Abstract:
:Despite the availability of successful prevention strategies to prevent excessive hyperbilirubinemia, the neurological sequelae of bilirubin neurotoxicity (BNTx) still occur throughout the world. Kernicterus, encephalopathy due to BNTx, is now understood to be a spectrum of severity and phenotypes known as kernicterus spectrum disorder (KSD). A better understanding of the selective neuropathology and molecular biology of BNTx and using consistent clinical definitions of KSDs as outcome measure can lead to more accurately predicting the risk and causes of BNTx and KSDs. In Part I of our two-part review, we will summarize current and recent advances in the understanding of the selective neuropathology and molecular biology of the disease. Herein we emphasize the role of unbound, free unconjugated bilirubin as well as genetic contributions to the susceptibility BNTx and the development of KSDs. In Part II, we focus on current and possible novel methods to prevent BNTx and ABE and treat ABE and KSDs.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Riordan SM,Shapiro SMdoi
10.1038/s41390-019-0608-0subject
Has Abstractpub_date
2020-01-01 00:00:00pages
327-331issue
2eissn
0031-3998issn
1530-0447pii
10.1038/s41390-019-0608-0journal_volume
87pub_type
杂志文章,评审abstract::125I-Tyr insulin (125I-Tyr Ins) was injected into the vitelline vein of rat fetuses in utero after 17, 19, or 21 days of gestation. Three min later, the weight and radioactivity of various organs and of the remaining carcass were measured. A radioactivity concentration index (RCI) was calculated by dividing the specif...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198109000-00014
更新日期:1981-09-01 00:00:00
abstract::The copper concentrations in organs of developing Long-Evans Cinnamon (LEC) rats (2 d to 13 mo) were measured to elucidate the pathogenesis of their hereditary hepatitis. Hepatic copper contents of LEC rats were significantly higher than those of control rats (26 to 92 times higher). The subcellular distribution of he...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199203000-00011
更新日期:1992-03-01 00:00:00
abstract:BACKGROUND:Maternal smoking impairs fetal growth; however, if postnatal growth differs between children born small for gestational age (SGA) with smoking and non-smoking mother is unknown. METHODS:Cohort-study of term born children born appropriate for gestational age with non-smoking mother (AGA-NS, n = 30,561), SGA ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0352-5
更新日期:2019-06-01 00:00:00
abstract::Epidemiologic studies have linked intrauterine growth restriction (IUGR) with an increased incidence of cardiovascular disease later in life; reduced cardiomyocyte number in IUGR hearts may underlie such prenatal programming. Our aim was to examine the effect of IUGR, as a result of maternal protein restriction, on th...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000157726.65492.CD
更新日期:2005-06-01 00:00:00
abstract::The first days of postnatal life are energetically demanding as metabolic functions change dramatically to accommodate drastic environmental and physiologic transitions after birth. It is increasingly appreciated that metabolic pathways are not only crucial for nutrition but also play important roles in regulating inf...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/s41390-019-0647-6
更新日期:2020-01-01 00:00:00
abstract::The purpose of the study was to investigate the effect of low doses of dopamine in children. Fourteen cases were studied after open heart surgery. Cardiac output and renal parameters were determined under baseline conditions and under continuous infusion of dopamine 2.5 and 5 micrograms/kg/min. During the control peri...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198909000-00009
更新日期:1989-09-01 00:00:00
abstract::Functional disturbances in microcirculation in juvenile type 1 diabetes (T1D) are believed to underlie, in part, the later occurrence of cardiovascular complications. Some epidemiologic studies suggested greater risk of microvascular complications in those with T1D-risk genotypes of human leukocyte antigen (HLA). We i...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e318165bfd4
更新日期:2008-04-01 00:00:00
abstract::The gastrointestinal (GI) barrier function is immature in the preterm neonate and might thus facilitate translocation of enteric bacteria and gut-derived septicemia. Circumstantial evidence suggests that bacterial uptake from the intestine may be further enhanced by an alteration of the host nutritional status. To tes...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199603000-00007
更新日期:1996-03-01 00:00:00
abstract:BACKGROUND:A growing body of evidence defines inflammation as a hallmark feature of disease pathogenesis of Duchenne muscular dystrophy. To tailor potential immune modulatory interventions, a better understanding of immune dysregulation in Duchenne muscular dystrophy is needed. We now asked whether dystrophin deficienc...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0427-3
更新日期:2019-08-01 00:00:00
abstract::We evaluated the effectiveness of administration of high-dose busulfan (BU), an alkylating agent with substantial myeloablative but negligible immunosuppressive properties, on the engraftment of congenic normal bone marrow and spleen cells (hematopoietic cell transplantation; HCT) in murine galactosylceramidase defici...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199103000-00016
更新日期:1991-03-01 00:00:00
abstract:BACKGROUND:Short forms of IQ (S-IQ) assessments are time efficient and highly predictive of the full IQ (F-IQ) in healthy individuals. To investigate the validity of S-IQs for patients with neurodevelopmental impairments, this study tested a well-established S-IQ version in patients with congenital heart disease (CHD)....
