Biliary epithelial cells from mice with congenital polycystic kidney disease are hyperresponsive to epidermal growth factor.


: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.


Pediatr Res


Pediatric research


Nauta J,Sweeney WE,Rutledge JC,Avner ED




Has Abstract


1995-06-01 00:00:00












  • Synergistic effect of influenza a virus on endotoxin-induced mortality in rat pups: a potential model for sudden infant death syndrome.

    abstract::Sudden infant death syndrome is the most common cause of postneonatal infant mortality in the developed world. It is a diagnosis of exclusion with peak age of incidence between 2 and 6 mo. Fifty to 63% of these infants have a preexisting upper respiratory tract infection before death. We hypothesized that the immature...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Blood-Siegfried J,Nyska A,Lieder H,Joe M,Vega L,Patterson R,Germolec D

    更新日期:2002-10-01 00:00:00

  • Fetomaternal potassium relations in the rat on the twentieth day of gestation. II. Effects of maternal hypokalemia.

    abstract::Time-corrected measurements of transplacental and transamniotic electrical potentials were made in potassium-deficient rats on the 20th day of gestation. These averaged 6.4 mV and 2.0 mV, respectively, in the deficient group as compared with 14.3 and 18.4, respectively, in the controls. The measured maternal potassium...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Fantel AG

    更新日期:1978-10-01 00:00:00

  • The evolution of primitive reflexes in extremely premature infants.

    abstract::A longitudinal study describes the pattern of appearance of eight primitive reflexes in a population of 47 viable extremely premature infants, beginning as early as 25 wk postconceptional age (PCA). Infants were examined weekly, from 1 wk of age until discharge from the neonatal intensive care unit. Primitive reflexes...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Allen MC,Capute AJ

    更新日期:1986-12-01 00:00:00

  • Increased expression of extracellular glutathione peroxidase in mice with dextran sodium sulfate-induced experimental colitis.

    abstract::Extracellular glutathione peroxidase (E-GPx) is a selenoenzyme that reduces hydrogen peroxide and organic peroxides. All plasma glutathione peroxidase (GPx) activity in humans is attributable to E-GPx. The gastrointestinal (GI) tract also synthesizes and secretes E-GPx into the extracellular milieu. Endogenously gener...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Tham DM,Whitin JC,Cohen HJ

    更新日期:2002-05-01 00:00:00

  • "Car. factor" deficiency revisited.

    abstract::Three members of a Virginia family with a bleeding disorder were found to have a serum defect in thromboplastin generation similar to the previously reported "Car. factor" deficiency. Sera from three members of the original Car. family did not correct the defect of affected members of the Virginia family. Partial thro...

    journal_title:Pediatric research

    pub_type: 临床试验,杂志文章


    authors: Komp DM

    更新日期:1975-04-01 00:00:00

  • Choline incorporation into lecithin in response to insulin or dexamethasone in homogeneous cell cultures of rat lung epithelial cells and fibroblasts.

    abstract::Labeled choline incorporation into adult rat lung alveolar epithelial cells and adult rat lung fibroblasts in monolayer culture was determined after incubation with insulin (Ins) 10 micrograms/ml, Dexamethasone (Dex) 10(-6)M, or no drug (ND). Incubation periods were 1, 3, 4, and 5 hours. The lecithin (phosphatidyl cho...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Sharp MJ,Borer RC Jr,Vadnay L,Douglas WH

    更新日期:1980-07-01 00:00:00

  • Pyritinol hydrochloride and cognitive functions: influence on children in slow learner classes.

    abstract::Pyritinol-HCl was tested for its impact on the cognitive functions of children with learning disabilities. This study is a contribution to scientific discussion on the complicated methodologic problems in evaluating the clinical efficacy of psychopharmacologic agents. Sixty-seven pupils of slow learner classes between...

    journal_title:Pediatric research

    pub_type: 临床试验,杂志文章


    authors: Wälti U,Kuenzler M,Schild J,Vassella F,Pavlincova E,Bircher J,Herschkowitz N

    更新日期:1975-09-01 00:00:00

  • Where to make a difference: research and the social determinants in pediatrics and child health in the COVID-19 era.

