Abstract:
:A new model has been developed for the study of maternal phenylketonuria. Beginning on the 12th day of gestation the diet of pregnant rats was supplemented with 0.5% alpha-methylphenylalanine and 3% phenylalanine. This resulted in an 83% reduction of hepatic phenylalanine hydroxylase activity. The maternal plasma phenylalanine was elevated 10-20-fold for two-thirds of the day, but the degree and persistence of the fetal hyperphenylalaninemia may have been even greater. The brain phenylalanine concentrations in the fetus were raised up to 2900 nmole/g brain, whereas the highest level observed in the dam was 382 nmole/g. Experimentally-treated fetuses showed small reductions in both body and brain weight when compared to age-matched controls; however, no differences were seen in crown to rump length, litter size, DNA and protein concentrations per g, or in postnatal survival. Initiation of the diet at conception rather than on the 12th day caused a significantly greater inhibition of fetal growth, and 21% mortality. The fetal cerebral concentrations of methionine and the branched chain amino acids (valine, leucine and isoleucine) were decreased by hyperphenylalaninemia. From the 16th day on, the concentration of the inhibitor neurotransmitter glycine was elevated. Cerebral serotonin showed a 20-30% deficit and its primary metabolite 5-hydroxyindoleacetic acid a 71-77% deficit. Of twelve enzymes quantified in the brains of hyperphenylalaninemic fetuses only phosphoserine phosphatase showed any change. From the 20th to the 22nd day of gestation its activity was 46-67% higher in experimental than in normal fetuses. Measurement on the 22nd day of gestation showed that the increases in phosphoserine phosphatase activity and glycine content were present in brain stem, cerebellum, and forebrain.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Brass CA,Isaacs CE,McChesney R,Greengard Odoi
10.1203/00006450-198205000-00014subject
Has Abstractpub_date
1982-05-01 00:00:00pages
388-94issue
5eissn
0031-3998issn
1530-0447journal_volume
16pub_type
杂志文章abstract::Angiotensin II-forming chymase is expressed in the pulmonary arteries of the monocrotaline-induced pulmonary hypertensive rats, but its actual role is unclear. We studied chymase-dependent angiotensin II formation in the pulmonary arteries of the monocrotaline-induced pulmonary hypertensive rats and observed the effec...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000219431.45075.d9
更新日期:2006-07-01 00:00:00
abstract::The ontogeny of glutamine uptake by jejunal brush border membrane vesicles was studied in suckling and weanling rats and compared with the data obtained from previous studies done on adult rats in our laboratory. Glutamine uptake represented transport into the intravesicular space rather than mere binding into the mem...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199005000-00021
更新日期:1990-05-01 00:00:00
abstract::Docosahexaenoic acid (DHA) is an omega-3 fatty acid that is highly concentrated in CNS tissues. Although breast milk contains the fatty acids DHA and arachidonic acid, infant formulas marketed in North America do not contain these nutrients. The potential deleterious effects of rearing infants with formulas devoid of ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200203000-00003
更新日期:2002-03-01 00:00:00
abstract::We have performed clinical, in vitro biochemical, and genetic studies of a patient with severe insulin resistance, considerable growth restriction, and Rabson-Mendenhall syndrome (patient RM-3). The blood IGF-I level was undetectable in this patient, although the GH level was moderately decreased. During the postprand...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199707000-00012
更新日期:1997-07-01 00:00:00
abstract::Near-infrared spectroscopy is a noninvasive method for monitoring brain oxygenation. The aim of the study was to investigate differences between cerebral oxygenation in different brain regions in newborns. In a prospective study, we monitored simultaneously left and right frontoparietal and temporo-occipital regional ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e31822a36db
更新日期:2011-10-01 00:00:00
abstract::An Epstein-Barr virus-transformed B-cell line derived from a patient with severe combined immunodeficiency who died of a lymphoreticular malignancy has been characterized. The line derived from bone marrow cultures and designated DV-1 shows surface and cytoplasmic IgM and staining with fluorescent monoclonal antibodie...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198704000-00003
更新日期:1987-04-01 00:00:00
abstract:BACKGROUND:Bicuspid aortic valve (BAV) is the most common type of congenital heart disease (CHD) and has a proposed genetic etiology. BAV is categorized by cusp fusion, with right-left (R-L) cusp fusion being associated with additional CHD, and right-noncoronary cusp (R-NC) fusion being associated with aortic valve dys...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2014.67
更新日期:2014-08-01 00:00:00
abstract::We report reference values for osteocalcin and bone and liver alkaline phosphatase (ALP) isoforms in serum from 114 healthy children and adolescents, age 7-16 y. The bone and liver ALP isoforms were measured by high performance liquid chromatography (HPLC). Six ALP isoforms were separated and quantified in all samples...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199512000-00021
更新日期:1995-12-01 00:00:00
abstract:BACKGROUND:The aim of this study was to determine whether small-for-gestational-age (SGA) infants born very prematurely had increased respiratory morbidity in the neonatal period and at follow-up. METHODS:Data were examined from infants recruited into the United Kingdom Oscillation Study (UKOS). Of the 797 infants who...
