Abstract:
PURPOSE OF REVIEW:To summarize recent progress in our understanding of immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) and IPEX-related disorders. RECENT FINDINGS:A number of Mendelian disorders of immune dysregulation and autoimmunity have been noted to result from defects in T regulatory cell, development and function. The best characterized of these is IPEX, resulting from mutations affecting FOXP3. A number of other gene defects that affect T regulatory cell function also give rise to IPEX-related phenotypes, including loss-of-function mutations in CD25, STAT5b and ITCH. Recent progress includes the identification of gain-of-function mutations in STAT1 as a cause of an IPEX-like disease, emerging FOXP3 genotype/phenotype relationships in IPEX, and the elucidation of a role for the microbiota in the immune dysregulation associated with regulatory T cell deficiency. SUMMARY:An expanding spectrum of genetic defects that compromise T regulatory cell function underlies human disorders of immune dysregulation and autoimmunity. Collectively, these disorders offer novel insights into pathways of peripheral tolerance and their disruption in autoimmunity.
journal_name
Curr Opin Pediatrjournal_title
Current opinion in pediatricsauthors
Verbsky JW,Chatila TAdoi
10.1097/MOP.0000000000000029subject
Has Abstractpub_date
2013-12-01 00:00:00pages
708-14issue
6eissn
1040-8703issn
1531-698Xpii
00008480-201312000-00013journal_volume
25pub_type
杂志文章,评审abstract:PURPOSE OF REVIEW:The purpose of this review is to describe current concepts on inflammation, immunity and insulin resistance. Metabolic and immune systems are closely involved in inflammation and significantly contribute to the pathology seen in the pediatric intensive care unit. The ability of insulin to decrease hep...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e3283464b3e
更新日期:2011-06-01 00:00:00
abstract:PURPOSE OF REVIEW:The prevalence of pediatric atopic dermatitis (AD) has increased throughout the world, now ranging from 10 to 20% in developed countries. Pediatric patients with AD make up a substantial proportion of patients seen by general pediatricians, allergists, dermatologists, and other specialists. As such, t...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000109
更新日期:2014-08-01 00:00:00
abstract:PURPOSE OF REVIEW:Diverse methodological approaches pose significant challenges to assess environmental exposure effects on child health outcomes. Although transdisciplinary research efforts offer unique opportunities for understanding the complex and multidimensional facets of lifespan health and disease trajectories,...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000606
更新日期:2018-04-01 00:00:00
abstract:PURPOSE OF REVIEW:Congenital hyperinsulinism is the most common cause of persistent hypoglycemia in infants and children. Early and appropriate recognition and treatment of hypoglycemia is vital to minimize neurocognitive impairment. RECENT FINDINGS:There are at least 11 known monogenic forms of hyperinsulinism and se...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000645
更新日期:2018-08-01 00:00:00
abstract:PURPOSE OF REVIEW:This study reviews the history of neonatal drug development, recent legislative efforts designed to facilitate the study of therapeutic agents in neonates, and future steps necessary to advance drug development. RECENT FINDINGS:Although many federal regulations have been introduced over the past 15 y...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000463
更新日期:2017-04-01 00:00:00
abstract:PURPOSE OF REVIEW:The obesity epidemic confronts the pediatrician every day in the office. Pediatricians should help curb the epidemic through prevention and the usual pediatric primary care tasks of screening, communication and anticipatory counseling. This review highlights findings from recent literature to guide of...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e328151c3e9
更新日期:2007-06-01 00:00:00
abstract::Nocturnal enuresis is a common problem occurring in 15% of children at 5 years of age. Although usually self-limiting, justification for early treatment is founded in the psychological impact on the child. The physician's role in treating enuresis is first to rule out structural causes for enuresis. After this has bee...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199404000-00012
更新日期:1994-04-01 00:00:00
abstract:PURPOSE OF REVIEW:Neurodevelopmental-behavioural paediatrics (NBP) is a field of medical practice that has arisen in response to recent changes in child health epidemiology. This review considers how the profession of NBP is addressing clinical need, and discusses possibilities for future development of the field. REC...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000819
更新日期:2019-12-01 00:00:00
abstract:PURPOSE OF REVIEW:Simulation has been widely adopted as a training and assessment tool in medical education. Conventional teaching methods may be inadequate to properly train healthcare providers for rare but potentially lethal events in pediatrics such as trauma and respiratory arrest. Recent studies suggest pediatric...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e32832b32dc
更新日期:2009-06-01 00:00:00
abstract:PURPOSE OF REVIEW:To provide clinicians with an updated overview of key considerations related to the clinical assessment and management of attention-deficit/hyperactivity disorder (ADHD) in adolescents. RECENT FINDINGS:The American Psychiatric Association's recently revised Diagnostic and Statistical Manual of Mental...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000053
更新日期:2014-02-01 00:00:00
abstract:PURPOSE OF REVIEW:This review will examine the current evidence regarding pediatric blunt abdominal trauma and the physical exam findings, laboratory values, and radiographic imaging associated with the diagnosis of intra-abdominal injuries (IAI), as well as review the current literature on pediatric hollow viscus inju...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e328352de97
更新日期:2012-06-01 00:00:00
abstract::Choledochal cysts are a relatively rare abnormality in the West but are more common in the East. The etiology of choledochal cysts remains unknown. Recently, the incidence in neonates and young infants has been increasing due to advances in diagnostic imaging, including antenatal diagnosis. Choledochal cysts can prese...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199706000-00018
更新日期:1997-06-01 00:00:00
abstract::Osteochondritis dissecans is a term used to describe the separation of an articular cartilage subchondral bone segment from the remaining articular surface. Juvenile osteochondritis dissecans describes an osteochondritis dissecans lesion found in skeletally immature children with a maximum incidence occurring between ...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-200302000-00007
