Abstract:
:Purified human liver arylsulfatase A on polyacrylamide gel electrophoresis at pH 4.0 is separated into two protein forms with enzymatic activity and two distinct inactive subunits. All of these components were immunologically distinguishable using different antisera preparations. In late infantile metachromatic leukodystrophy, only one of the two inactive subunits was immunologically detected, whereas in the juvenile form of metachromatic leukodystrophy, both inactive subunits were antigenically present.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Shapira E,DeGregorio RR,Nadler HLdoi
10.1203/00006450-197803000-00007subject
Has Abstractpub_date
1978-03-01 00:00:00pages
199-203issue
3eissn
0031-3998issn
1530-0447journal_volume
12pub_type
杂志文章abstract::Adenylate cyclase activity was measured in membrane preparations of cultured fibroblasts from controls and patients with cystic fibrosis. Enzyme activity increased as the transition from exponential growth to confluence occurred; sodium fluoride-stimulated activity more markedly displayed this relationship than basal ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197808000-00005
更新日期:1978-08-01 00:00:00
abstract::After the infusion of fructose, 0.25 g/kg body wt, the mean peak plasma uric acid level was 5.4 +/- 0.7 (SEM) mg/100 ml in six normal children and was not significantly increased compared with that of the mean basal value of 4.1 +/- 0.5 mg/100 ml. The mean blood inorganic phosphate (Pi) levels were significantly less ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197510000-00005
更新日期:1975-10-01 00:00:00
abstract::Automated microanalytic chromogenic coagulation assays allow serial monitoring of critically ill newborn infants. In this study 84 premature infants [26 healthy prematures and 58 neonates with idiopathic respiratory distress syndrome (IRDS)] were studied daily during the first week of life, to investigate the possible...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198403000-00012
更新日期:1984-03-01 00:00:00
abstract:BACKGROUND:To examine differences in comorbidities and surgical management based on socioeconomics in hospitalized children with Crohn's disease (CD). METHODS:Using the Kids' Inpatient Database for 2006-2012, we identified patients (<21 years) with a CD diagnosis. Cases were analyzed and stratified by median parental ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-0830-9
更新日期:2020-12-01 00:00:00
abstract::Responses of pial arterioles to topically applied arachidonic acid, conversion of exogenous arachidonic acid to prostanoids, and pial arteriolar dilation to hypercapnia were examined before and at progressive times after treatment with indomethacin (5 mg/kg i.v.) in chloralose-anesthetized newborn pigs with closed cra...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199306000-00016
更新日期:1993-06-01 00:00:00
abstract::The diagnosis of transient renal tubular acidosis was made in a 16 months old boy. Bicarbonate titration studies revealed that the acidification defect consisted of an association of proximal and distal tubular acidosis. The effect of experimentally induced potassium depletion revealed that hyperkalemia contributed to...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197910000-00020
更新日期:1979-10-01 00:00:00
abstract::In view of the suggested relationship between substrate availability, fetal growth and circulating fetal IGF-I concentrations, we investigated the effect of maternal starvation on plasma IGF-I levels in the late gestation ovine fetus. Ten fetuses aged 125-130 d gestation were sampled daily from indwelling arterial cat...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199004000-00015
更新日期:1990-04-01 00:00:00
abstract::Blood mononuclear cells from newborns and from adults, immune or nonimmune to herpes simplex virus, were cultured with IL 2 and herpes simplex virus and the amount of gamma-interferon in the supernatant measured after 3 days. The newborn and nonimmune adult cells made equivalent trace amounts of gamma-interferon in cu...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198605000-00004
更新日期:1986-05-01 00:00:00
abstract::This study was made to determine the life span of adult red cells transfused to early preterm infants. Nineteen very preterm infants (birth weight, 878.7 +/- 221 g; gestational age, 26.8 +/- 1.5 wk at birth) were sampled weekly after their last blood transfusion to determine the level (%) of fetal Hb in their circulat...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199707000-00002
更新日期:1997-07-01 00:00:00
abstract::Increased deposition of hyaluronan (HA) is part of the early response to fibrogenic stimulus in the lung exposed to bleomycin injury and has been associated with increased lung water in adult animals. Early respiratory distress syndrome (RDS) in premature infants is characterized by increased lung water, and late sequ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199402000-00024
更新日期:1994-02-01 00:00:00
abstract::Ventilation of immature airways has been shown to result in pressure-induced deformation and alteration of mechanical properties. These changes in mechanical properties may alter the effect of compressive pressures on pressure-flow relationships. To test this hypothesis, unventilated (Group I, n = 8) and ventilated (g...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198805000-00017
更新日期:1988-05-01 00:00:00
abstract:BACKGROUND:Prolonged hypoglycemia leads to brain injury, despite treatment with 10% dextrose. Whether induction of hyperglycemia or ketonemia achieves better neuroprotection is unknown. Hyperglycemia is neuroprotective in other brain injuries during development; however, it worsens hypoglycemia-induced injury in the ad...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2014.146
