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 hepatic urea cycle disorders was tested. In each case, the leukocytes showed the same enzyme deficiency as was apparent from a liver biopsy (Table 4). Leukocyte assays appear to be reliable indicators of the enzyme lesions in inherited urea cycle enzyme defects and therefore may obviate the need for liver biopsy.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Wolfe DM,Gatfield PDdoi
10.1203/00006450-197506000-00004subject
Has Abstractpub_date
1975-06-01 00:00:00pages
531-5issue
6eissn
0031-3998issn
1530-0447journal_volume
9pub_type
杂志文章abstract::Mucolipidosis 4 (ML4) is an autosomal recessive disorder with both lipid and mucopolysaccharide storage. The disease is characterized by severe visual impairment and psychomotor retardation. In our effort to find a phenotypic marker for ML4 fibroblasts, living cells were stained with fluorescent compounds. The stainin...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199506000-00003
更新日期:1995-06-01 00:00:00
abstract::We investigated the role of circulating bone marrow cells (BMC) in cardiomyocyte regeneration. BMC, isolated from transgenic mice expressing enhanced green fluorescent protein (GFP), were transplanted into lethally irradiated C57BL6 mice. Five weeks after bone marrow transplantation (BMT), flow cytometric analysis for...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000078275.14079.77
更新日期:2003-09-01 00:00:00
abstract::Naloxone, a specific opiate antagonist with no agonist properties, in doses of 0.4 and 4.0 mg/kg was found to markedly reduce the duration of primary apnea in asphyxiated newborn rabbits. There was no effect on the duration of the hyperpneic phase (time to primary apnea) or on survival time (time to last gasp). It is ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198004000-00021
更新日期:1980-04-01 00:00:00
abstract::The present study aimed to test whether an acute period of asphyxia during birth in the rat results in long-term alterations in CNS function. Morphologic studies have indicated that the hippocampus is particularly vulnerable to perinatal anoxia. Thus, the present study tested adult rats, which had undergone acute birt...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199504000-00018
更新日期:1995-04-01 00:00:00
abstract::BackgroundSotos syndrome (SoS) is an overgrowth disorder with various congenital anomalies and is usually accompanied by other clinical problems. However, anorectal malformations have not been documented as part of the SoS entity. Our objective is to report on a case of SoS associated with Hirschsprung's disease (HSCR...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.106
更新日期:2017-07-01 00:00:00
abstract:BACKGROUND:Pediatric myocarditis is a rare disease with substantial mortality. Little is known regarding its prognostic factors. We hypothesize that certain comorbidities and procedural needs may increase risks of poor outcomes. This study aims to identify prognostic factors for pediatric myocarditis. METHODS:The nati...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-01268-7
更新日期:2020-11-18 00:00:00
abstract:BACKGROUND:Heterozygous ATP-binding-cassette subfamily A member 3 (ABCA3) mutations are associated with neonatal respiratory complications. In an adult murine model, we investigated whether Abca3 haploinsufficiency is a predisposing factor for lung injury induced by hyperoxia or mechanical ventilation. METHODS:Abca3 h...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.127
更新日期:2013-10-01 00:00:00
abstract::Chitosan is a natural, cationic polysaccharide derived from fully or partially deacetylated chitin. Chitosan is capable of inducing large phospholipid aggregates, closely resembling the function of nonionic polymers tested previously as additives to therapeutic lung surfactants. The effects of chitosan on improving th...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000227558.14024.57
更新日期:2006-08-01 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::In view of their known high incidence of noninsulin dependent diabetes (NIDDM), we sought to determine whether Native American (Plains Indian) children and adolescents show evidence of risk factors for both NIDDM and cardiovascular disease. Children and adolescents between the ages of 4 and 19 y were recruited, and fi...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/00006450-199607000-00014
更新日期:1996-07-01 00:00:00
abstract::We report energy intake and selected nutrient intakes-protein, fat (total, saturated, and polyunsaturated), carbohydrate (total, starch, and sucrose), and cholesterol-for 1251 white children ages 6-19 years. The data were obtained, by means of a 24-h dietary recall, from children who were randomly selected from the No...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198302000-00009
更新日期:1983-02-01 00:00:00
abstract::A dietary-related deficiency of docosahexaenoic acid [C22:6(n-3)] in infant cerebral cortex has been identified. Absence or very low rates of hepatic synthesis from the essential fatty acid precursor, alpha-linolenic acid [C18:3(n-3)], in early life may have been a contributory factor. We have analyzed liver total lip...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199509000-00015
更新日期:1995-09-01 00:00:00
abstract::Twelve-h overnight urine and serum samples obtained simultaneously at 20-min intervals were assayed for growth hormone (GH). Ninety-one children, 5 to 16 y (Tanner stage 1 to 3) participated; group 1 were healthy children, group 2 were children with organic GH deficiency, and group 3 had idiopathic growth failure and ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199106010-00019
