Abstract:
:We have identified two unrelated girls with chronic neutropenia [absolute neutrophil counts (ANC) 10-870 and 10-940/microL in patients 1 and 2, respectively] and severe defect in superoxide anion generation by granulocytes. Formyl-methionyl-leucyl-phenylalanine-induced superoxide release was 1.2 +/- 0.9 and 1.9 +/- 1.9% (mean +/- SEM, n = 3) of normal controls', mean value in patients 1 and 2, respectively. However, granulocytes from both patients released a normal amount of superoxide upon stimulation with phorbol myristate acetate. Patient 2 exhibited characteristic features of Duane syndrome, a rare disorder of eye movement. Treatment of the patients with recombinant granulocyte colony-stimulating factor led to significant clinical improvements and reduction of infectious complications and to increases in the ANC, to 400-2100/microL in patient 1 and to 500-3000/microL in patient 2. Treatment with 5 micrograms/kg/d resulted in increased intracellular killing of opsonized Staphylococcus aureus by granulocytes and an enhancement of superoxide release upon stimulation with formyl-methionyl-leucyl-phenylalanine in both patients up to 11.1 +/- 6.0 and 13.5 +/- 7.0% (mean +/- SEM, n = 5) of normal controls', mean value in patient 1 and patient 2, respectively. These data suggested that recombinant human granulocyte colony-stimulating factor treatment enhanced resistance to bacterial infection by stimulation of superoxide generation and increasing the bactericidal capacity of peripheral blood granulocytes.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Káposzta R,Maródi Ldoi
10.1203/00006450-199501000-00011subject
Has Abstractpub_date
1995-01-01 00:00:00pages
50-5issue
1eissn
0031-3998issn
1530-0447journal_volume
37pub_type
杂志文章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::Periventricular leukomalacia (PVL), the major substrate of neurologic deficits in premature infants, is associated with reduced white matter volume. Using immunomarkers of axonal pathology [beta-amyloid precursor protein (beta-APP) and apoptotic marker fractin], we tested the hypothesis that widespread (diffuse) axona...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e31816c825c
更新日期:2008-06-01 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::Astrocyte end-feet ensheathe blood vessels in the brain and are believed to provide structural integrity to the cerebral vasculature. We sought to determine in developing infants whether the coverage of blood vessels by astrocyte end-feet is decreased in germinal matrix (GM) compared with cerebral cortex and white mat...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000214975.85311.9c
更新日期:2006-05-01 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::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
pub_type: 历史文章,杂志文章
doi:10.1203/01.PDR.0000101756.93542.09
更新日期:2004-01-01 00:00:00
abstract::To determine the influence of an altered carotid body function on the laryngeal chemoreflex (LCR) response, reflex apnea was induced by laryngeal water stimulation during normoxia or acute hypoxia in unanesthetized awake lambs in which the ventilatory response to acute hypoxia was attenuated by prolonged postnatal hyp...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199312000-00024
更新日期:1993-12-01 00:00:00
abstract::Although obstructive nephropathy is a frequent cause of renal insufficiency in infancy, factors influencing adaptation to chronic partial ureteral obstruction (CPUO) are poorly understood. Guinea pigs were subjected to unilateral CPUO within the first 2 days of life (Group I) and microsphere studies were performed at ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198412000-00008
更新日期:1984-12-01 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::Birth is characterized by swift and complex transitions in hemodynamic and respiratory variables. Unrecognized pathologies or incidents may quickly become fatal or cause permanent damage. This article introduces an essential component of an acute perinatal care simulator, namely a model for educational simulation of n...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181c2def3
更新日期:2010-02-01 00:00:00
abstract::Growth hormone (GH) regulates growth and development in the postnatal period but lacks somatotropic activity in the fetus. In contrast, the placental hormone placental lactogen (PL) stimulates amino acid transport, DNA synthesis, and somatomedin production in isolated fetal tissues, suggesting that PL may function as ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199208000-00015
更新日期:1992-08-01 00:00:00
abstract::Bilirubin-IX alpha photooxidation products were detected by high performance liquid chromatography in the urine of neonates undergoing phototherapy for hyperbilirubinemia. The in vivo photoproducts were identified by chromatographic comparison with authentic synthetic standards using two complementary methods. Bilirub...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198408000-00003
更新日期:1984-08-01 00:00:00
abstract::Leptin, a hormone secreted by adipocytes, is elevated in blood of obese adults. It is unknown whether the concentration is affected by gender, ethnicity, age, or stage of sexual maturation in children. We measured serum leptin levels in 183 children and 27 young adults using a double-antibody ELISA assay. Body fat mas...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199710000-00010
更新日期:1997-10-01 00:00:00
abstract::Keratinocyte growth factor (KGF, or fibroblast growth factor 7) was previously reported to enhance the synthesis of surfactant in alveolar type II cells. We investigated the possible interactions between KGF and a glucocorticoid, dexamethasone (Dex), on surfactant protein (SP) gene expression. In cultured fetal rat ty...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000047840.77635.AE
更新日期:2003-02-01 00:00:00
