Abstract:
:Cerebral magnetic resonance imaging (MRI) has revealed white matter changes in patients with phenylketonuria (PKU), an inborn error of metabolism with increased plasma phenylalanine level. Because the significance of these lesions is unknown, this study was undertaken to determine whether glucose metabolism was depressed in cerebral white matter MRI changes in patients with PKU. Four patients with PKU and nine healthy volunteers with an average age of 23 y (range 19-26 y) and 23 y (range 20-27 y), respectively, were studied. The IQ of patients with PKU was between 58 and 97. Cerebral MRI and positron emission tomography images with 18F-deoxyglucose were obtained, and arteriovenous differences for oxygen and glucose as well as cerebral blood flow was measured simultaneously to determine global cerebral oxygen and glucose metabolism. Cerebral MRI revealed that all patients with PKU had white matter changes with characteristic localization. In patients with PKU, regional glucose metabolism was 36% lower in the anterior periventricular areas, 0.14 +/- 0.06 compared with 0.22 +/- 0.04 mumol.g-1.min-1 in controls (mean +/- SD, p < 0.05, Mann-Whitney). Further, the ratio between glucose metabolism in the affected white matter and the cortex was 14% lower in the patients, decreasing from 0.57 +/- 0.05 to 0.48 +/- 0.06 (p < 0.05). Global cerebral blood flow, oxygen and glucose consumption were similar in the two groups. In conclusion, regional glucose metabolism is lower in MRI-demonstrated white matter changes. In mildly intellectually impaired patients with PKU, global cerebral glucose and oxygen metabolism remain intact.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Hasselbalch S,Knudsen GM,Toft PB,Høgh P,Tedeschi E,Holm S,Videbaek C,Henriksen O,Lou HC,Paulson OBdoi
10.1203/00006450-199607000-00004subject
Has Abstractpub_date
1996-07-01 00:00:00pages
21-4issue
1eissn
0031-3998issn
1530-0447journal_volume
40pub_type
杂志文章abstract::Several epithelial ion transporters are developmentally regulated in the preweaning period, at the time when the circulating levels of glucocorticoid and mineralocorticoid hormones increase. The specific role of glucocorticoids and mineralocorticoids in the maturation of epithelial ion transport is still disputed. In ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199508000-00005
更新日期:1995-08-01 00:00:00
abstract::To clarify the prevalence of Japanese children thought to be at a risk for infection with GB virus-C (GBV-C)/hepatitis G virus (HGV), we investigated the detection rates of serum GBV-C/ HGV ribonucleic acid (RNA) by reverse transcription-seminested PCR and serum anti-HGV-E2 antibody by ELISA in 162 children with histo...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/00006450-199901000-00021
更新日期:1999-01-01 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::Topical emollient therapy may reduce the incidence of serious infections and mortality of preterm infants in developing countries. We tested whether emollient therapy reduced the burden of pathogens on skin and/or prevented bacterial translocation. Neonates <33 wk gestational age were randomized to treatment with sunf...
