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 had a less severe deficiency. Hypertriglyceridemia was observed in the first patient, and elevations in triglyceride, cholesterol, apolipoprotein B (ApoB), and apolipoprotein C-III (ApoC-III) occurred predominantly in the very low density lipoprotein fraction (VLDL). This patient had normal lipoprotein lipase activity but mild glucose intolerance. The second patient had a borderline high cholesterol level with normal plasma triglycerides and apolipoproteins, whereas the third patient appeared to have normal total cholesterol but slightly higher triglycerides with elevated plasma apolipoprotein E (ApoE). No striking differences were observed in the chemical composition of all lipoprotein subfractions between patients and controls except that the neutral lipid content of VLDL was higher in patients than in controls. The ApoB was initially normal in molecular weight but degraded faster than the controls during storage. The appearance of the major low density lipoprotein (LDL) fraction of the first two patients was opaque white, in contrast to clear yellow in the third patient and in the age- and diet-matched controls. This abnormal appearance of LDL in these patients was associated with low plasma levels of beta-carotene and ceruloplasmin. These findings suggest that decreased serum copper levels may be associated with lipid and lipoprotein abnormalities and may enhance lipid peroxidation of LDL accounting for the color change.(ABSTRACT TRUNCATED AT 250 WORDS)
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Blackett PR,Lee DM,Donaldson DL,Fesmire JD,Chan WY,Holcombe JH,Rennert OMdoi
10.1203/00006450-198409000-00012subject
Has Abstractpub_date
1984-09-01 00:00:00pages
864-70issue
9eissn
0031-3998issn
1530-0447journal_volume
18pub_type
杂志文章abstract::Prenatal exposure to infection and subsequent inflammatory responses have been implicated in the etiology of schizophrenia and autism. In this review, we summarize current evidence from human and animal studies supporting the hypothesis that the pathogenesis of these two disorders is linked via exposure to inflammatio...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/PDR.0b013e318212c196
更新日期:2011-05-01 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::Concanavalin A surface receptors were detected on brush border enterocytes from child intestinal biopsies by the concanavalin A-peroxidase method. On electron microscopy, these receptors appeared as electron-dense deposits, located on a band running along the innermost part of the brush border membrane glycocalyx. In ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198206000-00008
更新日期:1982-06-01 00:00:00
abstract::Data are scarce regarding mineral bioavailability from human milk in older infants who may also be receiving solid foods (beikost). We measured the absorption of Ca, Zn, and Fe in 14 healthy, nonanemic 5-7-mo-old breast-fed infants whose mothers milk was extrinsically labeled with stable isotopes (44Ca, 70Zn, and 58Fe...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/00006450-199703000-00014
更新日期:1997-03-01 00:00:00
abstract::Leukotrienes C4 and D4 and thromboxane A2 are potent vasoconstrictors that may mediate pulmonary vasoconstriction in many clinical situations. There is a complex interaction among leukotrienes and thromboxane A2, because inhibition of thromboxane synthesis prevents some of the hemodynamic effects of exogenous leukotri...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198908000-00001
更新日期:1989-08-01 00:00:00
abstract::We studied the effects of anemia and ouabain administration on cardiac function, oxygen physiology, and blood catecholamine levels in nine newborn lambs. We measured oxygen consumption continuously, along with traditional hemodynamic variables. Oxygen transport was calculated. Following baseline measurements, lambs we...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198705000-00005
更新日期:1987-05-01 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
pub_type: 杂志文章
doi:10.1203/00006450-199112000-00021
更新日期:1991-12-01 00:00:00
abstract::Compliant immature airways sustain significant deformation following positive pressure ventilation. To evaluate the structural changes induced by in vivo positive pressure ventilation, tracheae of preterm lambs (107-116 d gestational age) were studied histologically. Nonventilated (group I: n = 7) and ventilated (grou...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198911000-00014
更新日期:1989-11-01 00:00:00
abstract::The microchemical study of growth cartilage biopsies may improve the classification and the genetic advice of some types of growth disturbances and contribute to the understanding of biochemical defects. Small tibial growth cartilage biopsies were performed during orthopedic surgery in cases with achondroplasia, pseud...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197510000-00006
更新日期:1975-10-01 00:00:00
abstract::The prevalence of iron deficiency was determined in Göteborg, Sweden, in a sample of 15- to 16-y-old girls (n = 220) and boys (n = 207) using serum ferritin (SF). In a recent study in women on the relationship between SF and stainable bone marrow iron, it was established that at a cutoff value for SF of < 16 microgram...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199311000-00023
更新日期:1993-11-01 00:00:00
abstract::We have explored the physiology of urinary epidermal growth factor (EGF) in the mouse by studying its ontogeny, and the effects of testosterone therapy on immunoreactive EGF (IR-EGF) concentrations in urine, kidney, serum and submandibular gland (SMG). Urine IR-EGF (U-EGF) increased about 100-fold relative to urea con...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198505000-00004
更新日期:1985-05-01 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::Nutrition in early life, growth, and subsequent health over a lifetime are significantly interrelated. The aim of this study was to assess body composition changes in exclusively breast-fed infants from birth up to 6 mo of age. An observational, prospective, cohort study was conducted. Fifty-nine full-term, healthy, e...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181f85a20
