Abstract:
:Cerebral concentrations of phenylalanine (PHE) were measured by means of quantitative in vivo 1H MR spectroscopy in 8 adult patients treated early for phenylketonuria type I. A 1.5-Tesla routine magnetic resonance scanner, localization sequence with short echo time (20 ms), and a fully automated data processing scheme were used. Baseline plasma PHE concentrations were 1.04 (0.70-1.39) mmol/L PHE with concurrent brain PHE concentrations of 0.27 (0.13-0.41) mmol/kg of wet weight resulting in a plasma/brain ratio of 4.12. Plasma and brain concentrations correlated significantly (Kendall tau b = 0.91, p < 0.01). During an oral load with a single dose of 100 mg L-PHE per kg of body weight in four patients, plasma levels steeply increased. Concurrent brain PHE increase was less steep, was significantly delayed, and still continued up to 20 h postload. Despite the proven rise in plasma and brain concentrations of PHE, neuropsychologic examinations revealed no impairment of attentional and fine motor abilities from preload up to 20 h postload.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Pietz J,Kreis R,Boesch C,Penzien J,Rating D,Herschkowitz Ndoi
10.1203/00006450-199511000-00005subject
Has Abstractpub_date
1995-11-01 00:00:00pages
657-63issue
5eissn
0031-3998issn
1530-0447journal_volume
38pub_type
杂志文章abstract::BackgroundAcute kidney injury (AKI) is common in pediatric inpatients and is associated with increased morbidity, mortality, and length of stay. Its early identification can reduce severity.MethodsTo create and validate an electronic health record (EHR)-based AKI screening tool, we generated temporally distinct develo...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.116
更新日期:2017-09-01 00:00:00
abstract::We studied the mechanisms by which C16:0 lysophosphatidylcholine (LPC) and albumin inhibit the surface activity of calf lung surfactant extract (CLSE) by using a pulsating bubble apparatus with a specialized hypophase exchange system, plus adsorption and Wilhelmy balance measurements. In the absence of inhibitors, CLS...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199907000-00015
更新日期:1999-07-01 00:00:00
abstract::Sudden infant death syndrome (SIDS) is multifactorial and may result from the interaction of a number of environmental, genetic, and developmental factors. We studied three major genes causing long QT syndrome in 42 Japanese SIDS victims and found five mutations, KCNQ1-K598R, KCNH2-T895M, SCN5A-F532C, SCN5A-G1084S, an...
journal_title:Pediatric research
pub_type: 杂志文章,多中心研究
doi:10.1203/PDR.0b013e3181841eca
更新日期:2008-11-01 00:00:00
abstract::The present study investigates the effects of polycythemia on renal hemodynamics and function in 15 anesthetized newborn dogs, 2-10 days of age. Microspheres were used to study renal blood flow. Experimental animals received an exchange transfusion with adult dog packed red blood cells resulting in an increase in hema...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198202000-00014
更新日期:1982-02-01 00:00:00
abstract::Alterations of the architecture of cerebral white matter in the developing human brain can affect cortical development and result in functional disabilities. A line scan diffusion-weighted magnetic resonance imaging (MRI) sequence with diffusion tensor analysis was applied to measure the apparent diffusion coefficient...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199810000-00019
更新日期:1998-10-01 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::We recently demonstrated that recombinant hGH exacerbates renal functional and structural injury in chronic puromycin aminonucleoside (PAN) nephropathy, an experimental model of glomerular disease. Therefore, we examined whether recombinant human (rh) IGF-I is a safer alternative for the treatment of growth failure in...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199601000-00002
更新日期:1996-01-01 00:00:00
abstract::The key enzymes in the synthesis of the naturally occurring polyamines, ornithine decarboxylase (ODC) and S-adenosyl methionine (SAM) decarboxylase, were investigated during cell growth and aging in fibroblast cultures from normal patients and patients with cystic fibrosis. A linear correlation between increased S-ade...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197703000-00009
更新日期:1977-03-01 00:00:00
abstract:BACKGROUND:The pathogenesis of BPD includes inflammation and oxidative stress in the immature lung. Corticosteroids improve respiratory status and outcome, but the optimal treatment regimen for benefit with low systemic effects is uncertain. METHODS:In a pilot dose escalation trial, we administered ≤5 daily doses of b...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-01343-z
更新日期:2021-01-19 00:00:00
abstract::The influence of maternal stress during pregnancy on the postpartum iron status and immune maturation of infants was investigated in a nonhuman primate model. Forty infant rhesus monkeys were generated from two types of disturbed pregnancies, early or late gestation stress, and compared with 24 undisturbed controls. P...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/pdr.0b013e318045be53
更新日期:2007-05-01 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::Escherichia coli which elaborate heat stable enterotoxin (ST) are a major cause of endemic diarrhea in infants. The reason(s) for this increased susceptibility of infants to ST-mediated diarrhea is unknown. We investigated the possibility that the immature (14 and 21 day old) rat small intestine is more sensitive to S...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198606000-00017
更新日期:1986-06-01 00:00:00
abstract::Phototherapy results in the conversion of bilirubin to more water-soluble isomers. Six clinically used phototherapy lamps which differ in their emission spectra have been compared in their ability to produce configurational and structural isomers of bilirubin in vitro. For all of the lamps, the initial rate of configu...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198407000-00021
