Abstract:
:To elucidate the impact of nutrition in cystic fibrosis (CF), we compared the phenotypic traits of Cftr -/- mice fed either a lipid-enriched liquid diet (Peptamen) or a standard chow combined with polyethylenglycol osmotic laxative (PEG), two strategies commonly used to prevent intestinal obstruction in CF mice. Survival, growth, liver, and ventilatory status were determined in Cftr -/- and Cftr +/+ mice, followed-up until 120 d. Ventilation was recorded in conscious animals using whole-body plethysmography. We found that the survival rate was similar in Peptamen and PEG Cftr -/- mice. Cftr -/- mice had lower minute ventilation than Cftr +/+ mice, whatever the diet. Both Cftr -/- and Cftr +/+ mice fed Peptamen displayed preadult growth delay compared with PEG-treated animals. Despite subsequent growth catch-up, Cftr -/- mice remained smaller than Cftr +/+ mice, whatever the diet. All Peptamen fed Cftr -/- mice showed hepatomegaly and liver steatosis, which also occurred but to a lesser extent in Peptamen fed Cftr +/+ animals. Therefore, while both treatment strategies are similarly efficient to avoid high mortality at weaning, Peptamen induces preadult growth delay and liver steatosis. These effects of diet are important to consider in future animal studies and also prompt to evaluate high-energy diets in CF patients.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Cottart CH,Bonvin E,Rey C,Wendum D,Bernaudin JF,Dumont S,Lasnier E,Debray D,Clément A,Housset C,Bonora Mdoi
10.1203/PDR.0b013e318155a61dsubject
Has Abstractpub_date
2007-11-01 00:00:00pages
528-32issue
5eissn
0031-3998issn
1530-0447journal_volume
62pub_type
杂志文章abstract::BackgroundAdults born preterm have higher levels of cardiometabolic risk factors than their term-born peers. Studies have suggested that at least those born smallest eat less healthily. We examined the association between early (<34 weeks) and late (34-36 weeks) preterm birth and diet and food preferences in adult age...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.301
更新日期:2018-03-01 00:00:00
abstract::Acute kidney injury (AKI) is common in premature infants and is associated with poor outcomes. Novel biomarkers can detect AKI promptly. Because premature infants are born with underdeveloped kidneys, baseline biomarker values may differ. We describe baseline values of urinary neutrophil gelatinase-associated lipocali...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3182275164
更新日期:2011-09-01 00:00:00
abstract::The isobutyryl-CoA dehydrogenase (IBD) enzyme is involved in the degradation of valine. IBD deficiency was first reported in 1998 and subsequent genetic investigations identified acyl-CoA dehydrogenase (ACAD) 8, now IBD, as the gene responsible for IBD deficiency. Only three individuals homozygous or compound heterozy...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000233085.72522.04
更新日期:2006-09-01 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::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::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::C3H/HeJ mice develop an increase in terminal air space area detectable by postnatal d 14 that persists into adulthood compared with strain-matched controls (C3H/SnJ, C3H/OuJ). Morphometric quantification revealed a 50% increase in terminal air space area by postnatal d 14 and a 2.3-fold increase by 2 mo of age in C3H/...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000246071.50268.51
更新日期:2006-12-01 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:BACKGROUND:Later life metabolic dysfunction is a well-recognized consequence of being born small for gestational age (SGA). This study has applied metabolomics to identify whether there are changes in these pathways in prepubertal short SGA children and aimed to compare the intracellular and extracellular metabolome in...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2016.72
更新日期:2016-08-01 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::Long-term EEG monitoring (LTM) with several electrodes could be a useful tool for surveillance of the brain during the first critical days of life. This study aimed to assess the feasibility of multichannel LTM for automated analysis of EEG activity from d 1 to 3 using eight electrodes. Premature infants (GA <31 wk; n...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e31821267d2
更新日期:2011-05-01 00:00:00
abstract::We evaluated bone mineralization by single photon absorptiometry at 2 y in a cohort of preterm infants studied since birth. Infants were fed human milk fortified with Ca [to achieve 80 mg/dL (19.96 mmol/L)] and P [40 mg/dL (12.91 mmol/L)] from wk 2 through 8 after birth. After hospital discharge, infants were divided ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199206000-00009
更新日期:1992-06-01 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::Mucosal samples from rabbit jejunum were incubated (30 min, 25 degrees C) with (125I)glycinin in the presence of buffer, soybean lectin (50 micrograms/ml) soyasaponins (1 mg/ml), or both lectin and saponins. The mucosal uptake of (125I)glycinin was negligible with buffer, and increased progressively with additions of ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198209000-00005
更新日期:1982-09-01 00:00:00
abstract::Neonatal PMN (polymorphonuclear neutrophils) exhibit altered inflammatory responsiveness and greater longevity compared with adult PMN; however, the involved mechanisms are incompletely defined. Receptors containing immunoreceptor tyrosine-based inhibitory motif (ITIM) domains promote apoptosis by activating inhibitor...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181b1bc19
更新日期:2009-09-01 00:00:00
abstract:BACKGROUND:The associations between prenatal exposure to endocrine disruptive chemicals (EDCs) and fetal growth are inconsistent, and few studies have considered small-for-gestational-age (SGA) birth as an outcome. Our current study of Scandinavian parous women aimed to address these inconsistencies and gaps in the lit...
