Brain development in infantile-onset Pompe disease treated by enzyme replacement therapy.

abstract：:Acute kidney injury (AKI) is common in premature infants and is associated with poor outcomes. Novel biomarkers can detect AKI promptly. Because premature infants are born with underdeveloped kidneys, baseline biomarker values may differ. We describe baseline values of urinary neutrophil gelatinase-associated lipocali...

journal_title：Pediatric research

authors： Askenazi DJ,Koralkar R,Levitan EB,Goldstein SL,Devarajan P,Khandrika S,Mehta RL,Ambalavanan N

更新日期：2011-09-01 00:00:00

abstract：:The head sizes of 38 patients, growth hormone (GH) deficient following craniospinal (n = 26) or cranial irradiation (n = 12), have been assessed before (n = 38) and on completion of GH therapy (n = 15) or at the end of a similar period of observation without GH (n = 7). These results were compared to the change in hea...

journal_title：Pediatric research

authors： Clayton PE,Shalet SM,Price DA,Surtees RA,Pearson D

更新日期：1987-10-01 00:00:00

abstract：:Gastrointestinal (GI) blood flow, O2 transport, and O2 uptake were measured during recovery from severe hypoxemia in newborn piglets. Hypoxemia was induced by lowering the inspired O2 concentration to 0.05 for 15 min. This resulted in an 82% decrease in GI O2 uptake. Recovery measurements were obtained 5 and 65 min af...

journal_title：Pediatric research

authors： Nowicki PT,Miller RR,Hansen NB,Hayes JR

更新日期：1985-11-01 00:00:00

abstract：BACKGROUND:Infantile hemangioma (IH) is the most frequent vascular tumor of early childhood. Recently, propranolol, a nonselective β1- and β2-adrenoceptor inhibitor, was introduced into the therapy of severe proliferating IH with excellent results. However, the underlying mechanism of action of propranolol is still unc...

journal_title：Pediatric research

authors： Rössler J,Haubold M,Gilsbach R,Jüttner E,Schmitt D,Niemeyer CM,Hein L

更新日期：2013-04-01 00:00:00

abstract：:To elucidate the impact of nutrition in cystic fibrosis (CF), we compared the phenotypic traits of Cftr -/- mice fed either a lipid-enriched liquid diet (Peptamen) or a standard chow combined with polyethylenglycol osmotic laxative (PEG), two strategies commonly used to prevent intestinal obstruction in CF mice. Survi...

journal_title：Pediatric research

authors： Cottart CH,Bonvin E,Rey C,Wendum D,Bernaudin JF,Dumont S,Lasnier E,Debray D,Clément A,Housset C,Bonora M

更新日期：2007-11-01 00:00:00

abstract：:The effect of Cis-platin on the glomerular filtration rate and effective renal plasma flow was determined using a radioisotope clearance technique in young (3 wk old) and adult (more than 12 wk old) rats. Cis-platin was administered intravenously in dosages ranging from 2.5 to 10 mg/kg body weight, either as a single ...

journal_title：Pediatric research

authors： Jongejan HT,Provoost AP,Wolff ED,Molenaar JC

更新日期：1986-01-01 00:00:00

abstract：BACKGROUND:The preterm newborn is at high risk of developing cardiovascular compromise during the first day of life and this is associated with increased risk of brain injury. Standard treatments are volume expansion and administration of inotropes, typically dopamine and/or dobutamine, but there is limited evidence th...

journal_title：Pediatric research

authors： Eiby YA,Shrimpton NY,Wright IM,Lumbers ER,Colditz PB,Duncombe GJ,Lingwood BE

更新日期：2016-12-01 00:00:00

abstract：:We have investigated the impaired secretion response of neonatal platelets. We compared the response of washed neonatal and adult platelets to thrombin and collagen, and to specific activators of calcium flux (inositol trisphosphate) and protein kinase C activation (oleoyl-acetyl glycerol). Neonatal platelets show no ...

journal_title：Pediatric research

authors： Israels SJ,Daniels M,McMillan EM

更新日期：1990-04-01 00:00:00

abstract：:Alveolar macrophages were isolated from three cystic fibrosis patients, and the structure and function of these cells were compared to that of normal alveolar macrophages. The cystic fibrosis (CF) and normal alveolar macrophages were able to phagocytize Pseudomonas in the presence of normal serum, but cells from both ...

