Abstract:
:The investigation was designed to explore the use of tests tapping reversible aspects of behavior and performance and to find out whether these data can be correlated with concurrent metabolic changes in different dietary phases. The four subjects had classic phenylketonuria, diagnosed on the basis of a high level of serum phenylalanine on a regular diet, and severe mental retardation. Three types of diet were used: the general institutional diet, a low phenylalanine diet, and a low phenylalanine diet with added L-phenylalanine. A position discrimination and reversal task was used. Blood samples were taken every week on the day before psychological testing, and one 24-hr urine sample was collected during each dietary phase. In subjects I and II performance deteriorated upon elevation of the serum phenylalanine level, but these changes were transient only, thus showing an adaptation to the effects of the high serum phenylalanine level. Subject III showed increased response latencies (slower test responses) during high phenylalanine diet phases and faster responses while on a low phenylalanine diet. The correlation between response time and phenylalanine level is highly significant (r = 0.86, P less than 0.001). On the low phenylalanine diet subject IV's responses were fast and her performance was very stable, which contrasted with erratic performance during the high phenylalanine phases. Calculations show a significant association with phenylalanine level, both for response time (r = 0.47, P less than 0.05) and for variability in response time within each session (r = 0.46, P less than 0.05). Biochemical variability was observed in metabolic responses to phenylalanine loading, especially in the excretion of tryptophan metabolites.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Anderson VE,Siegel FSdoi
10.1203/00006450-197601000-00003subject
Has Abstractpub_date
1976-01-01 00:00:00pages
10-7issue
1eissn
0031-3998issn
1530-0447journal_volume
10pub_type
杂志文章abstract::To date, many mutations, including intronic nucleotide changes, in the SLC12A3 gene encoding the thiazide-sensitive sodium-chloride cotransporter (NCCT) have been reported in Gitelman's syndrome (GS) patients. However, it has not been clarified whether intronic nucleotide changes affect mRNA content. Since mRNA analys...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/pdr.0b013e318031935a
更新日期:2007-04-01 00:00:00
abstract::Inasmuch as taurine biosynthesis is decreased during early postnatal life, an efficient mechanism for taurine uptake by the liver must be present to maintain intracellular stores of this beta-amino acid. Therefore, basolateral liver plasma vesicles prepared from 14-day and adult rats were used to examine taurine trans...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198802000-00009
更新日期:1988-02-01 00:00:00
abstract::This report deals with quantitative and qualitative investigations of alkaline phosphatase in two unrelated infants with the severe infantile form of hypophosphatasia. Both affected infants had no detectable leukocyte alkaline phosphatase activities and both sets of parents and one sibling tended to have low but varia...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197607000-00004
更新日期:1976-07-01 00:00:00
abstract::D-myo-Inositol 1,4,5-triphosphate (IP3) is a key second messenger in many cells, including macrophages, T and B cells, and neutrophils, in which it regulates free intracellular calcium ion levels. In human polymorphonuclear leukocytes the rise of intracellular [Ca2+] is the signal that activates a number of functions ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199510000-00015
更新日期:1995-10-01 00:00:00
abstract::Newborn rats exposed to 60% O(2) for 14 d demonstrated a bronchopulmonary dysplasia-like lung morphology and pulmonary hypertension. A 21-aminosteroid antioxidant, U74389G, attenuated both pulmonary hypertension and macrophage accumulation in the O(2)-exposed lungs. To determine whether macrophage accumulation played ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200108000-00003
更新日期:2001-08-01 00:00:00
abstract:BACKGROUND:Appearance of sleep cycling has been associated with good outcome in term and preterm infants, but the normal time of its appearance has not been determined. The objectives of this study were, to correlate the time of sleep cycling appearance and the length of quiet sleep in neonates with different degrees o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0560-z
更新日期:2020-03-01 00:00:00