journal_title:Pediatric research
pub_type: 杂志文章,随机对照试验
doi:10.1203/pdr.0b013e3180459f75
更新日期:2007-05-01 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::Altered pulmonary vascular resistance might be a factor for delayed closure of the ductus arteriosus (DA) in preterm infants. Angiotensin II plays a central role in the elevation of pulmonary vascular resistance. Angiotensin II exerts its vasoconstrictor effect on the angiotensin II type 1 receptor (AT1R). Homozygous ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000088016.67117.39
更新日期:2003-11-01 00:00:00
abstract::The placenta is essential to nutrition before birth. Recent work has shown that a range of clearly defined alterations can be found in the placentas of infants with intrauterine growth restriction (IUGR). In the mouse, a placental specific knockout of a single imprinted gene, encoding IGF-2, results in one pattern of ...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/01.PDR.0000181381.82856.23
更新日期:2005-11-01 00:00:00
abstract::The classical model of gene regulation by hormones involves a hormone-bound receptor interacting with a DNA response element to increase or decrease gene transcription. Steroid hormone regulation more commonly involves atypical cis-elements, co-receptors, accessory proteins, and unique modes of interaction on differen...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200111000-00007
更新日期:2001-11-01 00:00:00
abstract::The metabolism of proteoglycans in normal growth plate and the changes in growth plate morphology induced by diabetes and malnutrition were studied in rats. The proteoglycans had a significantly faster turnover (half-life measured with [35S]sulfate labeling: 25-30 h) than the cells in the growth plate. Morphometric st...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199001000-00014
更新日期:1990-01-01 00:00:00
abstract:BACKGROUND:Postnatal glucocorticoid therapy in the treatment of chronic lung disease benefits lung function, however it adversely affects brain development. We hypothesized that combined postnatal glucocorticoid and statin therapy diminishes adverse effects of glucocorticoids on the developing brain. METHODS:On postna...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.152
更新日期:2013-12-01 00:00:00
abstract::The mitochondrial trifunctional protein (TFP) is a multienzyme complex of the beta-oxidation cycle. Human TFP is an octamer composed of four alpha-subunits harboring long-chain enoyl-CoA hydratase and long-chain L-3-hydroxyacyl-CoA dehydrogenase and four beta-subunits encoding long-chain 3-ketoacyl-CoA thiolase. Mutat...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000103931.80055.06
更新日期:2004-02-01 00:00:00
abstract::Low IGF-I levels are found in patients with chronic liver disease, but it is not known whether these reductions in IGF-I are secondary to hepatic dysfunction or to malnutrition. To determine whether malnutrition associated with hepatic dysfunction causes the decrease in these levels, serum and liver IGF-I concentratio...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198911000-00009
更新日期:1989-11-01 00:00:00
abstract::Sequential magnitude and the rate of change in tracheal mechanics after application of intermittent positive pressure (IPP), and the time constant, tau, of this deformation were determined in fetal (21 and 27 days gestation), neonatal (term, 31 days gestation) and adult rabbits. In vitro tracheal mechanics were determ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198210000-00006
更新日期:1982-10-01 00:00:00
abstract::Carlo Agostoni, MD, and Kwang Sik Kim, MD, are the Guest Editors for this annual review issue on Nutrition and the Microbiome. Dr Agostoni is a Professor of Pediatrics at the Department of Clinical Sciences and Community Health, University of Milan, within the Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, ...
journal_title:Pediatric research
pub_type: 历史文章
doi:10.1038/pr.2014.195
更新日期:2015-01-01 00:00:00
abstract::Music is widely used to enhance well-being. We wished to assess music's effect on pediatric oncology outpatients. Patients who had leukemia and were in maintenance or consolidation outpatient treatment served as their own control at two visits. At visit 1, children rested for 20 min; at visit 2, for 20 min they listen...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e318174e6fb
更新日期:2008-07-01 00:00:00
abstract::Values of IGF-I after extraction, its binding proteins, and the high affinity GH-binding protein (BP) are not well established in pediatric patients with insulin-dependent diabetes mellitus (IDDM). We report data for IGF-I, IGFBP-1, and -3, and GHBP in 92 Spanish children with IDDM, separated according to pubertal sta...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199606000-00011
更新日期:1996-06-01 00:00:00
abstract::We studied the effects of exogenously administered adrenomedullin on fetal pulmonary arterial blood flow in near-term fetal sheep. The hemodynamic effects of a single injection of adrenomedullin into the left pulmonary artery were compared with those of acetylcholine; the effects of repeated injections of adrenomedull...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199704000-00007
