Abstract:
:The etiology of the tall stature almost invariably seen in homocystinuric patients is not known. The effect of metabolic control and the role of the GH-IGF system on growth were investigated in 10 patients with homocystinuria. There was a direct correlation between the plasma free homocyst(e)ine and growth velocity SD scores in 18 patient years (r, 0.46; p < 0.05). Plasma 2-y cumulative free homocyst(e)ine and height SD scores were directly correlated (r, 0.82; p < 0.01). Growth velocity SD scores were lower in patients with optimal metabolic control than in those with poorer control (-0.10 +/- 0.65 versus 0.95 +/- 0.68, p < 0.01). Height SD scores were also lower in the optimally controlled group (-0.01 +/- 0.81 versus 1.73 +/- 0.88, p < 0.05). GH and GH-related peptides did not deviate significantly from the reference ranges. These findings suggest that overgrowth is directly mediated by homocysteine, that the GH-IGF axis is not involved, and that it may be prevented by optimal metabolic control.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Topaloglu AK,Sansaricq C,Snyderman SEdoi
10.1203/00006450-200106000-00014subject
Has Abstractpub_date
2001-06-01 00:00:00pages
796-8issue
6eissn
0031-3998issn
1530-0447journal_volume
49pub_type
杂志文章abstract::We have previously demonstrated that enterally administered heparin-binding EGF-like growth factor (HB-EGF) produced in Escherichia coli decreases the incidence and severity of intestinal injury in a neonatal rat model of necrotizing enterocolitis (NEC). In preparation for upcoming human clinical trials, large-scale p...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181994fa0
更新日期:2009-04-01 00:00:00
abstract::The short-term (0-48 h) effects of maternal betamethasone administration on computerized fetal heart rate (FHR) parameters were studied in 36 pregnancies at increased risk for preterm delivery. FHR was recorded for 90 min immediately before the start of betamethasone treatment and again at 6-h intervals during the nex...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000155948.83570.EB
更新日期:2005-04-01 00:00:00
abstract::We investigated, using a human placental choriocarcinoma cell line as a model, the effects of the immunosuppressive drug cyclosporin A on several placental transport systems mediating the transfer of glucose and amino acids from mother to fetus. The results of the investigation show that the transport system responsib...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199207000-00024
更新日期:1992-07-01 00:00:00
abstract:BACKGROUND:Mevalonate kinase deficiency (MKD) is a rare genetic autoinflammatory disease caused by blocking of the enzyme mevalonate kinase in the pathway of cholesterol and isoprenoids. The pathogenic mechanism originating an immune response in MKD patients has not been clearly understood. METHODS:We investigated the...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.96
更新日期:2013-09-01 00:00:00
abstract::Tyrosine (Tyr) phosphorylation is implicated in the modification of several erythrocyte functions, such as metabolic pathways and membrane transport, as well as in signal transduction systems. Here we describe the map of Tyr-phosphorylated soluble proteins of newborn red blood cells (RBC) using an in vitro model simul...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000180545.24457.AC
更新日期:2005-10-01 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::Cerebral blood flow (CBF) measurement by near infrared spectroscopy (NIRS) using oxyhemoglobin (HbO2) as a tracer (CBF-HbO2) needs rapid changes in arterial oxygen saturation (SaO2) which often cannot be achieved in many sick infants. An alternative method based on the same adaptation of the Fick principle using i.v. ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199801000-00006
更新日期:1998-01-01 00:00:00
abstract::Carbohydrate and lipid intakes have both been found to modulate the metabolism of long-chain fatty acids. To define the respective influence of these two energy substrates on plasma fatty acid concentrations, 32 studies were performed in 16 parenterally fed newborn infants (mean +/- SEM, birth wt: 2.15 +/- 0.1 kg, age...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198910000-00002
更新日期:1989-10-01 00:00:00
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
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3182275164
更新日期:2011-09-01 00:00:00
abstract::C3H/HeJ mice develop an increase in terminal air space area detectable by postnatal d 14 that persists into adulthood compared with strain-matched controls (C3H/SnJ, C3H/OuJ). Morphometric quantification revealed a 50% increase in terminal air space area by postnatal d 14 and a 2.3-fold increase by 2 mo of age in C3H/...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000246071.50268.51
更新日期:2006-12-01 00:00:00
abstract::The fetal inflammatory response has been suggested as causal in neonatal morbidity. Serial levels of circulating cytokines were evaluated in 74 infants with a mean gestational age (GA) of 27.1 wk. Pro-inflammatory [tumor necrosis factor-alpha (TNF-alpha), interferon-gamma (IFN-gamma), IL-1 beta, IL-2, IL-6, IL-8, IL-1...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000182592.76702.E8
更新日期:2005-11-01 00:00:00
abstract::The deficiency of gamma-glutamyl transpeptidase activity, which was evident in some but not all cystic fibrosis amniotic fluids, could be corrected by mixing with either normal fluids or nondeficient cystic fibrosis fluids. Incubation of any amniotic fluid for 20 min at 62 degrees C resulted in total loss of gamma-glu...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198412000-00025
更新日期:1984-12-01 00:00:00
abstract:BACKGROUND:Maternal thyroid deficiency can increase Rho-kinase procontractile influence in arteries of 2-week-old progeny. Here we hypothesized that augmented role of Rho-kinase persists in arteries from adult progeny of hypothyroid rats. METHODS:Dams were treated with 6-propyl-2-thiouracil (PTU) in drinking water (0....