abstract::Five enzymes were measured in 50 liver specimens (18 normal liver, 20 Reye liver, 12 diverse liver disorders other than Reye syndrome). The enzymes were: glutamic dehydrogenase (E.C. 1.4.1.3), monoamine oxidase (E.C. 1.4.3.4), lactate dehydrogenase (E.C. 1.1.1.27), D-glucose-6-phosphate dehydrogenase (E.C. 1.1.1.49), ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198501000-00006
更新日期:1985-01-01 00:00:00
abstract::High-frequency oscillatory ventilation (HFOV) may improve pulmonary outcome in very preterm infants, but the effects on the brain are largely unknown. We hypothesized that early prolonged HFOV compared with low volume positive pressure ventilation (LV-PPV) would not increase the risk of delayed brain growth or injury ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181bb0cc1
更新日期:2009-11-01 00:00:00
abstract::Argininosuccinate synthetase and argininosuccinate lyase catalyze the conversion of citrulline to arginine in kidney. Immunohistochemical staining of mouse kidney sections with antibodies to these two enzymes, compared with the staining patterns of known markers for proximal tubules, demonstrated that these enzymes ar...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199102000-00010
更新日期:1991-02-01 00:00:00
abstract:BACKGROUND:Bradycardia and oxygen desaturation episodes are common among preterm very low birth weight (VLBW) infants in the Neonatal Intensive Care Unit (NICU), and their association with adverse outcomes such as bronchopulmonary dysplasia (BPD) is unclear. METHODS:For 502 VLBW infants we quantified bradycardias (HR ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-018-0223-5
更新日期:2019-06-01 00:00:00
abstract:BACKGROUND:The prevalence of autism spectrum disorders (ASD) is 5-fold higher in preterm (PT) infants born ≤28 weeks gestational age (GA) as compared to the general population. The relationship between placental pathologic lesions and ASD in PT infants has not been studied. OBJECTIVES:The objective of this study was t...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-01160-4
更新日期:2020-09-19 00:00:00
abstract::A 2-month-old white girl had severe liver disease (but without signs of hepatic necrosis, infection or cirrhosis), urinary cytomegalovirus, transient reduction of alpha 1-antitrypsin concentration and transient abnormal alpha 1-antitrypsin phenotype that were not present in her parents. Five serum specimens that were ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198203000-00006
更新日期:1982-03-01 00:00:00
abstract::In order to study the neonatal microcirculation, the capillary hemodynamics in skin was investigated in 43 full-term infants 2-7 days after birth. The nailfold capillaries of the thumb were visualized by means of television microscopy and the capillary blood cell velocity (CBV) was videophotometrically quantified in 1...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198806000-00011
更新日期:1988-06-01 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::Lung infections are a common cause of morbidity and mortality in neonates. To evaluate neonatal lung defenses against pneumococci, we challenged rats with aerosols of encapsulated pneumococci in an airborne infection apparatus. Whereas adult rats cleared greater than 95% of inhaled type 1 or type 25 pneumococci within...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198712000-00025
更新日期:1987-12-01 00:00:00
abstract:BACKGROUND:Sepsis is an important cause of neonatal morbidity and mortality worldwide. Diagnosis and treatment of neonatal sepsis relies on clinical judgment and interpretation of nonspecific laboratory tests. In a prospective cohort, we measured inflammatory cytokines as a potential biomarker for neonatal sepsis. MET...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2016.267
更新日期:2017-04-01 00:00:00
abstract::Sparse-fur mice, 28 d of age with the x-chromosomal inherited defect of ornithine carbamoyltransferase, were used to investigate if tryptophan and the serotonin pathway in the brain are affected in this animal model which closely resembles the human inborn error of metabolism. Increased concentrations of tryptophan an...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198404000-00014
更新日期:1984-04-01 00:00:00
abstract::Although fetal asphyxia, i.e. hypoxemia, acidosis, and hypercapnia, increases plasma arginine vasopressin (AVP) greater than 40-fold, hypoxemia and metabolic acidosis occurring independently cause only 5-fold and 2-fold increases, respectively. To determine the effects of hypercapnia on AVP release, we examined the ef...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199110000-00014
更新日期:1991-10-01 00:00:00
