Abstract:
:We evaluated the effectiveness of administration of high-dose busulfan (BU), an alkylating agent with substantial myeloablative but negligible immunosuppressive properties, on the engraftment of congenic normal bone marrow and spleen cells (hematopoietic cell transplantation; HCT) in murine galactosylceramidase deficiency (the twitcher mouse), a model of human sphingolipid storage disease. Untreated mice died with extensive demyelination and failure to thrive at a median age of 40 d (range, 28-47; n = 51). The life-span of twitcher mice given HCT at age 10 d after 100 mg/kg BU was significantly prolonged (median, 94 d; range, 55-140; n = 17); these animals did not develop the hindlimb paralysis seen in untreated twitchers. Histologic examination of twitcher sciatic nerves after HCT showed remyelinated areas and decreased globoid cell inclusions. Animals given HCT after conditioning with BU had both lymphoid and hematopoietic repopulation with donor cells by 90 d after HCT, as documented by presence of donor glucose phosphate isomerase-1A activity in blood, bone marrow, spleen, and lymph nodes. After BU and HCT, galactosylceramidase activity in livers and spleens of twitcher mice reached 45 and 80% of control, respectively; only modest elevations were observed in kidneys and lymph nodes. Hydrolase activity rose to 20% in the brains and exceeded control values in the sciatic nerves of transplanted twitcher mice, indicating entry of at least some donor-derived cells and/or hydrolase across the blood-brain and blood-nerve barriers after BU and HCT.(ABSTRACT TRUNCATED AT 250 WORDS)
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Yeager AM,Shinohara M,Shinn Cdoi
10.1203/00006450-199103000-00016subject
Has Abstractpub_date
1991-03-01 00:00:00pages
302-5issue
3eissn
0031-3998issn
1530-0447journal_volume
29pub_type
杂志文章abstract::The major variation in effect between undiluted cystic fibrosis and heterozygote sera on rabbit tracheal epithelium was in terms of the time after exposure at which changes occurred. Exposure to both types of sera for 3 min caused release of goblet cell mucus and ciliary resorption possibly by apical swelling of colum...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198011000-00005
更新日期:1980-11-01 00:00:00
abstract:BACKGROUND:Adult human airway smooth muscle (ASM) produce cytokines involved in recruitment and survival of leukocytes within airway walls. Cytokine generation by adult ASM is glucocorticoid-sensitive. Whether developing lung ASM produces cytokines in a glucocorticoid-sensitive fashion is unknown. METHODS:Cultured fet...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2015.168
更新日期:2015-12-01 00:00:00
abstract::Pregnane steroids have sedative and neuroprotective effects on the brain, due to interactions with the steroid-binding site of the GABAA receptor. In the adult brain, synthesis of the pregnane steroids is increased in response to stress. Therefore, we have used umbilicoplacental embolization to mimic chronic placental...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000088066.47755.36
更新日期:2003-12-01 00:00:00
abstract::Endothelin-1 can cause pulmonary vasoconstriction via endothelin-A (ET(A)) receptor activation. We hypothesized that ET(A) blockers (EMD 122946 and BQ 610) would reduce hypoxia-induced (HYP) but not group B streptococcal infusion (GBS)-induced pulmonary hypertension in a juvenile whole animal model. Pulmonary hyperten...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200212000-00017
更新日期:2002-12-01 00:00:00
abstract::Magnesium-deficient fetuses exhibited malformations (44%), anemia, and edema. Maternal plasma magnesium levels at day 21 of pregnancy reflected the level of dietary magnesium (2.43 +/- 0.09 mg Mg/100 ml, control; 0.74 +/- 0.02 mg Mg/100 ml, deficient). Plasma magnesium levels of deficient fetuses showed similar decrea...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197706000-00013
更新日期:1977-06-01 00:00:00
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:BACKGROUND:Infant adiposity is linked to both high maternal fat mass (FM) and excessive gestational FM gain, whereas the association with maternal adipokines is less clear. The aim was to determine how levels of maternal leptin, the soluble leptin receptor (sOB-R), adiponectin, and FM during pregnancy were linked to in...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-01115-9
更新日期:2020-09-14 00:00:00
abstract::To study the normal development of blood coagulation factor activities in a growing fetus while avoiding the effects of labor and delivery, a chronic fetal lamb model was developed in which serial blood samples from 10 fetuses were studied during the third trimester of pregnancy and 24 hr after birth. Under operating ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198107000-00014
