Abstract:
:Children with inborn errors of urea synthesis who survive neonatal hyperammonemic coma commonly exhibit cognitive deficits and neurologic abnormalities. Yet, there is evidence that ammonia is not the only neurotoxin. Hyperammonemia appears to induce a number of neurochemical alterations. In rodent models of hyperammonemia, uptake of L-tryptophan into brain is increased. It has been reported that in an experimental rat model of hepatic encephalopathy, in the ammonium acetate-injected rat, and in patients with hepatic failure and inborn errors of ammonia metabolism, quinolinate, a tryptophan metabolite, is increased. Elevations in quinolinate are of particular concern, as quinolinate could excessively activate the N-methyl-D-aspartate subclass of excitatory amino acid receptors, thereby causing selective neuronal necrosis. We sought to identify an animal model that would replicate the increases in quinolinate that have been associated with hyperammonemia in humans. Levels of quinolinate were measured in hyperammonemic urease-infused rats and ammonium acetate-injected rats. In the urease-infused rat, brain tryptophan was doubled, and serotonin and its metabolite 5-hydroxyindoleacetic acid were significantly increased. Yet, despite the increase in tryptophan and evidence for increased metabolism of tryptophan to serotonin, there were no observed increases of quinolinate in brain, cerebrospinal fluid, or plasma. In the ammonium acetate-injected rat, significant increases of 5-hydroxyindoleacetic acid in cerebral cortex were also observed, but quinolinate did not change in cerebrospinal fluid or cerebral cortex. In summary, we were unable to demonstrate an increase of quinolinate in brain or cerebrospinal fluid in these rat models of hyperammonemia.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Robinson MB,Heyes MP,Anegawa NJ,Gorry E,Djali S,Mellits ED,Batshaw MLdoi
10.1203/00006450-199210000-00021subject
Has Abstractpub_date
1992-10-01 00:00:00pages
483-8issue
4eissn
0031-3998issn
1530-0447journal_volume
32pub_type
杂志文章abstract::DNA segments that are separated from the promoter region of a gene by many thousands of bases may nonetheless regulate the transcriptional activity of that gene. This finding has led to the investigation of mechanisms underlying long-range chromatin interactions. In intermitotic cells, chromosomes decondense, filling ...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/pdr.0b013e31804575db
更新日期:2007-05-01 00:00:00
abstract::The mitochondria play a major role in the regulation of intracellular calcium. Despite the fact that the enterocytes receive the majority of absorbed calcium, the role of the intestinal mitochondria in calcium transport during maturation is not known. Therefore, the current studies were designed to characterize calciu...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198901000-00023
更新日期:1989-01-01 00:00:00
abstract::Phagocytosis of apoptotic cells, e.g., neutrophils, by monocytes is essential for resolution of inflammation. Delayed removal leads to secondary necrosis, perpetuating inflammation, and tissue destruction. Common histologic features in neonatal chronic inflammatory disorders are an accumulation of apoptotic cells in i...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181b9b470
更新日期:2009-11-01 00:00:00
abstract::Previous studies have reported differences in muscle function and metabolism between patients with cystic fibrosis (CF) and healthy controls (HC), but it is currently unknown whether these abnormalities are specific to CF or also seen in other airway diseases. In this study, we used magnetic resonance spectroscopy (MR...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181fff35f
更新日期:2011-01-01 00:00:00
abstract::Hypoxic-ischemic injuries can evolve over several days, and recent studies suggest that further neuronal death may occur 6 to 72 h later. Because cerebral temperature is an important determinant of outcome during the primary injury, we investigated the effect of temperature, on outcome, during the later phases of inju...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199604000-00005
更新日期:1996-04-01 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:Recent studies suggest that infantile hypertrophic pyloric stenosis (IHPS) and congenital heart defects (CHDs) may share some genetic risk factors, but little is known about the co-occurrence of the two conditions in patients. METHODS:Our study cohort included 2,212,756 persons born in Denmark 1977-2013. We...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0369-9