    abstract:IMPACT:Raises the importance of Social Determinants of Health in pediatrics. Considers the impact of Social Determinants of Health on COVID-19. Offers opportunities for research in the future. ...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Lachman P

    更新日期:2020-11-10 00:00:00

  • Insulin as a growth factor.

    abstract::Insulin is a potent mitogen for many cell types in vitro. During tissue culture, supraphysiological concentrations of insulin are necessary to promote cell replication in connective or musculoskeletal tissues. Insulin promotes the growth of these cells by binding, with low affinity, to the type I insulin-like growth f...

    journal_title:Pediatric research

    pub_type: 杂志文章,评审


    authors: Hill DJ,Milner RD

    更新日期:1985-09-01 00:00:00

  • Relationship between PCO2 and unfavorable outcome in infants with moderate-to-severe hypoxic ischemic encephalopathy.

    abstract:BACKGROUND:Abnormal PCO2 is common in infants with hypoxic ischemic encephalopathy (HIE). The objective was to determine whether hypocapnia was independently associated with unfavorable outcome (death or severe neurodevelopmental disability at 18 mo) in infants with moderate-to-severe HIE. METHODS:This was a post hoc ...

    journal_title:Pediatric research

    pub_type: 杂志文章,随机对照试验


    authors: Lingappan K,Kaiser JR,Srinivasan C,Gunn AJ

    更新日期:2016-08-01 00:00:00

  • Serum osteocalcin and bone and liver alkaline phosphatase isoforms in healthy children and adolescents.

    abstract::We report reference values for osteocalcin and bone and liver alkaline phosphatase (ALP) isoforms in serum from 114 healthy children and adolescents, age 7-16 y. The bone and liver ALP isoforms were measured by high performance liquid chromatography (HPLC). Six ALP isoforms were separated and quantified in all samples...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Magnusson P,Häger A,Larsson L

    更新日期:1995-12-01 00:00:00

  • Response to calcium of skinned gallbladder smooth muscle from newborn and adult guinea pigs.

    abstract::Guinea pig gallbladder smooth muscle contractility undergoes a period of postnatal maturation. Because tissues from adult animals contract more forcefully than tissues from newborn animals when stimulated with agonists that activate the contractile process through different mechanisms (receptor activation, membrane de...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Lambert R,Ryan JP

    更新日期:1990-10-01 00:00:00

  • Intestinal glucose transport in suckling rats fed artificial milk with and without added epidermal growth factor.

    abstract::Epidermal growth factor (EGF), a potent mitogen present in milk, is postulated to play an important physiologic role in the ontogeny of the intestine. An artificial rat milk, free of EGF, was developed and fed to newborn rats in an effort to evaluate the physiologic role of EGF on intestinal glucose transport. Compari...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Greene HL,Moore MC,Said HM,Ghishan FK,Orth DN

    更新日期:1987-04-01 00:00:00

  • The life span of erythrocytes transfused to preterm infants.

    abstract::This study was made to determine the life span of adult red cells transfused to early preterm infants. Nineteen very preterm infants (birth weight, 878.7 +/- 221 g; gestational age, 26.8 +/- 1.5 wk at birth) were sampled weekly after their last blood transfusion to determine the level (%) of fetal Hb in their circulat...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Bard H,Widness JA

    更新日期:1997-07-01 00:00:00

  • MicroRNA-93 may control vascular endothelial growth factor A in circulating peripheral blood mononuclear cells in acute Kawasaki disease.

    abstract:BACKGROUND:Kawasaki disease (KD) is the most common systemic vasculitis syndrome primarily affecting medium-sized arteries, particularly the coronary arteries. Though KD may be associated with immunological problems, the involvement of microRNAs (miRs) has not been fully described. METHODS:We enrolled 23 KD patients a...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Saito K,Nakaoka H,Takasaki I,Hirono K,Yamamoto S,Kinoshita K,Miyao N,Ibuki K,Ozawa S,Watanabe K,Bowles NE,Ichida F

    更新日期:2016-09-01 00:00:00

  • Salivary carcinoembryonic antigen (CEA) in cystic fibrosis.

