Abstract:
:The etiology of biliary atresia (BA) is not yet understood, but recent studies have shown inflammation with an up-regulated interferon (IFN) activity in the intra- and extrahepatic bile ducts of patients with BA. These findings support an inflammatory/infectious cause of BA as mimicked in our infective murine model. To study the role of the IFN receptors in our model, we used mice with inactivated INF-alpha/beta receptor A129, with inactivated IFN-gamma receptor G129, or inactivation of both interferon receptors AG129 as well as the wild type controls W129. Mice were infected with rotavirus within 48h of birth and 7 d postpartum. The incidence of BA in each group was determined during a 3 wk period. In the second week the virus load was measured. BA incidence was 76% in A129 and 67% in AG129 animals, whereas in the G129 group only 33% of the pups developed BA. The wild type presented with a BA-incidence of 15%, while 7 d old mice failed to develop BA. There was no significant difference in the virus load of the livers between the groups independent of clinical symptoms. In conclusion, inactivation of type I INF-receptor significantly increases the incidence of BA following postpartal rotavirus infection. This effect is independent of the presence of type II-INF-receptors. Thus, in our model a type I IFN-linked deregulation of the innate immune system appears to be crucial for the induction of biliary atresia.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Kuebler JF,Czech-Schmidt G,Leonhardt J,Ure BM,Petersen Cdoi
10.1203/01.pdr.0000219860.96732.09subject
Has Abstractpub_date
2006-06-01 00:00:00pages
790-4issue
6eissn
0031-3998issn
1530-0447pii
01.pdr.0000219860.96732.09journal_volume
59pub_type
杂志文章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
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更新日期:2017-05-01 00:00:00
abstract::Hypoxic ischemia is a common cause of damage to the fetal and neonatal brain. Although systemic and cerebrovascular physiologic factors play an important role in the initial phases of hypoxic-ischemic injuries, the intrinsic vulnerability of specific cell types and systems in the developing brain may be more important...
journal_title:Pediatric research
pub_type: 杂志文章,评审
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更新日期:2001-06-01 00:00:00
abstract::Innate immunity and urinary tract response play a central role in the development of urinary tract infection (UTI). Heat shock protein (HSP) 72 and Toll-like receptor (TLR) 4 are among the key elements of innate defence mechanisms. This study assesses the role of HSPA1B A(1267)G and TLR4 A(896)G polymorphisms using al...
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doi:10.1203/pdr.0b013e318030d1f4
更新日期:2007-03-01 00:00:00
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pub_type: 杂志文章
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更新日期:2017-10-01 00:00:00
abstract::Lung development requires extracellular matrix remodeling. This involves matrix metalloproteinases (MMPs) and their endogenous inhibitors [tissue inhibitors of metalloproteinases (TIMPs)]. Because these have been generally studied only in whole lung, we focused specifically on mesenchymal and epithelial cells freshly ...
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pub_type: 杂志文章
doi:10.1203/PDR.0b013e3180686cc5
更新日期:2007-07-01 00:00:00
abstract::The uterus is unique among smooth muscular organs in that, during pregnancy, it undergoes profound, largely reversible, changes orchestrated by the ovarian hormones. These changes facilitate uterine adaptation to the stretch induced by the growing fetus such that a state of myometrial contractile quiescence can be mai...
journal_title:Pediatric research
pub_type: 杂志文章,评审
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更新日期:1998-11-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199204000-00023
更新日期:1992-04-01 00:00:00
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doi:10.1203/00006450-200010000-00022
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pub_type: 杂志文章,多中心研究
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更新日期:2008-07-01 00:00:00
abstract::The objective was to determine in infants with perinatal depression whether the relative concentrations of N-acetylaspartate and lactate in the neonatal period are associated with (1) neurodevelopmental outcome at 30 mo of age or (2) deterioration in outcome from age 12 to 30 mo; and to determine whether socioeconomic...