abstract::The previous biochemical evidence had suggested that glutaric aciduria type II (GA II) is due to deficient dehydrogenation of multiple short-chain acyl coenzyme A's (CoA's), bu the precise biochemical mechanism underlying this disease was unknown. We investigated substrate oxidation and in vitro activities of isovaler...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198012000-00013
更新日期:1980-12-01 00:00:00
abstract::Despite the availability of successful prevention strategies to prevent excessive hyperbilirubinemia, the neurological sequelae of bilirubin neurotoxicity (BNTx) still occur throughout the world. Kernicterus, encephalopathy due to BNTx, is now understood to be a spectrum of severity and phenotypes known as kernicterus...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/s41390-019-0608-0
更新日期:2020-01-01 00:00:00
abstract:BACKGROUND:Diseases of adulthood, such as diabetes and hypertension, may be related to changes during pregnancy, particularly in kidney. We hypothesized that acute kidney injury progresses more rapidly in cases of fetal programming. METHODS:Diabetic dams' offspring were divided into: CC (controls, receiving vehicle); ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2014.205
更新日期:2015-03-01 00:00:00
abstract::We investigated the developmental changes in the pattern of urinary metabolites of theophylline, a substrate for CYP1A2, to study when CYP1A2, which is absent in the perinatal period, fully develops during childhood. The urinary ratios of three metabolites (1-methyluric acid, 3-methylxanthine, and 1,3-dimethyluric aci...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199901000-00011
更新日期:1999-01-01 00:00:00
abstract::Several hours after an hypoxic-ischemic injury to the developing brain, hyperemia, then seizures, edema, and infarction can develop. The roles of nitric oxide (NO) synthesis and excitotoxin accumulation during these later phases of injury are not known. The time course of extracellular levels of amino acids within the...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199605000-00008
更新日期:1996-05-01 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::We measured body composition, basal metabolic rate (BMR), and total energy expenditure in 28 nonobese and 35 obese adolescents aged 12-18 y using indirect calorimetry and the doubly labeled water method. BMR was highly correlated with fat-free mass in both the nonobese and obese groups (r = 0.77 and 0.84, respectively...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199002000-00022
更新日期:1990-02-01 00:00:00
abstract::The fetal respiratory and electrocortical effects of thyrotropin-releasing hormone (TRH) administered into the lateral cerebral ventricles, have been investigated in chronically catheterized unanesthetized fetal sheep at 125-140 days of gestation. Stimulatory effects on fetal breathing movements were seen at doses as ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198801000-00016
更新日期:1988-01-01 00:00:00
abstract::Premature rabbits delivered by cesarean section at 28 d of gestation were each given intratracheally 75 mg/kg of radiolabeled preparation of either natural rabbit surfactant, natural calf surfactant, or surfactant-TA. Each newborn rabbit was ventilated for up to 6 h in a ventilator-plethysmograph with individual adjus...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198904000-00024
更新日期:1989-04-01 00:00:00
abstract::Vitamin A and its precursor beta-carotene are naturally occurring antioxidants. The effects of diets deficient in beta-carotene and vitamin A on the alveolar-capillary membrane were studied in young adult BALB/C mice before and after exposure to 65% oxygen. One of three diets (standard complete, beta-carotene deficien...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198801000-00017
更新日期:1988-01-01 00:00:00
abstract::Kawasaki disease (KD) is an acute vasculitis of young childhood predominantly affecting the coronary arteries. IgA plasma cells have been found to infiltrate vascular and nonvascular tissues in fatal acute KD. To determine whether IgA B-lymphocytes were increased in the peripheral blood of patients with KD, we perform...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200110000-00019
更新日期:2001-10-01 00:00:00
abstract::Developmental disability, intrauterine growth retardation, renal anomalies, and dysmorphic features have been described in offspring of women who abuse toluene during pregnancy. A Sprague-Dawley rat model was developed to study this clinical syndrome. During d 6-19 of gestation, 11 treated dams received daily gavage d...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199412000-00023
更新日期:1994-12-01 00:00:00