Abstract:
:Epithelial hyperplasia is an early feature of the renal and biliary lesions in autosomal recessive polycystic kidney disease (ARPKD). To explore the cellular basis of this hyperplasia we isolated, cultured, and characterized biliary tract epithelium from common bile duct explants of mice with ARPKD (the BPK mouse) and controls. Primary cultures resulted in dense colonies of contact-inhibited epithelial cells with a homogenous growth pattern. Colony growth in serum-free basal medium (BM) of BPK-derived cells was not different from controls. Supplementation of BM with epidermal growth factor (EGF) induced a proliferative response in BPK-derived cells that was significantly increased over controls as assessed by [3H]thymidine uptake and expressed as percent change over growth in BM (BPK 239% and controls 131% of BM growth). In contrast, no differences between BPK- and control-derived cells were found with regard to the effects of BM supplementation with IGF-I, IGF-II, acidic fibroblast growth factor, keratinocyte growth factor, hepatocyte growth factor, or transforming growth factor-beta. Primary culture of biliary epithelium may provide a useful in vitro model for the study of the cellular pathophysiology of ARPKD. Our data demonstrate that increased epithelial sensitivity to EGF-like proteins may play a role in biliary epithelial proliferative changes which parallel renal tubular epithelial proliferation in ARPKD.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Nauta J,Sweeney WE,Rutledge JC,Avner EDdoi
10.1203/00006450-199506000-00014subject
Has Abstractpub_date
1995-06-01 00:00:00pages
755-63issue
6eissn
0031-3998issn
1530-0447journal_volume
37pub_type
杂志文章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::Increased fetal lung expansion profoundly inhibits surfactant protein gene expression and stimulates cellular proliferation in the fetal lung. Our aim was to determine whether increased expansion of the lung after birth, by the application of a continuous positive airway pressure (CPAP) for 12 h, inhibits surfactant p...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200112000-00015
更新日期:2001-12-01 00:00:00
abstract::Lung infections are a common cause of morbidity and mortality in neonates. To evaluate neonatal lung defenses against pneumococci, we challenged rats with aerosols of encapsulated pneumococci in an airborne infection apparatus. Whereas adult rats cleared greater than 95% of inhaled type 1 or type 25 pneumococci within...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198712000-00025
更新日期:1987-12-01 00:00:00
abstract::Milk gangliosides inhibit Vibrio cholerae enterotoxin and Escherichia coli heat-labile enterotoxin. Human milk gangliosides showed considerably higher enterotoxin-inhibitory activity compared to bovine and formula milk gangliosides as measured in vitro by enzyme-linked immunosorbent assay and in vivo in rabbit small b...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198605000-00008
更新日期:1986-05-01 00:00:00
abstract::Our aim was to compare white matter (WM) microstructure in preterm infants with and without punctate WM lesions on MRI using tract-based spatial statistics (TBSS) and probabilistic tractography. We studied 23 preterm infants with punctate lesions, median GA at birth 30 (25-35) wk, and 23 GA- and sex-matched preterm co...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3182182836
更新日期:2011-06-01 00:00:00
abstract:BACKGROUND:Kawasaki disease was well known for coronary artery abnormalities with few reports of incidence of acute kidney injury (AKI). Our aim was to identify the rate of AKI in patients with Kawasaki disease and its associated factors. METHODS:All patients with Kawasaki disease admitted to a medical center from Feb...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2016.81
更新日期:2016-08-01 00:00:00
abstract::Sarcoplasmic reticulum (SR) Ca release has been shown not to be the predominant mechanism responsible for excitation-contraction (E-C) coupling in fetal myocytes. However, most of the studies have been conducted either on primary cultures or acutely isolated cells, in which an apparent reduction of ryanodine receptor ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e31820bc69b
更新日期:2011-04-01 00:00:00
abstract::Many trials of mesenchymal stem cells (MSCs) have been published in the past 5-6 y. MSCs inhibit T-cell alloreactivity in vitro by soluble factors and direct cell-to-cell contact. They are safe to infuse in humans with no acute toxicity and no ectopic tissue formation. Promising results of MSC infusion for graft-vs.-h...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/pr.2011.56
更新日期:2012-04-01 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::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::Isolated TSH deficiency leading to hypothyroidism seems to be a rare condition, escaping the diagnosis by neonatal screening programs, which are based on the primary determination of TSH. This is the first report of a case with an autosomal recessive TSH defect caused by a homozygous mutation of the betaTSH gene that ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199908000-00007
更新日期:1999-08-01 00:00:00
abstract::Developmental differences in myocardial performance are known to exist. It is likely that the profile of protein isoforms present on the developing thin filament contributes to these observed differences. We have prepared thin filaments from developing and mature rat hearts by using an immunoprecipitation procedure de...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199109000-00006
更新日期:1991-09-01 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:BACKGROUND:Insulin-like growth factor (IGF)-I and -II play an important role in prenatal growth. During the first 2 months from birth, body fat doubles, and rapid weight gain during this time increases future risk of cardiometabolic disease. The aim of this study was to determine whether IGF measurements at birth assoc...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0331-x
更新日期:2019-04-01 00:00:00
abstract::The lipid profile, total cholesterol, triglycerides, and lipoproteins (beta-, pre-beta-, and alpha-) of cord blood, is presented for 419 black and white infants (94% of the eligible population) born during an 18-month period in Bogalusa, Louisiana. At birth, white neonates of both sexes had higher average levels than ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197808000-00011