journal_title:Pediatric research
pub_type: 杂志文章
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更新日期:2002-07-01 00:00:00
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pub_type: 杂志文章
doi:10.1038/s41390-019-0518-1
更新日期:2019-11-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200202000-00018
更新日期:2002-02-01 00:00:00
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pub_type: 杂志文章
doi:10.1203/01.PDR.0000103931.80055.06
更新日期:2004-02-01 00:00:00
abstract:BACKGROUND:Kawasaki disease (KD) is an acute febrile illness associated with the development of vasculitis. Administration of intravenous immunoglobulin (IVIG) is the standard treatment for KD. However, IVIG treatment is not effective in approximately 15% of children with KD. Some reports have presented evidence of imm...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2015.12
更新日期:2015-04-01 00:00:00
abstract:INTRODUCTION:Data regarding neonatal arterial ischemic stroke (NAIS) topography are still sparse and inaccurate. Despite the importance of locating NAIS to predict the long-term outcome of neonates, a map of arterial territories is not yet available. Our aim was therefore to generate the first three-dimensional map of ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0724-x
更新日期:2020-06-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章,评审
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更新日期:2010-05-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199508000-00005
更新日期:1995-08-01 00:00:00
abstract::Changes in high-density lipoprotein (HDL) subclass distribution were evaluated in a group of premature infants during the early postnatal period to ascertain whether enteral feeding brought about a rapid shift from neonatal to adult-like distributions. All infants were fed a combination of breast milk and formula. Cor...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198806000-00001
更新日期:1988-06-01 00:00:00
abstract::Progress has been made in the reduction of morbidity and mortality from neonatal sepsis. However, diagnosis continues to rely primarily on conventional microbiologic techniques, which can be inaccurate. The objective of this review is to provide the clinician with an overview of the current information available on di...
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pub_type: 杂志文章,评审
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更新日期:2017-10-01 00:00:00
abstract::Since the description of bronchopulmonary dysplasia (BPD) in premature infants, the supplemental oxygen administered has been suspect in the etiology of BPD. This has prompted studies on the effect of hyperoxia on lung growth in neonatal animals. So far, these have not led to a treatment which either prevents or mitig...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199608000-00007
更新日期:1996-08-01 00:00:00
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journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/00006450-197504000-00009
更新日期:1975-04-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200012000-00017
更新日期:2000-12-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197906000-00002
更新日期:1979-06-01 00:00:00
abstract::The effect of ingestion of uncooked cornstarch (2 g/kg body weight) in water, uncooked starch (1 g/kg) added to a meal, and glucose (2 g/kg) in water, was studied in eight patients with type IA glycogen storage disease (GSD) and one patient with type IB GSD. Blood glucose concentrations were determined at 30-min inter...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198409000-00015
更新日期:1984-09-01 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
abstract::A substantial proportion of the "apparently normal" preterm infants exhibit minor and moderate dysfunctions in neuromotor outcome as they grow older. Birth characteristics, minor abnormalities on the neonatal ultrasound scan of the brain, and motor milestones have only limited value in the early detection of these chi...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000170898.60160.09
更新日期:2005-08-01 00:00:00
abstract::The present study examined the extent to which abundance of the prolactin receptor (PRLR) and a range of primary mitochondrial proteins are influenced by either maternal nutrition and/or fetal number in adipose tissue. Pregnant sheep were control fed [consuming 100% of total metabolizable energy (ME) requirements (tak...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000047653.73271.C4
更新日期:2003-02-01 00:00:00
abstract::Programmed proliferative degeneration of the human fetal ductus arteriosus (DA) preceding definite postnatal closure has a large developmental variability and is controlled by several signaling pathways. Among these vascular endothelial growth factor (VEGF) and its receptors (VEGF-Rs) play an important role. Until now...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e318180a375
更新日期:2008-10-01 00:00:00
abstract::Cerebral malaria is the most severe neurological complication of infection with Plasmodium falciparum. With >575,000 cases annually, children in sub-Saharan Africa are the most affected. Surviving patients have an increased risk of neurological and cognitive deficits, behavioral difficulties, and epilepsy making cereb...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/PDR.0b013e3181eee738
更新日期:2010-10-01 00:00:00
abstract::Ambient oxygen concentration and vascular endothelial growth factor (VEGF)-A are vital in lung development. Since hypoxia stimulates VEGF-A production and hyperoxia reduces it, we hypothesized that VEGF-A down-regulation by exposure of airways to hyperoxia may result in abnormal lung development. An established model ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e31815b4857
更新日期:2008-01-01 00:00:00