更新日期:1984-07-01 00:00:00
abstract:BACKGROUND:Volatile organic compounds (VOCs) are hydrocarbons that originate within different healthy and diseased tissues. VOCs can be secreted into the circulation and then excreted in the urine and faeces. In the lungs, VOCs are locally produced and can be detected in exhaled breath. VOCs can be identified using non...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/s41390-020-0828-3
更新日期:2020-03-02 00:00:00
abstract:BACKGROUND:Though prenatal antidepressant exposure has been associated with adverse developmental outcomes, the extent to which the effects are due to prenatal drug exposure or underlying maternal mood disturbances is unclear. METHODS:This was a population-based retrospective cohort study using administrative data fro...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-01269-6
更新日期:2020-12-09 00:00:00
abstract::Sildenafil (Viagra) has been shown to be an effective pulmonary vasodilator and is increasingly used in patients with pulmonary hypertension. Its effects on the cerebral circulation are unclear and have not yet been described. We investigated the effect of i.v. sildenafil treatment on cerebral oxygenation in 13 childr...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/01.pdr.0000198772.26417.66
更新日期:2006-03-01 00:00:00
abstract:BACKGROUND:Observational studies suggest that asthma control improves after adenotonsillectomy, but longitudinal studies that correlate the effect of the procedure on the levels of biomarkers associated with airway inflammation are limited. METHODS:We conducted a longitudinal, observational study on pediatric patients...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.237
更新日期:2014-03-01 00:00:00
abstract::The flavin-containing monooxygenases (FMOs) are important for the metabolism of numerous therapeutics and toxicants. Six mammalian FMO genes (FMO1-6) have been identified, each exhibiting developmental and tissue- and species-specific expression patterns. Previous studies demonstrated that human hepatic FMO1 is restri...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200202000-00018
更新日期:2002-02-01 00:00:00
abstract::Genetic variants of surfactant protein B (SP-B) have been associated with respiratory distress syndrome (RDS) in the prematurely born infant. We wished to determine linkage between RDS and SP-B single nucleotide polymorphisms (SNPs) [-18 (A/C), 1013 (A/C), 1580 (C/T), and 9306 (A/G)] or SP-B-linked microsatellite [(D2...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000203145.48585.2c
更新日期:2006-04-01 00:00:00
abstract::Severe tetrahydrobiopterin (BH4) deficiency is a naturally occurring model of cerebral catecholamine and serotonin shortage. Examination of the stimulated release and physiologic secretion pattern of several hormones in affected individuals permits certain conclusions concerning the involvement of these neurotransmitt...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199804000-00006
更新日期:1998-04-01 00:00:00
abstract::Myocardial oxygen demand may be increased in chronically hypoxemic lambs because of their increased heart rate. Therefore, we determined whether left ventricular (LV) oxygen supply, oxygen uptake and oxygen demand were proportionally increased in 6-wk-old lambs, after 4 wk of hypoxemia (n = 15), as compared with contr...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199310000-00017
更新日期:1993-10-01 00:00:00
abstract::Alterations in the pancreatic secretion of fluid and of enzymes in response to either pilocarpine (15 mg/kg) or an octapeptide of cholecystokinin (0.1 microgram/kg) have been found in rats that received daily injections of reserpine (0.5 mg/kg) for 7 days. During a 3-hr secretory period, significant reductions in the ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198109000-00015
更新日期:1981-09-01 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:BACKGROUND:Cardiovascular instability immediately after birth is associated with intraventricular hemorrhage (IVH) in very-low-birth-weight (VLBW) infants. For circulatory management, evaluation of organ blood flow is important. In this study, the relationship between peripheral perfusion within 48 h after birth and IV...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.215
更新日期:2014-02-01 00:00:00
abstract::Serum copper (Cu) and zinc (Zn) concentrations were measured in neonates with an appropriate birth weight for gestational age (AGA) and in small-for-gestational age infants. At 7 days of age, there was a positive correlation between serum Cu concentration and gestational age (GA) (r = 0.63; P < 0.001) and a negative c...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198009000-00005
更新日期:1980-09-01 00:00:00
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::The perinatal ontogeny of preprotachykinin-A gene expression was assessed in central respiration-related structures. On the day of birth, there was an enhanced expression of preprotachykinin-A mRNA in the region of the nucleus tractus solitarii, the primary relay station for respiratory and cardiovascular reflexes. Th...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199104000-00008
更新日期:1991-04-01 00:00:00
abstract::In eight newborn lambs, the adaption of blood to acidosis was studied by sequential measurements of P50 in vitro (pH 7.4; 37 degrees C) and P50 in vivo (animal's pH and temperature, 39 degrees C) during the course of HCl-induced metabolic acidosis. The benefit of the change in both P50 on tissue oxygen tension was stu...
journal_title:Pediatric research
pub_type: 杂志文章
doi:
更新日期:1981-05-01 00:00:00
abstract::Monocyte chemoattractant protein-1 (MCP-1), acting through its C-C chemokine receptor 2 (CCR-2), has important roles in inflammation, angiogenesis, and wound repair. The individual and combined effects of inhaled nitric oxide (NO) and hyperoxia on lung MCP-1 and CCR-2 in relation to lung leukocyte dynamics are unknown...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200111000-00017
更新日期:2001-11-01 00:00:00
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