Abstract:
:To determine the importance of peroxisomes and mitochondria in hydroxyeicosatetraenoic acid (HETE) oxidation in vivo, urinary excretion of 12- and 15-HETE was measured in eight patients with a peroxisome deficiency disorder (Zellweger syndrome) showing normal mitochondrial beta-oxidation capacity, in three patients with a defect of mitochondrial long-chain fatty acid oxidation (long-chain acyl-CoA dehydrogenase deficiency), and in eight healthy subjects. 12- and 15-HETE were identified and quantified by gas chromatography/negative ion chemical ionization-mass spectrometry and specific RIA. The free compounds were found exclusively in the urine of peroxisome-deficient subjects (12-HETE: median 26 pg/mL, range 17-36 pg/mL; 15-HETE: median 40 pg/mL, range 29-61 pg/mL), whereas both compounds were below the detection limit (< 0.5 pg/mL) in the urine of patients with defective mitochondrial long-chain fatty acid oxidation and normal subjects (p < 0.002). These results implicate that peroxisomes are the main cellular organelle responsible for HETE oxidation in vivo. Analysis of HETE excretion in urine represents an additional new specific diagnostic tool in patients with Zellweger syndrome.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Mayatepek E,Lehmann WDdoi
10.1203/00006450-199601000-00022subject
Has Abstractpub_date
1996-01-01 00:00:00pages
146-9issue
1eissn
0031-3998issn
1530-0447journal_volume
39pub_type
杂志文章abstract::To delineate the role of growth hormone (GH) in the development and function of the immune system, immunological parameters including quantitative immunoglobulins, T and B lymphocytes, phytohemagglutinin lymphoproliferative response and delayed hypersensitivity skin tests were studied in nine GH-deficient children pri...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198503000-00009
更新日期:1985-03-01 00:00:00
abstract:BACKGROUND:Kawasaki disease (KD) is the most common systemic vasculitis syndrome primarily affecting medium-sized arteries, particularly the coronary arteries. Though KD may be associated with immunological problems, the involvement of microRNAs (miRs) has not been fully described. METHODS:We enrolled 23 KD patients a...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2016.93
更新日期:2016-09-01 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 relationship between cerebral blood flow (CBF) and the evolution of brain edema was investigated in an experimental model of perinatal hypoxic-ischemic brain damage. Seven-d postnatal rats were subjected to unilateral common carotid artery ligation followed by 3 h of hypoxia with 8% oxygen at 37 degrees C. This in...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199005000-00007
更新日期:1990-05-01 00:00:00
abstract::The aim of this study was to compare catecholamine concentrations in the fetal umbilical artery cord blood from the offspring of smokers versus the offspring of nonsmokers. Pregnant women who were self-identified as smokers (>/=10 cigarettes per day throughout pregnancy) or nonsmokers were recruited for study particip...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200301000-00020
更新日期:2003-01-01 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::We hypothesized that cardiac and respiratory modulation of postganglionic peroneal activity appeared in an age-related manner. In anesthetized, paralyzed and artificially ventilated piglets, simultaneous recordings of efferent phrenic and peroneal discharges were obtained during hyperoxia (fraction of inspired oxygen,...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199601000-00012
更新日期:1996-01-01 00:00:00
abstract::Uteroplacental insufficiency increases the risk of perinatal and long-term neurologic morbidity by depriving the fetus of oxidative substrate and causing intrauterine growth retardation. Skeletal muscle and liver from growth retarded fetal and juvenile rats respond to this deprivation by altering mitochondrial gene ex...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200006000-00019
更新日期:2000-06-01 00:00:00
abstract::The pathophysiology of kwashiorkor, a severe edematous manifestation of malnutrition, is still poorly understood. The syndrome is, however, known to be associated with alterations in redox metabolism. To further elucidate the role of oxidative stress in kwashiorkor, we carried out a longitudinal study on the major blo...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200102000-00018
更新日期:2001-02-01 00:00:00
abstract::Values of IGF-I after extraction, its binding proteins, and the high affinity GH-binding protein (BP) are not well established in pediatric patients with insulin-dependent diabetes mellitus (IDDM). We report data for IGF-I, IGFBP-1, and -3, and GHBP in 92 Spanish children with IDDM, separated according to pubertal sta...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199606000-00011
更新日期:1996-06-01 00:00:00
abstract::We have developed a method for measuring oxygen consumption (Vo2) in preterm infants in their normal incubator environment over prolonged periods. The results of measurements made over 24 h in 18 infants are presented. In normally grown infants, the mean Vo2 was 9.66 +/- 1.25 liters/kg X 24 h (SD) (6.71 +/- 0.87 ml/kg...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198410000-00003
更新日期:1984-10-01 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:The epithelial-mesenchymal transition (EMT) has been implicated as a key mechanism in the pathogenesis of liver fibrosis. The miR-200 family has been shown to inhibit EMT. METHODS:Liver fibrosis levels were assessed with Masson's trichrome staining of liver samples obtained from biliary atresia (BA) patient...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2014.181
更新日期:2015-02-01 00:00:00
abstract::We examined the long-term effects of dietary protein restriction during rat pregnancy on serum IGF-I, serum IGF binding proteins, and liver IGF-I gene expression during postnatal development. Pregnant Wistar rats were fed ad libitum throughout gestation a normal (20% casein diet; P20 controls) or a low (5% casein; P5)...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199604000-00015
更新日期:1996-04-01 00:00:00
abstract::Clinical trials demonstrated decreasing rates of bronchopulmonary dysplasia in preterm infants with hypoxic respiratory failure treated with inhaled nitric oxide (iNO). However, the molecular and biochemical effects of iNO on developing human fetal lungs remain vastly unknown. By using a well-characterized model of hu...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181d4f20f
