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 several associated syndromes. Molecular diagnosis allows for prediction of the effectiveness of diazoxide and the likelihood of focal hyperinsulinism. Inactivating mutations in the genes encoding the ATP-sensitive potassium channel (KATP hyperinsulinism) account for 60% of all identifiable mutations, including 85% of diazoxide-unresponsive cases. Syndromes or disorders associated with hyperinsulinism include Beckwith-Wiedemann syndrome, Kabuki syndrome, Turner syndrome, and congenital disorders of glycosylation. Although focal hyperinsulinism can be cured by resection of the lesion, therapeutic options for nonfocal hyperinsulinism remain limited and include diazoxide, octreotide, long-acting somatostatin analogs, and near-total pancreatectomy. Although sirolimus has been reported to improve glycemic control in infants with diazoxide-unresponsive hyperinsulinism, the extent of improvement has been limited, and significant adverse events have been reported. SUMMARY:Identification of the cause of congenital hyperinsulinism helps guide management decisions. Use of therapies with limited benefit and significant potential risks should be avoided.
journal_name
Curr Opin Pediatrjournal_title
Current opinion in pediatricsauthors
Vajravelu ME,De León DDdoi
10.1097/MOP.0000000000000645subject
Has Abstractpub_date
2018-08-01 00:00:00pages
568-575issue
4eissn
1040-8703issn
1531-698Xjournal_volume
30pub_type
杂志文章,评审abstract::Surfactant therapy has revolutionized neonatal care and is used routinely for preterm infants with respiratory distress syndrome. Recent investigation has further elucidated the function of surfactant-associated proteins and their contribution toward surfactant and lung immune defense functions. As the field of neonat...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-200304000-00002
更新日期:2003-04-01 00:00:00
abstract::Bone marrow transplantation has become well established in the treatment of malignant and nonmalignant disorders. Steady progress continues in improving survival after transplantation, but many problems remain to be solved. Relapsed disease is a major limitation, and new, more intensive preparative regimens are being ...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199302000-00016
更新日期:1993-02-01 00:00:00
abstract::Miniaturization of echocardiographic transducers, improved crystal technology, and enhanced digital processing of ultrasound signals have resulted in newer, more sophisticated equipment and imaging techniques. These technologic advances include transesophageal echocardiography, stress echocardiography (both pharmacolo...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199510000-00015
更新日期:1995-10-01 00:00:00
abstract::Complete deficiency of one of the early components (C1, C4, or C2) of the classical pathway of the complement cascade is one of the strongest genetic risk factors for systemic lupus erythematosus that has been recognized. The lupus that occurs in complement-deficient individuals typically presents in early childhood. ...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199810060-00011
更新日期:1998-12-01 00:00:00
abstract::von Willebrand disease (VWD) is a bleeding disorder caused by quantitative (type 1 and 3) or qualitative (type 2) defects of von Willebrand factor (VWF). The mechanisms of most inherited VWD types have been recently elucidated by genetic and molecular diagnosis, but the phenotypic tests based on measurements of plasma...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-200202000-00005
更新日期:2002-02-01 00:00:00
abstract:PURPOSE OF REVIEW:Screening, brief intervention and referral to treatment (SBIRT) offers a practical, integrated model for addressing substance use in primary care settings. This review provides an update of the research on SBIRT for adolescents in primary care, examines current dissemination challenges and suggests fu...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000371
更新日期:2016-08-01 00:00:00
abstract:PURPOSE OF REVIEW:Abdominal wall defects comprising both gastroschisis and omphalocele remain a source of significant morbidity and mortality, despite the advances in neonatal and pediatric surgical care. Survival has improved over the past few decades, especially with parenteral nutrition and surgical repair. Yet, sti...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e3282ffdc1e
更新日期:2008-06-01 00:00:00
abstract:PURPOSE OF REVIEW:Sepsis is the leading cause of pediatric death worldwide. In the United States alone, there are 72 000 children hospitalized for sepsis annually with a reported mortality rate of 25% and an economic cost estimated to be $4.8 billion. However, it is only recently that the definition and management of p...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000337
更新日期:2016-06-01 00:00:00
abstract:PURPOSE OF REVIEW:Children with chronic kidney disease (CKD) have impaired growth that leads to short stature in adulthood. The problem persists even with successful transplantation and steroid withdrawal protocols. The aim of this review is to provide an overview of the pressing issues related to growth failure in chi...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000070
更新日期:2014-04-01 00:00:00
abstract::Major advances in the genetic understanding of the limb-girdle (LGMD) and congenital (CMD) muscular dystrophies have led to a new, genetically based classification of these disorders. The definition of the complex of dystrophin-associated proteins on a biochemical and subsequently genetic level has greatly accelerated...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:
更新日期:1996-12-01 00:00:00
abstract::Medical informatics is both a science, which seeks to understand the information requirements of medical practice, research, and education, and an engineering discipline, which applies new technologies to help manage the explosion of data and new knowledge that confront contemporary physicians. Computerization of the ...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:
更新日期:1994-10-01 00:00:00
abstract:PURPOSE OF REVIEW:This study will outline recent advances in echocardiography and their impact in the understanding of cardiac morphology and ventricular function. RECENT FINDINGS:Evaluation of morphologic abnormalities in pediatric cardiology has benefited greatly from three-dimensional echocardiography, particularly...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e32832ff38e
