Abstract:
:Infantile Refsum's disease is characterized by high levels of phytanic acid and the absence of normal hepatic peroxisomes. We investigated the in vivo influence of phytol, a precursor of phytanic acid, on peroxisomes by both biochemical and morphological methods. Enhanced supply of phytol in the diet of adult mice causes proliferation of hepatic peroxisomes. The peroxisomal beta-oxidizing capacity as well as exchanges of acyl moieties between peroxisomes and mitochondria are raised around 5- and 2-fold, respectively. In parallel a 1.5-fold increase of total catalase and mitochondrial butyryl-CoA dehydrogenase activities occurs, whereas peroxisomal urate oxidase and glycolate oxidase remain normally active. Serum triglyceride levels are decreased after 3 wk of phytol feeding; serum cholesterol levels remain unaffected. Phytol feeding also induces peroxisome proliferation in duodenal epithelium, in myocardium and in skin sebaceous glands, but not in kidney.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Van den Branden C,Vamecq J,Wybo I,Roels Fdoi
10.1203/00006450-198605000-00007subject
Has Abstractpub_date
1986-05-01 00:00:00pages
411-5issue
5eissn
0031-3998issn
1530-0447journal_volume
20pub_type
杂志文章abstract::We used quin2, a high affinity fluorescent calcium indicator to measure free cytosolic Ca++ in neutrophils isolated from fresh heparinized blood of 13 cystic fibrosis (CF) patients (age range 6-32 yr) and 11 healthy individuals (age range 25-41 yr) as controls. The mean value of free cytosolic Ca++ for CF patients was...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198519040-00006
更新日期:1985-04-01 00:00:00
abstract::Long chain polyunsaturated fatty acids (LCPUFA)seem to be the most trophic macronutrients in inducing intestinal adaptation in adult short bowel syndrome (SBS), although their effects on intestinal adaptation in infants with SBS remain unknown.It is hypothesized that a high fat diet enriched with n-3 LCPUFA derived fr...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181eb2ee5
更新日期:2010-09-01 00:00:00
abstract::Hereditary surfactant protein B (SP-B) deficiency has been lethal in the first year of life without lung transplantation. We tested the hypothesis that SP-B gene mutations may result in milder phenotypes by investigating the mechanisms for lung disease in two children with less severe symptoms than have been previousl...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200009000-00003
更新日期:2000-09-01 00:00:00
abstract::Jejunal biopsies obtained from 45 children referred for a variety of clinical conditions were examined histologically and by standard immunofluorescence methods. The number of plasma cells was recorded per "mucosal tissue unit". The results showed that most of the immunoglobulin A (IgA) counts in infants under 3 yr wi...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197906000-00002
更新日期:1979-06-01 00:00:00
abstract::The ventricular epicardial activation sequence (VEAS) in 15 anesthetized lambs (near term fetus to 3.5 months of age) was determined using 40 simultaneously recorded bipolar electrograms for each animal. Isochrone maps were drawn by hand using relative activation times determined from the maximum first derivative of m...
