph Trasmonte, Rebecca J. Burke, Anna C. E. Hurst, Douglas M. Smith, Lauren J. Massingham, Laura Pisani, Carrie E. Costin, Betsy Ostrander, Francis M. Filloux, Amitha L. Ananth, Ismail S. Mohamed, Alla Nechai, Jasmin M. Dao, Michael C. Fahey, Ermal Aliu, Stephen Falchek, Craig A. Press, Lauren Treat, Krista Eschbach, Angela Starks, Ryan Kammeyer, Joshua J. Bear, Mona Jacobson, Veronika Chernuha, Bailey Meibos, Kristen Wong, Matthew T. Sweney, A. Chris Espinoza, Colin B. Van Orman, Arie Weinstock, Ashutosh Kumar, Claudia Soler-Alfonso, Danielle A. Nolan, Muhammad Raza, Miguel David Rojas Carrion, Geetha Chari, Eric D. Marsh, Yael Shiloh-Malawsky, Sumit Parikh, Ernesto Gonzalez-Giraldo, Stephen Fulton, Yoshimi Sogawa, Kaitlyn Burns, Myroslava Malets, Johnny David Montiel Blanco, Christa W. Habela, Carey A. Wilson, Guillermo G. Guzmán, Mariia Pavliuk

Department of Pediatrics Faculty Papers

IMPORTANCE: It is currently unknown how often and in which ways a genetic diagnosis given to a patient with epilepsy is associated with clinical management and outcomes.

OBJECTIVE: To evaluate how genetic diagnoses in patients with epilepsy are associated with clinical management and outcomes.

DESIGN, SETTING, AND PARTICIPANTS: This was a retrospective cross-sectional study of patients referred for multigene panel testing between March 18, 2016, and August 3, 2020, with outcomes reported between May and November 2020. The study setting included a commercial genetic testing laboratory and multicenter clinical practices. Patients with epilepsy, regardless of sociodemographic features, who received a …

A Diagnostic Dilemma Of Antiglutamic Acid Decarboxylase 65 (Anti-Gad 65) And Mycoplasma Pneumoniae Antibodies In A Girl Presenting With Acute-Onset Obsessive-Compulsive Disorder., Cecilia Freeman, Antanoid J Langeveldt, Robyn R Miller

Department of Pediatrics Faculty Papers

Acute-onset obsessive-compulsive disorder can be challenging, especially when triggered by an underlying disease process. Clinicians often turn to Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS), but it is important to consider a broad differential in these patients. We present a case of a 9-year-old girl with acute-onset obsessive-compulsive behavior likely triggered by a post-infectious phenomenon that ultimately resolved following treatment with plasmapheresis.

Prenatal Exposure To Methadone Or Buprenorphine: Early Childhood Developmental Outcomes., Karol Kaltenbach, Kevin E O'Grady, Sarah H. Heil, Amy L. Salisbury, Mara G. Coyle, Gabriele Fischer, Peter R. Martin, Susan Stine, Hendrée E. Jones

Department of Pediatrics Faculty Papers

BACKGROUND: Methadone and buprenorphine are recommended to treat opioid use disorders during pregnancy. However, the literature on the relationship between longer-term effects of prenatal exposure to these medications and childhood development is both spare and inconsistent.

METHODS: Participants were 96 children and their mothers who participated in MOTHER, a randomized controlled trial of opioid-agonist pharmacotherapy during pregnancy. The present study examined child growth parameters, cognition, language abilities, sensory processing, and temperament from 0 to 36 months of the child's life. Maternal perceptions of parenting stress, home environment, and addiction severity were also examined.

RESULTS: Tests of mean differences between children …

Fasoracetam In Adolescents With Adhd And Glutamatergic Gene Network Variants Disrupting Mglur Neurotransmitter Signaling., Josephine Elia, Grace Ungal, Charlly Kao, Alexander Ambrosini, Nilsa De Jesus-Rosario, Lene Larsen, Rosetta Chiavacci, Tiancheng Wang, Christine Kurian, Kanani Titchen, Brian Sykes, Sharon Hwang, Bhumi Kumar, Jacqueline Potts, Joshua Davis, Jeffrey Malatack, Emma Slattery, Ganesh Moorthy, Athena Zuppa, Andrew Weller, Enda Byrne, Yun R. Li, Walter K. Kraft, Hakon Hakonarson

