Abstract:
:In Krabbe's disease (globoid cell leukodystrophy), galactosylsphingosine (psychosine) is considered to be a causative agent of the pathology found in the nervous system of the patients. In our study, we examined the cytotoxic effect of psychosine in neural cell cultures derived from the rat nervous system. The concentration of toxic thresholds varied from cell type to cell type. The 50% of toxic doses for oligodendrocytes, astrocytes, and the sensory neurons of the dorsal root ganglia were 8, 20, and 30 micrograms/mL, respectively. Oligodendrocytes, therefore, appeared to show a higher sensitivity to psychosine than did astrocytes or neurons. When phorbol ester or DMSO was applied simultaneously with psychosine as protective agents in enriched cultures of rat oligodendrocytes, the total number of live cells and galactocerebroside-positive cells and the 2'3'-cyclic nucleotide 3'-phosphohydrolase activity in these cultures were considerably higher as compared with their levels in the experimental cultures treated with psychosine alone. These results indicate that phorbol ester and DMSO could serve as protective agents for psychosine neurotoxicity.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Sugama S,Kim SU,Ida H,Eto Ydoi
10.1203/00006450-199011000-00011subject
Has Abstractpub_date
1990-11-01 00:00:00pages
473-6issue
5eissn
0031-3998issn
1530-0447journal_volume
28pub_type
杂志文章abstract::A male child presented on the first day of life with metabolic acidosis with elevated blood lactate (15 mM), pyruvate (0.4 mM), and free fatty acid (1.3 mM) levels and a blood pH of 7.16. The severity of the acidosis was diminished by intravenous administration of glucose in large doses and by bicarbonate. On two occa...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197512000-00015
更新日期:1975-12-01 00:00:00
abstract::Left ventricular (LV) output nearly triples at birth, in association with increases in serum catecholamines. Similar increases in catecholamines in utero, however, do not increase output. We hypothesized that catecholamines increase contractility in utero, but that output cannot increase until LV loading conditions ar...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199701000-00009
更新日期:1997-01-01 00:00:00
abstract::Endothelin-1 can cause pulmonary vasoconstriction via endothelin-A (ET(A)) receptor activation. We hypothesized that ET(A) blockers (EMD 122946 and BQ 610) would reduce hypoxia-induced (HYP) but not group B streptococcal infusion (GBS)-induced pulmonary hypertension in a juvenile whole animal model. Pulmonary hyperten...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200212000-00017
更新日期:2002-12-01 00:00:00
abstract::Very young children with organic brain damage, intractable seizures, and developmental retardation are at particular risk of developing fatal hepatic dysfunction coincident with valproate therapy, especially if the children are also receiving other anticonvulsant drugs. The mechanism of valproate-associated hepatic fa...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199204000-00023
更新日期:1992-04-01 00:00:00
abstract::BackgroundThe mechanisms underlying aberrant activation of intestinal Toll-like receptor 4 (TLR4) signaling in necrotizing enterocolitis (NEC) remain unclear. In this study, we examined the role of single-immunoglobulin interleukin-1 receptor-related molecule (SIGIRR), an inhibitor of TLR signaling, in modulating expe...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.211
更新日期:2018-01-01 00:00:00
abstract::To study cardiovascular autonomic control, we assessed the effect of atropine on heart rate (HR) and blood pressure (BP) variability in 12 preterm infants (range 26-32 wk) before intubation for respiratory insufficiency. Spectral power analysis of R-R interval and systolic BP (SBP) series were estimated in a low-frequ...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/01.PDR.0000145257.75072.BB
更新日期:2004-12-01 00:00:00
abstract::BackgroundExtracellular adenine nucleotides contribute to ischemia-reperfusion injury following infant cardiopulmonary bypass (CPB), whereas conversion to adenosine may be protective. Alkaline phosphatase (AP), a key enzyme responsible for this conversion, decreases after infant CPB. Indirect evidence suggests that so...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.325
更新日期:2018-04-01 00:00:00
abstract::LBW infants are at risk of iron deficiency (ID), which is associated with impaired nervous system development and may lead to prolonged auditory brainstem response (ABR) latencies. We hypothesized that iron supplementation shortens ABR latencies in marginally LBW (MLBW, 2000-2500 g) infants. In a randomized, controlle...
journal_title:Pediatric research
pub_type: 杂志文章,随机对照试验
doi:10.1203/PDR.0b013e3182320cd0
更新日期:2011-12-01 00:00:00
abstract::Children with inborn errors of urea synthesis who survive neonatal hyperammonemic coma commonly exhibit cognitive deficits and neurologic abnormalities. Yet, there is evidence that ammonia is not the only neurotoxin. Hyperammonemia appears to induce a number of neurochemical alterations. In rodent models of hyperammon...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199210000-00021
更新日期:1992-10-01 00:00:00
abstract::We investigated whether obese children and adolescents have early echocardiographic signs of subclinical cardiac dysfunction and evaluated the respective influence of obesity per se versus parameters of carbohydrate and lipid metabolism that are frequently abnormal in obese subjects. The role of tissue Doppler imaging...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e318176182b
更新日期:2008-08-01 00:00:00
abstract::Serial plasma concentrations of immunoreactive 6-keto-prostaglandin F1 alpha (6-keto-PGF1 alpha), the stable hydration product of prostacyclin (PGI2), were measured with radioimmunoassay during the first month of life in 25 preterm infants with idiopathic respiratory distress syndrome (IRDS) and 38 preterm controls wi...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198210000-00005
更新日期:1982-10-01 00:00:00
abstract:BACKGROUND:Neonatal encephalopathy (NE) carries high risk for neurodevelopmental impairments. Therapeutic hypothermia (TH) reduces this risk, particularly for moderate encephalopathy (ME). Nevertheless, these infants often have subtle functional deficits, including abnormal memory function. Detection of deficits at the...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2016.169
更新日期:2016-12-01 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:BACKGROUND:Some of the health advantages of breast-fed as compared to formula-fed infants have been suggested to be due to metabolic programming effects resulting from early nutrition. METHODS:In a prospective double-blinded randomized trial, 160 infants <2 mo of age were randomized to experimental formula (EF) with a...
