Abstract:
:Irreversible congenital heart block (CHB) and the transient rash of neonatal lupus are strongly associated with maternal antibodies to SSA/Ro and SSB/La proteins; however, the precise mechanism by which these antibodies mediate organ-specific injury is not yet defined. Culturing of keratinocytes has provided critical insights. Accordingly, successful culturing of human fetal cardiac myocytes at high yield would constitute a powerful tool to directly examine conditions that promote expression of the target autoantigens. To accomplish this aim, fetal cardiac myocytes from 18- to 22-wk abortuses were established in culture using a novel technique in which cells were isolated after perfusion of the aorta with collagenase in a Langendorff apparatus. After preplating to decrease fibroblast contamination, cardiocytes were grown in flasks and slide chambers. Staining with monoclonal anti-sarcomeric alpha-actinin revealed the expected striations typical of cardiac myocytes in 70-90% of the cells after 4 d in culture. Furthermore, the cells were observed to beat at rates varying between 25-75 beats per minute (bpm) after the addition of 1.8 mM CaCl2. An average yield of 45-60 x 10(6) cells was obtained from a 3- to 5-g heart. Cellular localization of SSA/Ro and SSB/La by indirect immunofluorescence and demonstration of mRNA expression by reverse transcriptase polymerase chain reaction supports the feasibility of cultured cardiac myocytes for the study of congenital heart block. In contrast to the increased expression of SSA/Ro reported for keratinocytes, incubation of cultured human cardiac myocytes with either 17beta-estradiol or progesterone did not alter mRNA expression or cellular localization of 48 kD SSB/La, 52 kD SSA/Ro, or 60 kD SSA/Ro. In summary, we describe a novel method to successfully culture human fetal cardiac myocytes that should provide a valuable resource for investigation of the molecular mechanism(s) contributing to the development of congenital heart block. Differential constitutive and estradiol-induced expression of 52 and 60 kD SSA/Ro in human cardiac myocytes compared with keratinocytes may be a factor contributing to the marked discordance of clinically detectable injury in these two target tissues.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Tseng CE,Miranda E,Di Donato F,Boutjdir M,Rashbaum W,Chan EK,Buyon JPdoi
10.1203/00006450-199902000-00018subject
Has Abstractpub_date
1999-02-01 00:00:00pages
260-9issue
2eissn
0031-3998issn
1530-0447journal_volume
45pub_type
杂志文章abstract:BACKGROUND:To examine differences in comorbidities and surgical management based on socioeconomics in hospitalized children with Crohn's disease (CD). METHODS:Using the Kids' Inpatient Database for 2006-2012, we identified patients (<21 years) with a CD diagnosis. Cases were analyzed and stratified by median parental ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-0830-9
更新日期:2020-12-01 00:00:00
abstract::Tracheal aspirate IL-8 concentration and airway epithelial cell IL-8 expression are each increased in premature infants undergoing mechanical ventilation. We sought to determine the cytokines responsible for IL-8 expression in this context. Tracheal aspirates were collected from 18 mechanically ventilated premature in...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000145274.47221.10
更新日期:2004-12-01 00:00:00
abstract::Vitamin A and its precursor beta-carotene are naturally occurring antioxidants. The effects of diets deficient in beta-carotene and vitamin A on the alveolar-capillary membrane were studied in young adult BALB/C mice before and after exposure to 65% oxygen. One of three diets (standard complete, beta-carotene deficien...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198801000-00017
更新日期:1988-01-01 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 mitochondrial DNA (mtDNA) point mutation T8993G has been associated with maternally inherited Leigh syndrome (MILS) when very abundant (> 95%). MILS patients are usually severely affected and die in early infancy. In 1993, a novel T8993C point mutation was described in a juvenile form of Leigh syndrome (LS) charac...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199605000-00028
更新日期:1996-05-01 00:00:00
abstract::In seven patients with mucopolysaccharidoses (1 Hurler, 1 Hurler-Scheie, 4 Hunter, 1 Sly), cranial (1)H-magnetic resonance spectroscopy was performed to evaluate the accumulation of mucopolysaccharides and biochemical changes in the CNS in vivo before and after bone marrow transplantation (BMT). In two of seven patien...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200103000-00008
更新日期:2001-03-01 00:00:00
abstract::The purpose of this study was to investigate atrial myocardial blood flow in the fetus under conditions of acute right ventricular pressure load and adenosine infusion. Late gestation fetal sheep were instrumented for acute right ventricular pressure loading or adenosine infusion, and regional myocardial blood flow wa...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199403000-00010
更新日期:1994-03-01 00:00:00
abstract::Hemophagocytic lymphohistiocytosis (HLH) and Langerhans cell histiocytosis (LCH) are members of a group of rare heterogenous disorders, the histiocytoses, characterized by uncontrolled accumulation of pleomorphic infiltrates of leukocytes. The etiology of these diseases is mainly unknown. CD45 is a hemopoietic cell sp...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000106803.15344.72
更新日期:2004-03-01 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::To assess sleep organization in premature infants of < 35 wk gestational age (w GA), we performed polygraphic recordings in 24 neurologically normal neonates (eight per group): artificially ventilated 27-30 and 31-34 w GA infants and nonventilated 31-34 w GA infants. Sleep states were defined by concordance of EEG and...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199311000-00013
更新日期:1993-11-01 00:00:00
abstract::Momentum around the era of genomic medicine is building, and with it, anticipation of the benefits that whole genome analysis (personalized or individualized genomics) will bring for the provision of health care. These technologies have the potential to revolutionize genetic diagnosis; however, the expansive data gene...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/PDR.0b013e3181b0cbd8
更新日期:2009-10-01 00:00:00
abstract::L-selectin, a surface adhesion glycoprotein expressed on leukocytes, has a well-established role in mediating inflammation and lymphocyte recirculation. Recent evidence suggests that L-selectin may also influence hematopoiesis. We observed that a greater proportion of CD34+ cells express L-selectin in cord blood compa...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199906000-00015