abstract::The possible role played by albumin in regulating brain metabolism during development has been studied. The effects of fatty acid-free BSA on lactate, glucose, 3-hydroxybutyrate, and glutamine oxidation and lipogenesis by rat neurons and astrocytes from primary culture were studied. The rate of lactate oxidation and l...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199312000-00002
更新日期:1993-12-01 00:00:00
abstract::Plasma selenium concentration and glutathione peroxidase activity in red blood cells were determined in subjects from different age groups. The selenium level (mean +/- SD) found in infancy (0 to 6 months) was 2 +/- 0.6 micrograms/dl, with the lowest value of 1 microgram/dl observed in a 4-month-old infant. These leve...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198606000-00021
更新日期:1986-06-01 00:00:00
abstract::The purpose of this study is to determine whether the oxidative injury markers, protein carbonyls and malondialdehyde (MDA), are elevated in tracheal aspirates from very low birth weight (< 1500 g) infants; to determine whether levels correlate with myeloperoxidase as a marker of neutrophil inflammation; and to assess...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/00006450-200005000-00014
更新日期:2000-05-01 00:00:00
abstract:BACKGROUND:Hirschsprung disease (HSCR) is the third most common congenital disorder of the gastrointestinal tract. This study aims to elucidate changes in protein expression between the normal and aganglionic hindgut in human HSCR. METHODS:The biopsies were obtained from the normal and aganglionic hindgut in human HSC...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2014.33
更新日期:2014-06-01 00:00:00
abstract::Studies were conducted to assess the efficacy and safety of a synthetic peptide-containing surfactant in the treatment of respiratory distress syndrome (RDS) in preterm (approximately 80% of normal gestation) infant rhesus monkeys. Surfactant was prepared consisting of the phospholipids dipalmitoylphosphatidyl choline...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199604000-00025
更新日期:1996-04-01 00:00:00
abstract::A double-label, double-substrate incubation technique has been developed and used to study the conversion of progesterone to testosterone in testes extracts from incompletely virilized males. The procedure involves separation of the microsomes from a testicular homogenate, incubating the microsomes with 1 microM [7-3H...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198408000-00018
更新日期:1984-08-01 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
pub_type: 杂志文章
doi:10.1203/00006450-198107000-00014
更新日期:1981-07-01 00:00:00
abstract::To study cardiovascular autonomic control, we assessed the effect of atropine on heart rate (HR) and blood pressure (BP) variability in 12 preterm infants (range 26-32 wk) before intubation for respiratory insufficiency. Spectral power analysis of R-R interval and systolic BP (SBP) series were estimated in a low-frequ...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/01.PDR.0000145257.75072.BB
更新日期:2004-12-01 00:00:00
abstract:BACKGROUND:This study aimed to assess cortical gray matter growth and maturation in neonates with congenital heart disease (CHD). METHODS:Thirty-one (near) term neonates with severe CHD (8 univentricular heart malformation (UVH), 21 d-transposition of great arteries (d-TGA) and 2 aortic coarctation) underwent cerebral...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2016.145
更新日期:2016-11-01 00:00:00
abstract::The absolute and the percentage plasma NEFA pattern of healthy children, Cystic Fibrosis (C.F.) patients and their parents have been determined (table I, III, V) and compared with those of age and sex matched control subjects (table II, Iv, V). There is a striking difference in the percentage plasma NEFA pattern of C....
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198009000-00015
更新日期:1980-09-01 00:00:00
abstract::Continuous EEG monitoring has not been used widely in neonatal intensive care, especially in the care of extremely premature infants, probably in part because of a lack of a reliable quantitative method. The purpose of this study was to quantify the EEG of the very premature infants just after birth by using spectral ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000153868.77623.43
更新日期:2005-03-01 00:00:00
abstract::In Brazil, similar intrauterine growth restriction (IUGR) rates were observed between cities with distinct levels of socioeconomic development, challenging the current knowledge that higher rates of IUGR would necessarily be observed in poorer areas than in wealthier ones. Ribeirão Preto, a city located in the most de...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000156504.29809.26
更新日期:2005-05-01 00:00:00
abstract::Respiratory parameters of skeletal muscle were determined in permeabilized muscle fibers by adapting a technique described by Veksler et al. for cardiac fibers (Biochim Biophys Acta, 892:191-196, 1987). This method consists of the permeabilization of muscle fibers by saponin by allowing respiratory substrates and inhi...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199207000-00004
更新日期:1992-07-01 00:00:00
abstract::A new model has been developed for the study of maternal phenylketonuria. Beginning on the 12th day of gestation the diet of pregnant rats was supplemented with 0.5% alpha-methylphenylalanine and 3% phenylalanine. This resulted in an 83% reduction of hepatic phenylalanine hydroxylase activity. The maternal plasma phen...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198205000-00014
更新日期:1982-05-01 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::Programmed proliferative degeneration of the human fetal ductus arteriosus (DA) preceding definite postnatal closure has a large developmental variability and is controlled by several signaling pathways. Among these vascular endothelial growth factor (VEGF) and its receptors (VEGF-Rs) play an important role. Until now...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e318180a375
更新日期:2008-10-01 00:00:00