更新日期:2019-06-01 00:00:00
abstract::Spleens from 1-20-wk-old guinea pigs infected in utero with Treponema pallidum and age-matched controls, born to normal and heat-killed (56 degrees C, 2 h.) T. pallidum-injected mothers, were examined for their in vitro lymphoproliferative response to phytohemagglutinin, concanavalin A, and lipopolysaccharide. Additio...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199712000-00013
更新日期:1997-12-01 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: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
pub_type: 杂志文章
doi:10.1038/pr.2015.114
更新日期:2015-10-01 00:00:00
abstract::Thyroid hemiagenesis is a rare form of thyroid dysgenesis of which some familial cases have been reported, including one associated with a heterozygous mutation in the Pax8 gene. However, the physiopathology remains not well known. The objectives of this study were 1) to describe the clinical features, 2) to look for ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000161409.04177.36
更新日期:2005-06-01 00:00:00
abstract::Sudden infant death syndrome is the most common cause of postneonatal infant mortality in the developed world. It is a diagnosis of exclusion with peak age of incidence between 2 and 6 mo. Fifty to 63% of these infants have a preexisting upper respiratory tract infection before death. We hypothesized that the immature...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200210000-00005
更新日期:2002-10-01 00:00:00
abstract::Depression is one of the most debilitating chronic disorders in the United States, affecting 15 million children in homes with depressed mothers, many of whom endure household chaos, inconsistent nurturing, inadequate safety practices, and harsh discipline. Depressed mothers are under diagnosed and undertreated, yet t...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/pr.2015.214
更新日期:2016-01-01 00:00:00
abstract::In preterm infants, low levels of insulin like growth factor 1 (IGF-I) have been associated with impaired growth and retinopathy of prematurity. Our objective was to study safety and pharmacokinetics of i.v. administered rhIGF-I with its binding protein 3 (rhIGFBP-3) to preterm infants. At 3 d chronological age, an i....
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/PDR.0b013e31819d9e8c
更新日期:2009-05-01 00:00:00
abstract::BACKGROUNDThe aims of this study were to (i) compare the concentrations of two neural injury markers, S100B protein and neuron-specific enolase (NSE), in intrauterine growth-restricted (IUGR) fetuses and in fetuses with appropriate growth-for-gestational-age (AGA), and (ii) investigate potential relationships between ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.108
更新日期:2017-09-01 00:00:00
abstract::The incidence and prevalence of celiac disease vary internationally. We studied the incidence of identified cases of childhood celiac disease in six Dutch provinces that cover 47.9% of the surface area and 67.6% of the total population of The Netherlands. Children with celiac disease aged 0-14 y, diagnosed from Januar...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199502000-00015
更新日期:1995-02-01 00:00:00
abstract::Normal hemostasis depends in part on the balance achieved between proaggregatory and prothrombotic platelet thromboxane A2, measured as its stable end-product thromboxane B2 (TXB2), and vascular prostacyclin (PGI2), which inhibits platelet aggregation and is antithrombotic. Cystathionine-beta-synthase deficiency is ch...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198206000-00018
更新日期:1982-06-01 00:00:00
abstract::Conversion of adenosine to inosine is decreased in adenosine deaminase (ADA)-deficient fibroblasts at all concentrations of adenosine tested. Adenosine is not differentially toxic to ADA-deficient fibroblasts except at very high (5 X 10(-4) -1 X 10(-3) M) adenosine levels. Conversion of [14C] adenosine to GTP is not d...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197607000-00002
更新日期:1976-07-01 00:00:00
abstract::BackroundMicrocephaly can either be isolated or it may coexist with other neurological entities and/or multiple congenital anomalies, known as syndromic microcephaly. Although many syndromic cases can be classified based on the characteristic phenotype, some others remain uncertain and require further investigation. T...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.65
更新日期:2017-08-01 00:00:00