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0667-2
更新日期:2020-04-01 00:00:00
abstract::BackgroundAcute kidney injury (AKI) is common in pediatric inpatients and is associated with increased morbidity, mortality, and length of stay. Its early identification can reduce severity.MethodsTo create and validate an electronic health record (EHR)-based AKI screening tool, we generated temporally distinct develo...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.116
更新日期:2017-09-01 00:00:00
abstract::Tyrosine (Tyr) phosphorylation is implicated in the modification of several erythrocyte functions, such as metabolic pathways and membrane transport, as well as in signal transduction systems. Here we describe the map of Tyr-phosphorylated soluble proteins of newborn red blood cells (RBC) using an in vitro model simul...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000180545.24457.AC
更新日期:2005-10-01 00:00:00
abstract:BACKGROUND:Kawasaki disease (KD) is associated with expression and methylation of Fc gamma receptor genes. We characterized immunoglobulin A (IgA), IgE, IgG, and IgM receptor expression levels in KD. METHODS:Fc receptor expression levels were characterized using GeneChip Human Transcriptome Array 2.0 (HTA 2.0) with 18...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0707-y
更新日期:2019-12-09 00:00:00
abstract::Based on the serum phenylalanine levels under free diet patients with hyperphenylalaninemia are classified as "classical" (>1200 micromol/L), "mild" (600-1200 micromol/L), or "non-phenylketonuria (PKU)-hyperphenylalaninemia" (<600 micromol/L). Recent studies revealed intellectual, neurologic, and neuropsychologic defi...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199709000-00020
更新日期:1997-09-01 00:00:00
abstract::The effect of ingestion of uncooked cornstarch (2 g/kg body weight) in water, uncooked starch (1 g/kg) added to a meal, and glucose (2 g/kg) in water, was studied in eight patients with type IA glycogen storage disease (GSD) and one patient with type IB GSD. Blood glucose concentrations were determined at 30-min inter...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198409000-00015
更新日期:1984-09-01 00:00:00
abstract::Prenatal maternal therapy with glucocorticoid reduces the incidence of respiratory distress syndrome (RDS) in premature infants. To investigate the effects of this treatment on the fetal endocrine system, we determined serum concentrations of betamethasone, cortisol, dehydroepiandrosterone sulfate, growth hormone, and...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198002000-00011
更新日期:1980-02-01 00:00:00
abstract::Three members of a Virginia family with a bleeding disorder were found to have a serum defect in thromboplastin generation similar to the previously reported "Car. factor" deficiency. Sera from three members of the original Car. family did not correct the defect of affected members of the Virginia family. Partial thro...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/00006450-197504000-00009
更新日期:1975-04-01 00:00:00
abstract:BACKGROUND:Later life metabolic dysfunction is a well-recognized consequence of being born small for gestational age (SGA). This study has applied metabolomics to identify whether there are changes in these pathways in prepubertal short SGA children and aimed to compare the intracellular and extracellular metabolome in...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2016.72
更新日期:2016-08-01 00:00:00
abstract::The major variation in effect between undiluted cystic fibrosis and heterozygote sera on rabbit tracheal epithelium was in terms of the time after exposure at which changes occurred. Exposure to both types of sera for 3 min caused release of goblet cell mucus and ciliary resorption possibly by apical swelling of colum...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198011000-00005
更新日期:1980-11-01 00:00:00