更新日期:2009-10-01 00:00:00
abstract::Risk factors for infant development are considered in three areas: infant temperament, postpartum depression, and prenatal cocaine exposure. Investigations of all three areas indicate that contextual characteristics of infant development must be incorporated into models attempting to link early risk factors with later...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199408000-00009
更新日期:1994-08-01 00:00:00
abstract::Bacterial meningitis is an important problem in childhood. Vaccines can prevent Haemophilus influenzae type b disease and are being developed for infections caused by meningococci and pneumococci. Lumbar puncture is an important part of the diagnostic workup although care is required with its use. Once meningitis is d...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199402000-00006
更新日期:1994-02-01 00:00:00
abstract:PURPOSE OF REVIEW:Enhanced recovery protocols (ERPs) have been adopted for a variety of adult surgical conditions and resulted in markedly improved outcomes, including decreased length of stays, complications, costs, and narcotic utilization. In this review, we describe the development and implementation of an ERP for ...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000622
更新日期:2018-06-01 00:00:00
abstract::Disruptive behavior disorders present grave complications to normal childhood development. Their presence is frequently associated with additional psychiatric disorders. The hallmark features of the disruptive behavior disorders range from noncompliance to aggressive hostility toward authorities or peers. Oppositional...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199708000-00007
更新日期:1997-08-01 00:00:00
abstract:PURPOSE OF REVIEW:Continuous glucose monitoring (CGM) is increasingly used in the management of diabetes in children and adults, but there are few data regarding its use in neonates. The purpose of this article is to discuss the potential benefits and limitations of CGM in neonates. RECENT FINDINGS:Smaller electrodes ...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000592
更新日期:2018-04-01 00:00:00
abstract:PURPOSE OF REVIEW:This article evaluates the field of radiogenomics within recent developments in genomics and radiation biology. RECENT FINDINGS:Many pediatric cancer survivors have undergone treatment with radiation, putting them at risk for long-term side-effects associated with this therapy, especially cardiac dis...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000408
更新日期:2016-12-01 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:Rotavirus infection is the most common cause of severe diarrhea disease in infants and young children worldwide and has a major global impact on childhood morbidity and mortality. Vaccination is the only control measure likely to have a significant impact on the incidence of severe dehydrating rotavir...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/01.mop.0000147907.30720.04
更新日期:2005-02-01 00:00:00
abstract::The literature published in the past year on fractures in children has helped us to increase our understanding of the natural history of fracture healing in children and introduced new techniques for fracture treatment. There is an impetus for operative fixation of femur fractures in children. The possibility is raise...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199402000-00015
更新日期:1994-02-01 00:00:00
abstract::Over the past two decades, pharmacokinetic data have clearly demonstrated that development can markedly influence the absorption, distribution, excretion, and metabolism of xenobiotics. With respect to many of the processes that govern drug metabolism, the underlying pharmacogenetic determinants that may control eithe...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:
更新日期:1995-04-01 00:00:00
abstract::Inhaled corticosteroids (ICS) have become the mainstay of therapy in chronic childhood asthma. Despite the long history and the documented efficacy of these drugs in controlling asthma, concerns still abound regarding the safety of these drugs in children, most specifically related to the potential for adrenal suppres...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-200112000-00005
更新日期:2001-12-01 00:00:00
abstract:PURPOSE OF REVIEW:Lyme disease, caused by Borrelia burgdorferi, is the most common vector-borne illness in the United States. The pathogenesis, ecology, and epidemiology of Lyme disease have been well described, and antimicrobial treatment is very effective. There has been controversy about whether infection can persis...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000167
更新日期:2015-02-01 00:00:00
abstract::Acute bacterial rhinosinusitis is an infection of the nasal epithelium and paranasal sinus mucosa, usually caused in children by Streptococcus pneumoniae, Haemophilus influenzae, Moraxella catarrhalis, and, less frequently, group A Streptococcus species. The clinical diagnosis is based on daytime cough that may be wor...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-200202000-00015
更新日期:2002-02-01 00:00:00
abstract:PURPOSE OF REVIEW:The mid-gestation fetus is capable of regenerative healing with wound healing indistinguishable from surrounding skin. This review aims to evaluate the current knowledge of how the mid-gestation fetus heals without scar and the implications of these findings in efforts to recapitulate the fetal regene...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e3283535790
更新日期:2012-06-01 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:PURPOSE OF REVIEW:The term 'dual diagnosis' most commonly refers to the combination of severe mental illness and substance-use disorder (SUD). It is estimated that 7-10 million people in the USA alone have at a minimum one psychiatric disorder in addition to a SUD. As many of the psychiatric illnesses implicated have t...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e3283504fbf
更新日期:2012-04-01 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:Inherited disorders of calcium and phosphate homeostasis have variable presentation and can cause significant morbidity. An understanding of the mode of inheritance and pathophysiology of these conditions will help in the diagnosis and early institution of therapy. RECENT FINDINGS:Identification of g...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章
doi:10.1097/MOP.0000000000000064
更新日期:2014-04-01 00:00:00