Department of Pediatrics Faculty Papers

The glutamatergic neurotransmitter system may play an important role in attention-deficit hyperactivity disorder (ADHD). This 5-week, open-label, single-blind, placebo-controlled study reports the safety, pharmacokinetics and responsiveness of the metabotropic glutamate receptor (mGluR) activator fasoracetam (NFC-1), in 30 adolescents, age 12-17 years with ADHD, harboring mutations in mGluR network genes. Mutation status was double-blinded. A single-dose pharmacokinetic profiling from 50-800 mg was followed by a single-blind placebo at week 1 and subsequent symptom-driven dose advancement up to 400 mg BID for 4 weeks. NFC-1 treatment resulted in significant improvement. Mean Clinical Global Impressions-Improvement (CGI-I) and Severity (CGI-S) scores were, respectively, 3.79 …

Characterization Of Pulmonary Metastases In Children With Hepatoblastoma Treated On Children's Oncology Group Protocol Ahep0731 (The Treatment Of Children With All Stages Of Hepatoblastoma): A Report From The Children's Oncology Group., Allison F. O'Neill, Alexander J. Towbin, Mark D. Krailo, Caihong Xia, Yun Gao, M. Beth Mccarville, Rebecka L. Meyers, Eugene D. Mcgahren, Greg M. Tiao, Stephen P. Dunn, Max R. Langham, Christopher B. Weldon, Milton J. Finegold, Sarangarajan Ranganathan, Wayne L. Furman, Marcio Malogolowkin, Carlos Rodriguez-Galindo, Howard M. Katzenstein

Department of Pediatrics Faculty Papers

Purpose To determine whether the pattern of lung nodules in children with metastatic hepatoblastoma (HB) correlates with outcome. Methods Thirty-two patients with metastatic HB were enrolled on Children's Oncology Group Protocol AHEP0731 and treated with vincristine and irinotecan (VI). Responders to VI received two additional cycles of VI intermixed with six cycles of cisplatin/fluorouracil/vincristine/doxorubicin (C5VD), and nonresponders received six cycles of C5VD alone. Patients were imaged after every two cycles and at the conclusion of therapy. All computed tomography scans and pathology reports were centrally reviewed, and information was collected regarding lung nodule number, size, laterality, timing of resolution, and …

Lung Rest During Extracorporeal Membrane Oxygenation For Neonatal Respiratory Failure-Practice Variations And Outcomes., Deepthi Alapati, Zubair H. Aghai, Jobayer Hossain, Daniel R Dirnberger, Mark T. Ogino, Thomas H. Shaffer

Department of Pediatrics Faculty Papers

OBJECTIVE: Describe practice variations in ventilator strategies used for lung rest during extracorporeal membrane oxygenation for respiratory failure in neonates, and assess the potential impact of various lung rest strategies on the duration of extracorporeal membrane oxygenation and the duration of mechanical ventilation after decannulation.

DATA SOURCES: Retrospective cohort analysis from the Extracorporeal Life Support Organization registry database during the years 2008-2013.

STUDY SELECTION: All extracorporeal membrane oxygenation runs for infants less than or equal to 30 days of life for pulmonary reasons were included.

DATA EXTRACTION: Ventilator type and ventilator settings used for lung rest at 24 hours after …

A Double-Blind Randomized Trial Of Fish Oil To Lower Triglycerides And Improve Cardiometabolic Risk In Adolescents., Samuel S. Gidding, Carol Prospero, Jobayer Hossain, Frances Zappalla, Prabhakaran Babu Balagopal, Bonita Falkner, Peter Kwiterovich

Department of Pediatrics Faculty Papers

OBJECTIVES: To determine the efficacy of 4 g/day fish oil to lower triglycerides and impact lipoprotein particles, inflammation, insulin resistance, coagulation, and thrombosis.

STUDY DESIGN: Participants (n = 42, age 14 ± 2 years) with hypertriglyceridemia and low-density lipoprotein (LDL) cholesterol/dL were enrolled in a randomized, double-blind, crossover trial comparing 4 g of fish oil daily with placebo. Treatment interval was 8 weeks with a 4-week washout. Lipid profile, lipoprotein particle distribution and size, glucose, insulin, high-sensitivity C-reactive protein, interleukin-6, fibrinogen, plasminogen activator inhibitor-1, and thrombin generation were measured.

