Abstract:
:Mutations in multidrug resistance 3 gene (MDR3 or ABCB4) underlie progressive familial intrahepatic cholestasis type 3 (PFIC3), a severe pediatric liver disease progressing to cirrhosis. Abcb4-/- mice exhibit slowly developing hepatic lesions that can be accelerated by feeding a cholic acid (CA)-supplemented diet. We investigated the beneficial effects of a soybean lecithin (L)-supplemented diet in this model of liver disease. Abcb4-/- mice and wild-type (WT) controls were divided in four groups by the diet they were fed: control (C) diet, L-supplemented diet, CA-supplemented diet, and L- and CA-supplemented (L+CA) diet. After 2 wk on these regimens, liver enzymes and bilirubin were measured in serum with bile flow, total bile acids, and cholesterol (CHOL) and phospholipid (PL) concentrations in bile. Ductular hyperplasia, portal fibroblastic cell proliferation, myofibroblast activation, and hepatic fibrosis were quantified on liver sections. Abcb4-/- mice fed the C diet exhibited mild liver damage. CA produced very high elevations of serum liver enzymes and bilirubin with significant bile duct proliferation, peribiliary fibroblast activation, and fibrosis. The L-supplemented diet dramatically mitigated the hepatic damage in CA-supplemented diet animals. We conclude that L is protective against liver disease in Abcb4-/- mice and suggest that it could offer potential benefit in PFIC3.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Lamireau T,Bouchard G,Yousef IM,Clouzeau-Girard H,Rosenbaum J,Desmoulière A,Tuchweber Bdoi
10.1203/pdr.0b013e31802d7780subject
Has Abstractpub_date
2007-02-01 00:00:00pages
185-90issue
2eissn
0031-3998issn
1530-0447pii
00006450-200702000-00012journal_volume
61pub_type
杂志文章abstract::To investigate the difference in ventilatory response to exercise between children and young adults, we administered a treadmill progressive exercise test to seven boys (aged 8 to 11 y [group A]) and six male young adults (aged 14 to 21 y [group B]), who had a history of Kawasaki disease without significant coronary a...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199903000-00017
更新日期:1999-03-01 00:00:00
abstract::Contributions from multidisciplinary investigations have focused attention on the potential of tissue engineering to yield novel therapeutics. Congenital malformations, including cleft palate, craniosynostosis, and craniofacial skeletal hypoplasias represent excellent targets for the implementation of tissue engineeri...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/PDR.0b013e31816a743e
更新日期:2008-05-01 00:00:00
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
doi:10.1203/00006450-199602000-00025
更新日期:1996-02-01 00:00:00
abstract::Plasma amino acid levels in newborn babies are often measured as part of a screening for inherited metabolic disease, or less commonly as part of a nutritional assessment. We have examined certain factors which affect the levels as measured in low birth weight babies during the first 3 wk of life. As in other studies,...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198505000-00008
更新日期:1985-05-01 00:00:00
abstract::We have shown that vitamin A (VA) and retinoic acid (RA) synergistically increase lung retinyl ester content in neonatal rats. To confirm whether this biochemical synergism attenuates early neonatal hyperoxic lung injury in mice, we exposed newborn C57BL/6 mice to 95% O2 or air from birth to 4 d. The agent [vehicle, V...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181dbac3d
更新日期:2010-06-01 00:00:00
abstract:BACKGROUND:Extremely preterm birth is associated with subsequent behavioral problems. We hypothesized that perinatal systemic inflammation, a risk factor for cerebral white matter injury and cognitive impairment, is associated with behavior problems observed at 2 y. METHODS:In a cohort of 600 children born before 28 w...
journal_title:Pediatric research
pub_type: 杂志文章,多中心研究
doi:10.1038/pr.2014.41
更新日期:2014-06-01 00:00:00
abstract::Evidence is inconsistent or poorly understood for links between polychlorinated biphenyls (PCBs), 1,1'-dichloro-2,2'-bis(4-chlorophenyl)ethylene (DDE), and organophosphate (OP) pesticides and adverse pregnancy outcomes, although they are known developmental toxicants. We measured biomarkers of maternal exposure to DDE...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/pdr.0b013e31802d77f0
更新日期:2007-02-01 00:00:00
abstract::We have previously shown that generation of superoxide anion occurs in cerebral cortex during asphyxia/reventilation in newborn pigs and that a high dose of indomethacin (5 mg/kg i.v.) abolishes superoxide anion production. The purposes of this study were 1) to determine whether the generation of superoxide anion occu...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199309000-00025
更新日期:1993-09-01 00:00:00
abstract::The effect of hypoxia-ischemia (HI) on IL-1, and IL-6 bioactivity in relation to expression of IL-1 alpha, IL-1 beta, and IL-6 mRNA was studied, and the neuroprotective efficacy of IL-1 receptor antagonist (IL-1ra) was evaluated in neonatal rats. HI was induced in 7-d-old rats by unilateral carotid artery ligation and...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199610000-00015
更新日期:1996-10-01 00:00:00
abstract:BACKGROUND:The aim of our study was to understand the relationship between consanguineous marriages and reproductive outcomes. METHODS:A total of 999 families were recruited from five Muslim populations of Jammu region. Family pedigrees were drawn to access the family history and inbreeding status in terms of coeffici...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2016.177
更新日期:2017-01-01 00:00:00
abstract::Epidermal growth factor (EGF) and transforming growth factor-alpha (TGF-alpha) are mitogenic to the intestinal epithelium. To further clarify their role in the developing human fetal gut, their expression was studied in fetuses at 15 to 20 wk of gestation. TGF-alpha mRNA was present throughout the gastrointestinal tra...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199305000-00012
更新日期:1993-05-01 00:00:00
abstract::We have investigated the impaired secretion response of neonatal platelets. We compared the response of washed neonatal and adult platelets to thrombin and collagen, and to specific activators of calcium flux (inositol trisphosphate) and protein kinase C activation (oleoyl-acetyl glycerol). Neonatal platelets show no ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199004000-00004
更新日期:1990-04-01 00:00:00
abstract::Sudden infant death syndrome (SIDS) is multifactorial and may result from the interaction of a number of environmental, genetic, and developmental factors. We studied three major genes causing long QT syndrome in 42 Japanese SIDS victims and found five mutations, KCNQ1-K598R, KCNH2-T895M, SCN5A-F532C, SCN5A-G1084S, an...
