Abstract:
BACKGROUND:A growing body of evidence defines inflammation as a hallmark feature of disease pathogenesis of Duchenne muscular dystrophy. To tailor potential immune modulatory interventions, a better understanding of immune dysregulation in Duchenne muscular dystrophy is needed. We now asked whether dystrophin deficiency affects the cascade of leukocyte recruitment. METHODS:We performed intravital microscopy on the cremaster muscle of wild-type and dystrophin-deficient mdx mice. Recruitment was triggered by preparation alone (traumatic inflammation) or in combination with scrotal TNFα injections. Neutrophilic infiltration of the cremaster muscle was assessed on tissue sections. Integrin expression on circulating neutrophils and serum levels of pro-inflammatory cytokines were measured by flow cytometry. RESULTS:Mdx mice show increased rolling and adhesion at baseline (traumatic inflammation) and a more profound response upon TNFα injection compared with wild-type animals. In both models, neutrophilic infiltration of the cremaster muscle is increased. Upregulation of the integrins LFA-1 and Mac-1 on circulating leukocytes and pro-inflammatory cytokines IL-6 and CCL2 in the serum points toward systemically altered immune regulation in mdx mice. CONCLUSION:We are the first to show exaggerated activation of the leukocyte recruitment cascade in a dystrophin-deficient organism in vivo.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Kranig SA,Tschada R,Braun M,Patry C,Pöschl J,Frommhold D,Hudalla Hdoi
10.1038/s41390-019-0427-3subject
Has Abstractpub_date
2019-08-01 00:00:00pages
188-194issue
2eissn
0031-3998issn
1530-0447pii
10.1038/s41390-019-0427-3journal_volume
86pub_type
杂志文章abstract::Early-life stress has been shown to destabilize the homeostatic synaptic plasticity and compromise the developing brain to the later encountered insults. This study would determine the long-term epileptogenic effect of neonatal isolation (NI) on early-life seizure. There were five groups: normal rearing (NR) rats; NI ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181b337d2
更新日期:2009-10-01 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::The effect of increased red blood cell aggregation on uteroplacental blood flow was studied in 11 awake, late-pregnant guinea pigs. The aggregation of the red cells was increased by administering high molecular weight dextran (HMWD) to the previously hemoconcentrated animal. The purpose of the hemoconcentration before...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199201000-00017
更新日期:1992-01-01 00:00:00
abstract::The factors that mediate the postnatal fall in pulmonary vascular resistance, which is crucial for normal gas exchange, are not fully understood. The endothelium has been implicated in this phenomenon, through the release of vasorelaxant factors such as nitric oxide (NO). Human pulmonary expression of endothelial NO s...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/01.PDR.0000159575.58834.8D
更新日期:2005-05-01 00:00:00
abstract::Sugar alcohols have been found to play an important osmoregulatory role both in unicellular organisms and, more recently, in multicellular organisms, including mammals. This study shows that myo-inositol accumulates in the brains of chronically hypernatremic mice, as had been earlier found in rats, and demonstrates fo...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198911000-00024
更新日期:1989-11-01 00:00:00
abstract::It is customary to estimate the uteroplacental function in singletons by defining appropriateness of birth weight by gestational age. Such a measure, however, is not available for the entire multiple pregnancy set. We evaluate a new index, total triplet birth weight, expressed as multiples of the median (MOM) birth we...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000202757.47761.76
更新日期:2006-04-01 00:00:00
abstract::The association between lung injury and thrombocytopenia was investigated by comparing the megakaryocyte and platelet counts, and platelet activation using P-selectin as a marker, between the prepulmonary (right atrial) and postpulmonary (left atrial) blood in adult and neonatal (preterm and term) rats with and withou...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000079186.86219.29
更新日期:2003-09-01 00:00:00
abstract::We examined the effect of inspiratory resistive loaded breathing (IRL) on the electromyographic (EMG) activity of the costal and crural diaphragm in nine anesthetized spontaneously breathing piglets (age 10-23 days, weight 2.8-4.4 kg). Bipolar wire electrodes were inserted into the anterior paratendinous costal diaphr...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198701000-00007
更新日期:1987-01-01 00:00:00
abstract::The metabolic demands of the newborn heart are met primarily by glucose and lactate. Mitochondria are impermeable to the NADH produced by these cytosolic reactions. The malate/aspartate and alpha-glycerophosphate (alpha-GP) shuttles provide two pathways to transport reducing equivalents into mitochondria. The goals of...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199508000-00015
更新日期:1995-08-01 00:00:00
abstract::Prenatal maternal stress has been shown to impair functioning in nonhuman primate offspring. Little is known about the effects of prenatal stress on intellectual and language development in humans because it is difficult to identify sufficiently large samples of pregnant women who have been exposed to an independent s...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000136281.34035.44
更新日期:2004-09-01 00:00:00
abstract::A study using fractional clearances during orally induced water diuresis was designed to delineate the mechanism underlying defective tubular reabsorption of sodium in very low-birth-weight neonates. The use of clearance methodology during maximal water diuresis may give an indirect estimate of distal sodium delivery ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198312000-00017
更新日期:1983-12-01 00:00:00
abstract::The effect of hypertension in pregnant women on fetal maturation is an issue of considerable importance. Because of a possible role of prolactin in fetal adrenal steroidogenesis and in fetal lung maturation, we have investigated the relationship between hypertension in pregnant women and levels of prolactin and dehydr...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198609000-00014
更新日期:1986-09-01 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
abstract:INTRODUCTION:Corticosteroids have been widely used in clinical medicine as a first-line therapy to modify the inflammatory response in many pulmonary and systemic diseases. Inhaled and intratracheally administered corticosteroids have a particular interest in that their use allows the clinician to circumvent systemic s...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2011.78
更新日期:2012-04-01 00:00:00
abstract:BACKGROUND:Observational studies suggest that asthma control improves after adenotonsillectomy, but longitudinal studies that correlate the effect of the procedure on the levels of biomarkers associated with airway inflammation are limited. METHODS:We conducted a longitudinal, observational study on pediatric patients...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.237
更新日期:2014-03-01 00:00:00
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...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199506000-00014
更新日期:1995-06-01 00:00:00
abstract::The history of Pediatric Infectious Diseases closely parallels the history of Pediatrics at least until the last century, because historically infections comprised the major causes of childhood morbidity and mortality, as they still do in the developing world. This history reviews developments in the field through the...
