Abstract:
:Autoimmune Lymphoproliferative Syndrome (ALPS) is a recently recognized disease in which a genetic defect in programmed cell death, or apoptosis, leads to breakdown of lymphocyte homeostasis and normal immunologic tolerance. Some authors have referred to ALPS as Canale-Smith syndrome or lymphoproliferative syndrome with autoimmunity. Patients with ALPS have chronic enlargement of the spleen and lymph nodes, various manifestations of autoimmunity, and elevation of a normally rare population of "double negative T cells" (DNTs), T lymphocytes bearing alpha beta T cell receptors and expressing neither cluster differentiation (CD)4 nor CD8 surface antigens. When lymphocytes from patients with ALPS are cultured in vitro, they are resistant to apoptosis as compared to cells from healthy controls. Most patients with ALPS have mutations in a gene now named TNFRSF6 (tumor necrosis factor receptor gene superfamily member 6). This gene, previously known as apoptosis antigen 1 (APT1), encodes the cell surface receptor for the major apoptosis pathway in mature lymphocytes; this receptor has also had many names, including Fas (to be used here), CD95, and APO-1. ALPS is subdivided into: 1) Type Ia, ALPS with mutant Fas; 2) Type Ib, lymphadenopathy and mutation in the ligand for Fas in one patient with systemic lupus erythematosus; 3) Type II, ALPS with mutant caspase 10; and 4) Type III, ALPS as yet without any defined genetic cause.
journal_name
Curr Opin Pediatrjournal_title
Current opinion in pediatricsauthors
Jackson CE,Puck JMdoi
10.1097/00008480-199912000-00009keywords:
subject
Has Abstractpub_date
1999-12-01 00:00:00pages
521-7issue
6eissn
1040-8703issn
1531-698Xjournal_volume
11pub_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::Until recently, tetanus was estimated to be killing well over half a million children each year in the developing world, most commonly through neonatal tetanus, the incidence of which was around six per 1000 live births. Neonatal tetanus most commonly occurs through cord contamination of the umbilical stump. Vaccinati...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199802000-00002
更新日期:1998-02-01 00:00:00
abstract:PURPOSE OF REVIEW:Describe developments in the etiological understanding of Tourette syndrome. RECENT FINDINGS:Tourette syndrome is a complex heterogenous clinical syndrome, which is not a unitary entity. Pathophysiological models describe gamma-aminobutyric acid-ergic-associated disinhibition of cortico-basal ganglia...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000546
更新日期:2017-12-01 00:00:00
abstract:PURPOSE OF REVIEW:Physical activity has been highlighted internationally as a beneficial intervention for weight control and the improvement of physical and mental health. This review highlights findings from recent literature to guide office-based promotion of physical activity for obesity treatment and prevention. R...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e328013c88c
更新日期:2007-02-01 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::Herbal medicaments are in common use. In general, the judicious use of carefully selected and prepared herbal medications seems to cause few adverse effects and may be beneficial. However, toxic effects of these products have been reported with increasing frequency. Infants and children may be even more susceptible to...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-200104000-00014
更新日期:2001-04-01 00:00:00
abstract::Neonatal infections range from early-onset vertically acquired infections to nosocomial bacterial sepsis, and they account for significant morbidity and mortality globally. The recent emergence of perinatally acquired HIV-1 infection has also placed an enormous burden on existing meager health resources in developing ...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199704000-00003
更新日期:1997-04-01 00:00:00
abstract:PURPOSE OF REVIEW:The delineation of syndromes carrying a predisposition to malignancy has led to great insights into the molecular biology of malignancy. Many such syndromes have cutaneous findings which can precede the development of neoplasia. Early recognition of the cutaneous stigmata of the genodermatoses with ma...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e3282495939
更新日期:2007-08-01 00:00:00
abstract:PURPOSE OF REVIEW:Chronic disease management is an increasingly important aspect of caring for adolescents. Strength-based approaches hold tremendous promise in the face of current challenges. Recent clinical innovations and research findings that provide evidence of the efficacy of strength-based approaches and repres...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e32833a468e
更新日期:2010-08-01 00:00:00
abstract::Treatment of patients with anorexia nervosa is a topic that continues to spark debate. Two particular areas of controversy include the role of exercise and the use of selective serotonergic reuptake inhibitors (SSRI). Few randomized controlled trials have addressed these two issues. The psychological impact of exercis...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-200306000-00022
更新日期:2003-06-01 00:00:00
abstract:PURPOSE OF REVIEW:The right ventricle (RV) is uniquely at risk in many patients with repaired or palliated congenital heart disease (CHD) such as tetralogy of Fallot, corrected transposition, single right ventricle, and in those with pulmonary hypertension. These patients live with abnormal cardiac loading conditions t...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000268