journal_title：Pediatric research

authors： Thomassen MJ,Demko CA,Wood RE,Tandler B,Dearborn DG,Boxerbaum B,Kuchenbrod PJ

更新日期：1980-05-01 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

authors： Seidner SR,Jobe AH,Ruffini L,Ikegami M,Pettenazzo A

更新日期：1989-04-01 00:00:00

abstract：:The purpose of this study was to determine the effect of chest wall distortion on esophageal manometry by measuring simultaneous esophageal pressure changes at two sites in preterm infants. Fourteen infants were studied (mean +/- SD; birth weight, 1340 +/- 260 g; age, 8.5 +/- 4 d). Esophageal pressure was measured thr...

journal_title：Pediatric research

authors： Silva Neto G,Gerhardt TO,Claure N,Duara S,Bancalari E

更新日期：1995-05-01 00:00:00

abstract：:We investigated the relative contribution of the two pathways of phosphatidylcholine (PC) synthesis in lungs from chick embryos of 12 to 20 days of incubation as well as from hatched chicks and adult chickens. Lung "blocks" were incubated for 60 min in vitro in the presence of [3H]methionine and [14C]choline. PC was s...

journal_title：Pediatric research

authors： Compton SK,Goeringer GC

更新日期：1981-05-01 00:00:00

abstract：:To study the normal development of blood coagulation factor activities in a growing fetus while avoiding the effects of labor and delivery, a chronic fetal lamb model was developed in which serial blood samples from 10 fetuses were studied during the third trimester of pregnancy and 24 hr after birth. Under operating ...

journal_title：Pediatric research

authors： Kisker CT,Robillard JE,Clarke WR

更新日期：1981-07-01 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

authors： McNab TC,Tseng YT,Stabila JP,McGonnigal BG,Padbury JF

更新日期：2001-11-01 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

authors： Lindenskov PH,Castellheim A,Aamodt G,Saugstad OD,Mollnes TE

更新日期：2004-11-01 00:00:00

abstract：BACKGROUND:Increasing numbers of preterm-born children survive nowadays, and improving long-term health and neurodevelopment is becoming more important. Early-life growth has been linked to neurodevelopmental outcomes. We aimed to study whether this association has changed with time. METHODS:We studied two cohorts of ...

journal_title：Pediatric research

authors： Ruys CA,Hollanders JJ,Bröring T,van Schie PEM,van der Pal SM,van de Lagemaat M,Lafeber HN,Rotteveel J,Finken MJJ

更新日期：2019-02-01 00:00:00

abstract：BACKGROUND:Kawasaki disease (KD) is associated with expression and methylation of Fc gamma receptor genes. We characterized immunoglobulin A (IgA), IgE, IgG, and IgM receptor expression levels in KD. METHODS:Fc receptor expression levels were characterized using GeneChip Human Transcriptome Array 2.0 (HTA 2.0) with 18...

journal_title：Pediatric research

authors： Chang LS,Ming-Huey Guo M,Lo MH,Kuo HC

更新日期：2019-12-09 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

authors： Ballard PL,Torgerson D,Wadhawan R,Hudak ML,Weitkamp JH,Harris J,Asselin J,Chapin C,Ballard RA,McEvoy CT

更新日期：2021-01-19 00:00:00

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

authors： Goldin E,Blanchette-Mackie EJ,Dwyer NK,Pentchev PG,Brady RO

更新日期：1995-06-01 00:00:00

abstract：:Mother's milk represents a foundational step in the proper development of newborn immunity. This is achieved, in part, through the action of numerous regulatory proteins such as soluble cluster of differentiation 14 (sCD14) found in significant quantities in human milk (~25-50 μg/mL). In adults, CD14 stimulates cytoki...

journal_title：Pediatric research

authors： Spencer WJ,Binette A,Ward TL,Davis LD,Blais DR,Harrold J,Mack DR,Altosaar I