journal_title:Pediatric research
pub_type: 杂志文章
doi:
更新日期:1977-01-01 00:00:00
abstract::Genetic factors are implicated in pathogenesis of neonatal hyperbilirubinemia. In this nested case-control study, we determined 1) frequency of thymine-adenine (TA)n promoter polymorphism and Gly71Arg mutation in uridine diphosphoglucuronate-glucuronosyltransferase 1A1 (UGT1A1) gene in neonates > or =35-wk gestation p...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e31819ed5de
更新日期:2009-06-01 00:00:00
abstract::We probed possible developmentally related changes in thin filament activity in rat hearts with the aid of calmidazolium (CDZ). CDZ is a calmodulin antagonist that also binds to troponin C and stimulates Ca2+ troponin C-dependent activation of cardiac myofibrillar contractile activity. In paired experiments, we compar...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199007000-00011
更新日期:1990-07-01 00:00:00
abstract:BACKGROUND:Prematurity may influence the levels of amino acids, enzymes, and endocrine markers obtained through newborn screening. Identifying which analytes are the most affected by degree of prematurity could provide insight into how prematurity impacts metabolism. METHODS:Analytes from blood spots assayed by Newbor...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.212
更新日期:2014-02-01 00:00:00
abstract::Seven essentially healthy term infants who received gentamicin starting on the 1st day of life for prolonged rupture of membranes and maternal fever were compared with nine healthy term infants to determine whether this drug induces alterations in the auditory pathway. The auditory pathway was studied on the 3rd day o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199009000-00017
更新日期:1990-09-01 00:00:00
abstract:BACKGROUND:Caffeine is widely used to manage apnea of prematurity, and reduces the incidence of bronchopulmonary dysplasia (BPD). Deregulated transforming growth factor (TGF)-β signaling underlies arrested postnatal lung maturation in BPD. It is unclear whether caffeine impacts TGF-β signaling or postnatal lung develop...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.21
更新日期:2017-05-01 00:00:00
abstract::Labeled choline incorporation into adult rat lung alveolar epithelial cells and adult rat lung fibroblasts in monolayer culture was determined after incubation with insulin (Ins) 10 micrograms/ml, Dexamethasone (Dex) 10(-6)M, or no drug (ND). Incubation periods were 1, 3, 4, and 5 hours. The lecithin (phosphatidyl cho...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198007000-00014
更新日期:1980-07-01 00:00:00
abstract::Inhibition of IGF-I action by circulating IGF-I receptor autoantibodies is a potential mechanism of IGF-I resistance in growing children. To define the prevalence of IGF-I receptor antibodies in short-statured children, we have examined serum and plasma samples from a well-characterized group of 34 short, prepubertal,...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199210000-00016
更新日期:1992-10-01 00:00:00
abstract::The odds risk of vertical transmission of human immunodeficiency virus (HIV) to preterm infants is almost four times that of term infants and may relate to maternal and neonatal factors. We characterized the competence of early nonspecific cellular immunity, namely natural killer cytotoxicity (NKC) and antibody-depend...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199609000-00021
更新日期:1996-09-01 00:00:00
abstract::We examined 78 fetuses on d 21 of gestation (G21) and 83 neonates on d 2 after birth (A2), which were first generation offspring of WKY rats mated with normal Wistar rats (F1). In addition, we examined six groups of fetuses on d 19 of gestation (G19): 65 Wistar rats, 111 WKY rats, 85 F1, 100 F1 X F1, 92 F1 X Wistar, a...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199109000-00004
更新日期:1991-09-01 00:00:00
abstract:BACKGROUND:The present study examined the association between 9/11-related adolescent behavioral problems on mental health outcomes in early adulthood. METHODS:Data from enrollees of the World Trade Center Health Registry, who completed at least one adolescent (2006-2007 or 2011-2012) and adult survey (2011-2012 or 20...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-018-0050-8
更新日期:2018-08-01 00:00:00
abstract:INTRODUCTION:Data regarding neonatal arterial ischemic stroke (NAIS) topography are still sparse and inaccurate. Despite the importance of locating NAIS to predict the long-term outcome of neonates, a map of arterial territories is not yet available. Our aim was therefore to generate the first three-dimensional map of ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0724-x
更新日期:2020-06-01 00:00:00
abstract::Taurine is an essential amino acid during fetal life and appears to be vital for the growth of the fetus and for the development of the CNS. In intrauterine growth restriction (IUGR), fetal plasma concentrations of taurine are reduced, and we tested the hypothesis that this is caused by altered placental transport of ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199808000-00016
更新日期:1998-08-01 00:00:00
abstract::It is increasingly recognized that the abnormal physiologic consequences of pulmonary hypertension (PH) may contribute to poor cardiopulmonary health in premature babies. Conflicting literature has led to clinical uncertainty, pathological misinterpretation, and variability in treatment approaches among practitioners....