abstract::We have shown that vitamin A (VA) and retinoic acid (RA) synergistically increase lung retinyl ester content in neonatal rats. To confirm whether this biochemical synergism attenuates early neonatal hyperoxic lung injury in mice, we exposed newborn C57BL/6 mice to 95% O2 or air from birth to 4 d. The agent [vehicle, V...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181dbac3d
更新日期:2010-06-01 00:00:00
abstract:BACKGROUND:Near-infrared spectroscopy (NIRS) is an upcoming clinical method for monitoring regional cerebral oxygen saturation (rScO2) in neonates. There is a growing market offering different devices and sensors. Even though this technique is increasingly clinically applied, little is known about the similarities and/...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.133
更新日期:2013-11-01 00:00:00
abstract::Fetal lung cyclic AMP phosphodiesterase, cyclic AMP, phosphatidyl choline, and incorporation of precursors into phosphatidyl choline were measured in rabbits after maternal administration of hydrocortisone phosphate and aminophylline. Both agents inhibited lung phosphodiesterase activity and augmented cyclic AMP conce...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197606000-00011
更新日期:1976-06-01 00:00:00
abstract::Although cisapride is widely used to treat gastrointestinal motility disorders, it has been associated with QT prolongation, torsades de pointes, and cardiac arrest. Only in children, however, has atrioventricular (AV) block after cisapride been reported. This study used Langendorff perfusion to determine the direct e...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000050122.67091.1D
更新日期:2003-03-01 00:00:00
abstract::Insulin treatment of prepubertal children with insulin-dependent diabetes improves body protein balance by decreasing the rate of protein degradation without stimulating protein synthesis. However, insulin also causes hypoaminoacidemia, so the inability of insulin to stimulate protein synthesis may have been limited b...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000169976.20029.64
更新日期:2005-08-01 00:00:00
abstract::Spring steel coils have been used to occlude abnormal vessels in patients with a congenital heart malformation. However, long-term adverse effects of steel spring coils remain uncertain, although some long-term results appear to be good. The purpose of this study was to evaluate the angiographic and histologic results...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199804000-00016
更新日期:1998-04-01 00:00:00
abstract::This study was aimed at describing abdominal and laryngeal muscle responses to upper airway occlusion (UAO) in early life and the effect of sleep states on these responses. Twelve nonsedated, 9-26-d-old lambs were studied. We simultaneously recorded 1) airflow (pneumotachograph + face mask); 2) sleep states (electroco...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199706000-00011
更新日期:1997-06-01 00:00:00
abstract::Three patients with Menkes' disease, an inherited disorder of copper transport, were studied to determine whether the copper deficiency was associated with a lipoprotein disorder. Hypocuprinemia was documented in all three cases. Two patients had severe copper and ceruloplasmin deficiencies, whereas the third patient ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198409000-00012
更新日期:1984-09-01 00:00:00
abstract:BACKGROUND:Maternal smoking impairs fetal growth; however, if postnatal growth differs between children born small for gestational age (SGA) with smoking and non-smoking mother is unknown. METHODS:Cohort-study of term born children born appropriate for gestational age with non-smoking mother (AGA-NS, n = 30,561), SGA ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0352-5
更新日期:2019-06-01 00:00:00
abstract::Seven plasma protease inhibitors were analyzed in 8-yr-old children-75 PiZ (severely deficient), one PiZ-(with a null gene), 32 PiSZ (moderately deficient), and 35 normals. In PiZ and PiSZ alpha 1-antitrypsin-deficient children, significantly higher concentrations of alph 2-macroglobulin (p less than 0.001) and antith...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198508000-00011
更新日期:1985-08-01 00:00:00
abstract::Recombinant human erythropoietin (rEpo) is neuroprotective in neonatal models of hypoxic-ischemic brain injury. However, the optimal rEpo dose, dosing interval, and number of doses for reducing brain injury are still undetermined. We compared the neuroprotective efficacy of several subcutaneous rEpo treatment regimens...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/pdr.0b013e3180332cec
更新日期:2007-04-01 00:00:00