更新日期:1981-07-01 00:00:00
abstract::Necrotizing enterocolitis (NEC) remains among the most common and devastating diseases in neonates. Despite advances in neonatal clinical care, specific treatment strategies and diagnostic modalities remain lacking. As a result, morbidity and mortality remain high. Improved understanding of the pathogenesis of NEC has...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/s41390-020-1077-1
更新日期:2020-08-01 00:00:00
abstract::Intrauterine growth restriction (IUGR) is a recognized risk factor for neurologic deficits later in life. Abnormal fetal blood flow in the presence of fetal growth retardation helps to distinguish true fetal growth impairment from small but normally grown infants. The present study aimed to investigate the influence o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000129660.32875.09
更新日期:2004-07-01 00:00:00
abstract::Hypercholesterolemia is common in preterm infants administered 10% Intralipid perhaps because of excess phospholipid in plasma causing efflux of cholesterol from tissues. The purpose of this study was to determine if cholesterol synthesis (as measured by plasma lathosterol) is increased in preterm infants (23-32 wk ge...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199202000-00020
更新日期:1992-02-01 00:00:00
abstract::Human autoimmune lymphoproliferative syndrome (ALPS) is caused by defective CD95-mediated apoptosis of lymphocytes. In most patients, heterozygous mutations within the CD95 gene are found. Mutated proteins interfere with CD95-signaling in a dominant-negative way. However, the penetrance of clinical disease is variable...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e318191f7e4
更新日期:2009-02-01 00:00:00
abstract:BACKGROUND:Appearance of sleep cycling has been associated with good outcome in term and preterm infants, but the normal time of its appearance has not been determined. The objectives of this study were, to correlate the time of sleep cycling appearance and the length of quiet sleep in neonates with different degrees o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0560-z
更新日期:2020-03-01 00:00:00
abstract::High-frequency oscillatory ventilation (HFOV) may improve pulmonary outcome in very preterm infants, but the effects on the brain are largely unknown. We hypothesized that early prolonged HFOV compared with low volume positive pressure ventilation (LV-PPV) would not increase the risk of delayed brain growth or injury ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181bb0cc1
更新日期:2009-11-01 00:00:00
abstract:BACKGROUND:Infantile hemangioma (IH) is the most frequent vascular tumor of early childhood. Recently, propranolol, a nonselective β1- and β2-adrenoceptor inhibitor, was introduced into the therapy of severe proliferating IH with excellent results. However, the underlying mechanism of action of propranolol is still unc...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.16
更新日期:2013-04-01 00:00:00
abstract::Two infants with disorders of propionate metabolism were studied at 7 months of age to determine optimum levels of intake of protein and calories to meet the requirements for essential amino acid for growth in infancy, and at the same time minimize the accumulation of toxic intermediates. An effect of alanine was foun...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198501000-00023
更新日期:1985-01-01 00:00:00
abstract::Tracheal epithelial cells obtained from adult and infant ferrets were grown in primary culture in vitro. Cells from adult animals grew readily, and many ciliated cells were observed in the cultures. Successful cultures were derived from infant animals, but cell number in infant and adult cultures began to decrease aft...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199107000-00016
更新日期:1991-07-01 00:00:00
abstract::The length of the Y chromosome was measured in 42 black patients with trisomy 21 (47,XY,+21) and a similar number of normal individuals of American black ancestry. The length of the Y was expressed as a function of Y/F ratio and arbitrarily classified into five groups using subjectively defined criteria as follows: ve...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198209000-00012
更新日期:1982-09-01 00:00:00
abstract::The development of metabolic acidosis during neonatal sepsis with group B streptococci (GBS) has been attributed to progressive tissue ischemia resulting from reduced oxygen delivery (QO2). Using an animal model of GBS disease, we attempted to test this hypothesis by comparing the development of metabolic acidosis in ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198711000-00006