journal_title:Pediatric research
pub_type: 杂志文章,多中心研究
doi:10.1038/pr.2016.187
更新日期:2017-01-01 00:00:00
abstract::An infant with the acute neonatal form of pyruvate carboxylase deficiency (cross-reacting material negative) presented with severe intractable lactic acidosis within 4 h after birth. He also had hyperammonemia, hypercitrullinemia, and hyperlysinemia. Plasma glutamine was not elevated. He had a rapidly deteriorating cl...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198603000-00016
更新日期:1986-03-01 00:00:00
abstract::Pseudohypoaldosteronism is a hereditary salt-wasting syndrome usually seen in infancy with weight loss, dehydration, and failure to thrive. The pathophysiologic origin of pseudohypoaldosteronism is unknown. The defect could be related to the unresponsiveness of target organs to mineralocorticoids resulting in hyponatr...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199403000-00021
更新日期:1994-03-01 00:00:00
abstract::Despite effective antibiotic treatment, neuronal injury is frequent among children and adults with bacterial meningitis resulting in a high rate of death and neurologic sequelae. The hematopoietic cytokine erythropoietin (EPO) provides neuroprotection in models of acute and chronic neurologic diseases. We studied whet...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e318159af7a
更新日期:2007-12-01 00:00:00
abstract::We measured the cortisol and corticosterone responses to insulin-induced hypoglycemia in 13 growth hormone (GH)-deficient children and 30 short children without GH deficiency. Although there was no difference between the two groups in degree of hypoglycemia attained, baseline cortisol, baseline corticosterone, or cort...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198508000-00009
更新日期:1985-08-01 00:00:00
abstract::We studied the effect of maternal concentration and mode of administration on transfer rate of TSH-releasing hormone (TRH) in an in vitro model of dually perfused isolated lobule of human term placenta. Varying concentrations (4-40 microg dL(-1)) of TRH were added either to the open maternal circulation to mimic an in...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199705000-00012
更新日期:1997-05-01 00:00:00
abstract:BACKGROUND:To investigate whether the YAP/TAZ (Yes-associated protein/transcriptional coactivator with PDZ binding motif) pathway contributes to the pathogenesis of lymphatic malformations (LMs). METHODS:YAP, TAZ, CTGF (connective tissue growth factor), and Ki-67 were detected in LMs by immunohistochemistry. The coloc...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-0863-0
更新日期:2020-04-12 00:00:00
abstract::Lung development progresses through a process reliant on mechanical cell stretch. However, this process is not well defined at the molecular level. Our goal was to globally analyze the transcriptome of fetal mouse lungs following short periods of tracheal occlusion (TO) to identify cellular processes that are rapidly ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e31815eba47
更新日期:2008-02-01 00:00:00
abstract:BACKGROUND:Biliary atresia (BA) is an infantile disorder characterized by progressive sclerosing cholangiopathy leading to biliary obstruction. First-line treatment of BA is hepatoportoenterostomy, the prognosis of which is related to age at surgery and to histological variables such as extent of fibrosis and ductular ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.177
更新日期:2014-01-01 00:00:00
abstract::The age-related changes in fasting arterial levels of energy substrates and insulin were studied at birth and/or 6 wk in eleven baboon infants. In addition, the renal contribution to glucose release in the primate infant was estimated. Arterial blood glucose levels were similar in six fasted newborns and in nine faste...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198008000-00006
更新日期:1980-08-01 00:00:00
abstract::Treatment with phototherapy or with the lipase inhibitor orlistat decreases plasma unconjugated bilirubin (UCB) concentrations in hyperbilirubinemic Gunn rats. We investigated the mechanism(s) underlying the effects of orlistat, phototherapy, and combined treatment, using steady-state 3H-UCB kinetics. After three week...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000203180.79636.98
更新日期:2006-04-01 00:00:00
abstract::Whether severe hypoglycemia alone or in combination with hyperketonemia might cause deterioration of cardiac function has been controversial. Therefore, the influence of acute hypoglycemia (mean 33 mg/dL) with and without hyperketonemia (mean 1.3 and 3.3 mM) on cardiac function, substrate utilization, and myocardial h...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198912000-00002
更新日期:1989-12-01 00:00:00
abstract:BACKGROUND:Food allergy is common in children with prevalence up to 10%. We assessed the clinico-laboratory characteristics and frequency of food sensitization to the commonly consumed food among upper Egyptian preschool children with recurrent wheezy chest. METHODS:This cross-sectional descriptive study was conducted...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-0960-0
更新日期:2020-10-01 00:00:00
abstract::One of two slightly different influenza A/ts-1[E] recombinant candidate live vaccines was given intranasally to each of 23 young children. Twelve of 15 children who had no serum HI antibody but who did have serum ANAB at the time of administration became infected and 1 had mild rhinitis. All eight who lacked both type...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197604000-00008
更新日期:1976-04-01 00:00:00
abstract::To investigate the detailed pattern of change in circulating gonadotropin and testosterone concentrations around the onset of puberty and to determine the temporal relationship between the gonadotropin and testosterone secretion, plasma gonadotropin and testosterone were measured at 20-min intervals for 24 h in 21 nor...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199308000-00026
更新日期:1993-08-01 00:00:00