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Article Title Abstract (Partial) Journal Year PMID SOM Author
►Autism as Representative of Disability Pediatricians care for many children with autism spectrum disorder who demonstrate a wide range of abilities and needs. This population is vulnerable because of lags in diagnosis, difficulty accessing services, overlooked medical conditions, behavioral difficulties during medical visits, parental stress, bullying, comorbid mental health issues, and variable transitional care moving from adolescence to young adulthood. Comprehensive care includes earlier recognition of symptoms with timely referral to early intervention services. Pediatr. Clin. North Am. 2020 32122564 Aloisio, Denise; Huron, Randye F.
►Addressing Challenges Facing Today's Children   Pediatr. Clin. North Am. 2020 32122572 Kairys, Steven
►Making Object Shape Explicit for Toddlers With Late Language Emergence Purpose Toddlers with late language emergence have difficulty acquiring an object vocabulary that is well defined by shape early in development. Without object words, subsequent language growth is delayed. The current study tested an intervention scaffold that highlights object shape during word teaching so that toddlers with late language emergence may establish themselves in the early stages of object word learning. Method Four toddlers with late language emergence participated in a brief dose of two interventions that differed only in semantic scaffold-a co-speech shape gesture or a co-speech indicator gesture. Co-speech refers to the word model and gesture occurring simultaneously. J. Speech Lang. Hear. Res. 2020 32109170 Capone Singleton, Nina
►Distinct interferon signatures and cytokine patterns define additional systemic autoinflammatory diseases BACKGROUNDUndifferentiated systemic autoinflammatory diseases (USAIDs) present diagnostic and therapeutic challenges. Chronic interferon (IFN) signaling and cytokine dysregulation may identify diseases with available targeted treatments.METHODSSixty-six consecutively referred USAID patients underwent underwent screening for the presence of an interferon signature using a standardized type-I IFN-response-gene score (IRG-S), cytokine profiling, and genetic evaluation by next-generation sequencing. J. Clin. Invest. 2020 31874111 Li, Suzanne
►Combined l-citrulline and tetrahydrobiopterin therapy improves NO signaling and ameliorates chronic hypoxia-induced pulmonary hypertension in newborn pigs Newborn pigs with chronic hypoxia-induced pulmonary hypertension (PH) have evidence of endothelial nitric oxide synthase (eNOS) uncoupling. In this model, we showed that therapies that promote eNOS coupling, either tetrahydrobiopterin (BH4), a NOS cofactor, or l-citrulline, a NO-l-arginine precursor, inhibit PH. We wanted to determine whether cotreatment with l-citrulline and a BH4 compound, sapropterin dihydrochloride, improves NO signaling and chronic hypoxia-induced PH more markedly than either alone. Normoxic (control) and hypoxic piglets were studied. Some hypoxic piglets received sole treatment with l-citrulline or BH4, or were cotreated with l-citrulline and BH4, from day 3 through day 10 of hypoxia. Am. J. Physiol. Lung Cell Mol. Physiol. 2020 32073878 Aschner, Judy L.
►Pharmacokinetics and safety of VALTOCO (NRL-1; diazepam nasal spray) in patients with epilepsy during seizure (ictal/peri-ictal) and nonseizure (interictal) conditions: A phase 1, open-label study OBJECTIVE: To assess pharmacokinetics and safety of diazepam nasal spray (NRL-1; VALTOCO®) in pediatric and adult patients with epilepsy in seizure and nonseizure states. METHODS: A single dose of diazepam nasal spray (5, 10, 15, or 20 mg based on weight) was administered during each of two conditions (ictal/peri-ictal and interictal condition) to patients 6-65 years old with partial or generalized epilepsy with motor seizures or seizures with clear alteration of awareness; a second dose was permitted if needed for persistent seizures. Dosing could be interictal or ictal/peri-ictal first, with a washout of ≥14 days. Epilepsia 2020 32338380 Segal, Eric B.
