Abstract:
:Malignant osteopetrosis is a fatal congenital bone disorder characterized by defective osteoclastic function. Death frequently occurs within the first decade of life. The precise molecular defect(s) that causes osteopetrosis is not known. The possibility that osteoclasts, like macrophages, are controlled by interactions with cytokines suggests that these agents may provide a means of increasing osteoclastic function. Macrophage-colony stimulating factor (M-CSF), a cytokine known to enhance macrophage and osteoclast generation, and recombinant human interferon-gamma (rIFN), a cytokine known to stimulate superoxide generation by white cells, were administered to microphthalmic (mi/mi) mice in an attempt to improve the osteopetrotic condition. Each cytokine was administered separately and in combination to neonatal mi/mi mice for 7 consecutive d. Bone turnover, osteoclast numbers, superoxide generation by white cells, and hematocrit were assessed. rIFN, M-CSF, and a combination of the cytokines stimulates oxygen-derived free radical production by white cells and increased bone resorption. rIFN resulted in a reduction in the number of osteoclasts. This reduction in number was ameliorated by M-CSF. M-CSF alone and in combination with rIFN resulted in improved hematopoietic function, increased weight gain, and increased physical activity of the affected mutants.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Rodriguiz RM,Key LL Jr,Ries WLdoi
10.1203/00006450-199304000-00014subject
Has Abstractpub_date
1993-04-01 00:00:00pages
384-9issue
4 Pt 1eissn
0031-3998issn
1530-0447journal_volume
33pub_type
杂志文章abstract:BACKGROUND:We examined whether increased dairy intake was associated with changes in the levels of bone-related biochemical markers in overweight/obese adolescent girls undergoing a 12-week diet and exercise intervention. METHODS:Thirty-five girls were assigned to a low dairy group (LDa; 0-2 servings/day; n = 16) or a...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-0834-5
更新日期:2020-12-01 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:BACKGROUND:We examined the extent of fetal lung hypoplasia and lung maturation using the amniotic lamellar body count (LBC) in congenital diaphragmatic hernia (CDH). METHODS:We obtained 30 amniotic fluid samples from human CDH cases during cesarean section. We assessed LBC, magnetic resonance imaging (MRI), and ultras...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2012.187
更新日期:2013-03-01 00:00:00
abstract::Isolated TSH deficiency leading to hypothyroidism seems to be a rare condition, escaping the diagnosis by neonatal screening programs, which are based on the primary determination of TSH. This is the first report of a case with an autosomal recessive TSH defect caused by a homozygous mutation of the betaTSH gene that ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199908000-00007
更新日期:1999-08-01 00:00:00
abstract::Ca2+ transients were examined in embryonic chick hearts with an experimentally induced cardiac neural crest-related outflow tract defect known as persistent truncus arteriosus (PTA). In all of the animal models of neural crest-related heart defects, prenatal mortality is too high to be attributed to structural defects...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199712000-00002
更新日期:1997-12-01 00:00:00
abstract::Heart rate turbulence (HRT) is a chronotropic response of the sinus heart rhythm to ventricular premature beat (VPB). Children show decreasing heart rate together with the maturation of the autonomic nervous system. The aim of the research was to assess the relationship between HRT parameters, age, and heart rate and ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181598836
更新日期:2007-12-01 00:00:00
abstract::Circadian rhythms of plasma renin activity (PRA) and plasma aldosterone (PA) were studied in eight healthy children, 7 to 15 years old. Blood samples were obtained at 7:00 AM, 10:00 AM, 1:00 PM, 4:00 PM, 5:00 PM, 8.00 PM, and midnight on day 1 and 4:00 AM and 7:00 AM on day 2. Children had normal activity during this ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198205000-00007
更新日期:1982-05-01 00:00:00
abstract::We used an in vitro assay to study and compare the growth-promotional activity of protein and nonprotein components in human milk (HM) and cow milk (CM) samples for infant strains of Bifidobacterium species. HM samples varied considerably in growth-promotion activity for Bifidobacterium bifidum var pennsylvanicus, Bif...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199102000-00021
更新日期:1991-02-01 00:00:00
abstract::Newborn piglets exposed to acute hypoxia-ischemia (HI) received i.v. cannabidiol (HI + CBD) or vehicle (HI + VEH). In HI + VEH, 72 h post-HI brain activity as assessed by amplitude-integrated EEG (aEEG) had only recovered to 42 ± 9% of baseline, near-infrared spectroscopy (NIRS) parameters remained lower than normal, ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3182276b11
更新日期:2011-09-01 00:00:00
abstract::The previous biochemical evidence had suggested that glutaric aciduria type II (GA II) is due to deficient dehydrogenation of multiple short-chain acyl coenzyme A's (CoA's), bu the precise biochemical mechanism underlying this disease was unknown. We investigated substrate oxidation and in vitro activities of isovaler...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198012000-00013
更新日期:1980-12-01 00:00:00
abstract::Plasma selenium concentration and glutathione peroxidase activity in red blood cells were determined in subjects from different age groups. The selenium level (mean +/- SD) found in infancy (0 to 6 months) was 2 +/- 0.6 micrograms/dl, with the lowest value of 1 microgram/dl observed in a 4-month-old infant. These leve...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198606000-00021
更新日期:1986-06-01 00:00:00
abstract::Several neuropathologic findings in infants and children with human immunodeficiency virus type-1 (HIV-1) infection are different from those observed in adults, probably related to the fact that the retroviral infection occurs in the setting of neuro-development. This report describes the interaction and biologic acti...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199511000-00025
更新日期:1995-11-01 00:00:00
abstract::A dietary-related deficiency of docosahexaenoic acid [C22:6(n-3)] in infant cerebral cortex has been identified. Absence or very low rates of hepatic synthesis from the essential fatty acid precursor, alpha-linolenic acid [C18:3(n-3)], in early life may have been a contributory factor. We have analyzed liver total lip...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199509000-00015