更新日期:2015-10-01 00:00:00
abstract:PURPOSE OF REVIEW:Advances in the field of pediatric nutrition continue to help the pediatric office practitioner provide excellent clinical care to their patients. In 2003, several important papers were published in the field of clinical nutrition that are reviewed. RECENT FINDINGS:New recommendations by the American...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/01.mop.0000125070.51974.9d
更新日期:2004-06-01 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::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:The diagnostic approach to children with possible appendicitis remains challenging. Although advances in imaging have improved diagnostic accuracy over clinical judgment alone, new management strategies continue to evolve. This review serves as an update on imaging, biomarkers, and management of appen...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e32834604da
更新日期:2011-06-01 00:00:00
abstract::Atopic dermatitis is the most common chronic skin disease of young children and is frequently associated with asthma and allergies. Th2-type cytokine secreting T cells expressing the cutaneous lymphocyte-associated antigen play a central role in the induction of local IgE responses and recruitment of eosinophils in th...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199712000-00005
更新日期:1997-12-01 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:PURPOSE OF REVIEW:In September 2019, the United States was at risk of losing measles elimination status due to several large-scale outbreaks resulting in more than 1200 confirmed cases across 31 states. This resurgence caps approximately 10 years of increasing incidence, marked by a highly publicized outbreak in 2015 a...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000845
更新日期:2020-02-01 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:The management of displaced medial humeral epicondyle fractures in children remains controversial. The indications for surgery, the ideal surgical strategy and the implications of a painful nonunion remain unclear. RECENT FINDINGS:This article describes the state of the evidence and the art in the ma...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000707
更新日期:2019-02-01 00:00:00
abstract::The use of extracorporeal techniques for the treatment of pediatric diseases has expanded dramatically in the past decade. During this time, experience with both plasmapheresis and renal replacement therapy has become commonplace in ICUs and nephrology programs throughout the world. The indications for these therapies...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-200206000-00005
更新日期:2002-06-01 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::In the past decade a number of studies have systematically addressed the questions that existed regarding optimal ways to rehydrate and then refeed infants and children with diarrhea. Two very thorough papers are the highlights of the past year's publications in the topic of acute gastroenteritis in children. One summ...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/00008480-199710000-00010
更新日期:1997-10-01 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::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:The clinical importance of overgrowth syndromes in the pediatric patient population has been increasingly recognized during the past decade, but clinical overlap among overgrowth syndromes often makes diagnostic categorization difficult. Advances in the molecular delineation of overgrowth syndromes in...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/01.mop.0000187191.74295.97
更新日期:2005-12-01 00:00:00
abstract:PURPOSE OF REVIEW:This review will be covering dysfunctional voiding, its diagnosis, and treatment options. This will focus primarily on dysfunctional voiding rather than all lower urinary tract dysfunction and we will focus on some of the newer findings and progress within this disease. RECENT FINDINGS:Dysfunctional ...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章
doi:10.1097/MOP.0000000000000980
更新日期:2020-12-10 00:00:00
abstract:PURPOSE OF REVIEW:This paper reviews recent publications about the physiology associated with adaptation to malnutrition and refeeding (including the refeeding syndrome) and clinical outcomes of refeeding paradigms. RECENT FINDINGS:A number of recent reviews and original publications have highlighted important differe...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e3283487591
更新日期:2011-08-01 00:00:00
abstract:PURPOSE OF REVIEW:To summarize the recommended work-up in a child who presents with elevated blood pressure as well as innovative evaluation techniques under development. RECENT FINDINGS:'The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents' contains several ...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0b013e328013f647
更新日期:2007-04-01 00:00:00
abstract:PURPOSE OF REVIEW:The current review addresses the importance of the prospective cohort design in large, unselected populations starting early in life for understanding the origins of childhood health disorders. RECENT FINDINGS:Cohort studies originating in healthy populations have contributed to great advances in hea...
journal_title:Current opinion in pediatrics
pub_type: 杂志文章,评审
doi:10.1097/MOP.0000000000000596
更新日期:2018-04-01 00:00:00