更新日期：2010-12-01 00:00:00

abstract：:The history of Pediatric Infectious Diseases closely parallels the history of Pediatrics at least until the last century, because historically infections comprised the major causes of childhood morbidity and mortality, as they still do in the developing world. This history reviews developments in the field through the...

journal_title：Pediatric research

authors： Shulman ST

更新日期：2004-01-01 00:00:00

abstract：BACKGROUND:The basal ganglia are vulnerable to injury during cardiac arrest. Movement disorders are a common morbidity in survivors. Yet, neuronal motor network changes post-arrest remain poorly understood. METHODS:We compared function of the motor network in adult rats that, during postnatal week 3, underwent 9.5 min...

journal_title：Pediatric research

authors： Aravamuthan BR,Shoykhet M

更新日期：2015-10-01 00:00:00

abstract：BACKGROUND:Protein intake may modulate cardiac structure and function in pathological conditions, but there is a lack of knowledge on potential effects in healthy infants. METHODS:Secondary analysis of an ongoing randomized clinical trial comparing two groups of infants receiving a higher (HP) or lower (LP) protein co...

journal_title：Pediatric research

authors： Collell R,Closa-Monasterolo R,Ferré N,Luque V,Koletzko B,Grote V,Janas R,Verduci E,Escribano J

更新日期：2016-06-01 00:00:00

abstract：:The effect of ingestion of uncooked cornstarch (2 g/kg body weight) in water, uncooked starch (1 g/kg) added to a meal, and glucose (2 g/kg) in water, was studied in eight patients with type IA glycogen storage disease (GSD) and one patient with type IB GSD. Blood glucose concentrations were determined at 30-min inter...

journal_title：Pediatric research

authors： Smit GP,Berger R,Potasnick R,Moses SW,Fernandes J

更新日期：1984-09-01 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

authors： Goto K,Sugiyama K,Li R,Miyake Y,Ando T,Mizutani F,Terabe K,Mizuno K,Wada Y

更新日期：1999-01-01 00:00:00

abstract：:In this study, we have investigated the possible role of the pro-aggregatory arachidonic acid (AA) metabolite thromboxane, in the impaired function of neonatal platelets. In platelet-rich plasma thromboxane production (measured by radioimmunoassay of thromboxane B2) was not different between neonates and adults when s...

journal_title：Pediatric research

authors： Stuart MJ,Dusse J,Clark DA,Walenga RW

更新日期：1984-09-01 00:00:00

abstract：:Ethanol consumption during pregnancy can cause fetal alcohol syndrome (FAS). Although the exact mechanism is unknown, nutritional alterations caused by ethanol exposure may be an etiologic factor in FAS. The congenital heart defects seen in FAS are similar to those found in vitamin A teratogenesis. Because ethanol ing...

journal_title：Pediatric research

authors： DeJonge MH,Zachman RD

更新日期：1995-04-01 00:00:00

abstract：:The use of erythrocyte entrapment as a strategy to deliver and protect exogenously administered enzymes for replacement therapy in selected genetic diseases has been evaluated in a mammalian model system. The uptake, tissue distribution, intracellular localization, and in vivo lifetime of erythrocyte-entrapped bovine ...

journal_title：Pediatric research

authors： Thorpe SR,Fiddler MB,Desnick RJ

更新日期：1975-12-01 00:00:00

abstract：:We used in vivo microdialysis to determine the impact of a focal hypoxic-ischemic insult on hippocampal amino acid efflux in the immature brain. Microdialysis probes were inserted into the right hippocampus of postnatal d 7 rats. To induce hypoxic-ischemic injury, the right carotid artery was ligated and animals were ...

journal_title：Pediatric research

authors： Silverstein FS,Naik B,Simpson J

更新日期：1991-12-01 00:00:00

abstract：:Hirschsprung's disease, affecting one in 5000 live newborns, is the most common cause of neonatal intestinal obstruction. The obstruction or, later in life, constipation arises from the lack of enteric ganglia in the hindgut, thus resulting in poor coordination of peristalsis. Mutations in Hirschsprung patients have s...

journal_title：Pediatric research

authors： Svensson PJ,Von Tell D,Molander ML,Anvret M,Nordenskjöld A

更新日期：1999-05-01 00:00:00