Abstract:
PURPOSE OF REVIEW:Epidermolysis bullosa is a hereditary skin disorder characterized by skin fragility. However, the disease can manifest in many different organ systems, therefore children born with epidermolysis bullosa may have life long, complex medical needs. In this review, we will use a system-based approach to highlight important aspects of disease management and recent advancements in each of the areas. In addition, we will overview some of the cutting edge therapeutic developments in epidermolysis bullosa. RECENT FINDINGS:Recent advancements in supportive care of epidermolysis bullosa with focus on wound, pain, pruritus and nutrition status were discussed. Clinical surveillance and complication prevention are critical to improve clinical outcomes. Generalized epidermolysis bullosa is a systemic disease with increased morbidity and mortality; therefore, complex care using a multidisciplinary approach will provide the greatest benefits for patients. Current targeted treatments for epidermolysis bullosa aim at restoring the skin integrity using protein, cell, and gene therapies. SUMMARY:Improvement in care of epidermolysis bullosa in recent years results from keen clinical observation, novel molecular targeting, and the embracement of translational research.
journal_name
Curr Opin Pediatrjournal_title
Current opinion in pediatricsauthors
Cohn HI,Teng JMdoi
10.1097/MOP.0000000000000380subject
Has Abstractpub_date
2016-08-01 00:00:00pages
507-16issue
4eissn
1040-8703issn
1531-698Xpii
00008480-201608000-00017journal_volume
28pub_type
杂志文章,评审abstract:PURPOSE OF REVIEW:The purpose of this study is to review advances in both the pathogenesis and clinical management of biliary atresia. RECENT FINDINGS:Immunologic studies have further characterized roles of helper T-cells, B-cells, and natural killer cells in the immune dysregulation following viral replication within...
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::The importance of pediatric interhospital transport has increased dramatically in the past 5 to 10 years. Reasons include improved capabilities of tertiary care centers receiving transported patients, advances in availability of portable equipment that functions well in moving vehicles, and widespread recognition that...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199606000-00008
更新日期:1996-06-01 00:00:00
abstract:PURPOSE OF REVIEW:This update explores the current management options for adolescent obesity with a specific focus on bariatric surgery. RECENT FINDINGS:Research has highlighted the serious health complications associated with adolescent obesity and thus emphasized the need for effective interventions. With the increa...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000243
更新日期:2015-08-01 00:00:00
abstract:PURPOSE OF REVIEW:Dyskeratosis congenita, a rare condition characterized by mucocutaneous abnormalities and bone marrow failure, is caused by inherited defects in the telomerase complex. Autosomal dominant dyskeratosis congenita is associated with mutations in the RNA component of telomerase, hTERC, while X-linked dysk...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-200402000-00006
更新日期:2004-02-01 00:00:00
abstract:PURPOSE OF REVIEW:The small bowel has been a technically difficult area to examine. Indirect modalities such as barium follow-through have the disadvantage of attendant radiation exposure. Capsule endoscopy, a novel wireless method of investigation of the small bowel, has acquired an important role in both adult and pa...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e3282e2aaf8
更新日期:2007-10-01 00:00:00
abstract:PURPOSE OF REVIEW:The current article highlights recent developments in the field of pediatric cutaneous mastocytosis. Mastocytosis is a spectrum of conditions that range from fleetingly benign to aggressively malignant. Through recognizing the natural progression of disease, the role of biomarkers and mutational analy...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000922
更新日期:2020-08-01 00:00:00
abstract:PURPOSE OF REVIEW:Developmental speech and language disorders are common, seen in one in 20 preschool children, in the absence of frank neurological deficits or intellectual impairment. They are a key reason parents seek help from paediatricians. Complex neurogenetic and environmental contributions underpin the disorde...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000420
更新日期:2016-12-01 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:PURPOSE OF REVIEW:The purpose of this article is to present an overview of the state of breastfeeding in the United States and to examine recent evidence for strategies aimed at optimizing successful breastfeeding in the neonatal period. RECENT FINDINGS:Although rates of any and exclusive breastfeeding are rising, Ame...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e32832b325a
更新日期:2009-06-01 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:Adolescents, in particular those that are most disenfranchised, are increasingly at risk of acquiring HIV and, when acquiring HIV, have worse outcomes than adults. This article reviews the recent approaches to combination prevention aiming to optimize the HIV prevention and HIV treatment continua. RE...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000580
更新日期:2018-02-01 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::Two toddlers presented to their primary medical providers with anasarca and severe anemia. Laboratory evaluation revealed iron deficiency along with hypoproteinemia and hypoalbuminemia. Both children were diagnosed with iron deficiency anemia and were suspected to have an associated protein-losing enteropathy. A brief...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e32834dfc92
更新日期:2012-02-01 00:00:00
abstract:PURPOSE OF REVIEW:In fiscally challenging times, scientists must seek creative strategies and leverage existing resources to advance research. RECENT FINDINGS:This review describes programmes supported by the National Heart, Lung, and Blood Institute (NHLBI) of the National Institutes of Health (NIH) to promote resear...