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-018-0029-5
更新日期:2018-07-01 00:00:00
abstract::Early childhood overweight and obesity have alarmingly increased over the years. Adulthood obesity is a well demonstrated significant independent predictor of cardiovascular risk (CVR) and/or mortality, which predisposes to the major components of metabolic syndrome (MS). Evidence of MS in obese children has been also...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/pdr.0b013e31805d8a8c
更新日期:2007-06-01 00:00:00
abstract::Response characteristics of a transcutaneous PO2 electrode to step changes in PaO2 were determined. In vitro lag time and 95% response time of a transcutaneous PO2 monitor were compared to in vivo response. Release of arterial occlusion was used to produce rapid local PaO2 changes in human infants and adults. In vitro...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198102000-00012
更新日期:1981-02-01 00:00:00
abstract::Factors affecting bone turnover in premature infants are not entirely clear but certainly are different from those influencing bones of adults and children. To identify fetal and maternal factors that might influence bone turnover, we prospectively studied 50 infants (30 preterm and 20 full-term) born at Ain Shams Uni...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000160591.70409.C8
更新日期:2005-05-01 00:00:00
abstract::The effect of intrauterine growth retardation (IUGR) on cerebral and adrenal monoamine metabolism was studied in the fetuses of eight nulliparous rat dams after unilateral uterine artery ligation on d 18 of gestation. On d 22 (term = 23 d), four dams were subjected to normoxia and four to hypoxia (10% O2) for 58 min w...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199202000-00008
更新日期:1992-02-01 00:00:00
abstract::This study aimed to investigate 1) the plasma resistin concentration at birth, 2) the relationship of resistin with leptin and insulin, and 3) the association of resistin with anthropometric indexes in newborn infants. Blood samples for hormonal assay were obtained from preterm and term newborns within the first 2 h o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000180556.76864.9A
更新日期:2005-10-01 00:00:00
abstract::Newborn piglets exposed to acute hypoxia-ischemia (HI) received i.v. cannabidiol (HI + CBD) or vehicle (HI + VEH). In HI + VEH, 72 h post-HI brain activity as assessed by amplitude-integrated EEG (aEEG) had only recovered to 42 ± 9% of baseline, near-infrared spectroscopy (NIRS) parameters remained lower than normal, ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3182276b11
更新日期:2011-09-01 00:00:00
abstract:BACKGROUND:Adolescents born preterm have altered hypothalamic-pituitary-adrenal axis function with a blunted cortisol stress response, however, the influences of intrauterine growth restriction and race are unclear. METHODS:We measured salivary cortisol before and 20 min after a maximal-exercise stress test and calcul...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0682-3
更新日期:2020-05-01 00:00:00
abstract:BACKGROUND:Congenital portosystemic shunt (CPSS) has the potential to cause hepatic encephalopathy and thus needs long-term follow-up, but an effective follow-up method has not yet been established. We aimed to evaluate the importance of per-rectal portal scintigraphy (PRPS) for long-term follow-up of CPSS. METHODS:We...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2014.11
更新日期:2014-05-01 00:00:00
abstract:BACKGROUND:Using a large population-based sample, this study aims to verify whether televiewing at 29 mo, a common early childhood pastime, is prospectively associated with school readiness at 65 mo. METHODS:Participants are a prospective longitudinal cohort of 991 girls and 1,006 boys from the Quebec Longitudinal Stu...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.105
更新日期:2013-09-01 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::In Krabbe's disease (globoid cell leukodystrophy), galactosylsphingosine (psychosine) is considered to be a causative agent of the pathology found in the nervous system of the patients. In our study, we examined the cytotoxic effect of psychosine in neural cell cultures derived from the rat nervous system. The concent...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199011000-00011
更新日期:1990-11-01 00:00:00
abstract::Primary immunodeficiencies (PIDs) have traditionally been defined according to their immunologic phenotype. Far from being concluded, the search for human genes that, when mutated, cause PID is actively being pursued. During the last year, four novel genetic defects that cause severe combined immunodeficiency and seve...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e31819dbe1e
更新日期:2009-05-01 00:00:00
abstract::Intrauterine growth restriction (IUGR) is a recognized risk factor for neurologic deficits later in life. Abnormal fetal blood flow in the presence of fetal growth retardation helps to distinguish true fetal growth impairment from small but normally grown infants. The present study aimed to investigate the influence o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000129660.32875.09
更新日期:2004-07-01 00:00:00
abstract:BACKGROUND:Macrosomia and child obesity are growing health-care issues worldwide. The purpose of the study was to evaluate how extremely high or low birth weight affects metabolic markers evaluated in newborn screening. METHODS:The study was register-based and included full-term singletons born in Iceland from 2009 to...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-1033-0
更新日期:2020-06-29 00:00:00
abstract::Serial plasma concentrations of immunoreactive 6-keto-prostaglandin F1 alpha (6-keto-PGF1 alpha), the stable hydration product of prostacyclin (PGI2), were measured with radioimmunoassay during the first month of life in 25 preterm infants with idiopathic respiratory distress syndrome (IRDS) and 38 preterm controls wi...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198210000-00005
更新日期:1982-10-01 00:00:00
abstract::Tanshinone IIA is a compound purified from the Chinese herb Danshen (Radix Salviae Miltiorrhiza Bge). The neuroprotective effect of tanshinone IIA was investigated in a neonatal rat model of hypoxia-ischemia brain damage. Hypoxia-ischemia encephalopathy was induced in rats at day 7 of postnatal age by ligation of the ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000180550.99162.BC
更新日期:2005-10-01 00:00:00
abstract::This patient presented on the first day of life with pronounced lactic acidosis with an elevated lactate/pyruvate ratio. Urine organic acids showed Krebs cycle metabolites and mildly elevated methylmalonate and methylcitrate. The acylcarnitine profile showed elevated propionylcarnitine and succinylcarnitine. Amino aci...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181e5c3a4
更新日期:2010-08-01 00:00:00