    abstract::Increased concentrations of carcinoembryonic antigen (CEA) have been reported in the sera and sputum of patients with cystic fibrosis. We have found significantly higher concentrations of CEA in both extracted and unextracted saliva of patients with cystic fibrosis compared to age-matched controls and a comparable gro...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: MacSween JM,Gillespie CT

    更新日期:1980-03-01 00:00:00

  • Major circadian variations of glucose homeostasis in a patient with Rabson-Mendenhall syndrome and primary insulin resistance due to a mutation (Cys284-->Tyr) in the insulin receptor alpha-subunit.

    abstract::We have performed clinical, in vitro biochemical, and genetic studies of a patient with severe insulin resistance, considerable growth restriction, and Rabson-Mendenhall syndrome (patient RM-3). The blood IGF-I level was undetectable in this patient, although the GH level was moderately decreased. During the postprand...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Desbois-Mouthon C,Magré J,Duprey J,Caron M,Blivet-Van Eggelpoel MJ,Daubas C,Gourmelen M,Chevallier B,Rizkalla S,Robert JJ,Capeau J

    更新日期:1997-07-01 00:00:00

  • Intranasal fluticasone furoate in pediatric allergic rhinitis: randomized controlled study.

    abstract:BACKGROUND:Intranasal corticosteroids are the most efficacious anti-inflammatory medications for allergic rhinitis (AR). However, the efficacy and safety of intranasal corticosteroids in children have not yet been subject to specific research in China. The aim of this study was to investigate the efficacy and safety of...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Zhang Y,Wei P,Chen B,Li X,Luo X,Chen X,Xiang M,Li L,Zhao S,Xiao X,Yang X,Chen J,Fu Y,Xiao S,Liu H,Cheng L,Yao H

    更新日期:2020-10-02 00:00:00

  • Mucosal immune response in a case of sudden infant death syndrome.

    abstract::A prospective study to define the normal patterns of development of mucosal immunity in 263 children provided a unique opportunity to study the mucosal immune response in an infant who unexpectedly died from sudden infant death syndrome. The subject initially had a normal pattern of mucosal immune function, which was ...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Gleeson M,Clancy RL,Cripps AW

    更新日期:1993-06-01 00:00:00

  • Determinants of neurological outcome in neonates with congenital heart disease following heart surgery.

    abstract:BACKGROUND:The objective was to determine the association between perioperative risk factors and brain imaging abnormalities on neurologic outcome in neonates with hypoplastic left heart syndrome (HLHS) or d-Transposition of the great arteries (d-TGA) who underwent cardiac surgery including cardiopulmonary bypass. MET...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Kuhn VA,Carpenter JL,Zurakowski D,Reitz JG,Tague L,Donofrio MT,Murnick J,Axt-Fliedner R,Limperopoulos C,Yerebakan C

    更新日期:2020-07-25 00:00:00

  • Blood metabolomics in infants enrolled in a dose escalation pilot trial of budesonide in surfactant.

    abstract:BACKGROUND:The pathogenesis of BPD includes inflammation and oxidative stress in the immature lung. Corticosteroids improve respiratory status and outcome, but the optimal treatment regimen for benefit with low systemic effects is uncertain. METHODS:In a pilot dose escalation trial, we administered ≤5 daily doses of b...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Ballard PL,Torgerson D,Wadhawan R,Hudak ML,Weitkamp JH,Harris J,Asselin J,Chapin C,Ballard RA,McEvoy CT

    更新日期:2021-01-19 00:00:00

  • Poly-lactic-glycolic acid microspheres: a biodegradable marker for the diagnosis of aspiration in hamsters.

    abstract::Aspiration is a major cause of lung disease in infants and young children. As the symptoms and signs of aspiration are not specific, the diagnosis is delayed due to a low index of suspicion and low sensitivity and specificity of the available diagnostic tests. In the present study, we evaluated the utility of microsph...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Springer C,Benita S,Sherman Y,Gursoy N,Gilhar D,Avital A

    更新日期:2005-09-01 00:00:00

  • The effect of blue light exposure on the expression of circadian genes: bmal1 and cryptochrome 1 in peripheral blood mononuclear cells of jaundiced neonates.