►Child Abuse and Neglect: The Role of the Primary Care Pediatrician Child abuse affects more than 10% of children in the United States. For most children it is the result of family dysfunction. Child abuse affects children from all socioeconomic classes. Pediatricians have an important role to play in prevention and early detection of abuse. There are sentinel injuries, now summarized as Clinical Prediction Rules that can guide the general pediatrician to take more definitive steps to suspect and report child abuse and neglect. Primary prevention should be part of the anticipatory guidance and support that pediatricians provide to all of their families. Pediatr. Clin. North Am. 2020 32122563 Kairys, Steven
Overview of Juvenile localized scleroderma and its management BACKGROUND: Juvenile localized scleroderma (JLS) is a rare pediatric disease characterized by inflammation and skin thickening. JLS is associated with deep tissue and extracutaneous involvement that often results in functional impairment and growth disturbances. This article provides an overview of the disease with a focus on active features and treatment. DATA SOURCES: We searched databases including PubMed, Elsevier and MedLine and Wanfang, reviewing publications from 2013 to 2019. Selected earlier publications were also reviewed. RESULTS: Linear scleroderma is the most common JLS subtype. Several lines of evidence suggest that JLS is an autoimmune disease. World J Pediatr 2019 31786801 Li, Suzanne C.
Prenatal Opioid Exposure: Neurodevelopmental Consequences and Future Research Priorities Neonatal opioid withdrawal syndrome (NOWS) has risen in prevalence from 1.2 per 1000 births in 2000 to 5.8 per 1000 births in 2012. Symptoms in neonates may include high-pitched cry, tremors, feeding difficulty, hypertonia, watery stools, and breathing problems. However, little is known about the neurodevelopmental consequences of prenatal opioid exposure in infancy, early childhood, and middle childhood. Even less is known about the cognitive, behavioral, and academic outcomes of children who d Pediatrics 2019 31462446 Aschner, Judy L.
Risk of Wrong-Patient Orders Among Multiple vs Singleton Births in the Neonatal Intensive Care Units of 2 Integrated Health Care Systems Importance: Multiple-birth infants in neonatal intensive care units (NICUs) have nearly identical patient identifiers and may be at greater risk of wrong-patient order errors compared with singleton-birth infants. Objectives: To assess the risk of wrong-patient orders among multiple-birth infants and singletons receiving care in the NICU and to examine the proportion of wrong-patient orders between multiple-birth infants and siblings (intrafamilial errors) and between multiple-birth infants and JAMA pediatrics 2019 31449284 Aschner, Judy L.
Developing comparative effectiveness studies for a rare, understudied pediatric disease: lessons learned from the CARRA juvenile localized scleroderma consensus treatment plan pilot study BACKGROUND: We designed and initiated a pilot comparative effectiveness study for juvenile localized scleroderma (jLS), for which there is limited evidence on best therapy. We evaluated the process we used, in relation to the specific protocol and to the general task of identifying strategies for implementing studies in rare pediatric diseases. METHODS: This was a prospective, multi-center, observational cohort study of 50 jLS patients initiating treatment, designed and conducted by the jLS grou Pediatric Rheumatology Online Journal 2019 31307476 Li, Suzanne C.
Immunopathogenesis of Juvenile Systemic Sclerosis Juvenile-onset systemic sclerosis (jSSc) is a rare and severe autoimmune disease with associated life-threatening organ inflammation and evidence of fibrosis. The organ manifestations of jSSc resemble adult SSc, but with better outcomes and survival. The etiology of jSSc appears to reflect adult-onset SSc, with similar inflammatory mediators and autoantibodies, but with a significant population of children with uncharacterized anti-nuclear antibodies. The genetics of patients with jSSc differ fr Frontiers in Immunology 2019 31293569 Li, Suzanne C.