journal_title:Pediatric research
pub_type: 杂志文章,多中心研究
doi:10.1203/PDR.0b013e3181841eca
更新日期:2008-11-01 00:00:00
abstract::Quantitation of the body's fat and learn masses is an important component of nutritional assessment. Such measurements, however, are difficult to conduct routinely in infants due to the numerous limitations of traditional methods. The application of total body electrical conductivity measurements for quantitating fat-...
journal_title:Pediatric research
pub_type: 杂志文章
doi:
更新日期:1995-01-01 00:00:00
abstract::Maternal smoking doubles the risk of delivering a low birth weight infant. The purpose of this study was to analyze differential gene expression in umbilical cord tissue as a function of maternal smoking, with an emphasis on growth-related genes. We recruited 15 pregnant smokers and 15 women who never smoked during pr...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e31817c5507
更新日期:2008-08-01 00:00:00
abstract::Thrombocytopenia in the first days of life, in association with evidence of reduced megakaryocytopoiesis and platelet production at birth, is common in sick preterm babies. Thrombopoietin (Tpo) is the major regulator of platelet production in adults. However, these babies have low Tpo levels at birth, suggesting that ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199907000-00005
更新日期:1999-07-01 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::To evaluate the maturation of glucose homeostasis in the small for gestational age (SGA) neonate, glucose kinetics were measured with 78% enriched D-[U-13C]glucose by the prime plus constant infusion technique in nine SGA infants and compared with the rate obtained in seven term appropriate for gestational age infants...
journal_title:Pediatric research
pub_type: 杂志文章
doi:
更新日期:1984-01-01 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::Infection with human immunodeficiency virus (HIV) causes a progressive immunodeficiency, often manifested in adults by the development of infections with relatively rare, opportunistic organisms. However, in HIV-infected children, recurrent serious infections with common encapsulated bacteria, as well as recurrent min...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验,评审
doi:10.1203/00006450-199305001-00464
更新日期:1993-01-01 00:00:00
abstract::We studied the pre- and postnatal developmental regulation of the hepatic type I IGF receptor in the rat. Fetal rat liver membranes bound IGF-I throughout the latter part of gestation (d 17 to 21). After birth, binding diminished rapidly, reaching barely detectable levels by the 13th postnatal d. However, the presence...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199103000-00002
更新日期:1991-03-01 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::There is great interest in the regenerative potential of the neural stem cells and progenitors that populate the germinal zones of the immature brain. Studies using animal models of pediatric brain injuries have provided a clearer understanding of the responses of these progenitors to injury. In this review, we have c...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/pr.2017.261
更新日期:2018-01-01 00:00:00
abstract::N-formyl-L-methionyl-L-leucyl-L-phenylalanine (FMLP) and N-formyl-L-methionyl-L-phenylalanine (FMP) were used to investigate neutrophil (PMN) aggregation. Neutrophils were isolated from healthy adult volunteers and term newborn cord blood. Neutrophil aggregation was measured after the addition of FMLP and FMP. Adult P...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198312000-00013
更新日期:1983-12-01 00:00:00
abstract::The visual evoked potential (VEP) of five healthy, near-term exteriorized fetal lambs was compared with the VEP obtained from the same animals during the first hour after ventilation. Characteristics of the individual wave components and their relationship to blood pressure, heart rate, and arterial blood gas measurem...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198111000-00009
更新日期:1981-11-01 00:00:00
abstract::Hyperprostaglandin E syndrome (HPS), the prenatal variant of Bartter's syndrome, is characterized by a marked and selective stimulation of prostaglandin E (PGE2) synthesis. In the study group HPS patients showed increased urinary levels of PGE2, an index of renal, and of 11 alpha-hydroxy-9,15-dioxo-2,3,4,5,20-pentanor...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199708000-00019
更新日期:1997-08-01 00:00:00
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
doi:10.1203/00006450-199406000-00003
更新日期:1994-06-01 00:00:00
abstract::BackgroundLactoferrin (LTF) could play a beneficial role in insulin resistance and diabetes, but the association of its gene variants with cardio-metabolic disorders in children has not been investigated. This study aimed to examine the relationship between LTF variants, plasma LTF concentrations, and cardio-metabolic...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.72
更新日期:2017-11-01 00:00:00
abstract::This report describes a third mucopolysaccharidosis in animals: canine mucopolysaccharidosis VII. The affected dog was the offspring of a father-daughter mating. Weakness in the rear legs was evident at 8 weeks of age and became progressively worse. He had a large head, a shortened maxilla, and corneal granularities. ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198410000-00014
更新日期:1984-10-01 00:00:00
abstract::The carbohydrate-deficient glycoprotein syndromes are a recently individualized group of genetic multisystemic disorders. A predominant feature is a severe involvement of the central and peripheral nervous system resulting in psychomotor retardation, seizures, ataxia, and, mostly after infancy, stroke-like episodes. T...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199305000-00024
更新日期:1993-05-01 00:00:00