journal_title:Pediatric research
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1038/pr.2012.201
更新日期:2013-04-01 00:00:00
abstract::Reduced expression of bone morphogenetic protein receptors (BMPR) has been implicated in the pathogenesis of pulmonary hypertension (PH), but changes in the intracellular signaling pathway of BMPR have not been fully understood. We hypothesized that BMPR signaling in pulmonary endothelial cells is altered during the d...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/pdr.0b013e3180332cba
更新日期:2007-04-01 00:00:00
abstract::To describe the relation between scalp blood flow and transcutaneous PO2 and PCO2, these three signals were recorded simultaneously in eight experiments on four healthy lambs in the first weeks after birth. Scalp blood flow was recorded by a laser Doppler flow sensor that was incorporated in the transcutaneous PO2 ele...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199005000-00005
更新日期:1990-05-01 00:00:00
abstract::The sheep has served as an informative animal model for investigation of human fetal and newborn erythropoiesis and red blood cell (RBC) kinetics. We previously validated the permanent label (14C)cyanate for measuring red cell volume (RCV) in sheep. Here, we validate biotin labeling of RBCs as a nonradioactive method ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e318183f119
更新日期:2008-11-01 00:00:00
abstract::The development of the intestinal mucosal immune barrier is an important protective adaptation for postnatal life. The distribution and phenotype of T and B lymphocytes in human fetal intestine and lymphoid tissues have been characterized and compared to the distribution and phenotype of lymphocytes in postnatal intes...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199003000-00007
更新日期:1990-03-01 00:00:00
abstract::Iron is essential for fetal organ development, but the effect of isolated iron deficiency on nephrogenesis is unknown. Human premature infants are at risk for disrupted nephrogenesis because glomerular development is incomplete until 36-wk gestation. We modeled the effects of iron on postnatal glomerulogenesis in four...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181be79c2
更新日期:2009-12-01 00:00:00
abstract::A strongly positive tuberculin skin reaction (> 1.5 cm2) was observed during the acute phase of the illness in 11 children with Kawasaki disease (KD), but not in control pediatric patients with other febrile infections (41 patients) or diseases similar to KD (9 patients). The cutaneous sensitivity to intermediate stre...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/00006450-199704000-00017
更新日期:1997-04-01 00:00:00
abstract::Brain injury remains one of the major unsolved problems in neonatal care, with survivors at high risk of lifelong neurodisability. It is unlikely that a single intervention can ameliorate neonatal brain injury, given the complex interaction between pathological processes, developmental trajectory, genetic susceptibili...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/s41390-019-0729-5
更新日期:2019-12-18 00:00:00
abstract::The effect of hypoxia-ischemia (HI) on IL-1, and IL-6 bioactivity in relation to expression of IL-1 alpha, IL-1 beta, and IL-6 mRNA was studied, and the neuroprotective efficacy of IL-1 receptor antagonist (IL-1ra) was evaluated in neonatal rats. HI was induced in 7-d-old rats by unilateral carotid artery ligation and...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199610000-00015
更新日期:1996-10-01 00:00:00
abstract::Previous studies in adults have shown that chronic pulmonary hypertension is associated with decreased endothelial nitric oxide synthase (eNOS) expression in pulmonary arteries. However, the role of decreased eNOS expression in persistent pulmonary hypertension of the newborn (PPHN) is unknown. We investigated the hyp...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199809000-00012
更新日期:1998-09-01 00:00:00
abstract::Three members of a Virginia family with a bleeding disorder were found to have a serum defect in thromboplastin generation similar to the previously reported "Car. factor" deficiency. Sera from three members of the original Car. family did not correct the defect of affected members of the Virginia family. Partial thro...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/00006450-197504000-00009
更新日期:1975-04-01 00:00:00