RESULTS: Baseline lipid profile was total cholesterol 194 (5.4) mg/dL (mean …

Phenotypic Parameters Predict Time To Normalization In Infants With Hypogammaglobulinemia., Robert C. Van Winkle, Walter W. Hauck, Stephen J. Mcgeady

Department of Pediatrics Faculty Papers

PURPOSE: Infants with recurrent infection may be found to have hypogammaglobulinemia without impaired specific antibody responses. Many will be diagnosed with transient hypogammaglobulinemia of infancy.

METHODS: This study used a parametric survival analysis of 100 infants with hypogammaglobulinemia to predict time to normalization.

RESULTS: Aggregate initial immunoglobulins (IgG + IgA + IgM), as a percentage of age-adjusted normal, predicted time to resolution: median time to resolution for the infants in the lowest quartile of aggregate levels (≤81 % of age-adjusted lower limits) was greater than 5 years, with 34 % resolving in 3 years. For infants in the highest quartile …

The Differential Effects Of Maternal Age, Race/Ethnicity And Insurance On Neonatal Intensive Care Unit Admission Rates., Beatriz E De Jongh, Robert Locke, David A Paul, Matthew Hoffman

Department of Pediatrics Faculty Papers

BACKGROUND: Maternal race/ethnicity, age, and socioeconomic status (SES) are important factors determining birth outcome. Previous studies have demonstrated that, teenagers, and mothers with advanced maternal age (AMA), and Black/Non-Hispanic race/ethnicity can independently increase the risk for a poor pregnancy outcome. Similarly, public insurance has been associated with suboptimal health outcomes. The interaction and impact on the risk of a pregnancy resulting in a NICU admission has not been studied. Our aim was, to analyze the simultaneous interactions of teen/advanced maternal age (AMA), race/ethnicity and socioeconomic status on the odds of NICU admission.

METHODS: The Consortium of Safe Labor Database (subset …

Childhood Obesity And Blood Pressure: Back To The Future?, Bonita Falkner, Samuel Gidding

Department of Pediatrics Faculty Papers

The prevalence of childhood hypertension is increasing.^{1, 2} Studies that apply the 95^th percentile definition and repeat measurements on three separate visits, report a pediatric hypertension prevalence of approximately 3.5%^{3, 4}, and among obese children and adolescents the prevalence of both hypertension and prehypertension is even greater. The recently documented increase in hypertension among the young is due largely to the childhood obesity epidemic and possibly other secular changes in lifestyles. These publications and others confirm that hypertension is a prevalent child health condition, especially among overweight and obese children. A consistent positive association between body …

Periodic Fever, Aphthous Stomatitis, Pharyngitis, And Adenitis (Pfapa) Is A Disorder Of Innate Immunity And Th1 Activation Responsive To Il-1 Blockade., Silvia Stojanov, Sivia Lapidus, Puja Chitkara, Henry Feder, Juan C Salazar, Thomas A Fleisher, Margaret R Brown, Kathryn M Edwards, Michael M Ward, Robert A Colbert, Hong-Wei Sun, Geryl M Wood, Beverly K Barham, Anne Jones, Ivona Aksentijevich, Raphaela Goldbach-Mansky, Balu Athreya, Karyl S Barron, Daniel L Kastner

Department of Pediatrics Faculty Papers

The syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is the most common periodic fever disease in children. However, the pathogenesis is unknown. Using a systems biology approach we analyzed blood samples from PFAPA patients whose genetic testing excluded hereditary periodic fevers (HPFs), and from healthy children and pediatric HPF patients. Gene expression profiling could clearly distinguish PFAPA flares from asymptomatic intervals, HPF flares, and healthy controls. During PFAPA attacks, complement (C1QB, C2, SERPING1), IL-1-related (IL-1B, IL-1RN, CASP1, IL18RAP), and IFN-induced (AIM2, IP-10/CXCL10) genes were significantly overexpressed, but T cell-associated transcripts (CD3, CD8B) were down-regulated. On the …

An Evaluation Of Hospital Breastfeeding Policies In The Philadelphia Metropolitan Area 1994-2009: A Comparison With The Baby-Friendly Hospital Initiative Ten Steps., Andrea Crivelli-Kovach, Esther K Chung

Department of Pediatrics Faculty Papers

OBJECTIVE: The purpose of this study was to describe current breastfeeding policies and practices among Philadelphia, PA metropolitan hospitals and changes in their policies and practices over time.