journal_title:Pediatric research
pub_type: 杂志文章,随机对照试验
doi:10.1038/pr.2014.110
更新日期:2014-10-01 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::In blood vessels with diameters less than 500 microns, both the hematocrit and viscosity decrease with decreasing tube diameter [Fåhraeus effect (FE) and Fåhraeus-Lindqvist effect (FLE)]. Because both effects may be influenced by red blood cell (RBC) volume and osmolality, the effects of RBC type and suspending medium...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199310000-00031
更新日期:1993-10-01 00:00:00
abstract::Carlo Agostoni, MD, and Kwang Sik Kim, MD, are the Guest Editors for this annual review issue on Nutrition and the Microbiome. Dr Agostoni is a Professor of Pediatrics at the Department of Clinical Sciences and Community Health, University of Milan, within the Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, ...
journal_title:Pediatric research
pub_type: 历史文章
doi:10.1038/pr.2014.195
更新日期:2015-01-01 00:00:00
abstract::Preterm infants are more prone to bone mineral deficiency the lower their birth weight. To achieve the intrauterine bone mineral accretion rate postnatally, 74 low-birth-weight infants (median birth weight, 980 g; range 430-1.580 g) were each supplemented enterally and/or parenterally with calcium and/or phosphorus in...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/00006450-199401000-00027
更新日期:1994-01-01 00:00:00
abstract::The incidence and prevalence of celiac disease vary internationally. We studied the incidence of identified cases of childhood celiac disease in six Dutch provinces that cover 47.9% of the surface area and 67.6% of the total population of The Netherlands. Children with celiac disease aged 0-14 y, diagnosed from Januar...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199502000-00015
更新日期:1995-02-01 00:00:00
abstract::The proposed actions of atrial natriuretic factor (ANF) are mediated through specific plasma membrane (R1) receptors coupled to guanylate cyclase. A second receptor, R2, has been characterized by its ability to bind to an acyclic, truncated ANF analog (C-ANF4-23). The ANF-R2 receptor has not been identified in the fet...
journal_title:Pediatric research
pub_type: 杂志文章
doi:
更新日期:1991-04-01 00:00:00
abstract::The lymphocytes of human breast milk have been previously shown to be immunologically competent while the monocytes have been described as actively motile in some reports but not in another. Cells were separated from milk samples collected by lactating women 2 to 9 days postpartum. Milk cell migration and production o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198510000-00006
更新日期:1985-10-01 00:00:00
abstract:BACKGROUND:Real-time detection and classification of apneic episodes remain significant challenges. This study explores the applicability of a novel method of monitoring the respiratory effort and dynamics for rapid detection and classification of apneic episodes. METHODS:Obstructive apnea (OA) and hypopnea/central ap...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2015.69
更新日期:2015-07-01 00:00:00
abstract:BACKGROUND:Oral propranolol, a nonselective β-blocker, is able to reduce the progression of retinopathy of prematurity in newborns, but it appeared unsafe. This study aimed to find, in rabbits, a propranolol eye drop concentration able to induce lower plasma but higher retinal concentrations than those obtained after o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2014.108
更新日期:2014-10-01 00:00:00
abstract::Very preterm infants developing bronchopulmonary dysplasia frequently show a compromised growth in the neonatal period especially when steroids are given to facilitate weaning from the ventilator. The aim of this study was to evaluate the short-term effect of dexamethasone (DEXA) on the GH-IGF axis in ventilated very ...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/01.PDR.0000065727.45195.69
更新日期:2003-07-01 00:00:00
abstract::Active compression-decompression (ACD) cardiopulmonary resuscitation (CPR) with the inspiratory threshold valve (ITV) has been recently recommended by the American Heart Association for treatment of adults in cardiac arrest (class IIb: alternative, useful intervention), but this new technique has never been used in a ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200204000-00020
更新日期:2002-04-01 00:00:00
abstract:BACKGROUND:We examined the extent of fetal lung hypoplasia and lung maturation using the amniotic lamellar body count (LBC) in congenital diaphragmatic hernia (CDH). METHODS:We obtained 30 amniotic fluid samples from human CDH cases during cesarean section. We assessed LBC, magnetic resonance imaging (MRI), and ultras...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2012.187
更新日期:2013-03-01 00:00:00
abstract::Pathomechanisms involved in obstructive apneas remain obscure. Apnea arousal failure has been proposed as a cause for sudden death during sleep. The present study hypothesizes an interdependency between upper airway dilating submental muscle electromyogram (EMG) activity (EMGsub), diaphragmatic muscle activity (EMGdia...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199509000-00005
更新日期:1995-09-01 00:00:00
abstract::The ability of synthetic metalloporphyrins to suppress heme oxygenase activity and bilirubin formation has recently become of considerable clinical and experimental interest for suppression of jaundice in humans, including neonatal hyperbilirubinemia. The present investigation compares the biochemical effects of Sn- a...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199209000-00016
更新日期:1992-09-01 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::Premature birth causes significant health risks of the neonate and increases the cost for neonatal care. Urogenital infection, often caused by Gram-negative bacteria, is a known risk factor. Toll-like receptor-4 (TLR4) is the major endotoxin-signaling receptor and as such is crucial for the initiation of the innate im...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200209000-00011
更新日期:2002-09-01 00:00:00