更新日期:2003-02-01 00:00:00
abstract::Dyslipidemia increases the risk of cardiovascular events among individuals with renal disease, and there is a growing body of evidence that it hastens the progression of renal disease itself. Children with nephrotic syndrome or renal transplants have easily recognized hyperlipidemia. Among those with chronic renal ins...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-200204000-00009
更新日期:2002-04-01 00:00:00
abstract:PURPOSE OF REVIEW:The number of pediatric cancer survivors is growing rapidly as treatments become more effective. However, many current regimens cause gonadotoxicity and permanent infertility, significantly impacting quality of life. The purpose of this review is to update pediatric oncologists on risk factors for can...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e3283420fb6
更新日期:2011-02-01 00:00:00
abstract:PURPOSE OF REVIEW:Over 80% of children diagnosed with cancer are now cured. The burgeoning population of survivors of childhood cancer experiences high rates of morbidity and mortality due to 'late-effects' of treatment. These can be defined as any consequence of treatment that persists beyond or develops after the com...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000681
更新日期:2018-12-01 00:00:00
abstract:PURPOSE OF REVIEW:This article reviews current work investigating the neural bases of autism spectrum disorder (ASD) within the discipline of electrophysiological brain research. The manuscript focuses primarily on advances in understanding related to social information processing and interconnectivity among brain syst...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e32834cb9c9
更新日期:2011-12-01 00:00:00
abstract:PURPOSE OF REVIEW:Ras pathway mutations are one of the most common type of alterations in pediatric hematologic malignancies and are frequently associated with adverse outcomes. Despite ongoing efforts to use targeted treatments, there remain no Food and Drug Administration (FDA)-approved medications specifically for c...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章
doi:10.1097/MOP.0000000000000981
更新日期:2021-02-01 00:00:00
abstract::Hypoxic-ischemic brain injury occurs frequently in infancy and childhood. Events such as perinatal asphyxia, near drowning, respiratory arrest, and near sudden infant death syndrome cause significant mortality and morbidity. Despite current critical care practices, the outcomes from such injuries may be life-long neur...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199906000-00009
更新日期:1999-06-01 00:00:00
abstract::Now known as a Garrodian inborn error caused by the homozygous state of many different autosomal recessive mutations of the 7-dehydrocholesterol reductase gene leading to deficient conversion of 7-dehydrocholesterol to cholesterol, the RSH (so-called Smith-Lemli-Opitz) syndrome has become a paradigmatic metabolic malf...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199908000-00015
更新日期:1999-08-01 00:00:00
abstract:PURPOSE OF REVIEW:The HIV/AIDS epidemic is one of the most widespread public health problems today. Teens with mental health concerns are particularly vulnerable to infection because they engage in higher rates of risky behaviors than their peers. Families are vital influences in protecting teens from HIV exposure, but...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/01.mop.0000245356.68207.d2
更新日期:2006-10-01 00:00:00
abstract:PURPOSE OF REVIEW:The current review will focus on the current knowledge of the contribution of both germline and somatic mutations to the development and management of cancer in pediatric patients. RECENT FINDINGS:It has long been thought that genetic mutations in both germline and somatic cells can contribute to the...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000543
更新日期:2017-12-01 00:00:00
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journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000519
更新日期:2017-08-01 00:00:00
abstract::Abdominal imaging continues to grow in importance for diagnosis, anatomic staging, and functional analysis of gastrointestinal ailments. In this review, we attempt to delineate those publications, both original investigations and summary articles, that have advanced our understanding of abdominal imaging in infants an...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199610000-00012
更新日期:1996-10-01 00:00:00
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journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000214
更新日期:2015-06-01 00:00:00
abstract:PURPOSE OF REVIEW:Nonalcoholic fatty liver disease is a common condition that may progress to end stage liver disease. It is closely associated with obesity and insulin resistance, two conditions whose prevalence is increasing dramatically in children. This could potentially make nonalcoholic fatty liver disease the mo...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/01.mop.0000172816.79637.c5
更新日期:2005-10-01 00:00:00
abstract:PURPOSE OF REVIEW:An understanding of vaccine safety is important for all immunization providers, who have responsibilities to identify, report, and prevent adverse events. RECENT FINDINGS:New analytic methods can provide more rapid information on adverse events compared with traditional observational studies. Some ad...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e3283350425
更新日期:2010-02-01 00:00:00
abstract::Infectious disease emergencies are those in which delays in diagnosis or treatment may lead to untoward harm. Recent developments in the field offer few new therapies, but rather emphasize familiar infections in new guises or clarify approaches to troublesome entities. An enduring concern for the legal risks implicit ...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199406000-00013
更新日期:1994-06-01 00:00:00
abstract:PURPOSE OF REVIEW:P450 oxidoreductase deficiency--a newly described form of congenital adrenal hyperplasia--typically presents a steroid profile suggesting combined deficiencies of steroid 21-hydroxylase and 17alpha-hydroxylase/17,20-lyase activities. These and other enzymes require electron donation from P450 oxidored...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/01.mop.0000236395.71956.5c
更新日期:2006-08-01 00:00:00
abstract:PURPOSE OF REVIEW:This review summarizes for the pediatrician the current understanding of normal cerebellar and brainstem development, and then discusses selected malformations to highlight advances in the area. The impact of prematurity on cerebellar growth and development is then examined. The important insights pro...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e32801080e8
更新日期:2006-12-01 00:00:00