METHODS: In-person group interviews were conducted to obtain a composite picture of actual breastfeeding policies and practices. One questionnaire per hospital was completed based on responses from group consensus. Twenty-five hospitals providing maternity care were contacted. Information was obtained from personnel representing different areas of maternity services. Hospitals were classified according to the degree to which they were implementing the Ten Steps to Successful Breastfeeding.

RESULTS: Mean breastfeeding rates at suburban hospitals …

Does Treatment Of Premature Labor With Terbutaline Increase The Risk Of Autism Spectrum Disorders?, Patricia Rodier, Richard K Miller, Robert L Brent

Department of Pediatrics Faculty Papers

Beta-adrenergic agents have been used in pregnant women for the treatment of premature labor and for the treatment of asthma. Concerns have been expressed that exposure to terbutaline, a beta-2 adrenergic agonist, may increase the risk of autism spectrum disorders (ASDs) in the offspring. This hypothesis deserves critical review, given the number of patients exposed to the drug in the last two decades. The results are important to both the obstetricians and patients who weigh the risks and benefits of interventions and to the pediatricians who counsel the families of affected children.

A Case Study In Unethical Transgressive Bioethics: "Letter Of Concern From Bioethicists" About The Prenatal Administration Of Dexamethasone., Laurence B Mccullough, Frank A Chervenak, Robert L Brent, Benjamin Hippen

Department of Pediatrics Faculty Papers

On February 3, 2010, a "Letter of Concern from Bioethicists," organized by fetaldex.org, was sent to report suspected violations of the ethics of human subjects research in the off-label use of dexamethasone during pregnancy by Dr. Maria New. Copies of this letter were submitted to the FDA Office of Pediatric Therapeutics, the Department of Health and Human Services (DHHS) Office for Human Research Protections, and three universities where Dr. New has held or holds appointments. We provide a critical appraisal of the Letter of Concern and show that it makes false claims, misrepresents scientific publications and websites, fails to meet …

The Intellectual And Moral Integrity Of Bioethics: Response To Commentaries On "A Case Study In Unethical Transgressive Bioethics: 'Letter Of Concern From Bioethicists' About The Prenatal Administration Of Dexamethasone"., Laurence B Mccullough, Frank A Chervenak, Robert L Brent, Benjamin Hippen

Department of Pediatrics Faculty Papers

In our target article we showed that the Letter of Concern (LoC) fails to meet accepted standards for presenting empirical data for the purpose of supplementing a normative claim and for argument-based normative ethics. The LoC fails to meet the standards of evidence-based reasoning by making false claims, failing to reference data that undermine its key premises, and misrepresenting and misinterpreting the scientific publications it selectively references. The LoC fails to meet the standards of argument-based reasoning by treating as settled matters what are, instead, ongoing controversies, offering “mere opinion” as a substitute for argument, and making contradictory claims. The …

The Perils Of The Imperfect Expectation Of The Perfect Baby., Frank A Chervenak, Laurence B Mccullough, Robert L Brent

Department of Pediatrics Faculty Papers

Advances in modern medicine invite the assumption that medicine can control human biology. There is a perilous logic that leads from expectations of medicine's control over reproductive biology to the expectation of having a perfect baby. This article proposes that obstetricians should take a preventive ethics approach to the care of pregnant women with expectations for a perfect baby. We use Nathaniel Hawthorne's classic short story, "The Birthmark," to illustrate the perils of the logic of control and perfection through science and then identify possible contemporary sources of the expectation of the perfect baby. We propose that the informed consent …

State Infant Mortality: An Ecologic Study To Determine Modifiable Risks And Adjusted Infant Mortality Rates., David A. Paul. Md, Amy Mackley, Rnc, Robert G. Locke, Do, John L. Stefano, Md, Charlan Kroelinger, Phd

Department of Pediatrics Faculty Papers

OBJECTIVE: To determine factors contributing to state infant mortality rates (IMR) and develop an adjusted IMR in the United States for 2001 and 2002. DESIGN/METHODS: Ecologic study of factors contributing to state IMR. State IMR for 2001 and 2002 were obtained from the United States linked death and birth certificate data from the National Center for Health Statistics. Factors investigated using multivariable linear regression included state racial demographics, ethnicity, state population, median income, education, teen birth rate, proportion of obesity, smoking during pregnancy, diabetes, hypertension, cesarean delivery, prenatal care, health insurance, self-report of mental illness, and number of in-vitro fertilization …