abstract::Delivery of M. nemestrina at 80% of normal gestation provides a population of neonates at high risk for hyaline membrane disease (HMD). The diagnosis of HMD was made by the presence of reticulogranular densities and air bronchograms on chest radiographs. Patchy atelectasis was seen in the lungs of animals assigned by ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197905000-00017
更新日期:1979-05-01 00:00:00
abstract::Serum copper (Cu) and zinc (Zn) concentrations were measured in neonates with an appropriate birth weight for gestational age (AGA) and in small-for-gestational age infants. At 7 days of age, there was a positive correlation between serum Cu concentration and gestational age (GA) (r = 0.63; P < 0.001) and a negative c...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198009000-00005
更新日期:1980-09-01 00:00:00
abstract::Astrocyte end-feet ensheathe blood vessels in the brain and are believed to provide structural integrity to the cerebral vasculature. We sought to determine in developing infants whether the coverage of blood vessels by astrocyte end-feet is decreased in germinal matrix (GM) compared with cerebral cortex and white mat...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000214975.85311.9c
更新日期:2006-05-01 00:00:00
abstract:BACKGROUND:Volatile organic compounds (VOCs) are hydrocarbons that originate within different healthy and diseased tissues. VOCs can be secreted into the circulation and then excreted in the urine and faeces. In the lungs, VOCs are locally produced and can be detected in exhaled breath. VOCs can be identified using non...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/s41390-020-0828-3
更新日期:2020-03-02 00:00:00
abstract::Iron is essential for fetal organ development, but the effect of isolated iron deficiency on nephrogenesis is unknown. Human premature infants are at risk for disrupted nephrogenesis because glomerular development is incomplete until 36-wk gestation. We modeled the effects of iron on postnatal glomerulogenesis in four...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181be79c2
更新日期:2009-12-01 00:00:00
abstract::Gastrointestinal (GI) blood flow, O2 transport, and O2 uptake were measured during recovery from severe hypoxemia in newborn piglets. Hypoxemia was induced by lowering the inspired O2 concentration to 0.05 for 15 min. This resulted in an 82% decrease in GI O2 uptake. Recovery measurements were obtained 5 and 65 min af...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198511000-00017
更新日期:1985-11-01 00:00:00
abstract::A double-label, double-substrate incubation technique has been developed and used to study the conversion of progesterone to testosterone in testes extracts from incompletely virilized males. The procedure involves separation of the microsomes from a testicular homogenate, incubating the microsomes with 1 microM [7-3H...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198408000-00018
更新日期:1984-08-01 00:00:00
abstract::To investigate the detailed pattern of change in circulating gonadotropin and testosterone concentrations around the onset of puberty and to determine the temporal relationship between the gonadotropin and testosterone secretion, plasma gonadotropin and testosterone were measured at 20-min intervals for 24 h in 21 nor...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199308000-00026
更新日期:1993-08-01 00:00:00
abstract::The interactions between CD40 and CD40L (CD154) are critical for effective humoral immune response. CD40 signaling facilitates T lymphocyte dependent B cell proliferation and immunoglobulin isotype switch. The objective of our study was to investigate the CD40 and CD40L expression on peripheral blood mononuclear cells...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000219298.96471.18
更新日期:2006-06-01 00:00:00
abstract::The development of the intestinal mucosal immune barrier is an important protective adaptation for postnatal life. The distribution and phenotype of T and B lymphocytes in human fetal intestine and lymphoid tissues have been characterized and compared to the distribution and phenotype of lymphocytes in postnatal intes...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199003000-00007
更新日期:1990-03-01 00:00:00