abstract::The secreted protein sonic hedgehog (Shh) is essential for normal development of many organs. Targeted disruption of Shh in mouse leads to near complete absence of craniofacial skeletal elements at birth, and mutation of SHH in human causes holoprosencephaly (HPE), frequently associated with defects of derivatives of ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000199911.17287.3e
更新日期:2006-03-01 00:00:00
abstract::Our aim was to obtain a better understanding of the differences between breast-feeding and bottle-feeding, particularly with regard to how sucking performance changes from nonnutritive sucking (NNS) to nutritive sucking (NS). Twenty-two normal term infants were studied while breast-feeding at 4 and 5 d postpartum. Fiv...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000214993.82214.1c
更新日期:2006-05-01 00:00:00
abstract::The zeta subunit of the CD3 T-cell receptor complex and the major histocompatibility complex class 1 (MHC-I) are important not only for the immune response to antigens, they also function as signal molecules in the brain, where they play a role in the postnatal maturation process. The expression of these molecules can...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000238246.74817.a0
更新日期:2006-10-01 00:00:00
abstract::Milk gangliosides inhibit Vibrio cholerae enterotoxin and Escherichia coli heat-labile enterotoxin. Human milk gangliosides showed considerably higher enterotoxin-inhibitory activity compared to bovine and formula milk gangliosides as measured in vitro by enzyme-linked immunosorbent assay and in vivo in rabbit small b...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198605000-00008
更新日期:1986-05-01 00:00:00
abstract::The objective of this study was to establish longitudinal normative limits for home memory monitors during early infancy. Eighty-eight healthy infants were monitored overnight at 0.25-19 wk of age using the Healthdyne Smart Monitor. Apnea settings were 14 s for recording and 40 s for alarm; the bradycardia setting was...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199602000-00005
更新日期:1996-02-01 00:00:00
abstract::Gitelman disease was diagnosed in two unrelated children with hypokalemic metabolic alkalosis and growth failure (a boy and a girl aged 7 mo and 9.5 y, respectively, at clinical presentation) on the basis of mutations detected in the gene encoding the thiazide-sensitive NaCl cotransporter of the distal convoluted tubu...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199908000-00017
更新日期:1999-08-01 00:00:00
abstract::Long-term parenteral nutrition (TPN) in children is associated with sustained hyperinsulinemia due to a high nutriment infusion flow 12 h/24 h, with plausible lipotoxicity secondary to repeated lipid infusions and with changes in incretin hormone release. The aim of this study was to test whether long-term TPN can lea...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/PDR.0b013e3181559d5c
更新日期:2007-11-01 00:00:00
abstract::In artificial tubes as well as in blood vessels with diameters less than 500 microns, blood viscosity decreases with decreasing diameter (Fahraeus-Lindqvist effect). Our study measured viscosity of red blood cells (RBC) from 10 preterm infants, 10 term neonates, and 10 adults by means of a capillary viscometer. RBC we...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198906000-00009
更新日期:1989-06-01 00:00:00
abstract::Neutrophils contribute to ischemic brain injury in adult animals. The role of neutrophils in perinatal hypoxic-ischemic (HI) brain injury is unknown. Allopurinol reduces neutrophil accumulation after tissue ischemia and is protective against HI brain injury. This study was designed to investigate how neutrophils contr...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199705000-00002
更新日期:1997-05-01 00:00:00
abstract::To localize fetal rabbit lung beta-adrenoreceptors before and after glucocorticoid treatment, light microscopic autoradiography was performed with the reversible radiolabeled beta-adrenergic antagonist, [3H]dihydroalprenolol, on day 26 of gestation. Autoradiograms of adult lung and fetal myocardium were also prepared....
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198411000-00028
更新日期:1984-11-01 00:00:00
abstract::Intrauterine perfusion failure can cause cerebral malformations. We investigated the effect of transient maternal hypotension on newborn rat brain by inducing hypovolemic hypotension for 2.5 h during early embryonic day 7 (E7) or late (E15) gestation in pregnant rats. We found an increase in the number of TUNEL-positi...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200101000-00012
更新日期:2001-01-01 00:00:00