Hematologic Effects Of Placental Pathology On Very Low Birthweight Infants Born To Mothers With Preeclampsia., Kelly J. Zook, Md, Amy B. Mackley, Rnc, Jennifer Kern, David A. Paul. Md

Department of Pediatrics Faculty Papers

OBJECTIVE: To investigate the effect of placental pathology on neonatal neutrophils, platelets, hematocrit and nucleated red blood cells in very low birthweight (VLBW) infants born to mothers with preeclampsia. STUDY DESIGN: Retrospective cohort study of infants with birthweight < 1500 g born to mothers with preeclampsia from july, 2002 to july, 2006 at a single level III neonatal intensive care unit. Placental pathology was reviewed for the presence of placental infarction and vasculopathy. Hematologic parameters from day of life 0, 1 and 2 were obtained. Statistical analysis included repeated-measures analysis of variance and multivariable analysis using logistic regression. RESULT: The study sample included 203 infants with estimated gestational age of 28+/-3 weeks; 45% had placental infarctions and 26% placental vasculopathy. Infants with neutropenia and thrombocytopenia did not have an increased occurrence of placental infarction or maternal vasculopathy but were more likely to be of small gestational age (SGA) and of lower gestational age compared with infants without neutropenia or thrombocytopenia. After multivariable analysis, gestational age and SGA remained associated with both neutropenia and thrombocytopenia whereas placental infarction and vasculopathy did not remain in the models. CONCLUSION: In our population of VLBW infants born to mothers with preeclampsia, placental pathology was common. There was no association of placental infarction or vasculopathy with neonatal neutropenia and thrombocytopenia. The data suggest that neonatal hematologic effects of maternal preeclampsia, if related to the placenta, are associated with factors other than placental histology.

Infant Mortality In Delaware: The Need To Improve Maternal Health And Reduce Premature Births., David A. Paul. Md

Department of Pediatrics Faculty Papers

Delaware's high infant mortality rate continues to be driven by an increasing number of premature births. Premature birth is a complex problem with both social and medical roots. Based on the CDC road map, programs aimed at improving preconception health of women of childbearing age are blossoming in Delaware. In addition, Delaware's infant mortality rate can only be reduced if we are able to diminish disparities in health care delivery, and improve the social factors influencing infant mortality. These factors include diminishing family support, low high school graduation rate, poor housing, and a lack of health insurance.

Continuous Source Of Care Among Young Underserved Children: Associated Characteristics And Use Of Recommended Parenting Practices., Esther K Chung, Leny Mathew, Kelly F Mccollum, Irma T Elo, Jennifer F Culhane

Department of Pediatrics Faculty Papers

OBJECTIVES: The aim of this study was to 1) assess sociodemographic and health characteristics associated with having a continuous source of care (CSOC) among young children and 2) determine the relationship between having a CSOC and use of parenting practices.

METHODS: We conducted a prospective, community-based survey of women receiving prenatal care at Philadelphia community health centers. We conducted surveys at the first prenatal visit and at a mean age +/- standard deviation of 3 +/-1, 11 +/- 1, and 24 +/- 2 months postpartum, obtaining information on sociodemographic and health characteristics, child's health care provider, and 6 parenting practices. …

Microvascular Endothelial Cells Express A Phosphatidylserine Receptor: A Functionally Active Receptor For Phosphatidylserine-Positive Erythrocytes, Yamaja Setty, Suhita Gayen Betal

Department of Pediatrics Faculty Papers

Phosphatidylserine (PS)-positive erythrocytes adhere to endothelium and subendothelial matrix components. While thrombospondin mediates these inter-actions, it is unknown whether PS-associated erythrocyte-endothelial adhesion occurs in the absence of plasma ligands. Using ionophore-treated PS-expressing control HbAA erythrocytes, we demonstrate that PS-positive erythrocytes adhered to human lung microendothelial cells in the absence of plasma ligands, that this adhesion was enhanced following endothelial activation with IL-1alpha, TNF-alpha, LPS, hypoxia, and heme, and that this adhesive interaction was selective to erythrocyte PS. We next explored whether microendothelial cells express an adhesion receptor that recognizes cell surface-expressed PS (PSR) similar to that expressed on activated macrophages. …

Homocysteine Levels In Preterm Infants: Is There An Association With Intraventricular Hemorrhage? A Prospective Cohort Study., Wendy J Sturtz, Kathleen H Leef, Amy B Mackley, Shailja Sharma, Teodoro Bottiglieri, David A Paul