更新日期:1987-11-01 00:00:00
abstract:BACKGROUND:Continuous and intermittent bolus orogastric feedings are strategies used in infants unable to tolerate normal feeds. METHODS:To determine the effects of feeding modality on protein synthesis in different tissues, neonatal pigs received a balanced formula by orogastric tube as an intermittent bolus feed eve...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.89
更新日期:2013-08-01 00:00:00
abstract::We studied absorption of the potentially allergenic protein beta-lactoglobulin during acute rotavirus diarrhea in infants and assessed the relationship of this macromolecular absorption with intestinal sugar permeability. After oral rehydration, 38 patients with acute gastroenteritis were given orally a 100-ml solutio...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199109000-00019
更新日期:1991-09-01 00:00:00
abstract::The purpose of this project was to study the role of lipid peroxidation in oxygen-induced lung injury in the newborn lamb. It was our hypothesis that injury to the microvascular bed of the lung by oxygen would coincide with a burst of peroxidative activity and would be accompanied by an increased rate of excretion of ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199012000-00014
更新日期:1990-12-01 00:00:00
abstract::In eight newborn lambs, the adaption of blood to acidosis was studied by sequential measurements of P50 in vitro (pH 7.4; 37 degrees C) and P50 in vivo (animal's pH and temperature, 39 degrees C) during the course of HCl-induced metabolic acidosis. The benefit of the change in both P50 on tissue oxygen tension was stu...
journal_title:Pediatric research
pub_type: 杂志文章
doi:
更新日期:1981-05-01 00:00:00
abstract:BACKGROUND:Human milk oligosaccharides (HMOs) shape the intestinal microbiota in term infants. In premature infants, alterations in the intestinal microbiota (dysbiosis) are associated with risk of necrotizing enterocolitis (NEC) and sepsis, and the influence of HMOs on the microbiota is unclear. METHODS:Milk, urine, ...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1038/pr.2015.162
更新日期:2015-12-01 00:00:00
abstract::Clinical trials demonstrated decreasing rates of bronchopulmonary dysplasia in preterm infants with hypoxic respiratory failure treated with inhaled nitric oxide (iNO). However, the molecular and biochemical effects of iNO on developing human fetal lungs remain vastly unknown. By using a well-characterized model of hu...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181d4f20f
更新日期:2010-05-01 00:00:00
abstract::The objective of this study was to determine the plasma leptin concentrations in twin pregnancies in relation to chorionicity and discordant fetal growth. We studied 53 twin pregnancies of which 26 had growth discordance of > or =20% and 27 were concordant for growth (discordance of < or =10%). Paired maternal and fet...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200105000-00010
更新日期:2001-05-01 00:00:00
abstract::Lung infections are a common cause of morbidity and mortality in neonates. To evaluate neonatal lung defenses against pneumococci, we challenged rats with aerosols of encapsulated pneumococci in an airborne infection apparatus. Whereas adult rats cleared greater than 95% of inhaled type 1 or type 25 pneumococci within...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198712000-00025
更新日期:1987-12-01 00:00:00
abstract::In view of the suggested relationship between substrate availability, fetal growth and circulating fetal IGF-I concentrations, we investigated the effect of maternal starvation on plasma IGF-I levels in the late gestation ovine fetus. Ten fetuses aged 125-130 d gestation were sampled daily from indwelling arterial cat...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199004000-00015
更新日期:1990-04-01 00:00:00
abstract::To define respiratory effects of peripheral acidemia during early development, we injected lactic acid (LA) into the jugular vein of 32 1- to 7-d-old rabbit pups and four adult rabbits. The body weight of newborns varied between 67 +/- 17 g in 1-d-old and 119.8 +/- 33.7 g in 7-d-old animals versus 4 +/- 0.1 kg in adul...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199106010-00006
更新日期:1991-06-01 00:00:00
abstract::A child presented with obstructive sleep apnea (OSA) and a near-fatal airway obstruction and respiratory arrest shortly after receiving chloral hydrate (CH). We, therefore, hypothesize that CH might selectively depress upper airway maintaining muscles such as the genioglossus and so predispose to airway obstruction. G...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198406000-00006
更新日期:1984-06-01 00:00:00