更新日期:1997-04-01 00:00:00
abstract::Pesticides are chemicals that are designed specifically for the purpose of killing or suppressing another living organism. Human toxicity is possible with any pesticide, and a growing body of literature has investigated possible associations with neurodevelopmental disorders. Attention deficit disorder with or without...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/s41390-018-0200-z
更新日期:2019-01-01 00:00:00
abstract::Various assay methods for detection of thyroid hormones in human milk were evaluated in recovery and dilution experiments after which the concentrations of thyroxine (T4) and 3,5,3'-triiodothyronine (T3) were measured and compared with those in serum. The effect of breast feeding on pituitary thyroid function of norma...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198306000-00008
更新日期:1983-06-01 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::Patency of the ductus arteriosus in preterm infants is mediated by vasodilating prostanoids; however, reliable methods to monitor prostanoid activity or production in preterm infants are lacking. We measured the excretion rates of major and characteristic urinary metabolites of prostacyclin (PGI2), PGE1, and PGE2, 6-k...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198406000-00007
更新日期:1984-06-01 00:00:00
abstract::The worldwide burden of disease due to Epstein-Barr virus (EBV) infection is enormous. Diseases include endemic Burkitt lymphoma, infectious mononucleosis, cancers after transplantation, Hodgkin lymphoma, and nasopharyngeal carcinoma. A prophylactic EBV vaccine has the potential to significantly reduce the incidence a...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/s41390-019-0591-5
更新日期:2020-01-01 00:00:00
abstract::Prenatal maternal therapy with glucocorticoid reduces the incidence of respiratory distress syndrome (RDS) in premature infants. To investigate the effects of this treatment on the fetal endocrine system, we determined serum concentrations of betamethasone, cortisol, dehydroepiandrosterone sulfate, growth hormone, and...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198002000-00011
更新日期:1980-02-01 00:00:00
abstract:BACKGROUND:To minimize secondary lung injury, ventilated preterm infants are extubated as soon as possible. To maximize extubation success, they are often placed in prone position. The effect of extubation and subsequent prone positioning on lung volumes is currently unknown. METHODS:Changes in end-expiratory lung vol...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1038/pr.2014.201
更新日期:2015-03-01 00:00:00
abstract::Intracellular levels of cysteine (CSH) and reduced glutathione (GSH) in the kidney and liver of rats from the newborn to the adult period have been determined using a sensitive high performance liquid chromatography method. In the kidney, the intracellular level of free CSH increased 4-fold from 1 to 4 nmol/mg protein...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198711000-00025
更新日期:1987-11-01 00:00:00
abstract::Aceruloplasminemia is an autosomal recessive disorder of iron metabolism characterized by diabetes, retinal degeneration, and neurologic symptoms. Affected patients evidence marked parenchymal iron accumulation in conjunction with an absence of circulating serum ceruloplasmin and molecular genetic analysis reveals inh...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/00006450-199809000-00001
更新日期:1998-09-01 00:00:00
abstract:BACKGROUND:Prodromal symptoms are frequently reported in the atypical form of Hemolytic uremic syndrome (aHUS) suggesting implication of infectious triggers. Some pathogens may also play a role in the mechanisms of production of autoantibody directed against Factor H (FH), a complement regulator, leading to aHUS. METH...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-018-0009-9
更新日期:2018-07-01 00:00:00
abstract::This study aimed to characterize early neonatal microvascular function after preeclamptic pregnancy with respect to infant sex and in utero growth. Peripheral microvascular blood flow was examined prospectively from 6 to 72 h of age using laser Doppler flowmetry in a cohort of term infants of normotensive women and wo...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/pdr.0b013e318193edf1
更新日期:2009-03-01 00:00:00
abstract::To assess the National Institutes of Health extramural activity in the area of pediatric nephrology research, the rate of submission of grant applications to the National Institutes of Health and available indicators of the quality of these applications were analyzed. This was accomplished by utilizing the grant appli...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198511000-00001
更新日期:1985-11-01 00:00:00
abstract::Human intestinal mucosa consists of highly active epithelial cells in continual renewal and differentiation processes located at different portions of the villi. The crypt contains abundant replicating cells which, upon reaching the villus tip, acquire their fully differentiated state. Besides its well recognized role...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199611000-00004
更新日期:1996-11-01 00:00:00