更新日期:1978-08-01 00:00:00
abstract::Human Bifidobacterium sp strain bifidum (B. bifidum) was administered to BALB/c lactating mice (n = 58) and their litters (n = 327 pups) to evaluate the ingested strain's adherent properties and ability to inhibit murine rotavirus (MRV) infection. ELISA and anaerobic bacteriologic techniques were used to measure MRV s...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199406000-00014
更新日期:1994-06-01 00:00:00
abstract::Atherosclerosis originates in childhood and adolescence. The goal of this review is to highlight how exercise and physical activity during childhood and adolescence, critical periods of growth and development, can prevent adult cardiovascular disease (CVD), particularly through molecular mechanisms of monocytes, a key...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/s41390-019-0581-7
更新日期:2020-01-01 00:00:00
abstract::We determined the inhibitory effects of concentrations of tin- and zinc protoporphyrin (1-100 microM) and mesoporphyrin (0.1-10 microM) on the in vitro heme oxygenase (HO) (E.C.1.14.99.3) activity in liver, spleen, and intestine 13,000 x g tissue supernatants from fasted adult male Wistar rats through measurement of c...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198910000-00015
更新日期:1989-10-01 00:00:00
abstract::The regionalization of pediatric services has resulted in differential access to care, sometimes creating barriers to those living in underserved, rural communities. These disparities in access contribute to inferior healthcare outcomes among infants and children. We review the medical literature on telemedicine and i...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/pr.2015.192
更新日期:2016-01-01 00:00:00
abstract::A specific, enzyme-linked immunoabsorbent assay was used to determine the concentration of the 35,000 mol wt surfactant apoprotein (SP-A) in samples of amniotic fluid obtained from nondiabetic (n = 358) and diabetic (n = 29) women. The enzyme-linked immunoabsorbent assay was performed with rabbit antibodies directed a...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198812000-00016
更新日期:1988-12-01 00:00:00
abstract::Clinical trials demonstrated decreasing rates of bronchopulmonary dysplasia in preterm infants with hypoxic respiratory failure treated with inhaled nitric oxide (iNO). However, the molecular and biochemical effects of iNO on developing human fetal lungs remain vastly unknown. By using a well-characterized model of hu...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181d4f20f
更新日期:2010-05-01 00:00:00
abstract::The relationship between the development of cytotoxic cellular immune response to herpes simplex virus type I (HSV-1)-infected autologous cells and the production of interferon (IFN) was studied using in vitro secondary sensitization of peripheral blood leukocytes in subjects with recurrent herpes labialis (RHL) and i...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198609000-00020
更新日期:1986-09-01 00:00:00
abstract::Studies using stable isotopically labeled glucose and palmitate as precursors of pulmonary surfactant synthesis have demonstrated slow surfactant turnover in premature infants with respiratory distress syndrome (RDS). However, only limited data about surfactant turnover are available for term infants. Because acetate ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000075084.56255.E0
更新日期:2003-08-01 00:00:00
abstract::Adverse perinatal events affecting cerebral functions are a major cause of neonatal mortality, morbidity, and long-term neurologic deficit. Intrapartum fetal EEG, which records fetal brain electrical activity, provides a monitoring modality for evaluating the fetal CNS during labor. In this study, we describe a new ap...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200009000-00013
更新日期:2000-09-01 00:00:00
abstract:BACKGROUND:Caffeine is widely used to manage apnea of prematurity, and reduces the incidence of bronchopulmonary dysplasia (BPD). Deregulated transforming growth factor (TGF)-β signaling underlies arrested postnatal lung maturation in BPD. It is unclear whether caffeine impacts TGF-β signaling or postnatal lung develop...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.21
更新日期:2017-05-01 00:00:00
abstract::EBV has been associated with several malignancies in humans. EBV can also infect marmoset B lymphocytes, which, as opposed to human B cells, are permissive for lytic Epstein-Barr viral replication. Mice with a severe combined immunodeficiency phenotype (SCID mice) are extremely susceptible to EBV-induced lymphomagenes...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199410000-00007
更新日期:1994-10-01 00:00:00
abstract::Carbonic anhydrase II (CA II) is the only soluble isozyme of CA which is known to be expressed in kidney. We recently identified a deficiency of this enzyme as the basis for the autosomal recessive syndrome of osteopetrosis with renal tubular acidosis and cerebral calcification. In order to explore the physiological i...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198510000-00017
更新日期:1985-10-01 00:00:00
abstract::Epidemiologic studies have linked intrauterine growth restriction (IUGR) with an increased incidence of cardiovascular disease later in life; reduced cardiomyocyte number in IUGR hearts may underlie such prenatal programming. Our aim was to examine the effect of IUGR, as a result of maternal protein restriction, on th...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000157726.65492.CD
更新日期:2005-06-01 00:00:00
abstract::Breast-fed infants have higher bilirubin levels than formula-fed infants, possibly because of variations in the composition of the breast milk. The aim of this study was to investigate whether there is a relationship between cytokine levels in the colostrum of nursing mothers and neonatal jaundice (NJ). Breast milk sa...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e31809871c9
更新日期:2007-08-01 00:00:00
abstract::Various assay methods for detection of thyroid hormones in human milk were evaluated in recovery and dilution experiments after which the concentrations of thyroxine (T4) and 3,5,3'-triiodothyronine (T3) were measured and compared with those in serum. The effect of breast feeding on pituitary thyroid function of norma...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198306000-00008
更新日期:1983-06-01 00:00:00