journal_title:Current opinion in pediatrics
pub_type: 历史文章,杂志文章,评审
doi:10.1097/MOP.0b013e328364b8a7
更新日期:2013-10-01 00:00:00
abstract:PURPOSE OF REVIEW:To review the current evidence on the use of infection markers for diagnostic evaluation of sepsis in neonates. RECENT FINDINGS:Recent research in immunology has led to the discovery of cell surface antigens, chemokines, cytokines and acute phase proteins that can potentially be used to 'rule in' or ...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/01.mop.0000193293.87022.4c
更新日期:2006-04-01 00:00:00
abstract:PURPOSE OF REVIEW:To highlight recent advances in management of peanut allergy. RECENT FINDINGS:Peanut allergy presents during early childhood. The prevalence of peanut allergy in children in developed countries appears to be increasing. Several factors, such as peanut-specific or environmental, are hypothesized as co...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e32833d95cb
更新日期:2010-10-01 00:00:00
abstract:PURPOSE OF REVIEW:To illustrate potential risks and benefits of disease screening for newborns using the example of Duchenne muscular dystrophy. RECENT FINDINGS:There is a wide range in the reported positive predictive value for screening male newborns for Duchenne muscular dystrophy by the creatine kinase level on dr...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e3282f19f65
更新日期:2007-12-01 00:00:00
abstract:PURPOSE OF REVIEW:This review will outline the screening, diagnosis and management of cystic fibrosis related diabetes (CFRD). It will also discuss advances in the detection of early glucose abnormalities, their clinical significance and the emerging role for early insulin therapy. RECENT FINDINGS:Before the onset of ...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000251
更新日期:2015-08-01 00:00:00
abstract::Genetic causes account for 20% to 25% of human birth defects, but the largest proportion of birth defects have no definitive etiology and some of these malformations may be due to intrinsic, "nonpreventable" spontaneous errors of development. Environmental causes, which include maternal disease states, maternal infect...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199304000-00012
更新日期:1993-04-01 00:00:00
abstract::Studies using movement analysis confirm that multiple soft tissue releases improve function in the ambulant child with cerebral palsy, and highlight the fine adjustments of ankle-foot orthosis, which benefit children with myelomeningocele. For both of these disorders, there is information with regard to hip subluxatio...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199306000-00023
更新日期:1993-06-01 00:00:00
abstract::Progress in the pharmacotherapy of pediatric gastrointestinal diseases continued during 1998 despite ongoing obstacles encountered by clinicians and researchers. The major change involved warnings that cisapride, a widely used prokinetic agent, could cause potentially fatal arrythmias in susceptible people. The risk f...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199910000-00005
更新日期:1999-10-01 00:00:00
abstract::A number of growth factors have been identified that participate in lung growth and repair. The early stages of the repair cascade are important in preventing the later development of fibrosis. Both transforming growth factor-beta and basic fibroblast growth factor can have beneficial effects when given early in some ...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199506000-00002
更新日期:1995-06-01 00:00:00
abstract::In the past year, significant progress has been achieved in the research on aquaporins (AQPs), a family of structurally related molecular water channels. Three novel AQPs were identified, giving a total of ten mammalian AQPs. An important step forward in identifying the aqueous pore in AQP molecules was the determinat...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199808000-00018
更新日期:1998-08-01 00:00:00
abstract:PURPOSE OF REVIEW:The purpose of this review is to present an overview of sport-related concussion in the young athlete and review recent publications of clinical and scientific importance. RECENT FINDINGS:Recent findings show that young athletes are more susceptible to concussions than older athletes and more likely ...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/01.mop.0000236385.26284.ec
更新日期:2006-08-01 00:00:00
abstract:PURPOSE OF REVIEW:Complement mediated hemolytic uremic syndrome (aHUS) accounts for a significant proportion of non-shiga toxin HUS. The purpose of this review is to outline the pathophysiology, clinical features and therapeutic options for aHUS. RECENT FINDINGS:In the last decade, strides have been made in identifyin...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e32835df48a
更新日期:2013-04-01 00:00:00
abstract::This article reviews recent publications related to the epidemiology, pathogenesis, diagnosis, and management of otitis media. Otitis media is a continuum of conditions that includes acute otitis media, otitis media with residual or persistent effusion, unresponsive otitis media, recurrent otitis media, otitis media w...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199510000-00005
更新日期:1995-10-01 00:00:00
abstract:PURPOSE OF REVIEW:Recent literature regarding assessment, management and prevention of child maltreatment will be considered. Unexplained infant and child death will also be reviewed. RECENT FINDINGS:Controversies in the evaluation of unexpected infant death and inflicted traumatic brain injury are ongoing. Evidence c...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e32809f9543
更新日期:2007-04-01 00:00:00
abstract:PURPOSE OF REVIEW:We review recommendations from recent publications on the management of fever with antipyretics, the classification and diagnosis of fevers of unknown origin (FUO), and the evaluation of fever in infants under 90 days of age. RECENT FINDINGS:Anxiety about fever persists in the population, while the t...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e32835333e3
更新日期:2012-06-01 00:00:00
abstract::In the recent past, we have seen novel information on the bidirectional interactions between the endocrine and immune systems. Thus, the central nervous system, through the hypothalamic-pituitary-adrenal axis and the peripheral autonomic and sensory systems, influences the quantity and the quality of the immune and in...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199508000-00017
更新日期:1995-08-01 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