journal_title:Pediatric research
pub_type: 历史文章,杂志文章
doi:10.1203/01.PDR.0000101756.93542.09
更新日期:2004-01-01 00:00:00
abstract::BackgroundExtracellular adenine nucleotides contribute to ischemia-reperfusion injury following infant cardiopulmonary bypass (CPB), whereas conversion to adenosine may be protective. Alkaline phosphatase (AP), a key enzyme responsible for this conversion, decreases after infant CPB. Indirect evidence suggests that so...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.325
更新日期:2018-04-01 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::Twenty-four samples of breast milk from nine mothers of infants suffering from breast milk jaundice were studied. Eight samples of milk from mothers of nonjaundiced infants, along with five formula milks enriched with polyunsaturated fatty acids, served as controls. Milks from mothers with jaundiced infants had no inh...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197606000-00007
更新日期:1976-06-01 00:00:00
abstract:BACKGROUND:Deficiencies in phagocytosis may contribute to the increased susceptibility of infants to early life infections. Data on phagocytosis of the major neonatal pathogens Staphylococcus epidermidis (SE), Staphylococcus aureus (SA), and Escherichia coli (EC) by preterm infant leukocytes are inconsistent. METHODS:...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.145
更新日期:2013-11-01 00:00:00
abstract::The aim of this study was to produce a neonatal piglet model which, avoiding vessel ligation, exposed the whole animal to hypoxia and produced dose-dependent clinical encephalopathy and neuropathologic damage similar to that seen after birth asphyxia. Twenty-three piglets were halothane-anesthetized. Hypoxia was induc...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199611000-00014
更新日期:1996-11-01 00:00:00
abstract:BACKGROUND:Current tools to predict the severity of respiratory syncytial virus (RSV) infection might be improved by including immunological parameters. We hypothesized that a combination of inflammatory markers would differentiate between severe and mild disease in RSV-infected children. METHODS:Blood and nasopharyng...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2012.163
更新日期:2013-02-01 00:00:00
abstract::Bilirubin-IX alpha photooxidation products were detected by high performance liquid chromatography in the urine of neonates undergoing phototherapy for hyperbilirubinemia. The in vivo photoproducts were identified by chromatographic comparison with authentic synthetic standards using two complementary methods. Bilirub...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198408000-00003
更新日期:1984-08-01 00:00:00
abstract::Genetic typing of serum transferrin was performed in a group of 88 extremely premature infants (gestation age less than 32 wk) and in a control group of 351 full-term infants, using isoelectric focusing technique on ultrathin layer of polyacrilamide gel. A major incidence of C2 type was found among the preterm infants...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198212000-00011
更新日期:1982-12-01 00:00:00
abstract::Skin fibroblasts derived from 6 patients with cystic fibrosis (CF), 1-6 months old, and from 6 age matched donors were investigated for their ability to accumulate cyclic adenosine 3':5'-monophosphate (c-AMP) in response to isoproterenol and prostaglandin E1 (PGE1) using strictly defined culture conditions. In order t...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198003000-00018
更新日期:1980-03-01 00:00:00
abstract::The use of tissue engineering to develop new urinary bladder could have direct impact on children suffering from congenital bladder dysfunction. Current bladder tissue engineering protocols rely on scaffolds to support the ingrowth of new cells and the subsequent development of new tissues. The interaction between cel...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/PDR.0b013e3181d35034
更新日期:2010-05-01 00:00:00
abstract::BACKGROUNDThe aims of this study were to (i) compare the concentrations of two neural injury markers, S100B protein and neuron-specific enolase (NSE), in intrauterine growth-restricted (IUGR) fetuses and in fetuses with appropriate growth-for-gestational-age (AGA), and (ii) investigate potential relationships between ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.108
更新日期:2017-09-01 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::A son of related Turkish parents had grossly elevated serum tyrosine concentration and excreted tyrosine and p-hydroxyphenolic acids into the urine, whereas neither succinylacetone nor succinylacetoacetate could be demonstrated. The tyrosine concentration was normalized by a proper diet. This was not followed strictly...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198407000-00023
更新日期:1984-07-01 00:00:00