更新日期:1991-06-01 00:00:00
abstract::To determine whether epidermal growth factor (EGF) or EGF-like transforming growth factors (TGFs) are present in ovine fetal tissues, we have tested acid-ethanol extracts of ovine fetal kidney for the ability to induce anchorage-independent growth of normal rat kidney fibroblasts in soft agar and to compete with 125I-...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198711000-00026
更新日期:1987-11-01 00:00:00
abstract::BACKGROUNDThe aims of this study were to (i) compare the concentrations of two neural injury markers, S100B protein and neuron-specific enolase (NSE), in intrauterine growth-restricted (IUGR) fetuses and in fetuses with appropriate growth-for-gestational-age (AGA), and (ii) investigate potential relationships between ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.108
更新日期:2017-09-01 00:00:00
abstract::Premature newborn rabbits, delivered by hysterotomy on day 27 of gestation, were tracheotomized at birth, kept in body plethysmographs, and subjected to pressure-generated ventilation at a working pressure of 25 cm H2O, 100% O2, and frequency 40/min. Thirty-seven animals received 50 microliters of heterologous surfact...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198211000-00004
更新日期:1982-11-01 00:00:00
abstract::We determined the inhibitory effects of concentrations of tin- and zinc protoporphyrin (1-100 microM) and mesoporphyrin (0.1-10 microM) on the in vitro heme oxygenase (HO) (E.C.1.14.99.3) activity in liver, spleen, and intestine 13,000 x g tissue supernatants from fasted adult male Wistar rats through measurement of c...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198910000-00015
更新日期:1989-10-01 00:00:00
abstract:INTRODUCTION:The Special Supplemental Nutrition Program for Women, Infants, and Children (WIC) serves 50% of infants and 25% of preschool-aged children in the United States and collects height and weight measurements from eligible children every 6 mo, making WIC data a valuable resource for studying childhood growth an...
journal_title:Pediatric research
pub_type: 杂志文章,多中心研究
doi:10.1038/pr.2011.37
更新日期:2012-03-01 00:00:00
abstract::Although cisapride is widely used to treat gastrointestinal motility disorders, it has been associated with QT prolongation, torsades de pointes, and cardiac arrest. Only in children, however, has atrioventricular (AV) block after cisapride been reported. This study used Langendorff perfusion to determine the direct e...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000050122.67091.1D
更新日期:2003-03-01 00:00:00
abstract:BACKGROUND:For the second aim of the Kellogg Foundation grant, this double-blind RCT investigated the impact of plasma vitamin D metabolite 25-hydroxyvitamin D (25(OH)D) on plasma immune-mediators during pregnancy. We hypothesized that higher 25(OH)D concentrations would associate with reduced pro-inflammatory and incr...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-0885-7
更新日期:2020-04-20 00:00:00
abstract:BACKGROUND:Phytosterols in soybean oil (SO) lipids likely contribute to parenteral nutrition-associated liver disease (PNALD) in infants. No characterization of phytosterol metabolism has been done in infants receiving SO lipids. METHODS:In a prospective cohort study, 45 neonates (36 SO lipid vs. 9 control) underwent ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2015.78
更新日期:2015-08-01 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: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::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::Lung distensibility and airway mechanics were evaluated in 24 asthmatic children and adolescents, ages between 7 and 21 years, by quasi-static pressure-volume curves and by the static recoil-lung conductance relationship. The measurements were obtained by the step-wise inflation technique and the pressure-volume curve...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198411000-00020
更新日期:1984-11-01 00:00:00
abstract::Meconium aspiration syndrome (MAS) is a serious condition in newborns, associated with a poorly characterized inflammatory reaction. The aim of this study was to investigate a possible role for complement in pulmonary pathophysiology and systemic inflammation in experimental MAS. MAS was induced by instillation of mec...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000141983.32466.2A
更新日期:2004-11-01 00:00:00
abstract:BACKGROUND:Regular physical activity provides children with health and developmental benefits. This study investigated if active play and walking with the family dog was associated with better social-emotional development in young children. METHODS:We surveyed 1646 parents to ascertain if families with pre-schoolers o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-1007-2
更新日期:2020-07-06 00:00:00
abstract::Recent studies have shown 1,25(OH)2D3 receptor-mediated modulation of leukocyte proliferation, differentiation, and function. We examined the phagocytosis and killing of microorganisms by neutrophils and monocytes from five patients of three families with hereditary resistance to 1,25(OH)2D3. Phagocytosis of microorga...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198903000-00012
更新日期:1989-03-01 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::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