更新日期:2015-01-01 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: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::The ontogenetic renal responsiveness to exogenous cortisol was examined in the chronically cannulated ovine fetus. The contribution of effects at proximal and distal tubule of the kidney were studied also. Cortisol (81.5 micrograms/h) was infused into immature ovine fetuses (mean gestational age -113.9 days) on five o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198907000-00002
更新日期:1989-07-01 00:00:00
abstract:BACKGROUND:This study aimed to assess cortical gray matter growth and maturation in neonates with congenital heart disease (CHD). METHODS:Thirty-one (near) term neonates with severe CHD (8 univentricular heart malformation (UVH), 21 d-transposition of great arteries (d-TGA) and 2 aortic coarctation) underwent cerebral...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2016.145
更新日期:2016-11-01 00:00:00
abstract::In the fetal rat, nitrofen induces congenital diaphragmatic hernia (CDH) and pulmonary vascular remodeling similar to what is observed in the human condition. Airway hyperactivity is common in infants with CDH and attributed to the ventilator-induced airway damage. The purpose of this study was to test the hypothesis ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000057986.74037.7B
更新日期:2003-05-01 00:00:00
abstract::Matrix metalloproteinases (MMP) are likely effectors of normal lung development, especially branching morphogenesis, angiogenesis, and extracellular matrix degradation. Because hyperoxia exposure (>95% O(2)) from d 4 to 14 in newborn rat pups leads to arrest of alveolarization and mimics newborn chronic lung disease, ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000130658.45564.1F
更新日期:2004-07-01 00:00:00
abstract::This study was designed to evaluate upper airway protective mechanisms in response to pharyngeal fluid stimulation in healthy term and preterm infants at term equivalent age. Five term and seven preterm infants were studied and the following recorded: sleep state, cardiorespiratory function, and swallowing. Infusions ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199811000-00011
更新日期:1998-11-01 00:00:00
abstract::Uteroplacental insufficiency alters the anabolic metabolism of the fetus, resulting in intrauterine growth retardation (IUGR). The metabolic and physiologic factors that cause IUGR have long standing consequences after birth. Postnatal growth and glucose metabolism are altered in the IUGR infant. Skeletal muscle is an...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199805000-00001
更新日期:1998-05-01 00:00:00
abstract::Small for gestational age (SGA) children have a higher prevalence of cardiovascular risk factors at a young age. It is not known whether this increased risk is caused by their size at birth, a familial predisposition for cardiovascular disease or smallness at birth or a combination of these factors. The cardiovascular...
journal_title:Pediatric research
pub_type: 杂志文章,多中心研究
doi:10.1203/PDR.0b013e3181732922
更新日期:2008-07-01 00:00:00
abstract::IL-8/CXC ligand (CXCL) 8 is ingested in high concentrations by the human fetus/neonate with amniotic fluid and human milk, and is also produced constitutively by intestinal epithelial cells (IEC). We have shown that recombinant human IL-8/CXCL8 (rhIL-8/CXCL8) protects cultured IEC against tumor necrosis factor (TNF)-a...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000133196.25949.98
更新日期:2004-08-01 00:00:00
abstract::Primary immunodeficiencies (PIDs) have traditionally been defined according to their immunologic phenotype. Far from being concluded, the search for human genes that, when mutated, cause PID is actively being pursued. During the last year, four novel genetic defects that cause severe combined immunodeficiency and seve...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e31819dbe1e
更新日期:2009-05-01 00:00:00
abstract:BACKGROUND:Neonatal haemorrhaging is often co-observed with thrombocytopenia; however, no evidence of a causal relationship with low platelet count has been reported. Regardless, the administration of a platelet transfusion is often based upon this parameter. Accurate measurement of platelet function in small volumes o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0316-9
更新日期:2019-05-01 00:00:00
abstract:BACKGROUND:In infants admitted to an ICU with respiratory failure, there is an association between the ratio of CD8+ to CD4+ T cells within the upper respiratory tract and disease severity. Whether this ratio is associated with respiratory disease severity within children presenting to a pediatric emergency department ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-021-01364-2
更新日期:2021-01-27 00:00:00
abstract::Sarcoplasmic reticulum (SR) Ca release has been shown not to be the predominant mechanism responsible for excitation-contraction (E-C) coupling in fetal myocytes. However, most of the studies have been conducted either on primary cultures or acutely isolated cells, in which an apparent reduction of ryanodine receptor ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e31820bc69b
更新日期:2011-04-01 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::We have previously shown that generation of superoxide anion occurs in cerebral cortex during asphyxia/reventilation in newborn pigs and that a high dose of indomethacin (5 mg/kg i.v.) abolishes superoxide anion production. The purposes of this study were 1) to determine whether the generation of superoxide anion occu...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199309000-00025
更新日期:1993-09-01 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 objective was to investigate any possible relationship between functional and structural vascular changes in women with low gestational age and/or low birth weight by analyzing the retinal vascular pattern in women with thoroughly documented blood pressure. Retinal vessel morphology was evaluated by digital image ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200206000-00003
更新日期:2002-06-01 00:00:00