更新日期:1999-06-01 00:00:00
abstract::We evaluated the hypothesis that increased endogenous opioid activity mediates part or all of the left ventricular contractile and pump dysfunction previously demonstrated in HCl-induced metabolic acidemia. Eighteen Western newborn lambs were catheterized and instrumented; pacing wires were sutured to the right atrial...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198806000-00023
更新日期:1988-06-01 00:00:00
abstract::The functional activity of naturally acquired IgG and IgM with specificity for the capsular polysaccharide of type III, group B Streptococcus (III-GBS) was compared. Sera collected during convalescence from 14 infants who had developed specific antibodies following III-GBS infection were employed for separation of IgG...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198801000-00007
更新日期:1988-01-01 00:00:00
abstract::In the human fetus, liver macrophages appear to be the primary source of erythropoietin (Epo). Epo production shifts from the liver to peritubular cells in the kidney sometime during the 3rd trimester. Some preterm infants experience a hyporegenerative anemia that appears to be caused by inadequate Epo production. It ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199402000-00008
更新日期:1994-02-01 00:00:00
abstract::Endothelin (ET)-1 is a potent vasoconstrictor peptide that modulates basal pulmonary vascular resistance (PVR) in the normal ovine fetus and contributes to high PVR after chronic intrauterine pulmonary hypertension. Although high PVR is present in premature lambs with severe hyaline membrane disease (HMD), whether ET-...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199808000-00006
更新日期:1998-08-01 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::Evaluation of nonlinear heart rate (HR) dynamics has received considerable attention in the pediatric literature because such analyses not only provide insight into underlying control mechanisms, but may also help to differentiate between normal and abnormal infants. The purpose of this study was to determine, in eigh...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199807000-00018
更新日期:1998-07-01 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::Automated microanalytic chromogenic coagulation assays allow serial monitoring of critically ill newborn infants. In this study 84 premature infants [26 healthy prematures and 58 neonates with idiopathic respiratory distress syndrome (IRDS)] were studied daily during the first week of life, to investigate the possible...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198403000-00012
更新日期:1984-03-01 00:00:00
abstract::Escherichia coli which elaborate heat stable enterotoxin (ST) are a major cause of endemic diarrhea in infants. The reason(s) for this increased susceptibility of infants to ST-mediated diarrhea is unknown. We investigated the possibility that the immature (14 and 21 day old) rat small intestine is more sensitive to S...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198606000-00017
更新日期:1986-06-01 00:00:00
abstract::Gluconeogenesis decreases during septic shock, but its mechanism is not well known. Tumor necrosis factor alpha (TNF-alpha), which is a key cytokine in septic shock, can increase GLUT1 gene expression and glucose uptake in muscles and fatty tissues. TNF-alpha does not alter the metabolism of hepatocytes in which GLUT2...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200104000-00018
更新日期:2001-04-01 00:00:00
abstract::Dexamethasone (Dex) and triiodothyronine (T3) were administered to pregnant rats during late gestation to evaluate potential developmental effects on fetal lung vitamin K-dependent carboxylation. Maternal rats were injected on the 2 d before study with Dex (0.2 mg/kg intraperitoneally), with T3 (0.7, 3.5, or 7 mg/kg i...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198905000-00021
更新日期:1989-05-01 00:00:00
abstract:BACKGROUND:Toll-like receptor (TLR)-mediated inflammation may contribute to neonatal sepsis, for which pentoxifylline (PTX), a phosphodiesterase inhibitor that raises intracellular cAMP, is a candidate adjunctive therapy. We characterized the anti-inflammatory effects of PTX toward TLR-mediated production of inflammato...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.6
更新日期:2017-05-01 00:00:00
abstract:BACKGROUND:Serum caffeine concentrations >20 μg/ml (100 μmol/l) in infants treated for apnea of prematurity increases TNF-α and decreases IL-10, changes that perhaps are linked to comorbidities. We hypothesize that this proinflammatory cytokine profile may be linked to differential binding of caffeine to adenosine rece...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2016.50
更新日期:2016-07-01 00:00:00
abstract::Hypercholesterolemia is common in preterm infants administered 10% Intralipid perhaps because of excess phospholipid in plasma causing efflux of cholesterol from tissues. The purpose of this study was to determine if cholesterol synthesis (as measured by plasma lathosterol) is increased in preterm infants (23-32 wk ge...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199202000-00020
更新日期:1992-02-01 00:00:00
abstract::In spontaneously breathing newborn lambs, alveolar hypoxia increases lung microvascular pressure, which causes lung lymph flow to increase and the concentration of protein in lymph to decrease. To see if this response derives from hypoxia itself rather than from the change in breathing pattern that occurs during hypox...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198405000-00009
更新日期:1984-05-01 00:00:00
abstract::The effect of various lipids, proteins, and other substances on the adsorption rate of dipalmitoyl phosphatidylcholine (DPC) was investigated. Each substance was added to a sonicated suspension of DPC in saline, and the surface pressure of the adsorbed surface film was measured using a platinum plate suspended from an...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198107000-00010
更新日期:1981-07-01 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::It has been shown that glucocorticoids accelerate lung development by limiting alveolar formation resulting from a premature maturation of the alveolar septa. Based on these data, the aim of the present work was to analyze the influence of dexamethasone on cell cycle control mechanisms during postnatal lung developmen...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200202000-00008
更新日期:2002-02-01 00:00:00