更新日期:1995-09-01 00:00:00
abstract::Recent data suggest that umbilical venous perfusion of the fetal liver has an important influence on fetal growth and postnatal liver function, and that maternal factors in late pregnancy modify this circulation. In a longitudinal study of 160 low-risk pregnancies, we determined how umbilical and portal venous blood f...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/pdr.0b013e318163a1de
更新日期:2008-03-01 00:00:00
abstract::In neuronal cultures from the forebrain of 14-d-old rat embryos, transient hypoxia (95% N2/5% CO2, 37 degrees C) for 6 h has been shown to trigger delayed apoptotic death through sequential changes in protein synthesis, whereas preconditioning by a brief episode of hypoxia can rescue neurons. Because hypothermia has b...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200003000-00017
更新日期:2000-03-01 00:00:00
abstract::Four protein dietary groups of rats were used in this study, namely: 27% casein diet fed throughout gestation and lactation (group C-C); 27% gestation, 10% lactation (group C-D); 10% gestation, 27% lactation (group D-C); 10% throughout gestation and lactation (group D-D). Mean body weight of group D-D at warning was 1...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197503000-00008
更新日期:1975-03-01 00:00:00
abstract:BACKGROUND:Neonates with hypoxic-ischemic encephalopathy (HIE) frequently develop acute kidney injury (AKI). Aminophylline has been shown to reduce severe renal dysfunction in neonates after perinatal asphyxia. However, the effect of aminophylline on renal function in neonates undergoing hypothermia has not been studie...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-0999-y
更新日期:2020-06-05 00:00:00
abstract::A four-year-old Israeli Arab boy was found to have glycogen accumulation in both liver and muscle without clinical symptoms. Liver phosphorylase kinase (PK) activity was 20% of normal, resulting in undetectable activity of phosphorylase a. Muscle PK activity was about 25% of normal, resulting in a marked decrease of p...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198104000-00002
更新日期:1981-04-01 00:00:00
abstract::Exercise-induced arterial hypoxemia (EIAH) is a recognized phenomenon in highly trained adults. Like adult athletes, prepubescent trained children may develop high-level metabolic demand but with a limited lung capacity in comparison with adults. The purpose of this investigation was to search for evidence of EIAH in ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000114481.58902.FB
更新日期:2004-04-01 00:00:00
abstract::We measured the changes in tidal volume, duration of the various phases of the respiratory cycle, and peak nasal pressure during elastic and resistive loading in preterm infants. Values were calculated during the first loaded breath, when chemical drive was unchanged. Tidal volume decreased by equivalent percentages w...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197704000-00002
更新日期:1977-04-01 00:00:00
abstract::Energy and nitrogen balances were measured in growing low birthweight infants fed either mother's expressed breast milk or a 20 kcal per ounce formula to determine whether or not there were differences between the two dietary groups in (1) the partition of energy among excretion, expenditure, and storage and (2) the r...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198311000-00011
更新日期:1983-11-01 00:00:00
abstract::We have recently purified autoantigen polypeptides reacting with celiac disease patient sera IgA from the extracellular noncollagenous matrix compartment of fetal lung tissue. These molecules trigger the production of different tissue antibodies, the so-called antireticulin and antiendomysium antibodies in celiac dise...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199310000-00007
更新日期:1993-10-01 00:00:00
abstract::Previous studies in adults have shown that chronic pulmonary hypertension is associated with decreased endothelial nitric oxide synthase (eNOS) expression in pulmonary arteries. However, the role of decreased eNOS expression in persistent pulmonary hypertension of the newborn (PPHN) is unknown. We investigated the hyp...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199809000-00012
更新日期:1998-09-01 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::The purpose of this investigation was twofold: (1) to identify and characterize the enzymatic ATP hydrolysis system of epithelial cilia, and (2) to develop a quantitative, biochemical test for the ciliotoxic cystic fibrosis (CF) factor based on inhibition of ATP utilization by ciliary preparations. Our rationale for s...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197602000-00012
更新日期:1976-02-01 00:00:00
abstract::To determine the influence of an altered carotid body function on the laryngeal chemoreflex (LCR) response, reflex apnea was induced by laryngeal water stimulation during normoxia or acute hypoxia in unanesthetized awake lambs in which the ventilatory response to acute hypoxia was attenuated by prolonged postnatal hyp...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199312000-00024
更新日期:1993-12-01 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::Five patients with Bartter's syndrome were investigated. Sodium restriction (less than 10 mEq/day for at least 5 days) showed a renal sodium wastage in only two patients (I and II) in spite of increased aldosterone secretion rate (from 151-427 to 680-842 mug/day). The effect of angiotensin II (A II) 80ng/kg/min for 30...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197612000-00005
更新日期:1976-12-01 00:00:00
abstract::This report deals with quantitative and qualitative investigations of alkaline phosphatase in two unrelated infants with the severe infantile form of hypophosphatasia. Both affected infants had no detectable leukocyte alkaline phosphatase activities and both sets of parents and one sibling tended to have low but varia...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197607000-00004
更新日期:1976-07-01 00:00:00
abstract::Pearson's syndrome, a rare and fatal disorder characterized by refractory sideroblastic anemia and pancreatic insufficiency in infancy, is classified into mitochondrial cytopathies. To understand the molecular and genetic bases of this disorder, we have investigated the mitochondrial respiratory chain enzymes and the ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199307000-00024
更新日期:1993-07-01 00:00:00