Department of Pediatrics Faculty Papers

BACKGROUND: The purpose of this study was to characterize total homocysteine (tHcy) levels at birth in preterm and term infants and identify associations with intraventricular hemorrhage (IVH) and other neonatal outcomes such as mortality, sepsis, necrotizing enterocolitis, bronchopulmonary dysplasia, and thrombocytopenia. METHODS: 123 infants < 32 weeks gestation admitted to our Level III nursery were enrolled. A group of 25 term infants were enrolled for comparison. Two blood spots collected on filter paper with admission blood drawing were analyzed by a high performance liquid chromatography (HPLC) method. Statistical analysis included ANOVA, Spearman's Rank Order Correlation and Mann-Whitney U test. RESULTS: The median tHcy was 2.75 micromol/L with an interquartile range of 1.34 - 4.96 micromol/L. There was no difference between preterm and term tHcy (median 2.76, IQR 1.25 - 4.8 micromol/L vs median 2.54, IQR 1.55 - 7.85 micromol/L, p = 0.07). There was no statistically significant difference in tHcy in 31 preterm infants with IVH compared to infants without IVH (median 1.96, IQR 1.09 - 4.35 micromol/L vs median 2.96, IQR 1.51 - 4.84 micromol/L, p = 0.43). There was also no statistically significant difference in tHcy in 7 infants with periventricular leukomalacia (PVL) compared to infants without PVL (median 1.55, IQR 0.25 - 3.45 micromol/L vs median 2.85, IQR 1.34 - 4.82 micromol/L, p = 0.07). Male infants had lower tHcy compared to female; prenatal steroids were associated with a higher tHcy. CONCLUSION: In our population of preterm infants, there is no association between IVH and tHcy. Male gender, prenatal steroids and preeclampsia were associated with differences in tHcy levels.

A Turner Syndrome Neurocognitive Phenotype Maps To Xp22.3., Andrew R Zinn, David Roeltgen, Gerry Stefanatos, Purita Ramos, Frederick F Elder, Harvey Kushner, Karen Kowal, Judith L Ross

Department of Pediatrics Faculty Papers

BACKGROUND: Turner syndrome (TS) is associated with a neurocognitive phenotype that includes selective nonverbal deficits, e.g., impaired visual-spatial abilities. We previously reported evidence that this phenotype results from haploinsufficiency of one or more genes on distal Xp. This inference was based on genotype/phenotype comparisons of individual girls and women with partial Xp deletions, with the neurocognitive phenotype considered a dichotomous trait. We sought to confirm our findings in a large cohort (n = 47) of adult women with partial deletions of Xp or Xq, enriched for subjects with distal Xp deletions. METHODS: Subjects were recruited from North American genetics and …

Ochrobactrum Anthropi Bacteremia In A Child., Joel D. Klein, Stephen C. Eppes

Department of Pediatrics Faculty Papers

Children undergoing chemotherapy for cancer are at increased risk of supervening infection, including bacteremia. This may be due to immunosuppression as well as the presence of intravascular devices such as central venous catheters. Bacteria responsible for these infections include those found in the child's endogenous flora as well as in environmental sources. We report the case of a child with acute lymphoblastic leukemia (ALL) who developed bacteremia secondary to Ochrobactrum anthropi. Details of this extremely rare infection are the focus of this report.

Childhood Typhoid Fever Diagnosed In An Urban U.S. Setting., Surinder K. Sodhi, Stephen C. Eppes, Joel D. Klein

Department of Pediatrics Faculty Papers

The majority of cases of childhood typhoid fever diagnosed in the United States occur in persons recently returned from travel abroad. We report two childhood cases of typhoid fever acquired in Mexico and India and diagnosed in an urban United States setting. This report describes these cases and provides insights into the pitfalls in recognizing this condition in a nonendemic setting as well as information concerning differential diagnosis and treatment.

Malignant External Otitis And Mastoiditis Associated With An Igg4 Subclass Deficiency In A Child., Ricardo Castro, Md, Norman Robinson, Md, Joel Klein, Md, William Geimeier, Md

Department of Pediatrics Faculty Papers

We have presented the first child in Delaware with malignant external otitis associated with IgG4 deficiency. Our patient needed three courses of intravenous antibiotics and twice required mastoidectomy, but has recovered completely following the restoration of the natural barrier between the internal and external ear, using a fascial graft from the large temporalis muscle. Some hearing deficit remains.