    abstract::The purpose of this study was to investigate the effect of blue light phototherapy on the expression of circadian genes in peripheral blood mononuclear cells (PBMC) and plasma melatonin levels in neonates. Real-time reverse-transcriptase polymerase chain reaction (RT-PCR) was used to determine the expression of Bmal1 ...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Chen A,Du L,Xu Y,Chen L,Wu Y

    更新日期:2005-12-01 00:00:00

  • Ischemia-reperfusion injury in the intestines of newborn pigs.

    abstract::Although the pathogenesis of necrotizing enterocolitis remains uncertain, ischemia appears to be an important contributing factor to the development of this disorder. Reperfusion plays a major role in ischemia-related injury, and oxygen free radicals produced during reperfusion most likely contribute to the injury. Th...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Papparella A,DeLuca FG,Oyer CE,Pinar H,Stonestreet BS

    更新日期:1997-08-01 00:00:00

  • Stimulating and maintaining spontaneous breathing during transition of preterm infants.

    abstract::Most preterm infants breathe at birth, but need additional respiratory support due to immaturity of the lung and respiratory control mechanisms. To avoid lung injury, the focus of respiratory support has shifted from invasive towards non-invasive ventilation. However, applying effective non-invasive ventilation is dif...

    journal_title:Pediatric research

    pub_type: 杂志文章,评审


    authors: Dekker J,van Kaam AH,Roehr CC,Flemmer AW,Foglia EE,Hooper SB,Te Pas AB

    更新日期:2019-06-19 00:00:00

  • Adrenomedullin increases pulmonary blood flow in fetal sheep.

    abstract::We studied the effects of exogenously administered adrenomedullin on fetal pulmonary arterial blood flow in near-term fetal sheep. The hemodynamic effects of a single injection of adrenomedullin into the left pulmonary artery were compared with those of acetylcholine; the effects of repeated injections of adrenomedull...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: de Vroomen M,Takahashi Y,Gournay V,Roman C,Rudolph AM,Heymann MA

    更新日期:1997-04-01 00:00:00

  • Effect of gentamicin on the auditory brainstem evoked response in term infants: a preliminary report.

    abstract::Seven essentially healthy term infants who received gentamicin starting on the 1st day of life for prolonged rupture of membranes and maternal fever were compared with nine healthy term infants to determine whether this drug induces alterations in the auditory pathway. The auditory pathway was studied on the 3rd day o...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Kohelet D,Usher M,Arbel E,Arlazoroff A,Goldberg M

    更新日期:1990-09-01 00:00:00

  • Abnormal adrenal steroidogenesis in growth-retarded newborn infants.

    abstract::The results of prior studies suggest that abnormalities of development and function of the fetal adrenal occur in pregnancies complicated by intrauterine fetal growth retardation (IUGR). In the present investigation, we sought to extend such studies by matching IUGR infants with normally grown infants of women in whom...

    journal_title:Pediatric research

    pub_type: 临床试验,杂志文章


    authors: Parker CR Jr,Buchina ES,Barefoot TK

    更新日期:1994-06-01 00:00:00

  • Carbohydrate and energy metabolism in the brain of rats with thromboxane A2-induced fetal growth restriction.

    abstract::Fetal growth restriction (FGR) remains a cause of perinatal brain injury, sometimes leading to neurological and intellectual impairment. Although the mechanisms and pathophysiology of CNS injuries have not been elucidated completely, it is possible carbohydrate and energy metabolism may have an important role in the F...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Hayakawa M,Sato Y,Hattori T,Ichinohashi Y,Nakayama A,Yamamoto H,Hemmi H,Ito M,Ieda K,Kojima S

    更新日期:2011-07-01 00:00:00

  • Role of pili in Haemophilus influenzae adherence to, and internalization by, respiratory cells.

    abstract::To determine the role of pili in mediating adherence of type b and nontypeable Haemophilus influenzae to several respiratory cell types, piliated bacteria, nonpiliated phase variants, and nonpiliated mutants possessing insertionally inactivated pilin genes were tested. Both piliated and nonpiliated strains adhered to ...

    journal_title:Pediatric research

    pub_type: 杂志文章


    authors: Gilsdorf JR,Tucci M,Marrs CF

    更新日期:1996-02-01 00:00:00