Immunopathogenesis of Pediatric Localized Scleroderma. Localized scleroderma (LS) is a complex disease characterized by a mixture of inflammation and fibrosis of the skin that, especially in the pediatric population, also affects extracutaneous tissues ranging from muscle to the central nervous system. Although developmental origins have been hypothesized, evidence points to LS as a systemic autoimmune disorder, as there is a strong correlation to family history of autoimmune disease, the presence of shared HLA types with rheumatoid arthritis, high frequency of auto-antibodies, and elevated circulating chemokines and cytokines associated with T-helper cell, IFNgamma, and other inflammatory pathways. This inflammatory phenotype of the peripheral blood is reflected in the skin via microarray, RNA Sequencing and tissue staining. Research is underway to identify the key players in the pathogenesis of LS, but close approximation of inflammatory lymphocytic and macrophage infiltrate with collagen and fibroblasts deposition supports the notion that LS is a disease of inflammatory driven fibrosis. The immune system is dynamic and undergoes changes during childhood, and we speculate on how the unique features of the immune system in childhood could potentially contribute to some of the differences in LS between children and adults. Interestingly, the immune phenotype in pediatric LS resembles to some extent the healthy adult cellular phenotype, possibly supporting accelerated maturation of the immune system in LS. We discuss future directions in better understanding the pathophysiology of and how to better treat pediatric LS. Frontiers in immunology 2019 31114575 Li, Suzanne C.
Short term outcomes in term and late preterm neonates admitted to the well-baby nursery after resuscitation in the delivery room. OBJECTIVE: To determine the risk for deterioration in well-baby nursery (WBN) admissions after resuscitation. STUDY DESIGN: A single center retrospective study (2015-2016) of 370 resuscitated WBN admissions. RESULTS: Of the 11,307 admissions, 3.27% received resuscitation with 183 receiving continuous positive airway pressure (CPAP) alone and 187 receiving positive pressure ventilation (PPV) +/- CPAP. Resuscitated neonates were more frequently transferred to the NICU (11.6 versus 3.9%, p < 0.001) compared to those without resuscitation. More neonates requiring CPAP alone were transferred to the NICU compared to those requiring PPV +/- CPAP (15.85 versus 7.49%, p = 0.01). Univariate risk ratios for transfer were elevated for CPAP alone and lower gestational age categories. Multivariate regression analyses demonstrated increased transfer risk across gestational age categories only. CONCLUSIONS: Neonates admitted to the WBN after delivery room resuscitation are at increased risk for NICU transfer compared to those without resuscitation. This study supports the recommendation for post-resuscitation care. Journal of perinatology 2019 31101848 Spillane, Nicole T.
The Approach to the Child with Joint Complaints This article focuses on creating an orderly approach to history taking, examination, and ordering appropriate investigations when caring for a child with joint complaints. It classifies complaints as those with and without pain, swelling, or fever and of short or long duration. It recommends an approach to the physical examination and both suggests and discourages various laboratory and imaging studies. Pediatric Clinics of North America 2018 30031490 Haines, Kathleen A.
Practicing safety: a quality improvement intervention to test tools to enhance pediatric psychosocial care for children 0-3 years BACKGROUND: Child maltreatment is a significant public health issue in the United States. Yet, fewer than half of pediatricians discuss behavioral, developmental, or parenting issues with parents. OBJECTIVE: This paper describes the testing of bundles of tools and processes, part of a larger intervention, Practicing Safety, targeted at changing physician and staff behavior to identify families at risk for child maltreatment, provide anticipatory guidance, refer to community resources, and follow-up and track at-risk families. The intervention was implemented with 14 pediatric primary care practices throughout the United States; the study was completed in 2011. METHODS: A within-subjects repeated measures pre-post follow-up design was used t Primary Health Care Research & Development 2018 29248033 Kairys, Steven
Scleroderma in Children and Adolescents: Localized Scleroderma and Systemic Sclerosis Scleroderma is a rare disease that has two main forms: localized scleroderma (LS) and systemic sclerosis (SSc). Both are chronic diseases, can present in different patterns (subtypes), and are associated with extracutaneous involvement in pediatric patients. Morbidity and mortality is much worse for juvenile SSc with patients at risk for life-threatening lung, heart, and other visceral organ fibrosis and vasculopathy. Mortality is extremely rare in juvenile LS, but morbidity is common, with patients at risk for severe disfigurement and functional impairment. Scleroderma treatment is directed towards controlling inflammation and managing specific problems. Early diagnosis can greatly improve outcome. Pediatric Clinics of North America 2018 30031497 Li, Suzanne C.
Overview of Pediatric Rheumatology for the Primary Care Provider NA Pediatric Clinics of North America 2018 30031504 Li, Suzanne C.

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