更新日期:2010-05-01 00:00:00
abstract::Several studies analyzing the immune responses in patients with cow's milk allergy (CMA) have used T-cell lines or T-cell clones that require prolonged in vitro cell culturing and may result in a switched cell phenotype and function. We investigated immune responses to beta-lactoglobulin (b-LG) in peripheral blood mon...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e318232739c
更新日期:2011-12-01 00:00:00
abstract::The purpose of this study was to investigate atrial myocardial blood flow in the fetus under conditions of acute right ventricular pressure load and adenosine infusion. Late gestation fetal sheep were instrumented for acute right ventricular pressure loading or adenosine infusion, and regional myocardial blood flow wa...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199403000-00010
更新日期:1994-03-01 00:00:00
abstract:BACKGROUND:Gender is increasingly recognized as an important influence on brain development, disease susceptibility, and response to pharmacologic/rehabilitative treatments. In regenerative medicine, it remains entirely unknown whether there is an interaction between transplanted stem cells and host gender that might b...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2014.7
更新日期:2014-05-01 00:00:00
abstract::Necrotizing enterocolitis (NEC) is one of the leading causes of death in neonatal intensive care units. The underlying pathophysiology of NEC is poorly defined, although there is a suggestion that bacterial agents play an important role in the process. In this study, we evaluated bacterial isolates from 17 NEC cases a...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/00006450-199407001-00021
更新日期:1994-07-01 00:00:00
abstract::Opsonisation of heat-killed bakers yeast and C3 and C4 concentrations were determined in sera from 69 children with liver disease and 39 controls as simple screening procedures for abnormalities in the complement system. Where significant defects in these moieties were encountered, the activity of the following was me...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198304000-00014
更新日期:1983-04-01 00:00:00
abstract:BACKGROUND:Appearance of sleep cycling has been associated with good outcome in term and preterm infants, but the normal time of its appearance has not been determined. The objectives of this study were, to correlate the time of sleep cycling appearance and the length of quiet sleep in neonates with different degrees o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0560-z
更新日期:2020-03-01 00:00:00
abstract::BackgroundCaffeine promotes spontaneous breathing by antagonizing adenosine. We assessed the direct effect of caffeine on respiratory effort in preterm infants at birth.MethodsThirty infants of 24-30 weeks of gestation were randomized for receiving caffeine directly after birth in the delivery room (caffeine DR group)...
journal_title:Pediatric research
pub_type: 杂志文章,随机对照试验
doi:10.1038/pr.2017.45
更新日期:2017-08-01 00:00:00
abstract::Preterm infants are prone to abnormal bacterial colonization of the intestine with ensuing adverse health effects. To examine whether the oral application of Bifidobacterium lactis Bb12 (probiotic) may improve selected indicators of health status in preterm infants, a double blind, placebo controlled randomized clinic...
journal_title:Pediatric research
pub_type: 杂志文章,随机对照试验
doi:10.1203/PDR.0b013e318181b7fa
更新日期:2008-10-01 00:00:00
abstract::Changes in high-density lipoprotein (HDL) subclass distribution were evaluated in a group of premature infants during the early postnatal period to ascertain whether enteral feeding brought about a rapid shift from neonatal to adult-like distributions. All infants were fed a combination of breast milk and formula. Cor...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198806000-00001
更新日期:1988-06-01 00:00:00
abstract::The purpose of this study was to investigate the effect of blue light phototherapy on the expression of circadian genes in peripheral blood mononuclear cells (PBMC) and plasma melatonin levels in neonates. Real-time reverse-transcriptase polymerase chain reaction (RT-PCR) was used to determine the expression of Bmal1 ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000183663.98446.05
更新日期:2005-12-01 00:00:00
abstract::Infection with human immunodeficiency virus (HIV) causes a progressive immunodeficiency, often manifested in adults by the development of infections with relatively rare, opportunistic organisms. However, in HIV-infected children, recurrent serious infections with common encapsulated bacteria, as well as recurrent min...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验,评审
doi:10.1203/00006450-199305001-00464
更新日期:1993-01-01 00:00:00
abstract::The use of erythrocyte entrapment as a strategy to deliver and protect exogenously administered enzymes for replacement therapy in selected genetic diseases has been evaluated in a mammalian model system. The uptake, tissue distribution, intracellular localization, and in vivo lifetime of erythrocyte-entrapped bovine ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197512000-00011
更新日期:1975-12-01 00:00:00
abstract::The etiology of biliary atresia (BA) is not yet understood, but recent studies have shown inflammation with an up-regulated interferon (IFN) activity in the intra- and extrahepatic bile ducts of patients with BA. These findings support an inflammatory/infectious cause of BA as mimicked in our infective murine model. T...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000219860.96732.09
更新日期:2006-06-01 00:00:00
abstract::Inhibition of Pseudomonas cepacia (but not Pseudomonas aeruginosa) by beta-lactams was decreased in 5% CO2 in air compared with air alone. The effect of CO2 and pH (range, 6.0 to 8.0) on beta-lactam susceptibility, beta-lactamase expression, and outer membrane proteins was studied in isolates recovered from the sputum...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199403000-00003
更新日期:1994-03-01 00:00:00
abstract::Several neuropathologic findings in infants and children with human immunodeficiency virus type-1 (HIV-1) infection are different from those observed in adults, probably related to the fact that the retroviral infection occurs in the setting of neuro-development. This report describes the interaction and biologic acti...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199511000-00025
更新日期:1995-11-01 00:00:00