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Article Title Abstract (Partial) Journal Year PMID SOM Author
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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