更新日期:2010-12-01 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:BACKGROUND:Long-term prefrontal cortex (PFC)- and hippocampus-based cognitive deficits are the sequelae of perinatal iron deficiency, despite iron supplementation starting in the newborn period. Whether high-dose iron supplementation prevents these deficits is yet to be determined. METHODS:Perinatal iron deficiency wa...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2012.143
更新日期:2013-01-01 00:00:00
abstract::Fifty patients with haemophilia B, belonging to 29 kindreds, were investigated with a highly sensitive immunoradiometric assay based on a homologous antibody to factor IX. The assay measures factor IX antigen (f.IX:Ag) in plasma down to 0.025 U/dl. Seventeen of 18 investigated patients with severe haemophilia B had ve...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198203000-00019
更新日期:1982-03-01 00:00:00
abstract::Cerebellar injury has been increasingly recognized as a complication of preterm birth, with decreased cerebellar volumes seen on follow-up neuroimaging. A cohort of 38 preterm newborns, including 14 with two scans, was studied with MRI, including single-shot fast spin-echo diffusion tensor imaging (DTI) sequence speci...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181a1fb3d
更新日期:2009-07-01 00:00:00
abstract::Developmental disability, intrauterine growth retardation, renal anomalies, and dysmorphic features have been described in offspring of women who abuse toluene during pregnancy. A Sprague-Dawley rat model was developed to study this clinical syndrome. During d 6-19 of gestation, 11 treated dams received daily gavage d...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199412000-00023
更新日期:1994-12-01 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
pub_type: 杂志文章
doi:10.1203/00006450-198710000-00007
更新日期:1987-10-01 00:00:00
abstract::Neonatal jaundice is a common condition that could potentially lead to severe neurotoxicity. In this electrophysiological study we observed the effects of a short-term bilirubin injection on evoked potentials (population spike, PS) and long-term potentiation (LTP) in the hippocampal CA3 region of Sprague Dawley rats i...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000061563.63230.86
更新日期:2003-06-01 00:00:00
abstract::Alpha-mannosidosis is a lysosomal storage disorder resulting from deficient activity of lysosomal alpha-mannosidase. It has been described previously in humans, cattle, and cats, and is characterized in all of these species principally by neuronal storage leading to progressive mental deterioration. Two guinea pigs wi...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199911000-00003
更新日期:1999-11-01 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::The objective of this study was to establish longitudinal normative limits for home memory monitors during early infancy. Eighty-eight healthy infants were monitored overnight at 0.25-19 wk of age using the Healthdyne Smart Monitor. Apnea settings were 14 s for recording and 40 s for alarm; the bradycardia setting was...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199602000-00005
更新日期:1996-02-01 00:00:00
abstract::The response to the capsular polysaccharide of Haemophilus influenzae type b (Hib PS) has been used to determine the molecular basis of antibody gene diversity in humans. In contrast to the relatively restricted nature of anti-Hib PS heavy-chain variable region gene expression, a variety of light-chain variable region...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199303000-00022
更新日期:1993-03-01 00:00:00
abstract:BACKGROUND:To evaluate the apoptosis inhibitor of macrophage (AIM) deposition patterns on the kidneys of children with IgA nephropathy (IgAN) and Henoch-Schönlein purpura nephritis (HSPN) and to investigate the clinical usefulness of serum and/or urinary AIM levels as biomarkers for the disease activity. METHODS:Immun...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-0951-1
更新日期:2020-05-14 00:00:00
abstract::Ambulatory blood pressure (ABP) monitoring offers a reliable method for determining blood pressure (BP) in children. The aim of this cohort study was to examine whether maternal preeclampsia is associated with elevated BP in an offspring. The study population consisted of 57 children born to preeclamptic mothers (PRE)...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000196734.54473.e3
更新日期:2006-02-01 00:00:00
abstract::Glutaryl-CoA dehydrogenase (GCDH) deficiency is a rare inborn disorder of L-lysine, L-hydroxylysine, and L-tryptophan metabolism complicated by striatal damage during acute encephalopathic crises. Three decades after its description, the natural history and how to treat this disorder are still incompletely understood....
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000219387.79887.86
更新日期:2006-06-01 00:00:00
abstract:INTRODUCTION:The Coulter LH780 hematology analyzer can evaluate mean neutrophil volume (MNV), conductivity (MNC), scatter (MNS), and distribution width (DW). We sought to investigate the value of volume, conductivity, and scatter (VCS) parameters in diagnosis and treatment efficacy of neonatal sepsis. RESULTS:We obser...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2011.16
更新日期:2012-01-01 00:00:00
abstract::We probed possible developmentally related changes in thin filament activity in rat hearts with the aid of calmidazolium (CDZ). CDZ is a calmodulin antagonist that also binds to troponin C and stimulates Ca2+ troponin C-dependent activation of cardiac myofibrillar contractile activity. In paired experiments, we compar...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199007000-00011
更新日期:1990-07-01 00:00:00
abstract::The effects of ascorbic acid on the excretion of homogentisic acid and its derivative benzoquinone acetic acid were studied in two adults and three infants. The administration of relatively large amounts of ascorbic acid to the adults was followed by a disappearance of benzoquinone acetic acid from the urine, whereas ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198908000-00015
更新日期:1989-08-01 00:00:00