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/pr.2017.200
更新日期:2017-12-01 00:00:00
abstract::A prospective study to define the normal patterns of development of mucosal immunity in 263 children provided a unique opportunity to study the mucosal immune response in an infant who unexpectedly died from sudden infant death syndrome. The subject initially had a normal pattern of mucosal immune function, which was ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199306000-00003
更新日期:1993-06-01 00:00:00
abstract::DNA segments that are separated from the promoter region of a gene by many thousands of bases may nonetheless regulate the transcriptional activity of that gene. This finding has led to the investigation of mechanisms underlying long-range chromatin interactions. In intermitotic cells, chromosomes decondense, filling ...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/pdr.0b013e31804575db
更新日期:2007-05-01 00:00:00
abstract::The microchemical study of growth cartilage biopsies may improve the classification and the genetic advice of some types of growth disturbances and contribute to the understanding of biochemical defects. Small tibial growth cartilage biopsies were performed during orthopedic surgery in cases with achondroplasia, pseud...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197510000-00006
更新日期:1975-10-01 00:00:00
abstract::All enzymes of the urea cycle are demonstrable in circulating leukocytes. They show the same relative activities as those in liver except for argininosuccinate synthetase + lyase (combined) which seems to be disproportionately active. To see whether leukocytes reflect liver activity, blood from patients with three hep...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197506000-00004
更新日期:1975-06-01 00:00:00
abstract::Since the description of bronchopulmonary dysplasia (BPD) in premature infants, the supplemental oxygen administered has been suspect in the etiology of BPD. This has prompted studies on the effect of hyperoxia on lung growth in neonatal animals. So far, these have not led to a treatment which either prevents or mitig...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199608000-00007
更新日期:1996-08-01 00:00:00
abstract:BACKGROUND:Fetal hypoxia-ischemia (H-I) results in significant morbidity and mortality. Little is known about the timing of death in human stillbirths. The vulnerability of the fetus varies with age at the time of insult, but it is unknown what happens to the timing of fetal death in relation to a fetal insult. We aske...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2012.65
更新日期:2012-08-01 00:00:00
abstract::The effects of an emulsified intravenous fat preparation (Intralipid) on bilirubin transport were analyzed by cholestyramine extraction, spectrophotometric analysis, Sephadex gel filtration, peroxidase assay, bilirubin uptake by red blood cells, and by toxicity in tissue culture (L-929) cells. Intralipid is capable of...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197703000-00004
更新日期:1977-03-01 00:00:00
abstract::The interrelationship of 2,3-diphosphoglycerate (2,3-DPG) and P50 levels during the fetal and postnatal life were determined in two mammalian species which do not have a switchover of hemoglobin type at the end of their fetal development. In the guinea pig and rabbit, the 2,3-DPG levels remain low during fetal life an...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197903000-00006
更新日期:1979-03-01 00:00:00
abstract::In an effort to characterize the hepatic abnormality in patients with alpha1-antitrypsin deficiency, three unrelated children with the disorder (Pi types ZZ and SZ), two heterozygous parents (Pi type MZ), and three normal subjects (Pi type MM) were studied. As expected, the livers of the ZZ- and SZ-deficient subjects ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197601000-00007
更新日期:1976-01-01 00:00:00
abstract::Intrauterine ligation of the common bile duct was carried out in ten fetal lambs at approximately 80 days gestational age. Six of the fetal lambs survived until term and were sacrificed at varying periods until the 36th day of life. The lesion which developed resembled a "correctable" type of extrahepatic biliary atre...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198005000-00007
更新日期:1980-05-01 00:00:00
abstract::BackgroundTo examine the mechanism of urethral seam formation during embryonal development of rat urethra.MethodsTime-mated Sprague-Dawley rats were killed and the genital tubercles of male pups harvested on embryonic day (ED) 15, 16, 18, and 19. External morphology was observed under scanning electron microscope. Ser...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.185
更新日期:2017-12-01 00:00:00
abstract::Spontaneous preterm birth is a serious and common pregnancy complication associated with hormonal dysregulation, infection, inflammation, immunity, rupture of fetal membranes, stress, bleeding, and uterine distention. Heredity is 25-40% and mostly involves the maternal genome, with contribution of the fetal genome. Si...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/s41390-018-0180-z
更新日期:2019-03-01 00:00:00